Familial remitting chorea, nystagmus, and cataracts.

We report on 2 brothers with chorea and monocular horizontal nystagmus beginning in early infancy, both of which remit during the first decade of life, and peripheral cataracts. While this condition shares manifestations with benign hereditary chorea and several other familial movement disorders, the slowly remitting course of the chorea combined with the visual abnormalities appears to be unique.

A syndrome of microcephaly, eye anomalies, short stature, and mental deficiency.

We have studied a mother and son with a previously apparently undescribed syndrome of microcephaly, eye defects, small ears, mild mental deficiency, and short stature. The syndrome appears to be an autosomal or X-linked dominant trait. The cat eye syndrome, blepharophimosis or Kohn-Romano syndrome, Rieger syndrome, and other disorders are discussed in relationship to this entity.

Aphakic cystoid macular edema in children.

Twenty-five eyes of 18 pediatric patients (aged 7 1/2 months to 14 years 8 months) who had had cataract extractions were examined for evidence of cystoid macular edema (CME) with fluorescein angiography. No patient showed CME when studied from five weeks to four years after surgery. One patient with Peter's anomaly, glaucoma, and chronic iridocyclitis had intense fluorescence of the aqueous and vitreous that precluded evaluation of the macula. Even if this patient is assumed to have had CME, its incidence in our population of aphakic pediatric patients was no more than 4%.

Incidence of retinopathy of prematurity in a tertiary newborn intensive care unit.

This study determined the incidence of retinopathy of prematurity (ROP) in 2,958 admissions to the Newborn Intensive Care Unit of the James Whitcomb Riley Hospital for Children, Indianapolis, between January 1976 and December 1979. Among 2,484 survivors, acute ROP developed in 72 (2.9%); 60 (83%) of these newborns had birth weights of less than 1,500 g. The incidence of acute ROP among survivors with birth weights of less than 1,000 g (28%) was approximately three times that of the survivors with birth weights between 1,001 and 1,500 g (10.1%). The overall incidence of blindness was 4.5% of surviving infants less than 1,000 g and 1.2% of those surviving with birth weights of 1,000 to 1,500 g. Evidence of the strong influence of immaturity and low birth weight on the risk of development of ROP is reaffirmed. Increasing survival of the most susceptible infants may be the factor contributing most to the overall incidence of ROP.

Extraocular muscle-tendon transfer with scleral augmentation.

Two patients with 90 prism diopters of exotropia and an absent medial rectus muscle each had the nasal half of the superior and inferior rectus muscles joined with a thin strip of preserved sclera. The center of the scleral strip was first anchored to host sclera at the usual site of the medial rectus insertion. Alignment was improved in the primary position with a residual exodeviation of 25 prism diopters and 20 prism diopters, respectively. Each patient required secondary conjunctival recession because of a delle.

Photo-induced convulsion after using the translid binocular interactor.

A 12-year-old boy with small-angle secondary exotropia and minimal amblyopia experienced photo-induced epilepsy after using the Translid Binocular Interactor, a device designed to treat several of the motor and sensory abnormalities of the eyes alternately with a bright flash at 10 cps. Whether or not the device is effective, it is potentially dangerous to susceptible individuals.

Depth of strabismic amblyopia determined with neutral density filters.

We used a neutral density filter to assess motor responses in the fixation patterns of 25 patients, ranging in age from 5 to 54 years, with strabismic amblyopia. Shifts of fixation back to the preferred eye occurred when the visual acuity of the preferred eye through the filter was marginally better than that of the amblyopic eye. Thus, we were able to correlate the level of the filter at which this shift occurred with the level of amblyopia. This technique may be particularly helpful in the examination of preliterate children and in monitoring the progress in the treatment of amblyopia.

Persistent binocular diplopia after cataract surgery.

We reviewed the records of 38 consecutive patients who had persistent binocular diplopia after cataract surgery. The patients were divided into ten categories based on the suspected conditions underlying the diplopia. Of 16 patients who underwent strabismus surgery, five achieved the therapeutic goal of single binocular vision in the primary and reading position, and four attained this with prismatic or botulinum toxin therapy in addition to surgery. Seven patients continued to have diplopia despite strabismus surgery and adjunctive therapy. Even small residual deviations were often intolerable, because of highly diminished fusional amplitudes.

Linear subcutaneous fat atrophy after corticosteroid injection of periocular hemangiomas.

Two children developed evidence of subcutaneous fat atrophy after corticosteroid injection of periocular hemangiomas. The atrophy appeared at the site of injection and followed the expected course of lymphatic channels to the vicinity of regional lymph nodes.

Unilateral esotropia after enucleation in infancy.

Five patients developed esotropia in the remaining eye after unilateral enucleation in the first few weeks to months of life. Esotropia was associated with a face turn toward the opposite side and abduction nystagmus with a null point in extreme adduction. Our experience with these patients supports the reflexogenic theory for the development of a type of congenital-infantile esotropia. An intact globe-ocular muscle relationship, even in a blind eye, may have a stabilizing effect on the fellow eye in the first few weeks to months of life, and this should be considered before enucleation is done.

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.

Treatment of congenital glaucoma.

We reviewed 37 consecutive patients who had undergone at least one goniotomy, filtering surgery, and patching for amblyopia because of congenital glaucoma between 1969 and 1979. Of 32 eyes treated for uncomplicated congenital glaucoma, 25 (78%) achieved satisfactory control of intraocular pressure. Seven of the 12 eyes (58%) for which Snellen visual acuities could be obtained had visual acuities of 6/15 (20/50) or better. Of eight eyes treated for complicated congenital glaucoma, four (50%) achieved satisfactory control of intraocular pressure, but the visual acuities of these patients could not be tested.

Congenital blepharoptosis, anisometropia, and amblyopia.

A review of the records of 65 patients who had surgery for congenital blepharoptosis of the upper eyelid during a three-year period revealed the preoperative presence of anisometropia, strabismus, or amblyopia in 19 patients. Postoperatively, ten of the 65 patients developed or increased their astigmatism in the operated eye. In four of these patients amblyopia attributed to this postoperative astigmatism developed. The results of our study suggest that blepharoptosis surgery done in the first few years of life may contribute to the development of astigmatism and amblyopia in some patients.

Hibiclens keratitis.

Presumed accidental corneal exposure to Hibiclens (chlorhexidine 4% and detergent) in two patients resulted in severe and permanent corneal opacification. We investigated the corneal toxicity of Hibiclens by gross, biomicroscopic, and histopathologic studies of rabbit eyes exposed to Hibiclens for varying time intervals ranging from five to 15 minutes. Severe, irreversible, and progressive corneal damage resulted in all eyes studied.

Incidence of strabismus in neonates.

In an attempt to learn whether esotropia is present at birth or develops later in infancy, we observed 1,219 alert infants in a normal newborn nursery at a city hospital. Of these, 593 (48.6%) had orthotropic findings; 398 (32.7%) had exotropia, 40 (3.2%) had esotropia (intermittent in 17 with 14 varying between esotropia and exotropia and nine with a variable esotropia), and 188 (15.4%) were not sufficiently alert to permit classification. No infant displayed typical signs of congenital esotropia. We concluded that congenital-infantile esotropia is not connatal but rather develops in the first few weeks or months after birth.

Stereopsis in normal infants and infants with congenital esotropia.

We used electro-oculographic recordings of eye movement responses to a dynamic random-dot stereogram to assess stereopsis in normal infants and in infants with congenital esotropia. Normal infants showed an onset of stereopsis at about 4 months of age, consistent with previous reports. Four of nine infants with congenital esotropia demonstrated stereopsis when tested within two weeks of surgical alignment. No patient with congenital esotropia showed evidence of stereopsis when tested at a postoperative interval of more than two weeks.

Optic chiasmatic glioma in children.

We reviewed the records of 29 patients with optic nerve or chiasm glioma, or both, seen from 1955 to 1966. Sixteen patients were girls and 13 were boys. At the time of diagnosis, 14 patients were less than 2 years old. Optic atrophy was the most frequently seen physical finding, present in 26 of 29 patients. Twelve patients had diencephalic syndrome (41%). Proptosis was seen in eight. Eleven patients (38%) had associated neurofibromatosis. Pneumoencaphalogram was done on 26 patients and was abnormal in 23. The diagnosis was confirmed at surgery in 27 patients. All tumors were astrocytomas. Eighteen patients underwent radiotherapy. Surgery and radiotherapy were used as treatment for optic-chiasmatic glioma with diencephalic syndrome.

Visual function and academic performance.

Evaluation of 1,910 first-, second-, and third-grade students indicated that visual function and academic performance as measured by reading were not positively related. Visual function tests included visual acuity, muscle balance, preferred eye and hand, color vision, refraction, sensory and motor function, and a writing and drawing task. Academic tests included the Metropolitan Readiness Test, the Cognitive Abilities Test, the Iowa Test of Basic Skills, and the teacher's assessment of reading level. A simple test which can be completed during the office visit and interpreted by the ophthalmologist in the office included drawing, copying, and writing. A segment of this test, the "draw a bicycle test," can be used by an ophthalmologist to demonstrate the difference between vision and performance when examining a child up to third-grade level who is referred because of school failure.

Surgical treatment of congenital esotropia.

We conducted a retrospective study of 133 children (69 boys and 64 girls) who underwent bilateral medial rectus muscle recession (most by the augmented or en-bloc technique) for congenital esotropia. Esotropia was diagnosed before the age of 6 months in 84 patients and after the age of 6 months in the other 49. A total of 27 children underwent surgery before the age of 12 months; of these, three required second procedures. A total of 106 children underwent surgery after the age of 12 months; of these, eight required second procedures. The mean preoperative deviation was 40 prism diopters. Two patients had significant A pattern deviations and 17 had significant V pattern deviations. Six patients had dissociated vertical deviations. Five to 60 days after surgery, 52 patients had no deviation and 99 were within +/- 10 prism diopters of no deviation. Two months after surgery, 67 patients had no deviation and 114 were within +/- 10 prism diopters of no deviation. Final alignments (five months to seven years postoperatively) showed that 51 patients had no deviation and 109 were within +/- 10 prism diopters of no deviation. Despite adequate alignment, none of 13 patients whose esotropia was diagnosed before the age of 6 months, who underwent surgery before the age of 12 months, and who cooperated with testing achieved stereopsis. This suggested that there may be two types of congenital esotropia--one without fusion potential and one in which fusion is possible but lost secondarily because of peripheral esotropic factors.

X-linked recessive familial exudative vitreoretinopathy.

Familial exudative vitreoretinopathy is an inherited disorder characterized by retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates. We observed a family in which four boys (the children of three sisters) were affected with this disorder and an X-linked recessive inheritance was apparent. The differential diagnosis includes retinopathy of prematurity, primary hyperplastic primary vitreous, Coats' disease, peripheral uveitis, retinoblastoma, and Norrie's disease, but this differentiation can usually be made on the basis of clinical findings alone. Knowledge of X-linked recessive transmission is important for correct diagnosis and for genetic counseling.