Severe Unilateral Cystic Bronchiectasis in a Child with Down Syndrome and Prior Congenital Diaphragmatic Hernia Repair:  A Case Report

Mustafa El-Ahmer; Wesam Elsaghayer; Ahmed Aniba; Eman Alabani; Fatma Kannah

المؤلفون

Mustafa El-Ahmer Faculty of Medicine Misrata University Misrata Libya
Wesam Elsaghayer Department of Pathology Misrata Medical Center Misrata Libya
Ahmed Aniba Faculty of Medicine Misrata University Misrata Libya
Eman Alabani Department of Pediatric Surgery Misrata Medical Center Misrata Libya
Fatma Kannah Department of Pathology Misrata Medical Center Misrata Libya

الكلمات المفتاحية:

Bronchiectasis، cystic bronchiectasis، Down syndrome، pediatric pulmonology، congenital diaphragmatic hernia

الملخص

Background: Congenital bronchiectasis is a rare but serious condition in the pediatric population and may be more challenging to diagnose in children with Down syndrome due to overlapping clinical features and communication difficulties. Bronchiectasis, characterized by irreversible bronchial dilatation, is often associated with recurrent infections and may be particularly severe in children with underlying genetic conditions.

Case Presentation: We report a 12-year-old girl with Down syndrome and prior congenital diaphragmatic hernia repair who presented with a long-standing history of productive cough and recurrent lower respiratory tract infections since infancy. Physical examination revealed underweight status, digital clubbing, and inspiratory crackles predominantly in the left lower lung zone. Imaging demonstrated extensive cystic bronchiectasis with left lung volume reduction, and the patient underwent left pneumonectomy. Histopathology confirmed severe acute and chronic bronchiectasis with patchy fibrosis and superimposed acute bronchopneumonia.

Conclusion: This case highlights the rare occurrence of severe unilateral cystic bronchiectasis in children with Down syndrome and prior congenital diaphragmatic hernia repair. Early recognition and surgical intervention may be necessary in cases with extensive lung destruction

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