Outcomes of lensectomy with glued intraocular lens and factors associated with failure in eyes with spherophakia and glaucoma

Purpose: To evaluate the outcomes of lensectomy with a glued intraocular lens (IOL) in spherophakic eyes with secondary glaucoma and assess factors associated with failure. Methods: We prospectively evaluated outcomes of lensectomy with glued IOL in 19 eyes with spherophakia and secondary glaucoma (intraocular pressure (IOP) ?22 mm Hg and/or glaucomatous optic disc damage) between 2016 and 2018. The vision, refractive error, IOP, antiglaucoma medications (AGMs), optic disc changes, need for glaucoma surgery, and complications were assessed. Success was defined as complete when IOP was ?5 and ?21 mmHg without AGMs; qualified success as similar IOP with up to 3 AGM; the need for >3AGM/additional surgery for IOP control was considered a failure. Results: Preoperatively, the median (interquartile range: IQR) age was 18 (13.5–30) years. IOP was 16 (14–22.5) mmHg on a median of 3 (2,3) AGMs. Median postoperative follow up was 27.7 months (11.9, 39.7). Postsurgery, most patients achieved emmetropia, with significantly decreased refractive error from a median spherical equivalent of ?12.5D to + 0.5D, P < 0.0002. The complete success probability was 47% (95% confidence intervals (CIs): 29–76%) at 3 months and was 21% (8 ? 50%) at 1 year and 3 years. The qualified success probability was 93% (82–100%) at 1 year, which reduced to 79% (60–100%) in 3 years. None of the eyes had any retinal complications. The higher number of preoperative AGM was found to be a significant risk factor (p < 0.02) for the failure of complete success. Conclusion: One?third of the eyes had IOP control without the need for AGM postlensectomy with glued IOL. Surgery resulted in significant improvement in visual acuity. The higher number of preoperative AGM was associated with poor glaucoma control after glued IOL surgery

Kissing microvitreoretinal blade technique: A novel approach for safe and effective endocapsular lens aspiration in microspherophakia

We herein describe the kissing MVR technique in cases of microspherophakia for safe and effective endocapsular lens aspiration. Microspherophakia is associated with abnormally lax and broken zonules, which pose a great challenge to the operating surgeon, especially while creating the openings in the capsular bag. In this novel technique, simultaneous use of two 23 G MVR blades reduces the above problem associated with the severely mobile lens. Here, one MVR blade stabilizes the capsular bag and, at the same time, provides counter-traction for the opposite MVR blade while puncturing the capsular bag. Furthermore, the creation of openings in the lens at the equator or just posterior to it is beneficial as the capsule is relatively thicker and stronger at this location. This also minimizes the risk of premature extension to the anterior or posterior capsule, thereby avoiding complications like dropped lens matter, vitreous prolapse, and vitreous traction.

Outcomes of surgical intervention in cases of ectopia lentis

Purpose: To evaluate the outcomes of surgical intervention in cases of ectopia lentis. Methods: This retrospective study included all cases of ectopia lentis that presented between June 2015 and March 2019 in a tertiary care center. They were reviewed retrospectively. The corrected distance visual acuity (CDVA), severity of lens subluxation, type of surgery, intra?operative and post?operative complication, and specular count were recorded. Results: Seventy?eight eyes of 57 cases with a mean age at surgery of 14.73 years were analyzed. Intra?lenticular lens aspiration was the most common (n?62/78; 79.5%) surgical procedure followed by lens aspiration, intra?capsular cataract extraction, phaco?aspiration, and pars?plana lensectomy. Simultaneous intra?ocular lens (IOL) implantation was performed in 46.2% (n?32/78) of the eyes. The mean CDVA improved from 0.85 ± 0.55 logMAR to 0.44 ± 0.29 logMAR at 6 weeks follow?up. The post?operative CDVA was significantly better in the pseudo?phakic group compared to the aphakic group (p?0.02). The patient’s age at the time of surgery and the degree of subluxation did not impact the final visual outcome. Intra?operative complication included vitreous hemorrhage (n?1) and lens matter drop (n?1). Post?operative complications were noted in 26.9% of the eyes (n?21/78) with a higher complication rate in the pseudo?phakic group (p?0.00). A second intervention was required in 7.7% of the eyes (n?6/78). Conclusion: Age and degree of subluxation at the time of surgery do not influence the final visual outcome in cases of ectopia lentis undergoing lens extraction surgery. IOL implantation results in better visual outcomes but is associated with a high complication rate.

Retropupillary iris claw lens versus Gore-Tex assisted scleral fixated intraocular lens in children with large lens subluxations

Purpose: To compare the functional outcome of retropupillary iris claw lenses (RPIC?IOL) and scleral fixated intraocular lenses (SFIOL) in children with large lens subluxations. Methods: Sixty eyes of patients between 6 and 18 years of age having ?7 clock hour lens subluxation were included and equally divided into group A (RPIC?IOL implantation) and group B (Gore?Tex sutured SFIOL implantation). Cases with anterior and posterior segment abnormalities, trauma and glaucoma were excluded. Primary outcome was improvement in best?corrected visual acuity (BCVA) at 1.5 years. Secondary outcomes were assessment of intraocular lens (IOL) tilt, mean change in astigmatism at 1.5 years, and median operating time. All surgeries were performed by the same surgeon. Results: The mean improvement in BCVA in group A was 0.28 ± 0.41 logMAR and group B was 0.44 ± 0.45 logMAR (P = 0.3). Significant IOL tilt was seen in 4 eyes in group A (13.33%) and 5 eyes in group B (16.66%) (P = 0.120). Mean change in astigmatism was 4.38 ± 5.9D in group A and 4.91 ± 4.4D in group B (P = 0.299). The median operating time was 40 min in group A and 90 min in group B (P < 0.001). No significant posterior segment complications were seen in either technique. Conclusion: Both procedures had comparable visual outcomes. RPIC?IOL implantation was relatively quick and comparatively easier; it may be preferred in cases with high risk of retinal detachment.

Clinical profile and outcome of ocular manifestation in Marfans syndrome in India

Purpose: Marfan syndrome (MFS) is a genetic disorder associated with considerable morbidity and mortality. Presently, well?documented information on this condition is not available in India. Methods: In this retrospective cohort study, we recruited patients with clinically diagnosed MFS who presented to the outpatient department using revised Ghent nosology. We retrieved complete ophthalmic information, including vision, anterior and posterior segments, exported from electronic medical records, and relevant investigations, surgical details, and follow?up data were obtained in a specific, pretested format. Results: Our cohort consisted of 86 eyes of 43 patients and had a male preponderance. The prevalence was 20.5 per 100,000 individuals. The mean age of the patients was 23.9 years. All eyes were treated either optically with refraction or surgically using lensectomy and vitrectomy with suture supported scleral fixated intraocular lens (IOL), which significantly affected the visual outcome (P = 0.000). Conclusion: Although considered a rare disease, MFS is commonly found in the ophthalmological setting. Refraction and surgery (lensectomy with scleral fixated IOL) may improve the vision significantly

Peripheral iridectomy for preventing iris-related complications in glued intraocular lens surgery in children

Purpose: To assess the role of surgical peripheral iridectomy (PI) in preventing iris-related complications associated with glued intraocular lens (GIOL) surgery in children with bilateral ectopia lentis. Methods: Nonrandomized interventional case series of 34 eyes of 17 children (<15 years of age) who underwent pars plana lensectomy (PPL) and GIOL surgery between January 2013 and December 2016. Eyes with surgical PI (January 2013–June 2015) were compared with those without surgical PI (July 2015–December 2016). The primary outcome measure of the role of surgical PI in GIOL surgery was to account for complications such as optic capture, secondary glaucoma, intraocular lens (IOL) dislocation, or repeat surgery. The secondary outcomes were changes in the best-corrected visual acuity (BCVA). Results: The mean age at surgery was 8.8 years (range: 3.5–15 years). Surgical PI was conducted in 15 eyes. Among the 19 eyes without PI, 9 eyes had complications (optic capture –6; rise in IOP –4; IOL subluxation –4; repeat surgery –5). The complications were significantly less in the PI group, P = 0.02. There was a statistically significant improvement in BCVA (P = 0.0001) in all the patients. The mean presenting BCVA was 0.99 (±0.79) logMAR (Snellen ? 20/200) and post BCVA was 0.40 (±0.50) (Snellen ? 20/50). The mean preoperative refraction was ? 9 D (±8D) (range: ?5 D to ?23D) and postoperative was ?1 (±1.15) D. The mean follow-up was 25.4 months. Conclusion: Surgical PI along with GIOL surgery in children undergoing PPL is shown to reduce optic-capture-related complications.

Bimanual irrigation-aspiration for ectopia lentis and use of a small incision for 4-point scleral-sutured foldable intraocular lens and anterior vitrectomy in patients with Marfan syndrome

Purpose: This study evaluated bimanual intracapsular irrigation-aspiration for ectopia lentis with use of a small incision for 4-point scleral fixation of a foldable posterior-chamber intraocular lens (IOL) and anterior vitrectomy in patients with Marfan syndrome. Methods: We performed a retrospective study of 18 eyes from 10 patients with Marfan syndrome who underwent surgical intervention for ectopia lentis at our clinic between July 2012 and September 2018. In this study, intraoperative and postoperative complications, uncorrected visual acuity, best-corrected visual acuity, spherical equivalent, intraocular pressure, and endothelial cell density were evaluated. Results: No intraoperative complications were reported. In all cases, early postoperative evaluation revealed a clear cornea, round pupil, and well-centered IOL. Mean logMAR uncorrected visual acuity improved from 1.09 preoperatively to 0.56 postoperatively (P < 0.05). Mean logMAR best-corrected visual acuity improved from 0.45 preoperatively to 0.17 postoperatively (P < 0.05). Aside from transient ocular hypertension, no postoperative complications were reported. Conclusion: The combined surgical technique presented above yields excellent visual outcomes with an extremely low incidence of complications. This approach is simple, safe, and effective in the treatment of ectopia lentis in patients with Marfan syndrome.

La ectopia lentis y las soluciones quirúrgicas en la cirugía de catarata / Ectopia lentis and surgical solutions in cataract surgery

RESUMEN La extracción del cristalino en pacientes con ectopia lentis se convierte en una cirugía desafiante para el cirujano. En el pasado surgieron serias complicaciones asociadas a esta intervención quirúrgica, como desprendimiento de retina y glaucoma. El avance de las técnicas quirúrgicas en las últimas décadas ha brindado mayor seguridad para la cirugía de esta afección del cristalino. Se presenta una paciente femenina de 47 años aquejada de disminución de la visión. Al examen oftalmológico se encuentra subluxación superior del cristalino bilateral. Previa discusión con la paciente de los posibles escenarios a presentarse durante la cirugía, se le sugiere una facoemulsificación bilateral secuencial del cristalino conservando el saco capsular mediante la colocación de un anillo de tensión capsular fijado a la esclera más implante de lente intraocular plegable en el saco capsular. Se realiza primeramente cirugía del ojo izquierdo y se presenta un desgarro radial de la rexis con extensión posterior, por lo que se varía el abordaje quirúrgico y se fija el implante al iris. Un mes después se realiza cirugía del ojo derecho siguiendo el plan quirúrgico inicial sin complicaciones. La recuperación visual fue satisfactoria (0,7 en cartilla de Snellen) en ambos ojos y se demostró la efectividad de ambas alternativas quirúrgicas. La planificación correcta de la técnica quirúrgica y de las posibles modificaciones ante circunstancias sobrevenidas, así como la oportuna información preoperatoria y la experiencia del cirujano resultan fundamentales para alcanzar el éxito quirúrgico, la recuperación funcional y la satisfacción del paciente(AU)

ABSTRACT Crystalline lens removal in patients with ectopia lentis is a challenge for eye surgeons. Serious complications, such as retinal detachment and glaucoma, were associated in the past to this surgical procedure. However, the progress in surgical techniques achieved in recent decades provides greater safety in the surgical treatment of this lens disorder. A female 47-year-old patient attends consultation complaining of vision reduction. Ophthalmological examination reveals bilateral crystalline lens upper subluxation. The possible scenarios to occur during surgery are discussed with the patient, and it is suggested that she undergoes sequential bilateral lens phacoemulsification preserving the capsular bag with the placement of a capsular tension ring fixed to the sclera plus implantation of a foldable intraocular lens in the capsular bag. The first eye to be operated on is the left eye, and radial tear of the rhexis with posterior extension occurs. Therefore, the surgical approach is changed and the implant is fixed to the iris. Surgery of the right eye is performed one month later, following the initial surgical plan without any complications. Visual recovery was satisfactory (0.7 by the Snellen chart) in both eyes, proving the effectiveness of both surgical alternatives. Correct planning of the surgical technique and its possible modifications in response to unexpected circumstances, alongside timely preoperative information and the surgeon's experience, are all crucial to achieve surgical success, functional recovery and patient satisfaction(AU)

Iris-claw intraocular lens implantation in children with ectopia lentis / Implante de lente intraocular de fixação iriana em crianças com ectopia lentis

ABSTRACT Purpose: Artisan iris-claw lens implantation (AICLI) is a surgical technique for treating ectopia lentis. We aimed to compare visual outcomes and possible long-term complications of AICLI surgery in pediatric patients with ectopia lentis with or without a diagnosable hereditary disease. Methods: Seventeen children with non-traumatic ectopia lentis were retros pectively classified into two groups: group 1 included children with a diagnosable hereditary disease (11 patients, 65%), and group 2 included children without any definable hereditary disease (six patients, 35%). Patients were evaluated for post-surgical refraction, best-corrected visual acuity, and clinical follow-up complications. Results: The average follow-up time was 38 months, and the average age of the patients was 103 ± 53 months (30-196 months). Best-corrected visual acuity values were significantly increased in both groups after surgery (p<0.05). Target refraction values were achieved at a rate of 47% in group 1 and 22% in group 2. Post-surgery complications, such as lens dislocation (36%, 11 eyes of 10 patients) and hypotonia (10%, three eyes of three patients) were observed in both groups, and retinal detachments (10%, three eyes of three patients) were observed in three patients from group 1. Conclusions: Compared with previous similar studies, this study utilized the largest pediatric patient group and had the longest post-surgery follow-up time. Moreover, it is advisable that pediatric patients with non-traumatic ectopia lentis be carefully screened for any underlying hereditary disease, especially diseases related to connective tissue metabolism.

RESUMO Objetivo: A implantação de lentes intraoculares de fixação iriana em garra (AICLI) é uma técnica cirúrgica para o tratamento de ectopia lentis. Nosso objetivo foi comparar resultados visuais e possíveis complicações em longo prazo da cirurgia de AICLI em pacientes pediátricos com ectopia lentis com ou sem doença hereditária diagnosticável. Métodos: Dezessete crianças com ectopia lentis não-traumática foram classificadas retrospectivamente em dois grupos: o grupo 1 com pacientes apresentando doença hereditária diagnosticável (11 pacientes, 65%) e o grupo 2 com pacientes sem qualquer doença hereditária definível (6 pacientes, 35%). Os pacientes foram avaliados quanto à sua refração pós-operatória, acuidade visual melhor corrigida e complicações. Resultados: O tempo médio de seguimento foi 38 meses. A média de idade dos pacientes foi de 103 ± 53 meses (30-196 meses). Os valores de acuidade visual me lhor corrigida aumentaram significativamente em ambos os grupos (p<0,05). Os valores de refração alvo foram alcançados a uma taxa de 47% no grupo 1 e 22% no grupo 2. Complicações pós-operatórias como luxação da lente (36%, 11 olhos de 10 pacientes) e hipotonia (10%, 3 olhos de 3 pacientes) foram observados nos dois grupos e foram observados descolamentos de retina (10%, 3 olhos de 3 pacientes) em 3 pacientes do grupo 1. Conclusões: Em comparação com relatos anteriores na literatura, este estudo utilizou um grupo maior de pacientes pediátricos e tempo de seguimento pós-operatório mais longo. É aconselhável que pacientes pediátricos com ectopia lentis não-traumática sejam cuidadosamente selecionados em relação a doença subjacente hereditária, especialmente as doenças relacionadas com o metabolismo do tecido conjuntivo.

Bilateral Simple Ectopia Lentis Associated with FBN1 Gene Mutation

PURPOSE: To report a case of bilateral simple ectopia lentis associated with FBN1 gene mutation. CASE SUMMARY: A 47-year-old women presented with a one-month history of ocular pain and decreased visual acuity of the right eye. She had a family history of crystalline lens dislocation but showed no systemic abnormality or trauma history. Intraocular pressure was 45 mmHg in the right eye, which showed a myopic shift (−6.5 D). The crystalline lens of the right eye was subluxated to the anterior chamber, and the angle was closed. Phacoemulsification with scleral fixation of the posterior chamber intraocular lens of the right eye was performed. After that, best corrected visual acuity of the right eye was 1.0, and intraocular pressure was 15 mmHg. After 2 years, she presented with intermittent ocular pain and decreased visual acuity of the left eye. The crystalline lens of the left eye was subluxated to the anterior chamber. Phacoemulsification with scleral fixation of the posterior chamber intraocular lens of the left eye was performed. After that, intermittent ocular pain and visual acuity of the left eye were improved. Genetic testing confirmed an FBN1 gene mutation in the patient. CONCLUSIONS: A bilateral ectopia lentis patient without history of definite trauma should undergo complete systemic and ophthalmic examination to rule out accompanying disease, and a detailed family history should be collected. If hereditary ectopia lentis is suspected, genetic testing of probands and their family should be performed and will be helpful for genetic counseling and ophthalmic surveillance.

Clinical observation of 31 patients of congenital ectopia lentis treated with different operative manipulations / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To determine the optimal operation method and reduce operative complications by retrospective analysis of outcomes of different operative manipulation for patients with congenital ectopia lentis ( CEL) . ●METHODS: A retrospective study ranged from Jan. 1, 2010 to Jan. 1, 2015 was conducted and 31 CEL patients (57 eyes) treated with different operations were taken into this study. The postoperative outcome, operation related complications was analyzed in terms of different operative manipulations. ● RESULTS: The most common types of CEL are idiopathic and Marfan - related CEL. All eyes ( 98%) benefited from operation except 1 Marchesani syndrome eye concurrent with optic atrophy induced by glaucoma. A total of 3 eyes ( 5%) were treated by phacoemulsification combined with intraocular lens implantation; 8 eyes ( 14%) were treated by phacoemulsification, capsular tension ring implantation combined with intraocular lens implantation, among these 8 eyes, 1 eye was treated with capsular tension ring implantation combined with suture fixation; 39 eyes ( 68%) were treated by lens excision and anterior vitrectomy through corneal incision combined with intraocular lens implantation with suture fixation, among these 39 eyes, 2 eyes were treated with trabeculectomy , but not combined with intraocular lens implantation; 5 eyes ( 9%) were treated by intracapsular cataract extraction and anterior vitrectomy combined with intraocular lens implantation with suture fixation; 2 eyes ( 4%) were treated by lens excision and vitrectomy through pars plana combined with silicon oil injection. ● CONCLUSION: Almost all patients can obtain satisfactory outcomes through various operative manipulations. Phacoemulsification combined with intraocular lens implantation was the first choice for patients with lens dislocation range less than 90°. Phacoemulsification, capsular tension ring implantation combined with intraocular lens implantation was recommended for 90° - 180°, and lens excision and anterior vitrectomy through corneal incision combined with intraocular lens implantation with suture fixation was more suitable for lens dislocation range more than 180°. The appropriate operative methods for different types of CEL patients is the key to success.

Scleral- fixated intraocular lens implantation in microspherophakia.

Background: In microspherophakia, abnormal laxity of the lenticular zonules leads to development of a spherical lens and possible subluxation. We evaluated long-term results of lens removal with scleral-fi xated intraocular lens (SFIOL) implantation in microspherophakia. Materials and Methods: Case series. SF IOLs were implanted in four consecutive patients with bilateral microspherophakia (eight eyes [three with pupillary block and secondary glaucoma who underwent immediate surgery and fi ve with only subluxation who underwent elective surgery]). Post-operative best-corrected visual acuity (BCVA), intraocular pressure (IOP) and lens position were evaluated periodically from day 1 to 18 months. Results: All patients were females (mean age 28 ± 7.03 years). In group 1 eyes (three eyes that presented with pupillary block), the mean BCVA improved from 0.008 decimals (preoperative) to 0.50 decimals (fi nal post-operative visit); in group 2 eyes (the other fi ve eyes), the mean BCVA improved from 0.12 ± 0.21 decimals to 0.73 ± 0.14 decimals. The preoperative mean IOP (54.53 ± 7.33 mmHg) in group 1 eyes was signifi cantly (P = 0.03) higher than that (16 ± 4.30 mm Hg) in group 2 eyes. At fi nal post-operative visit, the mean IOP (11.67 ± 2.88 mmHg) in group 1 eyes was not signifi cantly diff erent from that in group 2 eyes (13.0 ± 3.08 mmHg). All SFIOLs were well- centred at the fi nal visit. None of the patients encountered any peroperative or postoperative complications. Conclusions: SFIOLs may be an option for surgical management of microspherophakia.

A tomografia de córnea e segmento anterior na propedêutica do exame complementar na avaliação de ectasia / Simplifying ectasia screening with corneal and anterior segment tomography

Descrevemos a importância da tomografia de córnea e segmento anterior na propedêutica do exame complementar na avaliação de ectasia. Esta descrição da interpretação clínica dos índices da tomografia de córnea e segmento anterior (Pentacam - Oculus, Wetzlar, Germany) neste relato de caso, demonstra a relevância de uma nova tecnologia na avaliação da córnea e segmento anterior na suspeita de ceratocone. O diagnóstico de ceratocone foi excluído pela análise dos índices tomográficos de ectasia. Detectou-se assimetria entre os lados nasal e temporal por meio da avaliação do mapa de profundidade de câmara anterior. No exame biomicroscópico sob midríase foi constatado subluxação do cristalino, sendo assim devemos estar atentos não somente aos índices tomográficos corneanos na avaliação do exame complementar e sim analisarmos outros dados importantes oferecidos pela tomografia de córnea e segmento anterior.

The methodology currently used for interpretation of the cornea and anterior segment tomography for the diagnosis of corneal ectasia. Description of the clinical interpretation of anterior segment tomography (Pentacam - Oculus, Wetzlar, Germany); case report of the ectopia lentis demonstrating the importance o evaluation of the cornea and anterior segment of the keratoconus. We excluded the disease, analyzing the tomographic analysis of rates of ectasia and observe an asymmetry between nasal and temporal sides in assessing the depth map of the anterior chamber. On the biomicroscopic examination in mydriasis, was found a ectopia lentis. We must be mindful not only to corneal tomography indices to evaluate a diagnostic test, but look at other important information provided by anterior segment tomography.

Ectopia lentis et pupillae / Ectopia lentis et pupillae

The Ectopia lentis et pupillae is a rare genetic syndrome, congenital, autosomal recessive with variable expression, characterized by ectopia of the lens and the pupil, usually bilateral and symmetrical, but without systemic manifestations. The pathogenesis of this anomaly is still unknown, but there are theories that the change is mesodermal, neuroectodermal, combined or mechanical. This article presents a clinical case of a patient with ectopia lentis et pupillae, describing its clinical and genetic aspects, secondary ocular complications and differential diagnosis.

A ectopia lentis et pupillae é uma síndrome genética rara, congênita, autossômica recessiva de expressividade variável, caracterizada por ectopia do cristalino e da pupila, geralmente bilateral e simétrica, sem associação com anormalidades sistêmicas. Sua patogênese ainda é desconhecida, porém se especula que haja alteração mesodérmica, neuroectodérmica, combinada ou mecânica. O artigo relata o caso clínico de um paciente portador de ectopia lentis et pupillae, descrevendo seus aspectos clínicos e genéticos, além de suas complicações secundárias e diagnósticos diferenciais.

Implantação de lente intraocular com uma alça amputada: proposta para o tratamento cirúrgico da subluxação do cristalino / Intraocular lens implantation with one loop haptic amputed: a new propose to the subluxation lens surgical treatment

OBJETIVO: Avaliar os resultados pós-operatórios da subluxação congênita do cristalino, corrigida por uma nova abordagem cirúrgica. MÉTODOS: Foram estudados 21 olhos de 13 pacientes, portadores de subluxação não traumática do cristalino submetidos à cirurgia na Fundação Altino Ventura, no período de abril de 1999 a abril de 2004. A idade média foi de 8,7 ± 5,4 anos, e o tempo médio de seguimento foi 21,5 ± 19,3 meses. Os pacientes foram submetidos à facoaspiração, implante do anel endocapsular e lente intraocular (LIO). Uma das alças da LIO foi amputada e apoiada sobre o anel, no interior do saco capsular, centralizando a LIO. RESULTADOS: Houve melhora da acuidade visual (AV) em todos os casos, e redução significante do equivalente esférico e componente esférico comparando-se a refração pré e pós-operatória (p<0,01). Não houve diferença entre o componente cilíndrico pré e pós-operatório (p=0,71). Opacificação da cápsula posterior do cristalino foi a complicação encontrada em 71,4 por cento dos casos. Foi realizada capsulotomia posterior precoce nestes pacientes sem intercorrências. CONCLUSÃO: Implante de LIO com uma alça amputada e apoiada sobre o anel endocapsular é uma opção no tratamento cirúrgico da subluxação congênita do cristalino, podendo-se observar centralização da LIO e melhora da AV no pós-operatório.

PURPOSE: To evaluate the postoperative results of congenital lens subluxation corrected by a new technique. METHODS: Retrospective chart review of 21 eyes of 13 patients with no traumatic lens subluxation who underwent surgery in Altino Ventura Foundation from April, 1999 to April, 2004. The mean age was 8.7 ± 5.4 years old, and the mean follow-up period was 21.5 ± 19.3 months. Patients underwent phacoaspiration, endocapsular ring and intraocular lens (IOL) implantation. The implanted IOL had one loop haptic excised and was supported above the ring, inside the capsular bag promoting intraocular lens centralization. RESULTS: Visual acuity improvement was observed in all cases. There was a significant reduction of the spherical equivalent and spherical component comparing the pre and postoperative refraction (p<0.01). There was no statistically significant difference between the pre and postoperative cylinder component (p=0.71). Posterior capsule opacification was a postoperative complication found in 71.4 percent of the cases. Early posterior capsulotomy was performed with no complications in these cases. CONCLUSION: Intraocular lens implantation with one loop haptic amputed and supported above the endocapsular ring is an option for the congenital lens subluxation surgical treatment, promoting lens centralization and postoperative visual acuity improvement.

Genotype/phenotype association in indian congenital aniridia.

The developmental birth eye disorder of iris is known as aniridia. Heterozygous PAX6 gene, which causes human aniridia and small eye in mice, is located on chromosome 11p13. The variability had been documented between the affected individuals within the families, is due to genotypic variation. Haploinsufficiency renders PAX6 allele non-functional or amorphic, however it presents hypomorphic or neomorphic alleles. India is not a well-studied ethnic group, hence the focus on congenital aniridia gene analysis supports the literature and the phenotypic association were analysed both in sporadic as well as familial. The consistent association of truncating PAX6 mutations with the phenotype is owing to non-sensemediated decay (NMD). It is presumed that the genetic impact of increased homozygosity and heterozygocity in Indian counter part arises as the consequence of consanguineous marriages. The real fact involved in congenital aniridia with other related phenotypes with PAX6 mutations are still controversial.

Transscleral IOL Fixation With Preserved Anterior Vitreous Face in Marfan Syndrome With Ectopia Lentis

PURPOSE: To evaluate the safety and efficacy of transscleral intraocular lens (IOL) fixation while preserving the anterior vitreous face in treating ectopia lentis of Marfan syndrome. METHODS: This study included six patients (12 eyes) who had undergone surgical intervention for ectopia lentis with or without lenticular opacity. We compared the best-corrected visual acuity (BCVA) before and after the surgery and evaluated perioperative complications. RESULTS: The mean age at the time of surgery was 18.2+/-10.7 years. The mean follow-up period was 11.2+/-7.1 months. Mean BCVA scores changed from 0.96+/-0.37 (LogMar Value) to 0.14+/-0.17 (LogMar Value). All patients showed more than two lines of improvement in visual acuity. In two eyes, pupillary capture was found. Medically controllable intraocular pressure elevation was found in three eyes. CONCLUSIONS: This study suggests that transscleral IOL fixation with a preserved anterior vitreous face can be a safe and effective technique in treating the ectopia lentis of Marfan syndrome.

Transscleral IOL Fixation With Preserved Anterior Vitreous Face in Marfan Syndrome With Ectopia Lentis

PURPOSE: To evaluate the safety and efficacy of transscleral intraocular lens (IOL) fixation while preserving the anterior vitreous face in treating ectopia lentis of Marfan syndrome. METHODS: This study included six patients (12 eyes) who had undergone surgical intervention for ectopia lentis with or without lenticular opacity. We compared the best-corrected visual acuity (BCVA) before and after the surgery and evaluated perioperative complications. RESULTS: The mean age at the time of surgery was 18.2+/-10.7 years. The mean follow-up period was 11.2+/-7.1 months. Mean BCVA scores changed from 0.96+/-0.37 (LogMar Value) to 0.14+/-0.17 (LogMar Value). All patients showed more than two lines of improvement in visual acuity. In two eyes, pupillary capture was found. Medically controllable intraocular pressure elevation was found in three eyes. CONCLUSIONS: This study suggests that transscleral IOL fixation with a preserved anterior vitreous face can be a safe and effective technique in treating the ectopia lentis of Marfan syndrome.

Long Term Results of the Lensectomy in Children with Ectopia Lentis

PURPOSE: To analyze the clinical features and the long term visual results of children with ectopia lentis after lensectomy. METHODS: Enrolled in this study were 79 eyes of 43 patients who were operated on and followed up for at least 3 years. We performed a retrospective analysis of the patient records. RESULTS: The mean age at diagnosis was 4.6 years, mean age at surgery was 5.7 years and average follow-up was 7.1 years. Of the 43 patients with bilateral ectopia lentis, 7 underwent monocular surgery. The mean age at diagnosis was higher and mean postoperative BCVA was significantly lower in the monocular surgery group than in the binocular surgery group. All eyes showed reduced hyperopia for 5 years postoperatively, but the reduction rate of the monocular operated eyes was significantly lower than that of the binocular operated eyes (P<0.05). No complication occurred during surgery and 1 eye (1.3%) with Marfan syndrome showed retinal detachment 9 years after surgery. CONCLUSIONS: Surgical treatment of ectopia lentis was safe and showed good visual outcomes after 7.1 years of follow-up. Nevertheless, consideration must be given to the possibility of amblyopia due to the difference of the degree of dislocation between the two eyes.