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Retinoschisis

Presentation

Acquired retinoschisis is most commonly peripheral, unilateral, asymptomatic, and present in an older demographic. XLRS is usually bilateral, central, symptomatic, and present in a younger male demographic (Table 1). [journal.opted.org]

Present Lattice in elevated retina Unlikely Suggestive Retinal pigment epithelium Normal (unless associated with retinal detachment, current or regressed) Acute: normal Chronic: atrophy and demarcation lines may be present Optical coherence tomography [entokey.com]

Rarely, white flecks or areas of schisis in the peripheral retina can be present. [institut-vision.org]

CONCLUSION: We present a case of atypical retinoschisis with clinical findings of retinitis pigmentosa.tinoschisis with clinical findings of retinitis pigmentosa. [cegat.de]

Peripheral bullous retinoschisis (seen in the current patient’s left eye) is present in approximately 50% of cases at presentation and may flatten spontaneously as the disease progresses. 1 The current case suggests that the likely mechanism for this [healio.com]

Entire Body System

  • Asymptomatic

    Management Observation for asymptomatic cases. Treatment is only indicated in symptomatic cases or cases with progressive retinal detachment. Back to top [vagelos.columbia.edu]

    Clinical features The disease is asymptomatic in majority of cases. Advanced cases may present a large peripheral visual field defect corresponding to the area of retinoschisis. [institut-vision.org]

    […] usually does not extend beyond the borders of the retinoschisis, schisis detachments are also usually asymptomatic. [entokey.com]

    Senile retinoschisis may not require medical treatment as it is asymptomatic. However, if it is causing you trouble with vision, you should consult an eye specialist to explore a suitable therapy option. [retinasocal.com]

  • Collapse

    First, the primary surgical goal is to close the outer wall breaks; treating inner wall breaks and collapsing the schisis cavity is optional. 14 If the schisis cavity is collapsed intraoperatively, it will likely recur postoperatively. [retinatoday.com]

    The inner layer collapses near the tip of the suction tube as the thick fluid is drained out of the tube, but the other area of schisis remains elevated, making the complete collapse of the inner layer very difficult. [doctorlib.info]

    Retinal detachment normally collapses under the sclera depression area while retinoschisis moves according to the retinal area being depressed. [myvision.org]

    This theory is also supported by the fact that transscleral cryopexy applied to the entire area of retinoschisis may cause irreversible damage to the Müller cells and therefore may result in a collapse of the schisis cavity. [slideplayer.org]

  • Surgical Procedure

    Surgical procedures may be a last resort of treatment. Most persons with juvenile X-linked retinoschisis usually retain functional vision. [rarediseases.org]

Skin

  • Blister

    It appears as a “water blister” on the retina. The surface of the lesion may show whitened blood vessels, or areas of small whitish flecks called Gunn’s dots. [flatbushoptical.com]

    Any form may be associated with the development of blisters (cysts) in the retina. In more advanced stages, Retinoschisis can lead to a serious vision loss. Female carriers rarely have vision problems. [canadiandisabilitybenefits.ca]

Eyes

  • Night Blindness

    Patients also typically have blind spots, poor peripheral vision, night blindness, and poor color perception. Many individuals with cone-rod dystrophy are considered legally blind by the time they reach early adulthood. [vrcny.com]

    However, a similar ERG pattern can be seen in other hereditary and acquired retinal disorders, notably, congenital stationary night blindness. [institut-vision.org]

    Nyctalopia/Night Blindness Cone Dystrophy Choroideremia Retinopathy of Prematurity Uveitis & Ocular Inflammation Retinoblastoma [retinasocal.com]

    Diagnostic clinical findings of a new syndrome with night blindness, maculopathy, and enhanced S cone sensitivity. Am J Ophthalmol 1990;110:124–34. [PubMed] [Google Scholar] 4. Lewis RA, Lee GB, Martonyi CL, et al. Familial foveal retinoschisis. [ncbi.nlm.nih.gov]

  • Photopsia

    The patient often complains of photopsia and floaters. A harmless self limiting retinal detachment may follow leakage of the cavity through a hole in the outer wall. No treatment is indicated. [gpnotebook.com]

    He denied photopsias and had no family history of retinal detachments. [webeye.ophth.uiowa.edu]

  • Vitreous Floaters

    Since the vessels in the inner layer are above the retinal surface like vitreous floaters, they can block the exiting light and produce a shadow effect ‘dark vessels’. [optos.com]

  • Esotropia

    On examination, he was found to have 30 Δ of esotropia and a preference for the right eye. Anterior segment examination was unremarkable. [ncbi.nlm.nih.gov]

Neurologic

  • Nystagmus

    Strabismus and nystagmus may occur. Rarely, a peripheral retinoschisis occurs in retrolental fibroplasia or diabetic retinopathy. [gpnotebook.com]

    This form of the disease is called bullous, usually seen in young children and is combined with a squint and nystagmus. Retinal cysts can spontaneously subside. [canadiandisabilitybenefits.ca]

    People living with X-linked Retinoschisis can also experience farsightedness (hyperopia), eyes looking in different directions (strabismus) and in more severe cases, involuntary eye movements (nystagmus). [retina-international.org]

    CXLRS is typically diagnosed in males between the ages of 3 months and school age with symptoms that include crossed eyes (strabismus), abnormal eye movements (nystagmus) and “lazy eye” (amblyopia). [rsnallc.com]

    In severe cases, nystagmus may also be observed. Severe cases involve full-thickness retinal detachment that leads to impaired vision or blindness. [orpha.net]

Treatment

Although treatment with oral CAIs has been shown to produce equal visual acuity outcomes, it is associated with a significantly greater side-effect profile when compared with topical treatment.8,17,19 Acute onset macular edema is more responsive to treatment [journal.opted.org]

Fortunately, this complication is rare, occurring in only 0.05% of patients with retinoschisis. 1 Treatment of progressive RRD associated with retinoschisis follows the same principles as treatment of any RRD, but with a few special considerations. [retinatoday.com]

What are the Treatment Options for Retinoschisis? Senile retinoschisis may not require medical treatment as it is asymptomatic. [retinasocal.com]

Treatment Treatment Options: There is presently no effective treatment for the disorder, but decreased vision later in life can be aided with low vision aids. Cases with posterior subcapsular cataract can be treated with cataract extraction. [disorders.eyes.arizona.edu]

There is no effective treatment for X-linked Retinoschisis at this time. [montanaretinaconsultants.com]

Prognosis

Patient’s perspective Medical counselling helped me and my family members to have better understanding of the disease nature, its inheritance pattern and prognosis. [casereports.bmj.com]

Prognosis Visual prognosis is highly variable, depending on the XLRS phenotypic variability. With prompt identification and management of the disease, the vision could be preserved adequately. [institut-vision.org]

There are many useful considerations and tools for evaluation and monitoring that can be used to determine the aetiology and prognosis of these retinal findings. [onlinelibrary.wiley.com]

This does not change the prognosis. If there are both inner and outer layer holes, then liquid vitreous can work its way under both retinal layers and cause a retinal detachment. Fortunately double layer holes occur very rarely. [retinalmd.com]

Treatment and Prognosis: The foveal and peripheral retinal splitting that occurs in schisis cannot be treated, but secondary complications may require treatment. [pediatricrrf.org]

Etiology

The etiology is most likely vitreous traction that physically pulls the retina apart. [optos.com]

[…] and Pathogenesis Although the exact etiology is unknown, there is no evidence to suggest a genetic, vascular, or nutritional etiologic component[3]. [webeye.ophth.uiowa.edu]

Etiology and pathogenesis The etiology of degenerative retinoschisis is poorly understood. Genetic, vascular, nutritional, or tractional mechanisms do not seem to be implicated in the pathogenesis of the condition. [institut-vision.org]

Epidemiology

Epidemiology, onset & clinical features Epidemiology Degenerative retinoschisis affects about 5-7% of the adult population. Occurrence in up to 22% of individuals older than 40 years has been reported. Both genders are equally affected. [institut-vision.org]

Summary Epidemiology X-linked retinoschisis prevalence is estimated to range between 1/5,000-1/25,000 males worldwide. Clinical description XLRS is a symmetrical bilateral macular disorder with onset in the first decade of life. [orpha.net]

Epidemiology Reported prevalence of disease varies widely due to population-based RS studies, difficulty of observing lesions due to its peripheral location, and the fact that most patients are asymptomatic. [webeye.ophth.uiowa.edu]

Pathophysiology

This experience reinforces the traction hypothesis of Shukla et al., Greven et al., and Shanmugam et al. [4–6] on the macula in pathophysiology. [hindawi.com]

Pathophysiology Using positional cloning, Sauer and associates identified XLRS1, the gene responsible for X-linked juvenile retinoschisis. [1] XLRS1 is located on band Xp22.2. [emedicine.medscape.com]

Prevention

Prevention How can I prevent retinoschisis? You can’t prevent retinoschisis. There’s no known cause of acquired retinoschisis. [my.clevelandclinic.org]

Complications Prompt diagnosis and management of retinoschisis are essential to prevent complications related to the condition, which can threaten a person’s vision. [verywellhealth.com]

For treatment, a barrage argon laser photocoagulation was performed to surround the retinoschisis and prevent further extension (Figure 1). [cureus.com]

It has never been shown that prophylactic treatment statistically preserves vision or prevents complications in cases of degenerative retinoschisis. [flatbushoptical.com]

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