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Copyright ©The Author(s) 2018.
World J Gastroenterol. Aug 28, 2018; 24(32): 3637-3649
Published online Aug 28, 2018. doi: 10.3748/wjg.v24.i32.3637
Table 1 Summary of mesenteric fibromuscular dysplasia reported to date
Case (n)Ref.Age (yr)Gender(Male/Female)Risk factorsSymptomsVessels InvolvedCTAngiographyPathologyTreatmentAnti-hypertensive drugAnti-coagulantsOutcome
1[5]62MN/AUpper abdominal pain, hemoperitoneum, shockCeliac, SMA, IMA, RAN/AN/AIntimal thickening in the branches of the SMA and IMA.LaparotomyNoneNoneDied
2[6]45FN/AAbdominal painSMA, RAN/AN/AN/AIleal resectionN/AN/AImproved
3[6]50FN/AHypertension, abdominal pain, diarrheaSMA, RA, iliacN/AStenosis and string-of-beads like Appearance in the SMAN/ASMA revascularizationN/AN/AImproved
4[7]73FN/AN/ACeliac, SMA, iliacN/AN/AN/AN/AN/AN/AImproved
5[7]42FN/AN/ASMA, RAN/AN/AN/AN/AN/AN/AImproved
6[7]50FN/AHypertensionCeliac, SMA, RAlN/AMinimal defects in the SMA and RA; stenosis of celiac arteryN/AN/AN/AN/AImproved
7[7]37FN/AVisceral ischemic symptomsCeliac, SMA, RAN/AN/AN/ARevascularizationN/AN/AImproved
8[7]47FN/AHypertension, abdominal painCeliac, SMA, RAN/ADefects in the SMA and RAMedial hyperplasiaNoneN/AN/ADied
9[8]41FN/AHypertensionSMA, internal carotid, RA, iliacN/ACorkscrew and string-of-beads like appearance in the RA, carotid, iliac arteryReplacement of the normal media with disorganized fibrous and muscular hyperplasiaThromboendarerectomy on SMAN/AN/ADied
10[9]64FN/AUnconsciousnessSMA, circle of WillisN/AN/AMedial hyperplasiaNoneNoneNoneDied
11[10]21MN/AHypertensionCeliac, SMA, RA, carotidN/AStenosis of celiac, SMA, RAIntimal fibroplasiaAnti-hypertensive drug; revascularization of carotid artery; angioplasty of RAYes, N/AN/AImproved
12[10]20FN/AHypertensionSMA, IMA, RA, carotidN/AStenosis of carotid, renal, SMA. Total occlusion of the IMA.Intimal fibroplasiaAnti-hypertensive drug, subclavian-carotid bypass; vascular reconstruction of the kidneyYes, N/AN/AImproved
13[11]55MN/AN/ASMAN/AN/AIntimal hyperplasia in SMANoneN/AN/AN/A
14[12]44FN/AAsymptomatic bruit of the aortoiliac systemCeliac, SMA, RA, iliacN/AString-of-beads like appearance of iliac artery; aneurysms of SMA, RAIntimal fibrosis with development of fibrosisResection and reconstructionN/AN/AImproved
15[13]58FN/ABody weight lossCeliac, SMA, IMA, RA, iliac, aortaN/AOcclusion of celiac, SMA, IMA.N/AOpen surgeryN/AN/AImproved
16[14]46FNone (non-smoker)Palpitations, headache, hypertensionCeliac, SMAN/AAneurysms in the right RA, celiac; occlusion in the left gastric arteryMuscle hypertrophy and disorganisation of elastic tissue of the media in celiac artery.Surgical ligationN/AN/AImproved
17[15]60MNone (non-smoker)Left abdominal pain, diarrheaSMAIrregular nodular thickening in transverse colon.Stenoses of the SMAIntimal fibrosis and focal replacement of medial smooth-muscle fibers by fibrous tissue .Splenic flexure resection and angioplastyN/AN/AImproved
18[16]54FSmokingHypertension, headache, abdominal painSMA, RA, coronaryLiver cystStenosis of the coronary arteriesIntimal hyperplasia in SMA, RA, coronary, splenic, intrahepatic arteryAnti-hypertensive drugα,β-blocker → Ca blockerN/ADied
19[17]39MN/AMelena, lower abdominal painJejunal, SigmoidN/AString-of-beads like appearance in the jejunal and sigmoid arteries.Adventitia is thickened by fibroplasiaResection of the jejunumN/AN/AImproved
20[18]23MN/AHypertensionCeliac, SMA, RA, carotid, vertebral, opthalmic, superficial temporal, iliac, lumber, intercostalHematomasin the paraduodenal and right superior gluteral lesion and splenic infarctionMultiple saccular aneurysms in the celiac, SMA, RA< splenic, hepatic, iliac, lumber, and intercostal arteriesMediolytic FMD with segmental dissection and thrombosisEmbolization of the gastroduodenal and right SMA to prevent hemorrhageN/AN/AImproved
21[19]33MN/AAbdominal painSMAN/AString-of-beads like appearance in the SMAThickening of the media due to hyperplasia in SMAIleal resectionN/AN/AImproved
22[20]78FN/AHypertension, abdominal pain, hemoperitoneum.SMA, RA, colonaryDilated loop of the small bowel and fluid in the peritoneal cavity.N/AMedial and perimedial fibrodysplasia, forms the characteristic petal-like appearance in SMA.NoneNoneNoneDied
23[21]43MNone (non-smoker)No symptomsSMA, iliacSMA aneurysmAneurysms in the SMA, hepatic artery, splenic artery, jejunal artery, iliac arteries.Medial fibrodysplasia in the arteral wallsAneurysm resection and arterial reconstructionN/AN/AImproved
24[22]48FNone (non-smoker)Abdominal pain, hemoperitoneumCeliac, SMA, RAN/AMultiple small aneurysms in celiac, SMA, RAN/ASurgical hemostasis and anti-hypertensive drugsβ-blockerN/AImproved
25[23]57FSmoking (40 packs/yr)Abdominal pain, weight loss, anorexia, nausea, vomiting, diarrhea.Celiac, SMANothing particularStenosis of the celiac artery and SMAMedial thickening, smooth muscle hyperplasia in SMA and celiac arteryAortoiliac and aorto-SMA bypassN/AN/ADied
26[24]48FSmoking (20 packs/yr)Abdominal painCeliac, SMA, IMAN/AOcclusion of the celiac, SMA; enlarged hypertrophic IMAIntimal fibroplasia and an increased deposition of fibrous tissue in the vessel wall mediaReimplantation of the SMAN/AN/AImproved
27[25]38MSmokingGastrointestinal bleeding, anemiaSMA, IMAN/AEctasia in IMA; string-of-beads like appearance in the SMAThickening and hyalinization of medium sized vessel walls, with intimal proliferation.Ileal resectionN/AN/AImproved
28[26]Not providedNot providedNone (non-smoker)Abdominal pain, distension, constipationSMAN/AN/AThick cuff (petal like) of smooth muscle proliferation with normal intima and media in mesenteric artery.Right hemicolectomyN/AN/AImproved
29[27]43FSmoking (10 cigarettes daily for 20 yr)Hypertension, headacheSMA, RAN/AString-of-beads like appearance in the right RA and SMA; stenosis and multiple irregularities in the left RAN/AAngioplasty and anti-hypertensive drugsYes, N/AN/AImproved
30[28]38MN/AN/AN/AN/AN/AN/AN/AN/AN/AImproved
31[29]43FN/AHypertension, abdominal pain, headacheSMA, RAAneurysms in the left RAAneurysms in the left RA with sever fibrodysplastic stenosis; string-of-beads like appearance in the right RA; stenosis in SMAIntimal fibroplasia, lost of internal elastic lamina, and massive destruction of the media in the aneurysm wallsAneurysm resection and aortorenal bypass and percutaneous transluminal angioplastyN/AN/AImproved
32[30]44FOral contraceptive pillsHypertension, abdominal pain, diarrhea, vomitingSMAStenosis of SMA and nonspecific colitisstenosis in SMAN/AAngioplastyN/AN/AImproved
33[31]30MN/AAbdominal pain, hypertensionCeliac, SMA, RA, iliacDissections of the celiac, SMA, left RA, and external iliac arterystenosis in the right RAN/AAnti-platelet and anti-hypertensive therapy and angioplasty for right renal artery.β-blocker, Ca blockerwarfarin, aspirin (100mg)Improved
34[32]47FN/AAbdominal pain, diarrhea, hypertensionAll abdominal arteriesA partial occlusion of the celiac artery and a total occlusion of the SMAN/AIntimal and medial proliferationAnti-hypertensive drugYes, N/AN/ADied
35[33]47FNone (non-smoker)Nausea, early satiety, abdominal painCeliac, SMAN/AStenosis of the SMA, hypertrophy of the gastroduodenal artery and pancreaticoduodenal arteriesN/AAn aorto-superior mesenteric artery and an aorto-hepatic artery bypass.N/AN/AImproved
36[34]19FN/AAbdominal pain, vomitingSMA, RAN/AStenosis of the origin of the SMA and multiple aneurysms involving the proximal SMA. Right renal artery is mild irregularity.N/AResection of the aneurysmal segment in the SMA; aorto-SMA interposition graft with polytetrafluoroethyleneN/AN/AImproved
37[35]52MSmokingAbdominal painIMAStenosis of the IMAN/ANecrosis of the mucosa; fibrosis of the inimaLeft hemicolectomyN/AN/AImproved
38[36]20FN/AAbdominal pain, hemorrhagic shockSMA, right gastroepiploic, jejunalIntraperitoneal bleeding in the omental bursa and mesentery of the transverse colonString-of-beads like appearance in the jejunal arteryN/ATranscatheter arterial embolizationN/AN/AImproved
39[37]61FN/AAbdominal painSMA, IMA, RAN/AMultiple aneurysms and stenoses in SMA, IMA, RAMultiple tears and dissections of the medial layer and fibrointimal thickeningAnti-coagulationNoneYes, N/AImproved