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Purpose: The aim of this study was to explore the potential benefits of retinal pigment epithelium replacement therapy in patients with Bietti crystalline dystrophy (BCD) by assessing the disease pathology with the distinctive relationship between fundus autofluorescence (FAF) abnormality and visual field defect., Methods: Sixteen eyes from 16 patients with BCD and 16 eyes from 16 patients with RHO-associated retinitis pigmentosa were included. Fundus autofluorescence, optical coherence tomography, and Goldmann perimetry results were retrospectively reviewed and assessed using image analyses., Results: In patients with BCD, the FAF abnormality area was not correlated with the overall visual field defect area and median overall visual field defect area (57.5%) was smaller than FAF abnormality area (98.5%). By contrast, the ellipsoid zone width was significantly correlated with the central visual field area (r = 0.806, P < 0.001). In patients with RHO-associated retinitis pigmentosa, the FAF abnormality area and ellipsoid zone width were significantly correlated with the overall visual field defect area (r = 0.833, P < 0.001) and central visual field area (r = 0.887, P < 0.001), respectively., Conclusion: The FAF abnormality shown in patients with BCD involves retinal pigment epithelium degeneration without complete loss of photoreceptors or visual function. These results suggest that patients with BCD are good candidates for retinal pigment epithelium replacement therapy for preservation of residual visual function., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Rationale: Relapsing polychondritis (RP) is a rare collagen disease characterized by inflammation and destruction of cartilage throughout the body. The paper details the clinical course of a case of RP with unique circumferential peripheral keratitis., Patient Concerns: A 54-year-old Japanese woman was referred to the hospital presenting with auricular and ocular pain., Diagnoses: Based on the auricle biopsy results and the three presenting symptoms (bilateral auricular chondritis, inflammatory arthritis and ocular inflammation), her condition was diagnosed as RP., Interventions: The three presenting symptoms gradually improved with prednisolone (PSL), methylprednisolone and cyclophosphamide combination therapy, followed by PSL, methotrexate and infliximab combination therapy. However, one month after the initial visit, despite ongoing treatment, a unique circumferential peripheral keratitis suddenly occurred, in which the corneal infiltration gradually clumped together and shrank at the peripheral area. The eye and ear pain showed exacerbations and remissions on reducing the dosage of steroid drugs. The general condition was improved on altering systemic therapy to PSL, methotrexate and tocilizumab., Outcomes: Keratitis gradually disappeared within 10 months of the initial visit., Lessons: This is the first report of a case of RP causing unique circumferential peripheral keratitis. This keratitis occurred despite use of focal and systemic steroids and showed improvement with general recovery. This may indicate that stabilization of general condition is important for recovery from keratitis in RP.

Purpose: To investigate when and how fast the foveal thinning occurs in central serous chorioretinopathy., Methods: Outer nuclear layer (ONL) thickness was measured in 60 eyes of 60 patients with active central serous chorioretinopathy. Patients were divided into 3 groups based on the duration of symptoms; within 1 month (Group A, 25 eyes), 1 to 6 months (Group B, 17 eyes), and >6 months (Group C, 18 eyes). Outer nuclear layer thickness and visual acuity were compared between each group. Some of the patients underwent several examinations and serial changes were analyzed., Results: The ONL thickness was correlated with the duration of symptoms (R = -0.61, P < 0.001). Visual acuity was worse in Group C compared with Group A (P = 0.003). The mean ONL thickness of each group and healthy contralateral eyes was 82.4, 70.2, 53.5, and 89.3 μm, respectively. Comparisons between each group showed statistical significances. Seventeen eyes in Group A were re-examined between 3 and 6 months after the first visit. The mean ONL thickness significantly decreased from 81.9 μm to 75.0 μm (P = 0.020)., Conclusion: In central serous chorioretinopathy, ONL thinning starts in the early stage and may continue as long as the subretinal fluid persists.

Purpose: Rheumatoid arthritis (RA) often presents with ocular complications: typically dry eye, peripheral corneal ulcer, and scleritis. We report for the first time a case of severe scleritis with RA, accompanying atypical continuous keratitis, which apparently differs from typical peripheral ulcerative keratitis (PUK)., Methods: Observational case report., Results: A 68-year-old woman with RA presented at our hospital complaining of worsening arthritis accompanying ocular injection and discharge. On examination, nodular scleritis and peripheral corneal infiltration were noted. In addition to administering topical steroid and antibiotics, cyclosporine and an oral steroid were added because of the patient's worsening scleritis. Despite gradual improvement of the scleritis, the efficacy of the additional treatments was limited. Four months after initial treatment, the patient presented with uveitis, thought to be caused by a herpetic virus. Antivirus treatment was effective for the uveitis, but atypical continuous keratitis suddenly appeared. The keratitis was located from 4-o'clock to 10-o'clock positions continuously in the midperipheral cornea and apparently differed from herpetic keratitis or PUK as typically seen in RA cases. Immune reaction was suspected, and the keratitis improved within 2 weeks. After that, the introduction of an anti-tumor necrosis factor α drug (infliximab) completely resolved the severe scleritis and there was no recurrence of ocular inflammation., Conclusion: As is shown in this case, RA can present with atypical continuous keratitis, thought to be a manifestation of an immunologic reaction other than PUK. In addition, although immunosuppressants are often used for the treatment of RA with scleritis, the efficacy is limited. Infliximab may be a useful treatment for treatment-resistant scleritis.

Background: Endovascular stent-grafting with intentional coverage of the left subclavian artery may be used to treat aortic isthmus injury, but this procedure may have serious neurologic sequelae and may not provide an adequate proximal landing zone. In 2005, in an effort to mitigate these problems, we began to use fenestrated stent-grafts for emergency repair of blunt aortic injury (BAI)., Methods: Between 2005 and 2007, all patients in our practice with a BAI with mediastinal hematoma (except young patients without an associated critical injury) were treated with immediate endovascular stent-grafting, if anatomically possible. A fenestrated stent-graft was placed from the aortic arch, if the BAI was less than 20-mm distal of the left subclavian artery. The records of the 13 patients in the series were reviewed retrospectively., Results: The BAI treatment was successful in all 13 patients. Eight patients (61.5%) were given a fenestrated stent-graft, placed distal to either the ascending aorta (n = 2), brachio-cephalic artery (n = 4), or left common carotid artery (n = 2), without concomitant bypass grafting or transposition of the head vessels. Two patients died of an associated critical brain injury (hospital mortality rate, 15.4%). There were no perioperative complications related to stent-graft usage and no unintentional occlusions of the head vessels by a fenestrated device. One patient underwent open repair of a newly developed type Ia endoleak 7 months after placement of a nonfenestrated stent-graft., Conclusion: Fenestrated stent-grafts can be used to treat BAI, without any concomitant procedures to provide an adequate proximal landing zone.

Background: Clivus epidural hematoma (CEH) is reported to be extremely rare. However, we consider that CEH may not be as rare as commonly thought, and that it may often go unrecognized because of diagnostic problems. The aim of this study was to evaluate the value of multidetector row computed tomography (MDCT) in the diagnosis of CEH., Methods: Twenty-seven children aged less than 16 years who had experienced traffic crashes or falls were retrospectively evaluated with MDCT including sagittal reconstruction of the cervical spine., Results: Sagittal reconstructed images showed definitive findings of CEH in 3 (13%) of the 24 traffic-crash patients., Conclusion: MDCT can show definitive findings of CEH, and diagnostic problems can be solved through the utilization of MDCT. We consider that CEH may be more common than previously thought.

Objective: Percutaneous catheter drainage (PCD) is not always effective in a case of hemopericardium. Acute occlusion of catheter and cardiac perforation can happen more often. To perform subxiphoid pericardiotomy within a minute for emergency cases, we have done this procedure in a blind method after finger dissection by subxiphoid approach. We report the usefulness of blind subxiphoid pericardiotomy (BSP) based on the results of a prospective control study., Methods: We designed a study to determine a favorable management for cardiac tamponade resulting from hemopericardium. In an emergency case of cardiac tamponade because of hemopericardium, board certified surgeons should perform BSP and other emergency physicians should perform PCD, with or without local anesthesia. PCD (n = 67) and BSP (n = 16) were performed for patients with cardio-pulmonary arrest (CPA) or near CPA because of cardiac tamponade secondary to trauma (n = 7), acute aortic dissection (n = 65), and cardiac rupture following acute myocardial infarction (n = 11) in our emergency medical center from January 2000 to December 2004., Results: BSP was effective in all cases but PCD was ineffective in five cases because of clotting in pericardium (p = 0.260). No complication was observed in the BSP group but five critical complications and three infeasible drainage complications were observed in the PCD group (p = 0.146). Ten patients (BSP, 4; PCD, 6; p = 0.077) survived after emergency surgery (n = 8) or conservative treatment (n = 2)., Conclusion: BSP was safe and could be performed quickly in an emergency situation. Percutaneous catheter drainage for hemopericardium could not avoid critical complications because of clotting in pericardium.

The purpose of this study was to investigate a suitable protocol of contrast-enhanced computed tomography (CECT) in cases with a cardiopulmonary support system. Contrast-enhanced computed tomography with intra-arterial injection (IAI) of contrast medium (CM) via a perfusion cannula showed sufficient contrast enhancement in 2 cases of cardiac decompensation (CD). Contrast-enhanced computed tomography with intravenous injection of CM showed insufficient and delayed contrast enhancement of the aorta in 2 cases of CD and 3 cases of pulseless electrical activity. We encourage administration of CM by means of IAI.

Purpose: To review the efficacy of a combination of triamcinolone acetonide (TA) injection and pars plana vitrectomy (PPV) for the treatment of macular edema associated with branch retinal vein occlusion (BRVO)., Methods: Seventeen eyes with macular edema associated with BRVO underwent PPV with an intraoperative injection of TA (10 mg) into the vitreous cavity. Residual or recurrent macular edema was treated with postoperative sub-Tenon capsule injections of TA (20 mg)., Results: With PPV and an intraoperative injection of TA, 82% of eyes showed rapid reduction of macular edema; foveal thickness decreased from 507 +/- 115 microm preoperatively to 261 +/- 123 microm 2 months after surgery (P = 0.0041). However, 59% of eyes showed recurrence of macular edema during the follow-up period. Twelve eyes with residual or recurrent macular edema received sub-Tenon capsule injections of TA; of these eyes, 9 showed substantial reduction of macular edema. Foveal thickness decreased from 381 +/- 102 microm to 256 +/- 56 microm (P = 0.0076) 2 weeks after postoperative injections of TA. At the final visit, visual acuity (logMAR) improved from 0.74 +/- 0.40 preoperatively to 0.40 +/- 0.34 (P = 0.010)., Conclusion: An intraoperative injection of TA in combination with PPV has the potential to facilitate the absorption of macular edema associated with BRVO. In addition, residual or recurrent macular edema can be treated with additional sub-Tenon capsule injections of TA.

Objectives: To report the satisfactory recovery of a woman with acute aortic dissection who underwent cardiopulmonary resuscitation out of hospital and to show that there are still chances of recovery for patients with prehospital cardiopulmonary arrest even due to acute aortic dissection., Design: Case report., Setting: An adult critical care unit of a university hospital., Patients: A 71-yr-old woman who had a cardiopulmonary arrest out of hospital. Cardiopulmonary resuscitation was performed before arriving at the emergency room of our hospital., Interventions: Cardiopulmonary resuscitation, echocardiography, computed tomography, aortography, coronary angiography, ascending aortic graft replacement, and brain hypothermia., Measurement and Main Results: Restoration of spontaneous circulation was gained 45 mins after onset of cardiopulmonary arrest. Echocardiographic and computed tomographic images revealed cardiac tamponade caused by type A acute aortic dissection. Since involuntary movements of the extremities in response to noxious stimulation were observed at 30 mins after restoration of spontaneous circulation, we performed an ascending aortic graft replacement and postoperative mild brain hypothermia. The patient recovered satisfactorily 1 month after the operation., Conclusion: The results of this case indicate that there are still chances of recovery for patients with prehospital cardiopulmonary arrest, even due to acute aortic dissection. We propose that an appearance of involuntary movements of the extremities in response to noxious stimulation is one key point that should be considered when deciding whether to perform an emergency operation for this kind of patient.

Although systemic administration of NO donors has been shown to attenuate the development of neointimal hyperplasia in the balloon injury model, this strategy has not been tested in a model of allograft vasculopathy. In this study, we investigated the effect of FK409, a spontaneous NO releaser, on the development of allograft vasculopathy, using a rat aortic transplant model. Thoracic aortas from ACI rats were transplanted heterotopically into the abdominal aorta of Wistar-Furth rats. Postoperatively, recipients received FK409 orally every 8 hours from the day of transplantation to the time of euthanization. Morphometric and immunohistochemical analyses were performed on the aortic grafts 8 weeks after transplantation. Control allografts showed severe neointimal hyperplasia, which consists mainly of alpha-actin-containing vascular smooth muscle cells. The FK409-treated allografts showed a dose-dependent reduction (statistically significant compared with the control) in the neointimal thickness as the dose increased from 1 to 10 mg/kg (thrice per day). However, there was no significant difference in the neointimal thickness between groups treated with 10 and with 20 mg/kg. FK409 treatment (10 mg/kg) caused a significant decrease in DNA synthesis (5-bromo-2-deoxyuridine [BrdU] uptake), an increase in DNA fragmentation (terminal deoxynucleotidyltransferase-mediated uridine nick-end labeling [TUNEL]), and upregulation of Fas expression, in the neointimal vascular smooth muscle cells. These data suggest that FK409 attenuates the allograft vasculopathy in a rat aortic transplant model.

We had obtained some immunohistochemical indications which suggested that peptide(s) containing GABA at the C-terminal could exist in specific neurons in brains. We therefore sought such GABA conjugate(s) by isolating an acidic GABA-dipeptide from bovine brains and determined its structure as gamma-L-glutamyl-gamma-aminobutyric acid (gamma-L-Glu-gamma Abu:GluGABA). Its gamma-linkage was determined by a mass-spectrometric (B/E linked scan SIMS) analysis; its chirality was established by a chiral column HPLC technique. This peptide is the first acidic GABA-peptide recognized in brain and the first brain peptide containing GABA at the C-terminal.