The disabled who help themselves.
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Persons with Disabilities↗
Biomedical subjects
R Bowell
Publications and source records attributed to R Bowell.
Outcome and complications of intraocular lenses in children with cataract.
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.
Cataract↗
Proteus syndrome: ocular complications.
Proteus syndrome is a recently recognized hamartoneoplastic malformation syndrome of uncertain etiology and variable expression, whose cardinal manifestations are pigmented nevi, hemihypertrophy, macrodactly, lipomata, and cerebroid-gyriform configuration of the skin on the soles of the feet. The characteristic features may be present at birth but become more apparent with time. In the past this syndrome has been confused with other overgrowth disorders such as neurofibromatosis, Klippel-Trenaunay-Weber syndrome, Maffucci syndrome, and Bannayan syndrome. The ophthalmic features of the proteus syndrome require clarification. We review the ocular findings in 16 previously described cases and describe the findings unique to our patient, in particular, unilateral epibulbar and suspected posterior segment hamartomas.
Child↗
Ophthalmic findings in classical galactosemia--a screened population.
Classical galactosemia due to a deficiency of galactose-1-phosphate-uridyl transferase, is an autosomal recessive disorder of galactose metabolism with an incidence in Ireland of one in 30,000 births. It can result in cataract formation through the accumulation of galactitol within the lens. Seventeen children with transferase deficient galactosemia were studied. Early diagnosis followed by a galactose-free diet and tight biochemical control prevented cataract formation in 13 cases after a mean follow-up of 6.3 years. Cataracts did not regress in all patients commenced on diet by 6 weeks but early treatment prevented progression. The ophthalmologist may play an important role in the monitoring of patients with this disease as the recognition of new lens opacities by slit-lamp biomicroscopy may be the most sensitive initial index of inadequate biochemical control.
Adolescent↗
Intellectual and educational attainment in albinism.
We studied the intellectual and educational ability of a group of 18 albino children as compared with that of a group of demographically matched controls. Intellectual ability was measured using Raven's Standard Progressive Matrices, and educational ability was measured by the reading, spelling, and arithmetic tests of the Wide Range Achievement Test. There was no statistically significant difference in intelligence of the two groups. However, there was a significant difference in reading, spelling, and arithmetic skills. No correlation was found between the clinical features of the albino children and their performance on the intelligence or educational tests.
Adolescent↗
Uniocular childhood blindness: a prospective study.
PURPOSE: This prospective study examines uniocular blindness among children younger than 16 attending a large pediatric ophthalmology department. The aim was to identify the causes of uniocular blindness and determine how much is preventable. We defined blindness according to the World Health Organization definition of vision worse than or equal to 3/60. METHODS: All children who attended the department and were blind in one eye during the period of the study were included. A history was taken, visual acuity was assessed by an age-appropriate method, and an ocular examination was carried out. When necessary, the child also was seen by an orthoptist or pediatrician. RESULTS: A total of 71 patients were identified. At the time of diagnosis, the patients varied in age from a few weeks to 15 years. The causes were classified into one of the following diagnostic categories: chromosomal/genetic (8.5%), prenatal (47.9%), perinatal (7.0%), or childhood (36.6%). Many cases are prenatal in origin and are not preventable. Other causes that are difficult to prevent include retinoblastoma, toxocariasis, and trauma. One cause, amblyopia, may be preventable or treatable in most instances if detected early. CONCLUSIONS: Most cases of uniocular blindness are not preventable; however, protective glasses should be encouraged to prevent injury to the good eye.
Adolescent↗
Ocular findings in glutaric aciduria type 1.
PURPOSE: To determine the nature and course of ocular abnormalities in glutaric aciduria (acidemia) type 1 (GA1). METHODS: Fifteen children with GA1 have been studied in the Republic of Ireland. A retrospective review of the records of the 6 children who died during their illness and prospective clinical examination of 9 survivors were performed. RESULTS: Seven of the 15 children had abnormal eye findings. Ocular complications included intraretinal hemorrhages, cataract, gaze palsy, strabismus, ametropia, and pigmentary retinopathy. CONCLUSION: Ocular involvement is common in glutaric aciduria. Complete ophthalmologic evaluation is recommended in all patients suspected to have this rare disease. Intraretinal hemorrhages due to GA1 could be misinterpreted as resulting from child abuse, and it is important to include this disorder with the differential diagnosis of child abuse.
Amino Acid Metabolism, Inborn Errors↗
Childhood penetrating eye injuries.
We reviewed 41 children with penetrating eye injuries from birth to 15 years of age between August 1982 to May 1990. Male to female ratio was 3.56:1. Thirteen (33%) cases resulted in monocular blindness. The distribution of penetrating injuries was based on the site of perforation: corneal, corneoscleral and scleral groups. The commonest cause of injury was child related from a thrown object (42%) and hazardous toys (21%).
Adolescent↗