Annals of Maxillofacial Surgery | July - December 2014 | Volume 4 | Issue 2 193 Supernumerary nostril: Congenital adrenal hyperplasia with a rare congenital anomaly Sinem Ciloglu, Alpay Duran, Hasan Buyukdogan, Ahmet K. Yigit Department of Plastic and Reconstructive Surgery, Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey Address for correspondence: Dr. Alpay Duran, Department of Plastic and Reconstructive Surgery, Haydarpasa Numune Training and Research Hospital, Istanbul 34688, Turkey. E-mail: dr.alpayduran@hotmail.com Multiple or supernumerary nostril is a rare congenital anomaly with unknown etiology. The first case was reported by Lindsay as bilateral supernumerary nostrils. Supernumerary nostril cases are mostly unilateral and isolated. They are also reported with other congenital malformations like facial clefts and congenital anomalies like congenital auricular hypoplasia, congenital cataracts, eusophageal atresia and patent ductus arteriosus. Here, we report a case of supernumerary nostril with congenital adrenal hyperplasia Keywords: Congenital adrenal hyperplasia, nostril, supernumerary INTRODUCTION Supernumerary nostril is one of the rarest congenital nasal deformities. [1] Supernumerary nostril cases are mostly unilateral and isolated. They are also reported with other congenital malformations such as facial clefts and congenital anomalies like congenital auricular hypoplasia, congenital cataracts, esophageal atresia and patent ductus arteriosus (PDA). [2,3] No case of an supernumerary nostril and congenital adrenal hyperplasia co-existence has been reported previously in English literature. Here, we report a case of supernumerary nostril with congenital adrenal hyperplasia. CASE REPORT A 4-year-old female patient with a nasal anomaly was admitted to our outpatient clinic. She was born 2300 g prematurely on her 37 th gestational week from a 34-year-old mother with a history of five pregnancies, two still births and two abortions. On her physical examination, she had a left-sided supernumerary nostril and cliteromegaly [Figure 1]. Her laboratory studies revealed low levels of androstenedione and her history showed that she had medical therapy for 1 year for her congenital adrenal hyperplasia. No additional anomaly except patent foramen ovale was detected in her work-ups. Paranasal sinus computed tomography study revealed that her left-sided accessory nostril opened to the left nasal cavity. The patient was operated, the opening of the supernumerary nostril to the nasal cavity was obliterated and the widened nostril was narrowed by excisions from the alar ground and lateral side [Figure 2]. A revision toward the nostril asymmetry is planned 1 year after the operation. Access this article online Website: www.amsjournal.com DOI: 10.4103/2231-0746.147133 Quick Response Code: Case Report – Developmental Anomalies ABSTRACT Figure 1: Preoperative view of the case