Liver Position Research Articles

Prenatal and postnatal markers of severity in congenital diaphragmatic hernia have similar prognostic ability.

The purpose of this study was to compare prenatal versus postnatal markers of congenital diaphragmatic hernia (CDH) severity at a single fetal-care center. A retrospective study was performed of patients having a complete prenatal evaluation and surgical repair (n = 55). Observed-to-expected lung-to-head ratio (o/eLHR), observed-to-expected total lung volume (o/eTLV), liver position (LP), a predictive dependent variable from logistic regression of o/eLHR and liver position (o/eLHR + LP), and diaphragmatic defect size per the CDH Study Group A-D classification were plotted into receiver-operating characteristics (ROC) curves. Survival and need for extracorporeal membrane oxygenation (ECMO) were primary outcomes. Survival was 69%, and ECMO utilization was 56%. Distribution was 80% left-sided defects. In the survival ROC curve, the area under the curve (AUC) for o/eLHR was 0.73, o/eTLV 0.74, LP 0.73, o/eLHR + LP 0.78, and defect size 0.84 (p = 0.23). The ROC curve for ECMO support showed o/eLHR had an AUC of 0.82, o/eTLV 0.89, LP 0.79, o/eLHR + LP 0.87, and defect size 0.90 (p = 0.19). The AUCs were similar when only left-sided CDH was analyzed. These data suggest that prenatal evaluation was equivalent to the postnatal diaphragmatic defect classification for predicting survival and need for ECMO in CDH patients.

Liver position is best prenatal predictor of need for ECMO and survival in left congenital diaphragmatic hernia

SURVIVAL IN LEFT CONGENITAL DIAPHRAGMATIC HERNIA HOLLY HEDRICK, ENRICO DANZER, HUAQING ZHAO, AZIZ MERCHANT, ALAN FLAKE, KENNETH LIECHTY, LORI HOWELL, MARK JOHNSON, DOUGLAS WILSON, MICHAEL BEBBINGTON, SCOTT ADZICK, Children’s Hospital of Philadelphia, Center for Fetal Diagnosis and Treatment, Philadelphia, Pennsylvania, Children’s Hospital of Philadelphia, Biostatistics, Philadelphia, Pennsylvania OBJECTIVE: To compare the ability of liver position (LP) and lung-to-head circumference ratio (LHR) to predict the need for extracorporeal membrane oxygenation (ECMO) and survival in fetuses with isolated left congenital diaphragmatic hernia (LCDH). STUDY DESIGN: We reviewed prenatal studies, hospital course, and postnatal outcomes of LCDH presenting to a single institution between January 1996 and January 2006. Logistic regression models were used to explain the need for ECMO using LP, LHR and gestational age (GA). Survival was determined by Kaplan-Meier analysis. Cox regression models were used to explore associations between LHR, LP, GA and survival. RESULTS: Eighty-nine cases with LCDH received prenatal and postnatal care at our institution. Overall 10-year survival was 66.3% at median follow up of survivors 57.8 months (range, 8-120 months). LP was intrathoracic in 49/89 (55%). In fetuses with liver-up, 39/49 (80%) required ECMO compared to 10/ 40 (25%) for those with liver-down (p!0.0001). Ten-year survival rate was 45% compared to 93% for those with liver-down (p!0.0001). Severe LHR (!1.0) predicted increased incidence of ECMO (75%, p=0.036) and lower 10year survival rate (34%, p=0.0003). Median GA at LHR measurement was 24.3 weeks. A lower LHR correlated with an increased need for ECMO and shorter time to survival independent of GA at time of measurement (ECMO, p=0.008; survival, p=0.007). When GA and LP were jointly fit into models, LHR (ECMO p=0.19, survival p=0.067) was no longer predictive of outcome at less than 24 weeks while LP (ECMO p!0.0005, survival p=0.001) remained highly predictive. CONCLUSION: LP is the best prenatal predictor of need for ECMO and survival in LCDH. LHR alone should not be used to counsel families regarding midgestational management choices.