Ophthalmological Aspects of von-Hippel-Lindau Syndrome.
Semin Ophthalmol
; 36(7): 531-540, 2021 Oct 03.
Article
en En
| MEDLINE
| ID: mdl-33780299
ABSTRACT
Background:
von Hippel-Lindau (VHL) syndrome is a multisystem neoplastic disorder involving eyes, central nervous system, kidneys, spine, and other tissues. A retinal capillary hemangioma (RCH) is the earliest manifestation of the VHL disease in most cases.Areas coveredThis paper aims to provide an up-to-date review of the current literature about von Hippel-Lindau syndrome. Molecular background, systemic and ocular features of the diseases as well as the utility of newer imaging modalities in diagnosis and monitoring of ocular VHL disease have been described. Besides, we have discussed newer treatment modalities and therapeutic targets.Conclusion:
Modern imaging technologies like optical coherence tomography and optical coherence tomography angiography are tools of the trade, in making an appropriate diagnosis and monitoring disease activity and response to treatment. Peripheral RCH may be treated using laser photocoagulation in tumors up to 3000 µm. Vascular endothelial growth factor suppression can help in reducing tumor activity and stabilize the tumor size; however, it does not regress the RCH.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Hemangioblastoma
/
Neoplasias de la Retina
/
Enfermedad de von Hippel-Lindau
Tipo de estudio:
Diagnostic_studies
Límite:
Humans
Idioma:
En
Revista:
Semin Ophthalmol
Asunto de la revista:
OFTALMOLOGIA
Año:
2021
Tipo del documento:
Article
País de afiliación:
Pakistán
Pais de publicación:
ENGLAND
/
ESCOCIA
/
GB
/
GREAT BRITAIN
/
INGLATERRA
/
REINO UNIDO
/
SCOTLAND
/
UK
/
UNITED KINGDOM