Biomedical subjects
Publications and source records attributed to R Bowell.
We report on binocular function and amblyopia in children who had surgery for infantile eosotropia in the first 24 months of life. Fourteen (56%) achieved peripheral fusion and no child obtained high grade stereopsis. Following surgery and treatment for amblyopia 20% remained amblyopic.
AIMS: To assess the outcome and complications of patients with advanced retinopathy of prematurity (ROP). METHODS: All patients with eyes achieving stage 4 or 5 retinopathy of prematurity were reviewed. Twenty one eyes were diagnosed during ROP screening in maternity hospitals and 10 eyes were of infants transferred for treatment. RESULTS: Thirty one eyes of 17 patients were included. Thirteen eyes were treated for acute disease but progressed to stage 4 or 5; seven had cryotherapy and six diode laser photocoagulation. Cataract was found in 17 eyes (54.8%), glaucoma in seven eyes (22.6%), microphthalmos in 15 (48.4%), and corneal opacification in four eyes (12.9%). Fifteen eyes had surgical procedures; two (6.5%) had trabeculectomy, four (12.9%) had lensectomy, and nine (29%) retinal detachment repair. Transferred infants had their initial eye examination later than infants in hospitals screened by the authors and 80% of them had progressed beyond threshold ROP by the time they were transferred for treatment. Twenty nine eyes (93.6%) had visual acuities of 3/60 or less and only two eyes (6.5%) achieved 6/18 or less. CONCLUSION: The visual outcome of the eyes undergoing retinal re-attachment surgery was disappointing. Cataract, microphthalmos, and glaucoma were the most frequent complications, and surgical intervention was often required. The need for children who are blind as a result of ROP to have long term follow up is shown.
BACKGROUND: The treatment for infantile glaucoma is surgical. Treatment options include goniotomy, trabeculotomy, combined trabeculotomy-trabeculectomy, and trabeculectomy. METHODS: Patients who had a follow up of 5 years or longer after primary trabeculectomy were examined to determine the long term stability in infantile glaucoma. RESULTS: In eyes with primary infantile glaucoma 92.3% achieved control of their glaucoma with a single trabeculectomy; 100% achieved control with two trabeculectomies; 85.7% of eyes with secondary infantile glaucoma achieved control with a single trabeculectomy. There were no serious complications experienced in either group. CONCLUSION: Primary trabeculectomy is a safe and successful operation for infantile glaucoma.
Congenital dacryocele is a distension of the naso-lacrimal sac usually present at birth or in the first three months of life. It occurs commonly in females and may present with dacryocystitis. Treatment includes probing and antibiotics if infection is present.
Thirty three children with classical galactosaemia diagnosed through newborn screening are considered. It is concluded that cataract formation has a direct relationship with poor dietary control. Erythrocyte galactose-1-phosphate (Gal-1-P) levels do not correspond to cataract formation unless many times higher than normal. The value of crystalline lens biomicroscopy is confirmed as a useful method for monitoring the dietary and biochemical control in classical galactosaemia.
Abnormalities of the central nervous system are frequently described in optic nerve hypoplasia. In a longitudinal study of 46 consecutive children (32 term, 14 preterm) with bilateral optic nerve hypoplasia 32 (69.5%) had associated neurodevelopmental handicap. Of these, 90% had structural central nervous system abnormalities on computed tomographic brain scans. Neurodevelopmental handicap occurred in 62.5% of the term and 86% of the preterm infants respectively. Term infants had a greater incidence of ventral developmental midline defects and proportionately fewer maternal and/or neonatal complications throughout pregnancy, while encephaloclastic lesions were commoner among the premature infants. An association of optic nerve hypoplasia with the twin transfusion syndrome and prenatal vascular encephalopathies is described.
Eight children with the fetal alcohol syndrome are described with ocular anomalies. They all had a strong history of maternal alcohol abuse throughout pregnancy, especially in the first trimester. All the children had eye abnormalities. These included external eye lesions, Peters' anomaly, lens opacification, ocular motility disorders, and optic nerve hypoplasia.
Maple Syrup Urine Disease is an autosomal recessive disorder of branched chain amino acid metabolism with an incidence in Ireland of one in 140,154 births. Ocular complications in untreated or late diagnosed patients includes optic atrophy, grey optic papilla, nystagmus, ophthalmoplegia, strabismus and cortical blindness. Seven patients with maple syrup urine disease were studied. All were diagnosed with the aid of newborn screening and commenced on early dietary treatment (mean age at diet introduction = 5 days). All remain physically well, with average intellectual performance, three having minor neurological defects and one strabismus. Early diagnosis, proper therapy and subsequent vigilant management may reduce substantially the risks of ophthalmic complications in this rare disease.
Eighty five visually handicapped children who attended the eye department were assessed. The children had a mean age of 3.6 years--range 0.3-14.7 years. The aetiological factors for severe visual handicap (visual acuity less than 3/60) were evaluated into prenatal factors; factors operating at birth and the immediate perinatal period; or factors operating in childhood. Genetic counselling and improvements in the management of pre-term infants is recommended in the reduction of childhood blindness. A national computerised childhood blind register should be established.
The reported success rates in the treatment of congenital glaucoma with goniotomy, trabeculotomy, and trabeculectomy suggest that trabeculectomy should be performed if the other procedures fail. We propose that the decision to perform primary trabeculectomy in primary and secondary congenital glaucoma reduces the effect which the many variable findings in surgical anatomy may have on the outcome of other procedures. This is a retrospective study of the results of primary trabeculectomy in 21 consecutive eyes of 15 patients with congenital glaucoma. Eighteen of 13 patients' eyes were controlled after a single trabeculectomy and remained controlled after a mean follow-up of 3.9 years (range 1.5 to 6.7 years). The role of primary trabeculectomy in congenital glaucoma merits further consideration.
Homocystinuria due to cystathionine-beta-synthetase deficiency is an autosomal recessive disorder of methionine metabolism with an incidence in Ireland of 1 in 52,544 births. Ocular complications in untreated patients include ectopia lentis, secondary glaucoma, optic atrophy, and retinal detachment. There are no characteristic signs or symptoms in infancy, and early detection relies on screening of newborn babies. Nineteen patients with homocystinuria were studied; 14 received dietary treatment and vitamin supplementation starting in the newborn period. Of these, none developed ectopia lentis after a mean follow-up of 8.2 years, compared with a 70% dislocation rate in untreated patients with a similar follow-up period. Ectopia lentis developed and progressed in five patients diagnosed later in life, despite tight biochemical control. The risk of ocular complications in homocystinuria can be substantially reduced in patients started on treatment within six weeks of birth.
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Seven patients, who satisfied the criteria laid down by Mason and Barnes (1969) for inclusion under the heading Behçet's disease, were assessed clinically and investigated with particular reference to immune status and blood fibrinolytic activity. In one patient in whom it was possible to obtain an eye for examination, there was evidence, on microscopic examination, of an immune vasculitis as well as clot formation, particularly in the veins. All the patients were treated with fibrinolytic agents and the favourable results obtained with this treatment suggest that it should be considered as the first line of therapy in what is otherwise a very difficult condition to manage.
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PURPOSE: To assess prospectively the complications and changes in refraction, axial length, and keratometry after intraocular lens (IOL) implantation in children with congenital, developmental, and traumatic cataracts. SETTING: The Children's Hospital, Dublin, Ireland. METHODS: The study comprised 24 eyes of 20 patients, aged 4 weeks to 12 years, who had extracapsular cataract extraction and posterior chamber IOL implantation. Mean follow-up was 103 weeks (range 34 to 270 weeks). RESULTS: Six eyes with congenital cataracts operated on between 4 and 28 weeks of age had central, steady, maintained fixation postoperatively. In the developmental cataract group, 64% achieved a visual acuity of 6/24 or better and 43%, 6/12 or better. In the three traumatic cases, visual acuities were 6/6, 6/9, and 6/24. Posterior capsule opacification occurred in 95.8% of eyes and was treated with a neodymium: YAG or Zeigler knife posterior capsulotomy as a secondary procedure. Other postoperative complications (membranous uveitis, iris capture, posterior synechias, iris prolapse) occurred in 29.2% of eyes. CONCLUSION: With careful management and patient selection, the use of IOLs in children can produce good visual results with a minimum of complications. Further follow-up is needed to assess the long-term visual outcome, complications, and changes in refraction.