Rare Diseases & Orphan Drug

Intestinal duplication cyst is a rare condition and may be the cause of small bowel obstruction in children. In pediatric age group it should be considered as an important diff erential diagnosis in children who presented with recurrent abdominal pain and or recurrent obstruction. Diagnosis of duplicated intestinal cyst is clinically always diffi cult; therefore, defi nitive diagnosis may only be made at laparotomy. Gastrointestinal Duplication (GIDs) is rare congenital malformation, which can arise from mouth to the anus. May vary greatly in presentation, size, location, and symptoms. It t prevalence of 1:4500 births, predominantly in white males2/3 of all intestinal duplication discovered in within fi rst 2 years of life with 1/3 identifi ed in the new born period. Due to its rarity of these lesions, they frequently present at both diagnostic and therapeutic Challenges. Duplication of the gastrointestinal tract are cystic or tubular structures whose lumen are linked by a mucous membrane usually supported by smooth muscle and intimately associated with the alimentary tube. Th e histology reveals the characteristic lining of intestinal mucosa. They occur because of congenital aberration during gut development which may be found anywhere from the tongue to the lower rectum. Although they both arise from a redundant morphogenesis, the dorsal non-vitelline enteric malformation of the duplication cyst have a diff erent embryological origin to those associated with the vitellointestinal duct (Meckel's diverticulum), and, about a half present within a month of birth and two-thirds in the fi rst year. The most common site is the small intestine (50%), particularly the ileum (35%) with the cystic type being more common than the tubular type. Jejunum (10% and duodenum (5%). Although rare, intestinal duplication cyst is an important diff erential diagnosis for recurrent abdominal pain in the pediatric age group, and rarer in adulthood.