Congenital cataract surgery: a retinal surgeon's viewpoint

Rhegmatogenous retinal detachments presenting many years after surgery for congenital cataracts frequently present formidable problems in management. The techniques and results of treatment are being presented and the implications for modern-day congenital cataract surgery are discussed.     

Glaucoma in aphakia and pseudophakia after congenital cataract surgery

Glaucoma is one of the most common causes of visual loss despite successful congenital cataract surgery. The overall incidence does not appear to have decreased with modern microsurgical techniques. The onset of glaucoma may be acute or insidious and notoriously refractory to treatment. Angle closure glaucoma may occur in the early postoperative period; but the most common type of glaucoma to develop after congenital cataract surgery is open angle glaucoma. Several risk factors have been identified and both chemical and mechanical theories have been proposed for its pathogenesis. Unlike children with congenital glaucoma, those with paediatric glaucoma following congenital cataract surgery are usually asymptomatic despite high intraocular pressure. They may require regular evaluation under anaesthesia, whenever there are any suspicious findings. Unlike congenital glaucoma, the first line of treatment for glaucoma in aphakia/pseudophakia may be medical. Traditional trabeculectomy in paediatric glaucoma following congenital cataract surgery has met with limited success. The addition of antimetabolites to trabeculectomy is known to inhibit fibrosis and enhance the success, but carries the lifelong risk of bleb-related endophthalmitis. Drainage implant surgery is a viable option to achieve longterm intraocular pressure control in this refractory group of patients. Cycloablative procedures may provide temporising treatment and should be reserved for patients with low visual potential. Diagnosis of glaucoma following congenital cataract surgery requires lifelong surveillance and continuous assessment of the problem. Further research is needed to understand the pathophysiology, prevention and treatment of this sight-threatening complication following successful cataract surgery in children.     

Nystagmus

Ophthalmologic nystagmus can be congenital and manifest/latent both of whose waveforms, compensatory mechanisms, and treatment are different. Physiologically, latent nystagmus may be reversed at will, whereas nystagmus and head nodding may be simultaneously triggered at will. Occasional oscillopsia is more frequent than is usually believed; extraretinal signals may be a reason for their absence in congenital nystagmus. Astigmatism could be the consequence of nystagmus, myopia is present in most of the cases of congenital nystagmus with blocking convergence, whereas depth-of-focus anomalies are probably due to the presence of nystagmus during its critical period of development. As for treatment, botulinum toxin injection, because of its temporary effect, is not used to treat nystagmus but to help in decision-making for surgery. Four large horizontal recti recession is the last-resort surgery which may be combined with other surgical procedures and can give unexpected results. Artificial divergence surgery by contrast is one of the most valuable procedures used in congenital nystagmus.     

额肌瓣悬吊术治疗重度先天性上睑下垂临床观察

目的:探讨额肌瓣悬吊术治疗重度先天性上睑下垂的临床效果。方法:采用额肌瓣悬吊术治疗重度先天性上睑下垂患者22例27眼。并对术后效果进行随访观察。结果:本组22例27眼,随访3～36mo,除1眼矫正不足外,余均取得良好效果。结论:额肌瓣悬吊术适用于治疗重度先天性上睑下垂,效果确切。     

Large bimedial rectus recessions in congenital esotropia.

The success rate of large (6 and 7 mm) bimedial rectus recessions in 45 congenital esotropes with deviations of 50 prism dioptres or greater was found to be 91%. Judgment of final alignment was made six weeks postoperatively, with an average follow-up of 13 months. Large bimedial rectus recessions are an effective surgical treatment for congenital esotropia. This procedure does not significantly alter adduction, and leaves other muscles available should further surgery be necessary. These findings show that initial surgery on three or more muscles is unnecessary in congenital esotropia.     

额肌瓣悬吊术矫正重度先天性上睑下垂

目的:探讨额肌瓣悬吊术矫正重度先天性上睑下垂的临床效果。方法:对28例35眼重度先天性上睑下垂患者,施行额肌瓣悬吊术,观察分析术中、术后并发症,随访观察术后效果。结果:本组病例均矫正理想,无严重术中术后并发症发生。结论:额肌瓣悬吊术矫正重度先天性上睑下垂的手术可靠,效果满意,是治疗重度上睑下垂较可靠的手术方法。     

重视儿童眼部整形手术时机和术式选择

儿童眼部畸形患者是眼部整形患者中的重要群体之一。儿童的生理、解剖与成年人有很大差异，儿童的视功能、眼眶及面部均处于发育期，其眼部畸形尤其是一些先天性畸形常伴有或最终导致视功能异常，先天或后天性的眼球缺失或发育不全则致眼眶及面部发育迟滞，因此儿童眼部整形不单纯为一种整形美容手术，更多需关注整形手术对儿童视功能及眼眶发育的影响，因此选择适当的手术时机及手术方式对于儿童眼部整形尤为重要。     

儿童先天性上睑下垂手术方法探讨

目的观察不同的手术方式矫治儿童先天性上睑下垂的治疗效果。方法对56例（100眼）儿童先天性上睑下垂采用上睑皮肤单切口,分3组：第1组利用额骨膜缝线式吊术,治疗年龄在3月～1．5岁的患儿。第2组利用上睑提肌缩短术治疗上睑提肌肌力为4—8mm的患儿。第3组利用额肌瓣悬吊术治疗上睑提肌肌力为0～6mm的患儿。结果随访6月～2a,额肌瓣组病例全达满意效果,而上睑提肌组12眼,矫正不足,额骨膜缝线悬吊组4眼上睑垂睑线消失。结论额肌瓣悬吊术为矫治儿童先天性上睑下垂的最佳手术方式。     

Second nonocular cancers in retinoblastoma: a unified hypothesis The Franceschetti Lecture

The procedure for congenital cataract surgery and its indications are discussed.     

Alternating ptosis after bilateral frontalis muscle suspension for congenital ptosis

PURPOSE: To report an unusual response to bilateral frontalis suspension surgery for congenital ptosis. METHODS: Case report. RESULTS: A 6-year-old child with infantile esotropia and bilateral congenital ptosis received a homologous fascia lata frontalis suspension. After surgery, the patient exhibited an alternating ptosis, caused by alternating unilateral frontalis muscle contraction, which has persisted throughout 3 years of postoperative observation. CONCLUSION: Symmetric frontalis muscle activation may not always occur after bilateral frontalis muscle suspension surgery for congenital ptosis. Amblyopia, strabismus, or fixation preference may predispose patients to use unilateral brow elevation, despite bilateral frontalis muscle suspension surgery.     

Management of congenital cataracts

The management of congenital cataracts at the Wilmer Institute is presented, with emphasis on the need for careful preoperative assessment. The indications and techniques for surgery in uncomplicated cataracts are discussed, and the advantages of using vitrectomy instruments in complicated congenital cataracts, such as those associated with persistent hyperplastic primary vitreous, are described.     

Visual acuity and binocular vision in children with pseudophakia

PURPOSE: To evaluate the results of cataract surgery in children with IOL implantation. MATERIALS AND METHODS: Our research involved 106 children (156 eyes) with congenital and traumatic cataract, in age from 18 months to 18 years, who underwent cataract surgery with intraocular lens implantation. We evaluated visual acuity for far vision and binocular vision after surgery. RESULTS: Visual acuity over 0.5 in unilateral congenital cataract was found in 27.25%, in traumatic cataract in 73%, whereas in bilateral congenital cataract visual acuity over 0.5 we received in 75% of operated children. Full binocular vision was obtained in children with good visual acuity (0.5 and more), with appropriate alignment of the eyes: 13.1% in unilateral congenital cataract, 45.4% in traumatic cataract and 55% in congenital bilateral cataract. CONCLUSIONS: Cataract surgery with intraocular lens implantation results in increase of visual acuity and in achievement binocular vision, if surgery is carried out in the early period, after it had been diagnosed.     

重度先天性上睑下垂的手术治疗

目的分析额肌筋膜瓣悬吊术和异体阔筋膜悬吊术矫正重度先天性上睑下垂的优缺点。方法对重度先天性上睑下垂66例87眼进行手术治疗,其中额肌筋膜瓣悬吊术30例45眼,异体阔筋膜悬吊术36例42眼,分析术后上睑下垂矫正效果、上睑弧度、双重睑成形及暴露性角膜炎发生情况。结果额肌筋膜瓣悬吊术取材方便、复发率较低、上睑弧度匀称;阔筋膜悬吊手术简单、重睑形成良好,但取材困难、复发率稍较高。结论额肌筋膜瓣悬吊术和阔筋膜悬吊术均能有效治疗重度先天性上睑下垂,前者无需特殊材料,术后效果较稳定。     

Ultrasound biomicroscopic patterns after glaucoma surgery in congenital glaucoma

OBJECTIVE: This study aimed to demonstrate specific morphologic patterns in congenital glaucoma after various surgical procedures by means of ultrasound biomicroscopy (UBM) and to investigate correlations between UBM morphology and the effectiveness of glaucoma surgery in reducing intraocular pressure. DESIGN: Observational case series. PARTICIPANTS AND INTERVENTIONS: Thirty four eyes of 18 consecutive patients, not older than 18 years, with congenital glaucoma and with a history of previous antiglaucomatous surgery underwent UBM examination of the anterior chamber angle in the treatment area and in an untreated region. MAIN OUTCOME MEASURES: The morphology of the anterior chamber angle region and the tissue reflectivity were analyzed. RESULTS: Specific UBM patterns of the anterior chamber angle in congenital glaucoma were observed after goniotomy, trabeculotomy, trabeculectomy, deep sclerectomy, and cyclodialysis. In the first months after surgery, a limited correlation was found between morphology and the success of filtering surgery. Adhesions of the iris or the ciliary processes to the trabeculectomy cleft were detected in 19 of 25 eyes after filtering procedures. CONCLUSIONS: In cases of cloudy cornea and unknown previous glaucoma surgery, UBM can be used to identify the type and localization of previous surgery in congenital glaucoma, thus assisting surgical planning for subsequent glaucoma management. The correlation between UBM morphology and the effectiveness of filtering surgery is less convincing than previously demonstrated in adults, possibly underlining the importance of individual nonsurgical factors for prognosis in congenital glaucoma.     

先天性白内障术后视网膜脱离的手术治疗

目的 探讨先天性白内障术后视网膜脱离的特点及手术方法。 方法 对42例先天性白内障患者44只白内障术后孔源性视网膜脱离眼的临床资料进行回顾分析,比较不同的视网膜脱离手术方法治疗后的效果。先天性白内障术后发生视网膜脱离平均间隔时间为14.8年。绝大多数白内障手术为吸出术并有后囊膜切开史 ;多数瞳孔不能散大并有后发性白内障形成。16只眼曾测眼轴,平均轴长(26.80±1.90) mm。视网膜脱离手术前裂孔发现率仅43.2%。 结果 巩膜手术成功率80.3%,玻璃体手术成功率85.7%。 结论 先天性白内障术后发生视网膜脱离间隔时间长;因瞳孔因素,周边眼底检查困难,巩膜手术成功率低,玻璃体手术是较理想的手术方式。 （中华眼底病杂志,2000,16：71-138）     

先天性膜性白内障临床研究进展

先天性白内障是导致世界范围内儿童盲的主要原因,其中先天性膜性白内障是临床上一种罕见的特殊类型的先天性白内障,由于晶状体纤维在母体内发生退行性变,皮质逐渐被吸收形成先天性膜性白内障。先天性膜性白内障临床表现类似于后发性白内障,但无屈光能力,因此先天性膜性白内障也被称为假性无晶状体。目前,国内外关于先天性膜性白内障的报道较少,而针对先天性膜性白内障发病机制的研究更少。明确先天性膜性白内障的发病机制,尤其是对基因遗传学的研究,有助于理解先天性白内障的发病机制和晶状体发育的分子机制。     

先天性白内障手术后计划外再手术原因及相关危险因素研究

目的　探讨先天性白内障手术后计划外再手术的原因及相关危险因素,为先天性白内障手术提供参考。方法　回顾性研究。收集2009年1月至2018年12月于青岛眼科医院就诊并行先天性白内障手术后计划外再次入院手术治疗的患者资料,分析患者再手术发生率、再手术时间以及发生原因等。结果　将白内障手术后计划外再手术36例（42眼）患者纳入本研究。计划外再手术占前三位的疾病分别为:后发性白内障(54.76%,23/42）、继发性青光眼(21.43%,9/42)、瞳孔闭锁（14.29%,6/42）;其他原因有人工晶状体异位（7.14%,3/42）、视网膜脱离（2.38%,1/42）。计划外再手术发生率在0～3个月龄组为13.29%,显著高于4～12个月龄组（3.82%）和>12个月龄组（1.96%）,组间差异有统计学意义（P=0.00）;低月龄（≤3个月）白内障手术、一期未植入人工晶状体与后发性白内障、继发性青光眼和瞳孔闭锁均高度相关（均为P＜0.05）。结论　后发性白内障、瞳孔闭锁和继发性青光眼为先天性白内障手术后计划外再手术的主要原因,低月龄（≤3个月）患者行白内障手术与计划外再手术高度相关。     

额肌瓣悬吊术治疗重度先天性上睑下垂

目的:探讨额肌瓣悬吊术治疗重度先天性上睑下垂的手术治疗效果。方法:对55例75眼重度先天性上睑下垂患者进行额肌瓣悬吊术,随访时间3～12(平均6)mo。结果:术后矫正满意69眼(92%),基本满意5眼(7%),欠矫1眼(1%),无明显过矫发生。结论:额肌瓣悬吊术是治疗重度先天性上睑下垂的一种疗效可靠的手术方法。     

Congenital and Developmental Cataracts: Focus on Strabismus Outcomes at Long-Term Follow-Up

Purpose: To evaluate the incidence of strabismus in congenital and developmental cataract surgery in patients with a follow-up longer than five years. Methods: All patients with congenital and developmental cataracts observed from 1996 to 2013 with a follow-up longer than five years were retrospectively included. Results: We included 117 patients (58 females and 59 males, mean age 0.62±0.3 years, 160 eyes) with congenital cataracts and 73 patients (32 females and 41 males, mean age 6.63±0.7 years, 121 eyes) with developmental cataracts. Before cataract surgery, strabismus was present in 88 patients with congenital cataracts (75.2%) and in 30 patients with developmental cataracts (41.1%) (p=0.01). After a follow-up of 9.26±1.3 years (range, 5–14 years), a significantly greater incidence of strabismus was observed after surgery only in patients with unilateral congenital cataracts who underwent cataract removal and primary IOL implantation (p=0.02). Distance BCVA and near BCVA were better after surgery for developmental cataracts (p<0.05). Presence of binocular vision was more frequent after surgery for developmental cataracts (p=0.001). Conclusion: Incidence of strabismus and postsurgical onset of strabismus at long-term follow-up was greater in patients with congenital cataracts.     

环扎带悬吊术治疗儿童重度先天性上睑下垂的临床分析

目的观察硅胶环扎带悬吊术在先天性重度上睑下垂术中的远期治疗效果。方法应用环扎带额肌悬吊术治疗先天性重度上睑下垂患儿23眼。随访6月~3年,平均1.9年。观察术后上睑下垂的矫正效果及并发症情况,并与在我院行额肌筋膜瓣悬吊术的病例对比分析。结果环扎带悬吊术23眼,满意17眼（73.9%）;额肌筋膜瓣悬吊术18眼,满意15眼（83.3%）,二者之间满意率差异无统计学意义（χ2=0.1308,P〉0.05）。结论环扎带悬吊术治疗先天性上睑下垂的手术简易,取材方便,术后并发症少,远期效果肯定,值得推广。