Multimodal Imaging of Choroidal Nodules in Neurofibromatosis Type I.

AIM: To clarify the possibilities and role of posterior segment imaging in patients with neurofibromatosis type I (NF1), and to show the prevalence of this disease in the pediatric population in Slovakia. MATERIAL AND METHODS: Until recently, ophthalmologic consultations in patients with NF1 were limited mainly to the observation of Lisch nodules of the iris and the presence of optic nerve glioma. However, advances in imaging capabilities have made it possible to investigate and describe new f indings concerning the ocular manifestations of this disease. Between October 2020 and November 2021, we examined the anterior and posterior segment of 76 eyes (38 children ­ 12 boys and 26 girls) with genetically confirmed NF1 gene mutation at our clinic. The age of the patients ranged from 4 to 18 years. The anterior segment was checked for the presence of Lisch nodules biomicroscopically with a slit lamp. On the posterior segment, the presence of choroidal nodules was checked by various imaging methods ­ fundus camera, infrared confocal selective laser ophthalmoscopy, MultiColor imaging, OCT, and OCT angiography. All the patients had magnetic resonance imaging performed in order to detect potential optic nerve gliomas for the purpose of diagnosis. We observed the correlation between the patients' age, presence of Lisch nodules and the presence of choroidal nodules. Eight patients also had other manifestations of the disease ­ optic nerve gliomas or microvascular changes (so-called "corkscrew" vessels). RESULTS: Out of 38 patients, Lisch iris nodules were present in 20 patients (53%) and choroidal nodules in 24 patients (63%). There was no positive correlation between the presence of these two manifestations within the same patient or eye, but there is a clear correlation between the presence of choroidal nodules and patient age. CONCLUSION: The results suggest that a previously unknown ocular manifestation of neurofibromatosis type I, namely choroidal nodules, has a higher prevalence than Lisch nodules also in the pediatric population and can be easily visualized using various imaging modalities. It will be important to include follow-up observation of this finding among the standard controls for ocular findings in NF1, and it will be very interesting to correlate this f inding with the exact NF1 mutation

Anterior Chamber Iris-Fixated Phakic Intraocular Lens Implantation for Treatment of High Anisometropia in Children: Long-term Results.

PURPOSE: To evaluate the long-term efficacy of anterior chamber iris-fixated phakic intraocular lens (PIOL) implantation in the treatment of high myopic (> -9.00 diopters [D]) or hyperopic (> +6.00 D) anisometropia in children with intolerance to spectacles or contact lenses. METHODS: A total of 58 children with anisometropic myopia (range: -9.25 to -18.50 diopters [D]) and 13 children with anisometropic hyperopia (range: +6.00 to +8.50 D) underwent unilateral iris-claw PIOL implantation from 2008 to 2018. The mean age was 6.7 years. The mean follow-up was 38.5 months. Target refraction was emmetropia. RESULTS: The cycloplegic refraction (mean spherical equivalent) improved from -12.47 D (range: -9.25 to -18.50 D) to -0.73 D (range: +0.75 to -1.50 D) in myopic eyes and reduced from +7.15 D (range: +6.00 to +8.50 D) to +0.94 D (range: -0.50 to + 1.75 D) in hyperopic eyes. Eighty-four percent of eyes were corrected to within ±1.00 D of emmetropia. The mean uncorrected distance visual acuity (UDVA) improved from 1.74 ± 0.36 to 0.45 ± 0.28 logarithm of the minimum angle of resolution (logMAR) (P = .0014). The mean corrected distance visual acuity (CDVA) changed from 0.68 ± 0.32 to 0.27 ± 0.15 logMAR (P = .02179). The safety index was 2.18 and the efficacy index was 1.51. The mean preoperative endothelial cell count of 2,874.7 cells/mm2 changed to 2,685.3 cells/mm2 at 2 to 6 years postoperatively. Binocular vision quality was improved in 55 patients. No patient had serious complications or lost any Snellen lines of CDVA. CONCLUSIONS: Iris-fixated PIOL implantation is an effective and safe method in the treatment of high anisome-tropic myopia and hyperopia and amblyopia in pediatric patients who are noncompliant with spectacles or contact lenses. [J Pediatr Ophthalmol Strabismus. 2023;60(3):203-209.].

Analysis of biometric parameters of 2340 eyes measured with optical biometer Lenstar LS900 in a Caucasian population.

OBJECTIVES: To describe the pattern and mutual relationships between basic biometric characteristics of the eye in a Central European Caucasian population. METHODS: A single-centre retrospective study of 2340 patients (965 males, 1375 females) scheduled for cataract surgery between 2014 and 2016. Measurements using laser interferometry included AL (axial length), K (average corneal curvature), ACD (anterior chamber depth), LT (lens thickness), CCT (central corneal thickness), AST (astigmatism) and WTW (white to white). Subjects were stratified by gender and controlled for age. Descriptive, correlation and regression analyses were performed on the data. RESULTS: The mean AL was 23.33 ± 1.01 mm - higher in males (23.59 ± 0.99 mm), in comparison to females (23.15 ± 0.99 mm). The elderly had lower ACD and higher LT, while males had higher AL independent of age. Furthermore, LT and K decreased with AL, while ACD decreased with LT and increased with AL independent of age and gender. CONCLUSIONS: The estimates of the biometrics are obtained on a large sample of subjects and can serve as normative values for Lenstar LS900 in the Central European Caucasian population.

The Natural History of Retinal Vascular Changes from Infancy to Adulthood in Wyburn-Mason Syndrome.

Wyburn-Mason syndrome is a rare, non-hereditary congenital neurocutaneous disorder leading to arteriovenous malformations. Malformations are characterized by an artery that is directly connected to veins without a capillary system and forms a fragile mass of abnormal vessels. It can be found in the midbrain, in the eyes, orbit, and rarely in cutaneous nevi. Neurological and ocular symptoms are the most common. Ocular signs and symptoms include abnormally dilatated vessels of conjunctiva, nystagmus, strabismus, vitreous hemorrhage, vein occlusions, retinal detachment, etc. Neurological symptoms may include headaches, paralysis, epistaxis, hydrocephalus, and hemiparesis. Imaging modalities such as MRI/CT angiography, optical coherence angiography, and fluorescein angiography are the most important for the identification of arteriovenous malformations. In our case report, we present an eight-month-old girl with an incidental finding of retinal angiomatosis on the left eye and was subsequently diagnosed with Wyburn-Mason syndrome. We compare the findings from the first visit to her clinical findings 20 years later.

Confluent diode laser coagulation: the gold standard of therapy for retinopathy of prematurity.

PURPOSE: The authors compare results of retinopathy of prematurity treatment with single-spot diode laser coagulation (DLC) versus confluent DLC. The final anatomical outcome and need for additional therapy, such as additional DLC, cryotherapy, scleral buckling, and intravitreal bevacizumab, were evaluated. METHODS: A retrospective review of patients with threshold retinopathy of prematurity treated between January 2001 and October 2012 was conducted. Single-spot laser treatment or confluent laser treatment was applied anterior to the ridge extending to the ora serrata. In the first group (the single-spot group), a single-spot DLC was used between January 2001 and May 2008. The single-spot group included 338 patients (671 eyes) with retinopathy of prematurity. In the second group (the confluent group), confluent DLC was used in 326 patients (652 eyes) between June 2008 and October 2012. The authors compared the need for re-treatment to achieve regression of retinopathy of prematurity in both groups. The rate of progression, frequency of re-treatment, complications, and structural outcomes were evaluated. RESULTS: In the single-spot group, re-treatment only with DLC was necessary in 43 (6.4%) eyes, additional cryotherapy was performed in 22 (3.3%) eyes, and scleral buckling in 107 (15.9%) eyes. Altogether, additional therapy was used in 172 (25.6%) eyes. In the confluent group, re-treatment with DLC was used in 5 (0.8%) eyes, additional cryotherapy in 6 (0.9%) eyes, scleral buckling in 16 (2.5%) eyes, and intravitreal bevacizumab in 14 (2.1%) eyes. Altogether, additional therapy was used in 41 (6.3%) eyes. The confluent group showed a favorable anatomical outcome in 99.1% of the cases compared with 96.4% in the single-spot group. The results were statistically significant (P = .001.) CONCLUSIONS: The DLC method was significantly more effective than single-spot DLC in the treatment of retinopathy of prematurity.