眼眶胚胎性横纹肌肉瘤一例

患者男,11岁。2002年10月发现左眼眶下侧肿胀,自行扪及下眶缘一绿豆大小硬结,右眼无异常。在外院手术切除肿物后,未予治疗。2个月后再次发现左下眼眶肿胀,并扪及一约0．5 cm×0．3 cm大小结节,再次于外院手术切除,并     

Periorbital necrotising fasciitis after minor trauma

Introduction Necrotising fasciitis or streptococcal gangrene is a rare and often fatal soft tissue infection usually affecting the limbs and trunk. Facial involvement is exceedingly rare due to the excellent blood supply of this region. Methods We report a case of initially misdiagnosed streptococcal gangrene of the eyelids precipitated by minor trauma and which progressed despite intensive medical therapy. Results A 53-year-old man with a history of alcohol abuse developed rapidly increasing left-sided periorbital oedema, erythema and skin vesicles soon after sustaining a laceration to his left upper lid. It was initially treated as herpes zoster ophthalmicus complicated by a secondary bacterial cellulitis. Bacterial cultures grew group A β haemolytic Streptococcus pyogenes. Despite 8 days of high-dose parenteral antibiotic therapy and oral acyclovir, characteristic blisters formed and necrosis of the periorbital skin and subcutaneous tissues ensued. Surgical debridement was performed and the fasciitis rapidly resolved. Conclusion Physicians and ophthalmologists must be aware of the risk factors, although rare, for periorbital necrotising fasciitis and the cardinal signs that differentiate this condition from common non-necrotising preseptal cellulitis. Prompt recognition and early surgical debridement are crucial in limiting the morbidity and mortality from severe forms of this disease.     

眼眶特发性炎性假瘤的CT及MRI表现特点

目的探讨眼眶特发性炎性假瘤（IOIP）的CT和MRI表现特征，以提高对眼眶特发性炎性假瘤的认识和影像诊断水平。方法回顾分析我院2000年1月-2006年10月间80例（89只眼）IOIP患者CT及MR／资料，总结IOIP的影像学特征。结果①肌炎型IOIP：多条眼外肌受累18只眼，单条7只眼，16只眼（66．7％），同时累及肌腹和肌腱。眼外肌受累频率由高到低依次为外、下、内、上直肌。MRI检查呈等T1、略高T2信号。眶内其它组织受累17例，眶外侵犯4例。②肿块型IOIP：多数病灶形态不规则（18／21），边界不清楚（15／21）。5例“铸型”征阳性，7例眶尖脂肪影消失。MRI检查呈等T1、等或略高T2信号，信号不均匀。邻近组织受累18例，眶外侵犯3例。③泪腺炎型IOIP：泪腺扁长形肿胀，“铸型”征阳性，边缘模糊，密度均匀，增强后明显强化。MRI检查呈略高T1，T2信号。邻近组织受累7例。④巩膜炎及视神经炎型IOIP：4例眼环增厚，边缘模糊。其中3例累及视神经眶内段，2例增强后眼环明显强化。1例仅视神经边缘毛糙。邻近组织受累4例。⑤混合型IOIP：融合以上几型表现，呈多样化特征。结论IOIP的影像学表现复杂多样，但多具有炎性特征，眶内多种组织同时受累而以某一种组织为主。CT扫描可作为IOIP的首选检查方法之一，MRI对IOIP有无颅内受累的显示比CT更有优越性。     

Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation

Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.     

经颅眶入路切除视神经鞘脑膜瘤

目的探讨早期手术切除视神经鞘脑膜瘤,保护眶尖部血管神经的临床效果。方法回顾性分析21例经颅眶入路切除视神经鞘脑膜瘤的患者临床资料,首发症状为视力障碍13例,其中失明6例,突眼8例。神经影像学检查显示15例肿瘤（扁平型）包绕视神经纵行生长呈现“双轨征”,其中5例侵犯颅内;6例肿瘤（结节型）呈类圆形无“双轨征”,均局限于眶内。术后随访2月至6年。结果肿瘤全切15例,部分切除6例,17例患者动眼神经及18例患者外展神经术后功能保留;2例早期手术的小型肿瘤患者患眼视力被成功保留,分别随访4年及5年均未复发。随访肿瘤复发2例（均为大型肿瘤）。结论早期手术切除视神经鞘脑膜瘤有在彻底切除肿瘤的同时保存眶尖部其他神经血管的优点,部分患眼的视力亦可保留。     

Orbital imaging: Part 1. Normal anatomy

Advanced imaging techniques enable the radiologist to detect an increasing number of structures within the orbit not previously identifiable. We describe the imaging techniques and orbital anatomy with an emphasis on radiologically identifiable structures. In a second review of orbital pathology we present pathological processes that may involve these structures.     

Progesterone receptor expression in orbital cavernous hemangiomas

Orbital cavernous hemangiomas (OCH) have thick and highly cellular vascular walls. Ultrastructural studies have demonstrated the smooth muscle nature of these cells. Vascular neoplasms can modify their morphological and clinical features under hormonal stimulation. The purpose of the present study was to investigate the presence of smooth muscle markers and sex steroid receptors in 12 cases of OCH. Orbital cases were compared with cutaneous hemangiomas and subcutaneous angioleiomyomas. Smooth muscle actin (SMA) and desmin were localized in spindle cells of the vascular walls of all 12 cases studied. OCH showed immunohistochemical positivity with progesterone receptor (PR) antibody both in smooth muscular and in endothelial cells. For comparison, sex steroid receptors were studied in 10 cases of cutaneous cavernous hemangioma and in 10 cases of subcutaneous angioleiomyoma. PR was found in smooth muscle and endothelial cells of 6 out of 10 cases of subcutaneous angioleiomyoma and in none of the cases of cutaneous cavernous hemangioma. No positivity was obtained with estrogen receptor (ER) antibody in any of the cases tested. The present data suggest that OCH share morphological and immunohistochemical features with subcutaneous angioleiomyomas. Furthermore, immunohistochemical positivity with PR antibody indicates that OCH have to be added to the list of mesenchymal lesions that express sex steroid receptors. Received: 15 July 1999 / Accepted: 14 September 1999     

Growth of the fetal lens and orbit.

OBJECTIVES: To present normal lens and orbital measurement ranges by gestational age. METHODS: Three hundred and thirty-five women with uneventful pregnancies were evaluated from the 15th to the 40th week of gestation. Lens and orbital measurements were added to routine biometric measurements for normal fetuses. RESULTS: A strong linear correlation was observed between gestational age (GA) and lens diameter (R(2)=0.89; P<0.0001), circumference (R(2)=0.89; P<0.0001), and surface (R(2)=0.90; P<0.0001). A linear correlation was also found between GA and orbital diameter (R(2)=0.92; P<0.0001), circumference (R(2)=0.92; P<0.0001) and surface (R(2)=0.95; P<0.0001). A linear growth function was observed between biparietal diameter and both lens diameter (R(2)=0.90; P<0.0001) and orbital diameter (R(2)=0.94; P<0.0001). CONCLUSION: Lens and orbital measurements provide normative data for fetal growth and development. These data may be also helpful in detecting fetal ocular anomalies.     

The steroid effect on the blood-ocular barrier change induced by triolein emulsion as seen on contrast-enhanced MR images.

OBJECTIVE: The purpose of this study is to evaluate the effect of dexamethasone on the damaged blood-ocular barrier caused by triolein emulsion, using contrast-enhanced MR imaging. MATERIALS AND METHODS: An emulsion of 0.1-mL triolein in 20 mL of saline was infused into the carotid arteries of 32 cats, 12 cats were placed in the treatment group and 18 cats were placed in the Control group. Thirty minutes after the infusion of triolein emulsion, a set of orbital pre- and post-contrast T1-weighted MR images (T1WIs) were obtained. Infusion of 10 mg/kg dexamethasone into the ipsilateral carotid artery of each of the cats in the treatment group cats and 20 mL saline in each of the cats in the control group was given. A second set of pre- and post-contrast orbital T1WIs were obtained three hours following triolein emulsion infusion. Qualitative analysis was performed for the the anterior chamber (AC), the posterior chamber (PC), and in the vitreous humor of the ipsilateral and contralateral eyes. The signal intensity ratios of the ipsilateral eye over the contralateral eye were quantitatively evaluated in the three ocular chambers on the first and second set of T1WIs, and were then statistically compared. RESULTS: Qualitatively, the AC, the PC or the vitreous did not show immediate contrast enhancement on the first and the second set of post-contrast T1WIs. However, the AC and the PC showed delayed contrast enhancement for both groups of cats on the second pre-contrast T1WIs. No enhancement or minimally delayed enhancement was seen for the vitreous humor. Quantitatively, the signal intensity ratios in the PC of the treatment group of cats were statistically lower than the ratios of the control group of cats for the second set of T1WIs (p = 0.037). The AC and vitreous showed no statistically significant difference between the feline treatment group and control group (p > 0.05). CONCLUSION: Contrast-enhanced MR images revealed increased vascular permeability in the PC of the eye after infusion of triolein emulsion. Dexamethasone seems to decrease the breakdown of the blood-aqueous barrier in the PC.     

Orbital alveolar soft part sarcoma: Histopathologic report of two cases

Alveolar soft part sarcoma is considered as a distinct histopathological entity with rare cases reported from the orbit area. Two cases of alveolar soft part sarcomas occurring in the orbit of two patients along with their histopathologic findings are reported herewith. In both cases, the patients presented with eyelid swelling and proptosis. The diagnosis was made by incisional biopsies and histopathology. The literature is reviewed regarding occurrence of this tumor, its diagnosis and management.