Two decades to diagnose bilateral congenital choanal atresia: is this delay acceptable?

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A 20‐year‐old woman was referred to us with a life‐long history of difficulty in breathing through her nose. She was treated by her general practitioner with topical nasal medications for suspected nasal polyposis. A detailed history obtained in the otorhinolaryngology clinic suggested that nasal suctioning and placement of a nasal catheter was performed in the neonatal period but no general anaesthetic procedure was carried out. The precise details of any intervention performed were not available as the patient was born 20 years ago in the Pacific Islands. On examination in the clinic, nasal endoscopy demonstrated retained secretions in the anterior and posterior aspect of the nasal cavities which when aspirated revealed the absence of a normal posterior choanae (Fig. 1). The clinical suspicion of bilateral choanal atresia was confirmed on computed tomogram of the paranasal sinuses (Fig. 2). Distorted anatomy of the posterior nasal cavity was noted. The posterior bony septum was deviated to the right occluding the right posterior choana to a conical space.
Both transnasal and transplatal approaches to address the bilateral choanal atresia were discussed with the patient. The senior author is an experienced rhinologist and prefers the endoscopic approach which is the currently favoured technique in many tertiary centres. The recommendation was based on the age of the patient and her complex bony nasal anatomy as depicted on the computed tomography scan images (Fig. 2). Following discussion with the patient and her family, a transnasal endoscopic creation of a neochoana was proposed (Fig. 3). The procedure was carried out under general anaesthesia using a 4 mm curved diamond burr and microdebrider. After identification of a pinpoint membranous part of the right choanal atresia, a right‐sided posterior nasal flap was elevated based anteriorly. Using Kerrison rongers, the pinhole opening in the right atretic plate was progressively widened. A left Killian incision was used to perform an endoscopic septoplasty and to facilitate removal of the posterior bony septum. The entire neochoana was widened by removing the bone over the base of the sphenoid and roof of the post nasal space superiorly and the hard palate inferiorly, extending laterally to the level of the eustachian cushions. Haemostasis was achieved using bipolar diathermy. No post nasal splints were used but to prevent adhesions between septum and lateral walls of the nose, silastic sheets were secured anteriorly on each side of the nasal septum. No palatal perforation was noted on examination of the oral aspect of the palate. The patient made an uneventful recovery and was discharged home the following day on regular saline douches, antibiotics and analgesia. The patient was last reviewed in clinic at 4 months where a wide communication between the nasal cavities and post nasal space was confirmed (Fig. 4).
The nose develops from the neural crest cells which undergo caudal migration to reach midface region during the fourth week of gestation. Two nasal placodes and pits form inferiorly. Inferior to the nasal complex, the stomodeum forms. Nasobuccal membrane separates the oral cavity inferiorly from the nasal cavity superiorly. There are four theories for the development of choanal atresia:1
Three years before our interaction, the patient was reviewed by the ophthalmologists and was noticed to have multiple eye abnormalities including significantly impaired vision, iridocorneal adhesions and pupillary fibrous tissue; anterior capsular lens inferomedial opacity all affecting the right eye. The left lower eyelid had a medial canthal notching and the upper eye lid had ptosis. All these findings were attributed to birth‐related trauma which remains questionable. The left lower lid abnormality was corrected by a medial canthoplasty. The upper lid ptosis is managed conservatively.
Our patient reports a complete lack of sense of smell since birth.
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