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Prune-belly syndrome with anorectal malformation
J K Mahajan1, S Ojha, K L N Rao
1Department of Pediatric Surgery, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India. jkmahajanjk@sify.com
Summary
Prune-belly syndrome (PBS) rarely occurs with anorectal malformations (ARM). This case highlights the severe complications and management challenges in neonates with this rare PBS-ARM association.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Neonatology
Background:
- Prune-belly syndrome (PBS) is a congenital disorder characterized by abdominal muscle deficiency, urinary tract anomalies, and undescended testes.
- Gastrointestinal anomalies, such as a wide mesentery, are common in PBS, but anorectal malformations (ARM) are rare associations.
Observation:
- A one-day-old male neonate presented with prune-belly syndrome (PBS) and a rare co-occurrence of high anorectal malformation (ARM) and urethral stenosis.
- The neonate underwent initial sigmoid colostomy for ARM, followed by emergency surgery for necrotizing gastritis and gastric perforation.
Findings:
- The rare association of PBS with high ARM and urethral stenosis presented significant management challenges.
- Postoperative complications included necrotizing gastritis, gastric perforation, requiring partial gastrectomy and vesicostomy, ultimately leading to fatal septicemia.
Implications:
- This case underscores the critical need for vigilant monitoring and multidisciplinary management in neonates with rare PBS-ARM associations.
- The review of similar cases highlights the complexities and potential pitfalls in surgical interventions, emphasizing the importance of early diagnosis and tailored treatment strategies.