1. No category

Overview
Mucoceles of the
Paranasal Sinuses
Francis T.K. Ling, MD BSc
Department of Otolaryngology – Grand Rounds
University of Ottawa
Wednesday, January 28th 2004
•
•
•
•
•
•
Anatomy and Development
Physiology and Pathophysiology
Epidemiology
Clinical Features
Treatment
Case Presentations
Introduction
Introduction
• Definition:
• Mucoceles known for > 100 years
• Epithelial lined mucous-containing sac completely filling a
paranasal sinus
• Capable of expansion by virtue of bone resorption and new bone
formation
• 1725:
• 1818:
Dezeimeris first described frontal mucoceles
Langenbeck commented on clinical complaints and
symptoms
• “hydatids”
• 1890:
Rollett introduced the term “mucocele”
• Most common lesion causing expansion of paranasal
sinuses
Anatomy and Development
Anatomy and Development
•
•
•
•
• Maxillary Sinuses
Maxillary sinuses
Ethmoid sinuses
Sphenoid sinus
Frontal sinuses
• Occupies body of maxilla
• First to develop in the
human fetus
• Biphasic growth:
• 3 years
• 7 years to adolescence
• Average volume 14.75 ml
• Drains into middle meatus
via maxillary ostium
1
Anatomy and Development
Anatomy and Development
• Ethmoid Sinuses
• Sphenoid sinus
• Located in superior half of
lateral nasal wall
• Development begins during 3rd4th month of fetal development
• Continue to grow through
childhood until age 12
• Average volume 15 ml
• Drainage:
• Anterior: infundibulum or
ethmoid bulla
• Posterior: superior meatus
•
•
•
•
In body of sphenoid bone
No significant sinus at birth
Development begins at 5 years
Final volume attained by 12-15
years
• Average volume: 7.5 ml
• Drainage:
• Sphenoethmoidal recess
Anatomy and Development
Anatomy and Development
• Frontal Sinuses
• Frontal recess
• Frontal bone
• Begins as evagination of frontal
recess
• Development begins at 2 ya and
reaches adult size at 15-20 ya
• Variable development:
• 10% unilateral
• 5% rudimentary
• 4% absent
• Drainage into frontal recess
• 2-20 mm in length
• Marked variation in configuration and attachment of uncinate
process
• Variable drainage patterns of frontal recess
Physiology
Physiology
• Sinus lining:
• Pattern of clearance:
• Ciliated, pseudostratified,
columnar epithelium
• Mucous glands and goblet
cells mucous blanket
• “sol-gel” phase
• Maxillary: floor
stellate pattern along walls
to natural ostium
• Frontal: inward flow
medially superior
lateral floor
frontal
recess
2
Pathophysiology
Pathophysiology
• Obstruction of sinus ostium or outflow tract
• Bone resorption:
•
•
•
•
Inflammation (ie. Chronic sinusitis)
Trauma
Iatrogenic (eg. FESS)
Mass/Tumour (eg. Polyps, ostioma, malignancy, ostioma)
• Obstruction of minor salivary gland located within lining
of paranasal sinus
• Eg. Mucous retention cyst of maxillary sinus
• Epithelium continues to secrete
causing expansion of the mucocele
• Increased pressure
devascularization of bone and
osteolysis
• Local inflammation
secretion of
cytokines
• Fibroblasts PGE2 + IL-1
• Epithelial cells TNF alpha
• Cause osteoclastic bone
resorption
Epidemiology
Epidemiology
•
•
•
•
• Rombaux et al (Belgium, 2000):
3rd or 4th decade
M:F ~ 7:1
10-15 years to develop
Frontal > ethmoid > maxillary > sphenoid
•
•
•
•
•
Fronto-ethmoidal ~65%
Maxillary ~ 20%
Sphenoid ~1-8%
Posterior ethmoid ~1-6%
Uncommon locations: middle turbinate, pterygomaxillary space
•
•
•
•
178 mucoceles
Primitive mucoceles: 35%
Post-traumatic: 2.1%
Post-operative: 62.9%
• Incidence after FESS not known
Clinical Presentation
FrontoFronto-ethmoidal Mucocele
• Slow expansion
• Most common clinically significant mucocele
• Classification (Har-El, 2001)
• Patients asymptomatic for many years
• May take 10 years or more to become symptomatic
• Symptoms depend on location/type of mucocele and extent
of bony erosion
• In general:
• Headache and facial pressure common
• Facial swelling with tenderness to palpation
• Ocular and neurological problems
• Type 1:
• Type 2:
• Type 3:
Limited to frontal sinus (+/- orbital extension)
Frontoethmoid mucocele (+/- orbital extension)
Erosion of posterior wall
• A. Minimal or no intracranial extension
• B. Major intracranial extension
• Type 4
• Type 5
Erosion of anterior wall
Erosion of both posterior and anterior wall
• A. Minimal or no intracranial extension
• B. Major intracranial extension
3
FrontoFronto-ethmoidal Mucocele
FrontoFronto-ethmoidal Mucocele
• General:
• Ocular:
• Frontal headache (common) and/or deep nasal pain
• Frontal swelling +/- infection/draining fistula
• Nasal obstruction and rhinorrea unusual
• Proptosis (common)
• Periorbital pain
• Displacement of globe
downward and outward
direction
• Reduced ocular mobility
• Diplopia
FrontoFronto-ethmoidal Mucocele
Maxillary Mucocele
• Neurologic:
• “mucous-retention” cyst
•
•
•
•
•
Destruction of posterior frontal sinus wall
Decreased LOC
Confusion
Meningitis
CSF leak
• Incidental finding
• Rarely achieve sufficient size to cause bony erosion
• Rarely require specific therapy if asymptomatic
• Spontaneous regression without therapy
Sphenoid Mucocele
Sphenoid Mucocele
• Rare lesion
• Extension:
•
General:
•
Ocular:
•
•
•
•
Superiorly into pituitary fossa
intracranial
Posteriorly towards clivus
Anteriorly into posterior ethmoids
Laterally into orbits
• Compression:
• Pituitary gland, optic chiasm, carotid artery, cavernous sinus, CN
III-VI, brain
• Headache with occipital, vertex or deep nasal pain
•
•
•
•
•
Diplopia
Visual field disturbance
Vision loss
Retro-orbital pain
Neurologic:
•
•
•
•
Decreased LOC
Confusion
Meningitis
CSF leak
4
Investigations
Investigations
• CT scan provides excellent anatomical information
• Findings:
• MRI scan:
•
•
•
•
Completely opacified sinus cavity
Thinned and expanded sinus walls
Loss of normal scalloped margin
Depression or erosion of supra-orbital ridge and extension of soft
frontal tissue mass across midline
• Allows differentiation of mucoceles from solid component of
neoplasms or meningoencephalocele
• Demarcates mucocels and soft-tissue structures in the event of
intracranial or intraorbital growth
• Findings:
• Signal intensity vary depending on state of hydration and age
• Majority show hyperintense T2 and hypointense T1
• Increased dehydration
T2 become hypointense and T1 become
hyperintense
Treatment
External Approaches
• Surgery is required
• Operate on non-infected mucocele unless acute
symptomatic mucopyocele
• Goals
• Traditionally preferable when there are intraorbital or
intracranial manifestations
• Typically for fronto-ethmoidal mucoceles
• Techniques:
• Reintegration of affected sinus into nasal circuit
• Sinus exclusion with obliteration and respect of posterior wall
• Cranialization
• Approaches
• External
• Endoscopic
• Combined
• External frontoethmoidectomy
• Lynch
• Killian
• Reidel
• Lothrop
• Osteoplastic flap
External Frontoethmoidectomy
External Frontoethmoidectomy
• Indications:
• Technique (Lynch)
• Acute infectious of frontal and ethmoid sinuses with orbital
extension
• Mucoceles, pyoceles, cutaneous fistulae and CSF leaks, or
intracranial complications from fronto-ethmoidal sinuses
• Exposure for benign tumours of fronto-ethmoidal sinuses, anterior
skull base, or superior nasal cavity
• Incision made near medial
orbital rim; avoid damage to
medial canthal ligament and
trochlea
5
External Frontoethmoidectomy
External Frontoethmoidectomy
•
• Killian procedure
Technique (Cont’d)
• Periosteum elevated to fronto-ethmoid
suture
• Anterior ethmoid artery divided
• Lamina papyracea removed and
ethmoidectomy performed
• Frontal sinus opened in medial part of
floor
• Diseased tissue within sinus is removed
• Large chute from frontal sinus through
ethmoid cavity into the nose
• +/- stent placement
• For tall sinuses in which
disease cannot be removed
through floor alone
• Floor and anterior wall
removed
• Supraorbital bony strut (10
mm)
External Frontoethmoidectomy
External Frontoethmoidectomy
• Reidel procedure:
• Lothrop procedure:
• Entire anterior wall and floor
of frontal sinus removed
• Mucosa removed
• Sinus obliteration
forehead soft tissue laid
against posterior table
• Significant deformity
• Rarely if ever used
• Unilateral or bilateral anterior ehtmoidectomy
• Interfrontal septum and superior nasal septum and frontal recesses
connected
• High risk of cribriform plate damage:
• Anosmia
• CSF leak
• Meningitis
Osteoplastic Flap
Osteoplastic Flap
• 1894: described by Brieger
• Fat obliteration:
• Incisions:
• First described in 1950 by Bergara
• Prevent recurrence
• Associated with varying degree of
necrosis and resorption
• Coronal approach
• Midline forehead approach
• Brow incision
• Indications:
• Neoplasms
• Fractures
• Chronic frontal sinusitis associated
with orbital or intracranial
complications
6
Osteoplastic Flap
Osteoplastic Flap
• Technique:
• Technique:
• Skin-tissue flap raised,
preserving periosteum and
supraorbital nerves
• Perimeter of frontal sinus
marked with template from
Caldwell-view radiograph
• Periosteum incised and lifted
off bone
• Bone cuts made to create
osteoplastic flap
Osteoplastic Flap
Osteoplastic Flap
• Technique:
• Technique:
• Bone flap removed
• Disease in frontal sinus
removed
• Mucosa lining stripped and
drilling of cortical bone
performed
• Minimum 2 mm
required to eliminate all
mucosal elements
• Mucosa lining stripped and
drilling of cortical bone
performed
• Minimum 2 mm
required to eliminate all
mucosal elements
Osteoplastic Flap
Osteoplastic Flap
• Technique:
• Technique:
• Once frontal recess reached,
mucosa is inverted down
toward nasal cavity
• Fat harvested from lower left
quadrant of the abdomen
over rectus muscle used to
obliterate sinus cavity
• Frontal recess is plugged
with fascia, muscle or bone
• Bone flap replaced and fixed
• Periosteum closed
• Skin closure
7
Osteoplastic Flap
Osteoplastic Flap
• Cranialization
• Complications:
• Indications:
• Large portions of posterior frontal sinus destroyed with
substantial epidural spread of mucocele
• Intracranial complications present
• Frontal craniotomy usually required
• Extradural dead space remains for extensive mucoceles
• Dead space obliterates by frontal brain over several weeks
• Oblteration of dead space by abdominal fat used to achieve
immediate closure and to avoid scarred adhesions
• Fat donor site:
• Seroma
• Hematoma
• Abscess
• Cellulitis
• Intracranial:
• Dural tears
• Frontal lobe injury
• CSF leaks
• Meningitis
• Brain abscess
Osteoplastic Flap
Osteoplastic Flap
• Complications (Cont’d):
• Complications (Cont’d):
• Ocular:
• Extraocular muscle injury
• Globe injury
• Hemorrhage retrobulbar hematoma
• Infection:
• Fat graft
• Osteomyelitis of bone flap
diplopia/blindness
• Nerve injury:
• Supraorbital nerves forehead paresthesia, hypoesthesia or
anaesthesia
• Facial nerve loss of frontalis function
• Olfactory nerve anosmia
• Cosmesis:
• Scar
• Depression or embossment
• Recurrence
External Approaches
Endoscopic Approach
• Recurrence:
• Introduced in 1980 by D.W. Kennedy
• “marsupialization”:
• Lund (1998):
• 28 patients with combined approach (Lynch)
• Recurrence rate: 11%
• Weber (2000):
• Osteoplastic flaps for various reasons
• 59 patients
• Mucoceles after procedure: 9.8% (5 patients)
• Conboy and Jones (2003)
• 23 patients with external (Lynch) or combined approach
• 26% recurrence
• Opening enlarged without complete removal of mucosal lining
• Lund (1991):
• Sinus lining returns to normal with re-establishment of
mucociliary activity
• Advantages:
• Short hospital stay
• No facial scarring
8
Endoscopic Approach
Endoscopic Approach
• Contraindications (Rombaux et al, 2000)
• Technique:
• Absolute:
• Mucocele not accessible to endoscope
• Mucocele located in external part of frontal or maxillary sinus
• Cutaneous fistula
• Relative:
• Loss of anatomical landmarks
• Revision surgery for recurrence lateral to frontal recess after
previous external approach
• Frontal recess stenosis with hypertrophic bone occluding area
• Associated disease (ie. Malignancy, large benign tumour)
• Polyps or polypoid
mucosa cleared
from frontal recess
Endoscopic Approach
Endoscopic Approach
• Technique:
• Technique:
• Identification of anterior
ethmoid artery
• Posterior reference
• Frontal opening located
2-4 mm anterior
• Agger nasi
cells removed
Endoscopic Approach
Endoscopic Approach
• Technique:
• Technique:
• Enlargement
anteriorly and
anteriormedially to
avoid accidental
intracranial entry
• Mucosa covering
posterior aspect of
frontal sinus
preserved
• Provides source of
epithelialization
9
Endoscopic Approach
Endoscopic Approach
• Technique (Cont’d)
• Postoperative Care:
•
•
•
•
Floor of frontal sinus anterior to outflow tract removed
Mucocele identified, opened and drained
Lining not curetted or removed
+/- stent insertion
• Antibiotics and saline spray
• Irrigation of stent
• Removal of stent 6-12 weeks after surgery
Endoscopic Approach
Endoscopic Approach
• Results:
• Results (Cont’d):
• Many studies show recurrence rates at or close to 0%
• Rombaux et al;Acta Oto-Rhino-Laryngologica Belg. 54:115-122,
2000
• 178 patients with 3 recurrences
• 97.9% successful
• Lund et al; J. Laryngol. Otol. 112(1): 36-40, 1998
• No recurrences in 20 patients
• Mean follow-up 34 months
• Har-El; Laryngoscope 111:2131-2134, 2001
• 108 sinus mucoceles
• 66 frontal and frontoethmoidal, 17 ethmoid, 7 sphenoethmoid,
12 sphenoid, 6 maxillary mucoceles
• 83% intraorbital extension
• 55% erosion of skull base with varying degrees of intracranial
extension; 31% major intracranial extension (intracranial
extent larger than sinus
• Follow-up: 1-13.5 years; median 4.5 years
• Recurrence of frontal mucocele in 1 patient (0.9%)
Endoscopic Approach
External vs. Endoscopic Approaches
• Results (Cont’d):
• Traditional teaching:
• Conboy and Jones; Clin. Otolaryngol. 28:207-210, 2003
• 68 mucoceles
• 66% endoscopic, 22% external, 12% combined
• Mean follow-up 6 years
• Recurrences:
• 9% endoscopic group
• 26% external or combined group
• Complete removal of mucocele lining
• Required external techniques
• Recent trend favouring endoscopic approach
• Marsupialization for large mucoceles controversial
• Long-term follow-up required
• Results of studies may not be final
• Follow-up in many series is short
10
External vs. Endoscopic Approaches
FollowFollow-up
• “small, well-positioned mucoceles may be attempted first
endoscopically, but in the setting of massive mucoceles
with risk of imminent complications and instability of the
facial skeleton, the more conservative approach may be the
more aggressive open techniques”
• “endoscopic transnasal approach best choice for
intracranially extended mucoceles because it is the least
invasive and can provide an adequate surgical view for
wide marsupialization”
• Mucoceles may recur many years after surgery
Case Presentation #1
Case Presentation #1
• Recurrences may be as long as 49 years after initial surgery
(Moriyama)
• Recurrences should be treated as early as possible
• 69 yo M
• Pituitary tumour removed 25 years ago
• Follow-up MRI incidental left frontal mucocele
• No orbital or intracranial extension
• Asymptomatic with no sinus complaints
Case Presentation #1
Case Presentation #2
• Dx: Frontal mucocele
• Treatment:
•
•
•
•
•
•
• Endoscopic removal of left frontal sinus mucocele
• Marsupialization and aspiration of thick fluid
• Well postoperatively
• No complications
• No recurrence
72 yo F
Referred from ophthalmology
Decreased vision of left eye
Left retro-orbital pain
No sinus symptoms
Rhinoscopy: normal
11
Case Presentation #2
Case Presentation #2
Case Presentation #2
Case Presentation #2
• Dx: sphenoethmoidal mucocele
• Treatment:
• Treatment (Cont’d):
•
•
•
•
• Marsupialization of
posterior ethmoid cells
• Removal of anterior and
inferior walls
Left functional endoscopic sinus surgery
Uncinectomy
Anterior ethmoidectomy
Posterior ethmoidectomy greenish fluid expelled and drained
Case Presentation #2
Case Presentation #3
•
•
•
•
•
•
•
•
Well postoperatively
Reduced pain
Vision still decreased
No recurrence at 4
months
73 yo M
History of chronic sinusitis
Previous septoplasty
Admitted for nausea and vomiting, dehydration, frontal
headaches and diplopia
• Previously on antibiotics and pain medication with no
improvement in symptoms
12
Case Presentation #3
Case Presentation #3
Case Presentation #3
Case Presentation #3
• Dx: sphenoethmoidal
mucocele
• Treatment:
• FESS
• Middle turbinate fractured to
expose large cystic formation
• Aspiration of purulent
secretions
• Marsupialized
• Dehiscence of LP
Case Presentation #3
Case Presentation #3
• Discharged home
• Returned to ER with progressive headache, nausea,
vomiting and dehydration
• CT report:
• Repeat CT scan
• “area of calcification in planum sphenoidale. It is uncertain
whether this is related to the mucocele, or possibly represents an
underlying meningioma”
13
Case Presentation #3
Case Presentation #3
• Repeat MRI
Case Presentation #3
Case Presentation #4
• Dx: Tuberculum sellae meningioma
• 49 yo M
• Progressive proptosis of
right eye
• No visual deficits
• Investigations:
• Involving:
• Pituitary gland
• Both cavernus sinuses
• Compression of left optic nerve
• Endocrinology: no endocrinopathy
• Ophthalmology: mild left visual field defect
• Patient not interested in craniotomy for biopsy or
decompression
• Will be followed regularly
• Large right frontal sinus
lesion
• Extension into orbit and
intracranial cavity
Case Presentation #4
Case Presentation #4
• Dx: Right frontal mucocele
• Treatment:
• Treatment (Cont’d)
• Combined ENT, Ophthalmology and Neurosurgery removal
• Osteoplastic flap
• Brow incision
• Supraorbital nerve cut for exposure
• Template osteoplastic flap raised
mucocele evacuated
• Roof of orbit and posterior sinus wall eroded
• Mucocele lining removed, sinus walls burred
• Osteoplastic flap:
• Dura dehiscent anteriorly with exposed brain dural patch
• Orbital roof defect reconstructed
• Frontal recess plugged, sinus obliterated with fat and Tisseel
• Bone flap replaced
14
Case Presentation #4
Summary
• Post-op
• Mucoceles most common lesion causing expansion of
paranasal sinuses
• Long asymptomatic progress
• When symptomatic, usually present with ocular symptoms
+/- neurologic symptoms depending on location of
expansion
• Fronto-ethmoidal mucoceles most common
• Caused by sinus obstruction secondary to chronic
infection, surgery or trauma
•
•
•
•
•
•
•
Accumulation of CSF under right forehead scalp
No rhinorrhea
Bed rest and aspiration of fluid
Persistent leak lumbar drain
Resolution of CSF leak
No infection
Discharge home
• Follow-up
• Well with no recurrence
Summary
• Treatment is surgical
• Traditionally, complete removal advocated via external approach
• Trend towards endoscopic management
• External or combined approaches usually reserved for extensive
involvement or failed endoscopic attempt
• Push towards endoscopic management of large intracranial
mucoceles
• Long term follow-up required to monitor for recurrence
15