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无脏器疝出的胎儿膈疝

Fetal diaphragmatic hernia without visceral herniation.

作者信息

Stringer M D, Goldstein R B, Filly R A, Howell L J, Sola A, Adzick N S, Harrison M R

机构信息

Fetal Treatment Center, University of California, San Francisco, USA.

出版信息

J Pediatr Surg. 1995 Sep;30(9):1264-6. doi: 10.1016/0022-3468(95)90481-6.

Abstract

Many cases of congenital diaphragmatic hernia (CDH) are currently detected before birth. The authors hypothesized that there is a subgroup of patients with CDH who have no evidence of visceral herniation in utero and who would be expected to have less pulmonary hypoplasia and a good prognosis. Among 41 neonates with left-sided CDH treated between January 1990 and October 1993, 17 cases were diagnosed after birth. Ten of the 17 had undergone detailed fetal sonographic imaging at or after 20 weeks' gestation. After independent review of the prenatal scans of these 10 patients, one was found to have evidence of a diaphragmatic hernia and was excluded from further analysis. The other nine fetuses survived, and prosthetic repair of the diaphragmatic defect and extracorporeal membrane oxygenation (ECMO) were each required in only one patient. This contrasted with the outcome for 18 control patients with prenatally diagnosed CDH: 4 (22%) died, 13 (72%) required prosthetic repair, 9 (50%) were supported with ECMO and the duration of ventilatory support and hospital stay were significantly longer. There is a spectrum of severity among both pre and postnatally diagnosed cases of CDH. In the neonate with an isolated left-sided diaphragmatic hernia, a good prognosis is to be expected if the condition was not detectable by detailed prenatal sonography in the second half of pregnancy.

摘要

目前,许多先天性膈疝(CDH)病例在出生前就被检测出来了。作者推测,存在一部分CDH患者,他们在子宫内没有内脏疝形成的证据,预计肺发育不全程度较轻且预后良好。在1990年1月至1993年10月期间接受治疗的41例左侧CDH新生儿中,17例在出生后被诊断出来。这17例中有10例在妊娠20周及以后接受了详细的胎儿超声成像检查。在对这10例患者的产前扫描进行独立复查后,发现1例有膈疝证据,被排除在进一步分析之外。其他9例胎儿存活,仅1例患者需要进行膈疝缺损的假体修复和体外膜肺氧合(ECMO)。这与18例产前诊断为CDH的对照患者的结果形成对比:4例(22%)死亡,13例(72%)需要假体修复,9例(50%)接受了ECMO支持,通气支持时间和住院时间明显更长。产前和产后诊断的CDH病例都存在严重程度的差异。对于孤立性左侧膈疝的新生儿,如果在妊娠后半期通过详细的产前超声检查无法检测到该疾病,则预后良好。

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