Epilens membrane simulating cataract in children with uveitis: a report of three cases / Membrana epicristaliniana simulando catarata em crianças com uveíte: relato de três casos

ABSTRACTHere we present the cases of three female children, of whom two were aged 6 years and one was aged 11 years. Two of the three children had bilateral uveitis and suspected cataract and Vogt-Koyanagi-Harada (VKH) disease. The third one had uveitis and suspected cataract in one eye and sympathetic ophthalmia (SO), and had undergone penetrating keratoplasty in the fellow eye following a trauma. After controlling the inflammation, we planned to perform phacoemulsification without intraocular lens implantation. However, intraoperatively, after removing the epilens membranes, the lenses appeared clear, and therefore phacoemulsification was not performed. During follow-up, the patients did not develop cataract, and visual acuity levels ranged from 20/30 to 20/100. Fundoscopy revealed VKH disease and SO. Ophthalmologists should not always assume that patients with uveitis have cataract; a transparent lens may exist behind the epilens membrane, allowing a less aggressive therapeutic approach.

RESUMOApresentamos os casos de três meninas (duas de seis anos e uma de onze anos), com uveíte bilateral e suspeita de catarata em ambos os olhos e doença de Vogt-Koyanagi-Harada (VKH) em dois casos. No terceiro caso, oftalmia simpática e suspeita de catarata em um olho e trauma com ceratoplastia penetrante no outro olho. Após controle da inflamação, indicou-se facoemulsificação sem LIO. No per-operatório, após retirada da membrana epicristaliniana, a transparência do cristalino foi percebida, não sendo realizada a facoemulsificação. No período de acompanhamento as pacientes não desenvolveram catarata e a acuidade visual variou de 20/30 a 20/100. O exame de fundoscopia reforçou o diagnóstico de VKH e oftalmia simpática. Portanto, é muito importante que o oftalmologista esteja sempre atento quando se deparar com um caso de uveíte com suspeita de catarata, porque, por trás da membrana epicristaliniana pode existir um cristalino transparente, o que leva a uma conduta terapêutica menos agressiva.

Successful cochlear implantation in a case of Vogt-Koyanagi-Harada disease.

OBJECTIVE: To characterize the auditory phenotype of Vogt-Koyanagi-Harada disease (VKH) and highlight the potential for successful cochlear implantation (CI). PATIENTS: Single case report INTERVENTION(S): Therapeutic MAIN OUTCOME MEASURE(S): AzBio Sentences in quiet and noise, CNC Words, BKB-SIN RESULTS: VKH is a systemic autoimmune disorder characterized by granulomatous uveitis accompanied by neurologic and auditory-vestibular symptoms. Development is attributed to an autoimmune response against pigmented cells, with destruction of melanocytes by T lymphocytes directed against an unidentified antigen; accordingly, the disease typically affects races with darkly pigmented skin. VKH presents as blurring of vision and eye inflammation, severe headaches, and meningismis. Auditory symptoms include sensorineural hearing loss (SNHL), tinnitus, and imbalance. SNHL can persist despite immunosuppressant therapy.This report describes successful cochlear implantation in a 26-year-old female patient with VKH who presented with complaints of blurred vision and bilateral sudden SNHL. Oral steroids initially restored hearing, but benefit ceased upon discontinuation of oral steroids and was not restored following a series of intratympanic steroid injections. The patient underwent bilateral CI, which was fast-tracked because of the autoimmune nature of her hearing loss. After implantation, her speech understanding in both quiet and noise far exceeds her pre-implant scores. CONCLUSION: This unique case is the first to report successful CI in VKH. It is important for hearing health professionals to be aware of the signs and symptoms of this disease as patients may present initially with hearing loss, tinnitus, or imbalance, and may benefit from CI in cases of persistent, steroid-unresponsive, severe-to-profound SNHL.

[Clinical research of anterior chamber inflammation after phacoemulsification surgery in patients with complicated cataract secondary to uveitis].

OBJECTIVE: To investigate anterior chamber inflammation after phacoemulsification with intraocular lens (IOL) implantation in patients with Vogt-Koyanagi-Harada (VKH) syndrome or Behçet's disease (BD). METHODS: Cohort study. Seventeen patients (20 eyes) with complicated cataracts and VKH syndrome or BD who underwent phacoemulsification with IOL implantation at Zhongshan Ophthalmic Center, Sun Yat-Sen University (SYSU) between January 2010 and June 2011 were included as the experimental group in this study. Cataract surgery was performed on these patients only when uveitis had been under control for more than three months. Thirty patients (40 eyes) with age-related cataracts who underwent phacoemulsification with IOL implantation in the same period were included as the control group. Quantitative measurements of anterior chamber aqueous flare and inflammatory cells were conducted preoperatively and postoperatively using a Laser Flare Cell Meter (LFCM). Independent t-test was used to compare patients' ages, and the energy and time of phacoemulsification between the two groups. The Student's t-test was used to assess the differences between paired data preoperatively and postoperatively. Independent t-test was also used to assess the quantitative data between groups. RESULTS: The study recruited 20 eyes in the experimental group and 40 eyes in the control group, including 11 eyes from 9 VKH patients and 9 eyes from 8 BD patients. The preoperative and postoperative flare values in the experimental group were (19.86 ± 6.47), (44.28 ± 18.47), (35.60 ± 12.65), (23.85 ± 8.41), and (13.86 ± 4.27) pc/ms, respectively, which were statistically higher than that of the control group preoperatively, and on days 1, 7, 30, and 90 after surgery (tpre = 4.643, Ppre < 0.01; t1 = 6.035, P1 < 0.01; t7 = 3.595, P7 = 0.001; t30 = 4.658, P30 < 0.01; t90 = 3.308, P90 = 0.002). Aqueous flare in Group A and Group B declined to preoperative levels on day 30 (t = 0.320, P = 0.753) and day 7 (t = 0.454, P = 0.653). For the experimental group, the inflammatory cell count on day 1 and 7 was (83.46 ± 27.08) and (27.56 ± 8.32) cells/0.5 mm(3), respectively, which was significantly higher than the preoperative level [(6.47 ± 3.56)cells/ 0.5 mm(3), t1 = 5.261, P1 < 0.01; t7 = 2.766, P7 = 0.012]. On days 30 and 90, the inflammatory cell count was (11.43 ± 4.81) and (4.82 ± 2.29) cells/0.5 mm(3), respectively, and there was no statistically significant difference in the inflammatory cell count compared with the preoperative level (t30 = 2.348, P30 = 0.042; t90 = 1.376, P90 = 0.186). For the control group, inflammatory cell count reduced to pre-operative level on day 7 (t7 = 2.464, P7 = 0.018). CONCLUSIONS: Anterior chamber inflammation reaches peak levels one week postoperatively in VKH and BD patients who receive phacoemulsification with IOL implantation. It takes three months for the inflammation to recede, and might take longer for complete restoration of the blood-aqueous barrier.

Visual rehabilitation in pre phthisis bulbi's patient: case report.

Uveitis is a group of intraocular inflammatory diseases that affects mainly patients of working age. Vogt-Koyanagi-Harada disease is a chronic, granulomatous, systemic and autoimmune disease with manifestations in the eye, skin, and hearing and nervous systems. Ocular complications include cataract, glaucoma, choroidal neovascular membrane formation, and subretinal fibrosis, which can lead to phthisis bulbi with profound low vision or even blindness. The purpose of this article is to present the visual rehabilitation of a young patient with pre phthisis bulbi as a complication of the uveitis. During the active stage of the disease the patient quit her studies in a crucial phase of her life. After clinical treatment and surgery, training program of optical device adaptation and residual visual optimization were performed. Due to the great patient's motivation, it was possible to reintegrate her to social life.

Visual rehabilitation in pre phthisis bulbi's patient: case report / Reabilitação visual em paciente com pré phthisis bulbi: relato de caso

Uveitis is a group of intraocular inflammatory diseases that affects mainly patients of working age. Vogt-Koyanagi-Harada disease is a chronic, granulomatous, systemic and autoimmune disease with manifestations in the eye, skin, and hearing and nervous systems. Ocular complications include cataract, glaucoma, choroidal neovascular membrane formation, and subretinal fibrosis, which can lead to phthisis bulbi with profound low vision or even blindness. The purpose of this article is to present the visual rehabilitation of a young patient with pre phthisis bulbi as a complication of the uveitis. During the active stage of the disease the patient quit her studies in a crucial phase of her life. After clinical treatment and surgery, training program of optical device adaptation and residual visual optimization were performed. Due to the great patient's motivation, it was possible to reintegrate her to social life.

Uveíte é o termo utilizado para descrever um grupo de doenças inflamatórias oculares que ocorrem predominantemente na idade produtiva. Vogt-Koyanagi-Harada é uma doença sistêmica, autoimune, granulomatosa, crônica com manifestações oculares, neurológicas, auditivas e cutâneas. As principais complicações incluem catarata, glaucoma, membrana neovascular e fibrose sub-retiniana, que podem evoluir para atrofia ocular, baixa visão profunda e cegueira. O propósito deste artigo é apresentar um caso de reabilitação visual em paciente jovem com pré phthisis bulbi consequente às complicações da uveíte. Após tratamento clínico e cirurgia, um programa de treinamento e adaptação de recursos ópticos foi realizado para otimizar o uso do resíduo visual, motivando a paciente para reintegrar-se socialmente e voltar aos estudos.

Vitreous surgery for macular hole in patients with Vogt-Koyanagi-Harada disease.

We describe two patients with Vogt-Koyanagi-Harada (VKH) disease, both in the convalescent stage, who presented with unilateral macular holes together with clinically significant epi-retinal membranes. Vitreo-retinal surgery was performed on the affected eyes and the surgical technique involved a standard three-port vitrectomy, peeling of the epi-retinal and internal limiting membrane (ILM). In both cases the retinae were tamponaded with air resulting in anatomical closure of the macular holes. The histology of the excised membrane was available in one case and this revealed multiple layers of presumed retinal pigment epithelial cells with cytoplasmic processes and intercellular junctions forming a basal lamina attached to the smooth surface of the ILM. Our findings demonstrate that macular holes can develop in patients with VKH but that the hole can be successfully closed with vitreo-retinal surgery. The convalescent stage tends to occur several weeks after the acute stage when the uveitic process has subsided and is characterized by choroidal depigmentation, producing a sunset glow appearance to the ocular fundus. Patients may also demonstrate varying degrees of cutaneous hypopigmentation, poliosis and/or alopecia. Macular holes have also been reported previously in patients during the convalescent stage of VKH and this communication describes the outcome of two patients who underwent vitreo-retinal surgery for this problem.

Síndrome de Vogt-Koyanagi-Harada en un niño de 4 años. Presentación de un caso clínico / Vogt-Koyanagi-Harada syndrome in a 4 years old child

Se presenta el caso de un niño de ascendencia mexicana de 4 años 8 meses de edad. El paciente más joven reportado en nuestro país, con síndrome de Vogt-Koyanagi-Harada. El cual presentó un cuadro de panuveítis bilateral severa que se complicó con catarata y glaucoma secundario, requiriendo de manejo médico y quirúrgico para mejorar su visión. Simultáneamente, presentó alopecia, vitiligo e hipoacusia, datos que en conjunto conformaron el diagnóstico de esta enfermedad