hypertrophic myopathic CIPO

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