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Retinal cavernous hemangioma

MedGen UID:
152678
•Concept ID:
C0730304
•
Neoplastic Process
Synonyms: Cavernous hemangioma of retina; Retinal cavernous angioma
SNOMED CT: Cavernous hemangioma of retina (312937006)
 
HPO: HP:0011513
Monarch Initiative: MONDO:0021281

Definition

A benign tumor of the retina that appears as a grouping of blood-filled saccules within the inner retinal layers or on the surface of the optic disc. Retinal cavernous angioma are described as having a 'cluster of grapes' appearance. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRetinal cavernous hemangioma
Follow this link to review classifications for Retinal cavernous hemangioma in Orphanet.

Professional guidelines

PubMed

Classification and management of hereditary retinal angiomas.
Augsburger JJ, Shields JA, Goldberg RE
Int Ophthalmol 1981 Aug;4(1-2):93-106. doi: 10.1007/BF00139583. PMID: 7298262
Treatment of retinal vascular anomalies.
Gass JD
Trans Sect Ophthalmol Am Acad Ophthalmol Otolaryngol 1977 May-Jun;83(3 Pt 1):OP432-42. PMID: 888256
Fluorescein angiography. An aid in the differential diagnosis of intraocular tumors.
Gass JD
Int Ophthalmol Clin 1972 Spring;12(1):85-120. doi: 10.1097/00004397-197203000-00007. PMID: 4566519

Recent clinical studies

Etiology

Swept-source optical coherence tomography of retinal cavernous hemangioma: a new imaging modality.
Pastor-Idoate S, Gil-Martinez M, Crim N, Quijano C, Biswas S, Charles S, McLeod D, Stanga PE
J Pediatr Ophthalmol Strabismus 2015 Mar 4;52 Online:e4-7. doi: 10.3928/01913913-20150224-02. PMID: 25735011
Retinal cavernous hemangioma: fifty-two years of clinical follow-up with clinicopathologic correlation.
Shields JA, Eagle RC Jr, Ewing MQ, Lally SE, Shields CL
Retina 2014 Jun;34(6):1253-7. doi: 10.1097/IAE.0000000000000232. PMID: 24849703
Twin vessels in familial retinal cavernous hemangioma.
Bottoni F, Canevini MP, Canger R, Orzalesi N
Am J Ophthalmol 1990 Mar 15;109(3):285-9. doi: 10.1016/s0002-9394(14)74551-9. PMID: 2309859
Cavernous hemangioma of the retina, cutaneous angiomas, and intracranial vascular lesion by computed tomography and nuclear magnetic resonance imaging.
Schwartz AC, Weaver RG Jr, Bloomfield R, Tyler ME
Am J Ophthalmol 1984 Oct 15;98(4):483-7. doi: 10.1016/0002-9394(84)90136-3. PMID: 6486224

Diagnosis

Hamartomas of the Retina and Optic Disc.
Mirzayev I, Gündüz AK
Turk J Ophthalmol 2022 Dec 28;52(6):421-431. doi: 10.4274/tjo.galenos.2022.25979. PMID: 36578224Free PMC Article
RETINAL CAVERNOUS HEMANGIOMA WITH INTRALESIONAL PHLEBOLITHS.
Yu MD, Dalvin LA, Shields CL
Retin Cases Brief Rep 2020 Fall;14(4):301-304. doi: 10.1097/ICB.0000000000000724. PMID: 29505489
New Interpretation of Multimodality Fundus Imaging for Retinal Cavernous Hemangioma.
Li J, Li Y, Li H
Curr Eye Res 2019 Apr;44(4):423-427. Epub 2018 Nov 26 doi: 10.1080/02713683.2018.1549263. PMID: 30433829
Retinal Cavernous Hemangioma.
Bloch E, Hakim J
Ophthalmology 2015 Oct;122(10):2037. doi: 10.1016/j.ophtha.2015.07.006. PMID: 26398050
Retinal vascular tumors.
Singh AD, Rundle PA, Rennie I
Ophthalmol Clin North Am 2005 Mar;18(1):167-76, x. doi: 10.1016/j.ohc.2004.07.005. PMID: 15763202

Therapy

RETINAL CAVERNOUS HEMANGIOMA WITH INTRALESIONAL PHLEBOLITHS.
Yu MD, Dalvin LA, Shields CL
Retin Cases Brief Rep 2020 Fall;14(4):301-304. doi: 10.1097/ICB.0000000000000724. PMID: 29505489
Is systemic infliximab therapy effective for retinal cavernous hemangioma?
Alsulaiman SM, Abouammoh MA, Al-Dahmash SA, Abu El-Asrar AM
Saudi Med J 2014 Sep;35(9):1127-30. PMID: 25228188Free PMC Article

Prognosis

Retinal cavernous hemangioma: fifty-two years of clinical follow-up with clinicopathologic correlation.
Shields JA, Eagle RC Jr, Ewing MQ, Lally SE, Shields CL
Retina 2014 Jun;34(6):1253-7. doi: 10.1097/IAE.0000000000000232. PMID: 24849703
Novel KRIT1/CCM1 mutation in a patient with retinal cavernous hemangioma and cerebral cavernous malformation.
Reddy S, Gorin MB, McCannel TA, Tsui I, Straatsma BR
Graefes Arch Clin Exp Ophthalmol 2010 Sep;248(9):1359-61. Epub 2010 Mar 20 doi: 10.1007/s00417-010-1329-6. PMID: 20306072Free PMC Article
A splice-site mutation in CCM1/KRIT1 is associated with retinal and cerebral cavernous hemangioma.
Kitzmann AS, Pulido JS, Ferber MJ, Highsmith WE, Babovic-Vuksanovic D
Ophthalmic Genet 2006 Dec;27(4):157-9. doi: 10.1080/13816810600977168. PMID: 17148043
Retinal vascular tumors.
Singh AD, Rundle PA, Rennie I
Ophthalmol Clin North Am 2005 Mar;18(1):167-76, x. doi: 10.1016/j.ohc.2004.07.005. PMID: 15763202

Clinical prediction guides

Optical coherence tomography angiography findings in retinal cavernous hemangioma: New cases and review of the literature.
Nhari M, Bailleul H, Bricout M, Loria O, Nguyen AM, Kodjikian L, Mathis T
J Fr Ophtalmol 2023 Dec;46(10):1222-1226. Epub 2023 Oct 21 doi: 10.1016/j.jfo.2023.06.012. PMID: 37872067
Hamartomas of the Retina and Optic Disc.
Mirzayev I, Gündüz AK
Turk J Ophthalmol 2022 Dec 28;52(6):421-431. doi: 10.4274/tjo.galenos.2022.25979. PMID: 36578224Free PMC Article
Multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma: a case report.
Zbiba W, Elleuch IE, Sayadi S, Laadheri M
BMC Ophthalmol 2020 Feb 4;20(1):46. doi: 10.1186/s12886-020-1326-4. PMID: 32019534Free PMC Article
Novel KRIT1/CCM1 mutation in a patient with retinal cavernous hemangioma and cerebral cavernous malformation.
Reddy S, Gorin MB, McCannel TA, Tsui I, Straatsma BR
Graefes Arch Clin Exp Ophthalmol 2010 Sep;248(9):1359-61. Epub 2010 Mar 20 doi: 10.1007/s00417-010-1329-6. PMID: 20306072Free PMC Article
A splice-site mutation in CCM1/KRIT1 is associated with retinal and cerebral cavernous hemangioma.
Kitzmann AS, Pulido JS, Ferber MJ, Highsmith WE, Babovic-Vuksanovic D
Ophthalmic Genet 2006 Dec;27(4):157-9. doi: 10.1080/13816810600977168. PMID: 17148043

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