Paul G. Steinkuller

An update on progress and the changing epidemiology of causes of childhood blindness worldwide

PURPOSE To summarize the available data on pediatric blinding disease worldwide and to present current information on childhood blindness in the United States. METHODS A systematic search of world literature published since 1999 was conducted. Data also were solicited from each state school for the blind in the United States. RESULTS In developing countries, 7% to 31% of childhood blindness and visual impairment is avoidable, 10% to 58% is treatable, and 3% to 28% is preventable. Corneal opacification is the leading cause of blindness in Africa, but the rate has decreased significantly from 56% in 1999 to 28% in 2012. There is no national registry of the blind in the United States, and most schools for the blind do not maintain data regarding the cause of blindness in their students. From those schools that do have such information, the top three causes are cortical visual impairment, optic nerve hypoplasia, and retinopathy of prematurity, which have not changed in past 10 years. CONCLUSIONS There are marked regional differences in the causes of blindness in children, apparently based on socioeconomic factors that limit prevention and treatment schemes. In the United States, the 3 leading causes of childhood blindness appear to be cortical visual impairment, optic nerve hypoplasia, and retinopathy of prematurity; a national registry of the blind would allow accumulation of more complete and reliable data for accurate determination of the prevalence of each.

Congenital Dacryocele: A Collaborative Review

Fifty-four cases of congenital dacryocele from several medical centers were reviewed retrospectively. There was strong female preponderance (73%) and unilateral involvement (88%). Lacrimal sac contents could be expressed by local massage through the puncta in 21% of cases. Probing and irrigation were done under general (27.8%) or local (55.6%) anesthesia, while in other cases (16.7%), the cyst resolved before intervention. Recurrence of the dacryocele occurred in 10 patients (22%) after probing. Nasal cysts were visualized in six cases. Marsupialization of nasal cysts was necessary in four cases. In one center, after conservative therapy, 80% of cysts resolved spontaneously and 20% developed dacryocystitis. Surgical intervention is indicated in cases of dacryocystitis, cellulitis, breathing difficulty from large nasal cysts, recurrent dacryocele, and lack of its resolution after a short trial of digital massage.

Therapeutic outcomes of cryotherapy versus transpupillary diode laser photocoagulation for threshold retinopathy of prematurity

INTRODUCTION We undertook this study to compare the clinical outcomes in patients treated for threshold retinopathy of prematurity (ROP) with transpupillary diode laser photocoagulation versus transscleral cryotherapy. METHODS A retrospective chart review was performed of patients treated for threshold ROP at our institution between 1988 and 1997. Cryotherapy was used to treat patients before 1992, and diode laser was used thereafter. One hundred fifteen eyes of 63 patients underwent cryotherapy, and 130 eyes of 70 patients underwent laser treatment. Because strong concordance exists between fellow eyes treated for ROP, statistical analysis was done for right eye outcomes only. Demographics, short-term complications, and treatment parameters were compared on the entire cohort of patients. Structural outcomes were compared between a group of 79 cryotreated eyes (39 OD, 40 OS) and a group of 113 laser-treated eyes (56 OD, 57 OS). Mean cycloplegic refraction was compared between a group of 38 cryotreated eyes (18 OD, 20 OS) and a group of 90 laser-treated eyes (46 OD, 44 OS). Visual acuity for preverbal and nonverbal children was estimated and converted to a Snellen visual acuity equivalent. Geometric mean visual acuity was then compared between a group of 44 cryotreated eyes (22 OD, 22 OS) and a group of 92 laser-treated eyes (47 OD, 45 OS). Patients were included in each outcome analysis if adequate documentation was present in the patient record. RESULTS Demographics and short-term complications were not significantly different between the 2 groups. In the statistical group, 22 of 39 right eyes (56.4%) in the cryotherapy group versus 49 of 56 right eyes (87.5%) in the laser group had resolution of ROP after treatment (P = .0008). Mean spherical equivalent cycloplegic refraction at 12 months of age and over the follow-up period was not significantly different between the cryotherapy and laser groups. Estimated geometric mean visual acuity in the cryotherapy group was 20/103 and in the laser group was 20/49 at 12 months of age (P= .0099). CONCLUSIONS Diode laser photocoagulation was associated with a better long-term structural outcome and visual acuity compared with cryotherapy for the treatment of threshold ROP. Refractive error was not significantly different between the 2 groups over an equivalent follow-up period.

Blindness and eye disease in Kenya: ocular status survey results from the Kenya Rural Blindness Prevention Project.

A series of eight regional eye surveys were conducted in Kenya as part of the Kenya Rural Blindness Prevention Project. Each survey consisted of clinical examinations of about 1800 individuals selected by a random cluster sampling technique in geographically distinct and culturally homogeneous rural areas; 13,803 examinations were completed in all. Together these surveys provide the basis for national estimates of the prevalence and aetiology of visual loss and ocular pathology. The results showed that 0.7% of rural Kenyans are blind in the better eye by WHO standards, and another 2.5% suffer significant visual impairment. Rates of visual loss tend to increase five-fold in each 20-year age cohort. Females have higher prevalence of visual loss than males over age 20, and certain geographical areas have markedly higher rates. The commonest cause of both blindness and visual impairment is cataract, accounting for 38% of all visual loss. Trachoma (a localised problem), glaucoma, macular degeneration, and severe refractive errors follow cataract as leading causes of blindness in the better eye. Trauma, corneal scars of various causes, phthisis, and staphyloma are important causes of monocular blindness. Nutritional eye disease does not appear to be a problem of any magnitude in rural Kenya.

Optic neuritis in children: Clinical features and visual outcome☆☆☆

BACKGROUND The purpose of this paper is to study the clinical spectrum of pediatric optic neuritis. We evaluated the presenting features, neuroimaging findings, cerebrospinal fluid abnormalities, associated systemic disease, and visual outcome in patients with this condition. METHODS A retrospective analysis was performed on all patients who came to Baylor College of Medicine with optic neuritis during a 6-year period from 1991 to 1997. The degree of initial visual loss, subsequent visual recovery, and associated disease were reviewed. Magnetic resonance images and cerebrospinal fluid findings were also analyzed. RESULTS Twenty-five patients (39 eyes) 21 months of age to 18 years of age were included in the study, with a mean follow-up of 11 months. Fourteen patients (56%) had bilateral optic neuritis, and 11 patients (44%) had unilateral disease. Thirty-three of 39 eyes (84%) had visual acuity of 20/200 or less at presentation. Twenty-one of 25 patients (84%) were given intravenous methylprednisolone (10 to 30 mg/kg/day). Thirty of 39 eyes (76%) recovered 20/40 visual acuity or better. Three of 39 eyes (7%) recovered vision in the 20/50 to 20/100 range. Six of 39 eyes (15%) recovered vision of 20/200 or less. Twenty-three of 25 patients (92%) underwent magnetic resonance imaging of the brain. A normal magnetic resonance image of the brain was associated with recovery of 20/40 or better visual acuity in 6 of 6 affected eyes (100%). Seven patients were 6 years of age or younger at presentation. Six of 7 (85%) had bilateral disease, and 12 of 13 (92%) affected eyes recovered 20/40 visual acuity or better. Eighteen patients were 7 years of age or older at presentation. Eight of 18 (44%) had bilateral disease, and 10 of 18 patients (56%) had unilateral disease. Eighteen of 26 affected eyes (50%) recovered 20/40 visual acuity or better. CONCLUSION Pediatric optic neuritis is usually associated with visual recovery; however, a significant number (22%) remain visually disabled. A normal magnetic resonance image of the brain may be associated with a better outcome. Younger patients are more likely to have bilateral disease and a better visual prognosis.

Pharmacokinetics of bevacizumab and its effects on serum VEGF and IGF-1 in infants with retinopathy of prematurity.

PURPOSE To measure serum levels of bevacizumab and to compare serum levels of free vascular endothelial growth factor (VEGF) and insulin-like growth factor-1 (IGF-1) in infants who were treated with either intravitreal injection of bevacizumab (IVB) or laser for type 1 retinopathy of prematurity (ROP). METHODS Twenty-four infants with type 1 ROP were randomized into three treatment groups: IVB at 0.625 mg per eye per dose, IVB at 0.25 mg per eye per dose, and laser. Blood samples were collected prior to treatment and on posttreatment days 2, 14, 42, and 60. Weekly body weights were documented from birth until 60 days post treatment. Serum levels of bevacizumab, free VEGF, and IGF-1 were measured with enzyme-linked immunosorbent assay (ELISA). RESULTS Serum bevacizumab was detected 2 days after the injection, peaked at 14 days, and persisted for up to 60 days with half-life of 21 days. Area under the curve (AUC) analysis showed that systemic exposure to bevacizumab was variable among the subjects and was dose dependent. Serum free VEGF levels decreased in all three subgroups 2 days post treatment, with more significant reductions found in both IVB-treated groups, P = 0.0001. Serum IGF-1 levels were lower in both IVB-treated groups. CONCLUSIONS Clearance of bevacizumab from the bloodstream in premature infants takes at least 2 months. Although serum free VEGF levels decreased following either laser or bevacizumab treatment, the reductions were more significant in the IVB-treated groups. Potential long-term effects of systemic exposure to bevacizumab in infants need to be studied further.

Ocular Medications in Children

Many ocular medications are used by pediatricians or ophthalmologists caring for pediatric patients. Topical antibiotics are commonly prescribed for bacterial conjunctivitis, nasolacrimal duct obstructions, and ophthalmia neonatorum. Many new antiallergy eye drops are now available for the treatment of seasonal (hay fever) conjunctivitis. Dilating eye drops and antiglaucoma medications are generally used or prescribed by ophthalmologists, but pediatricians must be aware of their potentially serious systemic side effects. Before initiating treatment, physicians should evaluate the risks and benefits of ophthalmic medications, establish minimum dosages necessary to achieve a therapeutic benefit, and monitor children for local and systemic side effects.

The Epidemiology of Trachoma in Rural Kenya: Variation in Prevalence with Lifestyle and Environment

PURPOSE Ocular examination surveys were carried out in Kenya by the International Eye Foundation as a component of the Kenya Rural Blindness Prevention Project to determine the national prevalence of blindness and ocular morbidity and major causes. A goal of the surveys was to determine the overall geographic distribution and severity of trachoma throughout Kenya. METHODS Using a random cluster household sampling technique, 13,803 people of all ages and of diverse cultural and ethnic backgrounds were identified in eight regions of Kenya. A detailed examination for active and inactive trachoma was carried out on each person surveyed as part of the general ocular examination. RESULTS The prevalence rate of visual loss (< 20/60) due to trachoma in the better eye was 7.2/1000. Active trachoma was present in 19% of all persons examined, and 50% of all those with trachoma were found to have moderate to severe inflammation. Prevalence varied according to survey region from less than 1% in four regions where agriculture is the economic base, to 57% and 63% in two arid pastoral regions. Trachoma prevalence varied from 28% in children younger than 3 years of age to 11% in persons older than 60 years of age. Potentially blinding eyelid deformities secondary to chronic trachoma occurred in 5.0% of the rural population, and 1.2% of the rural population displayed associated corneal scarring. Lid scarring, corneal scarring, and lid deformities were greater in prevalence among females of all age groups when compared with males. CONCLUSIONS Trachoma prevalence in Kenya varies widely from region to region. High prevalence is associated with high climatic aridity, and lower prevalence is associated with areas of greater rainfall, sustainable agriculture, and a higher general standard of living. Within high-risk regions, there are wide variations in age-specific prevalence and severity of the disease. Potentially blinding sequelae of trachoma are more prevalent in females than in males.

Early-onset refractive accommodative esotropia.

INTRODUCTION We studied the natural history of pure refractive accommodative esotropia after spectacle correction in patients with onset before 1 year old to determine whether their outcomes and characteristics were different from those of patients with more typical age at onset of refractive accommodative esotropia. METHODS We retrospectively reviewed the charts of 17 children with onset of refractive accommodative esotropia before 1 year old. Records of 26 children with onset after 2 years old were reviewed as controls. RESULTS The mean ages at diagnosis were 9 months and 48 months for the study and control groups, respectively. All 17 study patients and all 26 control patients were initially well aligned with spectacles at distance and near. Follow-up averaged 34 months for study patients and 41 months for control patients. Three study patients (17.6%) and 1 control patient (3.8%) had eventual deterioration and required strabismus surgery (P = .28). None of the study patients developed amblyopia, whereas 42% of control patients did (P = .001). Seven of 15 (47%) of the study patients with known birth history were born prematurely, whereas only 3 of 24 (12%) control patients were born prematurely (P = .03). CONCLUSIONS Refractive accommodative esotropia was diagnosed at as early as and age 4 months. Prematurity appeared to be a risk factor. Amblyopia was not detected in any patient with early-onset refractive accommodative esotropia. Treatment with full hyperopic spectacle correction led to long-term stable alignment, with relatively few patients requiring surgery.

Cataract: The leading cause of blindness and vision loss in Africa

Senile cataract is a non-preventable disease of aging, having its biggest impact in the over-60 age group. Published clinic and hospital data, population-based surveys and World Health Organization estimates indicate that 1.2% of the entire population of Africa is blind, and that cataract causes 36% of this blindness. Ocular disease and ophthalmic manpower status questionnaires mailed to every African country in 1982 by the International Eye Foundation support these contentions, and further demonstrate that cataract is the biggest single cause of blindness on the continent. Taking the population of Africa as a whole, this means that 6,000,000 people are blind, and that 2,000,000 of them are blind due to cataract. As this form of blindness is so closely related to aging, an estimate of the annual incidence of the condition can be made. One method is to use the formula I = P/D, where I = annual incidence, P = overall prevalence and D = duration of the condition. A 5-year survival has come to be a commonly used estimate in developing world epidemiology for the longevity of a person who becomes blind due to senile onset cataracts. In Africa then, I = P/D = 2,000,000/5 = 400,000 people blinded annually by cataract. An alternate method for this computation is to multiply the number of people entering the 60 and above age group annually by 0.08. This factor is derived from knowing the prevalence of cataract blindness in this age group, the life expectancy on becoming 60, and the number of people turning 60 annually. This gives an annual incidence of 160,000 and would signify that the development of cataract blindness does not reduce longevity. Follow-up prevalence surveys in the same population group will tell us which method (if either) is correct. The average number of cataract operations done annually by each ophthalmologist in Africa is surprisingly low, 120. The two most cost-effective ways of raising the number of cases done are: (1) shorten the average hospital stay, and (2) train more ophthalmic clinical officers to perform cataract surgery.