Rod Rotation with Outrigger Is Substantial for Correcting Apical Hypokyphosis in Patients with Adolescent Idiopathic Scoliosis: Novel Outrigger Device for Concave Rod Rotation.

The apical hypokyphosis of scoliotic patients is thought to lead to decreased lung capacity and cause shortness of breath. Additionally, concave rod curve reduction is a problem in the correction of apical hypokyphosis in posterior spinal fusion surgery in adolescent idiopathic scoliosis (AIS). We investigated the contributions of rod rotation (RR) with an outrigger device, followed by differential rod contouring (DRC) with the outrigger attached to the concave rod, designed to prevent concave rod curve-flattening. We analyzed and compared the results of segmental pedicle screw fixation without the outrigger in 41 AIS patients with thoracic curves (Lenke type I, 25; type II, 16) to those corrected using the outrigger in 36 patients (Lenke type I, 24; type II,12). The changes in the Cobb angle, apical kyphosis of five vertebrae, thoracic kyphosis (TK, T4-12), correction rate, correction angle of apical vertebral rotation, spinal penetration index (SPi), and rib hump index (RHi) before and after surgery were measured, and the contribution of the outrigger was analyzed. The mean scoliosis correction rates without and with the outrigger were 72.1° and 75.6°, respectively (p = 0.03). Kyphosis of the five apical vertebrae and TK were significantly greater in the surgery with the outrigger (p = 0.002). Significantly greater improvements in SPi and RHi were also noted in the surgery with the outrigger (p < 0.05). The use of concave RR and convex DRC with the outrigger appear to be advantageous for correcting apical hypokyphosis, followed by the subsequent formation of TK. As a result, breathing problems are less likely to occur during daily life because of improvements in SPi and RHi.

Involvement of Angiopoietin 2 and vascular endothelial growth factor in uveitis.

PURPOSE: Angiopoietin (Ang) 2 is released from vascular endothelial cells by the stimulation of vascular endothelial growth factor (VEGF)A. Ang2 increases the expression of leukocyte adhesion molecules on endothelial cells via nuclear factor κB. The aim of this study was to evaluate the effects of Ang2 and VEGFA on ocular autoimmune inflammation. METHODS: We measured the concentrations of Ang2 and VEGFA in vitreous samples among patients with uveitis. Vitreous samples were collected from 16 patients with idiopathic uveitis (uveitis group) and 16 patients with non-inflammatory eye disease (control group). Experimental autoimmune uveoretinitis (EAU) was induced in B10.BR mice with a human interphotoreceptor retinoid-binding protein-derived peptide. The retinochoroidal tissues of the EAU mice were removed, and the mRNA levels of Ang2 and VEGFA were examined. EAU mice treated with anti-Ang2, anti-VEGFA, a combination of anti-Ang2 and anti-VEGFA, anti-Ang2/VEGFA bispecific, or IgG control antibodies were clinically and histopathologically evaluated. RESULTS: The protein levels of Ang2 and VEGFA were significantly higher in the vitreous samples of patients with uveitis than in controls (P<0.05). The retinochoroidal mRNA levels of Ang2 and VEGFA were significantly upregulated in EAU mice compared to controls (n = 6, P<0.05). Although there was no significant difference, treatment with anti-VEGFA antibody reduced the clinical and histopathological scores. However, treatment with anti-Ang2 antibody reduced the clinical and histopathological scores (n = 18-20, P<0.05). Furthermore, these scores were further decreased when treated by inhibiting both Ang2 and VEGFA. CONCLUSIONS: Based on these results, VEGFA and Ang2 were shown to be upregulated locally in the eye of both uveitis patients and models of uveitis. Dual inhibition of Ang2 and VEGFA is suggested to be a new therapeutic strategy for uveitis.

Association between serum interleukin-17 levels and ectopic bone formation in OPLL patients with DISH.

OBJECTIVE: To investigate the relationship between the severity and morphology of heterotopic ossification in the spinal ligaments including sacroiliac (SI) joints, and serum interleukin-17 (IL-17) levels in patients with ossification of the posterior longitudinal ligament (OPLL) with or without diffuse idiopathic skeletal hyperostosis (DISH), as well as a non-OPLL group. METHODS: A total of 103 patients with OPLL (DISH (-), n = 50; DISH (+), n = 53) and 53 age- and gender-matched controls were included. The serum levels of IL-17 were analyzed, and the severity of ectopic ossification and the morphology of ectopic bone formation were evaluated. The SI joint morphological variations were categorized into four types. RESULTS: No significant differences were found in serum IL-17 levels between the OPLL and control groups. However, the DISH (+) group showed higher IL-17 levels than the DISH (-) group, especially in female patients (p = 0.003). Additionally, IL-17 levels were positively correlated with the number of Flat vertebral units, meaning one of the characteristics of DISH ossification type (R2 = 0.199, p = 0.012). IL-17 levels in type 4 were significantly higher in the DISH (+) group than in the DISH (-) group. CONCLUSIONS: The morphological characteristics of paravertebral bone formation in the entire spine, including the SI joint, are likely associated with serum IL-17 levels in OPLL. These findings provide pathological and serological evidence of local inflammation contributing to paravertebral ossification of OPLL patients.

A case of tubulointerstitial nephritis and uveitis syndrome accompanied by subclinical choroiditis.

BACKGROUND: Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uveits characterized by complications of idiopathic acute tubulointerstitial nephritis, and most cases present only anterior uveitis. We report a case of TINU syndrome in which the presence of choroiditis was revealed by multimodal imaging. CASE PRESENTATION: A 12-year-old male visited our hospital with a 6-day history of ocular pain and hyperemia. Conjunctival and ciliary injections, 1 + flare and 3 + cells of anterior chamber inflammation with mutton fat keratic precipitates were observed in both eyes (OU), together with redness and swelling of the optic disc OU. Laboratory tests showed slightly high levels of soluble IL-2R and serum ß2 microglobulin and markedly high levels of urinary ß2 microglobulin. The diagnosis of probable TINU syndrome was established on the basis of bilateral uveitis and urinalysis results in accordance with a clinical criteria of tubulointerstitial nephritis. With treatment with oral prednisolone (PSL) at 20 mg/day, ocular findings improved, and the dose of PSL was gradually reduced and withdrawn 6 months later. However, 1 month later from the withdrawal, ocular inflammation recurred with the presence of retinal exudates and snowball vitreous opacities in the peripheral retina OU. Fluorescein angiography showed leakages from peripheral retinal vessels and staining corresponding to retinal exudates. Indocyanine green angiography showed hypofluorescent dots scattered over the ocular fundus. Optical coherence tomography revealed the presence of choroidal thickening. Laser speckle flowgraphy color map showed a relatively cooler color. Findings from these multimodal images indicated the presence of subclinical choroiditis; therefore, oral PSL was administered again, and ocular inflammatory findings were improved. CONCLUSIONS: TINU syndrome can exhibit subclinical choroiditis detected with multimodal imaging. Further studies are necessary to determine the frequency of subclinical choroiditis in TINU syndrome.

Assessment of Cervical Myelopathy Risk in Ossification of the Posterior Longitudinal Ligament Patients With Spinal Cord Compression Based on Segmental Dynamic Versus Static Factors.

OBJECTIVE: Using segmental dynamic and static factors, we aimed to elucidate the pathogenesis and relationship between ossification of the posterior longitudinal ligament (OPLL) and the severity of cervical myelopathy. METHODS: Retrospective analysis of 163 OPLL patients' 815 segments. Imaging was used to evaluate each segmental space available for the spinal cord (SAC), OPLL diameter, type, bone space, K-line, the C2-7 Cobb angle, each segmental range of motion (ROM), and total ROM. Magnetic resonance imaging was used to evaluate spinal cord signal intensity. Patients were divided into the myelopathy group (M group) and the without myelopathy group (WM group). RESULTS: Minimal SAC (p = 0.043), (C2-7) Cobb angle (p = 0.004), total ROM (p = 0.013), and local ROM (p = 0.022) were evaluated as an independent predictor of myelopathy in OPLL. Different from the previous report, the M group had a straighter whole cervical spine (p < 0.001) and poorer cervical mobility (p < 0.001) compared to the WM group. Total ROM was not always a risk factor for myelopathy, as its impact depended on SAC, when SAC > 5 mm, the incidence rate of myelopathy decreased with the increase of total ROM. Lower cervical spine (C5-6, C6-7) showing increased "Bridge-Formation," along with spinal canal stenosis and segmental instability (C2-3, C3-4) in the upper cervical spine, could cause myelopathy in M group (p < 0.05). CONCLUSION: Cervical myelopathy is linked to the OPLL's narrowest segment and its segmental motion. The hypermobility of the C2-3 and C3-4, contributes significantly to the development of myelopathy in OPLL.

A case of APMPPE-like panuveitis presenting with extensive outer retinal layer impairment following COVID-19 vaccination.

BACKGROUND: Vaccination against the worldwide pandemic coronavirus disease 2019 (COVID-19) is underway; however, some cases of new onset uveitis after vaccination have been reported. We report a case of bilateral acute posterior multifocal placoid pigment epitheliopathy-like (AMPPE-like) panuveitis after COVID-19 vaccination in which the patient's pathological condition was evaluated using multimodal imaging. CASE PRESENTATION: A 31-year-old woman experienced bilateral hyperemia and blurred vision starting 6 days after her second inoculation of the COVID-19 vaccination. At her first visit, her visual acuity was decreased bilaterally, and severe bilateral anterior chamber inflammation and bilateral scattering of cream-white placoid lesions on the fundus were detected. Optical coherence tomography (OCT) showed serous retinal detachment (SRD) and choroidal thickening in both eyes (OU). Fluorescein angiography (FA) revealed hypofluorescence in the early phase and hyperfluorescence in the late phase corresponding to the placoid legions. Indocyanine green angiography (ICGA) showed sharply marginated hypofluorescent dots of various sizes throughout the mid-venous and late phases OU. The patient was diagnosed with APMPPE and was observed without any medications. Three days later, her SRD disappeared spontaneously. However, her anterior chamber inflammation continued, and oral prednisolone (PSL) was given to her. Seven days after the patient's first visit, the hyperfluorescent lesions on FA and hypofluorescent dots on ICGA partially improved; however, the patient's best corrected visual acuity (BCVA) recovered only to 0.7 OD and 0.6 OS, and the impairment of the outer retinal layer was broadly detected as hyperautofluorescent lesions on fundus autofluorescence (FAF) examination and as irregularity in or disappearance of the ellipsoid and interdigitation zones on OCT, which were quite atypical for the findings of APMPPE. Steroid pulse therapy was performed. Five days later, the hyperfluorescence on FAF had disappeared, and the outer retinal layer improved on OCT. Moreover, the patient's BCVA recovered to 1.0 OU. Twelve months after the end of treatment, the patient did not show any recurrences. CONCLUSIONS: We observed a case of APMPPE-like panuveitis after COVID-19 vaccination featuring some atypical findings for APMPPE. COVID-19 vaccination may induce not only known uveitis but also atypical uveitis, and appropriate treatment is required for each case.

Association of Inflammation, Ectopic Bone Formation, and Sacroiliac Joint Variation in Ossification of the Posterior Longitudinal Ligament.

Ossification of the posterior longitudinal ligament (OPLL) is considered a multifactorial condition characterized by ectopic new bone formation in the spinal ligament. Recently, its connections with inflammation as well as sacroiliac (SI) joint ankylosis have been discussed. Nevertheless, whether inflammation, spinal ligament ossification, and SI joint changes are linked in OPLL has never been investigated. In this study, whole-spinal computed tomography and serum high-sensitive C-reactive protein (hs-CRP) levels were obtained in 162 patients with cervical OPLL. Ossification lesions were categorized as plateau and hill shapes. Accordingly, patients were divided into plateau-shaped (51 males and 33 females; mean age: 67.7 years) and hill-shaped (50 males and 28 females; mean age: 67.2 years) groups. SI joint changes were classified into four types and three subtypes, as previously described. Interactions among ossification shapes, hs-CRP levels, and morphological changes in the SI joint were investigated. The plateau shape was more common in the vertebral segments (59.5%), compared to the hill shape, which was predominant in the intervertebral regions (65.4%). Serum hs-CRP levels in the plateau-shaped group (0.11 ± 0.10 mg/dL) were significantly higher than those in the hill-shaped group (0.07 ± 0.08 mg/dL). SI joint intra-articular fusion was the main finding in the plateau-shaped group and showed significantly higher hs-CRP levels compared to the anterior para-articular bridging, which more frequently occurred in the hill-shaped group. Our findings suggested a possible inflammation mechanism that might contribute to the new bone formation in OPLL, particularly the plateau shape.

Sacroiliac Joint Variation in Patients With Ossification of the Posterior Longitudinal Ligament.

STUDY DESIGN: Retrospective Cohort Study. OBJECTIVES: Ossification of the posterior longitudinal ligament (OPLL) reveals heterotopic ossification in the spinal ligament. OPLL also tends to ossify ligaments and entheses throughout the body. However, hallmarks of sacroiliac (SI) joint ossification and its variation in OPLL have not been clarified. Here, we investigated the morphological changes in SI joints in individuals with and without OPLL. METHODS: We included 240 age- and sex-matched patients (OPLL+, 120; OPLL-, 120) in the study. SI joint variations were classified into 4 types: Type 1, normal or small peripheral bone irregularity; Type 2, subchondral bone sclerosis and osteophyte formation; Type 3, vacuum phenomenon; and Type 4, bridging osteophyte and bony fusion. Type 4 was further divided into 3 subgroups as previously described. Interactions between the ossified spinal region in OPLL and morphological changes in the SI joint were evaluated. RESULTS: SI joint ankylosis occurs more frequently in patients with OPLL (51.7%) than in those without (non-OPLL) (33.3%). The SI joint vacuum phenomenon (49.2%) was the main finding in non-OPLL. SI joint ankylosis in OPLL was characterized by anterior bridging and intra-articular fusion. OPLL patients with multilevel ossification tend to develop degeneration and ankylosis of the SI joints. CONCLUSIONS: OPLL conferred a high risk of SI joint ossification compared with non-OPLL, and patients with extensive ossification had a higher rate of SI joint ankylosis. Understanding SI joint variation could help elucidate OPLL etiology and clarify the phenotypic differences in the SI joint between OPLL and other spinal disorders.

The Steroid-Sparing Effect of Adalimumab in the Treatment for the Recurrent Phase of Vogt-Koyanagi-Harada Disease.

PURPOSE: To reveal the steroid-sparing effect of adalimumab (ADA) in the treatment for the chronic recurrent phase of Vogt-Koyanagi-Harada (VKH) disease. CASES AND METHODS: Thirty-six eyes from 18 cases of the recurrent phase of VKH disease treated with ADA over 12 months were examined retrospectively. Before the introduction of ADA, 4 cases received prednisolone (PSL) monotherapy and other 14 cases received PSL and cyclosporine A (CYA) combination therapy. RESULTS: In cases treated with PSL and CYA, CYA was discontinued when ADA was introduced. The minimum dose of PSL to control intraocular inflammation (min dose of PSL) could be reduced in all cases after the introduction of ADA (from 16.9 ± 7.9 mg to 6.3 ± 3.1 mg). No serious adverse events were observed in the observational periods. CONCLUSION: By comparing the min dose of PSL before and after the introduction of ADA, the steroid-sparing effect of ADA was confirmed.

Reduction of Intraocular Pressure and Aqueous Outflow Resistance After Modified 360-degree Suture Trabeculotomy.

PRCIS: Decreasing aqueous outflow resistance at trabecular meshwork is the main mechanism of modified 360-degree suture trabeculotomy (ST), and the preoperative C-value, which shows aqueous outflow resistance, is likely to be useful for predicting intraocular pressure (IOP) reduction. PURPOSE: To clarify the mechanism of IOP reduction and the preoperative prognostic predictor of modified 360-degree ST. MATERIALS AND METHODS: Forty-three eyes of 32 patients with glaucoma who underwent ST at Hokkaido University Hospital between April 2017 and February 2020 were enrolled. The records of postoperative IOP and coefficient of aqueous outflow (C-value) after ST were reviewed from clinical charts retrospectively. Preoperative IOP and C-values were also reviewed and considered as the baseline. RESULTS: Although the differences were not significant, IOP decreased to 15.4±3.3 mm Hg at 3 months ( P =0.10) and 16.1±3.8 mm Hg at 6 months ( P =0.21). In addition, there were significant decreases in anti-glaucoma medication scores at both 3 and 6 months after surgery ( P <0.01). The C-value increased significantly to 0.24±0.11 µL/min/mm Hg at 3 months ( P <0.01) and increased significantly to 0.27±0.14 µL/min/mm Hg at 6 months ( P <0.01). The rates of change in IOP were negatively correlated with that in the C-value at 3 months ( r =-0.49 P <0.01) and 6 months ( r =-0.46 P <0.01). The success rate (IOP<21 mm Hg, IOP reduction>20%) was greater in the low baseline C-value group (≤0.17) than high baseline C-value group (>0.17) ( P <0.05), and the baseline IOP and C-values were statistically significant in association with the success ( P <0.05) at 6 months. CONCLUSIONS: Increased conventional outflow by the elimination of the aqueous outflow resistance at the trabecular meshwork is the main mechanism of IOP reduction after ST. Preoperative examination of tonographic outflow facility may be useful for predicting the IOP reduction and outcome of ST.

Usefulness of Wide-Awake Local Anesthesia No Tourniquet Surgery to Decide on Tendon Transfer Versus Grafting in Chronic Flexor Tendon Rupture.

Purpose: We investigated the clinical outcomes of flexor tendon reconstruction for chronic rupture of the flexor tendon based on an evaluation of the voluntary active contraction distance (ACD) of the ruptured musculotendinous unit and changes in intraoperative total active motion (TAM) that could only be observed during wide-awake local anesthesia no tourniquet (WALANT) surgery. Methods: Reconstructions of 19 tendons of the flexor pollicis longus (FPL) and 18 tendons of the flexor digitorum profundus (FDP) were performed during WALANT surgery to evaluate the ACD of the ruptured musculotendinous unit and TAM observed during the surgery. Tendon grafting or tendon transfer was selected during the surgery based on ACD. TAM, pinch strength, and grip power were evaluated before the surgery, during the surgery, and at final follow-up, and they were surveyed based on Quick Disabilities of the Arm, Shoulder, and Hand (q-DASH) scores. The final outcomes of tendon grafting and tendon transfer were compared. Results: In FPL tendon reconstruction, tendon grafting was performed in 10 patients with a total PDD and ACD value greater than 30 mm, and tendon transfer was performed in 9 patients with the value less than 30 mm. In FDP tendon reconstruction, tendon grafting was performed in 8 patients and tendon repair in 2 patients with a total PDD and ACD value greater than 40 mm, and tendon transfer was performed in 8 patients with the value less than 40 mm. The TAM value, q-DASH score, pinch power, and grip strength were improved in all patients. In both the tendon reconstructions, intraoperative TAM was significantly increased compared with preoperative TAM but significantly decreased at final follow-up. No significant differences were identified in final follow-up TAM and the q-DASH scores between tendon transfer and tendon grafting. Conclusions: The great advantage of WALANT surgery is that surgeons can evaluate the ruptured musculotendinous unit and measure TAM during the surgery. Type of study/level of evidence: Therapeutic I.

Clinical significance of serum and vitreous soluble interleukin-2 receptor in patients with intraocular lymphoma.

BACKGROUND: Intraocular lymphoma (IOL) is a masquerade syndrome that mimics uveitis, making diagnosis difficult. The serum soluble interleukin-2 receptor (sIL-2R), which is cleaved by matrix metalloproteinase (MMP) -2 and MMP-9, has been recognized as a tumor-related biomarker of malignant lymphomas. The aim of this study was to review the reliability of serum and vitreous sIL-2R for distinguishing IOL from uveitis. METHODS: Patients who underwent diagnostic vitrectomy for marked vitreous haze at Hokkaido University Hospital between April 2014 and June 2019 were enrolled. The patients were divided into an IOL group and a uveitis group, according to the pathology of their vitreous samples. The IOL group was further divided at the time of vitrectomy into patients who already had extraocular involvement (IOL with extraocular involvement group) and patients with no evidence of having extraocular involvement (IOL without extraocular involvement group). Serum sIL-2R, and intravitreal sIL-2R, MMP-2, and MMP-9 levels were assessed. RESULTS: Twenty-five eyes of 25 patients, and 15 eyes of 15 patients were included in the IOL group and uveitis group, respectively. The serum sIL-2R levels were significantly lower in the IOL group than in the uveitis group (P < 0.05), and 20.0% and 66.7% in the IOL and the uveitis group showed high sIL-2R value above the normal range. Vitreous sIL-2R tended to be higher in the IOL group than in the uveitis group (P = 0.80). Serum sIL-2R was significantly lower in the IOL without extraocular involvement group than in the IOL with extraocular involvement group (P < 0.05); 5.9% in the IOL without extraocular involvement group and 50.0% in the IOL with extraocular involvement group showed high sIL-2R value above the normal range. Vitreous sIL-2R, MMP-2, and MMP-9 tended to be higher in the IOL with extraocular involvement group than in the IOL without extraocular involvement group (P = 0.30, < 0.05, 0.16). CONCLUSIONS: Serum sIL-2R is often within the normal range in IOL patients. Even if it is within the normal range, the possibility of IOL should be considered. Serum sIL-2R is not a reliable biomarker for IOL, whereas vitreous sIL-2R may be useful for the diagnosis of IOL.

Fundus autofluorescence imaging in acute posterior multifocal placoid pigment epitheliopathy.

Purpose: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a disease characterized by multiple yellowish-white placoid lesions. Although most lesions resolve spontaneously, some turn into scars and lead to permanent visual dysfunction. In this report, we found suggestive findings in fundus autofluorescence (FAF) that may be useful for distinguishing severe lesions requiring treatment in APMPPE. Observation: Case 1: A 29-year-old woman was referred to our hospital with multiple yellowish-white placoid lesions on the fundi of both eyes (OU). FAF showed hyperautofluorescence in some of these placoid lesions. Based on the findings of fluorescein angiography, a diagnosis of APMPPE was established, and oral prednisolone (PSL) was initiated, given that some lesions were located in the macula. One week later, exacerbation occurred with the newly developed hyperautofluorescent lesions. Some lesions in the right eye (OD) that were hyperautofluorescent at the first visit became hypoautofluorescent. Afterward, although all hypoautofluorescent lesions persisted, most of the hyperautofluorescent lesions disappeared, so oral PSL could be stopped. Two months later, however, the recurrence occurred along with multiple new placoid lesions. Some lesions located at the macula were hyperautofluorescent on FAF OU, indicating the possibility of becoming scar lesions with hypoautofluorescence. Accordingly, oral PSL was given again. Case 2: A 47-year-old woman noticed decreased vision OD, and she was referred to us. Multiple yellowish-white placoid lesions were seen in the fundi OU. FAF showed hyperautofluorescence both with and without corresponding hypoautofluorescence in the placoid lesions OU. A diagnosis of APMPPE was established, and oral PSL was initiated. Four months later, some lesions that were hyperautofluorescent at the first visit had turned isoautofluorescent, and some lesions OU became hypoautofluorescent. However, all hypoautofluorescent lesions remained hypoautofluorescent OU. Only some hyperautofluorescent lesions recovered to isoautofluorescence without scars. Conclusions and Importance: In APMPPE, lesions showing hyperautofluorescence on FAF may change into hypoautofluorescence indicating scar formation. Therefore, the presence of hyperautofluorescent lesions in the macula may be a good indicator of the need for intensive corticosteroid treatments to avoid leaving hypoautofluorescent scars that are related to irreversible visual dysfunction.

Clinical features of ocular sarcoidosis: severe, refractory, and prolonged inflammation.

PURPOSE: To clarify the proportion of ocular sarcoidosis with severe, refractory, and prolonged inflammation and their association with ocular complications and visual prognosis. STUDY DESIGN: Multicenter, retrospective, longitudinal cohort study. METHODS: Three hundred and twenty-three eyes of 164 patients (45 men; 119 women) with ocular sarcoidosis who visited Hokkaido University Hospital and Yokohama City University Hospital from 2010 to 2015. We newly defined severe, refractory, and prolonged inflammation in ocular sarcoidosis, and investigated their proportions, ocular complications and final visual acuity from medical records of our sarcoidosis patients. RESULTS: The eyes with severe inflammation numbered 72/323 (22.3%), with refractory inflammation, 80/323 (24.8%), and with prolonged inflammation, 91/323 (28.2%). The number of eyes having neither severe, refractory, nor prolonged inflammation (defined as none) was 114/323 (35.3%). The numbers of eyes that reached irreversible visual dysfunction were 6/72 (8.3%) of those with severe inflammation, 10/80 (12.5%) with refractory inflammation, 12/91 (13.2%) with prolonged inflammation, and 4/114 (6.2%) with none. As complications, cataract (62.2%), glaucoma (28.5%), epiretinal membrane (24.1%), cystoid macular edema (22.6%), vitreous hemorrhage (2.8%), choroidal atrophy (2.5%), macular degeneration (1.2%), macular hole (0.9%) and retinal detachment (0.3%) were identified. Among them, secondary glaucoma (16 eyes) and macular degeneration (4 eyes) were major complications related to irreversible visual dysfunction. CONCLUSIONS: Although most of the patients with ocular sarcoidosis had a relatively good visual prognosis, some developed severe, refractory, and/or prolonged inflammation related to the development of ocular complications, that resulted in poor visual prognosis.

Efficacy and Safety of Adalimumab for Exacerbation or Relapse of Ocular Inflammation in Patients with Vogt-Koyanagi-Harada Disease: A Multicenter Study.

PURPOSE: We investigated efficacy and safety of adalimumab (ADA) treatment for exacerbation or recurrence of Vogt-Koyanagi-Harada (VKH) patients. METHODS: Medical records of 70 VKH patients who received ADA treatment for more than 6 months were retrospectively investigated. RESULTS: The mean age of VKH patients was 54.8 ± 15.1 years, and male/female ratio was 34/36, and sunset glow fundus was observed in 71.4%. Subfoveal choroidal thickness, indocyanine green angiography scores, and corticosteroid and cyclosporine doses were significantly reduced by ADA treatment for 6 months compared to baseline, while LogMAR and flare counts were also improved without being statistically significant. Adverse events were observed in 17.1%, in which tuberculosis was at 7.14% and psoriasis was at 2.86%; however, ADA treatment was continued in 91.4%. CONCLUSIONS: ADA was shown to be effective to achieve remission of VKH disease refractory to conventional treatments and was generally well tolerated with few serious adverse events.

BCOR-CCNB3 sarcoma arising in the proximal tibia: A case report.

BCL6 corepressor-cyclin B3 (BCOR-CCNB3) fusion sarcoma was classified as an emerging subgroup of undifferentiated small round cell sarcoma in 2020. The incidence of BCOR-CCNB3 fusion sarcoma is reportedly 1.5-14% among undifferentiated unclassified sarcomas, representing a rare entity among primary malignant bone tumors. The present study reports a case of BCOR-CCNB3 fusion sarcoma in the proximal tibia of a boy. A 12-year-old boy presented with a 6-month history of knee pain and a slowly growing mass in the anteromedial aspect of the left proximal tibia. Plain radiography and computed tomography of the knee demonstrated a lytic lesion with cortical destruction of the proximal tibia. Magnetic resonance imaging showed the bone tumor expanding into soft tissue with almost homogeneous hypointensity on T1-weighted imaging and slightly hyperintensity on T2-weighted imaging. On histopathological evaluation, the tumor comprised a proliferation of small, round to ovoid-shaped mesenchymal cells without osteoid formation. Histopathologically, BCOR-CCNB3 sarcoma of bone was finally diagnosed based on immunohistochemical staining and additional molecular analyses. The patient underwent bone tumor resection followed by pre- and post-operative chemotherapy according to a Ewing sarcoma protocol. The patient showed no evidence of local recurrence or distant metastasis at 12 months after completion of adjuvant chemotherapy. We present herein an additional case of BCOR-CCNB3 sarcoma of the proximal tibia, and review the relevant literature on BCOR-CCNB3 sarcoma of bone.

Diagnostic Accuracy of Cell Block Preparations and Clinical Features Affecting It in Vitreoretinal Lymphoma.

Purpose: The purpose of this study was to examine the diagnostic accuracy of the cell block (CB) method and clinical features affecting it in patients with vitreoretinal lymphoma (VRL). Methods: This study enrolled 38 eyes in 33 VRL patients, and 7 eyes in 7 patients with idiopathic uveitis who underwent diagnostic vitrectomy. Medical records including the results of CB cytology, interleukin (IL)-10/-6 concentrations, and immunoglobulin heavy chain gene (IgH) rearrangement were retrospectively searched. Results: Patients with VRL comprised 16 women and 17 men, and the age of onset ranged from 44 to 85 years (mean: 70 years). CB preparations detected large malignant cells in 35 eyes (92%), whereas the other 3 VRL eyes were negative. Two of the latter three eyes showed subretinal infiltrates, which existed in 7 of 35 CB-positive eyes. Intravitreal IL-10 and -6 concentrations were 1866 ± 4088 pg/mL and 98 ± 139 pg/mL, respectively, and the rate of IL-10/-6 >1 was 86.9%. The presence of IgH monoclonality was 63.2%. In patients with uveitis, CB specimens revealed no atypical but small inflammatory cells. IL-6 concentration was 311.1 ± 240 pg/mL, whereas IL-10 was undetectable in six eyes, and the IL-negative rate was 85.7%. Six eyes (85.7%) with uveitis showed no IgH monoclonality. Conclusions: Diagnostic accuracy of CB preparations in VRL could achieve an equivalent outcome to IL ratio calculation and IgH monoclonality detection. The appearance of subretinal infiltrates may diminish the CB positivity.

Posterior scleritis with anti-neutrophil cytoplasmic antibody-associated vasculitis utilizing rituximab therapy to maintain remission: A case report.

PURPOSE: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a necrotizing vasculitis syndrome characterized by the destruction of small vessels, leading to various organ disorders. Here, we report a case of posterior scleritis with AAV successfully treated with prednisolone and rituximab (RTX) combination therapy. OBSERVATIONS: A 69-year-old female suffered from ocular pain and redness in her left eye for 2.5 years. She had been diagnosed with idiopathic otitis media before a year. At her initial visit, scleral injection with nodular elevated scleral lesions, vitreous haze, and serous retinal detachment (SRD) in the inferior periphery were observed in the left eye. Enhanced computed tomography revealed the enhancement and thickening of the left sclera. The results of laboratory analysis were positive for myeloperoxidase ANCA. Accordingly, she was diagnosed with AAV. Owing to the exacerbation of vitreous haze and SRD, topical treatment and steroid pulse therapy were initiated. Following therapy, anterior and posterior scleritis improved, and additional RTX was administered to maintain the remission. Following treatment, the patient has maintained remission with 10 mg/day prednisolone to date. CONCLUSIONS AND IMPORTANCE: We encountered a case of posterior scleritis with AAV in which inflammatory manifestations subsided with RTX and glucocorticoid combination therapy. RTX administration likely contributed to the maintenance of remission.

A case of Epstein-Barr virus acute retinal necrosis successfully treated with foscarnet.

PURPOSE: Epstein-Barr virus (EBV) is a herpes virus known to cause infectious mononucleosis and several other human disorders. Ocular EBV infections that have been reported include uveitis, retinal vasculitis, and acute retinal necrosis (ARN). ARN is usually caused by herpes simplex virus (HSV) or varicella-zoster virus (VZV). ARN that is caused by EBV (EBV-ARN) is rarely seen, and only a few cases have been reported. The visual prognosis for EBV-ARN is poor, and no treatment strategy has been established. We report on a patient who was treated successfully for EBV-ARN. OBSERVATION: An 80-year-old female who had been treated with prednisolone at 5 mg/day and methotrexate at 2 mg/week for rheumatoid arthritis visited our hospital because of blurred vision in her left eye. Her left visual acuity was 20/50, and extensive white-yellowish retinal lesions at the temporal periphery with retinal hemorrhages were seen through vitreous haze. The DNA sequence of EBV, but not of HSV, VZV, or cytomegalovirus, was detected by a polymerase chain reaction (PCR) assay in the aqueous humor (4.2 × 106 copies/ml), with EBV also being positive in serum (3.5 × 102 copies/ml). The patient received 2 mg of intravitreal ganciclovir injections twice with a 3-day interval and intravenous infusion of acyclovir at 750 mg/day for 7 days; however, the retinal white lesions expanded rapidly, then dose of prednisolone was increased (40 mg/day) and vitrectomy was performed 10 days after the initial visit. After the surgery, the retinal lesion continued to enlarge. Vitreous samples showed high copies of EBV (1.2 × 108 copies/ml). Following treatment with intravenous foscarnet (4800 mg/day), which replaced the acyclovir application, the retinal white lesions gradually diminished, leaving retinal scars. To date, the patient has developed no retinal detachment and shows visual acuity over 6/60 in the left eye along with silicone oil. CONCLUSIONS: We experienced a case of EBV-ARN that was refractory to systemic acyclovir and topical ganciclovir but responded effectively to systemic foscarnet after vitrectomy. Although the clinical management remains challenging in this disease, foscarnet is considered to be one of the candidate drugs for EBV infections.