DRI Triton SS-OCT applied to detect choroidal nodules in paediatric patients affected by NF1.

Purpose: To examine whether image processing of non-mydriatic DRI Triton SS-OCT (Topcon Corporation, Tokyo, Japan) using the red free filter could assess the presence of choroidal nodules and thus include their detection as a diagnostic criterion in neurofibromatosis type 1 (NF1). Material and methods: We included 417 eyes from 210 patients, 377 - from 190 patients diagnosed with NF1 according to the criteria established by the National Institutes of Health Consensus Development Conference (NIH) and 40 from 20 healthy patients as a control group. The mean age was 9.4 years (range 2 years-18 years). All patients had their visual acuity measured by a test according to age, were examined for the presence of lisch nodules and an Optical Coherence Tomography (OCT) of the macular area was performed. All the OCT images were analysed to check if visible nodules could be identified. Results: Ages 14 (95% CI=(9.7,18.3)) and 12 years (95% CI=(9.1,14)) are the cut-off points that best separate those with choroidal nodules with Triton OCT and lisch with slit lamp, respectively, from those without. lisch nodules were detected in 50% of cases of NF1 patients. The presence of choroidal nodules did not present a statistically significant correlation with the occurrence of optic pathway glioma (p = 0.96) nor with the patient's visual worsening (p = 0.072). A statistically significant correlation was observed between the presence of choroidal nodules and the presence of lisch nodules (p < 0.05). Conclusion: The Topcon Triton OCT red free tool would not be a good tool to detect choroidal nodules in patients with NF1 because of its low sensitivity. If the presence of choroidal nodules were to be included in the diagnostic criteria for NF1, it would be convenient to use a device with red and infrared radiations.

An Unusual Case of Lacrimal Gland Dacryops in a 10-Year-Old Child.

Lacrimal gland dacryops are an unusual type of benign epithelial neoplasm. Most of the literature refers to dacryops of the lacrimal palpebral lobe, with orbital lobe involvement less frequent. The authors report a case of dacryops in a 10-year-old child involving the orbital lobe of the lacrimal gland. [J Pediatr Ophthalmol Strabismus. 2023;60(6):e79-e82.].

Dermoid Cyst: Outcome Analysis in a Pediatric Referral Hospital.

PURPOSE: To describe the management of dermoid cysts in a pediatric referral hospital. METHODS: Retrospective review of 115 patients with pathological diagnosis of dermoid cyst in a pediatric referral hospital between 2003 and 2019. RESULTS: One hundred fifteen patients, 51 (44.3%) males, and 64 (55.7%) females were retrospectively reviewed. There were fifty-one (44.3%) right eyes and 64 (55.7%) left eyes. Mean age at surgery was 39.15 months (5.6 months-16.4 years). One hundred four (90.4%) lesions were superficial, and 11 (9.6%) were deep. Quadrant location was 63 (54.8%) superior-temporal, 45 (39.1%) superior-nasal, 4 (3.5%) inferior-temporal, and 2 (1.7%) in the nasal inferior quadrant. Most patients only had aesthetic concerns, but 3 (2.61%) showed lacrimal drainage obstruction symptoms, 2 (1.74%) had proptosis, and 1 case spontaneously drained to the cutaneous surface. Imaging was performed in 51 (44.3%) patients. Regarding to their radiodensity, 71.9% had low density content, 28.1% high density, and only 1 patient showed full liquid content; 10.9% showed heterogeneous content; 53.1% showed bone remodeling. Every patient but 1 underwent surgery for a barely accessible asymptomatic retrobulbar cyst. Nine cysts (7.8%) were breached during surgery. Three recurrences were found (2.6%), but only 1 was related to intraoperative breach. CONCLUSIONS: Dermoid cysts are the most common benign periorbital tumors in the pediatric population. Imaging is required for evaluation of lesions in atypical locations, deep or fixed to underlying tissue. Surgical removal is the gold standard of treatment. Multidisciplinary approach may be required in the most complex cases. After surgery, few complications and recurrences were found in our series.

Anterior iris-claw intraocular lens implantation for the management of nontraumatic ectopia lentis: long-term outcomes in a paediatric cohort.

PURPOSE: To report the feasibility and long-term safety of lensectomy and iris-claw intraocular lens (IOL) implantation to treat children with severe ectopia lentis in a paediatric tertiary hospital. METHODS: Prospective cohort study of 21 eyes from 12 patients with severe ectopia lentis and visual acuity <20/63. All eyes underwent 23-gauge pars plana vitrectomy, lensectomy, iridectomy and Artisan IOL implantation in the anterior chamber with iris-claw enclavation via pars plana. Mean age at surgery was 8.0 ± 5.3 yo (range 3-17 years). A full ophthalmologic examination including best-corrected visual acuity (BCVA), biomicroscopy, intraocular pressure (IOP) measurement, fundus evaluation and central endothelial cell count (cECC) was performed pretreatment, at 3 months' postsurgery, and every 6 months thereafter. Ultrasound biomicroscopy (UBM) was performed 12 months after surgery. RESULTS: Mean follow-up was 39.3 ± 13.0 months. Best-corrected visual acuity (BCVA) (mean ± SD) improved from 0.91 ± 0.29 logMar preoperatively to 0.18 ± 0.23 logMar at final follow-up (p < 0.0001). Mean distance from the endothelium to the anterior IOL surface after surgery was 3.11 ± 0.61 mm. Postsurgically, cECC loss was 5.04% ± 9.58% with an annual cECC loss rate of 3.16% ± 4.46%. One patient developed IOL dislocation and retinal detachment after severe ocular contusion requiring vitrectomy, IOL refixation and gas tamponade. Another patient developed cystoid macular oedema, managed with intravitreal dexamethasone. CONCLUSION: This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.