- Case Report
- Open Access
Small bowel infarction due to fibro muscular dysplasia: a case report and literature review
© Dalmia and Hussain; licensee BioMed Central Ltd. 2010
- Received: 5 November 2009
- Accepted: 6 April 2010
- Published: 6 April 2010
We describe a rare case of small bowel infarction due to fibro muscular dysplasia in superior mesenteric artery in a young patient.
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.
Fibro muscular dysplasia of SMA is rare, is treatable but has a high mortality.
- Superior Mesenteric Artery
- Splenic Flexure
- Celiac Artery
- Celiac Axis
- Watery Diarrhea
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.
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:
Hydrogen ion - 43.4 nmol/litre
Reported cases of intestinal infarction due to fibro muscular dysplasia
Yamaguchi et al(Am J Gastroenterol, 1996, 91(8):1635-8)
Stenosis of jejunal and sigmoid branch
Conservative treatment leading to stenosis
Hamed et al.(J Ped Surg, 1997, 32(9):1379-80)
SMA Occlusion due to intimal Fibroplasia leading to intestinal gangrene
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
Fibro muscular dysplasia of SMA presents dramatically. Early diagnosis can be life saving. However outcome is generally poor in most cases.
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
- Curry TK, Messina LH: Fibro muscular dysplasia: when is intervention warranted?. Sem Vasc Surg. 2003, 16: 190-199. 10.1016/S0895-7967(03)00024-3.View ArticleGoogle Scholar
- Mettinger KL, Ericson K: Fibro muscular dysplasia and the brain: observation on angiographic, clinical, and genetic characteristics. Stroke. 1982, 13: 46-52.View ArticlePubMedGoogle Scholar
- Luscher TF, Lie JT, Stanson AW, Houser OW, Hollier LH, Sheps SG: Arterial fibro muscular dysplasia. Mayo Clin Proc. 1987, 62: 931-952.View ArticlePubMedGoogle Scholar
- Luscher TF, Keller HM, Imhof HG: Fibro muscular hyperplasia: extension of the disease and therapeutic outcome: results of the university hospital Zurich Cooperative study on fibro muscular hyperplasia. Nephron. 1986, 44 (Suppl 1): 109-114.PubMedGoogle Scholar
- Sang CN, Whelton PK, Hamper UM, et al: Etiologic factors in renovascular fibro muscular dysplasia: a case-control study. Hypertension. 1989, 14: 472-479.View ArticlePubMedGoogle Scholar
- Pannier-Moreau I, Grimbert P, Fiquet-kempf B: Possible familial origin of multifocal renal artery fibro muscular dysplasia. J Hypertens. 1997, 15: 797-801. 10.1097/00004872-199715120-00092.View ArticleGoogle Scholar
- Ruston A: genetics of fibro muscular dysplasia. Arch Intern Med. 1980, 140: 233-236. 10.1001/archinte.140.2.233.View ArticleGoogle Scholar
- Harrison EG, McCormack LJ: Pathological classification of renal arterial disease in renovascular hypertension. Mayo Clin Proc. 1971, 46: 161-167.PubMedGoogle Scholar
- Leadbetter WF, Burkland CE: Hypertension in unilateral renal disease. J Urol. 1938, 39: 611-625.Google Scholar
- Palubinskas AJ, Ripley HR: Fibro muscular hyperplasia in extra renal arteries. Radiology. 1964, 82: 451-455.View ArticlePubMedGoogle Scholar
- Gill CK, Benavides DC, Rees C, Fenves AZ, Burtin EC: Fatal Mesenteric Fibro muscular Dysplasia: A Case Report and Review of the Literature. Arch Intern Med. 2004, 164 (10): 1148-1153. 10.1001/archinte.164.10.1148.View ArticleGoogle Scholar
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.