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#