Congenital diaphragmatic hernia (CDH) is a birth defect resulting from the abnormal development of the diaphragm that allows the abdominal viscera to move up into the thoracic cavity. This condition is associated with varying degrees of pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction, factors that largely determine neonatal morbidity and mortality. Its incidence is estimated at 1 in 2000–4000 births.

Currently, most cases are diagnosed prenatally, which allows for appropriate prognostic stratification. This is chiefly based on the observed/expected lung area to head ratio (O/E LHR) and the position of the liver (intrathoracic or intraabdominal).1 Based on these criteria, cases can be classified as mild, moderate, or severe; this classification has prognostic and therapeutic implications. For example, fetoscopic endoluminal tracheal occlusion may be considered in fetuses with moderate or severe CDH.2

Traditionally, postnatal management guidelines have recommended, based on expert opinion, elective intubation in the delivery room to prevent gastric and bowel distension associated with noninvasive ventilation, which could further compromise lung function.3,4 However, the European guidelines published in 2015 already recognized a subset of patients with favorable prognostic characteristics who could benefit from initial management based on spontaneous breathing, thereby avoiding elective intubation.4 In recent years, several single-center studies have reported favorable outcomes with this strategy in carefully selected cases.5,6

The criteria proposed for identifying newborns who are candidates for conservative management include: mild CDH (O/E LHR ≥ 45%), left-sided location, intraabdominal liver, gestational age (GA) ≥ 37 weeks, and absence of associated major anomalies. In these patients, initial respiratory support may prioritize maintaining spontaneous breathing under close clinical monitoring.

The Hospital Sant Joan de Déu is a leading tertiary care center for the prenatal diagnosis and management of pregnant women carrying fetuses with CDH, including the option of treatment with fetoscopic endoluminal tracheal occlusion. Over the past 10 years, the neonatal unit has managed a total of 90 cases of CDH. In 2022, our hospital implemented a specific protocol for conservative respiratory management in the delivery room for patients who met the aforementioned prenatal criteria.

Since the introduction of this protocol in 2022, 7 of the 31 newborns with a diagnosis of CDH managed in our hospital met the criteria for this approach and were initially managed with a spontaneous breathing strategy in the delivery room. Table 1 summarizes the clinical characteristics and outcomes of these patients.

As can be seen in the table, of the seven patients who met the criteria, only one had to be intubated in the delivery room after failure of IPPV. Of the other six, two underwent elective intubation for surgery, and the rest required intubation at different times prior to surgery due to progressive respiratory deterioration, with increased oxygen requirements and work of breathing. None of the patients developed pneumothorax or other complications attributable to the delay of intubation. Likewise, there was no evidence of severe hemodynamic deterioration attributable to the initial conservative management, although one of the patients required vasoactive support and subsequently ECMO due to pulmonary hemorrhage, likely secondary to an infection by Klebsiella pneumoniae in the postoperative period.

In conclusion, prenatal risk stratification allows the individualization of initial respiratory management of newborns with CDH, reserving immediate intubation for moderate or severe cases. In carefully selected patients, an initial strategy based on spontaneous breathing may allow avoiding or delaying invasive mechanical ventilation, as we did not observe any acute complications attributable to delayed intubation in this case series. Most patients had mild or moderate pulmonary hypertension and required minimal pulmonary vasodilator or vasoactive support before surgery. Although all patients were assessed with point-of-care echocardiography after being admitted to the neonatal unit, our current protocol does not consider the use of these tools to predict failure of conservative management in the delivery room. In this regard, the incorporation of functional parameters from heart or lung ultrasound examinations could, in the future, help identify neonates at increased risk of requiring intubation at an early stage, optimize monitoring and select candidates for a spontaneous breathing-based strategy. Still, prospective multicenter studies are needed to better define the safety and long-term benefits of conservative management based on spontaneous breathing.