Where is my ear? – Cervical Chondrocutaneous Branchial Remnant

Özet

A 6-year-old boy presented with the absence of pinna on the left side since birth. A cutaneous appendage emerging from the neck on the left side was discovered during the examination (Figure 1). Deep palpation revealed a firm texture like cartilage. On the left side, a primitive firm cartilage was palpated in the helix region (Figure 2). The rest of the cartilaginous framework and external auditory canal (EAC) opening was absent on the left side. On the right side, the pinna was smaller in size and loped. High resolution computed tomography (HRCT) of temporal bone showed an atretic and stenosed EAC on the left and ride side, respectively. Ossicular lump was noted on both sides on HRCT, but all inner ear structures appeared normal in both ears. Pure tone audiometry revealed moderate and mild conductive hearing loss on the left and right sides, respectively. Magnetic resonance imaging of the neck was done to rule out any branchial abnormality. It did not reveal any cyst/sinus/fistula. A provisional diagnosis of cervical chondrocutaneous branchial remnant (CCBR) with leftsided microtia and EAC atresia was made on clinico-radiological basis. After a thorough discussion with the father, the patient was planned for the excision of the cervical mass and staged pinnaplasty and canaloplasty on the left side at a later stage. Histopathological examination after excision showed elastic cartilage rests covered by normal skin consisting of epidermis, dermis, adnexal structures, and subcutaneous fat compatible with CCBR. The development of six cartilaginous tubercles known as “Hillocks of His” initiates the development of pinna in the sixth week of pregnancy. The first three hillocks are formed by the first branchial arch, while the remaining three are formed by the second branchial arch (1). The lobule is the first external ear component to develop. The growing pinna is first found in the neck, but by the 20th week of pregnancy, it has ascended to its usual adult location and structure (2). An accessory tragus is formed from the first branchial arch and is usually found along an imaginary line drawn from the tragus to the angle of the mouth, whereas CCBR