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.