Vitreoretinal surgery without schisis cavity excision for the management of juvenile X linked retinoschisis

Abstract

Juvenile X linked retinoschisis (XLRS) is a congenital X linked recessive retinal disorder characterised by cystic maculopathy and peripheral schisis. This study presents the case of an 8-month-old boy with a documented positive family history of XLRS, with a large retinoschisis cavity affecting the macula, first in the left eye and 1 year later in the right eye. The patient underwent pars plana vitrectomy in both eyes using 23-G instruments, posterior hyaloid dissection, a small retinotomy, fluid drainage with a 42-G cannula, infrared diode laser and silicone oil as internal tamponade. The anatomical and functional outcomes at 3 years following the first surgery are described. To the authors' knowledge, there is no previously reported experience with this technique in patients with XLRS.

Surgery for Pediatric Vitreoretinal Disorders

“…117,120 In a current series of cases, the main indication for unilateral vitrectomy in a 7 year old and bilaterally in a 17-yearold was foveal retinoschisis, which was associated with peripheral retinoschisis or peripheral inner holes. 117,120 In a current series of cases, the main indication for unilateral vitrectomy in a 7 year old and bilaterally in a 17-yearold was foveal retinoschisis, which was associated with peripheral retinoschisis or peripheral inner holes.…”

“…31,117,118 García-Arumí et al 117 performed posterior hyaloid dissection after injection of 0.05 mL of diluted preservative-free triamcinolone acetonide in an 8-month old child. Norrie disease is believed to result from mutations in the 28-kb Norrie disease protein (NDP) gene, located on chromosome Xp11.3.…”

“…The disease is characterized by a retinal Recent studies advocate vitrectomy for a bullous schisis cavity with or without hemorrhage in children with successful surgically induced posterior vitreous detachment. 117 Photocoagulation should applied to the border of the attached retina and the schisis cavity to prevent the progression of retinal splitting. Trese 32 describe the adjuvant use of autologous plasmin for hyaloid separation.…”

Surgical treatment for complications of congenital retinoschisis

“…The surgical indications include rhegmatogenous or tractional retinal detachment, dense vitreous hemorrhage, peripheral schisis cavities involving the macula and macular pucker [20,21] . Many studies showed that vitreoretinal surgery in the treatment of complications of congenital retinoschisis is safe and effective; the visual function in most patients is improved or stabilized after the surgical treatment [21][22][23][24][25] .…”

Netzhautablösung im Kindesalter

“…Einige Studien beschreiben bei bullöser Schisisblase mit oder ohne Blutung die Durchführung einer Vitrektomie mit erfolgreicher Abhebung der hinteren Glaskörpergrenzmembran (HGM) und damit postoperativ eine Besserung der anatomischen Situation [41][42][43]. Die funktionellen Ergebnisse nach Vitrektomie sind jedoch limitiert, die Sehschärfe stieg bei der Mehrzahl der operierten Augen an, die postoperativ erreichte Sehschärfe betrug durchschnittlich 20/55 [42][43][44]. Postoperativ sieht man eine foveale Depression mit kleinen zystischen Räumen in der Netzhaut, allerdings persistierten die negativen b-Wellen im ERG (Elektroretinogramm) nach klinischer Rückbildung der Schisis, da sich die intraretinalen Nervenfasern trotz Repositionierung nicht neu vernetzen können [43].…”