Idiopathic intestinal pseudo-obstruction: a familial visceral neuropathy

Clin Genet. 1980 Oct;18(4):291-7. doi: 10.1111/j.1399-0004.1980.tb00888.x.


Four individuals with idiopathic intestinal pseudo-obstruction (IIP), a familial visceral neuropathy with autosomal dominant inheritance, are presented. The disorder is characterised by abdominal colic, abdominal distension and diarrhoea, and is of a progressive nature with relapses and remissions. The main feature is a disturbance of motility in the affected intestine, with histological evidence of a neuronal disorder, characterised by hyperplasia and eventual atrophy. Management of the condition is difficult; initially, treatment should be conservative by nasogastric suction with intravenous nutrition. When laparotomy is undertaken to exclude mechanical obstruction, resection of the intestine is inadvisable, and ileo-colic anastomosis may be beneficial.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Genes, Dominant
  • Humans
  • Intestinal Diseases / genetics*
  • Intestinal Diseases / pathology
  • Intestinal Diseases / therapy
  • Intestinal Obstruction / diagnosis*
  • Male
  • Neuromuscular Diseases / genetics*
  • Neuromuscular Diseases / pathology
  • Neuromuscular Diseases / therapy
  • Pedigree