Congenital Cholesteatoma Localized to the Mastoid Cavity and Presenting as a Mastoid Abscess

Abstract

Introduction. Congenital cholesteatoma is a pearly white mass that rarely originates from the mastoid process. Case Report. A 21-year-old male patient presented to our department with severe right mastoid pain and postauricular fluctuant swelling for 23 days. There was no preceding history of ear complaints and examination showed a normal right ear drum. Emergency exploration of the mastoid process was done on the same day and revealed localized cholesteatoma limited only to the mastoid cavity. Conclusion. Despite a rarity, the mastoid process should be always put in mind as a site of origin for congenital cholesteatoma.

Congenital mastoid‐isolated cholesteatoma in a 14‐year‐old boy

“…Presentation can be nonspecific, with vertigo, otorrhea, neck and mastoid pain described. It is often identified incidentally, however, can present with acute mastoiditis in adults 2 . All but one of the published cases have been managed surgically 3 …”

“…Congenital cholesteatoma isolated to the mastoid is very rare, with less than 30 published cases. The “Implantation theory” postulates that squamous cells become trapped during the closure of the mastoid fontanelle, eventually forming cholesteatoma 2 …”

“…The "Implantation theory" postulates that squamous cells become trapped during the closure of the mastoid fontanelle, eventually forming cholesteatoma. 2 As the middle ear is spared, hearing is usually normal and they can grow significantly before detection, with a mean age of presentation of 51. 3 Presentation can be nonspecific, with vertigo, otorrhea, neck and mastoid pain described.…”

“…It is often identified incidentally, however, can present with acute mastoiditis in adults. 2 All but one of the published cases have been managed surgically. 3 Careful review of preoperative imaging is vital as exposure of the facial nerve, sigmoid sinus or posterior fossa dura is frequent.…”

Congenital Cholesteatoma: The Silent Pathology

“…The incidence of CC is estimated to be between 4 and 24% of all cholesteatomas in children, but presentations in adults are common and should not be overlooked [3,6,[11][12][13][14]. Although small middle ear lesions are sometimes set apart from petrous bone cholesteatomas, they should be taken as seriously as they grow and will eventually cause much damage [9,12,14,15].…”