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hypertrophic myopathic CIPO

Thursday 3 December 2009

A hypertrophic pattern in VM, dominating the atrophic pattern, is exceedingly
rare. As a pattern of injury, hypertrophy is almost absent from the description of visceral myopathy in much of the literature.

Much of literature on CIP0 and CVM is contained within the
clinical and surgical medical specialties and is less well represented in
anatomical pathology, and it is possible that the hypertrophic patterns are
underreported.

Clinical synopsis

- hypertrophic pattern of visceral myopathy
- intestinal failure.
- hypertrophic changes in the muscularis propria
- congenital visceral myopathy (CVM)
- meconial ileus
- neonatal digestive obstruction
- emesis
- failure to thrive
- biliary obstruction
- hypertrophic muscularis propria
- cholecystitis and cholelithiasis
- abdominal adhesions
- ureteral obstruction
- no familial association

Pathological synopsis

- visceral myopathy
- small bowel dilatation to 15 cm
- thickened intestinal wall (0.8 cm)
- mucosa lacking the mucosal folds, red and “leathery”
- pancreatic ductal ectasia
- diffuse marked muscular hypertrophy of both layers of the
muscularis propria
- submucosa reduced to a thin web of connective tissue
- obliteration of medium caliber vessels and submucosal
plexus
- The 2 layers of muscularis propria maintain their outer longitudinal
and inner circular orientation but have more intersecting fascicles than
typically encountered.
- The intersecting fascicles crossed the plane normally occupied by the myenteric plexus.
- The myenteric plexus is nearly obliterated by the hypertrophic layers of the muscularis propria.
- The muscularis mucosae and remaining mucosa do not have any abnormality.
- No overt vascular abnormality.
- No vacuolar degeneration of myocytes.
- Some pericellular clearing at high power.
- Inflammation absent.

Treatment and prognosis

- multivisceral transplantation

See also

- achalasia
- pyloric stenosis

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#