Supernumerary Remnant Vas Deferens Like Wolffian-Derivatives in a Young Child With Abdominal Cocoon Syndrome—A Clinicopathological Novelty

• Published in: Pediatric and developmental pathology 2021-09, Vol.24 (5), p.455-459
• Main Authors: Agarwal, Shilpi, Gaur, Kavita, Agrawal, Nikita, Puri, Archana
• Format: Article
• Language: English
• Subjects: Child; Humans; Intestinal Obstruction - diagnosis; Intestinal Obstruction - etiology; Intestinal Obstruction - pathology; Intestine, Small - pathology; Male; Syndrome; Vas Deferens - abnormalities; Wolffian Ducts - abnormalities
• ISSN: 1093-5266; 1615-5742
• DOI: 10.1177/10935266211001639

The abdominal cocoon syndrome is a rare cause of recurring intestinal obstruction in children. It refers to encasement of the small bowel by a fibrocollagenous membrane forming a cocoon. We report a nine year old male presenting with abdominal pain, distension, bilious vomiting and inability to pass stool and flatus for two days. In view of a persistently increasing bilious nasogastric output, an urgent exploratory laparotomy was performed. The small bowel loops were matted together forming a cocoon densely adherent to the parietal peritoneum with supra-colic fibrous bands. The bands histologically displayed multiple ductal remnants with epithelium resembling that of ductus deferens. These structures showed immunopositivity for pan-cytokeratin and basal CD10.Workup for tuberculosis and other etiological causes was unremarkable. This is the first documented case of abdominal cocoon in a pediatric subject associated with supernumerary wolffian remnants.