|
Visceral myopathy: congenital
|
Rare. Involvement of the muscularis propria of the gut which is one of the possible causes of chronic intestinal pseudo-obstruction syndrome (CIPO) (see this term) because there is intestinal hypoperistalstism. Sporadic cases or familial cases with autosomal recessive or autosomal dominant inheritance.
Depending on the location of the disease and histologic picture, different types are identified:
|
Nme VM = visceral myopathy |
affected organs |
presentation |
vacuolated
|
atrophy of muscular fibers |
Hypertrophy of the muscularis propria |
|
VM sporadic |
esophagus, stomach, small bowel, colon |
dilatated digestive tract |
+ |
+ |
not reported |
|
VM I |
esophagus, colon, bladder |
dilatated esophagus |
+ |
+ |
not reported |
|
VM II gene ACTG2 (2p13.1) |
stomach, small bowel, ocular muscles |
dilatated stomach and small bowel, |
+ |
+ |
not reported |
|
VM III |
esophagus, stomach, small bowel, colon |
dilatated digestive tract |
+ |
+ |
not reported |
|
VM IV |
stomach, small bowel |
thin small bowel, normal esophagus and colon |
+ (external layer) |
+ (external layer) |
+ (external layer) |
|
VM hypertrophic |
stomach, small bowel, colon, sometimes bladder and biliary tract |
dilatated small bowel, and colon |
+ but focally |
+ very localized |
++ all layers are involved |
|
muscular idiopathic
of the digestive tract |
esophagus, stomach, small bowel |
no dilation : wall thickening |
- |
- |
+, internal layer or all muscular layers |
Anesthetic implications:
check the blood electrolyte and protein levels, liver function (risk of liver disease secondary to parenteral nutrition); difficult venous access - peripheral or central (thrombosis following repeated central venous access). "Full esophagus" (cfr achalasia) in case of esophageal involvement. Delay of gastric emptying. Risk of latex allergy, the primary prevention of which is recommended
References:
- Burt N, Williams AR.
A pseudosyndrome, a real risk : aspiration on induction in a child with chronic idiopathic pseudoobstruction.
Am J Anesthesiol 1999; 26: 223-5.
- Koh S, Bradley RF, French SW, Farmer DG, Cortina G.
Congenital visceral myopathy with a predominantly hypertrophic pattern treated by multivisceral transplantation.
Human Pathology 2008; 39:970-4.
- Dranove JE, Applegate K, Croffie JM.
Type I familial visceral myopathy presenting with CT findings of superior mesenteric artery syndrome in an adolescent.
J Pediatr Gastroenter Nutrition 2009; 49: 379 (image of the month).
Updated: August 2020