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Congenital vascular anomalies and persistent respiratory symptoms in children
P K Gormley1, M P Colreavy, N Patil
1Department of Otolaryngology, Head and Neck Reconstructive Surgery, University College Hospital Galway, Ireland. pgormley@tinet.ie
Insights
Congenital cardiovascular anomalies causing airway compression are treatable. Early endoscopic diagnosis is crucial, as symptoms like stridor are often misdiagnosed, delaying treatment for pediatric patients.
Area of Science:
- Pediatric Cardiology
- Thoracic Surgery
- Medical Diagnostics
Background:
- Congenital cardiovascular anomalies are a rare cause of pediatric stridor.
- Delays in endoscopic diagnosis are common, with symptom duration ranging from weeks to years.
- Anomalies of the aortic arch complex may be underdiagnosed, mistaken for conditions like croup or asthma.
Purpose of the Study:
- To highlight congenital vascular anomalies as a treatable cause of pediatric stridor.
- To emphasize the importance of early endoscopic diagnosis for tracheobronchial compression.
- To review the clinical presentation, diagnosis, and management of these anomalies.
Main Methods:
- Retrospective review of 16 pediatric cases with congenital vascular anomalies causing tracheobronchial compression.
- Diagnostic evaluation included direct laryngoscopy and bronchoscopy.
- Treatment involved aortopexy, surgical division of the aortic arch, or conservative management.
Main Results:
- Stridor was the most common presenting symptom (100%), followed by cough and dyspnea (75%).
- Innominate artery compression (15 cases) and double aortic arch (1 case) were identified.
- Endoscopic diagnosis was successful in all cases; 75% of patients experienced complete symptom resolution after treatment.
Conclusions:
- Congenital vascular anomalies causing tracheobronchial compression are a significant, treatable cause of pediatric stridor.
- A high clinical index of suspicion is essential for timely diagnosis and intervention.
- Endoscopic evaluation is the diagnostic method of choice for these potentially fatal conditions.
Abstract:
Congenital cardiovascular anomalies are a rare but treatable cause of stridor in the paediatric population. Despite this, we and the authors of other series have found a delay in referral for endoscopic diagnosis. The duration of symptoms prior to endoscopic diagnosis ranged from 6 weeks to 10 years. It has been estimated that up to 3% of the population have congenital anomalies of the aortic arch complex and we feel that many patients currently being diagnosed as having recurrent croup or atypical asthma must be excluded from this group. We had 16 cases of congenital vascular anomalies causing tracheobronchial compression. Fifteen were due to innominate artery compression and one was due to a double aortic arch. Presenting symptoms included stridor (100%), persistent cough (75%), chronic dyspnoea (75%), reflex apnoea (60%), recurring respiratory tract infections (56%) and dysphagia (25%). Direct laryngoscopy and bronchoscopy was diagnostic in all cases and is our investigation of choice. Eleven underwent aortopexy (69%), one had division of the non-dominant aortic arch (6%) and four were managed conservatively (25%). They were followed for up to 9 years post-operatively and 75% had complete resolution of their symptoms. We feel that only a high clinical index of suspicion will enable these potentially fatal anomalies to be diagnosed.