朗格汉斯细胞组织细胞增生症 (Langerhans cell histiocytosis,LCH) 是一种由骨髓细胞肿瘤性增殖引起的罕见疾病，多见于儿童。LCH临床表现多样，以骨骼受累最常见。该文报道了一例儿童颅眶沟通LCH，影像学检查结果提示患儿右侧眉弓处类圆形穿凿样骨质破坏，通过手术切除病灶，送组织病理学检查明确诊断，同时选择通过3D打印聚醚醚酮(polyetheretherketone,PEEK)材料修补颅眶缺损部位，PEEK材料匹配度高、安全性好，改善患儿预后，提升患儿生存质量。
Langerhans cell histiocyte (LCH) is a rare disease caused by the tumor-like proliferation of bone marrow cells, which is mostly seen in children. Its clinical manifestations can be diverse, in which the skeletal system is most involved. This paper reports a case of LCH in cranio-orbital communication of a child. The imaging results suggest that there is a round chisel damage at the patient’s right brow ridge. In terms of definitive diagnosis and treatment, this patient underwent surgical resection and histopathological examination. 3D-printed polyether-ether-ketone (PEEK) material was selected to repair the cranio-orbital defect. The material can achieve better biocompatibility, while 3D-printing technique allows higher matching degree, both help to improve the prognosis and quality of life of the patient.
甲状腺相关眼病(thyroid-associated ophthalmopathy,TAO)，又称Graves眼病，是与甲状腺疾病密切相关的一种器官特异性自身免疫性疾病。眼球突出是TAO的主要临床表现之一，也是临床上多数患者就诊的原因。眼球突出一方面会影响美观，另一方面可因眼睑闭合不全导致暴露性角膜炎或因眼眶压力增大导致压迫性视神经病变。眼眶减压术用于重度TAO已有过百年历史，从最早经外眦皮肤切开的传统外部切口入路进行骨性眼眶减压及脂肪减压到内镜下经鼻入路眼眶减压术，其安全性和有效性均已得到肯定。术后复视是眼眶减压术常见的并发症。近年来，随着眼眶减压术的发展，其越来越多地用于美容目的以矫正眼球突出。然而术后的新发复视仍然是困扰众多相关眼科医疗工作者的难题。近年来，多项研究对术后新发复视的相关因素进行了探讨，并由此对眼眶减压术进行改良，在对术后新发复视的减少方面取得不同程度的进展。该文对眼眶减压术后新发复视的研究进展进行综述，旨在促进专科医生更精准地开展TAO的手术，进而提高手术患者术后的生活质量及手术满意度。
Thyroid-associated ophthalmopathy (TAO), known as Graves’orbitopathy, is an organ specific autoimmune disease closely related to thyroid diseases. Exophthalmos is one of the main clinical manifestations of thyroid related ophthalmopathy and is also the reason for most patients seeking medical atention in clinical practice.Eyeball protrusion can afect aesthetics on the one hand, and on the other hand, it can lead to exposed keratitis due to incomplete closure of the eyelids or compressive optic neuropathy due to increased orbital pressure.Orbital decompression has been used to treat severe TAO that threatens vision for over 100 years, and its safety and efectiveness have been confrmed.However, postoperative new diplopia remains a challenge for many ophthalmic medical workers.In recent years, many studies have explored the relevant factors of postoperative new diplopia, and improved the surgery, achieving varying degrees of progress in reducing postoperative new diplopia.Tis article reviews the research progress of new diplopia afer orbital decompression, aiming to promote more accurate surgery for thyroid related eye diseases by specialized doctors.
本文总结了15例自膨胀水凝胶眶内植入术患儿围手术期的护理要点。术前主要评估患儿是否完善术前检查，给予患儿及其家属个性化的心理护理，进行术前准备以及禁食禁饮的管理。术后主要给予患儿安全管理，饮食、疼痛、眼部用药以及弹力绷带包扎护理，关注有无并发症的发生及给予相应的护理，并对患儿及其家属做好出院指导。15例患儿均顺利完成手术，术后均出现术眼疼痛，2例出现眶压增高，1例出现呕吐，均得到妥善处理。术后随访3~18个月，患儿均获得了较为满意的眼部外观，生活质量得到了提高。
This paper summarized the nursing experience of 15 children with self-expanding hydrogel orbital implantation during perioperative period. Before operation, children were fully evaluated, given with psychological care, preoperative preparation and management of fasting and drinking. After the surgery, the patients were mainly given with safety management, diet, pain, medicine and elastic bandage dressing care. Nurses should pay attention to the occurrence of complications and give corresponding nursing care and offer useful discharge guidance for the children and their parents. All 15 children completed the operation successfully, postoperative eye pain occurred in 15 cases after operation, and the orbital pressure increased in 2 cases, 1 case vomited, and all cases were properly treated. After postoperative follow-up for 3–18 months, 15 children were satisfied with the appearance of the eye,and the life quality was improved.
目前Wetlab眼科手术教学采用猪眼球作为教学模型，与实际手术差别较大，且无法满足外眼手术教学的需求。通过使用带有眼球、眼外肌、眶组织、眶骨及眼睑结构的全眼模型，可高度模拟真实手术场景，覆盖更多眼科手术教学需求。基于全眼模型构建多个眼科手术教学平台，依照培养阶段设计手术课程，创建新的评价体系，改变传统将“内眼”“外眼”分开的教学理念，可使年轻医师从一开始就建立眼科“分科而不分割”的整体思维，从而使眼科手术培训更加规范和完善。
Porcine eyes have been used as animal model in ophthalmic surgery training. However, it differs greatly from real surgery and cannot meet the needs of external eye surgery teaching. Porcine orbit model with eyeball, extraocular muscles, orbital tissue, bones and eyelids can be more realistic simulation of real surgeries and cover more needs for ophthalmic surgery teaching. By setting up ophthalmic surgery teaching platforms, designing staged course and creating new assessment methods based on porcine orbit model, the traditional concept about separation of internal and external eye is changed. This helps young doctors to establish a holistic view from the very beginning,that ophthalmic surgeries should not be split because of subspeciality. In this way, ophthalmic surgery training will become more standardized and perfected.
患者女，因左眼睑反复红肿3个月余就诊。眼眶CT检查：左眶前部可见边界清楚的低密度软组织影，病变向眶外上方延伸，通过位于蝶骨大翼与蝶骨嵴交汇处侵蚀性骨孔与颅内病变沟通。颅内可见额叶、颞叶散在分布的团块状高密度病灶。眼眶MRI检查：边界清楚的异常信号病灶从左上睑延伸至眶上方和眶外上方，呈囊性改变。颅内病变呈混杂信号，散在分布于颞叶和额叶。临床诊断为左眼眶、颅沟通性病变。于全身麻醉下行左眼眶病变切除术，术后病理诊断为眼眶棘球蚴囊肿(包虫病)。发生于眶、颅的沟通性包虫病少有报道，本病例提示对于眶、颅沟通性包虫病需要根据病变的性质、位置采取个性化治疗原则，术前影像学检查的判断分析是辅助治疗的重要手段。
A young female patient complained of recurrent redness and swelling of the right eyelid for more than 3 months.Orbital CT examination showed that low density soft tissue density shadow with clear boundary can be seen in the anterior part of the left orbit. The lesion extended to the upper part of the orbit and communicated with the intracranial lesion through the erosive foramen at the intersection of the great wing of the sphenoid bone and the sphenoid ridge. There were massive high-density lesions in the frontal and temporal lobes. MRI examination of orbit showed that the left upper eyelid extended to the upper orbit and the upper extraorbital region. There were cystic signal of the orbit lesion and mixed signals of intracranial scattering in temporal and frontal lobes. The clinical diagnosis was left orbital cranial communicating lesion. The patient underwent orbital tumor resection under general anesthesia and was diagnosed as echinococcosis by postoperative pathology. This case suggests that for orbital cranial communicating echinococcosis, individualized treatment should be adopted according to the nature and location of the lesions, in which imaging examination are important.
眼眶泪腺淋巴上皮癌是一种非常少见的恶性肿瘤。本例为一位46岁女性患者，表现为左眼睑红肿、眼球突出和视力下降，临床和影像学表现类似于泪腺炎型假瘤。手术完整切除后送检病理检查，其特征是未分化的癌巢之间有大量分化成熟的淋巴细胞、浆细胞浸润，确诊为淋巴上皮癌。术后行化学治疗和局部放射治疗，随访6.5年无复发。眼眶泪腺淋巴上皮癌的临床表现容易与泪腺炎型假瘤相混淆，应该进行病理学检查以明确其恶性表型，完整切除后辅助放化学治疗有较好的效果。
Lymphoepithelial carcinoma of the orbital lacrimal gland is a rare carcinoma. A 46-year-old female patient presented with red and swollen left eyelid, proptosis and blurred vision. The clinical and imaging manifestations were similar to inflammatory pseudotumor. Pathological examination was performed after complete resection.Pathological features showed undifferentiated carcinoma nests surrounded by obvious infiltration of non-neoplastic lymphocytes and plasma cells, which was consistent with the diagnosis of lymphoepithelial carcinoma.Chemotherapy and local radiotherapy were performed after the resection. The case did not relapse after 5 years of follow-up. The clinical manifestations of orbital lacrimal lymphoepithelial carcinoma were easily confused with inflammatory pseudotumor. Pathological examination should be performed to clarify its malignant phenotype.Adjuvant radiotherapy and chemotherapy after complete resection had a good effect.
目的：分析发生于眶骨的占位性病变的CT表现及其与组织病理学之间的关系。方法：回顾性收集15例经临床表现、影像学特征、病理证实为发生于眶骨占位病变患者的临床资料，分析其CT检查、组织病理学切片及其他临床表现。结果：在15例患者中，经病理诊断为骨瘤的患者有4例，骨内血管瘤3例，骨样骨瘤和骨化性纤维瘤各2例，嗜酸性肉芽肿3例，尤文肉瘤1例。骨瘤由成熟板层骨构成，对应在CT上呈现为高密度的骨样结节状隆起；骨内血管瘤病理主要表现为骨小梁间的畸形血管，在CT上表现为典型的“栅栏状”“蜂窝状”特征；骨样骨瘤以骨样组织和结缔组织为主，在CT上为类圆形的高密度影，中央为典型的低密度“瘤巢“影，外周为增生的骨密度影；骨化性纤维瘤由增生的纤维组织及骨样组织构成，CT表现为肿瘤表面常有骨壳包绕，病变多呈圆形或椭圆形；嗜酸性肉芽肿主要由大量朗格汉斯细胞组成，对应CT上的软组织肿块影，可伴溶骨性破坏；尤文肉瘤以低分化的小圆细胞为主，CT表现为虫蚀样骨质破坏，破坏的骨组织间有软组织病变。结论：发生于眶骨的各类病变有其特有的病理组成，不同的病理组成在CT上表现出相应的影像特征。
Objective: To analyze computed tomography (CT) manifestations of space-occupying lesions of the orbital bone and their relationship with histopathology. Methods: The clinical data of 15 patients with orbital bone occupying lesions confirmed by clinical manifestations , imaging features and pathological symptoms features were retrospectively collected, and their CT examination, histopathological sections and other clinical manifestations were analyzed. Results: Among the 15 patients, 4 cases were pathologically diagnosed with osteoma, 3 cases of intraosseous hemangioma, 2 cases of osteoid osteoma and 2 cases of ossifying fibroma, 3 cases of eosinophilic granuloma and 1 case of Ewing’s sarcoma. Osteoma was composed of mature lamellar bones, which corresponded to a high-density osteoid nodular protuberance on CT. The pathological features of intraosseous hemangioma were mainly malformed blood vessels between bone trabeculae and typical “fence” and “honeycomb” features on CT. Osteoid osteoma was mainly composed of bone-like tissue and connective tissue, mainly showing round high-density shadow, typical low-density “tumor nest” shadow in the center, and hypertrophic bone density shadow in the periphery. Ossifying fibroma was composed of proliferative fibrous tissue and bone-like tissue. CT showed that the surface of the tumor was often surrounded by bone shell, and the lesions were mostly round or oval. Eosinophilic granuloma was mainly composed of a large number of Langerhans cells, which corresponded to the soft tissue mass on CT and could be accompanied by osteolytic destruction. Ewing’s sarcoma was mainly composed of poorly differentiated small round cells, and CT manifestations were worm-eaten bone destruction with soft tissue lesions between destroyed bone tissues. Conclusion: Various lesions occurring in the orbital bone have their own special pathological composition. Different pathological components show corresponding imaging features on CT.
目的：探讨眼眶黏膜相关淋巴组织(mucosa-associated lymphoid tissue，MALT)结外边缘区B细胞淋巴瘤的临床表现、影像学特征及病理学特点，以期减少眼眶淋巴瘤的误诊，提高生存率。方法：对2020年1月至2020年12月中山大学中山眼科中心诊治的71例眼眶MALT结外边缘区B细胞淋巴瘤的临床、影像及病理学资料进行回顾性分析。结果：71例患者中，男38例，女33例；左侧眼眶31例，右侧眼眶34例，双侧眼眶6例；原发病例67例，复发病例4例；年龄23~84岁，病程为3个月~15年。最常见的临床表现是眼部肿块和不同程度的眼球活动受限；磁共振成像(magnetic resonance imaging，MRI)检查见密度均匀的软组织影，呈“铸造样”，眼球内未见侵犯；组织病理检查可见肿瘤由小至中等大小淋巴样细胞组成，瘤细胞呈弥漫或片状生长，核小到中等、不规则，核仁不明显，部分细胞呈单核样淋巴瘤细胞改变，其中9例可见浆细胞样分化，伴浆细胞分化的病例kappa与lambda的表达不对称。结论：眼眶MALT结外边缘区B细胞淋巴瘤有多种临床表现，影像学检查具有一定的特征，可辅助术前诊断。病理学检查可用于术后的准确诊断及分型，据此制定合适的治疗方案，提高疗效。
Objective: To investigate the clinical, imaging and pathological features of orbital extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT), so as to reduce misdiagnosis and improve survival rate.Methods: The clinical, imaging and histopathological data of 71 patients with orbital extranodal marginal zone B cell lymphoma of MALT who were treated in Zhongshan Ophthalmic Center, Sun Yat-sen University from Jan.2020 to Dec. 2020 were retrospectively analyzed. Results: There were 38 males and 33 females in the 71 patients.There were 31 cases located on the left orbit, 34 cases on the right orbit, and 6 cases were bilateral; there were 67 primary cases and 4 relapsed cases. The age ranged from 23 to 84 years old. The disease course ranged from 3 months to 15 years. The main clinical manifestations included ocular masses and limited eyeball movement.Magnetic resonance imaging showed a “cast-like” soft tissue shadow of uniform density, and no intraocular invasion is observed. Histopathological examination showed that the tumor was composed of small to medium sized lymphocyte-like cells. Tumor cells exhibited diffuse or lamellar growth, with small to medium-sized irregular nuclei, and the nucleoli were inconspicuous. Some of the lymphocyte-like cells showed mononuclear lymphoma cells changes, and plasmacytoid differentiation was observed in 9 cases, in which kappa and lambda expressions were asymmetric.Conclusion: The clinical manifestations of orbital extranodal marginal zone B cell lymphoma of MALT are various, and imaging examination has certain characteristics, which can assist preoperative diagnosis.Pathological examination can be used for accurate diagnosis and classification after operation, based on which appropriate treatment plan can be made and curative effect can be improved.
眼眶部副神经节瘤极为罕见，多属非功能性肿瘤。本病例为1位中年女性，以右眼眼睑抬举无力为主诉就诊，眼眶计算机断层扫描(computed tomography，CT)和磁共振成像(magnetic resonance imaging，MRI)检查发现右眼眼眶内肿物，手术完整切除，结合HE染色和免疫组织化学检查，病理组织学诊断为眼眶副神经节瘤，随访3年，肿瘤无复发。
Orbital paraganglioma is a rare disease and mostly belongs to non-functional tumors. In this report, we described a middle-aged female admitted to our hospital with the chief complaint of weak lifting of her right eyelid. Orbital computed tomography (CT) and magnetic resonance imaging (MRI) examination detected an intra-orbital mass in the right eye. Complete excision was conducted, and post-operative histopathological and immunohistochemical examination revealed the mass to be orbital paraganglioma. Within the 3-year follow-up,the patient showed no recurrence.
目的：探讨眼眶原发性滑膜肉瘤(synovial sarcoma，SS)的临床病理学及分子遗传学特点。方法：收集1例复旦大学附属眼耳鼻喉科医院眼科2020年10月收治并经病理学检查证实为眼眶原发性SS的病例，同时回顾性分析文献中已报道的10例眼眶原发性SS的临床及病理检查资料，包括临床表现、影像学检查、组织学特点、免疫表型及分子病理学检查结果。结果：患者女，53岁，因“复发性右眼眶内肿物13余年”收治入院。SS组织病理学：肿瘤由弥漫分布的单一短梭形细胞组成，肿瘤细胞异型性明显，胞质少，核分裂多见；肿瘤侵犯结膜下、巩膜表面、视神经鞘膜、眶内肌肉及纤维脂肪组织。免疫组织化学检查提示波形蛋白(Vimentin)、Calpolnin、CD99、Bcl-2均阳，SMARCB1(INI-1)部分阳/弱阳。荧光原位杂交(fluorescence in situ hybridization，FISH)法检测到SS18基因易位。回顾性总结文献中已报道的10例和本例(总共11例)眼眶SS患者，其中男性2例，女性9例，左眼6例，右眼5例；患者发病年龄为1~53岁，平均年龄22岁，中位年龄24岁。患者术前病程范围较广，为1周~13年。11例中，5例症状至少出现3年以上，多表现为进行性眼球突出伴眼球移位及运动受限，疼痛及视力下降。CT和MRI上多表现为分叶状或者卵圆形软组织肿块，部分因出血坏死出现囊性外观，增强扫描显示病灶呈不均匀强化。组织学上，本组11例眼眶SS中单相纤维型7例，双相型4例，单相纤维型中有2例存在分化差的成分。免疫组织化学染色显示：上皮样成分表达上皮标记(CKpan、CK7、CK19)和Vimentin；梭形细胞表达Vimentin、CD99、Bcl-2、Calpolnin、TLE1及灶性表达上皮标记。结论：眼眶原发性滑膜肉瘤罕见，形态上需要和眼眶其他软组织来源恶性肿瘤相鉴别，其具有特征性t(x:18)(p11;q11)染色体易位，产生SY T-SSX融合基因，分子病理学的检测有助于最后确诊。
Objective: To investigate the clinicopathological and molecular genetics features of synovial sarcoma (SS) of the orbit. Methods: We retrospectively reviewed 10 published cases of primary SS of the orbit, along with 1 case of primary SS of the orbit confirmed by pathology who was admitted to the ophthalmology department of Eye & ENT Hospital of Fudan University in October 2020. The clinical data, radiological findings,morphology, immunophenotype and genetic characteristics of the cases were analyzed. Results: Our case was a 53-year-old woman with an SS in the right orbit, which had recurred multiple times. Histopathologic examination showed a primitive tumor composed of spindled and ovoid cells. Focal infiltration was observed in adjacent structures, such as the sub-conjunctiva, scleral surface, optic nerve sheath, muscle, and fibro-fatty tissue. Immunohistochemistry showed positivity for vimentin, calponin, CD99, and Bcl-2 and loss of INI-1expression, which is typical of SS. Fluorescence in situ hybridization (FISH) showed the (X;18)translocation in the tumor cells. The analysis included 2 males and 9 females aged between 1 and 53 years old (mean: 22 years; median: 24 years). Among the SS cases, 6 left eyes and 5 right eyes (all monocular cases)were affected. Symptoms had been present from 1 week to 13 years in the case from our hospital, while in 5 cases, symptoms had been present for at least 3 years. Common clinical features of the patients included proptosis or globe displacement, decreased vision, and pain. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an ovoid mass with heterogenous enhancement and a cystic appearance,which was probably attributable to hemorrhage or necrosis. Of these 11 cases, 7 cases were biphasic SS,4 were monophasic fibrous SS, and 2 were poorly differentiated in monophasic SS. Immunohistochemistry revealed positivity for pan-cytokeratin (CKpan), CK7, CK19, vimentin, cluster of differentiation 99 (CD99),B-cell lymphoma 2 (Bcl-2), calponin and transducin-like enhancer protein 1 (TLE1). Conclusion: Primary SS of the orbit is extremely rare and needs to be distinguished from other spindle cell tumors of orbital soft tissue. The SS diagnosis is based on the presence of the t(X;18) (p11; q11) translocation, which results in an SYT-SSX fusion gene.
