Bronchial stenosis in an 8-year-old with Moebius syndrome: A case report

Abstract

Introduction

Moebius syndrome is a rare congenital condition involving underdevelopment of cranial nerves VI and VII, often causing facial palsy. Also rare, bronchial stenosis may cause recurrent respiratory infections. There is scarce evidence on the outcomes of pediatric patients undergoing open surgical repair for congenital bronchial stenosis.

Presentation of case

An eight-year-old male with Moebius syndrome and tracheostomy-dependent since infancy, presented with fever, severe respiratory compromise and left lung atelectasis. Bronchoscopy revealed severe stenosis of the left main bronchus and the right upper lobe bronchus. Despite two bronchoscopic balloon dilations, the obstruction recurred. The patient successfully underwent bronchoplasty under extracorporeal membrane oxygenation (ECMO) with resection of the stenotic segment and end-to-end anastomosis. After 97 inpatient days, he was discharged home with usual nocturnal ventilation support.

Discussion

The diagnosis of congenital bronchial stenosis (CBS) is difficult because of its rarity and nonspecific presentation. Severe cases may warrant definitive surgical repair and ECMO provides critical support to ensue oxygenation and hemodynamic stability while the airway procedure takes place. Open bronchoplasty offers a safe and effective option when bronchoscopic management fails.

Conclusion

Early recognition of CBS in children with complex comorbidities such as Moebius syndrome is key for prompt management. Multidisciplinary efforts are crucial for optimal patient outcomes.