Co-existence of Neurotrophic Keratopathy in Eyes With Limbal Stem Cell Deficiency.

PURPOSE: To investigate whether neurotrophic keratopathy is present in limbal stem cell deficiency (LSCD), by measuring corneal sensation and characterizing corneal subbasal nerve plexus. DESIGN: Prospective, cross-sectional, case-control comparative study. METHODS: A total of 46 eyes with LSCD and 14 normal eyes were recruited from 2019 to 2022. Corneal sensation was measured using a Cochet-Bonnet esthesiometer, and subbasal nerve plexus was imaged using in vivo confocal microscopy (IVCM) at the central cornea and 4 limbal regions. Subbasal nerve density (SND, number of nerves/mm2), subbasal nerve length (SNL, total length of nerves/mm2) and subbasal nerve branch density (SNBD, number of branches/mm2) were quantified. LSCD was graded to stage 1, 2, and 3 using a previously established staging method consisting of clinical scores, basal cell density, central corneal epithelial thickness, and SNL. RESULTS: The mean (±SD) cornea sensation in the central cornea and limbus were 29.2 ± 21.5 and 33.6 ± 15.1 mm in the LSCD group and 57.6 ± 5.8 and 54.3 ± 4.7 mm in the control group, respectively (all P < .001). In sectoral LSCD, the corneal sensation in the affected regions (29.1 ± 17.6 mm) decreased significantly compared to the unaffected regions (41.4 ± 18.2 mm, P < .001). Central corneal SND, SNL, and SNBD were reduced by 84.6%, 82.6%, and 89.2%, respectively, in LSCD compared to controls (all P < 0.05). The central corneal sensation negatively correlated with the severity of LSCD (rho = -0.64, P < .0001) and positively correlated with SND, SNL, and SNBD (rho = 0.63, 0.66, and 0.56, respectively; all P < .001). CONCLUSIONS: Corneal sensation was reduced in eyes with LSCD. The degree of corneal sensation reduction positively correlated with the severity of LSCD. This finding demonstrated the coexistence of neurotropic keratopathy in LSCD.

Autologous Serum Eye Drops in the Management of Limbal Stem Cell Deficiency Associated With Glaucoma Surgery.

PURPOSE: To evaluate safety and efficacy of autologous serum eye drops (AS) in the treatment of limbal stem cell deficiency (LSCD) associated with glaucoma surgery. METHODS: Retrospective case series of eyes with glaucoma surgery-induced LSCD treated with AS. Diagnosis of LSCD was confirmed by anterior segment optical coherence tomography, in vivo confocal microscopy, and/or impression cytology. Limbal stem cell deficiency severity was staged using a clinical scoring system (2-10 points). Outcome measures were changes (≥2 points) of the LSCD score and best-corrected visual acuity (BCVA) from the baseline to the last follow-up. RESULTS: Thirteen eyes of 12 consecutive patients treated with 50% AS for at least 3 months were included. The mean age was 78.9±7.5 years and the mean duration of AS use was 20.9±16.8 months. Indications of AS included LSCD progression in eight eyes (61.5%) and visual axis threatening in five eyes (38.5%). The mean LSCD score at baseline (6.7±1.6) was similar to that at last follow-up (6.5±2.2, P =0.625). Two eyes (15.4%) showed improvement, nine eyes (69.2%) were stable, and two eyes (15.4%) worsened. The mean baseline BCVA (0.89±0.64 logMAR) was similar to the mean final BCVA (1.05±0.63 logMAR, P =0.173). There were no serious adverse complications related to AS. CONCLUSION: AS appears to be well tolerated and may stabilize the progression of LSCD with limited effects. A larger study is necessary to confirm the findings.

Recombinant Human Nerve Growth Factor for Pediatric Neurotrophic Keratopathy.

ABSTRACT: A 4-year-old boy presented with right neurotrophic corneal ulcer, lagophthalmos, and facial palsy 8 months after neurosurgery for synchronous brain tumors. Initial treatment with topical antibiotics, topical corticosteroids, lubrication, and lateral tarsorrhaphy successfully treated the corneal epithelial defect; however, the cornea continued to demonstrate diffuse epitheliopathy and a dense stromal opacity and remained insensate on Cochet-Bonnet esthesiometry. After a course of topical cenegermin, central corneal sensation normalized, and the corneal epitheliopathy was markedly improved. Two years after the completion of cenegermin, corneal sensation was maintained; there were no recurrences of epithelial defects, and the stromal opacity had markedly improved. In vivo confocal microscopy (IVCM) demonstrated the presence of subbasal corneal innervation. This report highlights the safety and prolonged effects of cenegermin for the treatment of pediatric iatrogenic neurotrophic keratopathy, as evidenced by the clinical course and IVCM.

Unilateral Urrets-Zavalia syndrome after Implantable Collamer Lens implantation: a case report and review of the literature.

BACKGROUND: Fixed dilated pupil after ophthalmic surgery or Urrets-Zavalia syndrome occurs after anterior segment surgery and usually relates to postoperative elevation of intraocular pressure. Urrets-Zavalia syndrome results in complaints of glare, halo, and photophobia. Retention of the viscoelastic agent during Implantable Collamer Lens implantation can result in postoperative elevation of intraocular pressure and Urrets-Zavalia syndrome. However, reversibility of pupillary dilatation is possible in some cases. CASE PRESENTATION: A 20-year-old Thai man with myopic astigmatism in both eyes underwent Implantable Collamer Lens implantation in the right eye. The preoperative slit-lamp examination of both eyes was normal, and no ectatic changes were detected from corneal tomography. One hour after the uncomplicated surgery of the right eye, intraocular pressure increased to 48 mmHg and was immediately controlled with antiglaucoma medications. Postoperative pupillary dilatation was detected, presumably due to effect of preoperative application of mydriatic drops. At postoperative day 1, the right pupil remained dilated but still reactive to light and pilocarpine 2% eye drops. Two weeks later, the left eye underwent the Implantable Collamer Lens implantation and showed neither postoperative increase in intraocular pressure nor postoperative pupillary dilatation. Two months after surgery, the dilatation of the right pupil partially reversed. CONCLUSIONS: The findings of the right eye suggested diagnosis of Urrets-Zavalia syndrome. Compared with former reports, we noted an association between immediate control of elevation of postoperative intraocular pressure, light reactivity of the dilated pupil, and reactivity to pilocarpine 2% eye drops as potential predictors for reversibility of Urrets-Zavalia syndrome.

Time to Keratometric Stability After Pterygium Excision and the Associated Factors: A Clinical Perspective.

PURPOSE: To determine the percentage of eyes with corneal astigmatic power stability and mean corneal keratometric power at 6-month post-pterygium excision, and to identify the time, and the associated factors, required to achieve stability. METHODS: This prospective observational study enrolled patients undergoing pterygium excision. Patients were evaluated for baseline characteristics and keratometric data before and every month after pterygium excision for six months using IOL Master 500® (Carl Zeiss, Meditec). Clinically stable corneal astigmatic power and keratometric power were, respectively, defined as changes in these parameters of less than 0.25 and 0.27 diopters after two consecutive visits. Time to corneal astigmatic and keratometric power stability, as well as factors associated with the stability, were analyzed. RESULTS: Forty percent and 73.3% of eyes, respectively, demonstrated corneal astigmatic and corneal keratometric stability at six months post-operation. Within three months of reaching initial stability, the corneal astigmatic power and the mean keratometric power showed instability in 46.7% and 27.3% of patients, respectively. No patients with keratometric stability for more than three months became unstable during the study period. The extension of pterygium exceeding 3.0 mm was associated with a delay in time to corneal astigmatic stability (HRadjusted 0.41; 95% CI 0.19-0.89; P= 0.02). CONCLUSION: According to the clinical relevance, 40% and 73% of patients, respectively, presented corneal astigmatic and keratometric stability within six months post-operation. Patients with a pterygium extension of more than 3 mm required a longer time for corneal astigmatic stability. It is recommended that keratometric stability be achieved for at least three months before commencing with additional procedures.

Identification of A Novel TGFBI Gene Mutation (p.Serine524Cystine) Associated with Late Onset Recurrent Epithelial Erosions and Bowman Layer Opacities.

BACKGROUND: Most transforming growth factor beta-induced (TGFBI) corneal dystrophies are associated with a characteristic phenotype, clinical course, and a conserved mutation in the TGFBI gene. However, we report a novel TGFBI missense mutation associated with a late-onset, variant Bowman layer dystrophy. METHODS: Participants underwent slit-lamp examination and multimodal imaging. Polymerase chain reaction amplification and Sanger sequencing were performed on saliva-derived genomic DNA to screen TGFBI exons 4 and 12 as well as COL17A1 exon 46. PolyPhen-2 and SIFT were used to predict the functional impact of any identified variants. RESULTS: A 56-year-old Thai woman reported a four-year history of decreased vision and intermittent eye irritation, suggestive of recurrent epithelial erosions, in both eyes. Slit-lamp exam revealed bilateral, irregular, limbal-sparing Bowman layer opacities, which were also noted on anterior segment optical coherence tomography. Phototherapeutic keratectomy was performed in the right eye, improving the best-corrected visual acuity from 20/50 to 20/30. Sequencing of the TGFBI gene revealed a novel heterozygous, missense mutation in exon 12 (c.1571 C > G; p.Ser524Cys), which was present in an affected son and absent in an unaffected son, and was predicted to be damaging by PolyPhen-2 and SIFT. The patient was diagnosed with a variant Bowman layer dystrophy given the late onset of an atypical phenotype and the identification of a novel TGFBI mutation. CONCLUSIONS: A novel TGFBI missense mutation is associated with a late-onset Bowman layer dystrophy. Given the atypical clinical appearance and course, molecular genetic analysis was utilized to establish a definitive diagnosis.