A proposal of rehabilitative approach in the rare disease "De Barsy Syndrome": case report.

De Barsy syndrome is an autosomal recessive condition characterized by an progeroid appearance with distinctive facial features and cutis laxa. Ophthalmological, orthopedic, and neurological anomalies are generally also present. This syndrome is rare and the complex therapeutic management, from a surgical but also rehabilitative point of view, has not been recognized. The aim of this paper is to describe a possible rehabilitative protocol, after an orthopedic surgical treatment, in a child with De Barsy Syndrome. A 6-year-old boy was born with a congenital bilateral hip dysplasia associated with bilateral congenital foot deformity (vertical talus). Moreover, he showed stereotypic dyskinetic movements and psychomotor delay with cognitive impairment and absent language; the sitting position was maintained with orthoses to support the trunk control and the standing position was not acquired. He was treated with pinstripe knee-highs for the foot and double nappy for the hips. At 19 months old, he underwent a two stage surgical approach for a bilateral pronated valgus foot with severe talonavicular subluxation. Satisfactory hip range of motion was achieved by conservative treatment alone. Afterwards, for the foot laxity and the flat-pronated foot corrective shoes were prescribed. The main rehabilitative goals were: attention improvement, visual exploration for foot-eye and hand-eye coordination, encourage the essential prerequisites of language, controlling the upright position using support, improving hip-knee-foot relationship, improving load transfer between the right and left sides of the body, and bimanual coordination. The rehabilitation process lasted six months, three times a week, for a time from 30 minutes to 60 minutes per session. The results were encouraging and the patient acquired the possibility of sitting with the indicated postural system, the possibility of assuming an upright position and taking a few steps with the aid of rollator with a postural stabilization system for the pelvis.

Characterization of an Electronic Corneal Prosthesis System.

PURPOSE: Corneal opacity is a leading cause of reversible blindness worldwide. An electronic corneal prosthesis, or intraocular projector, could potentially restore high-quality vision without need for corneal clarity. MATERIALS AND METHODS: Four intraocular projection systems were constructed from commercially available electronic components and encased in biocompatible plastic housing. They were tested for optical properties, biocompatibility, heat dissipation, waterproofing, and accelerated wear. A surgical implantation technique was developed. RESULTS: Intraocular projectors were produced of a size that can fit within the eye. Their optics produce better than 20/200 equivalent visual acuity. MTT assay demonstrated no cytotoxicity of devices in vitro. Temperature testing demonstrated less than 2°C increase in temperature after 1 h. Three devices lasted over 12 weeks under accelerated wear conditions. Implantation surgery was demonstrated via corneal trephination insertion in a cadaver eye. CONCLUSION: This is the first study to demonstrate and characterize fully functional intraocular projection systems. This technology has the potential to be an important new tool in the treatment of intractable corneal blindness.

Defining success in infant penetrating keratoplasty for developmental corneal opacities.

BACKGROUND AND PURPOSE: Neonatal corneal opacities (NCO) are one of the common causes of visual impairment in infants. We present the two infants with penetrating keratoplasty (PKP) for unilateral NCO with different visual and graft outcomes, and illustrate the importance of timing of surgery, importance of visual rehabilitation, and the lack of necessity of a clear graft to achieve reasonable visual function in infants with NCO. PATIENTS AND METHODS: Two infants with unilateral NCO (Peters anomaly) underwent PKP at age of 5.5 weeks (Case One) and 16 weeks (Case Two). Postoperative optical correction, amblyopia therapy, visual and graft outcomes were recorded. RESULTS: At the last follow-up (9.5 years in both the cases), Case One achieved a best-corrected visual acuity (BCVA) of 20/80 with -22 D of contact lens. The graft had a small clear zone centrally but otherwise was opacified to some extent. Case Two achieved a BCVA of 20/125 at 30 cms eccentrically with a clear graft. Case Two was uncooperative for amblyopia therapy and optical treatment. CONCLUSION: For a successful visual outcome in NCO, early PKP, aggressive amblyopia therapy, optical correction, and commitment from the parents for longterm follow-up and demanding treatment are required.

Rehabilitation of vision disabling corneal opacities: is there hope without corneal transplant?

OBJECTIVE: To evaluate the efficacy of contact lenses in visual rehabilitation of a series of patients with corneal scars/opacities. METHOD: Retrospective review of case records of 158 patients (n=162 eyes) with poor vision subsequent to corneal scar/opacity, who underwent contact lens fitting, was done. Primary outcome assessed was quantum of additional improvement in vision with rigid gas permeable (RGP) or soft lenses over spectacles. Success was defined as visual improvement of ≥2 lines over that of spectacles and/or improvement of vision to ≥20/60 (LogMAR 0.48). RESULTS: Rigid gas permeable lenses were fitted in 137 eyes and soft contact lenses in 25 eyes. Lenses were successful in improving visual acuity by two or more lines over that of spectacles in 70% eyes (113 of 162 eyes), of which RGP lens accounted for 85% (96 of 113 eyes). Alternative outcome of success was defined as attainment of reasonable functional vision of >20/60 (LogMAR 0.48). This could be achieved in 65% cases (105 of 162 eyes) of which 83% (88 eyes) were with use of RGP lenses. Almost one-fourth (23.4%) patients were children less than 15 years old. Soft contact lenses were attempted in 25 instances, where RGP lens could not be fitted and were successful in 17 (68%) of these. All these 17 patients were aphakes. CONCLUSIONS: Rehabilitation of corneas with visually disabling corneal opacities is possible with usage of appropriate contact lenses even in young children.

Mechanical superficial keratectomy for corneal haze after photorefractive keratectomy with mitomycin C and extended wear contact lens.

PURPOSE: To evaluate the clinical results of a mechanical keratectomy with mitomycin C (MMC) and extended wear contact lens (EWCL) for the treatment of corneal haze after photorefractive keratectomy (PRK) of high myopia. SETTING: Eye Research Center, Khatam-al-Anbia Eye Hospital, Mashhad University of Medical Sciences, Iran. METHODS: : In a retrospective interventional case series, we enrolled 15 eyes of 9 patients who previously underwent PRK for high myopia and developed corneal haze and regression. Mechanical removal of corneal haze was done by using a surgical blade number 15. Then, MMC (0.02%) was used for 2 minutes. An EWCL was applied for 1 month. The main outcome measures were uncorrected visual acuity, best-corrected visual acuity, spherical equivalent (SE), and corneal haze grade. All patients were followed for a minimum of 6 months. RESULTS: The mean age of the patients was 25.66 ± 7.03 years; 7 patients were men and 2 patients were women. The mean best-corrected visual acuity before superficial keratectomy was 20/80 (range, 20/200-20/50) and improved to 20/20 after treatment (P < 0.05). Thirteen eyes (86.6%) achieved an uncorrected visual acuity of at least 20/40. The mean preoperative SE was 3.91 ± 1.30, and the mean final postoperative SE was -0.85 ± 1.19 (P < 0.05). Eight eyes (53%) were within 1 diopter of emmetropia. Corneal haze in all patients declined to a trace haze or complete clearness. No recurrence occurred during the mean follow-up time of 12 months. CONCLUSIONS: Superficial keratectomy with MMC and EWCLs is effective in reducing persistent and refractory corneal haze after PRK.

Permanent keratoprosthesis combined with pars plana vitrectomy and silicone oil injection for visual rehabilitation of chronic hypotony and corneal opacity.

PURPOSE: To present outcomes of combined pars plana vitrectomy, silicone oil (SO) injection, and permanent keratoprosthesis (Kpro) procedure in prephthisical eyes. METHODS: All 3 patients were monocular with chronic severe hypotony, aphakia, and total corneal opacity in their vital eye. Preoperative visual acuity ranged from light perception to counting fingers at 1 foot. Two patients had a history of failed corneal grafts because of SO, and one had funnel retinal detachment. Pars plana vitrectomy and long-term SO tamponade were performed, and a permanent Boston type 1 Kpro was used in lieu of a donor corneal transplantation. RESULTS: No unexpected intraoperative complications were encountered. Patients were followed for a period of 11-13 months. All patients had anatomic success with an attached retina and a clear visual axis. The procedures resulted in increased visual function in all patients ranging from hand motions to 20/800. No case has progressed to phthisis bulbi during the follow-up period. At the last visit, biomicroscopic examination revealed clear Kpro with an attached retina. CONCLUSIONS: Boston type 1 Kpro implantation, as the primary corneal procedure with pars plana vitrectomy and intraocular SO, may be a viable option in selected patients with prephthisical eyes.

[Pediatric contact lens users in public and private services: comparative analysis]. / Crianças usuárias de lente de contato nos serviços público e privado: análise comparativa.

PURPOSE: To analyze the indications, type and complications of contact lens use and visual acuity in children, in ophthalmological, public and private, services. METHODS: The information from the medical records of 59 contact lens users at a private service (Hospital de Olhos de Pernambuco - Recife - PE- Brazil - group 1), and 43 at public service (Fundação Altino Ventura - Recife - PE - Brazil - group 2), was analyzed. The collected data included: demographic information; age at first examination; indication of lens use; contact lens type; complications and visual acuity. RESULTS: The most common indications of contact lenses in group 1 were: ametropia (55.9%), anisometropia (18.6%) and esotropia (16.9%). In this group leukoma and phthisis were not present. In group 2 the most common indications were: anisometropia (23.2%), ametropia (18.6%), leukoma (18.6%) and phthisis (16.3%). Esotropia was not found in group 2. The most prescribed contact lens was soft and of permanent use in group 1 (45.8%) and in group 2 (32.6%). The most frequent complication in group 1 was discomfort (33.3%) and in group 2 was the loss of the lens (60%). CONCLUSIONS: The most frequent indication in private services was ametropia and anisometropia in the public ones. The type of lens mostly prescribed in both groups was soft and of permanent use. The most frequent complication in group 1 was discomfort and in group 2 loss of the lens. The visual acuity was the same in the majority of the patients.

Capsular opacification after vitreous-sparing cataract surgery in children.

BACKGROUND: The aim of this study was to evaluate different methods of intraocular lens (IOL) implantation and posterior capsule management in the absence of vitrectomy with respect to visual axis opacification (VAO) in children. PATIENTS AND METHODS: Seventy-three eyes of African children undergoing cataract extraction and IOL implantation between 1998 and 2001 were evaluated. In all eyes, the IOL haptics were placed in the bag and the vitreous was preserved. The IOL optic and posterior capsule were managed in the following 3 ways: The IOL optic was captured behind the posterior capsule in conjunction with posterior capsulotomy (PC) in 47 eyes (group 1). The IOL optic was implanted in the bag with PC in 14 eyes (group 2) and without PC in 12 eyes (group 3). Cox proportional hazard analysis and Kaplan-Meier survival curves were performed to evaluate the incidence of Elschnig pearls (EP) and VAO. RESULTS: Elschnig pearls developed in 14.9 % of the patients in group 1, in 56.8 % in group 2 and in 91.7 % in group 3. The occurrence of EP depended significantly on optic capture (p < 0.001) and child's age (p < 0.05), but not on PC (p = 0.084) and eye side (p = 0.1). The persistence of visual axis clarity depended significantly on optic capture (p < 0.001) but not on PC. CONCLUSIONS: In vitreous-sparing cataract surgery, posterior capsule opening does not effectively prevent VAO unless it is in conjunction with IOL optic capture.

Crianças usuárias de lente de contato nos serviços público e privado: análise comparativa / Pediatric contact lens users in public and private services: comparative analysis

Objetivos: Analisar as indicações, tipo, complicações do uso de lentes de contato e acuidade visual em crianças de serviços de Oftalmologia público e privado. Métodos: Os dados dos prontuários de 59 crianças usuárias de lentes de contato em serviço privado (Hospital de Olhos de Pernambuco - Grupo 1), e 43 no serviço público (Fundação Altino Ventura - Grupo 2), foram analisados. A coleta de dados incluiu características sociodemográficas, idade da primeira consulta, indicação do uso da lente, tipo de lente, complicações e acuidade visual. Resultados: As mais comuns indicações do uso de lente de contato no grupo 1 foram: ametropia (55,9%), anisometropia (18,6%) e esotropia (16,9%). Neste grupo o leucoma e phthisis não estavam presentes. No grupo 2, as indicações mais comuns foram: anisometropia (23,2%), ametropia e leucoma (18,6%) cada, e phthisis (16,3%). A esotropia não apareceu no grupo 2. O tipo de lente de contato mais prescrita foi a gelatinosa de uso permanente (não descartável) no grupo 1 (45,8%) e no grupo 2 (32,6%).A complicação mais encontrada no grupo 1 foi desconforto (33,3%) e no grupo 2 perda da lente (60%). Conclusões: A indicação de ametropia predominou nos pacientes privados e as anisometropias nos públicos. O tipo de lente de contato mais prescrita nos dois grupos foi a gelatinosa de uso permanente. A complicação mais frequente no grupo 1 foi desconforto e no grupo 2 perda da lente. A acuidade visual na maioria dospacientes manteve-se.

Purpose: To analyze the indications, type and complications of contact lens use and visual acuity in children, in ophthalmological, public and private, services. Methods: The informationfrom the medical records of 59 contact lens users at a private service (Hospital de Olhos de Pernambuco - Recife - PE - Brazil - group 1), and 43 at public service (Fundação Altino Ventura - Recife - PE - Brazil - group 2), was analyzed. The collected data included: demographic information; age at firstexamination; indication of lens use; contact lens type; complications and visual acuity. Results: The most common indications of contact lenses in group 1 were: ametropia (55.9%), anisometropia (18.6%) and esotropia (16.9%). In this group leukoma and phthisis were not present. In group 2 the most common indications were: anisometropia (23.2%), ametropia (18.6%), leukoma (18.6%) and phthisis (16.3%). Esotropia was not found in group 2. The most prescribed contact lens was soft and of permanent use in group 1 (45.8%) and in group 2 (32.6%). The most frequent complication in group 1 was discomfort(33.3%) and in group 2 was the loss of the lens (60%). Conclusions: The most frequent indication in private services was ametropia and anisometropia in the public ones. The type of lens mostly prescribed in both groups was soft and of permanent use. The most frequent complication in group 1 was discomfortand in group 2 loss of the lens. The visual acuity was the same in the majority of the patients.

Prosthetic contact lenses: adventure or miracle.

PURPOSE: To show that if fitted and handled properly, iris painted contact lenses can be good cosmetic prostheses for disfigured or blind eyes for which no evisceration or enucleation is indicated. METHODS: Twenty patients with an opaque cornea were enrolled in this study. All patients underwent detailed ophthalmologic examinations, including corneal topography and keratometry. The close-up photographs of healthy eyes of the subjects were taken by a camera with a mounted macro lens under daylight. Twenty custom hand-painted contact lenses (Net Lens, Istanbul, Turkey) were selected from the catalog, made according to the photographs, and applied to the patients. Fitting and centration were examined by slitlamp biomicroscopy. RESULTS: Because of troubles in fitting and centralization, contact lenses could not be applied in three of the patients. In the remaining 17 patients, the lenses were applied successfully. All eyes achieved the desired cosmetic and anatomic result. CONCLUSIONS: Besides providing superior cosmesis to evisceration or enucleation, prosthetic contact lenses significantly improve the social relationships and well-being of patients. Fitting a prosthesis over a disfigured or an absolute (blind) eye could be a successful remedy for maintaining the integrity of the orbital anatomy, enhancing the cosmetic appearance, and accelerating the rehabilitation of patients with disfigured blind eyes.

Micro displays as intraocular vision aid--design of an optical system.

All over the world about 10 million people suffer from blindness caused by an opaque, irreversible damaged and inoperable cornea. Many of these people still have intact retinal functions. After Prof. Heimann of the University of Köln miniaturized and implantable displays might help such people to restore partially their vision. Optical and physiological constraints of such vision aids are discussed.

LED-display for an intraocular microoptic system.

In this paper the design and fabrication of an LED array usable for an intraocular vision aid (IOVA) is presented. IOVA is intended for people with vastly opaque cornea caused by explosion or chemical burns. The LED array consists of 1024 single GaP/GaAsP LEDs. In a first step an 8 x 8 pixel LED array has been fabricated. This LED array is bonded to a CMOS driver circuit performing a miniaturized display by using flip-chip-technique. Thus each LED pixel can be driven separately and the generated light passes the substrate.

Image acquisition and image processing for the intraocular vision aid.

The contribution describes an "intraocular vision aid (IOVA)" system for patients suffering from corneal opacification. In order to gain patients' acceptance the system has to be miniaturized to a magnitude that image acquisition, image processing, and power supply can be integrated into a portable unit. A CMOS camera whose dynamic range covers more than 100 dB takes pictures of the scenery. Its image sensor has a resolution of 380 x 300 pixel. In order to reduce fixed pattern noise correlated double sampling is implemented on-chip. In addition, this sensor stands out for low power consumption, random pixel access, and local brightness adaptation. An analog-digital-converter allows direct coupling to an external signal processor or a monolithically integrated unit for image processing to compress data.

Wireless power and data transmission system for a micro implantable intraocular vision aid.

Wireless power and data transmission system developed for an intraocular vision aid for blind patients will be described. This system is applicable for patients suffering from bilateral corneal opacification but with intact posterior ocular. The system consists of an external unit as well as an implant. The external unit is required for image acquisition, channel coding, IR data transmission, and RF power transmission to the implant. The implantable unit contains a CMOS receiver, a receiver antenna coil, and the microdisplay based on a LED array. The CMOS receiver serves for reception and decoding of image data as well as driving circuits for the miniaturized LED array. In this case, mechanical wiring between the external unit and the implant is neither useful nor comfortable. An optimal technical solution needs a wireless data transfer. If the power is transferred to the implant wireless, too, the solution grows ideal. The system described in this communication employs wireless power and data transmission using an 13.56 MHz RF link for power transmission and an near IR (NIR) optical link for data transmission from an external CMOS camera and telemetry unit to the implantable micro display.

Biocompatibility tests on the intraocular vision aid IOVA.

Intraocular miniaturized image transmission systems are developed to restore vision in patients with irreversible destruction of the anterior ocular segment (i.e. chemical burns, explosion trauma, trachoma) and high risk for corneal transplantation, provided that the posterior ocular segment is intact. To ensure safety of such a device biocompatibility tests were conducted. In the present study the effects of light and temperature in various intensities and the toxicity of the implanted materials on neural function in the rabbit retina in-vivo was investigated. All tests give encouraging results concerning the feasibility of intraocular miniaturized image transmission systems.

[Keratoprosthesis from a personal collection 15-year old material]. / Keratoprotezowanie w 15-letnim materiale wlasnym.

The study presents the analysis of a 15-years material concerning the keratoprosthesis in severe corneal leukoma of various aetiology. The material comprises 82 cases of corneal leukoma of the 5th category (hopeless leukoma) caused in the majority of cases by chemical burns as well as post-inflammatory leukoma, on the background of pemphigus, after opacified corneal grafting. The authors discuss the characteristic complications of keratoprosthesis and present their personal results.

Ceratoprótese temporária durante vitrectomia via pars plana / Temporary keraprosthesis during pars plana vitrectomy

A realizaçäo da cirurgia vítreo-retiniana implica na transparência da córnea. Quando a córnea está opaca a observaçäo do fundo do olho se torna impossível, impedindo a cirurgia que, muitas vezes, tem caráter de urgência, como nos casos de descolameto de retina. Desta forma cirurgiöes vítreo-retinianos desenvolveram uma prótese para a substituiçäo temporária da córnea sem transparência. O uso da ceratoprótese temporária tem grandes vantagens, pois, além de permitir o ato operatório com bastante transparência de meios, propicia que ao final da cirurgia vítreo-retiniana seja feito um transplante da córnea, dando, muitas vezes, visäo útil ao paciente. No presente trabalho descrevemos um caso em que foi utilizada a ceratoprótese de Landers-Foulks, devido à opacidade total da córnea, para correçäo de um deslocamento de retina por proliferaçäo vítreo-retiniana