The aetiology in paediatric aphakic glaucoma.

PURPOSE: To investigate the causes of glaucoma in children following removal of cataracts. METHODS: In total, 24 patients (37 eyes) with uncomplicated congenital cataracts who developed glaucoma following cataract removal were studied retrospectively. Cataract morphology, surgical technique, postoperative complications, time to glaucoma onset, gonioscopic findings, the presence of microcornea, and the histopathologic characteristics of the filtration angle in one case were the studied parameters. RESULTS: We found a bimodal onset of glaucoma. Early-onset glaucoma occurred at a mean age of 6 months in 15 eyes and delayed-onset glaucoma at a mean age of 12 years in 22 eyes. Early-onset glaucoma was significantly (P=0.018) more likely to be due to angle closure. With delayed-onset glaucoma, the filtration angle is open in 86% of eyes and significantly (P=0.006) more eyes in the delayed-onset group had microcornea. CONCLUSIONS: Performing cataract surgery very early in life in microphthalmic eyes and leaving residual lens material increases the risk for glaucoma. We recommend a prophylactic iridectomy in eyes at risk for pupillary block. Eyes with delayed-onset glaucoma have open filtration angles but with findings consistent with incomplete development of filtration structures. Early age at cataract extraction and microcornea are risk factors for delayed-onset glaucoma.

The prevalence of uveitis in juvenile rheumatoid arthritis.

BACKGROUND: Because asymptomatic uveitis has been an important cause of visual loss in children with juvenile rheumatoid arthritis, periodic ophthalmologic screenings of such patients have been recommended. Recently, some authors have found a decreased prevalence of uveitis in children with juvenile rheumatoid arthritis. METHODS: We studied a total of 76 patients (63 girls and 13 boys, aged 1 to 16 years), referred to 3 pediatric ophthalmology practices between March 1976 and October 1999. Follow-up examinations were performed at intervals of 3 to 6 months according to current guidelines, during the following 6 months to 23 years (mean, 55 months). RESULTS: Uveitis developed in 10 children (13%). Of these 10 children, 2 were symptomatic (blurred vision, discomfort) and 7 were diagnosed with uveitis at the initial visit. Only 1 patient had asymptomatic uveitis after initial negative findings on screening examination. Final visual acuity for all the compliant children in the uveitis group was better than 20/30. DISCUSSION: The prevalence of uveitis in our study is similar to rates found by other recent authors. This decrease may reflect a tendency for systemic medications to prevent the development of ocular inflammation. We believe that screening guidelines should be reevaluated, especially for asymptomatic children with negative findings on initial examinations.

High levels of binocular function are achievable after removal of monocular cataracts in children before 8 years of age.

OBJECTIVE: To investigate the visual acuity and binocular function results achieved in children who had monocular cataracts removed before 8 years of age. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Clinical records of 171 patients who underwent a unilateral cataract removal between January 1986 and 1996 were reviewed retrospectively. Seventy-four eyes were included in the study: 19 congenital, 11 developmental, 19 posterior lenticonus, 19 traumatic, and 6 complicated cataracts. Patients with less than 2 years of follow-up; eyes with cataracts resulting from retinoblastoma; prematurity; and those associated with dense corneal scars, lens dislocation, and persistent hyperplastic primary vitreous were excluded. INTERVENTION: Visual acuity was measured by means of age-appropriate tests such as the fixation pattern, Allen object recognition cards, isolated optotypes with the Sheridan Gardiner test, and Snellen letters. Sensory fusion was assessed with the Worth 4-dot test, and stereo acuity was assessed with the Titmus stereo test. MAIN OUTCOME MEASURES: Cataracts were classified regarding type, extent, age at onset, duration of the opacity, age at surgery, method of removal, development of secondary membrane, form of optical rehabilitation, and presence of strabismus. Visual acuity levels between 6/6 and 6/12 were considered "good." Fusion of the Worth 4-dot test at distance and near, and presence of stereo acuity of 100 seconds of arc or better were considered "good" binocular function. Multiple logistic regression analysis was used to define factors that correlated with achieving good visual outcome. RESULTS: Visual acuity was 6/12 or better in 27 (36.5%) eyes. However, good binocular function was achieved in only 11 of these 27 patients. Results of univariate analysis showed that later age at onset of cataract and absence of strabismus were significant for good visual acuity and binocular function. The presence of strabismus increases the risk of not achieving good visual acuity by 5.45-fold. CONCLUSIONS: Good visual acuity and binocular function can be achieved after removal of monocular cataracts in visually immature children. Patients with strabismus at presentation or during the follow-up period have the least chance of achieving a good sensory result.

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.

Long-term outcome of cyclocryotherapy for refractory pediatric glaucoma.

OBJECTIVE: To evaluate the success rate and long-term outcome of cyclocryotherapy for refractory pediatric glaucoma. DESIGN: Retrospective interventional case series. PARTICIPANTS: A total of 64 eyes of 49 patients from 2 institutions with pediatric glaucomas resistant to conventional medical and surgical therapies treated with cyclocryotherapy from 1975 to 1996 were included in this review. INTERVENTION: Cyclocryotherapy was performed on eyes with pediatric glaucoma resistant to maximal medical and surgical interventions. Each cyclocryotherapy session was evaluated in terms of area treated, temperature, and number of applications placed. MAIN OUTCOME MEASURES: Criteria for success included intraocular pressure (IOP) of 21 mmHg or less without devastating complications or need for further glaucoma surgery. RESULTS: The mean baseline pretreatment IOP of all eyes was 30.0 +/- 8.1 mmHg. Six months after their last treatment, 42 eyes (66%) were successes. Longer term follow-up (mean, 4.8 +/- 3.3 years) yielded a lower final success rate in 28 eyes (44%). For these 28 eyes, mean IOP was reduced from 30.3 +/- 7.8 mmHg pretreatment to 16.8 +/- 4.0 mmHg after their last cyclocryotherapy treatment session (P < 0.001). The average number of cyclocryotherapy sessions for successful eyes was 4.1 +/- 4.0 (range, 1-17). The mean follow-up time for these successful eyes was 4.9 +/- 3.4 years. Devastating complications attributable to cyclocryotherapy included phthisis (5 eyes) and retinal detachment (5 eyes). Devastating complications occurred more frequently among eyes with aniridia than among all other eyes (nonaniridics) (50% vs. 11%, respectively; P < 0.05). CONCLUSION: Cyclocryotherapy is an effective means of lowering IOP and is a reasonable treatment option in selected pediatric patients with refractory glaucoma. Eyes with aniridia experienced a very high rate of phthisis after cyclocryotherapy and may be poor candidates for this treatment.

Results following treatment of third cranial nerve palsy in children.

PURPOSE: To investigate the etiology, sensory, motor, and cosmetic results of treatment for oculomotor (CNIII) palsy in children. METHODS: We conducted a retrospective review of the clinical records of children with a diagnosis of CNIII palsy who were followed up in our practice between 1981 and 1996. RESULTS: During the 15-year period, 49 children with 53 affected eyes were followed for a mean of 5.5 years. CNIII palsy was congenital in one third of cases and secondary to postnatal trauma in another third. Thirty-three of the eyes were affected before visual maturation (age 8 years) and 27 eyes developed amblyopia. None of the 6 eyes with amblyopia in which visual acuity could be quantitated had measurable improvement of Snellen acuity after treatment. Overall, visual acuity was between 6/5 and 6/12 at the last follow-up visit in 56% of affected eyes. Ocular alignment was greatly improved after recess-resect procedures on the horizontal rectus muscles, but binocular function was difficult to preserve or restore. Blepharoptosis improved after levator palpebrae muscle resection or eyelid suspension procedures. CONCLUSIONS: CNIII palsy may undergo partial resolution in children, but surgical treatment is frequently necessary. Although surgery can result in cosmetically acceptable alignment of the eyes, it rarely results in restoration or achievement of binocular function. Multiple procedures are often necessary to maintain good ocular alignment. Several surgical procedures may be needed to correct related blepharoptosis and maintain an acceptable eyelid position. Treatment of amblyopia is only effective in maintaining the level of visual acuity present at the onset of the CNIII palsy, and improvement in 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.

Scleral fixation of posterior chamber intraocular lenses in children.

BACKGROUND AND OBJECTIVE: Children with monocular aphakia who become contact lens intolerant require an intraocular lens (IOL) for visual rehabilitation. When there is inadequate support from the posterior lens capsule, use of an anterior chamber IOL or a sclerally fixated posterior chamber IOL may be considered. The authors report their experience with scleral fixation of posterior chamber IOLs in children. PATIENTS AND METHODS: Seven posterior chamber IOLs were sutured in the ciliary sulcus of children who could not wear contact lenses. In each eye, the lens capsule remnants were inadequate to provide sufficient support for the haptics of a posterior chamber lens. RESULTS: Six of seven patients had improved visual acuity, with an average improvement of 4 lines. Complications related to scleral fixation included exposure of the scleral fixation suture in one eye, lens decentration in one eye, and lens tilt in one eye. Follow-up ranged from 3 to 38 months, averaging 26 months. CONCLUSION: Implantation of a posterior chamber sclerally fixated IOL offers an alternative to placement of an anterior chamber lens in children who cannot wear contact lenses and who lack capsular support. Continued follow-up of these patients will help determine the long-term safety of this technique.

Infantile exotropia.

BACKGROUND: Exotropia is uncommon in healthy children between 6 months and 1 year old. METHODS: The charts of patients treated in our practice between January 1980 and August 1994 were reviewed for a diagnosis of infantile exotropia. All of them had an exodeviation during the first year of life. Children with neurologic defects, prematurity, trauma, craniofacial syndromes, or orbital abnormalities or ocular defects that would reduce vision were eliminated. RESULTS: Sixty-six healthy patients with normal eyes had an exodeviation of 15 prism diopters (delta) or more that persisted through the first year of life. Fifty-four were eliminated because the diagnosis had not been confirmed by a pediatric ophthalmologist before they were 1 year old or they had not been followed for more than 4 years. The 12 remaining patients were followed for at least 4 years (mean, 7.9 years). The mean age at the first examination was 7.8 months (range, 4 to 12 months). On early evaluation, exotropia was intermittent in four and constant in eight. Three had amblyopia. Ten required surgical correction. Exotropia was corrected with one operation in six patients; four required additional procedures, mostly to correct oblique muscle overaction and dissociated vertical deviation (DVD). At the most recent visit, all 12 patients had equal visual acuity in both eyes and satisfactory ocular alignment. Five had fusion at distance and near, but only two had stereo acuity of 100 seconds of arc or better. CONCLUSION: Infantile exotropia is rare. But, like patients with infantile esotropia, those with exotropia can be expected to have good visual acuity but unstable ocular alignment. Also, as with early-onset esotropia, although surgical intervention is usually required, high levels of binocular function develop in some patients with exotropia. Oblique muscle overaction and DVD are common, often becoming manifest by the first year of age. Unlike infantile esotropia, nystagmus is rare.

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.

Incidence of increased intracranial pressure after early surgical treatment of syndromic craniosynostosis.

The late development of intracranial hypertension after an aesthetically successful cranial vault expansion has been noted anecdotally in children with syndromic synostosis. If untreated, this process can lead to neurological and visual deterioration; however, the frequency of this problem is uncertain. In an attempt to detect this process before the onset of irreversible impairment, the authors have since 1991 incorporated routine ophthalmological evaluations into the multidisciplinary follow-up protocol for all patients with complex craniosynostosis. These examinations were performed at initial evaluation and at 6-month intervals thereafter. The present report focuses on the results in 22 consecutive infants with syndromic synostosis who underwent initial surgery between 1991 and 1994. All but 4 children underwent initial cranial reconstruction at 6-18 months of age. Four patients had papilledema preoperatively which in each instance resolved postoperatively. During the follow-up period, 8 children manifested evidence of late intracranial hypertension at a median of 16.5 months after initial operation(s): 4 developed asymptomatic papilledema, 1 had progressive proptosis, and 3 had other subtle clinical signs of increased intracranial pressure (ICP). Further evaluation disclosed a Chiari 1 malformation in 2 children; 4 had a 'beaten copper' appearance on skull radiographs, and 4 underwent lumbar puncture, which in each case demonstrated an opening pressure above 20 cm H2O. Only 3 children exhibited symptoms from the ICP elevation. Six children with a head size < or = the 25th percentile underwent a repeat cranial expansion; after the cranial vault expansion, one child who initially had slit-like ventricles developed ventriculomegaly and, because of persistent papilledema, underwent insertion of ventriculoperitoneal (VP) shunt. Two other patients with a head circumference above the 50th percentile and the development of moderate ventriculomegaly underwent VP shunt insertion alone. All children have had complete resolution of papilledema as well as other symptoms and signs of increased ICP without evidence of optic atrophy or neurological sequelae. This study indicates that the incidence of delayed, asymptomatic increases in ICP among children with complex craniosynostosis is higher than previously estimated. The pathophysiologic bases for this process are discussed. Because the detection of this problem at a presymptomatic stage should optimize the likelihood of a good functional outcome, we strongly recommend close ophthalmological and clinical follow-up as a part of the comprehensive care of all young children with complex craniosynostosis, even after a cosmetically successful cranial expansion.

Results following secondary intraocular lens implantation in children.

BACKGROUND: Placement of a secondary intraocular lens (IOL) in a child may be considered in children with congenital monocular cataracts who have had complete opacities removed early in life and who later become contact lens intolerant, in eyes that have received trauma which precluded placement of a primary IOL, and in young adults who have bilateral aphakia who become resistant to use of contact lenses or spectacles. METHODS: Clinical records of all children in our practice who received a secondary IOL between January 1988 and December 1994 were reviewed. Indications, biometry, type of procedures, preoperative and postoperative acuity, refractive error, binocular status, and complications were studied. RESULTS: During the 7-year period, 242 cataract operations were performed. Fifty-nine eyes received a lens implant, and 28 of these were secondary implants. There was a mean interval between the initial cataract operation and the procedure for the secondary implant of 77 months. The mean follow-up was 35 months (range, 3 to 71) for the 28 eyes that received a secondary implant. Two received anterior chamber implants. Eight eyes had insufficient capsular support for an IOL. Six implants were placed in the posterior chamber and required suture fixation to the sclera. Twenty of 28 eyes had a measurable improvement in visual acuity. Only 1 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, and 75% were within 3.00 diopters. During the follow-up period, 2 eyes developed glaucoma. One had a transient pressure elevation, and the second has required 2 filtration procedures. Three patients required a Nd: YAG capsulotomy. Six patients demonstrated Worth fusion at distance and near, but only 3 patients demonstrated 200 seconds of arc or better stereo acuity. CONCLUSION: Placement of contemporary-style, secondary intraocular lenses in children and young adults appears to provide a safe and effective alternative for correction of aphakia in children who become contact lens or spectacle-intolerant.

Strabismus associated with meningomyelocele.

INTRODUCTION: Strabismus occurs frequently in patients with spina bifida or meningomyelocele. PATIENTS AND METHODS: I performed 1544 eye examinations in 298 patients with spina bifida over a period of 15 years. The patient records were retrospectively reviewed for visual acuity, binocular function, and strabismus. Select patients with A-pattern strabismus were recalled for neuroimaging. RESULTS: Visual acuity was correctable to 20/40 in 85% of the patients. One hundred and eighty-three patients (61%) had strabismus at some time over the study period. Sixty-three percent had an esodeviation; 37% an exodeviation. A-pattern strabismus was more common in patients with exotropia (39%) than in those with esotropia (27%). Defects on neuroimaging in the region of the vertical gaze pathways in the brain stem were correlated with A-pattern strabismus. CONCLUSION: With increased longevity of the patient with spina bifida, quality-of-life issues become increasingly important. Amblyopia, strabismus, and other acquired defects in the visual system related to hydrocephalus should be closely monitored and treated when indicated.

The results of surgery for benign tumors of the cavernous sinus.

CAVERNOUS SINUS SURGERY has been performed increasingly in the last 2 decades because of new knowledge and technologies. With increasing international expertise in cavernous sinus surgery, the results must be analyzed critically to search for accurate prognosticators of outcome. We performed a retrospective review of 124 patients (40 male, 84 female; mean age, 45 years) who underwent cavernous sinus surgery for benign tumors from 1983 to 1992. Sixty-five percent had tumors encasing the internal carotid artery. Mean follow-up was 29 months (median, 26 mo). Gross total or near-total resection was possible in 80%. Patients with neurilemomas, angiofibromas, epidermoids, chondroblastomas, and hemangiomas were more likely to have total or near-total resection (100% versus 75%, P < 0.025). Disabling complications (five cerebral infarctions, two meningitis, and one hydrocephalus with chiasmal prolapse) occurred only in patients with meningiomas or pituitary adenomas. On follow-up, excellent/good binocular vision was achieved in 53% of patients entering surgery with excellent/good function versus 25% who entered surgery with fair/poor binocular vision (P < 0.025). Ninety-three percent of patients had a Karnofsky score > or = 70 on follow-up. There were a total of 12 recurrences (10%), 6 in patients with meningiomas, 2 in patients with angiofibromas, 2 in patients with craniopharyngiomas, 1 in a patient with a pituitary adenoma, and 1 in a patient with an osteoblastoma. Patients with tumor growth or neurological symptoms indicative of progressive cavernous sinus involvement should undergo cavernous sinus exploration. This surgery has acceptable morbidity and mortality and, if the tumor can be removed easily, the surgeon should try to perform radical tumor resection. To avoid major complications, the surgeon must exercise utmost care to preserve the neurovascular structures of the cavernous sinus, with special attention to tumors that extend into the petroclival region. Better results from surgery can be expected in those patients with neurilemomas, hemangiomas, or epidermoids than in patients with meningiomas, craniopharyngiomas, or pituitary adenomas. Good functional outcome can be expected, particularly if the patient's preoperative clinical status is good. Particular attention must be paid to the reconstruction of anatomic barriers in order to prevent cerebrospinal fluid leakage and subsequent meningitis.

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.

Intracranial hypertension after cranial vault decompression for craniosynostosis.

A 3-year-old boy with chronic papilledema, bilateral coronal synostosis and Pfeiffer syndrome underwent an orbitofrontal advancement. One month postoperatively, his papilledema had resolved, however, his vision progressively deteriorated over the ensuing 3 months to legal blindness. He had no symptoms or signs of increased pressure. Multiple imaging studies were unremarkable except for dilatation of the optic nerve sheaths. A lumbar puncture revealed intracranial hypertension, which resolved after treatment with a lumboperitoneal shunt. Intracranial hypertension may persist after craniofacial reconstruction. In patients who have progressive neurological deterioration after craniofacial reconstruction, direct measurement of intracranial pressure is indicated, despite a lack of clinical signs or symptoms of intracranial hypertension and normal imaging studies.

Control of eyelid retraction associated with Graves' disease with botulinum A toxin.

Two patients had satisfactory control of eyelid retraction associated with thyroid orbitopathy with repeated treatment of the levator palpebrae superioris muscle with botulinum A toxin. The effects of the toxin lasted for 3 to 4 months.