Société Francophone d'Imagerie Prénatale et Pédiatrique FEPUR Accreditation n° 16-2006-19 ORGANIZING COMMITTEE Jean-Nicolas Dacher, Rouen Patrick Le Dosseur, Rouen Béatrice Olier, Guerbet – France Danièle Eurin, Rouen Cécile Cellier, Rouen Fred Avni, Brussels Richard Medeiros, Rouen Marie Brasseur, Rouen Jacques Thiébot, Rouen Guy Sebag, Paris PROFESSORS By alphabetical order Dr C. Adamsbaum (Paris – France) Dr F. Avni (Brussels – Belgium) Dr H. Brisse (Paris – France) Dr M. Cassart (Brussels – Belgium) Dr JF. Chateil (Bordeaux – France) Dr Ph. Clapuyt (Brussels – Belgium) Dr JN. Dacher (Rouen – France) Dr V. Donoghue (Dublin – Ireland) Dr H. Ducou le Pointe (Paris – France) Dr C. Farnoux (Paris – France) Dr S. Franchi Abella (Le Kremlin Bicêtre – France) Dr C. Garel (Paris – France) Dr C. Owens (London – UK) Dr D. Pariente (Le Kremlin Bicêtre – France) Dr Ph. Petit (Marseilles – France) Dr G. Sebag (Paris – France) Dr A. Smets (Amsterdam – The Netherlands) Dr C. Veyrac (Montpellier – France) THURSDAY OCTOBER 5th 2006 08:00-08:50 Registration 08:50-09:00 Welcome 09:00-09:30 09:30-10:00 10:00-10:30 Intracranial hypertension and hydrocephalus. C. Adamsbaum – M. Zerah (Paris, France) Neonatal Brain Emergencies. C. Garel – C. Farnoux (Paris, France) Bone Infection G. Sebag (Paris, France) 10:30 Coffee break 11:00-11:30 11:30-12:00 Traumatism to the appendicular skeleton H. Ducou le Pointe (Paris, France) Postnatal emergencies (after prenatal diagnosis) M. Cassart (Brussels, Belgium) 12:00-13-30 Lunch break 13:30-16:10 WORKSHOP Room n°1 13:30-14:50 14:50-16:10 Cancer emergencies A. Smets (Amsterdam, The Netherlands) H. Brisse (Paris, France) Multiple trauma Ph. Petit (Marseilles, France) Room n°2 Multiple trauma Ph. Petit (Marseilles, France) Cancer emergencies A. Smets (Amsterdam, The Netherlands) H. Brisse (Paris, France) 16:10 Coffee break 16:40-17:20 Strategies in Emergency Pediatric Radiology (CNS excluded) : US, CT, MR. F. Avni (Brussels, Belgium) 20:30 Gala Dinner FRIDAY OCTOBER 6th 2006 09:00-10:20 WORKSHOP Room n°1 10:20-10:50 Room n°2 Acute lung disease Liver and biliary tract emergencies C. Owens (London, UK) D. Pariente, S. Franchi Abella (Le Kremlin bicêtre, France) Neonatal Gastrointestinal Tract Obtruction V. Donoghue (Dublin, Ireland) 10:50 Coffee break 11:20-12-40 WORKSHOP Room n°1 Liver and biliary tract emergencies Acute lung disease D. Pariente, S. Franchi Abella (Le Kremlin bicêtre, France) C. Owens (London, UK) 12:40-14:00 Lunch break 14:00-14:30 Abdominal emergencies, intra-peritoneal JF. Chateil (Bordeaux, France) 14:30-:15:00 Urinary tract infection C. Veyrac (Montpellier, France) 15:00-15:30 Room n°2 Liver transplant emergencies Ph. Clapuyt (Brussels, Belgium) Intracranial hypertension and hydrocephalus “Take - home messages” C. Adamsbaum*, M. Zerah** * Université Paris Descartes, Faculté de Médecine, AP-HP, Service de Radiologie, Hôpital St Vincent de Paul, Paris ** Service de Neurochirurgie, Hôpital Necker Enfant Malades, Paris 1- The 3 main components included in the closed cranial box are cerebro spinal fluid (CSF), vascular component and parenchyma. A hydrocephalus corresponds to an active expansion of the CSF component at the expense of the 2 others. 2- The CSF flow includes two components : a bulk flow and a pulsatile flow The bulk flow (around 600cc/24 hours) is produced by the choroid plexuses and mainly resorbed by the arachnoid granulations. The pulsatile flow is related to the cardiac contractions (around 15cc through each systole) and counterbalanced with the elastic resistance of the brain. Hydrocephalus can be due either to an abnormality of the global CSF flow (block) or of the pulsatile flow (cerebral dysmorphy) or a hypersecretion of CSF 3- As in neonates and infants a hydrocephalus is responsible for a macrocrania, the measurement of the cranial perimeter is a diagnostic clue. 4- The concept of « external hydrocephalus » should be replaced by “benign dilation of the subarachnoid spaces”. This entity is related to the immaturity of the granulations in neonates and young infants in whom the resorption of CSF is mainly venous. None neurologic sign should be observed in the typical pattern, which will spontaneously regress in the first year of life. 5- The aims of imaging (MRI) are to identify a hydrocephalus but also to evaluate the parenchyma, to look for an etiology and for the follow up. Hydrocephalus can be uni, bi, tri or tetraventricular. It is important to highlight that - a triventricular dilation is not necessary a stenosis of the aqueduct - the spinal canal must be studied if none intracranial cause is recognized 6- MRI can evaluate the consequences of the hydrocephalus : - acute effects can be a periventricular hyperhydratation, an edema, a mass effect, anoxia and/or ischemia - chronic effects can include a demyelination, gliosis and calcifications, outpoutching or ventricular walls abnormalities 7- The frequency of etiologies of hydrocephalus depends on the age of occurrence : - mainly post hemorrhagic or post infectious in neonates - mostly related to a tumor in childhood Bibliographie • • • • Barkovich AJ. Pediatric Neuroimaging. Lippincott Williams & Wilkins, Philadelphia, 4è Ed., 2005, 932p Bradley WG, Safar FG, Furtado C et al. Increased intracranial volume: a clue to the etiology of idiopathic normal pressure hydrocephalus ? AJNR, 2004;25:1479-1484. Cinalli C, Maixner WJ, Sainte-Rose C. Pediatric Hydrocephalus. Springer, 2004 Heidelberg, Choux M, Di Rocco C, Hockley A, Walker M. Pediatric neurosurgery. Churchill Livingstone, London, 1999 NEONATAL BRAIN EMERGENCIES Dr C Garel, Department of Paediatric Imaging -Dr C Farnoux, Department of Neonatology Hôpital Robert Debré, Paris, France According to the clinical findings and medical history, true neonatal brain emergencies can be differentiated from “delayed” emergencies. In true emergencies, cerebral imaging may have direct immediate consequences on the therapeutic management. In delayed emergencies, imaging must be performed rapidly because of parental anxiety but it will usually not influence immediately the child’s management. In neonates, brain damage may show through with very subtle clinical findings. Neonates may be scanned with different imaging techniques. Cranial ultrasound (US) has the advantage of being mobile and easily used on the neonatal unit. It is a good method for screening and monitoring the evolution of lesions. Computed Tomography (CT) is good at identifying acute hemorrhage but lacks sensitivity, notably regarding white matter lesions. Magnetic Resonance Imaging (MRI) has a higher sensitivity and is much better at determining the exact site and extent of lesions. True neonatal brain emergencies Some medical histories or clinical findings observed in a neonate are highly suggestive of brain damage : - hypoxic-ischemic encephalopathy in term neonates with fetal distress prior to delivery, abnormal Apgar scores and resuscitation at birth, - seizures with or without fever, - infectious context with neurological findings, - marked hypotonia, - clinical findings suggesting intracranial hypertension : increasing head circumference, vomiting, lethargy, sutural dysjunction, bulging anterior fontanel, - apnea, - transfontanellar murmur, - history of birth trauma, fall or accidental injury, - marked thrombopenia or thrombophilic disorders, - marked prematurity (under 30 weeks) within the first 48 hours or prematurity (under 32 weeks) with clinical instability. In hypoxic-ischemic encephalopathy in term neonates, imaging plays a key role and may predict the outcome. Some lesions may be difficult to detect using US and CT and evaluation with MRI is therefore optimal. An abnormal signal intensity within the posterior limb of the internal capsule is a good early predictor of abnormal outcome. It may be isolated but is usually associated with abnormalities in the basal ganglia and thalami and sometimes in the brain stem in severe acute injury. Brain swelling may appear during the first 24-48 hours following an asphyxial episode and it may be associated with white matter and cortical lesions (cortical highlighting around central and interhemispheric fissures and around the insula). The loss of gray/white matter differentiation comes as a result of cytotoxic edema due to severe ischemia. Early diffusion- weighted imaging helps detecting these lesions (1). Stroke is more common in neonates than in older children. Some neonates with stroke secondary to perinatal asphyxia are seriously ill but, in most cases, systemic signs of stroke are nonspecific and subtle and may be overlooked. They include hypotonia, lethargy or apnea. Seizures are noted in 25-40%. Risk factors include maternal and placental disorders, blood and cardiac disorders, perinatal asphyxia, infection and trauma. Most perinatal strokes occur in the territory of the middle cerebral artery with a predominance of left-hemisphere lesions. Cranial US is less sensitive than CT or MRI in the detection of such lesions. Cerebral infarction appears as an ill-defined region of hyperechogenicity with obliteration of the normal gyral pattern. Large infarctions may show a mass effect. CT shows a well-defined region of hypodensity affecting both the cortex and the underlying white matter in an arterial distribution. MRI is the best modality to identify infarctions in the hyperacute phase through the use of diffusion-weighted imaging. The acute infarct is hyperintense on diffusion-weighted images.Pseudonormalization takes place between 7 and 8 days after injury (2-6). Accidental and non accidental injury may be responsible for a wide spectrum of clinical signs: the mildest ones are non-specific (poor feeding, vomiting, lethargy and irritability) so that injury may be overlooked and the most severe ones are seizures and unconsciousness. Shaken baby syndrome is the most common cause of death or serious neurological injury resulting from child abuse. The younger the infant the greater the risk of injury. Sudural hematoma is a hallmark of non-accidental shaking head injury. It is usually bilateral and is visible typically in the interhemispheric fissure. Posterior fossa subdural hemorrhage is uncommon. In this location, it must be differentiated from clinically silent subdural hematomas arising in the newborn at term. Those hematomas are usually located in the posterior fossa and MRI follow-up shows complete resolution at age 4 weeks (7). CT is regarded as the most appropriate modality for detection of acute hemorrhage. However, MRI with Flair sequence has also been reported to be particularly useful for detecting very recent hemorrhage. MRI is better at delineating intraparenchymal damage, not shown or underestimated on early performed CT scan. Diffusion-weighted imaging makes it possible to detect cortical gray matter contusion and white matter tears in the subcortical brain tissue. MR findings of swelling and edema may also be observed (8-10). Birth traumas may be responsible for skull fractures. Such fractures have very little prognostic value concerning associated neurological damage. Epidureal hematoma is rare and is usually associated with a large cephalohematoma and a subjacent skull fracture. Neonates typically present with bulging anterior fontanel. In term neonates, intraventricular hemorrhage result most from thalamic or choroid plexus hemorrhage caused by deep venous thrombosis (6). Spontaneous superficial parenchymal and leptomeningeal hemorrhage have been reported in the temporal lobes in otherwise healthy term neonates (11). In premature neonates, intraventricular hemorrhage is well demonstrated by cranial US. It may be associated with ventricular dilatation resulting from blockage of the CSF pathways by hemorrhage. Ventriculoperitoneal CSF diversion may be necessary. These patients have an increased incidence of periventricular white matter injury (6,12). In neonates with meningitis, nonspecific signs of sepsis may be present. Stupor, with or without irritability is the most common neurological sign. Seizures are observed in up to 40% of affected neonates and hemiparesis may be present. Complications of bacterial meningitis are extremely common at that age; clinical and imaging follow-up is essential. These complications include cerebritis, infarction, brain abscess, empyema, sinus thrombosis and hydrocephalus. Cerebritis is the earliest stage of the abscess formation process. In case of brain abscesses, neonates usually present with symptoms of increased intracranial pressure. In neonates, brain abscesses are distinguished by three features: they are of relatively large size, capsule formation is relatively poor and they typically originate in the periventricular white matter. On cranial US, they are hypoechoic with a hyperechoic rim. This thin wall has an increased density on CT and enhances after contrast administration. On MRI, the abscess wall is T1 hyperintense and slightly T2 hypointense. Abscesses show reduced diffusion. Cerebral infarcts are related to vasculitis, thrombosis and arachnoiditis. They most often appear 1-6 days after infection. Focal cerebral infartcs with necrosis might mimic an abscess and possibility of abscess formation due to secondary infection of the infarcts should be considered. MRI is more sensitive than CT to inflammatory vasculitis. Small deep white-matter infarcts may lead to subtle tissue damage and be overlooked by routine MRI. Diffusion-weighted imaging can be used to detect small infarcts (13-16). A tranfontanellar murmur may reveal a congenital vascular malformation. Three lesions may be diagnosed in utero: the vein of Galen aneurysmal malformation, dural sinus malformations and pial arteriovenous fistulas. Brain damage usually arises from ischemia secondary to venous congestion or thrombosis. In pial arteriovenous fistulas, cerebral tissue injury can develop rapidly and shunting through the fistula must be reduced as soon as possible to avoid loss of brain substance (17-18). Delayed neonatal brain emergencies In two types of circumstances, due to parental anxiety, cerebral imaging has to be performed rapidly after birth, even, if in most cases, imaging findings will not have immediate therapeutic implications: - When prenatal imaging has diagnosed brain abnormalities and when termination of pregnancy has not been considered justified by the medical staff or has been rejected by the parents. All kinds of malformations or in utero acquired brain abnormalities may be concerned: septal agenesis, partial callosal agenesis, congenital infection with or without brain involvement…. - When a facial malformation or a syndrome with possible brain involvement are discovered at birth and have not been diagnosed during the prenatal period: neurocutaneous syndrome, meningocele, panhypopituitarism… Conclusion The clinical signs of neonatal brain emergencies are often poor and non specific. Therefore, imaging has a key role to play; it may detect cerebral damage with direct therapeutic implications. Conversely, it may eliminate a brain involvement and thus reassure the medical staff and the parents. Some imaging findings may predict the outcome. [1 ]Rutherford, M., MRI of the neonatal brain. 2002, London: WB Saunders. [2 ]Marret, S., Lardennois, C., Mercier, A., Radi, S., Michel, C., Vanhulle, C., Charollais, A., and Gressens, P. Fetal and neonatal cerebral infarcts. Biol Neonate 2001; 79: 236-240. [3 ]McKinstry, R.C., Miller, J.H., Snyder, A.Z., Mathur, A., Schefft, G.L., Almli, C.R., Shimony, J.S., Shiran, S.I., and Neil, J.J. A prospective, longitudinal diffusion tensor imaging study of brain injury in newborns. Neurology 2002; 59: 824-833. [4 ]Miller, V. Neonatal cerebral infarction. Seminars in Pediatric Neurology 2000; 7: 278-288. [5 ]Nelson, K.B. and Lynch, J.K. Stroke in newborn infants. Lancet Neurol 2004; 3: 150-158. [6 ]Barkovich, J., Brain and spine injuries in infancy and childhood, in Pediatric neuroimaging, B. JA, Editor. 2005, Lippincott Williams § Wilkins: Philadelphia. p. 190-290. [7 ]Whitby, E.H., Griffiths, P.D., Rutter, S., Smith, M.F., Sprigg, A., Ohadike, P., Davies, N.P., Rigby, A.S., and Paley, M.N. Frequency and natural history of subdural haemorrhages in babies and relation to obstetric factors. Lancet 2004; 363: 846-851. [8 ]Phillips, M.M., Non-accidental head injury in the young infant, in MRI of the neonatal brain, R. M, Editor. 2002, W B Saunders: London. p. 261-269. [9 ]Blumenthal, I. Shaken baby syndrome. Postgrad Med J 2002; 78: 732-735. [10 ]Parizel, P.M., Ceulemans, B., Laridon, A., Ozsarlak, O., Van Goethem, J.W., and Jorens, P.G. Cortical hypoxic-ischemic brain damage in shaken-baby (shaken impact) syndrome: value of diffusion-weighted MRI. Pediatr Radiol 2003; 33: 868-871. [11 ]Huang, A.H. and Robertson, R.L. Spontaneous superficial parenchymal and leptomeningeal hemorrhage in term neonates. AJNR Am J Neuroradiol 2004; 25: 469-475. [12 ]A Couture, C.V., Transfontanellar doppler imaging in neonates. 2001, Berlin: Springer. [13 ]S Blaser, V.J., LE Becker, EL Ford-Jones, Neonatal brain infection, in MRI of the neonatal brain, M. Rutherford, Editor. 2002, WB Saunders: London. p. 201-223. [14 ]Barkovich, J., Infections of the nervous system, in Pediatric neuroimaging, J. Barkovich, Editor. 2005, Lippincott Williams § Wilkins: Philadelphia. p. 801-868. [15 ]Jan, W., Zimmerman, R.A., Bilaniuk, L.T., Hunter, J.V., Simon, E.M., and Haselgrove, J. Diffusion-weighted imaging in acute bacterial meningitis in infancy. Neuroradiology 2003; 45: 634-639. [16 ]JA Ressler, M.N. Central nervous system infections in the pediatric population. Neuroimaging Clin N Am 2000; 10: 427-443. [17 ]Lasjaunias, P., Vascular disease in neonates, infants and children. Interventional neuroradiology management. 1997, Berlin: Springer. [18 ]Garel, C., Azarian, M., Lasjaunias, P., and Luton, D. Pial arteriovenous fistulas: dilemmas in prenatal diagnosis, counseling and postnatal treatment. Report of three cases. Ultrasound Obstet Gynecol 2005; 26: 293-296. TRAUMATISM TO THE APPENDICULAR SKELETON H. DUCOU LE POINTE – Hôpital d'Enfants Armand Trousseau - Paris TRAUMATISM TO THE APPENDICULAR SKELETON H. DUCOU LE POINTE Hôpital d ’Enfants Armand-Trousseau, Paris Objectives • Review how pediatric fractures are different of adult fractures in terms of anatomy, mechanism and physiology. • Review how to explore patients • Review specificities of : shaft and epiphyseal fractures • A focus point on elbow fracture • Few words about stress fracture and battered child syndrom 4M 4D Articular cartilage Epiphysis Metaphysis Medullary Cavity Diaphysis Cortex Periosteum Growth plate 9Y Epiphysis Resting chondrocytes Proliferating chondrocytes Prehypertrophic chondrocytes Hypertrophic chondrocytes Osteoblast/Osteoclast Bone trabeculae Metaphysis <10 Y > 10Y TRAUMATISM • First reason to consult in emergency. • Bone X-rays – 25% of emergency department patients – 65% of x-rays done for the emergency department • Diagnosis could be difficult. • Battered Child Syndrome HOW TO EXPLORE ? • • • • Conventional imaging Ultrasonography Computed tomography MRI HOW TO EXPLORE ? Conventional imaging : AP, lateral view CONVENTIONAL IMAGING WHICH VIEW? Oblique views – Distal humerus – Torus fracture – Complexe fracture COMPARATIVE VIEWS ? • Few indications • Th. E. Keats. Atlas of normal roentgen variants that may simulate disease (mosby) • Bowing (plastic) fracture • Secondary ossification center ULTRASONOGRAPHY • Few indications – X-ray efficacy and few irradiation. – Need medical knowledge – Newborn and young child WHEN TO PERFORM A CT? • • • • Complexe fracture Number of fragments Articular aligment +/- 2 mm Appreciation of orthopedic treatment WHEN TO PERFORM A CT? Complex fracture WHEN TO PERFORM A CT? To verify articular alignement Tillaux Fracture WHEN TO PERFORM A CT? Quality of orthopedic treatment WHEN TO PERFORM A MR STUDY ? • Rarely in emergency • Osteochondral fracture • To vizualise ligaments, cartilage or fibrocartilage. • Complications of fracture : epiphysiodesis MRI Osteochondral fracture MRI Meniscal injuries Cruciate ligament lesion MRI Epiphysiodesis WHICH TYPE OF LESIONS : • Shaft fractures. • Epiphyseal-metaphyseal fractures. • Secondary ossification center avulsion. • Dislocation and ligamentous injuries are uncommon. SHAFT FRACTURES Good prognosis: Thick periosteum Some pediatric specificities SHAFT FRACTURE Torus fracture Plastic fracture Hairline fracture Greenstick fracture TORUS FRACTURE • Compression fracture • Metaphyseal regions (cortex is weak) • Buckling of the cortex • Very good prognosis Bowing fracture • • • • Diagnosis could be difficult Begnin Radial and ulna Search for another fracture HAIRLINE FRACTURE • • • • Spiral fracture Tolder’s fracture Tibial location Diagnosis could be difficult PROGNOSIS OF SHAFT FRACTURE • Good prognosis : Growing bones have the ability to remodel angular formities. • Be aware: Malunion in rotation don’t improve spontaneously EPIPHYSEAL-METAPHYSEAL FRACTURES • Wide variety of injuries • Salter-Harrris classification SALTER-HARRRIS CLASSIFICATION I, II, III, IV, V Who is who ? SALTER I AND II : GOOD PROGNOSIS SALTER I AND II : GOOD PROGNOSIS SALTER I AND II : GOOD PROGNOSIS SALTER III SALTER IV Triplane fracture SALTER V EPIPHYSIODESIS Growth disturbance Complete arrest Partial arrest (progressive angulation) EPIPHYSIODESIS ELBOW FRACTURE Supracondylar 60 % Lateral epicondyle 20 % Medial epicondyle 10 % Radial head 7% Dislocation frequently associated with a fracture 3 % Ossification center - CRITOE 12 Y 8Y6m 12 Y Capitellum (3-6 months) Radial head (3-6 years) Internal (medial) epicondyle (4-7 years) Trochlea (9-10 years) Olecranon (6-10 years) External epicondyle (9-13 yeas) Radiographic lines 1 2 1 Anterior humeral line 2 Radiocapitellar line 3 Anterior fat pad Radiographic lines Radiocapitellar line 3 Ruben, 5 y 4 m LATERAL EPICONDYLAR FRACTURE • The second most frequent fracture of pediatric elbow. • It is usually a Salter IV fracture • Small fragment of distal humeral metaphysis and unossified epiphysis. • MRI or arthrography helps to define the extension into the unossified epiphysis • Open fixation is required in case of extension into the joint. Non union MEDIAL EPICONDYLE AVULSION • Simple separation to complete dislocation • Entrapment of the medial epicondyle is possible especially when avulsion accopanies true elbow dislocation Monique, 14 y SUPRACONDYLAR FRACTURE • Most common fracture of the pediatric elbow • Extension type with posterior displacement of distal fragment is the most frequent type (95 %) • Up to 15 % of pediatric supracondylar humerus fractures have nerve injury Mohand, 9 y, falls of a tree on an out-streched arm in full extension David,12 y, fall in hyperflexion ELBOW DISLOCATION • Unfrequent • the most frequent site of dislocation • Posterior dislocation (1/3) or posterolateral (2/3) • Associated frequently with medial epicondylar avulsion Solène, neonatal loss of elbow function Jules, 7 y D G G STRESS FRACTURE • Occurs frequently in the upper tibia • Difficult to diagnose at the early phase • Typical sclerosis and periosteal new bone formation allow the diagnosis • MRI could demonstrate the fracture at an early phase STRESS FRACTURE Boy 15 Y STRESS FRACTURE Battered Child Syndrome BATTERED CHILD SYNDROME Conclusion Don’t forget clinical examination Postnatal emergencies after prenatal diagnosis Marie Cassart – ULB Erasme Hospital- Brussels- Belgium Recent development in antenatal imaging modalities (3D-US, fetal MRI, fetal skeletal survey…) significantly improved antenatal diagnoses (1). The medical staff (obstetricians, neonatalogists …) and the family are actually informed before birth about the anomaly. Consequently, delivery may be planned in a tertiary center and the immediate neonatal cares can be organized according to the anomaly. The fetuses carrying malformations are therefore better managed in the neonatal period. The neonatal emergencies are numerous and of various origins, they can affect different systems. In our experience, the most frequent neonatal emergencies are of gastro-intestinal origin due to the associated risk of occlusion and perforation. But the neonatal emergencies may also be due to acute respiratory distress in cases of airway compressions (cervical masses) or to urinary tract obstruction (uretral valves) or major hydrocephaly…. The topic is wide, therefore, in the presentation, we will discuss the most frequent neonatal emergencies encountered in daily clinical practice. Thoraco-abdominal emergencies : - The digestive tract is frequently affected ( 9% of all fetal anomalies). It includes atresia (2), volvulus (3), digestive duplication (4), malrotation (5,6), perforation… Those conditions may represent emergencies due to the associated risk of extensive bowel ischemic damages (atresia, volvulus, malrotation) and possible secondary perforation and peritonitis. In cases of duplication cysts , surgery is requested to prevent intussusception, torsion, bleeding or malignant changes. In cases with anal atresia (which can only be suspected antenatally due to associated malformations), the surgical emergency consists in eliminating the meconial content of the colon, but also to treat frequently associated fistulas which can be responsible of urinary tract infections. - The abdominal walls can present defects that represent acute neonatal emergencies (diaphragmatic hernias (7,8), gastroschisis, omphalocele…) which should be promptly repaired because of the associated risk of respiratory distress or pulmonary hypertension (diaphragmatic hernia) or sepsis and fluid loss in neonates with gastroschisis. - The fetal masses (lymphangiomas, teratomas…) can encase the vascular structures, compress the airways (9) or induce digestive occlusion according to their nature, size and location. In the cases of neck masses compressing the airways, the obstetrician apply the EXIT procedure (which consists in intubation of the fetus before umbilical cord section). The solid masses can also be responsible of neonatal cardiac deficiency due to massive blood steal. Neonatal surgery is therefore advocated. - The genito-urinary tract can be severely dilated secondary to obstuction (uretral valves, uretral prolapse of an ureterocele, vesicu-ureteral junction obstruction…) or reflux (10). Those conditions must be rapidly managed and the newborn treated by prophylactic antibiotherapy to prevent upper urinary tract infection and secondary renal scarring. In cases of mecanical obstruction, a surgical intervention is needed to facilitate voiding and prevent bladder distention. The neurological emergencies The antenatal discovery of neurological malformations are often associated with poor prognosis and most often lead to medical interruption of the pregnancy. Some mild anomalies may lead to progressive neurologic developmental retardation which are not neonatal emergencies. The most common anomalies which need immediate neonatal care are the fetuses at risk of hydrocephalus and intracranial hypertension (CNS hemorrhages) (11) or fetuses at risk of infection (open dysraphysms). Skeletal dysplasia - Skeletal dysplasia are rare entities but some of them can be lethal. The precise antenatal diagnosis is often difficult to establish. Actually, 3D-CT is a good complementary imaging modality that can help in establishing the diagnosis and therefore the prognosis of the affection during fetal life (12). In cases of lethal dysplasia, the family may request the natural death of the newborn. The main cause of death is respiratory insufficiency due to thoracic hypoplasia. The emergency in those cases is to accompany the newborn to death assisting the family. Here again, the antenatal diagnosis is of great importance, it allows the parents to be prepared to this hard trial. Neonatal emergencies are numerous but the most frequent are of gastrointestinal origin. The antenatal diagnosis is of great help to prepare the family and the medical staff in the rapid and adequate management of these newborns. All those entities will be developed in the oral presentation associating the antenatal diagnosis with the post natal work-up and treatment. References 1. Breysem L, Bosmans H, Dymarkowski S, et al. The value of fast MR imaging as an adjunct to US in prenatal diagnosis. Eur Radiol 2003;13:1538-1548. 2. Langer J, Hussain H, Khan A et al. Prenatal diagnosis of esophageal atresia using sonography and magnetic resonance imaging. J Ped Surg 36:804-807. 3. Ogunyemi D. Prenatal ultrasonographic diagnosis of ileal atresia and volvulus in a twin pregnancy. J Ultrasound Med 2000,19:723-726. 4. Foley N, Sithasanan N, Mc Ewing R et al. Enteric duplication cyst presenting as antenatally detected abdominal cysts: is delayed resection appropriate? J Ped Surg 2003,38:18101813. 5. Rapfer S, Rappold J. Intestinal malrotation - not just a pediatric surgeon’s problem. J Am Coll Surg 2004;199(4):628-635. 6. Cassart M, Massez A, Lingier P et al. Sonographic prenatal diagnosis of malpositioned stomach as a feature of uncomplicated intestinal malrotation. Pediatr Radiol 2006;36 (4): 358-360. 7. Gorincourt G, Bouvenot J, Mourot MG et al. Prenatal prognosis of congenital diaphragmatic hernia using magnetic resonance imaging measurement of fetal lung volume. Ultrasound Obstet Gynecol 2005; 26:738-744. 8. Laudy J, Van Gucht M, Van Dooren MF et al. Congenital diaphragmatic hernia: an evaluation of the lung-to-head ratio and other prenatal parameters. Prenat Diagn 2003;23:634-639. 9. Kathary N, Bulas D, Newman K et al. MR imaging of fetal neck masses with airway compromise: utility in delivery planning. Pediatr Radiol 2001;31:727-731. 10. Cassart M, Massez A, Metens T et al. Complementary role of MRI after sonography in assessing bilateral urinary tract anomalies in the fetus. AJR 2004;182:689-695. 11. de Laveaucoupet J, Audibert F, Guis F et al. Fetal magnetic resonance imaging (MRI) of ischemic brain injury. Prenat Diagn 2001;21:729-736. 12. Ruano R, Mohlo M, Roume J et al. Prenatal diagnosis of fetal skeletal dysplasias by combining two-dimensional and three dimensional ultrasound and intrauterine threedimensional helical computer tomography. Ultrasound Obstet Gynecol 2004; 24:134-140. CANCER EMERGENCIES IN CHILDREN A.M.J.B. SMETS AMC - University of Amsterdam Amsterdam, The Netherlands [email protected] H. BRISSE Institut Curie Paris, France [email protected] Most common emergency situations and related tumors in paediatric oncology (CNS primary tumors not included) Anatomic location Brain and cranial nerves Symptoms Related tumors Comment References Optic nerve compression Haematologic malignancies Stage-4 Neuroblastoma Rhabdomyosarcoma Burkitt’s lymphoma UCNT Extensive retinoblastoma Haematologic malignancies Skull base invasion (1, 2) Intracranial invasion Intracranial haemorrhage And ischemia CNS infections Paraneoplastic neurologic syndromes Haematologic malignancies Neuroblastoma Hodgkin’s disease Brain metastasis Spine Spinal cord compression Germ cell tumors sarcoma Rhaboid tumor / ATRT neuroblastoma Neuroblastoma Ewing sarcoma Haematologic malignancies Germ cell tumors Chordoma (3) (4) rare Acute myeloblastic leukemia (Hyperleukocytosis, Thrombocytopenia) Especially if BMT Opsoclonusmyoclonus-ataxia (Kinsbourne syndrome) Encephalomyelitis Cerebellar and limbic encephalitis Myasthenia Limbic encephalitis rare (5) (6) (7) (7) (8, 9) (10) (11, 12) (13) (14) (15) (16, 17) (7, 18-21) (20) (22, 23) Sacro-coccygeal rare (24) Anatomic location Head and Neck Symptoms Related tumors Comment References Malignant exophtalmia Neuroblastoma Orbital Rhabdomyosarcoma Haematologic malignancies (chloroma) Retinoblastoma Lymphoma Haemangioma Cervical Neuroblastoma Cystic lymphangioma Neuroblastoma Nasal angiofibroma Lymphoma pPNET / Ewing sarcoma Thymic hyperplasia Germ cell tumor Cystic lymphangioma Neuroblastoma Lymphoma Lymphoma Germ cell tumors Stage 4 (19) (25, 26) Upper airway compression Chest Musculoskeletal Horner syndrome Epistaxis airway compression SVC compression Pleuro-pericardial compressive effusion Pneumothorax Intra-cardiac thrombosis Pathologic fracture Osteonecrosis Lung metastasis Wilms tumor Osteosarcoma Osteosarcoma Ewing sarcoma Bone metastasis Haematologic malignancies (27) Extensive cases (6) Laryngeal (28) (29) Stellate ganglion Male adolescent (29, 30) (31-33) (34, 35) Klinefelter syndrome (36, 37) (24) (38) (39-41) Osteosarcoma rare (42) (43-46) (47) (48) Neuroblastoma (49, 50) Anatomic location Abdomen Hypertrophic osteoarthropathy UCNT Paraneoplastic (lung metastases) (51-53) Symptoms Related tumors Comment References Tumor rupture Renal tumors Wilms tumor Large renal angiomyolipoma Germ cell tumors Juvenile granulosa cell tumor Urachal RMS (54-57) (58) Ovarian neoplasms Bowel obstruction Pelvis Severe haematuria Paraneoplastic syndrome Ovarian torsion Acute urine retention “Medical” emergenci es Acute renal failure Hypertension Hepatic failure Intra-vascular coagulation Hypercalcemia Inappropriate ADH secretion Abdominal RMS Burkitt’s lymphoma Pelvic tumors Wilms Neuroblastoma Germ cell tumors Ovarian cysts Pelvic RMS Neuroblastoma Germ cell tumors Haematologic malignancies Retroperitoneal or pelvic tumor Neuroblastoma Nephroblastoma Phaeochromocytoma Adrenocortical tumors Stage-4S neuroblastoma (Pepper sd) Liver multiple Haemangioendotheli omas Haematologic malignancies Neuroblastoma Congenital mesoblastic nephroma Haemangiomas (Kaposiforme type) Haematologic malignancies Neuroblastoma Rhabdoid tumor Bolande Brain tumors Lymphoma (59-61) (62) (63) Diarrhoea - VIP (64) (65) (66) (67, 68) (69) If urinary tract obstruction Renovascular HT (70, 71) (72-74) (75) (76) AML Promyelocytic Stage 4 (77, 78) Kasabach-Merritt syndrome (79) Bone metastases paraneoplastic paraneoplastic (80, 81) Symptoms Acute therapyinduced complicati ons Chemotherapy Related drugs and tumors Hematologgic malignancies Alkylating Agents (Busulfan) L-Asparaginase Actinomycine D Cyclosporine A Radiotherapy Vincristine Ifosmamide Methotrexate Cytarabine Abdominal tumors (mainly right-side Wilms tumor) Chest tumors Comment References Tumor lysis syndrome Liver veno-occlusive disease Portal vein thrombosis Thombosis (venous sinus) Liver veno-occlusive disease Hypertensive encephalopathy Bowel obstruction Acute encephalopathy (82) (83-86) (87, 88) (83, 84) (7) (89, 90) Liver veno-occlusive disease Alveolitis (91) References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 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Multiple Trauma - Workshop P Petit, M Panuel Centre Hospitalo-Universitaire Timone et Nord – Marseille- France Multiple trauma correspond to potentially life threatening injuries of at least two distinct anatomical sites. Multiple clinical scores, of still discussed relevance, are used to evaluate the clinical status of a traumatized child (Injury Severity Score, Paediatric Trauma Score, Revised Trauma Score, Paediatric Triage Tape, Careflight, Children Coma Score, Modified Glasgow Coma Scale,…). However, to our knowledge, none of them are correlated to a clear imaging strategy. Furthermore, the therapeutic management has changed over years and the role of imaging in non surgical conservative treatment has increased. The goal of this workshop will be : To discuss this strategy considering the suspected mechanism of trauma, the - child’s age and the clinical status, the available imaging equipment and the experience of the medical team in charge of the patient. To present the imaging features of the most frequent lesions encountered in chest, - abdomen, pelvis and spine. To describe the different tricks to limit the diagnostic traps - Since parts of this subject will be also addressed in others teaching courses, the authors pleased the lecturers to referred to specific texts for additional informations. Traffic accident, the leading cause, fall from height and child abused are responsible for the majority of polytraumatisms. Peaks of frequency are reported in toddlers and adolescents. Penetrating trauma is extensively rare in Europe. Traffic injury is also the first cause of morbidity and mortality in this age group. Blunt trauma to the head and chest are the two most frequent causes of death. (1). Head (80%) and peripheral skeleton (76%) are the most frequent sites involved (2). Short patient outcome is preferentially related to the severity of head trauma and long term impairment to both head and skeletal lesions. Chest, abdomen, pelvis and axial skeleton are interested in this decreasing order. The death rate for trauma in children is higher than that of adults with similar injuries. Paediatric particularities : - anatomy : - Probably due to its plasticity child’s brain has an ability to recover faster than adult’s brain. - Spinal cord are frequently injured without associated bone lesion. Physiological ligamentous and soft-tissue hyperlaxity, unfused epiphyses, more horizontal position of the facet joints and a disproportionate weight of the head compared to the underneath spine explain the frequency of spinal cord injury without radiological abnormality (SCIWORA). - This mechanism of lesions is also encountered in the lung parenchyma without rib fracture since child chest wall is of more compliant nature than in adults. The lowest ribs still cartilaginous are less traumatic than their ossified adult counterparts and are not responsible for spleen or liver lacerations. However, child’s kidneys are less protected by a poor volume of perirenal fat. They are proportionally larger than in adults and the persistence of fetal lobulations make them more prone to parenchymatous rupture. These anatomical factors explain that pediatric kidney trauma are more severe than the adults one (3). Deceleration abdominal lesions are less common since the kinetic energy is lower than in adult because of a reduced abdominal volume. - clinical exam : - Set apart from comatous patients, no valuable verbal informations can be obtained in children less than 5 years of age. This emergency exam is considered poorly reliable by some authors who advocate the need for liberal Multi Slice Computed Tomography (MSCT) explorations; however, such imaging strategy has not proved to be useful in mild to moderate pediatric trauma (4). - Hemodynamic instability can decompensate more abruptly than in adult and the need for patient’s transport especially to the imaging department must carefully be assessed compare to its attended benefits (5). - imaging : - Until closure of the anterior fontanelle (around 18 months), brain and its envelops can be explored by transfontanellar ultrasonography. However, this technique is not applicable in case of polytrauma; CT without IV injection is the modality of choice. - Rarely the use of sonography in the evaluation of spinal trauma has been reported, mostly during obstetrical trauma (6). It cannot be recommended in such situation. MRI will be the exam of choice in case neurologic symptoms or high suspicion of cord lesion because of the mechanism of trauma. - Radiation dose must be a constant concern especially when a whole body MSCT is proposed but will reasonably be discussed when compared to this clearly life threatening pathology. A large proportion of CT are normal in case of polytraumatism : head (62% (7)), abdomen (59 to 87%) (8,9) or will not significantly modify the therapeutic management (4). - Sedation for MR or CT imaging has to be realised in painful, agitated or less than five year old child. Needs for these exams have to be perfectly weighted compared to the risk of sedation on these fragile patients. Rare interventional radiology procedures will also be done under sedation. Imaging strategy : The imaging strategy will be decided by a multidisciplinary team (emergency and intensive care unit physicians, surgeon, radiologist), and will be directly related to mechanism of trauma (kinetic energy, seat belt related injuries), child’s age and clinical status. The goal of imaging will be to provide a fast and reliable diagnosis with the lowest invasiveness and radiation burden. Anatomical sites involved in the traumatism will also guide the indications for imaging. Whatever the scientific evidences published, non quantifiable parameters including stress and experience of the medical team involved in a polytraumatized child and fear to miss a significant injury may influence this imaging strategy. Head : CT without IV injection is the examination of choice If this exam is clearly indicated in severe head trauma with a Glasgow score less than 8, controversies still exist concerning the clinical features that can predict the risk of intracranial injury and then the need for imaging in minor trauma. Absence of : short loss of consciousness, amnesia, prolonged headache, drowsiness, clinical evidence of basal or non frontal skull fractures would identified 100% of children without brain lesions (7). Some authors have recommended an associated abdominal CT when Glasgow score is less than 10 since in their experience associated abdominal lesions are up to 25% (10). This percentage was far much lesser (2 to 5%) in a more recent article (4). Skull radiographs have limited indications mostly in suspected battered child. MR imaging will describe more efficiently white matter lesions but is less easy to perform in the acute phase of trauma. Spine : Injuries to the spine are rare. Craniocervical junction is the most frequent site of fracture in this clinical situation (11) especially in the youngest. Most trauma centers recommend to systematically explore the craniocervical junction during head CT (12). High risk patients for cervical spine injury included Glasgow score less than 14, neck tenderness, loss of consciousness, neurologic deficit, fall from a height greater than 3 meters and traffic injury with high kinetic energy (13). Cervicothoracic junction is rarely involved and routine CT has not been recommended even in severely injured patients (13). CT scout views (AP and lateral) may help to decide which patient will need a spiral CT of the neck. Optimal conventional radiography work up is still debated (AP, open mouth, cross-table lateral +/- two oblique views). Spinal cord symptoms are more frequent in the young child and require urgent MRI/CT evaluation. In SCIWORA syndrome the symptoms can be delayed. Launay and col. (14) considered than for any child who has a mechanism of injury that suggests trauma to the spine an urgent MR must be done since the prognosis can be improved if the syndrome is early diagnosed. Lap belt ecchymosis are associated with 21% hollow viscera injuries (15) and 8% had both spine (Chance’s fracture) and intestinal injury (16); this clinical presentation definitely require CT exploration. Chest : Thoracic injuries account for 14% of all trauma related death just after head injuries (1). Contusions, lacerations and pleural effusions are the most frequent lesions encountered. Diaphragmatic rupture may rarely occurred. Tracheobronchal rupture, oesphagal tears and mediastinal vascular lesions are very rare. When rib fractures are present they underline a major trauma. AP chest radiograph at admission is always necessary. Ultrasound may be very useful to evaluate presence and volume of pleural effusion. Renton and coll. (1) reported that in their experience chest CT with IV injection has not significantly changed the clinical management of pediatric thoracic blunt trauma but in their series 17.7% of therapeutic management was strongly influenced by this imaging exam. Abdomen : The spleen, the liver, the kidney and the pancreas are the more frequent involved organs in this decreasing order. Hollow viscera and the root of the mesentery are interested in less than 1% (15). The small bowel, the duodenum, the colon and the stomach are interested in this decreasing order. Plain film of the abdomen is of very limited interest. When performed in good conditions with high frequency probes abdominal doppler-ultrasound is highly reliable to exclude abdominal lesion (17,18). Doppler must be systematically associated to insure normal parenchymal blood supply and may help to reveal small contusions. However, when CT has already been proposed for evaluation of the head or the spine, an ultrasound examination appears to be time consuming in our experience. CT with IV injection allows to establish classifications of parenchymatous lesions (tables1-3) but in practice, imaging does not seem to modify the surgical management of these lesions except for kidney trauma. In fact, indications for surgery are mainly decided based on hemodynamic status, peritoneal reaction and clinical follow up (19). Pneumoperitoneum can be absent on CT despite an intestinal wall perforation (20). In a series of neurologically normal children who sustained blunt abdominal trauma, 84% major intestinal injuries were correctly suspected clinically (15). These authors do not recommend CT in case of blunt intestinal injury. However, if the need for imaging exploration could be discussed, surgeons and emergency physicians are definitely more confident with the knowledge of the existing lesions and precise organ’s damages. Rarely interventional imaging procedures (arterial embolization, vascular stenting, nephrostomy) must be necessary in emergency (21); drainage of fluid collections is more frequently required especially after surgery. Pelvis : Pelvic fractures are rare and considered as a marker of injury severity (22). When clinically suspected, no radiographs are mandatory; CT with multiplanar reformations (MPR) and 3D reconstructions allows a very accurate staging and a complete assessment of the frequent (88%) associated neurological, vascular, urinary (bladder) or orthopedic injuries (22). Peripheral skeleton : Plain radiograph is the exam of choice. Two orthogonal views must be done in case of suspicion of fracture. CT protocol : MSCT is now widely available. Its impact on child’s management has been recently assessed (23). The radiation dose in such situation has been poorly studied and for Kim et al.(24) CT represent 97% of the effective dose related to the radiologic assessment of injured children. When decision to perform a CT has been decided by the physicians on charge of the child care must be taken : - To ensure the control of hemodynamic status, pain and movements - To limit the artefacts due to resuscitation material and upper limbs - To reduce the dose as low as possible : Hormann et al. (23) proposed for a whole body study : o Children less than 15 kg : 120 kV, 25 mAs o Children between 15 and 34 kg : 120 kV, 50 mAs o Children between 35 and 54 kg : 120 kV, 90 mAs Depending of the number of available detectors, protocols may differ from an institution to another. However, 1 mm cut sections are usually required and reconstructed in 0.8 mm to obtain good multiplanar reconstructions. CT of the head must be performed without IV contrast injection and extended to the first two cervical vertebrae. Ideally, upper limbs are then repositioned above the head and lower cervical spine, chest, abdomen and pelvis must be then explored after contrast injection. Scan acquisitions must not be done too quickly after injection to obtain an homogenous enhancement of the parenchymas. An arterial phase is not necessary except when a very rare aortic dissection is suspected. A delayed acquisition of the abdomen or pelvis must be done when respectively kidney or bladder trauma are diagnosed or suspected on the first phase of acquisition. Reconstructed 5 mm cuts are usually sufficient to correctly explore the different parenchymas. Reconstructed 1 to 2 mm cuts are necessary to look for bone lesions. Multiplanar reformations are very useful especially in case of suspected spinal lesions. All images must be looked at with different window levels including lung, bone and soft tissues. Algorithm: Imaging evaluation of a polytraumatized child Hemodynamically stable Hemodynamically unstable Neurological and Spinal status Exploration required Hemodynamically controlled No need for exploration Chest Xray Abdominal Doppler-US Abnormal Whole Body CT MRI No Surgery Normal CT of the interested area suspected spinal cord lesion Yes Stop Table 1 : AAST Liver Injury Grading System I - Hematoma: subcapsular, <10% surface area - Laceration: capsular tear, <1 cm in parenchymal depth II - Hematoma: subcapsular, 10%–50% surface area; intraparenchymal, <10 cm in diameter - Laceration: 1–3 cm in parenchymal depth, <10 cm in length III - Hematoma: - subcapsular, >50% surface area or expanding or ruptured subcapsular hematoma with active bleeding; intraparenchymal, >10 cm or expanding or ruptured - Laceration: > 3 cm in parenchymal depth IV - Hematoma: ruptured intraparenchymal hematoma with active bleeding - Laceration: parenchymal disruption involving 25%–75% of a hepatic lobe or one to three Couinaud segments within a single lobe V - Laceration: parenchymal disruption involving > 75% of a hepatic lobe or more than three Couinaud segments within a single lobe - Vascular: juxtahepatic venous injuries (ie, retrohepatic vena cava or central major hepatic veins) VI - Vascular: hepatic avulsion Table 2 : American Association for the Surgery of Trauma Organ Injury: Spleen injury grading system I - Subcapsular hematoma, < 10% surface area; - Capsular laceration, < 1 cm parenchymal depth II - Subcapsular hematoma, 10%–50% surface area; intraparenchymal hematoma, < 5 cm diameter; - Laceration with 1–3 cm parenchymal depth, not involving a trabecular vessel III - Subcapsular hematoma, > 50% surface area or expanding; ruptured subcapsular or parenchymal hematoma; intraparenchymal hematoma >5 cm - Laceration with > 3 cm parenchymal depth or involving trabecular vessels IV - Laceration of segmental or hilar vessels that produces major devascularization >25% of spleen - Completely shattered spleen; vascular hilar injury with devascularized spleen V Table 3 : American Association for the Surgery of Trauma Organ Injury: Kidney injury grading system I - Hematuria with normal imaging studies - Contusions - Nonexpanding subcapsular hematomas II - Nonexpanding perinephric hematomas confined to the retroperitoneum - Superficial cortical lacerations less than 1 cm in depth without collecting system injury III - Renal lacerations greater than 1 cm in depth that do not involve the collecting system IV - Renal lacerations extending through the kidney into the collecting system - Injuries involving the main renal artery or vein with contained hemorrhage - Segmental infarctions without associated lacerations - Expanding subcapsular hematomas compressing the kidney V - Shattered or devascularized kidney - Ureteropelvic avulsions - Complete laceration or thrombus of the main renal artery or vein References : 1) Renton J, Kincaid S, Elhrich PF. Should helical CT scanning of the thoracic cavity replace the conventional chest x-ray as a primary assessment tool in pediatric trauma? An efficacy and cost analysis. J Pediatr Surg 2003; 38(5): 793-7 2) Schalamon J, v.Bismarck S, Schober PH, Hollwarth ME. Multiple trauma in pediatric patients. Pediatr Surg Int 2003;19:417-423 3) Brown SL, Elder JS, Spirnak JP. Are pediatric patients more susceptible to major renal injury from blunt trauma ? A comparative study. J Urol 1998; 160: 138-00. 4) Jindal A, Velmahos GC, Rofougaran R. Computed tomography for evaluation of mild to moderate pediatric trauma: are we overusing it? World J Surg. 2002;26(1):13-6 5) Carty H. Blunt abdominal trauma in children: Plain film? Ultrasound? CT? In: Willi U, editor. Difficulties in imaging and understanding of children disease. New York: Springer;1997.p28-36 6) Filippigh P, Clapuyt P, Debauche C, Claus D. Sonographic evaluation of traumatic spinal cord lesions in the newborn infant. Pediatr Radiol. 1994;24(4):245-7 7) Da Dalt L, Marchi AG, Laudizi L, Crichiutti G, Messi G, Pavanello L, Valent F, Barbone F. Predictors of intracranial injuries in children after blunt head trauma. Eur J Pediatr 2006;165:142-148. 8) Hulka F, Mullins RJ, Leonardo V et coll. Significance of peritoneal fluid as an isolated finding on abdominal computed tomographic scans in pediatric trauma patients. J Trauma 1998;44(6):1069-72. 9) Neish AS, Taylor GA, Lund DP, Atkinson CC. Effect of CT information on the diagnosis and management of acute abdominal injury in children. Radiology 1998;206(2):327-31. 10) Beaver BL, Colombani PM, Fal A, Fishman E, Bohrer S, Buck JR, Dudgeon DL, Haller Ja Jr. The efficacy of computed tomography in evaluating abdominal injuries in children with major head trauma J Pediatr Surg 1987;22(12):1117-22. 11) Cirak B, Ziegfeld S, Knigh V, Chang D, Avellino A, Paidas C. Spinal injuries in children. J Pediatr Surg 2004; 39(4):607-612. 12) Keenan HT, Hollingshead MC, Chung CJ, Michele K. Ziglar MK. Using CT of the cervical spine for early evaluation of pediatric patients with head trauma. Am J Roentgenol 2001;177:1405-9 13) Jelly LME, Evans DR, Easty MJ, Coats TJ, Chan O. Radiography vs Spiral CT in the evaluation of cervicothoracic junction injuries in polytrauma patients who have undergone intubation. Radiographics 2000; 20:S251-9. 14) Launay F, Leet AI, Sponseller PD. Pediatric spinal cord injury without radiographic abnormality: a meta-analysis. Clin Orthop Relat Res 2005;(433):166-70. 15) Jerby BL, RJ Attorri, D Morton Jr. Blunt intestinal injury in children: The role of the physical examination. J Pediatr Surg 1997; 32(4) :580-4. 16) Sivit CJ, Taylor GA, Newman KD, Bulas DI, Gotschall CS, Wright CJ, Eichelberger MR. Safety-belt injuries in children with lap-belt ecchymosis: CT findings in 61 patients. Am J Roentgenol 1991;157(1):111-4. 17) Richards JR, Knopf NA, Wang L et al. Blunt abdominal trauma in children: evaluation with emergency US. Radiology. 2002;222(3):749-54 18) Sirlin CB, Brown MA, Andrade-Barreto OA, et al. Blunt abdominal trauma: clinical value of negative screening US scans. Radiology 2004;230(3):661-8. 19) Ruess L, Sivit CJ, Eichelberger MR et coll. Blunt abdominal trauma in children: impact of CT on operative and nonoperative management. Am J Roentgenol 1997;169(4):1011-4. 20) Strouse PJ, Close BJ, Marshall KW et coll. CT of bowel and mesenteric trauma in children. Radiographics1999;19(5):1237-50. 21) Christensen R. Invasive radiology for pediatric trauma. Semin Pediatr Surg 2001; 10(1):7-11. 22) Spiguel L, Glynn L, Liu D. Pediatric pelvic fractures: a marker for injury severity. Am Surg 2006; 72(6):481-4. 23) Hörmann M, Scharitzer M, Philipp M, Metz VM, Lomoschitz F. First experiences with multidetector CT in traumatized children. Eur J Radiol 2003;48 :125-132. 24) Kim PK, Zhu X, Houseknecht E et al. Effective radiation dose from radiologic studies in pediatric trauma patients. World J Surg. 2005;29(12):1557-62. Strategies in Emergency Pediatric Radiology US – CT – MR (CNS excluded) F. Avni Department of Medical Imaging Erasme Hospital – Brussels - Belgium Defining strategies in emergency pediatric radiology is a difficult challenge. It cannot be approached through one single "decision tree" or one straight strategy. There are multiple diseases, multiple presentations and the need for tailoring imaging modalities. Furthermore, during the last years, there have been several significant shifts influencing emergency pediatric radiology. For instance and among others, we have observed: - The move towards perinatal medicine and the raising number of "neonatal emergencies". - The increasing use and development of computerized cross sectional imaging techniques. - The choice, we will have more and more in the future, between many different imaging techniques in order to achieve the correct diagnosis rapidly, safely and at lesser expense. - The growing interest and concern on irradiation hazards. - The growing complexity of cases in terms of type of diseases and their treatment that necessitate a continuous "upgrade" in knowledge and medical education. - Still, our biggest challenge in the next future is probably how to maintain a 24/24h quality reporting with a decreasing number of pediatric radiologists. 1. Neonatal emergencies The increasing use of obstetrical US and of fetal MR imaging has dramatically changed our approach to the neonatal management of congenital anomalies. Nowadays, fetuses with a potentially significant pathology are delivered in a tertiary care environment. They are managed rapidly even before any symptom occurs. In such patients, imaging has to be rapid, efficient and non-invasive. It must confirm the diagnosis and orient treatment. For uro-genital anomalies, plain films and ultrasound examination are usually sufficient at birth in order to confirm most anomalies. Chest CT can be necessary for the preoperative assessment of congenital thoracic malformation. CT or MR imaging will be necessary in case of solid type tumors. Enema (and less usually upper GI series) will be helpful in order to assess intestinal obstruction. A mistake would be not to confirm postnatally the diagnosis and to rely exceedingly upon the antenatal diagnosis. This would lead to wrong doing and poor treatment. Also, all the congenital conditions have been differentiated from any acquired acute neonatal condition (i.e. as necrotizing enterocolitis). 2. Use of computerized cross-sectional imaging techniques In abdominal imaging, the trend in adults is clearly towards a more systematic use of CT for the evaluation of many acute abdominal conditions (i.e. renal colic, acute appendicitis, intestinal obstruction). In pediatric radiology, this evolution is slower but does exist. To date, US is still the central imaging technique for the evaluation of most acute abdominal conditions; yet, several authors do advocate the use of (low dose) CT in children as well. For instance, in case of trauma, the tendency, although controversial, seems towards an increasing use of CT with contrast enhancement. US is being used essentially for the follow-up (it should be stressed that the newest CT scanners allow a better control on the irradiation dose). On the contrary, to date there has not been a clear evolution towards a use of MR imaging in acute abdominal conditions (appendicitis? abscesses?). Whatever technique used, it is mandatory that it should be adapted to the child size and weight. For acute respiratory conditions, the use of simple chest X-ray is still the rule. For skeletal trauma, conventional films of the bone answer most clinical questions. With the advent of digital acquisition techniques, the images must be optimized in terms of windowing so that no detail is missed. In some specific conditions, 3D-CT (i.e. trimalleolar fractures) or MR imaging (occult trauma) or even US (neonatal epiphyseal avulsion) can provide useful information. Ultrasound and MR imaging are also useful in detecting early evidence for osteomyelitis. 3. Proper use of irradiating techniques As concern grows about radiation hazards, one must be very cautious in using CT. The use of US and MR imaging should be emphasized and developed in order to skip the need for CT. 4. The evolution towards fixed-costs for subsidised health In many countries (Belgium, Netherlands…), an evolution has been imposed by the political authorities. More and more, the reimbursement of imaging examinations is included in a global "package" for the management of affections. On one hand, this might bring us to re-think our "decision trees" and to avoid "unnecessary" examinations, on the other the degree of diagnostic accuracy may drop rapidly. Evidence-based studies are mandatory in order to define best managements. 5. The complexity of diseases – New diseases With time, newer treatments are established especially in oncology patients. This leads to complications and to acute conditions that we need to recognize and to learn about. The range of diseases that a pediatric radiologist needs to know is widening. Permanent education and upgrade of knowledge is crucial. 6. 24 hours reporting One of the biggest challenges for the future is maintaining highest quality of reports and 24 hour reporting. This means, sufficient radiologists devoted to pediatric radiology with an optimized training. This means also continuing education of high level. Finally, this means enough staffing both medical and paramedical. In this perspective, the impact of PACS and teleradiology have still to be evaluated. References 1. Swischuk LE. Emergency pediatric imaging: changes over the years. (Part II). Emerg Radiol 2005; 11: 253-261. 2. Brousseau T, Sharief GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am 2006; 53: 69-84. 3. Kalifa G. Imaging in pediatric emergencies. J Radiol 2005; 86: 197-263. 4. John SD. Trends in pediatric emergency imaging. Radiol Clin N Amer 1999; 37: 995-1034. 5. Di Agostino J. Common abdominal emergencies in children. Emerg Clin N Amer 2002; 20: 139-153. 6. Chowdhary SK, Pimpalwar A, Narasimhan KL, Katariya S, Rao KL. Blunt injury of the abdomen: a plea for CT. Pediatr Radiol 2000; 30: 798-800. 7. Cody DD, Moxley DM, Krugh KT, O'Daniel JC, Wagner LK, Eftekhari F. Strategies for formulating appropriate MDCT techniques when imaging the chest, abdomen, and pelvis in pediatric patients. AJR 2004; 182: 849-859. 8. York D, Smith A, Philips JD, von Allmen D. The influence of advanced radiographic imaging on the treatment of pediatric appendicitis. J Pediatr Surg 2005; 40: 1908-1911. 9. Pedrosa I, Rofsky NM. MR imaging in abdominal emergencies. Radiol Clin N Amer 2003; 41: 1243-1273. 10. Moir CR. Abdominal pain in infants and children. Mayo Clin Proc 1996; 71: 984-989. 11. Makowitz RI, Meyer JS, Hegman JA, Fellows KA. The impact of extended radiology attending coverage in a children hospital. Pediatr Radiol 1998; 28: 167-170. 12. Helsted MJ, Kumar H, Paquin JJ, et al. Diagnostic errors by radiology residents by interpreting pediatric radiographs in an emergency setting. Pediatr Radiol 2004; 34: 331-336. 13. Dorias AS, Amernic H, Dick P, et al. Cost effectiveness analysis of weekday and weeknight or weekend shifts for assessment of appendicitis. Pediatr Radiol 2005; 35: 1186-1195. 14. Carver CD, Slovis TL. How many radiologic technologists are necessary to image patients efficiently in a pediatric radiology department. AJR 1990; 155: 187-189. 15. DeCorato DR, Kagetsu NJ, Ablow RC. Off-hours interpretation of radiologic images of patients admitted to the emergency department: efficacy of teleradiology. AJR 1995; 165: 1293-1296. 16. Gouin S, Patel H, et al. The effect of PACs on the accuracy of diagnostic interpretation of pediatric emergency physicians. Acad Emerg Med 2006; 13: 186-190. 17. Scott JN, Romans CC. On call services provided by radiology residents in a university hospital environment. Can Associ Radiol J 2003; 54: 104-108. Acute Chest infections in Children. ECPR Workshop Deauville 2006 Catherine M. Owens Department of Radiology, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK Ann-Marie Jeannes Department of Radiology, St Mary’s Hospital, Paddington. London W2 UK Summary Respiratory tract infection is the most common cause of illness in children carrying significant morbidity and mortality. Host immunity is important in helping to understand and classify the different end organ responses to a particular organism and indeed whether various pathogens are virulent, as well as their degree of virulence. It is therefore prudent to consider separately the immunocompetent from the immunocompromised child when considering the radiological profiles of acute chest infection. The chest radiograph is the primary imaging modality for acute lung disease as it is easy to obtain, inexpensive, and readily available with a low radiation burden. There are a number of scenarios where CT is playing an increasing role in evaluating acute lung disease in a more sensitive manner than the CXR at a price of higher delivered radiation dose. If CT is to be used it is vital that the attending radiologist is aware of the various tips and tricks to reduce CT radiation burden. When dealing with children, the issue of performing a low-dose examination is crucial and of outmost importance. Radiation dose is a contentious issue in paediatrics as it is well established that the lifetime cancer mortality risks attributable to CT examinations are considerably higher than for adults. As proposed by the ALARA principle “as low as reasonably achievable”, the selection of appropriate scanning parameters focuses on the optimization of the image quality whilst delivering the lowest possible radiation dose and shifting the risk-benefit balance towards benefit. Technical parameters that need to be selected for any scan include: thickness of collimation, tube current – milliamperage, and kilovoltage. The thickness of collimation is the minimum section thickness that can be acquired once the scan is finished and in a 16-row MDCT scanner is usually 1.5 mm. Thinner collimation (0.75 mm) increases the radiation dose by approximately 30% with our in house reduced protocol and is applied only in selected cases of vascular abnormalities, visualization of small structures, in cardiac CTs and when High resolution thin slice parenchymal lung imaging is required for detailed assessment of lung parenchyma (Tables 1 and 3). Table 1. Volumetric CT Chest Scanning Parameters [16 slice Somaton Scanner] according to child’s weight when routine [1.5mm collimation] and ‘Combiscan’ [0.75mm] protocol is performed < 15 kg. 15 – 24 kg. 25 – 34 kg. 35 – 44 kg. 45 – 55 kg. Volume Combi Volume Combi Volume Combi Volume Combi Volume Combi 1.5mm 0.75mm kVp 100 100 100 100 100 100 100 100 100 100 eff. mAs 20 20 25 25 35 35 55 55 75 75 Collimation mm 1.5 0.75 1.5 0.75 1.5 0.75 1.5 0.75 1.5 0.75 Scan Slice Width mm 5 5 5 5 5 5 5 5 8 8 Table feed mm 24 12 24 12 24 12 24 12 24 12 Scan time seconds 0.5 0.5 0.5 0.5 0.5 0.5 0.5 0.5 0.5 0.5 CTDI mGy 0.9 1.0 1.13 1.31 1.58 1.75 2.48 2.75 3.38 3.75 Table2. High Resolution CT Chest Scanning Parameters according to child’s weight < 15 kg. < 30 kg. > 30 kg. kVp 100 100 100 eff. mAs 20 30 55 Collimation mm. 1 1 1 Scan Slice Width mm. 1 1 1 Table feed mm. 10 10 10 Scan time seconds 0.36 0.75 0.75 CTDI mGy 0.21 0.32 0.59 Table 3. Dose Comparison for different scanning protocols in a phantom study in our institution Effective Dose mSv Volume (1.5mm) Combi (0.75mm) HRCT CTA CXR M F M F M F M F AP LAT < 15kg 0.77 0.90 0.9 1.05 0.36 0.42 1.30 1.51 0.00487 0.00799 15 – 24 kg 0.93 1.09 1.13 1.31 0.36 0.42 1.62 1.89 0.00874 0.01086 25 –34 kg 1.34 1.56 1.58 1.84 0.54 0.63 2.24 2.62 0.01163 0.00968 35 – 44 kg 2.11 2.46 2.48 2.89 1.00 1.17 2.57 3.0 0.01769 0.01452 Methods adopted to minimize radiation dose in MDCT include: 1. Applying a dose modulation function, where the system samples the patient thickness and adjusts (e.g. reduces) the exposure accordingly when the tube is in the AP/PA position, as patients are narrower in AP than side to side orientation. 2. Reduction of the kilo-voltage to 100 kVp when imaging the thorax. Further reduction to 80 kVp is possible for CTA, but as resolution of the parenchyma is not ideal this is applied only if lung pathology is unlikely. 3. If possible selecting tube collimation of 1.5mm. The 0.75mm collimation improves spatial resolution but as already mentioned increases the radiation dose and is therefore reserved for CTA or where thin slice reconstruction is indicated. 4. Appropriate mAs selection dependent on the patient’s weight or cross sectional diameter. Unlike the single slice scanner, an increase or decrease in table feed time on the MDCT scanner only affects the overall scanning time. An increase in table speed results in concomitant increase in mA and this has no effect on the dose delivered. The tube current is automatically compensated to ensure that the preset effective and total mAs is delivered, i.e. a fast table movement results in an automatic increase in the mA keeping the mAs constant. The immunocompetent child The roles of imaging in evaluation of community acquired pneumonias are multiple: confirmation or exclusion of pneumonia, characterisation and attempts to predict the infectious agents, exclusion of other causes of symptoms, evaluation when resolution is slow or incomplete and evaluation of related complications eg suspected lung abscess [or cavitatory necrosis] which is in fact more common and more benign in children than was previously believed with only 41% of CT proven cases being visible on CXR and a much better prognosis with conservative management than in adults with relatively little lung scarring [ Donnelly 1998]. CT is also valuable in detecting the presence of superinfected bronchopulmonary foregut malformations or underlying/ensuing bronchiectasis. The role of CT in management of empyema is controversial and will be discussed extensively in the Workshop. Mycobacterium tuberculosis. In children pulmonary TB usually occurs as a result of primary infection acquired from an infected adult. Disseminated or extrapulmonary tuberculosis is relatively rare in children and raises the possibility of underlying immunodeficiency and is classified as an AIDS indicator in children. Radiological features are usually similar to those in immunocompetent controls and include lobar consolidation, segmental or lobar atelectasis, pleural effusions and lymphadenopathy. Pulmonary cavitation is uncommon. Large-volume lymphadenopathy is a frequent finding, the paratracheal and hilar regions being the most common sites. Occasionally the chest radiograph appears normal. Miliary disease is uncommon, although when it occurs it may be indistinguishable from LIP. Contrast-enhanced CT (CECT) and HRCT are of particular value in the confirmation of mediastinal and miliary disease. Mediastinal lymphadenopathy is often large in volume and typically demonstrates prominent peripheral enhancement following intravenous contrast. Classical HRCT features of miliary tuberculosis are of a reticulonodular infiltrate. In adults the nodules are typically uniform in size measuring 1-3mm. however in children nodules are more varied in size and definition that the classic Fleischner description based on adult criteria. The immunocompromised child The immunodeficiency states in children may be sub-divided into two major groups; congenital (primary) and acquired (secondary). The spectrum of illness and imaging appearances are similar; regardless of the underlying cause of immunodeficiency. All immunodeficiency states are associated with an increased susceptibility to infection and neoplasia with the lymphoproliferative disorders being the most frequent. However, the type of infections encountered and the risks of neoplasia are influenced by the underlying defect (whether predominantly humoral or cell mediated), the use of immunosuppressive drugs or radiotherapy as well as the length of immunosuppression. A working knowledge of the underlying likely defect is therefore important when interpreting imaging functions in children with immunodeficiency states. The primary or congenital immunodeficiency disorders are inherited groups of disorders resulting from innate defects of the immune system. The secondary or acquired immunodeficiency disorders occur as a consequence of infection with the human immunodeficiency virus (HIV), or secondary to immunosuppressive drug therapy or chemo-radiotherapy (for haematological/solid organ malignancies or as preparation for bone marrow transplantation). Both primary and secondary immunodeficiency states result in an increased susceptibility to infection, with the respiratory tract being the most common disease site. Certain complications, however, particularly the infectious complications, are common to all immunodeficiency states and extensive overlap in imaging findings is observed. THE PRIMARY IMMUNODEFICIENCY DISORDERS Background The congenital or primary immunodeficiency disorders (PID) represent a rare heterogeneous group of genetically determined disorders characterised by defects of cell-mediated immunity, antibody production and/or the complement and phagocyte systems (table 4). Table 4 Immunodeficiency disorders Disorder Inheritance Combined immunodeficiencies Severe combined immunodeficiency X, AR Purine nucleosidase phosphorylase AR Common variable immunodeficiency Predominantly antibody deficiencies Selective 1g A deficiency AR, AD, S Selective 1g G subclass deficiency AR, S Hyper 1g M X, AR X linked Agammaglobulinaemia X Well defined immunodeficiencies Wiscott-Aldrich syndrome X Ataxia telangectasia AR Di George syndrome AD, S Other immunodeficiencies X linked lymphoproliferative disease X Hyper 1g E syndrome S Phagocytic defects Chediak-Higashi syndrome AR AR - autosomal recessive, AD - autosomal dominant, S - sporadic. X - X-linked Clinical manifestations vary according to the subtype of PID and include recurrent infections, infection with unusual or opportunistic organisms, failure to thrive, skin rashes, recurrent skin sepsis and unusual wound healing. A family history of recurrent infections or premature death among siblings may also be revealed. Perhaps the most important, and frequent, clinical manifestation of the congenital immunodeficiency disorders is recurrent sinopulmonary infections which if, adequately treated, may result in the development of obstructive lung disease, chronic respiratory failure and ultimately premature death. After infection, malignancy is the second leading cause of death. The lymphoproliferative disorders (LPD) account for the majority of tumours, with Epstein-Barr virus being associated in approximately 30-60% of cases . The risk of developing a malignancy varies, ranging from 1 to 25%. The children at greatest risk are those with Wiskott-Aldrich syndrome and combined variable immunodeficiency. Pulmonary Complications Diffuse lung disease Common to all the primary immunodeficiency disorders is an inability to mount a normal or adequate antibody response to antigenic stimulation, resulting in susceptibility to bacterial infections, particularly of the respiratory tract. Bronchiectasis is a common complication, occurring in 20-40% of patients. Typical radiographic features include hyperinflation and bronchial wall thickening, with of without dilation. The chest radiograph is neverthetheless insensitive and commonly appears normal or shows only subtle change. High-resolution CT (HRCT) detects abnormalities not visible on the plain. HRCT improves diagnostic accuracy compared to the chest radiograph and allows for an earlier diagnosis Pneumocystis carinii pneumonia Pneumonia caused by Pneumocystis carinii usually occurs in children whose primary immunodeficiency is undiagnosed and, indeed, is frequently the first indicator of an underlying immunodeficiency state. Classical radiographic appearances include hyperinflation with diffuse bilateral interstitial or nodular infiltrates which may be subtle initially, progressing rapidly to widespread alveolar shadowing Angio-invasive aspergillosis (IPA) IPA typically manifests as multi-focal areas of parenchymal inflammation caused by haematogenous dissemination of the organisms. It is associated with infarction and necrosis secondary to vascular obstruction. Classical radiographic features include either solitary or multiple nodules or masses, with or without cavitation. In some cases, however, the chest radiograph appears normal or may demonstrate a focal infiltrate indistinguishable from a pyogenic pneumonia. Specific HRCT features have been described, probably the most characteristic being a ‘halo’ of ground-glass attenuation representing peri-lesional necrosis and haemorrhage, surrounding a central focal fungal nodule or infarct. A second commonly described characteristic feature of IPA is lesional cavitation with the formation of an air-crescent Occasionally IPA may manifest as a necrotising pneumonia with infiltration of local structure or organs). Although these classical features have been described, in many cases, imaging appearances are non-specific. SECONDARY (ACQUIRED) IMMUNODEFICIENCIES Immunodeficiency secondary to immunosuppressive or myeloablative chemo-radiotherapy Respiratory complications may be sub-divided into two broad categories: infectious and noninfectious. The frequency and range and severity of pulmonary complications are influenced by the intensity and length of immunosuppression and are also dependant on whether the treatment goal is immunosuppression or myeloablation. Stem cell transplantation (SCT) SCT is followed by a temporal and predictable sequence of events. Initially there is a marked transient neutrpenia with neutrophil numbers returning to normal within 2-4 weeks. Recovery of lymphocytes is more prolonged with absolute numbers taking approximately 3 months to return to normal. Despite recovery of lymphocyte numbers, cellular (B and T cell) and humoral (particularly anti0body production) immunity usually remains impaired for a further 6-12 months with antibody production taking up to 1 year to return to normal . The development of graft-versus-host disease (GVHD) and the resultant requirement for increased immunosuppression, also delays immunological recovery and is associated with an increased risk of infectious pulmonary complications. Pulmonary complications occur in 40-60% of patients following SCT and are responsible for 1040% of transplant-related deaths. The type of pulmonary complication will be influenced by the type of underlying immune defect and may be subdivided into early (up to 100 days) and late (after 100 days) complications. Pulmonary complications - early Infectious complications. Within the early post-transplant period the most frequently infecting organisms are bacteria and fungi, with Gram-negative organisms and Aspergillus sp being the most prevalent. Children are also at risk from certain viral infections, the most important being respiratory syncytial virus (RSV) and herpes simplex virus (HSV). Bacterial pneumonias. Bacterial pneumonias occur in approximately 10% of patients during the pre-engraftment period and are associated with a high mortality. Viral pneumonias. Viral infections with, for example, respiratory syncytial virus, herpes simplex, adenovirus and varicella, typically occur within the early post-transplant period. Respiratory syncytial virus. Following stem cell transplantation (particularly in the very early posttransplant period), RSV infection commonly leads to a devastating viral pneumonia, with a mortality rate approaching 50% . Fungal pneumonias. Angio-invasive pulmonary aspergillosis (IPA) is reported to occur in approximately 4% of children undergoing SCT Children are at particular risk during episodes of prolonged neutropenia or with the use of high-dose corticosteroids for treating GVHD. The most common infecting organisms are Aspergillus fumigatus or Aspergillus flavus. The prognosis was dismal but introduction of expensive but efficacious triple antifungal therapy has altered mortality rates in children. As a consequence of a profound neutropenia, radiographic appearances may be atypical and, as a result, the diagnosis of IPA is often delayed. A definitive diagnosis can be established only with certainty after histological confirmation of hyphae within lung tissue. Using EORTC criteria probability rates can be high and empirical therapy begun following presence of CT findings of multifocal nodules, perifissural consolidation, the halo sign and cavitation [which is probably less often observed in children than adults]. Acute Non-infectious pulmonary complications. Those occurring within the early posttransplant period include idiopathic pneumonia syndrome (IPS), pulmonary oedema, pulmonary haemorrhage, pulmonary veno-occlusive disease (VOD) and relapse of the underlying malignancy. Diffuse alveolar haemorrhage (DAH). DAH occurs in approximately 10% of patients undergoing allo-BMT and usually occurs at the time of engraftment. IT is usually associated with other pulmonary complications, particularly infection and is associated with a high mortality . Classical radiographic features are those of air space shadowing, which may be patchy and multifocal or more confluent consolidation with air bronchograms. Cessation of bleeding typically results in rapid clearing over a few days . Idiopathic pneumonia syndrome (IPS). IPS is defined as diffuse lung injury for which no cause has been identified . Both GVHD and pre-transplant total body irradiation (TBI) are contributing factors. It usually occurs 6-8 weeks following BMT . Radiographic features are non-specific and variable; they include diffuse air-space shadowing and/or interstitial infiltrates, often with a nodular component. They may be indistinguishable from adult respiratory distress syndrome (ARDS) Pulmonary complications - late Infectious pulmonary complications. Patients continue to be at risk from bacterial infections even after engraftment due to continuing defective humoral immunity. The most common infecting organisms are encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae. Others include Pneumocystis carinii (particularly in those where antibiotic compliance is poor), cytomegalovirus and adenovirus. Non-infectious pulmonary complications. These include obliterative bronchiolitis (OB), diffuse alveolar damage (DAD) lymphocytic interstitial pneumonia (LIP) and relapse of the underlying disease. Obliterative (constrictive) bronchiolitis (OB). Obliterative bronchiolitis is otherwise known as constrictive bronchiolitis and occurs secondarily to chronic pulmonary GVHD. OB is characterised by airflow limitation secondary to submucosal and peri-bronchiolar inflammation, fibrosis and vascular sclerosis, predominantly affecting the medium-sized airways (respiratory bronchioles). Radiological features include hyperinflation, bronchial wall thickening and/or dilation. In the majority, however, the chest radiograph appears normal or shows only subtle change. HRCT improves diagnostic accuracy compared to the chest radiograph. Classical HRCT findings are of patchy areas of lung hypo-attenuation associated with a reduction in the number and calibre of vessels, with or without associated bronchiectasis of bronchial wall thickening. Solid organ transplantation As a consequence of long-term immunosuppressive therapy, recipients of solid organ transplants are at risk from recurrent infections as well as an increased incidence of neoplasia, the lymphoproliferative disorders being the most frequent. The type of organ transplantation in addition to the type and intensity of immunosuppression regimen used will influence the pulmonary complications encountered. Post-transplant lymphoproliferative disorders (PT-LPD). The post-transplant lymphoproliferative disorders represent a spectrum of disease ranging from polyclonal lymphoid hyperplasia to monoclonal malignant lymphoma. The majority of PT-LPD is of B cell origin and is almost invariably associated with Epstein-Barr virus (EBV) infection. The incidence varies from 1% in renal transplant recipient to 10% in heart and heart/lung transplant recipients The time between transplant and onset varies from 1 month to several years. Radiographic appearances of inthrathoracic LPD are variable and range from discrete parenchymal nodules, which may be solitary or multiple, to diffuse or focal reticulonodular infiltrates or consolidation. These findings may or may not be associated with mediastinal lymphadenopathy. Imaging appearances may mimic pyogenic or fungal infection and, as a consequence, biopsy is usually required. HUMAN IMMUNODEFICIENCY VIRUS (HIV) - ASSOCIATED ACQUIRED IMMUNODEFIENCY SYNDROME The human immunodeficiency virus (HIV) produces its dominant effects on T cells. CD4 cells being the main side of viral replication. It also infects CD4-negative cells (B and T cells) resulting in defects in both cell0mediated and humoral immunity. Since the epidemic began, in the late 1980s over 3.8 million children have died of AIDS. Pulmonary complications Pulmonary disease is the most common initial manifestation of HIV in children and is the primary cause of death in 50% of children with AIDS. The most common respiratory manifestations of HIV are bacterial pneumonias, Pneumocystis carinii pneumonia and LIP. Pneumocystis carinii pneumonia. This is frequently the initial manifestation of HIV, often presenting in early infancy. It is the most common opportunistic pulmonary infection in children with AIDS, occurring in up to 50%, and is the leading pulmonary cause of death. Radiographic appearances are variable and include hyperinflation with diffuse bilateral interstitial of nodular infiltrates and widespread alveolar shadowing. Asymmetric, focal or patchy infiltrates are also frequent. In contrast to the disease in children with primary immunodeficiency disorders, caviatory nodules and cysts are common occurring in up to one third of cases. Lymphadenopathy and pleural effusions are uncommon and, if present, an alternative diagnosis such as LIP of MTB should be considered. Pneumothoraces and/or pneumomediastinum are a frequent complication HRCT findings include patchy of diffuse ground-glass opacity, consolidation, cysts or cavities, centrilobular opacities, nodules and interlobular septal thickening. Lymphocytic interstitial pneumonitis (LIP). LIP occurs in approximately one-third of infected children. It occurs as part of the diffuse infiltrate lymphocytosis syndrome (DILS) and is thought to represent a direct ‘hyperimmune’ lung response to the presence of either HIV or EBV. It appears to be restricted to those children expressing the HLA DR 5 alleles and is associated with a slower rate of disease progression. Radiographic appearances include interstitial reticulonodular infiltrates which may progress to patchy air-space consolidation in contrast to Pneumocystis carinii pneumonia, lymphadenopathy is common and often becomes more prominent during episodes of super-added infection Viral infections. Infection may occur as a result of a primary infection of reactivation of latent virus. Viruses include RSV, influenza and parainfluenza virus, CMV and occasionally measles and varicella-zoster virus (VZV). Varicella-zoster virus. Children often present with a more protracted illness than in immunocompetent controls, with approximately 50% suffering from chronic or recurrent VZV infection. The risk of complications increases with increasing immunological impairment. The mortality rate in children with AIDS is 15%. The chest radiograph typically shows bilateral diffuse reticulonodular infiltrates. The nodules are variable in size, ranging from 3 to 10mm; they often coalesce, resulting in focal areas of consolidation. Superimposed bacterial infection is common. Mycobacterium tuberculosis. This may occur at any stage off HIV infection. In children it usually occurs as a result of primary infection acquired from an infected adult. Disseminated or extrapulmonary tuberculosis is classified as an AIDS indicator in children. Radiological features are usually similar to those in immunocompetent controls and include lobar consolidation, segmental or lobar atelectasis, pleural effusions and lymphadenopathy. Pulmonary cavitation is uncommon. Large-volume lymphadenopathy is a frequent finding, the paratracheal and hilar regions being the most common sites. CONCLUSION Pulmonary complications are common in immunocompetent and immunodeficient children and the CXR is the first port of call. Tuberculosis sadly has returned to challenge us and the radiologist must keep this in mind when analysing radiographs with abundant lymphadenopathy, or consolidation which is not resolving on conventional anti microbial agents. A respiratory presentation is the most common first manifestation of immunodeficiency states in children, with infectious complications being the most common. There is overlap in the radiological findings and correlation with the cause and severity of the underlying immunodeficiency state is important when interpreting imaging findings. All immunodeficiency states are also associated with an increased incidence of neoplasia, particularly LPD, the chest being a frequent site of disease. The radiological manifestations in addition to the clinical presentations are often atypical and may be misinterpreted as infection. It is therefore vital that both clinicians and radiologists be aware of the protean manifestations of these potentially treatable conditions. Suggested reading 1 Paediatric Chest Imaging: Lucaya and Strife. Springer Verlag 2002 2: Respiratory Infections following Haemopoetic Stem Cell Transplantation. Dr C M Owens Dr PA Veys. In Childhood Respiratory Infections :British Medical Bulletin Vol 61 2002 Oxford University Press 3 Webb WR, Muller NL, Naidich DP. Disease characterised primarily by parenchymal opacification. In High Resolution CT of the Lung, 3rd edn, pp 355-420. Philadelphia: Lippincott-Williams & Wilkins, 2001. LIVER AND BILIARY TRACT EMERGENCIES Stéphanie Franchi-Abella and Danièle Pariente Service de Radiologie pédiatrique - CHU de Bicêtre 78 rue du Général Leclerc - 94275 Le Kremlin Bicêtre cedex - France E mail : [email protected] ; [email protected] In this workshop we would like to share our experience of acute presentation of liver and biliary tract diseases in infants and children. Topics covered will include some aspects of acute presentation of liver tumours, delayed complications of liver trauma, acute liver failure in neonates and children, acute cholestasis in infants and children and acute hepatic vascular diseases. Illustrative cases will be presented and radiological findings of the main causes will be described. The pertinent clinical and biological findings will be recalled and some guidelines on the use of imaging techniques for the diagnosis and also the possible radiological treatment will be given. HEPATIC TUMOURS Hepatic tumors may present as a rapidly enlarging and painful abdominal mass, particularly when there has been acute tumoral bleeding. However they are often discovered fortuitously on an ultrasound examination performed sometimes on emergency for another acute clinical finding. Approximately two thirds of primary liver tumors in children are malignant and the causes to consider are different in the infant and the child. In neonates and patients less than 3 years of age, the most commonly encountered lesions are hemangioma, hepatoblastoma, metastases of neuroblastoma, mesenchymal hamartoma, and rarely choriocarcinoma. In patients greater than 3 years the most likely diagnoses are hepatocellular carcinoma, sarcomas, focal nodular hyperplasia and rarely adenoma. Infantile hemangioma (also called hemangioendothelioma) may present with congestive heart failure in newborns and infants less than 6 months of age, also with Kasabach-Merritt syndrome (platelet-trapping by the hemangioma), intra tumoral or peritoneal hemorrhage and rarely with hepatic failure. The diagnosis can be made on imaging: the lesion may be focal, heterogenous, with central calcifications or multiple, diffuse simulating a metastatic liver. Abnormally large hepatic artery with high flow and dilated hepatic veins are often evident on Ultrasound. Specific finding is the early marked peripheral enhancement , followed by the centripetal filling of the lesion, which can be observed as well on CT scan or on MR imaging. The mapping of the vasculature is better achieved with multidetector CT angiography. Angiography is nowadays only performed for embolization. Fistulas between portal vein branch and hepatic vein may be present associated with hemangiomas and can be depicted on ultrasound.These can be embolized if there is presence of hepatic failure, pulmonary hypertension or hyperammoniemia.On the contrary arterial embolization is recommanded to treat congestive heart failure and or consumptive coagulopathy. In the neonatal period infantile choriocarcinoma may simulate hemangioma and may present with multiple hemorrhagic and hypervascular lesions in the liver, lungs, brain, skin, ear-nose and throat . Diagnosis is easily made on high serum or urinary level of beta HCG in the newborn and also of the mother. Other differential diagnoses include hepatoblastoma and metastases of neuroblastoma. A traumatic delivery may be complicated by subcapsular or intrahepatic hematoma which may be the first sign of hemophilia. In the older child, acute presentation of hepatic tumor may be observed with traumatic rupture of hepatoblastoma and this is a factor of poor prognosis, with greater risk of distant metastases. Sarcomas also may present with sudden onset of pain due to intra tumoral bleeding. Hemorrhage and rupture are also characteristic findings of adenoma and predisposing factors have to be searched for: glycogen storage disease, hormonal treatment, familial diabetes, portocaval fistula... High fever, hepatomegaly and biological findings of infection may be indicative that a hepatic tumor is a pyogenic or fungal abscess, but necrosis of a non-secreting malignant tumor such as a fibrolamellar carcinoma has also to be considered. Imaging guided aspiration or biopsy is mandatory. LIVER TRAUMAS Management of acute liver trauma will be considered in the lecture on “multiple traumas. We would like to only focus on some of the delayed complications of liver traumas. As the conservative management of blunt hepatic trauma is becoming the primary approach, complications previously not encountered after surgery are more commonly described and can occur weeks to months after injury, often after patient’s discharge. These complications include delayed hemorrhage, hemobilia, non bleeding vascular lesions, biloma and bile peritonitis and intra abdominal abscess formation. Their rate is reported to be between 7 and 10 % in children but they usually occur after deep liver injuries. Post traumatic arterial pseudo aneurysms are thought to be secondary to biloma. They may complicate by decompression into the bile duct causing hemobilia which must be suspected when the patient present with melena or hematemesis, abdominal pain, anemia and cholestasis. US and multi detector CT angiography are reliable to detect these vascular lesions. These arterial lesions may also be the cause of delayed hemorrhage with secondary subcapsular hematoma or hemoperitoneum. Intrahepatic arteriovenous fistula is rare and usually located between a hepatic arterial branch and a portal vein. It may form from direct laceration of adjacent arteries and veins or often via a connection with a pseudo aneurysm. Patients with arterio portal fistulae may develop several days or years after injury gastrointestinal bleeding and or ascitis resulting from portal hypertension and high cardiac output failure. US with doppler is very sensitive to make this diagnosis, showing the zone of fistula, the increased flow in the hepatic artery and the inverted flow in the adjacent portal vein. Angiographic embolization is the best treatment of these lesions, less invasive and more selective than surgery which is only indicated in case of failure. Biloma must be suspected when there is continued growth of an intra or more often peri hepatic collection. The diagnostic may be confirmed either by cholescintigraphy or by percutaneous aspiration. Prolonged percutaneous drainage may be necessary to obtain healing of the bile leak. When the bile leak is massive and due to a large bile duct fistula, surgery may be necessary. MR cholangiography may be useful to demonstrate the origin of the leak. PTC or ERCP may be necessary. ACUTE LIVER FAILURE Acute liver failure is a rare event in infancy and childhood but it is often fatal and liver transplantation may be the only therapeutic option. Its etiology includes infections, metabolic disorders, infiltrative diseases, autoimmune hepatitis and in many cases the cause remains unknown. For making the diagnosis, the major role is played by biological studies but imaging may be helpful in orientating the investigations. US examination has to search for signs of underlying chronic disease such as dysmorphy of the liver, irregularities of its contours with presence of macro or micronodules, signs of portal hypertension ( increased thickness of the lesser omentum, splenomegaly, patent paraombilical vein, spontaneous splenorenal shunt, hepatofugal flow in the portal vein, ascitis), and also anomalies of the kidneys. In newborn or infant, presence of irregular hepatic contours, nodules, ascitis, and nephromegaly with hyperechoic medullary areas are suggestive of tyrosinemia. Intrahepatic nodules may also be seen in some cases of mitochondrial hepatopathies. MRI has been reported to be diagnostic of neonatal hemochromatosis. In older child, fuminant presentation of Wilson disease must be considered when US shows signs of micronodular cirrhosis, ascitis, and sludge in the gallbladder. In some cases, transjugular hepatic biopsy must be performed when the diagnosis remains uncertain. ACUTE CHOLESTASIS Imaging has a major role to play in acute cholestasis. The first goal of imaging is to establish if there is extrahepatic biliary obstruction that will present with dilatation of the biliary tract. Ultrasonography with color Doppler easily allows to distinguish intra-hepatic from extra-hepatic cholestasis. If there is a dilatation of the biliary tract, the second goal of imaging is to define the level and the cause of obstruction. The aetiology to consider differs according to the age. In the neonatal period, if there is a biliary tract dilatation, causes of extra-hepatic cholestasis to consider are cholelithiasis, choledocal cyst, spontaneous perforation of the bile duct, duodenal duplication, tumors with compression of the biliary tract. If there is no biliary tract dilatation, biliary atresia has to be ruled out as rapidly as possible as the prognosis is highly dependent on the delay of treatment. The diagnosis of biliary atresia mainly relies on clinical findings and acholic stools and firm hepatomegaly are strongly suggestive of the diagnosis . However US may be helpful in depicting a cyst in the porta hepatis or elements of the polysplenia syndrome which is associated with about 10% of cases of biliary atresia. In childhood, causes of acute extra-hepatic biliary obstruction are cholelithiasis, choledocal cyst, extrinsic compression by a tumor or a cavernoma in case of portal obstruction, liver trauma, postsurgical stenosis. Acute intra-hepatic cholestasis are secondary to viral hepatitis and drug induced hepatitis. Management of cholelithiasis will differ according to the age of the child. In neonates and infants, it is a rare event. Causative factors have been reported: haemolysis, dehydration, infection, parenteral nutrition, furosemide treatment and gastrointestinal disorders. Spontaneous resolution of the obstruction can occur rapidly. That is why, if the infant has no fever, we recommend observation during about 2 weeks. However, acute complications such as cholangitis and even abscess formation can occur. If the infant becomes septic or there is no spontaneous resolution, percutaneous transhepatic cholecystography or cholangiography to flush biliary tree with saline and contrast can be proposed in order to push the stones in the duodenum. This treatment is successful in about 75 % of our cases. It requires sometimes repeated lavage via an external drainage left in place. If this treatment fails surgery is required. In older children, main causes of gallstones are hemolytic anemia, liver disease, parenteral nutrition, biliary tract obstruction , drugs ( cextriaxone, furosemide), obesity. Cholelithiasis can remain asymptomatic and resolve spontaneously. If there is a migration and obstruction, a surgical treatment with cholecystectomy is recommended. Choledochal cyst or congenital dilatation of the common bile duct is the most common malformation of the biliary tree and may become symptomatic at any age with cholestasis, pain, acute pancreatitis or rarely bilious peritonitis. Antenatal diagnosis or fortuitous discovery is possible. It is in most cases secondary to an abnormally long common duct between biliary and pancreatic channels that allows reflux of the pancreatic secretions in the bile duct. Ultrasonography shows dilatation of the choledochus ranging from a minimal enlargement to a major cystic deformation often associated with dilatation of the intrahepatic ducts. Cholelithiasis can exist. In some cases it can be difficult to distinguish primary cholelithiasis from choledochal cyst complicated by lithiasis. MR cholangiography or percutaneous cholangiography or ERCP will show the abnormal long common biliary-pancreatic duct that makes the diagnosis of congenital dilatation of the common bile duct. Surgery consists of complete resection of the extra hepatic bile duct and hepatico-jejunostomy in order to deconnect bile and pancreatic ducts and to prevent the risk of cholangiocarcinoma, which has been reported as early as in the second decade. Hydrops of the gallbladder is an acute distension of the gallbladder without mechanical obstruction of the cystic duct. The pathogenesis is unknown. It can be associated with various diseases: streptococcus infection, kawasaki disease, leptospirosis, salmonella and shigella infection, extensive burning, polyarteritis nodosa, familial paroxysmal polyseritis, prolonged parenteral nutrition. Spontaneous resolution is the usual outcome. Perforation can occur but is rare. Surgery is usually not necessary. Percutaneous drainage may be useful. HEPATIC VASCULAR DISEASES Portal hypertension related to cirrhosis is usually an indolent process. Acute portal hypertension, however may occur in the setting of acute portal vein thrombosis, acute Budd-Chiari syndrome, veno-occlusive disease or even more rarely arterio-portal fistulas. Acute portal vein thrombosis can be related to ascending omphalitis or more often an umbilical vein catheter in the neonatal period. It can be secondary to liver abscess and also to complicated appendicitis. It has also been seen after surgical procedures involving the liver (difficult tumour resections, biliary tract surgery), and after splenectomy. Other hypercoagulable states have also been incriminated. Usually the liver size and function are normal. When it is associated with a hepatic mass, a malignant lesion must be suspected. The diagnosis can be made by US with doppler which shows echogenic and enlarged portal veins branches without flow. Later on (about 2 weeks in our experience) there is development of a portal cavernoma consisting of multiple collateral veins with hepatopetal flow. Hepatofugal veins can be rapidly demonstated by US or angioCT and can manifest with GI bleeding. In Budd-Chiari syndrome and veno-occlusive disease, the liver is enlarged and there may be severe ascites. Classically in acute Budd-Chiari syndrome (which is rare in children), US may show echogenic hepatic vein and or thrombus in the distal inferior vena cava and intra hepatic collateral veins joining the patent hepatic vein in a “spider web” pattern. There may be reversal flow in the portal vein. In veno-occlusive disease the diagnosis is suspected mainly because of predisposing conditions such as bone marrow transplantation, chemotherapy or radiation or more rarely now with ingestion of toxic plants as originally described. On US the liver appears heterogeneous and all the hepatic veins very thin. There may be reversal of the portal vein flow and increased hepatic artery resistive index. In both cases of supra hepatic level of portal hypertension CT scan and MR may demonstrate suggestive heterogeneous contrast enhancement of the liver parenchyma. Arterioportal fistula can be congenital, post-traumatic, iatrogenic or associated with a tumor or Rendu-Osler disease. The diagnosis is usually made by ultrasonography with Doppler that shows the abnormal communication between an hepatic artery and a portal vein. There is an hepatofugal flow in the portal vessel that is connected to the artery with arterialisation of the portal flow. Both vessels are usually enlarged, mostly in congenital forms with in some cases aneurysmal dilatation of the portal branch. If the fistula is large, all the portal flow can become hepatofugal and severe portal hypertension may be observed. Several veins and arteries can be part of the malformation. In post-traumatic or iatrogenic forms, the fistula is not always depicted on US, even if symptomatic. AngioCT or angioMR can provide important informations showing the anatomy of a complex fistula and can depict post-traumatic or iatrogenic fistula not visible on US. The treatment is necessary. In complex forms it can require several interventionnal radiological procedures using arterial or venous ways and can even require surgery for resection or transplantation in diffuse forms. REFERENCES - Goffette PP, Laterre PF. Traumatic injuries: imaging and intervention in post-traumatic complications (delayed intervention). Eur Radiol 2002, 12:994-1221 - Taylor GA, Acute hepatic disease. Radiologic Clinics of North America 1997, 35:799-813 - Helen Carty. Portal hypertension Danièle Pariente . Liver infections, the biliary tract, miscellaneous liver disease In Imaging Children, second edition, Elsevier, Churchill Livingstone 2005 NEONATAL GASTROINTESTINAL TRACT OBSTRUCTION. Veronica Donoghue Radiology Department, Children’s University Hospital, Temple Street, Dublin 1. Ireland. Introduction : Diseases which cause gastrointestinal tract obstruction in the neonatal period are generally divided into those of a medical nature and those that require surgical intervention. The role of diagnostic imaging is to help decide if the patient has a medical or surgical disease and following this to determine if possible the exact nature of the condition. In the premature infant necrotizing enterocolitis is the most frequent condition. In the term infant congenital intestinal obstruction at the various levels are the most common causes. Paralytic ileus, particularly associated with sepsis should also be considered. It is the most frequent cause of negative laparoscopy or laparotomy in neonates (1) The imaging evaluation should include supine and horizontal beam radiographs, either an erect, cross –table lateral or left lateral decubitus view, together with a chest radiograph. These are essential to determine the level of obstruction, air-fluid levels in the bowel and for detecting free intraperitoneal air. When examining the radiograph one should remember that the patterns of the jejunum, ileum and colon are not established in the neonate. The anteroposterior view should cover the abdomen from the diaphragm to the symphysis pubis and include the hernial orifices (2). Sonography is the next line of investigation as it is non-invasive and does not require radiation. Contrast studies of the bowel may also be required. Computed Tomography is rarely necessary. Necrotizing Enterocolitis : This condition is primarily a disease of the premature neonate. The precise aetiology is uncertain and is thought to be multifactorial in origin (3). Mucosal damage, bacterial invasion and inflammatory response are the most likely mechanisms of bowel wall necrosis (4). Clinically these infants have bile stained aspirates, abdominal distension, tenderness, bloody stools , hypotension and shock. Therapy involves nasogastric suction to decompress and rest the bowel and the use of broad spectrum antibiotics. The role of imaging is to confirm the diagnosis and monitor its course. Anteroposterior and crosstable lateral radiographs are performed. A left lateral decubitus view involves unnecessary repositioning if the ill neonate and is less sensitive in the demonstration of free intraperitoneal air (2). The timing of serial radiographs is determined clinically. They are important to determine when to stop therapy and to determine if surgical intervention is required. The earliest and most common radiographic sign is bowel loop distension. This sign however is non-specific. Pneumatosis intestinalis is diagnostic (Fig. 1). However interobserver variability in the diagnosis of pneumatosis is considerable (5). It maybe cystic (submucosal) or linear (intramuscular or subserosal) in nature. It tends to be an early rather than a late finding and maybe transient. It usually involves the colon or terminal ileum but may occur in other areas of the bowel. There is a poor correlation between the presence and extent of pneumatosis and the severity of the disease. Bowel wall thickening, a persistently isolated dilated bowel loop, ascites or portal venous gas may develop. Portal venous gas represents dissection of intramural pneumatosis into the lymphatics and mesenteric veins (Fig. 1). This may also be transient (6). Ultrasonography is useful to detect intraperitoneal fluid and to guide paracentesis. The technique is also useful to detect gas in the portal venous system. Gas in the bowel wall and abscess formation are also detected by sonography. Colour Doppler ultrasound findings of absence of bowel wall perfusion has been shown to be more accurate than clinical examination and plain radiography findings in the prediction of necrosis in neonates with necrotizing enterocolitis and therefore may alter clinical staging and management (7). The presence of a pneumoperitoneum (Fig. 1) is an absolute indication for surgical intervention and indicates full thickness bowel wall necrosis and perforation. Only 63% of infants with surgically proven perforation however, demonstrate a radiographically detectable pneumoperitoneum. Other recognised indications for surgery are: clinical deterioration despite aggressive medical management, fixed dilated bowel loops, abdominal mass and ascites (8). When a neonate is too unwell to undergo surgery a percutaneous drain maybe placed with delayed resection of necrotic bowel (9). Patients may go on to develop strictures at previously affected sites. They maybe solitary or multiple, are usually short and of varying severity. They are suspected in infants with recurrent intermittent obstruction, acute obstruction, low-grade continuing rectal bleeding or constipation. When performing a contrast enema for diagnosis one must take great care to show continuity of the bowel lumen as short strictures maybe obscured by overlapping loops. Strictures may resolve spontaneously particularly if they occur after an acute episode of necrotizing enterocolitis. Oesophageal Atresia : Oesophageal atresia and tracheoesophageal fistula are classified as: a. Oesophageal artesia without fistula (7.8%) b. Oesophageal atresia with proximal fistula (0.8%) c. Oesophageal atresia with distal fistula (85.8%) d. Oesophageal atresia with fistula to both pouches (1.4%) e. H-Type fistula without atresia (4.2%) In the majority of patients the atresia is between the proximal and middle third of the oesophagus and the gap varies in length. The proximal oesophagus forms a blind pouch which distends with air and indicates the diagnosis on the plain radiograph (Fig. 2). This can be confirmed by passing a radiopaque feeding tube which curls when it approaches the blind end of the proximal pouch. Chest radiographs should include the upper abdomen to assess the presence of air in the stomach which indicates the presence of a distal fistula. Routine contrast examination is not required. Contrast imaging of infants with a possible H-type fistula should be performed under fluoroscopic control in a prone position with horizontal fluoroscopy. A feeding tube with end holes is placed in the distal oesophagus. Non-ionic contrast medium is injected through the tube with enough pressure to distend the oesophagus as the tube is slowly withdrawn. If contrast appears in the trachea or lungs it is very important to be certain that it was not aspirated. The H-type fistula usually has an upward oblique course. Bronchoscopy and oesophagoscopy are sensitive methods of detection and maybe indicated to confirm the diagnosis. A right-sided aortic arch occurs in approximately 5% of patients and if present a left thoracotomy is preferred. Stricture formation at the anastamotic repair site is a common postoperative complication. It is related to surgical technique, ischaemia at the anastamotic ends, anastamotic leak and gastroesophageal reflux. Oesophageal dysmotility and tracheomalacia are also recognized complicarions. Between 50% - 70% of infants with oesophageal atresia have associated anomalies. The acronym VACTERL refers to a spectrum of associated congenital malformations that include vertebral abnormalities, anal atresia, cardiac defects, tracheoesophageal fistula, oesophageal atresia, renal and radial limb anomalies (2). Duodenal Obstruction : Intrinsic duodenal obstruction may be due to atresia, stenosis or webs. These result from failure of gut recanalization . Downs syndrome is present in about half of all patients with duodenal atresia. Other associated anomalies include congenital heart disease, malrotation, anorectal anomalies or other bowel atresias. In these patients the abdominal radiograph is diagnostic showing gaseous distension of the stomach and proximal and proximal duodenum without distal gas, the “double-bubble” appearance. (Fig. 3). No further radiological investigation is required (2). If partial obstruction is suspected an upper GI examination is appropriate to differentiate duodenal stenosis or a web from other extrinsic causes such as malrotation with volvolus or obstructing Ladd’s bands. Midgut Malrotation and Volvolus : During the first trimester of gestation the midgut leaves the abdominal cavity and enters the umbilicus and subsequently returns. As it returns the midgut rotates 270 degrees in an anticlockwise direction around the superior mesenteric artery. It is then fixed at the duodenojejeunal flexure and the caecum (10). The term malrotation encompasses a variety of anomalies of intestinal rotation and mesenteric fixation. Most commonly there is incomplete rotation. This leads to mesenteric shortening which may twist on its axis and lead to volvolus. At surgery there maybe Ladd’s bands which develop in an attempt to fix the bowel. These extend from the caecum across the duodenum to the hilum of the liver and may also cause extrinsic duodenal obstruction. Malrotation of the bowel does not in itself cause symptoms or complications. Two thirds of the patients who are symptomatic present in the first weeks of life with acute onset of bilious vomiting. If volvolus occurs around the superior mesenteric artery it may result in bowel obstruction and vascular compromise. It is a true emergency as bowel necrosis may follow with loss of the entire jejunum and ileum. Plain radiographs maybe unremarkable but may demonstrate partial or complete duodenal obstruction, This appearance may occur in duodenal stenosis or atresia with an incomplete diaphragm which is much less common. There may also be a pattern of ileus or small bowel obstruction which is due to the volvolus and closed loop obstruction. These signs are non-specific however. Upper gastrointestinal contrast examination is typically performed to document the location of the duodenojejunal flexure and to document duodenal obstruction. It is vital to obtain a true anteroposterior view of the upper abdomen with the first pass of contrast medium through the proximal small bowel. The antrum of the stomach should not be overfilled to avoid obscuring the duodenojejunal flexure (2). An abnormally located duodenojejunal junction is the most accurate indicator of malrotation (Fig. 4). It should be located to the left of the midline and at or almost at the level of the duodenal bulb. The duodenojejunal junction is mobile in children and can be displaced by an overdistended stomach, the presence of a nasogastric tube, chronic bowel dilatation or an enlarged spleen (11). With malrotation the ligament of Trietz is amost always in an abnormal position, usually lower and to the right of normal. There maybe a spiral or corkscrew appearance of the duodenum with early filling of the small bowel in the right upper quadrant. If duodenal obstruction is complete contrast may not pass far enough to identify the duodenojejunal flexure but this is irrelevant as surgery is then indicated. The normal duodenum is a retroperitoneal structure and on lateral views lies behind the level of the stomach with superimposition of the second and fourth parts of the duodenum. A malrotated duodenum courses anteriorly and this relationship is lost. Therefore a lateral view of the contrast filled duodenum is important. In 16% of these cases the caecum is in a normal location. Contrast enema is not diagnostic in evaluating symptomatic malrotation. The caecum maybe mobile in patients with a normally positioned duodenojejunal flexure. However in patients with malrotation the caecum is typically in the right upper quadrant or in the midline. In patients with malrotation, ultrasonography of the superior mesenteric artery and vein may reveal reversal of their normal orientation (12). The superior mesenteric artery is normally located to the left of the vein. When the superior mesenteric vein is to the left of the artery malrotation maybe present The vessels should be assessed as distally as possible from the superior mesenteric veinsplenic vein confluence in a direct anteroposterior orientation. The sign is not sensitive as one third of patients with malrotation may have a normal vessel orientation. In addition abnormal orientation does not definitely indicate malrotation. Occasionally the superior mesenteric vein and its mesentery rotates clockwise around the artery on colour Doppler ultrasound. This is called the “whirlpool” sign as it is indicative of a volvolus (13). Malrotation maybe associated with duodenal atresia, Meckel’s diverticulum, omphalocoele, gastroschisis, situs inversus, polysplenia and asplenia and Bochdalek hernia. At surgery the bowel is derotated, Ladd’s bands are divided and a Ladd’s procedure is performed. This involves placing the small bowel to the right of the spine and the colon to the left. An appendisectomy is usually performed at the time of surgery. Jejunoileal atresia and stenosis : The majority of jejunal and ileal atresias and stenoses, except those that are familial are thought to be secondary to vascular accidents as a result of intrauterine occlusion of the distal superior mesenteric artery (14). They may involve the bowel anywhere from the duodenojejunal flexure to the ileocaecal valve. The diagnosis may be suggested on antenatal ultrasound because of polyhydramnios and dilated fluid filled bowel loops. The neonate often presents with bilious vomiting and abdominal distension. There maybe failure to pass meconium, particularly in infants with a distal atresia. There are 4 types of small bowel atresia: Type 1: Simple intraluminal diaphragm (32%) Type 2: Atresia with a fibrous cord connecting the blind ends (26%) Type 3: Total atresia with complete separation of the blind ends and an associated mesenteric defect (26%) Type 4: The familial form of multiple atresias (17%) Plain abdominal radiographs demonstrate typical findings of small bowel obstruction with dilated bowel loops. In proximal jejunal atresia there may be very few dilated loops (Fig. 5). More distal atresias have a more uniform dilatation of small bowel loops with associated air-fluid levels (Fig. 6). In utero perforation at the time of the ischaemic insult resulting in meconium peritonitis may cause speckled peritoneal calcification. Bowel wall calcification maybe present as a result of previous ischaemia or infarction (15). Scrotal calcification maybe seen when there is a patent processus vaginalis. Large cystic areas in the abdomen maybe due to very dilated loops proximal to the atretic segment. Additional imaging is not usually required when there is high intestinal obstruction. A contrast enema is required to evaluate low bowel obstruction to distinguish between small and large bowel obstruction. Water soluble low osmolar contrast medium is introduced via a soft rectal catheter. Care must be taken not to precipitate colonic perforation. The more distal the small bowel obstruction the smaller the colon calibre. The microcolon is due to lack of use rather than an anatomic or functional abnormality. The rectum is distensible, distinguishing it from the microcolon occasionally found in long segment Hirschprung’s disease. In distal ileal atresia contrast stops at the atresia. An “apple-peel” atresia maybe suspected if the contrast filled distal ileum has a spiral pattern. There is occlusion of the superior mesenteric artery distal to its origin (16). The diagnosis is also very likely if there is a high intestinal obstruction and malrotation. Surgical treatment involves resection of the atretic or stenotic segment of the intestine with reanastamosis. The proximal dilated bowel may remain for some time in the post-operative period with altered mobility. Meconium Ileus : The condition is almost always associated with cystic fibrosis. 10-12% of patients with cystic fibrosis present with meconium ileus (17). The condition is due to distal ileal obstruction as result of thick tenacious meconium. Clinically, infants usually present with bilious vomiting, abdominal distension and failure to pass meconium in the first 24 hours of life. Abdominal radiographs show multiple dilated air filled bowel loops indicating low intestinal obstruction. In general these infants have fewer air-fluid levels than those with small bowel atresias. A “soap-bubble” appearance maybe seen in the right lower quadrant due to a mixture of air and meconium. Meconium ileus maybe complicated by volvolus, intestinal atresia and perforation with meconium peritonitis or pseudocyst formation. There maybe evidence of a pneumoperitoneum or ascites. If meconium peritonitis is present there maybe associated peritoneal calcification. A localised perforation may form a meconium pseudocyst which may have curvilinear peripheral calcification. Volvolus is caused by the weight of a meconium filled bowel. A contrast enema clinically demonstrates a microcolon with inspissated meconium identified in the distal ileum and dilated small bowel proximal to the obstruction (Fig. 7). Once the diagnosis is established a therapeutic enema should be performed to relieve the obstruction (18). The choice of contrast medium is controversial bur we use dilute gastrograffin (diatrizoate meglumine). It is hyperosmolar and causes fluid shift into the bowel lumen even when diluted. Extreme care must be taken with fluid and electrolyte balance to avoid hypotension and circulatory collapse in the newborn infant. The aim is to introduce contrast medium into the distal small bowel proximal to the obstructing inspissated meconium but care must be taken to avoid overdistension of the microcolon. Several attempts maybe made (1-2 per 24 hours) provided there is progressive clinical improvement in terms of abdominal distension and passage of meconium. The infants fluid and electrolyte balance must be closely monitored during and after each procedure. Overall the therapeutic enema success rate is 50-60% with a perforation rate of 3-10%. In unsuccessful cases surgery often reveals complications. Hirschprung’s Disease : Patients with Hirschprung’s disease typically fail to pass meconium in the first 48 hours of life and may present with abdominal distension, bilious vomiting or enteritis. Over 80% of patients present in the first six weeks of life (19). In this condition there is absence of myenteric plexus ganglion cells in the distal large bowel. It is though to result from arrest of the craniocaudal migration of neuroblasts. This results in a normal to reduced calibre of the aganglionic segment causing a functional obstruction with distension of the more proximal normal bowel. In approximately 80% of patients the disease involves the distal 10 centimetres of large bowel. Approximately 10 – 15% have a longer segment with 5 – 10% having total colonic disease. Abdominal radiographs reveal distal bowel obstruction with a paucity of rectal air. These are nonspecific findings. Pneumoperitoneum occurs in approximately 4% of infants, usually due to caecal perforation. Enterocolitis and bowel pneumatosis may also be seen. Rectal examination should not be performed prior to the contrast enema as this may distort and mask a low lying transition zone from aganglionic to normal colon. In addition bowel preparation should not be performed. The catheter should be inserted just inside the anal margin without dilating the balloon. Low osmolar contrast medium or barium maybe used. The bowel is filled slowly with the infant in the lateral position. The diagnosis is suggested if there is a transition zone between the narrow aganglionic distal bowel and the proximally dilated normal colon (Fig. 8). This however may not always be clearly identified. The aganglionic segment may have irregular contractions causing a saw-toothed mucosal pattern on fluoroscopy. Normally the rectum is 20-40% larger than the sigmoid colon diameter. In Hirschprung’s disease confined to the rectum this ratio, known as the rectosigmoid index (20), may be equal or reversed. Additionally there maybe delayed evacuation of the contrast medium used. A 24 hour delayed film may help in equivocal cases. Knowledge of the extent of the aganglionic bowel is important for pre-operative planning of transanal surgery in these patients. Contrast enema delineation of the transition zone needs to be regarded with caution however. This is especially true in long-segment disease where knowledge of the extent of the aganglionic bowel is most crucial to surgical planning (21). If a neonate demonstrates clinical signs of enterocolitis an enema examination should not be undertaken due to the risk of perforation. Enterocolitis is a potentially fatal complication and may occur before or after surgical treatment of the disease. Confirmation of Hirschprung’s disease is achieved by means of suction biopsies. Anorectal manometry may help in equivocal cases. The contrast enema may not be diagnostic in infants with total colonic Hirschprung’s disease or disease with an ultrashort segment. In total colonic aganglionosis the enema usually shows a normal colonic calibre . A true microcolon appearance is rare. There may however be colonic shortening with rounding of the hepatic and splenic flexures. The colonic wall maybe irregular throughout due to abnormal contractions. In rare cases there is variable small bowel involvement. Surgical treatment of Hirschprung’s disease is now usually undertaken in a one step procedure with resection of the aganglionic segment and confirmation of ganglia in the proximal bowel. Associated anomalies are listed in Table 1. Meconium Plug Syndrome & Small Left Colon Syndrome : Meconium Plug Syndrome and Small Left Colon Syndrome are related and usually present very early in life with failure to pass meconium and abdominal distension. They are the result of delayed peristaltic activity in a normally innervated colon. The aetiology is uncertain. It is associated with maternal drug ingestion, eclampsia, diabetes and prematurity. In meconium plug syndrome the colon has a normal calibre. The plug is in the rectosigmoid region but may extend proximally throughout the entire colon. The infant usually passes the plug following a contrast enema or rectal examination. In small left colon syndrome there is a distal microcolon with a normal calibre colon proximally. It may mimic Hirschprung’s disease and if there is any doubt suction biopsies should be performed. Anorectal Anomalies : A practical classification of these anomalies is the division of the lesions into high, intermediate and low. These divisions are related to the position of the distal rectal pouch in relation to the puborectalis muscle. High and intermediate lesions are treated with a diverting colostomy with delayed repair. Low lesions are treated by anoplasty. A more descriptive classification was introduced by Gans following an international symposium in 1970 (22). They are subdivided into four catergories. Ectopic anus is the most common abnormality where the anus opens onto the perineum, scrotum, vulva, vestibule, urethra, vagina or cloaca. This results from failure of the hindgut to descend correctly to join the anus, instead emptying ectopically through a fistula. The anal dimple and external anal sphincter is usually present to some degree. In imperforate anus the terminal bowel ends blindly and no fistula exists. There are two types – anal and anorectal atresia depending on the length of the atretic portion. The third catergory is rectal atresia where the anus is present and open and a variable segment of rectum is atretic above this. There is no associated fistula. The fourth catergory is anal or rectal stenosis. The outcome often depends on the presence of associated anomalies and their incidence is greater in infants with high anorectal anomalies. The associated abnormalities may manifest as part of the VACTERL association (vertebral, anorectal, cardiac, tracheoesophageal, renal and limb). Evaluation of infants with associated spinal anomalies by spinal ultrasonography and Magnetic Resonance Imaging revealed dysraphism and cord anomalies in up to 50% (23). These may lead to poor bladder and bowel control later in life. Infants usually fail to pass meconium or have an absent or abnormal anal dimple. Plain radiographs will show distal bowel obstruction. If there is an associated fistula gas may be seen in the bladder or rarely in the vagina in girls. It is important to allow at least 12 hours for gas to reach the rectum. A prone cross-table lateral with the buttocks elevated by a pillow for 15 to 20 minutes may be helpful (Fig. 9). However the presence of impacted meconium in the distal rectal pouch can make a low anomaly appear high. The “M” line dividing the lower third and upper two-thirds of the ischium is the most accurate line to classify the anomalies as high or low. Ultrasound of the perineum can also measure the rectal pouch-perineal distance. A distance of 10mm or less indicates a low lesion and 10 – 15mm a high lesion. The detection of associated fistulas and other associated anomalies usually takes place after a colostomy has been performed. Fistulas may be documented via a contrast study in a defunctioning colostomy, a micturating cystogram or occasionally a vaginogram in female infants. Renal tract anomalies are best detected initially using ultrasonography. Repair of high and intermediate anomalies is usually undertaken in the first year of life. Colonic Atresia : There are three types of colonic atresia: Type 1: membranous atresia Type 2: atresia connected by a thin atretic band Type 3: complete atresia with no connecting band which is the most common type found. Colonic stenosis is less frequent. The aetiology is very likely due to an intrauterine vascular accident in a normal colon. The infant usually presents with abdominal distension, vomiting and failure to pass meconium. A contrast enema reveals a microcolon distal to the obstruction. References : 1. Franken EA Jr, Kao SCS, Smith WL et al Imaging of the acute abdomen in infants and children. Am J. Roentgen. 1989;153:921-928 2. Donoghue V, Twomey EL The Neonatal Gastrointestinal Tract Carty H, Brunelle F. Stringer DA, Kao SCS editors Imaging Children, ed. 2. 2005; Elsevier Churchill Livingstone. Chapter 10, p 1305 3. Kanto WP Jr, Hunter JE, Stroll BJ Recognition and medical management of necrotizing enterocolitis. Clin. Perinat. 1994; 21: 335-346 4. Kosloske AM Pathogenesis and prevention of necrotizing enterocolitis: a hypothesis based on personal observation and a review of the literature. Pediatrics 1984;74:1086-1092 5. Mata AG, Rosengart RM Interobserver variability in the radiographic diagnosis of necrotizing enterocolitis Pediatrics. 1980;66:68-71 6. Donoghue V, Kelman CG Transient portal venous gas in necrotizing enterocolitis Br. J. Rad. 1982;55:681-683 7. Faingold R, Daneman A, Tomlinson G et al Necrotizing enterocolitis: assessment of bowel viability with color doppler US Radiology. 2005; 235:587-594 8. Morrison SC, Jacobson JM The radiology of necrotizing enterocolitis. Clin. Perinatol. 1994; 21: 347-363 9. Ein SH, Marshall DG, Girvan D Peritoneal drainage under local anaesthesia for perforations from necrotizing enterocolitis. J. of Ped. Surg. 1977; 12: 963-967 10. Torres AM, Ziegler NM Malrotation of the intestine. World J. Surg. 1993; 17: 326-331 11 Katz ME, Siegal MJ, Shackelford GD et al The position and mobility of the duodenum in children Am J. Roentgenol. 1987; 148: 947-951 12. Dufour D, Delaet MH, Dassonville M. et al Midgut malrotation, the reliability of sonographic diagnosis Pediatr. Radiol. 1992; 22:21-23 13. Pracros JP, Sann L. Genin G. et al Ultrasound diagnosis of midgut volvolus: the whirlpool sign. Pediatr. Radiol. 1992; 22: 18-20 14. Touloukian RJ Diagnosis and treatment of jejunoileal atresia. World J. Surg. 1993; 17: 310-317 15. Aharon M, Kleinhaus U, Lichtig C Neonatal intramural intestinal calcification associated with bowel atresia Am. J. of Roentgen. 1978; 130: 999-1000 16. Herman TE, Siegal MJ Neonatal radiology. Familial apple peel small bowel. J. Perinatol. 1992; 12: 381-382 17. Del Pin CA, Czyrko C, Ziegler MM et al. Management and survival of meconium ileus. A 30 – year review. Ann. Surg. 1992; 215: 179-185 18. Kao SCS, Franken EA Jr. Nonoperative treatment of simple meconium ileus: a survey of the Society for Pediatric Radiology. Pediatr. Radiol. 1995; 25: 97-100 19. Rescorla FJ, Morrison AM, Engles D et al. Hirschprung’s disease: evaluation of mortality and long-term function in 260 cases. Arch. Surg. 1992; 127: 934-941 20. Siegal MJ, Shackelford GD, McAlister WH The rectosigmoid index. Radiology. 1981; 139: 497-499 21. Jamieson DH, Dundlas SE, Belushi SA et al. Does the transition zone reliably delineate aganglionic bowel in Hirschprung’s disease? Pediatr. Rad. 2004; 34: 811-815 22. Gans SL Classification of anorectal anomalies: a critical analysis. J. of Pediatr. Surg. 1970; 5: 511-513 23. McHugh K, Dudley NE, Tam P Pre-operative MRI of anorectal anomalies in the newborn period. Pediatr. Radiol. 1995; 25: S33-S36 Figures for Neonatal Bowel Obstruction Deauville October 2006 Fig. 1. Infant with Necrotizing Enterocolitis. There is diffuse intramural air. Gas is present in the portal venous system (black arrow). There is free intraperitoneal air Indicating bowel perforation(oval arrow) Fig. 2. Infant with oesophageal atresia. The nasogastric tube is curled in the blind ending proximal oesophageal pouch. Fig. 3. Infant with Duodenal Atresia. There is a “double-bubble” appearance. Fig. 4. Midgut malrotation and volvolus. The duodeno-jejunal flexure is low in position and lies to the right of the midline. There is a cork-screw appearance suggesting a volvolus. Fig. 6. Ileal atresia. There are multiple dilated air filled bowel loops with air-fluid levels. Fig. 5 .Jejunal atresia. Air outlines dilated proximal bowel loops and there are air-fluid levels present on a cross table lateral radiograph. Fig. 7. Contrast enema in an infant with meconium ileus. There is a functional microcolon with inspissated meconium in the terminal ileum (black arrow). Fig. 9. Infant with high rectal atresia. Prone cross table lateral with the buttocks elevated outlining the level of the obstruction Fig. 8. Contrast enema in an infant with Hirschprung’s disease. A transition zone Is demonstrated between the narrow distal rectal aganglionic segment and the dilated normal colon (arrow) Intra-peritoneal emergencies in children J.F. Chateil, L. Harper, M. Brun, P. Pietrera, F. Mallemouche (Bordeaux, France) In the presence of acute abdominal pain in a child, the main preoccupation is (what is frequent) to eliminate or (what is rarer) to confirm the presence of a pathology requiring surgery. Other medical disorders can also require more or less urgent treatment. Imaging constitutes an often-useful diagnostic tool. Its results must, however, always be set against clinical examination. Abdominal trauma will not be discussed in this document. In first intention, two exams can help the practitioner: plain film radiography of the abdomen and abdominal ultrasound. Other investigations are rarely necessary immediately, and their indications have to be discussed for each specific case. 1. Imaging 1.1 Plain X-rays of the abdomen: The AP view, at least one incidence in the upright position with a horizontal incidental beam, can be completed by an AP incidence in supine position, with a vertical beam. It has to include the entire abdomen, with the diaphragm and the pubic symphysis. The classic lateral view with horizontal beam is rarely useful and is only performed to look for air-fluid levels or a pneumoperitoneum, when the supine AP view cannot be obtained. The sensibility of plain X rays is poor, and they are mainly used in occlusive diseases or to look for calcifications. Air-fluid levels are still not easy to interpret: they can be isolated or multiple, localized or diffuse and concern the small bowel or the colon. The distinction between true obstruction and multiple levels testifying of gastro-enteritis is not always easy. Calcifications can be seen, but these are often better analyzed on supine view. The fact that the lung bases can be seen sometimes can supply the key to the diagnosis, in which case a chest radiography must be performed... 1.2 Abdominal ultrasound: Abdominal sonography is the most useful examination. Its sensibility increases with higher knowledge of the suspected diagnosis. It is therefore essential to have a well clarified and explicit demand. For the study of the digestive tract, though fasting is preferable, its absence does not constitute a real contraindication and it is generally possible to perform an exam of decent quality. Standardized abdominal ultrasound has to study all the solid organs: liver, biliary tract and hepatic hilum, pancreas, spleen, kidneys and bladder; and search for signs of intraperitonal effusion. According to the suspected pathology, the exam can then be more focused: - Study of the digestive tract with a high frequency probe: parietal wall thickening, digestive peristaltism or distension, obstruction of the lumen; - search for signs in favour of gut malrotation or even of small bowel volvulus, by appreciating the position of superior mesenteric vessels; - careful study of the right iliac fossa: search for the appendix, for the mesenteric lymph nodes or ileitis; - study of the uterus and ovaries; - pulsed Doppler and/or colour Doppler study to appreciate the vascularization of the parenchyma, inflammatory hypervascularisation, or interruption of the vascular flow. This is not always technically easy to perform. We must emphasize again, the need for the investigation to be guided by an appropriate demand. 1.3 Other investigations: Digestive opacifications are rarely useful: upper digestive tract opacification has very few indications in emergency. Main indications of (opaque or air) enema are in relation with intussusception. The use of CT is very debated, because it has become the most useful exploration of acute abdomen in adult patients. In children, CT is important for traumatic lesions. Other indications are discussed according to the results of ultrasound. European guidelines tend to recommend sonography instead of CT. 2. Intussusception This frequent pathology results from the telescoping of an intestinal segment into the adjacent segment. From an anatomical point of view, the intussusception can be ileo-ileal, ileocecal or ileoileocecal. The peak of incidence is between 6 months and 3 years. At this age, “idiopathic forms” are the most frequent, and mesenteric adenolymphitis with hypertrophied lymph nodes and Peyer’s patches can be the lead points. Before 6 months and after 3 years, secondary intussusceptions are more frequent: Meckel’s diverticulum, polyp, digestive duplication, lymphoma, Henoch Schönlein disease... [1] Classical clinical signs associate paroxystic abdominal pain interrupted by calm spells, vomiting and blood in the stools in variable abundance. Abdominal palpation between the episodes of pain can sometimes reveal a mass corresponding to the intussuseption. Associated signs can be less suggestive: access of paleness, non-feverish occlusion, diarrhea..... As soon as the diagnosis is evoked, it is necessary to perform additional investigations in order to confirm or infirm the diagnosis. Hydrostatic reduction is easier when the delay after the beginning of the pain is short, that is before a true occlusion or necrosis of the intussusceptum by strangulation occurs. 2.1 Imaging of intussusception Three techniques can be useful: plain X ray, sonography and enema [2]. Plain X-ray of the abdomen has a poor sensibility. Sometimes, it can show the true image of the intussusception: elongated soft tissue mass, sometimes encircled by a gas crescent. Other signs include the relative poverty of gases, vacuity of the right iliac fossa and air-fluid levels in the occlusive form. Perforation is rare. Ultrasound is the best imaging study in the presence of suspected intussusception. It requires a high frequency probe and investigation of the entire abdomen. Sonography demonstrates the direct image in transverse section with the target sign, whose diameter is at least 25 mm, with a hyper echogenic central zone and a sonolucent crown, corresponding to the intestinal walls. In longitudinal section, there is a “doughnut” aspect. Lymh nodes are sometimes visible within the mass. In case of ileo-ileal intussusception, the mass is less voluminous and can be more difficult to recognize. There frequently is a sonolucent crescent. With doppler, the absence of vascular flow within the intussusceptum often signs a late diagnosis, with possible strangulation. The presence of a small amount of intraperitoneal effusion has no pejorative meaning. Plain X rays with right lower quadrant intussusception Two sonographic examples of intussusception, the second one ielo-ileal Enema and reduction techniques: Hydrostatic or pneumatic reduction can be performed. Hydrostatic reduction under fluoroscopic control allows confirmation of the diagnosis, and reduction of the intussusception in most cases. Contraindications are intestinal perforation, abundant intraperitoneal effusion and hypovolemic shock. The rectal and colonic injection of water-soluble contrast confirms the diagnosis by showing the halt in progress of the contrast media, with the classic image of a concave filling defect with a coiled spring appearance. The rectal injection is pursued until the intussusception is reduced. This is performed under fluoroscopic control, using a non-occlusive cannula, by progressively increasing the hydrostatic pressure (1 - 1,20 m of height). The reduction is complete when the caecum is in place and of normal morphology, and when there is a substantial amount of small bowel filled with the contrast medium at the end of exam. Opaque enema with colic intussusception and hydrostatic reduction After reduction, there is often a pseudotumoral oedema of the ileocecal valve with a specific aspect, which can be seen with ultrasound after evacuation: collapsed intestinal lumen, with a stellar aspect in transverse section and " a fish bone aspect" in longitudinal section. The child is kept in observation a few hours because premature recurrences can exist after reduction: if in doubt ultrasonography can be repeated. Failure of hydrostatic reduction (approximately 25 %) leads to surgery. Other teams perform pneumatic reduction under fluoroscopic control. This method seems faster and maybe more effective; digestive perforation can occur more often, but with less associated morbidity than when it arises with contrast media. Air enema with colic intussusception and pneumatic reduction 3. Acute appendicitis Acute inflammation of the appendix remains the most frequent surgical abdominal emergency in childhood. If in practice the diagnosis of the typical form can be easy for the surgeon, there are often atypical presentations and imaging examinations can play a useful role when the clinical findings are not conclusive. Common symptoms are the existence of pain in the right iliac fossa associated with abdominal tenderness, fever and hyper-leucocytosis. Localized peritonitis signals to an appendicular abscess. 3.1 Imaging of appendicitis Plain film of the abdomen is often not helpful. It can confirm the diagnosis only by showing a appendicolith; this is found in 5 to 10 % of cases, as a round or oval calcification (uniform or lamellated) in the right iliac fossa. Other signs have only an orientation value: presence of air-fluid levels within the last ileal loops, mass effect on the air-filled cecum, spinal splinting. Because of the poor sensibility of plain radiographies, the role of ultrasound is more and more important [3, 4].. Examination is performed with a high frequency probe, with graded compression, and oblique, transverse and longitudinal scans of the right lower quadrant. A normal appendix is found in healthy children in 50 to 90% of cases, regarding the experience of the operator. Appendicitis is diagnosed on sonography if the appendix is non compressible, with a maximal cross-sectional diameter exceeding 6 mm[5, 6]. An appendicolith, adjacent fluid collection or a mass with mixed echogenicity may be found. Colour Doppler study can find signs of loco-regional inflammation, with hypervascularization. Two sonographic examples of acute appendicitis, the second one with abscess When CT is performed, appendicitis is diagnosed when the appendix does not fill completely with air (or contrast media if enema is performed), exceeds 6 mm in cross-sectional diameter, or if an appendicolith, adjacent extra-luminal air or a complex fluid collection or mass are recognized [7]. 4. Mechanical obstructions 4.1 Midgut volvulus and malrotation In most of cases, diagnosis of midgut malrotation is made in the neonatal period. But in some cases, patients may remain asymptomatic, and intestinal obstruction comes later in life. Chronic or acute vomiting with abdominal pain are the most frequent signs. Plain X-rays demonstrate air-fluid levels, sometimes with a duodenal bubble. Sonography (or CT) shows the inversion of the superior mesenteric artery and vein position. The vein is normally echogenic. Volvulus can be recognized with the “whirlpool” sign [8, 9]. Malposition of superior mesenteric vessels and Volvulus with whirlpool sign 4.2 Acquired obstruction Incarcerated hernia is a clinical diagnosis. Adhesions are a common cause of obstruction in children who have undergone prior abdominal surgery, although this is less frequent after coelioscopic surgery. Acute abdominal pain and vomiting leads to the diagnosis. Sonography may be useful, by demonstrating dilated loops, with or without peristalsis, thickening of the intestinal wall and intraperitoneal fluid. 5. Abdominal pain and Henoch Schonlein disease Henoch Schonlein purpura (HSP) is the most common vasculitis in childhood and is characterized by a systemic angiitis, mainly affecting the small vessels of the skin, joints, gastrointestinal tract, and, more rarely, kidney. HSP usually affects children between the ages of 5 to 15 years. Abdominal pain is the initial symptom in 10% of cases, and abdominal involvement occurs in half these cases. Children have vomiting, gastrointestinal bleeding, and, rarely, intussusception or perforation [10]. Sonography may be useful for diagnosis, or to look for acute complications. In most cases with gastrointestinal involvement, there is submucosal infiltration with oedema or haematomas. This variable thickening (4 to 12 mm), is globally hypo echogenic, localized or diffuse. It realizes a regular circumferential infringement, without real obstruction of the lumen. Intraperitoneal effusion can be present. The evolution is mostly made towards a spontaneous regression, sometimes interrupted by new painful episodes. Intestinal intussusception can occur, favoured by this parietal infiltration, , and justifies sonographic controls to be repeated when paroxystic pain persists. HSP with parietal digestive involvement Renal involvement is impossible to assert with sonography. 6. Meckel’s diverticulum Meckel diverticulum is the most common congenital anomaly of the gastro-intestinal tract. The most common complications are haemorrhage and peptic ulceration, small intestinal obstruction, and diverticulitis [11]. The diagnosis of Meckel’s diverticulum is difficult to establish preoperatively. Bleeding is usually painless. It can be massive and dramatic, or slow and occult. Intestinal obstruction can be in relation with an inverted diverticulum, intussusception, volvulus or internal hernia or adhesions. Acute Meckel’s diverticulitis manifests as abdominal pain, fever and vomiting, as with acute appendicitis. Sonography or even CT can be useful for the diagnosis in cases of intestinal obstruction. Ileo-ieal intussusception is characterized by a target sign, sometimes with a sonolucent crescent at the top of the intussusceptum. Meckel’s diverticulum with intussusception 7. Miscellaneous disorders Other abdominal diseases can be responsible for acute abdominal pain and vomiting. The most frequent aetiologies are the following: [12]. - Haemolytic uremic syndrome, which often begins with acute digestive signs. Severe ulceration of the colon may occur. Sonography shows a infiltration of the bowel wall. It can be very important to rule out renal involvement, with hyperechogenicity of the cortex and elevated vascular resistance index depicted by Doppler study. Haemolytic uremic syndrome with digestive and renal involvement - Pathology of the urachus: secondary infection of a cyst [13]. - Digestive duplications, with perforation or intestinal obstruction. - Abdominal tumours, in particular B lymphomas. - Finally, it is necessary to remember that numerous disorders can cause acute abdominal pain: urologic diseases, acute ovarian torsion… and even otitis or acute chest infection ! 8. Few references: [1] Navarro O, Dugougeat F, Kornecki A, Shuckett B, Alton DJ, Daneman A. The impact of imaging in the management of intussusception owing to pathologic lead points in children. A review of 43 cases. Pediatr Radiol. 2000 Sep;30(9):594-603. [2] Daneman A, Alton DJ. Intussusception. Issues and controversies related to diagnosis and reduction. Radiol Clin North Am. 1996;34(4):743-56. [3] Garcia Pena BM, Cook EF, Mandl KD. Selective imaging strategies for the diagnosis of appendicitis in children. Pediatrics. 2004 Jan;113(1 Pt 1):24-8. [4] Taylor GA. Suspected appendicitis in children: in search of the single best diagnostic test. Radiology. 2004 May;231(2):293-5. [5] Kaiser S, Frenckner B, Jorulf HK. Suspected appendicitis in children: US and CT--a prospective randomized study. Radiology. 2002 Jun;223(3):633-8. [6] Sivit CJ, Applegate KE, Stallion A, Dudgeon DL, Salvator A, Schluchter M, et al. Imaging evaluation of suspected appendicitis in a pediatric population: effectiveness of sonography versus CT. AJR Am J Roentgenol. 2000 Oct;175(4):977-80. [7] Kaiser S, Finnbogason T, Jorulf HK, Soderman E, Frenckner B. Suspected appendicitis in children: diagnosis with contrast-enhanced versus nonenhanced Helical CT. Radiology. 2004 May;231(2):427-33. [8] Pracros JP, Sann L, Genin G, Tran-Minh VA, Morin de Finfe CH, Foray P, et al. Ultrasound diagnosis of midgut volvulus: the "whirlpool" sign. Pediatr Radiol. 1992;22(1):18-20. [9] Patino MO, Munden MM. Utility of the sonographic whirlpool sign in diagnosing midgut volvulus in patients with atypical clinical presentations. J Ultrasound Med. 2004 Mar;23(3):397-401. [10] Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, et al. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005 Dec;35(3):143-53. [11] Levy AD, Hobbs CM. From the archives of the AFIP. Meckel diverticulum: radiologic features with pathologic Correlation. Radiographics. 2004 Mar-Apr;24(2):565-87. [12] Scholbach TM. Changes of renal flow volume in the hemolytic-uremic syndrome--color Doppler sonographic investigations. Pediatr Nephrol. 2001 Aug;16(8):644-7. [13] Ueno T, Hashimoto H, Yokoyama H, Ito M, Kouda K, Kanamaru H. Urachal anomalies: ultrasonography and management. J Pediatr Surg. 2003 Aug;38(8):1203-7. Urinary tract infection (UTI) C. Veyrac, C. Baud, O. Prodhomme, M. Saguintaah, A. Couture Introduction Urinary tract infection commonly occurs in children, since it affects 8.4 % of girls and 1.7 % of boys by the age of 7 years. It includes several different clinical conditions from simple bacteriuria (colonic count of > 105/ml in a clean catch specimen of urine) to renal abscess or pyonephrosis. A lower UTI associates bacteriuria with dysuria, polakiuria and apyrexia. An upper UTI associates pyuria with fever (> 38.5°), vomiting, back or abdominal pain, and abnormal laboratory findings : increased C-Reactive protein (> 20 mg/l), increased Procalcitonin (> 0.8 ng/ml). The distinction between both entities is important since permanent renal damage may occur when the kidneys are involved. Many studies support the idea that delayed diagnosis and treatment of acute pyelonephritis, defined as an acute infection of the renal pelvis and parenchyma, are correlated with an increased incidence in the subsequent development of renal scarring. Acute pyelonephritis The clinicobiological diagnosis of acute pyelonephritis is difficult, especially in the neonate and young infant because of absence of specific clinical findings. Moreover, the severity of renal involvement cannot be assessed without imaging modalities. Physiopathologically, a common factor accounting for the imaging abnormalities in acute pyelonephritis appears to be focal ischemia secondary to an inflammatory response of the kidney to bacterial invasion. Therefore, focal decreased perfusion due to edema, tubular obstruction due to accumulation of granulocytes and/or edema, and altered tubular cell membrane transport mechanism are responsible for the imaging findings. Tc99m DMSA scintigraphy is a non invasive, low-radiation procedure, considered as the imaging of choice to detect acute pyelonephritis. It reflects the mass of functional tubular cells. A focal or diffuse reduction in the DMSA uptake by the renal parenchyma is observed in both acute parenchymal infection and renal scarring. The overall sensitivity, specificity and accuracy for these applications are 90%, 95 % and 92 % respectively. Ultrasound is usually performed in the acute phase to detect a congenital abnormality, especially renal or ureteral dilatation, but it is uncommonly proposed for the early positive diagnosis of pyelonephritis. Indeed in the literature, its sensitivity ranges from 20 % to 69 %. The sonographic findings that may be found are : - Renal enlargement with increase of both kidney diameters, especially the anteroposterior width. - Focal echodense areas that are extremely frequent, with different appearances : hyperechogenic corticomedullary triangles, small tubular or linear cortical bands separated by normal parenchyma resulting in a striated cortex, intramedullary triangles, large mass compressing the normal parenchyma and displacing the renal contour. These lesions are hypovascular on color and power Doppler. Most often, high frequency transducer first demonstrates the lesion ; in rare cases, power or color Doppler detects an hypovascular area that is subsequently shown by gray scale sonography. - Hypoechogenic focal nodules that are usually very small-sized, located within the subcapsular cortex and only detected by high frequency probes. Unfrequently, large strongly hypoechogenic lesions indicate abcedation or necrosis. - Thickening of the pelvic (and/or ureteral) wall : it is not specific of acute infection since it can result from non infected reflux. Nevertheless it often shows blurred margins, and associates with renal sinus hyperechogenic thickened fat. These two combined findings strongly suggest upper UTI. - Abnormal intraluminal renal or ureteral echoes : usually small echoes, mobile with breathing and child's movements, sometimes thicker, gravity-dependent with hydrohydric levels. - In most patients, several abnormal sonographic findings are associated and multifocal lesions are observed. Besides the signs of acute upper UTI, sonography is highly sensitive for detecting a pre existing renal abnormality : renal or/and ureteral dilatation, caliceal deformity, intra vesical ureterocele, renal hypoplasia or scarring suggesting reflux nephropathy. Hyperechogenic areas or loss of corticomedullary differentiation cannot be always recognized as the result of acute infection or chronic nephropathy. Probably power Doppler may help the diagnosis but dysplastic or fibrotic renal tissue exhibits lower vascularisation than normal parenchyma. At last, sonography easily enables the diagnosis of renal or ureteral stones, suggesting some bacterial agents (especially Proteus Mirabilis). It may change the therapeutic management. The infants with infected obstructed kidney usually present with severe clinical symptoms. Performing sonography as first investigation permits to obtain a very early diagnosis and to decide an appropriate treatment such as percutaneous nephrostomy with US guidance, endoscopic opening of a ureterocele or vesical drainage for bladder outlet obstruction. Nevertheless, in some acute pyelonephritis, the renal sonography remains normal. Thus, it is actually impossible to exclude a diagnosis of acute pyelonephritis from sonography alone. Computed Tomography is not routinely used in the diagnosis of acute pyelonephritis in children. It is less sensitive than DMSA and responsible for higher radiation dose to the patients. CT scan is obtained immediately after IV infusion of iodine contrast. It shows sharply bordered triangular areas of decreased attenuation involving both the medulla and cortex, or rounded masses of low attenuation with abnormal renal contours. In some doubtful cases, delayed scans (30 mn later) may be required, that demonstrate areas of increased attenuation. CT may be indicated when acute infection occurs in a previously abnormal renal parenchyma (for example hypodysplasia, reflux nephropathy, …). Indeed, renal scarring may be difficult to distinguish from acute parenchymal infection by DMSA scintigraphy (as well as with US). At last, CT is especially indicated when renal abscess or extra renal extension of the disease is suspected, that is rare occurrence in children. Follow-up imaging. A routine sonographic follow-up is usually not required to control the response to antibiotic therapy, except in the patients with persistent high fever at day 3 of treatment or with criteria of severity (infected dilated kidney for example). After healing of the acute UTI, we routinely perform a voiding cystography. A DMSA scintigraphy is indicated 6 to 8 months after the acute event in order to detect the development of a renal scarring but this is not our subject today. Renal abscess It is an unfrequent lesion in children, compared with the high incidence of acute pyelonephritis. Some rare infective agents such as Staphylococcus Aureus and anaerobic bacteria may be encountered besides the common E. Coli and Proteus. Urine cultures may remain negative. Thus, the diagnosis lies on imaging since US and CT permit 82-90 % accuracy. A renal abscess appears on sonography as a strongly hypoechogenic hypovascular rounded focal intraparenchymal lesion with blurred margins. The surrounding parenchyma may be hypo or hyperechogenic, hypovascular or hyperemic. Capsular thickening suggests an associated extension into the pararenal tissues. Nevertheless, this is better demonstrated by CT which is indicated, since 50 % of abscesses seem to have a perirenal component. On CT, renal abscess is a well defined mass of reduced attenuation, with a thick wall enhancing after IV contrast administration. Perirenal fat may be infiltrated with increased attenuation. Percutaneous drainage has large indications (usually with CT guidance), except in very small lesions which may respond to the medical treatment alone. Renal abscess may be difficult to differenciate from infected calyceal diverticulum or renal cyst that appear with smooth thin margins, intra cystic gravity-dependent echoes, suggestive relationship with the normal calices. The sonographic follow-up confirms the diagnosis. Fungal pyelonephritis Fungal pyelonephritis is most commonly secondary to Candida or Aspergillus species. It occurs in patients at risk : premature neonates, children with prolonged intensive care or with prolonged insertion of intraluminal renal or ureteral catheter, immunocompromised patients. Two different presentations are encountered : - Multiple granulomatous intraparenchymal microabscesses, that appear on sonography as small rounded target-like subcapsular cortical nodules. They result from hematogenous spread of the disease and are commonly associated with hepatic, splenic, pulmonary, CNS involvement. High resolution sonography is required to demonstrate the tiny lesions. CT also shows the multiple microabscesses and is indicated in older children with small lesions. On US follow-up, the nodules remain visible despite the efficiency of treatment, with a transient increase of their echodense core. - Fungal balls, that appear as rounded homogeneously echogenic structures, within the urinary tract lumen and may cause obstruction. The diagnosis is suspected from the imaging aspect and the clinical context. It is confirmed by isolation of the species in the urine or blood cultures. Obstructing fungal pyelonephritis usually requires percutaneous drainage combined with specific medications. Xanthogranulomatous pyelonephritis Xanthogranulomatous pyelonephritis is an extremely uncommon type of renal inflammatory disease in children. It is a distinct clinicopathologic entity characterized histopathologically by suppuration, parenchymal destruction and accumulation of lipid laden macrophages. It usually associates urinary tract obstruction, infection, renal stones and decrease in parenchymal tissue. The diagnosis is extremely difficult, especially in the focal form that mimicks renal malignancy. At ultrasound, the renal size is increased, the normal parenchyma is replaced with multiple fluid-filled masses, that contain renal stones in 70 to 79 % of patients (often staghorn calculi), the extremely thin parenchyma exhibits abnormal echostructure (granulomatous tissue). CT is indicated, showing an enlarged kidney, rounded masses of low attenuation representing caliceal dilatation or necrotic tissue (the bear-paw sign) with renal calculi. The walls of the cavities enhance after contrast but there is no renal excretion. A perirenal extension can be demonstrated. MRI has been described as able to demonstrate the specific fatty tissue in the enlarged multiloculated kidney. The treatment of choice is nephrectomy (total or partial), but can be difficult due to nephromegaly and the extent of pararenal inflammatory changes. Conclusion In our experience, imaging plays an important role in the early diagnosis of upper UTI. Besides DMSA scintigraphy (the gold standard for acute pyelonephritis), ultrasound has become more and more sensitive but it requires the use of high resolution devices with combined Doppler associated with experience of the radiologist (and patience ..). CT (and MRI) have specific indications. References Chang JW, Chen SJ, Chin TW, Tsai HL, Pan CC, Chu YK, Tiu CM, Yang LY (2004) Xanthogranulomatous pyelonephritis treated by partial nephrectomy. Pediatr Nephrol 19:1164-1167 Dacher JN, Avni F, François A, Rypens F, Monroc M, Eurin D, Le Dosseur P (1999) Renal sinus hyperechogenicity in acute pyelonephritis : description and pathological correlation. 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