Journal of Aapos最新文献

Purpose

To evaluate the outcomes of a novel modification of the Nishida procedure with medial rectus recession (Nishida-MRc) for myopic strabismus fixus (MSF) and to compare this modified procedure with the half Jensen’s union with medial rectus recession (U-MRc).

Methods

The medical records of MSF patients who underwent strabismus surgery at a single institution between January 2017 and June 2022 were retrospectively reviewed. The main outcome measures assessed were postoperative improvements in ocular alignment and motility. Surgical success was defined as horizontal and vertical deviations ≤15^Δ.

Results

A total of 45 patients were included, of whom 39 had no previous strabismus surgery. All but 3 had follow-up ≥8 months. Nishida-MRc, with or without a traction suture (Ts), had a success rate (9/16 [56%]) higher, though not statistically significantly so, than U-MRc with or without Ts (11/29 [38%]). The Nishida-MRc group tended to have less frequent use of Ts (25% vs 52%; P = 0.076), and 94% of these patients had a deviation within 20^Δ, compared with 59% for U-MRc (P = 0.012). In cases with esotropia of ≥123^Δ, final residual esotropia in the Nishida-MRc without Ts (12.40^Δ ± 8.30^Δ) and U-MRc-Ts (19.75^Δ ± 18.62^Δ) groups was significantly lower (P = 0.019) than in the U-MRc without Ts group (63.40^Δ ± 40.83^Δ), and the average correction of esotropia was significantly greater (P = 0.014).

Conclusions

In our study cohort, Nishida-MRc produced a greater effect in the treatment of MSF than U-MRc.

Purpose

To determine whether extremely premature infants require screening for retinopathy of prematurity (ROP) if <31 weeks’ postmenstrual age (PMA).

Methods

The medical records of infants born in community hospital settings at <31 weeks’ gestational age (GA) were reviewed retrospectively. Prevalence and progression of ROP in infants born at <24 weeks’ GA were compared with infants born at 24-30 weeks’ GA.

Results

A total of 2,061 records were reviewed: 1,969 infants were born at 24-30 weeks’ GA; 92, at <24 weeks. Infants born <24 weeks’ GA were more likely to develop pre-plus and plus disease or require treatment than infants born 24-30 weeks’ GA (P < 0.0001) and did so earlier (P = 0.0001). Eight infants developed pre-plus or greater ROP <31 weeks’ PMA; 6 were born <24 weeks’ GA. Three infants developed plus disease or required treatment <31 weeks’ PMA, the earliest at 27 and 3/7 weeks.

Conclusions

Clinicians should consider initiating ROP screening examinations before 31 weeks’ PMA, particularly for infants born <24 weeks’ GA and those with lower birth weights.

Background

Pediatric aphakia may be treated conservatively with aphakic contacts or spectacles. Many families and surgeons opt for a secondary intraocular lens (IOL) when the child is older. In certain situations, pediatric aphakic patients must undergo implantation earlier than planned. The purpose of this study was to investigate how often and why early implantation occurs.

Methods

We retrospectively reviewed the medical records of consecutive patients who were left aphakic after cataract surgery in infancy and were seen at our institute at ≥4 years of age. Early implantation was defined as occurring at <4 years of age.

Results

A total of 175 patients fit inclusion criteria. We found that 22 of 90 patients (24%) with unilateral cataracts had undergone early secondary IOL implantation before 4 years of age compared to 10 of 85 patients (12%) with bilateral cataracts, a statistically significant difference in the relative risk of early implantation (OR 2.43 [95% CI 1.07-5.49]). Of our patients undergoing early implantation, 15 of 31 (44%) had Medicaid as the primary insurance provider, which is representative of the practice overall. In patients requiring early implantation, failure with contact lens accounted for 26 of 32 cases (81%), with 7 of 26 (27%) of these failures attributed to nonmedical reasons.

Conclusions

Of the factors we analyzed, only the presence of unilateral aphakia was associated with increased risk of early IOL implantation in our study cohort.

Congenital fibrosis of the extraocular muscles (CFEOM) type 1 is associated with heterozygous missense variants in KIF21A, which encodes a kinesin-like motor protein. Individuals with CFEOM1 have severe paralysis of upgaze and ptosis, resulting in a pronounced chin-up head posture. There can also be limitations of horizontal eye movements. Loss of function of KIF26A, an unconventional kinesin motor protein that lacks ATP-dependent motor activity, has been recently reported to cause a spectrum of congenital brain malformations associated with defects in migration, localization, and growth of excitatory neurons. It has also been associated with megacolon resembling Hirschsprung’s disease. We report the case of a boy with homozygous loss of function of KIF26A with restricted eye movements, specifically restricted upgaze and downgaze with variable nystagmus and dissociated vertical eye movements. This case represents a congenital cranial dysinnervation disorder, most similar to CFEOM, and is the first report of a congenital cranial dysinnervation disorder caused by a kinesin other than KIF21A.

Terminal deletions of chromosome 3q are associated with a heterogenous clinical phenotype, which includes growth restriction, developmental delay, and intellectual disability. However, little has been published on the ophthalmic impacts of chromosome 3q deletions. We report a 9-year-old boy with a 1.4 megabase deletion of 3q27.1q27.2 whose ocular morbidities included retinal detachment in one eye, vitreous hemorrhage in the other eye, and foveal hypoplasia in both eyes that required acute care and continuous ophthalmologic follow-up.

Background

Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a recently defined optical coherence tomography (OCT) finding. The purpose of this study was to characterize the presence of PHOMS and their visual significance in pediatric patients with and without optic nerve pathologies.

Methods

This retrospective study evaluated 400 patients (<18 years of age) including normal control subjects and patients with optic neuritis, papillitis, optic nerve head drusen (ONHD), and papilledema. Information on demographics, visual function, and structural parameters were obtained.

Results

PHOMS were found in 7 of 258 normal control eyes (2.7%), 9 of 59 eyes with optic neuritis (15.3%), 58 of 76 eyes with ONHD (76.3%), 3 of 11 eyes with papillitis (27.3%), and 180 of 308 eyes with papilledema (58.4%). PHOMS were more prevalent in the papilledema (P < 0.001), ONHD (P < 0.001), and optic neuritis (P = 0.028) eyes than in control eyes. We identified 5 cases where PHOMS developed de novo. This occurred over an average of 2.3 years (range, 0.2-7.4 years). Sixteen cases of PHOMS resolved over an average of 1.1 years (range, 0.3-4.0 years). Cross-sectionally, PHOMS were not associated with visual acuity (P = 0.551), retinal nerve fiber layer thickness (P = 0.068), ganglion cell volume (P = 0.375), or visual field mean deviation (P = 0.795).

Conclusions

PHOMS are present in a majority of children with papilledema or ONHD. PHOMS are dynamic and may form de novo over time with optic nerve pathology and may resolve either through treatment or atrophy. There was no relationship between the presence of PHOMS and poor visual function in our study cohort.

Purpose

To evaluate the incidence of rise in intraocular pressure (IOP) in fellow eyes of patients with unilateral primary congenital glaucoma (PCG) and to identify risk factors for IOP increase over long-term follow-up.

Methods

The medical records of unilateral PCG patients who had completed at least 5 years of follow-up were reviewed retrospectively. The incidence of developing ocular hypertension / glaucoma in fellow eyes was analyzed. Fellow eye progressors were those which showed an increase in optic nerve cupping by at least 0.2 since the first presentation or had IOP of >21 mm Hg on two occasions. The risk factors for progression that were analyzed included IOP, visual acuity, axial length, central corneal thickness (CCT), corneal diameters (CD), presence or absence of angle dysgenesis on high-resolution anterior segment optical coherence tomography (AS-OCT), and morphology of aqueous outflow pathways.

Results

After a median follow-up of 8.2 years (range, 5-25.5) progression to bilateral disease was found in 17 of 54 patients (32%), of whom 8 (15%) developed ocular hypertension and 9 (17%) developed glaucoma in the fellow eye. Among the unaffected fellow eyes, those with a larger CD (>12 mm), measured after at least 5 years’ follow-up, were ten times more likely to progress (P = 0.01; OR = 9.5 [95% CI, 1.7-54.3]). The presence of a patent supraciliary channel was significantly more frequently associated in fellow eyes compared with affected eyes on AS-OCT (OR = 1.4 [95% CI, 0.46-4.68]).

Conclusions

One-third of unaffected fellow eyes of unilateral PCG eventually progress over time, most often after 5 years. Larger CD at follow-up in the fellow eye is strongly predictive for progression.

Background

Handheld optical coherence tomography (HH-OCT) can image awake, young children but lacks integrated segmentation/analysis software. OCT imaging of eyes with optic neuropathies demonstrates ganglion cell layer (GCL) and ganglion cell complex (GCC) thinning, with a normal or thickened inner nuclear layer (INL). We compared pediatric normative data with GCL/INL and GCC/INL ratios from HH-OCT macular scans of awake young children with clinically diagnosed optic neuropathies.

Methods

Macular HH-OCT from awake children with optic neuropathies was prospectively obtained using Bioptigen (Leica Microsystems, Wetzlar, Germany). The GCL, GCC, and INL were manually measured by two readers using ImageJ from single-line macular scans at the thickest points nasal and temporal to the fovea, respectively, and the GCL/INL and GCC/INL ratios were calculated and compared with normative data.

Results

HH-OCT images from 17 right eyes of 17 children (mean age, 4.3 ± 2.9 years) with optic neuropathies were analyzed. Mean nasal (17 eyes) and temporal (16 eyes) GCL/INL ratios with optic neuropathies were 0.44 ± 0.38 (95% CI, 0.26-0.62) and 0.26 ± 0.22 (95% CI, 0.15-0.36), respectively. Corresponding normative GCL/INL ratios are 1.26 ± 0.20 (95% CI, 1.19-1.34) and 1.23 ± 0.27 (95% CI, 1.13-1.33), respectively (P < 0.0001). Severe thinning precluded GCL measurements in 2 eyes nasally and 5 eyes temporally, resulting in GCL measurements of zero. Mean nasal (17 eyes) and temporal (16 eyes) GCC/INL ratios were 1.93 ± 0.70 (95% CI,1.60-2.27) and 1.67 ± 0.44 (95% CI,1.46-1.87). Corresponding normative ratios are 2.85 ± 0.38 (95% CI, 2.71-2.99) and 2.87 ± 0.42 (95% CI, 2.70-3.03), respectively (P < 0.0001).

Conclusions

GCL/INL and GCC/INL ratios calculated from single-line macular HH-OCT scans in awake young children with optic neuropathies differ significantly from normative values and may thus have utility in helping to establish a diagnosis of optic neuropathy.

Adams-Oliver syndrome (AOS) is a rare inherited disorder characterized by aplasia cutis congenita, cutis marmorata telangiectatica congenita, and terminal limb defects. Ocular associations have been rarely reported. We report a 6-month-old boy with AOS associated with refractory glaucoma, megalocornea, and anterior polar cataract. To our knowledge, this is the first case of glaucoma to be reported in association with AOS.

Among surveyed households, the transmission rate of pediatric conjunctivitis was 12%. Rates did not differ when the index child did or did not use an ophthalmic antibiotic (14% vs 11% [P = 0.6]). Transmission rates were lower than for other infections where children are not routinely excluded from school or daycare.