Table 1:

Abridged recommendations for diagnosing and managing congenital diaphragmatic hernia

RecommendationStrength of recommendation*Level of evidence
Prenatal diagnosis
Ultrasound measurement of O/E LHR should be used between 22 and 32 w of gestational age to predict the severity of pulmonary hypoplasia in isolated CDH.●●●●B-NR
In left-sided CDH, an O/E LHR < 25% predicts poor outcome. In right-sided CDH, an O/E LHR < 45% may predict poor outcome.●●●○B-NR
Fetal magnetic resonance imaging should be used (where available) for the assessment of lung volume and liver herniation in moderate and severe CDH.●●●●B-NR
Ventilation
Newborns with CDH and immediate respiratory distress should be preferentially intubated at birth. Bag-valve-mask ventilation should be avoided.●●●●C-EO
Sedation should be provided to all mechanically ventilated newborns with CDH. Deep sedation and neuromuscular blockade should be provided selectively to those with greater ventilation or oxygen requirements.●●●●B-NR
A T-piece should be used with the ventilator to avoid a peak inspiratory pressure > 25 cm H2O.●●●●B-NR
An arterial pCO2 between 45 and 60 mm Hg and a pH between 7.25 and 7.40 should be targeted in all newborns with CDH.●●●●B-NR
Supplemental oxygen should be titrated to achieve a preductal saturation of at least 85%, but not > 95%.●●●●B-EO
Gentle, intermittent mandatory ventilation should be the initial ventilation mode for newborns with CDH who require respiratory support. High-frequency oscillatory ventilation or high-frequency jet ventilation should be used when the peak inspiratory pressure required to control hypercapnia using intermittent mandatory ventilation exceeds 25 cm H2O.●●●●B-NR
Hemodynamic support
Treatment of poor perfusion (capillary refill > 3 s, lactate > 3 mmol/L, urine output < 1 mL/kg/h) and blood pressure below norms for age should include:
  • judicious administration of crystalloid, generally not exceeding 20 mL/kg;

  • inotropic agents such as dopamine or epinephrine; and

  • hydrocortisone.

If poor perfusion continues, assessment of cardiac function (i.e., echocardiogram, central venous saturation) should be performed
●●●●B-NR
Echocardiography
Two standardized echocardiograms, one within 48 h of birth and one at 2–3 w of life, are needed to assess pulmonary vascular resistance, as well as left ventricular and right ventricular function. Additional studies may be conducted as clinically indicated.●●●●C-LD
Management of pulmonary hypertension
iNO is indicated for confirmed suprasystemic pulmonary arterial hypertension without left ventricular dysfunction, provided lung recruitment is adequate. In the absence of clinical or echocardiographic response, iNO should be stopped.●●●○C-EO
Sildenafil should be considered in patients with refractory pulmonary hypertension (i.e., unresponsive to iNO) or as an adjunct when weaning iNO.●●●○B-R
Milrinone should be used to treat cardiac dysfunction, particularly if it is associated with pulmonary hypertension.●●●●B-NR
Prostaglandin E1 can be used to maintain ductus arteriosus patency and reduce right ventricular afterload in patients with pulmonary hypertension with right ventricular failure, or in the presence of a closing ductus.●●●○C-LD
Extracorporeal life support
The possibility of extracorporeal life support should be discussed during prenatal counselling for CDH, and should disclose that available evidence does not suggest a survival benefit to its use.●●●○B-R
Surgery
The following physiologic criteria should be met before surgery:
  • urine output > 1 mL/kg/h

  • FiO2 < 0.5

  • preductal oxygen saturation between 85% and 95%

  • normal mean arterial pressure for gestational age

  • lactate < 3 mmol/L

  • estimated pulmonary artery pressures less than systemic pressure.

Failure to meet these criteria within 2 w should prompt consideration of either attempted repair or a palliative approach.
●●○○C-EO
Patch repair: For diaphragmatic defects that are not amenable to primary repair, oversized, tension-free polytetrafluoroethylene/GORE-TEX patches should be used.●●●●C-LD
Open repair v. minimally invasive surgery: A minimally invasive surgical approach or technique should not be used in the repair of neonatal CDH because of the high rates of recurrence.●●●●B-NR
For patients on extracorporeal life support: Surgery should be avoided until after decannulation. If the patient cannot be weaned off extracorporeal life support, consideration should be given for either surgery or palliation, as appropriate.●●●●C-LD
Long-term follow-up
  • We recommend standardized multidisciplinary follow-up for children with CDH to provide surveillance and screening, optimal and timely diagnosis and clinical care adjusted to the level of risk.

●●●●B-NR
  • We recommend identifying the subset of CDH survivors at high risk for long-term morbidity as comprising those infants and children who require extracorporeal life support, who have been repaired with a patch or who required respiratory support at 30 days of life.

●●●●B-NR
  • Note: CDH = congenital diaphragmatic hernia, iNO = inhaled nitric oxide, O/E LHR = observed-to-expected lung–head ratio.

  • * Strength of recommendation: the number of circles represents the level of expert consensus during creation of recommendations (see Box 2).

  • Level of evidence: evidence supporting the recommendation (see Box 1).