To report outcomes of pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling for extensive macular schisis in choroideremia and provide surgical insights. Retrospective case series of five eyes from four patients with choroideremia and extensive macular schisis who underwent PPV with ILM peeling at a single tertiary center between 2011 and 2025. Data collected included pre- and post-operative best-corrected visual acuity (BCVA), surgical technique, spectral-domain optical coherence tomography (SD-OCT) imaging, intraoperative findings, and post-operative complications. All five eyes showed complete resolution of macular schisis on SD-OCT without foveal retinal pigment epithelium atrophy. BCVA improved in all cases. Intraoperative challenges included retinal fragility, peripheral schisis-like changes, and difficulty with ILM visualization and adherence. No iatrogenic retinal breaks or detachments occurred. Vitrectomy for extensive macular schisis in choroideremia can yield favourable anatomical and functional outcomes but requires tailored techniques to address retinal fragility and minimize iatrogenic trauma. Extensive macular schisis is a common end stage feature of choroideremia that is frequently missed as patients are no longer being followed up. The schisis however, can detach the small, remaining island of vision leading to potentially treatable vision loss. Due to retinal fragility, surgical intervention is traditionally considered high risk and of uncertain benefit. Pars plana vitrectomy with internal limiting membrane peeling and tailored surgical strategies can achieve favourable anatomical and functional outcomes in choroideremia patients.
Elsayed et al. (Wed,) studied this question.