Postgrad Med J (1990) 66, 61 - 62 i) The Fellowship of Postgraduate Medicine, 1990 Postgrad Med J: first published as 10.1136/pgmj.66.771.61 on 1 January 1990. Downloaded from

Spontaneous benign complicating scleroderma in the absence ofpneumatosis cystoides intestinalis

N.J.M. London, R.G. Bailey and A.W. Hall Department ofSurgery, Glenfield General Hospital, Leicester, UK.

Summary: We describe a 64 year old woman with a 3-year history ofscleroderma who presented as an emergency with increasing painless abdominal distention. Radiological investigations revealed a pneumoperitoneum in the absence ofeither visceral perforation or cystoides intestinalis. This is only the fourth report of spontaneous benign pneumoperitoneum complicating scleroderma without pneumatosis cystoides intestinalis. The possible aetiology of this condition is discussed.

Introduction Serious gastrointestinal involvement is present in 60 mmHg. The was grossly distended, approximately 50% of patients with scleroderma.' soft, non-tender and tympanitic on percussion. Spontaneous pneumoperitoneum is a rare comp- Urgent laboratory investigations showed a normal lication ofthe disease and is usually associated with haemoglobin and white cell count. Abdominal copyright. pneumatosis cystoides intestinalis.2 We report a (Figure 1) and chest X-rays revealed a large case of spontaneous pneumoperitoneum in a pneumoperitoneum and although there was small patient with scleroderma in whom there was no bowel and colonic dilatation there was no evidence of either visceral perforation nor of radiological evidence of a localized point of obs- pneumatosis cystoides intestinalis. truction within the bowel. Because the clinical picture was not compatible with a visceral perforation a peritoneal lavage was Case report performed. This yielded odourless gas with the

return of clear lavage fluid and a reduction in the http://pmj.bmj.com/ A 64 year old woman presented as an emergency patient's abdominal distension. It was concluded with constipation, vomiting and increasing painless that the diagnosis was that of benign spontaneous abdominal distension. Three years previously she peritoneum and conservative treatment was had been diagnosed as suffering from scleroderma initiated. Her condition improved over the next and had been admitted to hospital two years later week such that she was able to eat normally. with a self-limiting episode of intestinal obstruc- Repeat contrast studies including a gastrograffin tion. Contrast studies ofthe oesophagus, small and meal, small bowel enema and large bowel enema large bowel at that time had shown a dilated atonic did not show any evidence of visceral perforation on October 2, 2021 by guest. Protected oesophagus with free gastro-oesophageal reflux nor of pneumatosis cystoides intestinalis. and small bowel dilatation with an increased transit The patient's condition gradually improved until time. There was no evidence of pneumatosis cys- 19 days after admission when she suddenly toides intestinalis. Physical examination on this developed a dense right-sided hemiplegia and died. occasion revealed a typical sclerodermatous facies Permission for a post-mortem examination was with sclerodactyly and dry gangrene of the distal sought but was not obtained. left ring finger. She was apyrexial with a pulse rate of 88 per minute and blood pressure of 100/ Discussion Correspondence: N.J.M London, M.R.C.P., F.R.C.S., Department of Surgery, Clinical Sciences Building, Gastrointestinal involvement in scleroderma is Leicester Royal Infirmary, PO Box 65, Leicester LE2 characterized by atrophy of the muscularis propria 7LX, UK. and its replacement by collagen tissue. These Accepted: 16 May 1989 changes lead to decreased peristaltic activity with 62 CLINICAL REPORTS Postgrad Med J: first published as 10.1136/pgmj.66.771.61 on 1 January 1990. Downloaded from

Figure1Abdominal X r'ay showing neumoeritoneum with small and large.bowel.dilatation.. Figure I Abdominal X-ray showing pneumoperitoneum with small and large bowel dilatation.

consequent stasis and distension. Although any rowth, increased thickness of the unstirred water part of the may be involved, layer, or thickening, fragmentation and collagenous the organs involved in decreasing order of fre- infiltration of the muscularis mucosa. quency are the oesophagus, small bowel, colon and Spontaneous pneumoperitoneum is a rare com- .3 The oesophageal motor abnormalities plication in patients with intestinal scleroderma copyright. may lead to gastro-oesophageal reflux, and was first reported by Fallon in a patient with oesophagitis and stricture. Oesophageal moniliasis scleroderma and pneumatosis cystoides intes- is not infrequent in scleroderma. Abnormalities of tinalis.2 There have only been three previous small intestinal motility are present in 40% of reports of spontaneous pneumoperitoneum in the patients and may lead to atony, dilatation and absence of pneumatosis cystoides intestinalis.46 large, wide-necked diverticula. These diverticula The aetiology of the pneumoperitoneum in these represent localized areas of dilatation due to circumstances is obscure although it has been smooth muscle atrophy. Colonic involvement, suggested that recurrent microperforations along demonstrated by barium enema, occurs in 10 to the gastrointestinal tract allow air to leak into the http://pmj.bmj.com/ 50% of patients,3 the commonest finding being peritoneal cavity.' sacculations. A variety of complications may It is important that doctors should be aware of supervene in patients with scleroderma. Intestinal spontaneous benign pneumoperitoneum in ischaemia and perforation may be due to vas- patients with scleroderma and that the absence of culitis. The colonic motor disturbance predisposes pneumatosis cystoides intestinalis does not exclude to the formation offaecaliths, which, ifthey become the diagnosis. Unnecessary surgery in these lodged in a diverticulum, produce an appearance patients will not only compromise their intestinal that can be mistaken for a colonic tumour. Intestinal function but also place their lives at risk. on October 2, 2021 by guest. Protected malabsorption may result from bacterial overg-

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