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Arterial dissection-lentiginosis syndrome

MedGen UID:
325345
•Concept ID:
C1838122
•
Disease or Syndrome
Synonym: Arterial dissection with lentiginosis
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0010889
OMIM®: 600459
Orphanet: ORPHA1682

Definition

A rare association syndrome, reported in several members of two families to date, characterized by arterial dissection, occurring at an early age and presenting with a range of manifestations depending on the vascular territory involved (ex. headache, dysphasia, hemiparesis), in association with cystic medial necrosis and multiple lentigines (brown and black in color and mainly affecting the skin of the trunk and extremities). [from ORDO]

Clinical features

From HPO
Arterial dissection
MedGen UID:
8077
•Concept ID:
C0002949
•
Disease or Syndrome
A separation (dissection) of the layers of an artery.
See: Feature record | Search on this feature
Cystic medial necrosis
MedGen UID:
1638446
•Concept ID:
C4551473
•
Disease or Syndrome
A disorder of large arteries, in particular the aorta, characterized by an accumulation of basophilic ground substance in the media with cyst-like lesions associated with degenerative changes of collagen, elastin and the vascular smooth muscle cells.
See: Feature record | Search on this feature
Multiple lentigines
MedGen UID:
272242
•Concept ID:
C1328931
•
Disease or Syndrome
Presence of an unusually high number of lentigines (singular
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVArterial dissection-lentiginosis syndrome
Follow this link to review classifications for Arterial dissection-lentiginosis syndrome in Orphanet.

Professional guidelines

PubMed

2023 ACC/AHA/ACCP/HRS Guideline for the Diagnosis and Management of Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.
Joglar JA, Chung MK, Armbruster AL, Benjamin EJ, Chyou JY, Cronin EM, Deswal A, Eckhardt LL, Goldberger ZD, Gopinathannair R, Gorenek B, Hess PL, Hlatky M, Hogan G, Ibeh C, Indik JH, Kido K, Kusumoto F, Link MS, Linta KT, Marcus GM, McCarthy PM, Patel N, Patton KK, Perez MV, Piccini JP, Russo AM, Sanders P, Streur MM, Thomas KL, Times S, Tisdale JE, Valente AM, Van Wagoner DR; Peer Review Committee Members
Circulation 2024 Jan 2;149(1):e1-e156. Epub 2023 Nov 30 doi: 10.1161/CIR.0000000000001193. PMID: 38033089Free PMC Article
2023 AHA/ACC/ACCP/ASPC/NLA/PCNA Guideline for the Management of Patients With Chronic Coronary Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.
Virani SS, Newby LK, Arnold SV, Bittner V, Brewer LC, Demeter SH, Dixon DL, Fearon WF, Hess B, Johnson HM, Kazi DS, Kolte D, Kumbhani DJ, LoFaso J, Mahtta D, Mark DB, Minissian M, Navar AM, Patel AR, Piano MR, Rodriguez F, Talbot AW, Taqueti VR, Thomas RJ, van Diepen S, Wiggins B, Williams MS; Peer Review Committee Members
Circulation 2023 Aug 29;148(9):e9-e119. Epub 2023 Jul 20 doi: 10.1161/CIR.0000000000001168. PMID: 37471501
2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.
Isselbacher EM, Preventza O, Hamilton Black J 3rd, Augoustides JG, Beck AW, Bolen MA, Braverman AC, Bray BE, Brown-Zimmerman MM, Chen EP, Collins TJ, DeAnda A Jr, Fanola CL, Girardi LN, Hicks CW, Hui DS, Schuyler Jones W, Kalahasti V, Kim KM, Milewicz DM, Oderich GS, Ogbechie L, Promes SB, Gyang Ross E, Schermerhorn ML, Singleton Times S, Tseng EE, Wang GJ, Woo YJ; Peer Review Committee Members
Circulation 2022 Dec 13;146(24):e334-e482. Epub 2022 Nov 2 doi: 10.1161/CIR.0000000000001106. PMID: 36322642Free PMC Article

Recent clinical studies

Etiology

Diagnosis and Management of Acute Aortic Syndromes: Dissection, Penetrating Aortic Ulcer, and Intramural Hematoma.
Sorber R, Hicks CW
Curr Cardiol Rep 2022 Mar;24(3):209-216. Epub 2022 Jan 14 doi: 10.1007/s11886-022-01642-3. PMID: 35029783Free PMC Article
Patterns of ischemic posterior circulation strokes: A clinical, anatomical, and radiological review.
Salerno A, Strambo D, Nannoni S, Dunet V, Michel P
Int J Stroke 2022 Aug;17(7):714-722. Epub 2021 Sep 28 doi: 10.1177/17474930211046758. PMID: 34581223Free PMC Article
Inflammation during the life cycle of the atherosclerotic plaque.
Libby P
Cardiovasc Res 2021 Nov 22;117(13):2525-2536. doi: 10.1093/cvr/cvab303. PMID: 34550337Free PMC Article
Epidemiology and management of aortic disease: aortic aneurysms and acute aortic syndromes.
Bossone E, Eagle KA
Nat Rev Cardiol 2021 May;18(5):331-348. Epub 2020 Dec 22 doi: 10.1038/s41569-020-00472-6. PMID: 33353985
Diagnosis and management of acute aortic syndromes in the emergency department.
Morello F, Santoro M, Fargion AT, Grifoni S, Nazerian P
Intern Emerg Med 2021 Jan;16(1):171-181. Epub 2020 May 1 doi: 10.1007/s11739-020-02354-8. PMID: 32358680

Diagnosis

Diagnosis and Management of Acute Aortic Syndromes: Dissection, Penetrating Aortic Ulcer, and Intramural Hematoma.
Sorber R, Hicks CW
Curr Cardiol Rep 2022 Mar;24(3):209-216. Epub 2022 Jan 14 doi: 10.1007/s11886-022-01642-3. PMID: 35029783Free PMC Article
Patterns of ischemic posterior circulation strokes: A clinical, anatomical, and radiological review.
Salerno A, Strambo D, Nannoni S, Dunet V, Michel P
Int J Stroke 2022 Aug;17(7):714-722. Epub 2021 Sep 28 doi: 10.1177/17474930211046758. PMID: 34581223Free PMC Article
Epidemiology and management of aortic disease: aortic aneurysms and acute aortic syndromes.
Bossone E, Eagle KA
Nat Rev Cardiol 2021 May;18(5):331-348. Epub 2020 Dec 22 doi: 10.1038/s41569-020-00472-6. PMID: 33353985
Diagnosis and management of acute aortic syndromes in the emergency department.
Morello F, Santoro M, Fargion AT, Grifoni S, Nazerian P
Intern Emerg Med 2021 Jan;16(1):171-181. Epub 2020 May 1 doi: 10.1007/s11739-020-02354-8. PMID: 32358680
Reversible cerebral vasoconstriction syndrome.
Ducros A
Lancet Neurol 2012 Oct;11(10):906-17. doi: 10.1016/S1474-4422(12)70135-7. PMID: 22995694

Therapy

Clinical Opinion: The diagnosis and management of suspected fetal growth restriction: an evidence-based approach.
Lees CC, Romero R, Stampalija T, Dall'Asta A, DeVore GA, Prefumo F, Frusca T, Visser GHA, Hobbins JC, Baschat AA, Bilardo CM, Galan HL, Campbell S, Maulik D, Figueras F, Lee W, Unterscheider J, Valensise H, Da Silva Costa F, Salomon LJ, Poon LC, Ferrazzi E, Mari G, Rizzo G, Kingdom JC, Kiserud T, Hecher K
Am J Obstet Gynecol 2022 Mar;226(3):366-378. Epub 2022 Jan 10 doi: 10.1016/j.ajog.2021.11.1357. PMID: 35026129Free PMC Article
Acute Aortic Syndromes.
Rogers RK, Reece TB, Bonaca MP, Hess CN
Cardiol Clin 2021 Nov;39(4):495-503. doi: 10.1016/j.ccl.2021.06.002. PMID: 34686262
Pathologic Disparities Between Peripheral Artery Disease and Coronary Artery Disease.
Narula N, Olin JW, Narula N
Arterioscler Thromb Vasc Biol 2020 Sep;40(9):1982-1989. Epub 2020 Jul 16 doi: 10.1161/ATVBAHA.119.312864. PMID: 32673526
Transradial Artery Access Complications.
Sandoval Y, Bell MR, Gulati R
Circ Cardiovasc Interv 2019 Nov;12(11):e007386. Epub 2019 Nov 1 doi: 10.1161/CIRCINTERVENTIONS.119.007386. PMID: 31672030
Fibromuscular dysplasia.
Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X
Orphanet J Rare Dis 2007 Jun 7;2:28. doi: 10.1186/1750-1172-2-28. PMID: 17555581Free PMC Article

Prognosis

Patterns of ischemic posterior circulation strokes: A clinical, anatomical, and radiological review.
Salerno A, Strambo D, Nannoni S, Dunet V, Michel P
Int J Stroke 2022 Aug;17(7):714-722. Epub 2021 Sep 28 doi: 10.1177/17474930211046758. PMID: 34581223Free PMC Article
Supravalvular aortic stenosis: elastin arteriopathy.
Merla G, Brunetti-Pierri N, Piccolo P, Micale L, Loviglio MN
Circ Cardiovasc Genet 2012 Dec;5(6):692-6. doi: 10.1161/CIRCGENETICS.112.962860. PMID: 23250899
Reversible cerebral vasoconstriction syndrome.
Ducros A
Lancet Neurol 2012 Oct;11(10):906-17. doi: 10.1016/S1474-4422(12)70135-7. PMID: 22995694
Terson's syndrome.
Hassan A, Lanzino G, Wijdicks EF, Rabinstein AA, Flemming KD
Neurocrit Care 2011 Dec;15(3):554-8. doi: 10.1007/s12028-011-9555-2. PMID: 21604080
Lacunar infarct.
Lastilla M
Clin Exp Hypertens 2006 Apr-May;28(3-4):205-15. doi: 10.1080/10641960600549082. PMID: 16833026

Clinical prediction guides

Pathologic Disparities Between Peripheral Artery Disease and Coronary Artery Disease.
Narula N, Olin JW, Narula N
Arterioscler Thromb Vasc Biol 2020 Sep;40(9):1982-1989. Epub 2020 Jul 16 doi: 10.1161/ATVBAHA.119.312864. PMID: 32673526
Vascular compression syndromes.
Czihal M, Banafsche R, Hoffmann U, Koeppel T
Vasa 2015 Nov;44(6):419-34. doi: 10.1024/0301-1526/a000465. PMID: 26515219
Popliteal artery entrapment syndrome.
Lejay A, Ohana M, Lee JT, Georg Y, Delay C, Lucereau B, Thaveau F, Gaertner S, Chakfé N; Groupe Européen de Recherche sur les Prothèses Appliquées à la Chirurgie Vasculaire (GEPROVAS)
J Cardiovasc Surg (Torino) 2014 Apr;55(2 Suppl 1):225-37. PMID: 24796917
Popliteal entrapment syndrome.
Sinha S, Houghton J, Holt PJ, Thompson MM, Loftus IM, Hinchliffe RJ
J Vasc Surg 2012 Jan;55(1):252-262.e30. Epub 2011 Nov 23 doi: 10.1016/j.jvs.2011.08.050. PMID: 22116047
Clinical cerebrovascular anatomy.
Krishnaswamy A, Klein JP, Kapadia SR
Catheter Cardiovasc Interv 2010 Mar 1;75(4):530-9. doi: 10.1002/ccd.22299. PMID: 20049963

Recent systematic reviews

Direct-acting oral anticoagulants in antiphospholipid syndrome: A systematic review.
Girón-Ortega JA, Girón-González JA
Med Clin (Barc) 2023 Jul 21;161(2):65-77. Epub 2023 Apr 25 doi: 10.1016/j.medcli.2023.03.011. PMID: 37105842
Clinical features and complications of Loeys-Dietz syndrome: A systematic review.
Gouda P, Kay R, Habib M, Aziz A, Aziza E, Welsh R
Int J Cardiol 2022 Sep 1;362:158-167. Epub 2022 Jun 1 doi: 10.1016/j.ijcard.2022.05.065. PMID: 35662564
Cardiovascular Changes in Menopause.
Nair AR, Pillai AJ, Nair N
Curr Cardiol Rev 2021;17(4):e230421187681. doi: 10.2174/1573403X16666201106141811. PMID: 33155924Free PMC Article
Global vascular guidelines on the management of chronic limb-threatening ischemia.
Conte MS, Bradbury AW, Kolh P, White JV, Dick F, Fitridge R, Mills JL, Ricco JB, Suresh KR, Murad MH; GVG Writing Group
J Vasc Surg 2019 Jun;69(6S):3S-125S.e40. Epub 2019 May 28 doi: 10.1016/j.jvs.2019.02.016. PMID: 31159978Free PMC Article
Ocular ischemic syndrome - a systematic review.
Terelak-Borys B, Skonieczna K, Grabska-Liberek I
Med Sci Monit 2012 Aug;18(8):RA138-144. doi: 10.12659/msm.883260. PMID: 22847215Free PMC Article

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