Background:Pseudotumors of the orbit comprise a group of idiopathic inflammatory processes and are,except for endocrine orbitopathy,the most common reason for exophthalmos in adults.Orbital pseudotumors,also called id...Background:Pseudotumors of the orbit comprise a group of idiopathic inflammatory processes and are,except for endocrine orbitopathy,the most common reason for exophthalmos in adults.Orbital pseudotumors,also called idiopathic orbital inflammatory syndrome(IOIS),can be determined from or bital involvement in systemic fibrosing diseases.Finding the correct diagnosis can be challenging.Due to the topographic relations of the orbit to neighbouring structures,a multidisciplinary cooperation is highly recommended.Case Report:We report a case of a 42-year-old woman with unilateral exophthalmos.Additionally we found impaired motility of the affected bulbus,ptosis and reduction of visual acuity.Orbital MR imaging demonstrated dense fibrotic masses filling the whole orbita including the extraocular muscles as well as the optic nerve.Tissue specimens were extracted while performing orbital decompression via a lateral orbitotomy.Histological examination revealed a lymphatic infiltration and fibrotically destroyed tissue containing the lacrimal gland.After surgical decompression,oral steroid therapy and immunotherapy,a recovery of the visual loss could be seen.Conclusions:Intraorbital fibrosclerosing pseudotumors often require a difficult long-term treatment.Therapeutic options are steroid therapy,immunotherapy,radiotherapy and surgery.The diagnostic steps include blood tests,ultrasound,CT and/or MRI as well as histological differentiation.Solid tumors and orbital involvement in diseases of the hematopoetic system have to be excluded.Since intraorbital fibrosis can be accompanied by manifestations in various other organs,a complete investigation of the body and thorough follow up are crucial.展开更多
文摘Background:Pseudotumors of the orbit comprise a group of idiopathic inflammatory processes and are,except for endocrine orbitopathy,the most common reason for exophthalmos in adults.Orbital pseudotumors,also called idiopathic orbital inflammatory syndrome(IOIS),can be determined from or bital involvement in systemic fibrosing diseases.Finding the correct diagnosis can be challenging.Due to the topographic relations of the orbit to neighbouring structures,a multidisciplinary cooperation is highly recommended.Case Report:We report a case of a 42-year-old woman with unilateral exophthalmos.Additionally we found impaired motility of the affected bulbus,ptosis and reduction of visual acuity.Orbital MR imaging demonstrated dense fibrotic masses filling the whole orbita including the extraocular muscles as well as the optic nerve.Tissue specimens were extracted while performing orbital decompression via a lateral orbitotomy.Histological examination revealed a lymphatic infiltration and fibrotically destroyed tissue containing the lacrimal gland.After surgical decompression,oral steroid therapy and immunotherapy,a recovery of the visual loss could be seen.Conclusions:Intraorbital fibrosclerosing pseudotumors often require a difficult long-term treatment.Therapeutic options are steroid therapy,immunotherapy,radiotherapy and surgery.The diagnostic steps include blood tests,ultrasound,CT and/or MRI as well as histological differentiation.Solid tumors and orbital involvement in diseases of the hematopoetic system have to be excluded.Since intraorbital fibrosis can be accompanied by manifestations in various other organs,a complete investigation of the body and thorough follow up are crucial.
文摘目的观察自拟清热化坚汤联合糖皮质激素治疗眼眶特发性炎性假瘤(IOIP)的临床疗效。方法 2013年1月至2014年1月收集符合纳入标准的30例IOIP患者,采用随机数字表法分为治疗组和对照组,每组各15例。治疗组予中药联合口服糖皮质激素治疗。对照组予单纯口服糖皮质激素治疗。两组治疗半年后比较中医证候积分、临床疗效(眼球突出度、眼压、影像学检查)及复发率的变化差异。结果 (1)临床疗效:治疗组痊愈5例,显效6例,有效2例,无效2例,总有效率86.67%;对照组痊愈2例,显效3例,有效4例,无效6例,总有效率60.00%(Mann-Whitney U检验,Z=-2.081,P=0.037)。治疗组眼球突出度、眼压以及肿块直径的变化值分别为(-4.20±1.08)mm、(-13.73±4.06)mm Hg(1 mm Hg=0.133 k Pa)、(-1.50±0.30)cm;对照组相应数值为(-2.27±0.73)mm、(-8.13±1.41)mm Hg、(-1.21±0.24)cm,其中眼压变化的组间差异有统计学意义(配对t检验,t=-5.05,P<0.001)。(2)中医证侯疗效:治疗组痊愈5例,显效7例,有效3例,无效0例,总有效率100.00%;对照组痊愈1例,显效2例,有效6例,无效6例,总有效率60.00%(Z=-3.353,P=0.010)。(3)复发率:随访半年,治疗组15例,失访1例,复发2例,复发率14.29%(2/14);对照组15例,失访1例,复发7例,复发率50.00%(7/14)(Fisher确切概率法,P=0.103)。结论自拟清热化坚汤联合糖皮质激素治疗IOIP可以明显改善患者的中医症状及临床体征,对降低IOIP复发率亦有一定作用。
