Patient and Method
Preoperative measurements: Article was structured according to the guideline of CARE. A 31-year-old male patient was admitted to the Ear Nose Throat Clinic in Semptember 2019 with complaint of left fullnes, discharge and conductive hearing loss. Symmetrical nodular cutaneous lesions on malar region, tragus and lateral portion of external ear canal (EAC) bilaterally were noted first (Fig. 1). The patient had diagnosed with TSC during adolescence clinically and genetically. The patient also had hypopigmented macules on the trunk and lower extremities and renal angiomyolipoma. There was no neurological symptom and intracranial finding in the patient’s previous magnetic resonance imaging (MRI). The patient reported that the his father and sibling had similar skin lesions without any neurological symptoms. The complaint for the reason of admission to Otorhinolaryngology Clinic was recurrent discharge and hearing loss, especially in the left ear. On physical examination, total obstruciton of EAC was detected due to TSC angiofibroma (Fig. 2). The lesions were pushed with a rigid endoscope (2.7 mm X 100 mm, Karl Storz SE & Co., Tuttlingen, Germany) by passing through the angiofibromas for EAC examination. On EAC examination, there was a slight purulent secretions and cerumen impaction was detected, the tympanic membrane was intact. Temporal bone Computed Tomography showed bilaterall soft tissue thickening of the one-third externall part of EAC. The structures of middle ear cavity and temporal bone were natural. Bone conduction was normal in audiometric examination, but mild conductive hearing loss was detected in the left (Pure tone Average for 0.5, 1, 2, 4 kHz; right ear: 15 dB and left ear: 25 dB).
Treatment regimen: EAC aspiration for cerumen and discharge was performed weekly. Ciprofloxacin / dexamethasone local therapy (Siprogut Plus Drop, 0.3% / 0.1%, Bilim Pharmaceutical Co., Istanbul, Turkey) prescirebed for three consequtive week, However, no improvement was achieved with medical treatment. So, it was decided to EAC meatoplasty. Surgery was planned to obtain a favorable, self-cleaning EAC rather than total excision of angiofibromas. The surgery was performed under local anesthesia. Angiofibromas on the EAC and tragus were excised. Suprapeichondrial dissection was performed especially for the excision of angiofibromas on the external meatus of EAC. The defect was repaired by sliding a 3x2x3 cm fasciocutaneous island flap inferiorly created in the preauricular area (Fig. 3 and 4). Skin marking for the required flap was made in the preauricular hairless region according to the size and shape of the defect. Local anesthesia was then infiltrated. The skin incision is complete. To preserve blood supply to the flap, it was not completely separated from the underlying temporal fascia. Peripheral dissection was performed for adequate movement and rotation. A self-cleaning and well ventilated EAC was achieved with an open and external meatus.
Treatment outcome: Immunohistochemistry analysis showed that some tumor cells were positive for CD31, Actin(SM), CD34 and vimentin, negative expression for smooth muscle actin (SMA), desmin, S100, and AE1/AE3. Ki-67 proliferation index was less than 5%. The pathology specimen was histopathologically diagnosed as angiofibroma. The patients was positive TSC1gene mutation. The patient was followed-up with monthly visits for six months postoperatively, and no EAC stenosis or angiofibroma recurrence was observed.