准分子激光原位角膜磨镶术(laser-assisted in situ keratomileusis,LASIK)是矫正屈光不正的重要角膜屈光手术方式之一。经过准分子激光切削的角膜，生物测量数据发生改变。对于此类患者，通过常规测量获得的参数数据以及使用常规计算公式确定的IOL屈光度将变得不再准确，由此将会导致术后较大的屈光误差，进而影响患者的视觉质量。本文报道一例46岁的男性白内障患者。该患者既往双眼屈光不正，曾接受过LASIK手术治疗。白内障术前角膜地形图检查发现该患者双眼存在角膜偏心切削，这为IOL屈光度的确定带来困难。手术医生通过角膜地形图判断角膜切削的居中性，在特定区域内选择角膜曲率K值，并采用Barrett True K公式计算出IOL屈光度。白内障术后患眼屈光误差相对较小，视力提高，视觉质量改善。
Laser-assisted in situ keratomileusis (LASIK) is a crucial corneal refractive surgery for correcting refractive errors. The cornea, after undergoing excimer laser ablation, undergoes changes in biometric measurements. For such patients, conventional measurements and IOL power calculations based on standard formulas may no longer be accurate, leading to significant postoperative refractive errors and subsequently impacting the patient's visual quality. This article presents a case of a 46-year-old male cataract patient who had a history of refractive errors in both eyes and had previously undergone LASIK surgery. Preoperative corneal topography revealed corneal eccentric ablation in both eyes, posing challenges in determining IOL power. The surgeon assessed the centration of corneal ablation using corneal topography, selected the keratometry value (K value) within specific corneal regions, and calculated the IOL power using the Barrett True K formula. Postoperatively, the cataract patient experienced relatively minor refractive errors, leading to improved vision and enhanced visual quality.
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：通过真实的临床病例，分析青光眼与非青光眼性视神经病变的不同表现，提高疾病的鉴别诊断能力。方法：采用病例研究与文献回顾方法，记录3例易误诊为正常眼压性青光眼（normal tension glaucoma, NTG）的神经眼科病例，通过视力、眼压、瞳孔对光反应、视盘形态、RNFL厚度、视野损害等特点，抽丝剥茧，得出诊断。结果：病例1视野呈弓形缺损，但视盘凹陷不深，且对侧眼“视盘拥挤”，随访期间发生急性前部缺血性视神经改变(anterior ischaemic optic neuropathy, AION)，最终确诊“AION遗留视神经萎缩”。病例2视力下降、视野缺损与视神经结构损害不相符，经完善MRI确诊为“左侧视神经鞘膜瘤”。病例3青年男性，中心视力下降，视盘凹陷不深，根据DNA结果确诊Leber遗传性视神经病变（Leber hereditary optic neuropathy, LHON）。结论：通过几个典型的神经眼科病例，掌握AION、视神经鞘膜瘤、LHON的结构和功能损害特征，如视盘凹陷不深、中心视力下降、视神经结构损害与视功能不相符，与NTG相鉴别。
Objective: To analyze the differentiating manifestations of glaucomatous and non-glaucomatous optic neuropathies using real clinical cases and to enhance the ability to make differential diagnosis. Methods: By using case study and literature review methods, we recorded three neuro-ophthalmic cases that were prone to being misdiagnosed as normal tension glaucoma (NTG). The diagnosis was established by extracting features related to visual acuity, intraocular pressure, pupillary light reflex, optic disc morphology, retinal nerve fiber layer (RNFL) thickness and visual field damage. Results: Case 1 presented with arcuate visual field defects, shallow optic disc cupping, and contralateral crowded discs. During follow-up, the patient subsequently developed acute anterior ischemic optic neuropathy (AION), and the final diagnosis confirmed was confirmed as optic atrophy secondary to AION. Case 2 exhibited a disproportionate visual acuity decline and visual field defects that were inconsistent with glaucomatous structural damage. MRI confirmed the diagnosis of left optic nerve sheath meningioma. Case 3 involved a young male who presented with central vision loss and shallow optic disc cupping. Genetic testing confirmed Leber's hereditary optic neuropathy (LHON). Conclusions: Through these representative neuro-ophthalmology cases, we illustrate the characteristic patterns of structural and functional damage associated with AION, optic nerve sheath meningioma, and LHON, such as shallow optic disc cupping, central vision loss, and inconsistency between optic nerve structural damage and visual function. These findings highlight the key differentiating features of these conditions from NTG.
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.
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.
