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Retinoschisis (Retinoschisis, Juvenile)

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A vitreoretinal dystrophy characterized by splitting of the neuroretinal layers. It occurs in two forms: degenerative retinoschisis and X chromosome-linked juvenile retinoschisis.

Also Known As:

Retinoschisis, Juvenile; Retinoschisis, X-Linked; Juvenile Retinoschisis; Retinoschisis, Juvenile, X Chromosome-Linked; Degenerative Retinoschises; Degenerative Retinoschisis; Juvenile Retinoschises; Juvenile Retinoschises, X-Linked; Juvenile Retinoschisis, X-Linked; Juvenile, Retinoschisis X-Linked; Juveniles, Retinoschisis X-Linked; Retinoschises; Retinoschises, Degenerative; Retinoschises, Juvenile; Retinoschises, X-Linked; Retinoschises, X-Linked Juvenile; Retinoschisis X Linked Juvenile; Retinoschisis X-Linked Juveniles; Retinoschisis, X Linked; Retinoschisis, X-Linked Juvenile; X Linked Juvenile Retinoschisis; X Linked Retinoschisis; X-Linked Juvenile Retinoschises; X-Linked Juvenile, Retinoschisis; X-Linked Juveniles, Retinoschisis; X-Linked Retinoschises; Retinoschisis X-Linked Juvenile; Retinoschisis, Degenerative; Retinoschisis, Juvenile, X-Linked; X-Linked Juvenile Retinoschisis; X-Linked Retinoschisis

Networked: 344 relevant articles (16 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Eye Diseases: 2132
- Retinal Diseases: 1278
  - Retinal Degeneration: 2246
    - Retinoschisis: 344
      - Retinoschisis of Fovea: 15
      - Retinoschisis autosomal dominant

Related Diseases

1.	Retinal Detachment (Retinal Detachments)
2.	Vitreous Hemorrhage
3.	Hemorrhage
4.	Myopia
5.	Vitreous Detachment (Posterior Vitreous Detachment)

Experts

1.	Sieving, Paul A: 9 articles (08/2015 - 09/2004)
2.	Weber, Bernhard H F: 9 articles (12/2014 - 04/2002)
3.	Molday, Robert S: 8 articles (05/2012 - 04/2002)
4.	Fishman, Gerald A: 7 articles (06/2014 - 09/2006)
5.	Hirakata, Akito: 6 articles (10/2015 - 09/2005)
6.	Bush, Ronald A: 5 articles (08/2015 - 09/2004)
7.	Inoue, Makoto: 4 articles (10/2015 - 11/2008)
8.	Ohno-Matsui, Kyoko: 4 articles (10/2015 - 09/2006)
9.	Zeng, Yong: 4 articles (07/2015 - 09/2004)
10.	Vijayasarathy, Camasamudram: 4 articles (07/2015 - 09/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Retinoschisis:

1.	Retinaldehyde (Retinal)IBA 05/01/2003 - "The results showed statistically significant difference of average response densities and latencies in six ring retinal regions between the normal control and retinoschisis. " 06/01/2014 - "We compared the efficacy of rescue mediated by retinoschisin secretion from these specific subtypes of retinal cells in the Rs1h-/- mouse model of retinoschisis. " 06/01/2009 - "The purpose of this study was to investigate Fourier domain optical coherence tomography (FD OCT) as a noninvasive tool for retinal imaging in the Rs1h-knockout mouse (model for X-linked juvenile retinoschisis). " 03/01/2004 - "X-linked retinoschisis is an important condition to study, both to improve the clinical management of this disorder, and to better understand retinal function and development. " 11/01/2003 - "To study local retinal cone function in patients with X-linked retinoschisis (XLRS) by multifocal ERGs (mfERGs). "
2.	dorzolamide (Trusopt)FDA LinkGeneric 02/01/2010 - "To determine the efficacy of sustained topical therapy with dorzolamide hydrochloride, 2%, on visual acuity and cystic macular lesions in patients with juvenile X-linked retinoschisis (XLRS). " 02/01/2010 - "Efficacy of sustained topical dorzolamide therapy for cystic macular lesions in patients with X-linked retinoschisis." 03/01/2008 - "There have been no therapeutic interventions known to be effective for patients with X-linked juvenile retinoschisis, but some studies are trying to determine the importance of dorzolamide for the treatment of foveal lesions in this disease. " 06/01/2014 - "Resolution of mid-peripheral schisis in x-linked retinoschisis with the use of dorzolamide." 07/01/2011 - "To correlate the response of topical dorzolamide (Trusopt; Merck) in patients with X-linked retinoschisis (XLRS) with genotype. "
3.	ranibizumab (Lucentis)FDA Link 01/01/2013 - "The CNV responded well to intravitreal ranibizumab therapy, and visual acuity in the left eye improved and stabilized; however, retinoschisis due to fluctuations in intraretinal fluid persisted. " 01/01/2014 - "This study aimed to explore the incidence and features of retinoschisis after intravitreal ranibizumab injection for myopic choroidal neovascularization. " 01/01/2014 - "No new onset of retinoschisis took place in group 2 and group 3. Intravitreal ranibizumab injection may be a risk factor for aggravation of retinoschisis in eyes with preexisted retinoschisis and epiretinal membrane." 01/01/2014 - "Eleven eyes that had retinoschisis and epiretinal membrane were in group 1, six eyes that had simple epiretinal membrane were in group 2, and 66 eyes that had neither retinoschisis nor epiretinal membrane were in group 3. Six contralateral eyes in group 1 which had retinoschisis and epiretinal membrane but were not treated with intravitreal ranibizumab injection were set as the control group. " 01/01/2014 - "Intravitreal ranibizumab injection is effecitve on treating myopic CNVs, but it could be a risk factor for developing more severe retinoschisis in eyes with preexisted retinoschisis and epiretinal membrane. "
4.	Acetazolamide (Diamox)FDA LinkGeneric 08/01/2015 - "To report the results of an acetazolamide (Diamox(®)) treatment regimen in a genetically confirmed case of X-linked juvenile retinoschisis (XLRS). " 08/01/2015 - "Rapid resolution of retinoschisis with acetazolamide." 03/01/2014 - "Acetazolamide in retinoschisis: a prospective study." 02/01/2009 - "Treatment of adult-onset acute macular retinoschisis in enhanced s-cone syndrome with oral acetazolamide." 04/01/2007 - "Acetazolamide in the treatment of X-linked retinoschisis maculopathy."
5.	Carbonic Anhydrases (Carbonic Anhydrase)IBA 08/01/2014 - "A combination of topical and systemic carbonic anhydrase in the treatment of chromosome X-linked retinoschisis." 09/01/2006 - "To determine the value of a topical carbonic anhydrase inhibitor for the treatment of foveal lesions in patients with X-linked retinoschisis (XLRS). " 02/01/2009 - "To report on the efficacy of the oral carbonic anhydrase inhibitor (CAI) acetazolamide in treating macular retinoschisis (RS) in the rare vitreoretinal dystrophy best known as the enhanced S-cone syndrome (ESCS). "
6.	tranilast (N 5')IBA 06/01/2011 - "The clinical efficacy of surgical treatments was retrospectively analyzed in 10 patients with congenital retinoschisis (10 eyes) complicated with rhegmatogenous retinal detachment (n=5), vitreous hemorrhage (n=2) and macula- involving schisis (n=1). " 11/01/2006 - "Clinical diagnoses included inherited retinal dystrophies [X-linked congenital retinoschisis (XRS) (n=17), congenital stationary night blindness (CSNB) (n=6), retinitis pigmentosa (RP) (n=6), cone (-rod) dystrophy (n=5), choroideremia (n=1), Müller cell sheen dystrophy (MCSD) (n=1)] and acquired retinopathies (melanoma-associated retinopathy (MAR) (n=1), vigabatrin retinotoxicity (n=1)). "
7.	Fluorescein (Funduscein)FDA LinkGeneric 09/01/2005 - "The two brothers with ophthalmic disease were evaluated with color fundus photography, fluorescein angiography, optical coherence tomography (OCT), molecular genetic study (Group VI of Retinoschisis Consortium), pattern visual evoked potential (PVEP), and full field ERG. " 01/01/1970 - "Fluorescein angiographic studies on x-chromosomal retinoschisis." 10/01/2009 - "We studied in detail by means of fluorescein angiography (FLA), microperimetry (fundus related perimetry), and ocular coherence tomography (OCT) the case of a 30-year-old woman who had an optic pit with secondary retinoschisis in her right eye." 09/01/2005 - "In juvenile X-linked retinoschisis (XLRS), fluorescein angiography is usually unremarkable and contributes poorly to the diagnosis. " 09/01/1991 - "Without therapy we saw a slowly progressive flat central retinoschisis with a serous macular exsudation in fluorescein-angiography. "
8.	DNA ProbesIBA 09/01/1987 - "A linkage study was carried out in nine families with 24 males affected by X-linked recessive retinoschisis (RS), using three polymorphic DNA probes from the distal segment of Xp. " 12/01/1994 - "Linkage analysis was carried out in seven X linked juvenile retinoschisis (XLRS) families using four DNA probes and four CA repeat polymorphisms from the Xp22 region. " 10/01/1988 - "Use of linked DNA probes for carrier detection and diagnosis of X-linked juvenile retinoschisis." 12/01/1990 - "In this report a family with X-linked retinoschisis is described in which segregation analysis with DNA probes strongly suggests that the deceased grandfather, who was said to have had good vision, had been affected by juvenile retinoschisis."
9.	Prostaglandins D (PGD)IBA 06/01/2008 - "The aim of this study was to perform preimplantation genetic diagnosis (PGD) for X-linked retinoschisis using multiple displacement amplification (MDA) for whole genome amplification and linked markers to the RS1 gene. " 06/01/2008 - "As a result, the development of an MDA-PGD protocol for X-linked retinoschisis allowed for the diagnosis of 20 embryos in the four PGD cycles performed. " 06/01/2008 - "MDA products were used for polymerase chain reaction analysis of two polymorphic markers flanking the RS1 gene and a new X/Y marker, X22, to sex embryos in an X-linked retinoschisis PGD programme. "
10.	congenital stationary Night blindnessIBA 02/01/2012 - "Overall in this group of patients, the most common diagnoses associated with a negative ERG were congenital stationary night blindness (CSNB, n = 29) and X-linked retinoschisis (XLRS, n = 7). " 09/01/2003 - "Abnormalities of the scotopic threshold response correlated with gene mutation in X-linked retinoschisis and congenital stationary night blindness." 07/01/1993 - "Differential diagnosis between these unusual cases of cone dystrophies and X linked retinoschisis or congenital stationary night blindness was possible with funduscopy, adaptometry, and evaluation of progression, but not with the electroretinogram. " 07/01/1993 - "These patients are compared with others who characteristically have negative electroretinograms-one patient with incomplete congenital stationary night blindness and another with X linked congenital retinoschisis. " 04/01/1990 - "A male preponderance was observed for juvenile retinoschisis (P less than 0.005) and congenital stationary night blindness (P less than 0.05), and a female dominance in the groups of patients with 'functional' symptoms (P less than 0.01). "

Therapies and Procedures

1.	Vitrectomy 03/01/2008 - "Pars plana vitrectomy without ILM peeling is effective for treating macular retinoschisis and foveal detachment in highly myopic eyes with posterior staphyloma. " 09/01/2005 - "After vitrectomy, OCT showed a marked fluid resolution in the retinoschisis, and the patient's vision improved rapidly. " 09/01/2015 - "The retinoschisis in the RNFL resolved immediately after the vitrectomy, and the BCVA improved significantly (p = 0.004). " 11/01/2013 - "These results indicate that vitrectomy with ILM peeling can lead to improvement in vision in patients with macular retinoschisis or foveal detachment with visual impairments." 01/01/2006 - "To evaluate the efficacy of vitrectomy for posterior retinoschisis (RS) or foveal detachment (FD) associated with posterior staphyloma in myopic eyes. "
2.	Vitreoretinal Surgery 06/01/2011 - "It was concluded that vitreoretinal surgery in the treatment of complications of congenital retinoschisis is safe and effective, and helps improve and maintain the visual function." 06/01/2011 - "This study examined the clinical features of complications of congenital retinoschisis and the clinical efficacy of vitreoretinal surgery in the treatment of these complications. " 03/01/1998 - "Vitreoretinal surgery is often helpful in stabilizing or improving visual function in patients with posterior segment complications from X-linked retinoschisis." 11/01/2008 - "Vitreoretinal surgery without schisis cavity excision for the management of juvenile X linked retinoschisis." 03/01/1998 - "To assess the outcomes of vitreoretinal surgery in the treatment of vision-threatening posterior segment complications of X-linked retinoschisis. "
3.	Light Coagulation 09/01/2002 - "To provide a detailed description of the clinical features that are considered forerunners of symptomatic complications in asymptomatic degenerative retinoschisis, and to show that in selected cases at this stage prophylactic photocoagulation may be a better choice than mere observation. " 12/01/2001 - "Congenital retinoschisis: successful collapse with photocoagulation." 07/01/1990 - "Photocoagulation and cryopexy were applied in 5 patients with retinoschisis; in 3 they were connected with a temporary invagination of the sclera by means of a Lincoff-Kreissig balloon. " 02/01/1990 - "After analysis of spontaneous evolution of the untreated eyes and review of literature, the authors conclude that preventive photocoagulation in bullous retinoschisis is not advised, because of spontaneous regression with age and the possibility of complications. " 02/01/1990 - "[Photocoagulation in sex-linked heredofamilial retinoschisis]."
4.	Traction 03/01/2010 - "Release of traction exerted by a shrinking membrane is a surgical option to promote regression of retinoschisis and improvement in visual field and vision in schisis-membrane cases." 09/01/2015 - "A 53-year-old woman with macular and diffuse retinoschisis complicated by presumed vitreomacular traction underwent unilateral intravitreal ocriplasmin injection. " 09/01/2015 - "We suggest that the traction on the structurally fragile RNFL contributed to the retinoschisis." 11/01/2013 - "A complete resolution of macular retinoschisis can occur after a release of traction on the retina. " 11/01/2013 - "The natural course of myopic traction maculopathy varies, and eyes with more extensive macular retinoschisis tend to progress more commonly. "
5.	Lasers (Laser) 01/01/2012 - "Scanning laser ophthalmoscope retro-mode imaging of foveal schisis in eyes with X-linked retinoschisis." 11/01/2010 - "[Alternative of laser treatment optimization in retinoschisis]." 04/01/2010 - "To determine the characteristics of a macular retinoschisis using noninvasive retromode imaging by a new, commercial confocal scanning laser ophthalmoscope (F10; Nidek, Aichi, Japan) and to compare the findings with those obtained by conventional optical coherence tomography (OCT). " 11/01/2003 - "The laser test was positive for retinoschisis in 40 of 41 eyes. " 11/01/2003 - "To describe a modified laser technique for identification of bullous retinoschisis. "