[Frequency of classified forms in endogenous uveitis considering the anatomical location of the inflammation].

PURPOSE: To investigate the frequency of classified forms in endogenous uveitis in Asahikawa Medical College considering the anatomical location of the inflammation. METHOD: 116 new uveitis patients, who visited our hospital between 2001 and 2008, were retrospectively analyzed. We reviewed medical charts of the patients' age at disease onset, unilateral or bilateral uveitis, anatomical location of inflammation and diagnosis of classified form of uveitis. RESULTS: Age ranged from 5 to 83 years and the distribution of age showed two peaks. Sarcoidosis was the most common form accounting for 33% of all uveitis, 40% of bilateral uveitis and 43% of panuveitis. Vogt-Koyanagi-Harada disease and Behçet's disease followed sarcoidosis. In the unilateral uveitis cases, herpes virus infectious uveitis was the most common form followed by Behçet's disease and ulcerative colitis. In anterior uveitis, herpes virus infectious uveitis was the most common form followed by sarcoidosis and HLA-B27-positive anterior uveitis. CONCLUSION: The most common form amang the entire endogenous uveitis patient's in our hospital was sarcoidosis, and it was also the commonest in panuveitis and bilateral uveitis. The high frequency of sarcoidosis was remarkable when compared with other studies from other facilities.

Pluripotent Stem Cell Model of Nakajo-Nishimura Syndrome Untangles Proinflammatory Pathways Mediated by Oxidative Stress.

Nakajo-Nishimura syndrome (NNS) is an immunoproteasome-associated autoinflammatory disorder caused by a mutation of the PSMB8 gene. Although dysfunction of the immunoproteasome causes various cellular stresses attributed to the overproduction of inflammatory cytokines and chemokines in NNS, the underlying mechanisms of the autoinflammation are still largely unknown. To investigate and understand the mechanisms and signal pathways in NNS, we established a panel of isogenic pluripotent stem cell (PSC) lines with PSMB8 mutation. Activity of the immunoproteasome in PSMB8-mutant PSC-derived myeloid cell lines (MT-MLs) was reduced even without stimulation compared with non-mutant-MLs. In addition, MT-MLs showed an overproduction of inflammatory cytokines and chemokines, with elevated reactive oxygen species (ROS) and phosphorylated p38 MAPK levels. Treatment with p38 MAPK inhibitor and antioxidants decreased the abnormal production of cytokines and chemokines. The current PSC model revealed a specific ROS-mediated inflammatory pathway, providing a platform for the discovery of alternative therapeutic options for NNS and related immunoproteasome disorders.

Resection and anterior transposition of the inferior oblique muscle for treatment of inferior rectus muscle hypoplasia with esotropia.

PURPOSE: To report a case of inferior rectus muscle hypoplasia with esotropia, which was treated successfully by resection and anterior transposition of the inferior oblique muscle. OBSERVATIONS: A 1-year-old boy presented with esotropia. He had esotropia of 15-30° and intermittent left hypertropia. At the age of 3 years, the alternate prism cover test showed esotropia of 35Δ and left hypertropia of 25Δ. Magnetic resonance imaging of the orbit revealed left inferior rectus muscle dysgenesis. Strabismus surgery was performed and a hypoplastic left inferior rectus muscle was identified. We performed bilateral medial rectus muscle recession, and resection and anterior transposition of the left inferior oblique muscle. Nine months after the surgery, the patient had esotropia of 8Δ and left hypertropia of 6Δ. CONCLUSIONS AND IMPORTANCE: Resection and anterior transposition of the inferior oblique muscle is useful for hypoplasia of the inferior rectus muscle accompanied by horizontal strabismus.

RETINAL CAVERNOUS HEMANGIOMA DOCUMENTED BY SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY AND CONFOCAL SCANNING LASER OPHTHALMOSCOPE RETRO-MODE IMAGING.

PURPOSE: To report a case of retinal cavernous hemangioma documented by spectral domain optical coherence tomography and retro-mode retinal imaging by confocal scanning laser ophthalmoscope. METHODS: Clinical examination, fluorescein angiography, spectral domain optical coherence tomography, and scanning laser ophthalmoscope retro-mode imaging were performed. RESULTS: Fundus examination of the left eye of an asymptomatic 39-year-old woman showed an irregularly elevated dark red lesion with a cluster of various-sized saccular aneurysms. The retinal cavernous hemangioma lesions were in the upper vascular arcade. A late-phase fluorescein angiography image showed a typical finding, known as fluorescence capping, characterized by incomplete perfusion because of plasma-erythrocyte separation, which was seen in some larger aneurysms. Spectral domain optical coherence tomography showed that various-sized dilated cystic spaces corresponded to the aneurysms. The image showed the horizontal line, which corresponded to the fluorescence capping in some larger aneurysms. Scanning laser ophthalmoscope retro-mode images showed the individual sizes and septum of the aneurysms. The image also showed the characteristic dots-and-lines pattern around the retinal cavernous hemangioma at the corresponding area of inner retinoschisis attributed to the aneurysms without outer retinoschisis on the spectral domain optical coherence tomography images. CONCLUSION: Spectral domain optical coherence tomography and scanning laser ophthalmoscope retro-mode imaging may be useful for evaluating the pathophysiologic features and the full extent of the retinal cavernous hemangioma.

Temporal posttraumatic limited ocular movement with suspected trapdoor fracture.

Trapdoor fractures, or blowout fractures, result from muscle entrapment after orbital floor fractures. The incarcerated muscles may become necrotic because of ischemia; immediate surgery is recommended for symptomatic persistent diplopia or clinical evidence of entrapment. We report a case of spontaneous resolution of diplopia in a patient with a high suspicion of a trapdoor fracture. A 15-year-old girl presented with diplopia after being hit in the eye while playing volleyball. Computed tomography did not show a fractured orbital bone, but the forced duction test was positive when the left eye was pulled forward toward the left. Magnetic resonance imaging was negative for edema and inflammation in the extraocular muscles. With observation only, the diplopia resolved 2 weeks after onset. A negative forced duction test confirmed the resolution. Observation only may be appropriate in cases with posttraumatic limited ocular movement, after imaging has excluded an emergent condition.

Central corneal thickness measurements with Fourier-domain optical coherence tomography versus ultrasonic pachymetry and rotating Scheimpflug camera.

PURPOSE: To compare the accuracy and repeatability of Fourier-domain optical coherence tomography (FD-OCT) with ultrasonic pachymetry (USP) and a rotating Scheimpflug camera for measuring the central corneal thickness (CCT). METHODS: The CCT was measured in 30 subjects (30 normal corneas) by the same examiner using RTVue-100 FD-OCT with an anterior segment adaptor, Pentacam rotating Scheimpflug camera, and SP-2000 USP. Two examiners obtained one FD-OCT measurement from 10 eyes of 5 subjects to assess interexaminer reproducibility. RESULTS: The mean CCT (±SD) measured by FD-OCT, USP, and the Pentacam were 530 ± 33, 544 ± 34, and 552 ± 35 µm, respectively. Significant correlations were found between FD-OCT and USP (r = 0.97; P < 0.0001), FD-OCT and Pentacam (r = 0.97; P < 0.0001), and USP and Pentacam (r = 0.96; P < 0.0001). Pairwise comparisons showed that the FD-OCT CCT measurement was significantly thinner than those of the other 2 methods (P < 0.001 for all comparisons). Regarding intraexaminer repeatability, the intraclass correlation coefficients ranged between 0.97 and 0.98. There was high repeatability of the CCT measurements with all methods. FD-OCT also had high interexaminer reproducibility (intraclass correlation coefficient = 0.98). CONCLUSIONS: RTVue-100 FD-OCT may be a useful alternative for measuring the CCT; however, it significantly underestimates the CCT compared with the USP and the Pentacam with slight differences. Although highly correlated, the measurements are not directly interchangeable in clinical practice.