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.