Affiliation:
1. Departement of Pediatrics, Hedi Chaker Hospital, Sfax, TUNISIA,Faculty of Medicine of Sfax, University of Sfax, Sfax, TUNISIA
2. Department of Pediatric Surgery, Hedi Chaker Hospital, Sfax, TUNISIA,Faculty of Medicine of Sfax, University of Sfax, Sfax, TUNISIA
Abstract
Bannayan-Zonana syndrome (BRRS) is a rare genetic disorder characterized by macrocephaly, numerous soft tissue and visceral hamartomas, and lipomas. Because of the risk of fatal bleeding and visceral neoplasia in adulthood, recognizing this disease is critical. We reported a new pediatric case of BRRS in an 18-month-old female infant with lipomas, macrocephaly, and gastrointestinal hamartomatous polyps that prompted ileo-ileal intussusception and protein-losing enteropathy. Patients with BRRS require a comprehensive approach. Our case is unique in its sporadic occurrence and the presence of protein-losing enteropathy as a gastrointestinal polyposis manifestation. Pediatric patients with multiple lipomas, protein-losing enteropathy, and intestinal intussusception should be assessed for BRRS.
Subject
History,History and Philosophy of Science,Multidisciplinary,Social Sciences (miscellaneous),Geography, Planning and Development,Marketing,Strategy and Management,Industrial relations,Business and International Management,Business, Management and Accounting (miscellaneous),Management of Technology and Innovation,Management Science and Operations Research,Information Systems and Management,Organizational Behavior and Human Resource Management,Electrical and Electronic Engineering,Computer Science Applications,Mechanical Engineering,Transportation,Computational Mechanics,Management Science and Operations Research,Computer Science Applications,Theoretical Computer Science,Computer Science Applications,General Mathematics,Software,Political Science and International Relations,Sociology and Political Science,Mechanical Engineering,Mechanics of Materials,Algebra and Number Theory