Clark R H, Hardin W D, Hirschl R B, Jaksic T, Lally K P, Langham M R, Wilson J M
J Pediatr Surg. 1998 Jul;33(7):1004-9. doi: 10.1016/s0022-3468(98)90522-x.
Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult.
The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients.
Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours).
The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.
在过去十年中，先天性膈疝（CDH）的修复手术已从急诊手术转变为择期手术。自首次成功修复以来，管理的许多其他方面也有所发展。然而，大多数报告来自单一机构。缺乏大型多中心数据库阻碍了先天性膈疝（CDH）管理方面的进展，使得确定当前标准变得困难。
1995年成立了CDH研究小组，以收集来自北美、欧洲和澳大利亚多个机构的数据。参与中心填写了1995年和1996年所有存活的CDH婴儿的登记表。收集了所有患者的人口统计学信息、手术管理数据和结局。
62个中心参与，纳入461例患者。在记录存活情况的442例患者中，总体存活率为280例（63%）。缺损为左侧的占78%，右侧的占21%，双侧的占1%。91%的患者采用肋下入路，76%的患者使用胸腔引流。略超过一半（51%）的患者使用了某种补片，在使用补片的患者中，聚四氟乙烯是最常用的材料（81%）。平均手术时间为102分钟，平均失血量为14毫升（范围为0至500毫升）。绝大多数患者在上午6点至下午6点之间接受修复手术（329例中的289例，88%）。19%的患者在体外膜肺氧合（ECMO）支持下进行手术修复，平均时间为ECMO治疗过程开始后的170小时（范围为10至593小时）。未接受ECMO治疗的患者手术时的平均年龄为73小时（范围为1至445小时）。
本报告的多中心性质使其成为当前管理情况的一个快照。数据表明，对缺损进行假体修补已变得常见，非工作时间修复很少见，延迟手术修复已成为许多中心的首选方法。此外，63%的平均存活率表明，尽管经过数十年的个人努力，CDH问题远未解决。这凸显了未来建立集中数据库和开展合作多中心研究的必要性。
