Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
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        GI Motility Disorders
      
GI or intestinal motility disorders show up with clinical complaints or findings, even something as acute as volvulus. Cessation of abdominal intestinal motor activity might result in a clinical working diagnosis of "ileus". Abnormalities may vary from loss of action to hyperactivity to malcoordinated peristaltic activity...acute, subacute, or chronic & organic (we can define a definite anatomic or chemical disorder) or "functional" (we can't yet find a definite anatomic or chemical disorder...psychosomatic). Though pathologists might routinely process gut cross-sections, myenteric evaluation calls for longitudinal sections (maybe 12-20 in a colectomy)4. Enteric neural IHC markers might be: NSE for ganglion cells, S100 for glial tissue, CD117 for Cajal cells, and CD34 for fibroblast-like cells associated with the Cajal cells. In chronic situations, be aware of specialty GI motility disorders clinics such as at Mayo Clinic (their gastroparesis page HERE). Proximal disorders may be evaluated in nuclear medicine using a radio-labelled meal & distally maybe with external imaging of such as SITZMARKS capsules or internally with capsule endoscopy. There is a Foundation for functional GI disorders, many of which manifest as motility disorders.

Slowing or cessation of activity:

  • biliary dyskinesia: HERE.
  • esophagus: swallowing problems (dysphagia & measure with barium swallow &/or manometry)
    • mechanical:
      1. acute: viral & fungal (candida) esophagitis.
      2. chronic:
        • eosinophilic esophagitis (mechanical?) HERE
        • achalasia: term used for apparent distal obstructions but is more specific after EGD for obstruction apparently due to increased distal sphincter tone (functional achalasia ?) & often with some dilation of esophagus.
        • crico-pharyngeus muscle syndrome: by physical exam, the normal upward movement of the "Adam's apple" is reduced or absent9; diagnosible by barium swallow.
        • esophageal dysmotility...ultimately progressing to presbyesophagus.
        • Schatzki ring: an organic partially obstructing membrane.
        • stricture/stenosis:
          1. benign
          2. malignant
    • non-mechanical:
      1. acute:
        • esophagoparesis (organic vs. functional)
      2. chronic:
        • globus<: functional disorder = sensation of lump or tightness in throat; may be as recurrent acute episodes.
        • eosinophilic esophagitis (non-mechanical vs. mechanical?)
        • functional dysphagia: absence of clinical or histological evidence of GERD or other organic disorder.
        • chronic pseudo-obstruction (e. g., scleroderma/CREST).
        • as part of the more generic "chronic idiopathic pseudo-obstruction" (CIIP).
        • organic deposits: hemochromatosis, amyloidosis, etc.
  • stomach: Normal action: Peristaltic waves push food through the pyloric outlet as the muscle normally relaxes; then, toward closure, the final closing is due to normal mucosa slightly prolapsing to form the pyloric plug6. A physiological pseudodiverticular bulge forms during normal peristalsis, preceding the expulsion and the pyloric plug action 6. Peptic or other fibrosis of the channel mucosa/submucosa can prevent plug action and cause a component of either or both of low-grade gastric dumping or duodeno-gastric reflux6 emptying problems (measure with post-fasting food present at EGD, barium meal studies, gastroduodenal manometry, or SITZMARKS study, or gastric emptying study). One is looking for gastric outlet obstruction (GOO), outlet incompetence (with or without dynamic obstruction), and hyperperistaltic GERD (often interpreted by patients as postprandial vomiting when it is actually more like over-capacity regurgitation or rumination).There is a time-line spectrum between beginning pyloric outlet pathology and full, unremittant GOO.
    • mechanical dysmotility: see "normal action", above.
      1. acute outlet obstruction:
        • stenotic:
          • distal ulcer: edema and inflammatory tissue reactions cause an acute obstruction of a not-yet-diagnosed lesion.
          • prolapse: as edematous folds or polypoid lesion.
          • congenitally tight sphincter: non-hypertrophic, tight outlet gets obstructed by a chunk of food or a beazor (FA12-203).
          • hypertrophic pyloric sphincter: gets obstructed by other issues.
          • duodenal obstruction: manifesting as GOO, for the same variety of reasons as gastric obstructions, the duodenum can obstruct & seem to be GOO. An unusual variant is Bouveret Syndrome of gallstone duodenal obstruction due to choledochoduodenal fistula.
          • acute complication of stenotic: as an acute complication of a not-previously-obstructive, chronic lesion of subclinical outlet restriction or obstruction...for whatever reason (thickened pyloric sphincter, etc., suddenly with peptic [acid] or other irritations [alcohol, too spicey, viral gastritis, etc.]), the lining of the gastric outlet swells the outlet shut. This follows a meal or alcoholic or other irritant intake. If too overloaded by the meal, there may be involuntary (maybe seeming somewhat voluntary), bulemia-like or rumination-like or more intense regurgitation as the stomach (the patient) attempts to decompress & rid itself of the too large volume of contents. Avoid repeat episodes by (1) removal of the eating or drinking of the irritant and/or (2) treating the hyperacidity with daily Tums & Prilosec or such. In a too sudden or too volume-heavy obstruction, the stomach may shut down (a complexifying episode of acute gastroparesis). Peptic (acid) gastropathy or reactive (irritants) gastropathy may flare up enough to close a tight sphincter area. Also, a beazor or polypoid lesion can ball-valve the outlet into an intermittent or permanent occlusion.
        • non-stenotic: CMV or eosinophilic gastritis7.
      2. chronic: There is a time-line spectrum between beginning pyloric outlet pathology and full, unremittant GOO; causes may be pinpointed with EGD, barium swallow studies, and gastric emptying studies (barium studies and/or a combination of EGD findings and insertion of SITZMARKS capsules or capsule endoscopy distal to the suspected obstruction to show that rest of bowel has normal transit time).
        • benign peptic ulcer:
          • stenotic obstruction: most commonly, pyloric lesions are located in the superior channel over the lesser curve pyloric muscle "torus".
          • dynamic obstruction: if ulcer situates on distal torus aspect, it may erode the distal sphincter loop and rarely result in incompetence rather than stenosis; Torus plus distal-loop-dominant hypertrophy draws the radial plane of the outlet (in resected specimens) into a horizontal situation (with outlet directed more superiorly) such that an expanding inferolateral pseudodiverticulum forms [FA12-111] between the proximal & distal pyloric radial loops & enlarges far beyond the physiological pseudodiverticular bulge that forms during normal peristalsis6.
        • distal tumor.
        • hypertrophic pyloric sphincter: the supero-medial torus at the distal lessor curve & the distal pyloric loop of the sphincter can enlarge by congenital influence (sometimes with manifestation first in adulthood8); but adult distortions are more commonly secondary (highest % etiology is peptic), see above.
        • peptic & other obstructions: see above, acute.
        • as part of the more generic "chronic idiopathic pseudo-obstruction" (CIIP).
        • chronic pseudo-obstruction, secondary (e. g., scleroderma/CREST).
        • ischemic spincter: superior mesenteric artery syndrome7.
    • non-mechanical:
      1. acute:
        • gastroparesis: at level of CNS, autonomic, enteric nervous systems via gastric overload, traumatic, toxic, metabolic, medicinal, infectious, degenerative, and autoimmune etiologies (diabetes is by far most common cause). Acute intermittent gastroparesis episodes can complicate incomplete gastric outlet restriction or obstruction (GOO) [FA12-111] (and, see above).
        • gastromalacia: at autopsy one may find areas of marked mural gastric thinning...sometimes perforated (considered to be an agonal dissolution of the mural tissue).
      2. chronic:
        1. gastroparesis: diabetes ("gastroparesis diabeticorum"...L-06-7445, L-07-1465) is a most common cause & etiology of gastroparesis (autoimmune& others...see acute, above) & diagnosed in nuclear medicine via "gastric emptying study" with nothing by mouth after midnight before & then next day with Radiology meal of scrambled eggs containing a tracer; usually, half of activity has passed out of stomach within 90 minutes. If longer than 110 minutes to half empty, abnormal. Chronic pseudo-obstruction (see histology below) can be a gastroparesis cause. A combination of EGD findings and insertion of SITZMARKS capsules or capsule endoscopy distal to the sphincter or distal duodenum to show that rest of bowel has normal transit time. As with acute, there are many potential etiologies. By 2009, treated by dietary modification & maybe OTC or Rx domperidone (Motilium) or cisapride, erythromycine, or maybe Reglan = all being motility enhancers.
          • as part of the more generic "chronic idiopathic pseudo-obstruction" (CIIP).
          • excessive belching disorders: true aerophagia vs. functional.
          • nausea & vomiting disorders: when one is bothered several times per week with nasea & EGD is normal = " chronic idiopathic nausea"; "functional vomiting" is an average of an episode per week of vomiting that is in absence of chronic canabinoid use, absence of major psychiatric disorder or an eating disorder or rumination syndrome & negativity for CNS or metabolic disorder or abnormal EGD or barium study. "Cyclic vomiting" is 3 or more episodes per year of acute vomiting lasting a week or less (usually a family HX of migraines).
          • rumination syndrome in adults: this entity is when one recurringly burps up or regurgitates partly digested food into the mouth with spitting it out or rechewing & reswallowing. Differs from GERD in that tends to be before food has become acidic; etiology = functional vs. outlet partial obstruction.
        2. organic deposits: hemochromatosis, amyloidosis, etc.
        3. idiopathic regurgitation (functional?): while this may due to episodic dietary volume overload, it may happen ever in the absence of large volume intake.
  • duodeno-enteric: emptying or slow transit problems (measure with malabsorption tests or possibly capsule endoscopy).
    • mechanical:
      1. acute: when significantly myopathic, can result in elevated CPK.
        • volvulous/incarceration: myopathic effect will tend to affect lumenad components worse than mural periphery (such as muscularis propria externa) [L-06-9956].
        • stricture/ulcer: as with volvulous; ulcer & with obstructing passive capsule endoscope [L10-7512].
        • torsion/incarceration: as with volvulous.
        • external obstruction/incarderation: adhesions, endometriosis, etc.; changes as with volvulous.
      2. subacute & chronic:
        • the above causes of acute: but at a slower temporal profile. And, see Bouveret Syndrome, above.
        • chronic pseudo-obstruction: (e. g., scleroderma/CREST).
    • non-mechanical:
      1. acute:
        • duodenoparesis, enteroparesis: potential same causes as acute or chronic gastroparesis & rarely just as an isolated segment. In fact, though there may be a worst segment, paresis beyond the stomach likely to effect entire tract...enterocoloparesis.
        • enteromegally: Ogilvie's syndrome (colonic is more common)
        • "ileus" (adynamic ileus): due to effects of shock and/or anesthesia or operative-associated medications.
        • vague etiologies: at level of CNS, autonomic, enteric nervous systems via traumatic, toxic, vascular insufficiency (FA08-139), metabolic, medicinal, infectious, degenerative, and autoimmune etiologies.
      2. chronic:
        • chronic intestinal pseudo-obstruction1: see colonic, below.
        • irritable bowel syndrome (IBS): any duodeno-enteric component of IBS.
        • vague etiologies: at level of CNS, autonomic, enteric nervous systems via traumatic, toxic, metabolic, medicinal, infectious, degenerative, and autoimmune etiologies.
      3. with organic deposits: hemochromatosis, amyloidosis (L-06-6141), etc.
  • colonic/rectal: slow or rapid transit and pain & urgency problems (measure with transit time measures such as the SITZMARKS radiopaque rings sequence; rectal function with anorectal manometry or ability to expell an inserted baloon). Chronic constipation (category includes Rome III criteria "functional defecation disorder") falls into this category and consists of 2 main types: (1) obstructed defecation and (2) slow transit (chronic slow transit constipation syndrome)...traditionally thought due to recto-anal dysfunction (pelvic floor failure to relax, paradoxical floor contraction, defective or deficient rectal motor function and/or sensitivity4. Almost any of these etiologies can be tilted over into an acute or subacute status clinically in the medicated elderly, especially postoperatively.
    • mechanical:
      1. acute:
        • fecal impaction/constipation, with or without stercoral ulcer; volvulus of dilated bowel [L06-8447; L07-10934]. May see myopathic degeneration. See enteric acute volvulous, above.
      2. chronic:
        • histologically unremarkable: this suggests mechanical etiology likely (1) "functional defecation disorder" or (2) slow transit due to "obstructed defecation" of recto-anal dysfunction (pelvic flow failure to relax, paradoxical floor contraction, defective or deficient rectal motor function and/or sensitivity).
        • chronic stasis histology: in chronic stasis colonopathy [L12-3600], one might find (1) muciphages, even to a copious degree, in non-rectal colonic areas and even (2) mucosal and mesenteric lymphoid hyperplasia.
        • stenosis: chronic stenosing diverticulitis; IBD-C; neoplasia/tumor; mural constricting scar; externally variably occlusive adhesions, such as at splenic flexure in post-splenctomy case [L12-3600].
        • dolichocolon or redundant colon: abnormally long colon with chronic low-grade incompletely occlusive torsion [L12-3600].
        • diverticulosis and diverticulitis: diverticular protrusions probably secondary to effects of chronic malcoordinated peristalsis; as blind pockets of intestine, they are subject to acute & chronic inflammation with abscess production and even perforation. Strangely, even when not really inflammed, they are subject to bleeding of significant...event emergent... degrees, without one always finding a macroscopic bleed site when examining the colectomy specimen (often associated with chronic aspirin intake). Bleeding may be appreciated microscopically as RBCs mixed with lumenal mucous [L06-9851] in the diverticula. Associated muscular hypertrophy can cause a setting leading to a colitis within the segment that looks like IBD-UC, microscopically.
    • non-mechanical:
      1. acute:
        • colonoparesis: potential same causes as gastroparesis & rarely just as an isolated segment; if so problematic as to require hospital-based treatment, best to use the term "acute pseudo-obstruction".
        • acute idiopathic pseudo-obstruction (nontoxic megacolon) [mega= enlarged/dilated]...Ogilvie's syndrome1; studies have documented that up to 95% of the cases of acute colonic pseudo-obstruction are associated with medical [LMC-05-9716 venous congestion, mucosal hemorrhage & then localized pseudomembraneous colitis] or surgical conditions (secondary), with the rest being classified as idiopathic, sometimes with myopathic "pseudo-obstruction" [L07-8628]. The most commonly associated conditions for secondary etiology include trauma, pregnancy, cesarean delivery, severe infections, and cardiothoracic, pelvic, or orthopedic surgery2. This degeneration can be very focal & acute, subacute, or chronic and a basis for colonoscopic pneumatic perforation [L08-13361].
        • toxic megacolon:
          • Hirschsprung's complicated:
          • ulcerative colitis (IBD-UC) complicated:
          • ischemic colonopathy/colitis complicated:
          • pseudomembraneous colitis complicated:
          • chronic intestinal pseudo-obstruction complicated:
      2. chronic:
        • chronic inertia: absence of our diagnosing any of the below diagnoses in a patient with slow transit time and medically refractory constipation [L-05-2255]. May be diagnosed by oral ingestion of a "Sitz-Mark diagnostic test" capsule containing 24 radiopaque rings & transit time checked. See vague etiologies, above, with gastroparesis & enteroparesis.
        • Hirschsprung's disease: as pediatric or child or even adult onset...histology of full thickness biopsy in search of intestinal neuronal dysplasia (IND)...decrease or loss of ganglion cells & other abnormalities of myenteric innervation. Meissner's plexus just beneath the muscularis mucosae normally has a small cluster of, say, 1-5 ganglion cells for about every 1mm of length [L07-2013]. Auerbach's plexus between the interna and externa layers of the muscularis propria normally has a regularly intermittently visible nueral zone with fibers and ganglion cells. If ganglion cells seem decreased or absent, look for hypertrophy (increased prominence) of the tiny nerves in the plexi.
        • zonal colonic aganglionosis (ZCA): motility dysfunction (constipation) which is due to patchy loss of ganglia [L07-2992; may see ZCA & CIPO combo L07-10605] and therefore harder to diagnose.
        • intestinal neuronal dysplasia: hyperplastic...even giant...ganglion cells4.
        • obstructed defecation, colonic type: one finds enteric S100-pos. glial cell loss (a cell which "orchestrates motility") & reduced NSE-pos. neuroganglionic cells in superficial plexus...possibly explains lake of this variant of obstructed defecation to respond to biofeedback therapy4
        • cathartic colon: melanosis coli [L07-2992, L09-11652], when demonstrated, can imply this designation which is a sign that there is some as yet undiagnosed cause for laxative medication.
        • megacolon or megarectum: congenital, acquired, idiopathic (due to infectious or other neurological disease, systemic disease, metabolic disorders, medication or drug effects...Chagas disease is most common world-wide cause)[LMC-02-5403].
        • dolichocolon or redundant colon: abnormally long colon.
        • chronic intestinal pseudo-obstruction (CIPO): idiopathic (primary) or secondary (long outline of causes) dilated colon with thin walls (colonic atrophy, mural atrophy, "leiomalacia") [LMC-05-8635] to dilated but mostly normal (L06-8447; L07-10605) to stiff colon with thick muscular wall (mural colon hypertrophy) [LMC-04-8885; LMC-04-9493; L-05-2533]. Can just affect part of a colonic area. Always some dilatation with variable smoothing of mucosal folds and usually thick & thin areas of muscularis propria. The muscularis propria is normally 2-3MM thick (about 8-12g/cm of colonic length with most of paracolic tissue trimmed off). May be secondarily complicated by stercoral ulcers (or other secondary etiology) with perforation [LMC-07-6010; L07-7094]; could perforate following colonic inflation for colonoscopy [L07-6869]. Can be familial or sporadic, segmental to systemic (best review here1). Don't confuse this disorder with the mural thickening often seen in chronic diverticulosis/diverticulitis, which thickening (1) tends to be closely limited to the diverticular bowel segment, and is (2) negative for myopathic or neuropathic degeneration:
          • myopathic forms: thin...colonomalacia...[LMC-05-8635; LMC-05-9384] or thick walls show muscle degeneration and fibrous replacement. Scleroderma might be a cause.This degeneration can be very focal & acute, subacute, or chronic and a basis for colonoscopic pneumatic perforation [L08-13361].
          • neuropathic forms: reduced or nearly absent enteric neural apparatus and/or ganglia with CMV-like non-viral inclusions.
        • functional disorders:
          • irritable bowel disorder (IBS): abdominal pain which improves with defecation and either predominantly associated with diarrhea IBS-D) or with constipation (IBS-C).
          • functional bloating: in absence of any other functional GI syndrome, patient senses abdominal distension whether it can be measured or not.
          • functional constipation (chronic idiopathic constipation): in persons not fulfilling IBS-C criteria, there is persistently difficult, infrequent, or incomplete defecation.
          • functional diarrhea: at least 75% of stools are mushy or watery and without pain.
          • unspecified functional bowel disorder: cases of b owel symptoms not attributable to an organic etiology and not fitting criteria of the above 1-4.
        • with organic deposits: hemochromatosis, amyloidosis, etc.

Malcoordinated action or peristalsis:

  • esophagus:
  • stomach:
  • duodeno-enteric:
  • colonic/rectal:

Hyperactivity:

  • esophagus:
  • stomach: "dumping syndrome"
  • duodeno-enteric:
  • colonic/rectal: diarrhea of all sorts; be aware of the post-"starvation" "Refeeding Syndrome" with possible copious diarrhea, acute dementia, cardiac failure, coma, and convulsions. Re-intake of unregulated carbohydrates too quickly and hypokalemia low potassium likely present.

Combination hyper & malcoordinated defects:

  • esophagus:
  • stomach:
  • duodeno-enteric:
  • colonic/rectal:
    • irritable bowel syndrome (IBS): malcoordination and functional

References:

  1. Surgical Pathology of the GI Tract, Liver, Biliary Tract, and Pancreas. Odze, Goldblum, & Crawford, 1067 pages, 2004. [EBS]
  2. e-Medicine website: http://www.emedicine.com/
  3. "ROME III, The Functional Gastrointestinal Disorders", the entire issue of Gastroenterology 130(5):1377-1556, April 2006.
  4. Bassotti G, et. al., "Colonic neuropathological aspects in patients with intractable constipation due to obstructed defecation", Modern Pathology 20(3):367-374, March 2007.
  5. Lembo A & Camilleri M, Review Article: Current Concepts, Chronic Constipation, NEJMed 349(14):1360-8, 2 October 2003.
  6. Lu CC, et. al., "Pyloric deformation from peptic disease Radiographic evidence for incompetence rather than obstruction" [pyloric anatomy & function], DIGESTIVE DISEASES AND SCIENCES, 35(12):1459-1467, 1990 HERE.
  7. Mohammed AA, et. al., Gastric Outet Obstruction, Hepato-gastroenterology [non-peptic acquired causes], 54(80):2415-2420, 2007 HERE.
  8. Zaval C, et. al., "HypertrophicPyloricStenosis: Adult and Congenital Types Occurring in the Same Family", J. Med. Genetics 6(2):126-128, June 1969 HERE.
  9. Gulling J, et. al., "Surgical Management of Cricopharyngeal Disorders at MUSC as Compared to Statewide Trends", The J. of the SCMA, 109(1):6-8, March/April 2013.
(posted 25 August 2002; latest adjustment, 25 May 2014)
 
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