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myopathic CIPO

Thursday 26 November 2009

Synopsis

- chronic intestinal pseudoobstruction (CIPO)
- intestinal failure
- visceral myopathy
- atrophic muscularis propria or hypertrophic muscularis propria
- atrophic pattern of injury to the muscularis propria

  • vacuolar degeneration of myocytes
  • loss of muscle fibers
  • honeycomb pattern of fibrosis

Hypertrophic changes can be observed in visceral myopathy, but the hypertrophy typically represents only a minor feature, typically limited to the internal layer of the muscularis propria.

The muscular hypertrophy can be the dominant pattern of injury and can involve both layers of muscularis propria rather than just the inner circular layer.

See also

- chronic intestinal pseudoobstruction (CIPO)
- visceral myopathy

  • infantile visceral myopathy (congenital visceral myopathy)

References

- Congenital visceral myopathy with a predominantly hypertrophic pattern treated by multivisceral transplantation. Koh S, Bradley RF, French SW, Farmer DG, Cortina G. Hum Pathol. 2008 Jun;39(6):970-4.
PMID: #18329691#

- 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#