Congenital Subglottic Stenosis: Report of a Familial Case Series
Authors List
Barber, Lucy, Starship Children's Hospital, Auckland, New Zealand
Gomes, Ariana, Starship Children's Hospital, Auckland, New Zealand
Johnston, James, Auckland City Hospital, Auckland, New Zealand
Van Der Meer, Graeme, Starship Children's Hospital, Auckland, New Zealand
Objective: Congenital subglottic stenosis (CSGS) is the third most common upper airway congenital anomaly in infants, after laryngomalacia and vocal cord paralysis. There are very few reported cases of familial congenital subglottic stenosis. We discuss a case series of three siblings with grade III subglottic stenosis (Myer-Cotton grading) and detail their different presentations.
Method: Analysis was performed retrospectively using each patient’s clinical records with the patient’s initial presentation highlighted in our discussion. A literature review revealed six articles with subglottic stenosis occurrence in families.
Results: All three cases presented at 3 months of age with varying symptoms. Laryngoscopy and bronchoscopy subsequently diagnosed CSGS Cotton Myer grade III in each patient. The first case underwent laryngotracheal reconstruction with anterior rib graft at 4 months of age. The other two cases (twins) have had a tracheostomy and are awaiting further management.
Conclusion: Three interesting presentations of familial congenital subglottic stenosis have been outlined in our case series. Further research is required to investigate a possible genetic predisposition to this disease.
Barber, Lucy, Starship Children's Hospital, Auckland, New Zealand
Gomes, Ariana, Starship Children's Hospital, Auckland, New Zealand
Johnston, James, Auckland City Hospital, Auckland, New Zealand
Van Der Meer, Graeme, Starship Children's Hospital, Auckland, New Zealand
Objective: Congenital subglottic stenosis (CSGS) is the third most common upper airway congenital anomaly in infants, after laryngomalacia and vocal cord paralysis. There are very few reported cases of familial congenital subglottic stenosis. We discuss a case series of three siblings with grade III subglottic stenosis (Myer-Cotton grading) and detail their different presentations.
Method: Analysis was performed retrospectively using each patient’s clinical records with the patient’s initial presentation highlighted in our discussion. A literature review revealed six articles with subglottic stenosis occurrence in families.
Results: All three cases presented at 3 months of age with varying symptoms. Laryngoscopy and bronchoscopy subsequently diagnosed CSGS Cotton Myer grade III in each patient. The first case underwent laryngotracheal reconstruction with anterior rib graft at 4 months of age. The other two cases (twins) have had a tracheostomy and are awaiting further management.
Conclusion: Three interesting presentations of familial congenital subglottic stenosis have been outlined in our case series. Further research is required to investigate a possible genetic predisposition to this disease.
