Malignant astrocytoma of the optic nerve in a child.

Malignant gliomas of optic nerve and chiasm are rare, rapidly fatal neoplasms of adulthood. This report documents the occurrence of a malignant astrocytoma of the optic nerve in an 11-year-old boy who 9 years previously had a cerebellar medulloblastoma treated with surgery and irradiation. This malignant optic nerve glioma followed the same aggressive clinical course as that seen in adults, with death 9 months after diagnosis despite surgery and chemotherapy. Radiation may have been an important factor in the development of this malignant tumor which is almost never seen in the pediatric age group.     

Optico-hypothalamic glioma: an analysis of 16 cases

We reviewed our experience of 16 patients with histologically proven optico-hypothalamic gliomas. They ranged in age from 0.3 to 15 years at the time of diagnosis. Fifteen tumors were located in the optic chiasm, optic nerve, optic tract and/or hypothalamus, while one tumor was confined to one optic nerve. All tumors were classified as low-grade astrocytomas, which were mainly composed of pilocytic astrocytes. No patient had associated neurofibromatosis. The initial treatment for tumors included surgery in 12, radiotherapy in 7, and chemotherapy in 4 patients. After treatment, visual function improved in 3 out of 14, and endocrine function improved in 1 out of 4 evaluable patients. The 5-year actuarial survival rate was 84.0%, and that at 10 years 71.1%. Our experience and the literature indicate that: (1) patients with disease limited to the optic nerve are adequately managed by resection alone; (2) chiasmal-hypothalamic gliomas behave variably, and progressive disease may occur late in the course of the illness; (3) gliomas that arise in patients under 2 years of age and involve the optic chiasm may act aggressively despite their histological benignity; (4) the beneficial effects of radiotherapy occur in about half of the patients; (5) although chemotherapy may be an effective adjuvant treatment modality, it is not an alternative to radiation therapy at present. Both surgery and irradiation therefore offer the best treatment now available for patients with progressive disease.     

Bilateral sphenoorbital hyperostotic meningiomas with proptosis and visual impairment: a therapeutic challenge. Report of three patients and review of the literature

Objective

Bilateral hyperostotic sphenoorbital meningiomas are extremely uncommon. Due to extensive infiltration of the orbits and the frontotemporal skull base, often only a subtotal tumor resection is feasible. Thus far, no treatment algorithms have been suggested for this rare tumor entity. We report on the surgical management of 3 patients.

Methods

All 3 patients underwent a pterional approach for surgical resection. Surgery was performed in two stages, primarily treating the most affected side. Treatment consisted of microsurgical resection of the infiltrated sphenoid wing and orbital walls, intraorbital tumor removal and optic nerve decompression. Orbital wall reconstruction was performed using titanium mesh allografts. Radiation therapy was administered in 1 patient with residual tumor infiltration of the cavernous sinus.

Results

Our series includes 2 women (51 and 68 years old) suffering from simultaneous progressive bilateral loss of vision and proptosis and 1 woman (69 years old) who developed contralateral disease after surgical resection of a hyperostotic sphenoorbital meningioma 16 years earlier. After optic nerve decompression, vision improved in 2 cases after surgery. Initial visual deterioration was observed in 1 case but improved on longterm follow-up. The degree of proptosis was reduced in all treated eyes.

Conclusion

In bilateral hyperostotic sphenoorbital meningiomas we propose staged surgery when clinical and radiological progression is observed. Subtotal tumor resection with the aim of optic nerve decompression and subsequent orbital reconstruction provides satisfactory results. The most affected eye should be treated first. In case of additional cavernous sinus infiltration, focal radiation therapy can be considered.     

立体定向内放疗联合γ-刀治疗复发性颅咽管瘤

目的评价立体定向穿刺引流加内放疗结合γ-刀治疗复发性颅咽管瘤的有效性和安全性。方法回顾性分析26例手术后复发性囊实性颅咽管瘤的治疗经验。对16例肿瘤实体部分靠近视神经、视交又及视束者，先行立体定向穿刺抽吸加核素内放疗，再行γ-刀治疗；对10例肿瘤囊性部分靠近视神经、视交又及视束者，先行γ-刀治疗，再行穿刺抽吸加核素内放疗。结果随访6～42个月，平均24．5个月；临床症状和体征消失10例，改善13例，无变化2例，加重1例。影像学检查结果显示病变消失4例，缩小17例，无变化3例。增大2例；有效控制率为92．3％。结论对复发性囊实性颅咽管瘤联合应用立体定向穿刺抽吸引流、^32P内放疗及γ-刀等治疗手段，具有并发症少，复发率及病死率低等优点。     

视路胶质瘤的诊治分析（附8例报道并文献复习）

目的 探讨视路胶质瘤的诊断及治疗方法。方法 回顾性分析2009~2018年收治的经病理确诊的8例视路胶质瘤的临床资料,并结合相关文献复习,总结视路胶质瘤的诊断、治疗方法。5例首发病人采取经冠状切口单侧额纵裂入路手术治疗,3例外院手术后复发病人接受伽玛刀治疗。结果 5例手术中,肿瘤大部分切除4例,近全切除1例。8例中,毛细胞型星形细胞瘤7例 , 胶质母细胞瘤1例。2例术后视力、视野损害较术前明显改善,2例视力、视野损害同术前。3例复发病人伽玛刀治疗后6~12个月复查示肿瘤明显缩小。8例出院后随访1~3年,改良Rankin量表评分1分6例,2分2例。结论 对于视路胶质瘤,多种影像技术结合可能更利于明确诊断,术中应在护视神经、下丘脑等重要结果的情况下尽量全切肿瘤。大部分视路胶质瘤,综合手术、放化疗能取得较好的效果。     

Friendly fire: neurogenic visual loss from radiation therapy.

The author's experience and review of the medical literature suggest that radiation-induced neurogenic visual loss presents on average 18 months after treatment and usually after cumulative doses of radiation that exceed 50 Gy or single doses to the visual apparatus of greater than 10 Gy. Visual loss may result from lesions of the disc, retrobulbar segment of the optic nerve, optic chiasm, or retrogeniculate pathways. Magnetic resonance imaging, the best means of demonstrating radiation injury to the visual pathway, may show abnormalities before the loss of vision. The second eye may show clinical manifestations of optic neuropathy many months after the diagnosis in the first involved eye. Spontaneous improvement in visual function may rarely occur. Treatment has been disappointing, but if visual dysfunction is detected early, hyperbaric oxygen might be beneficial. The risk of neurogenic visual loss must be factored into the decision to irradiate the brain.     

儿童眶内视神经胶质瘤的手术治疗和随访

目的 探求儿童眶内视神经胶质瘤的手术方法.方法 总结3例15岁以下(分别是1.5岁、3岁和10岁)眼眶内视神经胶质瘤临床症状和体征,肿瘤影像学发现,采用经额底入路打开前颅底(眶顶),切除视神经肿瘤.根据肿瘤切除程度和患儿年龄采用术后放疗.结果 3例患儿视力下降,2例有下凸眼,患侧瞳孔扩大且无光反射.2例肿瘤向颅内生长其视乳头呈苍白色,1例眶内局限生长视乳头呈水肿改变.MRI和CT显示3例均为右侧眶内肿瘤,呈梭状外观,其中2例肿瘤向颅内生长,但没有超过视交叉.经冠状切口额底入路,去除眶顶板,眶内段肿瘤全部切除3例,其中2例向颅内生长部分没有全切除.术后1例10岁患儿行放疗,另2例为3岁以下患儿,未行放疗.3例患儿术后连续随访,最长8年,均没有肿瘤复发.结论 对于局限存眶内段的视神经胶质瘤应予全切除,对4岁以上患儿,如有肿瘤颅内生长,应行术后放疗,患儿可获得长期生存.     

Fractionated radiotherapy for optic nerve sheath meningiomas

Optic nerve sheath meningiomas (ONSM) are rare tumors of the meninges that surround the optic nerve as it enters the orbit. If left untreated, these benign tumors lead to progressive compression of the optic nerve and vascular compromise resulting in visual loss. Surgical resection of ONSM is associated with a high morbidity due to injury to the vascular supply of the optic nerve, with up to 94% of patients reporting worsened vision post-operatively. Fractionated radiotherapy is a non-invasive alternative to surgery for the treatment of ONSM that has demonstrated improved outcomes. The reported long-term tumor control rates approach 100%, with greater than 80% vision preservation or improvement after treatment. Recently, improved technology for delivery of radiotherapy, including stereotactic and three-dimensional conformal radiotherapy has emerged. The literature suggests that the modality of radiotherapy does not affect the outcomes as long as conformal targeting with a total dose of 50 Gy to 54 Gy and a fractional dose of less than 2.0 Gy is used. Radiosurgery is not generally used for ONSM due to the high toxicity to the optic nerve when high-dose single fraction radiation is given. Therefore, conformal fractionated radiotherapy appears to be the most effective treatment for ONSM, and should be used as a primary therapy unless there is a specific indication for surgical intervention.     

Solitary intracranial extra-osseous plasmacytoma presenting with ophthalmic signs.

Solitary plasmacytomas rarely develop in the skull, meninges, or brain. Ophthalmic signs as the initial manifestations of solitary intracranial plasmacytoma have rarely been described. We report the neuro-ophthalmologic, imaging, and pathologic findings for two patients. One patient presented with optic neuropathy, the second with bilateral sixth nerve palsies. Plasmacytoma is a treatable intracranial tumor that should be considered in the differential diagnosis of patients who present with optic neuropathy or sixth nerve palsy.     

Efficacy and complications of radiotherapy of anterior visual pathway tumors

A progressive disturbance in visual acuity or visual field, along with an unexplained optic nerve atrophy, suggests the possibility of a tumor. Tumors that frequently affect the anterior visual pathway include primary optic nerve sheath meningiomas, intracranial meningiomas, optic gliomas, pituitary tumors, and craniopharyngiomas. The location of these tumors sometimes prohibits a complete surgical excision that might jeopardize the visual system. Radiation therapy, however, can be beneficial in these cases. This article reviews the indications for radiotherapy of tumors that involve the anterior visual pathway, along with the possible complications. Cases that present the effect of radiation therapy and radiation damage are presented.     

Delayed Radiation Injury to the Optic Nerves

Until recently, diagnosis of delayed radiation-induced injury to the optic nerves was based only on a visual deficit detected after cranial irradiation and an absence of direct optic nerve involvement by tumor. Now contrast-enhanced magnetic resonance imaging (MRI) can demonstrate breakdown of the bloodbrain barrier in the optic nerves to provide stronger evidence for the diagnosis. This case illustrates that the MRI abnormality, and abnormalities of the ocular fundus, may precede visual symptoms. More precisely delivered radiation may prevent this complication.     

A Long-Term Survival Case of a Primary Malignant Intracerebral Nerve Sheath Tumor

We report a long-term survival case of a primary malignant intracerebral nerve sheath tumor (MINST) occurring in the right frontal lobe of a 13-year old boy. After the gross total resection (GTR), we have performed radiation therapy but it recurred 50 months after the surgery, so the second GTR was performed. Later, second tumor recurrence was found 4 months after the second surgery. Subsequently the third GTR, radiotherapy, and chemotherapy were carried out. At present, the patient has been remaining alive for 77 months without evidence of tumor recurrence. According to the previous reports, the primary MINST is very rare : there are only 8 cases reported. It is also a fast-growing, invasive tumor with poor outcome. This is the first case that had no recurrence for 50 months after the surgery among the reported cases that had been followed up for more than 5 years. It is supposed that a period of recurrence free survival after GTR and low mitotic activity are associated with the patient''s prognosis. A GTR followed by adjuvant radiation therapy and chemotherapy will be recommended to patients of MINST.     

Lack of radiation optic neuropathy in 72 patients treated for pituitary adenoma.

The incidence of radiation optic neuropathy (RON) after external photon beam radiation therapy for nonfunctioning pituitary adenoma (NFA) is not well-studied. Retrospective review of ophthalmological and imaging data in 72 patients with NFA treated between 1985 and 1998 with external beam radiation therapy after surgery Clinical follow-up after radiation therapy had to be at least 18 months. RON was defined as a sudden and profound irreversible visual loss affecting the optic nerve or chiasm A review of previously published cases of RON was then performed. In our cohort, no patient had RON. A total of 11 adequately documented series reports of RON were found in the medical literature on radiation-treated NFAs. The incidence of RON in NFA from these series is 0.53% (95% CI, 0.26%-0.96%). An additional 14 single RON cases have been reported, bringing the total of adequately documented RON cases to 25. RON is a rare complication after external beam radiation therapy for NFA.     

Optic nerve enhancement in hypotensive ischemic optic neuropathy.

A 57-year-old woman who had hypotension and cardiac arrest during coronary artery bypass grafting developed hypotensive ischemic optic neuropathy with no light perception vision OU. Bilateral mid-orbital optic nerve enhancement was found on magnetic resonance imaging (MRI) eight weeks after surgery. Re-examination 16 weeks after surgery showed no light perception vision, dilated un-reactive pupils, and pale optic discs. Bilateral optic nerve enhancement persisted on MRI. Optic nerve enhancement has been reported commonly in radiation-induced ischemic optic neuropathy, occasionally in arteritic ischemic optic neuropathy, and rarely in nonarteritic ischemic optic neuropathy. It has never been reported in hypotensive ischemic optic neuropathy.     

Therapeutic effect of naloxone on radiation optic neurapathy★

BACKGROUND: Radiation therapy is widely used to treat tumor of brain; however, irradiation of radiation into eye tissues may easily cause ischemia and hypoxia in retina and optic nerve tissue so as to induce radiation optic neurapathy. Noloxone is a specific antagonist of opiate receptor, and it can change injured effect of β-endorphin. OBJECTIVE: To observe the axoplasmic transport of optic nerve at various phases after radiation injury so as to investigate the mechanism and regularity of optic nerve injury; meanwhile, to verify the therapeutic effects of naloxone on radiation optic neurapathy. DESIGN: Randomized controlled animal study. SETTINGS: Medical College of Qingdao University, Changzhi Medical College. MATERIALS: A total of 40 healthy adult New Zealand rabbits, weighing 2–2.5 kg, of either gender, were checked by using slit lamp and ophthalmoscop before radiation in order to exclude eye diseases. FCC-7000 vertical kilocurie 60Co therapeutic machine was made in Yantai, China; in addition, naloxone was provided by Beijing Sihuan Pharmaceutical Factory. METHODS: The experiment was carried out in the Animal Experimental Laboratory, Medical College of Qingdao University from January 2005 to December 2006. The experimental rabbits were randomly divided into radiation group (n =16), treatment group (n =16), blank control group (n =4) and injured control group (n =4). Except blank control group, rabbits in other three groups were irradiated in as the center of optic chiasma including the area of optic nerve by using 60Co therapeutic machine. Radioactive source was 85 cm away from head, and the size of operative field was 5 cm×5 cm. The radiation was performed once a day with the dosage of 3 Gy for 8 days in total. The total dosage was 24 Gy. When radioactive dosage reached 24 Gy, 2 mg/kg naloxone was dropped into ear vein of rabbits in the treatment group once a day for 10 days in total. Rabbits in the injured control group were only irradiated but not given any drugs. MAIN OUTCOME MEASURES: At 1, 10, 30 and 60 days after 24-Gy radiation, anterogradely labeled horseradish peroxidase (HRP) was used to measure optical density mean (OPTDM) in the radiation group and the treatment group; while, OPTDM was directly measured in the blank control group, and OPTDM was directly measured after radiation in the injured control group. RESULTS: All 40 experimental rabbits were involved in the final analysis. There were significant differences in OPTDM at various phases between radiation group and blank control group (P < 0.05); in addition, there was significant difference in OPTDM in the treatment group at 1, 10, 30 and 60 days after 24-Gy radiation (P < 0.05); otherwise, at 30 and 60 days after 24-Gy radiation, there was significant difference in OPTDM between radiation group and treatment group (P < 0.05). CONCLUSION: Naloxone may improve optic nerve axoplasmic transport disorder induced by radiation so as to protect optic nerve.     

Ophthalmic artery occlusion secondary to radiation-induced vasculopathy.

A 35-year-old man with neurofibromatosis type 1 (NF1) had a left ophthalmic artery occlusion that caused no light perception OS 28 years after having been treated with external beam radiation therapy for a presumed glioma of the right optic nerve and chiasm. Clinical and imaging findings were consistent with radiation-induced cerebral vasculopathy. This ophthalmic complication has never been reported, despite the common occurrence of severe carotid-ophthalmic artery junction stenosis after radiation in NF1 patients. Even though modern radiation techniques limit collateral damage, this modality should be used with discretion in NF1 patients, given the vulnerability of their immature cerebral vasculature to radiation.     

Optic glioma with progressive occlusion of the aqueduct of Sylvius in monozygotic twins with neurofibromatosis

Monozygotic twins sisters with optic glioma "in mirror image" (one with involvement of the left optic nerve and the other with the right optic nerve) and hydrocephalus secondary to progressive stenosis of the aqueduct have been found in a series of 128 cases below 14 years of age with neurofibromatosis. The optic glioma was diagnosed in each of the twins at 2 years of age. In one twin the tumor involved only the optic nerve but in the other the glioma affected the optic nerve and spread to the homolateral zone of the optic chiasm. First symptoms of hydrocephalus appeared at 8 years and 11 years of age respectively but ventriculo-peritoneal shunting procedures were performed to relieve intracranial hypertension at 11 years and 15 years of age respectively. At 2 years of age both twins had pneumoencephalography which demonstrated normal air passage through the aqueduct and cerebral ventricles of normal size and morphology. Posterior studies with CT-scan demonstrated progressive obstruction of the aqueduct with very slow progression of the hydrocephalus in each twin, although it was not observed simultaneously. The increased intracranial pressure was tolerated for many years in each twin without obvious symptoms which could be attributed to the slow progression of the aqueduct obstruction.     

Rearrangement of the retino-collicular projection after partial optic nerve crush in the adult rat

The establishment of retino-collicular topography is a well-investigated model of axon pathfinding and it was believed that this topography is irreversibly fixed throughout life. We now report that, after partial crush of the adult rat optic nerve, the anterograde transport of intravitreally-injected tracers via axons of surviving retinal ganglion cells (RGC) in all retinal quadrants is confined to the rostro-medial part of the superior colliculus (SC). This indicates that the retino-collicular topography is rearranged after partial crush of the adult rat optic nerve. The reorganization starts in the injured optic nerve where surviving axonal fibres are demyelinized and bundled in the periphery of the optic nerve distal to the crush site. This is followed by a displacement of surviving axons to the medial part of the optic tract (OT) within 2 weeks. The infiltration of macrophages with the subsequent production of tumour necrosis factor-alpha at the lesion site is a prerequisite for the altered retino-collicular projection as blockade of tumour necrosis factor-alpha signalling with the neutralizing antibody Infliximab abolishes reorganization in the SC and lateralization of RGC axons in the optic nerve and OT. This suggests that optic nerve inflammation is necessary for a progressive bundling of surviving RGC axons, probably via clearance of cellular debris which, in turn, may lead to a redistribution of RGC axons to the medial OT and rostro-medial SC.     

Optic neuropathy associated with periostitis in relapsing polychondritis.

Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.     

Retinal ganglion cell damage induced by spontaneous autoimmune optic neuritis in MOG-specific TCR transgenic mice

Multiple sclerosis (MS) and its animal model experimental autoimmune encephalomyelitis (EAE) are marked by inflammatory demyelinating lesions throughout the central nervous system, including optic nerve. Neuronal loss also occurs in EAE, including retinal ganglion cell (RGC) loss in eyes with optic neuritis, but the finding of RGC loss in relation to optic nerve inflammation differs in different EAE settings. Recently, Myelin oligodendrocyte glycoprotein (MOG)-specific TCR transgenic mice were found to develop spontaneous isolated optic neuritis in the absence of EAE. In the current study, the relationship of inflammation to retinal ganglion cell (RGC) loss during isolated optic neuritis is examined. RGCs of MOG-specific TCR transgenic mice were labeled with Flourogold and then treated with pertussis toxin (PT) or observed untreated. At various time points, RGCs were counted, retinas were TUNEL labeled, and optic nerves were examined for inflammatory cell infiltrates. 29% of untreated MOG-specific TCR transgenic mice developed periocular inflammation by 4 months of age, and 32% of optic nerves of TCR transgenic mice had histological lesions in the optic nerve. Incidence of histological optic neuritis was 20% at day 8 following injection of PT and increased to 48% by day 12, and 68% by day 16. In contrast, no RGC loss or TUNEL staining was detected in eyes with optic neuritis until day 12 in the mice injected with PT. A 28% reduction in RGC numbers at day 12 increased to 39% by day 16, and RGC loss of eyes with severe or massive inflammation was significantly higher than that of eyes with mild or moderate inflammation. No RGC loss occurred in TCR transgenic mouse eyes without optic neuritis. The fact that inflammation precedes RGC loss suggests that neuronal loss during optic neuritis occurs secondary to the inflammatory process in isolated optic neuritis.