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Articles Regarding Visual Loss in Children,
its Prevention and Treatment:

Riad, SF, Dart, JK, Cooling, RJ: Primary care and Ophthalmology in the United Kingdom. British Jnl Ophthalmology, 87:380, 2003.
The National Health Service of Great Britain is now primary care led. Because of many structural problems, there is an urgent need to structure where such care should be targeted. The obvious areas are management of minor ills and trauma; screening and detection as well as prevention of disease where possible; follow up and support for chronic conditions where possible. Because of the limited supply of ophthalmologists in the British Isles the problem has surfaced there earlier than it might in the US.

Mafee, MF, Mafee, RF, et al: Medical Imaging in Pediatric Ophthalmology. Pediatric Clinics of North America, 50:259, 2003.
The use of Computed Tomography (CT) and Magnetic Resonance (MR) Imaging has improved our understanding of many congenital and acquired conditions in pediatric ophthalmology, resulting in more appropriate therapeutic interventions and, as well, giving insight into the pathogenisis.

Tran, T, Kaufman, LM: The Child’s Eye In Systemic Disease. Pediatric Clinics of North America, 50:241, 2003.
Ocular manifestations of many pediatric systemic diseases as well as tips in practical management are the subject of this review article. Practical advice is given regarding the management of the ocular involvement for the primary physician.

Thompson, L, Kaufman, L: The Visually Impaired Child. Pediatric Clinics of North America, 50:225, 2003.
This article discusses the causes of childhood blindness and outlines how the primary care provider may begin appropriate steps toward the management of the impaired child. How to access community resources, low vision programs, school resources are discussed. The strategies which have world wide application may prevent some conditions which result in blindness. Among others diet, hygiene and immunizations play a role.

The Eye Examination in Infants, Children and Young Adults. Ophthalmology, 110:860,2003.
The principles to guide Pediatricians in the art and science of a thorough examination of the eyes of their patients is the subject of this article. This is often a neglected area in the education and practice of Pediatricians.

Josiatis, C, Matisoff, M: Familial Dysautonomia (Riley Day Syndrome) in review: Diagnosis, Treatment of Ocular Manifestations. Advances in Experimental Medicine & Biology, 506 (A): 71, 2002.
Review of the diagnosis and treatment of ocular manifestation in this rare but very damaging syndrome to the eye.

Hanioglu-Kargi, S, Koksal, M, Tomac, S, et al: Ophthalmologic abnormalities in children from a Turkish school for the deaf. Turkish Jnl of Pediatrics, 45: 39-42, 2003.
Complete ophthalmologic examinations were performed on 104 deaf children age 7 to 20 years. 42 children had some ophthalmologic abnormality, 31 being refractive error (29.8%). Nineteen had an eye movement disorder (mainly strabismus and nystagmus). Two had corneal or lens opacities and nine had retinal abnormalities. Routine eye exams are important in this population because of the use of sight to compensate for the handicap of deafness.

Edelsten, c, Reddy, M, Stanford, M, Graham, E: Visual loss associated with Pediatric Uveitis in English Primary and Referral Centers. Amer Jnl Ophthal, 135:676, 2003.
This multicenter, retrospective case control (patients recruited had the disease) of 249 patients under the age of 20 years from three primary and two referral centers were studied for different type of uveitis (An inflammation of the lining coats of the eye), associated systemic disease, age, sex and visual loss (Acuity and Visual Field) between hospitals. In the primary centers the most frequent diagnosis was idiopathic (no known cause) in 78%. In the referral centers the diagnosis was arthritis- associated uveitis ( Juvenile Rheumatoid Arthritis in USA)in 67%. Visual loss to the level of less than 20/50 was found in one out of six children. Visual loss was more common in children requiring systemic treatment ( pills or shots) 2 times, or surgery, eight times than in children requiring only topical eye medications.

Bullard, S, Donahue, S, Feman, S, Sinatrea, RB, et al: Decreasing incidence and severity of Retinopathy of Prematurity(ROP). Jnl of American Assoc of Ped Ophth and Strabismus, 3:321, 1999.
Two cohorts of premature infants of the same birth weight were studied. The first was infants seen from 1/1/86 to 11/30/87. The second was 7/1/ 95 to 6/30/96. The overall incidence was found to have decreased by 27% and this was clinically and statistically significant. The incidence of ROP requiring treatment also decreased. While this is encouraging it should be interpreted with care. The population of infants with serious eye disease was small (11) and it may be a statistical fluke. Some centers are experiencing an increase in the number and percentage of serious disease in infants less than 750 grams ( the lowest birth weigh tin this study) which are now routinely surviving (500-650 grams).

Gair , E, Adams, G: Normal Visual Development after Unilateral Complete Ptosis. Jnl Amer Assoc of Ped Ophth & Strabismus, 2: 127, 2000.
A five year old boy was born with unilateral complete Ptosis (drooping of the lid to completely cover the eye). It was due to an hemangioma (Blood vessel ) tumor. After six weeks it resolved spontaneously . Vision, alignment, depth perception developed normally thereafter. This adds evidence that complete closure of one eye can be tolerated for up to six weeks without permanent damage to the developing visual system.

Functional Magnetic Resonance Imaging as a tool for investigating amblyopia in the human visual cortex. A pilot study. Jnl Amer Assoc of Ped Ophth & Strabismus, 6: 300, 2002.
This is a preliminary study to determine if differences exist between the amblyopic and normal eye when studied by magnetic resonance imaging -a new and sophisticated technique. Subjects with amblyopia exhibit a smaller “area of activation” when studied by this technique than does the normal eye in the same person stimulated by the same stimulus. This difference also persists when compared to controls in subjects that have no amblyopia. This is in keeping with current concepts of amblyopia, i.e., that it is deprived of cortical processing area as compared to the normal eye.

Axial Myopia associated with dense vitreous hemorrhage of the neonate Jnl Amer Assoc of Ped Ophth & Strabismus, 6: 348, 2002.
A massive bleed into the vitreous is known to be a rare cause of the development of axial myopia (elongated growth of the eye). This study was designed to determine if the myopia was axial (current thinking) or refractive (change in the curvature or power of the optical elements of the eye) or both. Four infants were studied. Their refractive error, and A scan ultrasonography were done. Three of the four developed an elongated growth of the posterior segment of the eye, a myopia in excess of -11.00 diopters, and dense amblyopia. The fourth infant’s vitreous bleed cleared within 11 days and developed mild amblyopia. It was the conclusion of this study that hemorrhages persisting longer than 4 weeks in a new born infant should be removed surgically by vitrectomy to avoid deprivation amblyopia.

Watts, P, Neveu, M, Holder, G, et al: Visual Evoked Potentials in successfully treated Amblyopia. Jnl Amer Assoc of Ped Ophth & Strabismus, 6: 389, 2002.
The pattern reversal visual evoked potential were recorded in 11 successfully treated amblyopes age 7 to 11 years, and 10 age matched normal children. No pre treatment VEP were recorded. Result: VEP’s from the amblyopic eye showed significantly longer latencies for small check size stimuli. However the same defect in latency (time of arrival of the stimulus to the visual cortex) was seen in the fellow eye of the amblyopes suggesting a central processing defect.

Lit, ES, et al: International Ophthalmolgy Clinics 42:107, 2002.
Review article of the damage that can be done to the eye by retained foreign bodies due to injuries.

Blomdahl, S. et al; Patient focused urban Tele-ophthalmology services. Jnl of Telemedicine & Telecare 8: Suppl 2:43-4, 2002.
This article sets out how telemedicine network can be established so that general practioners, pediatricians, family physicians can access eye specialists via a telemedicine network. It’s problems too.

Mansour, AM et al. :Ocular pathology in acquired immunodeficiency syndrome. Comprehensive Therapy. 28: 155-9, 2002.
A comprehensive study including pathology of the ocular lesions of the HIV virus infected individuals.

Berrocal, AM,et al: Uveitis following intraocular surgery. Ophthalmolgy Clinics of No America. 15:357-64,2002.
A not infrequent complication, often overlooked, following intraocular surgery, which may prolong or retard recovery from the surgery if not diagnosed and adequately treated.

Mafong, DD, et al: Ocular findings in children with sensorineural hearing loss. Archives of Otolaryngology-head and neck. 128:1303-6,2002.
Thirty one percent of children with sensorineural hearing loss had ocular abnormalities. Refractive errors were commonest. The remaining patients had other, treatable in most cases, abnormalities. Children with SNHL should have a complete eye exam to detect these defects.

Madden, AC, et al: Eye Health in rural Australia. Clinical & Experimental Ophthalmology. 5: 316-21, 2002.
Rural health care is one of the most woorisome areas of health care primarily due to neglect and under service. This study is a population based survey of a rural population not unlike that found in America. An increased prevalence (more people in the total population) are affectred with common eye disorders including children in this study. There is a need to develop telemedicine networks and to increase the rural work force of ophthalmologists to meet the needs of rural populations.

Bulgan, T.,et al: Prevalence and causes of severe visual impairment in children in Mongolia. Ophthalmic Epidemiology. 9:271-81.
Mongolia is far away from the United States yet in this study which was fairly complete among the children an incidence of 19/ 100,000 children with blindness or severe visual impairment was found. Not very different than population based studies in the US and other developed countries! The causes were somewhat different in that infection, particularly meningococcal meningitis was high on the list. A vaccine is available for that. Cataract untreated and refractive errors, the usual suspects were among the other causes. Both are treatable.

Al-Til, M.et al: Ophthalmic findings in Wolfram syndrome. European Jnl of Ophthalmology. 12:84-8, 2002.
Wolfram Syndrome (diabetes insipidus, (water losing diabetes) optic atrophy, deafness) was studied in 15 patients in Amman, Jordan. Optic atrophy was present in 93%, color vision defects in 92% (probably secondary to OA), cataract 66.6%, pigmentary retinopathy, 30 %, (resembles retinitis pigmentosa) and diabetic retinopathy, Except for the prevalence of cataract this group resembles what has been reported and suggests the need to see and follow these children by an ophthalmologist.

Kim, JH, Characteristic ocular findings in Asian children with Down Syndrome. Eye.: 16: 710-14, 2002.
The ocular findings, quite similar to what is seen in Caucasian children, are: Upward slant of the lid fissures(hence the name) epicanthus, an extra fold of skin over the eyelid by the nose, epiblepharon, an extra fold of skin along the lower lid, assorted refractive errors which tend to be high, strabismus, cross or wall eye condition, tear duct obstruction, cataracts, and glaucoma. Interestingly Brushfield spots, tiny freckles on the Iris and keratoconus, a conical deformity of the cornea, though uncommon in Caucasians, was not found at all, in the Korean children. Children with Downs Syndrome need careful and liflong follow up by ophthalmologists.

Shanske, S,et al: Identical Mitochondrial DNA deletionin a woman with ocular myopathy and in her son with Pearson Syndrome. Amer Jnl of Human Genetics. 71:679-83, 2002.
Single deletions of mitochondrial DNA, (the DNA which comes exclusively from the cytoplasm of the mother’s ovum) produces major disorders among which are three of eye importance: Kearns Sayre syndrome involving eye muscles and retina, the heart muscle, hearing and other systems; Pearson syndrome involving the blood forming system and Progressive Ophthalmoplegia Externa (PEO) affecting the eye muscles and other larfe mucle groups as well. The cases reported of a mother and son with both having identical deletion of the cytoplasmic DNA showed very different findings: the mother with PEO of very extensive involvement and the son who died at 1 year of a very rare type of anemia. The authors reach the conclusion that in this case the single gene deletion was transmitted through the germ line. (I am not sure I understand how they came to that conclusion.)

Mackey, DA, et al: Congenital fibrosis of the vertically acting extraocular muscles maps to FEO locus. Homan Genetics, 11510-12, 2002.
Several syndromes of fibrosis (loss of elasticity) of the extraocular muscles have been described. It usually occurs in families and tends to be dominantly inherited. This group in Australia report a family where they were able to trace the gene to s specific site in the genome where other mutations reside to provide the genetic basis for its inheritance. All individuals with this familial syndrome need to be seen by an ophthalmologist for treatment can help there disfiguring strabismus as well as the othe rocular abnormalities that accompany it.

Farr, AK, et al: Ocular manifestations of Ataxia-Telangectasia.
This rare syndrome has widespread effects in the body of affected individuals. Telangectatic (a term to describe an extraordinary collection of vessels on the conjunctiva, hence the name) vessels were present in 91%, also on the skin in 33%, 38% had strabismus. Apraxia (inability to perform a purposeful movement) of the eyes was noted in 30% as well as nystagmus and hypometric (slow) saccades in others. Poor accommodation (focusing) were noted as well. These are treatable.

Chritiansen, SP, et al: Ocular outcomes in low birthweight premature infants with intraventricular hemmorhage. Jnl Pediatric Ophthalmology & Strabismus. 39: 157-65.
Sixty of a total of 490 patients with very low birthweight (less than 1500 grams at birth) were studied in follow up. Intraventiricular hemmorhage (bleeding in the brain during the early weeks of life) was documented in all. In those children with serious bleeds, 73% had strabismus compared to 14% with not serious bleeds. Optic atrophy, other ocular motility defects and retinal abnormalities occurred in significant percentages as well. This study emphasizes the need for continued follow up by ophthalmologists of these children.

Onofrey CB, et al: The Outcome of Retinopathy of Prematurity: Screening for Retinopathy of Prematurity Using an Outcome Predictive Program. Ophthalmology 2001; 108:27.
Retinopathy of prematurity (ROP) is a potentially blinding disease of very premature infants. This study was designed to determine if a risk assessment program (computer-based) could accurately predict which infants went on to threshold disease requiring laser therapy versus those who did not. If the risk assessment program determined that the risk was 0.5 (= 1 chance out of two), then the program performed as well as standard ophthalmological exam. If the risk was set lower than that (1 chance out of 8), then the program predicted far too many babies would reach threshold than actually did. This might cause a significant number of babies to be treated who did not need it.

Subhani M, et al: Screening Guidelines for Retinopathy of Prematurity: The Need for Revision in Extremely Low Birth Weight Infants. Pediatrics 2001; 107:656.
In this article, the authors retrospectively examined the medical records of all premature infants of extremely low birth weight (ELBW = less than 1,000 grams at birth). Standard ophthalmological examinations are performed today on premature infants between 31 and 33 weeks of age. In these 258 extremely low birth weight infants an examination perhaps at 28-30 weeks might uncover the disease at an earlier stage. The importance of this paper rests on the fact that it brings to our attention the possibility of making an earlier diagnosis of ROP in these extremely high-risk infants.

Balcer LJ, et al: Visual Loss in Children with Neurofibromatosis Type 1 and Optic Pathway Gliomas: Relation to Tumor Location by Magnetic Resonance Imaging. American Journal of Ophthalmology 2001; 131:442.
Optic pathway gliomas occur in 15-20% of children afflicted with neurofibromatosis-1. This paper cites evidence that by using refined magnetic resonance imaging (MRI) scanning, the location of the tumor can be related to the risk of the child developing severe visual loss. The use of the MRI provides a new diagnostic method to determine what is the risk of the occurrence of visual loss in these children.

Lotery AJ, et al: Mutations in the CRB1 Gene Cause Leber’s Congenital Amaurosis. Archives of Ophthalmology 2001; 119:415.
Leber’s congenital amaurosis is an inherited eye disease causing blindness or severe visual impairment in children afflicted with the disease. Most probably it occurs because rods and cones, which are the photoreceptors, never develop normally. It has been known for a long time that it is inherited, probably as an autosomal recessive disorder, ie both parents must carry one of the genes. In this paper of a total of 233 children with Leber’s congenital amaurosis (LCA), 21 of 190 affected individuals and 2 of 140 controls were found to harbor mutations in the CRB1 gene. This work adds to an increasing body of knowledge of the molecular diagnosis which can confirm clinical diagnosis of patients with LCA. Though no specific treatment at this time is known as a result of this, it will facilitate counseling of patients and their parents regarding the visual prognosis and provide for the possibility of the development in the future of gene therapy.

Blair M, et al: Decreased Incidence of Retinopathy of Prematurity, 1995-1997. Journal of American Association for Pediatric Ophthalmology & Strabismus 2001; 5:118.
This retrospective study reviewed the records over a two-year period in a neonatal intensive care unit locally in that unit as well as a data base compiled internationally. It reviewed factors in the patient care of these premature infants that may have contributed to a change in the incidence of retinopathy of prematurity. In all, 191 infants from the University of Kentucky NICU and a data base from the Vermont Oxford Network which comprised 9,989 infants were compared. The data would seem to indicate, first of all, that there was a decrease in the incidence from 1995 to 1997 from 57% to 36%. The factors that would seem to account for this were the use of Cortisone-like medications given to the mother before birth, the use of oxygen at 36 weeks postconceptional age, as well as the use of oxygen on discharge of the infants to their home. If this is borne out by further studies, it would seem to indicate that these factors (or others) are operating to reduce the overall number of infants with ROP.

Fulton AB, et al: The Rod Photoreceptors in Retinopathy of Prematurity: An Electroretinographic Study. Archives of Ophthalmology 2001; 119:499.
This study links severity of acute phase retinopathy of prematurity to the function of rod photoreceptors after the disease has resolved. Using special methods to document changes in these rod photoreceptors which are used primarily for night vision, the authors determined in a group of 25 infants and children that these photoreceptors were affected most severely when the ROP was most severe during infancy. Because retinopathy of prematurity is a disease of developing blood vessels, presumably destroying these blood vessels. This in turn results in damage to a greater or lesser extent to these photoreceptors which are essential to night vision.

Kerr NC, et al: Botulinum Toxin for Sixth Nerve Palsies in Children with Brain Tumors. Journal of American Association for Pediatric Ophthalmology & Strabismus 2001; 5:21.
Children with brain tumors often develop an inward deviation of their eye secondary to a paralysis of their external rectus muscle and as a result experience double vision. Botulinum Toxin (Botox) is a non-operative office intervention in which the Botox is delivered by means of a fine needle into the medial muscle opposing the external or lateral paralyzed muscle. In comparing two groups of children managed conservatively (ie no Botox or surgery), versus Botulinum injections to control or treat the weakness of the lateral muscle, the spontaneous recovery rate in the group receiving no treatment was as good as the recovery rate in those who received the Botox injection. The conclusion was that this form of therapy was of no benefit to the children who received it.

Brown RT, et al: Prevalence and Assessment of Attention-Deficit/Hyperactivity Disorder in Primary Care Settings. Pediatrics 2001;107:43.
Attention-deficit/hyperactivity disorder (ADHD) is an apparently common finding in today’s educational setting in America. Epidemiological studies reveal prevalence (number of diagnosed patients in a given population) as between 4% and 12% in children between the ages of 6 and 12 years. This article surveys the current medical tests, rating scales and the like that are employed in evaluating ADHD. For purposes of this paper, the take-home message is that recommendations are made regarding the assessment of children in the pediatric primary care setting.

Oren B, et al: The Prevalence of Uveitis in Juvenile Rheumatoid Arthritis. Journal of American Association for Pediatric Ophthalmology and Strabismus 2001; 5:2-4.
Juvenile rheumatoid arthritis can have as an accompaniment severe uveitis with secondary complications of cataracts, glaucoma and retinal detachment. It is an important cause of visual loss in children with the primary condition, juvenile rheumatoid arthritis. Periodic ophthalmological screenings have been recommended. Recently, a noted decrease in the prevalence of uveitis in children with JRA has been mentioned. In this study, a total of 76 patients (63 girls and 13 boys, ages 1-16 years) were studied following current guidelines. Uveitis was found in 10 children (13%). Only 2 of the children were symptomatic on presentation. The remaining were discovered on screening. The study seems to agree with other current studies in finding a lower rate of uveitis in JRA. The authors attribute this to the use of more and better medications.

Parsa CF, et al: Redefining Papillorenal Syndrome: An Underdiagnosed Cause of Ocular and Renal Morbidity. Ophthalmology 2001; 108:738.
The papillorenal syndrome, also known as renal-coloboma syndrome is an autosomal dominant genetic entity consisting of bilateral optic nerve anomalies associated with severe kidney disease. This study describes 3 families with this syndrome, all of whom had typical changes in the blood vessels entering the eye at the optic disc. This enabled the ophthalmologist to make a very early diagnosis of the condition and refer the patient to pediatric kidney specialist for appropriate care. In so doing, this allows earlier recognition of the kidney disease which, if neglected, is essentially fatal, also avoids unneeded eye surgery and enables the parents to seek genetic counseling regarding other children.

Mohney BG: Common Forms of Childhood Esotropia (Crossed Eyes). Ophthalmology 2001; 108:805.
Strabismus (crossed or wall-eyed) is a common affliction which begins in childhood. This study describes in rural Appalachia the incidence of these defects in the community. In order of frequency, accommodative esotropia was the most common (this is the type of strabismus that can be treated with glasses primarily). The second most common cause was crossed eyes associated with central nervous system defects in this childhood entity.