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

Josee Paradis; Agnieszka Dzioba; Hamdy El-Hakim; Paul Hong; Frederick K Kozak; Lily H P Nguyen; Demitri Perera; Evan Jon Propst; Jennifer M Siu; Monika Wojtera; Murad Husein; National Choanal Atresia Study Working Group

doi:10.1186/s40463-021-00517-x

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

J Otolaryngol Head Neck Surg. 2021 Jul 12;50(1):45. doi: 10.1186/s40463-021-00517-x.

Authors

Josee Paradis^{1

2}, Agnieszka Dzioba³, Hamdy El-Hakim⁴, Paul Hong^{5

6}, Frederick K Kozak^{7

8}, Lily H P Nguyen^{9

10

11}, Demitri Perera¹², Evan Jon Propst¹³, Jennifer M Siu¹³, Monika Wojtera¹⁴, Murad Husein^{3

14}; National Choanal Atresia Study Working Group

Affiliations

¹ Department of Otolaryngology- Head and Neck Surgery, Children's Hospital at London Health Sciences Centre, London, ON, Canada. [email protected].
² Otolaryngology-Head and Neck Surgery, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada. [email protected].
³ Department of Otolaryngology- Head and Neck Surgery, Children's Hospital at London Health Sciences Centre, London, ON, Canada.
⁴ Division of Pediatric Surgery and Otolaryngology Head and Neck Surgery, Departments of Surgery and Pediatrics, The Stollery Children's Hospital, University of Alberta Hospital, Edmonton, AB, Canada.
⁵ IWK Health Centre, Halifax, NS, Canada.
⁶ Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, Dalhousie University, Halifax, NS, Canada.
⁷ Faculty of Medicine, University of British Columbia, Vancouver, BC, Canada.
⁸ Division of Pediatric Otolaryngology-Head and Neck Surgery, BC Children's Hospital, Vancouver, BC, Canada.
⁹ Department of Otolaryngology-Head and Neck Surgery, McGill University, Montreal, Canada.
¹⁰ Institute for Health Science Education, McGill University, Montreal, Canada.
¹¹ Department of Pediatric Surgery, Montreal Children's Hospital, Montreal, Canada.
¹² Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.
¹³ Department of Otolaryngology-Head & Neck Surgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
¹⁴ Otolaryngology-Head and Neck Surgery, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada.

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.

Keywords: Choanal atresia; Clinical presentation; Family history.

Publication types

Multicenter Study

MeSH terms

Canada
Child
Choanal Atresia* / diagnosis
Choanal Atresia* / epidemiology
Female
Humans
Infant
Infant, Newborn
Male
Retrospective Studies
Tertiary Care Centers