A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation

Josée Paradis1,2, A Dzioba2, Hamdy El-Hakim3, Paul Hong4,5, Frederick K. Kozak6, Lily H. P. Nguyen7,8, Demitri Perera9, Evan J. Propst10, Jennifer Siu10, Monika Wojtera1, Murad Husein1,2
1Otolaryngology-Head and Neck Surgery, Schulich School of Medicine and Dentistry, Western University, London, Canada
2Department of Otolaryngology- Head and Neck Surgery, Children’s Hospital at London Health Sciences Centre, London, Canada
3Division of Pediatric Surgery and Otolaryngology Head and Neck Surgery, Departments of Surgery and Pediatrics, The Stollery Children’s Hospital, University of Alberta Hospital, Edmonton, Canada
4Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, Dalhousie University, Halifax, Canada
5IWK Health Centre, Halifax, Canada
6Faculty of Medicine, University of British Columbia, Vancouver, Canada
7Department of Pediatric Surgery, Montreal Children’s Hospital, Montreal, Canada
8Institute for Health Science Education, McGill University, Montreal, Canada
9Faculty of Medicine, University of Queensland, Brisbane, Australia
10Department of Otolaryngology - Head and Neck Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada.

Tóm tắt

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract

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Carpenter RJ, Neel HB. Correction of congenital choanal atresia in children and adults. Laryngoscope. 1977;87(8):1304–11. https://doi.org/10.1288/00005537-197708000-00010.

Biron VL, O'Connell DA, Barber B, et al. Transoral robotic surgery with radial forearm free flap reconstruction: case control analysis. J Otolaryngol Head Neck Surg. 2017;46(1):20.

Duncan NO, Miller RH, Catlin FI. Choanal atresia and associated anomalies: the CHARGE association. Int J Pediatr Otorhinolaryngol. 1988;15(2):129–35. https://doi.org/10.1016/0165-5876(88)90063-8.

Keller JL, Kacker A. Choanal atresia, CHARGE association, and congenital nasal stenosis. Otolaryngol Clin N Am. 2000;33(6):1343–51. https://doi.org/10.1016/S0030-6665(05)70285-1.

Blake KD, Prasad C. CHARGE syndrome. Orphanet J Rare Dis. 2006;1(1):34. https://doi.org/10.1186/1750-1172-1-34.

Bergman JEH, Janssen N, Hoefsloot LH, Jongmans MCJ, Hofstra RMW, van Ravenswaaij-Arts CM. CHD7 mutations and CHARGE syndrome: the clinical implications of an expanding phenotype. J Med Genet. 2011;48(5):334–42. https://doi.org/10.1136/jmg.2010.087106.

Holland BW, McGuirt J, William F. Surgical management of choanal atresia: improved outcome using mitomycin. Arch Otolaryngol Head Neck Surg. 2001;127(11):1375–80. https://doi.org/10.1001/archotol.127.11.1375.

Freitas RPD, Berkowitz RG. Bilateral choanal atresia repair in neonates—a single surgeon experience. Int J Pediatr Otorhinolaryngol. 2012;76(6):873–8. https://doi.org/10.1016/j.ijporl.2012.02.063.

Romeh HE, Albirmawy OA. A 13-year experience and predictors for success in transnasal endoscopic repair of congenital choanal obliteration. Int J Pediatr Otorhinolaryngol. 2010;74(7):737–42. https://doi.org/10.1016/j.ijporl.2010.03.027.

Newman JR, Harmon P, Shirley WP, Hill JS, Woolley AL, Wiatrak BJ. Operative management of choanal atresia: a 15-year experience. JAMA Otolaryngol Head Neck Surg. 2013;139(1):71–5. https://doi.org/10.1001/jamaoto.2013.1111.

Friedman NR, Mitchell RB, Bailey CM, Albert DM, Leighton SEJ. Management and outcome of choanal atresia correction. Int J Pediatr Otorhinolaryngol. 2000;52(1):45–51. https://doi.org/10.1016/S0165-5876(99)00298-0.

Gosepath J, Santamaria VE, Lippert BM, Mann WJ. Forty-one cases of congenital choanal atresia over 26 years--retrospective analysis of outcome and technique. Rhinology. 2007;45(2):158–63.

Teissier N, Kaguelidou F, Couloigner V, François M, Van Den Abbeele T. Predictive factors for success after transnasal endoscopic treatment of choanal atresia. Arch Otolaryngol Head Neck Surg. 2008;134(1):57–61. https://doi.org/10.1001/archoto.2007.20.

Van Den Abbeele T, François M, Narcy P. Transnasal endoscopic treatment of choanal atresia without prolonged stenting. Arch Otolaryngol Head Neck Surg. 2002;128(8):936–40. https://doi.org/10.1001/archotol.128.8.936.

Eladl HM, Khafagy YW. Endoscopic bilateral congenital choanal atresia repair of 112 cases, evolving concept and technical experience. Int J Pediatr Otorhinolaryngol. 2016;85:40–5. https://doi.org/10.1016/j.ijporl.2016.03.011.

Statistics Canada. Live births and fetal deaths (stillbirths), by type of birth (single or multiple). 2021. https://www150.statcan.gc.ca/t1/tbl1/en/tv.action?pid=1310042801&pickMembers%5B0%5D=1.2&cubeTimeFrame.startYear=1991&cubeTimeFrame.endYear=2010&referencePeriods=19910101%2C20100101. Accessed March 20, 2021.

Statistics Canada. Estimates of the components of natural increase, quarterly. https://www150.statcan.gc.ca/t1/tbl1/en/tv.action?pid=1710005901&pickMembers%5B0%5D=1.3&cubeTimeFrame.startMonth=01&cubeTimeFrame.startYear=1980&cubeTimeFrame.endMonth=10&cubeTimeFrame.endYear=1990&referencePeriods=19800101%2C19901001. Accessed March 20, 2021.

Burrow TA, Saal HM, de Alarcon A, Martin LJ, Cotton RT, Hopkin RJ. Characterization of congenital anomalies in individuals with choanal atresia. Arch Otolaryngol Head Neck Surg. 2009;135(6):543–7. https://doi.org/10.1001/archoto.2009.53.

Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope. 1996;106(1 Pt 1):97–101. https://doi.org/10.1097/00005537-199601000-00019.

Bhattacharyya AK, Lund VJ. Unilateral choanal atresia in siblings – a rare occurrence. J Laryngol Otol. 1996;110(7):665–7. https://doi.org/10.1017/S0022215100134553.