Retinoschisis is a poorly documented form of retinal degeneration characterized by cyst-like splitting that occurs between the inner nuclear and outer plexiform layers. The pathogenesis of retinoschisis is incompletely understood, but congenital, acquired and secondary aetiologies (glaucoma, inflammation, neoplasia) are described in humans. This retrospective study investigated the prevalence and associated histological and clinical features of retinoschisis in cats and dogs submitted for biopsy over a 10-year period. Of 140 samples with documented \'retinal vacuolation\', four out of 120 (3%) canine samples and one out of 20 (5%) feline samples had changes consistent with retinoschisis. In most cases (80%), there was concurrent retinal detachment. In cases with available histories, increased intraocular pressure, proptosis and retinal detachment were reported clinical findings. In cats and dogs, retinoschisis is a retinal change that is generally secondary to other ocular lesions.

To explore the etiologies, characteristics, and prognosis of lamellar macular hole (LMH) in pediatric patients.
A consecutive series of 59 patients (62 eyes) aged <16 years with MHs (lamellar and full-thickness) treated from 2013 to 2021 in a tertiary center was reviewed. Data collected included demographic and clinical characteristics, management, and outcomes of patients presenting with LMH.
Twelve eyes (19.4%) of 11 children had LMH. Seven patients were male, with an average age of 6.9 years. The primary pathologies included X-linked retinoschisis in six eyes (50%); familial exudative vitreoretinopathy in two (16.7%); and ocular toxocariasis, Coats disease, persistent hyperplastic primary vitreous, and idiopathic LMH with associated lenticonus in one eye (8.3%) each. Four eyes (36.4%) showed tractional appearance and seven (63.6%) degenerative. All degenerative LMH showed ellipsoidal zone defect, significantly higher than that in the tractional group (25%, 1/4) ( P = 0.024). Five eyes achieved closed LMH and limited visual gain, four underwent surgery, and one closed spontaneously.
X-linked retinoschisis was the most frequent primary cause in pediatric LMH. Two types of LMH can be classified: tractional and degenerative. The latter showed a higher rate of ellipsoidal zone defect. Vision improved after LMH closed, regardless of surgery or spontaneous closure.

To evaluate the influence of tamponade on the visual and anatomic outcomes of pars plana vitrectomy for myopic traction maculopathy (MTM).
Multicenter, retrospective clinical cohort study.
Consecutive eyes that underwent vitrectomy for advanced MTM with tamponade of air, sulfur hexafluoride (SF6), or perfluoropropane (C3F8) or without tamponade with a minimum follow-up of 12 months were included. Main outcome measures included postoperative visual acuity (VA) at 12 months in eyes with vs without tamponade.
We included a total of 193 eyes (193 patients) in this study; 136 eyes (70%) treated with tamponade were compared with 57 eyes (30%) treated without tamponade. Baseline characteristics did not differ significantly between the groups. Both groups showed significant visual improvement at 12 months (both P < .001). However, postoperative visual acuity and visual improvement at 12 months were significantly better (P = .003 and P = .028, respectively) in eyes without tamponade, although the MTM in these eyes without tamponade took longer to resolve (P = .039). Retinal thickness and the ellipsoid zone were more preserved in eyes without tamponade (P < .001 and P = .001, respectively). Complications such as macular holes did not differ between the groups. A novel imaging finding of \"schisis bending (accordioning)\" was identified during MTM resolution.
Vitrectomy either with or without tamponade for MTM was effective in improving vision in this study. However, eyes without tamponade experienced even better visual improvement and preserved retinal anatomy, despite a longer schisis resolution time. Surgery without tamponade may achieve better visual outcomes.

OBJECTIVE: The study aimed to evaluate the macular microvasculature of X-linked retinoschisis (XLRS) and identify correlations between vascular changes, structural changes, and functional outcome.
METHODS: Genetically confirmed XLRS patients and heathy control subjects underwent complete ophthalmic examination, dilated funduscopic examination, optical coherence tomography, and optical coherence tomography angiography. Schisis distribution, outer plexiform layer discontinuation, photoreceptor layer thickness, and photoreceptor outer segment length were reviewed using optical coherence tomography. Vascular flow density and foveal thickness at foveal and parafoveal area were measured using optical coherence tomography angiography.
RESULTS: A total of 17 eyes of 9 XLRS patients and 22 eyes of 11 control subjects were examined from July 2018 to August 2020. Flow density in the deep capillary plexus at foveal and parafoveal area decreased in XLRS patients compared with control subjects (P = 0.014 and 0.001, respectively), whereas foveal avascular zone area and perimeter remarkably increased (P = 0.015 and 0.001, respectively). Although outer and total retinal layers were significantly thicker in XLRS, inner retinal layer was thinner with reduced photoreceptor layer thickness and shortened photoreceptor outer segment length (P < 0.001 and P < 0.001, respectively). Foveal flow loss in deep capillary plexus, foveal avascular zone enlargement, thinner inner retina and photoreceptor layer thickness, and shortened photoreceptor outer segment length correlated with best-corrected visual acuity.
CONCLUSIONS: X-linked retinoschisis eyes exhibit decreased flow density in the deep capillary plexus and variable foveal avascular zone with enlarged perimeter. Structural deterioration of the photoreceptor best reflects the degenerative changes, whereas microvascular alteration shows considerable correlation with functional outcome in XLRS.

The inheritance pattern of genetically confirmed hereditary juvenile retinoschisis reported so far is X-linked recessive with limited number of female cases. We identified a female patient with retinoschisis, and this study reports the clinical features as well as the underlying genetic defect of this family.
Detailed family history and pedigree analysis were performed. All affected subjects underwent detailed ophthalmic examinations, including best corrected visual acuity (BCVA), dilated fundoscopy, optical coherent tomography (OCT) and fundus autofluorescence (FAF). DNA sample of the proband was sequenced by next-generation sequencing (NGS). Sanger sequencing was performed for validation and segregation.
Three affected subjects including one female and two males were confirmed in this consanguineous family. The BCVA ranged from 20/50 to hand motion. Foveoschisis, hyperopia, subcapsular cataracts, vitreous opacity, retinal pigmentation, and macular atrophy were present in all three patients, with variable severity. Nystagmus, esotropia, and retinal vessels transposition were noted in the female patient. Retinal detachment occurred in the female patient and her affected brother. A small deletion in RS1 gene c.97delT (p.W33Gfs*93) (NM_000330.3) was found, which was co-segregated in the pedigree.
Consanguineous family having XLRS female patient could manifest as pseudo-dominant inheritance. Significant intrafamilial phenotypic variation was revealed.

OBJECTIVE: To evaluate a case of macular retinoschisis associated with a TJP2 mutation in a young woman affected by a Progressive Familiar Intrahepatic Cholestasis (PFIC) using multimodal imaging.
METHODS: Observational case report.
RESULTS: A 35-year-old woman, undergone a liver transplant for PFIC, was referred to Eye Clinic for complete ophthalmological examination and multimodal imaging. Fundus examination showed no significant alterations in both eyes while multicolor image revealed the presence of several cystes in macular region. The autofluorescence image highlighted small areas of iperautofluorescence in macular region. En-face image showed a \"coral shape pattern\" and structural optical coherence tomography (OCT) revealed foveal hyporeflective cystic spaces in outer and inner nuclear layers, areas of splitting within the nerve fiber layer (schisis areas) and an initial preretinal fibrosis. Lastly, OCT-angiography (OCT-A) demonstrated small perifoveal teleangectasias and slight reduction of the foveal avascular zone area.
CONCLUSIONS: Multimodal imaging could help to highlight the presence of a rare retinal disorder associated with a gene related systemic disease.

OBJECTIVE: To evaluate the effect of vitrectomy combined with scleral shortening for eyes with myopic macular retinoschisis.
METHODS: Thirty-seven patients with myopic macular retinoschisis who underwent pars plana vitrectomy (PPV) combined with scleral shortening were reviewed. Axial length (AL), the height of macular retinoschisis, the height of retinal detachment if existed, the diameter of macular hole if existed and best corrected visual acuity (BCVA) were obtained. The preoperative and postoperative parameters were compared.
RESULTS: At postoperative 24mo, the mean AL and height of macular retinoschisis were reduced significantly by 0.79 mm and 256.51 µm (t=8.064, P<0.0001; Z=-5.086, P<0.0001) respectively. In addition, the mean height of retinal detachment and diameter of macular hole were also reduced significantly by 365.38 µm and 183.68 µm (Z=-4.457, P=0.000008; Z=-2.983, P=0.003) respectively. Meanwhile, the postoperative BCVA was improved markedly (Z=-2.126, P=0.033).
CONCLUSIONS: Vitrectomy combined with scleral shortening is an effective surgical method for eyes with myopic macular retinoschisis, whether or not macular hole and retinal detachment are present.

OBJECTIVE: Until now, the diagnosis of optic nerves hemorrhages in abusive head trauma (AHT) has been obtained only in the postmortem setting. The aim of the IRB-approved study was to assess the presence of optic nerves hemorrhages in AHT patients using 3D-SWI.
METHODS: Thirteen children with a final confirmed multidisciplinary diagnosis of AHT underwent coronal and axial 3D-SWI imaging of the orbits. The presence of optic nerve sheath (ONS) hemorrhages was defined by thickening and marked 3D-SWI hypointensity of the ONS, resulting in mass effect upon the CSF space. Optic nerve (ON) hemorrhages were defined by areas of susceptibility artifacts in the ON parenchyma. Superficial siderosis was defined by susceptibility artifact coating the ON. Furthermore, data about post-traumatic deformity of the ONS at the head of the optic nerve were collected.
RESULTS: The average age of the population was 7.9 ± 5.9 months old. The average GCS was 11.8 ± 4.5. The male to female ratio was 7:6. ONS hemorrhages were identified in 69.2% of cases. Superficial siderosis and ON hemorrhages were identified in 38.5 and 76.9% of cases, respectively. 3D-SWI also depicted traumatic deformity of the ONS at the level of the optic nerve head in 10 cases (76.9%). No statistical correlations were identified between RetCam findings and 3D-SWI findings or GCS and ON hemorrhages.
CONCLUSIONS: This research shows that dedicated MRI with volumetric SWI of the orbits can depict hemorrhages in the ON, ONS, and ONS injury, in AHT victims.

The purpose of this study was to analyze the clinical features of dome-shaped macula (DSM) in highly myopic eyes and its morphological relationship with myopic retinoschisis (MRS).
In this cross-sectional study, 409 eyes of 409 patients with high myopia who had spectral-domain optical coherence tomography (OCT) examinations were included. The associations of DSM with the distribution of MRS and ocular biometry were evaluated.
Of 409 eyes, DSM was detected in 64 eyes (15.6%). The eyes with DSM were more myopic (-18.8 ± 3.9 vs. -13.4 ± 5.9; P < 0.001) and had longer axial length (31.7 ± 2.4 vs. 29.5 ± 2.5; P < 0.001) compared with those without DSM. A higher rate of extrafoveal retinoschisis (35.9% vs. 9.6%; P < 0.001) and a lower rate of foveoschisis (10.9% vs. 26.1%; P = 0.01) were detected in the eyes with DSM compared with those without DSM. In the eyes with DSM, MRS was detected in 30 eyes (46.9%). MRS predominantly affected the extrafoveal area (76.7%), especially the base of the dome (82.6%). The extrafoveal retinoschisis was most frequently distributed in the superior quadrant (52.2%). None of the eyes with DSM displayed fovea-only retinoschisis. The ratio of the height and width of the macular bulge was higher in eyes with MRS than those without MRS (0.05 vs. 0.04; P = 0.001).
A DSM is found in highly myopic eyes with a longer axial length. MRS in eyes with DSM is more likely to affect the extrafoveal area, especially the base of the dome. A steeper macular bulge is associated with the occurrence of MRS.

To explore the role of foveal and parafoveal Müller cells in the morphology and pathophysiology of tractional macular disorders with a mathematical model of mechanical force transmission.
In this retrospective observational study, spectral-domain optical coherence tomography images of tractional lamellar macular holes and patients with myopic foveoschisis were reviewed and analysed with a mathematical model of force transmission. Parafoveal z-shaped Müller cells were modelled as a structure composed of three rigid rods, named R1, R2 and R3. The angle formed between the rods was referred to as θ . R1, R2 and R3 lengths as well as the variation of the angle θ were measured and correlated with best corrected visual acuity (BCVA).
In tractional lamellar macular holes, there was a significant reduction of the angle θ towards the foveal centre (p<0.001). By contrast, there were no significant differences in θ in myopic foveoschisis (p=0.570). R2 segments were more vertical in myopic foveoschisis. There was a significant association between lower θ angles at 200 µm temporal and nasal to the fovea and lower BCVA (p<0.001 and p=0.005, respectively). The stiffness of parafoveal Müller cells was predicted to be function of the angle θ , and it grew very rapidly as the θ decreased.
Parafoveal Müller cells in the Henle fibre layer may guarantee structural stability of the parafovea by increasing retinal compliance and resistance to mechanical stress. Small values of the angle θ were related to worse BCVA possibly due to damage to Müller cell processes and photoreceptor\'s axons.