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Retinal Angioma

Retinal Angiomata


Presentation

  • Because of the high risk of the presence of other VHL lesions, thorough screening for VHL is mandatory for patients presenting with a solitary juxtapapillary angioma.[ncbi.nlm.nih.gov]
Vascular Disease
  • This second volume covers tumors, the phacomatoses, and vascular disease. Volume 1 covers the visual sensory system, the autonomic nervous system, the ocular motor system, the eyelid, facial pain and headache, and nonorganic disease.[books.google.de]
  • Ryan Springer Science & Business Media , 29 mar 2010 - 779 pagine Vascular diseases of the retina are a major cause of blindness among all age groups.[books.google.it]
  • Retinal Perforations, Breaks Surgical Procedures and Postoperative Cases Toxic Retinopathies; see also Light Toxicitiy Trauma (Injury) Tumors, Neoplasma Vascular Diseases (see also: Systemic Immunologic Diseases) Sclera Strabismus, Ocular Motility Disorders[atlasophthalmology.net]
  • Machemer R, Williams JM (1988) Pathogenesis and therapy of traction detachment in various retinal vascular diseases. Am J Ophthalmology 105: 170–181 26.[springermedizin.de]
  • Machemer R, Williams JM (1988) Pathogenesis and therapy of traction detachment in various retinal vascular diseases. Am J Ophthalmology 105: 170–181 Google Scholar 26.[link.springer.com]
Flame-shaped Hemorrhage
  • Flame-shaped hemorrhage was found in the temporal and inferior retina, along with dilated retinal veins, whereas macular central reflection was not identified (Figure 1 A).[bmcophthalmol.biomedcentral.com]
Facial Pain
  • Volume 1 covers the visual sensory system, the autonomic nervous system, the ocular motor system, the eyelid, facial pain and headache, and nonorganic disease.[books.google.de]
Neck Mass
  • Enhance your understanding with multiple-choice questions accompanying each case, emphasizing cranial nerves, skull base lesions, sinonasal, orbital, salivary gland, aerodigestive system mucosal lesions and deep space neck masses.[books.google.com]

Workup

  • A workup for the presence of VHL is pertinent in patients presenting with this tumor entity.[ncbi.nlm.nih.gov]
  • Cases for the Diagnostic and Therapeutic Challenges section should include a detailed history of the patient, the diagnosis, the workup, the management, and finally, the question or questions that the submitter wishes to have answered by the consultants[journals.lww.com]
  • The patient was referred to her internal medicine physician for genetic testing and workup for the associated systemic manifestations of von Hippel-Lindau disease.[westcoastretina.com]

Treatment

  • Treatment was rendered over 21 months' time. The treatment resulted in graduate resolution of marked macular edema and exudation, with a gain in visual acuity.[ncbi.nlm.nih.gov]
  • Treatment of leaking angioma from VHL treated with laser.[aao.org]
  • As a general rule; since these tumors are not cancer and cannot metastasize; treatment should only be undertaken in case of tumor progression or a threat to visual acuity due to the adverse effect of treatment on the optic nerve and major blood vessels[eyecancer.com]

Prognosis

  • While the overall visual prognosis for VHL-associated RCH is good, a recent report demonstrated that with mean follow-up of 8.2 4.2 years that on average patients lost of 5.1 0.6 letters.[aao.org]
  • The visual prognosis for patients with retinal capillary hemangiomas is variable and dependent on a variety of factors.[westcoastretina.com]
  • Prognosis is poor, with a median survival of 50 years, with the most common cause of death being RCC and cerebellar hemangioblastomas 1.[radiopaedia.org]
  • Prognosis Prognosis depends on the occurrence of multiple tumors. RCC is the main cause of death, followed by CNS hemangioblastomas.[orpha.net]
  • Cerebral cavernous malformations: natural history and prognosis after clinical deterioration with or without hemorrhage . J Neurosurg 1997; 87 :190–7. 13. Detwiler P , Porter R , Zabramski J, et al.[nature.com]

Etiology

  • Indoor Mold Spore Exposure: A Possible Factor in the Etiology of Multifocal Choroiditis Reuven Rudich, MD, John Santilli, MD, and William J.[kundoc.com]
  • Etiology VHL is caused by highly penetrant mutations in the VHL gene (3p25.3), a classic tumor suppressor. Most cases are diagnosed via a germline mutation.[orpha.net]
  • The exact etiology and pathogenesis behind overlap of these unique syndomes are not clear. [6] Trabecular hyperpigmentation and coexistant structural damage add on to the severity and bilaterality of glaucoma in such overlap syndromes. [7] Both of our[kjophthal.com]
  • This subtype, called VHL type 1B, protects patients from developing renal cell carcinoma. [20] In patients within kindreds having a missense mutation as their genetic etiology for VHL disease (type 2 VHL disease), the risk for developing pheochromocytoma[emedicine.medscape.com]

Epidemiology

  • Epidemiology RAP is now considered a well-established neovascular phenotype of AMD, with distinct clinical and epidemiologic features, most likely due to genetic differences still not completely understood (46).[amdbook.org]
  • A review of the physiopathlology, epidemiology, diagnosis, and treatment for this rare condition. By Hadas Newman, MD ; and Paul T.[retinatoday.com]
  • Summary Epidemiology Prevalence is estimated at 1/53,000 and annual birth incidence at 1/36,000. Men and women are equally affected. Mean age at diagnosis is 26 years (range: infancy - 7th decade).[orpha.net]
  • Children's Mercy Hospital, Molecular Genetics Laboratory, Kansas City, Missouri Johns Hopkins Hospital, DNA Diagnostic Laboratory, Baltimore, Maryland University of Pennsylvania School of Medicine, Genetic Diagnostic Laboratory, Philadelphia, Pennsylvania Epidemiology[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Comprehensively updated to reflect everything you need to know regarding retinal diagnosis, treatment, development, structure, function, and pathophysiology, this monumental ophthalmology reference work equips you with expert answers to virtually any[books.google.com]
  • Useful For Suggests clinical disorders or settings where the test may be helpful Diagnosis of suspected von Hippel-Lindau (VHL) disease Diagnosis of suspected VHL-related hereditary erythrocytosis Clinical Information Discusses physiology, pathophysiology[mayomedicallaboratories.com]
  • Further longitudinal studies with OCTA may bring further insights on the pathophysiology of RAP and establish OCTA imaging as an additional biomarker to guide the treatment and monitoring of these lesions.[amdbook.org]
  • Pathophysiology VHL results from a germline mutation in the VHL gene which is a tumour suppressor gene located on the short arm of chromosome 3 (3p25–26).[eyewiki.aao.org]

Prevention

  • Early diagnosis and treatment of retinal angiomas with an appropriate systemic workup for associated systemic diseases can prevent visual loss and morbidity and mortality.[ncbi.nlm.nih.gov]
  • Early detection and treatment of VHL disease may prevent loss of vision and loss of life.[sjopthal.net]
  • In the hope of preventing retinal detachment when faced with large angiomas, the procedure was begun with circular photocoagulation of the adjacent retina. The feeder vessels were not coagulated in Dr.[healio.com]
  • Laser photocoagulation is directed onto the tumor surface and may be supplemented with barricade and/or grid laser temporally to prevent exudate into the macula.[aao.org]
  • The normal VHL gene acts as a tumor-suppressor gene, with the function of preventing the formation of tumors.[rarediseases.org]

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