Flap technique-assisted surgeries for advanced retinitis pigmentosa complicated with macular hole: a case report and literature review

Abstract

Background Full-thickness macular hole (FTMH) is a rare complication in retinitis pigmentosa (RP) patients and may increase intraoperative challenges. Furthermore, lens capsular flap transplantation and inverted internal limiting membrane (ILM) flap were reported to close complicated FTMH successfully. Here, we present a case of bilateral advanced RP complicated by a FTMH treated with a novel lens capsular flap transplantation and inverted internal limiting membrane flap. Case presentation A 46-year-old presented to our hospital with a complaint of progressively blurred vision and metamorphopsia in both eyes. Spectral-domain optical coherence tomography revealed a FTMH with retinoschisis in the right eye and another FTMH in the left eye. ILM peeling with inverted ILM flap technique was performed on the right eye and ILM peeling with anterior lens capsular flap technique was performed on the left eye. Post-operative follow-up showed successful closure of the FTMH and improved vision in both eyes. Conclusions In our present case, flap-assisted techniques for retinitis pigmentosa with macular hole result in excellent visual and anatomic outcomes.

Autosomal Dominant Retinitis Pigmentosa Secondary to TOPORS Mutations: A Report of a Novel Mutation and Clinical Findings

“…It is characterized by a defect in the foveal neurosensory retina that extends through all retinal layers and causes detrimental central vision loss. The pathogenesis of FTMH in RP patients is not well understood, but has been previously described to be related to chronic VMT, ERM formation, or retinal atrophy [ 13 , 14 ]. FTMH can be surgically repaired by PPV with ILM peeling and supplemental gas tamponade.…”

“…FTMH can be surgically repaired by PPV with ILM peeling and supplemental gas tamponade. Repair of macular holes in RP patient is rare and long-term outcomes are poorly understood [ 13 , 14 ]. As in the case of our patient (P7), we performed a successful repair of a FTMH surgery, which resulted in improved visual acuity and persistent primary surgical success given persistent closure of the macular hole over three years of follow up.…”

Macular abnormalities in retinitis pigmentosa. Management and treatment

“…Closure rates reach 90%, vision improvement has been observed in 62.5% of patients, and failure of hole closure is higher in RP cases with high myopia [23], chronic FTMH [24] and diabetic retinopathy [25]. There are documented cases of FTMH in patients with RP who had posterior staphyloma with significant choroidal and retinal atrophy despite normal eye length (23.24 mm and 23.43 mm) [26]. Surgical treatment of this type of FTMH is difficult due to poor retinal adhesion to the pigment epithelium within the staphyloma.…”

“…Vitreomacular traction syndrome (VMTS) in patients with RP has a prevalence of 0.6-27.3% [26]. There is evidence that patients with RP have vitreous degeneration, including vitreous gel collapse and posterior vitreous detachment, and that such lesions can cause macular complications related to vitreoretinal tractions [34].…”

“…In the case of FTMH, vitreoretinal surgery is the most effective treatment option, involving not only anatomical improvement of the retina after surgery, but also improved visual acuity [26].…”