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.
眼附属器淋巴组织增生性疾病作为一类疾病的总称,包括了良性淋巴组织增生、非典型性淋巴组织增生、IgG4相关眼病以及多种恶性淋巴瘤在内的数十种疾病类型。临床诊断此类疾病应将患者眼部体征、影像学检查与病理学检查紧密结合。随着免疫表型及分子病理等检测技术的进步,此类疾病之间的鉴别诊断正逐渐清晰。本文就眼附属器淋巴组织增生性疾病进行系统性描述,并重点探讨该类疾病的病理鉴别诊断。
Ocular adnexal lymphoproliferative disease, as a general term, contains reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, IgG4 related ocular disease and malignant lymphoma. The clinical diagnosis of this kind of disease should integrate patient’s symptoms, imaging features and pathology characteristics. Development of immunophenotyping, molecular pathology and other detection technology will help with the differential diagnosis of ocular adnexal lymphoproliferative disease. This article is going to discuss the etiology, epidemiology, diagnosis and treatment of ocular adnexal lymphoproliferative disease, with a focus on the clinicopathological differential diagnosis of such disease.
近年来日益强大的眼内液分子/细胞生物学检测技术因其简便、快捷和高效的特点,使得眼科医生在诊断眼内淋巴瘤时倾向于单纯只依据此类方法而淡化病理诊断的重要性。眼内液分子/细胞生物学技术因其本身只能“间接提示肿瘤细胞存在”的局限性而不能作为眼内淋巴瘤的确诊依据。眼内组织/细胞病理仍然是眼内淋巴瘤诊断的金标准,其价值和地位不能被其他任何分子/细胞生物学检测手段所替代。理解并掌握各种诊断、检测技术的优势和局限性,规范和优化眼内组织/细胞病理标本的采集、保存和送检流程有助于提高眼科临床医生对眼内淋巴瘤的诊断效率和医疗质量。
In recent years, more and more powerful molecular/cellular biological techniques of intraocular fluid have made ophthalmologists tend to only rely on these methods in the diagnosis of intraocular lymphoma because of their features of simplicity, fastness and efficiency. The molecular/cellular biological techniques of intraocular fluid cannot be used as the basis for the diagnosis of intraocular lymphoma because it can only indicate the existence of tumor cells indirectly. Intraocular tissue/cell pathology remains the gold standard for the diagnosis of intraocular lymphoma, and its importance cannot be replaced by any other molecular/cell biological methods. Understanding and mastering the advantages and limitations of various diagnostic techniques, standardizing and optimizing the collection, preservation and submission process of intraocular tissue/cell specimens will help ophthalmologists improve the diagnostic efficiency and medical quality of intraocular lymphoma.
黏膜相关淋巴组织(mucosa-associated lymphoid tissue lymphomas,MALT)淋巴瘤是原发性眼附属器淋巴瘤(primary ocular adnexal lymphoma,POAL)中最常见的病理类型。目前,原发性眼附属器黏膜相关淋巴组织淋巴瘤(primary ocular adnexal mucosa-associated lymphoid tissue lymphoma,POAML)的临床类型和临床表现尚未被眼科医师熟练掌握,临床治疗亦无共识和指南。本文根据POAML起源位置,重点介绍各临床类型的早中期临床表现,以及针对各临床类型和病变范围的个体化治疗方法。
Mucosa-associated lymphoid tissue lymphomas (MALT) lymphoma is the most common pathologic type in primary ocular adnexal lymphoma (POAL). Currently, the clinical types and manifestations of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) have not been well understood by ophthalmologists, and there is no consensus or guideline for clinical treatment. According to the original location, this paper focuses on the clinical manifestations of early and intermediate-stage POAML, as well as the individualized treatment for each clinical type and lesion range.
目的:分析各种病因摘除眼球的患儿中视网膜母细胞瘤(retinoblastoma,RB)的误诊率,并总结其临床和病理特点。方法:回顾性分析2003年以来在中山眼科中心因临床诊断为RB并接受眼球摘除的患者563例(577眼),比较术前临床诊断和术后病理诊断,从中筛选出误诊病例,分析这些病例的临床和病理特点,包括患者年龄、性别、治疗过程、临床影像学、肿瘤病理分期、病理诊断以及分化程度。结果:共发现误诊病例22例(22眼),误诊率3.91%,所有误诊病例均为单眼摘除患者。常见的临床误诊类型为:与Coats病相混淆12例(12眼),与眼内炎症反应混淆5例(5眼),以及与眼内其他肿瘤混淆5例(5眼)。所有患者中,>4岁者55例,其中误诊12例,误诊率21.82%。结论:与RB相关的临床误诊并不罕见,其中最常见的误诊病因是Coats病,>4岁患者是误诊的主要人群。
Objective: To investigate the misdiagnosis rate in enucleated eyes for retinoblastoma (RB), analyze the clinicopathological features and summarize differential diagnosis. Methods: Retrospective analysis was performed on 563 cases (577 eye) undergoing eyeball enucleation in Zhongshan Ophthalmic Center Sun Yat-sen University since 2003. Misdiagnosed cases were screened out by comparing the preoperative clinical diagnosis and postoperative pathological diagnosis. The clinical and pathological features of those misdiagnosed cases were summarized, including age, gender, therapeutic process, imagological examination, pathological stage and pathological diagnosis. Results: Twenty-two cases had been misdiagnosed, which account for 3.91% of 563 enucleated eyeball cases. All of misdiagnosed cases had underwent monocular eyeball enucleation. Diseases that were easily misdiagnosed with RB were Coats disease (12/22), endophthalmitis (5/22) and other intraocular tumor (5/22). Among all patients, 55 patients were older than 4 years old, of which 12 cases were misdiagnosed, with a misdiagnosis rate of 21.82%. Conclusion: It is not unusual for clinical misdiagnosis of RB. Coats disease is the most frequent cause of RB misdiagnosis. Misdiagnosis rates is higher in patients over 4 years old.
目的:探索用细胞块制备试剂盒对眼内玻璃体液微量细胞制备细胞块的成功率,苏木素-伊红(hematoxylin-eosin,HE)染色效果及技术要点。方法:收集中山大学中山眼科中心临床病理科2020年9月至2021年1月由临床送检的25例玻璃体液(含玻璃体切割液),应用细胞块制备试剂盒制备细胞块后,常规固定、脱水、包埋、切片,随后进行HE染色,观察染色效果。结果:25例玻璃体细胞蜡块制作成功率达到100%,制片后HE染色效果好,背景干净,细胞形态清晰,核质对比分明。结论:应用细胞块制备试剂盒能将眼内玻璃体液微量细胞制成蜡块,极大提高了标本的利用率,为后续的病理研究提供丰富的材料。
Objective: To explore the effect and technical key points of the cell block preparation kit for collecting a few cells in ocular vitreous humor. Methods: A total of 25 cases of vitreous humor (including vitrectomy fluid) were collected from Zhongshan Ophthalmic Center, Sun Yat-sen University from September 2020 to January 2021. Cell block preparation kit was used to prepare cell blocks, which were routinely fixed, dehydrated, embedded, sectioned, and then hematoxylin-eosin (HE) stained. Results: The success rate of 25 cases of vitreous cell paraffin blocks reached 100%, and the morphology of the cells was clear with clean background and shape contrast of nucleus and plasma in HE staining. Conclusion: The cell block preparation kit can make the cells of intraocular vitreous humor into paraffin blocks, which greatly improves the utilization rate of specimens and is conducive to providing abundant materials for pathological studies.
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SYT-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.
目的:探讨眼囊尾蚴病的临床病理学特点。方法:回顾性分析7例眼囊尾蚴病的临床资料、大体与镜下病理改变。结果:男女性别比1:6,平均年龄24.7(中位数20)岁。临床以眼睑与眼周红肿、肿胀为主要症状3例,视物不清、视力下降2例,复视1例,结膜红肿1例。囊尾蚴寄生于球结膜1例,寄生于眼球内2例,寄生于眼眶内4例。7例均见到猪囊尾蚴,均在虫体内找到石灰小体,其中2例伴有坏死,可在坏死物中找到石灰小体。结论:眼囊尾蚴病以青少年女性多见,眼内型以视力下降为主要症状,眼眶型以眼睑与眼周红肿、肿胀为主要症状。找到囊尾蚴虫体与头节是眼囊尾蚴病明确诊断依据之一,石灰小体是该病重要病理诊断线索。
Objective: To explore the clinicopathological characteristics of ocular cysticercosis. Methods: The clinical data, grossing and microscopic pathological changes of 7 cases of ocular cysticercosis were analyzed retrospectively. Results: In this study, the male-to-female ratio was 1:6, the mean age was 24.7 (median age 20) years old. Of these patients, 3 patients presented with eyelid and periocular redness and swelling, 2 with blurred vision and decreased vision, 1 with diplopia, and 1 with conjunctival swelling and redness. In addition, 1 case was parasitized by cysticercus in the bulbar conjunctiva, 2 cases were in the eyeball and 4 cases were in the orbit. Cysticercus cellulosae containing calcareous corpuscle were observed in all cases, including 2 were accompanied by necroses with calcareous corpuscle. Conclusion: Ocular cysticercosis is found to be more common in female adolescents. Decreased vision is the main clinical symptom in intraocular infections, while orbital infections often show redness and swelling of eyelid and periocular. The finding of cysticercus and scolex in specimens is one of the confirmed diagnostic bases of ocular cysticercosis and calcareous corpuscles are important pathological diagnostic clues for this disease.
目的:比较重力液流与主控液流2种灌注方式下行白内障超声乳化手术对青光眼患者视盘血流的影响。方法:采用随机数字表法将患者分为2组,分别为重力液流灌注组和主控液流灌注组。记录术中超声乳化累积释放能量(cumulative dissipated energy,CDE),术后1天、1周、1个月和3个月患者最佳矫正视力(best corrected visual acuity,BCVA)、眼压、视盘血流密度及视网膜神经纤维层厚度。结果:主控液流灌注组术中CDE小于重力液流灌注组(5.6±1.3 vs 6.3±1.2,P=0.034)。术后1天重力液流灌注组视盘周围血管密度(circumpapillary vascular density,cpVD)、整个图像血管密度(whole en face image vessel density,wiVD)和视盘内血管密度(inside disc vascular density,inside disc VD)均高于主控液流灌注组(P<0.05),其余时间点差异无统计学意义(P>0.05)。术后1周和1个月重力液流灌注组视网膜神经纤维层厚度大于主控液流灌注组(P<0.05),术后1天和3个月未见明显差异。结论:相较于传统的重力液流灌注,主控灌注能够在青光眼患者白内障超声乳化手术中减少超声能量的使用,术后早期可减轻由术中高眼压引起的视盘炎症性充血,可以减轻对视网膜神经纤维层的影响。
Objective: To compare the influence of active versus passive phacoemulsification fluidics systems on optic disc blood flow in patients with glaucoma. Methods: Patients were divided into 2 groups by a random number table method, namely the active fluidics system group and the passive fluidics system group. The intraoperative cumulative dissipated energy (CDE) was recorded, and the best corrected visual acuity (BCVA), intraocular pressure, optic disc blood flow density and retinal nerve fiber layer thickness were measured at the follow-up of 1 day, 1 week, 1 month and 3 months. Results: During phacoemulsification, CDE in the active fluidics system group was lower than that in the passive fluidics system group (5.6±1.3 vs. 6.3±1.2, P=0.034). One day after the surgery, the circumpapillary vessel density (cpVD), whole image vessel density (wiVD) and inside disc vascular density (inside disc VD) in the passive fluidics system group were higher than those in the active fluidics system group (P<0.05), and the differences were not statistically significant at the rest of the follow-ups (P>0.05).The retinal nerve fiber layer in passive fluidics system group was thicker than that in active fluidics system group at the follow-ups of 1 week and 1 month (P<0.05), and the difference was not statistically significant at the follow-up ofs 1 day and 3 months. Conclusion: Compared with the traditional passive fluidics system, the active fluidics system can reduce the CDE during phacoemulsification surgery. It can reduce the inflammatory congestion of the optic disc caused by intraoperative high intraocular pressure on the early postoperative stage. In addition, it can also protect retinal nerve fiber layer.
目的:比较活体共聚焦显微镜和病理检查在角膜后部真菌感染的诊断阳性率,探讨两种检查方法在角膜后部真菌感染诊断中的价值。方法:回顾性病例对照研究。收集2009年11月至2020年12月在青岛眼科医院就诊并进行穿透性角膜移植手术治疗角膜后部真菌感染患者,术前均进行角膜刮片KOH涂片检查和活体共聚焦显微镜检查,术后病变角膜进行病理组织切片、过碘酸-Schiff法(PAS)染色和六亚甲基四胺银法(GMS)染色检查,比较不同检查方法诊断的阳性率。结果:18例角膜后部真菌感染患者角膜刮片KOH涂片均未检查到真菌菌丝,其中有16例患者经活体共聚焦显微镜检查到真菌菌丝(88.9%),而2例患者在术前活体共聚焦显微镜检查中未查到病原体。术后病理检查PAS染色联合GMS染色,18例患者中18例均可检查到真菌菌丝,角膜后部真菌感染患者病理切片中可见角膜深基质层变性坏死,大量炎症细胞浸润,PAS染色和GMS染色可见典型真菌菌丝侵犯角膜基质深层,而角膜基质浅层及上皮层均未查见真菌菌丝。结论:活体共聚焦显微镜诊断角膜后部真菌感染具有一定的局限性,联合术后病理组织切片和特殊染色检查有助于提高角膜后部真菌感染的诊断率。
Objective: To compare the diagnostic rate between in vivo confocal microscopy and pathological examination in retrocorneal fungal infection. Methods: It is a retrospective study. A total of 18 patients with retrocorneal fungal infection and received PKP surgery in the Qingdao Eye Hospital from November 2009 to December 2020 were enrolled. KOH smear and in vivo confocal microscopy examination were performed before surgery, and pathological examination including periodic acid-schiff (PAS) stain and Grocott Methenamine Silver (GMS) stain were performed after surgery. Patients were diagnosed retrocorneal fungal infection based on in vivo confocal microscopy and pathological examination. The diagnostic rates of the two methods were compared. Results: None of the 18 patients with posterior corneal fungal infection were found to have fungal hyphae in the corneal smear. Sixteen patients (88.9%) were found fungal hyphae by in vivo confocal microscopy. Corneal stroma necrosis and a large number of inflammatory cells were shown by postoperative pathologic examination, and all patients were found fungal hyphae in posterior corneal stroma with PAS stain and GMS stain. Conclusion: Confocal microscopy has unique advantages such as non-invasive and rapid examination in the diagnosis of fungal keratitis. However, it needs to combine with pathological examination for diagnosing the retrocorneal fungal infection.