JRSM >
Volume 96, Number 8 >
Pp. 406-407
Wycombe General Hospital, High Wycombe HP11 2TT, UK
Correspondence to: J Ramus E-mail: jimramusuk{at}yahoo.co.uk
CASE HISTORY
A man of 43 was referred after 24 hours of feeling generally unwell with intermittent rigors. PNH had been diagnosed 18 years previously, and regular blood transfusions for his anaemia had resulted in iron-overload diabetes mellitus. 8 years before the present episode he had had a small-bowel resection for ischaemia secondary to venous thrombosis.
On examination his abdomen was distended and slightly tender in the left lower quadrant, without obvious peritonism. Haemoglobin was 6.4 g/dL and platelet count 16 x 109/L. Blood indices were otherwise normal. A plain abdominal X-ray showed dilated small-bowel loops. Adhesional small-bowel obstruction was diagnosed and initial management was conservative. After three days the abdominal distention had not resolved and a CT scan (Figure 1) showed gross small-bowel dilatation with an area of collapsed small bowel distally, highly suggestive of a mechanical obstruction. At laparotomy, with blood-transfusion and platelet cover, he was found to have a segment of gangrenous small bowel with a stricture. This was resected and the bowel was anastomosed by an extramucosal technique. The patient spent the first three postoperative days on the intensive care unit for close monitoring, and received total parenteral nutrition for five days until enteral feeding was started. No further blood or platelet transfusions were required. Histopathological examination of the operative specimen showed extensive haemorrhagic infarction secondary to venous thrombosis.
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COMMENT
PNH is an acquired disease characterized by attacks of intravascular haemolysis and haemoglobinuria. It can lead to profound anaemia, thrombocytopenia and leucopenia and is often complicated by venous thrombosis.1 The thrombotic tendency may be related to deficiency of a glycosyl-phosphatidylinositol anchor protein.2 Thrombotic complications are the most common cause of death in PNH, and about 40% of patients have one or more episodes of venous thrombosis during their illness.3 The incidence of mesenteric venous thrombosis leading to bowel ischaemia is between 3% and 8%.4
In 1966, Blum and Gardner5 described successful laparotomy in two patients with PNH—one with infarction of the terminal ileum, who underwent segmental resection; the other with an infarcted caecum, treated with a limited right hemicolectomy. Subsequently, Doukas et al.6 reported resection of two areas of ischaemic bowel in another patient, with good postoperative results. A fourth case, a man of 37 who survived after small-bowel resection for bowel gangrene secondary to extensive venous thrombosis, was reported by Williamson et al.7 in 1987. These were the only reports we could find of patients with PNH surviving laparotomy for bowel ischaemia. None had survived more than one laparotomy for this condition, as ours did.
A difficulty in dealing with these cases is that abdominal pain is a frequent symptom in PNH and is seldom due to bowel thrombosis. The possibility simply has to be borne in mind. In some cases, conservative management with intravenous fluids, antibiotics and low-molecular-weight heparin is successful.8
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