Abstract

Congenital choanal atresia is a rare but well recognized entity. It is characterized by the developmental failure of nasal cavity to communicate with nasopharynx. While Roederer first described this condition in 1755 it was Oto who further described it in relation to the deformity involving palatine bones. Congenital atresia can be unilateral or bilateral. The reported incidence is 1 out of 6000-7000 live births. Females are more commonly affected than males. It may be associated with other congenital anomalies like coloboma, heart defect, retarded growth, genitourinary abnormalities, and ear anomalies (CHARGE anomalies). In 2/3 of the cases choanal atresia is bony-membranous type while approximately 1/3 cases are that of pure bony type. While unilateral choanal atresia may be subtle in presentation and the diagnosis may be missed in neonates and infants bilateral choanal atresia is life threatening and may present in immediate postnatal life with signs of severe airway obstruction and cyanosis.

Aims and Objectives: (1) To study the cases of choanal atresia through clinical evaluation (2) To study the surgical management of choanal atresia (3) Post-operative follow-up of patients with choanal atresia.

Materials and Methods:This was a multicentric prospective cohort study conducted in Department of Oto-Rhino-laryngeology at a government medical college and a ENT hospital. Detailed history was noted and clinical examination was done. A pediatric reference was done in each patient to rule out associated congenital anomalies. CT-PNS was done in all cases to confirm the diagnosis and type of choanal atresia. Surgery was done using transnasal endoscopic approach. Nasal stents were kept for 7-10 days after which they were removed under general anaesthesia. Follow up diagnostic nasal endoscopy was done at 6 weeks and 6 months to assess the size of posterior nasal aperture. The data was analyzed with a special emphasis on demographic characteristics, clinical presentation, surgical intervention required and postoperative outcome.  

Results: Total 7 cases with either unilateral or bilateral choanal atresia were included in this study. Out of these cases 4 were males and 3 females with a M: F ratio being 1:0.75. 4 patients had bilateral choanal atresia while 3 patients had unilateral choanal atresia. Out of the 3 patients who had unilateral choanal atresia 2 had right sided and 1 had left sided choanal atresia. Common signs present in bilateral choanal atresia were tachypnoea and central cyanosis while in children with unilateral choanal atresia the most common complaint was refusal to take proper feeding. The median age of surgery in children with unilateral choanal atresia was 6 years. Since bilateral choanal atresia is an emergency immediate intervention was done and median age of these patients at surgery was 12 days. Follow up of the patients was done every 6 monthly.

Conclusion: Choanal atresia is a rare but well recognised entity. While the clinical features of unilateral choanal atresia may be subtle bilateral choanal atresia usually present with respiratory distress and cyanosis in immediate postnatal period. Trans-nasal endoscopic surgical repair of choanal atresia with postoperative stenting achieves good results in patients.

Keywords: Choanal Atresia, CHARGE, Trans-nasal endoscopic surgery, Respiratory distress.