title: Intestinal neuronal dysplasia (type B) causing fatal small bowel ischaemia in an adult: a case report.
abstract: Intestinal neuronal dysplasia (IND) is a well-documented but controversial entity defined as a congenital malformation of gastrointestinal innervation caused by dysplastic embryonal development of the enteric nervous system. Mainly described as a colonic disorder mimicking Hirschsprung's disease in the paediatric population, involvement of the ileum is rare. We report a case of fatal IND of the small intestine in a 58-year-old man presenting with a recent onset of features of small intestinal obstruction. IND has been described as a cause of colonic pseudo-obstruction and chronic constipation in adults, but to the best of our knowledge, this is the first report of IND of the ileum occurring in an adult causing fatal small bowel obstruction. A Medline search yielded no cases of ileal involvement with IND in adults.
authors.affiliation: Departments of Surgery, RMV Hospital, No. 138, AECS Layout, Sanjaynagar, Bangalore 560094, India. wetware@sify.com
authors.forename: Rajagopalan
authors.initials: R
authors.lastname: Vijayaraghavan
authors.forename: Rangashamaiah
authors.initials: R
authors.lastname: Chandrashekar
authors.forename: Avinash
authors.initials: A
authors.lastname: Melkote Jyotiprakash
authors.forename: Ravi
authors.initials: R
authors.lastname: Kumar
authors.forename: M V
authors.initials: MV
authors.lastname: Rashmi
authors.forename: Charalingappa
authors.initials: C
authors.lastname: Shanmukhappa Belagavi
mesh_terms: D017615:Enteric Nervous System / Q000002:abnormalities*; D017809:Fatal Outcome; D005726:Ganglia, Parasympathetic / Q000473:pathology; D006801:Humans; D007077:Ileal Diseases / Q000209:etiology / Q000473:pathology; D007082:Ileum / Q000098:blood supply*; D007415:Intestinal Obstruction / Q000209:etiology / Q000473:pathology; D007511:Ischemia / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged