Familial visceral myopathy is the most common cause of chronic primary (idiopathic) intestinal pseudo-obstruction (CPIPO).

Synopsis

 segmental dilatation of the alimentary tract

• multiple sites
• megaduodenum

 degenerating muscle cells
 smooth muscle fibrosis

• full thickness of the muscularis propria
• often more prominent in or limited to the external layer

 degenerating muscle cells: pale, poorly defined, and fragmented
 residual thread-like remnants surrounded by collagen
 destroyed muscle cells
 apparent spaces surrounded by collagen
 vacuolated appearance of longitudinal and circular muscles

Differential diagnosis

 progressive systemic sclerosis

Synopsis

 congenital myopathic pseudo-obstruction
 smooth muscles of the digestive tract (megaduodenum and megacolon) and the urinary bladder (megacystis)
 megaduodenum
 megacystis

See also

 megacystis-microcolon-intestinal peristalsis syndrome

References

 Guze CD, Hyman PE, Payne VJ. Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome. Am J Med Genet. 1999 Jan 15;82(2):114-22. PMID: #9934973#

 Schuffler MD, Pagon RA, Schwartz R, Bill AH. Visceral myopathy of the gastrointestinal and genitourinary tracts in infants. Gastroenterology. 1988 Apr;94(4):892-8. PMID: #3345889#

 Alstead EM, Murphy MN, Flanagan AM, Bishop AE, Hodgson HJ. Familial autonomic visceral myopathy with degeneration of muscularis mucosae. J Clin Pathol. 1988 Apr;41(4):424-9. PMID: #3366929#

 Fitzgibbons PL, Chandrasoma PT. Familial visceral myopathy. Evidence of diffuse involvement of intestinal smooth muscle. Am J Surg Pathol. 1987 Nov;11(11):846-54. PMID: 3674282

 Mitros FA, Schuffler MD, Teja K, Anuras S. Pathologic features of familial visceral myopathy. Hum Pathol. 1982 Sep;13(9):825-33. PMID: #6896696#

 Smith JA, Hauser SC, Madara JL. Hollow visceral myopathy: a light- and electron-microscopic study. Am J Surg Pathol. 1982 Apr;6(3):269-75. PMID: #7102905#