Incidence and predisposing factors of intraocular Lens tilt following secondary ciliary sulcus implantation in children: An ultrasound biomicroscopic study.

PURPOSE: To evaluate the incidence and causes of intraocular lens (IOL) tilt and changes in anterior chamber angle after secondary IOL sulcus implantation following congenital cataract removal. METHODS: A retrospective observational study was conducted on children who underwent secondary sulcus IOL implantation following pediatric cataract removal in the period from 2017-2020 in Cairo university Hospitals. Children were examined for IOL position, centration, and tilt. Intraocular pressure (IOP) measurement, fundus and gonioscopic examination was performed. Ultrasound biomicroscopy (UBM) was performed on both eyes in children with clinically detected tilt. RESULTS: Ciliary sulcus secondary IOL implantation was performed in 102 eyes (57 children). IOL tilt was detected clinically in 16 eyes of 14 children (15.7%). UBM showed clinically undetected tilt in the fellow eye in additional 4 eyes. The mean angle of tilt was 12.8 ± 3.9° in clinically detected tilt compared to 7.5 ± 1.2° in UBM detected tilt. Mean anterior chamber depth (ACD) was 2.4 ± 0.5 mm IOP was >21 mmHg in 1.9% of eyes. Narrowing of the anterior chamber angle (ACA) after sulcus implantation occurred in 40% of eyes with open angle. Sulcus proliferations and obliterated sulcus were detected in all 20 eyes. Sommering's ring was found in 7 eyes (35%). Axial length, corneal diameter, and presence of persistent fetal vasculature did not affect IOL position. CONCLUSION: The presence of residual lens matter or an obliterated ciliary sulcus is associated with a higher incidence of IOL malposition following ciliary sulcus implantation.

Anterior chamber angle features in primary congenital glaucoma infants using hand-held anterior segment-oct.

PURPOSE: To describe anterior chamber angle (ACA) structures and parameters in primary congenital glaucoma (PCG) and normal infant eyes, using Hand-held anterior segment optical coherence tomography (HH AS-OCT), as an in-office, non-contact technique. METHODS: Normal and PCG-infants <24 months were examined, using HH AS-OCT (RTVue RT- 100, Optovue Inc., Fremont, CA). Sedation was not required. Corneal pachymetry map, ACA width and iris thickness (IT) were measured. Trabecular meshwork (TM), Schlemm's canal (SC), and scleral spur (SS) identification were assessed in both groups. RESULTS: Forty-eight infants; (26 PCG-eyes and 22 normal-eyes) aged 9.12 ± 6.7 months, were included. Nasal and temporal ACA width in PCG infants was found significantly larger (39.3 ± 6.6° vs. 30.4 ± 5.6, and 40.1 ± 5.3° vs. 32.5 ± 6.2 respectively) (p < 0.001). IT was significantly reduced (121.7 ± 43.9 µm in PCG-infants, vs. 160.3 ± 38.6 µm in normal-eyes) (p < 0.01). TM was identified in all normal eyes (100%) and nine (34.6%) PCG- eyes. SC was identified in 16 (72.7%) normal eyes versus four (15.4%) PCG. In PCG-eyes, an abnormal structure occluding the angle was seen in seven (26.9%), and a hyper-reflective membrane in five (19.2%), the iris was anteriorly inserted in all PCG-eyes, and iridotrabeculodysgenesis was clearly identified (with constant iris anterior insertion). The abnormal tissue obscuring the angle was seen in younger PCG-infants and iris thinning appeared to be part of the pathology, not a result of IOP elevation. CONCLUSION: Using HH AS-OCT permits tomographic examination of the ACA in PCG infants and may help in the understanding of disease pathology. Hence, may assist in optimizing treatment.

Serum cortisol and adrenocorticotrophic hormone (ACTH) in infants receiving topical and subconjunctival corticosteroids following cataract surgery.

PURPOSE: Cushingoid features are occasionally encountered in infants after pediatric cataract surgery. The aim of this study is to evaluate whether the use of topical glucocorticoids (GCs) following congenital cataract surgery can result in endogenous adrenal suppression and/or systemic side effects similar to those seen with systemic steroids. METHODS: A prospective study was performed on 20 infants with bilateral congenital cataract. All infants received a single subconjunctival betamethasone injection of 1 mg at the end of surgery in addition to topical dexamethasone eye drops 1 mg/ml for 6 weeks. All infants had anthropometric measurements and blood pressure measurements, serum cortisol, and ACTH level measurements before surgery and 2 months after. In addition, the total administered glucocorticoid adjusted per weight was calculated. RESULTS: The mean age of the infants was 4.93 ± 2.58 months. Thirteen were males (65%). The total administered glucocorticoid dose was 18.7 mg and the mean cumulative dexamethasone equivalent dose administered was 2.75 ± 1.31 mg/kg. There was a statistically significant increase in the adjusted weight percentile for age (P = 0.009). Both the systolic and diastolic blood pressure were significantly elevated (P = 0.005 and P = 0.025 respectively). There was a statistically significant reduction in both the morning and afternoon serum ACTH levels (P = 0.023 and P = 0.014). The reduction in serum cortisol levels was statistically non-significant. CONCLUSIONS: Topical steroids following pediatric cataract surgery can result in both subclinical and clinical changes in the hypothalamic-pituitary-adrenal axis that can be easily overlooked and need careful attention and follow-up.

Management strategy and novel ophthalmological findings in neonatal severe hypertriglyceridemia: a case report and literature review.

BACKGROUND: Neonatal severe hypertriglyceridemia is rarely reported in the literature and there is no consensus for hypertriglyceridemia management at this age group. METHODS: The index case is a 4-week-old male infant with severe hypertriglyceridemia accidentally discovered during a circumcision surgery. His clinical and genetic characteristics and his successful management strategy are described. Furthermore, a detailed ophthalmological examination of the proband was conducted at 3 and 6 months of age using Fourier-domain-optical coherence tomography. RESULTS: Triglycerides level at presentation was extremely high 33,727 mg/dL (380.8 mmol/L). Two sessions of exchange blood transfusion on two consecutive days successfully reduced triglycerides to 382 mg/dL (4.3 mmol/L) with no adverse effects. The infant was discharged 3 days later. At discharge, the mother was advised to continue breastfeeding together with a medium-chain triglycerides formula. Satisfactory growth parameters and lipid profile values were obtained for a follow-up duration of 5 months with no reported attacks of acute pancreatitis. Lipoprotein lipase deficiency was confirmed by the detection of the LPL homozygous pathogenic variant c.805G > A; p.(Glu269Lys). Early corneal and macular lesions were detected and persisted on follow-up despite relatively good lipemic control. CONCLUSION: This case highlights the importance of the early discovery of severe hypertriglyceridemia during the neonatal period, which is needed for prompt management and prevention of severe complications. Rationalized breastfeeding can be tolerated within the diet plan of the disease with satisfactory outcomes. To our knowledge, it is the first study reporting early corneal and macular affection by severe hypertriglyceridemia in a neonate. Prolonged follow-up is needed to determine the extent of ophthalmological lesions.

Changes in Intraocular Pressure and Anterior Chamber Angle After Congenital Cataract Extraction.

PRECIS: Anterior chamber angle (ACA) narrowing continues to occur for at least 2 years after congenital cataract surgery. Risk factors for intraocular pressure (IOP) elevation after congenital cataract surgery were higher central corneal thickness (CCT) and surgery at <2 months. PURPOSE: The purpose of this study was to study the changes in IOP and in the ACA during the first 2 years after pediatric cataract surgery and to determine risk factors for such changes. PATIENTS AND METHODS: A retrospective observational study was done on infants who underwent pediatric cataract surgery in Cairo University Hospitals and completed a 1-year follow-up. Demographic and clinical characteristics were recorded including age at surgery, sex, corneal diameter, CCT pupil diameter, IOP, gonioscopic findings, presence of persistent hyperplastic primary vitreous, surgical approach, primary intraocular lens implantation, and perioperative subconjunctival steroid injection. Changes in IOP and in the ACA were recorded, and the risk factors for such changes were analyzed. RESULTS: Postoperative IOP elevation >18 mm Hg occurred in 23 eyes of 206 eyes (11%), who completed Year 1 and in 9 (13%) of 86 eyes who completed Year 2. Risk factors for IOP elevation were larger preoperative CCT (P=0.01) in Year 1, and younger age at surgery (P=0.01), and aphakia (P=0.05) in Year 2. In multivariate analysis only younger age at surgery was a risk factor for IOP elevation in Year 2. ACA narrowing occurred in 49% and in 21% of the examined eyes in Years 1 and 2, respectively. Aphakia was not a significant risk factor of angle narrowing in Years 1 and 2 (P=0.17 and 0.42, respectively). CONCLUSIONS: Higher preoperative CCT was a risk factor for early-onset IOP elevation. Surgery at >2 months was associated with lower susceptibility to late-onset IOP elevation.

Horizontal Extraocular Muscle Insertion Site in Relation to Axial Length Using Swept-Source Anterior Segment OCT.

PURPOSE: The purpose of this study was to correlate between the axial length of the globe and the insertion site of horizontal extraocular muscles using swept-source anterior segment optical coherence tomography (SS-ASOCT), with posing an equation to calculate the muscle insertion site from the axial length. METHODS: The study design was observational and cross-sectional. It was performed on 157 eyes of 157 healthy subjects. The distance of the medial rectus (MR) and the lateral rectus (LR) insertion sites from the limbus were measured using SS-ASOCT. The insertion sites' distances were correlated to the axial length (hypermetropes < 22.5 mm, myopes > 24.5). Correlation between numerical variables was done by Pearson's correlation coefficient and confirmed by linear regression analysis and scatter diagrams. RESULTS: The mean MR insertion site was 5.47 ± 0.19 mm in hypermetropes versus 5.68 ± 0.23 mm in myopes, whereas the mean LR insertion site was 6.81± 0.23 mm in hyperopes versus 7.08 ± 0.16 mm in myopes. The axial length showed a moderate positive, but significant, correlation to the insertional position for the medial and lateral rectus muscles (MR: r=0.417, p<0.001; LR: r=0.410, p<0.001). CONCLUSION: Comparing the horizontal extraocular muscle insertion site to axial length using SS-ASOCT showed a significant positive correlation. The model equation for MR insertion: MR (mm) = 4.522 + 0.045 (AXL in mm) with an R = 0.437, R2= 0.191, F=12.071, P<0.001. The model equation for LR insertion: LR (mm) = 5.72 + 0.048 (AXL in mm) with an R = 0.438, R2= 0.192, F=12.116, P<0.001.

Torsional Changes After Vertical Transposition of Horizontal Recti in V-pattern Exotropia Without Oblique Dysfunction.

PURPOSE: To evaluate pattern collapse and torsional changes following vertical transposition of horizontal recti in patients with V-pattern exotropia and no oblique dysfunction. METHODS: A prospective study was performed on patients who had V-pattern exotropia and no oblique dysfunction. Lateral recti were transposed upward half-tendon width in V-pattern of 25 prism diopters (PD) or less (n = 14) and full-tendon width in V-pattern of greater than 25 PD (n = 10). Amblyopic patients had unilateral lateral rectus recession with upward transposition and medial rectus resection with downward transposition (n = 8). Ductions, versions, pattern strabismus, disc foveal angle, and astigmatic axis were analyzed before and 6 months after surgery. RESULTS: In the 32 patients (21 females), the mean age was 8.25 ± 1.23 years. Only amblyopic patients showed preoperative fundus extorsion (mean disc foveal angle = 16.9°, P < .01). Mean pattern collapse was 13.1 ± 3.8 PD with half-tendon transposition, 35.6 ± 13.7 PD with full-tendon transposition, and 13.8 ± 7.9 PD in the unilateral group. Pattern collapse increased gradually so that pattern normalization occurred after 6 months in most patients. There was a statistically significant correlation between the preoperative V-pattern and the magnitude of pattern collapse after surgery (r = 0.80, P < .01). There were no significant changes in the mean disc foveal angle (< 0.5°) or axis of astigmatism (< 0.5°) in all three groups. CONCLUSIONS: Vertical transposition of horizontal muscles can correct V-pattern exotropia, although the effect might not be immediate. Torsional changes are minimal. Amblyopic patients with V-pattern exotropia showed a preoperative extorsion that did not change after surgery. [J Pediatr Ophthalmol Strabismus. 2019;56(2):107-115.].

Ocular Manifestations in Children with Juvenile-Onset Systemic Lupus Erythematosus.

PURPOSE: To study the ocular manifestations of juvenile systemic lupus erythematosus (JSLE), including the ocular side-effects of the systemic medications used. METHODS: A descriptive cross-sectional study on 40 children diagnosed with JSLE was conducted. Ophthalmological and laboratory investigations as well as a calculation of the Systemic Lupus Disease Activity Index 2000 (SLEDAI-2K) were performed. RESULTS: Forty consecutive children, 32 females and 8 males, with JSLE were examined. Their mean age was 13±2.8 years and the mean SLEDAI-2K was 4.3±3.1. An abnormal Schirmer test was found in 16 patients (40%), retinal vascular changes were found in seven patients (17.5%), and one patient (2.5%) had faint posterior subcapsular cataract. CONCLUSION: Serious sight-threatening complications were not detected in our study; dry eye was the most common ocular finding, and the detected retinopathy was related to systemic hypertension and could not be correlated to either disease activity or duration.

Assessment of Corneal and Tear Film Parameters in Rheumatoid Arthritis Patients Using Anterior Segment Spectral Domain Optical Coherence Tomography.

PURPOSE: To study the corneal changes in rheumatoid arthritis (RA) patients in vivo, using spectral domain anterior segment optical coherence tomography (AS-OCT). METHODS: A case-control study was done on 43 RA patients and 40 controls. The disease activity score (DAS28-ESR) was calculated and all participants had lower tear meniscus, corneal thickness, and epithelial thickness evaluation using AS-OCT. RESULTS: The lower tear meniscus height (LTMH) and the lower tear meniscus area (LTMA) were significantly lower in the RA patients than in controls (p < 0.001). RA patients also had a significantly thinner central corneal thickness (p = 0.02) and their epithelium was found to be thinner in the superotemporal peripheral sector. CONCLUSIONS: The LTMH and LTMA are significantly reduced in RA patients, despite the absence of clinical diagnosis of dry eye. RA patients have thinner corneal thickness and epithelial thickness than controls, which did not correlate with either disease duration or activity.

Progression of High Anisometropia in Children.

PURPOSE: To investigate the onset and rate of progression of high anisometropia in myopic children younger than 13 years. METHODS: A retrospective study was performed on children with anisometropia younger than 13 years with myopia of more than 4.00 diopters (D) in the more ametropic eye and a difference in spherical equivalent refraction of 4.00 D between both eyes. All children had a complete ophthalmologic examination, including measurement of visual acuity and cycloplegic refraction every 3 to 6 months for at least 5 years. Change in the spherical equivalent and the cylindrical error for both eyes and changes in the difference in spherical equivalent refraction between both eyes were calculated for each patient at each visit. Linear, polynomial, logarithmic, and exponential fitting models were tested for both eyes and for the anisometropic difference between both eyes. The regression line with the greatest R2 value was considered best fit. RESULTS: Sixty-three patients fulfilled the inclusion criteria. The more ametropic eye grew in a regular fashion during the first 2 years of life, followed by a rapid decrease in the rate of growth to become almost stable after 4 years of age. The increase in myopia best fit a third-degree polynomial (cubic) model (R2 = 0.98). The less ametropic eye showed only a small increase in myopia during the follow-up period. The anisometropic difference between both eyes increased gradually during the first 2 years, then remained stable. CONCLUSIONS: High anisometropic myopia progresses rapidly in the first few years of life before becoming stable. [J Pediatr Ophthalmol Strabismus. 2017;54(5):282-286.].

Value of Microperimetry in Detecting Early Retinal Toxicity of Hydroxychloroquine in Children with Juvenile Systemic Lupus Erythematosus.

PURPOSE: To evaluate retinal sensitivity in children who are on hydroxychloroquine (HCQ) for systemic lupus erythematosus using microperimetry and compare the results with those of the Humphrey visual field (HVF) 10-2 and spectral-domain optical coherence tomography (SD-OCT). PROCEDURE: A case-control cross-sectional study including 19 patients (less than 18 years old) on HCQ for at least 5 years. Controls were 21 normal children. Participants underwent a complete ophthalmic examination, then were investigated using HVF 10-2, SD-OCT, and microperimetry. RESULTS: Ocular examination revealed no abnormalities. The overall mean microperimetry sensitivity of the patients (15.75 dB) was not significantly different from that of the controls (16.35 dB). The HVF 10-2 showed a significant difference in the mean deviation of the patients. Conclusions and Message: Microperimetry was not more revealing than HVF 10-2 and SD-OCT. Larger studies are required to compare the diagnostic accuracy of screening modalities of retinal toxicity in children on HCQ.

Fluorescein Angiographic Findings in Patients with Active Systemic Lupus Erythematosus.

PURPOSE: To evaluate the retina of patients with active systemic lupus erythematosus (SLE) using fundus fluorescein angiography (FFA), irrespective of their visual acuity or fundus examination. METHODS: A descriptive study was performed on 30 patients with active SLE; disease activity was calculated using The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Fundus examination and FFA angiography were done to all patients. RESULTS: A total of 60 eyes of 30 patients were included. Their mean age was 32.6 ± 1.17 years. All patients showed disease activity at time of examination according to SLEDAI. Retinopathy was detected by FAF in 24 eyes (40%), 10 eyes of which had normal fundus examination. SLEDAI was positively correlated to the presence of retinopathy. CONCLUSIONS: All patients with ocular lupus should be carefully evaluated for systemic involvement and, vice versa, all patients diagnosed with SLE should have a thorough ocular examination and FFA, even if they had normal fundus.

Retinal Nerve Fiber Layer and Ganglion Cell Complex Thicknesses Are Reduced in Children With Type 1 Diabetes With No Evidence of Vascular Retinopathy.

PURPOSE: To determine whether type 1 diabetes (T1DM) in children with a mean age of 12.21 ± 3.04 years affects the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) when compared to age- and sex-matched healthy children. METHODS: Forty-six children with T1DM with no diabetic retinopathy (DR) and 50 normal age- and sex-matched controls underwent full clinical ophthalmic and spectral-domain optical coherence tomography (SD-OCT) examination. Using RTVue Fourier-Domain OCT (version 6.11.0.12) average, superior, and inferior RNFL and GCC thicknesses (in µm) were measured. Mean values of patients and the control group were compared. RESULTS: In children with T1DM with no DR, the mean average RNFL thickness was 110.9 µm ± 10.46, and the mean GCC thickness was 95.59 µm ± 5.13; both were significantly thinner than the control group (115.62 µm and 99.30 µm, respectively). The retinal nerve fiber layer and GCC thickness showed no correlation to either age of onset, duration of the disease, or glycosylated hemoglobin (HbA1c). A positive correlation was found between the daily insulin dose and the average RNFL thickness (r = 0.378, P = 0.01). The average GCC in children with dyslipidemia was thinner than those with normal lipid profile (91.29 ± 6.46 µm, 97.11 ± 3.59 µm, respectively) with a P value of 0.011. CONCLUSIONS: Thinning of the RNFL and GCC in children with T1DM with no DR compared to healthy controls suggests that neurodegenerative changes occur in the absence of vascular changes. It also shows that neurodegeneration is not related to either disease duration, onset, or control.

A New Ultrasound Biomicroscopic Sign seen after Deep Sclerectomy (Dolphin Head Sign).

PURPOSE: To describe a new ultrasound biomicroscopic (UBM) sign seen in patients who underwent deep sclerectomy (DS) as a surgical procedure for the management of uncontrolled primary open-angle glaucoma (POAG). The presence of this sign in ultrasound biomicroscopy is suggested to be an indicator of successful surgery. We would like to name this sign as the "dolphin head sign." DESIGN: Prospective interventional study. MATERIALS AND METHODS: Twenty-eight eyes of 17 patients with POAG underwent DS with intraoperative mitomycin C (MMC) 0.3% applied for 2 minutes under the superficial scleral flap. Patients were followed up for a minimum of 6 months. Ultrasound biomicroscopy was done at the third postoperative month to evaluate the surgical area in both successful and failed cases. RESULTS: The study included 28 eyes of 17 patients. The mean age of the study group was 42.90 ± 14.37 years (20-64 years). The study included 10 females and 7 males. The mean preoperative intraocular pressure (IOP) was 24.57 ± 6.37 mm Hg (20-38 mm Hg). The mean best corrected visual acuity (BCVA) was 0.57 ± 0.3 (0.05-1.00). Complete success has been achieved in 21 eyes (75%) during the follow-up period, with a mean IOP of 12.00 ± 3.86 mm Hg (6-20 mm Hg). The dolphin head sign was demonstrated only in successful cases, whereas the unsuccessful cases failed to show the typical sign. CONCLUSION: The presence of a "dolphin head" configuration in UBM images could be taken as an indicator of successful DS. How to cite this article: Abdelrahman AM, El Cheweikh HM, El-Fayoumi DMS, Allam RSHM. A New Ultrasound Biomicroscopic Sign seen after Deep Sclerectomy (Dolphin Head Sign). J Curr Glaucoma Pract 2016;10(2):56-59.

Changes in the axis of astigmatism and in fundus torsion following inferior oblique muscle weakening.

PURPOSE: To study the changes in fundus torsion and in the axis of astigmatism following inferior oblique (IO) myectomy in patients with inferior oblique overaction (IOOA). METHODS: The degree of fundus torsion and corneal astigmatism were prospectively evaluated before and 3 months after IO myectomy in patients with IOOA grade +2 or more in one or both eyes and an astigmatic error of ≥1 D. Fundus torsion was evaluated by measuring the disk foveal angle (DFA) using fundus photography. The axis of astigmatism was identified from the anterior sagittal map using Pentacam corneal imaging. RESULTS: A total of 54 eyes of 27 patients were included. Patients were divided into two groups: those with esotropia and those with exotropia. All patients had a preoperative DFA >8°, which decreased postoperatively in both groups (P < 0.01). Postoperatively, there was incyclorotation of the axis of astigmatism by >5° in 80% of the esotropic group and 75% of the exotropic groups (P < 0.01). CONCLUSIONS: Incyclorotation of the axis of astigmatism occurs after IO myectomy. Measurement of the change in the axis of astigmatism can be used to assess the torsional changes after IO myectomy in patients with IOOA.

Surgical management of monocular elevation deficiency combined with inferior rectus restriction.

PURPOSE: To evaluate the results of ipsilateral inferior rectus (IR) recession, when performed alone or in combination with contralateral superior rectus (SR) recession in patients with monocular elevation deficiency (MED) and IR tightness. METHODS: The medical records of patients with MED and tight IR muscle were retrospectively reviewed. Patients underwent either ipsilateral IR recession alone (IR group) or combined with contralateral SR recession (IR+SR group). Pre- and postoperative ocular motility and alignment and fundus torsion were analyzed. Abnormal head posture was recorded. All patients were followed for at least 6 months. RESULTS: A total of 23 patients were identified. Mean patient age at time of surgery was 9.8 ± 8.7 years (range, 3-45 years). Ten patients underwnet ipsilateral IR recession 5-8 mm; 13 patients, 4 mm ipsilateral IR recession combined with 5-12 mm contralateral SR recession. Both groups experienced marked improvement in ocular alignment in the primary position (P = 0.34). However, there was a statistically better ocular alignment in both upgaze (P = 0.03) and downgaze (P < 0.01) in the IR+SR group, with a lower degree of fundus intorsion (P < 0.01). An ipsilateral head tilt developed in 70% of patients in the IR group and in 54% of patients in the IR+SR group. CONCLUSIONS: In this patient cohort, combined contralateral SR recession with ipsilateral IR recession reduced postoperative IR underaction and achieved better ocular alignment in upgaze and downgaze.