Visual Outcomes and Complications of Intraocular Lens Placement in the Absence of Capsular Support in a Philippine Tertiary Hospital.

Background: Surgical correction of aphakia without capsular support continues to be a challenge. Improvements in the technology of cataract surgery have provided advancements in techniques in surgical management of aphakia. Locally, we have limited data on the outcomes of the different intraocular lenses used in aphakia. Objective: This study aimed to determine visual outcomes and complications associated with different techniques of intraocular lens implantation in the absence of capsular support. Methods: We reviewed the medical charts of 207 patients who underwent intraocular lens implantation without capsular support. Excluded were patients with incomplete follow up, pediatric patients, and lost records. Best corrected visual acuity at day 1, 1st month, 3rd month and 6th month postoperatively, and the complications were noted. Results: Mean age was 60 and 51% (n=105) were females. The mean follow-up time was 9.33 ± 0.71 months. Loss of capsular support was most frequently caused by intraoperative complication (n=146, 70%) and trauma. Retropupillary fixation iris claw intraocular lens was frequently used (n=93, 44.9%). Across all patients, visual acuities showed excellent outcomes with 20/50 or better. Across IOL types, the most frequent postoperative complication was increase in IOP. Statistically significant results were set at P <0.05. Conclusion: There is a notable preference towards iris claw retropupillary lenses through time. Iris claw lenses showed the shortest operative time. All intraocular lenses used in aphakia showed comparably good postoperative visual acuities, except for the superior visual acuity trend seen among retropupillary iris claw and anterior chamber IOL groups. Complications included elevated intraocular pressures, corneal edema, and pigment dispersion.

Online Questionnaire-Based Study on the Knowledge, Attitude, and Practices of Non-Ophthalmic Physicians-in-Training Regarding Exposure Keratopathy Among Patients Admitted in a Tertiary Referral Hospital.

Objective: This study aimed to determine the knowledge, attitude, and practices of non-ophthalmic physicians-in-training regarding recognition and prevention of exposure keratopathy among patients admitted in a tertiary referral hospital. Methods: This study was a single-center cross-sectional study. An anonymous self-administered online questionnaire was distributed among non-ophthalmic physicians-in-training at the Philippine General Hospital (PGH) using convenience and snowball sampling. Results: A total of 77 responses were recorded with a response rate of 37%. The majority of the respondents uniformly reported the absence of an established eye care protocol or policy being implemented for different subsets of patients at risk for developing exposure keratopathy. Ninety-six percent (96%) was aware of exposure keratopathy. The most urgent reason for referral to the ophthalmology service was the presence of whitish of opacity at 46.8%, while the least urgent reason was the presence of eye redness at 36.4%. Only 43% of the patients with incomplete eyelid closure was referred to the ophthalmology service. The most frequently used modality of prophylactic eye protection treatment was eyelid taping (84.4%) followed by lubricants (drops and gels, 79.1%). The most common reason for not referring was the absence of an established protocol on the appropriate indication for referral to the ophthalmology service. Conclusion: This study showed that the majority of non-ophthalmic physicians-in-training had adequate knowledge and favorable attitude regarding recognition and prevention of exposure keratopathy among patient admitted at the PGH; however, the absence or the unavailability of an eye care protocol among patients with inadequate eyelid closure in our institution on the prevention of exposure keratopathy and its early treatment and appropriate indications for ophthalmology referral resulted in non-uniformity and varied practice patterns on its management.

Isolated aniridia caused by a novel PAX6 heterozygous deletion mediated by multi-exon complex rearrangement.

PURPOSE: Mutations in PAX6 gene (chromosome 11p13) encoding a transcriptional regulator involved in oculogenesis mostly present with aniridia. Aniridia is not uncommon in the Philippines but only limited information is available as yet. The purpose of this study was to present a novel, deletion mediated by complex rearrangement in PAX6 gene causing an isolated aniridia in a Filipino girl. PATIENTS AND METHODS: The patient is an 8-year-old girl who came in due to leukocoria with associated nystagmus and esotropia. She presented with subnormal vision, nystagmus, aniridia, and cataractous lenses in both eyes. The family history reveals presence of the aniridia and cataract with the mother and a sibling. The patient underwent lens extraction without intraocular lens implantation bilaterally, where patient subsequently underwent intraocular lens implantation on her left eye. Systemic workup was performed including whole abdomen, renal ultrasound, blood chemistry, and urinalysis. Targeted cataract panel with WT1 and PAX6 genes revealed a novel, heterozygous PAX6-inherited mutation from the mother. This variant is a complex rearrangement in PAX6 involving partial deletions of exons 3-5, including the initiator codon. Deletions of PAX6 are part of a contiguous gene deletion syndrome - Wilms tumor, aniridia, genitourinary anomalies, and intellectual disability syndrome - and therefore evaluation of the WT1 gene was necessary to rule out this life-threatening syndrome. CONCLUSION: This rare, complex rearrangement of multiple exons and deletions in PAX6 causing an isolated aniridia phenotype is probably the first reported case. The patient was managed by a multidisciplinary team and the guardians were counseled regarding the prognosis and complications.

Large bilateral medial rectus recession versus three-to-four horizontal muscle surgery for large-angle esodeviations.

PURPOSE: The best surgical approach to correcting large angle esodeviations is still debated. Options for correcting large esodeviations include recessing both medial rectus (BMR) muscles more than 5.5 mm or performing surgery on three or four horizontal rectus muscles. There remains no consensus on which surgery has better survival outcomes. In this research, we are interested in determining the survival rates and long-term outcomes of both surgeries locally in the Philippines. RESULTS: A total of 74 medical records were retrospectively reviewed. The mean age at onset of deviation was 2 ± 2.9 years old, and the mean age at surgery was 14 ± 12.5 years, with a mean of 12 ± 12.1 years from onset to surgery. The mean follow-up period was 9.9 months (range 6-24) . The mean preoperative deviation at near was 59.3 ± 13.6 PD (range: 35-95) while at distance was 58.5 ± 13.6 PD (range 10-95). The most common type of esotropia (ET) was infantile ET (45%), followed by basic ET. Majority of the esodeviations did not have associated vertical strabismus (67.6%, n = 50). Overall success rate was 48.1% for BMR recessions and 54.5% for 3-4 muscles surgery. Survival analysis revealed the decreasing trends of survival but plateauing of outcomes after 6 months. CONCLUSION: We reported the outcomes for both surgeries. Neither had shown superiority over the other. After a mean follow up of 9.9 months, the overall success was relatively good: 50% were successful, 39.2% overall recurrence and 13.8% overcorrections. Survival plots showed a plateauing of results after 6 months.

Novel compound heterozygous pathogenic BBS5 variants in Filipino siblings with Bardet-Biedl syndrome (BBS).

PURPOSE: Bardet-Biedl syndrome (BBS) is rare in the Philippines and only limited information on the prevalent subtypes is available as yet. The purpose of this study is to present the clinical characteristics of two Filipino siblings presenting with mutations in BBS5. PATIENTS AND METHODS: The Filipino female siblings, aged 11 and 14 years underwent comprehensive ophthalmologic evaluation. Fundus photography, macular optical coherence tomography (OCT) and electroretinography (ERG) were also obtained. Systemic workup was performed including radiographic imaging of limb defects, renal ultrasound, blood chemistry, and transvaginal ultrasound. Targeted Bardet-Biedl sequence analysis and deletion/duplication analysis were performed to determine potential pathogenic mutations. RESULTS: Both children had poor visual acuity with a myopic refraction. There was a pigmentary retinopathy with retinal pigment epithelium changes and attenuation of vessels without waxy disc pallor. Generalized macular thinning and undetectable ERG responses were recorded. Physical examination revealed obesity, facial anomalies, brachydactyly, postaxial polydactyly, and clinodactyly of fifth digits. Both patients displayed cognitive developmental delay and hypogonadism. Molecular analysis revealed novel compound heterozygous mutations in BBS5 with c.143-1 G > A (splice acceptor) and c.925_931del (p.Gln309ilefs*14), each inherited from one asymptomatic parent. CONCLUSION: These are probably the first reported BBS5 mutations causing Bardet-Biedl syndrome in the Philippines. Patients were managed by a multi-disciplinary team and the parents were counseled regarding the prognosis and additional complications associated with the syndrome.