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Bronchobiliary fistula: a clinical and surgical challenge. Presentation of a pediatric case
L Marcano Sanz1, M Endis Miranda2, M Siavichay Romero2
1Pediatric Cardiothoracic Surgery Unit. Vicente Corral Moscoso Regional Hospital. Cuenca, Azuay (Ecuador).
Insights
Congenital bronchobiliary fistula is a rare condition requiring multidisciplinary care. This case highlights the importance of considering bile duct hypoplasia for effective treatment of this rare malformation.
Area of Science:
- Medical Malformations
- Surgical Case Reports
- Rare Diseases
Background:
- Congenital bronchobiliary fistula is an extremely rare congenital malformation.
- High morbidity and mortality rates are associated with this condition.
- Only 36 cases were reported worldwide by 2016.
Observation:
- An 11-year-old male presented with chronic lung disease, respiratory insufficiency, jaundice, and other symptoms.
- Diagnosis was confirmed via fibrobronchoscopy and CT scan.
- Recurrence after initial treatment indicated underlying bile duct hypoplasia.
Findings:
- A complex case involving congenital bronchobiliary fistula with bile duct hypoplasia was managed.
- Multiple surgical interventions including pneumonectomy, segmentectomy, and fistula repairs were performed.
- Successful surgical closure of the bronchopleural fistula using a bovine pericardial patch led to symptom resolution.
Implications:
- Cross-disciplinary treatment and a high degree of clinical suspicion are crucial for managing this rare condition.
- Bile duct hypoplasia must be considered as it may necessitate a thoracoabdominal surgical approach.
- This case underscores the importance of comprehensive management strategies for rare congenital malformations.
Introduction:
Congenital bronchobiliary fistula is an extremely rare malformation with high morbidity and mortality rates. Up to 2016, 36 cases had been reported worldwide.
Clinical Case:
11-year-old male patient with history of chronic lung disease and respiratory insufficiency, bile ptyalism and 66-80% arterial saturation, jaundice, asymmetric thorax, finger clubbing, and disseminated crackling rales. He was diagnosed through fibrobronchoscopy and CT-scan. After fistula closure and right pneumonectomy, recurrence occurred due to bile duct hypoplasia as evidenced by endoscopic retrograde cholangiopancreatography. Left lateral hepatic segmentectomy and fistula closure from the abdomen were carried out. Bronchopleural fistula persisted following intensive nutritional and antibiotic treatment. It was surgically closed using a bovine pericardial patch. Six months later, the patient had no symptoms.
Discussion:
Given how extremely rare this malformation is, cross-disciplinary treatment and a high grade of suspicion are needed. The presence of bile duct hypoplasia is to be considered, since it requires a thoracoabdominal approach.
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