Several recent articles which have some importance for diaphragmatic hernia management
Madenci AL, Sjogren AR, Treadwell MC, Ladino-Torres MF, Drongowski RA, Kreutzman J, et al. Another dimension to survival: predicting outcomes with fetal MRI versus prenatal ultrasound in patients with congenital diaphragmatic hernia. J Pediatr Surg. 2013;48(6):1190-7. Counselling parents about how likely it is that their infant may survive is very tricky. In general. And especially for diaphragmatic hernia patients where the mortality and morbidity are high, but the long term outcome is generally excellent. This study looks at several different indices of lung hypoplasia to determine which, if any, are better than others. It points out, as we already knew, but it bears emphasizing, that the lung head ratio differs during pregnancy, so rather than a single number, the ratio between observed lung-head ratio and the expected ratio is what is predictive of important outcomes. Also fetal MRI with calculation of lung volume (which also obviously should increase during gestation, so the percentage of normal is used as the measure) is a better discriminant of survival, need for ECMO, and ventilator dependency.
Walleyo A, Debus A, Kehl S, Weiss C, Schonberg SO, Schaible T, et al. Periodic MRI lung volume assessment in fetuses with congenital diaphragmatic hernia: prediction of survival, need for ECMO, and development of chronic lung disease. AJR American journal of roentgenology. 2013;201(2):419-26. In this study there were 226 mothers whoer fetuses had repeated fetal MRI. The percentage of expected lung volume was strongly assocaited with survival and need for ECMO. They didn’t compare to ultrasound, though.
Hagelstein C, Weidner M, Kilian AK, Debus A, Walleyo A, Schoenberg S, et al. Repetitive MR measurements of lung volume in fetuses with congenital diaphragmatic hernia: individual development of pulmonary hypoplasia during pregnancy and calculation of weekly lung growth rates. Eur Radiol. 2014;24(2):312-9. This article from the same group, presumably with many of the same babies, calculated the rate of antenatal lung growth. They found :
Individual development of FLV in patients with CDH during pregnancy is extremely variable. Follow-up MR-FLV measurements are advisable before deciding upon pre- and postnatal therapeutic options.
Babies could change from poor to reasonable prognosis lung volumes during pregnancy, and vice versa. Something to keep in mind.
Kays DW, Islam S, Larson SD, Perkins J, Talbert JL. Long-term Maturation of Congenital Diaphragmatic Hernia Treatment Results: Toward Development of a Severity-Specific Treatment Algorithm. Annals of Surgery. 2013;258(4):638-45. An observational study that suggests that one size does not fit all. Suggests different treatment algorithms for different severities of lesion, which makes sense, they suggest that less severe lesions should have delayed surgery, more severe lesoins should have surgery prior to ECMO, rather than after. Interesting but far from conclusive.
Kays DW, Islam S, Richards DS, Larson SD, Perkins JM, Talbert JL. Extracorporeal Life Support in Patients with Congenital Diaphragmatic Hernia: How Long Should We Treat? Journal of the American College of Surgeons. 2014;218(4):808-17. When you start ECMO for a diaphragmatic hernia, you know you may be in for the long run. this review of a large experience (96 ECMO cases of a total of 240), by the sam eauthors as the study above showed :
Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors.
Babies on ECMO up to 4 weeks still had good long term pulmonary outcomes. So be patient, but after 4 weeks it looks pretty desperate.
Wright T, Filbrun A, Bryner B, Mychaliska G. Predictors of early lung function in patients with congenital diaphragmatic hernia. J Pediatr Surg. 2014;49(6):882-5. This study looked at lung function in follow up of a group of very high risk CDH patients, during the first 3 years of life. They are actually pretty good. Things that you would expect to be predictors of somewhat poorer function (such as need for ECMO) were indeed, but even with those factors, only moderate abnormalities were found.