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Gut Online First, published on October 23, 2014 as 10.1136/gutjnl-2014-308319 Editor’s quiz: GI snapshot
A rare cause of abdominal pain, diarrhoea and GI bleeding PART 1 A 62-year-old woman presented with abdominal pain and diarrhoea. She had a history of primary biliary cirrhosis and renal transplantation secondary to reflux nephropathy. Cross-sectional imaging and a colonoscopy demonstrated terminal ileal inflammation and ulceration, but histological biopsies were inconclusive and cytomegalovirus staining was negative. The patient subsequently developed spontaneous small bowel perforation due to deep ulceration, necessitating an emergency right hemicolectomy. Her postoperative period was complicated by several episodes of rectal bleeding. After a prolonged admission at her local hospital, the patient was referred to our unit for further management. On arrival, the patient’s immunosuppression consisted of tacrolimus and low-dose prednisolone. Mycophenolate mofetil (MMF) had been discontinued prior to transfer due to concerns that MMF toxicity was the cause of her ileal disease. Laboratory evaluation revealed a haemoglobin of 83 g/L with a raised C-reactive protein (21 mg/L). CT angiography showed no active bleeding despite clinical evidence of ongoing rectal blood loss. A colonoscopy showed a hemi-circumferential anastomotic ulcer but no active bleeding (figure 1). Push enteroscopy was unremarkable. Histological slides of the right hemicolectomy resection specimen were reviewed at our centre (figures 2–4). What are the unusual histological features demonstrated in figures 2–4?
Figure 2 Histological slide from right hemicolectomy specimen (low-power view, H&E staining).
PART 2 Figure 2 shows a punched-out ulcer involving the full thickness of the small bowel wall. In figures 3 and 4, the mesenteric veins demonstrate marked luminal narrowing with myointimal hyperplasia. These features are pathognomonic of idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV). IMHMV is an extremely rare disease of unknown aetiology that classically occurs in the rectosigmoid colon of relatively
Figure 1 Endoscopic view (at colonoscopy) of a hemi-circumferential anastomotic ulcer with a clean base.
Figure 3 Histological slide from right hemicolectomy specimen (high-power view, H&E staining).
Figure 4 Histological slide from right hemicolectomy specimen (high-power view, elastica van Gieson staining).
Laskaratos F-M, et al. Gut Month 2014 Vol 0 No 0
1
Copyright Article author (or their employer) 2014. Produced by BMJ Publishing Group Ltd (& BSG) under licence.
Downloaded from http://gut.bmj.com/ on November 2, 2014 - Published by group.bmj.com
Editor’s quiz: GI snapshot young, otherwise healthy males.1 The clinical presentation of IMHMV mimics that of IBD, and colonic resection has been invariably required in these patients typically after a protracted course of medical therapy.2 Biopsies are almost always nondiagnostic with non-specific inflammatory features, although the presence of ischaemic type changes may help to differentiate IMHMV from IBD. The key point is that the biopsies do not support the presumptive diagnosis of IBD.3 This condition has only been diagnosed after histopathological review of the resected colon and bowel resection appears to be curative.4 In our case, the disease affected the terminal ileum resulting in deep ulceration and spontaneous perforation. This is an extremely unusual presentation, and only one similar case has been reported in the literature previously.5 After a prolonged admission, the patient’s rectal bleeding settled with conservative measures and she made a full recovery, leading to her discharge. Faidon-Marios Laskaratos,1 Mark Hamilton,1 Marco Novelli,2 Neil Shepherd,3 Gareth Jones,4 Christopher Lawrence,5 Miriam Mitchison,6 Charles D Murray1
London NHS Foundation Trust, Pond Street, Hampstead, London NW3 2QG, UK; [email protected] Contributors F-ML prepared the manuscript. MN and NS prepared the histopathology images. MH, MN, NS, GJ, CL and MM reviewed the manuscript and contributed with their comments to the final version of the manuscript. CDM conceived and finalised the manuscript. All authors approved the submitted manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; internally peer reviewed. To cite Laskaratos F-M, Hamilton M, Novelli M, et al. Gut Published Online First: [ please include Day Month Year] doi:10.1136/gutjnl-2014-308319 Received 26 August 2014 Revised 29 September 2014 Accepted 30 September 2014 Gut 2014;0:1–2. doi:10.1136/gutjnl-2014-308319
REFERENCES 1
1
Centre for Gastroenterology, Royal Free Hospital NHS Foundation Trust, London, UK 2 Department of Histopathology, University College Hospitals NHS Foundation Trust, London, UK 3 Gloucestershire Cellular Pathology Laboratory, Cheltenham General Hospital, Sandford Road, Cheltenham, Glos, UK 4 Centre for Nephrology, Royal Free Hospital NHS Foundation Trust, London, UK 5 Department of Nephrology, East and North Hertfordshire NHS Trust, Hertfordshire, UK 6 Department of Histopathology, East and North Hertfordshire NHS Trust, Hertfordshire, UK Correspondence to Dr Charles D Murray, Centre for Gastroenterology, Royal Free
2
2
3 4
5
Sherman J, Kao P, West B, et al. Focal myointimal hyperplasia of mesenteric veins is associated with previous trauma in surgical specimens. Pathol Res Pract 2006;202:517–22. Korenblit J, Burkart A, Frankel R, et al. Refractory pancolitis: a novel presentation of idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol Hepatol 2012;8:696–700. Platz J, Hyman N. Idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol Hepatol 2012;8:700–3. Kao P, Vecchio J, Hyman N, et al. Idiopathic myointimal hyperplasia of mesenteric veins. A rare mimic of idiopathic inflammatory bowel disease. J Clin Gastroenterology 2005;39:704–8. Lanitis S, Kontovounisios C, Karaliotas C. An extremely rare small bowel lesion associated with refractory ascites. Gastroenterology 2012;142:e5–7.
Laskaratos F-M, et al. Gut Month 2014 Vol 0 No 0
Downloaded from http://gut.bmj.com/ on November 2, 2014 - Published by group.bmj.com
A rare cause of abdominal pain, diarrhoea and GI bleeding Faidon-Marios Laskaratos, Mark Hamilton, Marco Novelli, Neil Shepherd, Gareth Jones, Christopher Lawrence, Miriam Mitchison and Charles D Murray Gut published online October 23, 2014
Updated information and services can be found at: http://gut.bmj.com/content/early/2014/10/23/gutjnl-2014-308319
These include:
References
This article cites 5 articles, 0 of which you can access for free at: http://gut.bmj.com/content/early/2014/10/23/gutjnl-2014-308319#BIBL
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Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
Gut Online First, published on October 23, 2014 as 10.1136/gutjnl-2014-308319 Editor’s quiz: GI snapshot
A rare cause of abdominal pain, diarrhoea and GI bleeding PART 1 A 62-year-old woman presented with abdominal pain and diarrhoea. She had a history of primary biliary cirrhosis and renal transplantation secondary to reflux nephropathy. Cross-sectional imaging and a colonoscopy demonstrated terminal ileal inflammation and ulceration, but histological biopsies were inconclusive and cytomegalovirus staining was negative. The patient subsequently developed spontaneous small bowel perforation due to deep ulceration, necessitating an emergency right hemicolectomy. Her postoperative period was complicated by several episodes of rectal bleeding. After a prolonged admission at her local hospital, the patient was referred to our unit for further management. On arrival, the patient’s immunosuppression consisted of tacrolimus and low-dose prednisolone. Mycophenolate mofetil (MMF) had been discontinued prior to transfer due to concerns that MMF toxicity was the cause of her ileal disease. Laboratory evaluation revealed a haemoglobin of 83 g/L with a raised C-reactive protein (21 mg/L). CT angiography showed no active bleeding despite clinical evidence of ongoing rectal blood loss. A colonoscopy showed a hemi-circumferential anastomotic ulcer but no active bleeding (figure 1). Push enteroscopy was unremarkable. Histological slides of the right hemicolectomy resection specimen were reviewed at our centre (figures 2–4). What are the unusual histological features demonstrated in figures 2–4?
Figure 2 Histological slide from right hemicolectomy specimen (low-power view, H&E staining).
PART 2 Figure 2 shows a punched-out ulcer involving the full thickness of the small bowel wall. In figures 3 and 4, the mesenteric veins demonstrate marked luminal narrowing with myointimal hyperplasia. These features are pathognomonic of idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV). IMHMV is an extremely rare disease of unknown aetiology that classically occurs in the rectosigmoid colon of relatively
Figure 1 Endoscopic view (at colonoscopy) of a hemi-circumferential anastomotic ulcer with a clean base.
Figure 3 Histological slide from right hemicolectomy specimen (high-power view, H&E staining).
Figure 4 Histological slide from right hemicolectomy specimen (high-power view, elastica van Gieson staining).
Laskaratos F-M, et al. Gut Month 2014 Vol 0 No 0
1
Copyright Article author (or their employer) 2014. Produced by BMJ Publishing Group Ltd (& BSG) under licence.
Downloaded from http://gut.bmj.com/ on November 2, 2014 - Published by group.bmj.com
Editor’s quiz: GI snapshot young, otherwise healthy males.1 The clinical presentation of IMHMV mimics that of IBD, and colonic resection has been invariably required in these patients typically after a protracted course of medical therapy.2 Biopsies are almost always nondiagnostic with non-specific inflammatory features, although the presence of ischaemic type changes may help to differentiate IMHMV from IBD. The key point is that the biopsies do not support the presumptive diagnosis of IBD.3 This condition has only been diagnosed after histopathological review of the resected colon and bowel resection appears to be curative.4 In our case, the disease affected the terminal ileum resulting in deep ulceration and spontaneous perforation. This is an extremely unusual presentation, and only one similar case has been reported in the literature previously.5 After a prolonged admission, the patient’s rectal bleeding settled with conservative measures and she made a full recovery, leading to her discharge. Faidon-Marios Laskaratos,1 Mark Hamilton,1 Marco Novelli,2 Neil Shepherd,3 Gareth Jones,4 Christopher Lawrence,5 Miriam Mitchison,6 Charles D Murray1
London NHS Foundation Trust, Pond Street, Hampstead, London NW3 2QG, UK; [email protected] Contributors F-ML prepared the manuscript. MN and NS prepared the histopathology images. MH, MN, NS, GJ, CL and MM reviewed the manuscript and contributed with their comments to the final version of the manuscript. CDM conceived and finalised the manuscript. All authors approved the submitted manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; internally peer reviewed. To cite Laskaratos F-M, Hamilton M, Novelli M, et al. Gut Published Online First: [ please include Day Month Year] doi:10.1136/gutjnl-2014-308319 Received 26 August 2014 Revised 29 September 2014 Accepted 30 September 2014 Gut 2014;0:1–2. doi:10.1136/gutjnl-2014-308319
REFERENCES 1
1
Centre for Gastroenterology, Royal Free Hospital NHS Foundation Trust, London, UK 2 Department of Histopathology, University College Hospitals NHS Foundation Trust, London, UK 3 Gloucestershire Cellular Pathology Laboratory, Cheltenham General Hospital, Sandford Road, Cheltenham, Glos, UK 4 Centre for Nephrology, Royal Free Hospital NHS Foundation Trust, London, UK 5 Department of Nephrology, East and North Hertfordshire NHS Trust, Hertfordshire, UK 6 Department of Histopathology, East and North Hertfordshire NHS Trust, Hertfordshire, UK Correspondence to Dr Charles D Murray, Centre for Gastroenterology, Royal Free
2
2
3 4
5
Sherman J, Kao P, West B, et al. Focal myointimal hyperplasia of mesenteric veins is associated with previous trauma in surgical specimens. Pathol Res Pract 2006;202:517–22. Korenblit J, Burkart A, Frankel R, et al. Refractory pancolitis: a novel presentation of idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol Hepatol 2012;8:696–700. Platz J, Hyman N. Idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol Hepatol 2012;8:700–3. Kao P, Vecchio J, Hyman N, et al. Idiopathic myointimal hyperplasia of mesenteric veins. A rare mimic of idiopathic inflammatory bowel disease. J Clin Gastroenterology 2005;39:704–8. Lanitis S, Kontovounisios C, Karaliotas C. An extremely rare small bowel lesion associated with refractory ascites. Gastroenterology 2012;142:e5–7.
Laskaratos F-M, et al. Gut Month 2014 Vol 0 No 0
Downloaded from http://gut.bmj.com/ on November 2, 2014 - Published by group.bmj.com
A rare cause of abdominal pain, diarrhoea and GI bleeding Faidon-Marios Laskaratos, Mark Hamilton, Marco Novelli, Neil Shepherd, Gareth Jones, Christopher Lawrence, Miriam Mitchison and Charles D Murray Gut published online October 23, 2014
Updated information and services can be found at: http://gut.bmj.com/content/early/2014/10/23/gutjnl-2014-308319
These include:
References
This article cites 5 articles, 0 of which you can access for free at: http://gut.bmj.com/content/early/2014/10/23/gutjnl-2014-308319#BIBL
Email alerting service
Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
Topic Collections
Articles on similar topics can be found in the following collections GUT Snapshot (294)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
