INTRODUCTIONPrimary optic nerve sheath meningiomas (ONSMs) are the most common primary tumours of the optic nerve sheath. The diagnosis and management of ONSMs have changed dramatically in the last decade. In this review article, we discuss the latest information regarding these issues.

MATERIALS AND METHODSReferences for this manuscript were obtained by searching the database PubMed using the phrase "optic nerve sheath meningioma". Approximately 150 articles were identified and reviewed. These articles served as reference sources for other articles, books and chapters on the subject. The results were combined with our personal experience, which includes over 100 cases of ONSMs that have been observed or treated with one or more of the modalities described below.

RESULTSThe diagnosis of ONSM can be suspected in most cases from clinical findings and supported by the results of neuroimaging, obviating tissue biopsy in the majority of cases. Management depends on several factors. Observation may be appropriate in patients with mild or no visual deficit or in whom visual loss is not progressing, whereas stereotactic fractionated radiation therapy has been documented to improve or stabilise vision in progressive or advanced cases. Attempts at excision of ONSMs are associated with a high risk of blindness and should be reserved for the rare case of an anteriorly located, primarily exophytic tumour with focal involvement of the dural sheath. Rare patients with acute visual deterioration may benefit from optic nerve sheath fenestration.

CONCLUSIONThe majority of ONSMs can be suspected on clinical grounds and diagnosed with readily available non-invasive neuroimaging. Stereotactic fractionated radiotherapy is currently the treatment of choice for ONSMs that require therapy.

The most cases with orbital metastases have been reported in patients with a prior established diagnosis of cancer and widespread systemic involvement. However, ocular symptoms can be developed as an initial presentation of cancer in patients without cancer history. We report a case of rapid progression from trochlear nerve palsy to orbital apex syndrome as an initial presentation of advanced gastric cancer.
Diagnosis ; Diplopia ; Humans ; Neoplasm Metastasis ; Optic Nerve Diseases ; Orbit ; Stomach Neoplasms ; Trochlear Nerve Diseases ; Trochlear Nerve

No abstract available.
Diagnosis, Differential ; Female ; Humans ; Middle Aged ; Optic Disk Drusen/*diagnosis ; Optic Nerve Neoplasms/*diagnosis ; Tomography, Optical Coherence

No abstract available.
Diagnosis, Differential ; Female ; Humans ; Middle Aged ; Optic Disk Drusen/*diagnosis ; Optic Nerve Neoplasms/*diagnosis ; Tomography, Optical Coherence

Pilocytic astrocytomas (PAs) are World Heath Organization Grade I tumors and are most common in children. PA calcification is not a common finding and has been reported more frequently in the optic nerve, hypothalamic/thalamus and superficially located cerebral tumors. We present a cerebellar PA in a 3-year-old male patient with cystic components and massive calcification areas. The residual tumor grew rapidly after the first operation, and the patient was operated on again. A histopathological examination revealed polar spongioblastoma-like cells. Massive calcification is not a common feature in PAs and can lead to difficulties in radiological and pathological differential diagnoses.
Astrocytoma* ; Child ; Child, Preschool ; Diagnosis, Differential ; Humans ; Male ; Neoplasm, Residual ; Neoplasms, Neuroepithelial ; Optic Nerve ; Recurrence*

Attention has been called to the sudden expansion of a pituitary tumor by hemorrhage within it, causing sudden loss of vision. Management of the pituitary apoplexy should be regarded as a neurological emergency. The outstanding clinical features of acute pituitary apoplexy were sudden headache, depressed consciousness, opthalmoplegia, meningismus and signs of compression of the optic nerve or chiasm. The authors report two cases of pituitary apoplexy, one was chromophobe adenoma and pathological diagnosis was not obtained in the other. The first case was 45-year-old man admitted to the Department of Neurosurgery, Catholic Medical Center on June 20, 1977, with headache, double vision and impared left side visual acuity. On April 26, 1977, he experienced sudden severe headache and diplopia then became drowsy. The headache and impairment of consciousness improved subsequently. He was admitted to a university hospital following the onset. The work up at the hospital revealed an enlarged sella with hazy dorsum sellae on plain skull films. The carotid angiogram revealed an elevation of the A1 segments bilaterally and cisternal pneumogram revealed an evidence of suprasellar extension of the pituitary tumor. He was advised to have immediate surgery. The patient, however, refused the surgery and noticed the impaired visual acuity and diplopia were persisting. The studies in this hospital revealed marked impairment of left side vision and oculomotor nerve palsy of the left eye. The carotid angiogram and cisternal pneumogram revealed the no evidence of sellar mass or suprasellar extension of the tumor. The second case was 59-year-old woman admitted to the neurosurgical ward on September 30, 1977, after a sudden onset of severe bifrontal headache, vomiting, bilateral ocular pain and drowsy mental state. She was drowsy with panopthalmoplegia of the right side, and spinal fluid was xanthochromic. Skull X-ray, carotid angiogram and air study revealed an evidence of pituitary tumor with minimal suprasellar extension. Removal of pituitary tumor through the trans-sphenoidal approach was performed, and the tumor was proved to be chromophobe adenoma. Panopthalmoplegia was progressively improved postoperatively.
Adenoma, Chromophobe ; Consciousness ; Diagnosis ; Diplopia ; Emergencies ; Female ; Headache ; Hemorrhage ; Humans ; Meningism ; Middle Aged ; Neurosurgery ; Oculomotor Nerve Diseases ; Optic Nerve ; Pituitary Apoplexy* ; Pituitary Neoplasms ; Skull ; Visual Acuity ; Vomiting

152 biopsy proven orbital lesions were seen during 1991-1995. Cystic tumors were the most frequent type of lesions, accounting for 36 cases(23.68%). Secondary tumors accounted for 17 cases(11.18%), lacrimal lesions 14 cases(9.21%), lymphoid tumors 14 cases(9.21%), inflammatory pseudotumors 13 cases(8.55%), peripheral nerve tumors 11 cases(7.24%), lipocytic and myxoid tumors 10 cases(6.58%), vasculogenic tumors 10 cases(6.58%), optic nerve and meningeal tumors 8 cases(5.26%), rhabdomyosarcomas 4 cases (2.63%), osteomas 3 cases (1.97%), metastatic tumors 3 cases (1.97%), histiocytic tumors 2 cases(1.32%), and other lesions 4 cases(2.63%). The most frequent primary orbital tumor was dermoid and most frequent maliganant tumor was maliganant lymphoma. In pediatric group, the most frequent primary orbital tumor was dermoid and most frequent maliganant tumor was rhabdomyosarcoma. Overall the frequency of maligant tumors was about 20%. Also this survey has a certain bias it will con tribute the recent classification and incidence of space-occupying lesions of the orbit in Korea.
Bias (Epidemiology) ; Biopsy ; Classification* ; Dermoid Cyst ; Diagnosis ; Granuloma, Plasma Cell ; Incidence* ; Korea ; Lymphoma ; Meningeal Neoplasms ; Optic Nerve ; Orbit* ; Osteoma ; Peripheral Nervous System Neoplasms ; Rhabdomyosarcoma

A 13-year-old girl with a right intraorbital optic nerve glioma (ONG) was referred to our glaucoma clinic because of uncontrolled intraocular pressure (IOP) in her right eye. The IOP reached as high as 80 mmHg. Several months earlier, she had undergone stereotactic image-guided robotic radiosurgery using the CyberKnife for her ONG; the mass had become smaller after treatment. Her visual acuity was no light perception. Slit lamp examination revealed rubeosis iridis, a swollen pale optic disc, and vitreous hemorrhage. After medical treatment, the IOP decreased to 34 mmHg, and no pain was reported. Although the mass effect of an ONG can cause neovascular glaucoma (NVG), this case shows that stereotactic radiosurgery may also cause NVG, even after reducing the mass of the tumor. Patients who undergo radiosurgery targeting the periocular area should be followed carefully for complications.
Adolescent ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Glaucoma, Neovascular/diagnosis/*etiology/physiopathology ; Glioma/diagnosis/*surgery ; Gonioscopy ; Humans ; Intraocular Pressure ; Magnetic Resonance Imaging ; Optic Nerve Neoplasms/diagnosis/*surgery ; Radiosurgery/*adverse effects

PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18+/-10.5 months, and diagnosis after symptom onset was made at the mean age of 11+/-9.7 months. The mean z score was -3.15+/-1.14 for weight, -0.12+/-1.05 for height, 1.01+/-1.58 for head circumference, and -1.76+/-1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.
Astrocytoma ; Brain Neoplasms ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis ; Diarrhea ; Drug Therapy ; Early Diagnosis ; Failure to Thrive* ; Follow-Up Studies ; Head ; Humans ; Hydrocephalus ; Hypothalamic Diseases ; Infant* ; Optic Nerve Glioma ; Palliative Care ; Retrospective Studies ; Seoul ; Strabismus ; Vomiting ; Weight Gain

PURPOSE: Intracranial lesions may produce ophthalmic symptoms as chief complaints. The purpose of this study is to help ophthalmologists make an earlier diagnosis of the presence of an intracranial lesion. METHODS: The medical records of the patients diagnosed with an intracranial lesion at the department of ophthalmology were reviewed retrospectively. RESULTS: The mean age of 38 patients (Male: Female=18: 20) was 42.2 years. Visual disturbance was the most frequent symptom at presentation (63.2%), and there was strabismus, visual field defects, diplopia, and other complaints. The underlying problem was that for 23 patients, there was an initial misdiagnosis for 10 of them as having a retinal lesion or an optic neuropathy in 10, paralytic strabismus in 9, and there were other misdiagnoses as well. There were afferent pupillary defects in 12 and optic disc abnormalities in 8 patients. The most common type of visual field defects was a bitemporal hemianopsia. The final diagnosis was the presence of a brain tumor in 28, cerebral infarction in 5, carotid-cavernous fistula in 3, aneurysm in one, and histiocytosis in one patient. CONCLUSIONS: The patients that presented with ophthalmologic symptoms were diagnosed as having variable intracranial lesions. Therefore, it is important for the ophthalmologist to pay closer attention so as to discover the specific intracranial lesion through the exam of the pupil, the optic disc, and the visual field test.
Aneurysm ; Brain Neoplasms ; Cerebral Infarction ; Diagnosis ; Diagnostic Errors ; Diplopia ; Fistula ; Hemianopsia ; Histiocytosis ; Humans ; Medical Records ; Ophthalmology* ; Optic Nerve Diseases ; Pituitary Neoplasms ; Pupil ; Pupil Disorders ; Retinaldehyde ; Retrospective Studies ; Strabismus ; Visual Field Tests ; Visual Fields