[先天性膈疝。产前诊断与新生儿结局]

Lamberti A, Liguori M, Teodoro A, Tartaglione A, Caccioppoli U, Paladini D

Dipartimento di Ginecologia, Ostetricia, Università degli Studi Federico II, Napoli.

Minerva Ginecol. 1999 Jul-Aug;51(7-8):283-9.

BACKGROUND

The objective of this study is to determine the management and the outcome of 17 cases of congenital diaphragmatic hernia observed, and to compare the results with the literature data.

METHODS

The study was made between June 1994 and June 1998 in the Department of Obstetrics and Gynecology of the University of Naples Federico II and it collected 17 pregnant women with diagnosed or suspected congenital diaphragmatic hernia, referred to our diagnostic unit from other institutes: between 18 and 37 weeks' gestation, a detailed ultrasound examination and an echocardiography were performed to confirm the diagnosis, to establish the site and contents of the hernia exactly and to detect associated structural malformations. Fetal kariotyping was made in 7 cases. When the women decided to continue pregnancy, or the legal limit for termination of pregnancy was exceeded, the pregnancy was monitored with ultrasound examination, delivery took place in our department and the baby was transferred to an intensive care unit.

RESULTS

Ultrasound examination led to the diagnosis of 14 postero-lateral left-sided diaphragmatic hernias (82.3%), 1 antero-lateral left-sided (5.9%), 1 bilateral postero-lateral sided (5.9%) and 1 diaphragmatic eventration (5.9%). Associated structural malformation were diagnosed in 4 fetuses (27%). In 1 case only (14.3% of examinated kariotyping) an abnormal result was found. Five pregnancies (29.4%) were terminated, 2 (11.7%) are still going on and 10 fetuses (58.9%) were born alive: 5 fetuses (50%) died in the first days of life before surgical intervention, 2 (40% of operated children and 20% of born alive) died after the operation and 3 (60% of operated children and 30% of born alive) are actually alive and in good health. The total postnatal mortality was 70% and 40% after operation.

CONCLUSIONS

From the analysis of these data and from the international literature the conclusion is drawn that congenital diaphragmatic hernia is associated with a high postnatal mortality, although potentially it can be corrected with surgery: a better postnatal management and a better knowledge of evaluable in uterus prognostic factors are necessary to improve postnatal outcome.

背景

本研究的目的是确定所观察的17例先天性膈疝的处理方法及结果，并将结果与文献数据进行比较。

方法

该研究于1994年6月至1998年6月在那不勒斯费德里科二世大学妇产科进行，收集了17例已诊断或疑似先天性膈疝的孕妇，她们是从其他机构转诊至我们诊断科室的：在妊娠18至37周期间，进行了详细的超声检查和超声心动图检查，以确诊、准确确定疝的部位和内容物，并检测相关的结构畸形。7例进行了胎儿核型分析。当孕妇决定继续妊娠或超过妊娠终止法定时限时，通过超声检查监测妊娠情况，在我们科室分娩，婴儿被转入重症监护病房。

结果

超声检查诊断出14例左侧后外侧膈疝（82.3%）、1例左侧前外侧膈疝（5.9%）、1例双侧后外侧膈疝（5.9%）和1例膈膨升（5.9%）。4例胎儿（27%）被诊断出伴有结构畸形。仅1例（所检查核型分析的14.3%）结果异常。5例妊娠（29.4%）终止，2例（11.7%）仍在继续，10例胎儿（58.9%）存活出生：5例胎儿（50%）在生命的头几天在手术干预前死亡，2例（手术患儿的40%和存活出生者的20%）术后死亡，3例（手术患儿的60%和存活出生者的30%）目前存活且健康。产后总死亡率为70%，术后为40%。