Vitrectomy for proliferative retinopathy in patient with advanced duchenne muscular dystrophy.

PURPOSE: Retinal vascular abnormalities are rare in patients with Duchenne muscular dystrophy. We present a patient with Duchenne muscular dystrophy who developed severe proliferative retinopathy for which vitrectomy was successfully performed in one eye. METHOD: Case presentation. A 23-year-old Japanese man with Duchenne muscular dystrophy complicated by cardiac and respiratory insufficiency had reduced vision in both eyes. His best-corrected visual acuity was 0.01 in the right eye and hand movements in the left eye. Ophthalmoscopy showed vitreous hemorrhage and proliferative tissue attached to the optic disk bilaterally. Ultrasound echography showed tractional retinal detachment in the left eye. RESULTS: Because general anesthesia was considered to be a high risk, vitrectomy, lensectomy, neovascular membrane removal, endolaser photocoagulation, and silicone oil injection were performed under local anesthesia on the right eye. After removal of the silicone oil and intraocular lens implantation, the best-corrected visual acuity was 0.8 in the right eye. Vitrectomy was performed on the left eye, but the retina could not be attached. CONCLUSION: The etiology of the proliferative retinopathy in our case is not known. Because early treatment has the potential to improve and maintain vision, we recommend periodic fundus examinations in patients with Duchenne muscular dystrophy.

Focal functional and microstructural changes of photoreceptors in eyes with acute zonal occult outer retinopathy.

PURPOSE: Acute zonal occult outer retinopathy (AZOOR) is characterized by an acute zonal loss of outer retinal function with minimal ophthalmoscopic changes in one or both eyes. We present a patient with AZOOR whose ultrastructural and functional findings were followed for 8 months. CASE: A 22-year-old woman developed an acute central scotoma in her right eye. Her best-corrected visual acuity (BCVA) was 0.5 OD and 1.2 OS. The ophthalmoscopic examinations, fluorescein angiography, and full-field electroretinograms (ERGs) were normal in both eyes. The amplitudes of the multifocal ERGs (mfERGs) were attenuated in the area corresponding to the scotoma. Spectral domain optical coherence tomography showed an absence of both the inner and outer segment (IS/OS) line of the photoreceptors and the cone outer segment tip (COST) line between the IS/OS line and the retinal pigment epithelium. These changes were seen in the area corresponding to the scotoma. One month later, the scotoma disappeared and the BCVA improved to 1.2 OD. The mfERGs increased to almost the same amplitude as the fellow eye. The IS/OS line became discernible but the COST line was still absent. The ophthalmological findings of the right macula remained normal during the 11-month follow-up period. CONCLUSIONS: Our findings indicate that the selective loss of the IS/OS and the COST lines is probably the morphological alterations corresponding with the reduced BCVA and the mfERGs in the areas of the visual field defects in the acute phase of AZOOR. But in the recovery phase, only the abnormality of the COST line is a subclinical sign for the disease. These findings should be important in understanding and evaluating the pathological mechanism in other outer retinal diseases.