Autosomal dominant zonular cataract with sutural opacities is associated with a splice mutation in the betaA3/A1-crystallin gene.

PURPOSE: Congenital cataracts constitute a morphologically and genetically heterogeneous group of diseases that are a major cause of childhood blindness. Autosomal Dominant Zonular Cataracts with Sutural Opacities (CCZS) have been mapped to chromosome 17q11-q12 near the betaA3A1-crystallin gene (CRYBA1). The betaA3A1-crystallin gene was investigated as the causative gene for the cataracts. METHODS: The betaA3/A1-crystallin gene was sequenced in affected and control individuals. Base changes were confirmed and assayed in additional family members and controls using NlaIII restriction digestion of PCR amplified DNA sequences. Base changes were assessed for their effects on splicing by information analysis. RESULTS: The cataracts are associated with a sequence change in the 5' (donor) splice site of intron 3: GC(g->a)tgagt. The sequence change also creates a new NlaIII site. This base change cosegregates with the cataracts in this family, being present in every affected individual. Conversely, this base change was not seen in 140 chromosomes examined in 70 unaffected and unrelated individuals. Information theory mutational analysis shows that the base change lowers the information content of the splice site from 6.0 to -6.8 bits, so that splicing would not be expected to occur at the altered site. CONCLUSIONS: Taken together, these observations suggest that the observed mutation might be causally related to the cataracts in this family.

Familial subepithelial corneal amyloidosis (gelatinous drop-like corneal dystrophy): exclusion of linkage to lactoferrin gene.

PURPOSE: Because corneal tissue with familial subepithelial corneal amyloidosis (FSCA; gelatinous drop-like dystrophy of the cornea) contains lactoferrin the possibility that the FSCA gene was the human lactoferrin (hLF) gene was investigated. Due to contradictory published information we also mapped the hLF gene. METHODS: We mapped the hLF gene using a genomic clone of the entire hLF gene as a probe by fluorescence in situ hybridization (FISH). Utilizing PCR primers that are specific to the hLF gene, we also mapped the hLF via radiation somatic cell hybrid analysis. Linkage of the FSCA gene to the hLF gene was evaluated by genetic linkage analysis using polymorphic markers within and in the vicinity of the hLF gene. RESULTS: The hLF gene mapped to the short arm of chromosome 3 at 3p21. Linkage analysis using polymorphic markers for hLF and haplotype analysis of the 3p21 loci indicates that the FSCA gene is not linked to the 3p21 locus. CONCLUSIONS: The gene for FSCA is not the hLF gene in these families.

Autosomal dominant zonular cataract with sutural opacities in a four-generation family.

PURPOSE: We identified and examined four generations of a family with coexisting autosomal dominant zonular cataracts and sutural opacities and sought to determine their genetic basis. METHODS: Twenty-four of the 48 members in the family were examined. Systemic and ocular histories were obtained, and a detailed ophthalmic examination was performed. From each individual, 20 ml of blood was drawn for linkage studies with microsatellite markers in regions to which zonular cataracts had previously been localized (chromosomes 1, 2, and 16). RESULTS: Individuals of the first generation were reportedly asymptomatic. Several members of the second generation had morphologically identical zonular cataracts. Affected members of the third generation showed morphologic heterogeneity, with the zonular opacity varying from a uniform lamella to a segregation of dots. A high degree of consanguinity in the second generation suggested recessive inheritance with a pseudodominant inheritance pattern. However, examination of one member of the asymptomatic first generation disclosed senile cataractous changes superimposed on a faint zonular cataract enclosing sutural opacities and a pulverulent fetal nucleus. The latter findings were reconfirmed to be present in affected members of all generations, suggesting an autosomal dominant mode of inheritance. Initial efforts at linkage analysis excluded the gene locus causing this cataract from the Duffy, haptoglobin, and gamma-crystallin regions. CONCLUSIONS: The cataract in this family is both phenotypically and genetically distinct from previously described and mapped cataracts.

Computerized topography of selective versus all-suture release to manage high astigmatism after cataract surgery.

PURPOSE: To compare the efficacy of selective suture release (SSR) with all-suture release (ASR) in controlling corneal astigmatism after cataract surgery. SETTING: Sight Saver's Cornea Training Centre, L.V. Prasad Eye Institute, Hyderabad, India. METHODS: This prospective, randomized study evaluated the effect on astigmatism of two techniques of suture release in 30 patients with more than 3.00 diopters (D) of corneal astigmatism after cataract surgery. All patients had interrupted sutures with well-healed wounds. Fifteen patients had ASR irrespective of the location of the steep meridian. In the other 15, only the suture located in the steep meridian was selectively released. The pattern of decay of astigmatism after suture release was studied using computerized videokeratography. RESULTS: Mean pretreatment corneal cylinder was 6.30 D +/- 2.72 (SD) in the ASR group and 6.95 +/- 1.67 D in the SSR group. In the ASR group, corneal cylinder dropped to 3.70 +/- 1.15 D immediately after suture release and further decreased to 1.82 +/- 0.66 D at 1 week (P < .001). In the SSR group, astigmatism swung erratically to the adjoining sutures and decreased unpredictably at an average of 1.32 +/- 2.00 D with each suture release. CONCLUSION: The ASR technique was more predictable and less cumbersome than the SSR method.

Globe rupture during digital massage after peribulbar anesthesia.

A 75-year-old woman developed globe rupture with extrusion of intraocular contents during digital massage after periocular injection of an anesthetic. The rupture was repaired. At the last follow-up 6 months after surgery, visual acuity was 20/160. The retina was attached, and the vitreous hemorrhage was resolving. This complication of peribulbar anesthesia and digital massage highlights the importance of using quantitative techniques to induce hypotony during peribulbar anesthesia.

Secondary capsule-supported intraocular lens implantation in children.

PURPOSE: To evaluate surgical problems, postoperative complications, and visual results of secondary posterior chamber intraocular lens (IOL) implantation in children. SETTING: L.V. Prasad Eye Institute, Hyderabad, India. METHODS: A retrospective study was done of secondary IOL implantation in 27 children (35 eyes) who were not satisfied with aphakic glasses and were intolerant of or reluctant to use contact lenses. The extent of posterior capsular support was assessed prior to surgery. Additional surgical procedures were posterior synechiolysis (11 eyes), anterior vitrectomy (8 eyes), pupilloplasty (2 eyes), and membranectomy (2 eyes). RESULTS: Postoperative complications included wound leak (1 eye), uveitis (5 eyes), peripheral anterior synechias (2 eyes), and retinal detachment (1 eye). Visual acuity improved or remained at the preoperative level in 34 eyes. CONCLUSION: Secondary posterior chamber IOL implantation is an effective optical modality for managing pediatric aphakia. Observation must continue to determine the long-term safety of the procedure.

Effect of two anterior capsulotomy techniques on the corneal endothelium.

We performed a prospective, randomized, clinical study to assess the effect of two anterior capsulotomy techniques on the corneal endothelium. Specular microscopy was done preoperatively and postoperatively in 57 patients having manual extracapsular cataract surgery with a can-opener capsulotomy or with a retained anterior capsule flap. The mean percentage of cell loss following the can-opener capsulotomy was 0.43 (P = .22). There was no statistically significant alteration in endothelial morphology after surgery. The cell loss with a retained anterior capsule flap was zero. However, there was a statistically significant difference in polymegathism (P = .024) and pleomorphism (P = .045) after surgery. A comparison of the two techniques showed no statistically significant difference in cell loss or in pleomorphism. The statistically significant difference in polymegathism (P = .01) was not clinically significant. Our findings indicate that, when carefully performed, both methods of anterior capsulotomy for manual extracapsular cataract surgery are safe for the corneal endothelium.

Capsular bag distension after optic capture of a sulcus-fixated intraocular lens.

Capsular bag distension from occlusion of the circular anterior capsule opening by the intraocular lens (IOL) optic is a rare complication of capsulorhexis. The nature and source of fluid remain unclear. We report a case of capsular bag distension in an eye with an optic-captured, all-poly(methyl methacrylate), sulcus-fixated IOL. The fluid behind the IOL optic was visible on the first postoperative day and resolved spontaneously by the sixth postoperative week. We believe that retained viscoelastic material in the capsular bag was a major contributor to the fluid.

Outside-in transscleral fixation for ciliary sulcus intraocular lens placement.

We describe a new technique of transscleral intraocular lens fixation in the absence of adequate posterior capsular support. This technique of suture placement within the ciliary sulcus employs routinely used instruments, requires minimum manipulation, and has good visual outcome and negligible complications. We performed five intraocular lens implantations using the technique with good visual outcomes in each. No significant intraoperative or postoperative complications were noted.

Experience during the learning curve of laser in situ keratomileusis.

PURPOSE: To identify pitfalls and offer hints on achieving a successful outcome during the early laser in situ keratomileusis (LASIK) learning process. SETTING: Gimbel Eye Centre, Calgary, Alberta, Canada. METHODS: This was a retrospective review of the preoperative planning, surgical procedure, intraoperative and postoperative problems, and early postoperative anatomic and refractive results in the first 73 eyes that had LASIK. RESULTS: Intraoperative complications included failure of the keratome to make a cut, excessively thin cap, repositioning difficulty, and inadequate intraocular pressure elevation. Early postoperative complications included excessive central and peripheral wrinkling of the cap, peripheral lipid deposits, and central edema of the cap. One month postoperatively, mean spherical equivalent refraction was -0.90 diopters (D) (range +1.75 to -6.00 D), and 45 eyes had a best corrected visual acuity between 20/15 and 20/40. CONCLUSION: Our retrospective review of the problems experienced during the early LASIK learning process should help novice lamellar refractive surgeons avoid such problems and shorten the learning curve.

Prospective evaluation of corneal endothelial cell loss after pediatric cataract surgery.

PURPOSE: To study the alterations in endothelial cell count and morphology after pediatric cataract surgery using currently practiced techniques. SETTING: L.V. Prasad Eye Institute, Hyderabad, India. METHODS: In a prospective nonrandomized series comprising 20 eyes of 14 children with congenital or developmental cataract, endothelial cell loss from cataract surgery was evaluated. Mean patient age was 9.3 years (range 5 to 15 years). Extracapsular cataract extraction (ECCE) with intraocular lens (IOL) implantation was performed in 11 eyes (Group 1). Primary posterior capsulotomy and anterior vitrectomy were performed with ECCE and IOL implantation in 9 eyes (Group 2). Noncontact specular microscopy was done preoperatively and 6 to 8 and 24 to 36 weeks postoperatively. Endothelial cell loss, alteration in the coefficient of variation, and the change in the number of hexagonal cells were determined by semiautomated analysis of endothelial pictures. RESULTS: Mean endothelial cell loss was 198.39 cells/mm2 (5.28%) in Group 1 and 295.17 cells/mm2 (7.50%) in Group 2 at 24 to 36 weeks. There was no statistically significant difference in alteration in endothelial cell count and morphology between the 2 groups. CONCLUSIONS: The results suggest that endothelial cell loss with currently practiced techniques of pediatric cataract surgery is within acceptable limits.

Silicone oil adhesion to intraocular lenses: an experimental study comparing various biomaterials.

PURPOSE: To perform an in vitro experimental study comparing the degree of adherence of silicone oil to various rigid and foldable intraocular lens (IOL) designs and to the human lens capsule. SETTING: Center for Research on Ocular Therapeutics and Biodevices, Department of Ophthalmology, Storm Eye Institute, Medical University of South Carolina, Charleston, South Carolina, USA. METHODS: Seven IOL styles comprising various biomaterials were studied: fluorine-treated (Fluorlens), heparin-surface-modified (HSM), hydrogel, Memory-Lens, Poly(methyl methacrylate) (PMMA), soft acrylic, and silicone lenses; the human crystalline lens was also studied. Each lens was immersed in silicone oil for 12 hours, than photographed, studied by scanning electron microscopy (except the crystalline lens), and subjected to computer-generated image analysis to determine the silicone oil coverage. RESULTS: Silicone oil coverage of dry silicone lenses was 100% and of lenses immersed in normal saline, 82.5%. The least coverage was on the heparin-surface-modified lens (mean score 9.4%). Coverage of the other four lenses ranged from approximately 15.1% to 33.7%. Mean coverage of the human lens capsule was 10.9%. CONCLUSION: Although a silicone IOL shows maximal adherence to silicone oil, other lens biomaterials are not immune to this complication. Silicone oil coverage was related to the dispersive energy component of the surface charge of the IOL biomaterial. Low dispersive energy materials had less silicone oil coverage, while those with higher dispersive energy had more oil coverage.

Heparin-surface-modified intraocular lenses in pediatric cataract surgery: prospective randomized study.

PURPOSE: To evaluate the performance of heparin-surface-modified (HSM) intraocular lenses (IOLs) in pediatric eyes after cataract surgery. SETTING: L.V. Prasad Eye Institute, Hyderabad, India. METHODS: This prospective, randomized, double-masked, controlled clinical trial comprised 90 children aged 2 to 14 years with cataract. The patients were consecutively randomized to receive an HSM (Group 1) or an unmodified (Group 2) poly(methyl methacrylate) (PMMA) IOL. Extracapsular cataract extraction (ECCE) with IOL implantation was performed in children 8 years and older and ECCE with primary posterior capsulotomy, anterior vitrectomy, and IOL implantation in children younger than 8 years. Outcome parameters were inflammatory cell deposits on the IOL surface, posterior synechias, and anterior chamber reaction. RESULTS: Follow-up data were available for 73, 70, 60, and 68 patients at 1 week, 1 month, 3 months, and 6 months, respectively. Significantly fewer cell deposits were noted in Group 1 at 1, 3, and 6 months (P < .001). Synechia formation and anterior chamber reaction were comparable in the 2 groups. CONCLUSION: The lower incidence of inflammatory cell deposit formation in eyes with HSM PMMA IOLs indicates that these IOLs have greater bicompatibility than unmodified IOLs in pediatric cataract surgery.

Nocardial necrotizing scleritis after trauma. Successful outcome using cefazolin.

Nocardial necrotizing scleritis is uncommon, but potentially sight threatening. We report the occurrence of necrotizing scleritis in a 58-year-old farm worker after he sustained trauma with vegetable matter. Microbiological studies isolated Nocardia asteroides, sensitive only to cefazolin. Clinically the patient responded well to this drug, attaining a final visual acuity of 20/40, with complete resolution of the scleritis. Early isolation and testing for antibiotic sensitivity were responsible for the successful management of this patient.

Use of ultrasound biomicroscopic evaluation in preoperative planning of penetrating keratoplasty.

PURPOSE: To evaluate the use of ultrasound biomicroscopy (UBM) in examining the status of anterior-segment structures in eyes with corneal opacification. METHODS: Evaluation with UBM was performed between 1 and 2 weeks before surgery in 21 patients (21 eyes) with corneal opacities who were scheduled for penetrating keratoplasty. UBM findings were correlated with the findings noted during penetrating keratoplasty. The efficacy of UBM evaluation was calculated in terms of positive and negative predictive values (PPVs and NPVs) for the presence or absence of each finding. RESULTS: Seventeen eyes had either aphakic or pseudophakic bullous keratopathy (ABK or PBK). UBM findings of anterior synechiae, peripheral anterior synechiae, posterior synechiae, peripheral and sector iridectomies positively correlated with those observed during surgery in five (55.6%) of nine locations, 19 (73.1%) of 26 locations, 12 (80%) of 15 locations, eight (88.9%) of nine locations) and six (85.7%) of seven locations, respectively. The NPVs for these findings were 100%, six (54.5%) of 11 locations), 10 (71.4%) of 14 locations, 12 (100%) of 12 locations, and 14 (100%) of 14 locations, respectively. Correlation for findings pertaining to the posterior capsule (PC) were presence of PC/PC remnants [PPV, 13 (92.9%) of 14 locations; NPV, two (66.7%) of three locations], presence of central PC tears [PPV, two (66.7%) of three locations], intactness of PC [PPV, six (85.7%) of seven locations], and PC thickening [PPV, 13 (81.25%) of 16 locations; NPV, four (100%) of four locations]. For vitreous in the anterior chamber, the PPV was eight (100%) of eight locations, and NPV was 12 (92.3%) of 13 locations. Pseudophakos-related information in eyes with PBK showed a PPV of 100% for presence, centration, and anatomic location (seven of seven, four of four, and seven of seven locations, respectively). CONCLUSION: In eyes with corneal opacity, UBM determines the status of various anterior-segment structures. It is thus a useful adjunct in the preoperative planning and prognostication of patients requiring penetrating keratoplasty.

Co-occurring adenoviral keratitis and hemorrhagic cystitis. A case report.

Adenoviruses are ubiquitous viruses, commonly causing ocular infections. They are also known to cause multisystem infections. External ocular infections caused by adenovirus are most often due to contaminated secretions. We report a patient in whom genitourinary symptoms developed and subsided along with ocular symptoms. Adenovirus was isolated on ocular culture. This is the first reported case of concurrent adenoviral keratitis and hemorrhagic cystitis. Eliciting a history of genitourinary and respiratory symptoms is important in patients with ocular adenoviral infections.

Results of sutureless wound construction in children undergoing cataract extraction.

A prospective study was performed to evaluate the efficacy of self-sealing (sutureless) wound construction for cataract surgery in children. Self-sealing wounds were constructed during cataract surgery in 25 eyes of 24 children between 7 and 16 years of age. Absence of wound leak at the conclusion of surgery was taken as indicating a secure wound. Watertight wounds were achieved in 19 eyes, 11 of those in children older than 11. Six eyes demonstrated wound leak, and sutures had to be applied to achieve a secure wound. All wound leaks occurred in children younger than 11. Patients younger than 11 who underwent primary posterior capsulotomy and anterior vitrectomy had a significantly higher incidence of wound leak after sutureless cataract surgery (P < .001). We conclude that the self-sealing wound probably should not be used for cataract surgery in children younger than 11.

Physical and mechanical principles of phacoemulsification and their clinical relevance.

A clear understanding of the physical and mechanical principles that govern phacoemulsification can facilitate usage of this technique for effective and efficient cataract removal in a variety of clinical situations. This article addresses separately, concepts pertaining to the three essential components of phacoemulsification, namely, irrigation, aspiration and emulsification. Machine settings are suggested for the various techniques presently in use. Finally, alternative approaches for lens removal that are currently being investigated are briefly discussed.

Conjunctival transplantation for corneal surface reconstruction--case reports and review of literature.

Corneal persistent epithelial defects (PED) can occur due to various causes. In diffuse ocular surface disease, they often occur secondary to depletion of limbal stem cells. A number of complications occur secondary to PED and successful treatment usually requires conjunctival surgeries for corneal surface reconstruction. We report two cases of chemical injury successfully treated by two such procedures. This report highlights the encouraging results of limbal transplantation and reviews the literature in the management of PED with limbal stem cell loss.

Principles and paradigms of pediatric cataract management.

Propensity for increased postoperative inflammation and capsular opacification, a refractive state that is constantly in a state of flux due to growth of the eye, difficulty in documenting anatomic and refractive changes due to poor compliance, and a tendency to develop amblyopia, makes management of cataract in the child different from that in the adult. The recent past has unraveled several caveats of pediatric cataract management-the importance of atraumatic surgery and complete removal of lens matter, benefits of in-the-bag intraocular lens (IOL) implantation, role of titrating IOL power to counter refractive changes due to growth of the eye, prudery of continuously following these eyes for early detection of aphakic glaucoma and benefits of some surgical innovations. Although these promise to significantly improve our management of pediatric cataract, their long-term benefits are yet to be determined. We will also have to harness newer techniques, especially in the areas of wound construction and capsule management, and will have to develop effective strategies for the refractive management of infantile aphakia.