Li et al. Journal of Medical Case Reports 2011, 5:583 http://www.jmedicalcasereports.com/content/5/1/583 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Bilateral congenital choanal atresia and osteoma of ethmoid sinus with supernumerary nostril: a case report and review of the literature Xue-zhong Li*, Xiao-lan Cai, Lei Zhang, Xue-feng Han and Xiao Wei Abstract Introduction: Congenital choanal atresia is a relatively rare deformity, especially bilateral congenital choanal atresia. We report a case of bilateral congenital choanal atresia in a 22-year-old Chinese man, who was also diagnosed with congenital right accessory nasal deformity, osteoma of his left ethmoid sinus and congenital keratoleukoma of his right eye. Case presentation: A 22-year-old Chinese man presented with mouth breathing, sleep snoring and difficult feeding after birth, with no olfactory sensation. Three-dimensional computed tomography revealed bilateral choanal atresia and a high density bony shadow in his left ethmoid sinus that extended to his left frontal sinus. Conclusions: Choanal atresia is often accompanied by other congenital abnormalities. To the best of our knowledge, this is the first report of choanal atresia accompanied by congenital accessory nasal deformity and congenital keratoleukoma. Background Choanal atresia was first reported by Johann Roderer in 1755 [1]. Its incidence is about 8.2 per 100, 000 [2] and the primary symptoms, such as bilateral imperforation, dyspnea and inability to suck after birth, are attributed to postnatal nasal obstruction. In serious cases, ensuing suffocation can lead to death. We present a rare case of bilateral congenital choanal atresia, which was accompa- nied by congenital right accessory nasal deformity, osteoma of his ethmoid sinus, and congenital keratoleu- koma of his right eye. Case presentation A 22-year-old Chinese man presented with mouth breathing, sleep snoring, and loss of olfactory sensation. Difficult feeding after birth was reported, without dys- pnea or asphysia. He had no relevant family history. Physical examination revealed severe closed rhinolalia and no airflow in both of his anterior naris. Three nos- trils were identified on his external naris. His left naris, round-shaped and about 1 cm in diameter, was normal. * Correspondence: zhangleicome@126.com Department of Otolaryngology, Qilu Hospital, Shandong University, Ji’nan 250012, China However, his right anterior naris was divided into two parts by a barrier diaphragm which protruded from the lateral wall (Figure 1). The medial part of his right naris was relatively large, similar to the left, and connected to the nasal cavity, while the lateral part was approximately 0.3 cm in diameter and did not communicate with the nasal cavity. Three-dimensional computed tomography (CT) revealed bilateral choanal atresia (Figure 2) and a high- density bony shadow in his left ethmoid sinus (Figure 3), which extended to the left frontal sinus. The cornea of his right eye was covered by keratoleukoma (Figure 4) without reflection to light, while his left eye was normal. His hearing and intelligence were normal. Our primary diagnosis was congenital right accessory nasal deformity (Figure 1), congenital bilateral atresia of the posterior nares (Figures 2 and 5), osteoma of the left ethmoid sinus (Figure 3) and congenital keratoleukoma of his right eye (Figure 4). He was operated on under general anesthesia in March 2007. The procedures included orthopedics of the right accessory nasal defor- mity, exploration and recanalization of the posterior nares, and resection of the osteoma in his left ethmoid sinus. © 2011 Li et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.