Posterior polymorphous dystrophy associated with astigmatism and amblyopia in children.

BACKGROUND: Posterior polymorphous dystrophy has been described in the scientific literature as having more than a casual association with keratoconus in adults. We studied four consecutive children with posterior polymorphous dystrophy who had coexisting astigmatism. In our patients, the posterior changes appeared to be associated with the corneal astigmatism, as evidenced by videokeratography. METHODS: Four consecutive cases of posterior polymorphous dystrophy in children were found over a 3-year period by one of the authors (PD). They presented with varying degrees of amblyopia in the eyes with the greater amount of posterior polymorphous dystrophy. Cycloplegic refractions and keratometry readings were performed. The more affected eyes had greater degrees of astigmatism, presumably causing the amblyopia. Whenever possible, the results were supplemented by confirmation by a corneal specialist, specular microscopy, slit-lamp photographs and videokeratography. RESULTS: Greater asymmetry of posterior polymorphous dystrophy was associated with greater astigmatism in the more affected eye. When more astigmatic anisometropia was present there was a greater amblyopia. Videokeratography of one of the patients showed that an area of confluent blebs forming a crescent shape compared exactly with a steep cylinder along the same axis. With-the-rule astigmatism was associated with a more diffuse pattern of blebs. CONCLUSION: Decreases in visual acuity in children with posterior polymorphous dystrophy may sometimes be due to an amblyopia caused by the astigmatism of asymmetric disease.

Duane's retraction syndrome.

Duane's retraction syndrome (DRS) has been a recognized clinical entity for nearly a century. It is a clinically well described ocular disorder consisting of retraction of the globe with narrowing of the lid fissure in attempted adduction, frequent abduction deficiency with variable limitation to adduction, and upshoot and/or downshoot of the affected eye on adduction. Among strabismus patients the incidence of DRS is probably not more than 5%. Most cases are sporadic, but familial cases have been estimated at 10% by most authors. Numerous theories concerning the etiology and pathogenesis of DRS have been proposed, including agenesis of the abducens nucleus, but the majority of investigators concur that the characteristic findings are best explained by a paradoxical innervation of the lateral rectus muscle, which subsequently causes a cocontraction of the horizontal rectus muscles. The frequent association of DRS with other congenital anomalies suggests a teratogenic event occurring between the fourth to eighth week of gestation as an etiological factor. In this review historical aspects and theories of the syndrome are studied and statistical data are compiled and analyzed. Clinical features, differential diagnoses and variants of the syndrome are examined. Testing and treatment objectives are discussed.

The sit-up test: an alternate clinical test for evaluating pediatric torticollis.

The sit-up test, a new evaluation method for differentiating between ocular and orthopaedic torticollis, was evaluated against the monocular occlusion test, using results of three-step testing for standardization. The study group consisted of 31 patients with torticollis between the ages of 4 and 12.5 years. Subjects were selected based only on their ability to cooperate with three-step testing. Three-step testing identified 27 of the 31 patients as having ocular torticollis, with the remaining 4 having an orthopaedic etiology. Sit-up testing correctly identified all 27 ocular torticollis patients, with no false positives or false negatives. Monocular occlusion testing detected at best 22 (81.4%) of the ocular torticollis patients, with no false positives.

Calibration of Hirschberg test photographs under clinical conditions.

The feasibility of determining the angle of ocular deviation from clinical photographs was assessed. Corneal reflex photographs were obtained from 30 strabismic patients and 5 orthophoric control subjects, using a centered electronic flash, with a millimeter ruler placed across the forehead for calibration. Measurements of corneal reflex displacement were obtained from the photographs and compared with standard prism-cover results. The value of 20.89 prism diopters (PD) per millimeter of corneal reflex displacement was obtained for the factor of conversion from reflex displacement to strabismic angle. This value of the Hirschberg coefficient is valid only for measurements of reflex displacement along a true frontal plane, as provided in a photograph. Detailed algorithms for the calculation of strabismic angle from photographic data are provided in an appendix.

A survey of severe eye injuries in children.

We reviewed the medical records of 258 children from newborn to 20 years of age who had ocular injuries severe enough to warrant admission to United Hospitals Medical Center, Newark, NJ, over a 3 1/2-year period. In this time there were 1737 pediatric admissions for eye treatment and of these, 258 (14.85%) were directly related to trauma. Only strabismus, with 676 (38.9%) admissions, accounted for more pediatric hospitalizations for eye injuries. Balls, fists, and sticks were the most common causes of injury, and the most common diagnosis was hyphema. The majority of injuries occurred in children between 11 and 15 years of age (31%). Ocular injuries in children above 10 years of age were most commonly sports-related, whereas injuries in the home accounted for the majority of trauma in children younger than 10 years of age.