Refractive error at birth and its relation to gestational age.

PURPOSE: The refractive status of premature infants is not well studied. This study was done to find the norms of refractive error in newborns at different gestational ages. METHODS: One thousand two hundred three (1203) eyes were examined for refractive error by streak retinoscopy within the first week of life between June 2001 and September 2002. Tropicamide eye drops (0.8%) with phenylephrine 0.5% were used to achieve cycloplegia and mydriasis. The refractive error was measured in the vertical and horizontal meridia in both eyes and was recorded to the nearest dioptre (D). The neonates were grouped in five gestational age groups ranging from 24 weeks to 43 weeks. RESULTS: Extremely preterm babies were found to be myopic with a mean MSE (mean spherical equivalent) of -4.86 D. The MSE was found to progressively decrease (become less myopic) with increasing gestation and was +2.4 D at term. Astigmatism of more than 1 D spherical equivalent was seen in 67.8% of the eyes examined. Among newborns with > 1 D of astigmatism, the astigmatism was with-the-rule (vertical meridian having greater refractive power than horizontal) in 85% and against-the-rule in 15%. Anisometropia of more than 1 D spherical equivalent was seen in 31% babies. CONCLUSIONS: Term babies are known to be hypermetropic, and preterm babies with retinopathy of prematurity (ROP) are known to have myopia. This study provides data on the mean spherical equivalent, the degree of astigmatism, and incidence of anisometropia at different gestational ages. This is the largest study in world literature looking at refractive errors at birth against gestational age. It should help understand the norms of refractive errors in preterm babies.

The eyes of young chickens grow toward emmetropia.

The distribution of refractive errors was followed in chicks from hatching to 8 weeks of age. A dramatic progressive decrease in the variability of refractions was observed over this period. In addition, there appeared to be a parallel decline in hyperopia, even when the artifactual hyperopia of retinoscopy was taken into account. These results are evidence for a postnatal development regulatory mechanisms, most likely dependent on vision, which directs growth of the eye toward emmetropia.

Visual acuity development in normal and abnormal preterm human infants.

The grating acuity of preterm infants was determined by measurements of the visual evoked potential (VEP) produced by phase alternation of sinusoidal luminance gratings. The development of visual acuity in healthy preterm infants appears to be accelerated when compared with full term infants of the same post-conceptual age. Cortical insults and the more advanced stages of retinopathy of prematurity may adversely affect acuity development as indexed by the VEP. Preterm twins also appear to have lower acuity than preterm monoparous infants.

Ophthalmologic findings in the Cornelia de Lange syndrome.

Cornelia de Lange Syndrome (CDLS) is characterized by mental retardation, growth retardation, skeletal anomalies, and a characteristic facies. We examined 22 children with CDLS to ascertain the relative frequencies of oculofacial and ophthalmologic abnormalities. We confirm prior reports of the frequent findings of brow hypertrichosis, synophrys, and long arcuate eyelashes. In addition we documented the association with ptosis, nystagmus, and high myopia. Contrary to previous reports, we did not find blue sclera or corneal, pupillary, or retinal disorders to be part of this syndrome. Hypertelorism and telecanthus were found, but they were dependent on the method of calculation. Two children had optic nerve pallor. We also report the new findings of poor macular reflex, chin lifts induced by ptosis, hypertropia, and nasolacrimal duct fistula.

High heritability of posterior corneal tomography, as measured by Scheimpflug imaging, in a twin study.

PURPOSE: Anterior corneal curvature shows significant heritability. Scheimpflug imaging also permits assessment of posterior tomography. We estimated heritability of posterior and anterior tomographic parameters. METHODS: Oculus Pentacam images were obtained in twins from the TwinsUK cohort. Mean anterior and posterior radii of curvature (right cornea) were compared within twin pairs, and heritability calculated (maximum likelihood structural equation modeling, using OpenMx package). Heritability estimates also were calculated for other parameters (anterior and posterior elevation at apex and thinnest point; pachymetry at apex and thinnest point; average pachymetry progression index). RESULTS: Images from 138 twins were included (32 monozygotic [MZ] and 37 dizygotic [DZ] twin pairs). Mean (SD) age was 61 (11) years; 91% were female. Coefficients for intrapair correlation for MZ and DZ twins were, respectively, 0.89 and 0.42 for anterior curvature, and 0.93 and 0.46 for posterior curvature (P ≤ 0.0001 for differences between MZ and DZ correlations; Fisher r-to-z transformation). Heritability estimates (95% confidence interval [CI]) for anterior and posterior curvature were 89% (79%-93%) and 90% (83%-94%), respectively. Estimates for all other parameters were 75% or higher, except anterior apical elevation (61%). Point estimates for posterior parameters were consistently higher than anterior parameters, although CIs overlapped. Age-matching yielded similar estimates. Intereye correlations were high; correlations with age were weak (r < 0.30). CONCLUSIONS: This is the first study to explore heritability of a number of different parameters of corneal tomography, including posterior curvature. Almost all parameters appeared highly heritable, with a trend toward higher heritability estimates for posterior (versus anterior) parameters.

Treatment of congenital esotropia with botulinum toxin type A.

PURPOSE: To examine the effect of botulinum toxin type A injections given transconjunctivally into the medial recti muscles in patients with congenital esotropia. METHODS: Eighty-two patients with congenital esotropia were treated with botulinum toxin type A transconjunctival injections into the medial recti muscles. RESULTS: A Kaplan--Meier curve shows a 24-month success rate of 87% (n = 53) with alignment within 20 prism dioptres (PD) of the 80 patients who were seen at least 1 year following the last injection. Seven of the 80 patients underwent standard monocular surgery with recession of the medial rectus and resection of the lateral rectus muscle. Two patients were lost during follow-up. CONCLUSION: Botulinum toxin type A injections into the medial recti muscles are a valuable alternative to conventional strabismus surgery.

[Non-oculomotor eye involvement in Moebius sequence]. / Nichtokulomotorische Augenbeteiligung bei Möbius-Sequenz.

Moebius sequence is a congenital disorder that not only affects the oculomotor system but also the eyes themselves. Ocular involvement might be sight-threatening and needs regular follow-up by an ophthalmologist.

[Refractive errors in visually handicapped children]. / Refraktionsanomalien bei sehbehinderten Kindern.

Aside from hereditary juvenile macular dystrophy (Stargardt's disease), the author observed a significant increase in the incidence of spheric and astigmatic refractive errors in a further six hereditary or congenital deformities with poor vision. A shift toward myopia was seen in cases of coloboma, both of the optic nerve head as well as of the macular region, and in cases of macular aplasia and hypoplasia. A shift toward hyperopia was found in complete and ocular albinism, in retinitis pigmentosa and congenital achromatopsia. This hyperopic shift is in contradiction to the hypothesis that myopia can be deprivation-induced. It is assumed that a disturbance of visual stimulation early in life can disrupt emmetropization and thus induce the eye to become either myopic or hyperopic, in combination with a high incidence of astigmatism. This disturbance of visual stimulation may be due to clouding of the dioptric media, though equally to congenital or hereditary defects in the retina or the visual pathways of visual cortex.

[Congenital facial hemiatrophy. Report of two pediatric cases]. / Kongenitale Hemiatrophia faciei. Bericht über zwei kindliche Fälle.

In facial hemiatrophy two different types are known: the progressive acquired type, which is relatively more common, and the rare congenital non progressive form. Two children--both with congenital facial hemiatrophy--are presented, both showed a (horizontally and vertically) smaller lid fissure and antimongoloid configuration of the lid. In addition in one case we could observe: small corneal diameter, staphyloma posticum, paving stone degeneration of the retina, convergent strabismus, and vertical deviation, in the other case: ptosis, major astigmatism, and latent divergent strabismus.

[Congenital ptosis: amblyogenic refractive errors, amblyopia, manifest strabismus and stereopsis related to the types of ptosis. Data on 77 patients and review of the literature]. / Kongenitale Ptosis: Refraktionsfehler, Amblyopie und Störungen des Binokularsehens der einzelnen Ptosisformen--Befunde von 77 Patienten und Literaturübersicht.

BACKGROUND: Former reports on amblyogenic refractive errors, amblyopia and binocular vision in congenital ptosis usually comprise all forms of ptosis without any differentiation. This study is an analysis of different kinds of ptosis. PATIENTS AND METHODS: 154 eyes (98 ptotic eyes) of 77 patients with congenital ptosis aged > or = 1 year (56 unilateral ptoses: 45 simple, 1 with rectus superior paresis, 7 with Marcus Gunn's syndrome, 2 congenital oculomotor palsies, 1 unilateral fibrosis syndrome; 21 bilateral ptoses: 10 simple, 2 with bilateral double elevator paresis, 7 blepharophimosis syndromes and 2 bilateral fibrosis syndromes) were investigated concerning visual acuity, refractive error (94 % in cycloplegy), strabismus and stereo acuity. As amblyogenic refractive errors were defined: astigmatism > or = ldpt, anisometropia > or = 1 dpt (spherical equivalent) and hyperopia > or = 3 dpt; amblyopia was defined as visual acuity less than 1.0 or a difference between both eyes of at least 0.2. RESULTS: Altogether were found: Hyperopia > or = 3 dpt in 28.6 % (n = 28); astigmatism > or = 1 dpt in 63.3 % (n = 62), anisometropia in 27.3 % (n = 21), amblyopia in 65.3 % (n = 64), strabismus in 29.9 % (n = 23) and stereopsis in 76.6 % (n = 59). In unilateral simple ptosis, astigmatism of the fellow eye was found in 26.8 % (n = 14). In unilateral ptosis, esotropia was 21.4 % (n = 12), in bilateral ptosis, exotropia 19 % (n = 4), as well as astigmatism > or = 3 dpt 26.2 % (n = 11). As was to be expected, ptosis groups with motility disorders were associated with higher rates of strabismus and amblyopia. The blepharophimosis syndrome could be differentiated by the typical lid anomalies. CONCLUSION: The subgroups of congenital ptosis differ in frequency of amblyogenic factors. In unilateral ptosis these are also frequent in the fellow eye.

Síndrome de Ciancia: resultados quirúrgicos / Ciancia's syndrome: surgical results

El síndrome de Ciancia, representa aproximadamente el 10 por ciento de las esotropías congénitas, se caracteriza por ángulo de desviación generalmente grande, limitación de la abducción y nistagmos en resorte en el intento de la misma. Se realizó un estudio descriptivo prospectivo de 21 pacientes, que asistieron a la Consulta de Oftalmología Pediátrica del Hospital Oftalmológico Docente Pando Ferrer desde enero del 2000 a enero del 2002 con los objetivos describir la edad de aparición, de diagnóstico y de cirugía de los casos estudiados, identificar el defecto refractivo predominante, describir los signos y enfermedades asociadas, determinar los resultados quirúrgicos y la correlación existente entre la edad del alineamiento y los resultados sensoriales obtenidos. En la muestra estudiada la edad de aparición de la desviación ocular fue en el 62 por ciento de los pacientes entre el nacimiento y los 2 meses de edad, en el primer año de vida se diagnosticaron el 66,7 por ciento de los niños los cuales fueron operados entre los 13 y 17 meses de edad. Quedaron alineados quirúrgicamente el 81,0 por ciento de los pacientes. La asociación más frecuente fue con hiperfunción de oblicuos inferiores. Los 14 pacientes alineados entre los 13 y 17 meses de edad alcanzaron fusión y estereopsia

Síndrome de Ciancia: resultados quirúrgicos / Ciancia's syndrome: surgical results

El síndrome de Ciancia, representa aproximadamente el 10 por ciento de las esotropías congénitas, se caracteriza por ángulo de desviación generalmente grande, limitación de la abducción y nistagmos en resorte en el intento de la misma. Se realizó un estudio descriptivo prospectivo de 21 pacientes, que asistieron a la Consulta de Oftalmología Pediátrica del Hospital Oftalmológico Docente "Pando Ferrer" desde enero del 2000 a enero del 2002 con los objetivos describir la edad de aparición, de diagnóstico y de cirugía de los casos estudiados, identificar el defecto refractivo predominante, describir los signos y enfermedades asociadas, determinar los resultados quirúrgicos y la correlación existente entre la edad del alineamiento y los resultados sensoriales obtenidos. En la muestra estudiada la edad de aparición de la desviación ocular fue en el 62 por ciento de los pacientes entre el nacimiento y los 2 meses de edad, en el primer año de vida se diagnosticaron el 66,7 por ciento de los niños los cuales fueron operados entre los 13 y 17 meses de edad. Quedaron alineados quirúrgicamente el 81,0 por ciento de los pacientes. La asociación más frecuente fue con hiperfunción de oblicuos inferiores. Los 14 pacientes alineados entre los 13 y 17 meses de edad alcanzaron fusión y estereopsia(AU)