Isolated ileal ganglioneuromatosis in an 11-year-old boy: Case report and review of literature

• Main Authors: Subhashis Mitra, Sanghamitra Mukherjee, Hema Chakraborty
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2016-01-01
• Series: Indian Journal of Pathology and Microbiology
• Subjects: Diffuse; ganglioneuromatosis; intestinal; isolated
• Online Access: http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2016;volume=59;issue=3;spage=365;epage=367;aulast=Mitra

Ganglioneuromatous proliferation in the gastrointestinal tract is a rare occurrence and is usually associated with specific syndrome complexes such as multiple endocrine neoplasia Type 2B or von Recklinghausen's disease. We report here a case of diffuse intestinal ganglioneuromatosis, presenting as intestinal obstruction and chronic constipation in an 11-year-old boy. Sporadic cases of intestinal ganglioneuromatosis in the absence of any systemic manifestations are a very rare cause of enteric motility disorders in childhood, and we discuss the pathological and clinical significance of this finding. Histopathological identification of this uncommon cause of a common pediatric problem is important since the condition is amenable to surgical treatment.