Nobutsugu Hayashi

Retinal pigment epithelial cell transplantation after subfoveal membranectomy in age-related macular degeneration: Clinicopathologic correlation

PURPOSE To report the histopathology after retinal pigment epithelial cell transplantation and subfoveal membranectomy in age-related macular degeneration. METHODS An 85-year-old white woman with bilateral choroidal neovascularization underwent subfoveal membranectomy combined with transplantation of a sheet of human adult retinal pigment epithelium (retinal pigment epithelium) under the foveal center in the right eye. The patient was immunosuppressed postoperatively with prednisone, cyclosporine, and azathioprine. The patient died from congestive heart failure 114 days after surgery. RESULTS A patch of hyperpigmentation was visible at the transplant site under the foveola after surgery. Mound-like clusters of individual round, large densely pigmented cells were present in the subretinal space and outer retina in this area. There was loss of the photoreceptor outer segments and native retinal pigment epithelium in the center of the transplant bed, with disruption of the outer nuclear layer predominantly over regions of multilayered pigmented cells. Cystic spaces were present in the inner and outer retina. A residual intra-Bruchs membrane component of the original choroidal neovascular complex was present under the transplant site. CONCLUSIONS The transplant site contained clusters of round, pigmented cells that did not form a uniform monolayer in most areas. The morphology at the transplant site is consistent with the lack of visual improvement seen after surgery in this patient.

Indocyanine Green Facilitates Removal of Epiretinal and Internal Limiting Membranes in Myopic Eyes With Retinal Detachment

PURPOSE To describe the use of intravitreal indocyanine green as an aid to identifying epiretinal membranes and internal-limiting membranes during surgery for a retinal detachment resulting from a macular hole. METHODS A 62-year-old man who had a retinal detachment resulting from a macular hole underwent vitrectomy. During the surgery, intravitreal indocyanine green was injected intravitreally. RESULTS The internal-limiting membrane was stained green, but the epiretinal membrane was unstained. Because the epiretinal membrane and internal-limiting membrane were clearly identified, they could be completely removed. The clinical observations of the epiretinal membrane and internal-limiting membrane excised were confirmed by electron microscopy. Successful reattachment was obtained without damage to the retina. CONCLUSION Removal of epiretinal membrane and internal-limiting membrane can be facilitated by using intravitreal indocyanine green during vitrectomy. We recommend further studies to confirm the benefit of this technique.

Giant cell angiofibroma of the orbit and eyelid

PURPOSE To report the clinicopathologic features of a newly recognized tumor, giant cell angiofibroma. DESIGN Observational case series. MAIN OUTCOME MEASURES Clinical and histopathologic features of giant cell angiofibroma. METHODS Light and electron microscopy and immunohistochemistry of five cases of giant cell angiofibroma. RESULTS A total of five patients (4 women and 1 man) are described: two presented with a painless mass in the eyelid, two with a mass in the orbit, and one presented with a conjunctival lesion. All lesions were well demarcated with no capsule and were composed of blood vessels, a patternless spindle-shaped cell proliferation with a solid and pseudovascular appearance, and multinucleated giant cells. Both spindle-shaped and giant tumor cells were intensely positive for CD34 and vimentin. CONCLUSION Giant cell angiofibroma resembles solitary fibrous tumor and giant cell fibroblastoma and should be considered in the differential diagnosis of spindle-cell tumors in the eyelid, orbit, and conjunctiva.

Congenital cystic eye: report of two cases and review of the literature.

A 13-month-old boy and a 2-week-old girl, who were considered to be anophthalmic and who later each developed a cystic lesion in the left orbit with protrusion of the lower eyelid, were studied. The fellow eye in case 1 was subsequently found to be microphthalmic with cyst and was normal in case 2. Histopathologic study of each case revealed a cyst lined externally by dense fibrous connective tissue to which skeletal muscle and adipose tissue were attached. The inner aspect of the cyst was lined by neuroglial tissue, possible immature retinal tissue, and cuboidal epithelium. No fully developed ocular structures or microphthalmos were identified. Fourteen cases of congenital cystic eye, including our cases, have been published in the English-language literature since 1964. We discuss and illustrate the findings in our cases and 10 others in which histopathologic findings were reported. Congenital cystic eye, microphthalmos with cyst, and microphthalmos with cystic teratoma should be suspected in patients with a small or unrecognizable eye and an orbital cystic mass that is detected by palpation or visualization.

Ocular histopathologic study of a patient with the T 8993-G point mutation in Leigh's syndrome.

OBJECTIVE To report the histopathologic findings of eyes from a patient with Leighs syndrome associated with the T 8993-G point mutation in mitochondrial DNA (mtDNA). DESIGN Case report. INTERVENTION A child with hypotonia, developmental delay, persistent lactic acidosis, seizures, and ataxia died of aspiration pneumonia at 15 months of age. Analysis of DNA isolated from blood was positive for the T to G point mutation at position 8993 in mtDNA, and the proportion of mutant genomes was estimated at approximately 95%. The type and distribution of abnormalities seen in the brain at autopsy were consistent with those in patients with Leighs syndrome. MAIN OUTCOME MEASURES The left eye was examined by light microscopy, and segments of the right eye were examined by transmission electron microscopy. Genetic analysis on DNA isolated from blood was performed. RESULTS Thinning of the nerve fiber and ganglion cell layers was present in the nasal aspect of the macula, and mild atrophy of the temporal aspect of the optic nerve head and optic nerve was present. Electron microscopic study disclosed numerous distended mitochondria in all cells, but particularly in the retinal pigment epithelium, nonpigmented ciliary epithelium, and corneal endothelium. CONCLUSION This is a report of the ocular histopathologic findings in Leighs syndrome with the T 8993-G point mutation. The light microscopic findings were similar to those of patients with similar features reported previously. In addition, ultrastructural abnormalities of mitochondria were present.

Ameloblastoma of the mandible metastasizing to the orbit with malignant transformation. A histopathological and immunohistochemical study.

Abstract We report here a case of ameloblastoma of the mandible with multiple local recurrences and metastasis to the orbit. The patient was a 63-year-old Japanese woman with visual disturbance of her right eye. Diagnostic imaging revealed a mass occupying the right orbital apex with partial intracranial involvement. She had been surgically treated for mandibular ameloblastoma 27 years previously, and the tumour had recurred three times in the past 5 years. The orbital tumour and recurrent ameloblastomas were investigated histopathologically and immunohistochemically. The tumour changed in morphology as it recurred, from follicular ameloblastoma without atypia to apparent malignant tumours disclosing undifferentiated or squamoid features. On immunohistochemical analysis, staining for cytokeratin was positive in the squamoid cells but not in the undifferentiated cells. Both histopathologically and immunohistochemically, the orbital tumour was almost identical to the undifferentiated recurrent tumour. The orbital tumour was distinct from the primary site or sites of recurrence of ameloblastoma, and we concluded that the mandibular ameloblastoma underwent malignant transformation with multiple recurrences and finally metastasized to the orbit.

A Giant Cell Angiofibroma Involving 6q

Giant cell angiofibroma, a recently proposed new clinicopathological entity, arises exclusively in the orbit in adults and histologically exhibits features intermediate between those of giant cell fibroblastoma and solitary fibrous tumor. In a typical case of giant cell angiofibroma, abnormalities of chromosome 6 with a common pattern involving 6q13 were detected together with various other chromosomal aberrations. To our knowledge, this is the first reported case of giant cell angiofibroma demonstrating chromosomal abnormalities.

Juvenile Xanthogranuloma Presenting with Unilateral Prominent Nodule of the Eyelid: Report of a Case and Clinicopathological Findings

BackgroundThe purpose of this study is to report and describe the clinical and histopathologic findings in a patient with juvenile xanthogranuloma presenting as a relatively large, solitary, pedunculated mass on the left upper eyelid.CaseA 31-month-old Japanese girl presented with a pedunculated nodular lesion that had been developing since 4 months earlier. Otherwise, no abnormal findings were identified in either eye. In addition, no other abnormalities, including skin eruptions or nodules, were detected anywhere. The lesion was completely excised and examined histopathologically.ObservationsMicroscopic study revealed a histiocytic proliferation accompanied by lymphocytes and occasional plasma cells and eosinophils within the dermis. A moderate number of giant cells of the foreign-body and Touton types were present. In the immunohistochemical analysis, the proliferative histiocytes and giant cells showed positive immunoreactivity for vimentin and macrophage markers and negativity for S-100 protein. The diagnosis of juvenile xanthogranuloma was made. The findings of further detailed ophthalmologic and systemic examinations and evaluations were unremarkable. Therefore, the diagnosis was made of juvenile xanthogranuloma, solitary type, occurring on the eyelid.ConclusionsAlthough juvenile xanthogranuloma of the eyelid is uncommon and its occurrence anywhere in the eye is rare, ophthalmologists should be familiar with this entity. Juvenile xanthogranuloma should be included in the differential diagnosis of eyelid mass lesions.

Ultrastructural analyses of internal limiting membrane excised from highly myopic eyes with myopic traction maculopathy

PurposeTo evaluate the ultrastructure of the internal limiting membranes (ILMs) excised during vitrectomy from highly myopic eyes with myopic traction maculopathy (MTM). The clinical findings before and after the vitrectomy were compared.MethodsSeven eyes of 7 patients with macular retinoschisis were studied. Four of these eyes also had a foveal detachment but without a retinal break. All the eyes underwent vitrectomy with the creation of a posterior vitreous detachment and ILM peeling. The excised ILMs were examined by transmission electron microscopy (TEM).ResultsThe retinas were reattached in all eyes after the vitrectomy. No retinal breaks including macular holes were identified intraoperatively. Transmission electron microscopy showed glial cells in 4 eyes, retinal pigment epithelium-like cells in 4 eyes, and myofibroblast-like cells in 4 eyes on the excised ILMs. A newly produced basement membrane appeared to merge with the ILM in 5 eyes. Thick collagen was seen in 2 eyes, and fibrous long-spacing collagen in the newly synthesized collagen fibers was seen in 3 eyes. The cellular components of the glial cells appeared to have migrated through the thinner parts of the retina or through a defect of the ILM in 2 eyes.ConclusionsCells that migrate onto the surface of the ILM synthesize new collagen, which can create tangential traction. This may explain the success of vitrectomy with ILM peeling in treating MTM in highly myopic eyes.

Wavefront analysis and ultrastructural findings in an eye with posterior lentiglobus

An 8-year-old girl with unilateral posterior lenticoglobus underwent lens aspiration and intraocular lens implantation. Wavefront analysis performed before surgery to assess the surgical indication showed a higher-order aberration of 0.502 μm root mean square. After surgery, the value decreased to 0.132 μm root mean square. Preoperative best-corrected visual acuity of 0.1 improved to 0.3 postoperatively. Electron-microscopic examinations of the posterior capsule revealed unusual nodular structures and variations in capsular density.