DDSEP 2024 Motility Flashcards
What is chronic intestinal pseudobstruction (CIPO)?
Long term intestinal dilation and dysmotility in the absence of mechanical obstruction.
Many causes for this
What are the classifications of disorders in CIPO?
Myopathic disorders (those affecting smooth muscle of intestines) and neuropathic disorders (those affecting central, autonomic, or ENS
What is the distinguishing feature of neuropathic disorders on EMG studies?
What about myopathic?
Neuropathic- intestinal contractions are normal amplitude but are uncoordinated.
Myopathic- contractions are either absent or low average amplitude.
Some disorders have overlap (amyloidosis, systemic sclerosis etc)
What workup do you do for suspected CIPO?
Exclude obstruction (CT-E, SBFT) and measure the GI and colonic transit.
Consider manometry and blood tests (list on evernote) and an autonomic reflex scan.
How can you treat CIPO? (Other than meds)
Maintain nutrition, facilitate bowel clearance, and avoid surgery if possible.
Osmotically active fiber containing an emollient agent may help with slow colonic transit.
Other meds discussed on another slide.
Effective treatment is lacking.
What meds can help with CIPO?
Octreotide can help symptoms but delay gastric emptying, so dose 2 hours after last meal at bedtime to sweep stuff into colon with MMC.
Erythromycin can help but not as helpful with lower GI motility.
Reglan/domperidone doesn’t work.
May need abx for SIBO rotating.
What is Familial Visceral Neuropathy
Rare group of disorders that affect the ENS and thus degeneration of the myenteric plexus with 2 phenotypes
Type I- AD inheritance with onset at any age
Type II- AR with infancy onset
What is familial visceral myopathy (FVM)?
You get degeneration and fibrosis of the GI SM and there are 3 types
Type 1- AD and manifests after 1st decade with pseudoobstruction. Will get megacystitis, uterine inertia, mydriasis
Type 2- AR inheritance (“Mitochondrial neurogasrointestinal encephalomyopathy”) with severe GI dysmotility, pitosis, skeletal muscle weakness. Skeletal muscle biopsy shows “ragged red fibers”
Type 3 FVM- AR onset middle age with pseudoobstruction, no extra GI symptoms
What are the non-esophageal manifestations of systemic sclerosis?
SB 2nd most affected, you get pseudoobstruction.
May also get delayed gastric emptying, colonic inertia, fecal incontinence (nighttime typically with weakness of internal anal sphincter which is SM)
Octreotide may help (give bedtime after meals)
What are the extra-esophageal manifestations of Chagas?
Usually cardiac involvement but can have bowel involvement with gastroparesis, SB dysmotility, or megacolon
What should you suspect in a middle aged smoker with recent onset of nausea, vomiting, or feeding intolerance?
Paraneoplastic visceral neuropathy with intestinal pseudoobstruction a/w IgG ab’s cross reacting with tumor antigens destrying ENS
