Weber T R, Kountzman B, Dillon P A, Silen M L
Department of Surgery, Saint Louis University Health Sciences Center, and Cardinal Glennon Children's Hospital, MO 63104, USA.
Arch Surg. 1998 May;133(5):498-502; discussion 502-3. doi: 10.1001/archsurg.133.5.498.
To compare the survival rates for 3 therapeutic eras, each using different treatment strategies for the management of newborns with congenital diaphragmatic hernia (CDH).
Retrospective review of all infants with CDH from 1970 through 1997.
Tertiary care children's hospital.
A total of 203 newborns with CDH.
Extracorporeal membrane oxygenation (ECMO) was performed with arterial and venous cannulation connected to a membrane oxygenatorroller pump perfusion apparatus, using systemic heparinization. Delayed operative therapy involved operative repair 2 to 5 days after birth using preoperative ventilation support only. Since 1970, 203 newborns with CDH were managed in 3 therapeutic eras: era 1 (1970-1983, 102 patients) was immediate CDH repair with postoperative ventilator and pharmacologic support; era 2 (1984-1988, 45 patients) was immediate repair with postoperative ventilator support (18 patients), immediate ECMO with CDH repair on ECMO (4 patients), or immediate repair with postoperative ECMO (23 patients); and era 3 (1989-1997, 56 patients) was immediate ECMO with repair on ECMO (23 patients), immediate repair with postoperative ECMO (9 patients), or delayed (2-5 days) CDH repair (24 patients).
Survival, defined as discharge from the hospital, and morbidity.
Survival was 42% (43/102 patients) in era 1, 58% (26/45 patients) in era 2, and 79% (44/56 patients) in era 3 (P<.02 vs eras 1 and 2). In era 3, the survival for immediate ECMO with repair on ECMO was 57% (13/23 patients), 89% (8/9 patients) for immediate repair with postoperative ECMO, and 96% (23/24 patients) for delayed repair. Eight late deaths were caused by pulmonary hypertension (1 death), sudden infant death syndrome (1 death), and other causes (6 deaths). Morbidity in survivors included mild neurologic deficit (5 patients) and pulmonary disease (3 patients).
These data demonstrate a significant improvement in survival in CDH with preoperative ECMO and with delayed repair with and without ECMO support and suggest that immediate repair of CDH without the availability of ECMO support should be abandoned.
比较三个治疗时代对先天性膈疝（CDH）新生儿采用不同治疗策略的生存率。
对1970年至1997年所有CDH婴儿进行回顾性研究。
三级护理儿童医院。
共203例CDH新生儿。
体外膜肺氧合（ECMO）通过动脉和静脉插管连接到膜式氧合器-滚压泵灌注装置进行，使用全身肝素化。延迟手术治疗包括出生后2至5天进行手术修复，仅术前给予通气支持。自1970年以来，203例CDH新生儿在三个治疗时代接受治疗：时代1（1970 - 1983年，102例患者）为立即进行CDH修复并术后给予呼吸机和药物支持；时代2（1984 - 1988年，45例患者）为立即修复并术后给予呼吸机支持（18例患者）、立即进行ECMO并在ECMO上进行CDH修复（4例患者）或立即修复并术后进行ECMO（23例患者）；时代3（1989 - 1997年，56例患者）为立即进行ECMO并在ECMO上进行修复（23例患者）、立即修复并术后进行ECMO（9例患者）或延迟（2 - 5天）CDH修复（24例患者）。
生存（定义为出院）和发病率。
时代1的生存率为42%（43/102例患者），时代2为58%（26/45例患者），时代3为79%（44/56例患者）（与时代1和2相比，P<0.02）。在时代3中，立即进行ECMO并在ECMO上进行修复的生存率为57%（13/23例患者），立即修复并术后进行ECMO的生存率为89%（8/9例患者），延迟修复的生存率为96%（23/24例患者）。8例晚期死亡原因包括肺动脉高压（1例死亡）、婴儿猝死综合征（1例死亡）和其他原因（6例死亡）。幸存者的发病率包括轻度神经功能缺损（5例患者）和肺部疾病（3例患者）。
这些数据表明，术前使用ECMO以及在有或无ECMO支持的情况下延迟修复，CDH的生存率有显著提高，并表明在没有ECMO支持的情况下立即修复CDH应被摒弃。
