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

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...

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Main Authors: 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
Format: Article
Language:English
Published: SAGE Publishing 2021-07-01
Series:Journal of Otolaryngology - Head and Neck Surgery
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Online Access:https://doi.org/10.1186/s40463-021-00517-x
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author 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
author_facet 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
author_sort Josee Paradis
collection DOAJ
description 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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spelling doaj-art-627b6f0c18b4456cb1190f232c74c2db2025-02-03T00:22:57ZengSAGE PublishingJournal of Otolaryngology - Head and Neck Surgery1916-02162021-07-015011810.1186/s40463-021-00517-xA national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentationJosee Paradis0Agnieszka Dzioba1Hamdy El-Hakim2Paul Hong3Frederick K. Kozak4Lily H. P. Nguyen5Demitri Perera6Evan Jon Propst7Jennifer M. Siu8Monika Wojtera9Murad Husein10National Choanal Atresia Study Working GroupDepartment of Otolaryngology- Head and Neck Surgery, Children’s Hospital at London Health Sciences CentreDepartment of Otolaryngology- Head and Neck Surgery, Children’s Hospital at London Health Sciences CentreDivision of Pediatric Surgery and Otolaryngology Head and Neck Surgery, Departments of Surgery and Pediatrics, The Stollery Children’s Hospital, University of Alberta HospitalIWK Health CentreFaculty of Medicine, University of British ColumbiaDepartment of Otolaryngology-Head and Neck Surgery, McGill UniversityFaculty of Medicine, University of QueenslandDepartment of Otolaryngology-Head & Neck Surgery, Hospital for Sick Children, University of TorontoDepartment of Otolaryngology-Head & Neck Surgery, Hospital for Sick Children, University of TorontoOtolaryngology-Head and Neck Surgery, Schulich School of Medicine and Dentistry, Western UniversityDepartment of Otolaryngology- Head and Neck Surgery, Children’s Hospital at London Health Sciences CentreAbstract 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 abstracthttps://doi.org/10.1186/s40463-021-00517-xChoanal atresiaClinical presentationFamily history
spellingShingle 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
A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation
Journal of Otolaryngology - Head and Neck Surgery
Choanal atresia
Clinical presentation
Family history
title A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation
title_full A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation
title_fullStr A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation
title_full_unstemmed A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation
title_short A national study of choanal atresia in tertiary care centers in Canada – part I: clinical presentation
title_sort national study of choanal atresia in tertiary care centers in canada part i clinical presentation
topic Choanal atresia
Clinical presentation
Family history
url https://doi.org/10.1186/s40463-021-00517-x
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