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.
马方综合征(Marfan syndrome,MFS)是与晶状体异位有关的常见的全身性疾病，约50%~80%的MFS患者存在晶状体异位。该文报道一例21岁的男性患者，因患有MFS致双眼晶状体半脱位伴双眼并发性白内障，先后实施白内障摘除人工晶状体植入合并囊袋张力环悬吊固定术，术后保留低度近视，随访期间未发现眼压升高、人工晶状体严重移位及囊袋皱缩等并发症。
Marfan syndrome (MFS) is a common systemic disease associated with lens heterotopia, and about 50%~80% of Marfan patients have lens heterotopia. This article reports a case of a 21-year-old male patient who suffered from bilateral lens subluxation and concurrent cataract due to MFS, who underwent cataract extraction and IOL implantation with CTR suspension fixation was performed to preserve low-grade myopia after surger y. No complications such as increased intraocular pressure, intraocular lens severely displacement and capsular bag shrinkage were found during follow-up.
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.
强直性瞳孔在临床工作中较为少见，容易被误诊，典型的强直性瞳孔为单侧，随着病程的进展，逐渐转变为双侧。新发双侧强直性瞳孔更是极为罕见，应当引起重视。其典型的临床表现包括瞳孔散大、瞳孔运动迟钝、节段性瞳孔麻痹、无其他眼部器质性病变。依据病因可以分为继发于急性眼内肌麻痹、合并有全身疾病以及特发性三类。目前治疗方面多以病因治疗为主。
Tonic pupil is rare in clinical work and likely to be misdiagnosed. The typical tonic pupil is unilateral, which gradually turns to bilateral with the progress of disease. Newly bilateral tonic pupil is extremely rare, which captivates widespread attention. Typical clinical manifestations include dilated pupils, slow pupil movement, segmental pupillary palsy, and no other ocular diseases. On this basis, the causes can be divided into three categories, including secondary to acute intraocular myoparalysis, complicated with systemic disease and idiopathic type. At present, etiological treatment is the main treatment.
Two patients aged of 30 and 22 (female in cases 1, and male in case 2) both complained of unilateral blurring of vision and scotoma within a week of being diagnosed with dengue fever. No other abnormal findings were found in their anterior segment. Retinal examination revealed blurring of the optic disc margin and several white spots in the posterior in both cases. Optical coherence tomography (OCT) imagery revealed that the white spots were only located in the retinal outer layers. Macular cystic foveolitis were also found in case 1 and diff used macular edema in case 2. In case 1, visual and retinal recovery were seen to resolve spontaneously. In case 2, patient had complete visual recovery two months after onset of the disease after being treated with steroids but central scotomata has continued to persist.
Two patients aged of 30 and 22 (female in cases 1, and male in case 2) both complained of unilateral blurring of vision and scotoma within a week of being diagnosed with dengue fever. No other abnormal findings were found in their anterior segment. Retinal examination revealed blurring of the optic disc margin and several white spots in the posterior in both cases. Optical coherence tomography (OCT) imagery revealed that the white spots were only located in the retinal outer layers. Macular cystic foveolitis were also found in case 1 and diff used macular edema in case 2. In case 1, visual and retinal recovery were seen to resolve spontaneously. In case 2, patient had complete visual recovery two months after onset of the disease after being treated with steroids but central scotomata has continued to persist.
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.
暴发性脉络膜上腔出血(subchoroidal expulsive hemorrhage，SEH)是内眼手术中罕见且严重的并发症，广东省人民医院眼科收治1名因晶状体完全脱位继发青光眼的女性患者，73岁，其手术过程中发生SEH，现报告如下。通过回顾病例，讨论及分析SEH的原因、危险因素及治疗。
Subchoroidal expulsive hemorrhage (SEH) is one of the rarest and worst complications of intraocular surgery. We treated one patient with secondary glaucoma due to complete dislocation of the lens, who developed SEH during the surgery. In this case report, the causes, risk factors and treatment of subchoroidal expulsive were discussed and analyzed by reviewing the case.
该文报道一例30岁的男性患者因“双眼自幼视力不佳，强光下视物模糊加重4年余”就诊，经过眼部检查评估，诊断为双眼瞳孔残膜、双眼屈光不正。患者接受一期双眼瞳孔残膜切除、二期双眼行有晶状体眼后房型环曲面人工晶状体(toric implantable collamer lens,TICL)植入手术，术后视力恢复良好。文章回顾了该例患者的诊治过程，为临床屈光不正同时伴有瞳孔残膜患者的诊治提供参考。
A 30-year-old male patient presented at our institution with a history of poor vision in both eyes since childhood, exacerbated by blurriness under bright light for over four years. Following a comprehensive ophthalmic examination, the patient was diagnosed with bilateral pupillary membrane remnants and refractive errors. The patient underwent a two-stage surgical intervention, starting with the removal of the pupillary membrane remnants, followed by the implantation of toric implantable collamer lenses (TICL) in the posterior chamber of the lensless eyes. Postoperative outcomes were favorable, with significant improvement in visual acuity. This article reviews the therapeutic journey of the patient, offering insights into the diagnosis and management of individuals with concurrent refractive anomalies and pupillary membrane remnants, thereby contributing to the clinical discourse on the subject.
患者女，60岁，因“右眼前黑影飘动1月，视力下降8天”就诊。视力：右眼0.1，不能矫正；左眼0.6矫正0.9。右眼眼底见视盘周围边界清晰不规则灰白色区，并波及中心凹。视野检查：右眼对应眼底病灶的视野缺损；左眼正常。光学相干断层成像术(optical coherence tomography，OCT)显示灰白色区域椭圆体带不规则、缺失，视网膜色素上皮(retinal pigment epithelium，RPE)层见数个指状隆起。眼底自发荧光(autofluorescence，AF)示：受影响区域内呈高荧光和部分不规则低荧光区。荧光素眼底血管造影(fundus fluorescein angiography，FFA)示：早期见荧光渗漏，晚期荧光着染、蓄积。吲哚菁绿血管造影(indocyanine green angiography，ICGA)示：见以视乳头为中心，边界清晰的低荧光区。诊断：右眼急性轮状外层视网膜病变。治疗：给予抗炎和改善血循环4周，眼底灰白色环状带消失，视力明显好转。随访6个月，患者病情控制良好。
A 60-year-old woman was admitted to Chengdu Aidi Eye Hospital because of “dark shadow fluttering in the right eye for 1 month and vision loss for 8 days”. Visual acuity—with a myopic correction—was 0.1 with the right eye and 0.9 with the left eye. The right eye fundus presented a well-defined, irregular, grayish white area around the optic disc, and affected the fovea, corresponding to the visual field defect of the fundus lesion. Optical coherence tomography (OCT) showed that the ellipsoid bands in this region were irregular and absent, and several finger-like ridges were seen in the retinal pigment epithelium (RPE) layer. Fundus autofluorescence (AF): High fluorescence and some irregular low fluorescence in the affected area. Fundus fluorescein angiography (FFA): Fluorescence leakage was seen in the early stage, fluorescence staining and accumulation in the late stage. Indocyanine green angiography (ICGA): A well-defined low-fluorescence area centered on the optic nipple was observed. Diagnosis:Acute annular outer retinopathy. Treatment: Anti-inflammatory and improved blood circulation for 4 weeks, the gray and white ring of fundus disappeared and the visual acuity improved obviously
眼黄斑囊样水肿(cystoid macular edema,CME)是白蛋白结合型紫杉醇的罕见并发症。该文报告了一例60岁女性患者，在右侧乳腺癌根治术后进行为期7周的白蛋白结合型紫杉醇化学治疗，治疗过程中出现双眼视力下降，经眼科检查诊断为由白蛋白结合型紫杉醇引起的双眼CME。确诊后即刻停用白蛋白结合型紫杉醇，并采用口服乙酰唑胺治疗。经随访，患者停药20个月时双眼CME基本消失，同时双眼矫正视力恢复至1.0。该病例为化学治疗药物引起的CME，机制可能与紫杉烷类药物对Müller细胞和视网膜色素上皮层产生毒性作用有关。值得注意的是，其典型的特征表现为荧光素眼底血管造影未见明显的荧光渗漏。文章回顾了该病例的病程发展，并对其他文献中报道的白蛋白结合型紫杉醇诱导的CME病例的临床特点及诊疗进行了总结。同时，对白蛋白结合型紫杉醇诱导CME的潜在发病机制进行了讨论，旨在为眼科医生提供早期诊断和治疗此类疾病的思路。
Cystoid macular edema (CME) is a rare complication of nab-paclitaxel.. In our article, it is reported a case of a 60-year old woman who had undergone nab-paclitaxel chemotherapy for 7 weeks after a radical surgery for breast cancer.During the treatment, she reported vision declined, and was diagnosed as CME caused by nab-paclitaxel through ophthalmic examinations. The nab-paclitaxel was immediately discontinued after the diagnosis, and the patient was treated with oral acetazolamide instead. In the follow up visit, after stopping nab-paclitaxel for 20 months, CME was found to disappear basically, and the corrected visual acuity was restored to 1.0 in patient's both eyes. his case is CME caused by chemotherapy drugs. Its mechanism may be related to toxic effects of paclitaxel to Müller cells and the retinal pigment epithelial layer. Notably, its typical feature is that there is no obvious fluorescence leakage could be observed on fundus fluorescein angiography. In the article, the course and development of this case is reviewed, and the clinical characteristics and diagnosis and treatment of nab-paclitaxel induced CME cases reported in other literature are also summarized. At the same time, the potential the potential pathogenesis of nab-paclitaxel-induced CME is discussed, to provide reference to ophthalmologists for early diagnosis and treatment for this disease.
