Congenital fibrosis of the extraocular muscles (CFEOM) is a rare, congenital, non-progressive disorder presenting with partial or total ophthalmoplegia, with variable degrees of ptosis in both eyes. We present the clinical manifestations of congenital fibrosis of the extraocular muscles in two patients. Both patients presented with bilateral ptosis and variable ophthalmoplegia with a chin-up posture. The ocular deviations have been noted since birth. No patient demonstrated a Marcus-Gunn jaw-winking phenomenon. Both patients had a mild refractive error with with-the-rule astigmatism. Deviation for both patients revealed exotropia with varying amounts of hypotropia and limitations in the movement of extraocular muscles. Both patients presented no abnormalities in the pupils. Neuroimaging revealed atrophy of the extraocular muscles. Diagnosis of CFEOM in a resource-poor setting is also challenging due to inaccessible gene testing. Manifestations of CFEOM vary across affected patients. CFEOM proposes challenges to the ophthalmologist with regards to management.
congenital fibrosis ; extraocular muscles ; congenital cranial dysinnervation disorders

Silver-Russell Syndrome (SRS) is a rare disorder associated with prenatal and postnatal growth retardation with associated characteristic facial and ocular features including strabismus. We report the outcome of strabismus surgery performed for exotropia in a 4-year-old patient with SRS. The patient presented with decreased visual acuity and constant exotropia of the right eye noted since 3 months of age. Systemic SRS characteristics consisted of relative macrocephaly, short stature, forehead prominence and stunted growth pattern. An X-pattern exotropia is consistent with bilateral tight lateral recti muscles with overelevation in adduction of the left eye was present. Patient underwent unilateral right lateral rectus recession and right medial rectus resection for a 50-prism diopter constant exotropia. Patient had a favorable outcome of within 8 prism diopters from orthotropia at 1st, 3rd-, 6th- and 12th-month post-operatively.
Russell Syndrome ; exotropia ; macrocephaly

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.
Aphakia ; Philippines

Objective: Toric intraocular lens implantation has been used to correct corneal astigmatism during cataract surgery. The study aimed to compare the visual outcomes between manual vs markerless toric intraocular lens implantation in astigmatic correction. Methods: The medical records of patients at American Eye Center who underwent phacoemulsification by multiple surgeons with insertion of monofocal or multifocal toric lenses via manual marking and markerless method from 2010-2019 were reviewed. Results: A total of 70 patients were included in the study. Results showed no significant difference in the following characteristics between manual and markerless method at one month and two months post-cataract surgery: uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), uncorrected near vision acuity (UNVA), corrected near vision acuity (CNVA), refraction spherical equivalent. The UDVA, CDVA, UNVA, CNVA and astigmatism had significantly lower median/mean-rank at one and two months postoperatively compared to preoperative values. Conclusion In conclusion, our findings indicated that both manual-based and markerless systems effectively facilitated accurate placement of the toric IOL on the desired axis. Notably, there was no significant difference observed between the two methods. Both systems are straightforward to execute. In low-resource settings like the Philippines, the manual marking method can be employed when markerless guidance equipment is unavailable.
Phacoemulsification ; Astigmatism ; Philippines

OBJECTIVE

Successful intraocular lens (IOL) placement in cataract surgery is synonymous with the IOL being placed in the capsular bag. When the capsular bag is violated, the ciliary sulcus becomes an option to approximate an inthe- bag position. Studies report that single-piece foldable acrylic (SPA) IOLs are a poor choice for the sulcus. This study aimed to report the visual outcomes and complications of sulcus placement of single-piece intraocular lenses and three-piece intraocular lenses, and compare the design and characteristics to the occurrences of complications.

The medical records of patients were retrospectively reviewed in a single center from 2016-2019.

A total of 245 eyes from 237 patients were included in the study with a mean age of 61 years and male predominance. Majority of sulcus implantation occurred during phacoemulsification (87%). Around 82% (n=202) were implanted with single-piece IOLs and 18% (n=43) were three-piece IOLs. Best corrected distance visual acuity (BCDVA) was 20/20 after six months for both groups. Comparison between two groups showed no superiority with each other. Complications notable were elevated intraocular pressure, corneal edema, loss of IOL centration, and pigment dispersion. Smaller optic diameter and overall length predispose to higher probabilities of loss of centration. Pliability, hydrophobicity/hydrophilicity, and material do not correlate with postoperative complications. There were significantly higher numbers of pigment dispersions in IOLs with square-edged design.

In conclusion, visual outcomes remain equally excellent for both single-piece and threepiece groups. In contrast, there were more notable complications in single-piece group. Loss of centration tends to occur more with mean optic diameters lower than or equal to 5.50 mm and an overall length of less than 12.50 mm or lower. While appropriate for the capsular bag, square-edged designs were found to be inappropriate for the sulcus. The retrospective design does not allow strong inferences hence caution should be taken in correlating results.