Diagnosis and management of fibromuscular dysplasia and segmental arterial mediolysis in gastroenterology field: A mini-review

Table 1 Summary of mesenteric fibromuscular dysplasia reported to date

Case (n)	Ref.	Age (yr)	Gender(Male/Female)	Risk factors	Symptoms	Vessels Involved	CT	Angiography	Pathology	Treatment	Anti-hypertensive drug	Anti-coagulants	Outcome
1	[5]	62	M	N/A	Upper abdominal pain, hemoperitoneum, shock	Celiac, SMA, IMA, RA	N/A	N/A	Intimal thickening in the branches of the SMA and IMA.	Laparotomy	None	None	Died
2	[6]	45	F	N/A	Abdominal pain	SMA, RA	N/A	N/A	N/A	Ileal resection	N/A	N/A	Improved
3	[6]	50	F	N/A	Hypertension, abdominal pain, diarrhea	SMA, RA, iliac	N/A	Stenosis and string-of-beads like Appearance in the SMA	N/A	SMA revascularization	N/A	N/A	Improved
4	[7]	73	F	N/A	N/A	Celiac, SMA, iliac	N/A	N/A	N/A	N/A	N/A	N/A	Improved
5	[7]	42	F	N/A	N/A	SMA, RA	N/A	N/A	N/A	N/A	N/A	N/A	Improved
6	[7]	50	F	N/A	Hypertension	Celiac, SMA, RAl	N/A	Minimal defects in the SMA and RA; stenosis of celiac artery	N/A	N/A	N/A	N/A	Improved
7	[7]	37	F	N/A	Visceral ischemic symptoms	Celiac, SMA, RA	N/A	N/A	N/A	Revascularization	N/A	N/A	Improved
8	[7]	47	F	N/A	Hypertension, abdominal pain	Celiac, SMA, RA	N/A	Defects in the SMA and RA	Medial hyperplasia	None	N/A	N/A	Died
9	[8]	41	F	N/A	Hypertension	SMA, internal carotid, RA, iliac	N/A	Corkscrew and string-of-beads like appearance in the RA, carotid, iliac artery	Replacement of the normal media with disorganized fibrous and muscular hyperplasia	Thromboendarerectomy on SMA	N/A	N/A	Died
10	[9]	64	F	N/A	Unconsciousness	SMA, circle of Willis	N/A	N/A	Medial hyperplasia	None	None	None	Died
11	[10]	21	M	N/A	Hypertension	Celiac, SMA, RA, carotid	N/A	Stenosis of celiac, SMA, RA	Intimal fibroplasia	Anti-hypertensive drug; revascularization of carotid artery; angioplasty of RA	Yes, N/A	N/A	Improved
12	[10]	20	F	N/A	Hypertension	SMA, IMA, RA, carotid	N/A	Stenosis of carotid, renal, SMA. Total occlusion of the IMA.	Intimal fibroplasia	Anti-hypertensive drug, subclavian-carotid bypass; vascular reconstruction of the kidney	Yes, N/A	N/A	Improved
13	[11]	55	M	N/A	N/A	SMA	N/A	N/A	Intimal hyperplasia in SMA	None	N/A	N/A	N/A
14	[12]	44	F	N/A	Asymptomatic bruit of the aortoiliac system	Celiac, SMA, RA, iliac	N/A	String-of-beads like appearance of iliac artery; aneurysms of SMA, RA	Intimal fibrosis with development of fibrosis	Resection and reconstruction	N/A	N/A	Improved
15	[13]	58	F	N/A	Body weight loss	Celiac, SMA, IMA, RA, iliac, aorta	N/A	Occlusion of celiac, SMA, IMA.	N/A	Open surgery	N/A	N/A	Improved
16	[14]	46	F	None (non-smoker)	Palpitations, headache, hypertension	Celiac, SMA	N/A	Aneurysms in the right RA, celiac; occlusion in the left gastric artery	Muscle hypertrophy and disorganisation of elastic tissue of the media in celiac artery.	Surgical ligation	N/A	N/A	Improved
17	[15]	60	M	None (non-smoker)	Left abdominal pain, diarrhea	SMA	Irregular nodular thickening in transverse colon.	Stenoses of the SMA	Intimal fibrosis and focal replacement of medial smooth-muscle fibers by fibrous tissue .	Splenic flexure resection and angioplasty	N/A	N/A	Improved
18	[16]	54	F	Smoking	Hypertension, headache, abdominal pain	SMA, RA, coronary	Liver cyst	Stenosis of the coronary arteries	Intimal hyperplasia in SMA, RA, coronary, splenic, intrahepatic artery	Anti-hypertensive drug	α,β-blocker → Ca blocker	N/A	Died
19	[17]	39	M	N/A	Melena, lower abdominal pain	Jejunal, Sigmoid	N/A	String-of-beads like appearance in the jejunal and sigmoid arteries.	Adventitia is thickened by fibroplasia	Resection of the jejunum	N/A	N/A	Improved
20	[18]	23	M	N/A	Hypertension	Celiac, SMA, RA, carotid, vertebral, opthalmic, superficial temporal, iliac, lumber, intercostal	Hematomasin the paraduodenal and right superior gluteral lesion and splenic infarction	Multiple saccular aneurysms in the celiac, SMA, RA< splenic, hepatic, iliac, lumber, and intercostal arteries	Mediolytic FMD with segmental dissection and thrombosis	Embolization of the gastroduodenal and right SMA to prevent hemorrhage	N/A	N/A	Improved
21	[19]	33	M	N/A	Abdominal pain	SMA	N/A	String-of-beads like appearance in the SMA	Thickening of the media due to hyperplasia in SMA	Ileal resection	N/A	N/A	Improved
22	[20]	78	F	N/A	Hypertension, abdominal pain, hemoperitoneum.	SMA, RA, colonary	Dilated loop of the small bowel and fluid in the peritoneal cavity.	N/A	Medial and perimedial fibrodysplasia, forms the characteristic petal-like appearance in SMA.	None	None	None	Died
23	[21]	43	M	None (non-smoker)	No symptoms	SMA, iliac	SMA aneurysm	Aneurysms in the SMA, hepatic artery, splenic artery, jejunal artery, iliac arteries.	Medial fibrodysplasia in the arteral walls	Aneurysm resection and arterial reconstruction	N/A	N/A	Improved
24	[22]	48	F	None (non-smoker)	Abdominal pain, hemoperitoneum	Celiac, SMA, RA	N/A	Multiple small aneurysms in celiac, SMA, RA	N/A	Surgical hemostasis and anti-hypertensive drugs	β-blocker	N/A	Improved
25	[23]	57	F	Smoking (40 packs/yr)	Abdominal pain, weight loss, anorexia, nausea, vomiting, diarrhea.	Celiac, SMA	Nothing particular	Stenosis of the celiac artery and SMA	Medial thickening, smooth muscle hyperplasia in SMA and celiac artery	Aortoiliac and aorto-SMA bypass	N/A	N/A	Died
26	[24]	48	F	Smoking (20 packs/yr)	Abdominal pain	Celiac, SMA, IMA	N/A	Occlusion of the celiac, SMA; enlarged hypertrophic IMA	Intimal fibroplasia and an increased deposition of fibrous tissue in the vessel wall media	Reimplantation of the SMA	N/A	N/A	Improved
27	[25]	38	M	Smoking	Gastrointestinal bleeding, anemia	SMA, IMA	N/A	Ectasia in IMA; string-of-beads like appearance in the SMA	Thickening and hyalinization of medium sized vessel walls, with intimal proliferation.	Ileal resection	N/A	N/A	Improved
28	[26]	Not provided	Not provided	None (non-smoker)	Abdominal pain, distension, constipation	SMA	N/A	N/A	Thick cuff (petal like) of smooth muscle proliferation with normal intima and media in mesenteric artery.	Right hemicolectomy	N/A	N/A	Improved
29	[27]	43	F	Smoking (10 cigarettes daily for 20 yr)	Hypertension, headache	SMA, RA	N/A	String-of-beads like appearance in the right RA and SMA; stenosis and multiple irregularities in the left RA	N/A	Angioplasty and anti-hypertensive drugs	Yes, N/A	N/A	Improved
30	[28]	38	M	N/A	N/A	N/A	N/A	N/A	N/A	N/A	N/A	N/A	Improved
31	[29]	43	F	N/A	Hypertension, abdominal pain, headache	SMA, RA	Aneurysms in the left RA	Aneurysms in the left RA with sever fibrodysplastic stenosis; string-of-beads like appearance in the right RA; stenosis in SMA	Intimal fibroplasia, lost of internal elastic lamina, and massive destruction of the media in the aneurysm walls	Aneurysm resection and aortorenal bypass and percutaneous transluminal angioplasty	N/A	N/A	Improved
32	[30]	44	F	Oral contraceptive pills	Hypertension, abdominal pain, diarrhea, vomiting	SMA	Stenosis of SMA and nonspecific colitis	stenosis in SMA	N/A	Angioplasty	N/A	N/A	Improved
33	[31]	30	M	N/A	Abdominal pain, hypertension	Celiac, SMA, RA, iliac	Dissections of the celiac, SMA, left RA, and external iliac artery	stenosis in the right RA	N/A	Anti-platelet and anti-hypertensive therapy and angioplasty for right renal artery.	β-blocker, Ca blocker	warfarin, aspirin (100mg)	Improved
34	[32]	47	F	N/A	Abdominal pain, diarrhea, hypertension	All abdominal arteries	A partial occlusion of the celiac artery and a total occlusion of the SMA	N/A	Intimal and medial proliferation	Anti-hypertensive drug	Yes, N/A	N/A	Died
35	[33]	47	F	None (non-smoker)	Nausea, early satiety, abdominal pain	Celiac, SMA	N/A	Stenosis of the SMA, hypertrophy of the gastroduodenal artery and pancreaticoduodenal arteries	N/A	An aorto-superior mesenteric artery and an aorto-hepatic artery bypass.	N/A	N/A	Improved
36	[34]	19	F	N/A	Abdominal pain, vomiting	SMA, RA	N/A	Stenosis of the origin of the SMA and multiple aneurysms involving the proximal SMA. Right renal artery is mild irregularity.	N/A	Resection of the aneurysmal segment in the SMA; aorto-SMA interposition graft with polytetrafluoroethylene	N/A	N/A	Improved
37	[35]	52	M	Smoking	Abdominal pain	IMA	Stenosis of the IMA	N/A	Necrosis of the mucosa; fibrosis of the inima	Left hemicolectomy	N/A	N/A	Improved
38	[36]	20	F	N/A	Abdominal pain, hemorrhagic shock	SMA, right gastroepiploic, jejunal	Intraperitoneal bleeding in the omental bursa and mesentery of the transverse colon	String-of-beads like appearance in the jejunal artery	N/A	Transcatheter arterial embolization	N/A	N/A	Improved
39	[37]	61	F	N/A	Abdominal pain	SMA, IMA, RA	N/A	Multiple aneurysms and stenoses in SMA, IMA, RA	Multiple tears and dissections of the medial layer and fibrointimal thickening	Anti-coagulation	None	Yes, N/A	Improved

M: Male; F: Female; N/A: Data not applicable; SMA: Superior mesenteric artery; IMA: Inferior mesenteric artery; RA: Renal artery; CT: Computed tomography.

Table 2 Summary of mesenteric segmental arterial mediolysis reported to date

Case (n)	Ref.	Age (yr)	Gender (Male/Female)	Risk factors	Symptoms	Vessels Involved	CT	Angiography	Pathology	Treatment	Anti-hypertensive drug	Anti-coagulants	Outcome
1	[48]	65	F	N/A	Abdominal pain	SMA	N/A	Beaded appearance and stenosis of the MCA	Lysis and destruction in the media and intima	Resection of aneurysm in MCA	N/A	N/A	Improved
2	[49]	56	F	N/A	Abdominal pain	IMA	Intraabdominal hemorrhage	Aneurysm in IMA	N/A	Left hemicolectomy	N/A	N/A	Improved
3	[50]	78	M	N/A	Abdominal pain, diarrhea, shock	SMA	N/A	N/A	Destruction of the tunica interna and media in MCA	Emergent surgery (right hemicolectomy); a large hematoma and a ruptured aneurysm upon the surgery	N/A	N/A	Improved
4	[51]	35	F	N/A	Abdominal pain, perforation on transverse colon	SMA	Occlusion of the mesenteric vein and ischemic colitis	Unremarkable	Segmental vacuolar degeneration of smooth muscle with areas of wall thinning	Resection of terminal ileum	N/A	N/A	Died
5	[52]	52	M	N/A	Sudden hemiparesis, hypertension	Celiac, SMA, IMA hepatic artery	Aneurysm in the celiac, hepatic, SMA	Aneurysms in celiac, SMA, ICA, hepatic; stenoses in celiac and SMA	Multiple segmental mediolysis lesions of the muscular and elastic fibers of the media	Reconstruction of hepatic and celiac artery using autologous saphenous vein graft	N/A	N/A	Improved
6	[53]	49	M	N/A	Abdominal pain, shock	SMA	Large hematoma surrounding a high-density aneurysm	Beaded appearance in SMA	Multifocal fragmentation of the elastic fibers of the media	Right hemicolectomy	N/A	N/A	Improved
7	[54]	57	M	N/A	Abdominal pain	SMA, hepatic	Small aneurysm at the middle colic artery and mesenteric hematoma	Aneurysm and stenosis of the celiac, SMA, hepatic artery	N/A	Embolization with N-butyl cyanoacrylate for aneurysm in the SMA	N/A	N/A	Improved
8	[54]	76	F	N/A	Abdominal pain, nausea	IMA	Mesenteric hematoma	Aneurysm in IMA	N/A	Embolization with coil	N/A	N/A	Died
9	[55]	59	M	N/A	Abdominal pain, shock	SMA, RA, gastroepiploic, splenic	SMA dissection, aneurysm in RA, gastroepiploic, splenic artery; rupture of the splenic aneurysm	Saccular aneurysms and multiple stenotic region in gastroepiploic artery	Medial island spared from mediolysis	Emergency embolization of the splenic artery, resection of aneurysm in the gastroepiploic	N/A	N/A	Improved
10	[56]	57	M	N/A	Abdominal pain, diarrhea	SMA	Ascites throughout the abdomen	Aneurysm in SMA	N/A	Transcatheter arterial embolization	N/A	N/A	Improved
11	[57]	60	M	N/A	N/A	SMA	Rupture of the aneurysm of the MCA	Multiple beaded patters and aneurysm in SMA	N/A	Surgical resection	N/A	N/A	Improved
12	[47]	25	F	N/A	Anorexia, abdominal pain, diarrhea	SMA, hepatic	Ischemic colitis of the splenic flexure	Occlusion of IMA; stenoses of the hepatic artery	Patchy, isolated destruction of the arterial media involving both the internal and external elastic laminae	Partial colectomy of the splenic flexure	N/A	N/A	Improved
13	[58]	53	M	N/A	None	Celiac, SMA, splenic	Aneurysm in splenic, celiac, SMA; dissection in the celiac.	Aneurysm in the celiac, splenic, and SMA	N/A	Embolization with coil and aortic stent graft	N/A	N/A	Improved
14	[59]	51	M	N/A	Abdominal pain, shock	SMA, IMA	Abdominal hemorrhage	Active bleeding from SMA	N/A	Embolization and ligation of the branches of the SMA	N/A	Warfarin	Improved
15	[60]	29	F	N/A	Hypertension	SMA, RA, hepatic	Renal cortical nephrograms	Scattered microaneurysms in SMA, RA, hepatic artery	Segmental lesions of the media with loss of smooth muscle cells	Anti-coagulants	N/A	Warfarin	Improved
16	[61]	55	F	N/A	Abdominal pain	Celiac, SMA, hepatic, splenic	Unremarkable in vessels	Aneurysms in celiac, SMA, hepatic, splenic artery	N/A	Anti-coagulants	N/A	Warfarin followed by aspirin	Improved
17	[62]	56	M	N/A	Abdominal pain, shock	SMA	Aneurysm in MCA, SMA dissection	Saccular aneurysms in the MCA; dissections in the SMA	N/A	Embolization with coil	N/A	N/A	Improved
18	[63]	64	F	N/A	Abdominal pain, back pain, nausea	SMA, IMA, hepatic	Hematoma in the anterior pararenal space inferior to pancreatic tail; bleeding from aneurysm	Multiple aneurysms in the SMA, IMA, hepatic artery	N/A	Conservative	N/A	N/A	Improved
19	[64]	60	F	Hypoxia	Hypoxia, hypotension, cardiopulmonary arrest	SMA	Large hematoma in the retroperitoneal and intraperitoneal space; SMA aneurysm	Aneurysms and beaded appearance in the SMA	N/A	Conservative	N/A	N/A	Improved
20	[65]	36	M	N/A	Abdominal pain	Celiac, hepatic, anterior inferior pancreaticoduodenal artery	Stenosis and aneurysms in anterior inferior pancreatic duodenal artery	Aneurysms and beaded like appearance in the anterior inferior pancreaticoduodenal artery	N/A	Embolization with coil	N/A	N/A	Improved
21	[66]	47	M	N/A	Loss of consciousness, headache, abdominal pain	SMA	SAH, massive intraperitoneal hematoma	Beaded like appearance in SMA; dissection in VA	Medial islands and medial degenerations in SMA	Embolization with coil for VA and SMA. Surgical resection of part of middle colic artery and descending colon.	N/A	N/A	Improved
22	[67]	79	M	N/A	Abdominal pain, hypotension	IMA	Active bleeding from IMA and hemorrhage	N/A	Reduplication of the internal elastic lamina with arterial dissection within the tunica media and thrombus at the site of rupture	Surgical resection of left colic artery	N/A	N/A	Improved
23	[68]	40	M	N/A	Abdominal pain	Celiac, SMA	Extensive dissection of SMA with the thrombotic occlusion. stenosis and dilation of celiac artery	N/A	N/A	Conservative	N/A	N/A	Improved
24	[69]	32	M	N/A	Abdominal pain	IMA, RA	Aneurysm in renal and IMA, massive amount of hemorrhage	Stenosis and aneurysm in the RA	Media shows myxoid degeneration in the outer one-third adjacent to the adventitia	Surgical hemostasis and left hemicolectomy	Yes, N/A	N/A	Improved
25	[70]	58	M	N/A	Abdominal pain	SMA	Mesenteric hematoma and right inguinal hernia with unremarkable small bowel	Beaded like appearance in SMA	N/A	Immunosuppressive therapy	N/A	N/A	Improved
26	[71]	57	M	N/A	Hypertension, abdominal pain	SMA	Arterial dissection with luminal stenosis and aneurysm formation at the distal portion of the SMA	Segmental dilatation, aneurysm in the SMA	Vacuolization and decrease in the number of vascular smooth muscles	Aneurysmectomy and bowel resection	Ca-blocker	N/A	Improved

M: Male; F: Female; N/A: Data not applicable; SMA: Superior mesenteric artery; IMA: Inferior mesenteric artery; RA: Renal artery; CT: Computed tomography; MCA: Middle cerebral artery.

Table 3 Clinical characteristic of the fibromuscular dysplasia and segmental arterial mediolysis

	Fibromuscular dysplasia	Segmental arterial mediolysis
Gender	Female (9:1)[2]	No presentation[74]
Age of presentation	Young to middle age[2]	No preference[74]
Laboratory findings	No serological markers[74]	No serological markers[74]
Risk factors	Smoking and extracranial arteries[4]	Hypoxia and shock or other vasoconstrictor stimuli[47]
Vascular distribution	Renal and extracranial arteries[4]	Celiac and mesenteric arteries[48]
CT	Alternating stenosis and aneurysms, less commonly dissections[38]	Dissections with alternating stenosis and aneurysms, dissecting aneurysms[48]
Angiography	Beaded aneurysmal appearance (string-of-beads)[38]	Beaded aneurysmal appearance (string-of-beads)[38]
Pathology	Fibrous or fibromuscular thickening of the arterial wall[38]	Vecuolization and lysis of the outer media[47]
Symptoms	Renovascular hypertension, Headache, Pulsatile tinnitus[4]	Acute abdominal pain, Intraperitoneal bleeding[47]
Treatment	Anti-platelet therapy and anti-hypertensive therapy. Balloon angioplasty and stenting[45]	Anti-hypertensive therapy and endovascular management, surgical management[74]

CT: Computed tomography.