视盘内出血伴视盘旁视网膜下出血(intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage, IHAPSH)的病例报道较为少见。此病好发于患有轻、中度近视的中青年，且伴有视盘向倾斜或小视盘的患者，具有自限性，视力预后良好，无后遗症，而且很少复发。IHAPSH的眼底表现为视盘内出血、视盘旁视网膜下出血，严重者可出现玻璃体积血。文章报道一例IHAPSH，患者为26岁中度近视女性，主要症状为左眼无痛性视力下降伴眼前黑影飘动6 d。双眼最佳矫正视力均为1.0。眼底检查发现双眼视盘偏小，呈倾斜位，右眼视盘鼻侧见红色视网膜下出血灶；左眼视盘内及视盘鼻侧、上方、颞上方视网膜浅层出血，视盘鼻侧见边界清晰的新月形红色视网膜下出血。未进行特殊治疗，2个月后出血完全吸收。此病病因复杂多样，需与多种眼底疾病相鉴别，及时行眼科相关检查以排除其他原因导致的视盘水肿、视盘出血。文章通过文献回顾，总结其临床特征、发病机制、诊断、鉴别诊断、治疗及预后。

Intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage (IHAPSH) is rarely reported. IHAPSH commonly affects young myopic eyes with tilted optic discs and spontaneously resolves without treatment, with an excellent prognosis for vision recovery and very rarely recur. The fundus manifestations of IHAPSH are intrapapillary hemorrhage, peripapillary subretinal hemorrhage, or even with vitreous bleeding. An 26-year-old female with mild myopia presented with symptoms of blurry vision and black filamentous floaters for 6 days. Her BCVA was 1.0 in both eyes. Fundus examination showed that both discs appear small, and elevated in the nasal regions. Intrapapillary hemorrhage, peripapillary subretinal hemorrhage and vitreous hemorrhage were observed in the left eye, while only peripapillary subretinal hemorrhage was seen in the right eye. The hemorrhage was resolved spontaneously without any complication after 2 months. The etiology of IHAPSH is complex, and it should be differentiated from other causes of papilledema and disc hemorrhage. The clinical characteristics, pathogenesis, diagnosis, differential diagnosis, treatment and prognosis were summarized through the literature review.

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.

        文章报道了一例5岁女性Dandy-Walker综合征（Dandy-Walker syndrome，DWS）患儿的临床表现、眼科检查及影像学特征。该患儿在确诊DWS后行后颅窝蛛网膜囊肿部分切除术，然而术后视力仍进行性下降，眼科随访过程中发现视网膜神经纤维层（Retinal Nerve Fibers Layer，RNFL)进行性变薄，提示颅内压尚未有效控制，视神经损害在进一步发展。由于DWS病变位置特殊性，头颅磁共振成像（Magnetic Resonance Imaging，MRI）及常规腰椎穿刺未能确定幕上颅内高压，后经侧脑室直接测压，证实仍然存在颅压异常增高。行右侧脑室-腹腔分流术后，患儿双眼视力有所提高，双眼RNFL）厚度无继续变薄。目前DWS的治疗方式包括后颅窝囊肿切除术、脑脊液分流术及内镜下第三脑室造瘘术。 术后DWS患者的颅内压的测量有特殊性，幕上与幕下区域之间的脑实质内压力可能形成显著的压力梯度，腰椎穿刺测压可能无法准确反映颅内压，脑室内测压方法则创伤更大。此时需要眼科检查提供支持颅内高压的证据，如RNFL厚度进行性下降、视盘水肿及视网膜血管形态异常，这些检查无创、简便、可多次重复，是临床医生评估颅内压的有效手段，建议纳入DWS患者术后颅内压的常规管理中。

This article reports the clinical presentation, ophthalmologic examination, and imaging features of a 5-year-old girl with Dandy-Walker syndrome (DWS). She underwent partial resection of an arachnoid cyst in the posterior cranial fossa after the diagnosis of DWS. However, her visual acuity continued to deteriorate after the surgery, and the ophthalmologic follow-up revealed progressive thinning of the retinal nerve fibers layer (RNFL), suggesting that the intracranial pressure (ICP) had not yet been effectively controlled, and the damage to the optic nerve was further developing. However, due to the special pathological changes of DWS, MRI and routine lumbar puncture was not able to exactly identify the supratentorial ICP, subsequent ICP measurement through the lateral ventricle confirmed that increased ICP was still existed. After performing a right ventriculoperitoneal shunt, the child's binocular vision improved, and the thickness of the RNFL in both eyes remained stable. Current treatment modalities for DWS include cystectomy, cerebrospinal fluid shunt and endoscopic third ventriculostomy. The measurement of postoperative ICP in DWS patients is complicated, because there may be a significant pressure gradient between the supratentorial and infratentorial regions, which is not accurately reflected by lumbar puncture manometry, and intraventricular manometry is much more invasive. Ophthalmologic examinations including progressive decrease in RNFL thickness, optic disc edema, and abnormal retinal vascular morphology are required to provide evidences of increased ICP. These examinations are noninvasive, simple, and repeatable. They are an effective means of assessing ICP, and are recommended to be included in the routine management of ICP in patients with DWS after surgery.

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.