12-Year Outcomes of Microkeratome-Assisted Anterior Lamellar Therapeutic Keratoplasty (ALTK) for Disorders of the Anterior Part of the Corneal Stroma - A Comparative Review of Adult and Children.

PURPOSE: To report the visual outcomes and complications of automated anterior lamellar therapeutic keratoplasty (ALTK) in adults and children, and to examine these outcomes as a function of age and etiology. METHODS: A consecutive series of cases undergoing automated ALTK procedures performed at the Jules-Gonin Eye Hospital Lausanne, Switzerland, between June 2003 and January 2015. Only patients with at least 3 months of follow-up were included. RESULTS: There were 53 eyes (24 right) of 51 patients (17 female, 16 juvenile), with a mean age of 34.8 years (range from 3 months to 88 years), analyzed. The mean follow-up was 35 (± 26) months. Diagnosis in the adult (n = 37) vs. juvenile (n = 16) eyes was different: opacity following surgical complication 8 vs. 0, congenital 1 vs. 1, dystrophy 5 vs. 2, infection 12 vs. 5, keratectasia 3 vs. 0, trauma 7 vs. 0, tumor 1 vs. 3, and allergy 0 vs. 5. Visual impairment as a consequence of corneal scarring was the principle indication for surgery in both adult (70%; 26) and juvenile eyes (63%; 10); other indications were choristoma, dermoid, other tumors, astigmatism, and congenital opacity. In adult vs. juvenile eyes, the mean visual acuity (spectacle and contact lenses) was, at last visit, 0.55 vs. 0.45 LogMAR (p = 0.78), with a range of 100% to hand movements. Failure occurred in 6 (16%) vs. 2 (13%) cases and complications were observed in 14 (38%) vs. 9 (56%) cases, however, more surgical revision was required in juvenile eyes, 4 (11%) vs. 7 (43%) (p = 0.01, Fisher test). CONCLUSIONS: This study shows that anterior lamellar keratoplasty in children retains good visual function when combined with adequate amblyopic therapy. However, the rate of complications is higher in juveniles and requires more intensive interdisciplinary follow-up.

Refractive outcomes and complications after combined copolymer phakic intraocular lens explantation and phacoemulsification with intraocular lens implantation.

PURPOSE: To assess safety, efficacy, and predictability of combined Implantable Collamer Lens phakic intraocular lens (pIOL) explantation and phacoemulsification and to report the rate of complications. A secondary aim was to examine whether intraocular lens (IOL) power calculation was affected by the pIOL in situ. SETTING: Jules-Gonin Eye Hospital, Lausanne, Switzerland. DESIGN: Retrospective case series. METHODS: The records of patients having pIOL implantation with subsequent phacoemulsification were reviewed. Data analysis was performed between July 1, 2015, and December 31, 2015. The mean corrected distance visual acuity (CDVA), safety, stability of refraction, predictability of IOL power calculation, and postoperative complications were recorded. RESULTS: The study comprised 38 eyes (29 patients). Of the eyes, 32 (82%) were within ±1.00 diopter (D) of the target refraction and 21 (57.9%) were within ±0.50 D. The mean CDVA after IOL implantation was significantly better than before pIOL implantation (P < .001) and similar to after pIOL implantation (P = .87). The safety of this multistepped procedure was good; on average, 1.6 Snellen lines of CDVA were gained over before pIOL implantation. Branch retinal vein occlusion occurred in 1 eye; no other sight-threatening complications were observed. Posterior capsule opacification developed in 15 eyes (39.5%) and was treated successfully with neodymium:YAG capsulotomy. CONCLUSIONS: The highest mean CDVA was recorded at the final visit. Good safety, stability, and predictability were also observed. Postoperative complications were few and in all but 1 case, not sight threatening. Overall, results indicate the combination of procedures does not negatively affect the final visual outcome.

Clinical Outcomes and Cataract Formation Rates in Eyes 10 Years After Posterior Phakic Lens Implantation for Myopia.

IMPORTANCE: Intraocular collamer lenses (ICLs) are posterior chamber phakic lenses that provide a refractive surgery option for those with high myopia or astigmatism. The short-term and midterm results indicate good refraction stability, efficacy, and safety. Cataract has been suggested to be an important long-term complication of ICL implantation. OBJECTIVE: To report the rates of cataract development and refractive outcomes 10 years after ICL implantation. DESIGN, SETTING, AND PARTICIPANTS: The study included 133 eyes of 78 patients undergoing consecutive V4 model ICL implantations, which took place from January 1, 1998, through December 31, 2004, at Jules-Gonin Eye Hospital, Lausanne, Switzerland. Data analysis was performed from January 1, 2014, to May 31, 2014. The lenses implanted were as follows: 53 V4 model ICLs of -15.5 D or greater, 73 V4 model ICLs of less than -15.5 diopter (D), and 7 V4 model toric ICLs for myopia. MAIN OUTCOMES AND MEASURES: Rate of cataract surgery, lens opacity, ocular hypertension, refractive safety, predictability, and stability. RESULTS: A total of 133 eyes of 78 patients (34 men and 44 women, with a mean [SD] age of 38.8 [9.2] years at enrollment) met the inclusion criteria. The rate of lens opacity development was 40.9% (95% CI, 32.7%-48.8%) and 54.8% (95% CI, 44.7%-63.0%) at 5 and 10 years, respectively. Phacoemulsification was performed in 5 eyes (4.9%; 95% CI, 1.0%-8.7%) and 18 eyes (18.3%; 95% CI, 10.1%-25.8%) at 5 and 10 years after ICL implantation, respectively. The vault height (distance between the posterior ICL surface and anterior lens surface) measured a mean (SD) of 426 (344) µm immediately postoperatively, decreasing to 213 (169) µm at 10 years. A smaller vault height was associated with the development of lens opacity and phacoemulsification (P = .005 and .008, respectively). The intraocular pressure was 15 mm Hg postoperatively, and there was no significant increase in intraocular pressure observed until the 10-year follow-up (16 mm Hg, P = .02). At 10 years, 12 eyes (12.9%; 95% CI, 5.6%-19.6%) had developed ocular hypertension that required topical medication. At 10 years, the mean (SD) safety index was 1.25 (0.57), with a manifest spherical equivalent of -0.5 D at 1-year postoperatively vs -0.7 D at 10 years postoperatively in eyes aimed at emmetropia. CONCLUSIONS AND RELEVANCE: This retrospective single center study indicates that ICL implantation provides good long-term safety and stability of refraction in patients with high myopia compared with similar short-term studies. However, the rates of cataract formation and ocular hypertension at 10 years have important clinical implications, and as such this information should be part of the available patient information before ICL implantation.

Modern cataract surgery for radiation-induced cataracts in retinoblastoma.

BACKGROUND: Surgery of radiation-induced cataracts in children with retinoblastoma (RB) is a challenge as early intervention is weighted against the need to delay surgery until complete tumour control is obtained. This study analyses the safety and functional results of such surgery. METHODS: In a retrospective, non-comparative, consecutive case series, we reviewed medical records of RB patients ≤ 14 y of age who underwent either external beam radiotherapy or plaque treatment and were operated for radiation-induced cataract between 1985 and 2008. RESULTS: In total, 21 eyes of 20 RB patients were included and 18 out of the 21 eyes had Reese-Ellsworth stage V or ABC classification group D/E RB. Median interval between last treatment for RB and cataract surgery was 21.5 months, range 3-164 months. Phacoaspiration was performed in 13 eyes (61%), extra-capsular cataract extraction in 8 (39%) and intraocular lens implantation in 19 eyes (90%). The majority of cases, 11/21 (52%), underwent posterior capsulorhexis or capsulotomy and 6/21 (28%) an anterior vitrectomy. Postoperative visual acuity was ≥ 20/200 in 13 eyes and < 20/200 in 5 eyes. Intraocular tumour recurrence was noted in three eyes. Mean postoperative follow up was 90 months ± 69 months. CONCLUSIONS: Modern cataract surgery, including clear cornea approach, lens aspiration with posterior capsulotomy, anterior vitrectomy and IOL implantation is a safe procedure for radiation-induced cataract as long as RB is controlled. The visual prognosis is limited by initial tumour involvement of the macula and by corneal complications of radiotherapy. We recommend a minimal interval of 9 months between completion of treatment of retinoblastoma and cataract surgery.

CRYBA3/A1 gene mutation associated with suture-sparing autosomal dominant congenital nuclear cataract: a novel phenotype.

PURPOSE: To identify the genetic defect leading to the congenital nuclear cataract affecting a large five-generation Swiss family. METHODS: Family history and clinical data were recorded. The phenotype was documented by both slit lamp and Scheimpflug photography. One cortical lens was evaluated by electron microscopy after cataract extraction. Lenticular phenotyping and genotyping were performed independently with short tandem repeat polymorphism. Linkage analysis was performed, and candidate genes were PCR amplified and screened for mutations on both strands using direct sequencing. RESULTS: Affected individuals had a congenital nuclear lactescent cataract in both eyes. Linkage was observed on chromosome 17 for DNA marker D17S1857 (lod score: 3.44 at theta = 0). Direct sequencing of CRYBA3/A1, which maps to the vicinity, revealed an in-frame 3-bp deletion in exon 4 (279delGAG). This mutation involved a deletion of glycine-91, cosegregated in all affected individuals, and was not observed in unaffected individuals or in 250 normal control subjects from the same ethnic background. Electron microscopy showed that cortical lens fiber morphology was normal. CONCLUSIONS: The DeltaG91 mutation in CRYBA3/A1 is associated with an autosomal dominant congenital nuclear lactescent cataract. A splice mutation (IVS3+1G/A) in this gene has been reported in a zonular cataract with sutural opacities. These results indicate phenotypic heterogeneity related to mutations in this gene.

Implantable contact lens for moderate to high myopia: relationship of vaulting to cataract formation.

PURPOSE: To study cataract formation in eyes with an implantable contact lens (ICL) used for moderate to high myopia. SETTING: University Eye Hospital, Lausanne, Switzerland. METHODS: An ICL (model V3 or V4, Staar Surgical AG) was implanted in 75 eyes. Three months after surgery and again at the last follow-up examination, the transparency of the crystalline lens was assessed on transilluminated photographs and the vaulting of the ICL over the crystalline lens was evaluated. Central vaulting was measured precisely on digitized photographs taken with a 75 SL Zeiss slitlamp camera, while peripheral vaulting was estimated on photographs obtained with a Scheimpflug camera. The minimum follow-up was 12 months; the mean was 21.8 months. RESULTS: At the last follow-up, 20 of the 75 eyes (27%) had an ICL-induced anterior subcapsular cataract (ASCC). The number of cataracts increased with the duration of the follow-up. Cataracts developed more commonly in older patients than in younger patients. All 20 cataracts occurred when the central vaulting was equal to or less than 0.09 mm. In 26 eyes with the same range of vaulting (among which 11 had no vaulting), the lenses were clear at the last visit. The 20 patients with cataract and the 26 patients with clear lenses matched in age and duration of follow-up but not in myopia. No touch between the ICL and the crystalline lens was encountered when the central vaulting was equal to or greater than 0.15 mm. Vaulting showed a slight decrease over time. No statistically significant difference in vaulting was found between models V3 and V4. CONCLUSION: Central and/or peripheral contact between the ICL and the crystalline lens may be responsible for the high incidence of ASCC formation in this study. Central vaulting greater than 0.09 mm appears to protect the crystalline lens from cataract formation. However, we recommend aiming for higher central vaulting (0.15 mm) to avoid contact between the ICL and the crystalline lens. This should be attainable by implanting longer ICLs.

BIGH3 mutation spectrum in corneal dystrophies.

PURPOSE: To investigate the molecular pathology underlying BIGH3-related corneal dystrophies (CDs) and to further delineate genotype-phenotype specificity. METHODS: Sixty-one index patients with CDs were subjected to phenotypic and genotypic characterization. The corneal phenotypes of all patients were assessed by biomicroscopy and documented by slit lamp photography. The BIGH3 gene was amplified exon by exon from constitutional DNA to perform single-strand conformation polymorphism (SSCP) analysis, followed by direct bidirectional sequencing of abnormal conformers. RESULTS: The phenotypes of CDs were classified as lattice CD in 30 patients, Groenouw type I in 12 (CDGGI), Avellino in 7 (CDA), Reis-Bückler in 8 (CDRB), and Thiel-Behnke in 4 (CDTB). Fifty occurrences of 16 distinct mutations were identified, including 8 novel mutations responsible for lattice type IIIA in three patients (CDLIIA), intermediate type I/IIIA (CDLI/IIIA) in four patients, and atypical CDL with deep deposits in one patient (CDL-deep). CONCLUSIONS: Disease-causing mutations were identified in 80% of the patients (50/61). All mutations localize in two regions of kerato-epithelin: the amino acid R124 and BIGH3 fasc domain 4. This study also confirms the mutation hot spot at positions R124 and R555 with nearly 50% of the mutations targeting these two amino acids (24/50). In addition the corneal phenotypes induced by changes at R124 and R555 are amino acid specific: R124C in CDLI, R555W and R124S in CDGGI, R124H in CDA, R124L in CRRB, and R555Q in CDTB. In CDLIIIA, CDLI/IIIA, and CDL-deep the genotype-phenotype correlation is domain specific, with all changes occurring at the boundary or within the fasc4 domain.