A case of cleft lip and palate associated with unilateral choanal atresia

Choanal atresia is a congenital anomaly of narrowing or obliteration at posterior nasal aperture, blocking the posterior nasal cavity from the nasopharynx. The incidence of choanal atresia is one in 5,000–8,000 births. It can be bilateral or unilateral, and unilateral choanal atresia is rarely associated with other malformations compared with bilateral choanal atresia. Here we report a rare case of cleft lip and palate associated with unilateral choanal atresia. The patient was born with multiple congenital anomalies including left cleft lip and palate, amniotic band syndrome, right radial paralysis, skin defects at the right elbow and right back, and anophthalmia. Left cheiloplasty was conducted at 5 months, along with the release of circumferential constriction bands, neurolysis of the right radial nerve, and transfer of the left sural nerve. At the age of 1 year and 4 months, he underwent left palatoplasty, and transtympanic ventilation tubes were placed for bilateral otitis media with effusion. Viscous nasal discharge from his right nostril continued, and right congenital choanal atresia and bilateral adenoid vegetation were diagnosed by the age of five years. Endoscopic perforation of choanal atresia and adenoidectomy were conducted by his otolaryngologist during the same operation for extraction of the deciduous anterior tooth, which was also necessary before bone grafting for the left alveolar cleft. Although the association of cleft lip and palate with unilateral choanal atresia is rare, it is important for surgeons to have knowledge of this malformative association, as that would enable careful examination and timely operation.