There have been many publications recently for this group of our patients:
Hagadorn JI, et al. Trends in treatment and in-hospital mortality for neonates with congenital diaphragmatic hernia. J Perinatol. 2015;35(9):748-54. This multi-center cohort study shows, in term babies, that mortality has been improving (which is always a problematic outcome, we don’t know the influence of pregnancy termination for more sever lesions, for changes in referral patterns etc etc) and that this has been associated with less ECMO in the uncomplicated cases, with more sildenafil and milrinone use and more multiple vasodilator use. Despite my comments in parentheses above, there were more cases overall which were complicated, so the trends in survival might not be due to changes in case-mix, but despite those changes. Trends in preterm babies with diaphragmatic hernia were not significant, despite more sildenfail and more multiple vasodilator use there was no improvement in mortality.
Gien J, Kinsella JP. Differences in preductal and postductal arterial blood gas measurements in infants with severe congenital diaphragmatic hernia. Archives of Disease in Childhood – Fetal and Neonatal Edition. 2015. You would think someone would have done this before, but apparently not: pre-ductal blood gases are often significantly better oxygenated than post-ductal. So if you use pre-ductal blood gases, you can reduce oxygen therapy and respiratory support and so on. Should be routine whenever possible.
Puligandla PS, et al. Management of congenital diaphragmatic hernia: a systematic review from the APSA outcomes and evidence based practice committee. J Pediatr Surg. 2015. A very interesting systematic review of the literature. Can do no better than quote the results section of the abstract.
Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO (by which they mean VV compared to VA:KJB)) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material.
Bojanic K, et al. Survival of outborns with congenital diaphragmatic hernia: the role of protective ventilation, early presentation and transport distance: a retrospective cohort study. BMC pediatrics. 2015;15(1):155. Less aggressive ventilation associated with permissive hypercapnia led to improved survival, which was not affected by transportation distance. Of course, as a Canadian, I can laugh at the idea that a 280 km transfer is “distant”. More than 1000 km, now that qualifies as a long distance transport.
Huang JS, et al. Thoracoscopic repair of congenital diaphragmatic hernia: two centres’ experience with 60 patients. Pediatr Surg Int. 2015;31(2):191-5. We have been doing this at my center for a while now, with good success, but the procedure seemed to take longer than an open repair, more recent cases have been quicker. Putting in a patch is still a challenge.
Yamoto M, et al. Cardiac fetal ultrasonographic parameters for predicting outcomes of isolated left-sided congenital diaphragmatic hernia. J Pediatr Surg. 2015. The major finding from this study was that the worst outcomes were among cases where the tricuspid valve was relatively larger than the mitral valve. on the fetal echocardiogram in infants with a left-sided hernia. The larger that ratio (TV/MV) the higher the mortality. To put it the other way around the smaller the left heart, the worse off the patients were.
DeKoninck P, et al. Right-sided congenital diaphragmatic hernia in a decade of fetal surgery. BJOG: An International Journal of Obstetrics & Gynaecology. 2015;122(7):940-6. In this paper, exclusively interested in right-sided hernia in infants with a fetal diagnosis, the prediction of outcome from the lung-head ratio, compared to the same index for left-sided hernias, was worse. It seemed to be improved by fetal surgery using tracheal occlusion.
Spoel M, et al. Pulmonary ventilation and micro-structural findings in congenital diaphragmatic hernia. Pediatr Pulmonol. 2015 Finally when you get your patients through the neonatal period, and they survive to adulthood, their lungs are still not normal. The subjects included int his study had normal oxygen saturation and a reasonably normal FVC.