1 Mucoceles Mucoceles of the of the Paranasal Paranasal Sinuses Sinuses Francis T.K. Ling, MD BSc Department of Otolaryngology – Grand Rounds University of Ottawa Wednesday, January 28 th 2004 Overview Overview • Anatomy and Development • Physiology and Pathophysiology • Epidemiology • Clinical Features • Treatment • Case Presentations Introduction Introduction • Definition: • Epithelial lined mucous-containing sac completely filling a paranasal sinus • Capable of expansion by virtue of bone resorption and new bone formation Introduction Introduction • Mucoceles known for > 100 years • 1725: Dezeimeris first described frontal mucoceles • 1818: 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 • Ethmoid sinuses • Sphenoid sinus • Frontal sinuses Anatomy and Development Anatomy and Development • Maxillary 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 2 Anatomy and Development Anatomy and Development • Ethmoid Sinuses • Located in superior half of lateral nasal wall • Development begins during 3 rd - 4 th 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 Anatomy and Development Anatomy and Development • Sphenoid sinus • 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 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 Anatomy and Development Anatomy and Development • Frontal recess • Marked variation in configuration and attachment of uncinate process • Variable drainage patterns of frontal recess Physiology Physiology • Sinus lining: • Ciliated, pseudostratified, columnar epithelium • Mucous glands and goblet cells  mucous blanket • “sol-gel” phase Physiology Physiology • Pattern of clearance: • Maxillary: floor  stellate pattern along walls to natural ostium • Frontal: inward flow medially  superior  lateral  floor  frontal recess 3 Pathophysiology Pathophysiology • Obstruction of sinus ostium or outflow tract • 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 Pathophysiology Pathophysiology • Bone resorption: • 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 • 3 rd or 4 th 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 Epidemiology Epidemiology • Rombaux et al (Belgium, 2000): • 178 mucoceles • Primitive mucoceles: 35% • Post-traumatic: 2.1% • Post-operative: 62.9% • Incidence after FESS not known Clinical Presentation Clinical Presentation • Slow expansion • 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 Fronto Fronto - - ethmoidal Mucocele ethmoidal Mucocele • Most common clinically significant mucocele • Classification (Har-El, 2001) • Type 1: Limited to frontal sinus (+/- orbital extension) • Type 2: Frontoethmoid mucocele (+/- orbital extension) • Type 3: Erosion of posterior wall • A. Minimal or no intracranial extension • B. Major intracranial extension • Type 4 Erosion of anterior wall • Type 5 Erosion of both posterior and anterior wall • A. Minimal or no intracranial extension • B. Major intracranial extension 4 Fronto Fronto - - ethmoidal Mucocele ethmoidal Mucocele • General: • Frontal headache (common) and/or deep nasal pain • Frontal swelling +/- infection/draining fistula • Nasal obstruction and rhinorrea unusual Fronto Fronto - - ethmoidal Mucocele ethmoidal Mucocele • Ocular: • Proptosis (common) • Periorbital pain • Displacement of globe downward and outward direction • Reduced ocular mobility • Diplopia Fronto Fronto - - ethmoidal Mucocele ethmoidal Mucocele • Neurologic: • Destruction of posterior frontal sinus wall • Decreased LOC • Confusion • Meningitis • CSF leak Maxillary Maxillary Mucocele Mucocele • “mucous-retention” cyst • Incidental finding • Rarely achieve sufficient size to cause bony erosion • Rarely require specific therapy if asymptomatic • Spontaneous regression without therapy Sphenoid Sphenoid Mucocele Mucocele • Rare lesion • Extension: • 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 Sphenoid Sphenoid Mucocele Mucocele • General: • Headache with occipital, vertex or deep nasal pain • Ocular: • Diplopia • Visual field disturbance • Vision loss • Retro-orbital pain • Neurologic: • Decreased LOC • Confusion • Meningitis • CSF leak 5 Investigations Investigations • CT scan provides excellent anatomical information • Findings: • 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 Investigations Investigations • MRI scan: • 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 Treatment • Surgery is required • Operate on non-infected mucocele unless acute symptomatic mucopyocele • Goals • Reintegration of affected sinus into nasal circuit • Sinus exclusion with obliteration and respect of posterior wall • Cranialization • Approaches • External • Endoscopic • Combined External Approaches External Approaches • Traditionally preferable when there are intraorbital or intracranial manifestations • Typically for fronto-ethmoidal mucoceles • Techniques: • External frontoethmoidectomy • Lynch • Killian • Reidel • Lothrop • Osteoplastic flap External External Frontoethmoidectomy Frontoethmoidectomy • Indications: • 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 External External Frontoethmoidectomy Frontoethmoidectomy • Technique (Lynch) • Incision made near medial orbital rim; avoid damage to medial canthal ligament and trochlea 6 External External Frontoethmoidectomy Frontoethmoidectomy • 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 External External Frontoethmoidectomy Frontoethmoidectomy • Killian procedure • For tall sinuses in which disease cannot be removed through floor alone • Floor and anterior wall removed • Supraorbital bony strut (10 mm) External External Frontoethmoidectomy Frontoethmoidectomy • Reidel 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 External External Frontoethmoidectomy Frontoethmoidectomy • Lothrop procedure: • 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 Osteoplastic Flap Flap • 1894: described by Brieger • Fat obliteration: • First described in 1950 by Bergara • Prevent recurrence • Associated with varying degree of necrosis and resorption • Indications: • Neoplasms • Fractures • Chronic frontal sinusitis associated with orbital or intracranial complications Osteoplastic Osteoplastic Flap Flap • Incisions: • Coronal approach • Midline forehead approach • Brow incision 7 Osteoplastic Osteoplastic Flap Flap • Technique: • Skin-tissue flap raised, preserving periosteum and supraorbital nerves • Perimeter of frontal sinus marked with template from Caldwell-view radiograph Osteoplastic Osteoplastic Flap Flap • Technique: • Periosteum incised and lifted off bone • Bone cuts made to create osteoplastic flap Osteoplastic Osteoplastic Flap Flap • 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 Osteoplastic Osteoplastic Flap Flap • Technique: • Mucosa lining stripped and drilling of cortical bone performed • Minimum 2 mm required to eliminate all mucosal elements Osteoplastic Osteoplastic Flap Flap • 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 Osteoplastic Osteoplastic Flap Flap • Technique: • Bone flap replaced and fixed • Periosteum closed • Skin closure 8 Osteoplastic Osteoplastic Flap Flap • Cranialization • 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 Osteoplastic Osteoplastic Flap Flap • Complications: • Fat donor site: • Seroma • Hematoma • Abscess • Cellulitis • Intracranial: • Dural tears • Frontal lobe injury • CSF leaks • Meningitis • Brain abscess Osteoplastic Osteoplastic Flap Flap • Complications (Cont’d): • Ocular: • Extraocular muscle injury • Globe injury • Hemorrhage  retrobulbar hematoma  diplopia/blindness • Infection: • Fat graft • Osteomyelitis of bone flap Osteoplastic Osteoplastic Flap Flap • Complications (Cont’d): • 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 External Approaches • Recurrence: • 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 Endoscopic Endoscopic Approach Approach • Introduced in 1980 by D.W. Kennedy • “marsupialization”: • 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 9 Endoscopic Endoscopic Approach Approach • Contraindications (Rombaux et al, 2000) • 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) Endoscopic Endoscopic Approach Approach • Technique: • Polyps or polypoid mucosa cleared from frontal recess Endoscopic Endoscopic Approach Approach • Technique: • Identification of anterior ethmoid artery • Posterior reference • Frontal opening located 2-4 mm anterior Endoscopic Endoscopic Approach Approach • Technique: • Agger nasi cells removed Endoscopic Endoscopic Approach Approach • Technique: • Enlargement anteriorly and anteriormedially to avoid accidental intracranial entry Endoscopic Endoscopic Approach Approach • Technique: • Mucosa covering posterior aspect of frontal sinus preserved • Provides source of epithelialization 10 Endoscopic Endoscopic Approach Approach • Technique (Cont’d) • Floor of frontal sinus anterior to outflow tract removed • Mucocele identified, opened and drained • Lining not curetted or removed • +/- stent insertion Endoscopic Endoscopic Approach Approach • Postoperative Care: • Antibiotics and saline spray • Irrigation of stent • Removal of stent 6-12 weeks after surgery Endoscopic Endoscopic Approach Approach • Results: • 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 Endoscopic Endoscopic Approach Approach • Results (Cont’d): • 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 Endoscopic Approach Approach • Results (Cont’d): • 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 External vs. External vs. Endoscopic Endoscopic Approaches Approaches • Traditional teaching: • 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 [...]... Follow- • “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... 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. .. 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... 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... 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... • • 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... 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... 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 ... 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 . 1 Mucoceles Mucoceles of the of the Paranasal Paranasal Sinuses Sinuses Francis T.K. Ling, MD BSc Department of Otolaryngology –. well-positioned mucoceles may be attempted first endoscopically, but in the setting of massive mucoceles with risk of imminent complications and instability of the