Congenital Diaphragmatic Hernia Guidelines

Updated: Nov 19, 2021
  • Author: Daniel S Schwartz, MD, MBA, FACS; Chief Editor: Jeffrey C Milliken, MD  more...
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Canadian CDH Collaborative Guidelines for Congenital Diaphragmatic Hernia

In 2018, the Canadian Congenital Diaphragmatic Hernia Collaborative issued guidelines for diagnosis and management of congenital diaphragmatic hernia (CDH). [28]

Recommendations for antenatal diagnosis included the following:

  • Ultrasonographic measurement of observed-to-expected (O/E) lung-head ratio (LHR) should be done between 22 and 32 weeks of gestational age to predict the severity of pulmonary hypoplasia in isolated CDH.
  • In left-side CDH, an O/E LHR < 25% predicts poor outcome. In right-side CDH, an O/E LHR < 45% may predict poor outcome. 
  • Fetal magnetic resonance imaging (MRI) should be used (where available) for the assessment of lung volume and liver herniation in moderate and severe CDH.

Recommendations for ventilation included the following:

  • Newborns with CDH and immediate respiratory distress should be preferentially intubated at birth. Bag-valve-mask ventilation should be avoided.
  • 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. 
  • A T-piece should be used with the ventilator to avoid a peak inspiratory pressure (PIP) higher than 25 cm H 2O.
  • An arterial carbon dioxide tension (PaCO 2) between 45 and 60 mm Hg and a pH between 7.25 and 7.40 should be targeted in all newborns with CDH.
  • Supplemental oxygen should be titrated to achieve a preductal saturation of at least 85% but no greater than 95%.
  • Gentle intermittent mandatory ventilation (IMV) should be the initial ventilation mode for newborns with CDH who require respiratory support. High-frequency oscillatory ventilation (HFOV) or high-frequency jet ventilation (HFJV) should be used when the PIP required to control hypercapnia using IMV exceeds 25 cm H 2O.

Recommendations for hemodynamic support included the following:

  • Treatment of poor perfusion (capillary refill >3 s, lactate >3 mmol/L, urine output < 1 mL/kg/hr) and blood pressure below norms for age should include (1) judicious administration of crystalloid, generally not exceeding 20 mL/kg; (2) inotropic agents such as dopamine or epinephrine; and (3) hydrocortisone.
  • If poor perfusion continues, assessment of cardiac function (via echocardiography or central venous saturation) should be performed.

Recommendations for echocardiography included the following:

  • Two standardized echocardiograms, one within 48 hours of birth and one at 2-3 weeks of life, are needed to assess pulmonary vascular resistance, as well as left ventricular (LV) and right ventricular (RV) function. Additional studies may be conducted as clinically indicated.

Recommendations for management of pulmonary hypertension included the following:

  • Inhaled nitric oxide (iNO) is indicated for confirmed suprasystemic pulmonary arterial hypertension without LV dysfunction, provided that lung recruitment is adequate. In the absence of clinical or echocardiographic response, iNO should be stopped.
  • Sildenafil should be considered in patients with refractory pulmonary hypertension (ie, hypertension unresponsive to iNO) or as an adjunct in weaning iNO.
  • Milrinone should be used to treat cardiac dysfunction, particularly if it is associated with pulmonary hypertension.
  • Prostaglandin E1 can be used to maintain ductus arteriosus patency and reduce RV afterload in patients with pulmonary hypertension with RV failure or in the presence of a closing ductus. 

Recommendations for extracorporeal life support included the following:

  • The possibility of extracorporeal life support should be discussed during antenatal counselling for CDH, and the discussion should disclose that available evidence does not suggest a survival benefit to its use. 

Recommendations for surgery included the following:

  • The following physiologic criteria should be met before surgery: (1) urine output >1 mL/kg/hr, (2) fraction of inspired oxygen (FiO 2) < 0.5, (3) preductal oxygen saturation between 85% and 95%, (4) normal mean arterial pressure for gestational age, (5) lactate < 3 mmol/L, and (6) estimated pulmonary artery pressures less than systemic pressure.
  • Failure to meet these criteria within 2 weeks should prompt consideration of either attempted repair or a palliative approach. 
  • For diaphragmatic defects that are not amenable to primary repair, oversized tension-free polytetrafluoroethylene/GORE-TEX patches should be used. 
  • A minimally invasive surgical approach or technique should not be used in the repair of neonatal CDH, because of the high rates of recurrence. 
  • In 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 to either surgery or palliation, as appropriate. 

Recommendations for long-term follow-up included the following:

  • Standardized multidisciplinary follow-up is recommended for children with CDH to provide surveillance and screening, optimal and timely diagnosis, and clinical care adjusted to the level of risk.
  • It is recommended to identify 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.