Autopsy Case of Bilateral Optic Nerve Aplasia with Microphthalmia: Neural Retina Formation Is Required for the Coordinated Development of Ocular Tissues.

Human congenital anomalies provide information that contributes to the understanding of developmental mechanisms. Here we report bilateral optic nerve aplasia (ONA) with microphthalmia in the autopsy of the cadaver of a 70-year-old Japanese female. The gross anatomical inspection of the brain showed a cotton thread-like cord in the presumed location of the optic nerve tract or chiasm. Histologically, no neural retina, optic nerve bundle or retinal central vessels were formed in the eye globe, and the retinal pigment cells formed rosettes. The cornea, iris, and lens were also histologically abnormal. Immunohistochemically, no retinal cells expressed beta III tubulin, and Pax6- immunoreactive cells were present in the ciliary non-pigmented epithelial cells. This case of ONA could be attributed to the agenesis of retinal projection neurons as a sequel to the disruption of neural retina development. The neural retina formation would coordinate the proper development of ocular tissues.

Flap suturing endonasal dacryocystorhinostomy assisted by ultrasonic bone aspirator.

BACKGROUND: In the external dacryocystorhinostomy (DCR), a sutured anastomosis technique performed between the nasal mucosal and lacrimal sac flaps reported by Dupuy-Dutemps and Bourguet was the gold standard and was believed to lead to the success of the surgery. However, because of the small working space, a flap suturing technique has not been completely established in endonasal DCR (END-DCR). OBJECTIVES: The effect of the modified flap suture anastomosis technique using a Sonopet ultrasonic bone aspirator was retrospectively compared to that using a diamond burr in patients with nasolacrimal duct obstruction. MATERIALS AND METHODS: One hundred ten patients underwent the modified flap suturing technique using the Sonopet, and 30 patients were operated on using a diamond burr. RESULTS: Successful patency of the lacrimal ostium (LO) was obtained in all patients in both groups. The rates of successful suturing during the operation and of a large diameter of the LO 3 months after the operation were significantly higher in patients in whom the Sonopet rather than the burr was used. CONCLUSIONS AND SIGNIFICANCE: The Sonopet might offer similar surgical outcome to the traditional microdrill DCR and is a safer means of bone removal in END-DCR in the small working space.

Lacrimal Drainage System Obstruction Following Inferior Turbinate Surgery.

Objective: To provide information on the clinical characteristics and management of an uncommon acquired nasolacrimal drainage system obstruction (NLDSO). Methods: A patient was treated with microdebrider-assisted endoscopic marsupialization for a cystic lesion located in the synechiae between the inferior turbinate and the lateral wall of the inferior meatus. Results: A 21-year-old woman suffered from epiphora and purulent discharge 1 week after inferior turbinate surgery. Endoscopy reveled synechiae between the inferior turbinate and the lateral wall of the inferior meatus. Computed tomography-dacrocystography (CT-DCG) showed enlargement of the nasolacrimal duct and cystic accumulation of contrast medium from the lacrimal orifice posteriorly. The patient was treated with nasal endoscopic marsupialization of the cyst and then powered inferior turbinoplasty. Six months after the operation, both endoscopy and CT scan showed a widely patent left inferior meatus and nasolacrimal orifice. Conclusions: We report the first case of post-operative NLDSO following inferior turbinate surgery. Nasal endoscopy and CT-DCG are useful to diagnose the location of a NLDSO. Otorhinolaryngologists should be aware that surgery may lead to the formation of a NLDSO and that endoscopic marsupialization is a curative treatment for these distal-end NLDSO. NLDSOs are caused by synechiae, which are a complication of inferior turbinate surgery.

Reconstructive Surgery of the Upper Eyelid Using the Residual Tarsus after Excision of Sebaceous Gland Carcinoma.

PURPOSE: To report a successful eyelid reconstruction performed using the residual tarsus after excision of a sebaceous gland carcinoma. CASE REPORT: An 86-year-old woman presented with a sebaceous gland carcinoma of the upper eyelid margin. After excision of the tumor, she underwent reconstructive surgery of the superior eyelid performed using the residual tarsus. Because the tumor was localized on the upper lid margin, a tarsus of about 4-5 mm in height remained after the excision; we therefore used the residual tarsus to reconstruct the upper eyelid. No functional or cosmetic problems arose as a result of this method. CONCLUSIONS: Reconstructive surgery of the upper eyelid using the residual tarsus may be a viable option provided that surgeons can ensure a sufficient safety margin, and that more than 4 mm of the tarsus remains.

Analysis of retinal vessels in eyes with retinitis pigmentosa by retinal oximeter.

PURPOSE: To study changes in the oxygen saturation and calibre of retinal vessels in the eyes of patients with retinitis pigmentosa (RP). METHOD: In this cross-sectional study, 63 eyes of 63 patients with RP and 14 eyes of 14 healthy subjects were enrolled and examined at Toyama University Hospital. Retinal oximetry measurements were performed using a spectrophotometric oximetry unit (Oxymap T1, Reykjavik, Iceland), which is coupled to a fundus camera base (TRC-50DX; Topcon Corporation, Tokyo, Japan). Retinal arterioles of 28 eyes and retinal venules of 35 eyes with adequate image quality were selected for analyses. We calculated the residual areas of visual field (I-4e and V-4e isopters) in each eye and determined the correlations between the data of retinal vessels and the areas of visual field in the RP eyes. RESULTS: The mean oxygen saturation of the venules in the RP eyes (60.2 ± 6.5%) was significantly higher than that in healthy eyes (54.6 ± 6.3%, p = 0.0089). The oxygen saturation of the venules in the RP eyes correlated significantly with the residual areas of the visual field of V-4e (r = -0.50; p = 0.0030). The mean calibres of the retinal arterioles (91.1 ± 9.1 µm) and retinal venules (116 ± 13 µm) of the RP eyes were significantly narrower than those of the healthy eyes (115 ± 11 µm and 152 ± 15 µm, respectively, p < 0.01). The retinal arteriolar and venular calibres in the RP eyes correlated with the residual areas of the visual field of V-4e (r = 0.43; p = 0.033 and r = 0.45; p = 0.011, respectively). CONCLUSIONS: The severity of RP was correlated with increased oxygen saturations in the retinal venules and decreased retinal vessel calibres.

Endonasal flap suture-dacryocystorhinostomy (eFS-DCR): a new surgical technique for nasolacrimal duct obstruction (NLDO).

CONCLUSION: Endonasal flap suture-dacryocystorhinostomy (eFS- DCR) is a new and valuable technique for the treatment of nasolacrimal duct obstruction (NLDO). This technique could improve the success rate of endonasal DCR in comparison to external DCR. OBJECTIVE: The standard procedure for NLDO is DCR. DCR can be performed via either an external or endonasal approach. External DCR is now regarded as the gold standard in the treatment of NLDO because of its higher success rate. However, we report a new endonasal surgical technique, eFS-DCR, and assess its efficacy by comparison with the standard endonasal DCR. METHODS: We prospectively investigated a series of 62 consecutive patients with NLDO undergoing endonasal DCR. There were two surgical intervention groups: group 1 (24 patients, 28 sites) undergoing endonasal DCR without eFS; and group 2 (38 patients, 47 sites) undergoing eFS-DCR. Study end points were success rate, the ostium diameter (OD) of the lacrimal sac after DCR, and the duration of stent placement. We compared the two groups on these three points. RESULTS: The success rate was 78.6% (22 of 28 sites) for group 1 (DCR without eFS) and 97.9% (46 of 47 sites) for group 2 (eFS-DCR). The ostium size in patients in group 2 was larger than that in group 1. The median time for the duration of stent placement was 42.5 days for group 1 and 31.5 days for group 2. There was a statistical difference (p < 0.01) in all these parameters between the two groups.

Adaptive optics fundus images of cone photoreceptors in the macula of patients with retinitis pigmentosa.

BACKGROUND: The purpose of this study was to examine cone photoreceptors in the macula of patients with retinitis pigmentosa using an adaptive optics fundus camera and to investigate any correlations between cone photoreceptor density and findings on optical coherence tomography and fundus autofluorescence. METHODS: We examined two patients with typical retinitis pigmentosa who underwent ophthalmological examination, including measurement of visual acuity, and gathering of electroretinographic, optical coherence tomographic, fundus autofluorescent, and adaptive optics fundus images. The cone photoreceptors in the adaptive optics images of the two patients with retinitis pigmentosa and five healthy subjects were analyzed. RESULTS: An abnormal parafoveal ring of high-density fundus autofluorescence was observed in the macula in both patients. The border of the ring corresponded to the border of the external limiting membrane and the inner segment and outer segment line in the optical coherence tomographic images. Cone photoreceptors at the abnormal parafoveal ring were blurred and decreased in the adaptive optics images. The blurred area corresponded to the abnormal parafoveal ring in the fundus autofluorescence images. Cone densities were low at the blurred areas and at the nasal and temporal retina along a line from the fovea compared with those of healthy controls. The results for cone spacing and Voronoi domains in the macula corresponded with those for the cone densities. CONCLUSION: Cone densities were heavily decreased in the macula, especially at the parafoveal ring on high-density fundus autofluorescence in both patients with retinitis pigmentosa. Adaptive optics images enabled us to observe in vivo changes in the cone photoreceptors of patients with retinitis pigmentosa, which corresponded to changes in the optical coherence tomographic and fundus autofluorescence images.

Treatment of macular edema due to branch retinal vein occlusion with single or multiple intravitreal injections of bevacizumab.

PURPOSE: We examined the predictive factors for final visual acuity (VA) with macular edema of branch retinal vein occlusion (BRVO) treated by intravitreal injection of bevacizumab (IVB) and examined the differences between patients without recurrent macular edema due to BRVO after a single IVB and patients treated with multiple IVB because of recurrent macular edema. METHODS: In this retrospective study, 37 eyes of 37 patients with BRVO were treated with IVB and followed up for more than 24 weeks. Eighteen eyes showed no recurrence of macular edema after a single IVB (single IVB group). The remaining 19 eyes showed recurrent macular edema and underwent multiple IVB (multiple IVB group). VA and morphologic parameters of optical coherence tomography were examined. RESULTS: Mean VA, central retinal thickness, and mean retinal thickness in a circular region of 1-mm diameter at the fovea improved significantly with IVB treatment in both groups. Final VA was correlated with baseline VA and integrity grade of the photoreceptor inner and outer segment (IS/OS) line beneath the fovea. CONCLUSION: Baseline VA and IS/OS line grade at 4 weeks may be predictive factors for final VA.

One-year results of photodynamic therapy combined with intravitreal ranibizumab for exudative age-related macular degeneration.

Purpose. To evaluate the effects of photodynamic therapy (PDT) combined with intravitreal injection of ranibizumab (IVR) for exudative age-related macular degeneration (AMD). Methods. Retrospective case series. Thirty eight eyes of 38 patients with exudative AMD underwent combined therapy consisting first of IVR, followed by PDT within a week and the second IVR at 1 month. All patients were followed up for more than 12 months. The best corrected visual acuity (BCVA) and central macular thickness (CMT) were examined. Results. The mean number of IVR and PDT sessions were 2.9 ± 1.3 and 1.1 ± 0.3, respectively. The mean BCVA and CMT were significantly improved to 0.38 logMAR units (P < 0.01) and 240 µm (P < 0.01) at 12 months, respectively. Thirty-six of 38 eyes (94.8%) improved or maintained BCVA at 12 months. Conclusion. PDT combined with IVR for exudative AMD was effective at improving visual acuity and CMT with a low recurrence rate for 12 months.

Intraoperative changes in idiopathic macular holes by spectral-domain optical coherence tomography.

PURPOSE: To examine anatomical changes in idiopathic macular holes during surgery using handheld spectral-domain optical coherence tomography (SD-OCT). METHODS: Five eyes of 5 patients who underwent surgery for the repair of idiopathic macular holes were examined. The surgery included standard 25-gauge, 3-port pars plana vitrectomy, removal of the internal limiting membrane (ILM), fluid-air exchange, and 20% sulfur hexafluoride tamponade. Intraoperative SD-OCT images of the macular holes were obtained after ILM removal and under fluid-air exchange using a handheld SD-OCT. From SD-OCT images, the macular hole base diameter (MHBD) was measured and compared. RESULTS: All macular holes were successfully closed after the primary surgery. The mean MHBD under fluid-air exchange was significantly smaller than the mean MHBD after ILM removal and the preoperative mean MHBD. In 1 eye with a stage 3 macular hole, SD-OCT images revealed that the inner edges of the macular hole touched each other under fluid-air exchange. CONCLUSION: Fluid-air exchange significantly reduced MHBD during surgery to repair macular holes. Fluid-air exchange may be an important step for macular hole closure as it reduces the base diameter of the macular hole.