Ocular findings and genetic test in Alström syndrome in childhood.

This study aimed to investigate the mutation spectrums and ocular features of Alström syndrome (AS) patients. Six AS patients from five unrelated families were included. Ocular and systemic examinations were performed in all subjects. Whole-exome sequencing (WES) was performed in the probands, and Sanger sequencing was performed for mutation validation and segregation analysis. Among the six patients, the first symptoms included nystagmus, poor fixation, and photophobia. Five patients had high hyperopia, four of whom (80%) were initially diagnosed with amblyopia before referral with prescribed corrective lenses and amblyopia treatment, but no improvement was obtained. Optical coherence tomography (OCT) revealed progressive damage to the photoreceptor layer, including blurred ellipsoid zone (EZ) and lack of interdigitation zone (IZ) within the macula, and thorough loss of photoreceptor layer in the peripheral retina. Electroretinograms (ERG) demonstrated severely diminished cone and rod responses. WES identified biallelic variants of ALMS1 in all the six patients, including two novels, c.3892C > T (p.Gln1298*) and c.2888_2897del (p.Ser963Thrfs*15) and five knowns, c.10819C > T (p.Arg3607Trp), c.2090C > A (p.Ser697*), c.4891C > T (p.Gln1631*), c.10825C > T (p.Arg3069*) and c.6430C > T (Arg2146*). In conclusion, this study expanded the ocular features and genotypic spectrum of AS. High hyperopia is a significant and common feature of AS. OCT and ERG are essential accessory techniques for the diagnosis of AS. If a patient had high hyperopia with a noneffective response to amblyopic treatment, the diagnosis of AS should be suspected, and detailed ocular examination, systemic evaluation, and genetic testing recommended.

Posterior microphthalmos with achievement of good visual acuity and disappearance of papillomacular retinal folds: a case report.

BACKGROUND: Posterior microphthalmos (PM) is a rare condition with poor visual prognosis even after amblyopia treatment. We report a case of PM with achievement of good visual acuity and disappearance of papillomacular retinal folds (PFs) over a period of 7 years. CASE PRESENTATION: A girl aged 3 years and 5 months was referred to our hospital, after poor visual acuity was identified at a medical checkup for 3-year-olds. She had severe spherical hyperopia: + 17.25 D in the right eye (RE) and + 18 D in the left eye (LE). Her corrected visual acuity was 20/200 in the RE and 20/250 in the LE. PFs were observed in both eyes on optical coherence tomography (OCT), and the diagnosis of PM was made based on the normal corneal diameter and anterior chamber depth. During the course of the disease, a gradual decrease in the height of the PFs was observed on OCT. The corrected visual acuity at age 10 years was 20/20 in the RE and 20/25 in the LE. CONCLUSIONS: The visual prognosis of PM is poor, and only one case with good visual acuity has been reported in the literature. The patient in the present case not only developed good visual acuity, but also showed improvement in macular morphology, which was not noted in previous reports. Early diagnosis of PM and early amblyopia treatment is important for the visual development in PM.

Investigation of corneal biomechanics at moderate to high refractive errors.

PURPOSE: Corneal hysteresis (CH) and corneal resistance factor (CRF) are corneal biomechanical parameters which were measured by ocular response analyzer (ORA). Aim of this study was to define the CH and CRF in high myopic and hyperopic patients and compare the results with emmetropic control group. METHODS: A total of 193 eyes of 100 healthy volunteers were included. Study groups were high myopic patients (n = 27) with spherical refractive errors (SRE) of greater than -5.00D, high hyperopic patients (n = 20) with SRE of greater than +3.00D and controls (n = 53) with SRE between ± 1.00D. All subjects underwent IOP and corneal biomechanical evaluation with the ORA. Also Goldmann applanation tonometry (GAT), central corneal thickness (CCT), corneal curvature and axial length measurements were taken. RESULTS: Mean age of groups was 30.7 ± 6.9, 29.1 ± 7.7, 28.9 ± 5.6 years (p > 0.05). Among study groups except CRF and CCT, all parameters were significantly different between study groups. CH was lowest in the high myopic group and highest in the high hyperopia. In all groups, there were significant correlations between CH and CRF, CCT, SRE, SE (spherical equivalent), AL (axial length) and between CRF and CCT. GAT and IOPg (Goldmann-correlated intraocular pressure) measurements were significantly correlated with CCT (p < 0.05). One of the major findings was as the CH approaches 11.2 mmHg, IOPcc (corneal-compensated intraocular pressure) and IOPg get close to each other. CONCLUSIONS: The results revealed that CRF is not affected by refractive errors and IOPcc is not affected by any other ocular parameter. The difference between IOPcc and IOPg was greatest in myopic group, and IOP (intraocular pressure) measurement in these patients deserves high suspicion.

Longitudinal Changes in Refractive Development in Highly Hyperopic Children: A 2.6-11.2 Year Follow-up of Preschoolers Diagnosed with High Hyperopia.

PURPOSE: This study aims to elucidate the longitudinal refractive and ocular biometric alterations in preschool children with high hyperopia who underwent early interventions. METHODS: We conducted a retrospective analysis of preschool children diagnosed with high hyperopia at Tianjin Medical University Eye Hospital between 2011 and 2023. Inclusion criteria required an initial examination with cycloplegic refraction, bilateral spherical equivalent power (SE) ≥ +5.00D with a difference <1.00D, a minimum two-year follow-up, and at least three ocular biometric measurements. The annual axial growth rate evaluated emmetropization in highly hyperopic children. We applied Restricted Cubic Spline (RCS) models to explore potential nonlinear relationships between age and spherical equivalent, axial length, corneal curvature, and axial length-to-corneal curvature ratio. Additionally, Mixed-effects models were employed to investigate factors associated with changes in refractive error and axial length. RESULTS: The study enrolled 60 eligible subjects, with a median initial diagnosis age of 3.5 years (IQR, 2.8-4.9 years) and a median last visit age of 9.3 years (IQR, 8.1-10.8 years). The average follow-up duration was 5.7 years. RCS analysis revealed notable nonlinear changes in spherical equivalent power, axial length, and axial length-to-corneal curvature ratio, although corneal curvature displayed no statistically significant nonlinear trend. Factors affecting SE changes included the presence of strabismus, the use of cycloplegia, baseline SE, and age. Conversely, changes in axial length solely correlated with baseline axial length and age. CONCLUSION: Highly hyperopic preschool children undergoing early intervention display a marked emmetropization tendency, though most still remain moderately to highly hyperopic, with the progression of refractive changes showing non-uniform patterns with respect to age.

Bilateral helicoid peri-papillary sub-retinal fibrosis due to a biallelic NR2E3 mutation: Describing variable expressivity of a mutation.

BACKGROUND: To describe different clinical presentations of a same NR2E3 recessive mutation in two families and within one family. DESIGN: Interventional family study. RESULTS: Our first case was a one-year-old male child with high hyperopia and refractive accommodative esotropia. In retinal examination, peri-papillary sub-retinal fibrosis with a helicoid configuration was observed in both eyes. The parents and the only sibling had no pathologic findings in the eyes. The child showed to have severely reduced responses in both photopic and scotopic electroretinogram components. In the genetic investigation, a homozygous autosomal recessive mutation in the NR2E3 gene (IVS1-2A > C) was discovered in the affected child, while the other family members were heterozygous for this mutation. We followed up with the patient for 3 years and no new lesion developed during this period. The second case was a 13-year-old male child referred to the retina clinic for decreased vision in the right eye. In retina examination, there were nummular pigmentary changes at the level of retinal pigment epithelium and along the vascular arcades with foveo-schitic changes in both eyes. A choroidal neovascularization (CNV) was noticed in the macula of his right eye. The genetic evaluation proved the same mutation in the NR2E3 gene as in the first case. Family history was remarkable for an uncle, an aunt, and two cousins with night blindness. CONCLUSION: Same NR2E3 gene mutation can cause heterogeneous clinical manifestations such as slight retinal changes in the absence of any visual symptoms to high hyperopia associated with helicoid peri-papillary sub-retinal fibrosis.

Clinical update in nanophthalmos: Features, diseases and complications associated.

Nanophthalmos is a rare congenital condition of the eyeball that is characterised by a smaller size of the anterior and posterior segments without associated ocular malformations. Typical features that have traditionally been described in these eyes are short axial length, thickened sclera, cornea with a smaller diameter, narrow anterior chamber, and an increased lens to globe volume ratio. However, at present, there is still a lack of recognised diagnostic criteria for nanophthalmos and a classification of its severity. Its clinical relevance stems from the increased risk of multiple ocular conditions, such as high hyperopia, amblyopia, angle-closure glaucoma, retinal detachment, and cataracts. Likewise, in relation to surgery in these eyes, there are particularities in cataract and glaucoma surgery and with a greater risk of associated intra- and postoperative complications. In this way, the treatment of nanophthalmos focuses on controlling the associated eye conditions and reducing and controlling surgical complications. This review aims to update what has been published in recent years regarding nanophthalmos.

Choroidal thickness and vascular microstructure parameters in Chinese school-age children with high hyperopia using optical coherence tomography.

Background: The current study was to evaluate the choroidal thickness (CT) and vascular microstructure parameters in Chinese children with high hyperopia through enhanced depth imaging optical coherence tomography (EDI-OCT). Methods: Cross-sectional study. A total of 23 children with high hyperopia and 29 children with normal refractive status were retrospectively enrolled in the study. The measurement of the macular CT, 7 points: the sub-foveal area point, the temporal and nasal points at a radius of 0.5-mm, 1.5-mm, and 3-mm were measured. After binarization of the OCT images, the total choroidal area (TCA), stromal area (SA) as well as the luminal area (LA) were identified and measured. The choroidal vascularity index (CVI) was defined as the ratio of LA to TCA. The independent t-test for normal distributions and Kruskal-Wallis tests for non-normal distributions were used to compare other parameters between groups. The Tamhane's T2 test was performed to adjust for multiple comparisons between groups within each analysis. Results: The subfoveal CT (SFCT) in the high hypermetropic group was significantly thicker than that in normal controls (309.22 ± 53.14 µm vs. 291.27 ± 38.27 µm; P = 0.019). At 0.5 mm, 1.5 mm, and 3.0 mm in diameter, the nasal choroidal sectors of the high hyperopia eyes were significantly thicker than that of the control (P < 0.05). There was significant difference in the choroidal vascular parameters. TCA and LA in the high hyperopia eyes was significantly larger than that of the normal control eyes (3078129.54 ± 448271.18 µm2 vs. 2765218.17 ± 317827.19 µm2, 1926819.54 ± 229817.56 µm2 vs. 1748817.18 ± 191827.98 µm2; P = 0.009, P = 0.011; Table 2). SA values were 1086287.55 ± 212712.11 um2 in the high hyperopia eyes and 999712.71 ± 209838.12 µm2 in the control eyes. The CVI and LA/SA ratio values were differed significantly in the two groups (P = 0.019, P = 0.030, respectively). AL was significantly correlated with SFCT (r = -0.325, P = 0.047), but not significantly correlated with other parameters. Spherical equivalent (SE) was significantly correlated with AL and SFCT (r = -0.711, r = 0.311; P = 0.001, P = 0.016), whereas no significant association between sphere and other parameters. Conclusion: The choroidal structure of the high hyperopia eyes was different from the normal control eyes. The thicker SFCT, higher LA, and TCA were characteristic of high hyperopia eyes. Choroidal blood flow may be decreased in amblyopic eyes. SFCT of high hyperopia children abnormally increased and correlated with shorter AL and higher SE. AL and SE affect choroidal structure and vascular density.

Bacillary Layer Detachment in an Atypical Case of Central Serous Chorioretinopathy Associated with High Hyperopia.

A 16-year-old boy with elevated hyperopia presented to the office with a 24-h history of bilateral blurred vision, mainly of the left eye, and bilateral central serous chorioretinopathy. He showed a clinically recognizable bacillary layer detachment in one eye and excellent multimodal diagnostic image correlation, with the best-corrected visual acuity as 20/400. He had bilateral serous retinal detachment, as confirmed by optical coherence tomography. Laser photocoagulation was performed with good results, and reestablishment of the foveal anatomical structure was documented 16 days after treatment.

Comparison of optical coherence tomography measurements between high hyperopic and low hyperopic children.

PURPOSE: To identify the peripapillary retinal nerve fiber layer, total macular, ganglion cell layer, and inner plexiform layer thicknesses in children with high hyperopia using spectral domain optical coherence tomography. METHODS: Twenty-one children with high hyperopia and 20 controls were enrolled in this study. Subjects with spherical equivalent +5.0 D or higher were evaluated in the study group and subject with spherical equivalent between +0.25 and +2.0 D in the control group. The retinal nerve fiber layer thickness, macular thickness, macular ganglion cell layer and inner plexiform layer thicknesses were measured using a spectral domain optical coherence tomography, and results were compared between groups. RESULTS: The nasal and inferior quadrant and the global retinal nerve fiber layer thickness were significantly thicker in the study group. The mean thickness of inferior quadrant of the inner macula was significantly thicker in the study group than those in the control group. The mean thickness of the ganglion cell layer in nasal, temporal and inferior quadrant of outer macula was significantly thinner in the study group than the control group. The mean thickness of the inner plexiform layer in the inferior quadrant of the inner macula and nasal and inferior quadrant of the outer macula were significantly higher in study group than those in control group. CONCLUSION: High hyperopic children had thicker retinal nerve fiber layer when compared to the controls. This difference should be taken into account when evaluating children with glaucoma or other optic disc disorders.

Ocular findings in posterior microphthalmos.

AIM: To report a critical case series of six patients with posterior microphthalmos (PM). METHOD: Complete ophthalmologic examinations of all patients were performed using best-corrected visual acuity (BCVA), cycloplegic refraction, applanation tonometry, slit lamp biomicroscopy of the anterior segment, fundoscopy, A and B mode ultrasonography (USG), keratometry, and optic coherence tomography (OCT). RESULTS: The most significant clinical characteristics of male patients aged 10-25 years was the presence of shorter posterior segments (mean: 15.27-18.91 mm) accompanying high hyperopia (mean +9.00 - +18.50 diopter) despite the normal anterior segment findings. The BCVA ranged between 20/320 and 40/100. Retinal folds were detected bilaterally on the papillomacular band in all patients. Although neurosensory retina was included in the fold in OCT images, retinal pigment epithelium, choroid, and sclera were not included in the fold. Pigmentary retinopathy was detected in one patient. CONCLUSION: Despite normal anterior segment, posterior microphthalmos is characterized with high hyperopia, and shorter axial length and bilateral papillomacular retinal fold. Refractive amblyopia, uveal effusion syndrome, retinal detachment and macular hole are complications that can be corrected. Posterior microphthalmos must be kept in mind in patients with a normal anterior segment, and high hyperopia.

Bioptics for high hyperopia with combined multifocal intraocular lens implantation and excimer ablation in young patients.

PURPOSE: To analyze the visual and refractive outcome of the bioptics procedure combining multifocal intraocular lens implantation and excimer laser surgery in young patients with high hyperopic eyes not suitable for a single surgical procedure. METHODS: This retrospective case series included 10 eyes of five patients (age range 18-30 years) with high hyperopia (spherical equivalent +8.51 ± 0.85 diopters (D)). They had been treated with serial multifocal intraocular lens implantation followed 6 weeks later by laser in situ keratomileusis for residual hyperopia. Uncorrected distance visual acuity, uncorrected near visual acuity, corrected distance visual acuity, corrected near visual acuity, and manifest refraction were evaluated before surgeries, after multifocal intraocular lens implantation, and 3 months post laser in situ keratomileusis. RESULTS: No patients were lost to follow-up (6 months). The mean spherical equivalent decreased to +2.05 ± 1.33 D after multifocal intraocular lens implantation and to -0.10 ± 0.58 D after the laser in situ keratomileusis procedure. Success of the procedures was determined by uncorrected visual acuity. LogMAR uncorrected distance visual acuity improved by a total of more than six lines from 1.05 ± 0.18 LogMAR to 0.46 ± 0.12 LogMAR post multifocal intraocular lens implantation and to 0.15 ± 0.06 LogMAR after both surgeries. The LogMAR uncorrected near visual acuity increased by 0.81 ± 0.82 LogMAR after lens implantation due to loss of accommodation, and all eyes reached a LogMAR of 0 at 1 month postoperatively following laser in situ keratomileusis. CONCLUSIONS: A bioptics approach involving multifocal intraocular lens followed 6 weeks later by a laser in situ keratomileusis procedure for the correction of very high hyperopia enabled the resolution of the residual refractive error in young very high hyperopic patients.

A Patient with Keratoconus, Nanophthalmos, Lipodermoids, and Pigmentary Retinopathy.

A 44-year-old male with no pertinent history other than poor vision for more than 25 years was examined. Best corrected visual acuity was 20/80 OD [MR: +14.25 +1.00 × 15°] and 20/200 OS [MR: +15.00 +1.50 × 175°]. Significant limitation in ocular movements and the presence of an orbital lipodermoid in the infero-temporal aspect of each eye were noted. Forced duction test was positive for the same directions of limitation indicating possible extraocular muscle fibrosis. Ophthalmoscopy was remarkable for the presence of peripheral bony spicules. Corneal topography was compatible with keratoconus (Kmax = 55.04D OD and 52.87D OS). A-scan revealed axial lengths of 16.96 mm OD and 16.32 mm OS, compatible with a diagnosis of nanophthalmos. OCT revealed diffuse macular thickening for both eyes with foveal thickness of 350 µm OD and 353 µm OS. Over the next 12 years the patient had stable visual acuity, manifest refractions and anterior segment examination. Ophthalmoscopy revealed only minimal progression of pigmentary changes. We report the first case of these simultaneous multiple findings which may refer to a possible syndromic association of congenital or early childhood onset.

Clinical Features of Children with +4.00 Diopters or More Hyperopia Weaning with Age

PURPOSE: To evaluate the clinical features of children with high hyperopia weaning with age. METHODS: The medical records of 203 children wearing spectacles due to hyperopia of +4.00 diopters (D) or greater in at least one eye based on the cycloplegic refraction and with follow-up for 3 years or more were reviewed. The patients were divided into those who showed a decrease in the spherical equivalent (SE) of 1.50 D or greater and those who maintained. The age of wearing spectacles, the magnitude of hyperopia, the angle of deviation, the ratio of accommodative-convergence to accommodation (AC/A), and the frequency of amblyopia and anisometropia were compared. RESULTS: Forty seven patients with decreased hyperopia and 156 patients with sustained hyperopia were included. The decreased-group started to wear spectacles later than the sustained-group (5.0 ± 2.3 years vs. 4.1 ± 2.4 years). The mean SE of the hyperopic eye in the decreased-group was significantly greater at the initial visit than in the sustained-group (6.29 ± 2.18 D vs. 5.47 ± 1.38 D); was identical at the 1 year follow-up (4.83 ± 1.72 D vs. 4.89 ± 1.55 D); and significantly lower at the last follow-up (3.15 ± 1.72 D vs. 4.65 ± 1.56 D). In the decreased-group, the mean hyperopia of 3.14 ± 2.02 D decreased during a mean period of 3.9 years, especially during the first year after spectacle correction. At baseline, the frequency and angle of esotropia at both distant and near with/without hyperopic correction was significantly larger in the sustained-group. The frequency of amblyopia and anisometropia and the AC/A were identical between the two groups, while the frequency of amblyopia at the last follow-up was significantly lower in the decreased-group. CONCLUSIONS: Some patients with hyperopia of +4.00 D or greater who had none or a small angle of esotropia and improved amblyopia showed a decrease in hyperopia with age.

Long-term results of clear lens extraction combined with piggyback intraocular lens implantation to correct high hyperopia.

AIM: To assess the refractive outcome of clear lensectomy combined with piggyback intraocular lens implantation in highly hyperopic patients. METHODS: This case review included 19 eyes of 10 patients with high hyperopia and axial length less than 21mm. Intraocular lens power was calculated for emmetropia using the Holladay II formula in 17 eyes, and SRK/T formula in 2 eyes following clear lens extraction and piggyback intraocular lens implantation. Patients were examined periodically over 24 months for visual acuity and spherical equivalent (SE). RESULTS: The mean postoperative SE at 24 months was 0.20±1.39D (range, -3.00 to 2.50D), better than preoperative 9.81±2.62D (range, +6.00 to +14.50D) (P<0.001). Five eyes had SE within ±0.5D of emmetropia and 11 eyes within ±1.00D at postoperative 24 months. The mean postoperative uncorrected visual acuity (UCVA) at 24 months was 0.60±0.36, significantly improved compared to preoperative 1.39±0.33 (P<0.001). The mean best-corrected visual acuity (BCVA) at 24 months was 0.49±0.35, not statistically different compared to preoperative 0.38±0.30 (P=0.34). Twelve eyes maintained and 1 gained 1 or more Snellen line of BCVA, 4 eyes lost 1 line, and 2 eyes lost 2 lines at 24 postoperative months. Twelve eyes best-corrected near visual acuity (BCNVA) achieved J1 at postoperative 24 months compared to preoperative 7 eyes and the other 7 eyes better than J3. CONCLUSION: Clear lens extraction combined piggyback intraocular lens implantation appears to be an effective procedure to correct high hyperopia but mild overcorrection and intralenticular opacification may require secondary procedure.

Actualización clínica en nanoftalmos: características, enfermedades y complicaciones asociadas / Clinical update in nanophthalmos: Features, diseases and complications associated

El nanoftalmos es una condición congénita infrecuente del globo ocular que se caracteriza por un menor tamaño de los segmentos anterior y posterior en ausencia de una malformación ocular definida. Tradicionalmente se han descrito alteraciones anatómicas asociadas como una longitud axial corta, esclera engrosada, córnea con menor diámetro, cámara anterior estrecha y aumento de la relación entre el volumen del cristalino y el globo ocular. La causa se desconoce, aunque se han descrito múltiples mutaciones que podrían estar asociadas. En la actualidad, falta todavía una definición y una clasificación consensuada. Su relevancia clínica deriva de la asociación con múltiples afecciones oculares que pueden limitar la función visual como son hipermetropía elevada, ambliopía, glaucoma de ángulo cerrado, desprendimiento de retina y catarata. Asimismo, tanto la cirugía de catarata como la de glaucoma siguen constituyendo un desafío en estos ojos en los que el riesgo de numerosas complicaciones tanto intraoperatorias como postoperatorias es significativamente más elevado. El tratamiento del nanoftalmos tiene una doble orientación. Es preciso tratar las enfermedades asociadas y, además, reducir y controlar las complicaciones quirúrgicas. Esta revisión pretende realizar una actualización de lo publicado en los últimos años en relación con esta enfermedad. (AU)

Nanophthalmos is a rare congenital condition of the eyeball that is characterized by a smaller size of the anterior and posterior segments without associated ocular malformations. Typical features that have traditionally been described in these eyes are short axial length, thickened sclera, cornea with a smaller diameter, narrow anterior chamber, and an increased lens to globe volume ratio. However, at present, there is still a lack of recognized diagnostic criteria for nanophthalmos and a classification of its severity. Its clinical relevance stems from the increased risk of multiple ocular conditions, such as high hyperopia, amblyopia, angle-closure glaucoma, retinal detachment, and cataracts. Likewise, in relation to surgery in these eyes, there are particularities in cataract and glaucoma surgery and with a greater risk of associated intra- and postoperative complications. In this way, the treatment of nanophthalmos focuses on controlling the associated eye conditions and reducing and controlling surgical complications. This review aims to update what has been published in recent years regarding nanophthalmos. (AU)

Surgical Outcomes Dependent on the Amount of Hyperopia in Infantile Esotropia

PURPOSE: In the present study, short-term and long-term surgical outcomes dependent on the amount of hyperopia in patients with infantile esotropia were analyzed. METHODS: In this study, 80 patients with infantile esotropia who underwent both medial rectus recession from 2007 to 2011 and followed up for at least 36 months were retrospectively examined. The patients were divided into two groups according to the degree of hyperopia: ≥ +3.0 D (high hyperopia [HH], n = 59 patients) and < +3.0 D (non-high hyperopia [NH], n = 21 patients). Clinical characteristics analyzed included surgical success rate and dose-response relationship at the 3-month and 3-year postoperative follow-ups. RESULTS: Initial preoperative alignment (NH: 44.8 ± 10.2 PD, HH: 42.7 ± 11.6 PD, p = 0.450), surgical success rates (NH: 69.5% (41/59), HH: 71.4% (15/21), p = 0.837), under-correction rates (NH: 23.7% (14/59), HH: 9.5% (2/21), p = 0.191) and over-correction rates (NH: 6.8% (4/59), HH: 19.1% (4/21), p = 0.138) were not statistically significantly different between the NH and HH groups. A tendency towards a larger dose-response relationship was observed with HH (NH: 3.9 PD/mm, HH: 4.3 PD/mm) at the 3-month postoperative follow-up, but was not significant (p = 0.105). At the 3-year postoperative follow-up, exodrift was in progress and the dose-response relationship was significantly higher in the HH group than NH group (NH: 3.9 PD/mm, HH: 4.9 PD/mm, p = 0.010). A difference between the groups with amblyopia was observed (NH: 8.5% (5/59), HH: 23.8% (5/21), p = 0.146), although without statistical significance. CONCLUSIONS: The surgical success rate of infantile esotropia was not statistically associated with the amount of hyperopia. There was no statistical association between the dose-response relationship and amount of hyperopia at the postoperative 3-month follow-up, but a statistical association was found in the high dose-response relationship in the HH group at the postoperative 3-year follow-up. Therefore, the conventional amount of recession or muscle resection should be modified in high hyperopic (≥ +3.0 D) infantile esotropia, and long-term postoperative follow-up is necessary.

Long-term Observations on the Emmetropization of the High Hyperopia

PURPOSE: We are to investigate the reduction rate of high hyperopia, that is, process of emmetropization. METHODS: Thirty children who had hyperopia more than spherical equivalent of+4.0 diopter and could be followed at least 3 years were reviewed retrospectively. Data including visual acuity and the magnitude of hyperopia were collected every 6 months from 3 years of age. Annual reduction rates of hyperopia were analysed. Changes in the level of hyperopia were compared with those who had strabismus or astigmatism and those who did not. RESULTS: The children who had higher degree of initial hyperopia showed the faster rates of annual reduction but also significantly higher level of remained hyperopia (p=0.003). The concurrent presence of astigmatism was associated with a lesser reduction in hyperopia (p=0.01). CONCLUSIONS: Our data show that, as a whole, the emmetropization of high hyperopia occurs as a linear function of the initial level and converges toward a low hyperopic value. We have also found that astigmatism might impede emmetropization.