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Retinal Cavernous Hemangioma


Presentation

  • The authors present a case of a previously undiagnosed retinal cavernous hemangioma that hemorrhaged during labor.[ncbi.nlm.nih.gov]
  • Hundreds of full-color images present conditions as they present in real life. Common diagnostic pitfalls discuss what to look out for when making a difficult diagnosis.[books.google.com]
Asymptomatic
  • […] is a rare, benign, retinal tumor characterized by angiomatous proliferation of vessels within the inner retina or the optic disc.1 Here we report a case of retinal cavernous hemangioma on the margin of the optic disc in the right eye of a 61-year-old asymptomatic[ncbi.nlm.nih.gov]
  • […] a rare, benign, retinal tumor characterized by angiomatous proliferation of vessels within the inner retina or the optic disc. 1 Here we report a case of retinal cavernous hemangioma on the margin of the optic disc in the right eye of a 61-year-old asymptomatic[healio.com]
  • Rarely, the hemangiomas may bleed into the vitreous, but they usually remain asymptomatic. Vitreous traction is thought to be the cause of hemorrhage in these cases.[aao.org]
  • Almost always asymptomatic, although macular involvement or vitreous hemorrhage has been reported. Skin and central nervous system hemangiomas may co-exist, and autosomal dominant pedigrees have been reported.[webeye.ophth.uiowa.edu]
Ascitic Fluid
  • Tumor cells secrete a vascular permeability factor that promotes accumulation of ascites fluid. ‎[books.google.de]
Movement Disorder
  • […] conjunctiva swelling were seen in 19 cases, decreased visual acuity and eye movement disorder with eye pain were seen in 9 cases, and there were no obvious symptoms and signs in 3 cases.[omicsonline.org]
Macula
  • A 10-month-old infant with the diagnosis of retinal cavernous hemangioma involving the macula presented with an outward deviation of the right eye that has been noticed by her parents.[ncbi.nlm.nih.gov]
  • The macula was flat and dry, and the vessels were normal. The periphery was intact. There were no vitreous opacities or heme. No PVD or cells.[webeye.ophth.uiowa.edu]
  • Cavernous hemangiomas are visually insignificant in the majority of cases unless the macula is involved or vitreous hemorrhage occurs (an uncommon occurrence).[westcoastretina.com]
  • Retinal cavernous hemangiomas are symptomatic when they are located in or adjacent to the macula which has been reported in approximately 10% of the cases in the literature. 1, 5, 6 In these cases, visual acuity can be decreased on the basis of both tumor[clinicalgate.com]
Scotoma
  • Visual field testing may reveal a scotoma in the area corresponding to the tumor.[entokey.com]
  • Choroidal melanoma is usually asymptomatic with painless, progressive visual loss and paracentral scotoma in routine examination of the orbit.[omicsonline.org]
Eye Pain
  • Our patient was recovering from gastroenteritis, during which she described multiple episodes of forceful emesis shortly before the development of eye pain.[journals.lww.com]
  • Clinical symptoms and signs We studied 87 patients with orbital tumors: Clinical symptoms of eye pain and proptosis were seen in 44 cases, orbital masses with eye pain, decreased visual acuity were seen in 17 cases, eye movement disorder with eye pain[omicsonline.org]
Enlarged Blind Spot
  • An enlarged blind spot may be demonstrated on the visual field, but visual acuity is normal.[clinicalgate.com]
Intracranial Hemorrhage
  • There are previous reports of labor-induced intracranial hemorrhage from central nervous system vascular malformations, but none have demonstrated intraocular hemorrhage during labor.[ncbi.nlm.nih.gov]
  • CMs may be seen as an incidental finding on MRI studies, or they may present with symptoms, such as seizures or intracranial hemorrhage, often causing severe neurologic deficit.[books.google.com]
  • These are benign aberrant growths of capillary endothelium which develop shortly after birth and cause a variety of signs and symptoms including seizures, intracranial hemorrhage, and focal neurologic deficits.[disorders.eyes.arizona.edu]
  • Cavernous hemangioma of the retina Benign vascular tumor most commonly diagnosed in children or young adults and may be associated with intracranial hemorrhage resulting in stroke, cranial nerve palsies, and their related signs and symptoms. [64] Although[ijo.in]

Workup

  • However, genetic diagnostics alone in a patient who presents with a retinal cavernous hemangioma is not a substitute for the neurologic and systemic workup that is required, since the condition is genetically heterogeneous.[link.springer.com]
  • Diagnosis, Workup and Treatment The patient’s presentation of proptosis and diplopia was in line with an orbital process, as confirmed with MRI.[reviewofophthalmology.com]
Gliosis
  • Inferiorly from 5:00 to clockwise 6:30 in the mid-periphery, there are two almost contiguous clusters of red dilated saccules in the retina, with central gray-white surface gliosis. There are two similar but smaller lesions at 4:00 and 7:00.[webeye.ophth.uiowa.edu]
  • Fundus examination of left eye revealed a group of blood-filled saccules resembling a ‘cluster of grapes’ within the inner retinal layers with surface gliosis (Figure 1A). There was no subretinal exudation or feeder vessels.[djo.org.in]
  • Discussion Cavernous hemangiomas of the retina are rare, congenital hamartomas composed of multiple, thin-walled dilated vascular channels with surface gliosis. 2 The lesions are generally asymptomatic unless the macula is involved or there is associated[westcoastretina.com]
  • In the end stage, total retinal detachment with massive retinal gliosis, cataract, and phthisis bulbi can occur.[retinatoday.com]

Treatment

  • Put the very latest scientific and genetic discoveries, diagnostic imaging methods, drug therapies, treatment recommendations, and surgical techniques to work in your practice.[books.google.de]
  • Helps you make optimal use of the newest drug therapies, including Anti-VEGF treatment for wet ARMD and bevacizumab treatment for complications of diabetes.[books.google.com]
  • As a general rule, retinal cavernous hemangioma requires no active treatment. If vitreous hemorrhage should occur, laser or cryotherapy to the tumor can be attempted. If vitreous blood does not resolve, removal by vitrectomy may be necessary.[alpfmedical.info]
  • Treatment of retinal cavernous hemangiomas is usually not indicated unless recurrent vitreous hemorrhage develops, in which case photocoagulation or cryotherapy may be effective.[aao.org]

Prognosis

  • Prognosis This is varied between patients and may be non-progressing (or slowly progressing) in some patients and rapidly progressing in others. There is a guarded visual prognosis.[patient.info]
  • […] compression and chiasmal syndrome. 3 The average life expectancy of patients with VHL is less than 50 years, with morbidity most commonly due to renal cell carcinoma. 1,3,5 Early detection of affected gene carriers can play a critical role in improved prognosis[reviewofoptometry.com]
  • Though genetic results to date have no implication with regard to health or prognosis, the demonstration of a causative mutation in the KRIT1/CCM1 gene does provide greater certainty of the diagnosis, allows us to better justify future screening for the[link.springer.com]
  • The presenting visual acuity also correlates with the long-term prognosis.[institut-vision.org]
  • Prognosis The prognosis for all forms of nevi tends to be good with iris nevi showing enlargement in less than 5% of cases (Territo et al., 1988).[atlasgeneticsoncology.org]

Etiology

  • The bleeding recurred throughout childhood and the etiology was not determined.[ncbi.nlm.nih.gov]
  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • Disease Medulloepithelioma Etiology Medulloepithelioma is a tumor that arises from the epithelium of the medullary tube, most often the ciliary body.[atlasgeneticsoncology.org]
  • Epidemiology and Etiology Rb occurs in approximately 1 in 15,000 live births. Most children are diagnosed at a mean of 18 months. No predilection for race or gender has been shown.[entokey.com]

Epidemiology

  • Epidemiology and Etiology Rb occurs in approximately 1 in 15,000 live births. Most children are diagnosed at a mean of 18 months. No predilection for race or gender has been shown.[entokey.com]
  • Epidemiology Choroidal melanomas are found most often in countries with large populations of people of northern European decent.[atlasgeneticsoncology.org]
  • The Epidemiology of Diabetic Retinopathy 46. Diabetic Retinopathy: Genetics and Etiologic Mechanisms 47. Nonproliferative Diabetic Retinopathy and Diabetic Macular Edema 48. Proliferative Diabetic Retinopathy 49. Hypertension 50.[elsevier.com]
  • Epidemiology Frequency International Henderson reported an incidence of 4.3% among orbital neoplasms. [1] Mortality/Morbidity The morbidity associated with cavernous hemangiomas is the threat of compressive optic neuropathy, extraocular muscle dysfunction[emedicine.medscape.com]
  • Epidemiology Coats disease is a very rare eye disorder. The exact prevalence is unknown. There is a clear male predominance (more than 75% of patients) and no ethnic predisposition.[institut-vision.org]
Sex distribution
Age distribution

Pathophysiology

  • Spectral domain optical coherence tomography and scanning laser ophthalmoscope retro-mode imaging may be useful for evaluating the pathophysiologic features and the full extent of the retinal cavernous hemangioma.[ncbi.nlm.nih.gov]
  • 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.de]
  • Kaposi SarcomaKaposi Sarcoma Histopathology, Pathophysiology,Histopathology, Pathophysiology, TreatmentTreatment Combination of proliferating spindled &endothelial cells – Extravasated erythrocytes – Staghorn clefts (veins) Slowly enlarge New lesions[slideshare.net]
  • Retina-Related Clinical Trials: A Resource Bibliography Part 1: Surgical Retina Section 1: The Pathophysiology of Retinal Detachment and Associated Problems 95. Pathogenetic Mechanisms of Retinal Detachment 96.[elsevier.com]

Prevention

  • Optical shadowing was present, preventing establishment of any subretinal component to the lesion. Swept-source OCT (SS-OCT) showed an intraretinal lesion consisting of a group of clearly defined grape-like caverns with overlying preretinal tissue.[ncbi.nlm.nih.gov]
  • […] prognosis, the demonstration of a causative mutation in the KRIT1/CCM1 gene does provide greater certainty of the diagnosis, allows us to better justify future screening for the development of new lesions, and contributes to the genetic counseling and preventive[link.springer.com]
  • Alloplant is a biomaterial, which is low (small), the antigenicity of, preventing the formation of scars in the area of the transplant, the selective growth of the tissue of the recipient, provided the wider its introduction into practice.[elitemedical.rs]
  • A number of medications are being investigated to prevent hemorrhage and to prevent the growth of new lesions in those with the familial form. Expanded information on each of these topics can be found using our menus on the left.[angiomaalliance.org]
  • Ophthalmic Plastic Surgery Prevention and Management of Complications . New York: Raven Press; 1994. 312-25. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit. J Neurosurg . 1979 Aug. 51(2):219-28. [Medline] . Mercandetti M, Cohen AJ.[emedicine.medscape.com]

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