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Bidirectional Glenn shunt in an infant with prune-belly syndrome
1Department of Thoracic and Cardiovascular Surgery, Hacettepe University, Faculty of Medicine, 06100 Ankara, Turkey. rdogan@hacettepe.edu.tr
Insights
Prune-belly syndrome (PBS) typically involves abdominal muscle deficiency, undescended testes, and urinary issues. This case highlights a rare PBS association with complex congenital heart disease, requiring specialized surgical and postoperative care.
Area of Science:
- Pediatric Cardiology
- Congenital Anomalies
- Medical Case Reports
Background:
- Prune-belly syndrome (PBS) is characterized by abdominal muscle deficiency, bilateral cryptorchidism, and urinary tract malformations.
- Complex congenital heart disease (CHD) can occur alongside PBS, presenting unique clinical challenges.
Observation:
- An eleven-month-old boy presented with PBS and a complex cardiac anomaly.
- The patient exhibited isolated dextrocardia, single ventricle, pulmonary atresia, and other cardiovascular abnormalities.
Findings:
- A bilateral bidirectional Glenn shunt was successfully performed.
- The patient had a patent ductus arteriosus-dependent pulmonary blood flow, necessitating careful management.
Implications:
- This case underscores the importance of recognizing and managing complex CHD in patients with PBS.
- Specialized postoperative pulmonary care is crucial for improving outcomes in such cases.
- Further research into the co-occurrence of PBS and complex CHD may inform treatment strategies.
Abstract:
The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.