结节性硬化症(tuberous sclerosis complex，TSC)是一种累及多系统的常染色体显性遗传病，早期呈单一表现，容易漏诊、误诊，以眼部症状为首发特征的新生儿期病例少有报道。本文报告1例早产男婴，出生后1 d眼底筛查发现右眼视网膜散在多个灰白色半透明隆起灶及脱色素斑，回溯胎儿期超声心动图异常高度怀疑TSC，进一步行头颅MRI检查及家族基因检测，在新生儿期明确了这一诊断。

Tuberous sclerosis complex is a multisystemic disease with an autosomal dominant inheritance pattern. Missed diagnosis and misdiagnosis are common for patients with single manifestation in the early stage. There are few documented neonatal cases with ocular symptoms as primary presentation. Here we report a newborn boy presented with retinal hamartoma, retinal achromic patch, fetal cardiac rhabdomyoma and subependymal nodules.Subsequent genetic tests confirm a diagnosis of TSC.

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.

Merkel细胞癌(Merkel cell carcinoma，MCC)是一种少见的高度恶性的皮肤原发性神经内分泌癌，发生于眼睑的MCC更加罕见。本文对2例眼睑MCC患者的临床病史和治疗过程进行回顾和总结。1例初发患者经手术彻底切除，效果良好，随访7年，未再复发；1例患者在外院切除术后2个月，于眼睑原位复发，再次行手术彻底切除，2个月后同侧腮腺淋巴结及颈前淋巴结扩散，目前仍在肿瘤科进一步放化疗中。复习相关文献并结合本组病例提示，MCC诊断主要依靠病理检查，彻底切除病灶并结合术后放化疗是其治疗的主要手段，免疫治疗是目前新的发展趋势。

The Merkel cell carcinoma (MCC) is a rare highly malignant primary neuroendocrine carcinoma of the skin,especially in the eyelids. In this report, the clinical history and treatment course of 2 patients with MCC of the eyelid were reviewed and summarized. Patient 1 with primary MCC of eyelid was treated with complete surgical excision, with good results. Follow-up for 7 years had shown no recurrence. Patient 2 with the eyelid relapse in situ two months after resection in another hospital, was treated with complete surgical excision again, but the tumor metastasized to the ipsilateral lymphonodi parotidici and cervical lymph nodes two months after the surgery. The patient is now going through radiotherapy and chemotherapy in oncology department. According to the review of literatures, the diagnosis was based on the pathologic evidence. Complete removal of lesion combined with postoperative radiotherapy and chemotherapy is a major means of treatment for MCC. Immunotherapy will play a more important role in the future.

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.

线状皮脂腺痣综合征(linear nevus sebaceous syndrome，LNSS)是一种以皮脂腺痣(nevus sebaceous，NS)为特征性改变，同时合并癫痫、智力迟钝、神经缺陷或骨骼畸形等病变的疾病。本文报道1例经病理组织学检查确诊的LNSS患者，同时伴有双眼脉络膜骨瘤和脑部先天发育异常。由于线状皮脂腺综合征伴双眼多发异常较为少见，本文将总结该例患者的临床和病理表现，旨在为临床诊疗提供一定参考资料。

Linear nevus sebaceous syndrome (LNSS) is a disease characterized by nevus sebaceous (NS) and accompanied by epilepsy, mental retardation, nerve defect or skeletal deformity. We report a case of linear sebaceous nevus syndrome diagnosed by histopathological examination with bilateral choroidal osteoma and congenital developmental abnormalities of the brain. Since linear sebaceous gland syndrome with binocular abnormalities is relatively rare, this paper will summarize the clinical and pathological manifestations of this patient, aiming to provide certain reference for clinical diagnosis and treatment.

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.

睫状体髓上皮瘤是一种源自神经上皮层的恶性肿瘤，易因其伪装特性而被漏诊和误诊。本文报道了1例3岁9个月的男性患儿，眼部表现似晶状体破裂，经部分肿物切除活检，病理诊断为睫状体恶性髓上皮瘤。

Medulloepithelioma of the ciliary body is a kind of malignant tumor which arises from neuroepithelium. It is easily misdiagnosed or miss diagnosed due to its masquerade feature in the clinical practice. We report a boy with a tumor in his right eye at age of 3 years and 9 months presented first with lens rupture. Diagnosis of malignant medulloepithelioma of ciliary body was made pathologically by biopsy of the partial tumor.

霜样树枝状视网膜血管炎是一种少见的急性视网膜血管炎，多发生于健康青少年，病因不明，可能与病毒感染有关，糖皮质激素治疗有效，预后良好；也可继发于感染性疾病和全身疾病，预后较差。该文回顾了一例继发于异基因造血干细胞移植后的霜样树枝状视网膜血管炎，治疗后病情缓解，预后良好。

Frosted branch angiitis (FBA) is a rare acute retinal vasculitis which often occurs in healthy adolescents. The etiology of FBA is unknown, but its occurrence may be related to viral infection, glucocorticoid therapy is effective and has a good prognosis. FBA may also be secondary to infectious and systemic diseaseswith poor prognosis. In this paper, we reviewed a case of FBA secondary to allogeneic hematopoietic stem cell transplantation, which was relieved after treatment and had a good prognosis.

报告1例因电路短路产生电弧光烧伤患者双眼角膜的病例。专科检查:双眼角膜缘颞侧球结膜缺血区小于1/3，角膜上皮呈白色混浊，上皮部分脱落。入院给予清除角膜表面白色坏死组织、抗炎、扩瞳对症治疗，2周后角膜恢复透明，随访3个月患者晶状体及眼底未见明显异常；探讨电弧光在眼球不同部位的致病机制。

A case of corneal burn caused by arc light due to electrical short is reported. Specialist examination: ischemic areas of the temporal limbus bulbar conjunctiva in both eyes were less than 1/3, the corneal epithelium was white and cloudy, and the epithelium was partially peeled. The corneas became transparent after 2 weeks’ treatment of removing the white necrotic tissue, anti-inflammatory and dilating pupil. During the follow-up within 3 months, the patient’s lens and fundus showed no obvious abnormalities. The injury mechanism of arc light caused by electrical short to the eyeball is discussed.

患儿因“左眼红肿12 d”就诊。左上睑红肿(++++)，眼眶鼻侧可触及约黄豆大小硬结，界不清，质地中等，压痛(?)，伴上睑下垂，遮盖角膜下缘。双眼眼位正，左眼眼球外转轻度受限。眼眶及视神经MRI显示左眼上睑软组织肿胀，考虑炎性改变。入院诊断为左眼特发性眼眶炎性假瘤(Idiopathic orbital inflammatory pseudotumor，IOIP)。予以激素治疗后症状好转且随访期间无复发。本例患儿为拒绝外院活检而就诊于暨南大学附属第一医院的小儿IOIP病例，提示早期规范治疗是降低此病复发率的关键，尤其是儿童患者。

The pediatric patient was referred to our hospital with a 12-day history of left eye redness and swelling. On the orbital and nasal side of the eye, there was a firm, nontender mass about the size of soybeans, with unclear boundary, medium texture. Orbital examination showed left ptosis, covering limbus. Motility examination revealed both eyes were in normal position, and the left eye was slightly limited in extraocular rotation. Magnetic resonance imaging (MRI) revealed evidence of diffuse inflammation of left eyelid, and orbital inflammatory was considered. Excluding other etiologies, the child was diagnosed initially with idiopathic orbital inflammatory. The symptoms improved after hormone therapy and there was no recurrence during follow-up. This case was a case of IOIP in a child who was admitted to the First Affiliated Hospital of Jinan University for refusing biopsy from another hospital. The paper suggests that early standardized treatment is the key to reduce the recurrence rate of this disease, especially in children.