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Ocular perspective of mucopolysaccharidosis 粘多糖病的眼部透视
Pub Date : 2023-07-01 DOI: 10.4103/hjo.hjo_7_23
 . Shrinkhal, Ashish Kothari, G. Mamgain
A hereditary condition known as mucopolysaccharidosis is characterized by a lack of enzymes necessary for the breakdown of glycosaminoglycans (GAGs). GAG is improperly broken down, resulting in extensive lysosomal buildup throughout the different organs. From minor systemic and ocular involvement to a severe phenotype that can be lethal even in the few months of life, the clinical spectrum varies. Physical and cognitive skills are further hampered by visual impairment brought on by corneal clouding, retinopathy, and optic nerve involvement. Enzyme replacement therapy and bone marrow transplants are two examples of treatment methods that help patients live longer and better lives. The preferred course of treatment for people with visibly significant corneal clouding is keratoplasty. A thorough preoperative evaluation and patient counseling regarding the anticipated visual outcomes as well as postoperative complications such as graft re-opacification, rejection, and glaucoma should be conducted. Future therapies such as substrate reduction therapy and targeted gene therapy may be able to reverse corneal clouding. These therapeutic approaches are still in the testing phase.
粘多糖病是一种遗传性疾病,其特征是缺乏分解糖胺聚糖(GAGs)所需的酶。GAG被不恰当地分解,导致溶酶体在不同器官中广泛积聚。从轻微的全身和眼部受累到甚至在几个月的生命中就可能致命的严重表型,临床谱各不相同。由于角膜混浊、视网膜病变和视神经受累导致的视力障碍,身体和认知能力进一步受到阻碍。酶替代疗法和骨髓移植是帮助患者延长寿命和改善生活的两种治疗方法。对于有明显角膜混浊的人,首选的治疗方法是角膜移植。术前应进行全面的评估和患者咨询,以了解预期的视力结果以及术后并发症,如移植物再混浊、排斥反应和青光眼。未来的疗法,如底物还原疗法和靶向基因疗法可能能够逆转角膜混浊。这些治疗方法仍处于试验阶段。
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引用次数: 0
An approach to proptosis: A review 一种预测方法:综述
Pub Date : 2023-07-01 DOI: 10.4103/hjo.hjo_13_23
Shreya V. Mishra, Sushovit Sharma Luitel, Sucharita Das, Anupam Singh
Proptosis is a vision-threatening condition, where abnormal protrusion of one or both eyeballs develop outwards from the orbit. Clinically, it is defined as a protrusion of the eyeball beyond 21 mm from the orbital rim or an asymmetry of more than 2 mm between both eyes. It can be classified based on onset as acute, sub-acute and chronic proptosis. The causes of proptosis can be broadly classified into infectious, inflammatory, autoimmune, endocrine, vascular, traumatic, and neoplastic entities. Quantitative measurement of proptosis is done by exophthalmometers such as Hertel’s, Luedde’s, Naugle and Gormaz exophthalmometers. Proptosis is evaluated using multiple investigations involving laboratory, ocular, radiological and histopathological studies. This article provides insight into various types, etiologies, and approaches toward the evaluation, diagnosis, and management of proptosis.
眼球突出是一种威胁视力的疾病,指一只或两只眼球异常突出,从眼眶向外发展。临床上定义为眼球突出距眶缘超过21mm或双眼不对称超过2mm。它可以根据发病分为急性、亚急性和慢性预后。前列腺突出的病因可大致分为感染性、炎症性、自身免疫性、内分泌性、血管性、外伤性和肿瘤性。通过Hertel’s, Luedde’s, Naugle和Gormaz等凸眼计来定量测量眼球突出。通过包括实验室、眼科、放射学和组织病理学研究在内的多种调查来评估畸形。本文提供了各种类型,病因,以及评估,诊断和治疗的方法。
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引用次数: 0
Bilateral vision loss as first presentation of vestibular schwannoma 以前庭神经鞘瘤为首发表现的双侧视力丧失
Pub Date : 2023-07-01 DOI: 10.4103/hjo.hjo_6_23
 . Shrinkhal, Arviand Kumar, Annanya Soni, Suyash Singh, Arvind Kanchan, Shailja Maurya, Shruti Gupta
Vestibular schwannoma is a benign, slow-growing tumor of the vestibular nerve, located at cerebellopontine angle. It is derived from the Schwann cells of the myelin sheath of cranial nerve eight. We present here a unique case of vestibular schwannoma, who visited us with a primary complaint of bilateral decreased vision. Detailed examination revealed bilateral papilloedema associated with right-sided cranial nerve five and eight involvements. Patient was operated for the right-sided cerebellopontine angle tumor under intraoperative neuromonitoring. Postoperatively, patient showed significant improvement, both symptomatically and clinically.
前庭神经鞘瘤是一种位于脑桥小脑角的良性、生长缓慢的前庭神经肿瘤。它来源于颅8神经髓鞘的雪旺细胞。我们在这里提出一个独特的前庭神经鞘瘤的情况下,谁访问了我们的主要投诉双侧视力下降。详细检查显示双侧乳头水肿与右侧颅神经5和8受累有关。患者在术中神经监测下行右侧桥小脑角肿瘤手术。术后患者症状及临床均有明显改善。
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引用次数: 0
Pediatric cataract: Current perspectives and management strategies 儿童白内障:目前的观点和管理策略
Pub Date : 2023-07-01 DOI: 10.4103/hjo.hjo_12_23
S. Khokhar
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引用次数: 0
Comparative study of effect of phacoemulsification on central macular thickness in diabetic and nondiabetic patients assessed with spectral domain optical coherence tomography 超声乳化术对糖尿病和非糖尿病患者黄斑中央厚度影响的光谱域光学相干断层成像比较研究
Pub Date : 2023-07-01 DOI: 10.4103/hjo.hjo_4_23
A. Agrawal, MohdGhaniul Hasan, Ashutosh Kumar Singh, S. Mittal, Smita Sinha, R. Samanta
Purpose: The aim of this work was to study the effect of uneventful phacoemulsification on central macular thickness (CMT) in diabetic and nondiabetic patients as assessed with spectral domain-optical coherence tomography. Materials and Methods: A prospective, observational, comparative, cohort study was carried out at a tertiary care center in North India from January 1, 2018 to June 30, 2019. A total of 90 diabetic patients and 95 nondiabetic controls undergoing phacoemulsification surgery were evaluated. CMT and visual acuity (VA) were noted preoperatively and postoperatively at the 6th and 12th weeks, respectively. Results: A total of 185 patients, 95 nondiabetic, and 90 diabetic patients were included in the study. The mean age (years) of the study population was 64.56 (10.38). The subclinical increase in macular thickness was maximum at 6 weeks postoperatively (P < 0.001) and was more in diabetic as compared to nondiabetic patients. Final macular thickness at 12 weeks was comparable in both diabetic and nondiabetic patients (P < 0.05). However, the difference in change in macular thickness (preoperative versus postoperative) between the two groups was not clinically significant at 6 weeks (0.67 µm) and 12 weeks (0.08 µm). Conclusion: Uncomplicated phacoemulsification surgery with posterior chamber implantation of an intraocular lens is associated with subclinical macular thickening. However, there is no significant increase in macular thickness after phacoemulsification, irrespective of the glycemic status.
目的:本研究的目的是研究平稳超声乳化术对糖尿病和非糖尿病患者中央黄斑厚度(CMT)的影响。材料和方法:2018年1月1日至2019年6月30日,在印度北部的一家三级医疗中心进行了一项前瞻性、观察性、比较性、队列研究。对接受超声乳化术的90例糖尿病患者和95例非糖尿病患者进行了评估。术前、术后第6周、第12周分别观察CMT和视力(VA)。结果:共纳入185例患者,95例非糖尿病患者和90例糖尿病患者。研究人群的平均年龄为64.56岁(10.38岁)。术后6周黄斑厚度的亚临床增加最大(P < 0.001),糖尿病患者比非糖尿病患者增加更多。糖尿病和非糖尿病患者12周的最终黄斑厚度相当(P < 0.05)。然而,在6周(0.67µm)和12周(0.08µm)时,两组之间黄斑厚度变化(术前与术后)的差异无临床意义。结论:单纯超声乳化手术合并后房型人工晶状体植入术与亚临床黄斑增厚有关。然而,无论血糖状态如何,超声乳化术后黄斑厚度没有明显增加。
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引用次数: 0
Determinants of postoperative alignment in patients of infantile esotropia 婴儿内斜视患者术后视位的决定因素
Pub Date : 2023-01-01 DOI: 10.4103/hjo.hjo_21_22
Anupam Singh, A. Ramawat, A. Agrawal, Rakesh Panyala, R. Verma, S. Mittal, B. Kumar
Purpose: The purpose of this article is to study the factors affecting postoperative alignment in patients of infantile esotropia with deviation more than or equal to 30 prism diopter (PD) after 12 weeks of follow-up. Materials and Methods: A total of 23 patients with infantile esotropia were included in this study over a period of 18 months. All the patients were assessed preoperatively and postoperatively at day 1, 3rd week, 6th week, and 12th week. The patients underwent horizontal muscle surgery alone or vertical combined with horizontal muscle surgery wherever required. All surgeries were planned and performed according to W.E. Scott table by a limbus-based approach. Results: Mean age of our study population was 13.36 ± 8.0 years (1.5–30 years). Fifteen (65.2%) patients were hypermetropic, 5 (21.7%) patients were emmetropic and 3 (13.1%) were myopic. Among the study participants, amblyopia was present in 13 patients (56.6%). The mean preoperative horizontal deviation was 48.04 PD ± 13.3 PD (range = 30–70 PD). Eleven patients had preoperative esotropia of >50 PD and 12 had <50 PD. The mean postoperative horizontal deviation was 5.1 ± 3.1 PD (range = 2–14 PD). Twenty-one (91.30%) patients had orthophoria, 2 (8.70%) patients had residual esotropia and none had overcorrection. Conclusion: The postoperative alignment was not influenced by age at the time of surgery (P = 0.67) and magnitude of preoperative deviation (P = 0.67) Further, the postoperative outcome was independent of the laterality of surgery (unilateral vs. bilateral, P = 0.6) and extraocular muscles operated (horizontal vs. combined horizontal and vertical, P = 0.537).
目的:研究偏差大于等于30棱镜屈光度(PD)的婴幼儿内斜视患者术后对齐的影响因素,随访12周。材料与方法:本研究共纳入23例婴幼儿内斜视患者,为期18个月。所有患者术前和术后分别于第1天、第3周、第6周和第12周进行评估。患者在需要时接受单行水平肌手术或垂直肌联合水平肌手术。所有手术均按照W.E. Scott表,采用边缘法进行计划和实施。结果:我们研究人群的平均年龄为13.36±8.0岁(1.5-30岁)。远视15例(65.2%),准远视5例(21.7%),近视3例(13.1%)。在研究参与者中,弱视患者有13例(56.6%)。术前平均水平偏差为48.04 PD±13.3 PD(范围30 ~ 70 PD)。术前内斜视> 50pd 11例,< 50pd 12例。术后平均水平偏差为5.1±3.1 PD(范围= 2-14 PD)。21例(91.30%)有正斜视,2例(8.70%)有残余内斜视,无矫直过度。结论:手术时的年龄(P = 0.67)和术前偏移程度(P = 0.67)不影响术后对齐,术后结果与手术侧边(单侧vs双侧,P = 0.6)和眼外肌(水平vs水平垂直联合,P = 0.537)无关。
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引用次数: 0
Premyopia: An esoteric concept in evolution 近视眼:进化中的一个深奥概念
Pub Date : 2023-01-01 DOI: 10.4103/hjo.hjo_3_23
P. Pandey
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引用次数: 0
Large consecutive esotropia after uneventful surgical correction of infantile exotropia: A post-operative surprise 婴儿外斜视手术矫正后的大范围连续内斜视:一个术后惊喜
Pub Date : 2023-01-01 DOI: 10.4103/hjo.hjo_2_23
Ashutosh Kumar Singh, Nisha Rani, Stuti Chand, S. Verma, Mittali Khurana, S. Mittal
The incidence of consecutive esotropia developing after exotropia surgery has been reported to be 6%–20%. Consecutive esotropia with limited abduction requires reoperation on the muscles that were operated during the first surgery. It is difficult to predict the result of the second surgery because it may convert to exodeviation after the advancement of lateral rectus (LR) muscle, the contracture of the previously recessed muscle, or hypertrophy of resected medial rectus (MR). We present a case of 20-year-old woman with infantile exotropia who underwent bilateral lateral rectus recession and left MR resection in view of a large primary position deviation of 55 prism diopter (PD) at a 6-meter distance. But the patient developed large consecutive esotropia of 50 PD within 3 weeks of surgery with abduction limitation in the left eye, which was highly suspicious of slippage of the left LR. But when the LR was explored, it was found at 16 mm from the limbus without any evidence of slippage. In view of the abnormal response after the first surgery, the LR was advanced by 9 mm, and was inserted at 7 mm from limbus. The patient was orthophoric on the first postoperative day, and the alignment was maintained till 6 months of follow-up. This case is unique in terms of unexpected large consecutive esotropia of 50 PD after uncomplicated routine strabismus surgery, which was corrected by advancement of one LR only. This event could be possibly due to lack of binocularity and fusion in these patients with an associated component of dissociated horizontal deviation. This report emphasizes the possibility of some unusual postoperative outcomes in spite of careful and meticulous preoperative workup, and uneventful squint surgery in patients with infantile deviation.
据报道,外斜视术后发生连续内斜视的发生率为6%-20%。有限外展的连续内斜视需要对第一次手术时手术过的肌肉进行再手术。很难预测第二次手术的结果,因为它可能在外侧直肌(LR)前进,先前凹陷的肌肉挛缩或切除的内侧直肌(MR)肥大后转化为外偏。我们提出一个20岁的女性婴儿性外斜视的病例,她接受了双侧直肌衰退和左MR切除,因为在6米距离上有55棱镜屈光度(PD)的大原发位置偏差。但患者术后3周内出现50 PD连续大内斜视,左眼外展受限,高度怀疑为左LR滑脱。但是当我们探查下腭时,发现它在离边缘16毫米的地方没有任何滑动的迹象。鉴于第一次手术后反应异常,将LR向前推进9mm,并在距角膜缘7mm处插入。患者术后第一天为正位,并一直保持到随访6个月。这个病例的独特之处在于,50 PD患者在简单的常规斜视手术后出现了意想不到的大范围连续内斜视,仅通过一个LR的推进进行了矫正。这一事件可能是由于这些患者缺乏双眼和融合,并伴有游离的水平偏差。本报告强调了一些不寻常的术后结果的可能性,尽管仔细和细致的术前检查,以及婴儿斜视患者的平稳性斜视手术。
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引用次数: 0
Bilateral retinochoroidal coloboma with left eye posterior dislocated lens 双侧视网膜脉络膜结肠瘤伴左眼后晶状体脱位
Pub Date : 2023-01-01 DOI: 10.4103/hjo.hjo_20_22
 . Shrinkhal
We present here a typical case of bilateral aphakia with bilateral retinochoroidal Ida Mann’s type 1 coloboma with posteriorly dislocated lens in the left eye. There was no associated retinal detachment in both the eyes and no vitreous reaction in the left eye. Taking into consideration, the poor visual prognosis and no inflammation in the left eye despite dislocated lens; no active intervention was planned.
我们报告一例典型的双侧无晶状体合并双侧视网膜脉络膜Ida Mann 's 1型结肠并左眼晶状体后部脱位的病例。双眼无视网膜脱离,左眼无玻璃体反应。考虑到左眼晶状体脱位,视力预后差,无炎症;没有计划积极干预。
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引用次数: 0
Crouzon syndrome and the eye 克鲁松综合征和眼睛
Pub Date : 2023-01-01 DOI: 10.4103/hjo.hjo_22_22
Anushree Gupta
Crouzon syndrome is characterized by premature fusion of cranial sutures (craniosynostosis) which can impair proper brain and craniofacial development. Craniofacial abnormalities include frontal bossing, midface hypoplasia, parrot beak nose, and hypoplastic maxilla. The most common ophthalmic manifestations of Crouzon syndrome are proptosis, exotropia, exposure keratopathy, hypertelorism, and optic atrophy secondary to chronic papilledema. Here we report a case of Crouzon syndrome with ocular complications.
Crouzon综合征的特征是颅缝过早融合(颅缝闭合),这可能损害大脑和颅面的正常发育。颅面畸形包括额部隆起、中脸发育不全、鹦鹉喙鼻和上颌骨发育不全。Crouzon综合征最常见的眼部表现是眼球突出、外斜视、暴露性角膜病变、远视和继发于慢性乳头水肿的视神经萎缩。我们在此报告一例伴有眼部并发症的Crouzon综合征。
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引用次数: 0
期刊
Himalayan Journal of Ophthalmology
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