SUMMARY We treated 2 patients with unilateral acute retinal necrosis in association with uveitis. The disorder began as anterior uveitis, secondary glaucoma, and retinal vasculitis, and then developed into retinal necrosis within a few months. The aetiology was obscure and the appropriate treatment debatable. We also review reports of patients showing the same clinical characteristics, termed Kirisawa-type uveitis and bilateral acute retinal necrosis, and believe the disorders to be identical.

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A 44-year-old woman with atypical gyrate atrophy and iminoglycinuria was described.The serum ornithine level and ornithine-ketoacid transaminase (OKT) activity were both normal. Urinary excretion of proline, hydroxyproline and glycine was markedly increased. This finding, together with the existence of gyrate atrophy with hyperornithinemia due to OKT deficiency, suggests that proline deficiency in the chorioretinal tissues may concern the development of gyrate atrophy.-chorioretinal atrophy; hyperornithinemia; iminoglycinuria; proline Gyrate atrophy is a rare, inherited disease of chorioretinal atrophy. The plasma level of ornithine was found to be increased in affected patients (Simell and Takki 1973), and OKT in cultured skin fibroblasts was deficient in these patients.However, the mechanism of the ocular disturbance is still obscure because other patients with a similar degree of hyperornithinemia have no ocular problems (Gatfield et al. 1975) and there are cases of gyrate atrophy without hyperornithinemia (Jaeger et al. 1979). Recently we had a peculiar case which showed fundus changes similar to gyrate atrophy but lacked hyperornithinemia and in which urinary concentrations of iminoacids and glycine were markedly increased. PATIENT AND METHODSThe patient was a 44-year-old woman whose parents were first cousins, and had a 5-year history of night blindness.She was referred to our University Hospital because of progressive visual disturbances.Ophthalmologic examination revealed myopia, dark blindness, constricted visual fields and chorioretinal atrophy in both eyes. Characteristic lesions resembling gyrate atrophy were noted at the lower periphery (Fig. 1).Serum and urinary amino acid levels were measured on a Hitachi amino acid analyzer (type 835). OKT activity in cultured skin fibroblasts was determined by spectrophotometric methods (Katsunuma et al. 1964). RESULTS AND DISCUSSIONAll of the routine laboratory tests showed normal values. The serum ornithine level was 1.51 mg/100 ml (normal 0.36-1.56 mg/100 ml) and other serum amino acid levels were also within normal range. OKT activity in cultured skin fibroblasts was 178 nmoles/mg protein/hr (normal controls: 182±8 nmoles/mg protein/hr).

We present 2 cases of battered child syndrome with retinal hemorrhage. Case 1 is a 6-year-old boy who was blinded in both eyes following abuse by his mother’s common-law husband. Retinal hemorrhages and optic atrophy were found in both eyes. Case 2 is a 6-month-old boy who developed dyspnea following episodes of abuse by his mother’s common-law husband. Retinal hemorrhages were visible in both fundi. We believe that retinal hemorrhage is the most frequent clinical finding in abused children and its presence should alert the ophthalmologist to suspect battered child syndrome.

A young adult patient suddenly began to have episodes of unconsciousness that each lasted for several hours. Brief episodes of lethargy occurred monthly. Biochemical tests disclosed high levels of ammonia and citrulline in the serum. Brain oedema was suggested by CT scan, and optic disc swelling was observed ophthalmoscopically, indicating papilloedema. He died in coma. Deficient activity of argininosuccinate synthetase in the liver was demonstrated. Papilloedema was confirmed histologically. We believe that papilloedema is a serious sign indicating poor prognosis in late-onset citrullinaemia.