PMID: 12658043
Issn Print: 0277-2116
Publication Date: 2003/04/01
Chronic Intestinal Pseudoobstruction Associated With Altered Interstitial Cells of Cajal Networks
Ariel E. Feldstein; Steven M. Miller; Mounif El-Youssef; David Rodeberg; Noralane M. Lindor; Lawrence J. Burgart; Joseph H. Szurszewski; Gianrico Farrugia
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Excerpt
Chronic intestinal pseudoobstruction (CIPO) is a group of disorders characterized by repeated episodes of symptoms and signs of obstruction without a mechanical cause (1–4). CIPO is thought to be the result of a motility disorder of the gastrointestinal tract and can be broadly divided into myopathic, in which the primary defect is at the level of the smooth muscle cell, and neuropathic, in which the primary defect is at the level of the enteric nervous system (1–4). CIPO is further divided into familial and sporadic types. Sporadic CIPO may be the result of connective tissue diseases, general neurologic diseases, infections, drugs, and tumors (1–4). A subset of CIPO, idiopathic CIPO, is thought to result from a defective neuronal myenteric network (1). Full-thickness biopsies from patients with idiopathic CIPO may show neuronal damage, but many times the biopsies are unrevealing (1). A role for interstitial cells of Cajal (ICC) in coordinating intestinal motility has become more apparent in recent years (5–10). ICC are found throughout the gastrointestinal tract interspersed within the circular and longitudinal muscle layers and also form a dense network at the level of the neuronal myenteric plexus in the stomach, small intestine, and colon, and a second network at the level of the deep muscular plexus in the small intestine and at the submucosal plexus in the large intestine (11–13). This report describes a patient with CIPO with an intact enteric nervous system and a normal distribution of ICC within the muscle layers but with absent ICC networks in the myenteric and submucosal regions.
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