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Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left 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: 3.97). 11/2007; 197(4):422.e1-4. DOI: 10.1016/j.ajog.2007.07.001
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ABSTRACT 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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    • "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.
    Journal of Pediatric Surgery 06/2012; 47(6):1058-62. DOI:10.1016/j.jpedsurg.2012.03.003 · 1.31 Impact Factor
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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.
    Journal of Pediatric Surgery 05/2011; 46(5):808-13. DOI:10.1016/j.jpedsurg.2011.02.009 · 1.31 Impact Factor
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    • "Earlier it seemed that outcome could be better predicted when using a combination of markers. Herniation of the liver is in itself related to survival and therefore remains a logical candidate (Hedrick et al., 2007). Liver has comparable echogenecity to the lung, and Doppler interrogation of the umbilical vein and hepatic vessels help in determining its position. "
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    ABSTRACT: Congenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies with poor prognosis. In isolated cases, survival is dependent on the degree of lung hypoplasia and liver position. Cases should be referred in utero to tertiary care centers familiar with this condition both for prediction of outcome as well as timed delivery. The best validated prognostic indicator is the lung area to head circumference ratio. Ultrasound is used to measure the lung area of the index case, which is then expressed as a proportion of what is expected normally (observed/expected LHR). When O/E LHR is < 25% survival chances are < 15%. Prenatal intervention, aiming to stimulate lung growth, can be achieved by temporary fetal endoscopic tracheal occlusion (FETO). A balloon is percutaneously inserted into the trachea at 26-28 weeks, and reversal of occlusion is planned at 34 weeks. Growing experience has demonstrated the feasibility and safety of the technique with a survival rate of about 50%. The lung response to, and outcome after FETO, is dependent on pre-existing lung size as well gestational age at birth. Early data show that FETO does not increase morbidity in survivors, when compared to historical controls. Several trials are currently under design.
    Prenatal Diagnosis 07/2008; 28(7):581-91. DOI:10.1002/pd.2033 · 3.27 Impact Factor
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