Summary:Alveolar rhabdomyosarcoma (RMS) has a predilection for the deep soft tissues of the extremities and mainly occurs in children. Although the tumor may originate in other sites, such as the nasal cavity or paranasal sinus, invasion of the orbit is unusual. We describe the clinicopathological features of 2 cases of alveolar RMS of the nasal cavity or paranasal sinus in adult patients with orbital extension. These cases of alveolar RMS of the nasal cavity or paranasal sinuses are described in 2 men, both in the third decade of life. These patients were evaluated with radiological studies. The histological diagnosis was confirmed by immunohistochemical methods. Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy. Alveolar subtype RMS is an extremely aggressive neoplasm that rarely presents in the orbit or paranasal sinuses of adults but should be considered in the differential diagnosis of tumors with this localization. Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

Importance Optical coherence tomography software classifies abnormality of macular ganglion cell‐inner plexiform layer thickness and macular retinal nerve fibre layer thickness based on adult series. Background We assessed the impact of using paediatric reference macular ganglion cell complex values instead of adult reference values. Design Cross‐sectional study. Primary and tertiary health‐care setting. Participants Out of 140 healthy participants aged 5 to 18 years, 90% were eligible. Methods Following a dilated eye examination and cycloplegic refraction, participants underwent optical coherence tomography ganglion cell scans (Topcon 3D OCT‐2000; Topcon Corporation, Tokyo, Japan). Right eye measurements for superior, inferior, and total layer thickness and spherical equivalent were reported, together with age, sex and origin. Main Outcome Measures Paediatric reference values by age and spherical equivalent were produced, and the specific agreement between paediatric and adult ganglion cell complex reference values below or equal to percentile 5 was estimated. Results The multivariate analysis confirmed a positive association between spherical equivalent and macular ganglion cell‐inner plexiform layer thickness, and between age and macular retinal nerve fibre layer (five out of six regression coefficients P values were ≤ 0.03). Specific agreement was 25% for ganglion cell‐inner plexiform layer thickness and > 80% for macular retinal nerve fibre layer. Adult‐based software identified low ganglion cell values in one in seven children compared to paediatric reference values (0.8% vs 5.5%, P = 0.031). Conclusions and Relevance The availability of optical coherence tomography ganglion cell complex reference values for paediatric age and spherical equivalent groups can be used to improve detection of children with low cell layer thickness.

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Background: Assessment of interobserver reproducibility and interocular symmetry using optical coherence tomography (OCT)-based measurements of the macular ganglion cell complex (GCC) in healthy children facilitates interpretation of OCT data. We assessed the interobserver reproducibility and interocular symmetry of GCC and evaluated candidate determinants. Methods: This was a cross-sectional study performed in a primary and tertiary health-care setting. A total of 126 healthy participants aged 5 to 18 years were eligible. GCC scans were performed by 4 operators using the Topcon 3D OCT-2000 device. Intraclass correlation coefficients (ICCs) were used to estimate reproducibility and symmetry. Cutoff points for symmetry were defined as the 95th percentile of the absolute interocular difference for 6 GCC parameters. Percentile distributions of interocular difference were generated based on age and difference in absolute interocular spherical equivalent (SE). Results: The reproducibility ICC ranged from 0.96 to 0.98 for all 6 GCC parameters. Cutoff points for interocular symmetry of the superior and inferior quadrants and total macular retinal nerve fibre layer thickness (mRNFL) and macular ganglion cell layerinner plexiform layer thickness were 3.5, 4.5, 3.0, 3.0, 2.5, and 2.5 μm respectively. A positive association was observed between the absolute interocular difference of SE and superior and total mRNFL symmetry values (p = 0.047 and p = 0.040, respectively). Conclusions: OCT measurements of GCC in healthy children show excellent reproducibility. Interocular differences in SE should be assessed when mRNFL differences exceed the 95% cutoff. These findings can contribute to establish reference values for interocular symmetry in paediatric GCC parameters.

Purpose: To investigate the association between the ganglion cell complex (GCC) thickness at early school-age and prematurity and other neonatal factors. Methods: Cross-sectional study. The sample included very preterm children with gestational age (GA) below 32 weeks or birthweight below 1500 g enrolled in a follow-up program (n = 101) and a comparison group of term-born children (n = 49). Ganglion cell complex (GCC) thickness was measured at 4-8 years using high-quality optical coherence tomography (OCT) images. Data on neonatal and postnatal features were extracted from clinical records; analyses included mixed linear models. Results: Ganglion cell layer (GCL) and retinal nerve fiber layer (mRNFL) were thicker in term than in preterm born children (2.9 lm and 2.4 lm respectively, p < 0.001). Within the preterm group, lower GA was associated with a decrease in total GCL (0.7 lm per week, p < 0.001). Being small for GA was associated with further thinning in both layers (1.4 and 2.8 µm). Postnatal corticosteroids therapy and severe brain lesion were associated with thinning in the total GCL of 6 µm (p < 0.001) and 4.1 µm (p = 0.002), respectively, and shock was associated with thinning in total mRNFL of 6 µm (p < 0.001). Conclusions: Lower GA or birthweight are associated with thinning of GCC layers. When performing an OCT examination at school-age and a decrease in GCC thickness is observed, it may be relevant to ask about a history of prematurity, and further enquire about neonatal shock, postnatal corticosteroids therapy or severe brain lesion that are related to additional decrease in GCC thickness.

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Methods:We describe 4 patients suffering from open-angle glaucoma and cataract, and one patient with secondary glaucoma after Fuchs' heterochromic uveitis. All of them underwent phacoemulsification and non-penetrating deep sclerectomy with 5-fluoracil. Two patients presented hematoma in scleral lake 24 h after surgery, other 2 of them presented it one week after the intervention, and one of them presented it 2 weeks after the surgery. The hematoma was found by gonioscopy in all cases. Moreover, all patients had elevated intraocular pressure. Result: Hematoma usually appears within 2 weeks after surgery. Goniopunctures were performed in 5 patients within 5 months of the complication. A second goniopuncture was needed for one of them. Bleb needling was made in one of them. Three of the patients required treatment with beta-blocker eye drops since intraocular pressure exceeded pre-set values. Conclusion: Hematomas in scleral lake are a rare complication that may appear within 2 weeks after non-penetrating deep sclerectomy. They may cause surgery malfunction and antihypertensive eye drops might be required for tensional control. Goniopuncture could be used for solving the complication.