侧脑室三角区脑膜瘤的眼部表现及视放射损害的相关因素分析

目的：分析侧脑室三角区脑膜瘤患者的眼部表现特点,探讨导致视功能损害的可能相关因素,以及在临床诊治中的注意事项。方法：回顾分析2011－10／2015－10在首都医科大学附属北京天坛医院诊治的45例90眼侧脑室三角区脑膜瘤患者病例资料,对患者术前视力、视野、视乳头改变、肿瘤大小、肿瘤周围组织变化等进行分析。结果：患者年龄12～68（平均41．7±13．7）岁,男女比例1∶4．6;视力下降18眼,视乳头水肿24眼,视乳头色淡6眼,视野缺损47眼,其中视野缺损类型以双眼对侧同向性缺损为主;肿瘤最大径2．1～9．6（平均4．8±1．7）cm;肿瘤体积3．02～193．2（平均48．3±47．8）cm3。 Spearman分析显示肿瘤体积、肿瘤最大径和脑中线结构移位与术前出现视野缺损呈正相关。而患者年龄、性别、病程、脑室是否扩大、肿瘤周围组织是否水肿与术前出现视野缺损无明显相关性。经Mann－Whitney U检验,术前视野受损组和视野正常组之间肿瘤体积、肿瘤最大径、脑中线移位值均有显著性差异。结论：较多三角区脑膜瘤患者存在眼部症状和体征,患者视功能损害的原因主要是由于肿瘤对周围视放射的破坏导致。肿瘤大小、生长方向、对周围视放射压迫的具体位置决定了患者是否出现视野缺损以及视野缺损的类型和程度。对于靠近视放射等后视路的颅脑占位,眼科医生和神经外科医生要重视其神经眼科学检查,注意肿瘤与周围视路等功能区的位置关系,这对于手术时机选择、手术方案制定及改善预后具有重要意义。     

The optic nerve pathology in magnetic resonance imaging

Magnetic resonance imaging (MRI) delineates the orbital soft tissue excellently and is a valuable diagnostic tool in optic nerve pathology. By using different imaging parameters some examples are shown of demarcation of optic nerve tumors, optic nerve inflammations and orbital diseases surrounding the optic nerve. Whereas CT appears to be superior to MRI only in the detection of calcified perioptic meningiomas, MRI provides unique visualization of optic canal and even subarachnoidal space of retrobulbar optic nerve in peculiar cases.     

Zur Computer-Tomographischen Differentialdiagnose der Erkrankungen des Sehnerven

Zusammenfassung Die Computer-Tomogramme von 166 Patienten mit Sehnervenläsionen werden hinsichtlich der Kriterien Verlauf, Dicke, Dichte und Abgrenzbarkeit des Sehnerven, orbitaler und intrakranieller Weichteilveränderungen sowie knöcherner Veränderungen im Bereich des Canalis opticus untersucht. Die charakteristischen computer-tomographischen Merkmale der einzelnen Krankheitseinheiten wie der Optikus-Gliome, Optikusscheiden-Meningiome, neoplastischer und entzündlicher Infiltrationen etc. werden herausgestellt.Die differentialdiagnostische Bedeutung der einzelnen computer-tomographischen Kriterien wird untersucht und diskutiert.Die meisten Sehnervenläsionen lassen sich auf Grund computer-tomographischer Kriterien gut lokalisieren und identifizieren. Die häufigsten Tumoren des Sehnerven lassen sich unterscheiden. Bei der Neuritis nervi optici im Rahmen einer klinisch gesicherten Multiplen Sklerose und bei totalen Optikusatrophien finden sich keine Kaliberveränderungen des Sehnerven. Die Darstellung des intraorbitalen Sehnerven erfordert die Anwendung besonderer Untersuchungstechniken. Gute Darstellungen des knöchernen Sehnervenkanals lassen sich durch schräge Computer-Einstellungen 90° zum Canalis opticus erzielen. Der Ausschluß einer intrakraniellen Ursache einer Sehnervenläsion erfordert eine intravenöse Kontrastmittelgabe.

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Computed tomograms of 166 optic nerve lesions were analyzed: 97 were mainly orbital and 69 mainly intracranial. The criteria were clinical course, size, density and delineation of the optic nerve shadow, orbital and cerebral soft tissue abnormalities, and bony changes in the optic canal. Characteristic CT features are described of individual disease entities such as optic gliomas, optic nerve sheath meningiomas, neoplastic and inflammatory infiltrations. The differential diagnostic importance of individual CT criteria is evaluated and discussed.Simultaneous visualization of orbital and intracranial soft tissue changes as well as bony changes in the optic canal allow the location and identification of the majority of optic nerve lesions based on the criteria mentioned above, and optic nerve tumors can be differentiated. In 9 patients with optic neuritis due to clinically proven encephalitis and in 17 patients with total optic atrophy, no changes in the size of the optic nerve could be found.CT evaluation of the intraorbital portion of the optic nerve requires special examination techniques. Oblique computer reformations through the optic canal provide excellent visualization of bony changes in the optic canal. The exclusion of intracranial causes of optic nerve lesions requires intravenous injection of contrast material.]

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William F. Hoyt zum 55. Geburtstag gewidmet     

Optic Nerve Biopsy via a Medial Transconjunctival Orbitotomy Approach in the Diagnosis of Optic Nerve and Sheath Tumors

Purpose:?To evaluate the role of optic nerve biopsy in the diagnosis and management of optic nerve and sheath tumors.

Methods:?Seven patients with progressive optic nerve and sheath tumors for whom treatment was deemed necessary were included in this study. Optic nerve biopsy via a medial transconjunctival orbitotomy approach was performed in all patients

Results:?There were no complications related to the surgical procedure. Histopathological examination revealed that five patients had juvenile pilocytic astrocytoma (JPA) and two patients had optic nerve sheath meningioma (ONSM). All patients received external beam radiotherapy (EBRT).

Conclusions:?Many other benign and malignant optic nerve and sheath tumors have imaging features similar to those seen in JPA and ONSM. Therefore, it is important to establish histopathologic diagnosis before embarking on treatment. Furthermore, tissue diagnosis is required prior to EBRT in many institutions because of concerns about medicolegal liability. Optic nerve biopsy via a transconjunctival orbitotomy procedure in seven patients yielded histopathologic confirmation of the existing pathology and was not associated with any complications in this series.     

CT和MRI检查在视神经鞘脑膜瘤诊断中的价值

目的探讨CT和MRI检查在诊断视神经鞘脑膜瘤中的价值。方法本研究15例视神经鞘脑膜瘤均经手术及病理检查确诊,并均于术前行CT和MRI检查。结果15例视神经鞘脑膜瘤患者中,增强CT仅显示2例颅内蔓延;平扫MRI显示2例颅内蔓延;而增强MRI显示9例颅内有肿瘤。结论增强MRI在诊断视神经鞘脑膜瘤颅内蔓延中非常有价值,可显示增强CT和MRI平扫未显示的病变,应将其作为视神经鞘脑膜瘤术前、术后常规检查。     

OCT和视野对颅咽管瘤患者视功能损伤的评价

目的：通过分析光学相干断层扫描(OCT)测量颅咽管瘤患者黄斑区视网膜神经节细胞复合体(GCC)及视盘周围视网膜神经纤维层(pRNFL)厚度的变化及其与视野平均缺损度(MD)及视野平均敏感度(MS)的相关性,探讨OCT检查应用于评估颅咽管瘤患者视路损伤的可行性。

方法：对2014-09/2015-04在我神经外科就诊的颅咽管瘤患者95例175眼进行视野和OCT检查,测量神经纤维层厚度和GCC层厚度,利用Spearman等级相关系数(r_s)评价其与MD、MS的相关性,并分析其视乳头及视野改变的特点。

结果：pRNFL厚度异常眼占53.1%(93/175),其中,视乳头水肿占3.4%(6/175),视神经萎缩占47.4%(83/175),视乳头类青光眼样改变者占7.4%(13/175)。视野缺损占71.4%(125/175)。双眼的pRNFL平均厚度(r_s右眼=-0.411,r_s左眼=-0.354)及GCC平均厚度(r_s右眼=-0.400,r_s左眼=-0.314),与MD相关(P<0.01); 双眼的pRNFL平均厚度(r_s右眼=0.412,r_s左眼=0.342)及GCC平均厚度(r_s右眼=0.414,r_s左眼=0.299),与MS相关(P<0.01)。

结论：pRNFL和GCC厚度与视野损害相关,可定量的评估鞍区肿瘤患者的视神经损害程度。pRNFL和GCC厚度越薄,视功能损害越严重。在临床工作中,将视野检查和OCT检查结合起来,更有利于发现及评估患者的视路损害及预后。     

Optic Nerve Sheath Meningioma in the First Decade of Life: Case Report and Review of the Literature

Background/Aim

Tumors of the optic nerve are mostly either optic nerve gliomas or optic nerve sheath meningiomas (ONSMs). While gliomas occur in children, most meningiomas are slow-growing tumors affecting middle-aged individuals with progressive visual loss, proptosis, disc edema and optociliary veins on fundus examination. ONSMs are extremely rare in children, with only 14 cases reported in children under the age of 10 years. The purpose of this study is to describe an additional case and review clinical, radiologic and histopathological findings that can help differentiate such tumors in children and allow an early and accurate diagnosis.

Case Report

An 8-year-old girl had a 2-year history of progressive proptosis and total visual loss on the right side. A computed tomography scan revealed a well-defined intraconal mass with perioptic calcification. Magnetic resonance imaging showed a tumor surrounding the optic nerve and extending intracranially, with enhancement following gadolinium injection. The patient was submitted to fronto-orbital craniotomy for complete tumor excision. Histological studies identified the neoplasm as ONSM.

Conclusions

Though unusual, ONSMs may occur in children and, in these cases, present a much more aggressive behavior than in adults. ONSM should therefore be differentiated from optic glioma in children because of its aggressive behavior and need for different treatment modalities.Key Words: Optic nerve neoplasms, Benign optic nerve sheath neoplasm, Meningioma     

The Surgical Management and Outcomes for Spheno-orbital Meningiomas: A 7-year Review of Multi-disciplinary Practice

Aims/Purpose: Spheno-orbital meningiomas account for 9% of all adult intracranial meningiomas. Complete resection is extremely difficult with this condition. We report on our experience in the surgical management of spheno-orbital meningiomas.

Methods: A retrospective review was performed of all patients with spheno-orbital meningiomas who underwent joint neurosurgical and ophthalmic procedures between January 2000 and December 2007. Radiological findings, presenting clinical signs, indications for surgery, surgical approach, histopathological findings, surgical complications and post-operative results were recorded.

Results: Twelve patients were included in the study. Visual function deterioration was the main indication for surgery. Six patients underwent an optic canal decompression along with their initial tumour resection and all 12 patients underwent an extensive lateral orbital wall decompression. Post-operatively 6 patients had reduced proptosis. Two patients had an improvement in their visual acuity, 5 patients had a stable visual acuity and 5 patients had a progressive deterioration in visual acuity following surgery. Six patients maintained a stable visual field. Cranial nerve palsy was the commonest post-operative complication. Three patients required postoperative fractionated radiotherapy. Three patients required further surgery.

Conclusion: Spheno-orbital meningiomas are difficult tumours to manage. Surgical resection can reduce the degree of proptosis and stabilise visual function in patients with failing vision, although sustained improvement is difficult to achieve if the tumour behaves in an aggressive manner. The risk of post-operative visual loss is considerable, either due to surgery or tumour progression. Outcomes from surgical decompression may not necessarily be better than the natural history of these tumours.     

The Role of Intravenous Methylprednisolone Immunosuppression in the Management of Active Thyroid Eye Disease

Purpose:?To analyze the efficacy of intravenous methylprednisolone (IVMP) immunosuppression in the management of active thyroid eye disease (TED).

Methods:?This is a consecutive case series of 36 patients treated with 1?g of IVMP over 3 days, between January 2000 and January 2008. The indications for this treatment were moderate to severe active TED with or without optic nerve compression.

Results:?There were 32 hyperthyroid, 2 hypothyroid, and 2 euthyroid patients in this series. Nine patients presented with optic nerve compromise, 3 had severe active TED and 24 had moderately active TED. Twenty-seven patients received one course of IVMP and nine patients received two courses. All patients had tapering oral prednisolone over 1 month following the IVMP. The mean improvement in proptosis was 1.31?mm (range?=?0–3?mm). Optic nerve function improved in seven of nine patients (77.8%). Extra ocular muscle movement improved in 18 patients (50%). Overall benefit from IVMP was seen at 1/12 in 27 patients (75%) and at 3/12 in 30 patients (83%). After IVMP one patient underwent radiotherapy and three patients had nonsteroidal immunosuppression.

Conclusions:?IVMP immunosuppression is effective in 83% of patients. The effect lasts from 3 months to 8 years, with a mean of 15 months in 30.5%. It remains a useful modality of treatment especially when the optic nerve or cornea is threatened because of moderate to severe active TED. We encountered no severe side effects of pulsed IVMP and 3?g over 3 days is a safe dose.     

Orbital Nerve Seath Myxoma with Extraocular Muscle Involvement: A Rare Case

Abstract

A 66-year-old woman with breast cancer presented with a painless mass in the left orbit. MRI revealed a well-defined intraconal mass in the temporal quadrant of the orbit. Fifteen months later, a further MRI indicated the mass had grown, displacing the left optic nerve and making contact with the lateral rectus muscle, suggesting its possible intramuscular origin. Despite the clinical and radiological characteristics of the lesion and its slow growth, a PET/CT study was developed because of the history of malignant disease. No metabolic activity of the mass or malignant lesion in other locations was observed. After surgical excision, histopathological examination revealed an abundant myxoid matrix with few spindle-shaped cells and no signs of malignancy. The cells were immunopositive for CD34, positive for S-100 protein, and negative for EMA, actin, and CD57. A diagnosis was made of a nerve sheath myxoma. The orbital location of these tumors is extremely rare.     

磁共振扩散张量及3D-OCT对高眼压症患者视路的初步研究

目的：应用磁共振扩散张量成像(DTI)技术及三维光学相干断层成像(3D-OCT)技术研究高眼压症患者(OHT)视路的微观改变。

方法：纳入我院眼科2016-01/2019-10确诊为双眼OHT的患者26例52眼作为病例组,选取同期年龄、性别均与高眼压组相匹配的健康体检者26例52眼作为对照组。所有受检眼均进行最佳矫正视力检测、中央角膜厚度(CCT)测量、非接触性眼压、视盘3D-OCT扫描检测及视神经、视交叉、视束及视放射DTI检测,OHT患者增加房角镜检查,并对比两组的差异。

结果：OHT组CCT值比正常对照组薄,眼压值较正常对照组增高(均P<0.05)。OHT视盘面积较正常对照组变大,视杯面积增大; 平均青光眼视盘旁神经纤维层(CP-RNFL)厚度、鼻侧RNFL厚度比正常对照组变薄(均P<0.05),上方、下方及颞侧RNFL无差异(均P>0.05)。双侧视神经及视放射的FA值较正常对照组下降(均P<0.05)。两组视交叉、双侧视束FA值、双侧视路ADC值无差异(均P>0.05)。

结论：3D-OCT可以获取CP-RNFL厚度值,视盘的参数; DTI可以重建颅内视觉通路,能早期发现视神经、视交叉、视束、视放射的微观变化。联合3D-OCT及新兴的DTI技术可有效了解OHT患者视路的微观改变,为临床研究高眼压症提供新的思路和研究模式。     

Magnetic resonance diffusion tensor imaging and diffusion tensor tractography of human visual pathway

AIM: To investigate the visual pathway in normal subjects and patients with lesion involved by diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT). METHODS: Thirty normal volunteers, 3 subjects with orbital tumors involved the optic nerve (ON) and 33 subjects with occipital lobe tumors involved the optic radiation (OR) (10 gliomas, 6 meningiomas and 17 cerebral metastases) undertook routine cranium magnetic resonance imaging (MRI), DTI and DTT. Visual pathway fibers were analyzed by DTI and DTT images. Test fractional anisotropy (FA) and mean diffusivity (MD) values in different part of the visual pathway. RESULTS: The whole visual pathway but optic chiasm manifested as hyperintensity in FA maps and homogenous green signal in the direction encoded color maps. The optic chiasm did not display clearly. There was no significant difference between the bilateral FA values and MD values of normal visual pathway but optic chiasm, which the FA values tested were much too low(all P＞0.05). The ONs of subjects with orbital tumors were compressed and displaced. Only one subject had lower FA values and higher MD values. OR of 9 gliomas subjects were infiltrated, with displacement in 2 and disruption in 7 subjects. All OR in 6 meniongiomas subjects were displaced. OR in 17 cerebral metastases subjects all developed displacement while 7 of them had disruption also. CONCLUSION: MR-DTI is highly sensitive in manifesting visual pathway. Visual pathway can be analyzed quantitatively in FA and MD values. DTT supplies accurate three dimensional conformations of visual pathway. But optic chiasm’s manifestation still needs to improve.     

Effect of oral felodipine on ocular circulation

Purpose: To evaluate the effects of felodipine, a calcium channelblocker, on ocular circulation. Methods: In a double blind, randomized,crossover design, 10 volunteers received placebo or felodipine on 2 separatedays. Bidirectional laser Doppler velocimetry (BLDV), laser Doppler flowmetry(LDF), and monochromatic fundus photography (MFP) were employed to assessretinal, choroidal, and optic nerve rim circulatory parameters. Measurements wereobtained at baseline, 1.5, and 3 hours after dosing. Blood pressure, intraocularpressure (IOP), and heart rate were monitored, and perfusion pressure (PP) wascalculated. Results: In comparison to placebo, significant average percentagedecreases in diastolic blood pressure (BPd; p = 0.001), mean blood pressure (BPm;p = 0.003), and perfusion pressure (PP; p = 0.003) were observed 1.5 hours following felodipine. No significant differences were observed following placebo. Retinal, choroidal and optic nerve rim circulatory parameters showed no significant changes after placebo or felodipine. A significant positive correlation between BPd and optic nerve velocity (ON_vel) was observed 3 hours following felodipine (R = +0.654; p = 0.040). In contrast, no significant correlation between BPd and ON_vel was noted (R = +0.198; p = 0.583) following placebo. Conclusions: No significant change in retinal, optic nerve rim, or foveolar choroidal blood flow were observed, a factor that may be important in the treatment of systemic hypertension in patients with glaucoma. Abbreviations:BLDV – Bidirectional laser Doppler velocimetry;BPd – diastolic blood pressure;BPm – mean blood pressure;BPs – systolic blood pressure;CH_flow – choroidal flow;CH_vel – choroidal velocity;CH_vol – choroidal volume;D – vessel diameter;LDF – Laser Doppler flowmetry;MFP – monochromatic fundus photography;ON_flow – optic nerve flow;ON_vel – optic nerve velocity;ON_vol – optic nerve volume;PP – perfusion pressure;Q – retinal volumetric blood flow rate;V_max – maximum velocity of whole blood;V_mean – mean velocity of whole blood.     

OCT-guided management of subclinical recurrent retinoblastoma

Purpose: Retinoblastoma (Rb) tumor recurrence in the papillary or macular region is a threat to life and visual prognosis respectively, making early detection indispensable. This study demonstrates the value of optical coherence tomography (OCT) in the early detection of subclinical tumor recurrence.

Methods: Since June 2012, hand-held SD-OCT (spectral domain Optical Coherence Tomography) of retro-equatorial foci, the optic head nerve and macula, is systematically performed under anesthesia in children treated and followed for Rb.

Results: Between June 2012 and January 2017, 16 subclinical recurrent tumors in 14 children were detected only by OCT in flat pigmented scars (n = 9), in type 2 regression (n = 3), on the optic nerve head (n = 3), and as secondary retinal seeding (n = 1).

Conclusion: OCT has become invaluable in the modern management of Rb. It allows not only early detection of a lesion before any tumor extension towards the macula or optic nerve head, but also the monitoring of the therapeutic response.     

Letter to the editor

Abstract

An inflammation or injury to optic nerve anywhere its pathway by extrinsic lesions causes the optic neuropathy. These lesions are more effective in confined spaces like optic canal, orbital apex. We present a 61 year old woman with optic neuropathy due to lesion in an Onodi cell. In this case patient presented with progressive loss of vision in her right eye within a few days. Computed tomography (CT) revealed mucosal thickening and inflammatory signs in right sphenoid sinus and presence of Onodi cell in that side. Magnetic Resonance Imaging confirmed compression to the optic nevre. Systemic antibioterapy and endoscopic sinus surgery was performed. Postoperatively, the visual acuity and control CT views were better than initial findings. Histopathologic evaluation revealed polyps in Onodi cell. Ophthalmologists should be aware of the Onodi cell pathologies that caused compressive optic neuropathy.     

原发性眼眶脑膜瘤临床及影像诊断分析

目的：探讨原发性眼眶脑膜瘤的临床表现，影像学特征，诊断及治疗原则，方法：1979－1998年间诊治的原发性眼眶脑膜瘤115例进行回顾性分析，详细复习了全部病例的临床，病理资料及X线，B超，彩色多普勒超声，CT和（或）MRI的图像并进行比较，结果：原发性眼眶脑膜瘤可起源于视神经鞘和眶骨膜，多发生于中年女性，眼球突出，视神经萎缩，视力丧失和视睫状静脉为视神经鞘脑膜瘤的四联征，起源于眶骨膜的脑膜早期出现眼球突出和移位，视力减退出现较晚，X线，B超，CT和MRI有特征性表现，CT可以同时显示视神经增粗，眶内软组织肿块和眶骨改变，MRI可以更好地观察视神经管内及颅内蔓延状况，治疗原则主要为手术切除，结论：原发性眼眶脑膜瘤是种较为常见的眼眶肿瘤，影像学检查有助于诊断和鉴别诊断，一旦确诊，应早期手术治疗。     

Ultrasonic tomography of neurogenic tumors of the orbit

The results of a comprehensive ultrasonic examination of 12 patients with tumors of the eye orbit are described. A low echogenicity as well as heterogeneity of the echo-structure and of the contours' sharpness are the main sonographic features of the mentioned tumors. An examination of a vascularization degree in patients with gliomas of the optic nerve showed no blood circulation inside tumors; while a collateral blood circulation was detected in patients with meningiomas of the optic nerve. A compression of the ocular artery by the tumor and reduced hemodynamic indices were found to be typical. Finally, a triplex examination of the orbit makes it possible to diagnose tumors of the optic nerve.     

Neurodegenerative Genes Polymorphisms of the -491A/T APOE,the -877T/C APP and the Risk of Primary Open-angle Glaucoma in the Polish Population

Background: Glaucoma is characterized by optic neuropathy of the retinal ganglion cell. It may be possible that β-amyloid (Aβ) and apolipoprotein E (APOE), the main proteins of the pathogenesis of AD, play a role in glaucoma development. The aim of this study was to evaluate a relationship between the APP and APOE gene polymorphisms and the risk of primary open-angle glaucoma (POAG) occurrence.

Materials and methods: The study consisted of 183 patients with POAG and 209 healthy subjects. Genomic DNA was extracted from peripheral blood. Analysis of the gene polymorphisms was performed using PCR-RFLP.

Results: We found a statistically significant increase of the -491?T allele frequency (p?=?0.02; OR?=?1.48; 95% CI?=?1.06–2.08) of APOE in POAG compared to healthy controls. There were no differences in the genotype and allele distributions and odds ratios of the APP polymorphism between patients and controls group. We also found an association between APOE polymorphic variant and retinal nerve fiber layer (RNFL). There was a statistically significant difference in the APOE gene A/T genotype frequency in the early POAG stage and middle-advanced POAG stage in comparison to the advanced POAG stage (p?=?0.04; OR?=?3.38; 95% CI?=?1.04–10.97).

Conclusions: The -491?T allele of APOE polymorphism may be associated with a risk of POAG occurrence in the Polish population.     

Orbital color Doppler imaging of optic nerve tumors

Objective: To report changes in retinal arterial and venous blood flow pattern in two patients with tumors involving the entire optic nerve. Methods: Retrospective review of one patient with clinical and neuroimaging characteristics typical of bilateral optic nerve gliomas and one patient with a probable meningioma of the left optic nerve sheath. Results: The optic nerve glioma patient had reduced peak systolic velocity of central retinal arteries bilaterally, while the patient with an optic nerve sheath meningioma had relatively low central retinal artery flow velocity and intermittent blood flow in the central retinal vein on the affected side. Conclusions: Reduced retinal arterial flowvelocities in the setting of optic nerve gliomas may correlate with thepresence of optic nerve disease. Phasic blood flow in the central retinalvein with optic nerve sheath meningioma may be the reason that somepatients with this tumor develop retinal choroidal venous anastomoses.