Childhood glaucoma profile in a tertiary centre in Egypt according to the childhood glaucoma research network classification.

PURPOSE: To describe the prevalence and clinical characteristics of a large cohort of childhood glaucoma patients that presented to a tertiary Egyptian children's hospital using the childhood glaucoma research network (CGRN) classification. METHODS: A retrospective review of the medical records of all patients ≤ 14 years with a diagnosis of childhood glaucoma or glaucoma suspects who presented to Children's Hospital between January 2014 to December 2019 was conducted. Data collected included age at the time of diagnosis, gender, laterality, prenatal history, parental history, including consanguinity, intraocular pressure, horizontal corneal diameter, and cup-to-disc ratio. RESULTS: A total of 1113 eyes of 652 patients with diagnoses of either childhood glaucoma or glaucoma suspects were included in the study. Six hundred and sixteen patients (94%) were born full-term. A history of positive parental consanguinity was identified in 334 patients (51.2%). Almost 60% of patients were males. Primary congenital glaucoma (PCG) was the most prevalent diagnosis (68.2%), followed by glaucoma suspects (10.4%) and glaucoma following cataract surgery (GFCS) (8.4%). Juvenile open-angle glaucoma was the least prevalent category (0.3%). Other categories including glaucoma associated with non-acquired systemic disease, glaucoma associated with non-acquired ocular disease, and glaucoma associated with acquired conditions represented 5.8%, 4.7%, and 1.9%, respectively. CONCLUSIONS: PCG is the most common form of glaucoma in Egypt. More than half of the pediatric glaucoma patients had a positive history of parents' consanguinity.

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.

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.

Outcomes and Complications Rate of Resident versus Attending Performed Eye Muscle Surgeries.

Purpose: to compare the type, complications rate, and success rate of eye muscle surgeries performed by residents to those performed by attending consultants. Methods: A retrospective review was done on the charts of all children who underwent an eye muscle surgery in Cairo University Hospital during the period from January 2015 to December 2017. Preoperative data including age, sex, and type of deviation were tabulated. Details of the operative procedure including the surgeon, the type of eye muscle surgery, the number of muscles operated upon, perioperative complications, and the final ocular alignment at the end of the third month after surgery were recorded. Results: A total of 319 patients were included; 143 patients (315 muscles) in the supervised resident group and 176 patients (387 muscles) in the attending group. More vertical and oblique muscles surgeries were done by the attending consultants than by residents. Surgical success after three months was higher in the attending group (n = 126, 72%) than the resident group (n = 91, 64%). However, the difference was not statistically significant (P = .129). The perioperative complications rate was significantly higher (P = .004) in the resident group (14%) than the attending group (6%). Accidental scleral perforation was the most commonly encountered complication in the resident group (n = 6, 1.9%) followed by extruded/exposed Tenon (n = 5, 1.6%), and muscle slippage (n = 4, 1.3%). Conclusions: Success rate is similar in eye muscle surgeries performed by residents and attending consultants. However, perioperative complications are still more common among residents.

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.