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
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
Table 2 Summary of mesenteric segmental arterial mediolysis reported to date
Case (n)Ref.Age (yr)Gender (Male/Female)Risk factorsSymptomsVessels InvolvedCTAngiographyPathologyTreatmentAnti-hypertensive drugAnti-coagulantsOutcome
1[48]65FN/AAbdominal painSMAN/ABeaded appearance and stenosis of the MCALysis and destruction in the media and intimaResection of aneurysm in MCAN/AN/AImproved
2[49]56FN/AAbdominal painIMAIntraabdominal hemorrhageAneurysm in IMAN/ALeft hemicolectomyN/AN/AImproved
3[50]78MN/AAbdominal pain, diarrhea, shockSMAN/AN/ADestruction of the tunica interna and media in MCAEmergent surgery (right hemicolectomy); a large hematoma and a ruptured aneurysm upon the surgeryN/AN/AImproved
4[51]35FN/AAbdominal pain, perforation on transverse colonSMAOcclusion of the mesenteric vein and ischemic colitisUnremarkableSegmental vacuolar degeneration of smooth muscle with areas of wall thinningResection of terminal ileumN/AN/ADied
5[52]52MN/ASudden hemiparesis, hypertensionCeliac, SMA, IMA hepatic arteryAneurysm in the celiac, hepatic, SMAAneurysms in celiac, SMA, ICA, hepatic; stenoses in celiac and SMAMultiple segmental mediolysis lesions of the muscular and elastic fibers of the mediaReconstruction of hepatic and celiac artery using autologous saphenous vein graftN/AN/AImproved
6[53]49MN/AAbdominal pain, shockSMALarge hematoma surrounding a high-density aneurysmBeaded appearance in SMAMultifocal fragmentation of the elastic fibers of the mediaRight hemicolectomyN/AN/AImproved
7[54]57MN/AAbdominal painSMA, hepaticSmall aneurysm at the middle colic artery and mesenteric hematomaAneurysm and stenosis of the celiac, SMA, hepatic arteryN/AEmbolization with N-butyl cyanoacrylate for aneurysm in the SMAN/AN/AImproved
8[54]76FN/AAbdominal pain, nauseaIMAMesenteric hematomaAneurysm in IMAN/AEmbolization with coilN/AN/ADied
9[55]59MN/AAbdominal pain, shockSMA, RA, gastroepiploic, splenicSMA dissection, aneurysm in RA, gastroepiploic, splenic artery; rupture of the splenic aneurysmSaccular aneurysms and multiple stenotic region in gastroepiploic arteryMedial island spared from mediolysisEmergency embolization of the splenic artery, resection of aneurysm in the gastroepiploicN/AN/AImproved
10[56]57MN/AAbdominal pain, diarrheaSMAAscites throughout the abdomenAneurysm in SMAN/ATranscatheter arterial embolizationN/AN/AImproved
11[57]60MN/AN/ASMARupture of the aneurysm of the MCAMultiple beaded patters and aneurysm in SMAN/ASurgical resectionN/AN/AImproved
12[47]25FN/AAnorexia, abdominal pain, diarrheaSMA, hepaticIschemic colitis of the splenic flexureOcclusion of IMA; stenoses of the hepatic arteryPatchy, isolated destruction of the arterial media involving both the internal and external elastic laminaePartial colectomy of the splenic flexureN/AN/AImproved
13[58]53MN/ANoneCeliac, SMA, splenicAneurysm in splenic, celiac, SMA; dissection in the celiac.Aneurysm in the celiac, splenic, and SMAN/AEmbolization with coil and aortic stent graftN/AN/AImproved
14[59]51MN/AAbdominal pain, shockSMA, IMAAbdominal hemorrhageActive bleeding from SMAN/AEmbolization and ligation of the branches of the SMAN/AWarfarinImproved
15[60]29FN/AHypertensionSMA, RA, hepaticRenal cortical nephrogramsScattered microaneurysms in SMA, RA, hepatic arterySegmental lesions of the media with loss of smooth muscle cellsAnti-coagulantsN/AWarfarinImproved
16[61]55FN/AAbdominal painCeliac, SMA, hepatic, splenicUnremarkable in vesselsAneurysms in celiac, SMA, hepatic, splenic arteryN/AAnti-coagulantsN/AWarfarin followed by aspirinImproved
17[62]56MN/AAbdominal pain, shockSMAAneurysm in MCA, SMA dissectionSaccular aneurysms in the MCA; dissections in the SMAN/AEmbolization with coilN/AN/AImproved
18[63]64FN/AAbdominal pain, back pain, nauseaSMA, IMA, hepaticHematoma in the anterior pararenal space inferior to pancreatic tail; bleeding from aneurysmMultiple aneurysms in the SMA, IMA, hepatic arteryN/AConservativeN/AN/AImproved
19[64]60FHypoxiaHypoxia, hypotension, cardiopulmonary arrestSMALarge hematoma in the retroperitoneal and intraperitoneal space; SMA aneurysmAneurysms and beaded appearance in the SMAN/AConservativeN/AN/AImproved
20[65]36MN/AAbdominal painCeliac, hepatic, anterior inferior pancreaticoduodenal arteryStenosis and aneurysms in anterior inferior pancreatic duodenal arteryAneurysms and beaded like appearance in the anterior inferior pancreaticoduodenal arteryN/AEmbolization with coilN/AN/AImproved
21[66]47MN/ALoss of consciousness, headache, abdominal painSMASAH, massive intraperitoneal hematomaBeaded like appearance in SMA; dissection in VAMedial islands and medial degenerations in SMAEmbolization with coil for VA and SMA. Surgical resection of part of middle colic artery and descending colon.N/AN/AImproved
22[67]79MN/AAbdominal pain, hypotensionIMAActive bleeding from IMA and hemorrhageN/AReduplication of the internal elastic lamina with arterial dissection within the tunica media and thrombus at the site of ruptureSurgical resection of left colic arteryN/AN/AImproved
23[68]40MN/AAbdominal painCeliac, SMAExtensive dissection of SMA with the thrombotic occlusion. stenosis and dilation of celiac arteryN/AN/AConservativeN/AN/AImproved
24[69]32MN/AAbdominal painIMA, RAAneurysm in renal and IMA, massive amount of hemorrhageStenosis and aneurysm in the RAMedia shows myxoid degeneration in the outer one-third adjacent to the adventitiaSurgical hemostasis and left hemicolectomyYes, N/AN/AImproved
25[70]58MN/AAbdominal painSMAMesenteric hematoma and right inguinal hernia with unremarkable small bowelBeaded like appearance in SMAN/AImmunosuppressive therapyN/AN/AImproved
26[71]57MN/AHypertension, abdominal painSMAArterial dissection with luminal stenosis and aneurysm formation at the distal portion of the SMASegmental dilatation, aneurysm in the SMAVacuolization and decrease in the number of vascular smooth musclesAneurysmectomy and bowel resectionCa-blockerN/AImproved
Table 3 Clinical characteristic of the fibromuscular dysplasia and segmental arterial mediolysis
Fibromuscular dysplasiaSegmental arterial mediolysis
GenderFemale (9:1)[2]No presentation[74]
Age of presentationYoung to middle age[2]No preference[74]
Laboratory findingsNo serological markers[74]No serological markers[74]
Risk factorsSmoking and extracranial arteries[4]Hypoxia and shock or other vasoconstrictor stimuli[47]
Vascular distributionRenal and extracranial arteries[4]Celiac and mesenteric arteries[48]
CTAlternating stenosis and aneurysms, less commonly dissections[38]Dissections with alternating stenosis and aneurysms, dissecting aneurysms[48]
AngiographyBeaded aneurysmal appearance (string-of-beads)[38]Beaded aneurysmal appearance (string-of-beads)[38]
PathologyFibrous or fibromuscular thickening of the arterial wall[38]Vecuolization and lysis of the outer media[47]
SymptomsRenovascular hypertension, Headache, Pulsatile tinnitus[4]Acute abdominal pain, Intraperitoneal bleeding[47]
TreatmentAnti-platelet therapy and anti-hypertensive therapy. Balloon angioplasty and stenting[45]Anti-hypertensive therapy and endovascular management, surgical management[74]