RESUMEN
PURPOSE: To describe the biometric and corneal characteristics of patients with Marfan Syndrome (MFS) and ectopia lentis. STUDY DESIGN: Observational, descriptive, prospective study. Subjects Individuals with MFS with ectopia lentis (EL). METHODS: Fourty-four eyes of 23 patients underwent Scheimpflug analysis using the Pentacam (Oculus, Wetzlar, Germany), axial length (AL) using the IOL master 700 (Carl Zeiss AG, Oberkochen, Germany), endothelial cell count (ECC) using the CEM-350 (NIDEK, Maihama, Japan) and corneal biomechanics evaluation with the Ocular Response Analyzer: ORA (Reichert Ophthalmic Instruments, Buffalo, New York, USA) and Corvis (Oculus, Wetzlar, Germany). Statistical analysis was performed using IBM SPSS Statistics 25.0. RESULTS: The direction of lens subluxation was most frequently supero-nasal 40.9% (18/44). Mean keratometry (Km) was 40.22±1.76 Diopters (D); mean corneal astigmatism was 1.68±0.83 D; total corneal aberrometric root mean square (RMS) was 2.237±0.795µm; higher-order aberrations (HOAs) RMS were 0.576±0.272µm; mean AL was 25.63±3.65mm; mean ECC was 3315±459cell/mm2; mean CBI was 0.13±0.24, mean TBI was 0.31±0.25, mean posterior elevation was 4.3±4.5µm; mean total corneal densitometry was 16.0±2.14 grayscale units (GSU). CONCLUSION: Increased axial length, flatter and thicker corneas with higher regular astigmatism, normal densitometry, normal corneal biomechanical indices and normal posterior elevation were observed in Marfan patients with EL.
Asunto(s)
Astigmatismo , Desplazamiento del Cristalino , Síndrome de Marfan , Humanos , Biometría , Córnea/diagnóstico por imagen , Desplazamiento del Cristalino/diagnóstico , Desplazamiento del Cristalino/epidemiología , Desplazamiento del Cristalino/etiología , Síndrome de Marfan/complicaciones , Síndrome de Marfan/diagnóstico , Estudios Prospectivos , Agudeza VisualRESUMEN
BACKGROUND: Classic homocystinuria (HCU), or cystathionine beta-synthase (CBS) deficiency, is a rare inborn error of methionine metabolism. Main clinical features may include skeletal and vascular manifestations, developmental delay, intellectual disability and eye disorders. MATERIAL AND METHODS: This is an observational and retrospective study aiming at describing eye abnormalities presented by a cohort of late-diagnosed HCU patients. Data regarding ophthalmological evaluation included visual acuity, refraction, biomicroscopy, Perkins tonometry, fundus examination, retinography, biometry, ocular ultrasound, optical coherence tomography, anterior segment photography and topography. RESULTS: Ten patients with HCU (20 eyes) were included. The most frequent findings were ectopia lentis(n = 20) and myopia (n = 9). Biometry, ultrasound, OCT and topography findings were available for four patients. One patient had keratoconus; one had abnormal retinal pigmentation; and two had lens surgery scars with irregular astigmatism. CONCLUSIONS: Eye abnormalities are very frequent in late-diagnosed HCU patients. The presence of ectopia lentis should always raise the diagnostic hypothesis of HCU.
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Astigmatismo/patología , Desplazamiento del Cristalino/patología , Homocistinuria/complicaciones , Miopía/patología , Adolescente , Adulto , Astigmatismo/etiología , Desplazamiento del Cristalino/etiología , Femenino , Humanos , Masculino , Miopía/etiología , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
A new technique for fixating the capsular bag in patients with ectopia lentis is presented. In this technique, the capsulorhexis is performed using a femtosecond laser, followed by the insertion of a standard capsular tension ring to redistribute capsular forces. The nucleus is hydroprolapsed into the anterior chamber and nuclear disassembly is performed above the iris plane to reduce zonular stress. Finally, a 5-0 polypropylene monofilament is used to fixate a capsular tension segment and subluxated capsular bag. This novel double-flanged method, achieved with cautery, does not require direct suturing of the monofilament on the sclera. This article describes the use of this new technique in 3 eyes, 2 in patients with Marfan syndrome and 1 in a patient with microspherophakia.
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Capsulorrexis/métodos , Enfermedades de la Córnea/cirugía , Desplazamiento del Cristalino/cirugía , Glaucoma/cirugía , Iris/anomalías , Síndrome de Marfan/cirugía , Facoemulsificación/métodos , Técnicas de Sutura , Adulto , Enfermedades de la Córnea/complicaciones , Desplazamiento del Cristalino/complicaciones , Desplazamiento del Cristalino/etiología , Femenino , Glaucoma/complicaciones , Humanos , Iris/cirugía , Terapia por Láser/métodos , Cápsula del Cristalino/cirugía , Implantación de Lentes Intraoculares/métodos , Síndrome de Marfan/complicaciones , Persona de Mediana Edad , Refracción Ocular/fisiología , Esclerótica/cirugía , Agudeza Visual/fisiologíaRESUMEN
INTRODUCTION: Previous studies have reported that patients with the CHARGE association have congenital anomalies including: Coloboma; heart defects; choanal atresia, retarded growth and development; genital hypoplasia; and ear anomalies. Ocular findings in patients with the association include: poor visual acuity; anisometropia; myopic astigmatism; strabismus; microcornea; cataracts; staphyloma; and reduced stereopsis. METHODS: We conducted a non-concurrent prospective study of 13 patients with coloboma from Puerto Rico. RESULTS: Seven patients out of the 13 patients (53.8%) had the CHARGE association. Age ranged from 10 to 84 (mean = 38 years). Ocular findings in all the patients with coloboma include: nystagmus; strabismus; poor visual acuity; refractive errors; microcornea; cataracts; glaucoma; and dry eyes. Patients in our study had systemic findings such as: heart defects; ear anomalies; and endocrine problems. CONCLUSIONS: To our knowledge this is the first case series of patients with Coloboma and the CHARGE association reported in Puerto Rico and the Caribbean basin.
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Anomalías Múltiples/epidemiología , Coloboma/epidemiología , Adulto , Anciano , Anciano de 80 o más Años , Niño , Atresia de las Coanas/epidemiología , Córnea/anomalías , Oído/anomalías , Desplazamiento del Cristalino/etiología , Femenino , Glaucoma/etiología , Cardiopatías Congénitas/epidemiología , Humanos , Hidrocortisona/deficiencia , Hipotiroidismo/etiología , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Puerto Rico/epidemiología , Síndrome , Xeroftalmia/etiologíaRESUMEN
PURPOSE: To assess the results as to visual acuity of two different surgical procedures for ectopia lentis. METHODS: Fifty-one eyes of 28 patients (16 males and 12 females, mean age 16.00 +/- 8.5) with simple (19 cases) or Marfan syndrome-associated (9 cases) ectopia lentis with different levels of subluxation underwent lens extraction with implantation of intraocular lenses (IOL) with scleral fixation (21 cases) or by lens extraction with implantation of the intraocular lenses in the capsular bag expanded by endocapsular ring (RING) (30 cases). Result analysis emphasized pre- and postoperative visual acuity during a six-month follow-up. RESULTS: Both techniques showed significant increase of postoperative visual acuity with and without correction, but it was better among the cases operated on by scleral fixation of the intraocular lenses. More than the used technique, the preoperative subluxation grades were crucial as to the results. CONCLUSION: The two surgical techniques for correction of simple or Marfan syndrome-associated ectopia lentis are safe and effective, resulting in significant recovery of visual acuity, although surgical results are completely dependent on the preoperative lens subluxation grades.
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Desplazamiento del Cristalino/cirugía , Implantación de Lentes Intraoculares/métodos , Esclerótica/cirugía , Agudeza Visual/fisiología , Adolescente , Adulto , Niño , Preescolar , Desplazamiento del Cristalino/etiología , Desplazamiento del Cristalino/patología , Femenino , Estudios de Seguimiento , Humanos , Cápsula del Cristalino/cirugía , Implantación de Lentes Intraoculares/efectos adversos , Lentes Intraoculares , Masculino , Síndrome de Marfan/complicaciones , Síndrome de Marfan/patología , Síndrome de Marfan/cirugía , Estadísticas no Paramétricas , Técnicas de Sutura , Resultado del TratamientoRESUMEN
OBJETIVOS:Avaliar os resultados quanto à acuidade visual, de dois procedimentos cirúrgicos para a ectopia lentis. MÉTODOS: Foram operados 51 olhos de 28 pacientes (16 do sexo masculino e 12 do feminino, com média de idade de 16,00±8,5 anos) com ectopia lentis simples (19 casos), ou associada à síndrome de Marfan (nove casos), com diferentes graus de subluxação cristaliniana. Em 21 casos a técnica empregada foi a facectomia com implante de lente intra-ocular (LIO) por fixação escleral e em 30 casos foi utilizada a facectomia com implante da lente intra-ocular no saco capsular, previamente expandido por anel endocapsular (ANEL). Os resultados enfatizaram a acuidade visual pré e pós-operatória em seguimento de seis meses. RESULTADOS:Em ambas as técnicas, a acuidade visual pós-operatória sem e com correção teve aumento significante, que foi maior nos casos operados com fixação escleral da lente intra-ocular. Mais do que a técnica empregada, os graus de subluxação pré-operatórios foram determinantes para os resultados. CONCLUSÕES: Mediante as técnicas operatórias apresentadas, a correção cirúrgica da ectopia lentis simples ou associada à síndrome de Marfan é segura e eficaz, com recuperação significante da acuidade visual, embora os resultados operatórios dependam, intrinsecamente, dos graus pré-operatórios de subluxação do cristalino.
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Humanos , Masculino , Femenino , Preescolar , Niño , Adolescente , Adulto , Agudeza Visual/fisiología , Desplazamiento del Cristalino/cirugía , Esclerótica/cirugía , Implantación de Lentes Intraoculares , Cápsula del Cristalino/cirugía , Desplazamiento del Cristalino/etiología , Desplazamiento del Cristalino/patología , Estudios de Seguimiento , Implantación de Lentes Intraoculares , Lentes Intraoculares , Estadísticas no Paramétricas , Técnicas de Sutura , Síndrome de Marfan/complicaciones , Síndrome de Marfan/patología , Síndrome de Marfan/cirugía , Resultado del TratamientoRESUMEN
O cristalino ectópico está presente em diversas doenças, que apesar de raras, relacionam-se a complicações severas. A doença mais freqüentemente encontrada no presente estudo foi a Síndrome de Marfan, na qual o deslocamento do cristalino ocorreu para a região superior e temporal. O deslocamento acarreta importante diminuição da acuidade visual e pode gerar ambliopia. Esse estudo apresenta a freqüencia e as causas de cristalino ectópico nos pacientes examinados no Setor de Genética do Departamento de Oftalmologia da Escola Paulista de Medicina no periodo de 1985 a 1993, enfatizando o diagnóstico e reabilitação ocular precoces para a prevenção da ambliopia e estrabismo.