AAP paper
Defining “liver-up”: does the volume of liver herniation predict outcome for fetuses with isolated left-sided congenital diaphragmatic hernia?

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Abstract

Purpose

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).

Methods

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.

Results

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.

Conclusion

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.

Section snippets

Study cohort

Permission to conduct this study was obtained from the institutional review board (H-26176) of Baylor College of Medicine, Houston, TX. A retrospective medical record review was performed for all fetuses with isolated left-sided Bochdalek-type CDH evaluated with an ultrafast fetal magnetic resonance imaging (MRI) at our fetal center and delivered at our hospital between January 2004 and December 2010. Data were collected and included basic demographics, MRI-based measurements of liver

Results

A total of 53 fetuses with isolated left-sided CDH were evaluated with ultrafast MRI during the study period. Mean maternal age was 27.7 ± 6.1 years, with a mean gestational age at diagnosis of 23.0 ± 5.6 weeks and mean gestational age at birth of 37.9 ± 1.6 weeks (Table 1). The 6-month mortality rate was 21% (n = 11). Extracorporeal membrane oxygenation was in used 36% (n = 19) of cases with a survival rate of 47%. Diaphragmatic repair was performed in 93% (n = 49) of newborns at an average of

Discussion

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. In utero, a wide variation in the amount of herniated liver can be observed by ultrafast fetal MRI. This imaging modality offers the opportunity to assess liver position and calculate liver volume and percentage of herniation. An earlier

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1

Both authors contributed equally to this manuscript as co-first authors. Winner of the Jens Rosenkrantz Award for the best oral presentation by a trainee at the American Academy of Pediatrics, Section on Surgery Meeting, October 2011.

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