婴幼儿先天性角膜混浊穿透性角膜移植术临床疗效分析

Clinical effects of pediatric penetrating keratoplasty for congenital corneal opacity

目的探讨3岁以下婴幼儿先天性角膜混浊行穿透性角膜移植的临床疗效。方法回顾性系列病例研究。收集2009年6月至2016年12月于爱尔眼科医院集团行穿透性角膜移植术（PKP）的患儿12例（16只眼），其中男性6例（9只眼），女性6例（7只眼），年龄平均为（16．04±12．48）个月。所有患儿均为先天性角膜混浊，其中Peter异常I型8例患者（11只眼）；巩膜化角膜2例患者（3只眼）；中央部角膜皮样瘤合并虹膜粘连者2例（2只眼）。手术时年龄〈1岁的7例（7只眼）。术前观察患眼均有光照反应，其中4例（6只眼）有追光反应。除1例（2只眼）行PKP联合白内障摘除术外，其余均行单纯PKP术。术后严密复查角膜植片、缝线、前房情况，并检查眼压及视力。结果术后随访（33．17±22．60）个月，除1例单眼皮样瘤全层混浊患儿因3岁时手术，术后经过半年视力部分提高外，其余患者术后均在1周至1个月时间内视力获得明显提高。所有患儿手术均获得成功，术中无并发症发生。随访期内，4例（4只眼）发生排斥反应，其中3例Peter异常患儿发生急性排斥反应，药物控制后1例33个月的患儿植片保持半透明，但视力较前明显提高；2例1岁以下患儿植片恢复完全透明；1例3岁Peter异常患儿视力明显提高，术后2年发生免疫排斥反应，植片混浊，再次PKP术后半年又发生免疫排斥反应，导致植片混浊；该患儿另一只眼术后早期继发青光眼，抗青光眼手术失败，植片混浊。1例（2只眼）巩膜化角膜患儿术后1年时缝线松动未能及时发现，导致植片溃疡感染穿孔。结论婴幼儿先天性角膜混浊可以通过PKP获得有效的治疗，术后密切随访，及时处理并发症，家长积极配合给予规范视功能训练，均能获得比较好的视力。

Objective To report the clinical results of pediatric penetrating keratoplasty （PKP） in patients under 3 years old with congenital corneal opacity. Methods Retrospective study. Sixteen eyes of 12 patients who were treated with PKP in Aier Eye Hospital Group from June 2009 to December 2016 were enrolled in this study. All the patients were diagnosed as congenital corneal opacities： 8 cases （11 eyes） with Peter＇s anomaly I, 2 cases （3 eyes） with sclerocornea, and 2 cases （2 eyes） with corneal dermoid tumor combined with iris synechia. Seven cases （7 eyes） were under 1 year old. Eight cases （10 eyes） could not follow the light. Only 1 case （2 eyes） received PKP with extracapsular cataract extraction, and the others only had PKP. Postoperative examinations were performed more frequently than in aduhs, and sometimes general anesthesia was needed. Results The follow-up period was from 8 months to 6 years（33.17~22.60 months）. The postoperative visual acuity improvement was found in all eyes from 1 week to 1 month after surgery except a 3-year-old patient with corneal dermoid tumor with serious esotropia. All the surgeries were successful without intraoperative complications. Graft rejection occurred in 4 cases （4 eyes）. The graft of a 33-month-old patient became semitransparent. The grafts of 2 cases under 1 year old were clear after drug therapy. And the vision of a 3-year-old patient with Peter anomaly improved obviously, but immune rejection occurred 2 years after surgery. The second PKP was performed, but rejection occurred again. Secondaryglaucoma was found in the other eye early after operation; anti-glaucoma surgery failed, and the graft became cloudy. Graft infection associated with loosened sutures was observed in one case （2 eyes） of sclerocornea, and the second PKP failed. Conclusions For the patients with congenital corneal opacities, there is often a noticeable visual improvement after PKP. Good postoperative care, appropriate amblyopia treatment and timely examination are the keys to success.