Discussion
TSC is a multisystemic neurocutaneous tumor syndrome caused by mutation
of tumor suppressor genes causing hamartomas in different organs such as
skin, brain, lungs, and kidneys.1 Multiple facial
angiofibromas occur in most of the patients with TSC particularly around
the nose, appearing as firm skin-coloured telangiectatic papules. Also,
oral mucosal and gingival fibrous proliferation is another accompanied
lesions on the head and neck region.3 Severe facial
and nasal involvement of the disease can cause nasal breathing and
feeding difficulty in addition to cosmetic problems. Auricular and EAC
skin involvement is an expected but unusual presentation of TSC
syndrome. The main complaints were resistant EAC infection and
conductive hearing loss in the presented case due to obstruction of MAE.
While topical antineoplastic drugs have priority for common and small
lesions on the face, surgical treatment is recommended for more specific
lesions and in a limited area due to the risk of
scar.1, 2, 4 Treatments such as dermabrasion, chemical
peeling, cryosurgery, laser surgery are for cosmetic purposes and are
insufficient for serious complications such as bleeding and feeding
problems.4, 5 Complete surgical excision of the
lesions is more effective for to solve urgent complications and prevent
recurrence.5 Therefore, surgical excision and
reconstruction was considered in the presented case for the treatment of
complications due to EAC obstruction. In this case, remove of the
lesions on the tragus was aimed to cosmetical results and meatoplasty
planned to provide a functional MAE patency. Preauricular
fasciocutaneous island flap was adequate for defect reconstruction. The
result of operation was aesthetically and functionally satisfactory for
the patient and the surgeon.