Vascularization of the optic pathway. IV. Optic tract and external geniculate body.
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Eye↗
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Optic pathway gliomas.
Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children and infants further complicates selection of therapy. Historically, surgery and radiotherapy have played a primary role in management, however, in the last 15 years, chemotherapy has evolved into the first-line treatment of choice. Nonetheless, chemotherapy frequently fails, but serves to delay implementation of radiotherapy or surgery until the child has progressed neuropsychologically. An overall favorable prognosis for this tumor emphasizes the need for careful selection of therapy. Herein, we review the major features of optic pathway glioma, including epidemiology, pathology, therapeutic interventions, outcome, and treatment sequelae.
Combined Modality Therapy↗
[Analysis of orthograde rapid axonal transport of the optic pathway. Biochemical analysis of adult albino rabbit optic pathway (I)].
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Animals↗
Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force.
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Brain Neoplasms↗
Optic radiation involvement in optic pathway gliomas in neurofibromatosis.
PURPOSE: Optic pathway gliomas (pilocytic astrocytomas) in neurofibromatosis type 1 (NF-1) typically involve some combination of the optic nerves, chiasm, or optic tracts. Involvement of the optic radiations is rare. DESIGN: This paper describes seven patients with NF-1 with gliomas involving the pregeniculate optic pathway in addition to the optic radiations. METHODS: A retrospective database review was made of all patients with NF-1 and optic pathway gliomas seen by one of the authors (G.T.L.) at the Children's Hospital of Philadelphia from July 1993 to October 2001. Patients with involvement of pregeniculate optic pathway and the optic radiations were identified. From November 2001 to February 2003, patients were sought prospectively. Cases were also identified from the practice of another author (M.C.B.) at Arkansas Children's Hospital. RESULTS: Four patients from Children's Hospital of Philadelphia (three of 83 total NF-1/optic pathway gliomas from July 1993 to October 2001 and one prospectively) and three from Arkansas Children's Hospital were identified. Two had expanding mass lesions within the white matter of the temporal or parietal lobes, which were histopathologically demonstrated to be pilocytic astrocytomas. The other five had radiographic involvement of the optic radiations but did not undergo biopsy. In three of the cases the vision was 20/200 or worse in each eye. CONCLUSIONS: Optic pathway gliomas in NF-1 may rarely involve the optic radiations. Optic radiation involvement may signal a more aggressive optic pathway glioma in patients with neurofibromatosis-1.
Child, Preschool↗
Bilateral optic pathway glioma with intracranial calcification: magnetic resonance imaging and magnetic resonance spectroscopy findings.
Optic nerve glioma is the most common primary neoplasm of the optic nerve in childhood. It can extend intracranially along the optic pathway (optic pathway glioma). The lesion tends to present with decreased visual acuity in the affected eye, but can cause additional symptoms when it is large. Local involvement within the orbit can be characterized using CT, but MRI is superior in showing the intracranial extent of the lesion. Intracranial calcification in optic pathway glioma is rare. We present a rare case of optic pathway glioma with calcification in the intracranial component. Also, we describe MR spectroscopy (MRS) findings in this case.
Calcinosis↗
The relationship between the putative optic pathway potential and the electroretinogram.
The putative optic pathway flash visual evoked potential (FVEP) and the electroretinogram (ERG) were recorded consecutively in the lightly anesthetised rat. The purpose was to test the hypothesis that the optic pathway FVEP is only an artifact created by distorted volume-conducted retinal activity. A comparison of the timing of the ERG with that of the optic pathway FVEP confirmed this suspicion. It is shown that there is a close temporal correspondence between individual subcomponents of the optic pathway potential and those of the ERG (i.e., the a-wave, b-wave, and the oscillatory potentials). In addition, it was found that when ERG currents are recorded far field from the vicinity of the optic nerve or tract, they acquire a triphasic positive-negative-positive waveform, thereby heightening the illusion that the optic pathway FVEP is a genuine compound action potential. It is concluded that experimental findings derived from the recording of the optic pathway FVEP must be treated with caution.
Animals↗
[A case of cystic optic glioma involving chiasma and bilateral posterior optic pathway].
A case of cystic optic glioma involving chiasma and bilateral posterior optic pathway was reported. A 26-year-old male was admitted to our hospital complaining of dysarthria and left hemiparesis. CT, MRI revealed a cystic tumor at the right basal ganglia to midbrain, a calcified one at the bilateral optic tract and left temporal to thalamic region, and a small one at the chiasma. Radiotherapy and chemotherapy were performed because anaplastic astrocytoma was suspected after stereotactic biopsy of the tumor at the right basal ganglia. The subsequent MRI showed continuity among the above three lesions to be well defined. About 2 years later, however, enlargement of the cyst, tumor invasion beyond the optic pathway and growth of the chiasmal lesion were noted, and direct surgery to the chiasmal lesion was performed. The chiasma was swollen and grayish soft tumor tissue was partly resected after aspiration of the intrachiasmal cyst. The definitive pathological diagnosis was pilocytic astrocytoma. This case was designated as a peculiar optic glioma in the following respects; the patient was an adult man suffering from dysarthria and left hemiparesis, the tumor involved not only the chiasma and the bilateral optic tract, but also the outside optic pathway and was accompanied by a large cyst.
Adult↗
Optic pathway gliomas in children with and without neurofibromatosis 1.
Optic pathway gliomas represent 2 to 5% of brain tumors in children. Frequently asymptomatic, sometimes they demonstrate rapid growth, causing considerable visual dysfunction, neurologic deficits, and endocrine disturbances. Most optic pathway gliomas are diagnosed in patients with neurofibromatosis 1. Little is known about their natural course; therefore, there are no clear and widely accepted guidelines for their treatment. This study compared the clinical manifestations and natural history of sporadic and neurofibromatosis 1-associated optic pathway gliomas with regard to age at diagnosis, gender, and findings on neurologic, ophthalmologic, and neuroradiologic examinations in 83 children with optic pathway gliomas: 51 children with neurofibromatosis 1 and 32 children without any symptoms or signs of neurofibromatosis 1. A prospective study was performed in 21 patients with neurofibromatosis 1. In the rest of the patients with neurofibromatosis 1 and in 32 children with sporadic tumors, the analysis was carried out retrospectively. There was an increased incidence of females in the group of patients with neurofibromatosis 1 with optic pathway gliomas compared with the entire group of patients with neurofibromatosis 1 remaining for follow-up (P = .013). All optic pathway gliomas were found in children below 10 years of age, slightly earlier in the group without neurofibromatosis 1 (median age 4.6 vs 4.8 years). Children with optic pathway gliomas associated with neurofibromatosis 1 had predominantly multifocal lesions (P = .0001), whereas in the group without neurofibromatosis 1, isolated chiasmal involvement was more common (P = .002). Children with sporadic gliomas had significantly more frequently increased intracranial pressure, decreased visual acuity, and abnormalities of fundus of the eye at the time of diagnosis. The radiologic progression, visual deterioration, and endocrinologic complications were documented on follow-up more commonly in children with sporadic tumors. Our findings support the concept that there is an earlier and more severe clinical presentation of optic pathway gliomas in children with sporadic tumors than in those associated with neurofibromatosis 1.
Adolescent↗
Association of magnetic resonance imaging of anterior optic pathway with glaucomatous visual field damage and optic disc cupping.
PURPOSE: To investigate the association of magnetic resonance imaging (MRI) of anterior optic pathway with glaucomatous visual field damage and optic disc cupping. SUBJECTS AND METHODS: Twenty-three healthy volunteers (controls) and 31 glaucoma patients (14 with primary open angle glaucoma and 17 with normal tension glaucoma) were enrolled. All the participants showed no abnormal signs in their intracranial space and optic tract causing optic nerve atrophy and visual field defect, as confirmed by MRI. Multislice T1-weighted spin-echo imaging was performed in the sagittal plane followed by the coronal plane. MRI enabled the evaluation of the diameter of the optic nerve located in the retro-bulb space and the height of the optic chiasm in an observer-masked fashion. The MRI data were compared with the mean deviation (MD) score of the full threshold static visual field test and the optic cup-disc ratio (C/D ratio). RESULTS: The optic nerve diameter was significantly smaller in glaucoma patients (2.25 +/- 0.33 mm) than in controls (2.47 +/- 0.24 mm) and the height of the optic chiasm was significantly shorter in glaucoma patients (2.12 +/- 0.37 mm) than in controls (2.77 +/- 0.36 mm). The optic nerve diameter showed significant correlation with MD score (r = 0.547, P = 0.001) and C/D ratio (r = 0.407, P = 0.009). These correlations are similar to that between MD score and C/D ratio (r = 0.490, P = 0.001). The height of the optic chiasm showed significant correlation with MD score (r = 0.503, P = 0.01) and low correlation with C/D ratio (r = 0.339, P = 0.113). CONCLUSION: Glaucoma affects the anterior visual pathway anterogradely at least up to the optic chiasm, and these morphologic changes in the anterior visual pathway are correlated with glaucomatous optic nerve damage. MRI of the anterior visual pathway may be a good tool for evaluating glaucomatous damage objectively.
Adult↗
Adult height after cranial irradiation for optic pathway tumors: relationship with neurofibromatosis.
OBJECTIVE: Optic pathway tumors decrease adult height by central precocious puberty (PP) or hypothalamic-pituitary disorders, particularly growth hormone (GH) deficiency caused by the tumor, management of the tumor, or associated neurofibromatosis 1. The objective of this study was to evaluate the respective roles of these factors on disorders and adult height. STUDY DESIGN: Thirty-five patients with optic pathway tumors diagnosed at 6.4 +/- 0.6 years treated by cranial irradiation (30-56 Gy) reached adult height after treatment with GH alone (n = 16), gonadotropin hormone-releasing hormone analogue alone (n = 2), or both (n = 16). RESULTS: Central precocious puberty occurred before irradiation in four cases and after irradiation in 18. Eleven of the 17 patients with neurofibromatosis 1 had PP. Before irradiation, five of 21 patients lacked GH, zero of 21 lacked thyroid-stimulating hormone, and zero of 13 lacked adrenocorticotropin. After irradiation, 35 of 35 lacked GH, 28 of 35 lacked thyroid-stimulating hormone, and six of 35 lacked adrenocorticotropin; puberty was delayed in 15 patients. Adult height was -0.8 +/- 0.2 SD, below target height (0.2 +/- 0.2 SD, P <.0001), similar in patients with and without PP, but lower in those with neurofibromatosis 1 (-1.4 +/- 0.4 SD) than in those without (-0.3 +/- 0.3 SD, P =.04). CONCLUSIONS: Optic pathway tumors cause PP, but cranial irradiation causes most of the other hypothalamic-pituitary disorders. Adult height of patients given GH or gonadotropin hormone-releasing hormone analogue seems to depend on neurofibromatosis 1.
Body Height↗
Prognostic value of the pattern electroretinogram in cases of tumors affecting the optic pathway.
BACKGROUND: Tumors compressing the optic pathway may lead to irreversible loss of vision which may be detected by the pattern electroretinogram (PERG) because of its relation to ganglion cell function. METHODS: Eyes of 19 patients were tested shortly before and 5-10 days after tumor surgery. Visual acuity, the 30-deg visual field and the transient and steady-state pattern reversal ERG were measured. RESULTS: Using patterns of 1.5 x 1.2 deg there was a good correlation between the change of pre- and post-surgical visual performance and most of the pattern ERG amplitudes. For all variables tested--P50, N95- and steady-state amplitude--there was a critical value beyond which the visual outcome could be bad or favorable, whereas patients showing higher amplitudes always remained stable or improved after surgery. CONCLUSION: The positive correlation between pattern ERG amplitudes and the post-surgical outcome in the case of tumors affecting the optic pathway may be helpful in predicting the outcome for these patients.
Adolescent↗
Carboplatin-induced regression of an optic pathway tumor in a child with neurofibromatosis.
Optic pathway tumors are common in children with neurofibromatosis-1 (NF-1). The optimal management of these tumors is unknown, particularly when the optic chiasm and other brain structures are involved. We report the dramatic response to carboplatin in a 10-year-old girl with NF-1 and a progressive optic pathway tumor. Tumor shrinkage was accompanied by striking improvement in visual fields, return of color discrimination, and marked improvement in visual acuity. No significant toxicity was observed. One year following completion of chemotherapy the glioma remains as small or smaller than it was at the conclusion of therapy, and there has been no deterioration of vision. Carboplatin is a promising agent for the treatment of optic pathway tumors in children with NF-1.
Carboplatin↗
Spontaneous involution of optic pathway lesions in neurofibromatosis type 1: serial contrast MR evaluation.
PURPOSE: To evaluate with contrast MR the evolution in size, signal, and contrast enhancement of optic pathway lesions in four patients with neurofibromatosis type 1. METHODS: The four reported patients are children with ages ranging from 21 months to 13 years affected by neurofibromatosis type 1 and optic pathway lesions. No treatment of the optic pathway lesions was carried out in these patients. They have been followed by serial contrast MR. RESULTS: In all patients a change in size, signal, and enhancement of optic pathways lesions was noted with time, and in the last follow-up study a marked reduction in size and enhancement of optic pathway lesions was observed in all cases. CONCLUSIONS: Modification and regression of optic pathway lesions with spontaneous disappearance of the enhancement is demonstrated. This finding could have a crucial influence on the therapeutic approach of the optic pathway lesions.
Adolescent↗
Management of optic pathway and chiasmatic-hypothalamic gliomas in children with radiation therapy.
BACKGROUND AND PURPOSE: Optic pathway and chiasmatic-hypothalamic gliomas are rare childhood tumors. This study presents the experience in management of these tumors with radiation therapy. MATERIALS AND METHODS: Thirty-three children with the diagnosis of optic pathway and chiasmatic-hypothalamic gliomas were treated with radiation therapy from 1973 through 1994 in the Department of Radiation Oncology at Ankara University Faculty of Medicine. Twenty-four children had optic pathway gliomas and nine had chiasmatic-hypothalamic gliomas. Evidence of neurofibromatosis was present in six children. Subtotal resection was performed in 22 children and a biopsy in seven. The most common prescription for total tumor dose was 50 Gy, delivered in 2 Gy daily fractions. Follow-up ranged from 0.5 to 16.1 years (mean, 13.6 years). RESULTS: Overall, progression-free and cause-specific survival probabilities for the entire group were 93%, 82% and 93%, respectively, at 5 years and 79%, 77% and 88%, respectively, at 10 years. Differences in overall, progression-free and cause-specific survival probabilities between optic pathway and chiasmatic-hypothalamic gliomas were not statistically significant. Absence of evidence of neurofibromatosis correlated with significantly better progression-free and cause-specific survival probabilities. CONCLUSION: Radiation therapy is effective in stabilization or improvement of vision and prevention of tumor progression in both optic pathway and chiasmatic-hypothalamic gliomas.
Adolescent↗
Demonstration of the optic pathway in sellar/juxtasellar tumours with visual disturbance on MR imaging.
OBJECTIVE: To prospectively compare the demonstration of the intracranial optic pathway in patients with sellar/juxtasellar tumours and with clinical evidence of visual disturbance using either spoiled gradient recalled acquisition in steady state (SPGR) or conventional spin echo (CSE) T1-weighted imaging. MATERIALS AND METHODS: We studied 108 patients with sellar/juxtasellar tumours presenting visual disturbance. Visualization of the optic pathway (nerves, chiasm, tracts) was compared between CSE T1-weighted coronal image and SPGR coronal image. In 18 patients, SPGR imaging was performed before and after administration of Gd-DTPA and visualization of the optic pathway was compared. RESULTS: On CSE T1-weighted coronal images of 108 patients with visual disturbance, the rates for visualization of the optic nerves, chiasms and tracts were 50%, 77.8% and 89.8% respectively. In contrast, on SPGR coronal image the rates were 80.6%, 96.3% and 92.6% respectively. The rates of visualization of the optic pathway were greater in non-enhanced that in those with enhancement. The rates of visualization in patients with recurrent tumours were less than those in patients with primary tumours. The rate of visualization of optic nerves in patients with meningioma was less than in patients with pituitary adenoma, craniopharyngioma or Rathke cleft cyst. CONCLUSION: The rate of visualization of optic pathway structures on SPGR imaging (without enhancement) is greater than that on CSE T1-weighted imaging. It is important to understand the accurate position of the optic pathways with using new MR modality especially in surgical planning for lesions around the sella turcica.
Adolescent↗
Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1.
Neurofibromatosis-1 (NF-1) is a multisystem disorder presenting with a variety of clinical and imaging manifestations. Neural and non-neural tumours, and unusual benign miscellaneous conditions, separately or combined, are encountered in variable locations. We present a 2(1/2)-year-old boy with NF-1 who demonstrated coexisting optic pathway glioma with involvement of the chiasm and optic nerve, orbital alveolar rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia.
Child, Preschool↗
Ganglioglioma of the optic pathway. A case report.
A case of ganglioglioma of the optic pathway associated with congenital exophthalmos and strabismus is presented. Since the tumor extended from the right optic nerve to the right geniculate body, it was diagnosed as an optic glioma before operation. However, optic nerve biopsy showed that the lesion was a ganglioglioma. Although a literature review yielded two previous cases of ganglioglioma of the optic pathway, this is the first case in which the tumor involved the whole optic pathway.
Adolescent↗