4.

17 Chronic Intestinal Pseudo-Obstruction

Annu Rev Med 1999;50:37; Gut 1997;41:675
Epidem: Rare.
Pathophys: This disorder is defined by the radiographic picture of chronic obstruction in the
absence of a mechanical obstruction. A variety of underlying diseases may result in a chronic
defect in gut motility. In most pts, the disorder is secondary to diseases such as scleroderma,
amyloid, or a paraneoplastic syndrome associated with malignancy. In some pts, there is a defect
in enteric smooth muscle (hollow visceral myopathy), and in others there is a defect of the
enteric nervous system.
Sx: The predominant sx are pain, distension, vomiting, constipation, and diarrhea. A family
history may suggest one of the primary disorders.
Si: Distension or signs of an associated collagen-vascular, or neurologic disease.
P.213
Crs: The course in pts with the secondary form depends on the associated disorder. The course in
those with primary gut myopathy or neuropathy is that of chronic illness, pain, and malnutrition.
Cmplc: Bacterial overgrowth, malnutrition.
Diff Dx: Mechanical obstruction and mucosal disease such as Crohn's must be excluded.
Lab: A CMP, thyroid studies, Mg
++
, and CBC are obtained. In specialty centers, motility and
transit studies may be performed. Fibrosis and other morphologic abnormalities are seen on full
thickness biopsies or resected specimens.
X-ray: Plain films show dilated bowel, giving a radiographic impression of obstruction though
no mechanical obstruction exists.
Rx: Promotility agents are usually ineffective. Therapy is supportive with nutrition and rx of
bacterial overgrowth. Surgery is indicated in severe, symptomatic distension in order to place a
decompressive tube enterostomy (Am J Gastro 1995;90:2147). When there is localized pseudo-
obstruction, resection or bypass can be performed.