Surgical outcome after prism adaptation for esotropia with a distance-near disparity.

INTRODUCTION: Prism adaptation for patients with esotropia and a distance-near disparity is controversial. The purpose of this study was to evaluate the surgical outcome for patients who underwent prism adaptation for esotropia with a distance-near disparity and determine whether both preoperative sensory and motor fusion are necessary to determine surgical success. METHODS: The medical records of 65 prism-adapted patients with a distance-near disparity of 9 PD or more were reviewed. Prism responders had a fusion response to near Worth 4-dot test and a deviation with prisms of 8 PD or less of esotropia at near, and 5 PD or less of exotropia at distance, or both. The same criteria were used postoperatively to assess a successful surgical outcome. Patients were operated for the near angle or greater than the near angle. RESULTS: Fifty-eight of 65 patients (89%) demonstrated fusion with prisms. Twenty of 65 patients (31%) had increased deviations that were greater than their original near angle (prism builders). Eleven percent (7 of 65) had no fusion. Postoperatively, 88% (51 of 58) of all fusers, 95% (18 of 19) of prism builders, and 71% (5 of 7) of nonfusers had a good surgical outcome of 8 PD or less of esotropia at near, 5 PD or less of exotropia at distance, or both. CONCLUSIONS: Prism response for distance-near disparity esotropia is a good indicator of postoperative outcome. Responders to prism adaptation had a better surgical outcome compared with nonresponders. In particular, prism adaptation aids in detecting those patients who will benefit from larger amounts of surgical correction.

Clinical characterization and linkage analysis of a family with congenital X-linked nystagmus and deuteranomaly.

OBJECTIVES: To identify a congenital nystagmus locus on the X chromosome and to characterize the phenotype of a 4-generation family affected with congenital nystagmus and color deficiency. METHODS: Sixty-five patients underwent an eye examination, including evaluation for the presence of nystagmus and color vision abnormalities. Affected patients and obligate carriers of the congenital nystagmus mutation were genotyped with short tandem repeat polymorphisms located on the X chromosome, and these data were subjected to linkage analysis. RESULTS: Fourteen patients were affected with a horizontal, conjugate, congenital nystagmus. All examined patients had a visual acuity of 20/60 or better. There were no associated ocular or systemic findings except that 18 of the family members had deficient red-green color vision, which was classified as deuteranomaly (the most common form of anomalous trichromacy). Five patients exhibited nystagmus and deuteranomaly. Significant linkage was demonstrated between the nystagmus phenotype and 11 markers from Xq. The maximum lod score was 4.84 (theta = 0) and was obtained with marker DXS8041. Analysis of recombinants defined the disease interval to lie between markers ATA59C05 and DXS1192 (a 5.4-centimorgan region). The proximity of this locus to the red-green opsin gene cluster (11 centimorgans more telomeric) explains the frequent coexistence of nystagmus and color vision deficiency in this family. CONCLUSIONS: We have identified the genetic locus of the X-linked congenital nystagmus gene in this family. The critical interval in this report is less than half the size of the previously described nystagmus locus. These findings will aid in identifying the gene responsible for this condition.

The psychosocial effects of amblyopia study.

PURPOSE: The purpose of this study was to assess the psychosocial effects of growing up with and living with amblyopia and to determine whether patients with amblyopia but without strabismus encounter psychosocial problems similar to those encountered by individuals with strabismus. METHODS: A 20-question survey focusing on medical background, education, self-image, history of amblyopia, treatment of amblyopia, and effects of amblyopia on work, school, friendships, and self-esteem was provided to patients with amblyopia but not strabismus at Saint Louis University Eye Institute and at the University of Iowa Department of Ophthalmology. In addition, patients were evaluated in terms of somatization, obsession-compulsion, interpersonal sensitivity, depression, and anxiety. Results for patients with amblyopia were compared with those of strabismic, normative, and psychopathologic groups using the Hopkins Symptom Checklist (HSC). RESULTS: Twenty-five patients with amblyopia but without strabismus or previous surgery responded. A significant number of patients felt that amblyopia interfered with school (52%) and work (48%) to some degree and were generally affected in their lifestyle (50%). Fewer were affected in their play of sports (40%) or were influenced as to their job choice (36%). Patients with amblyopia as a whole had a greater degree of somatization, obsessive-compulsive behavior, interpersonal sensitivity, depression, and anxiety than patients with strabismus and HSC control subjects. Differences between patients with amblyopia and those with strabismus were not statistically significant (P > .05), but differences between patients with amblyopia and HSC control subjects were significant (P < .05) in each category. Patients with amblyopia, however, were less symptomatic in these areas than HSC Anxious and HSC Depressed groups. CONCLUSION: Psychosocial difficulties related to amblyopia affect individuals' self-image, work, school, and friendships. Amblyopia has a significant effect on psychosocial functioning and warrants aggressive screening, prevention and treatment during the amblyogenic years.

The effect of adjustable suture techniques on scleral resistance.

BACKGROUND AND OBJECTIVE: The effect of adjustable suture strabismus surgery techniques on the ease of the postoperative adjustment has not been determined. An in vitro animal model was developed to compare several intraoperative variables with the ability to adjust the sutures. MATERIALS AND METHODS: The amount of force required to advance and recess the suture was measured using different sutures, suture coatings, needles, and scleral tunnel techniques. RESULTS: Scleral tunnels placed at an angle to the muscle insertion caused significantly greater scleral resistance as compared with tunnels placed perpendicular to the insertion. Although differences were noted in the scleral resistance with different needles, suture coatings, and suture materials, there was a considerable overlap of the effect. CONCLUSIONS: Scleral tunnels placed perpendicular to the muscle insertion and parallel to the plane of action of the muscle offer the best technique for minimizing scleral resistance. Other less important factors include shorter scleral tunnels, larger needles, and special suture coatings.

Chorioretinal colobomas in a pediatric population.

OBJECTIVE: The purpose of the study was to determine the prevalence of retinal detachment and associated anomalies in pediatric patients with chorioretinal colobomas. DESIGN: Observational case series. PARTICIPANTS: Forty-eight patients (86 eyes) of patients with chorioretinal colobomas followed in two academic pediatric eye clinics were reviewed. MAIN OUTCOME MEASURES: The frequency of associated systemic abnormalities and the prevalence of retinal or choroidal detachment during the follow-up period. RESULTS: Forty-eight patients (86 eyes) were identified; ocular involvement ranged from small colobomas with isolated chorioretinal involvement to large colobomas affecting the iris, choroid, retina, and optic nerve. The mean ages at initial and most recent examinations were 27 and 100 months, respectively. Follow-up averaged 6 years, 1 month. Six retinal detachments were found in four patients. Patient ages at the time of detection of retinal detachment were 5 months, 29 months, 10 years, and 15 years. A choroidal detachment was found in a child at age 9 years. The combined prevalence of retinal or choroidal detachment was 10.4% of patients and 8.1% of affected eyes. Thirteen eyes were microphthalmic, and six had microcornea. Eighteen patients (38%) had other systemic abnormalities. CONCLUSIONS: The 8.1% prevalence of retinal or choroidal detachment among eyes in our series was much smaller than previously cited estimates of 23% to 43%. The higher figures probably reflect a referral bias toward patients with vitreoretinal problems and an older patient population. Because of the acknowledged difficulty of managing retinal detachments in patients with chorioretinal colobomas, emphasis should be directed toward early detection and possible prophylactic therapy. The high proportion of patients who have associated systemic abnormalities indicates the need for thorough evaluation of these children by other pediatric specialists.

Congenital lymphocytic choriomeningitis virus syndrome: a disease that mimics congenital toxoplasmosis or Cytomegalovirus infection.

OBJECTIVE: To describe the clinical characteristics of intrauterine infection with lymphocytic choriomeningitis (LCM) virus, an uncommonly recognized cause of congenital viral infection. PATIENTS: Three infants born in the midwestern United States in 1994 and 1995 with clinical features and serologic studies consistent with congenital LCM virus infection and cases of congenital infection identified by review of the medical literature between 1955 and 1996. RESULTS: Twenty-six infants with serologically confirmed congenital LCM virus infection were identified. Twenty-two infants were products of term gestations, and birth weights ranged from 2384 to 4400 g (median, 3520 g). Ocular abnormalities, macrocephaly, or microcephaly were the most commonly identified neonatal features. Twenty-one infants (88%) had chorioretinopathy, 10 (43%) had macrocephaly (head circumference >90th percentile) at birth, and 3 (13%) were microcephalic (head circumference <10th percentile). Macrocephaly and hydrocephalus developed postnatally in one of the latter infants. Hydrocephalus or intracranial calcifications were documented in five infants by computed tomography or magnetic resonance imaging. Nine infants (35%) died, and 10 (63%) of the 16 reported survivors had severe neurologic sequelae, consisting of spastic quadriparesis, seizures, visual loss, or mental retardation. One-half of the mothers reported illnesses compatible with LCM virus infection, and 25% reported exposures to rodents during their pregnancies. CONCLUSIONS: These cases suggest that congenital LCM virus infection could be an underrecognized cause of congenital infection among infants born in the United States. Because of the clinical similarities of these congenital infections, cases of congenital LCM virus infection can be confused with infections with cytomegalovirus or Toxoplasma gondii.

Glaucoma and ocular hypertension in pediatric patients with cataracts.

BACKGROUND: The pathogenesis of open-angle glaucoma and ocular hypertension in patients who have undergone surgical correction of their congenital cataracts remains undetermined. This study examines the prevalence of glaucoma and ocular hypertension in a population of patients who did not undergo surgical correction of their pediatric cataracts. METHODS: Fifty-eight eyes of 41 patients had cataracts before 2.5 years of age and were followed up until at least 5 years of age without operative correction. The patients were studied for the following parameters: age at diagnosis, type of cataract, etiology, bilaterality, optic nerve head cup-to-disc ratio, intraocular pressures, and reason why the patient did not undergo an operation. Glaucoma was defined as the presence of glaucomatous optic nerve head cupping with intraocular pressures of greater than 22 mm Hg. Ocular hypertension was defined as intraocular pressures greater than 22 mm Hg with no optic nerve changes. RESULTS: Nine of the 58 eyes had cataracts caused by persistent hyperplastic primary vitreous. The average age to the last intraocular pressure measurement was 19 years (range 5 to 48 years). Closed-angle glaucoma developed in two patients with persistent hyperplastic primary vitreous. Neither open-angle glaucoma nor ocular hypertension developed in any patients. CONCLUSION: Pediatric cataracts not of the persistent hyperplastic primary vitreous type were not associated with ocular hypertension or glaucoma in the absence of surgical cataract correction. In eyes with persistent hyperplastic primary vitreous cataracts, spontaneous closed-angle glaucoma developed in two of nine patients and open-angle glaucoma developed in none. Surgical cataract correction, or the aphakic state that follows such operations, may be responsible for pediatric aphakic glaucoma.

Baerveldt implant surgery in the treatment of advanced childhood glaucoma.

BACKGROUND: The efficacy of Baerveldt implant (Pharmacia & Upjohn, Inc., Kalamazoo, Mich.) surgery in the treatment of advanced childhood glaucoma is unknown. METHODS: We reviewed the results of 23 consecutive 350 mm Baerveldt implants in 20 eyes of 17 children. Results were classified as follows: (1) success; no further reoperation, no decrease in vision, and intraocular pressure at last follow-up less than 21 mm Hg with no medications; (2) qualified success; medication necessary to bring intraocular pressure to less than 21 mm Hg or complication not associated with tube failure; and (3) failure; intraocular pressure >20 mm Hg, tube failure complication or reoperation causing tube removal, phthisis, or enucleation. RESULTS: Original glaucoma types were bilateral aphakic (five), unilateral aphakic or persistent hyperplastic primary vitreous (four), primary infantile (four), juvenile (three), secondary(two), Peter syndrome (one), and Lowe syndrome (one). Patients had undergone a mean of 2.8 previous intraocular procedures. Mean preoperative intraocular pressure was 33.6 mm Hg; average number of preoperative glaucoma medications was 3.0. Mean follow-up was 19 months (range, 6 to 32 months). Eight procedures were considered successful (mean intraocular pressure 15.5 mm Hg), six were qualified successes (mean number of medications 0.8; mean intraocular pressure 16 mm Hg), and nine failed. Two eyes in the qualified success group do not have useful vision as a result of complications. Complications included retinal detachment (five), corneal decompensation (five), corneal graft rejection in five of six grafts; dislocated tubes (three), and recurrent uveitis (two). One of these eyes is phthisic and one has been enucleated. Only two of nine procedures in eyes with a history of one or no previous intraocular operations failed,whereas seven of 13 procedures in eyes with a history of three or more previous procedures failed. Only seven of 13 procedures in aphakic eyes were successes or qualified successes, whereas seven of 10 procedures in phakic eyes had good results. CONCLUSION: Baerveldt implants can produce good short-term results, especially in phakic eyes. Aphakic eyes and eyes that have undergone multiple procedures are at a much higher risk for devastating complications.

Prevalence of glaucoma after surgery for PHPV and infantile cataracts.

The prevalence of glaucoma associated with pediatric aphakia has been reported to range from 0% to 27%. Few studies have included a significant number of patients with persistent hyperplastic primary vitreous (PHPV) cataracts. The purpose of this study was to evaluate the prevalence of glaucoma in young children undergoing surgery for PHPV and infantile cataracts. The charts of all of the patients who underwent lensectomy/vitrectomy for infantile and PHPV cataracts at the University of Iowa Hospitals and Clinics between 1975 and 1992 were reviewed. Patients with ocular abnormalities that might predispose to glaucoma and patients with less than 6 months follow up were excluded. A total of 72 patients (109 eyes) were identified: 25 patients (26 eyes) with PHPV cataracts and 47 patients (83 eyes) with infantile cataracts. Mean age at time of surgery for both groups was 3.5 months. Mean follow up was 84.5 months for those with PHPV, and 76.9 months for those with infantile cataracts. Glaucoma developed in eight (32%) of the 25 patients with PHPV and in 15 (32%) of the 47 with infantile cataracts. Mean time to onset of glaucoma was 64.6 months for those with PHPV and 47.5 months for those with infantile cataracts (P = .70). Although there was no significant difference between the prevalence of postoperative glaucoma in those with infantile and those with PHPV cataracts, the prevalence of glaucoma in these patients is high. Children with aphakia should be closely monitored for glaucoma throughout their lives.

Effectiveness of inferior transposition of the horizontal rectus muscles for acquired inferior rectus paresis.

Full inferior transposition of the horizontal recti (inverse Knapp procedure) was performed on four patients with unilateral inferior rectus muscle weakness. The patients were followed up over time to determine the medium- and long-term effectiveness of the procedure. All of the patients had single binocular vision without the use of prisms in the primary position of gaze and three in the reading position after a mean postoperative follow-up interval of 21/2 years. The mean primary position preoperative vertical deviation was 20.3 delta and the mean primary position postoperative vertical correction was 19.5 (range, 16 to 24) delta. The field of binocular single vision was assessed quantitatively and scored 2.4% preoperatively compared with 52.3% postoperatively. There was no decrease in the effect of the procedure over time. Additional surgery may still be required to correct symptomatic residual vertical down-gaze diplopia.

Upper age limit for the development of amblyopia.

The medical records of 64 patients between 1 and 11 years old with known onset of an amblyopia-inducing condition were retrospectively reviewed to determine the upper age limit for the development of amblyopia. Thirty seven patients (group 1) developed amblyopia and 27 patients (group 2) did not. The mean patient age at the onset of the amblyopia-inducing condition was 41.0 months (2.90 SE) for group 1 and 92.3 months (4.02 SE) for group 2. The difference was statistically significant (P = .0001). No patient developed amblyopia after 73 months of age. The duration of the amblyopia-inducing condition and the type of amblyopia-inducing condition were not significantly different between the two groups. The age of the patient when exposed to an amblyopia-inducing condition is the most important determinant for the development of amblyopia. Patients 6 years or older with a normal visual system have a low probability of developing amblyopia following the onset of an amblyopia-inducing condition.

Factors affecting visual outcome after surgery for bilateral congenital cataracts.

The medical records of 33 patients with dense bilateral congenital cataracts were analyzed for factors affecting visual outcome. The average postoperative follow-up was 6.3 years. Of the 23 patients tested with optotypes (verbal children), 14 had visual acuities of 20/80 or better. Eight patients had binocular vision. Preoperative nystagmus, age at surgery, microphthalmos, and postoperative strabismus were not prognostically significant in visual outcome. Postoperative nystagmus was common and was not indicative of a poor visual outcome. Ocular anomalies, systemic anomalies, and postoperative complications were associated with a lower percentage of good postoperative visual acuities.

Superior oblique tuck surgery in the management of superior oblique palsies.

Three hundred and twenty-four patients with superior oblique muscle palsies required surgery over a 15-year period. Forty-four patients underwent superior oblique tuck surgery. Fifteen patients had unilateral and six bilateral tucks alone. Seventeen had unilateral tucks and three bilateral tucks in conjunction with other extraocular muscle surgery. Three had unilateral tucks with a contralateral Harada-Ito procedure. The best results were obtained with isolated superior oblique tucks and tucks in conjunction with a contralateral inferior rectus muscle recession or an ipsilateral inferior oblique muscle weakening procedure. The mean vertical correction following isolated, unilateral surgery was 3.6 prism diopters (range, 0 to 11 delta) in primary gaze and 15.3 delta (range, 0 to 40 delta) in the field of maximum deviation. The mean eso correction in downgaze with bilateral superior oblique tucks was 15.2 delta (range, 10 to 21 delta). There was no statistically significant correlation between the size of the tuck and the amount of deviation corrected. Some degree of postoperative Brown syndrome was seen in all patients, but became less marked with time and in no patient was it severe enough to require reversal of the tuck. In only four patients was there a significant lessening of the effect of the procedure with time. The results show that the superior oblique tuck procedure is an effective operation. In patients with unilateral muscle palsies, 64.3% with an abnormal head posture, 37.5% with diplopia, and 100% with both an abnormal head posture and diplopia achieved an excellent result. In patients with bilateral muscle palsies, 50% with an abnormal head posture and 66% with both an abnormal head posture and diplopia achieved an excellent result.

Long-term results of adjustable suture surgery for strabismus secondary to thyroid ophthalmopathy.

PURPOSE: To review the results of adjustable suture surgery in patients with strabismus secondary to thyroid ophthalmopathy to determine long-term outcome and to identify postoperative complications. METHODS: The records of 1524 patients with thyroid ophthalmopathy were retrospectively reviewed to identify those who required treatment for strabismus in the ocular motility clinic. Treatment consisted of adjustable suture surgery, prisms, or both. Elimination of diplopia in primary and reading positions was used at the criterion for success. RESULTS: Forty-seven patients were treated with adjustable suture surgery, with an average follow-up of 41 months. Results after 1 or more surgeries were: 47% excellent, 26% good, 19% fair, and 9% poor. Significant postoperative complications included eyelid retraction and A-pattern exodeviation. Sixteen of 18 patients with fair or poor outcomes after the initial surgery were recognized within 6 months. Postoperative changes in vertical deviation from primary position to downgaze were predictive of postoperative diplopia in downgaze. Analysis of multiple preoperative characteristics showed no statistically significant associations with outcome. Eight additional patients had adequate relief of diplopia using prisms alone, with an average follow-up of 49 months. CONCLUSIONS: Long-term symptomatic relief of diplopia was obtained in the majority of patients using adjustable suture strabismus surgery, combined occasionally with small amounts of prism postoperatively. Prisms alone provided effective long-term relief in patients with small-to-moderate deviations.

Factors associated with high postoperative myopia after penetrating keratoplasties in infants.

Sixteen penetrating keratoplasties performed in patients younger than 2 years of age were reviewed to determine which factors were associated with high postoperative refractive errors. When tissue from donors younger than 2 years was used, the mean postoperative spherical equivalent was -13.7 diopters, compared with +2.0 diopters when tissue from donors older than 2 years was used (P = 0.001). Analysis of variance suggests that the use of a 0.5-mm oversized transplant, as opposed to a 0- to 0.3-mm oversized transplant, contributed to the production of high myopia when tissue from donors younger than 2 years was used (P = 0.009). The high myopia appears to have resulted from steep transplant curvatures. Based on these results, we recommend against the combined use of corneal transplant tissue from very young donors and 0.5-mm oversized transplants for infant penetrating keratoplasties.