Diagnosis and Management of Congenital Diaphragmatic Hernia (CDH): A Clinical Practice Guideline

Recommendation Strength 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
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
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

By using this site you acknowledge that you have read, understand, and agree to be bound by our terms of use and privacy policy. All content and tools are for educational use only, are not meant to be a substitute for professional advice and should not be used for medical diagnosis and/or medical treatment.