Recurrent proliferative vitreoretinopathy in a patient with morning glory syndrome and intellectual disability.

PURPOSE: To report a case of morning glory syndrome (MGS) with retinal detachment, in whom unusually severe proliferative vitreoretinopathy (PVR) developed after surgery. OBSERVATIONS: A 6-year-old boy with intellectual disability underwent vitrectomy for retinal detachment associated with MGS in the left eye. Vitrectomy was performed five times. C3F8 gas tamponade was used for the first and second surgeries. However, the retina developed PVR with a nearly 360-degree giant retinal tear after the second surgery. The third surgery required 360-degree retinotomy, followed by short-term perfluoro-n-octane (PFO) tamponade, which was removed ten days later. During the fourth surgery, the retina was found to be flipped over in a funnel-shape on the retinal pigment epithelium under the PFO. Silicone oil (SO) tamponade was used. During the fifth surgery, the retina was flipped over under the SO again. We found that the patient shook his head rapidly and vigorously while crying. CONCLUSIONS AND IMPORTANCE: We speculate that excessive head shaking associated with the patient's intellectual disability induced an unusual shape of the retina under PFO or SO. Although difficult to achieve, postoperative resting seems important in preventing such complications in intellectually disabled patients with retinal detachment.

TEMPORAL INVERTED INTERNAL LIMITING MEMBRANE FLAP TECHNIQUE FOR A MACULAR HOLE PATIENT UNABLE TO MAINTAIN POSTOPERATIVE PRONE POSITIONING.

PURPOSE: To report the surgical technique and efficacy of the temporal inverted internal limiting membrane (ILM) flap technique for a patient with an idiopathic macular hole (MH) who is unable to maintain postoperative prone positioning. METHODS: Case report. RESULTS: A 73-year-old man with a Stage III MH in his left eye was scheduled to undergo surgery. Owing to his inability to maintain postoperative prone positioning for continuous placement of a transdermal bladder catheter after radical cystoprostatectomy to treat urinary bladder cancer, he underwent pars plana vitrectomy combined with the temporal inverted ILM flap technique and intraocular sulfur hexafluoride gas tamponade. The technique included ILM peeling at a temporal area of the macula to create one 2-disk-diameter semicircular ILM flap and inversion of the ILM flap nasally to cover the MH. Optical coherence tomography showed that MH closure started from the top of the MH just beneath the covered ILM flap; the closure process gradually extended toward the bottom of the MH. The well-aligned fovea recovered in 5 weeks postoperatively. The visual acuity was 20/200 preoperatively and improved to 20/50 postoperatively. CONCLUSION: The temporal inverted ILM flap technique, a simple surgery to treat MHs, provides scaffolding for retinal gliosis and may facilitate bridge formation between the walls of the MH beneath the flap. The procedure may be a good option to achieve MH closure without postoperative prone positioning.

Traditional Japanese herbal (kampo) medicines and treatment of ocular diseases: a review.

Herbal medicines have been used clinically in Eastern Asia, and traditional Japanese herbal (Kampo) formulas are approved as ethical drugs. The Kampo formulas are mixtures of the crude extracts of several herbs, each of which contains multiple components. Numerous investigators have reported that some herbal medicines are efficacious for treating several human diseases. We reviewed the literature on traditional herbal medicines and treatment of ocular diseases. Oral Orengedoku-to and Kakkon-to inhibit postoperative uveitis in humans. Oral Goshajinki-gan improved ocular surface disorders in patients with type 1 diabetes mellitus. Oral Hachimijio-gan increased retinal blood flow. Keishi-bukuryo-gan Sho might be associated with vitreoretinopathy in patients with type 2 diabetes mellitus. Oral Hachimijio-gan and Goshajinki-gan delayed lens opacification in rats and mice. Oral Sairei-to, Orengedoku-to, Senkanmeimoku-to, Scutellariae radix extract, Gardeniae fructus extract, topical Liguisticum wallichii rhizoma extract, and intravenous injection of tetramethylpyrazine, baicalin, baicalein, wogonin, and crocetin inhibited some forms of experimental uveitis in rabbits. Topical glycyrrhizinate improved allergic conjunctivitis in humans and rats. Oral crocetin improved eyestrain in humans. Oral berberine diminished experimental uveitis in rats. Baicalein, wogonin, berberine, and berberrubine inhibited in vitro expression of several cytokines in cultured retinal pigment epithelial cells. Some Kampo formulas are efficacious for treating several ocular diseases in humans and animals. Some herbal extracts and their components inhibit some forms of experimental uveitis.

Retinal risks of high-dose ornithine supplements: a review.

We reviewed the literature on ornithine supplementation and related topics. Nutritionists and physicians have reported that ornithine supplementation is useful. Paediatricians and biochemists have reported that ornithine is supplemented for NH(3) detoxification in the hyperornithinaemia-hyperammonaemia-homocitrullinuria (HHH) syndrome. In contrast, ophthalmic researchers have reported retinotoxicity associated with high-dose ornithine. In vivo and in vitro experiments have shown that high concentrations of ornithine or its metabolites are toxic to the retinal pigment epithelial (RPE) cells. Long-term (exceeding a few years) and high concentrations (exceeding 600 µmol/l) of ornithine in the blood induce retinal toxicity in gyrate atrophy of the choroid and retina (GA). Intermittent high levels of ornithine do not lead to retinal lesions. Constant blood ornithine levels between 250 and 600 µmol/l do not induce retinal lesions or cause a very slowly progressive retinal degeneration. Blood ornithine levels below 250 µmol/l do not produce retinal alteration. We concluded that short-term, low-dose or transient high-dose ornithine intake is safe for the retina; its nutritional usefulness and effect on NH(3) detoxification are supported by many researchers, but the effect may be limited; and long-term, high-dose ornithine intake may be risky for the retina. Patients with GA should avoid taking ornithine; amino acid supplementation should be administered carefully for patients with the HHH syndrome, relatives of patients with GA (heterozygotes) and subjects with RPE lesions; and blood ornithine levels and retinal conditions should be evaluated in individuals taking long-term, high-dose ornithine.

Unusual progression of pleomorphic adenoma of the lacrimal gland: case report.

A 72-year-old female complained of acute pain on left eye movement followed by progressive exophthalmos. Neuroimaging revealed a large well-demarcated lesion consisting of solid and cystic parts, as well as bone destruction and hemorrhage, within the left orbital cavity. The preoperative diagnosis was pleomorphic adenoma with or without malignant transformation, or cavernous angioma. En bloc excision including adjacent tissues was planned to resolve the progressive symptoms and to obtain a histological diagnosis. The transcranial route was chosen since tumor invasion to the cranial base was possible. The histological diagnosis was pleomorphic adenoma. Pathological and preoperative radiological examinations indicated that repeated intratumoral hemorrhage had caused the orbital bone destruction and acute orbital pain. Neoplasms should be differentiated from a wide spectrum of other possible pathologies. Accurate clinical diagnosis of neoplasm in the orbital cavity is important for correct therapeutic management. Malignancy is generally suspected if painful and progressive signs and symptoms are associated with an orbital mass lesion. The present case suggests that pleomorphic adenoma should also be considered in the differential diagnosis. The therapeutic strategy for lacrimal gland tumors remains controversial, so a flexible management approach is required.

Concomitant cavernous hemangioma and venous angioma of the orbit.

BACKGROUND: An unusual case of cavernous hemangioma coexisting with venous angioma in the ipsilateral orbit is described. CASE: A 67-year-old woman had a mass in the lower eyelid of her right eye and proptosis. Imaging examinations showed two masses connected to each other in the extraconal space of the right orbit. OBSERVATIONS: Anterior orbitotomy was performed to remove the tumors. Histopathological evaluation of the anteriorly located tumor revealed cavernous hemangioma. The posteriorly located tumor was found to be a venous angioma. Endothelial cells in both tumors showed positive immunostaining for factor VIII-related antigen and smooth muscle actin. Immunoreactivity of smooth muscle actin was more prominent in the interstitium of the posteriorly located tumor. CONCLUSION: Coexistence of cavernous hemangioma and venous angioma in the same area suggests that they are a continuum and of the same origin.

Sclerosing sweat duct carcinoma of the eyelid.

BACKGROUND: Sclerosing sweat duct carcinoma (SSDC) is a rare variant of sweat gland carcinomas. We report a case of SSDC of the eyelid. CASE: A 33-year-old woman presented with a painless, slowly enlarging nodule in her left lower eyelid. Incisional biopsy revealed proliferated ducts lined by two layers of epithelium embedded in a dense fibrous stroma. Cytologic atypia was not evident. Initial histopathological diagnosis was a syringoma or an inflammatory reaction. OBSERVATIONS: Four years after initial biopsy, the mass had enlarged slightly. Madarosis was observed thereafter. The lesion was excised completely with frozen section evaluation of the margins and repaired by a semicircular rotation flap. Microscopically, variable sized tubular structures could be seen invading the deep dermis and muscle. The final diagnosis was SSDC. CONCLUSIONS: This tumor is seldom recognized and is frequently confused with benign syringoma or inflammation. Wide and deep excisional biopsy is necessary for correct diagnosis and successful treatment.

Neuroretinitis associated with cat-scratch disease in Japanese patients.

PURPOSE: To clarify the prevalence and clinical profile of neuroretinitis associated with cat-scratch disease (CSD) in Japan. METHODS: Japanese patients were searched using a computer web site, Japana Centra Revuo Medicina. Location of patients, medical history, systemic and intraocular findings, treatments and visual outcome were examined. RESULTS: We reviewed 15 eyes of 14 patients, including our own case. Incidences of the disease were more common in summer than in winter and presented in the warmer parts of Japan. All patients had been previously healthy and had been exposed to cats or flea bites. Of the 14 patients, 13 (93%) had febrile illness and seven of these (54%) had lymphadenopathy. Uveitis and macular stars were observed in 11 of 15 eyes (73%). Most patients were treated with antibiotics and systemic steroids and visual prognoses were good. CONCLUSIONS: The clinical features of CSD-associated neuroretinitis in Japan are similar to those in other countries. Its distribution may correlate with the prevalence of Bartonella henselae-seropositive cats in Japan.