Abstract
Bilateral choanal atresia (CA) is a rare congenital malformation frequently associated with other anomalies. CHARGE association is closely linked to bilateral CA. The aim of this study was to describe the outcomes of the endoscopic repair in bilateral CA, and to assess the role of postoperative nasal stenting in two cohorts of CHARGE-associated and non-syndromic CA. Thirty-nine children were retrospectively analyzed (16 patients had CHARGE-associated CA). The rate of postoperative neochoanal restenosis was 31.3% in the CHARGE population, and 47.8% in the non-syndromic CA cohort. Data on postoperative synechiae and granulation tissue formation, need for endonasal toilette and dilation procedures, and number of procedures per patient were presented. Stent positioning led to a higher number of postoperative dilation procedures per patient in the non-syndromic cohort (p = 0.018), and to a higher rate of restenosis both in the CHARGE-associated, and non-syndromic CA populations. Children with CHARGE-associated and non-syndromic bilateral CA benefitted from endonasal endoscopic CA correction. The postoperative application of an endonasal stent should be carefully evaluated.
Highlights
In 1979, Hall described 17 children with choanal atresia (CA) and multiple congenital anomalies [1], and Hittner reported on 10 children with ocular colobomas and multiple congenital anomalies, including CA
2000, an international survey of pediatric otolaryngologists belonging to the American Society of Pediatric Otolaryngology (ASPO) stated that 85% of the interviewees preferred the endoscopic approach for CA repair, even if transpalatal repair and puncture with Fearon dilators were still advocated by 60% and 17%, respectively
Children with CHARGE association and bilateral CA benefit from endonasal endoscopic correction of the atretic plate, showing comparable results to that observed for non-syndromic-associated bilateral CA
Summary
In 1979, Hall described 17 children with choanal atresia (CA) and multiple congenital anomalies [1], and Hittner reported on 10 children with ocular colobomas and multiple congenital anomalies, including CA. This association was called “Hall–Hittner syndrome” [2]. Because of the heterogeneous clinical presentation, in 1998, Blake et al described four major criteria (the classical 4C’s: Choanal atresia, Coloboma, Characteristic ears, and Cranial nerve anomalies), and minor characteristics (genital hypoplasia, developmental delay, cardiovascular malformations, growth deficiency, orofacial cleft, tracheoesophageal fistula, and distinctive face) to define the new entity. Otolaryngologists are involved in the treatment of several conditions in CHARGE patients, including hearing loss, inner ear malformations, and CA [8,9,10,11]
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