Toward Standardized Management of Congenital Diaphragmatic Hernia: An Analysis of Practice Guidelines

Standardized care may improve outcomes in many diseases including congenital diaphragmatic hernia (CDH). Our study assesses the variability of CDH clinical practice guidelines (CPG) among North American centers. North American member institutions of the CDH Study Group and the Pediatric Surgical Research Collaborative were solicited to submit their CDH CPG. Elements from each CPG were collected and classified according to therapeutic purpose. Elements were assigned to umbrella topics of prenatal assessment, delivery plus initial resuscitation, ventilatory and cardiovascular management, therapeutic targets, analgesia, and criteria for transitions in care. Descriptive analyses were performed to characterize the scope and variability of CPGs. Sixty-eight centers provided 40 responses (59%). Of these, 29 (73%) had a CDH CPG, of which 27 were obtained for review. All CPGs had a primary focus of preoperative care. Conventional ventilation was the first-line strategy in all CPGs. Ninety-three percent reported a peak inspiratory pressure limit (mean: 25.2 ± 2 cm H2O). Target oxygenation and ventilatory variables had low coefficients of variation. Two-thirds of CPGs discussed echocardiography, with indications for inhaled nitric oxide, sildenafil, and prostaglandins detailed in 81%, 30%, and 22% of CPGs, respectively. Extracorporeal life support and operative indications were specified in 93% and 59%, respectively, although specific targets for each were highly variable. This synthesis of North American CDH CPGs identifies areas of both alignment and variability and provides objective data about individual institutional guidelines in CDH care. These data may inform the development of a consensus-based, multi-institutional approach to standardized CDH management in North America.