Background and Clinical Significance: Biallelic pathogenic variants in the PCARE gene (photoreceptor cilium actin regulator), also known as C2orf71 (chromosome 2 open reading frame 71), are typically associated with retinitis pigmentosa type 54 (RP54) and, less frequently, with cone–rod dystrophy (CORD23). Case Presentation: A 52-year-old man presented with an eight-year history of progressive visual loss, without photophobia or nyctalopia. He underwent a comprehensive ophthalmological evaluation, including multimodal retinal imaging, automated perimetry, and full electrophysiological testing, in accordance with International Society for Clinical Electrophysiology of Vision (ISCEV)’s standards. Genetic testing was performed using next-generation sequencing (NGS) with an inherited retinal dystrophy gene panel, and findings were confirmed by Sanger sequencing. Clinical examination revealed bilateral macular atrophy with minimal foveal sparing and a central scotoma. Optical coherence tomography (OCT) showed disruption of the outer retinal layers and retinal pigment epithelium (RPE) abnormalities. Fundus autofluorescence (FAF) demonstrated central hypoautofluorescence surrounded by a hyperautofluorescent ring. Electrophysiological testing revealed severely reduced rod- and cone- mediated responses on full-field electroretinography (ERG), absent pattern ERG responses, and markedly reduced multifocal ERG responses, indicating widespread retinal dysfunction with significant macular involvement. Genetic analysis identified a homozygous pathogenic nonsense variant in PCARE [c.3289C>T; p.(Gln1097*)], confirming the diagnosis of an autosomal recessive inherited retinal dystrophy. Conclusions: Biallelic PCARE variants can cause late-onset severe retinal dystrophy, with predominant macular involvement and cone–rod dysfunction. Given its phenotypic overlap with other inherited retinal diseases, accurate diagnosis requires the integration of multimodal retinal imaging, electrophysiological testing, and comprehensive genetic analysis.

Purpose: To systematically review the available evidence on the indications, efficacy, timing, and complications of Goniopuncture (GP) of the Trabeculo-Descemet’s Membrane (TDM) following Non-Penetrating Deep Sclerectomy (NPDS), and to illustrate clinical presentations with three representative cases of iris incarceration. Methods: A structured literature search was conducted in PubMed, EMBASE, and the Cochrane Library (inception to December 2024) following PRISMA guidelines. Peer-reviewed studies reporting GP outcomes after NPDS with at least 12 months of follow-up were included. Quality assessment was performed using the Newcastle-Ottawa Scale for cohort studies. Results: Fifteen studies encompassing 1,837 eyes met inclusion criteria. The GP rate ranged from 4% to 67% across series. Intraocular Pressure (IOP) reduction after GP ranged from 30% to 54%, and reported success rates (IOP ≤21 mmHg or ≥20% reduction without additional medication) ranged from 58% to 93%. Iris incarceration at the TDM was the most frequent complication (7-13%). Heterogeneity across studies was attributable to differences in surgical technique, patient demographics, implant type, follow-up duration, and success definitions. Conclusion: GP is a safe and effective adjunctive procedure that substantially improves IOP control after NPDS. Optimal outcomes require appropriate patient selection, careful technique, and close gonioscopic surveillance. Further prospective, standardized studies are needed to define best-practice protocols.

We report the case of a 75-year-old woman presenting with acute angle-closure glaucoma in the left eye, refractory to maximal medical therapy. Fixed mydriasis prevented laser peripheral iridotomy. One week later, the patient underwent phacoemulsification with intraocular lens implantation combined with a Single-Pass Four-Throw (SFT) pupiloplasty in four quadrants. The procedure restored pupillary anatomy, deepened the anterior chamber and achieved a sustained Intraocular Pressure (IOP) of 10 mmHg without topical medication. At two-week follow-up, best-corrected visual acuity improved to 0.8 (decimal notation; approximately 20/25 Snellen), with an open angle and complete symptom resolution. This case illustrates the role of SFT pupiloplasty as an effective adjunct in the surgical management of complex acute angle-closure glaucoma when laser peripheral iridotomy is not feasible.

Purpose: To report the surgical management of late-onset Pseudophakic Bullous Keratopathy (PBK) in an eye with a Baikoff-type angle-supported Anterior Chamber Intraocular Lens (AC-IOL) with all three haptics synechiated to the peripheral iris stroma, in the context of a pre-existing traumatic superior iridodialysis. Methods: A 72-year-old male with a history of traumatic cataract and superior iridodialysis who had undergone extracapsular cataract extraction with implantation of a three-haptic Baikoff-type AC-IOL approximately 30 years prior presented in 2021 with decreased visual acuity. Best-Corrected Visual Acuity (BCVA) was Hand Motions (HM) in the affected eye. Diffuse corneal edema (central corneal thickness 838 µm) with bullous keratopathy unresponsive to medical therapy was diagnosed. Slit-lamp examination revealed synechiae of all three IOL haptics to the peripheral iris stroma, precluding safe explantation. Descemet Stripping Automated Endothelial Keratoplasty (DSAEK) was performed retaining the AC-IOL in-situ. Results: Intraoperative air tamponade was achieved, but the air bubble migrated to the posterior chamber through the superior iridodialysis in the immediate postoperative period. Despite the absence of sustained anterior chamber air tamponade, the endothelial graft remained adherent and bullous keratopathy resolved. At the final follow-up visit (6 months postoperatively), BCVA had improved to 0.1 (20/200), Intraocular Pressure (IOP) was 14 mmHg and progressive corneal clearing was documented on biomicroscopic examination and Anterior Segment Optical Coherence Tomography (AS-OCT). Conclusion: DSAEK may be considered a viable surgical option for PBK secondary to retained AC-IOLs when explantation carries prohibitive risk. The presence of iridodialysis, though complicating air tamponade, does not necessarily preclude successful graft adhesion. The inherent rigidity of the DSAEK lenticule may confer an advantage over DMEK in anatomically complex anterior segments.