209例眼眶特发性炎性假瘤的临床分析

目的 :探讨眼眶特发性炎性假瘤的临床特点和治疗效果。方法 :回顾分析我中心自 1978年 1月到 1999年 12月收集的眼眶特发性炎性假瘤 2 0 9例。结果 :2 0 9例中 ,男 118例 ,女 91例 ;就诊年龄 4～ 80岁 (平均 44 4岁 ) ;右眼 90例 ,左眼 81例 ,双眼 38例。眼球突出( 6 6 % )、眶缘可触及肿物 ( 6 5 % )、眼睑肿胀 ( 5 5 % )、眶压增高 ( 5 5 % )和眼球运动受限 ( 4 8% )为最常见的表现。根据影像学和手术所见 ,以眼眶内占位性病变表现者最多见 ( 4 3% ) ,其次为泪腺炎型 ( 32 % )、弥漫型 ( 10 % )和肌炎型 ( 8% )。视神经周围炎型 ( 2 % )、巩膜周围炎型 ( 2 % )、急性型 ( 2 % )与眼睑型 ( 1% )少见。经手术、全身用激素或免疫抑制剂和眼眶局部放射治疗后随访结果 (平均随访 1 5年 )显示完全治愈率和部分治愈率分别为 40 %和 5 7% ,其中随访半年以上的 80例病人中有 43%复发。结论 :尽管眼眶特发性炎性假瘤容易复发 ,但手术切除、全身用激素和眼眶局部小剂量放射治疗等的成功率高     

肌炎型特发性眼眶炎性假瘤

目的探讨肌炎型眼眶特发性炎性假瘤的临床特点.方法回顾分析我院1978年1月～1999年12月特发性眼眶炎性假瘤病例,每例均有完整的临床特点、影像表现、诊断和治疗结果等记录.随访时间为1周到9年(平均4个月).结果　209例眼眶特发性炎性假瘤中,16例(8%)属于肌炎型眼眶特发性炎性假瘤.其中男6例,女10例;右眼6例,左眼7例,双眼3例;平均就诊年龄39岁.以眼球运动受限与斜视(87.5%)、复视(68.8%)、眼球突出(62.5%)为最常见表现,眼部充血(43.8%)和疼痛(43.8%)是与其它类型斜视不同的重要特点.各条眼外肌中,上直肌受累最常见(62.5%),其次为外直肌(43.8%),内直肌(31%)和下直肌(31%).经全身激素或激素联合局部放射治疗后,治愈率为56%,有效率为75%,复发率为75%.结论肌炎型是特发性眼眶炎性假瘤中较少见的临床亚型之一,以上直肌受累最常见,其次为外直肌、内直肌与下直肌.全身激素或激素联合放射治疗的治愈率只有56%,而复发率高达75%.如何提高治愈率与减少复发率是今后努力的方向.     

甲状腺相关眼病的放射治疗

目的探讨甲状腺相关眼病(TAO)放射治疗的适应证和临床效果。方法选择1992至2004年在四川大学华西医院肿瘤科放射治疗室行眼眶照射、资料较齐全的23例TAO患者,其中9例是浸润性突眼、14例是压迫性视神经病变,均为糖皮质激素治疗不敏感、不能接受糖皮质激素治疗者,或者因较严重的糖尿病、高血压等不能做眼眶减压或不愿意做手术的活动期且病程短的TAO患者。采用直线加速器和Donaldson眼眶外照射法,照射野大小为4cm×5cm,放射总剂量为20GY,治疗分10次,每次2GY。放射治疗期间其中11例炎性反应较重的患者同时口服泼尼松。全部患者放射治疗前后均行外眼照像和眼眶CT检查。结果14例压迫性视神经病变,放射治疗前矫正视力0.04~0.2,放射治疗后矫正视力0.1~0.8,CT检查显示肿大的眼外肌缩小,眼外肌缩小的程度与视力提高的程度成正相关。9例浸润性突眼,放射治疗前眼部炎性反应明显,放射和口服泼尼松治疗后眼睑、结膜肿胀显著减退,眶压降低,眼睑能闭合,暴露性角膜炎痊愈,眼球活动受限好转,眼球突出度降低1~3mm。随访1~3年,3例患者又出现视力减退,4例眼眶炎性反应复发,2例眼睑仍部分闭合不全。结论活动性难治的TAO患者采用放射治疗效果较好,对于眼眶炎性反应严重的患者可加用糖皮质激素治疗。(中华眼科杂志,2006,42:218-221)     

皮质激素联合小剂量放疗治疗眼眶炎性假瘤的临床观察

目的 探讨采用皮质激素联合小剂量放疗治疗眼眶炎性假瘤的有效性。方法 27例眼眶炎性假瘤患者(其中肌炎型12例，弥漫型7例，后部型3例，前部型5例)，首先采取口服强的松40—60rag／日。1～2周后逐渐减量，20—30天后停药。停药后采用小剂量放射治疗(钴—60经眶外侧壁眶尖部照射，总量20Gy，2周内分10次给予，2Gy／次)。结果 随访3年，27例患者经上述综合治疗后眼痛、眼球突出、眼球运动障碍等症状消失者23例，占85．2％；部分减轻者2例，占7．4％；无效者2例。占7．4％；随访3年，23例均无复发。结论 皮质激素联合小剂量放射治疗可显著减轻眼眶炎性假瘤患者的临床症状，提高治愈率，减少复发。     

眼眶肿瘤手术治疗18例

眼眶肿瘤严重威胁患者健康,手术摘除是有效的治疗手段。我院自1998~2005年共收治眼眶肿瘤18例18眼,现就临床效果、手术进路、手术体会及术后并发症等总结如下。1资料与方法1.1一般资料自1998~2005年我院共收治眼眶肿瘤18例18眼,其中男12眼,女6眼;年龄7~56岁,平均31.5岁;病程3个月~5a。良性肿瘤16眼,包括眶内皮样囊肿6眼,海绵状血管瘤2眼,眼眶静脉曲张3眼,特发性炎性假瘤3眼,泪腺良性多形性腺瘤2眼;恶性肿瘤2眼,包括泪腺多形性腺癌1眼,泪腺腺样囊性癌1眼。眼球突出度15~19mm,平均17mm。1.2方法本组患者均经过CT、MRI、超声检查进行初步诊断…     

眼眶特发性炎性假瘤的手术治疗

目的：眼眶特发性假瘤通常首选全身激素治疗,本文指在探讨其手术治疗效果。方法 回顾分析我中心１９８０年１月至１９９４年１月住院病人中用手术治疗的５２例患者,随访时间６个月至２０年,平均２．９年。结果 ２９例完全治愈,２例部分有效,２１例复发。２１例复发病人中,２例经再次手术,５例经再手术加用全身激素,５例加用激素和环磷酰胺等免疫抑制药,５例加用激素和低剂量放射治疗（２０ＧＹ,分２个疗程）后最终治愈。     

眼眶炎性假瘤271例临床分析

目的 分析眼眶炎性假瘤的临床特征及影像学检查在眼眶炎性假瘤的诊断和鉴别诊断中的价值,以及各种治疗方法效果的比较。方法 对眼眶炎性假瘤患者271例（303只眼）进行回顾性分析。结果 根据眼眶肿物组织学特征的不同,其超声和CT扫描检查具有多样的表现。弥漫性淋巴细胞浸润型炎性假瘤;B超检查显示眶内肿物为低回声,透声性强的占位性病变;纤维增生型炎性假瘤;B超检查显示眼眶内低回声,透声性差,边界不清的肿物;CT扫描可见肿物与眼球接触紧密,呈“铸造样”改变。糖皮质激素及放射治疗对于弥漫性淋巴增生型炎性假瘤效果显著,而纤维增生型炎性假瘤对各种治疗方法均不敏感。结论 影像学检查对于眼眶炎性假瘤的诊断,鉴别诊断,病理组织学分型的判断及治疗方法的选择有应用价值。     

眼眶炎性假瘤所致的眶尖综合征

目的　探讨眼眶炎性假瘤所致的眶尖综合征的临床特点和治疗等。方法　回顾分析我院自1 978年 1月到 1 998年 1 2月眼眶炎性假瘤病例 1 94例 ,其中 9例有典型的眶尖综合征表现 :患眼向各个方向运动受限或眼球固定 ,上睑下垂 ,调节麻痹 ,瞳孔散大 ,下睑皮肤麻木和视力下降或丧失等。治疗方法包括全身口服皮质激素、局部放射治疗、手术切除和上述方法联合治疗等。结果　 9例病人中 ,6例有眼球突出 ,5例有眼眶疼痛与结膜或眼睑充血。 5例经全身激素治疗后 ,4例视力有提高 ;3例上睑下垂基本消失 ,2例睑裂部分开大 ;5例病人眼球运动均恢复正常。 4例经开眶手术活检后用全身激素治疗 ,1例视力恢复正常 ,上睑下垂消失 ,眼球运动恢复正常 ;3例病情无好转。结论　眼眶炎性假瘤是眶尖综合征的常见原因之一 ,约一半病人没有眼部疼痛与充血 ,全身激素治疗效果好 ,其与眼眶恶性淋巴瘤的鉴别诊断十分重要     

眼眶病的诊断和治疗原则

眼眶病是指眶隔之后的眶骨和眶内软组织发生的疾病 ,或眶周和全身病的眼眶侵犯。眼眶病虽非常见病或多发病 ,但其种类繁多 ,与眶周及全身各系统联系密切。眼眶病的分类、临床表现、诊断及治疗均与眼球疾病有较大差异。一、眼眶病的分类[1 3 ]1.眼眶先天或发育异常 :常见为颅面骨畸形、先天性小眼球合并眼眶囊肿及脑膜 脑膨出等 ,其中以先天性小眼球合并眼眶囊肿最为多见。2 .眼眶炎性病变 :分为 3类 ,即急性炎性病变 ,如蜂窝组织炎、脓肿等 ;特发性炎性病变 ,如炎性假瘤、痛性眼肌麻痹等 ;慢性炎性病变 ,如血管炎、异物性肉芽肿及Ｗｅｇｅｎ…     

眼眶血管瘤的皮质类固醇疗法

眼眶血管瘤的皮质类固醇疗法广东医学院附属医院眼科王映芬，林晨本文报告应用皮质类固醇治疗眼眶毛细血管瘤及海绵状血管瘤８例，有效率１００％。本疗法对婴幼儿患者疗效最佳，可以治愈；成人患者经治疗可使瘤体缩小，眼球突出减轻，但不能根治，停药后可复发。眼眶血管...     

Treatment of recalcitrant idiopathic orbital inflammation (chronic orbital myositis) with infliximab

PURPOSE: To report results of treatment with a monoclonal antibody (infliximab) directed against tumor necrosis factor alpha in seven patients with chronic and difficult-to-control idiopathic orbital inflammation (orbital myositis). DESIGN: Observational case series. METHODS: Retrospective data were collected from seven patients who had idiopathic orbital inflammation and who were evaluated at three medical centers. All patients were treated with infliximab after the failure of traditional therapy, which included corticosteroids, radiotherapy, or anti-inflammatory chemotherapeutic agents. RESULTS: All seven patients had a favorable response to treatment with infliximab. One patient with Behcet disease required supplemental oral corticosteroids. Pain, swelling, and need for concomitant corticosteroids were the primary measures of treatment success. Symptoms of comorbid disease in four patients also improved (Crohn disease in two, Behcet disease in one, and psoriasis in one). There were no untoward effects of treatment after a mean follow-up of 15.7 months (range, 4 to 31 months). CONCLUSIONS: Treatment with infliximab appears to offer another therapeutic option in cases of recalcitrant or recurrent idiopathic orbital inflammation in which conventional treatment fails.     

Two cases of orbital myositis with monoclonal gammopathy of undetermined significance

PURPOSE: We report two cases of idiopathic orbital myositis with monoclonal gammopathy. CASE: Case one was a 47-year-old man, who had bilateral swelling of the extraocular muscles and impairment of the left optic nerve. Case two was a 27-year-old woman, who had bilateral proptosis. An immunological test showed that both patients had monoclonal gammopathy, and they were diagnosed as having monoclonal gammopathy of undetermined significance(MGUS). RESULTS: In case one, the patient achieved remission with steroid pulse therapy followed by administration of high doses of a steroid. In case two, because of repeated recurrence, the patient was treated with steroid pulse therapy and then radiation therapy to achieve final remission. CONCLUSION: We need to pay attention in the diagnosis of orbital myositis to distinguish MGUS. Such patients have an atypical clinical course and are resistant to ordinary steroid therapy.     

Supervoltage orbital radiotherapy in 36 cases of Graves' disease.

Thirty-six patients who had clinically progressive Graves' dysthyroid orbitopathy were treated with supervoltage orbital radiotherapy. Twenty-three of the patients had discontinued the use of orally administered prednisone after developing intolerable side effects. Medical contraindications prevented seven patients from receiving systemic corticosteroid treatment. Six additional patients declined to take prednisone and chose orbital radiotherapy as their primary treatment. All patients were treated with one radiotherapy protocol wherein a 6 MV linear accelerator delivered 2,000 cGy to the midplane of both orbits in ten fractions. None of the 36 patients was treated with corticosteroids during the orbital radiotherapy treatment interval. We encountered three patients who failed to respond to orbital radiotherapy and required supplemental immunosuppression to arrest progression of Graves' disease. The remaining 33 patients experienced stabilization or clinical improvement of their condition. None of the patients experienced complications from orbital irradiation. We believe supervoltage orbital radiotherapy is an effective means of treating Graves' dysthyroid orbitopathy.     

Clinical presentation and treatment of secondary orbital lymphoma

PURPOSE: To describe the clinical features and histologic classification of orbital lymphoma secondary to systemic non-Hodgkin lymphoma, to evaluate the efficacy of various treatment modalities for controlling the orbital component of disease, and to analyze vision-related outcomes. METHODS: In this retrospective case series, the clinical records of patients diagnosed with orbital lymphoma who were treated between January 1999 and May 2001 at the University of Texas M.D. Anderson Cancer Center were reviewed. Demographic data, histologic classification of lymphoma, immunophenotype, stage of disease, treatment outcomes, vision-related outcomes, and duration of follow-up after the diagnosis of orbital lymphoma were recorded in each case. RESULTS: At the time of diagnosis, all 15 patients identified either had a previous history of non-Hodgkin lymphoma or had simultaneous involvement of lymph node or other sites. Ten patients (67%) had intermediate-grade or high-grade lymphoma. All 15 patients had a B-cell phenotype. Systemic chemotherapy or immunotherapy was the initial treatment modality in 13 patients; the other 2 patients were treated with radiation as the initial mode of therapy. Eleven patients (73%) achieved complete regression of the orbital tumor; one patient had partial regression of the orbital lymphoma. Loss of vision secondary to compressive optic neuropathy was seen in 5 patients (33%); in 3 of these patients, the orbital lymphoma was diagnosed early and treatment was successful in reversing the loss of vision. One patient had irreversible vision loss secondary to compressive optic neuropathy; in this case, therapy had not been initiated until several months after onset of symptoms. Another patient died of lymphoma before the orbital disease could have responded to therapy. CONCLUSIONS: Because of often widespread systemic involvement and a usually more aggressive histologic classification than primary orbital lymphomas, secondary orbital lymphomas warrant treatment with systemic chemotherapy or systemic immunotherapy. In some cases, combining such systemic therapy with local radiation treatment is beneficial. Loss of vision as the result of compressive optic neuropathy in this setting can be reversed if systemic therapy is initiated soon after the onset of progressive signs and symptoms of orbital disease.     

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma: comparison of long-term outcome between radiotherapy and chemotherapy

AIM: To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), and to compare the treatment outcome between postoperative radiotherapy (RT) and chemotherapy in a retrospective analysis nearly 20y. METHODS: A retrospective cohort study of 56 patients with orbital NRSTS were reviewed, 34 of whom received postoperative RT, and 22 received postoperative chemotherapy. The clinicopathological features, local recurrence, metastases, and survival data were recorded. Survival analysis was performed using the Kaplan-Meier method. RESULTS: During follow-up (111.8mo, ranged 8-233mo) for 56 patients, 19 patients of them developed local recurrence, and 7 patients developed distant metastases. Fifteen patients died during follow-up period. Overall survival rates considering the whole study group was 78.57% at 5y, and 72.16% at 10y after the initial diagnosis. Compared with chemotherapy, RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy, 0.263, 95% confidence interval (CI), 0.095-0.728, P=0.0015]; with lower risk of distant metastasis (hazard ratio for RT vs chemotherapy, 0.073, 95%CI, 0.015-0.364, P=0.0014); and with lower risk of death from disease (hazard ratio for RT vs chemotherapy, 0.066, 95%CI, 0.022-0.200, P<0.0001). The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group. CONCLUSION: In patients with orbital NRSTS, postoperative RT provides better control of local recurrence, distant metastasis, and death from disease than chemotherapy. RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.     

Traumatic superior orbital fissure syndrome: report of 4 cases and review of literature

PURPOSE: The authors retrospectively analyzed four cases of posttraumatic superior orbital fissure syndrome and reviewed the literature. METHOD: Four patients (three male and one female) were followed for this complication in the department from october 1995 to december 1996. RESULTS: The mean age was 31 years, the median follow-up was 8 month. Computed tomography showed involvement of the superior orbital fissure but no in three patients, in the fourth. The treatment consisted of osteosynthesis of craniofacial fracture in three patients and corticosteroid therapy in the last one. All patients presented partial recuperation of external ophthalmoplegia and ptosis. Twelve months after the traumatism, one patient underwent surgery for persistant ptosis and diplopia. CONCLUSION: The superior orbital fissure syndrome is an exceptional complication of orbital traumas. Partial recuperation of the neuro-ophthalmologic function usually occurs within a period of several months.     

Multimodal therapy in the management of primary orbital mesenchymal chondrosarcoma

AIM:To evaluate the ophthalmic manifestations,radiographic features,and prognosis of Chinese patients with primary orbital mesenchymal chondrosarcoma(MCS).METHODS:The study included 6 cases with primary orbital MCS treated at Tianjin Eye Hospital from January 2009 to December 2019.Patitent ophthalmic manifestations,radiographic features,diagnosis,pathology,therapeutic regimens,and prognosis were retrospectively reviewed.RESULTS:Six patitents with primary orbital MCS were identified.The mean age at the first visit was 33y(range,25-42y).All six patients displayed manifestations of exophthalmos,diplopia,limitation of eye displacement,upper eyelid oedema,decreased visual acuity and ptosis.The mean disease history and range were 5 and 2-8mo,respectively.The tumors were located in the superonasal extraconal compartment(2/6,33.3%),intraconal compartment(2/6,33.3%),and bitemporal extraconal compartment(2/6,33.3%),respectively.Radiographic features were a well-defined,orbital mass with calcification and ossification on computed tomography(CT),and marked heterogenous enhancement on dynamic magnetic resonance imaging(MRI).Five patients were treated with tumor resection and one patient received orbital exenteration.Five patients in the cohort received postoperative radiation therapy,two patients received chemotherapy,and one patient did not receive postoperative adjuvant therapy because he refused.The histopathologic classification revealed a tumour composed of a mixture of mature chondroid tissue surrounded by small,round,and undifferentiated mesenchymal cells.Immunohistochemistry revealed Bcl-2,vimetin,CD99,and S-100 were expressed were expressed.After surgeries,two patients have developed a local recurrence.The median recurrence time of 58 mo(52-64 mo).One patient had distant recurrence included the lungs occurred 52mo after the initial surgery.CONCLUSION:The possibilty of orbital MCS need to be considered when a painless,slowly growing orbital mass with calcification and ossification.From our experience,trimodality treatment of radiation therapy,chemotherapy and surgery maybe the best option.Orbital MCS has a high tendency for late recurrence,regular long-term follow-up after complete excision is mandatory.     

Successful treatment of idiopathic orbital inflammation with infliximab: an alternative to conventional steroid-sparing agents

Therapy for orbital pseudotumor, also called idiopathic orbital inflammation, is nonspecific and mainly based on the use of corticosteroids. Two patients with steroid-dependent idiopathic orbital inflammation, one with myositis and the other with dacryoadenitis, and intolerant of standard steroid sparing agents (methotrexate or azathioprine), were treated with infliximab, a monoclonal antitumor necrosis factor alpha antibody. In both patients, orbital manifestations disappeared following treatment. After follow-up for at least 20 months, the 2 patients still receiving infliximab remained event free. Infliximab enabled steroid tapering to less than 5 mg per day, suggesting that infliximab could constitute an alternative to conventional steroid sparing agents.