Deslocamento espontâneo do núcleo do cristalino para câmara anterior / Spontaneous displacement of the lens nucleus into the anterior chamber

RESUMO Ao longo da vida, o cristalino produz novas fibras dispostas de forma concêntrica, que aumentam seu diâmetro anteroposterior e peso, tornando seu núcleo mais compacto e endurecido. A catarata hipermadura é uma forma de progressão avançada dessa proliferação de fibras, que pode desencadear uma variedade de complicações. A ruptura espontânea da cápsula anterior do cristalino, evoluindo com deslocamento anterior do núcleo, é uma complicação rara e com poucos casos publicados na literatura. Descrevemos o caso de uma paciente do sexo feminino, 68 anos, que apresentou ruptura espontânea da cápsula anterior do cristalino com deslocamento anterior do núcleo em olho esquerdo sem histórico de trauma ocular. A paciente foi submetida à facoemulsificação do cristalino e ao controle da pressão intraocular, evoluindo com melhora do quadro clínico.

ABSTRACT Throughout life, the lens produces new fibers arranged concentrically, which increase its anteroposterior diameter and weight, making its nucleus more compact and hardened. Hypermature cataract is an advanced stage of this fiber proliferation, which can trigger a variety of complications. Spontaneous rupture of the anterior lens capsule evolving with anterior displacement of the nucleus is a rare complication, with few cases published in the literature. We describe the case of a 68-year-old female patient, who presented spontaneous rupture of the anterior lens capsule with anterior displacement of the nucleus in the left eye, without a history of ocular trauma. The patient underwent phacoemulsification and clinical control of intraocular pressure, improving her condition.

Facoanafilaxia por cristalino mergulhado na Síndrome de Marfan / Phacoanaphylactic by dislocation of the lens in the Marfan Syndrome

Resumo A síndrome de Marfan é uma doença de herança autossômica dominante e que afeta o tecido conjuntivo com manifestações fenotípicas que envolvem os sistemas esquelético, cardiovascular e ocular. As principais manifestações oculares são a subluxação do cristalino, a miopia e o descolamento da retina. O objetivo deste artigo foi relatar a conduta clínico-cirúrgica de um paciente portador da síndrome de Marfan com cristalino luxado para a cavidade vítrea e que evoluiu com severa reação facoanafilática caracterizada por um glaucoma secundário severo e descompensação corneana.

Abstract Marfan syndrome is an autosomal dominant inheritance disease that affects connective tissue with phenotypic manifestations involving the skeletal, cardiovascular and ocular systems. The main ocular manifestations are the subluxation of the lens, myopia and retinal detachment. The aim of this article was to report the clinical and surgical management of a patient with Marfan syndrome with luxated lens for the vitreous cavity and who developed a severe phacoanaphylactic reaction characterized by severe secondary glaucoma and corneal decompensation.

WAGRO syndrome: a rare genetic condition associated with aniridia and additional ophthalmologic abnormalities / Síndrome WAGRO: uma condição genética rara associada à aniridia e a anormalidades oftalmológicas adicionais

ABSTRACT Aniridia is a congenital eye disorder with a variable degree of hypoplasia or absence of iris tissue. It is caused by loss of function of the PAX6 gene and may be an isolated ocular abnormality or part of a syndrome. WAGRO refers to a rare genetic condition leading to Wilms tumor, aniridia, genitourinary anomalies, mental retardation, and obesity and is caused by a deletion of the short arm of chromosome 11 (11p), where the PAX6 gene is located. Here, we report on an 8-year-old boy with aniridia, polar cataract, and lens subluxation along with neuropsychomotor and speech delays. Karyotype evaluation showed an interstitial deletion including region 11p13-p14, confirming the diagnosis of WAGRO syndrome. In cases of aniridia, a diagnosis of WAGRO syndrome should be considered.

RESUMO A aniridia é uma doença ocular congênita com grau variável de hipoplasia ou ausência do tecido da íris. É causada pela perda de função do gene PAX6 e pode ser uma anormalidade ocular isolada ou parte de uma síndrome. WAGRO refere-se a uma condição genética rara que leva ao tumor de Wilms, aniridia, anomalias geniturinárias, déficit intelectual e obesidade e é causada por uma deleção do braço curto do cromossomo 11 (11p), onde o gene PAX6 está localizado. Aqui, nós relatamos um menino de 8 anos de idade com aniridia, catarata polar e subluxação do cristalino, além de retardo neuropsicomotor e de fala. A avaliação cariotípica revelou uma deleção intersticial envolvendo a região 11p13-p14, confirmando o diagnóstico da síndrome WAGRO. Em casos de aniridia, um diagnóstico de síndrome de WAGRO deve ser considerado.

Deslocamento espontâneo do núcleo do cristalino para câmara anterior / Spontaneous dislocation of the lens nucleus to the anterior chamber

Resumo Ruptura espontânea da cápsula anterior do cristalino, evoluindo com deslocamento anterior do núcleo é uma complicação rara da catarata hipermadura. Relatamos o caso de uma paciente do sexo feminino, 79 anos, que se apresentou com ruptura espontânea da cápsula anterior do cristalino com deslocamento anterior do núcleo em olho direito sem histórico de trauma ocular. O diagnóstico foi confirmado pela visualização das abas da cápsula anterior, principalmente quando o núcleo foi removido. Realizamos facoemulsificação do núcleo pelo risco de descompensar a córnea. É importante destacar a raridade do caso, sendo o primeiro relatado no Brasil.

Abstract Spontaneous rupture of the anterior lens capsule, evolving with anterior dislocation of the lens nucleus is a rare complication of hypermature cataract. We report a case of a female patient, 79 years old, who presented with spontaneous rupture of the anterior lens capsule with anterior dislocation of the nucleus in the right eye with no history of ocular trauma. The diagnosis was confirmed by the visualization of the tabs of the anterior capsule, especially when the nucleus was removed. We perform phacoemulsification of the nucleus by the risk of decompensating the cornea. It is important to highlight the rarity of the case, the first being reported in Brazil.

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.

Magnetic resonance imaging of intact globe superior subluxation into the intracranium.

A 67-year-old man with right-sided blunt ocular trauma is reported here. Despite having received primary medical care, the patient complained of severe headache for 14 days. Initial computed tomography (CT) indicated hematoma in the right frontal lobe. However, magnetic resonance imaging (MRI) indicated that the right globe along with its optic nerve had been intactly dislocated into the intracranium and differentiated from hematoma. In this case, the significance of MRI, in blunt ocular trauma work-up, and also regaining successful ocular function are highlighted.

Comparison of immersion ultrasonography, ultrasound biomicroscopy and anterior segment optical coherence tomography in the evaluation of traumatic phacoceles.

Blunt ocular trauma in the elderly can result in anterior dislocation of the crystalline lens into the subconjunctival space (phacocele). Although rare, this presentation can be missed, especially if the patient presents several days after the injury and if the lid is not everted on examination. While a careful clinical examination is adequate in the diagnosis, imaging techniques can be put to use for the accurate location of the associated sclera rupture. We report three cases of post-traumatic phacocele wherein ultrasound biomicroscopy (UBM) was compared to the anterior segment optical coherence tomography (AS-OCT) and B-scan ultrasonography (B-scan), in order to establish the best imaging tool for this condition. We concluded, based on image quality, that UBM could be the imaging modality of choice to aid in the diagnosis of phacocele.

Results of intraocular lens implantation with capsular tension ring in subluxated crystalline or cataractous lenses in children.

Purpose: To evaluate the outcome of intraocular lens (IOL) implantation using capsular tension ring (CTR) in subluxated crystalline or cataractous lenses in children. Setting: Tertiary care setting Materials and Methods: We prospectively studied 18 eyes of 15 children with subluxation of crystalline or cataractous lenses between 90° up to 210° after phacoemulsification, CTR and IOL implantation. Each child was examined for IOL centration, zonular dehiscence and posterior capsular opacification (PCO). Results: Age of the patient ranged between five to 15 years. Out of 18 eyes, seven had traumatic and 11 had spontaneous subluxation of crystalline or cataractous lens. Phacoemulsification was successfully performed with CTR implantation in the capsular bag. Intraoperative zonular dialysis occurred in two eyes. Anterior vitrectomy was performed in six eyes to manage vitreous prolapse. IOL implanted was polymethyl methacrylate (PMMA) in eight eyes, hydrophobic acrylic in seven and hydrophilic acrylic in three. Follow-up ranged from 24 months to 72 months. Sixteen eyes had a best corrected visual acuity of 20/40 or better. Nine eyes developed significant PCO and were managed with Neodymium Yttrium Aluminum Garnet (Nd:YAG) laser posterior capsulotomy. One eye with acrylic IOL in the capsular bag had IOL dislocation after two years which was managed with vitrectomy and secondary trans-scleral fixation of IOL. Conclusions: Phacoaspiration with CTR implantation makes capsular bag IOL fixation possible in most of the eyes with subluxated crystalline or cataractous lenses. PCO still remains a challenge in children with successful phacoaspiration with CTR implantation

Anterior dislocation of a sulcus fixated posterior chamber intraocular lens in a high myope.

A 31-year-old man with high axial myopia and strabismus fixus convergens underwent bilateral refractive lens exchange followed by a squint surgery (bilateral superior partial Jensen's procedure and medial rectus recession). After one year he presented with traumatic anterior dislocation of the sulcus fixated posterior chamber polymethyl methacrylate lens. The lens was dialed back into the ciliary sulcus without any complications. This case highlights the importance of implanting an intraocular lens (IOL) in-the-bag. If the IOL needs to be implanted in the sulcus, a larger diameter of the IOL with larger optic size and overall length is desirable, especially in highly myopic eyes.

Traumatic phacocele: review of eight cases.

Blunt trauma can result in indirect scleral rupture with subsequent dislocation of the crystalline lens in the subconjunctival or subtenon space. This retrospective review of eight patients with traumatic phacocele highlights the clinical presentation, management and visual outcome. This study provides evidence that timely and effective intervention can ensure good visual recovery.

Luxación traumática del globo ocular: presentación de un caso / Traumatic luxation of eyeball: a case presentation

Se presenta un caso de luxación traumática de globo ocular en el seno maxilar derecho en una paciente de 68 años de edad, femenina, de la raza negra, con traumatismo órbitofacial derecho atendida en el Hospital Roberto Rodríguez de Morón . El diagnóstico se realizó por el examen físico, ultrasonido ocular y exámenes imagenológicos. Se describen la evolución y las complicaciones presentadas.

There has been presented a case of traumatic luxation of eyeball in right maxillary sinus in a 68 years old patient, feminine, black and with right facial orbit trauma attended in Moron´s Hospital Roberto Rodriguez. The diagnosis was done by the physical exam, eye ultrasound and imaging exams. There are described the evolution and presented complications.

Weill-Marchesani syndrome associated with retinitis pigmentosa.

Retinitis pigmentosa (RP) is associated with a wide variety of ocular and systemic disorders. The Weill-Marchesani syndrome is a multi-system disorder with microspherophakia as one of the common manifestations. A 14-year-old girl presented with short stature, short and stubby fingers, hypodontia and low-set ears. Slit-lamp examination revealed microspherophakia, with shallow anterior chambers with irido and phacodonesis. Ultrasonographic biomicroscopy confirmed the clinical findings and revealed hypoplastic ciliary body. Electroretinogram confirmed the diagnosis of RP. Though RP has been associated with ectopia lentis in earlier reports, this is, to the best of our knowledge, the first case report describing the association of RP and Weill-Marchesani syndrome.

Bilateral Spontaneous Anterior Lens Dislocation in a Retinitis Pigmentosa Patient

PURPOSE: To report a case of bilateral spontaneous anterior lens dislocation associated with retinitis pigmentosa (RP). METHODS: A 45-year-old male with RP presented with elevated intraocular pressure (IOP) in the right eye and was treated with laser iridotomy (LI). After LI, complete crystalline lens dislocation into the anterior chamber occurred. Surgical intervention, including anterior vitrectomy, intracapsular cataract extraction (ICCE), and IOL scleral fixation was performed. Two years later, the same episode occurred in his left eye and a similar treatment was done. RESULTS: Surgery was successful in both eyes. CONCLUSIONS: This is the first report of bilateral spontaneous anterior lens dislocation in a RP patient.

A Case of Weill-Marchesani Syndrome with Inversion of Chromosome 15

PURPOSE: To present a case of Weill-Marchesani syndrome with corneal endothelial dysfunction due to anterior dislocation of a spherophakic lens and corneolenticular contact. METHODS: A 17-year-old woman presented with high myopia and progressive visual disturbance. She was of short stature and had brachydactyly. Her initial Snellen best corrected visual acuity (BCVA) was 20/50 (-sph 20.50 -cyl 3.00 Ax 180) in her right eye and 20/40 (-sph 16.00 -cyl 6.00 Ax 30) in her left eye. Slit lamp examination revealed a dislocated spherophakic lens touching corenal endothelium. A microspherophakic lens, hypoplastic ciliary body, and elongated zonules were confirmed on rotating Scheimpflug camera (Pentacam(R)) and on ultrasound biomicroscopy. Specular microscopy showed corneal endothealial dysfunction. Systemic evaluation was performed, and chromosomal study showed 46, XX, inv (15) (q13qter). The patient was diagnosed with Weill-Marchesani syndrome. RESULTS: Due to impending corneal decompensation, phacoemulsification and suture fixation of the intraocular lens were performed. The operation and postoperative course were uneventful. Three months postoperatively, the visual acuity was 20/30 (OD) and 20/40 (OS) without correction, and BCVA was 20/20 (+sph 0.50 -cyl 2.00 Ax 160 : OD) and 20/25 (+sph 1.50 -cyl 3.00 Ax 30 : OS). During the follow-up period, increased corneal endothelial counts, hexagonality, and decreased corneal thickness were achieved. CONCLUSIONS: In Weill-Marchesani syndrome with a chromosomal anomaly, a dislocated spherophakic lens may cause severe corneal endothelial dysfunction due to corneolenticular contact, and prompt lensectomy is important to prevent such complications.

Manifestaciones oculares del síndrome de Marfán / Ocular manifestations of Marfan syndrome

El síndrome de Marfán (SM) es una patología que afecta múltiples órganos y cuando no se maneja de manera adecuada es muy discapacitante con alto costo social para los sistemas de salud. Dentro de los órganos afectados se encuentra el visual, con manifestaciones que no exhiben un patrón de tipicidad debido a la falta de estudios que incluyan el examen oftalmológico completo como parte integral de la valoración y el tratamiento. La presencia de alteraciones oftalmológicas y la identificación de ciertas características al examen físico pueden anteceder las manifestaciones de los otros sistemas que comprenden el complejo diagnóstico de este síndrome, lo que da lugar a un abordaje precoz con manejo temprano y eficaz de alteraciones visuales. El objetivo del presente trabajo es analizar los hallazgos oftalmológicos de los pacientes con SM y describir las alteraciones más comunes en esta población.

Luxación traumatica del cristalino / Traumatic lens dislocation

Después de un traumatismo contuso el daño ocular es variable y el pronóstico es reservado.La ultrasonografía ocular es de gran utilidad para valorar el estado anatómico del ojo especialmente cuando los medios oculares han perdido su transparencia, aportando información muy útil en el tratamiento.Se presentan imágenes ecográficas de un caso de traumatismo ocular contuso con luxación de cristalino.

A ecografia nas opacidades dos meios transparentes da infância / The echography in the transparence means opacity of childhood

A ecografia ocular, quer do tipo A como do tipo B, é um recurso diagnóstico importante nos casos de opacidades dos meios transparentes, nas medidas do globo ocular, no diagnóstico de descolamento de retina, nas opacidades vitreanas e no estudo dos tumores oculares. Nos casos de catarata congenita a ecografia é fundamental para um prognóstico funcional