Superior Mesenteric Vein Stenosis Complicating Crohn's Disease
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Gut 1999;45:459–462 459 CASE REPORT Gut: first published as 10.1136/gut.45.3.459 on 1 September 1999. Downloaded from Superior mesenteric vein stenosis complicating Crohn’s disease R S Hodgson, J E Jackson, S D Taylor-Robinson, JRFWalters Abstract Case 1 Background—Superior mesenteric vein A 34 year old man with a 21 year history of stenosis as a consequence of mesenteric Crohn’s disease was referred to this hospital for fibrosis, causing the development of small visceral angiography and consideration of bowel varices, is an unrecognised associ- embolisation of a reported arteriovenous mal- ation of Crohn’s disease. formation in the small intestine. Case reports—Two cases of gastro- The patient had had predominantly inflam- intestinal bleeding occurring in patients matory small intestinal disease causing relapses with Crohn’s disease, and a third case, of right iliac fossa pain, nausea, and vomiting presenting with pain and diarrhoea, are which was successfully treated with courses of described. In all three patients, visceral oral prednisolone and sulphasalazine or me- angiography showed superior mesenteric salazine for the first 17 years of his disease. He vein stenosis with dilatation of draining then presented with massive rectal bleeding collateral veins in the small bowel. Overt requiring blood transfusion. Exploratory gastrointestinal bleeding or iron defi- laparotomy revealed extensive inflammatory ciency anaemia resulting from mucosal Crohn’s disease involving the entire small ulceration is common in Crohn’s disease, intestine. The small intestine was oedematous, but acute or chronic bleeding from small ulcerated, and thick walled, and was noted to bowel varices as a result of superior be encroaching on the mesentery. A “haeman- mesenteric vein stenosis due to fibrosis gioma” at the duodenojejunal junction was http://gut.bmj.com/ has not previously been reported. resected, and an inflamed caecum adherent to (Gut 1999;45:459–462) the posterior abdominal wall was found. The Keywords: Crohn’s disease; superior mesenteric vein colon was noted to be full of blood. stenosis; mesenteric fibrosis; small bowel varices A limited right hemicolectomy to the hepatic flexure was performed and 20 cm of aVected terminal ileum was resected to remove the sus- Bleeding from the gastrointestinal tract is a pected source of blood loss. The mesentery was common presenting symptom or complication on September 26, 2021 by guest. Protected copyright. 1–3 noted to be oedematous and it bled easily. of Crohn’s disease. Acute or chronic blood Macroscopic examination of the resected loss results from mucosal ulceration. Vasculitis specimens revealed oedematous and fibrosed involving small vessels is a frequent finding in small and large bowel with loss of the normal aVected segments of gut,4 but involvement of Gastroenterology Unit, mucosal pattern and numerous ulcers which larger blood vessels such as the superior Department of caused a cobblestone appearance. Microscopi- mesenteric vein is reported rarely.5 The aeti- Medicine, Imperial cally there was a moderate chronic inflamma- College School of ology in the described cases is venous thrombo- tory infiltrate and numerous fissures and ulcers Medicine, London, UK sis caused by a procoagulant tendency. Periph- R S Hodgson eral venous thrombosis and pulmonary with granuloma formation, which were consist- S D Taylor-Robinson ent with the diagnosis of Crohn’s disease. Dur- JRFWalters embolism occur most commonly, but superior mesenteric vein thrombosis or portal vein ing the subsequent three years the disease was diYcult to control, requiring treatment with Department of thrombosis causing recurrent gastrointestinal Diagnostic Radiology, bleeding from varices has also been described. oral prednisolone 10–30 mg/day and azathio- Imperial College Occlusion of the superior mesenteric vein prine 150 mg/day. Over the next six months the School of Medicine, caused by a fibrotic reaction has not been patient presented with severe rectal haemor- London, UK rhage on six further occasions necessitating J E Jackson described. We describe three patients with Crohn’s dis- blood transfusion of 20 units in total. Barium Correspondence to: ease who were found to have superior me- meal and follow through examination showed Dr J E Jackson, Department senteric vein stenosis as a consequence of inflammation involving the jejunum in a of Diagnostic Radiology, pattern typical of Crohn’s disease. Visceral Imperial College School of mesenteric fibrosis and small bowel varices at Medicine, Hammersmith visceral angiography. One patient presented angiography performed at the referring hospi- Hospital, Du Cane Road, with abdominal pain, another with iron defi- tal was thought to show a vascular malforma- London W12 0NN, UK. ciency anaemia, but in the third case, frank tion in the proximal jejunum and a second, Accepted for publication variceal bleeding from the jejunum was the smaller lesion in the terminal ileum. He was 5 March 1999 predominant symptom. then referred to this hospital for repeat visceral 460 Hodgson, Jackson, Taylor-Robinson, et al Gut: first published as 10.1136/gut.45.3.459 on 1 September 1999. Downloaded from Figure 1 Case 1: venous phase from cannulation of the superior mesenteric artery shows a tight stenosis of the main superior mesenteric vein (long curved arrow) with Figure 3 Case 2: venous phase from the main superior numerous surrounding small bowel varices most prominent mesenteric arteriogram shows a tight stenosis of the superior within the jejunum (straight arrow). The stenosis is regular mesenteric vein (long arrow) and resultant small bowel and concentric with tapered shoulders strongly suggesting a varices (short arrows). fibrotic aetiology as opposed to recanalisation following venous occlusion by thrombus. Varices are also seen crossing meal and follow through examination, and the ileocolic anastomosis (arrowhead). technetium-99m pertechnate scintigraphy for Meckel’s diverticulum performed at the refer- SMV ring hospital were reported as normal. Visceral angiography performed at this Tight stenosis hospital showed a stenosis of the superior Jejunal mesenteric vein with resultant dilatation of col- varices lateral veins in the jejunum and ileum (fig 3). The ileal branches of the superior mesenteric vein were noted to be stretched around several loops of dilated ileum. A small bowel enema was therefore performed. This allowed the mucosa to be distended to maximum advan- tage as a result of the enteroclysis procedure in SMV http://gut.bmj.com/ order to highlight small bowel pathology. This revealed multiple tight strictures in the ileum accompanied by proximal dilatation of the ileal loops, with no evidence of mucosal ulceration Figure 2 Diagram illustrating the stenosis present in the or fistula formation. Laparotomy confirmed angiographic picture shown in fig 1. the presence of multiple ileal strictures with a normal colon. Two segments of small intestine angiography and consideration of embolisation were resected and stricturoplasty was per- on September 26, 2021 by guest. Protected copyright. of the vascular lesions. formed on a further narrowed segment. Histol- The repeat visceral angiogram revealed an ogy of the resected specimens revealed an extremely tight stenosis of the superior me- active, patchy, chronic inflammatory bowel senteric vein and notable dilatation of the col- disease featuring mucosal ulceration and fis- lateral jejunal veins (figs 1 and 2). He was suring, full thickness inflammation of the bowel commenced on propranolol to reduce the wall including submucosal, intramural, and splanchnic vascular pressure, and has not had a serosal lymphoid aggregates, and the presence further episode of rectal bleeding for 10 of granulomas which were consistent with a months. diagnosis of Crohn’s disease. The patient was subsequently treated with oral prednisolone Case 2 and mesalazine. She recovered well from the A 23 year old woman with a two year history of operation and is being followed up at the refer- occult gastrointestinal blood loss of uncertain ring hospital. aetiology was referred to this hospital for visceral angiography. Case 3 The patient had experienced cramping A 63 year old man presented to this hospital upper abdominal pain and lethargy lasting sev- with abdominal pain and diarrhoea. His past eral weeks on two occasions, but was otherwise medical history included two myocardial inf- asymptomatic. She had an iron deficiency arctions, bilateral carotid endarterectomies, anaemia and was dependent on oral iron and a sigmoid colectomy performed for severe supplementation. Her menstrual cycle was diverticular disease. There were no features regular with normal blood loss. Physical exam- indicating a diagnosis of Crohn’s disease in the ination was unremarkable, but multiple faecal history or examination. Blood tests performed occult blood tests were positive. Upper gastro- included a haemoglobin of 125 g/l, with normal intestinal endoscopy, colonoscopy, barium mean corpuscular volume, a normal white cell Superior mesenteric vein stenosis complicating Crohn’s disease 461 count and platelet count, an erythrocyte Venous and arterial thrombosis is a rare but sedimentation rate of 36 mm in the first hour, recognised complication of inflammatory C reactive protein 22 mg/l (normal range 0–10 bowel disease described by Talbot et al,5 which Gut: first published as 10.1136/gut.45.3.459 on 1 September 1999. Downloaded from mg/l), and albumin 34 g/l. A plain abdominal may be associated with coagulation defects,