Open AccessIsolated ileal ganglioneuromatosis in an 11-year-old boy: Case report and review of literature
Author(s) -
Subhashis Mitra,
Sanghamitra Mukherjee,
Hema Chakraborty
Publication year - 2016
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/0377-4929.188109
Subject(s) - medicine , pathological , gastroenterology , gastrointestinal tract , constipation , pathology , disease , intestinal pseudo obstruction , rare disease , endocrine system , hormone
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.