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The main goal of treating patients with an angioma (or angiomas) of the retina is to prevent the development of exudativex retinal complications leading to vision loss. In this regard, the treatment of patients with Hippel-Lindau disease should be started as early as possible, since the therapy of large tumors is ineffective.Learn More
With retinal angiomas, the diameter of which does not exceed 2.5 RD, laser coagulation is usually performed. The most commonly used for this purpose are argon green and blue-green, neodymium: YAG green and krypton yellow lasers. The exposure is 0.2–1.0 s, the diameter of the coagulates is 200–500 µm, and the power is 200–800 mW.
For small angiomas, direct tumor coagulation is most effective, which is usually carried out in several sessions to avoid the development of secondary exudative retinal detachment.Read and Watch
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As our experience shows, in angiomas with a diameter greater than 3 mm, laser coagulation in the form of successive series is more effective: at the first stage, barrier coagulation is performed around the body of the tumor and the vessels that feed it (coreg), at the second - coagulation adductor arterioles, on the third - angioma coagulation during several sessions.
The afferent arteriole must be coagulated until the ce segment is completely obliterated, when it becomes impossible to spontaneously open the vessel and restore blood flow through it. Intraocular hypertension artificially created during laser coagulation reduces blood flow in arterioles and promotes obliteration of the supply vessels. Treatment should be repeated every 2–8 weeks until the angioma is no longer perfused. According to J.S.Shields (1994), after complete obliteration of the adducting vessels, coagulation of the draining veins is advisable.
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Most often, these are retinal angiomas, hemangioblastomas of the central nervous system, pheochromocytoma, neoplasms of the kidneys and pancreas. Sometimes a single tumor process is a manifestation of carvedilol pills. The diagnosis is verified after neurological and ophthalmological examinations, CT or MRI of the brain and spine, ultrasound or CT scan of the kidneys, pancreas, adrenal glands, genetic diagnosis. Treatment consists in early detection and removal of emerging tumor formations.
The first signs also include epileptic seizures, they can be generalized or focal. Over time, signs of damage to the cerebellum appear, forming a symptom complex of cerebellar ataxia: static and dynamic discoordination, adiadochokinesis, hypermetria and asynergia, intentional tremor, myodystonia.
They manifest with cerebral symptoms (diffuse headaches, nausea without food intake, vomiting, tinnitus) due to increased intracranial pressure.
CNS damage. The most common source of primary symptoms are cerebellar cysts (cerebellar cysts).
Tumors affect the cerebellum and retina, intracerebral tumors of the hemispheres, neoplasms of subcortical structures and the medulla oblongata are less common, and tumors of the spinal cord and peripheral nerves are even less common. Due to the incomplete manifestation of genetic aberrations, some patients may have only one clinical sign of the disease.
As a result of insufficient oncosuppression, neoplasms grow, mainly angioreticulum and hemangioblastoma.
In another 20% of cases, Hippel-Lindau disease occurs due to new mutations. The aberrations affect the p25-26 region located on the 3rd chromosome, namely the VHL gene, which plays the role of a suppressor that suppresses the growth of neoplasms. To date, about 140 mutations of this gene are known.
Hippel-Lindau disease is a genetic pathology. In about 80% of cases, it is inherited in an autosomal dominant manner with incomplete gene penetrance.