Development of grating acuity in infants with regressed stage 3 retinopathy of prematurity.

The acuity card procedure was used to measure grating acuity in 17 infants with regressed Stage 3 retinopathy of prematurity (ROP) who had no lasting anatomic changes in the retina or optic nerve. Results were compared with those of 28 healthy preterm infants and 28 infants matched by birth weight and gestational age who did not have Stage 3 ROP. Infants in the ROP group showed delayed grating acuity development until 2 years of age. This difference among groups was significant at the 3-5- and 10-12-month test ages but not at the 0-1-, 8-9-, and 16-18-month test ages. Post hoc analyses indicated that the delay in acuity development shown by the ROP group was due to the poor acuity scores of the infants in that group who had central nervous system abnormalities of periventricular leukomalacia or severe (Grade III or IV) intraventricular hemorrhage. When the data of these infants were removed from the analysis, the ROP group showed acuity development similar to that of both the healthy preterm group and the group of infants with matched birth weights and gestational ages who did not have Stage 3 ROP.

Visual field development in infants with stage 3 retinopathy of prematurity.

Binocular visual field development was measured in 11 infants who had stage 3 ROP in early infancy and in 11 infants without ROP, matched for birthweight and gestational age. Kinetic perimetry was used to measure visual fields along the 45 degree, 135 degree, 225 degree and 315 degree half-meridia. Infants were tested at 4, 9, and 18 months from due date. Analyses of variance were used to compare results of the two groups for each age tested. Results at the 4-month test age indicated that both groups had visual fields within the normal range for their age. However, at the 9-month test age the ROP group showed a significantly (P less than 0.05) smaller visual field than the control group. At 18 months, the ROP group still showed smaller visual fields than the control group, but the difference was not significant. The results suggest that dysfunction of the peripheral retina associated with ROP may produce a constriction of the visual field or a delay in visual field development.

Acuity card assessment of visual function in the cryotherapy for retinopathy of prematurity trial.

The primary outcome measure of the effectiveness of cryotherapy in the original design of the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) was the anatomic status of the retina, as documented by fundus photography 3 months and 12 months after infants had received treatment. The authors describe the addition of a measurement of visual function, the Acuity Card procedure, to the CRYO-ROP study. After training, four visual-acuity testers attempted to measure monocular grating acuity in all randomized infants and approximately one fourth of the natural-history infants in the study, tested at 1 year postcryotherapy or 1 year postterm. In 95% of infants on whom testing was attempted, monocular acuity values from each eye were obtained. Interobserver test-retest results on 25 eyes of 13 randomized infants agreed to within one octave or better in all but one of the eyes. The high testability rate and good interobserver agreement suggest that the Acuity Card procedure has been a successful method of assessing visual function in the CRYO-ROP study.

Structural features of extraocular muscles of children with strabismus.

Ninety extraocular muscle (EOM) biopsy specimens from 80 children with strabismus and normal ocular alignment were studied by light microscopy, enzyme histochemistry, and electron microscopy. Light microscopy showed considerable variation in muscle fiber shape and size with sarcomere disruption, sharp increases in endomysial and perimysial collagen, numerous vacuoles and subsarcolemmal inclusions. Electron microscopy showed disruption of myofilaments, nemaline rods, abnormal mitochondria, leptomeric profiles, occasional "myelin figures," glycogen, and lipid-like droplets. Some intramuscular nerves contained long-spacing collagen ("Luse bodies"). Unlike somatic skeletal muscle, EOM showed a consistent lack of mosaic pattern and reciprocal stain activity between fiber types noted by enzyme histochemistry. Quantitative observations indicate that minimal but consistent morphologic differences exist between strabismic and nonstrabismic EOM. The role these changes play in the pathogenesis of strabismus and their significance remains unclear.

Characteristics of infantile esotropia following early bimedial rectus recession.

A homogeneous group of infantile esotropes were studied who underwent surgical alignment before 1 year of age. Three groups emerged: those who remained stable following their initial early alignment, those who were well aligned and remained stable for prolonged periods of time and then decompensated, and those who were unstable throughout the observation period. Preoperatively, no factors were identified that predicted into which group each patient might enter. Other associated factors were identified that affected the strabismic angle and these were overactions of the inferior oblique muscles, dissociative vertical deviation, rotary and other forms of nystagmus, amblyopia, and changing refractive errors. The treatment modalities consisted of miotics, glasses, bifocals, occlusion, and repeated operations. This study underscores the instability of the conditions of patients with infantile esotropia and the need for repeated observations throughout the first decade of life.

HLA antigens in Stevens-Johnson syndrome with ocular involvement.

Fifteen white patients and three black patients with Stevens-Johnson syndrome (SJS) with ocular involvement had HLA typing performed. The HLA-Bw44 antigen was found to have an increased frequency of 66.7% in white patients with SJS with ocular involvement, which was statistically significant when compared with a frequency of 20.4% in the white control population. The results of this study suggest that there is an immunogenetic susceptibility to the development of SJS with ocular manifestations in white patients.

Congenital superior oblique palsy in infants.

A retrospective review of 20 cases of infants with congenital superior oblique palsy showed that all cases had been diagnosed and treated surgically before the patient was 2 years old. Single inferior oblique weakening procedures, single superior oblique tucks, and combined inferior oblique weakening procedures and superior oblique tucks were performed as the initial surgical procedures. Best results were obtained with the two-muscle procedure. The presence of preoperative primary position hypertropia correlated significantly with failure of single inferior oblique weakening to eliminate the signs of superior oblique palsy.

Early retinal vessel development and iris vessel dilatation as factors in retinopathy of prematurity. Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) Cooperative Group.

OBJECTIVE: To determine whether the extent of retinal vessel development present on early screening examinations for retinopathy of prematurity has prognostic value? DESIGN: The prospectively collected data from the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity were used to compare the development of acute retinopathy of prematurity and long-term structural and visual outcomes for eyes with differing extents of retinal vessel development. PATIENT: Study patients had eyes with the following vessel development. In zone I eyes, vessels extended from the disc less than twice the distance from the disc to the macula. In zone II eyes, vessels extended beyond zone I but not to the nasal ora serrata. Transitional eyes had vessels partly in zone I and partly in zone II. RESULTS: The chance of developing threshold retinopathy of prematurity was inversely related to the early degree of vessel development: 54% for zone I eyes, 25% for transitional eyes, and 8% for zone II eyes. The presence of prominent iris vessels at 34 to 35 weeks of postmenstrual age was associated with increased risk for all three groups; zone I eyes almost always needed treatment (94%). The chance of having an unfavorable anatomic alteration of the posterior fundus, or poor vision at the ages of 1 year and 3 1/2 years, was also inversely related to the degree of early vessel development. Vessel development was an independently important factor even when birth weight, gestational age, and race were considered. CONCLUSIONS: The degree of early retinal vessel development is a significant predictor of outcome from retinopathy of prematurity. Iris vessel dilatation is an important indication for greater vigilance in following these infants.

Congenital horizontal tarsal kink.

A 3-week-old male infant had an extreme form of entropion caused by a congenital horizontal tarsal kink, which led to corneal ulceration and visual impairment. The combination of absence of the upper eyelid fold, irritability, and blepharospasm in the absence of congenital blepharoptosis is a sign that may facilitate early recognition of this problem.

Supranuclear oculomotor palsy and exotropia after tetanus.

An 11-year-old boy developed generalized tetanus complicated by a supranuclear gaze palsy and exotropia. This case led us to include supranuclear palsy in an expanded definition of cephalic-tetanus.

Episcleral osteocartilaginous choristoma.

PURPOSE/METHOD: A 31-year-old woman had a hypertropia and an episcleral choristoma in the superotemporal quadrant of the globe. The choristoma was examined. RESULTS/CONCLUSIONS: Gross and histopathologic examination showed bone and cartilage components in the tumor, which refutes the previously accepted membranous growth pattern of episcleral osseous choristomas.

Coats' disease in a patient with Cornelia de Lange syndrome.

A 16-month-old boy had the mental and physical retardation, low-pitched cry, phocomelia with syndactyly, hirsutism, low-set ears, bushy eyebrows, elongated eyelashes, blepharoptosis, and strabismus characteristic of Cornelia de Lange syndrome along with ophthalmoscopic findings characteristic of Coats' disease. Cryotherapy made the abnormal telangiectatic vessels less prominent, but macular scarring produced poor visual acuity. Occlusion therapy was unsuccessful.

External carotid-cavernous fistula in an infant.

A 7-week-old infant developed a nontraumatic fistula of the external carotid artery and the cavernous sinus. Therapeutic embolization produced an uncomplicated clinical and angiographic cure. Treatment was prompted by the availability of new methods of treatment and our hope of preventing irreversible anatomic and visual sequelae.

Serum and vitreous Toxocara antibody in nematode endophthalmitis.

Five patients had a clinical diagnosis of nematode endophthalmitis and had both a serum and vitreous titer for Toxocara antibody. All five patients had a vitreous titer that was equal to or greater than the serum titer. Serum and vitreous obtained from ten other patients who had no evidence of endophthalmitis did not show this correlation. This study provides the clinician with a further understanding of the meaning of a positive enzyme-linked immunosorbent assay titer as well as an additional area for analysis in selected patients with ocular inflammation.

Keratoglobus and blue sclera.

Five patients from two families had similar features including keratoglobus, blue scleras, hyperextensibility of the hand, wrist, and ankle joints, sensorineural conduction hearing alterations, and mottling of the teeth. Keratoglobus had been observed in all patients at, or shortly after, birth. Corneal perforations developed in seven of the ten eyes after minimal trauma. Repair of these perforations was complicated by the extremely thin corneas and six eyes had to be either enucleated or eviscerated. Histopathological examination of two of the enucleated eyes showed the corneal stromas of both eyes to be estremely thin, Bowman's membrane was absent, and Descemet's membrane was unusually thick. This condition has an autosomal-recessive inheritance pattern.

Congenital lacrimal sac mucoceles.

Seven infants had tense, blue-gray swellings inferior to the medial canthal tendon with otherwise normal-appearing eyelids and puncta. All lacrimal sacs transilluminated and A-scan ultrasonography performed in one case demonstrated a nonloculated cystic cavity. Four (57%) infants had uncomplicated mucoceles. One was treated with massage, and has remained asymptomatic for 14 months. The other three were cured with a single probing and irrigation of the entire lacrimal system. Three (43%) infants had developed erythema of the tissues overlying the swollen lacrimal sacs by the time of referral. Because none of the mucoceles could be decompressed by massage, prompt probing and irrigations were performed in each case. Cultures from the aspirates of all three sacs yielded Staphylococcus organisms. In contrast to the uncomplicated cases, two patients developed recurrences that necessitated additional treatment.

A histopathologic study of bilateral aphakia with a unilateral intraocular lens in a child.

A 7-year-old boy who had had electrically induced cataracts since he was 6 months old underwent a lens extraction by phacoemulsification in his right eye. He later refused to wear a contact lens. When the boy was 10 year old, the lens of his left eye was removed by phacoemulsification and an intraocular lens was implanted. The intraocular lens functioned well and was tolerated by the boy until he died in an automobile accident at the age of 16 years. A histopathologic study of the eyes, which were obtained post mortem, showed a decreased corneal endothelial cell population, mild focal atrophy of iris structures, and mild lymphocytic infiltration in the implanted eye. Vitreous liquefaction and epiretinal membrane formation occurred in both eyes.

Third cranial nerve palsy in children.

PURPOSE: To report the causes and the sensory, motor, and cosmetic results after treatment for oculomotor (third cranial nerve) palsy in children. METHODS: Review of the clinical records of children with a diagnosis of third cranial nerve palsy followed up in a university-based pediatric ophthalmology practice between 1981 and 1996. RESULTS: Forty-nine children with 53 affected eyes were followed up for a mean of 5.5 years. Third cranial nerve palsy was partial in 31 children (32 eyes) and complete in 18 children (21 eyes). The palsy was congenital in 20 eyes and caused by postnatal trauma in 17 eyes. Seventeen eyes had aberrant regeneration and four eyes with partial third cranial nerve palsy had spontaneous resolution. Thirty-six children (38 eyes) were affected before visual maturation (age 8 years), and 25 (27 eyes) had amblyopia. Of the five amblyopic eyes with quantifiable visual acuity, none had measurable improvement of Snellen visual acuity during the follow-up period. Overall, visual acuity was between 6/5 and 6/12 at the last follow-up visit in 31 eyes (58%). Ocular alignment was greatly improved after strabismus procedures, with a mean of 1.5 procedures for patients with partial third cranial nerve palsy and 2.3 procedures for those with complete palsy. Binocular function was difficult to preserve or restore but was achieved for some patients with partial third cranial nerve palsy. CONCLUSIONS: Surgical treatment of third cranial nerve palsy is frequently necessary, especially in cases of complete palsy. Multiple strabismus procedures are often needed to maintain good ocular alignment. Surgery can result in cosmetically acceptable alignment of the eyes, but it rarely results in restoration or achievement of measurable binocular function. Treatment of amblyopia is effective in maintaining the level of visual acuity present at the onset of the third cranial nerve palsy, but improvement in visual acuity is difficult to achieve.

Secondary intraocular lens implantation after cataract surgery in children.

PURPOSE: To report results of secondary intraocular lens implantation after cataract surgery in children. METHODS: We reviewed clinical records for a 5-year period of patients who had cataract surgery in childhood and received a secondary intraocular lens implant. We studied indications for secondary intraocular lens placement; surgical procedures for intraocular lens implantation; preoperative and postoperative visual acuity, refractive error, and binocular status; and complications of the procedure. RESULTS: A secondary intraocular lens was placed in 28 eyes of 25 patients who had cataract surgery in childhood. In 20 eyes, the lenses were placed in the ciliary sulcus. The other eight eyes had insufficient capsular support for an intraocular lens; in two, the intraocular lens was placed in the anterior chamber and, in six, in the posterior chamber with suture fixation to the sclera. Twenty of 28 eyes (71%) had measurable improvement in visual acuity; only one eye had a decrease in visual acuity of 2 lines. Fifteen patients (54%) had a final refraction within 1.50 diopters of the fellow eye; 21 (75%) were within 3.00 diopters. During follow-up, two eyes developed glaucoma. One had transient pressure elevation; one required two filtration procedures. Three patients required Nd:YAG capsulotomy. Six patients demonstrated Worth fusion at distance and near; three demonstrated 200 seconds of arc or better stereo visual acuity. CONCLUSION: Secondary placement of an intraocular lens in the posterior chamber appears to be a safe, effective alternative for correction of aphakia in the contact lens- or spectacles-intolerant child or young adult.

Absence of antibody production in patients treated with botulinum A toxin.

To test the possibility of the formation of an antibody to botulinum A toxin after multiple injections of this potent neurotoxin, we collected serum samples from 28 patients who received 57 doses. These injections over a nine-month period with as much as 50 units per injection formed no detectable antibody.