Congenital cataract surgery with intraocular lens implantation in microphthalmic eyes: visual outcomes and complications / Cirurgia de catarata congênita com implante de lente intraocular em olhos microftálmicos: resultados visuais e complicações

PURPOSE: To report the visual outcomes and complications of congenital cataract surgery with primary intraocular lens implantation in microphthalmic eyes of children younger than 4 years of age. METHODS:This retrospective interventional case series included 14 microphthalmic eyes from 10 children who underwent congenital cataract surgery with primary intraocular lens implantation younger than 4 years of age. Seven patients had bilateral cataracts (11 eyes met the study's inclusion criteria) and 3 patients had unilateral cataract. Patients' medical charts were reviewed to obtain information regarding the preoperative and postoperative ophthalmological examination. Main outcome measures were intraocular pressure (IOP), best-corrected visual acuity, and intraoperative and postoperative complications. RESULTS: Mean age at the time of surgery was 21.7 ± 2.9 months. Mean ocular axial length was 19.2 ± 0.9 mm. Mean preoperative IOP was 9.7 ± 1.7 mmHg and 10.3 ± 3.1 mmHg on final follow-up (P=0.18). There were no intraoperative complications. Two (15.4%) eyes developed secondary visual axis opacification, of which only one needed to be reoperated due to significantly decreased vision (0.5 logMAR). Preoperative and postoperative best-corrected visual acuity was 2.09 ± 0.97 logMAR and 0.38 ± 0.08 logMAR in bilateral cases and 1.83 ± 1.04 logMAR and 0.42 ± 0.13 logMAR in unilateral cases, respectively. CONCLUSION: Primary intraocular lens implantation in congenital cataract surgery in microphthalmic eyes resulted in a significant best-corrected visual acuity improvement with no intraoperative complications and minimal postoperative complications.

OBJETIVOS: Relatar os resultados visuais e as complicações da cirurgia de catarata congênita com implante primário de lente intraocular em olhos microftálmicos de crianças menores de 4 anos. MÉTODOS: Esta série de casos retrospectiva incluiu 14 olhos microftálmicos de 10 crianças menores de 4 anos que foram submetidas à cirurgia de catarata congênita com implante primário de lente intraocular. Sete pacientes tinham catarata bilateral (11 foram incluídos no estudo) e 3 tinham catarata unilateral. Os prontuários dos pacientes foram revisados para se obter informação sobre o exame oftalmológico pré- e pós-operatório. As principais variáveis analisadas foram pressão intraocular, acuidade visual com melhor correção e complicações intra- e pós-operatórias. RESULTADOS: A média da idade dos pacientes na época da cirurgia foi de 21,7 ± 2,9 meses. O diâmetro antero-posterior médio foi de 19,2 ± 0,9 mm. A pressão intraocular média pré-operatória foi 9,7 ± 1,7 mmHg e 10,3 ± 3,1 mmHg no último exame de acompanhamento pós-operatório (P=0,18). Não houve complicações intraoperatórias. Dois (15,4%) olhos desenvolveram opacificação secundária do eixo visual, dos quais um foi reoperado devido à baixa visual significativa (0,5 logMAR). AV pré- e pós-operatórias foram 2,09 ± 0,97 logMAR e 0,38 ± 0,08 logMAR em casos de catarata congênita bilateral e 1,83 ± 1,04 logMAR e 0,42 ± 0,13 logMAR em casos unilaterais, respectivamente. CONCLUSÃO: O implante primário de lente intraocular em cirurgia de catarata congênita em olhos microftálmicos resultou em uma melhora significativa da acuidade visual com nenhuma complicação intraoperatória e com pouca complicação no pós-operatório.

Posterior Chamber Intraocular Lens Implantation in Pediatric Cataract with Microcornea and/or Microphthalmos

PURPOSE: To report the results of secondary posterior chamber intraocular lens (PC-IOL) implantation in pediatric cataract eyes with microcornea and/or microphthalmos. METHODS: Retrospective studies were conducted by reviewing the charts of 26 eyes of 15 patients with secondary PC-IOL implantations for microcornea and/or microphthalmos associated with cataract in children between 1999 and 2002. The corneal diameter was 9.5 mm or less at the time of secondary PC-IOL implantation. Preoperative examinations were conducted for bilaterality, corneal diameter, eye abnormalities and systemic abnormalities. Postoperative results were evaluated in terms of visual developments, refractive changes, axial length measurements and the occurrence of complications. The follow-up period was at least one year after secondary PC-IOL implantation. RESULTS: Age at the first diagnosis for cataract with microcornea and/or microphthalmos was 2.7 months on average. Among 15 patients, 8 (53.3%) had a family history. All patients received an initial irrigation and aspiration, posterior capsulectomy, and anterior vitrectomy at 0.8 years (0.1-3.3 years) of age and a secondary PC-IOL implantation surgery at 6.7 years (1.6-17.2 years) of age on average. The postoperative follow-up period was 2.1 years (1.1-4.3 years) on average. The average power of the implanted PC-IOL was +21.2D. Postoperative complications were secondary glaucoma in two eyes, secondary pupillary membrane formation in two eyes. Best corrected visual acuities in 20 eyes in children capable of the vision test at the last follow-up were 20/60 or better in 11 eyes, 20/80 to 20/150 in eight eyes, and 20/200 or worse in one eye. CONCLUSIONS: The secondary PC-IOL implantation in pediatric cataract with microcornea and/or microphthalmos is recommended as a means of improving vision, but must be conducted carefully to avoid possible complications.

Two Cases of Uveal Effusion Syndrome

PURPOSE: To report a case of uveal effusion syndrome associated with hypotony and a case of uveal effusion syndrome in nanophthalmos. METHODS: The first case was a 25-year-old man who presented with decreased visual acuity in the left eye and hypotony. Fundus examination revealed choroidal effusion and retinal detachment with a thickened eyeball. Partial thickness sclerotomy and sclerectomy were performed. The second case was a 13-year-old boy who had uveal effusion syndrome with a nanophthalmic eye. RESULTS: In the patient with hypotony, intraocular pressure was well maintained following partial thickness sclerotomy and sclerectomy, and choroidal effusion and retinal detachment were reduced. The visual acuity of the nanophthalmic patient was well maintained during a 3-year follow-up period without treatment. CONCLUSIONS: appropriate treatment modalities should be considered depending on the ophthalmic condition of the individual patient.

Choroidal effusion following laser peripheral iridotomy for the treatment of angle closure glaucoma in a patient with nanophthalmos.

We report a case of Nanophthalmos with choroidal effusion and retinal detachment following a peripheral laser iridotomy for the treatment of angle closure glaucoma. Angle closure glaucoma was diagnosed in the right eye of the patient after finding an elevated intraocular pressure and narrow angles on gonioscopy. The glaucoma was treated by lowering the intraocular pressure medically followed by laser peripheral iridotomy. The first day after a laser peripheral iridotomy, choroidal effusion was noticed that was followed by an exudative retinal detachment within a week. The medical and laser therapies for the lowering of intraocular pressure for the treatment of angle closure glaucoma in patients with nanophthalmos can be associated with choroidal effusion, retinal detachment and loss of vision.

Orbital aspiration as treatment of microphthalmos with orbital cyst: a case report.

A 6-month-old girl came to the hospital with swelling of the right lower eyelid, exophthalmos, chemosis and upward deviation of the eyeball--all of which had been present since birth. Iris, optic disc, and chorioretinal coloboma were also apparent. Magnetic resonance imaging revealed a small globe with a large cystic lesion in the orbit of the right eye. Pre- and post-operative photographs and magnetic resonance imaging indicated a safe, simple single orbital aspiration as an alternative treatment for mild microphthalmos with an orbital cyst.

Angle closure glaucoma in nanophthalmos and pigmentary retinal dystrophy: a rare syndrome.

The simultaneous occurrence of nanophthalmos, angle closure glaucoma and pigmentary retinal dystrophy documented in the present case represents a rare syndrome.

Adaptaçäo de lente de contato em microcórnea; relato de um caso / Contact lens fitting on microcornea; a case report

Os autores relatam um caso de adaptaçäo de lente de contato em uma paciente portadora de microcórnea e microftalmia que foi submetida a facectomia sem lente intraocular (LIO). Adaptou-se uma lente de contato fluorsiliconada de +14,25 dioptrias com curva base (CB) mais curva que K (46,00) e diâmetro pequeno (8,3), proporcionando acuidade visual de 20/60 a paciente. Esta lente mostrou-se adequada para o caso clínico

Familial nanophthalmos: management and complications.

Nanophthalmos is a rare form of congenital hypermetropia, in which, the individual is at a high risk of developing angle-closure glaucoma. We report a family of nanophthalmos affecting a brother and sister, as confirmed by biometry. Genetic analysis revealed an autosomal recessive mode of inheritance. The sister developed angle-closure glaucoma, which was refractory to medical and laser therapy. She underwent surgical interventions which resulted in complications. The clinical presentation, management, and complications encountered are discussed.

Microphthalmos with cyst: a clinical study.

Defects in closure of embryonic fissure and invagination of optic vesicle results in varied clinical presentation of microphthalmos with cyst. In our study, three patients had microphthalmos with cyst in one of their orbits. The cyst presented either as a swelling in the lower lid or as a sinus with purulent discharge associated with absent or small sized eyeball. The microphthalmic eye showed a spectrum of posterior segment abnormalities such as retinal disorganisation, gliosis, choroidal, and scleral coloboma. Microscopically, the cyst connected to the coloboma consisted of an outer layer of fibrovascular tissue and an inner gliotic neuroectodermal layer. The treatment consisted of excision of cyst alone or of microphthalmic eye with cyst. The use of conformers was mandatory after removal of cystic eyeball for near normal development of both orbits to maintain facial symmetry in our paediatric patients.

Microphthalmos with cyst: case presentation

The author has experienced a case of microphthalmos with large orbital cyst in a 4 months old female, that was found at the time of birth. To facilitate fitting a cosmetic prosthesis, the microphthalmos with cyst was removed surgically. On serial section I could find an area of discontinuation of the sclera that was suspected to be the defective closure of the embryonic cleft. Some aberrant retinal tissue was found in the wall of the cyst, and markedly disorganized ocular tissue forming a tumor-like mass filled the microphthalmic eyeball. In view of these histopathologic findings I could draw the conclusion that developmental failure of the embryonic eyeball and consequential proliferation of the embryonic neuroepithelial cells occurred at an early developmental stage causing the formation of microphthalmos with cyst.