Purpose: To present a series of 4 patients from the Ohio Amish or Mennonite populations with isolated ectopia lentis. Methods: A case series was evaluated. Results: Four cases with bilateral lens subluxations were diagnosed with a homozygous c.767_786del pathogenic variant in ADAMTSL4. Their ages ranged from 2 to 22 years. Three cases were symptomatic and were managed surgically with lensectomy, vitrectomy, and endolaser photocoagulation with or without secondary intraocular lens (IOL) implantation. One asymptomatic patient was observed. The postoperative visual acuity ranged from 20/20 to 20/60 in nonamblyopic eyes. Conclusions: The pathogenic homozygous c.767_786del variant in ADAMTSL4 may be a cause of bilateral isolated ectopia lentis in the Ohio Amish and Mennonite populations, likely as a result of a founder effect. Vitrectomy and lens extraction with or without secondary IOL implantation may lead to good visual outcomes. There were no cases of retinal detachment.

OBJECTIVE: To study the clinical presentations, visual, and refractive profiles of children with congenital ectopia lentis in a large cohort of patients from a tertiary eye care network in India.
METHODS: A retrospective review of electronic medical records from December 2012 to December 2020 was conducted. Two hundred and ninety-seven consecutive children ≤18 years of age at presentation were identified and analyzed for demographic details, patient distribution, lens subluxation, visual, and refractive profiles before and after the interventions.
RESULTS: Five hundred and ninety-four eyes of 297 (male 56%; n = 166) patients were analyzed. The mean age at presentation was 8.74 ± 3.89. Best-corrected visual acuity (BCVA) at presentation ranged from 0.3 logMAR to 3.5 logMAR; (Snellen: 6/9 - close to face [CF]) (mean 0.89 ± 0.68). High myopia (n = 201; 33.83%) and mild astigmatism (n = 340; 57.23%) were more frequent. Temporal (n = 108; 18.18%) subluxation was most common followed by superior. Lensectomy with limited vitrectomy was performed in 243 eyes of 127 patients (40.90%). Median preoperative BCVA was 1.0 (range: 0.3-3.5 logMAR; 20/40 - CF). Median postoperative BCVA was 0.5 logMAR (6/18) in the pseudophakic group and 0.6 logMAR (6/24) in the aphakic group. Spherical equivalent in myopic children reduced from -12.06 ± 6.84D to -1.57D (-0.25D to - 5.5D) in the pseudophakic group and +9.3D (+5.5D to 15.5D) in the aphakic group.
CONCLUSIONS: This study is a large cohort of children presenting with ectopia lentis. Following intervention, an improvement in the median BCVA and refractive correction was noted in the entire cohort.

UNASSIGNED: To present a case involving a rarely seen prototype posterior chamber phakic IOL (PC-pIOL) in a highly myopic patient with bilateral cataract.
UNASSIGNED: A 64-year-old male presented to our clinic with poor vision in both eyes. Clinical examination revealed bilateral mature cataract, phacodonesis as well as a PC-pIOL implanted 35 years ago to address his high myopia. The visual acuity (VA) was 20/200 in the right eye and no light perception in the left eye. PC-pIOL extraction as well as 23G pars plana vitrectomy (PPV) and fragmentation surgery was scheduled for the right eye. The left eye was treated conservatively. Successful extraction of the PC-pIOL was performed while it was easy to remove. It was a bow-tie shaped lens with a collar-stud-like button in the middle which extended anteriorly into the anterior chamber through the pupil. PPV with lens fragmentation was successful and the patient was left aphakic in order to avoid the placement of a zero diopter IOL. Final best corrected VA was 20/25 one month post-surgery.
UNASSIGNED: Removal of this rarely seen pIOL was performed without difficulty while excellent VA was achieved. Aphakia following complete vitrectomy represented a viable option in this case. Furthermore, we highlight the clinical manifestations associated with this IOL more than three decades after implantation.

UNASSIGNED: Non-traumatic ectopia lentis is a rare ocular disorder usually associated with syndromes like Marfans\'s syndrome, Weil-Marchesani and Homocystinuria. Ectopia lentis can lead to profound visual loss from refractive errors, glaucoma as well as retinal detachment if left unattended.
UNASSIGNED: The aim of this study was to describe the clinical profile of patients with non-traumatic ectopia lentis in a paediatric ophthalmology clinic in Ibadan, Nigeria.
UNASSIGNED: The clinical records of children ≤16 years diagnosed with non- traumatic ectopia lentis at the Paediatric Ophthalmology Clinic, University College Hospital Ibadan, from May 1, 2015 to Dec 31, 2019 were retrospectively reviewed. Information on demography, family history, visual acuity (VA), mean refractive error (spherical equivalent), and management was retrieved.
UNASSIGNED: Clinical records of 25 patients were reviewed. The mean age was 8.9 (±3.41) years with a range of 2-15 years. Sixteen (64%) patients were males. All the patients had bilateral involvement. A positive family history of ectopia lentis was elicited in 6 (24%) patients. Thirteen (52%) patients had a Marfanoid habitus and superior displacement of the lens was observed in 26 (52%) eyes. The presenting visual acuity was <6/60 in 22 (44%) eyes. The spherical equivalent of the refractive errors ranged from -20.00DS to +13.25DS. Twenty (40%) eyes underwent surgery within the period of the study and the best corrected postoperative visual acuity improved by 2 or more lines in 12 (60%) of operated eyes.
UNASSIGNED: Severe visual morbidity was common in this cohort of patients with ectopia lentis in our practice. Treatment provided some improvement in vision which highlights the need to encourage early presentation for care. Detailed family history is important as a few of the patients were diagnosed following acceptance of our invitation to siblings for ophthalmic evaluation.

OBJECTIVE: The aim of this study was to retrospectively evaluate vision outcomes of dogs with primary angle closure glaucoma (PACG) that underwent Ahmed valve gonioimplantation (AVG) followed by lensectomy and endolaser cyclophotocoagulation (ECP) with or without bleb resection after AVG failure.
METHODS: Medical records from 2008 to April 2022 were reviewed. All patients selected had an AVG performed first. Following valve failure, lensectomy-ECP was performed in addition to bleb resection as indicated. Additional ECP and AVG replacement surgeries were performed as deemed medically necessary. Main outcomes evaluated included vision preservation, average IOP reduction, and the number of glaucoma medications (both oral and topical) at 6, 12, 24, and 36 months.
RESULTS: Fourteen eyes from 13 patients were included in the study. Females were predominant (n = 9) compared with males (n = 4). Mean age at initial glaucoma diagnosis was 6.82 years. Following AVG and lensectomy-ECP, vision was preserved in 93%, 84%, 60%, and 48% of eyes at 1, 2, 3, and 4 years respectively. Following lensectomy-ECP, there was a significant reduction in mean IOP of 9.64 mmHg (p = .015) and 9.71 mmHg (p = .016) at 2 and 6 months, respectively. There was a reduction in mean IOP of 2.45, 7.25, and 12.25 mmHg at 12, 24, and 36 months, respectively, which was not statistically significant. There was a significant decrease in the number of glaucoma medications at all evaluated time points except 24 months.
CONCLUSIONS: Combined AVG and lensectomy-ECP is successful in maintaining vision long term as well as decreasing IOP, and the number of glaucoma medications administered.

OBJECTIVE: This study aims to investigate surgical outcomes of eyes with severe anterior persistent fetal vasculature (PFV) and the role of associated anatomical anomalies on prognosis.
METHODS: This is a retrospective, comparative case series of 32 eyes of 31 patients who underwent vitreoretinal surgery for severe anterior PFV, defined as fibrovascular tissue totally covering the back of cataractous lens. Based on the degree of anterior retinal elongations, cases were classified as follows: group 1, eyes with well-developed pars plana and minor/no abnormalities (n = 11, 34%); group 2, eyes with partially developed pars plana and broad-based elongations (n = 9, 28%); and group 3, eyes with no visible pars plana and fibrovascular membrane having 360° continuity with peripheral retina (n = 12, 38%). Complications and functional and anatomical outcomes were investigated.
RESULTS: The median surgical age was 2 (1-12) months. The median follow-up was 26 (6-120) months. Seventy-three percent in group 1 achieved finger counting or better vision with a single surgery and without any pupillary/retinal complication. Groups 2 and 3 required 2.1 ± 0.9 and 2.6 ± 1.2 surgeries on average. Pupillary obliteration and RD occurred in 33% and 22% in group 2 and 58% and 67% in group 3. Retina remained attached after silicone oil removal in 89% of group 2 and 25% of group 3. Phthisis developed in 50% in group 3.
CONCLUSIONS: Peripheral retinal anomalies are common in severe anterior PFV and have a major impact on prognosis. Prognosis is favorable in cases with mild-to-moderate anomalies with appropriate management of possible retinal tears. In eyes with 360° retinal elongations, severe fibrous proliferation and eventual eye loss are common.

OBJECTIVE: To describe a modified ab externo method of sulcus intraocular lens (IOL) fixation and report outcomes of eyes treated with this approach.
METHODS: Records of patients with lens instability or luxation that underwent a lensectomy and sulcus IOL implantation from January 2004 to December 2020 were reviewed.
RESULTS: Nineteen eyes of 17 dogs had a sulcus IOL placed via a modified ab externo approach. The median follow-up time was 546 days (range 29-3387 days). Eight eyes (42.1%) developed POH. A total of six eyes (31.6%) developed glaucoma and required medical management long term to control IOP. The IOL position was satisfactory in most cases. Nine eyes developed superficial corneal ulcers within 4 weeks following surgery, all of which healed without complication. At the time of the last follow-up, 17 eyes were visual (89.5%).
CONCLUSIONS: The technique described represents a potentially less technically challenging option for sulcus IOL implantation. The success rate and complications are similar to previously described approaches.

OBJECTIVE: The purpose of this report is to demonstrate the patient education ability and benefits in treating glaucoma and preventing blindness with the NIDEK GS-1 Gonioscope and earlier surgical intervention with cataract surgery/lensectomy and microinvasive glaucoma surgery (MIGS).
METHODS: This data was collected using a NIDEK GS-1 Gonioscope. Informed consent was obtained from all participants following explanation of possible risks.
CONCLUSIONS: NIDEK GS-1 automated gonioscopy offers many advantages, including (1) the ability to capture high quality, 360-degree chromatic documentation of the iridocorneal angle and trabecular meshwork, (2) improved patient education on the condition at hand through images; and (3) visualization of the change in the angle and trabecular meshwork before and after surgical intervention in patients with glaucoma.
CONCLUSIONS: Gonioscopic imaging is helpful in educating patients on the anatomy of the angle and how its anatomical configuration can contribute to glaucoma. It also gives clinicians a supplementary tool to document features of the ICA; to evaluate anatomical changes before and after surgical treatment of glaucoma and cataracts; and to demonstrate to patients how a specific surgical device or technique is controlling their intraocular pressure (IOP).

OBJECTIVE: To verify the feasibility and safety of staged lensectomy and vitrectomy in stage 5C retinopathy of prematurity (ROP) with corneal opacification.
METHODS: This was a retrospective, interventional, consecutive case series. Twenty-two eyes of 18 stage 5C ROP patients with corneal opacification were included. Regular combined lensectomy and vitrectomy were not prescribed due to the invisible fundus. Staged lensectomy and posterior vitrectomy were performed. The anatomical and visual outcomes were reviewed at the final follow-up visit.
RESULTS: The mean gestational age of ROP patients was 29.3±1.6wk (range: 27-32wk), comprising 8 males and 10 females. The average birth weight was 1363.0±300.0 g. All the eyes had corneal opacity and flat or disappeared anterior chambers pre-operatively. Two eyes had complicated cataract and 7 eyes had retrolental fibroplasia. Six eyes had posterior pupillary synechiae or membranes. Seven (31.8%) eyes had vascularly active retinas. The average interval between two procedures was 6.8±4.6mo (2.5-18.5mo). After surgeries, all the patients had normal anterior chambers. Fourteen eyes had clear corneas. The intraocular pressure of 3 eyes with glaucoma was controlled by medication. Two eyes had ocular phthisis. The retina was reattached in 3 eyes and partially attached in 11 eyes. Visual acuity ranged from no light perception to hand motion.
CONCLUSIONS: Staged lensectomy and vitrectomy are procedures that can halt progression to further complications and preserve some useful eyesight in stage 5C ROP patients with corneal opacification. The earlier the lensectomy is performed, the better the prognosis is.

To report the anatomic and functional outcomes of retinectomy without lensectomy in eyes with rhegmatogenous retinal detachment (RRD) and proliferative vitreoretinopathy (PVR).
Retrospective, noncomparative, and interventional case series.
One hundred twelve eyes of 112 patients with RRD complicated by PVR who underwent retinectomy without lensectomy.
Retrospective review of patients treated with vitrectomy and retinectomy without lensectomy from January 1, 2015, to January 1, 2020.
The primary outcome was the final attachment rate and single surgery anatomic success (SSAS) at 3 and 6 months after retinectomy. Secondary outcomes included predictors of final visual acuity (VA), the mean number of subsequent operations required for complete retinal reattachment, cataract surgery, and the number of eyes that ultimately had successful silicone oil removal.
Complete final retinal reattachment was achieved in 111 of 112 (99.1%) patients, with a mean (standard deviation [SD]) follow-up of 29 (14) months (range, 8-62 months) after retinectomy. The SSAS was achieved in 84 of 112 (75%) patients at 3 months and 73 of 112 (65.2%) patients at 6 months. The final VA improved or stabilized in 76 of 112 (67.9%) eyes. Silicone oil removal was performed in 72 of 112 patients (64.3%) at a mean (SD) of 6.6 (3.3) months, and cataract surgery was performed on 101 (90.2%) eyes before the last follow-up visit.
Retinectomy without lensectomy to repair RRDs complicated by PVR showed acceptable anatomic and functional results. This study suggests that removing the lens when there is no significant cataract may not be necessary in these cases to obtain reasonable outcomes.