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Sinus Cholesteatoma

● Epidermoid tumors, or cholesteatomas, develop from squamous epithelium.


● They commonly arise in the mastoid and middle ear secondary to chronic otitis media.
● but when cholesteatomas arise from the paranasal sinuses, the most common location is the
frontal sinus, followed by the ethmoid and maxillary sinuses.

○ They are a rare entity, with a paucity of reported cases.


○ The frontal sinus is the most common site of origin, with 30 reported cases, followed by the
ethmoid sinus and, rarely, the maxillary sinus. 1

● Either congenital or acquired.


● Congenital lesions likely develop from implantation of ectodermal epithelium during
development,
● Acquired epidermoids may occur secondary to several pathophysiologic mechanisms.
i. migration of squamous epithelium from a contiguous squamous epithelial region,
ii. traumatic implantation of squamous epithelium,2
iii. squamous metaplasia of respiratory epithelium.
○ In the paranasal sinuses, the latter two mechanisms appear most likely due to the lack of
native squamous epithelial surfaces in any proximity to the sinonasal tract.
○ Another proposed etiology, based on location, is buccal epithelium invasion of the maxillary
sinus via an oral antral fistula.

Clinical Features
○ Symptomatology is based on the location and expansile nature of the lesion.
○ Generally, they will present with increasing pressure and pain.
○ If the lesion obstructs sinus outflow, sinusitis symptoms will develop.
○ Patients can also present with facial swelling, palatal swelling, proptosis, restriction of
extraocular movements, nasal obstruction, and, in cases of skull base erosion, central nervous
system findings .

Diagnostic Work-up
● The first-line radiographic study for the majority of paranasal sinus lesions is a CT scan.
● On CT, a cholesteatoma appears as a nonenhancing, expansile lesion with irregular borders.
It is usually inhomogeneous in nature.
● At this point, an MRI is generally obtained. It will demonstrate low intensity on T1 and high
intensity on T2 sequences.

Treatment
● Surgical intervention is necessary secondary to the potential for continued expansion and
erosion of surrounding structures.
● The treatment is ranging from endoscopic marsupialization and exteriorization to complete
excision of the cyst lining, which may require an open procedure.
● cases located in the maxillary, ethmoid, sphenoid, and frontal sinuses generally can be
treated with endoscopic marsupialization, aggressive removal of cholesteatoma contents, and
subsequent exteriorization with the use of stereotactic image guidance systems
● The success of marsupialization in these cases depends on functional mucociliary clearance
in the paranasal sinuses into which the cyst is marsupialized.

1
2Adham Baniyounes , MD OTOLARYNGOLOGY H&N SURGERY

1
● Note: , enough respiratory mucosa must be present in the region of the exteriorized
squamous matrix to permit mucociliary clearance of debris.
● Management of lesions arising in the frontoethmoid region is perhaps more controversial,
given the options of marsupialization versus osteoplastic flap with obliteration of the frontal
and supraorbital cells.

Outcomes .
○ They are assumed to have the same pathophysiologic mechanisms of growth as temporal
bone cholesteatomas; accordingly, residual epithelium will likely promote recurrence.
○ Ultimately, the key to management relies on adequate long-term follow-up, ensuring patency
of the drainage pathway, which includes regular endoscopy and thorough debridement, as
well as postoperative radiographic surveillance.
○ Note that if the drainage of the contents cannot be guaranteed (e.g., as a result of the
extreme lateral localization of the cholesteatoma), a full excision must be performed

2
References

● Hartman JM, Stankiewicz JA. Cholesteatoma of the parana- sal sinuses: case report and review of the
literature. Ear Nose Throat J 1991;70(10):719–725
● Storper IS, Newman AN. Cholesteatoma of the maxillary sinus. Arch Otolaryngol Head Neck Surg
1992;118(9):975–977
● Palacios E, Robertson H. Cholesteatoma of the maxillary sinus. Ear Nose Throat J 2005;84(12):758
● Campanella RS, Caldarelli DD, Friedberg SA. Cholesteatoma of the frontal and ethmoid areas. Ann
Otol Rhinol Laryngol 1979;88(4 Pt 1):518–523
● Urken ML, Som PM, Lawson W, Edelstein D, Weber AL, Biller HF. Abnormally large frontal sinus: 2.
Nomenclature, pathology, and symptoms. Laryngoscope 1987;97(5):606–611

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