Reversible hypothalamic dysfunction in optic nerve germinoma.

Primary optic apparatus germ cell tumors are rare. There have been only 6 cases reported in the literature. Although they often disturb the hypothalamus-pituitary-adrenal axis and cause progressive visual loss, the influence of treatment outcomes on hypothalamic autoregulation has never been mentioned. Here, we report a patient with an optic nerve germinoma who presented with reversible visual and hypothalamic dysfunction, and we discuss the possible mechanisms and pathogenesis.

Pathological optic-disc cupping.

PURPOSE OF REVIEW: Pathological optic-disc cupping is most often caused by glaucoma, but may be seen in many less-common neuro-ophthalmic conditions. The goal of this article is to examine a host of entities causing optic-disc cupping, present key differentiating characteristics and pathophysiologies, and outline diagnostic approaches. RECENT FINDINGS: Multiple entities not associated with elevated intraocular pressure or glaucomatous optic-nerve disease may result in pathologic optic-nerve excavation. Even with the photography and imaging of today, it is still difficult for the clinician to accurately diagnose other causes of optic-disc cupping. Up to 20% of patients may be misdiagnosed and treated for glaucoma due to misinterpretation of the optic-disc cupping. Newer forms of imaging including optical coherence tomography may assist the clinician in decision making. A scrutinizing history, close observation of disc appearance, and the vasculature will aid in the diagnosis of glaucoma or other entity of optic-disc cupping. SUMMARY: Optic-disc cupping is a consequence of myriad disorders. Knowledge of the anatomy and vasculature of the disc is quintessential to the understanding of how, why, when, and what type of optic-disc cupping occurs in various conditions. Cupping can be seen with neurological processes, including benign tumors, which are treatable. Patient history, visual fields assessment, and funduscopic findings are the key to unlocking the diagnosis of glaucomatous versus nonglaucomatous optic-disc cupping. As clinicians, we must remain vigilant and receptive to the findings of potentially ominous forms of nonglaucomatous optic-disc cupping.

Pituitary cysts in childhood evaluated by MR imaging.

BACKGROUND AND PURPOSE: Pituitary cysts are common findings on pathologic examination and imaging studies. They are generally considered to be rarer in children than in adults; however, no good data exist to substantiate this opinion. We reviewed MR imaging studies to evaluate the frequency and imaging features of pituitary cysts in children. METHODS: We retrospectively reviewed T1-weighted sagittal images in 341 patients <15 years of age to evaluate for pituitary cysts. Paramagnetic contrast was administered in 86 of the 341 patients. Sagittal or coronal fast spin-echo T2-weighted images were performed in 166 patients. For patients having pituitary cysts, pituitary function was examined by assessing blood levels of pituitary hormones. RESULTS: A cystic pituitary lesion was recognized in 4 patients (1.2%) aged 1-4 years. None of the 4 manifested endocrinologic signs or symptoms or were the results of their laboratory studies abnormal. All the lesions were sharply demarcated and situated just posterior to the anterior pituitary lobe. All were iso- or hypointense compared with the pons on T1-weighted images without contrast enhancement, suggesting a Rathke cleft cyst. MR imaging of a patient with probable low-grade gliomas in the left hypothalamic region and optic chiasma showed complete resolution of a pituitary cyst at a 1-year follow-up study. CONCLUSION: The frequency of pituitary cysts on MR imaging in childhood is almost equal to that of Rathke cleft cysts, as assessed in autopsy studies of subjects aged 10 to 29 years. These cysts are common in children and should be considered, in the absence of signs or symptoms of pituitary dysfunction, as incidental findings.

Presumed optic nerve sheath meningioma diagnosed after complicated retrobulbar anesthesia.

We present a patient who developed acute visual loss and light flashes at retrobulbar anesthesia for cataract extraction. Vision improved only slightly after cataract surgery. Although traumatic optic neuropathy was suspected, the patient was diagnosed one year later with an optic nerve sheath meningioma (ONSM). ONSM is a rare, slow growing, benign tumour with highly variable clinical features. Diagnosis is often delayed. This case report demonstrates the diagnostic difficulty of this tumour.

[Pilocytic astrocytoma and diencephalic syndrome in an adult with neurofibromatosis type 1].

We report a rare case of pilocytic astrocytoma and diencephalic syndrome occurring together in a 30-year-old woman with neurofibromatosis type 1 (NF-1). Diencephalic syndrome included emaciation and somnolence. Gadolinium-enhanced magnetic resonance imaging revealed a brain tumor in the optic chiasmal-hypothalamic region, which invaded the thalamus, brain stem and cerebellum. A biopsy specimen from the chiasmal tumor was compatible with pilocytic astrocytoma. Although she received radiation therapy, she died 22 months after the onset of the disease.

Visual improvement despite radiologically stable disease after treatment with carboplatin in children with progressive low-grade optic/thalamic gliomas.

BACKGROUND: The purpose of this study was to examine the clinical and radiologic response to carboplatin by children with progressive optic/thalamic gliomas. PATIENTS AND METHODS: Between July 1997 and July 1999, 12 consecutive children were treated with monthly carboplatin for progressive optic/thalamic gliomas. RESULTS: Five children have completed 12 cycles of carboplatin and five children are currently receiving treatment. Two children had progressive disease noted both clinically and radiologically. Nine children have stable radiologic disease and one child has had a partial radiologic response to chemotherapy. Eight children have had regular visual assessments. Four children (three with stable radiology and one with a partial radiologic response) have had improvement in their vision. Three children with radiologically stable disease have had no change in vision. One child has had deterioration in vision despite radiologically stable disease. CONCLUSIONS: The results suggest that the clinical response of optic/thalamic gliomas to carboplatin, as measured by visual acuity and visual fields, may be better than predicted by radiologic assessment. These data suggest that a prospective clinical study is warranted of the role of carboplatin in children with progressive optic/thalamic gliomas and visual impairment.