报告一例视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)合并人体免疫缺陷病毒(human immunodeficiency virus,HIV)感染/获得性免疫缺陷综合征(acquired immune deficiency syndrome,AIDS)，并通过文献复习，总结其发病机制、临床特征、治疗及预后。检索文献包括7篇英文文献(8个病例)，1篇中文文献，共报道9例NMOSD合并HIV感染/AIDS病例，结合本文报道的1例共10例，其中5例为女性，5例为男性，3例HIV感染/AIDS为新发，其他病例的HIV感染/AIDS发病均早于NMOSD。临床表现上，7例均为视神经炎和脊髓炎同时或相继发生，2例表现为单相病程或复发性脊髓炎，1例仅表现为双眼相继发生的视神经炎，10例患者头或脊髓MRI均有典型的视神经或脊髓异常信号，伴或不伴强化。2例患者未进行水通道蛋白4(aquaporin protein-4,AQP4)抗体IgG检测，其余8例中5例AQP4抗体阳性、3例阴性。针对AIDS的治疗，10例患者中，8例接受了高效抗逆转录病毒治疗(highly active antiretroviral therapy,HAART)。针对NMOSD的治疗，10例患者中，急性期有8例患者接受糖皮质激素冲击治疗、3例患者接受血浆置换、2例接受丙种球蛋白治疗，序贯治疗期有6例患者接受免疫抑制剂治疗，其中1例因高胆红素血症停药。发生视神经炎的7例中，2例患者经治疗仍失明、5例视力部分恢复，发生脊髓炎的8例中，5例患者遗留截瘫或轻瘫、3例肌力部分恢复。1例因严重并发症去世。NMOSD合并HIV感染/AIDS临床较罕见，预后差，往往遗留严重的视力障碍及瘫痪等，临床治疗较为棘手，糖皮质激素和免疫抑制剂并非使用禁忌证，但制定治疗决策前需要充分考虑风险与获益的平衡。

A case of neuromyelitis optica spectrum disorders(NMOSD) complicated with human immunodeficiency virus(HIV) infection/acquired immunodeficiency syndrome(AIDS) was reported, and the pathogenesis, clinical characteristics, treatment and prognosis were summarized through the literature review. The retrieved literatures included seven English literatures (eight cases) and one Chinese literature, in which a total of nine cases of NMOSD co-infected with HIV infection/AIDS were reported. Combined with the case reported in this paper, the total number of cases was ten, among which five cases were female and five cases were male, three cases of HIV infection/AIDS were newly developed, and the other cases had earlier onset of HIV infection/AIDS than NMOSD. In terms of clinical manifestations, seven cases all had simultaneous or sequential optic nerve and myelitis, two patients presented with a uniphasic course or recurrent myelitis, and one case presented only with bilateral optic neuritis occurring sequentially in both eyes. All ten patients had typical abnormal signals of the optic nerve or spinal cord with or without enhancement on cranial or spinal MRI. Two patients did not undergo AQP4 antibody IgG testing , and of the remaining seven cases, five were positive for AQP4 antibodies and three were negative. For AIDS treatment, eight of the ten patients received highly active antiretroviral therapy(HAART). For NMOSD treatment, among the ten patients, eight patients received intravenous methylprednisolone,three patients received plasmapheresis, and two patients received intravenous immunoglobulin in the acute phase. Six patients received immunosuppressive therapy during the sequential treatment period, and one of them was discontinued due to hyperbilirubinemia. Of the seven cases with optic neuritis, two patients remained blind after treatment and five had partial recovery of vision. Of the eight cases with myelitis, five patients were left with paraplegia or mild paralysis, and three had partial recovery of muscle strength. One case died due to serious complications.NMOSD combined with HIV infection/AIDS is rare in clinic and has a poor prognosis. Patients are often left with severe visual impairment and paralysis. Clinical treatment is quite difficult, hormones and immunosuppressive agents are not considered as contraindications. Treatment decisions need to be made with fully considered about the balance of risks and benefits.

泪道肿瘤是比较罕见的泪道疾病。文献报道有超过55%的泪道肿瘤为恶性肿瘤，如果肿瘤不能完全切除或已经发生转移，可能会使复发率和死亡率增加。故临床上对表现为泪囊肿物的病例应详细检查，比如眼眶彩色多普勒超声、计算机断层扫描(computed tomography, CT)及磁共振成像(magnetic resonance imaging, MRI)检查，必要时行增强扫描，有条件还可以进行泪道内镜检查，充分预估病情，设计合理的手术入路和切除范围。如肿物较大或病情复杂，经过皮肤或者鼻内镜下入路作单一切口不能完整切除，可以内外路联合手术，以获得良好的视野，对肿物进行充分的游离分离，进行合适的切除。文章汇报了通过皮肤切口联合鼻内镜入路完成的2例泪道肿瘤切除术，1例术后病理为良性的乳头状瘤，术后5个月时复查未见复发，长期观察；另一例为NUT癌，确诊后患者已经至肿瘤科接受了化学治疗。提示表现为泪囊肿物的病例在术前应完善眼眶影像学检查，怀疑为恶性肿瘤时应完善全身重要脏器的检查，以充分评估病情。对于有手术指征的应选择合适的手术方案，尽量完整切除。术后应长期随访，以早期发现复发和转移，及时治疗。

Lacrimal tumors are a relatively rare disease of the lacrimal system. According to the literature, more than 55% of lacrimal tumors are malignant tumors. If the tumor cannot be completely removed or has already metastasized, it may increase the recurrence rate and mortality rate. Therefore, in clinical practice, if a case of a mass in the lacrimal sac is encountered, a thorough examination should be performed, such as orbital ultrasound scan, CT and MRI with or without contrast enhancement, and endoscopic examination of the lacrimal duct, if possible. The condition should be fully assessed to design a reasonable surgical approach and the extent of surgical resection. If the lesion is large or the condition is complex, a single incision through the skin or endoscopic approach may not be able to completely remove it, and an combined approach can be used to obtain a good view and fully dissect and separate the lesion for appropriate resection. This article reports two cases of lacrimal tumor resection performed through a skin incision combined with an endoscopic approach, one of which had a postoperative pathological diagnosis of benign papilloma and no recurrence was observed.5 months after surgery and Long-term follow-up is planned. The other case was NUT carcinoma. The patient had received chemotherapy in the oncology department of the general Hospital after diagnosis. It is suggested that the imaging examination of the orbit should be improved in the case of lacrimal tumor before operation, and the examination of the main organs of the body should be perfected when malignant tumor is suspected, so as to fully evaluate the condition. For the case with surgical indications, the appropriate surgical plan should be selected and the complete resection should be carried out as much as possible. Long-term follow-up should be carried out after surgery, so that recurrence and metastasis can be detected early, and timely treatment can be carried out.

目的：分析先天性泪腺皮肤瘘伴异位泪腺的临床表现，总结其治疗方案。方法：采用病例研究与文献回顾方法，记录1例确诊为先天性泪腺皮肤瘘伴异位泪腺患儿的外观照片、泪道探查冲洗情况、泪腺瘘管数字减影检查、泪腺瘘管CT造影检查等结果。予患者行泪腺瘘管下段及异位泪腺切除+泪腺瘘管上段转位结膜囊吻合代泪腺导管术，术中切除的病变组织行病理检查，术后随访。以“泪腺瘘”、“异位泪腺”为检索词，在 PubMed、CNKI 数据库中进行文献检索，检索到相关文献共 25 篇。结果：患儿为男性，5岁，眼科检查于左眼上睑中外1/3处见直径约1 mm瘘口，瘘口皮肤凹陷并有簇状毛发生长，有透明液体从瘘口阵发性流出。泪器检查示左上睑皮肤瘘管开口朝外上方，瘘管探查从瘘口进针，瘘管先是内下方走行再向外下走行，在距皮面3.5 mm处有一软性抵抗，加压不能突破，冲洗液原路反流，无脓液或血性液体反流。左眼泪腺瘘管数字减影检查示左眼泪腺瘘管造影剂存留，瘘管深部存在扇形腔隙。泪腺瘘管CT造影结果显示左眼外侧泪腺高密度影。术后随访6个月，患儿左眼上睑切口愈合好，未见瘘管复发，转位的泪腺瘘管成功将泪液引流入上穹隆结膜囊内。结论：先天性泪腺瘘同时合并先天性泪腺异位和毛发异位者临床上罕见，术前详细检查和精确诊断对指导治疗很有帮助；在明确主泪腺是正常的情况下，完整切除异位的泪腺组织，并行泪腺瘘管转位吻合于上穹隆结膜囊代泪腺导管术，是一种较好的治疗选择。

Objective: To demonstrate the clinical characteristics and surgical effects of congenital lacrimal gland cutaneous fistula with ectopic lacrimal gland. Methods: Observational case study and literature review. The results of appearance photographs, lacrimal duct probing and irrigation, digital subtraction imaging of the lacrimal fistula, and CT scanning of lacrimal fistula in a patient diagnosed with lacrimal gland cutaneous fistula with ectopic lacrimal gland were recorded. The surgical treatment for the patient was explored. The surgical strategy entailed resecting the lower segment of the lacrimal gland fistula and ectopic lacrimal gland, combined with transposing the upper segment of lacrimal gland fistula for conjunctival sac anastomosis, instead of using lacrimal gland catheter. Pathological examination and postoperative follow-up were conducted. Results: Ophthalmic examination revealed a fistula with a diameter of approximatedly 1 mm in medial-temporal 1/3 of the upper eyelid of the left eye. The skin surrounding the fistula was sunken and covered with tufts of hair. There was a paroxysmal discharge of clear fluid from the fistula. Lacrimal examination showed that the opening of the fistula on the skin of the left upper eyelid was directed outward and upward. Fistula exploration was conducted through the opening. The fistula tract initially coursed medially and caudally, then laterally and caudally. At a depth of 3.5mm from the dermal surface, a soft resistance was encountered that could not be overcome with forced pressure. The irrigation fluid refluxed along its original pathway, with no pus or bloody fluid regurgination. Digital subtraction imaging of the lacrimal fistula in the left eye demonstrated that contrast media remained and formed fanshaped spaces in the depth of the fistula. CT results of lacrimal fistula revealed a high density of lacrimal gland in left eye. During outpatient follow-up six months after surgery, the incision on the left upper eyelid was observed to have healed well, with no recurrence of the fistula The transposed lacrimal fistula successfully diverted tears into the conjunctival sac of the upper fornix. Conclusions: Congenital lacrimal gland cutaneous fistula combined with congenital ectopic lacrimal gland and ectopic hair is rare in clinical practice. Detailed preoperative examination and accurate diagnosis are extremely beneficial for guiding treatment. When the main lacrimal gland is normal, a better treatment choice involves completely removing the ectopic lacrimal gland tissue and transferring the lacrimal gland fistula to the conjunctival sac of the upper fornix to replace the lacrimal gland catheter.

Diabetic macular edema (DME) is a major sight-threatening cause in diabetic patients. We review the long-term outcome of four approved pharmacotherapy for treating DME, including intravitreal injections of corticosteroids (dexamethasone implants and fl uocinolone acetonide inserts) and anti-vascular endothelial growth factor (VEGF) (ranibizumab and aflibercept). They all show superior ability to improve vision and reduce macular thickness, comparing with sham injections or macular focal/grid laser treatment. Anti-VEGF agents result in low incidence of severe ocular or systemic adverse effects, but glaucoma and cataract should be aware after intravitreal corticosteroids. Prompt treatment with these agents can lead to a better outcome.

Diabetic macular edema (DME) is a major sight-threatening cause in diabetic patients. We review the long-term outcome of four approved pharmacotherapy for treating DME, including intravitreal injections of corticosteroids (dexamethasone implants and fl uocinolone acetonide inserts) and anti-vascular endothelial growth factor (VEGF) (ranibizumab and aflibercept). They all show superior ability to improve vision and reduce macular thickness, comparing with sham injections or macular focal/grid laser treatment. Anti-VEGF agents result in low incidence of severe ocular or systemic adverse effects, but glaucoma and cataract should be aware after intravitreal corticosteroids. Prompt treatment with these agents can lead to a better outcome.

Myopic choroidal neovascularization (mCNV) can cause severe visual impairment in highly myopic patients. We review the randomized trials of two approved pharmacotherapy for treating mCNV, including intravitreal injections of ranibizumab and afl ibercept. These two vascular endothelial growth factor (VEGF) antagonists show superior ability to improve vision and reduce macular thickness, comparing with sham injections or verteporfin photodynamic therapy (vPDT). There is no severe ocular or systemic adverse reaction reported in studies associated with ranibizumab and afl ibercept for mCNV. Prompt treatment with these agents can lead to a better outcome.

Myopic choroidal neovascularization (mCNV) can cause severe visual impairment in highly myopic patients. We review the randomized trials of two approved pharmacotherapy for treating mCNV, including intravitreal injections of ranibizumab and afl ibercept. These two vascular endothelial growth factor (VEGF) antagonists show superior ability to improve vision and reduce macular thickness, comparing with sham injections or verteporfin photodynamic therapy (vPDT). There is no severe ocular or systemic adverse reaction reported in studies associated with ranibizumab and afl ibercept for mCNV. Prompt treatment with these agents can lead to a better outcome.

The present study reports a case of a patient with choroidal neovascularization (CNV) associated with pseudoxanthoma elasticum (PXE). We observed the functional and anatomical improvement of the patient treated with intravitreal vascular endothelial growth factor (VEGF) inhibitor bevacizumab. The study also systematically searched the database for similar cases to provide a literature review. Data concerning the clinical features, treatment strategies and outcomes were extracted and analyzed. Retrospective interventional case report and systematic literature review. A 56-year-old healthy Chinese woman with CNV secondary to PXE was reported. Examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography and digital fundus photography. The patient managed with intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections (bevacizumab 1.25 mg/0.05 mL). The Cochrane Library, PubMed, OVID, and UpToDate databases were searched using the term pseudoxanthoma elasticum or Gr?nblad-Strandberg syndrome with the limits English. Articles that predated the databases were gathered from current references. Fundus examination revealed angioid streaks bilaterally and CNV in left eye (LE). After the patient underwent three intravitreal injections of bevacizumab, the LE showed absorption of the subretinal fluid and shrinkage of the CNV. Visual acuity (VA) was improved in her treated LE. Bevacizumab treatment was well tolerated with no adverse events reported. Approximately ten articles about 45 patients (49 eyes) describing CNV secondary to angioid streaks in PXE treated with anti-VEGF were found in the literature search. In the present case, bevacizumab of an initial three injection loading dose, achieved maintenance of visual function in the treatment of CNV associated with angioid streaks in PXE. Literature articles concluded that the intravitreal application of anti-VEGF is highly efficient for improving and stabilizing the lesion as well as the eyesight. So we believe that anti-VEGF therapy can be a great choice of treatment for CNV secondary to angioid streaks related PXE.

The present study reports a case of a patient with choroidal neovascularization (CNV) associated with pseudoxanthoma elasticum (PXE). We observed the functional and anatomical improvement of the patient treated with intravitreal vascular endothelial growth factor (VEGF) inhibitor bevacizumab. The study also systematically searched the database for similar cases to provide a literature review. Data concerning the clinical features, treatment strategies and outcomes were extracted and analyzed. Retrospective interventional case report and systematic literature review. A 56-year-old healthy Chinese woman with CNV secondary to PXE was reported. Examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography and digital fundus photography. The patient managed with intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections (bevacizumab 1.25 mg/0.05 mL). The Cochrane Library, PubMed, OVID, and UpToDate databases were searched using the term pseudoxanthoma elasticum or Gr?nblad-Strandberg syndrome with the limits English. Articles that predated the databases were gathered from current references. Fundus examination revealed angioid streaks bilaterally and CNV in left eye (LE). After the patient underwent three intravitreal injections of bevacizumab, the LE showed absorption of the subretinal fluid and shrinkage of the CNV. Visual acuity (VA) was improved in her treated LE. Bevacizumab treatment was well tolerated with no adverse events reported. Approximately ten articles about 45 patients (49 eyes) describing CNV secondary to angioid streaks in PXE treated with anti-VEGF were found in the literature search. In the present case, bevacizumab of an initial three injection loading dose, achieved maintenance of visual function in the treatment of CNV associated with angioid streaks in PXE. Literature articles concluded that the intravitreal application of anti-VEGF is highly efficient for improving and stabilizing the lesion as well as the eyesight. So we believe that anti-VEGF therapy can be a great choice of treatment for CNV secondary to angioid streaks related PXE.

前段巨眼(anterior megalophthalmos, AM)是一种罕见的双侧非进展性先天性眼前段扩大疾病，表现为大角膜、角膜厚度正常或轻中度变薄、前房明显加深、睫状环扩大和悬韧带松弛。早期症状可仅表现为角膜散光和屈光不正等，并发性白内障和晶状体脱位是AM患者视力下降的主要原因。眼前段解剖结构的异常使AM患者的白内障手术具有很大的挑战性。首先，极端前房深度引起的有效晶状体位置(ELP)预测误差及公式选择不当是导致其术后较大屈光误差的主要原因；其次，悬韧带松弛易导致晶状体脱位、后囊膜破裂和玻璃体脱出等术中并发症的发生；由于超大囊袋及悬韧带松弛，人工晶状体(IOL)偏心甚至脱位也是术后常见的并发症。因此，需根据患者悬韧带情况、晶状体混浊程度采取合适的手术方式及谨慎选择IOL的类型。采用手法小切口晶状体囊外摘除术，可避免超声乳化的高灌注压对悬韧带的进一步损伤，增加手术的安全性；植入光学面及襻宽大的IOL术后具有较好的稳定性；新公式如Barrett Universal Ⅱ、Kane和EVO等公式具有较好的屈光预测准确性。然而，目前关于AM患者的白内障手术治疗报道仍属于个案报道，未来还需要更大样本量的临床研究进一步证实。

前段巨眼(anterior megalophthalmos, AM)是一种罕见的双侧非进展性先天性眼前段扩大疾病，表现为大角膜、角膜厚度正常或轻中度变薄、前房明显加深、睫状环扩大和悬韧带松弛。早期症状可仅表现为角膜散光和屈光不正等，并发性白内障和晶状体脱位是AM患者视力下降的主要原因。眼前段解剖结构的异常使AM患者的白内障手术具有很大的挑战性。首先，极端前房深度引起的有效晶状体位置(ELP)预测误差及公式选择不当是导致其术后较大屈光误差的主要原因；其次，悬韧带松弛易导致晶状体脱位、后囊膜破裂和玻璃体脱出等术中并发症的发生；由于超大囊袋及悬韧带松弛，人工晶状体(IOL)偏心甚至脱位也是术后常见的并发症。因此，需根据患者悬韧带情况、晶状体混浊程度采取合适的手术方式及谨慎选择IOL的类型。采用手法小切口晶状体囊外摘除术，可避免超声乳化的高灌注压对悬韧带的进一步损伤，增加手术的安全性；植入光学面及襻宽大的IOL术后具有较好的稳定性；新公式如Barrett Universal Ⅱ、Kane和EVO等公式具有较好的屈光预测准确性。然而，目前关于AM患者的白内障手术治疗报道仍属于个案报道，未来还需要更大样本量的临床研究进一步证实。

近些年来，眼科疾病的临床诊断治疗及其病理发展的研究对医学影像学技术的要求日益增高，磁共振技术已广泛应用于研究眼科疾病的发病机制、治疗和分析预后。基于体素的形态学分析(voxel-based morphometry，VBM)作为一种新型的磁共振图像的分析方式，VBM可以对活体脑进行无创的形态学研究，定量分析磁共振图像中每一个单独体素内的白质、灰质的密度和体积的变化，从而反映对应区域的解剖学结构差异，能发现常规MRI不能检测到的灰质和白质结构的细微改变。不同于那些只作用于预设的感兴趣区域的分析方法，VBM完全没有偏向性，它探测全脑的异常变化，无需对感兴趣区的先验性假设，不会被研究人员的主观思维影响。这提供了一种全新的方法来探索眼科疾病中的神经病理变化，尤其在青光眼和弱视的研究中应用最多。

With the increasing requirements for medical imaging technologies in clinical diagnosis, treatment and pathological basis research of ophthalmic diseases, magnetic resonance imaging (MRI) has been broadly used in the diagnosis and prognostic evaluation of ophthalmic diseases. As a novel analytic method of MR images, voxel-based morphometry (VBM) quantitatively analyzes the changes in brain gray, white matter density and volume in each individual voxel in MR images to reflect the differences of anatomical structures in the corresponding areas, and it provides a novel way to reveal the neuronal pathological changes in ophthalmic diseases.

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.

脉络膜破裂大部分是由撞击引起的对冲伤，冲击力通过玻璃体传导眼底引起，因此一般脉络膜破裂会有比较明确的外伤或者钝挫伤病史。本文将报告1例16岁体校男生在无明显外伤史出现多发性脉络膜破裂伤，通过查阅文献发现有文献报道在隐匿性假性黄色瘤(pseudoxanthoma elasticum，PXE)疾病中可在无明显外伤或轻微外伤出现脉络膜破裂，并根据文献复习考虑本病例为隐匿性PXE可能。

Most of the choroidal rupture is mostly caused by impact injury, and the impact force is caused by the vitreous conduction through the fundus. Therefore, the choroidal rupture generally has a clear history of trauma or blunt trauma. This article will report a case of a 16-year-old boy in a sports school who developed multiple choroidal ruptures without obvious trauma history. Through literature review, it was found that choroidal rupture can occur without obvious or minor trauma in subtle pseudoxanthoma elasticum (PXE) disease, and based on literature review, this case was considered as a possibility of subtle PXE.