- Case Report
- Open Access
Small bowel infarction due to fibro muscular dysplasia: a case report and literature review
- Sanjay Dalmia1Email author and
- Amir Hussain2
https://doi.org/10.1186/1757-1626-3-79
© Dalmia and Hussain; licensee BioMed Central Ltd. 2010
- Received: 5 November 2009
- Accepted: 6 April 2010
- Published: 6 April 2010
Abstract
Introduction
We describe a rare case of small bowel infarction due to fibro muscular dysplasia in superior mesenteric artery in a young patient.
Case presentation
A 28 year old Asian female presented with acute onset left sided abdominal pain and watery diarrhea. She had a laparotomy due to further deterioration. It showed infracted small intestine, gall bladder and parts of liver. Abdomen had to be closed without any therapeutic procedure. She died in early post operative period. Autopsy showed fibro muscular dysplasia of superior mesenteric artery.
Conclusion
Fibro muscular dysplasia of SMA is rare, is treatable but has a high mortality.
Keywords
- Superior Mesenteric Artery
- Splenic Flexure
- Celiac Artery
- Celiac Axis
- Watery Diarrhea
Introduction
We report a case of 28 year old female who presented with acute abdomen and subsequent laparotomy showed complete infarction of small bowel due to fibro muscular dysplasia of SMA affecting the distribution of superior mesenteric artery with occlusion of celiac axis. Fibro muscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular occlusive disease that most commonly affect the renal and internal carotid arteries. On rare occasions it also affects other arteries.
Case presentation
A 28-year-old Asian female presented to accident and emergency department with 6 weeks history of intermittent left sided abdominal pain, which got worse over last 48 hours. Abdominal pain was associated with 2 episodes of watery diarrhea not mixed with blood and several episodes of vomiting. Drug history included nothing else apart from oral contraceptive pill. On examination she was comfortable and afebrile with pulse rate of 96 and blood pressure of 160/96. Abdomen was soft but tender in left iliac fossa and left lumber region with no guarding or rebound tenderness. Digital rectal examination was unremarkable. Full blood count showed white cell count to be elevated at 24.5 × 106/L. Urea, electrolyte, amylase and liver function tests were all within normal limits. Flexible sigmoidoscopic examination up to distal descending colon did not reveal any abnormality. Computer tomographic scan of the abdomen showed dilated large bowel up to splenic flexure along with dilated loops of small bowel. She was started on supportive treatment with IV Normal Saline, O2 inhalation, catheterization and antibiotics. However after treatment she failed to respond and progressively became more unwell. Gradually she developed hypotension and oliguria. Arterial blood gas analysis at 4 litre of oxygen showed compensated acidosis with following picture:
Po2-10.7 kPa
PCO2-4.04 kPa
Hydrogen ion - 43.4 nmol/litre
HCO3-17.5 nmol/litre
×10-Elastica van Gieson stain - Superior mesenteric artery origin.
Macroscopic picture showing origin of coeliac trunk and superior mesenteric artery.
Discussion
Reported cases of intestinal infarction due to fibro muscular dysplasia
Author | Pathology | Treatment offered | Result |
---|---|---|---|
Yamaguchi et al(Am J Gastroenterol, 1996, 91(8):1635-8) | Stenosis of jejunal and sigmoid branch | Conservative treatment leading to stenosis | Favourable |
Hamed et al.(J Ped Surg, 1997, 32(9):1379-80) | SMA Occlusion due to intimal Fibroplasia leading to intestinal gangrene | Exploratory Laparotomy | Unfavourable-Died |
Mertens et al. (Acta chir belg, 2005, 105: 523-527) | Occlusion of SMA and celiac trunk leading to visceral ischaemia | Reimplantation of Superior Mesenteric Artery | Favourable-Well two years after surgery |
Conclusion
Fibro muscular dysplasia of SMA presents dramatically. Early diagnosis can be life saving. However outcome is generally poor in most cases.
Consent
All reasonable attempts to gain consent have been made after patient has died the patient is anonymous there is no reason to think that the patient or their family would object to publication
Declarations
Authors’ Affiliations
References
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Copyright
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.