Moyamoya phenomenon after radiation for optic glioma

1993 ◽  
Vol 79 (1) ◽  
pp. 32-35 ◽  
Author(s):  
John R. W. Kestle ◽  
Harold J. Hoffman ◽  
Antonio R. Mock
Keyword(s):  
Moyamoya Disease ◽  
Treatment Plan ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Optic Glioma ◽  
Optic Pathway ◽  
Content Type ◽  
Sick Children ◽  
Moyamoya Phenomenon ◽  
Fine Print

✓ The role of radiotherapy in the management of patients with optic pathway glioma is controversial. In a series of patients with optic pathway glioma treated at The Hospital for Sick Children in Toronto, five children were encountered who developed moyamoya phenomenon after radiotherapy. A retrospective review of the medical records was undertaken in order to assess the relationship between optic pathway glioma, neurofibromatosis type 1 (NF1), radiation therapy, and moyamoya disease. Forty-seven patients with optic pathway glioma were operated on at The Hospital for Sick Children between 1971 and 1990. The moyamoya phenomenon did not occur in any of the 19 patients not receiving radiotherapy. Among the 28 patients who received radiotherapy, five developed moyamoya disease (two of 23 without NF1 and three of five with NF1). There was a statistically significant relationship between radiotherapy and moyamoya disease when the analysis was stratified according to the presence of NF1 (Mantel-Haensel chi-squared test 15.23, p < 0.01). The high incidence of moyamoya disease (three of five cases, or 60%) in patients with NF1 who have undergone radiotherapy suggests a synergistic relationship that should be considered when formulating a treatment plan for NF1 patients with optic pathway glioma.

Optic pathway glioma infiltrating into somatostatinergic pathways in a young boy with gigantism

1994 ◽  
Vol 81 (4) ◽  
pp. 595-600 ◽  
Author(s):  
Thomas J. Manski ◽  
Charles S. Ha worth ◽  
Bertrand J. Duval-Arnould ◽  
Elisabeth J. Rushing
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Resonance Imaging ◽  
Content Type ◽  
Hyperintense Signal ◽  
Immunocytochemical Studies ◽  
Stereotactic Biopsies ◽  
Fine Print

✓ The authors report gigantism in a 16-month-old boy with an extensive optic pathway glioma infiltrating into somatostatinergic pathways, as revealed by magnetic resonance imaging and immunocytochemical studies. Stereotactic biopsies of areas showing hyperintense signal abnormalities on T2-weighted images in and adjacent to the involved visual pathways provided rarely obtained histological correlation of such areas. The patient received chemotherapy, which resulted in reduction of size and signal intensity of the tumor and stabilization of vision and growth velocity.

The surgical treatment of childhood moyamoya disease

1987 ◽  
Vol 66 (5) ◽  
pp. 675-680 ◽  
Author(s):  
Meredith V. Olds ◽  
Robert W. Griebel ◽  
Harold J. Hoffman ◽  
Marilyn Craven ◽  
Sylvester Chuang ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Moyamoya Disease ◽  
Permanent Disability ◽  
Content Type ◽  
The Past ◽  
History Of ◽  
Sick Children ◽  
Progressive Disorder ◽  
Progressive Stage ◽  
Fine Print

✓Moyamoya disease is a progressive disorder, predominantly seen in childhood, that can cause severe permanent disability. The search for effective treatment has largely been unsuccessful in the past, but recent efforts at surgical intervention have shown promising results. The natural history of moyamoya disease, the options for treatment, and a series of patients from the Hospital for Sick Children in Toronto are reviewed. The results of surgical treatment are encouraging and the authors believe that it should be offered to all pediatric patients in the progressive stage of the disease.

scholarly journals Dural Ectasia of the Optic Nerve: A Rare Presentation in Neurofibromatosis Type I

2019 ◽  
Vol 10 (02) ◽  
pp. 349-351
Author(s):  
Minhaj Shaikh ◽  
Pushpinder Khera ◽  
Taruna Yadav ◽  
Pawan Garg
Keyword(s):  
Optic Nerve ◽  
Neurofibromatosis Type ◽  
Optic Chiasm ◽  
Optic Pathway Glioma ◽  
Optic Glioma ◽  
Type I ◽  
Optic Pathway ◽  
Rare Presentation ◽  
Dural Ectasia ◽  
Magnetic Resonance Imaging Mri

ABSTRACTNeurofibromatosis Type 1 (NF-1) is a common neurocutaneous syndrome with a characteristic spectrum of pathologies affecting the optic pathway. Optic pathway glioma and optic nerve meningioma are two such common afflictions of the optic nerve in NF-1. Dural ectasia of the optic nerve also known as optic nerve meningocele is a rare manifestation of optic nerve involvement in NF-1. Magnetic resonance imaging (MRI) is an excellent modality to accurately identify, characterize, delineate, and differentiate dural ectasia of the optic nerve from the commoner lesions such as optic glioma and meningioma in NF-1. We describe a case of a young woman with NF and a large recurrent palpebral neurofibroma. MRI evaluation of the orbits revealed extensive ectasia of the dura lining the cerebrospinal fluid sheath around all the segments of the optic nerve and around the optic chiasm.

Functional stereotactic radiosurgery involving a dedicated linear accelerator and gamma unit: a comparison study

2004 ◽  
pp. 373-380 ◽  
Author(s):  
Timothy D. Solberg ◽  
Steven J. Goetsch ◽  
Michael T. Selch ◽  
William Melega ◽  
Goran Lacan ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Linear Accelerator ◽  
Treatment Plan ◽  
Single Shot ◽  
Submillimeter Range ◽  
Primate Model ◽  
Content Type ◽  
Stereotactic Frame ◽  
Targeting Ability ◽  
Fine Print

Object. The purpose of this work was to investigate the targeting and dosimetric characteristics of a linear accelerator (LINAC) system dedicated for stereotactic radiosurgery compared with those of a commercial gamma knife (GK) unit. Methods. A phantom was rigidly affixed within a Leksell stereotactic frame and axial computerized tomography scans were obtained using an appropriate stereotactic localization device. Treatment plans were performed, film was inserted into a recessed area, and the phantom was positioned and treated according to each treatment plan. In the case of the LINAC system, four 140° arcs, spanning ± 60° of couch rotation, were used. In the case of the GK unit, all 201 sources were left unplugged. Radiation was delivered using 3- and 8-mm LINAC collimators and 4- and 8-mm collimators of the GK unit. Targeting ability was investigated independently on the dedicated LINAC by using a primate model. Measured 50% spot widths for multisource, single-shot radiation exceeded nominal values in all cases by 38 to 70% for the GK unit and 11 to 33% for the LINAC system. Measured offsets were indicative of submillimeter targeting precision on both devices. In primate studies, the appearance of an magnetic resonance imaging—enhancing lesion coincided with the intended target. Conclusions. Radiosurgery performed using the 3-mm collimator of the dedicated LINAC exhibited characteristics that compared favorably with those of a dedicated GK unit. Overall targeting accuracy in the submillimeter range can be achieved, and dose distributions with sharp falloff can be expected for both devices.

Visual changes after gamma knife surgery for optic nerve tumors

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 143-146 ◽  
Author(s):  
Yang Kwon ◽  
Jun Seok Bae ◽  
Jae Myung Kim ◽  
Do Hee Lee ◽  
Soon Young Kim ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Gamma Knife ◽  
Gamma Knife Surgery ◽  
Optic Glioma ◽  
Content Type ◽  
Nerve Sheath ◽  
Optic Nerve Sheath Meningioma ◽  
Optic Nerve Tumors ◽  
Visual Changes ◽  
Fine Print

✓ Tumors involving the optic nerve (optic glioma, optic nerve sheath meningioma) are benign but difficult to treat. Gamma knife surgery (GKS) may be a useful treatment. The authors present data obtained in three such cases and record the effects of GKS.

Optic pathway glioma with intraocular extension in a child with neurofibromatosis type-1

2020 ◽  
Vol 43 (5) ◽  
pp. e179-e181
Author(s):  
F. Palma-Carvajal ◽  
H. González-Valdivia ◽  
J.P. Figueroa-Vercellino ◽  
C. Saavedra-Gutiérrez ◽  
C. Rovira-Zurriaga ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Optic Pathway

scholarly journals High-Dose Intravenous Vitamin C Treatment of a Child with Neurofibromatosis Type 1 and Optic Pathway Glioma: A Case Report

2016 ◽  
Vol 17 ◽  
pp. 774-781 ◽  
Author(s):  
Nina Mikirova ◽  
Ronald Hunnunghake ◽  
Ruth C. Scimeca ◽  
Charles Chinshaw ◽  
Faryal Ali ◽  
...  
Keyword(s):  
Case Report ◽  
Vitamin C ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
High Dose ◽  
Optic Pathway ◽  
Intravenous Vitamin

Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2

2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida
Keyword(s):  
Symptom Onset ◽  
Survival Rates ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Content Type ◽  
Fine Print

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.

Establishment and characterization of a novel malignant astrocytoma cell line derived from a tumor removed in a patient with neurofibromatosis Type 1

2001 ◽  
Vol 94 (2) ◽  
pp. 301-308 ◽  
Author(s):  
Masanori Kurimoto ◽  
Yutaka Hirashima ◽  
Tsuneaki Ogiichi ◽  
Hideo Hamada ◽  
Hironaga Kamiyama ◽  
...  
Keyword(s):  
Cell Line ◽  
Neurofibromatosis Type 1 ◽  
Proliferative Activity ◽  
Neurofibromatosis Type ◽  
Malignant Astrocytoma ◽  
Content Type ◽  
Astrocytoma Cell Line ◽  
Astrocytoma Cell ◽  
Fine Print

Object. Patients with neurofibromatosis Type 1 (NF1) have a predisposition to development of a variety of benign and malignant tumors including neurofibromas, astrocytomas, pheochromocytomas, and malignant peripheral nerve sheath tumors. The availability of an astrocytoma cell line derived from NF1 would be useful in studies in which sporadic astrocytomas could be compared with NF1-derived astrocytomas. In this article the authors describe a novel astrocytoma cell line, TM-31, that they established from a tumor removed in a 42-year-old woman with NF1. Methods. The TM-31 cell line was prepared from a surgical specimen of malignant astrocytoma and was serially subcultured over 250 times throughout a 6-year period without showing any sign of cell senescence. Immunocytochemical analyses demonstrated that TM-31 cells are negative for glial fibrillary acidic protein but positive for vimentin and S-100 protein. The TM-31 cells display little neurofibromin expression when subjected to immunoblotting, indicating that there is an NF1 gene mutation. Polymerase chain reaction—single-strand conformational polymorphism analysis revealed that TM-31 cells harbor a p53 point mutation in exon 7, codon 238. Chemosensitivity testing of TM-31 cells revealed a resistance to 1-(4-amino-2-methyl-5-pyrimidinyl)methyl-3-(2-chloroethyl)-3-nitrosourea, although they are sensitive to cisplatin and etoposide. In addition, TM-31 cells displayed no morphological differentiation after all-transretinoic acid and dibutyryl cyclic adenosine monophosphate treatments. Pharmacological inhibition of farnesyltransferase of the Ras oncoprotein led to decreased proliferative activity and inhibition of anchorage-independent growth of TM-31 cells in soft agar. Conclusions. The TM-31 cell line is an immortalized astrocytoma cell line derived from a tumor obtained in a patient with NF1. Ras activation may be the major event of proliferative activity and of the transformed phenotype of TM-31 cells, and the farnesyltransferase inhibitor may be potentially important as a novel antiproliferative therapy for NF1-derived astrocytomas.

Complications of invasive subdural grid monitoring in children with epilepsy

2003 ◽  
Vol 98 (5) ◽  
pp. 1017-1026 ◽  
Author(s):  
Çagatay Önal ◽  
Hiroshi Otsubo ◽  
Takashi Araki ◽  
Shiro Chitoku ◽  
Ayako Ochi ◽  
...  
Keyword(s):  
Intractable Epilepsy ◽  
Intracerebral Hematoma ◽  
Content Type ◽  
Acceptable Risks ◽  
Grid Monitoring ◽  
Superficial Wound ◽  
Cerebrospinal Fluid Leaks ◽  
Sick Children ◽  
Fine Print

Object. This study was performed to evaluate the complications of invasive subdural grid monitoring during epilepsy surgery in children. Methods. The authors retrospectively reviewed the records of 35 consecutive children with intractable localization-related epilepsy who underwent invasive video electroencephalography (EEG) with subdural grid electrodes at The Hospital for Sick Children between 1996 and 2001. After subdural grid monitoring and identification of the epileptic regions, cortical excisions and/or multiple subpial transections (MSTs) were performed. Complications after these procedures were then categorized as either surgical or neurological. There were 17 male and 18 female patients whose mean age was 11.7 years. The duration of epilepsy before surgery ranged from 2 to 17 years (mean 8.3 years). Fifteen children (43%) had previously undergone surgical procedures for epilepsy. The number of electrodes on the grids ranged from 40 to 117 (mean 95). During invasive video EEG, cerebrospinal fluid leaks occurred in seven patients. Also, cerebral edema (five patients), subdural hematoma (five patients), and intracerebral hematoma (three patients) were observed on postprocedural imaging studies but did not require surgical intervention. Hypertrophic scars on the scalp were observed in nine patients. There were three infections, including one case of osteomyelitis and two superficial wound infections. Blood loss and the amounts of subsequent transfusions correlated directly with the size and number of electrodes on the grids (p < 0.001). Twenty-eight children derived significant benefit from cortical resections and MSTs, with a more than 50% reduction of seizures and a mean follow-up period of 30 months. Conclusions. The results of this study indicate that carefully selected pediatric patients with intractable epilepsy can benefit from subdural invasive monitoring procedures that entail definite but acceptable risks.

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