A Review of Complicated Cataract in Retinitis Pigmentosa: Pathogenesis and Cataract Surgery

Abstract

Retinitis pigmentosa (RP) is a set of inherited retinal degenerative diseases that affect photoreceptor and retinal pigment epithelial cells (RPEs), possibly associated with some ocular complications, including cataract. The complicated cataract formation is most likely the result of RP-related inflammation response, and the most common morphology category is posterior subcapsular cataract (PSC). Despite the absence of curative pharmacologic treatment, phacoemulsification with intraocular lens implantation to deal with opacification in the lens is preferred due to the considerable visual outcomes. However, the incidence of intraocular and postoperative complications is higher in RP patients than those without, including intraoperative phototoxic retinal damage, posterior capsular opacification (PCO), capsular contraction syndrome (CCS), pseudophakic cystoid macular edema (PCME), increased postoperative intraocular pressure (IOP), and intraocular lens (IOL) dislocation. Hence, it needs much attention to surgery progress and close follow-up. In this review, we discuss the current understanding of RP patients with complicated cataracts from morphology to potential pathogenesis to cataract surgical procedure and provide a concise description and the recommended management of related surgery complications to broaden the knowledge and lower the latent risks to yield better clinical outcomes.

Effect of lens capsular tension ring on preventing capsular contraction syndrome in the surgery of retinitis pigmentosa combined with cataract: Retrospective case series

“…CCS is defined as an exaggerated reduction in anterior capsulectomy and capsular bag diameter after extracapsular cataract surgery 20 . The fibrotic reaction of residual lens epithelial cells associated with anterior capsule opacification and extracellular matrix (ECM) disorder might work together as CCS's pathogenesis in RP 21 . CCS may also continuously exacerbate into severer IOL dislocation leading to refractive changes and glare, followed by retinal and ciliary body detachment without intervention 21 …”

“…20 The fibrotic reaction of residual lens epithelial cells associated with anterior capsule opacification and extracellular matrix (ECM) disorder might work together as CCS's pathogenesis in RP. 21 CCS may also continuously exacerbate into severer IOL dislocation leading to refractive changes and glare, followed by retinal and ciliary body detachment without intervention. 21 In this study, four cases of CCS occurred and were treated with capsule loosening operation at 6 months after the cataract surgery.…”

“…21 CCS may also continuously exacerbate into severer IOL dislocation leading to refractive changes and glare, followed by retinal and ciliary body detachment without intervention. 21 In this study, four cases of CCS occurred and were treated with capsule loosening operation at 6 months after the cataract surgery. These four cases of CCS were only found during follow-up.…”

Commentary: Cataract surgery in retinitis pigmentosa

“…[ 2 ] The ongoing inflammatory process from rapid photoreceptor degeneration is implicated as the probable cause for both these conditions. [ 3 ] The most common reported morphology of cataract in RP is posterior subcapsular cataract (PSC) which often requires cataract surgery for visual rehabilitation. [ 3 4 ] However, the rate of both intraoperative and postoperative complication following cataract surgery in eyes with RP is high when compared to routine cases of cataract.…”

“…[ 3 ] The most common reported morphology of cataract in RP is posterior subcapsular cataract (PSC) which often requires cataract surgery for visual rehabilitation. [ 3 4 ] However, the rate of both intraoperative and postoperative complication following cataract surgery in eyes with RP is high when compared to routine cases of cataract. [ 4 ] The high rate of reported complication and risk of guarded visual outcome following cataract surgery due to the associated retinal pathology often result in apprehension when treating these cases.…”