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.