Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis

Chronic primary intestinal pseudo-obstruction from visceral myopathy

“…Histological lesions found at the intestinal wall allow the inclusion of our patient in this etiologic group, since myenteric plexa and ganglion cells were free of involvement but muscle layers were deeply disrupted. These are sometimes intestinal smooth muscle lesions that develop secondary to other conditions (scleroderma, autoimmune disease, amyloido-296 (18,35,36). On other occasions these are intestinal morphogenesis alterations (presence of additional muscle layers; fusion of muscle layers together) (37), yet in others these are likely primary myocytic changes (atrophy and loss of muscle cells, and their replacement by fibrosis; focal accumulation of nuclei; absence of alpha-actin in the smooth muscle) (37,38).…”

A Case of Chronic Intestinal Pseudo-Obstruction

“…A nonabsorbable carbohydrate such as lactulose is ingested and a premature rise in exhaled H2 suggests the presence of intestinal bacteria. The diagnosis of chronic intestinal pseudoobstruction from scleroderma can be made with small bowel barium imaging or biopsy 20,21 . A "hidebound" configuration of the small bowel is a characteristic mucosal fold pattern in which there is a relative decrease in the distance separating the valvulae conniventes.…”

“…Less common presentations include diarrhea, rectal prolapse, spontaneous perforation, and colonic infarction. Radiographic evaluation of colonic involvement includes barium enema, which may show characteristic "wide mouth", or "fish mouth" diverticula, which are broad based true diverticula caused by the uneven distribution of atrophic muscularis 20 . Treatment includes conservative measures such as increased fluid and fiber intake.…”

Diagnosis and Treatment of Chronic Idiopathic Intestinal Pseudo-Obstruction Syndromes

“…Plain films of the abdomen show either a pattern of mechanical small bowel obstruc tion or diffuse intestinal ileus with both di lated small intestine and colon [26,27], Ap proximately 15% of patients have no evi dence of obstruction or ileus on their plain films, but do have abnormalities on barium study. A small number of patients may have pneumatosis intestinalis, and/or benign pneumoperitoneums.…”

“…We have never en countered peritoneal signs or evidence of real intestinal perforation in such cases, and ex ploratory surgery should be avoided in the absence of clinical signs of perforation. Bar ium contrast studies of the entire gastrointes tinal tract should be done whenever intesti nal pseudo-obstruction is suspected [26,27], The findings of multiple sites of abnormality increases the probability of CIIP. In contrast.…”