Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated congenital diaphragmatic hernia

Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
American journal of obstetrics and gynecology (Impact Factor: 4.7). 11/2007; 197(4):422.e1-4. DOI: 10.1016/j.ajog.2007.07.001
Source: PubMed


The purpose of this study was to determine the ability of liver position and lung-to-head ratio to predict outcome in isolated left congenital diaphragmatic hernia.
We reviewed prenatal studies and postnatal outcomes of congenital diaphragmatic hernia between January 1996 and January 2006.
Eighty-nine patients received prenatal and postnatal care at 1 institution. In fetuses with liver up, extracorporeal membrane oxygenation was required in 39 of 49 fetuses (80%), compared with 10 of 40 fetuses (25%) for those with liver down (P < .0001). Overall survival rate was 45%, compared with 93% for those with liver down (P < .00005). Low lung-to-head ratio (<1.0) predicted increased incidence of extracorporeal membrane oxygenation (75%; P = .036) and lower survival (35%; P = .0003). However, when measured at <24 weeks of gestation, lung-to-head ratio was not predictive of outcome (extracorporeal membrane oxygenation, P = .108; survival, P = .150); liver position remained highly predictive (extracorporeal membrane oxygenation, P = .006; survival, P = .001).
Liver position is the best prenatal predictor of outcome in isolated left congenital diaphragmatic hernia. Lung-to-head ratio alone should not be used to counsel families regarding mid gestational management choices.

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    • "Previously, some researchers suggested the merit of pulmonary artery–based measurements, whereas the predictive value of observed-to-expected lung-to-head ratio was extensively discussed during the past decade [9,10]. In contrast, liver position has gained wide acceptance as an excellent prognostic factor and is considered by many to be the best prenatal predictor of outcome [11,12]. "
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    ABSTRACT: Congenital diaphragmatic Bochdaleck hernia is an anatomical defect of the diaphragm, which allows protrusion of abdominal viscera into the chest, causing serious pulmonary and cardiac complications in the neonate. In this study we aimed to present a case of congenital Bochdaleck hernia. We investigated a 40 weeks old child, with a pregnancy carried out in a public hospital in Passo Fundo, Rio Grande do Sul, Brazil. We suggest that if diagnosis occurs in the prenatal period, the prognosis of this disease improves. As a consequence, it allows the parity of the fetus to occur in a higher complexity center, optimizing the chances of survival.
    Full-text · Article · Oct 2012 · International Archives of Medicine
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    • "Liver herniation has been consistently reported as an indicator of poor prognosis in fetuses with CDH [9] [10] [11] [12]. These studies, however, evaluate liver herniation as a dichotomous variable (absent or present) rather than on a continuous spectrum. "
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    ABSTRACT: The aim of the study was to evaluate the relationship between the degree of liver herniation and mortality or need for extracorporeal membrane oxygenation (ECMO) in isolated left-sided congenital diaphragmatic hernia (CDH). This is a retrospective review of all fetuses with isolated left-sided Bochdalek-type CDH evaluated at our center with ultrafast fetal magnetic resonance imaging between January 2004 and December 2010. Percentage of liver herniation was defined as the ratio of herniated liver volume to total fetal liver volume. The liver/thoracic volume ratio was defined as the ratio of herniated liver volume to total thoracic volume. Data were analyzed using receiver operating characteristic curves and Fisher's Exact and Mann-Whitney U tests. Fifty-three fetuses with isolated left-sided CDH were evaluated. Fetuses with "liver-up" (n = 32) and "liver-down" (n = 21) anatomy had similar rates of mortality (25% vs 14%, P = .49) and ECMO use (41% vs 29%, P = .40). The accuracy of liver-up (a dichotomous variable) to predict mortality or need for ECMO was 49% and 53%, respectively. Percentage of liver herniation greater than 21% was associated with mortality (P < .001) or need of ECMO (P < .001), with an accuracy of 87% and 79%, respectively. Liver/thoracic volume ratio of greater than 14% was also associated with mortality or ECMO use (P < .001 and P = .01, respectively), with an accuracy of 85% and 72%, respectively. Increased amounts of liver herniation in fetuses with isolated left-sided CDH are associated with higher rates of mortality or the need for ECMO support. Quantification of liver herniation (a continuous variable) is superior to overall liver herniation as a dichotomous variable (liver-up vs liver-down) in the prediction of perinatal morbidity and mortality.
    Full-text · Article · Jun 2012 · Journal of Pediatric Surgery
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    • "Sophisticated imaging techniques, such as 3-dimen- sional ultrasound and ultrafast fetal magnetic resonance imaging (MRI), have increased the amount of information that can be gathered about the anatomic malformation of the fetus and the severity of disease. Liver position, observed-toexpected lung-to-head ratio (LHR), and the presence of associated anomalies have been described in the literature to predict morbidity and mortality [13] [14] [15] [16] [17] [18] [19] [20] [21]. Prognostic data are useful in prenatal nondirective counseling to provide families with an understanding of the disease and potential outcomes . "
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    ABSTRACT: Although intuitive, the benefit of prenatal evaluation and multidisciplinary perinatal management for fetuses with congenital diaphragmatic hernia (CDH) is unproven. We compared the outcome of prenatally diagnosed patients with CDH whose perinatal management was by a predefined protocol with those who were diagnosed postnatally and managed by the same team. We hypothesized that patients with CDH undergoing prenatal evaluation with perinatal planning would demonstrate improved outcome. Retrospective chart review of all patients with Bochdalek-type CDH at a single institution between 2004 and 2009 was performed. Patients were stratified by history of perinatal management, and data were analyzed by Fisher's Exact test and Student's t test. Of 116 patients, 71 fetuses presented in the prenatal period and delivered at our facility (PRE), whereas 45 infants were either outborn or postnatally diagnosed (POST). There were more high-risk patients in the PRE group compared with the POST group as indicated by higher rates of liver herniation (63% vs 36%, P = .03), need for patch repair (57% vs 27%, P = .004), and extracorporeal membrane oxygenation use (35% vs 18%, P = .05). Despite differences in risk, there was no difference in 6-month survival between groups (73% vs 73%). Patients with CDH diagnosed prenatally are a higher risk group. Prenatal evaluation and multidisciplinary perinatal management allows for improved outcome in these patients.
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