Pneumatosis and pseudo-obstruction in scleroderma.

Abstract

Two cases of pneumatosis cystoides intestinalis in scleroderma have been recently published (1, 2). Pseudo-obstruction in this disease is a better known phenomenon (9–12). The simultaneous occurrence of these findings has not yet been reported. During the years 1964 to 1967, three unusual cases of generalized scleroderma with both pneumatosis and pseudo-obstruction were seen at the University of Colorado Medical Center. The classical gastrointestinal changes in scleroderma (3–9) consist of a dilated, atonic esophagus with decreased peristalsis in the lower two-thirds, delayed gastric emptying, decreased motility of the gut with malabsorption patterns and findings of intestinal obstruction, and colonic atony with sacculation or pseudodiverticulum formation. The roentgenologic findings of pneumatosis cystoides intestinalis (PCI) are also well known (1, 2, 13–15). Multiple radiolucent cysts or linear streaks of gas are seen within the bowel wall or mesentery. Sometimes this condition is associated with a pneumoperitoneum which is usually of long duration and nearly always asymptomatic. Multiple theories on the etiology of PCI ((2, 16–19, 21–23, 29) have been discussed at great length in the recent literature and will not be argued or amplified here. Case Reports Case I: L. C., a 58-year-old white female, had suffered from Raynaud's phenomenon since 1959, In 1962 she noticed definite hardening of the skin of the hands, arms, and face. Dysphagia, nausea and vomiting, constipation, and severe abdomina distension led to her admission at another hospital for exploratory laparotomy. Filmy adhesions were noted in the right upper quadrant, secondary to a previous cholecystectomy. The surgical conclusion was “a small bowel obstruction probably caused by paralytic ileus.” The patient presented at University of Colorado Medical Center (UCMC) on Aug. 20, 1964, with obvious clinical scleroderma. Films of the hands, esophagus, small bowel, and colon confirmed clinical findings. She became progressively more cachectic and intermittently and chronically obstructed. Laparotomy revealed an “ileal adhesion.” However, she showed no objective improvement and eventually required long tube intestinal decompression for the eight months prior to her death. Pneumatosis intestinalis without pneumoperitoneum was first observed in January 1965 and became more marked two weeks before death. Autopsy in April 1965 revealed severe cutaneous and subcutaneous sclerosis. There was marked gastrointestinal smooth muscle atrophy with replacement by fibrous connective tissue. Most of the small bowel serosa was distended with small air bubbles. The lungs demonstrated delicate alveolar walls with only scattered minimal perivascular fibrosis. Bronchopneumonia was thought the terminal event.