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
hypertrophic pattern of visceral myopathy
hypertrophic changes in the muscularis propria
congenital visceral myopathy (CVM)
neonatal digestive obstruction
failure to thrive
hypertrophic muscularis propria
cholecystitis and cholelithiasis
no familial association
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
submucosa reduced to a thin web of connective tissue
obliteration of medium caliber vessels and submucosal
The 2 layers of muscularis propria maintain their outer longitudinal
and inner circular orientation but have more intersecting fascicles than
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.
Treatment and prognosis
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#