Introduction
Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder
caused by TSC1 or TSC2 mutations is characterized by hamartomas in
various organs (e.g., skin, brain, lungs, and kidneys) and appears with
an incidence rate of approximately 1 in 5000–10,000 [1].
Angiofibromas are the most frequent skin lesions occurred in patients
with TSC older than 5 years and characteristically consist of numerous
pink to reddish papules or nodules that are typically located on the
cheeks, nose, and chin [2]. The traditional and current treatment
modalities for facial angiofibromas include topical sirolimus, topical
rapamycin cryosurgery, curettage, dermabrasion, chemical peeling,
excision, and laser therapies. Treatment options generally vary
according to the clinical presentation of the disease and the location
of the TSC related lesions. Treatments for skin lesions in the face and
head and neck area are usually intended for cosmetical issues, except
for ocular manifestations, and the treatment decision is made according
to the severity of the disease (e.g. Facial Angiofibroma Severity
Index). Auricula and external auditory canal (EAC) involvement in TSC
related angiofibroma a rare and specific pathology due to the aesthetic
and functional feature of the ear.
The obstruction of the EAC causes recurrent infections by preventing
aeration and excretion of cerumen. It can also cause conductive hearing
loss. Adequate meatoplasty is important to achieve a dry, self-cleaning
EAC canal to protect recurrent external otitis. Bony canaloplasty,
cartilage excision, Z-plasty techniques, or V-Y flaps are available for
EAC meatoplasty (3).