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Although 8q21.11 microdeletion syndrome (8q21.11 DS) has been reported in association with congenital corneal opacities, reports of the clinicopathological features and management are scarce.We reviewed medical records including ophthalmic evaluations, imaging, operative reports, and pathology reports of two unrelated patients referred to the Ophthalmology Clinic of UPMC Children's Hospital of Pittsburgh with a cytogenetic diagnosis of 8q21.11 DS.Ophthalmological evaluation of both children revealed bilateral enlarged, staphylomatous, and cloudy corneas with neovascularization. These findings were consistent with the diagnosis of congenital corneal staphyloma (CCS). In one patient, anterior segment optical coherence tomography and high-frequency ultrasound revealed materials consistent with lens remnants embedded in the cornea; this was confirmed by histopathology. In the second patient, lens was found to be adherent to the cornea during surgery. One eye underwent enucleation for corneal perforation secondary to elevated intraocular pressure. In the other eyes, treatment consisted of penetrating keratoplasty combined with vitrectomy. Ahmed tube was subsequently placed to control intraocular pressure.8q21.11 microdeletion syndrome can be associated with bilateral CCS, likely related to a combination of anterior segment developmental anomalies and elevated intraocular pressure. Tectonic penetrating keratoplasty is necessary to prevent corneal perforation, together with a strict control of the intraocular pressure.

PURPOSE: To evaluate management of keratoconic eyes with anterior stromal necrosis overlying the intracorneal ring segment (ICRS), by either ICRS explantation alone or exchange with corneal allogenic intrastromal ring segments (CAIRS). METHODS: Among 643 Intacs SK (Additional Technology, Inc) inserted at one institutional center, 16 eyes (15 patients) with overlying spontaneous anterior stromal necrosis were identified. Data included size of stromal defect and refractive and topographical findings before ICRS insertion, before anterior stromal necrosis, and 6 months after intervention. RESULTS: The 10-year incidence of anterior stromal necrosis after femtosecond laser–assisted ICRS insertion was 5.5%. Eight eyes underwent ICRS removal only and 8 eyes had ICRS exchanged with CAIRS. In the first group, CDVA worsened from 0.14 before melt to 0.28 logMAR after removal ( P = .10), simulated keratometry (SimK) and maximum axial keratometry (Kmax) increased from 44.73 to 46.34 diopters (D) ( P = .14) and from 49.23 to 52.26 D ( P = .14), respectively, and coma worsened from 0.87 to 1.52 D ( P = .02). In the CAIRS group, CDVA of 0.16 before melt improved to 0.11 logMAR postoperatively ( P > .99), and topographic indices stabilized with SimK, Kmax, and coma mildly altering from 45.31 to 45.44 D ( P > .99), from 49.25 to 49.64 D ( P > .99) and from 0.87 to 0.81 D ( P > .99), respectively. Whether the ICRS were explanted or exchanged, the visual and topographic mean values were better than those reported before ICRS implantation, whereas higher order aberrations in eyes without CAIRS regressed to levels before ICRS insertion. At the site of melt, thinnest residual stromal thickness averaged 327 µm with ICRS removal and 490 µm with CAIRS. Eyes with larger melt areas resulted in less optimal results with CAIRS implantation. CONCLUSIONS: Early experience in the management of anterior stromal necrosis by exchange of polymethylmethacrylate ICRS with CAIRS seems to avoid stromal thinning and confer better visual and topographic results, which were more pronounced with thicker segments. The benefit of CAIRS in large stromal melts needs to be investigated. [ J Refract Surg . 2022;38(4):256–263.]

A 40-year-old woman was referred for the assessment of bilateral corneal opacities with gradual visual decline over the course of the past decade. Her past ocular history is significant for bilateral amblyopia and strabismus surgery in both eyes before age 5. The patient's parents were told by her childhood ophthalmologist that she had a hereditary disorder. Her systemic review was significant for anal fissure and human leukocyte antigen-B27 ankylosing spondylitis. Her past ocular record revealed corrected distance visual acuity (CDVA) of 20/80 in both eyes in 2018 with central corneal haze. On presentation, her uncorrected distance visual acuity was 20/150 in both eyes. Her CDVA was 20/100 in both eyes with manifest refraction of +0.50 -2.50 × 075 in the right eye and +5.00 -2.25 × 094 in the left eye. Corneal topography reflected keratometry of 35.75/38.97 × 171 in the right eye and 36.45/38.35 × 32 in the left eye. Central corneal thickness was 669 μm and 652 μm, respectively. External slitlamp examination revealed a central faint stromal opacity inferior to the visual axis in the right eye and a central faint stromal opacity in the left eye, and both were associated with steep posterior curvature of the cornea (Figure 1). Further findings included 0.5 corneal haze with mild guttata, normal irides, and clear lenses in both eyes. Intraocular pressure was 23 mm Hg and 26 mm Hg, respectively (Figure 2, Supplemental Figures 1 and 2, http://links.lww.com/JRS/A543). Gonioscopy was unremarkable. Dilated fundus examination revealed a 0.15 cup-to-disc ratio bilaterally, but otherwise no pertinent vitreoretinal pathologies were noted. What is the most likely diagnosis? What medical or surgical interventions would you recommend for this patient? What is the prognosis for this patient?

PURPOSE: To compare the clinical and tomographic properties of adult patients with keratoconus treated with accelerated corneal cross-linking (A-CXL) versus accelerated contact lens–assisted corneal cross-linking (A-CACXL). METHODS: Patients who underwent A-CXL and A-CACXL due to progressive keratoconus were enrolled from January 2015 to January 2018 in this retrospective case–control study. The treatment group (minimum corneal thickness of less than 400 µm after epithelium removal; 30 patients, 30 eyes) was treated with A-CACXL; the control group (minimum corneal thickness of 400 µm or greater, 32 patients, 32 eyes) was treated with A-CXL. Assessments occurred before treatment and 12 months postoperatively. Demographic, clinical, and tomographic data were obtained from outpatient clinic reports. RESULTS: Significant improvement in visual acuity was evident at 12-month follow-up for the control group in uncorrected distance visual acuity (0.62 ± 0.42 vs 0.43 ± 0.31 logMAR, P = .01) and the treatment group in corrected distance visual acuity (0.51 ± 0.30 vs 0.40 ± 0.49 logMAR, P = .03). Progression of keratoconus was halted at similar rates for both groups (76.7% treatment, 84.4% control, P = .21). Mean minimum corneal thickness showed minor but significant thinning at the 12-month follow-up visit compared to baseline (control group = 463 ± 31 vs 450 ± 35 µm, P > .01; treatment group = 398 ± 32 vs 388 ± 41 µm, P = .02). CONCLUSIONS: A-CACXL halted keratoconus progression in 76.7% of eyes and achieved regression in 33.3% of eyes, with rates comparable to A-CXL. Visual outcomes improved for both groups, with similar keratometry changes. A-CACXL is an effective and safe option for patients with keratoconus and thin corneas, with results similar to A-CXL treatment in patients with a minimum corneal thickness of 400 µm or greater. [ J Refract Surg . 2021;37(9):623–630.]

Contact lens-assisted corneal cross-linking (CACXL) was introduced by Jacob et al. in 2012 for treating thin keratoconic corneas using riboflavin soaked soft contact lens to artificially increase the functional corneal thickness. It is advantageous over other thin corneal cross-linking techniques as it works independent of swelling properties of the cornea, is an epi-off technique and does not require additional time, additional expensive equipments or special solutions. The only additional requirement as compared to all other techniques is a UV barrier-free soft contact lens (SoflensTM, B&L) which is easily available and inexpensive. Advantages include simplicity, easy adaptability, early visual rehabilitation, good visual outcomes, safety, and efficacy. Progression rates are acceptable and the need for re-treatment has been low. CACXL can help regularize corneal shape and may be used in isolation or synergistically with Intracorneal ring segments (ICRS) or Corneal allogenic intrastromal ring segments (CAIRS). It gives about 70% stiffening as compared to standard Dresden protocol CXL in less ideal porcine eye studies. Murine eye models that closely mimic thin corneas and show greater cross-linking effect as compared to porcine eyes may be a better model for evaluation of CACXL, however further studies are needed. Care should be taken in selecting the right kind of contact lens. Proper technique should be followed, especially by confirming thinnest functional pachymetry to be above 400 microns intra-operatively before application of UV-A. The sub-contact lens riboflavin film should be avoided as also an excessively thick supra-contact lens riboflavin film and too many re-applications.

Purpose of review To review current, effective and more popular techniques for scleral fixation of intraocular lens (IOLs) and IOL-capsular bag complex. Recent findings Scleral fixation of IOLs became popular, originally with sutured scleral fixated IOLs and later the Scharioth technique of intrascleral haptic fixation. This was further developed as the Glued IOL technique which enjoys widespread adoption all over the world. Recently the Yamane technique has also become popular and is being widely adopted as well. Summary Scleral fixated IOLs have evolved in the last 2 decades with technical modifications, extended indications and improvised instrumentation. Though sutured and sutureless techniques have been growing equally, the sutureless scleral fixation techniques have attracted special interest. Reduced suture-related complications, technical ease and high-quality functional outcomes may be possible reasons. Sutureless capsular bag fixation also has distinct advantages.

The main postoperative complication of Descemet membrane endothelial keratoplasty and pre-Descemet endothelial keratoplasty (PDEK) is graft detachment. We report a technique in which the host Descemet membrane or the incision is used for graft scaffolding. The descemetorhexis is created 0.5 mm smaller than the graft from between 1 quadrant to 360 degrees, preferably in two opposite quadrants. Using the Jacob technique of pressurized air infusion (air pump-assisted PDEK), the host Descemet membrane is gently teased from under the graft with a reverse Sinskey hook and allowed to overlap the graft from the posterior aspect in a scaffolding manner. Wound scaffolding within the incision is attained by pulling any overlapping part of the graft into the inner part of the corneal incision instead of allowing it to lie posterior to the inferior lip. Both these scaffolding maneuvers give an extra degree of scaffolding or support that provides graft stability and thereby decreases the risk for graft detachment.

Purpose To report a case with multiple macroperforations and a double anterior chamber (AC) after predescemetic deep anterior lamellar keratoplasty. Methods A patient was referred after undergoing complicated excision of pseudopterygium that extended onto the cornea. At presentation, an eccentric lamellar patch graft extending over the pupillary axis with a double AC was seen. Surgical intervention showed a predescemetic plane of dissection with 2 macroperforations and iris plugging of the peripheral perforations. Suturing and fibrin glue alone did not seal the macroperforation, and a donor lenticule from small incision lenticule extraction (SMILE) was used to close the larger perforation using fibrin glue. A trephine was then used to mark a larger area of predescemetic dissection that included the original patch graft. A donor corneal graft was sutured after stripping Descemet membrane (DM). Results Postoperatively, the double AC resolved, and vision improved to 0.50 decimal equivalent (20/40) by 3 weeks. At 18-month postoperative follow-up, corrected distance visual acuity (CDVA) was 0.67 (20/30). The donor graft remained clear, and the SMILE lenticule with underlying sutures could be visualized. Conclusions A donor lenticule from SMILE surgery is a useful adjunct to seal macroperforations in deep anterior lamellar keratoplasty because the thin uniform lamellar tissue is easily applied using fibrin glue and gives uniform and good apposition on both host and donor sides. It can be used immediately without further preparation unlike hand-fashioned patch grafts.