Literature DB >> 33752607

Fibromuscular dysplasia of mesenteric arteries: a rare cause of multiple bowel resections-a case report and literature review.

Shuwen Du1, Shanbing Yang1, Kangmei Jia1, Peng Du2, Limin Zhang1, Jiheng Wang3.   

Abstract

BACKGROUND: Fibromuscular dysplasia (FMD) is a type of unexplained nonatherosclerotic vascular disease that usually involves the renal and internal carotid arteries and rarely involves the mesenteric artery. Mesenteric artery FMD is difficult to distinguish from Crohn's disease (CD) and Behcet's disease (BD) solely based on symptoms. Patients with mesenteric artery FMD can present with an acute abdomen, but case reports of patients who have a long medical history and undergo multiple bowel resections are extremely rare. CASE
PRESENTATION: The patient was a 45-year-old woman with an 11-year history of intermittent lower abdominal pain and fever. At the age of 34 years, she underwent right hemicolectomy and appendectomy due to an acute abdomen. She suffered from oral ulcers between 34 and 36 years old. A clinical diagnosis of presumed CD was made by the age of 41, and she was treated with mesalazine; however, the effect was poor. At the age of 42, she came to our centre, and based on her atypical symptoms and examination results, we thought she had CD. Hence, she was treated with glucocorticoids for 3 years. However, when she was 45, due to steroid dependence, thalidomide tablets were added. Unfortunately, she suffered from another episode of intestinal obstruction. Therefore, she underwent enterectomy. The postoperative histopathological diagnosis was mesenteric artery FMD. She no longer underwent pharmacotherapy after the surgery. Although she did not have any of her previous symptoms and postoperative colonoscopy showed no signs of recurrence, splenomegaly and abnormal routine blood results were still present.
CONCLUSIONS: Patients with mesenteric artery FMD can present with an acute abdomen. In addition, the symptoms and endoscopic manifestations of mesenteric artery FMD may appear similar to CD and BD. Hence, it is difficult to make a clear clinical diagnosis and proceed with treatment. Mesenteric artery FMD often requires surgical pathology to confirm its diagnosis. For patients who suffer from this disorder, surgery may be the best choice to improve the patient's quality of life.

Entities:  

Keywords:  Behcet’s disease; Bowel resections; Case report; Crohn’s disease; Fibromuscular dysplasia

Mesh:

Year:  2021        PMID: 33752607      PMCID: PMC7986496          DOI: 10.1186/s12876-021-01702-y

Source DB:  PubMed          Journal:  BMC Gastroenterol        ISSN: 1471-230X            Impact factor:   3.067


  8 in total

1.  Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association.

Authors:  Jeffrey W Olin; Heather L Gornik; J Michael Bacharach; Jose Biller; Lawrence J Fine; Bruce H Gray; William A Gray; Rishi Gupta; Naomi M Hamburg; Barry T Katzen; Robert A Lookstein; Alan B Lumsden; Jane W Newburger; Tatjana Rundek; C John Sperati; James C Stanley
Journal:  Circulation       Date:  2014-02-18       Impact factor: 29.690

2.  Abdominal angina and intestinal gangrene--a catastrophic presentation of arterial fibromuscular dysplasia: case report and review of the literature.

Authors:  R M Hamed; K Ghandour
Journal:  J Pediatr Surg       Date:  1997-09       Impact factor: 2.545

3.  Unusual mesenteric fibromuscular dysplasia a rare cause for chronic intestinal ischaemia.

Authors:  R Chaturvedi; P Vaideeswar; A Joshi; S Pandit
Journal:  J Clin Pathol       Date:  2008-02       Impact factor: 3.411

Review 4.  Nonatherosclerotic Vascular Disease in Women.

Authors:  Hiroyuki Jinnouchi; Aloke V Finn; Renu Virmani
Journal:  Tex Heart Inst J       Date:  2018-08-01

5.  Mesenteric Arteriovenous Dysplasia/Vasculopathy Is Distinct From Fibromuscular Dysplasia.

Authors:  Deepa T Patil; Juliana Kissiedu; E Rene Rodriguez; Erinn Downs-Kelly; Xiuli Liu; Lisa A Rybicki; Carmela D Tan
Journal:  Am J Surg Pathol       Date:  2016-10       Impact factor: 6.394

6.  Pathologic classification of renal arterial disease in renovascular hypertension.

Authors:  E G Harrison; L J McCormack
Journal:  Mayo Clin Proc       Date:  1971-03       Impact factor: 7.616

7.  The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients.

Authors:  Jeffrey W Olin; James Froehlich; Xiaokui Gu; J Michael Bacharach; Kim Eagle; Bruce H Gray; Michael R Jaff; Esther S H Kim; Pam Mace; Alan H Matsumoto; Robert D McBane; Eva Kline-Rogers; Christopher J White; Heather L Gornik
Journal:  Circulation       Date:  2012-05-21       Impact factor: 29.690

Review 8.  Arterial fibromuscular dysplasia.

Authors:  T F Lüscher; J T Lie; A W Stanson; O W Houser; L H Hollier; S G Sheps
Journal:  Mayo Clin Proc       Date:  1987-10       Impact factor: 7.616

  8 in total

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