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Introduction: Retinopathy of prematurity (ROP) is a vision-threatening disease of premature infants. Practice guidelines recommend that all infants screened for ROP receive follow-up eye examinations to screen for ophthalmic complications. 1 The purpose of this study was to identify risk factors for the development of strabismus, amblyopia, high refractive error, and cataracts among ROP-screened, non-treated infants., Methods: Retrospective single-centre study of ROP-screened, non-treated premature infants with ophthalmic follow-up. Clinical variables were screened for association with ocular findings at follow-up. Multivariable logistic regression was used to determine the risk factors associated with ocular findings., Results: 309 patients were seen for follow-up at 0.97 (0.69) [mean (SD)] years after neonatal intensive care unit (NICU) discharge. Strabismus was predicted by occipitofrontal circumference (OFC) z-score at NICU discharge (OR 0.61; 95% CI [0.42, 0.88]; p = 0.008), intraventricular haemorrhage (IVH) grade III or IV (OR 3.18; 95% CI [1.18, 8.54]; p = 0.02), and exclusive formula feeding at NICU discharge (OR 2.20; 95% CI [1.07, 4.53]; p = 0.03). Significant predictors of amblyopia were OFC z-score at discharge (OR 0.55; 95% CI [0.31, 0.96]; p = 0.03) and necrotising enterocolitis (NEC) (OR 6.94; 95% CI [1.38, 35.00]; p = 0.02). NEC was a significant risk factor for high refractive error (OR 7.27; 95% CI [1.39, 37.94]; p = 0.02)., Conclusions: Among premature infants screened but not treated for ROP, severe IVH, NEC, low OFC z-score, and exclusive formula feeding at NICU discharge were risk factors for ocular morbidity. These findings affirm the value of ophthalmic follow-up for all ROP-screened infants, particularly those with the identified risk factors., (© 2024. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Introduction: Childhood retinoblastoma (RB) survivors are known to experience long-term morbidity; however, eye-related quality of life (QoL), which may significantly impact activities of daily living (ADL), has not been extensively studied in this population. The purpose of this cross-sectional study was to assess QoL and ADL morbidity among school-age RB survivors., Methods: The Pediatric Eye Questionnaire (PedEyeQ) and Roll Evaluation Activities of Life (REAL) were administered to childhood RB survivors between ages 5 and 17 followed at St. Louis Children's Hospital. Visual outcomes and demographic predictors of ADL and QoL were examined., Results: Total 23 patients (mean age 9.6 years) consented for participation in this study. All children experienced at least one domain on the PedEyeQ ≤ 80%. Subjects and parents marked functional vision to be the most impacted domain with a median score of 82.5 and 83.4, respectively. Only 10.5% of participants scored above 75% on the ADL percentile rank. On multivariable analysis, decreased visual acuity (VA) was associated with worse "Child Functional" (odds ratio [OR] -59.2, p = .004) and "Parent Worry Function" (OR -66.5, p = .03) metrics. Decreased contrast sensitivity was associated with worse "Parent Impact" (OR 21.0, p = .02) and "Parent Worry Function" (OR 3.70, p = .04) metrics. Longer saccade horizontal latency was associated with a worse "Parent Worry Function" metric (OR 43.0, p = .009). On multivariable analysis, no variable was significantly associated with ADL., Conclusion: RB survivors have impaired QoL and ADL. Screening for such difficulties should strongly be considered for all RB patients. Additional studies may help predict morbidity based on visual metrics and demographic data., (© 2023 Wiley Periodicals LLC.)

Background: Retinoblastoma (RB) and its associated treatments can significantly impact visual acuity. However, little is known regarding other measures of vision, such as contrast sensitivity or saccades. The aim of this study was to describe contrast sensitivity and saccades in children treated for retinoblastoma., Methods: This cross-sectional study included children aged 5-17 years who had completed treatment for RB. Visual acuity, saccades via fixation analysis, and contrast sensitivity by Cardiff contrast sensitivity were assessed, and multivariable linear regression was performed., Results: Eleven children were enrolled (mean age, 10.7 ± 3.9 years). Treatment included enucleation (8 children [73%]) and chemotherapy (10 [91%]). Of the 11, one participant was unable to complete testing of saccades, and another was unable to complete contrast sensitivity testing. Decreased saccade parameters (velocity, latency, or accuracy) and impaired contrast sensitivity were observed in all 10 participants who underwent visual testing. Multivariable analysis revealed that worse logMAR visual acuity (B, -4.54 [-6.8, -2.2]; P = 0.004) and bilateral disease (B, -3.9 [-6.4, -1.4]; P = 0.009) were associated with worse contrast sensitivity. Germline disease was associated with decreased vertical saccade accuracy (P = 0.02)., Conclusions: Decreased contrast sensitivity and impaired saccades were universally observed in this cohort of RB survivors. Comprehensive visual evaluation should be considered for all RB survivors to provide optimal rehabilitative services for these patients., (Copyright © 2023 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To determine practice patterns of pediatric ophthalmologists with respect to types of medical conditions cared for and age of patients treated due to limited data regarding pediatric ophthalmologists' scope of practice., Methods: A survey was sent to 1408 international and United States members of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) membership through the group's internet listserv. Responses were collated and analyzed., Results: Ninety members (6.4%) responded. 89% of respondents confine their practices to pediatric ophthalmology and adult strabismus. The percentages of respondents who provide primary surgical and medical treatment of the following conditions include 68% for ptosis and anterior orbital lesions, 49% for cataracts, 38% for uveitis, 25% for retinopathy of prematurity, 19% for glaucoma, and 7% for retinoblastoma. For conditions other than strabismus, 59% restrict their practice to patients less than 21 years of age., Conclusion: Pediatric ophthalmologists provide primary medical and surgical care for children with a wide variety of ocular conditions, including complex disorders. Awareness of this variety of practice might prove beneficial in encouraging residents to consider careers in pediatric ophthalmology. Consequently, fellowship education in pediatric ophthalmology should include exposure to these areas.

Purpose: Differing techniques have been reported for focal laser therapy for patients with small and medium retinoblastoma. We report the technique used at our center; and report the functional and anatomical outcomes for small and medium retinoblastomas treated with focal laser therapy with or without systemic chemotherapy., Methods: A retrospective case study was conducted including pediatric patients with macular retinoblastoma treated with systemic chemotherapy and laser ablation from July 1990 to July 2015 at Washington University School of Medicine/Saint Louis Children's Hospital., Results: Fourteen eyes (11 patients) with small and medium retinoblastoma tumors were treated with repetitive indirect laser hyperthermia and seven of those patients were treated with systemic chemotherapy as well. Using the International Retinoblastoma classification, one eye was stage A, 10 eyes were stage B, and three eyes were stage C. The mean follow-up time was 7.7 years. There were no recurrences of tumor in the patients. Final visual acuity outcomes were 20/20 to 20/50 in four eyes, 20/60 to 20/200 in four eyes, and 20/400 or less in six eyes. None of the patients developed metastatic disease., Conclusions: The evidence for systemic chemotherapy and diode laser therapy is limited to case series and retrospective reviews, but evidence suggests that it is an effective treatment for small and medium sized retinoblastoma tumors involving the macula with the potential for good visual outcomes.

Purpose: The purpose of this study was to describe the academic performance of childhood retinoblastoma (RB) survivors., Design: Retrospective cohort study., Methods: Retrospective chart review of children followed in a survivorship clinic., Results: A total of 73 patients with RB (median age at diagnosis: 9.97 months; range: 0.29-65.1) were followed for a median of 6.4 years (0.2-1.76). A total of 48 patients (65.8%) had unilateral RB; 43 patients (63.0%) received systemic chemotherapy; and 57 patients (78.1%) underwent enucleation. At last follow-up, 5 children (6.8%) had bilateral visual acuity (VA) <20/70. Seventeen subjects (23.3%) reported school difficulties, and 10 subjects (13.7%) had an individualized education program (IEP). Multivariate analysis revealed that a history of receiving chemotherapy" Multivariate analysis revealed that a history of receiving chemotherapy was associated with self-reported school difficulties (odds ratio [CI]: 5.44; 95% confidence interval [CI]: 1.36-21.69; P = .016), and undergoing an IEP (OR: 11.47; 95% CI: 1.34-98.16; P = .03). The degree of visual impairment and history of enucleation did not influence the risk of self-reported school difficulties or the implementation of an IEP. Among unilateral RB patients, chemotherapy was an independent risk factor for self-reported school difficulties (OR: 12.8; 95% CI: 1.45-113; P = .009) and implementation of an IEP (OR: 15.2; 95% CI: 0.78-292; P = .02)., Conclusions: Academic difficulties in childhood RB survivors are associated with chemotherapy treatment, a risk factor independent of VA., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To report outcomes of nasolacrimal duct (NLD) probing in children 4 years and older with simple membranous NLD obstruction., Methods: The records of all patients 4 years and older with congenital NLD obstruction who underwent surgery from 1997 to 2015 at Washington University School of Medicine were retrospectively reviewed. Of 47 patients reviewed, 18 (38.3%) were found to have simple membranous obstructions and were included in this study. Simple membranous obstruction was present at the distal duct and was relieved with passage of the probes in all patients. Children with canalicular or diffuse distal NLD stenosis (as defined by a tight, gritty feeling or multiple obstructions when passing the probe through the bony portion of the NLD), trisomy 21, lacrimal trauma, or craniofacial abnormalities were excluded. A successful outcome was determined by resolution of epiphora and periocular crusting., Results: Eighteen patients with ages ranging from 4.1 to 10.6 years with simple membranous NLD obstruction were treated. Sixteen of 18 (88.9%) patients had good outcomes following NLD probing. Two patients had persistent symptoms that resolved following balloon dilation and stent placement., Conclusions: This study found that the success rate of probing in older patients with simple membranous NLD obstruction was comparable to that of younger patients. NLD probing alone is a good treatment option for older children with simple membranous NLD obstruction. Additional procedures such as balloon catheter dilation or stent placement may not be necessary at the time of initial probing. [J Pediatr Ophthalmol Strabismus. 2020;57(1):44-47.]., (Copyright 2020, SLACK Incorporated.)

Peroxisomal biogenesis disorders are caused by disruption of long chain fatty acid metabolism due to mutations in PEX genes. Individuals with these disorders often have vision loss due to optic atrophy and pigmentary retinopathy. We report an unusual retinal manifestation of peroxisomal biogenesis disorder.

Competing Interests: Conflict of Interest: No conflicting relationship exists for any author.

Introduction: A subpopulation of children with anisometropic myopia, amblyopia, and neurobehavioral disorders is noncompliant with spectacle wear and ill-suited to correction using contact lenses. We report the results of refractive surgery in a series of these children treated using lensectomy alone (clear lens extraction) or lensectomy with simultaneous implantation of an intraocular lens (refractive lens exchange)., Methods: Clinical course and outcome data were collated retrospectively in a group of 7 children and adolescents (mean age, 9.1 years; range, 4-20 years) with neurobehavioral disorders and noncompliance with spectacle wear for anisometropic myopia. Myopia in the 7 eyes ranged -11.9 to -24.5 D (mean, -16.7 D). Goal refraction was 0 to + 4 D. Correction was achieved by lensectomy in 5 eyes and lensectomy with intraocular lens implantation in 2 eyes. Primary posterior capsulectomy/subtotal vitrectomy was performed during the primary procedure in 5 eyes (71%). Mean follow-up was 3.8 years (range, 0.5-5.4 years)., Results: Myopia correction averaged 17.3 D. A total of 86% (6 eyes) were corrected within +/- 3 D of the goal refraction and the remaining 14% to within +/- 4 D. Uncorrected visual acuity improved postoperatively in all 7 eyes, albeit modestly (average gain 0.14 Snellen fraction; gain from a mean 20/2550 to a mean 20/130). Myopic regression averaged approximately 0.43 D/year. Capsular opacification necessitated YAG-laser membranectomy in the 2 eyes that had preservation of the posterior capsule at the primary procedure. Retinal detachment has not occurred in any of the eyes during the follow-up period., Conclusions: Refractive myopic lensectomy reduced high anisometropia and improved functional vision in children who have high myopia beyond the range of excimer laser correction and who will not wear glasses. Further study is indicated to determine the long-term safety of this procedure in similar pediatric populations.

Introduction: A subpopulation of children with high myopia and neurobehavioral disorders is noncompliant with spectacle wear and ill-suited to correction using contact lenses. We report the results of refractive surgery in a series of these children treated using lensectomy alone (clear lens extraction), or lensectomy with simultaneous implantation of an intraocular lens (refractive lens exchange)., Methods: Clinical course and outcome data were collated prospectively in a group of 13 children (mean age 10.4 years, range 1 to 18 years) with neurobehavioral disorders exacerbated by chronic noncompliance with spectacle wear, causing profoundly low functional vision. Myopia in the 26 eyes ranged from -14.25 to -26.00 D (mean -19.1 D). Goal refraction was approximately +1 D. Correction was achieved by lensectomy alone in 10 eyes, and lensectomy with intraocular lens implantation in 16 eyes. Primary posterior capsulectomy/subtotal vitrectomy was performed during the primary procedure in 11 eyes (42%). Mean follow-up was 4.5 years (range 1.3 to 7.5 years)., Results: Myopia correction averaged 19.9 D. Eighty-one percent (21 eyes) were corrected to within +/-2 D of goal refraction and the remaining 19% (5 eyes) to within +/-4 D. Uncorrected acuity improved substantially (ie, an average 2 log units) in all 26 eyes, with commensurate gains in behavior and environmental visual interaction in 85% [corrected] of children (11/13) [corrected] Myopic regression averaged -0.16 D/year. Capsular regrowth and/or opacification necessitated vitrector or YAG-laser membranectomy in 13 [corrected] eyes (50%) [corrected] Focal retinal detachment (successfully repaired) occurred after eye contusion in one eye (4%) with cicatricial retinopathy of prematurity., Conclusions: Bilateral refractive lensectomy is effective for improving functional vision in neurobehaviorally impaired children who have high myopia (beyond the range of excimer laser correction: see companion publication) and difficulties wearing glasses. Posterior capsule regrowth/opacification is common, necessitating secondary membranectomy. Further study is indicated to determine the long-term safety of this procedure in similar pediatric populations.

Purpose: To report longer term results of transscleral sutured intraocular lens (TSSIOL) implantation in a sizable cohort of aphakic children, who were not suitable for contact lens wear and lacked adequate capsular support for sulcus fixation of an intraocular lens., Methods: Clinical outcome data were collated by retrospective review after surgery on 43 consecutive eyes in 32 aphakic children (mean age at implantation = 10 years; 33% < or = age 7 years). Outcome measures included visual acuity, postoperative refractive error, postoperative complications, and rate of reoperation. Follow-up averaged 37 months., Results: Visual acuity improved after surgery in 70% (30) of operated eyes (in 69% or 22/32 children). Fifty-one percent (22/43 eyes) improved by two lines or more. No patient suffered a loss of acuity or exacerbation of preexisting amblyopia. Postoperative refraction was within +/-2.0 D of the predicted refraction in 93% (40/43) of eyes. Complications, with the exception of one eye (2%), were minor/transient and resolved in the first week after surgery. Complications included small hyphemas (7%, 3/43 eyes), vitreous hemorrhage (5%, 2/43 eyes), and ocular hypertension or hypotony (5%). Two eyes (5%) exhibited episodes of iris capture of the IOL optic, one of which (2%, 1/43) eventually necessitated reoperation for IOL exchange. No retinal detachments or other retinal complications were encountered., Conclusion: TSSIOL implantation appears to be a safe and effective method for correcting aphakia in pediatric eyes that lack adequate capsular support. Safety over a follow-up period longer than the average 3 years reported here remains to be determined. The surgery is more difficult to perform than capsular-bag or sulcus implantation and potentially carries greater risks.

Purpose: To describe and evaluate alternate techniques for implanting intraocular lenses in children in the absence of adequate capsular support for traditional lens implantation., Methods: Postoperative results of 18 eyes with posterior chamber intraocular lenses (PCIOLs) sutured to the ciliary sulcus and 10 eyes implanted with anterior chamber intraocular lenses (ACIOLs) were reviewed. Visual outcomes were divided into 2 groups: onset of aphakia during the critical period of visual development (< or = 9 years) and onset after the critical period (> 9 years). Visual outcomes and complications were recorded., Results: Average follow-up was 10.3 months in the PCIOL group and 49.2 months in the ACIOL group. Eyes that became aphakic after the critical period of visual development achieved better overall final visual acuity than the eyes that became aphakic during the critical period; indeed, the eyes that became aphakic during the critical period did not achieve significantly improved vision. There were no complications in the PCIOL group. Complications in the ACIOL group included corectopia, haptic migration through the operative wound requiring removal, and pigment deposits on the lens., Conclusions: PCIOLs sutured to the ciliary sulcus offer a superior option to ACIOLs for correction of childhood aphakia in children lacking capsular support. ACIOLs had a high rate of serious complications (10%) in this small series. Secondary implantation with transsclerally sutured PCIOLs should be considered in complicated cases when more conservative options have been exhausted.

The association of alveolar capillary dysplasia with misalignment of pulmonary veins (ACD-MPV) and ocular abnormalities has not been previously reported. We present a case of ACD-MPV and anterior segment dysgenesis of the eye in a full-term infant as well as a review of the relevant literature.

Huntington's disease (HD) is a progressive neurodegenerative disorder with no effective treatment. Geldanamycin is a benzoquinone ansamycin that binds to the heat shock protein Hsp90 and activates a heat shock response in mammalian cells. In this study, we show by using a filter retardation assay and immunofluorescence microscopy that treatment of mammalian cells with geldanamycin at nanomolar concentrations induces the expression of Hsp40, Hsp70 and Hsp90 and inhibits HD exon 1 protein aggregation in a dose-dependent manner. Similar results were obtained by overexpression of Hsp70 and Hsp40 in a separate cell culture model of HD. This is the first demonstration that huntingtin protein aggregation in cells can be suppressed by chemical compounds activating a specific heat shock response. These findings may provide the basis for the development of a novel pharmacotherapy for HD and related glutamine repeat disorders.

The huntingtin exon 1 proteins with a polyglutamine repeat in the pathological range (51 or 83 glutamines), but not with a polyglutamine tract in the normal range (20 glutamines), form aggresome-like perinuclear inclusions in human 293 Tet-Off cells. These structures contain aggregated, ubiquitinated huntingtin exon 1 protein with a characteristic fibrillar morphology. Inclusion bodies with truncated huntingtin protein are formed at centrosomes and are surrounded by vimentin filaments. Inhibition of proteasome activity resulted in a twofold increase in the amount of ubiquitinated, SDS-resistant aggregates, indicating that inclusion bodies accumulate when the capacity of the ubiquitin-proteasome system to degrade aggregation-prone huntingtin protein is exhausted. Immunofluorescence and electron microscopy with immunogold labeling revealed that the 20S, 19S, and 11S subunits of the 26S proteasome, the molecular chaperones BiP/GRP78, Hsp70, and Hsp40, as well as the RNA-binding protein TIA-1, the potential chaperone 14-3-3, and alpha-synuclein colocalize with the perinuclear inclusions. In 293 Tet-Off cells, inclusion body formation also resulted in cell toxicity and dramatic ultrastructural changes such as indentations and disruption of the nuclear envelope. Concentration of mitochondria around the inclusions and cytoplasmic vacuolation were also observed. Together these findings support the hypothesis that the ATP-dependent ubiquitin-proteasome system is a potential target for therapeutic interventions in glutamine repeat disorders.

Background: Nasolacrimal duct (NLD) obstruction and tear film abnormalities occur frequently in children with trisomy 21. This study describes the outcomes of treatment for NLD obstruction in this population., Methods: The records of 15 children with trisomy 21 treated for NLD obstruction were reviewed. Eight patients were initially treated with NLD probing with or without placement of nonfixated lacrimal stents. Because of poor success with this procedure, the initial treatment of children with trisomy 21 and NLD obstruction was changed to balloon catheter dilation in 1997. Outcomes were considered excellent if the patient had complete resolution of epiphora and dacryocystitis, good if the patient had only mild residual symptoms, fair if the patient had significant residual symptoms, and poor if there was no improvement., Results: Of 8 patients treated initially with NLD probing, 5 had fair or poor outcomes. These patients all had good outcomes after placement of nasally fixated lacrimal stents, balloon catheter dilation, or both. Of 7 patients treated initially with balloon catheter dilation, 5 had excellent or good outcomes and 2 had fair or poor outcomes., Conclusion: Simple NLD probing is often unsuccessful in treating NLD obstruction in children with trisomy 21. Balloon catheter dilation appears to be a reasonable alternative first treatment in these patients.

Background: Anomalous retinal correspondence (ARC) is a neural adaptation to eye misalignment in which non-corresponding retinal points are linked in the visual cortex to provide binocular fusion. ARC within the striate cortex would require that horizontal neurons link right-eye and left-eye ocular dominance columns (ODCs) separated by a distance in the cortex proportional to the angle of strabismus. Two hypothetical mechanisms are possible: (1) The ODCs can be linked by axons of horizontal neurons that project monosynaptically from a right-eye to a left-eye ODC. The further apart the ODCs, the longer the axons; hence, axon length should be greater in subjects with strabismus than in healthy subjects (elongated axon, monosynaptic hypothesis). In this case, the clinical probability of developing ARC should be independent of the angle of strabismus, until an upper-limit angle of strabismus is reached equally to the maximal length of axons available to link nonadjacent ODCs, at which point an abrupt decline of ARC probability should be evident. (2) Alternatively, ODCs can be linked by a chain of horizontal neurons, the number of which increases as the distance among ODCs increases; axon length in subjects with strabismus would be expected to be the same as in healthy subjects (normal axon, polysynaptic hypothesis). In this case, the greater the angle of strabismus, the more horizontal neurons and synapses required for linkage, and the greater the probability of signal degradation. Thus, the clinical probability of developing ARC through a polysynaptic mechanism should be inversely proportional to the angle of strabismus. The purpose of this study was to test these 2 hypotheses neuroanatomically in primates and clinically in children., Methods: For the neuroanatomic portion of the study, biotinylated dextran amine was injected into ODCs of area V1 to label individual neurons. The length of the horizontal axons from these neurons was then compared in strabismic and normal monkeys. In the clinical portion of the study, the medical records of 192 children with strabismus were reviewed retrospectively. The angle of strabismus (prism cover test) and the presence of ARC (Bagolini striated lenses, Worth/Polaroid 4-dot) were recorded. Plots of the presence of ARC as a function of the angle of strabismus were obtained., Results: There was no significant difference in axon length between healthy (7. 02 +/- 0. 83 mm) and strabismic monkeys (6. 60 +/- 1. 07 mm) (P =.16). In children with strabismus, ARC decreased as the angle of strabismus increased (P <. 05). ARC was more prevalent in children who had primary or postsurgical deviations of

The Escherichia coli SeqA protein has been found to affect initiation of replication negatively, both in vivo and in vitro. The mechanism of inhibition is, however, not known. SeqA has been suggested to affect the formation and activity of the initiation complex at oriC, either by binding to DNA or by interacting with the DnaA protein. We have investigated the binding of SeqA to oriC by electron microscopy and found that SeqA binds specifically to two sites in oriC, one on each side of the DnaA binding site R1. Specific binding was found for fully and hemimethylated but not unmethylated oriC in good agreement with earlier mobility shift studies. The affinity of SeqA for hemi-methylated oriC was higher than for fully methylated oriC. The binding was in both cases strongly cooperative. We suggest that SeqA binds to two nucleation sites in oriC, and by the aid of protein-protein interaction spreads to adjacent regions in the same oriC as well as recruiting additional oriC molecules and/or complexes into larger aggregates.

Purpose: Myectomy of the inferior oblique muscle is a common procedure used to correct overaction of this muscle. Previous descriptions of the surgery have not discussed how the surgeon should handle the proximal muscle stump following myectomy. This study examined the effectiveness of inferior oblique muscle myectomy with tucking of the muscle into Tenon's capsule for treatment of inferior oblique muscle overaction., Methods: The procedure was studied prospectively in 22 patients who underwent 32 inferior oblique muscle myectomies. In 18 patients, the intraoperative behavior of the inferior oblique muscle was recorded. Complete ocular motility examinations were performed before and after surgery. Success was defined as the elimination of inferior oblique muscle overaction and the elimination of hypertropia secondary to persistent ipsilateral inferior oblique muscle overaction in primary gaze., Results: The surgery corrected inferior oblique muscle overaction in 94% of eyes. Hypertropia secondary to persistent ipsilateral inferior oblique muscle overaction was eliminated in 92% of patients. There was no correlation between outcome and the intraoperative behavior of the inferior oblique muscle., Conclusion: Tucking of the inferior oblique muscle stump into Tenon's capsule is recommended following inferior oblique myectomy. This procedure is safe and effective for correcting inferior oblique muscle overaction.

Unusual ocular motility abnormalities have been rarely reported to result from anomalous extraocular structures. These structures, which may be either muscular or fibrotic, attach to the globe and produce a mechanical restriction resulting in incomitant strabismus. To our knowledge, we report the first patient with an anomalous extraocular muscle in whom the clinical, radiologic, surgical, and histopathologic findings are described.

Objective: Allen figure optotypes commonly are used to measure visual acuity in young children. Children with normal acuity measured with Allen figures sometimes are found to have unsuspected amblyopia that is detected when they are tested with Snellen letters. The correlation between visual acuities measured with these two optotype charts has not been well studied. The authors compared visual acuities measured with Allen figure and Snellen letter optotypes using the Mentor B-VAT II monitor., Design: The study design was a nonrandomized, comparative clinical trial., Participants: The study was composed of 12 adult subjects., Intervention: Visual acuities were measured using both Allen figure and Snellen letter optotypes using the B-VAT II monitor. The images were progressively blurred using plus lenses., Main Outcome Measures: Visual acuity was measured., Results: At visual acuity levels of 20/60 or better, Allen figure testing averaged 1.5 lines better than Snellen letter testing; between 20/70 and 20/200 visual acuities, the difference was 2.5 lines., Conclusions: Allen figure testing with the B-VAT II monitor overestimates visual acuity compared with testing with Snellen letters. This appears to result primarily from the construction of the optotypes. This discrepancy should be considered when visual acuity is measured in young children.

Background: Dacryocystitis in infants and older children is a serious complication of congenital or acquired nasolacrimal duct obstruction. To define the modes of presentation and treatment strategies of this disorder better, we reviewed the clinical courses of 54 children treated for dacryocystitis at St Louis Children's Hospital., Methods: Clinical, neuroradiologic, and laboratory data were collated for all cases of dacryocystitis treated from 1990 to 1995. Average follow up of the children in this consecutive series was 1.75 years (range, 4 months to 5 years)., Results: Of the 54 patients, 36 (67%) had chronic low-grade dacryocystitis, which was treated with nasolacrimal duct probing on an outpatient basis. The remaining 18 patients (33%) had acute dacryocystitis, which was treated with a combined medical/surgical strategy. Medical treatment consisted of hospital admission for administration of intravenous antibiotics followed by inpatient surgery, which varied according to the age of the patient and the clinical history: 1) Acute dacryocystitis in neonates was treated surgically by nasolacrimal duct probing and nasal endoscopy for excision of intranasal duct cyst; 2) Acute dacryocystitis with periorbital cellulitis was treated surgically by nasolacrimal duct probing; 3) Acute dacryocystitis due to facial trauma was treated surgically by dacryocystorhinostomy and stent placement; and 4) Acute dacryocystitis complicated by orbital abscess was treated by inferior orbitotomy for orbital abscess drainage, simultaneous nasolacrimal duct probing, and stent placement., Conclusion: Dacryocystitis in the pediatric population may present in either chronic or acute forms. An effective and safe treatment for acute dacryocystitis is hospital admission, both for administering intravenous antibiotics and monitoring to rule out orbital cellulitis or abscess formation. Intravenous antibiotic therapy is followed within a day or two by surgery tailored to the clinical history. In the majority of both chronic and acute cases, nasolacrimal duct probing appears to be an effective treatment strategy.

Purpose: "Touching four" dots on the Worth 4-dot test is used sometimes as an indication of fusion in young children. The authors examined the reliability of this test., Methods: A computer simulation of the Worth 4-dot test generated images representing fusion, suppression, and alternate fixation. Sixteen children, ranging in age from 32 to 48 months, were examined using this test., Results: None of the children could accurately describe the images verbally. Alternate fixation could not be distinguished from fusion by asking the subjects to touch the dots. Monocular suppression was identified accurately in all subjects., Conclusion: Touching four dots on the Worth 4-dot test does not distinguish fusion from alternate fixation in children with normal ocular alignment. This has important implications regarding the diagnosis of monofixation syndrome and assessment of the response to a prism adaptation trial in young children.

Purpose: To determine whether treatment of macular retinoblastoma with chemotherapy and laser hyperthermia can lead to satisfactory visual acuity., Methods: A child with unilateral macular retinoblastoma was treated with chemotherapy and laser hyperthermia. Visual function was assessed one year after treatment., Results: At age 25 months, the patient had normal visual behavior and a normal spatial-sweep visual-evoked potential., Conclusion: Treatment of macular retinoblastoma with chemotherapy and laser hyperthermia may enable recovery of satisfactory visual acuity.

Background: Incontinentia pigmenti (IP) is an inherited disorder with many systemic and ocular manifestations. Abnormalities of the retinal pigment epithelium, including peripheral hypopigmented areas, have been reported occasionally in patients with IP. These have not been symmetric, and they have not involved the fovea. The visual function in affected areas has not been described., Methods: The ophthalmic examination and results of fluorescein angiography are described in a patient with IP and bilateral discrete hypopigmented macular lesions., Results: The patient had normal vision. Fluorescein angiography of the macular lesions revealed transmission of fluorescence in the early phase and staining in the late phase., Conclusion: The authors believe that this is the first report of bilateral macular hypopigmented lesions in a patient with IP, and it is the first to document normal vision in an affected area.