Document 12313

Complex and Innovative Cases in
General Surgery
Marketa Rejtarova, DNP CPNP-AC/PC
Christina Allcox, CPNP-PC
Learning Objectives
Discuss the needs of complex pediatric general surgery
Describe cutting-edge surgical techniques used in
pediatric surgery
Describe the need for collaboration among disciplines
for care of these complex patients
Neither we, nor any member of our immediate families
currently have or have had in the past two years a
financial interest/arrangement or affiliation with one or
more organizations that could be perceived as a real or
apparent conflict of interest in the context of the subject
of this presentation
Inpatient General Surgery PNP Group
at Boston Children’s Hospital
14 PNPs (10 FT, 4 PT)
Inpatient General Surgery
Subspecialities (coordinator,
NP(s) and/or advanced RN(s)
Bariatric Program
Congenital Diaphragmatic
Esophageal Atresia
Short Bowel
Vascular Anomalies
Case #1 N.S.
N.S. was born at 38wks via csection to 42yF G2P1->2; IVF
Followed by MFM at OSH; selfreferred to AFCC at BCH
Prenatal imaging:
polyhydramnios, absent fluid
in stomach, no fluid-filled
structure within mediastinum,
fetal brain MRI normal, rest of
fetal survey fine
Esophageal Atresia /
Tracheoesophageal Fistula
EA/TEF con’t
2 separate issues commonly occurring together
The foregut at 19-23days of gestation is a single cell-layer
Gives rise to esophagus and pharynx
Dorsal foregut, primitive esophagus, begins to divide
from the ventral trachea at the level of the carina
(completed by 26th day of gestation)
Disruption in this process TEF
Unavailability of tissue d/t rapid growth and elongation
of the primitive esophagus and possibly with effect of
interruption of the vascular supply EA
Failure of esophagus to become a
continuous tube
Separate proximal and distal pouch
Abnormal connection between the
esophagus and trachea
Proximal, distal, or both
EA/TEF Types
EA/TEF Con’t
Type A often referred to as pure EA
TEF type C most common
Type E is also called type H
EA/TEF Workup
Incidence: 1/3,000 - 4,500, slight male predominance, more
common in preemies
Differential Diagnosis
Pneumonia, GER
50% have other anomalies
EA/TEF is part of VATER or VACTERL associations
Most common are cardiac
Dependent on associated anomalies
EA/TEF Management
Polyhydramnios, small or empty fetal stomach, and/or presence
of a dilated proximal esophageal pouch
Pooling of secretions in the upper esophageal pouch
Inability to tolerate feeds, coughing, choking
Inability to pass a feeding or NG tube*
Plain XR (gasless abdomen or abdominal gas present)
Esophagram (blind pouch); Bronchoscopy (fistula)
ECHO, renal u/s, spinal u/s, spinal xrays, and other based on
clinical findings/suspicion
Prior to repair
Suction of secretions is critical
Parenteral nutrition +/ Evaluation of esp. cardiac anomalies
Case #1 N.S. con’t: After birth
Apgars 9/9, unable to pass NG/Repogle left in pouch to
LWS, occasional desats
Bweight 2.84kg
HEENT: AFOS, eyes grossly normal, ears normal
shape/position, palate intact, NG in place
Resp: Scattered crackles R>L, good air entry
CV: S1/S2 audible, no M/R/G, femoral pulse 2+ B/L
Abd: Soft, NT, on mases, umbilical stump clamped
GU: Normal external, testes descended B/L, anus patent
MSK: No limb deformities appreciated, no sacral dimple
Case #1 N.S. con’t: Workup/PreFoker
Plain XR with gasless abdomen and NG that stops at T2 level,
lungs w/some streaky opacities c/w ?TTN
Sepsis workup d/t desats (negative)
ECHO on DOL #3: normal structure, small PDA, transitional
elevated right side pressures
DOL #3: DL/bronch, EGD, open GT placement, Intraoperative
esophagram, PICC line placement
Identification of proximal TEF
Renal u/s and spine u/s and x-rays normal
No other obvious congenital defects
Pouch decompression, nutritional support,
pulmonary toilet
EA/TEF Types
Type A often referred to as pure EA
TEF type C most common
Type E is also called type H
EA/TEF Surgical Management
A. Gasless abdomen (pure EA and proximal fistula)
Primary repair (short): very rare: +/- ligate and divide fistula
Delayed repair (intermediate): +/- Bougie upper pouch, +/Foker process, +/- esophagostomy
Staged repair (long): Foker process, +/- esophagostomy, +/replacement/interposition
B. Abdominal gas present (distal fistula)
Short gap (ligation and division w/primary repair)
Intermediate (ligation and division w/primary or delayed
repair, GT)
Wide (rare) (GT, ligation and division w/staged repair)
Repairs postponed till baby is medically stable
Long-Gap EA Repair: Foker
EA Team at BCH
Foker Process
Based on the foundation that
esophagus grows under tension
in utero
Placement of traction sutures at
the upper and lower ends of the
esophagus and applying tension
daily in order to stimulate
esophageal tissue growth
Stage 1
Stage 2
Stage 3 +/ Serial EGDs/dilations
LGEA Foker Process
Case #1 N.S. con’t: Foker I
DOL #32: Foker I procedure with external traction (gap 7.5cm,
lower pouch 2cm)
Paralyzed and sedated; on Zosyn
Chest tube d/c’d POD #5
DOL #2: Repair of traction sutures 2/2 looseness
DOL #13: Repair of traction sutures 2/2 looseness
Case #1 N.S. con’t: Foker II
EA/TEF Complications
DOL #56: Foker II procedure with primary anastomosis of
Paralyzed x7 day postop , Versed drip d/c’d POD #17 and
morphine drip d/c’d POD #23; continued on Methadone
Unasyn till POD #5
Extubated POD #8, issues w/secretions, then weaned to RA
Esophagram POD #14 no leak; advanced to full GJT feeds
(24 kcal/oz BM 33 cc/hr)
Diuretics prn; circumcision on POD #17
Routine x-ray to check bone density POD #10: left humerus
and right femur fracture (calcium level ok but PTH high;
ergocalciferol started)
Anastomotic leak (contained vs. not)
Sepsis, mediastinitis d/t leak
GER (>50%)
Difficulty swallowing
Anastomotic strictures
Secondary (exogenous) adrenal insufficiency
Fistula recurrence
Aspiration PNA
Plus complicated by associated anomalies
Acute and long-term
Case #1 N.S. con’t: After Foker II
Transferred to general surgery floor
POD #22: 2m immunizations (except Hep B, parents deferred)
POD #24: EGD/dil
POD# 29: MBS shows aspiration of both thin/honey thick liquids;
thus, pacifier dips only and slowly increased POs w/FT
POD #30: EGD, bronchoscopy, no laryngeal cleft, no recurrence of
TEF, moderate right bronchomalacia
POD #37: EGD/dil (stricture 7mm, dilated to 10mm, steroids)
POD #43: Follow-up bone x-rays: healed fractures (L humerus, R
POD #44: EGD/dil
POD #69: EGD/dil
On full PO bolus feeds (110cc q3 7x/da) plus breastfeeding
POD #71 (HD #121): Discharged locally!
Case #1 N.S. con’t: Readmits con’t
DOL #169: admission after EGD/dil previous day, w/vomiting
Fever upon admission, CXR w/opacity, Zosyn started, bcx
Progressive resp. distress, O2 started, tx to ICU, CXR w/large
PTX, to IR for pigtail chest tube placement/esophagram (large
leak) and OR for EGD (two perforations), stent placement and
NPO/PN/IL, Zosyn, extubated POD #2 and weaned to RA, GT
changed back to GJT for feeds, transferred back to general
surgery floor
Chest tube d/c’d POD #5, CXRs with enlarging PTX, chest tube
Pleural cultures w/mod Pseudomonas and abundant Hemophilus
(sensitive to Zosyn)
POD #12: Esophagram w/persistent leak, stent d/c’d, clips placed
Case #1 N.S. con’t: Readmits
DOL #125: s/p EGD/dil with steroid injection: routine observation,
d/c’d next day
DOL #146: s/p EGD/dil with steroid injection: admission d/t fever
in PACU, no recurrence or other issues, d/c’d next day
Case #1 N.S. con’t: Readmits
DOL #169: admission after EGD/dil previous day, w/vomiting
POD #18: Esophagram without leak, chest tube removed,
CXR no PTX/pneumomediastinum
POD #23: Transitioned to GT feeds
POD #25 & 26: Esophagram w/tight stricture and EGD
confirming this, stricture not amenable to dilation
POD #31: Esophagram w/persistent tight stricture but
allowing passage of clear liquids, allowed to take PO ad lib
Developed fevers, vomiting, loose stools (sick contact: gma):
sepsis rule out (negative), stool studies/cultures w/+norovirus
type 2, supportive therapy
Case #1 N.S. con’t: Readmits
DOL #169: admission after EGD/dil previous day, w/vomiting
POD #37: Esophagram w/increased esophageal stricture
POD #47: Taken to OR for esophageal stricture resection,
NPO/PN/IL postop, Esophagram POD #10 with patent
esophagus, no leak; feeds resumed
POD #62 and 15: EGD/dil
POD #70 and 23: EGD/dil (upto 12mm, steroid injection),
Discharged locally!
DOL #302: s/p EGD/dil (upto 12mm), admission for observation
d/t “wisp” leak at end of procedure, CXR without PTX or
pneumomediastinum, Unasyn x 24 hours, did well, discharged
next day
For those not as fortunate…
Case #1 N.S. con’t: Follow-up
Serial EGDs/dilations outpatient
Last EGD/dil on DOL #379 (very minimal stricture, upto 12mm,
oral budesonide x1 month postop)
Palliation w/esophagostomy and dependence on GJT feeds
Esophageal replacement
Good blood
Easy to
length usually
similar to
Ease of procedure
Adequate length
usually attained
Good blood supply
Readily available
Blood supply
length usually
Rapid transit
of food
Redundancy of
Slow transit
difficult to
Possible respiratory
Delayed GE
Poor blood supply if
need high in neck
Transient dysphagia
Possibly transient
dumping syndrome
es at cervical
May not be
long enough
Gastric Tube
Case #2 S.K.
8yM with hx neurofibromatosis
type 1 and severe midaortic
stenosis (MAS) presents to
BCH surgical clinic with
associated medically refractory
renovascular hypertension,
failure to thrive from intestinal
angina, and claudication
symptoms of his lower
Case #2 S.K. con’t
Case #2 S.K.: CTA on 5/28/13
CT scan findings
Marked stenosis of the abdominal aorta extending from just
above the level of the celiac axis to just below the level of the
renal arteries (~3cm)
Severe stenosis of celiac artery and complete obliteration of
his superior mesenteric artery, as well as stenosis centered
around mesenteric vessels
Midaortic Stenosis
Coarctation of the proximal abdominal aorta (the origin of the
renal and visceral arteries)
Rare condition and accounts for 0.5-2% of aortic coarctations
Can cause renovascular hypertension and ultimately end organ
Surgical bypass with artificial or autologous graft
Renal autotransplantation
Percutaneous transluminal angioplasty with stenting
New Approach to MAS
Review of MAS for past 30 years (at BCH)
Percutaneous intervention acutely brought down BP gradient
Surgical technique offered longer period of time from reintervention (Porras etal., 2013)
Novel surgical approach to lengthen the aorta
Tissue Expander-Stimulated Lengthening of Arteries (TESLA)
Developed by Dr. Heung Bae Kim (Kim etal., 2012)
Performed in three stages
Placement of tissue expander
Serial filling of tissue expander
Removal of tissue expander, resection of stenotic
segment, and primary end-to-end anastomosis
Case #2 S.K.: Presenting
BP maintained on 5mg Amlodipine daily
Marked gradient between UE and LE BP (~48 mmHg)
Persistent claudication
Weight loss
Over the next six months, the episodes of severe abdominal pain
increased and his weight remained stagnant
He was referred to general surgery clinic for further intervention
Case #2 S.K.: 1st Stage of TESLA
on 5/13/13
Specially made tissue expander (4x10cm, fill volume of 125 mL)
with a complete circumferential suture tab and suture tabs coming
from each end
A “tunnel” is made behind the aorta and the inferior vena cava
(IVC) with lumbar veins and arteries incorporated
Tissue expander is placed in the retro-aortic space just above the
Tabs on the expander are placed against the psoas muscle and
sutured into the fascia of the muscle
Longer sutures are then applied directly to the iliac crest
Case #2 S.K.: 1st Stage of TESLA
on 5/13/13 con’t
TESLA Procedure
Micro-injection port placed on the right lower costal margin and its
tubing tunneled into the abdomen and connected to the tubing
from the expander
Port is then secured with two silk ties
30 ml of blue-dyed saline is injected into the expander to assess
expansion and tension on the blood vessels
Barely palpable distal pulse of aorta was noted in OR
Case #2 S.K.: 2nd Stage of TESLA
Case #2 S.K.: 3rd Stage of TESLA
on 7/3/13
Routine filling of tissue expander with ultrasound guidance in
outpatient surgical clinic
SK went to office three times a week for expander filling
Unfortunately, tissue expander noted to be shifted to left side
which was confirmed on CT scan
Able to get to 145 ml of fill volume with noted 3 cm of
anterior displacement of aorta, which was felt adequate to
proceed with final repair early
Stenosed aorta from beginning of renal arteries to above the level
of celiac artery with noted sharp line of demarcation
~3 cm segment of aorta was resected, noted to contain origins of
celiac, SMA and right renal artery
After anastomosis secured, surgeons back-bled prior to removing
clamps (except aortic clamp), restoring flow to lower limbs,
hemostasis was satisfactory
Noted excellent pulse in distal aorta
The celiac artery, SMA and right renal artery were then each
implanted on the newly lengthened aorta via aortic punch
Case #2 S.K.: 3rd Stage of TESLA
Postoperative CTA
Case #2 S.K.: 3rd Stage of TESLA
Postoperative Course
CTA on POD #9 with
good aortic repair with
patent vessels
Celiac small but patent;
left main renal artery
tortuous but patent
Complicated by
Tachycardia and hypertension, antihypertensives restarted
Hematoma on ultrasound
Feeding intolerance, PICC placed and PN initiated
Able to advance diet, PN stopped and PICC removed
Discharged home on POD #15 on Amlodipine 5 mg BID, DAT
with nutritional shakes for extra calories
Weight on d/c 19.8 kg
Case #2 S.K.: Follow-up
Case #2 S.K.: Follow-up CTA
Renal clinic 11/20/13
Gradient between upper and lower BP down to 20 mm Hg
Able to titrate down on Amlodipine 2.5 mg BID
Weight 21.3 kg
Surgical Clinic 12/4/13
BP stable on decreased Amlodipine
Only claudication symptoms with feet and ankle pain
occurring after prolonged Taekwondo work-out
Weight 20.7 kg
Case #2 S.K.: Follow-up con’t
CT angiogram on 12/3/13
New narrowing of the abdominal
aorta at the site of the prior
anastomotic cyst
The aorta measuring 4mm in
diameter vs. 6mm on the last scan
Unsure if this is progressive disease or
narrowing at the anastomosis but
patient asymptomatic
Should this become a problem in the
future, next step would be to pursue
balloon dilation of the stricture
Next follow-up with surgery in 6 months
with CTA
Short Bowel Syndrome / Intestinal
Case #3 M.W.
HPI: 4 mM (DOL #137) with
history of gastroschisis and
s/p resection of necrotic
bowel w/subsequent short
bowel syndrome transferred
from OSH due to diagnosis of
Short Bowel Syndrome,
progressive PN-associated
liver disease, and inability to
advance enteral feeds
Prenatal/Birth Hx: Prenatal
diagnosis of gastroschisis.
Mom was HSV+; she received
Valtrex for viral suppression.
Born at 36 weeks via induced
vaginal delivery d/t poor fetal
growth. Apgar 9/9.
Incidence: ~ 0.7-1.1% of live hospitalized births
Decreased gastrointestinal mucosal surface area and faster
GI transit time
Can lead to malabsorption, electrolyte abnormalities,
dehydration, and ultimately malnutrition
Most common cause of intestinal failure: significant
reduction in functional small bowel mass, leading to
inadequate digestion and absorption, with subsequent
growth failure
Functional definition: PN dependence for >3 months
SBS Causes
What Patients Can You Encounter
Neonates adolescents (adults)
Necrotizing enterocolitis
Intestinal atresia(s)
Malrotation with midgut volvulus
Total intestinal aganglionosis/HD w/SB involvement
Trauma, vascular injury
IBD, tumors, radiation enteritis, intestinal pseudoobstruction, enteropathies, motility disorders
Combination of above
SB loss vs. mucosal enteropathy/motility d/o vs.
combination as causes of intestinal failure
Initial hospitalization versus readmissions
Second opinion
Case #3 M.W. con’t: PMH/PSH
Ventilator Dependence, CLD, s/p tracheostomy (DOL #123)
Hypertension, renal u/s c/w renal medical disease, received
amlodipine (DOL #77-127)
Gastroschisis: Placement of silo (DOL #1), return of bowel loops
into silo d/t compartment syndrome (DOL #2), daily reductions of
bowel, second stage of gastroschisis closure w/vicryl mesh (DOL
#14), washout w/replacement of vicryl mesh (DOL # 20), mesh
removal and final closure of gastroschisis (DOL #54)
Bowel perforation: Ex-lap, repair of enterotomy (DOL #1)
Case #3 M.W. con’t: PMH/PSH
Bowel necrosis; Enterocutaneous fistula; Short bowel syndrome:
Ex-lap, bowel resection with 25cm of jejunum and 15cm of colon
left, repair of jejunocutaneous fistula, jejunocolonic anastomosis
DOL #54)
Gastrostomy placement (DOL #54)
Oral aversion
GERD, s/p Nissen fundoplication (DOL #89)
Failure to advance enteral feeds, on trophic feeds only
TPN dependency, progressive PNALD
Hx Tracheal and urine +candida, treated w/Fluconazole, then
peritoneal cx +candida, changed to Micafungin and Amphotericin
B (dx’d DOL #12 and then DOL #20)
This image cannot currently be
display ed.
Case #3 M.W. con’t: PMH/PSH
Hx abdominal wall celullitis, concern for sepsis, treated w/Vanco
and Zosyn (dx’d DOL #50)
Bacterial overgrowth, started on Flagyl (DOL #82)
Hx Enterococcal bacteremia, urine +serratia and pseudomonas,
tracheal aspirate +serratia (thought to be colonization) (dx’d DOL
#118) , treated with Zosyn
Abdominal wall cellulitis and concern for sepsis (dx’d DOL #134),
treated with Vanco and Zosyn
CVL placement (DOL #1), CVL leak w/subsequent removal (DOL
#80) and PICC placement, CVL replacement (DOL # 89)
SBS: Major Predictors at Play for
Ultimate Enteral Feeding Tolerance
Length and portion of small bowel resected
Presence or absence of ileocecal valve (or terminal ileum?)
+/- Colon resection
Adaptive and functional capacity of the remaining bowel
Health of other organs that assist in digestion and
absorption, PNALD/IFALD
Bacterial overgrowth
Bowel Length
Bowel Conservation
• Normal SB length
• 217 ± 24 cm 27-35 weeks gestation
• 304 ± 44 cm > 35 weeks gestation
• At term, mean length is reported
250-300 cm
• Another 2-3m addition to adulthood
• Normal LB length
• 30-40 cm at birth
• Growing to 1.5 – 2 m in adulthood
• Loss of intestinal length reduces exposure
to nutrients to brush-border hydrolytic
enzymes as well as pancreatic and biliary
secretions; thus, limits digestion and
Limiting as much bowel loss as possible
Utilization of a “second-look” operation for bowel of
questionable viability
Utilization of temporary closure to avoid risk of inducing
abdominal compartment syndrome
In case of intestinal atresia with pre-existing bowel dilation
and limited distal small intestine (usually <35cm), a primary
STEP bowel lengthening procedure may be done
Re-establishment of bowel continuity via stoma takedown is
associated with more rapid weaning from PN
Bowel Length and Area Resected
Nutrient Absorption Sites
To avoid life-long dependence on TPN, one needs
10-35 cm SB with intact ICV (or terminal ileum)
35-60 cm SB without ICV
Nutrient Loss Based on Portion Resected
Duodenum: iron, calcium, magnesium, zinc
Jejunum: cholecystokinin w/decreased biliary and pancreatic
secretions (malabsorption), motilin function (dysmotility)
Ileum: fat absorption, ADEK
Distal Ileum: bile acid reabsorption, vitamin B12 with intrinsic
factor absorption
Colon: delays in bowel adaptation, loss of energy from
absorption of short-chain fatty acids, fluid and electrolyte
Area Resected and Transit Time
Normal intestinal transit time
Rapid in jejunum
Slow in distal ileum (ileal
Small bowel resection
Transit time decreases
Ileal resection reduces transit
time more than
duodenal/jejunal resection
Gastric emptying is also more
rapid with ileal resection
Colon resection shortens
transit time further
Presence or Absence of Ileocecal
ICV regulates the flow of enteric contents from SB to LB
Absence of ICV (or terminal ileum?)
Reduction of GI transit time
Increased loss of fluid and nutrients
Colonic bacteria can contaminate and colonize SB causing
inflammatory response that damages the bowel mucosa that
leads to further malabsorption (bacterial overgrowth)
Bile salts and B12 may be deconjugated by the bacteria
Adaptive and Functional Capacity of
the Remaining Intestine: Key Points
Ability to compensate (area of rxn, other trophic
Ileum more able to adapt and compensate for proximal
bowel loss
Intestinal adaptation
Gross anatomic and histological changes,
lengthening of villi, increased intestinal absorptive
surface area, and improved absorptive function
Digestive enzymes are decreased (functional
immaturity); thus
Functional improvement does not immediately
follow increase in absorptive surface area
The younger the patient, the bigger the advantage d/t
opportunity for further growth
Enteral nutrition as a stimulant of mucosal growth
Citrulline Level
Reflection of functional absorptive capacity
Non-structural amino acid primarily synthesized in intestinal
mucosa reflecting mucosal mass
Highly positively correlated with intestinal length and ability
to wean from PN
Persistent level <12 µmol/L, patients usually unable to wean
from PN
Case #3 M.W. con’t: Hospital Course
at BCH: 4m-8m (DOL #137-255)
Case #3 M.W. con’t: Hospital Course at
BCH: 4m-8m (DOL #137-255) con’t
Prolonged narcotic wean
Dandy-Walker Malformation
Deformational plagiocephaly (fitted for helmet)
Weaned off ventilator (DOL #141) to CPAP then trach collar 28%
Failed trach capping trial (DOL #176)
Periodic increase in secretions requiring optimization of pulm
regimen and/or antibiotic coverage
DL/bronch and kenalog injection of tracheal granuloma (DOL
DL/bronch, excision of suprastomal granulation tissue , baloon
dilation of subglottis, tracheostomy downsized from 4.0 Neo to
3.5 Pedi Shiley (DOL #167)
EKG (DOL #137) showed RV hypertrophy suspicious
for Pulmonary hypertension, ECHO (DOL #249) was of
poor quality but overall normal ventricular function,
Pulm HTN not excluded by cardiac service, planned f/u
Continued on TPN (cycled to 14 hours prior to dispo),
started on Ursodiol
Initiated on Omegaven for PNALD (BILIs DOL #137
7.9/5.3, 1month later 1.2/0.7, then normalized and wnl
Continued on maximal PPI therapy
Citrulline level on admit = 10 µmol/L, on discharge =
Case #3 M.W. con’t: Hospital Course at
BCH: 4m-8m (DOL #137-255) con’t
Case #3 M.W. con’t: Hospital Course at
BCH: 4m-8m (DOL #137-255) con’t
FEN/GI con’t
Slow feed advancement, immodium initiated, developed bilious
vomiting and high stool output on multiple occasions, back down to
trophic feeds only
Only PO stim d/t marked oral aversion
UGI/SBFT: No obstruction ; transit ~15 min (DOL #145)
Contrast enema: Caliber change between markedly dilated small
bowel (likely SB) and nondilated distal colon, no obvious stricture
(DOL #161)
Rectal suction biopsy to r/o Hischsprung’s disease: + ganglion cells
(DOL #188)
UGI/SBFT: consistent with Hx SBS, no obstruction, transit ~40 min
(DOL #247)
Circumcision (DOL #167)
Non-obstructive clot in RIJ noted on routine Doppler u/s, probably
d/t prior PICC line, chronic in nature, not treated per hematology
Abdominal wall cellulitis and concern for sepsis on admission,
completed Vanco/Zosyn course (dx’d DOL #137)
Increased secretions thought to be d/t viral infection (dx’d DOL
Increased secretions 2/2 ?Serratia and Pseudomonas colonization,
received Bactrim IV course and Tobramycin nebulizer treatment
(dx’d DOL #244)
PICC line removal, placement of a tunneled CVL (DOL #167)
Special Aspects of Fluid and
Nutritional Therapy in SBS Patients
SBS Medical Management
• Goal
• To maintain normal growth, promote intestinal adaptation, and
avoid complications associated with intestinal resection and PN
• Fluid, Electrolytes, and PN
• Large fluid losses common
• Usually start w/standard PN solution for age and titration based
on fluid/electrolyte balance after such losses stabilize
• TPN indicated until SB growth and adaption permit growth on
enteral nutrition
• Increased needs
• Enteral Feedings
• After postoperative ileus resolves and fluid/electrolyte status
Parenteral nutrition
Enteral nutrition
Prokinetic agents
Controlling stool output
Hormonal therapy
Parenteral Nutrition
SBS Enteral Nutrition
Life-saving therapy in children with intestinal failure
Goal is to provide adequate caloric intake, macronutrients and
micronutrients to optimize growth and development
Tailored to meet specific individual requirements
Lipids seems to be the major factor in PNALD/IFALD: lipid-limited
and/or fish-oil based formulas are being investigated as to their
effects on prevention and/or reversal of PNALD/IFALD
Reduced intestinal absorptive capacity leads to low bicarbonate
level and low sodium level
Risk factors of PNALD/IFALD, sepsis, mucosal atrophy, and
bacterial overgrowth
Initiation of enteral nutrition is the most important factor as it
obviates IFALD and catheter-associated bloodstream infections
Continuous feeds provide constant saturation of carrier transport
NG, GT to start, later +/- GJT
Trophic feeds as soon as feasible
Advancing of feeds based on stool output, gastric residuals, and s/s
As feeds advance, PN is weaned
Initiation of small amounts of PO feedings asap
BID-TID to promote sucking/swallowing and prevent oral aversion
Bolus feeds, once tolerating full continuous feeds
Usually start with 1hr worth bolus and transition slowly to Q3-4 hrs
SBS Enteral Nutrition con’t
Nutrient Deficiency in SBS
Immunologic and anti-infective factors, growth factors, nucleotides,
glutamine, other amino acids aiding in intestinal adaptation
Other formulas (somewhat controversial re ideal formula)
Consider age, functional anatomy and capacity of digestion and
Complex nutrients tend to stimulate intestinal adaptation more
effectively; however, limited mucosal surface area leads to lactose,
protein, and long-chain fatty acid malabsorption
Thus, protein hydrolysate formula or amino acid-based formula that
is lactose free and has MCT is used to facilitate absorption
Protein is generally tolerated well; fat high caloric
density/neurodevelopment/less adverse effect than carbohydrates
Most SBS patients have absent or compromised terminal ileum
While on PN, these get repleted IV
Regular assessment of vitamin/trace element levels (serum
vitamin A, 25-OH vitamin D3, vitamin E, Zinc, vitamin B-12, and
PT/INR as a reflection of vitamin K)
SBS Feeding Advancement
SBS Feeding Advancement
Principles con’t
Quantify feeding intolerance primarily by stool/ostomy output
and secondarily by reducing substances/gastric
Tolerance assessment no more than twice per 24hrs and no more
than 1 advancement in 24hrs
Ultimate goals (patient-dependent)
s/o < 10 stools/day or 2cc/kg/hr (advance by 10-20 cc/kg/d)
s/o 10-12 stools/day or 2-3 cc/kg/hr (no change)
s/o > 12 stools/day or >3cc/kg/hr (reduce or hold feeds)
RS < 1% (advance feeds per s/o)
RS = 1% (no change)
RS > 1% (reduce rate or hold feeds)
Gastric aspirate < 4x previous hour’s infusion (advance feeds)
Gastric aspirate > 4x previous hour’s infusion (reduce or hold)
No s/s dehydration (advance feeds per s/o)
Present s/s dehydration (reduce or hold)
Causes of Increased Gastric Output
and/or Vomiting in SBS Patients
Underlying dysmotility in a subset of SBS patients
Stricture, adhesions/SBO
Acute infection (GI or non-GI)
Bacterial overgrowth
~100-140 kcals/kg/d
If ostomy/stool output prevents advancement at 20 kcal/oz for 7
days, then increase kcal density
Weaning of PN simultaneously as advancing feeds
Prokinetic Agents in SBS
Relatively frequent in patients with gastroschisis
Aside from true intestinal pseudoobstruction, motility issues tend
to improve with time
Erythromycin: improves gastric emptying and antroduodenal
coordination; azithromycin also possible
(Octreotide: may accentuate bowel ischemia)
(Metoclopromide: can induce tardive dyskinesia; black-box
(Domperidone: treatment of gastroparesis; only under
investigational FDA use; can induce cardiac arrhythmias)
Cisapride: promotes motility in SB and the stomach; under
investigational FDA protocol; occasional use
Causes of Increased Stool/Ostomy
Output in SBS Patients
Hormonal Therapy
2 cc/kg/hr
Baseline stool output in SBS patients
Higher due to limited absorptive surface
Continuous feeds tend to cause less diarrhea in SBS patients
Worsening stool output
Reaching or going over the maximum present absorptive capacity
Quantify feeding intolerance primarily by stool/ostomy output and
secondarily by reducing substances/gastric aspirates/dehydration
Small bowel bacterial overgrowth
History of recurrent abdominal distention, foul-smelling stools, and
Acute change in stool output, associated s/s, +/- ABX use, ID
If no mechanical or infectious etiology exists, loperamide can be used
to decrease stool/ostomy output; stomal refeeding also a strategy in
patient with a long mucous fistula
Hormonal manipulation in attempt to improve
bowel adaptation
Glucagon-like peptide 2 (GLP-2)
Acts on bowel itself and leads to marked
bowel adaptation esp. in porcine models
Long-acting GLP-2 analogue (teduglutide)
Can improve water and to a lesser degree
nutrient absorption (studied in adults with
Trials are ongoing
Theoretical concerns about induction of GI
malignancy exist
Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
1/13-2/4/2011 (DOL# 314-336)
Short Bowel Syndrome, Inability to Advance Feeds, Bowel Dilation
DOL#315: DL/bronch (ORL) and ex-lap, LOA, STEP procedure (88cm
SB to 115cm SB), revision of jejunocolic anastomosis, liver bx (mild
portal inflammation and fibrosis), and GT ex-change (GNS)
NPO/TPN/OM, tx’d from ICU POD #3, UGI/SBFT POD #10 no
leak/obstruction (30 min transit), GT clamped and feeds started,
advanced to 10cc/hr, back on Imodium, PN cycled to 12 hours
Attempted trach capping trial but d/t increased WOB additional
attempts postponed till later
4/7-4/15/2011 (DOL #398-406) ~13 month
Respiratory distress/sepsis
Vent support, Vanco/Zosyn (Redman’s w/Vanco), tx’d from ICU HD #4
Bcx +SNA, resp cx +E-coli, +paraflu, Zosyn x7d, Vanco x10d
Citrulline level = 15 µmol/L
5/9-5/27/2011 (DOL #430-448)
Transfer from OSH after 12hr PN volume given over 1hr w/subsequent
seizures and pulm edema, CVL damaged/removed, bcx +SNA, no more
s/z activity, required PICU stay, now in need of a new CVL
Bradycardia, EKG RV hypertrophy
HD #7 Insertion of cuffed CVL; sedated ECHO (RV hypertrophy, and
HD #8 Lung perfusion scan w/decresed oxygenation 37% L vs. 63% R
HD #10 CTA w/mild/mod PVS left, mild PVS right lower PV
HD #14 Cardiac cath w/RVP 2/3 systemic sensitive to oxygen therapy
RV hypertrophy felt to be RLT to CLD and not PVS, plan to keep on
oxygen to keep pressures low, cont w/stable sinus brady (50’s to 80’s)
Intolerance of feed advancement, stool cx negative, finally able to adv
to ½ rate (8cc/hr)
Vancomycin x 14d for SNA line infection
Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
6/14-6/17/2011 (DOL #466-469) ~15 month
Fever of unknown origin, sepsis workup negative
6/28-7/11/2011 (DOL #480-493)
DL/bronch, trach decannulation, O2 wean
8/2-8/6/2011 (DOL #515-519)
Fever, Klebsiella line infection (Zosyn x14 days)
Con’t Elecare 20kcal/oz at 30cc/hr
MBS 8/4/2011 cleared him for purees
8/15-8/17/2011 (DOL #528-530)
Fever of unknown origin, sepsis workup negative
8/24-9/1/2011 (DOL #537-545)
Fever, increased stool output, sepsis workup negative, feeds
restarted (upto 32cc/hr)
Citrulline level = 39 µmol/L
9/20-10/1/2011 (DOL #564-575) ~18 month
Fever, vomiting, loose stools, sepsis workup negative, stool cultures
Feeding intolerance, UGI/SBFT w/interval increase in bowel dilatation,
slower transit time, restarted feeds and advanced to 20cc/hr
10/13-11/3/2011 (DOL #587-608)
Watery diarrhea despite stopping feeds, then low grade fever
Bowel rest, stool cultures negative, UGI/SBFT unchanged (moderate
bowel dilatation and transit time), no improvement
EGD/C-scope on HD #6 w/+bacterial overgrowth (E-coli, Strep
viridans, other GPC, and Peptostreptococcus species), started on
Cipro alternating w/ Flagyl each 1wk/month, feeds slowly restarted to
20cc/hr, cont PN/OM
CVL broke, repaired at bedside, then Bcx +Pseudomonas aeruginosa,
Zosyn x14 days
Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
11/7-11/17/2011 (DOL #612-622)
Fever, rigors, increased resp distress, still on Zosyn
HD #2, CVL removed in OR, changed to Meropenem d/t multiple
infections, Bcx + Klebsiella, sensitive to Unasyn (total x14 days)
HD #7, CVL placement and ECHO (worsening PVS and RV
hypertrophy), need for continued oxygen therapy, plan for repeat
12/21-12/27/2011 (DOL #656-662) ~21 month
Bloody stools, sepsis workup negative, feeding intolerance, cont
w/heme positive stools, restarted on bacterial overgrowth
regimen, advanced back to 32cc/hr
1/4-1/4/2012 (DOL #670)
?Dehydration, labs wnl, tolerating feeds
1/11-1/20/2012 (DOL #677-686)
Fever, vomiting, dark stools, +sick contact (gastro)
Septic workup negative, bowel rest, stool cx w/+cdiff, IV Flagyl and
enteral Vanco, then monotherapy w/enteral Vanco (total x14d), feeds
restarted upto 28cc/hr, imodium restarted
2/27-3/15/2012 (DOL #724-741) ~2 years
Fever, lethargy, Bcx +GNR, Zosyn/Gent, transferred from OSH
Vanco/Zosyn, Bcx +Klebsiella, changed to Cefepime (x14 d total), cdiff
Inability to restart feeds, bowel rest, then slowly upto 20cc/hr
Fever, fatigue, septic workup negative, slowly advanced back to
This image cannot currently be display ed.
Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
Bilious vomiting, watery stools, low grade fever, Flagyl started
empirically outpatient
Septic workup negative, bowel rest, GT changed to GJT 4/13/2012
Stool +cdiff, started enteral Vanco (x14 days)
EGD/c-scope 4/18/2012 w/cx Klebsiella resistant to Cipro, changed to
Gentamicin for bacterial overgrowth (together w/Flagyl)
Citrulline level = 21 µmol/L
Barium enema 4/25/2012 w/mildly dilated colon and SB
W/many bumps, slowly advanced to 10cc/hr via JT
Lethargy, low grade fever, vomiting
Bcx +SNA, V/Mero changed to Cefazolin (x14 days), initially NPO
advanced to 10cc/hr via JT w/imodium
6/9-6/18/2012 ~2 ¼ years
Feculent output from GT, vomiting
KUB nonobstructive, fluid resuscitated, bowel rest, bcx NGTD, slowly
restarted GJT feeds (10cc/hr)
Note 6/8-6/25: Transplant team consultation for isolated intestine
transplant, not meeting criteria (multiple bouts of CVL/enteral infections,
recent conversion to GJT, tolerating some JT feeds currently, need to
give more time to assess)
Feculent emesis, increased GT output
KUB nonobstructive, bowel rest, Bcx NGTD
GJT exchanged in IR, then tip too proximal again, GJT exchange
No stool x 48 hours, glycerin w/+stool, unable to restart feeds, stool
output increased (imodium restarted), GJT changed back to MICKey for comfort measures under his vest and kept to gravity
Plan for manometry testing outpatient
Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
Admission for EGD/c-scope/manometry testing by GI (normal,
tortuous colon, normal fasting/medication challenge manometry)
Fever, lethargy, septic workup negative, higher stool
output/imodium restarted, GT clamped
“Bubble” in CVL, brought to OSH where leukocytosis WBC 18.3,
hypotensive 50’s, bilious emesis x1, transferred to BCH
Vanco/Mero rule out, bcx NGTD, line repaired at bedside
UGI/SBFT 8/9/2012 w/no concerns
Exchange of GT to GJT in IR 8/10/2012, advanced to 7cc/hr via
CVL leak again, to OR for CVL replacement over guidewire
GJT exchange to skin-level GJT 8/14/2013, feeds increased to
15cc/hr over 20 hours
9/7-9/15/2012 ~ 2 ½ years
Decreased stool output, brown GT output, low grade fever
Septic workup negative, stooling back at baseline, JT feed restarted
Sinus bradycardia into 50’s asleep, sedated ECHO 9/12 w/mild pulm
hypertension, lung perfusion scan 9/12 improved (L43%/R57%),
EKG w/right axis deviation and RV hypertrophy, recs to continue
supplemental O2
URI, started Amox by PCP, then desats, CXR at OSH w/PNA,
changed to Azithromycin, coming for eval
CXR without consolidation, septic rule out negative, though cont
w/desats into 80’s (upto 2L O2), increased neb therapy, cards/pulm
rec 14 day Cefepime and cont O2
No change to JT feeds (Elec JR at 15cc/hr)
Citrulline level = 20 µmol/L
Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
12/18-12/23/2012 ~2 ¾ years
URI s/s, wheezing, fever, +sick contact (URI)
CXR w/perihilar interstitial markings, septic workup negative,
supportive therapy (O2, nebs added 3% NS nebs, viral DFA/flu
Tolerating feeds at 26cc/hr
Increased O2 requirement and PNA on CXR at OSH
V/Z started, bcx NGTD, changed to Augmentin for PNA (x10
days), supportive therapy
Attempt at unsedated ECHO to eval RVP but unable
Broken CVL, clamped, line replaced over guidewire in OR 1/14
Septic workup negative, due for GJT exchange (done), feeds
upto 27cc/hr
Bilious vomiting, worsened, lethargy
Aggressive IVF resuscitation, still oliguric, abdominal pain,
inconsolable, HD #2 taken to OR for Ex-lap w/LOA but no focal point
found, ARBF postop, then JT restarted and advanced to 5cc/hr
Left hip cellulitis, u/s negative for joint involvement/collections,
received 7d Vanco
3/12-3/15/2013 ~3 years
Line repaired x2 at OSH, no ABX given, presents w/fever and
Vanco/Zosyn, developed hives w/Zosyn, got Epinephrine/Benadryl,
changed to Meropenem, d/c’d w/negative rule out
Found to have swelling/erythema R submandibular neck w/enlarged
lymph nodes (largest 1.6cm on u/s, inflammation), afebrile, throat
wnl, CRP 6.3, completed 7d Ancef for infected lymph node
Feeds increased to 18cc/hr x 20 hours
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Case #3 M.W. con’t: Readmits
Case #3 M.W. con’t: Readmits
Vomiting, becoming feculent, increased GT output
Septic rule out negative, bowel rest, then slow restarting of JT
feeds upto 26cc/hr, GT clamped
Citrulline level = 26 µmol/L
Fever, septic workup negative
Underwent EGD, DL/bronch on 5/6 as previously scheduled
(reassuring findings)
Fever, Bcx +GNR at OSH, transferred for further care
Vanco/Mero, bcx + Enterococcus and Enterobacter, changed to
V/Cefepime, completed 14d ABX, test of cure Bcx +
w/Enterococcus on 6/3/2013, to OR for CVL removal, ECHO
without signs of vegetation, changed to Vanco/Mero, then Vanco
only x 14 days since 1st negative, PICC placed in meantime for
TPN/OM, feeds upto 15cc/hr
Fever, fatigue, increased stools
Septic workup negative, JT feeds restarted
Bilious vomiting, septic workup negative, unable to restart JT
feeds w/stable plain films and no obstruction/stable bowel
dilatation on UGI/SBFT 8/1/2013
8/25-8/29/2013 ~3 ½ years
Fever, fussy, not tolerating feeds, ?viral enteritis
Septic workup negative, able to restart feeds (upto 8cc/hr)
SBS Complications
Intestinal failure associated liver disease (IFALD) / PN-associated
liver disease (PNALD): cholesterol gallstones, cholestasis,
steatorrheic liver disease, portal fibrosis, cirrhosis, liver failure
Catheter-associated bloodstream infection (CABSI)
Vein thrombosis, loss of access
Hyperacid secretions leading to impairment in carbohydrate and
protein digestion and absorption, fat lipolysis and then to diarrhea
Vitamin/mineral deficiencies (esp. once off PN)
Bacterial overgrowth
Oxalate renal stones
Major cause of morbidity/death in children with SBS
Pathophysiology is unclear but vegetable (soy) oil-based lipid
emulsions (VBLEs) are associated with liver injury
Accumulation of potent pro-inflammatory mediators derived
from omega-6 fatty acids
Dysfunctional bile homeostasis due to high levels of phytosterols
PN dependence and DBILI persistently >2 mg/dL (34 µmol/L) not
due to other diseases
Prevention is key
Push enteral nutrition
Avoid overfeeding, use lower lipids or Omegaven as indicated
Cycle PN off for at least 2-6 hours/day
Ursodiol to help body digest fats
Aggressively prevent and treat infections
Fish oil-based Lipid Emulsion (FOLE)
vs. Lower VBLE/Combination
Catheter-Associated Bloodstream
Infections (CABSI)
FOLE – Omegaven (only under FDA
VBLE – High in omega-6 fatty acids,
for compassionate use; 10% lipid
contain phytosterols
Lower VBLE (Lipid restriction to 0.5 Rich in omega-3 fatty acids, low in
1g/kg/d; 20% Intralipid emulsion)
omega-6 fatty acids, no phytosterols
Possible reversal of PNALD
Significant decrease in TBILI, DBILI,
Caloric requirements must be met
TG, CRP, cholesterol, LDL, and trend
by glucose
towards increase in HDL
Risk of essential fatty acid
Reversal of PNALD; potentially also
deficiency (documented cases
prevents PNALD (studies underway)
esp. w/0.5g/kg/d)
Longterm neurodevelopmental
Essential fatty acid deficiency, but
outcomes are unknown
no evidence to date
Combination emulsions
Longterm outcomes are
Even small amount of VBLE leads
to some liver injury/cholestasis
Need for CVL for PN administration
Aseptic technique
Effort to avoid ligating the vessel (esp. large vessel so can be
reused in the future thus prevent “loss of access”)
Line care (various protocols)
Any suspicion, s/s fever, lethargy, irritability, ileus blood culture
High incidence of CABSI d/t enteric organisms, broad spectrum
If fungal infection or evidence of hemodynamic instability, CVL
Ethanol locks: promising role in preventing CABSI
Bacterial Overgrowth
Complicated SBS: Options
• In upto 60% patients with SBS
• Bowel dysmotility and dilation offer ideal environment for
abnormal bacterial growth
• Leads to: abdominal pain, worsening motility, mucosal ulceration
with bleeding, deconjugation of toxic byproducts such as D-lactic
• Also thought to potentiate translocation and hence septicemia
• Treatment is largely empiric
• Possible to obtain duodenal culture aspirate to guide therapy esp.
in challenging cases
• Enteral ABX x 7days followed by a period of no ABX (anaerobic
and GNR coverage)
• Bowel tapering if medical management becomes refractory
• Probiotics are NOT recommended in PN-dependent SBS patients
Especially in those who are extremely difficult or impossible to
wean from parenteral nutrition
Very short remaining small bowel segment (esp <35 cm), IFALD,
CABSI, marked dysmotility, and bacterial overgrowth
Enteral feedings
Wean and cycle TPN as much as possible
Omegaven and/or 1g/kg Intralipid
Meticulous line care, bowel prophylaxis
Surgical options
SBS Surgical Options
STEP Procedure
Nontransplant operations
Intestinal lengthening procedures: Bianchi, STEP, other
Intestinal tapering for dilated dysfunctional bowel segments
Creation of intestinal valves or reversed bowel segments for
patients with rapid intestinal transit times
Multivisceral or intestinal transplantation
First reported in 2003 (initial idea by Dr. H.B. Kim)
Technically straightforward zig-zag lengthening and tapering of
To increase intestinal length and/or taper dilated proximal bowel
in SBS patients and ameliorate bacterial overgrowth
International STEP Data Registry (incepted 2004)
In 2010, >110 patients
Overall survival rate 89%
~50% of patients with refractory IF to maximal medical
management were converted to full enteral feeds
Median time to enteral autonomy ~21 months
5 patients eventually required SB transplant
Case #3 M.W. con’t: Follow-up
Last CAIR Clinic Visit 10/21/2013
Slowly advanced on JT feeds outpatient upto 18cc/hr x 20 hours/d
Stage 1 babyfood via GT 3x/day
Mom happy w/developmental progress, speech
PN/OM providing 66 kcal/kg/d and Elecare JR giving 25 kcal/kg/d
A/AE: Vanco (Redman’s), Zosyn (hives)
Meds: ETOH locks 0.5mL IV 3x/wk, Albuterol prn, Atrovent 2puffs
BID, Flovent 2puffs BID, Ursodiol 150mg JT BID, Imodium 6mg JT
BID, Ferrous Sulfate 30mg JT daily, Cholecalciferol 4,000 units JT
daily, Gentamicin 29mg BID x 7 days via JT every other week,
Miconazole ointment prn topically for fungal rash
PE: Unremarkable (CVL and GJT in place/intact)
Plan: Transition to Compleat Pediatric feeds, increasing PN calories
for weight/growth, d/c cholecalciferol d/t high vit D level, set up due
for cardiology follow-up
Case #3 M.W. con’t: Yet Another
1/9-1/13/2014 ~ 3 ¾ years
Transfer from OSH d/t presentation after anaphylactic reaction to
Pediatric Compleat (hives, face/tongue swelling, resp. s/s)
S/s resolved, transitioned back to Elecare 30kcal/oz via JT
without issues (at 34cc/hr x 20 hours/day), PN over 14 hours
Allergy team c/s (RAST testing for major components in Pedi
Compleat and other major food allergens), follow-up arranged
Routine GJT ex-change in IR
F/u liver ultrasound (slight prominence of the CBD, small
gallbladder with likely sludge, and abnormally small caliber of the
visualized portion of the hepatic IVC (all similar to prior imaging),
Doppler deferred by radiology d/t normal on prior imaging)
Case #4 N.N.
Now 21 year old male s/p
isolated intestine and renal
transplant ~1 year ago for
short bowel syndrome
secondary to ischemic injury
from mesenteric venous
thrombosis after laparoscopic
appendectomy for perforated
Case #4 N.N. con’t: PMH/PSH
At age 15 yr, presented to OSH on 2/26/08 with ruptured appendicitis
Laparoscopic appendectomy with drainage of abdominal abscess
Developed mesenteric venous thrombosis
OR on POD #2 for Exploratory laparotomy (Ex-lap), small bowel
resection of 10 cm of gangrenous bowel
OR on POD #3 for Ex-lap, second look with further small bowel
resection of 15cm d/t necrosis
OR on POD #6 for Ex-lap with anastomosis of small bowel and
abdominal closure
Mesenteric Venous Thrombosis
Rare condition (1 in 5,000 to 15,000 admissions)
Accounts for 6 to 9 % of acute mesenteric ischemia
Mesenteric arterial thrombosis is most common cause of
Predisposing factors
Prothrombotic states, vessel wall injury, and venous stasis
Factors causing vessel wall injury include inflammatory causes
like appendicitis, peritonitis, IBD, and intra-abdominal
Mesenteric Venous Thrombosis con’t
Case #4 N.N. con’t: PMH/PSH
Remained in the ICU intubated
Developed hemoperitoneum and returned to the OR on 3/8/07 (POD
#10 from appendectomy) for Ex-lap with lysis of adhesions and
abdominal washout
Continued with bloody stools and pressor requirement
Colonoscopy performed 3/13/08 (POD 15) secondary to rectal
bleeding with grossly normal mucosal surface; rectal biopsy with
ulcerated mucosa
EGD with gastric ulcer
Tagged RBC scan without bleeding source
Abdominal CTs noted pan-colitis and no evidence for abscess
Case #4 N.N. con’t: ROS upon
Intubated from 2/26-3/8/08 and 3/10-3/15/08
Briefly on 3 pressors while in septic shock
Developed acute renal failure secondary to ATN
Initially on CRRT, transitioned to hemodialysis three times a week
Oliguric, on fluid restriction.
Case #4 N.N. con’t: ROS upon
Family history of hypercoaguable state (father's side)
Work-up ongoing at time of transfer
Persistent fevers despite broad-spectrum antibiotics, blood
cultures negative
Multiple CT scans of abdomen without evidence of abscess
Case #4 N.N. con’t: Transfer to BCH
Case #4 N.N. con’t: Workup at BCH
Transferred to ICP at BCH in 4/4/08 for further care
He was followed by GI service closely
Initial CT scan
Diffuse mucosal enhancement throughout the entire colon
and rectum, most pronounced in the ascending colon,
transverse colon, and descending colon, and least pronounced
in the rectosigmoid
Moderate to large amount of diffuse ascites
Liver, gallbladder, pancreas, and spleen are within normal
Noted relatively diminished enhancement of the kidneys
Follow up scans on 4/30, 5/21, and 6/13 continued to show pancolitis
NN suffered from chronic abdominal pain, with limited
response to narcotics
TENS unit used with success
A trial of Neurontin proved to have no added benefit
Stable on room air during hospitalization
LLL pneumonia, s/p treatment
Bilateral effusions identified on CT on 3/27 and 4/9/08.
Case #4 N.N. con’t: Workup at BCH
Case #4 N.N. con’t: Workup at BCH
Non-sustained ventricular tachycardia 5/08; then
hemodynamically stable; evaluated by Cardiology
ECHO (4/16, 5/9, 5/12, 5/30) showed normal anatomy, no
vegetation, low normal LV function, and trace pericardial effusion
EKG showed incomplete bundle branch block, normal QTc, no
evidence of atrial or ventricular hypertrophy
Had episode of chest pain with transient elevation in troponins,
leading to gated cardiac CTA for coronary arteries and function
on 5/12; this showed normal anatomy and coronary function.
He was started on Metoprolol XL
No arrhythmias since 5/31/08
EKG and signal-averaged EKG before discharge with Q waves
consistent with old ischemia
ECHO on 7/15/08 done because of a new gallop; no major
changes from before.
Feeding intolerance due to abdominal pain and nausea
Continued on full parenteral nutrition with resulting
cholestasis with max indirect/direct bilirubin of 4/2.5; bilirubin
on discharge normalized to 0.4/0.2
Electrolytes fairly stable and controlled through dialysis
Patient continued on calcitriol for vitamin D supplementation,
Ursodiol for cholestasis
Colonoscopy on 4/23/08 showed severe segmental
inflammation characterized by congestion, erythema, friability
and confluent ulcerations in the entire colon
Case #4 N.N. con’t: Workup at BCH
Case #4 N.N. con’t: Workup at BCH
GI/FEN con’t
CT angiography on 4/30/08 showed decreased mucosal
enhancement of colon, dilated small bowel, bleeding in left colon
and normal vessels
Abdominal US on 4/24/08 showed normal portal venous pressure
Endoscopy 5/29/08 showed diffuse moderately erythematous,
edematous and friable mucosa with no bleeding found in the
entire stomach
Sigmoidoscopy on 5/29 /09 showed patchy areas of severely
friable, erythematous, edematous mucosa with no bleeding
UGI on 6/2/08 showed terminal ileum narrowing in caliber with a
loss of the normal mucosal fold pattern, no discrete stricture
Hemodialysis catheter changed on admission
3x/wk HD schedule for several months
Erythropoeitin w/HD
2L/day fluid restriction
Evaluation for kidney transplant initiated
Hypothyroidism, likely from Hashimoto thyroiditis (Anti-TPO
elevated at 15.4 and Anti-thyroglobulin <20 on 5/24/08)
Started on Synthroid
Growth hormone started
Case #4 N.N. con’t: Workup at BCH
Case #4 N.N. con’t: Workup at BCH
Completed hypercoagulation workup without any inherited
coagulopathy or risk factors
Treated with subcutaneous heparin and transitioned to oral
warfarin starting 6/27/08
Consulted for hypogammaglobulinemia
Low vaccine titers, borderline low memory B cells (CD 27+), and
low T and B cells, and poor T cell response to tetanus antigen
Received IVIG therapy x2 for low levels, thought to have CVID,
work-up ongoing
Consulted due to concerns for vasculitis; autoimmune workup
Infectious Disease
Blood cultures remained negative
No evidence of abscess formation on multiple scans (4/23, 4/30,
5/21, 6/13/08)
CMV studies 5/23/08: shell antigen negative, IgG positive, IgM
negative, PCR negative
C. diff infection (positive on 4/13/08), treated with
metronidazole, and has remained negative thereafter
VRE swab positive 5/26, 6/3, and 6/6/08, all other cultures
Case #4 N.N. con’t: ICP Discharge
Case #4 N.N. con’t: Readmit 8/20/08
Discharged home on 7/26/08 with plan to return for OR in August
for exploratory laparotomy for persistent nausea and feeding
Parenteral nutrition (PN) cycled over 12 hours with restricted
clears diet by mouth
Discharge meds
Metoprolol, Prednisone 1 mg BID, Omeprazole, Zofran,
Ursodiol, Warfarin, Levothyroxine, Somatropin daily, Calcitriol,
PRN meds of Oxycodone, Promethazine, Nystatin powder,
Pre-operative UGI on 8/14/08
Distal jejunum and ileum
markedly abnormal,
featureless with multiple
Case #4 N.N. con’t: Readmit 8/20/08
Planned Ex-lap surgery 8/20/08
Two small bowel strictures, one 112 cm from Ligament of
Treitz and another 136 cm from Ligament of Treitz, at sites of
prior anastomoses
Other two anastamoses noted to be wide open and slightly
Extensive lysis of adhesions
Stricturoplasty x 2
5 cm Rxn of terminal ileum
ICV preserved
End-ileostomy creation
Case #4 N.N. con’t: Readmit 8/20/08
Hyperglycemia requiring sliding scale insulin, stopped upon
discharge home
HPA axis was evaluated per endocrine
AM cortisol 29.7 (high, but patient on prednisone), DHEAS low
at 28.4 (adrenals suppressed), ACTH 23 (normal)
On 9/22/08, repeat AM cortisol was 42; high-dose
Dexamethasone suppression test deferred
Requires stress dose steroids for procedures per endocrine
Discharged home on 9/23/08 on PN and Peptamen Jr. 1.5 @ 20
ml/hr x 12 hr nightly via GT, diet as tolerated by mouth; new
medication of sliding scale insulin for BG > 250
Case #4 N.N. con’t: Readmit 11/8/08
OR on 11/8/08
Ileoscopy with the GI team that showed two strictures,
consistent with pre-operative contrast study, and normal
looking bowel proximally
Ex-lap with tedious lysis of inflamed adhesions
A large diverticulum was noted at one of the stricturoplasty
sites just proximal to his ostomy, with the second stricture just
proximal to the stricturoplasty at the presumed anastamosis
Noted 60 cm abnormal bowel with flattening of normal folds;
left in place with potential for absorptive capacity; mesentery
very edematous
Resection of 25 cm of strictured small bowel with new endileostomy creation
At end of case, 118 cm of small bowel remaining
Case #4 N.N. con’t: Readmit 8/20/08
Planned Ex-lap surgery 8/20/08 con’t
Stamm gastrostomy tube placement and needle liver biopsy
144 cm of small bowel from Ligament of Treitz to ileostomy at
end of case
Pressor requirement from POD #1-POD #2, given stress dose
Wound dehiscence, abdominal CT on 9/5/08 showed large midline
abscess underlying surgical scar. Abscess drained by IR with drain
placement, drain removed 9/9/08. Treated with Vancomycin and
Zosyn x 7 days.
Liver biopsy showed mild steatosis, no fibrosis
Continued hemodialysis 3x/wk and IVIG infusions q 2 weeks
Case #4 N.N. con’t: Readmit 9/27/08
Abdominal pain, vomiting and increased ostomy output
CT with IV and PO contrast with no evidence of vascular
compromise or obstruction, but did show persistent anterior
abdominal abscesses and lesion concerning for a second stricture
proximal to the ostomy site
Contrast study with ileal stricture in the region of the ileostomy
Treated with IV antibiotics for persistent abscess and discharged
home on Moxifloxacin on 9/30/08
His nausea and abdominal pain persisted
Seen in surgical clinic outpatient and the plan was made to return
to the OR for full intraoperative enteroscopy to determine what
portions of bowel were functional and possible further bowel
Case #4 N.N. con’t: Readmit 11/8/08
Restarted enteral feeds on POD #4 and advanced slowly to rate of
Post-op course c/b mild wound infection, treated effectively with
Discharged home on 11/28/08 on PN and enteral feeds of
Peptamen 1.0 @ 20 ml/hr x 8 hr nightly via GT
Referred to the CAIR program (BILIs slightly bumped to 0.8/0.5;
abd u/s 11/7/08 w/enlarged echogenic liver, diffuse cortical
thinning of the kidneys)
CAIR managed his parenteral nutrition and enteral feeding
regimen outpatient
Per CAIR team started on pancreatic enzymes for malabsorption
Case #4 N.N. con’t: Readmits 4/09
Case #4 N.N. con’t: Readmits 3/9/10
and 3/19/10
Progressive renal failure
Left arm AV fistula graft on 4/9/09
Complicated by stenosis related to an old IV site
Revision on 6/25/09
Started aspirin to maintain patency
Readmit 4/15-4/29/09 for fever
CT scan with abdominal wall collection near ostomy, surgery
consulted but did not feel it needed intervention
Stomagram 4/21 showed no fistula, possible descending colon
Discharged home on 6 weeks of IV antibiotics (original cultures
not drawn from HD line) with plan for colonic manometry
Readmit 3/09-3/12/10 for abdominal pain
CT scan of the abdomen revealed an ileostomy stricture, a colonic
stricture at the splenic flexure, and a possible small bowel to colon
Plan for OR with GI to scope and general surgery for Ex-lap
OR 3/19/10
Colonoscopy with patchy area of moderately erythematous mucosa
up to the splenic flexure with blind end there
Extensive lysis of adhesions
Blind loop obstruction of the colon caused by the long segment
splenic flexure stricture splenic flexure stricture, resected
No connection between the small and large bowel was found
Ileum remnant scarred down so ileocecectomy was performed
He then had a jejunostomy creation and right colon mucous fistula
Case #4 N.N. con’t: Readmit 3/19/10
Case #4 N.N. con’t: 2010
Final pathology
Resected terminal ileum and strictured splenic flexure segment
c/w remote ischemia
Multiple samples of mesentery and intestine without evidence of
persistent vasculopathy
Post-op period unremarkable except for excessive ostomy output, for
which he was started on Loperamide
Discharged home on combination enteral feeds and PN on 3/26/10,
BILIs normalized 0.4/0.2
Persistent abdominal pain
Abdominal US on 5/6/10 showed no fluid collection or hernia, did
note interval slight worsening in appearance of the echogenic
kidney with parenchymal thinning and loss of corticomedullar
Readmitted 5/13/10-5/14/10 to GI service with bloody ostomy
Coagulation panel normal, other labs stable, KUB unchanged
Discharged home with plan for scopes by GI team
5/21/10 endoscopy unremarkable, ileoscopy with mild erythema
Abdominal CT on 5/26/10 showed no bowel loop dilatation or air
fluid levels seen to suggest obstruction, did note atrophic kidneys
Case #4 N.N. con’t: Transplant
Case #4 N.N. con’t: 2011-2012
Transplant referral 7/29/10 from CAIR clinic
Due to intestinal failure, dependence on parenteral nutrition, and
complication risk related to immunosupression associated with
isolated kidney transplant, combined small bowel and kidney
transplant was advised by the transplant team
LFTs remained stable outside of acute infections; thus liver
transplant was not pursued
N.N. with his family open to suggestion for transplant but wanted to
continued to continue to try to purse increasing enteral feeds while
weaning parenteral nutrition for the time being
Maintained on full PN
Worsening kidney disease, HD progressed to 6x/wk
Metoprolol stopped by cardiology outpatient; started on Midodrine
for hypotension
Several central line infections, required CVL removal and
replacement 1/11/11 and 1/14/11
Readmit 3/18/11- 4/13/11 for partial small bowel obstruction,
ultimately requiring ileostomy revision in situ on 3/25/11
CT in ED on 3/18/11 showed small atrophic kidneys with
progression of multiple hypoattenuating lesions
Indications for Intestinal Transplant
Intestinal failure
Short bowel syndrome is the most common reason for intestinal
transplantation in children
Every effort is made to optimize enteral nutrition and
decrease the need for parenteral nutrition in patients with
SBS to promote bowel rehabilitation and because of the risk
of liver damage
Unfortunately, some patients are not able to progress on
enteral feeds and become dependent on full PN with
resulting PNALD necessitating liver transplant as well
Congenital malformations, infections of the gastrointestinal
tract, absorptive impairment
There are three types of transplant for SBS
Intestinal Transplant
Isolated Small
Case #4 N.N. con’t: Decision to
Evaluate & Transplant Listing
UNOS: Listing Criteria
UNOS (United Network for Organ Sharing)
National transplant waiting list
In order to:
Confirm transplant is the best
Determine the urgency for
Assure a good organ match
Decision to pursue transplant option
in May of 2012
Listed for a combined kidney and
intestine transplant on 6/21/12
Transplant Evaluation
Small Bowel Transplant
Blood type, HLA typing
CBC w/diff, coags
Chem 10, LFTs w/GGTP +/Titers
HIV, Hepatitis A, B, C, HLA
+/- Tb, MMR IgG, RPR, Ammonia
CXR, KUB, Abd u/s w/Doppler
GI Endoscopy (upper and lower)
Upper GI series and barium enema
Abdominal CT &/or CTA abd/pelvis
+/- Motility studies
+/- Liver biopsy
Other (hypercoagulability workup
w/hx mesenteric venous thrombosis)
Surgery, Tx Coordinator
Hepatology/GI; Renal/Urology;
Dialysis Nurse
Infectious Disease
Transplant pharmacy
Social work
Child life specialist
Physical therapy
Financial coordinator
+/- Other comorbidity dependent
Difficult organ to transplant because
of its immunologic properties
Approximately 80% of immune cells
normally reside in the gut
After transplant the immune cells are
repopulated with recipient cells
However the genotype of the epithelium
remains that of the donor, making the graft
immunogenic and chimeric
Gut barrier can breached by ischemia or
reperfusion injury, recipient immune cells or
impaired microbial control mechanism
causing inflammation and tissue damage,
increasing chance of infection
Small Bowel Transplant con’t
A multivariate analysis of cases
within the last 5 years revealed
that transplantation of patients
waiting at home, recipient age,
antibody induction immune
suppression, and center
experience with at least 10 cases
were associated with improved
patient survival (Grant etal., 2003)
Case #4 N.N. con’t: BIG DAY
Called in for a deceased donor kidney and intestinal transplant on
Admitted to general surgery floor preop
Medication consideration, fever/sick, NPO
Possibility of Tx cancellation
Donor CMV positive, recipient negative
Donor EBV negative, recipient positive
Kidney Transplant
Most common etiology
Congenital renal and urologic anomalies
Renal allograft and patient survival rates have
increased in the past few years with
advancements in immunosuppressive therapy
Living donor transplants have increased graft
survival as compared to deceased donor renal
Important part of pre-transplant evaluation is
detection of anti-Human Leukocyte Antigen
(HLA) antibodies to the donor
Transplant Preop Orderset: Intestine
Admit, +/- PIV, full labs, T&S, ABORh, blood on hold (PRBC, PLT,
FFP), CXR, notify anesthesia/blood bank/radiology, GI fellow
Repeat serologies only if negative in the past 2 months
ABX on-call to OR
Campath 0.3mg/kg IV <66kg or 20mg IV >66kg
Premedications for Campath (GIVEN IN OR), frequent VS
Tylenol and Benadryl 30-60 minutes prior
Methylprednisolone 1mg/kg IV <50kg or 50mg IV >50kg 30-60
minutes prior
Zofran prn N/V; Meperidine prn shivering
Procurement of donor organ and enterectomy (+/- LOA)
Cross-clamping (ischemia times!)
Graft implantation: vascular then bowel anastomoses
To ICU postop
Transplant Preop Orderset: Kidney Specific:
Deceased Donor Steroid Avoidance Protocol
Case #4 N.N. con’t: BIG DAY con’t
Campath in OR for deceased donor, night before for living donor
Same dosing as for intestinal transplant, 0.3mg/kg IV <66kg
or 20mg IV >66kg
Premeds for Campath
Methylprednisolone dosing based on BSA: <1.67m2 IV 300
mg/m2, >/= 1.67m2 IV 500 mg
Tylenol and Benadryl
Vaclganciclovir on POD -1, <15kg: PO 7.5 mg/kg, >/= 15kg: PO
250 mg/m2
Zofran prn N/V, Meperidine prn shivering
Extensive lysis of adhesions was performed first.
Completion enterectomy (small segment at ligament of Treitz was
left for anastomosis)
Remaining colon was preserved (RUQ mucous fistula in place)
Mobilization of infrarenal aorta and vena cava for anastomosis
Small bowel graft was procured at the same time
Donor vessels were used to create two small grafts coming off the
infrarenal aorta and vena cava
Patient was fully heparinized
Intestinal graft was then brought up to the table
Case #4 N.N. con’t: BIG DAY con’t:
Small Intestine Tx
Case #4 N.N. con’t: BIG DAY con’t:
Small Intestine Tx con’t
Donor superior mesenteric artery (SMA) was anastomosed end-toside to the infrarenal aorta via an iliac artery donor graft
Donor superior mesenteric vein (SMV) was anastomosed end-toside to the infrarenal vena cava via a donor innominate vein graft
Arterial flow was restored to the graft
Several bleeding points in the upper mesentery of the graft, which
were controlled
The entire graft appeared well perfused
Donor proximal jejunum to the native jejunum end-to-end
Gastrojejunostomy tube passed well past this anastomosis while
being secured at the anastomosis
Distal graft ileum brought through the prior right lower quadrant
ileostomy site
Abdomen thoroughly irrigated with warm saline solution
Final inspection for hemostasis
Two Jackson-Pratt drains placed through right upper quadrant
incisions (at right retroperitoneum and near the jejunal
anastomosis on the left)
Abdomen was then closed
Case #4 N.N. con’t: BIG DAY con’t:
Kidney Tx
Case #4 N.N. con’t: BIG DAY con’t:
Kidney Tx con’t
LLQ prepped and draped
Incision was made in the left iliac fossa through the external
oblique and internal oblique and into the retroperitoneum
Due to multiple prior surgeries, the retroperitoneal space was
slightly difficult to get into, and surgeons did make a small hole in
the peritoneum
Donor kidney was a left kidney with single artery, single vein, and
single ureter
Surgeons mobilized the external artery and vein of the recipient
The donor renal vein anastomosed end-to-side to the recipient
external iliac vein
The donor renal artery was then anastomosed end-to-side to the
recipient external iliac artery
Small blood flush of the kidney prior to releasing the renal vein
The renal vein anastomosis was secured and the kidney reperfused nicely
Surgeons then filled the bladder via the Foley that had been
placed previously
Bladder was dissected with exposing a site on the lateral and
anterior portion for the ureteral anastomosis
The ureter was cut to the appropriate length and spatulated,
creating a diamond-shaped opening
Ureter then anastomosed to the bladder mucosa opening
The muscle was then closed over creating a long submucosal
tunnel to prevent reflux
Transplant Postop Orderset: Intestine
Transplant Postop Orderset: Kidney
To ICU postop
GJT to gravity, +/- NGT to LWS, JP x2 to bulb, Foley to gravity, stoma
to gravity
+/- CVP monitor, SCDs >25kg, +/- Heparin gtt
IVF +/- replacements, ABX periop
Morphine/Tylenol prn, PPI ppx
Frequent VS, labs q6 x3d, then q12 x2d, then qday
Abd u/s w/Doppler & CXR postop, +/- UGI plan
Immunossupression postop
Infection prophylaxis
Methylprednisolone taper
• Periop ABX
Prograf when able
• Fungal (Nystatin)
• PCP (Atovaquone)
• CMV (Ganciclovir,
Foley catheter x 5 days, JP x 1
Morphine prn, IV PPI
IVF for insensible losses
1:1 replacements with ½ NS for UOP and JP OP until tolerating fluids
Immunosuppression: Methylprednisolone until Prograf therapeutic
then tapered, Cellcept, Prograf
Infection ppx: Atovaquone (PCP), Ganciclovir then Valganciclovir
Frequent VS; labs post-op: q6hr x 48hr, q12hr x 48hr then daily
US with Doppler in PACU, Mag 3 scan on POD 1
• +/- HSV, VZV (Acyclovir)
Mainstay drug for intestinal and kidney transplants
Retrospective reviews (Fishbein etal., 2009; Grant etal., 2003)
Have shown increased graft survival rate over time, especially
with the use of Tacrolimus, which inhibits signal-1 activation
of T lymphocytes through the inhibition of calcineurin
One-year patient survival rates of 81% are being achieved
using antibody-based pretreatment/induction therapy and
tacrolimus-based maintenance immunosuppression
This is comparable to survival rate for liver transplants
Most common complication of Tacrolimus is renal damage
(Grant etal., 2003)
Transplant Complications
Surgical complications
Graft rejection
Graft dysfunction
Case #4 N.N. con’t: Post-Transplant
POD #0-2
Initially hypotensive; on pressors through the eve of POD #1
Resumed parenteral nutrition on POD #1
Ostomy with green liquid output
On IVF for insensible losses and 1:1 replacement of UOP and JP output
Made good urine post-op with serial labs showing slow decline of BUN
and creatinine
Mag 3 scan on POD #2 showed fair uptake of kidney, no obstruction, or
Continued Methylprednisone, Prograf and Cellcept started on the eve of
POD #0
Ganciclovir started on POD #0
Transferred to floor on eve of POD #2
Left arm edema on POD #2; non-occlusive thrombus in left basilic vein;
started on prophylactic lovenox per hematology team
Case #4 N.N. con’t: Immediately
Tolerated transplantation procedure well
Extubated at the end of the case
Immediate post-op ultrasounds
Patent vessels with good flows and low resistive
waveforms, no fluid collections
Limited 2/2 bandage
Visualized portions of the abdominal aorta and IVC patent
with good arterial and venous waveforms
The infrarenal anastomoses could not be visualized
No free fluid.
Case #4 N.N. con’t: Post-Transplant
POD #2-10
Ileal pluck biopsies performed frequently post transplant, all negative
for rejection immediately post-op
Weaned off steroids by POD #10 with therapeutic Prograf level
He continued Cellcept and Ganciclovir per protocol
Started on continuous Pedialyte via JT on POD #3, slowly advanced
over the next day to goal volume and transitioned to formula
After reaching goal feed volume via JT, he had acute increase in his
Gtube output, thus his enteral feeds were suspended for several days
UGI series on 1/22/13 showed patency of the graft with mild
An abdominal ultrasound that same day without intra-abdominal fluid
Case #4 N.N. con’t: 2-5 Weeks PostTransplant
Case #4 N.N. con’t: 2-5 Weeks PostTransplant con’t
Gtube output slowly decreased; enteral feeds were reinitiated
Persistent nausea with increasing enteral feeds; abdominal US on
2/2/13 showed increasing fluid collection
CT on 2/3/13 with large interloop abscess in right hemiabdomen
adjacent to distal ileum and ileostomy. Blood supply to intestinal
graft patent with no signs of poor perfused bowel.
Pigtail catheter placed by IR on 2/3/13; a lot of pain with the
tube and it was removed on 2/5 as the drainage was low and
collection too thick to pass through the catheter
Feeds advanced to goal thereafter
His goal minimum fluid intake of 4.5 liters/day
Of note, PN was discontinued on 2/17/13
Adequate urine output
Serum BUN and creatinine trending down (43/1.2)
Required Amlodipine daily for hypertension/nephroprotection
Developed hyperglycemia in the post-operative period
Requiring insulin (Lantus and Humalog)
Continued on Synthroid
Received 1 week of Lovenox for LUE clot, stopped per
hematology service recommendations
Required PRBC transfusion during the stay
Case #4 N.N. con’t: 2-5 Weeks PostTransplant con’t
Case #4 N.N. con’t: Discharge PostTransplant
Pluck biopsy on 2/4/13 with moderate rejection; treated with IV
pulse steroids 10mg/kg for 3 days, then slowly tapered over next
two weeks
Repeat pluck biopsy on 2/20/13 with no evidence of rejection
Transitioned from IV to oral steroids on 2/20/13 with plan for
Continued on cellcept and prograf
Infectious Disease
Transitioned to valganciclovir and started on Mepron for
On discharge CMV and EBV PCR were negative
Discharged home on 2/22/13, POD #40
Allowed regular diet by mouth with feeds of Peptamen 1.0 at 50cc/hr
x 10 hrs plus free water
Discharge meds: Prograf, Cellcept, Prednisone, Amlodipine, Protonix,
Baking Soda, Loperamide, Magnesium Oxide, ADEK, Humalog sliding
scale and carb coverage, Lantus, Levothyroxine, Nystatin,
Atovaquone, Valganciclovir
Enteral feeds weaned to off outpatient secondary to his advancement
of diet and ability to meet goal kcal and fluid requirements all by
Case #4 N.N. con’t: Readmission 3/43/26/13 (~2m post tx)
Case #4 N.N. con’t: Readmission 3/43/26/13 (~2m post tx) con’t
Concern for infected bilateral middle finger infection (blisters)
WBC 3, ANC 3.22
Area unroofed and dressed by plastic surgery
I&D on 3/4 and 3/6; dressing changes; wicks removed on 3/7
Wound cultures with abundant staph aureus
HSV, VZV DFA, culture and PCR all negative
Initially Vancomycin and Zosyn then switched to Cefazolin and IV
Acyclovir per ID recs
Transitioned to oral Keflex and then switched to Bactrim; stopped on
3/18/13 per ID recs
Course complicated by secretory diarrhea, stool culture +Norovirus
Received extra fluid of ~ 5 liters daily to compensate
He started on enteral IgG for norovirus on 3/13 for a 3 day course
Intestinal biopsies on 3/8, 3/13, and 3/20 were negative for rejection;
continued on steroid taper
Bedside scope on 3/21 appeared normal on exam but biopsies
showed focal mild rejection in the setting of norovirus
Course also complicated by fall on left knee, which became swollen
with a mobile knee cap and an effusion on XR. Ortho was consulted
and recommended a patellar brace and outpatient follow up.
Case #4 N.N. con’t: Readmission
4/12-4/14/13 (~ 3m post tx)
Case #4 N.N. con’t: Readmission
4/22-5/21/13 (~ 3-4m post tx)
Admit for fever and sepsis rule-out
Also note recent history of outpatient decrease in immunosuppression
secondary to +BK virus
Low WBC 4 and electrolytes with mild dehydration
Blood cultures ultimately negative and empiric antibiotics stopped
Bedside pluck biopsy of his ostomy showed > 6 apoptotic bodies but
no inflammation
CMV and EBV titers negative, repeat BK PCR pending on discharge
Prograf dosage increased on discharge
Note weaned off insulin upon admission, none required in-house
Case #4 N.N. con’t: Readmission
4/22-5/21/13 (~ 3-4m post tx) con’t
Case #4 N.N. con’t: Readmission 6/36/4/13 (~5m post tx)
Incisional hernia and abscess formation at his midline abdominal
incision; I&D with packing done
Culture with yeast and staph non-aureus; started on Fluconazole and
Pluck biopsy on 5/20 showed continued improvement with areas of
ulceration mixed with healthy regenerating mucosa
Final ileoscopy on 5/21 with no areas of ulceration but 1 area with
white fibrinous exudate; overall marked improvement
BK virus decreased to 365,615
High blood sugars in-house requiring restarting of sliding scale insulin
Discharged home on new immunosuppression of Leflunomide, Keflex
and Fluconazole to stop on 5/31/13, and sliding scale Humalog prn
Increased ostomy output
Biopsy showed 8-10 apoptotic bodies with concerns for rejection
Bedside ileoscopy showed resolving ulcerations, and appeared similar
in appearance to his last ileoscopy on 5/21/13
BK virus was resulted at 37, 786
Remained stable in-house, decision made to discharge home and
follow-up on biopsies outpatient
Biopsies did show ongoing rejection, for which he continued enteral
steroids and increased dose of Leflunomide
Repeat scope 6/24/13 showed active CMV, and was treated with BID
Valcyte 900mg
Admit for fever and friability of stoma
Intestinal biopsy positive for severe rejection
Started on pulse steroids, Zosyn and Valcyte
Renal biopsy consistent with mild rejection and evidence of BK virus
(1.2 million on 5/14); started on Leflunomide; Cellcept dose was
decreased by half and discontinued on 5/17
Serial intestinal biopsies next few days showing gradual improvement
of rejection on steroid treatment; thus the Zosyn and Valcyte were
stopped, he was restarted on oral Valgancyclovir
Scope on 5/16 showed healing ulcers and areas without rejection;
steroids transitioned to oral
Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx)
Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) con’t
Low grade fever, decreased hunger and film over ostomy; admit
sepsis rule out
Ileoscopy with a couple of areas of ulceration, biopsies were sent
Labs with WBC low at 0.6 with ANC 440, stable liver function tests
and creatinine
High dose Methylprednisone and empiric antibiotics started;
ultimately cultures negative so antibiotics stopped
Repeat scope on 7/15 with continued rejection so pulse steroids
Hospital course c/b RLE then LUE swelling and pain; u/s negative
Midline abdominal incision w/purulent drainage; culture positive for
candida (started on Fluconazole); wound con’t open; VAC applied and
ultimately discontinued on 8/1; persistent fluid collection on u/s 8/3;
bedside I&D (fascia intact)
Ileoscopies on HD #21, 27, and 30 (7/31, 8/6 and 8/9) with signs of
rejection found on 8/6; EBV and CMV PCR were negative
CMV stains of the ileal biopsies were positive and he was started on
IV ganciclovir
Per ID team, persistent CMV disease indicating resistance to
Ganciclovir; started on Cytogam and on renally dosed Foscarnet
Adenovirus PCR also returned positive (73k copies)
Abdominal midline wound required VAC reapplication on 8/9
Fluconazole was changed to Micafungin per ID recs
Received GCSF therapy for neutropenia until his ANC normalized
Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) con’t
Case #4 N.N. con’t: Readmission 7/99/7/13 (~6-8m post tx) con’t
VAC changes in OR until VAC discontinued on 8/16 and wet to dry
dressings instituted
RLE swelling and pain persisted, repeated US negative for DVT
MRI on 8/11 showed extensive subcutaneous/intramuscular edema,
bilateral lower extremity bone infarcts, and bilateral knee effusions
Orthopedics team was consulted, and saw no fluid collection
amenable to tapping
He continued on Micafungin for abdominal wound, Vancomycin and
Meropenem for lower extremity cellulitis, and Foscarnat and Cytogam
for CMV. Valganciclovir was stopped.
Bedside scope on 8/13 and 8/19 showed apoptosis and CMV but
overall improvement; steroids began to wean slowly
Viral studies on 8/19 with negative DFA and serum CMV, inconclusive
stool CMV, BK virus 1.5 million copies, Adenovirus 76K in the blood.
Viral studies on 8/27 showed: CMV and adeno PCRs negative, BK
virus 739,097 copies; viral shell for CMV and wound fungal culture
Vancomycin and Micafungin stopped on 8/30
Meropenem stopped on 9/3 with resolution in RLE swelling
Bedside scope on 9/3 grossly improved with the ulcer @ 15cm
appearing much smaller. Biopsies were negative for rejection,
showed an improvement in the inflammation, and CMV; adenovirus
was negative
Case #4 N.N. con’t: Readmission
10/18-12/30/13 (~ 9-11m post tx)
Case #4 N.N. con’t: Readmission 10/1812/30/13 (~ 9-11m post tx) con’t
Increasing abdominal pain
Abdominal US showed multiple fluid collections in the SQ tissue in the
anterior abdominal wall at the level of the wound packing
Started on Zosyn
HD #1: Aspiration of one collection in IR with culture ultimately
yielding Candida Albicans
Ileoscopy and biopsies on HD #1 unremarkable and biopsies with no
I&D of midline abdominal wound abscess on 10/21; VAC applied
Culture grew out candida (sent to an outside laboratory for further
sensitivity testing; sensitive to Micafungin)
Over the next couple of weeks, multiple OR wound washouts and VAC
Wound culture from 11/7 returned negative for candida and
micafungin was stopped
Later wound culture showed GNR; started on Zosyn on 11/13
Serum PCR positive for BK virus, CMV, and EBV; CMV also positive
from stains on intestinal biopsies, including his native stomach; thus,
he was started on foscarnet
Zosyn course x 2 weeks and samples were sent for CMV genotype
testing per ID recs (resistance to Foscarnet; stopped on 11/21)
Enrolled in Maribavir study at BWH to treat his CMV per ID team
Repeat CMV PCR on 11/29 and 12/5 negative; BK virus positive on
12/4 at 97, 000
Repeat scope 11/18 with persistent non-bleeding ulcers in gastric
antrum, but overall mucosa looked better
Case #4 N.N. con’t: Readmission 10/1812/30/13 (~ 9-11m post tx) con’t
Case #4 N.N. con’t: 12/30/13 Discharge
and Follow-up Outpatient After
Finally, VAC changes were done at bedside with premedication.
Hospital course c/b hypertension, for which his Amlodipine was
increased and he was started on hydrochlorothiazide
Serum labs showed stable BUN and creatinine
GT site continued to leak; definitive GT closure on 12/10 with an
endoscopy and VAC dressing change
Overall improvement with ongoing ulceration of the stomach;
ileostomy biopsies showed: CMV in the gastric antrum only
VAC placed at old gastrostomy site
Abdominal site additional purulent fluid; culture positive for candida
albicans; Micafungin was restarted
Finally discharged home with double wound VAC in place
Discharge meds: Leflunomide, Prednisone, Maribavir, Valganciclovir,
Atovaquone, Valtrex, Nystatin, Micafungin, Amlodipine,
Hydrochlorothiazide, Pantoprazole, Loperamide, Levothyroxine,
Humalog sliding scale, Lantus, Vitamins, Potassium Phosphate,
Magnesium Oxide, and Zinc Sulfate
VAC changes outpatient once weekly coordinated with his Maribavir
He will continue on Maribavir as prophylaxis per ID team at BCH
Seen in clinic 1/13/14 and prednisone decreased
Clinic 1/21/14 with no significant changes
Thank You!
Have fun in Boston!
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Other Sources
Boston Children’s Hospital Experts & Website
National Guideline Clearinghouse