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

Recommendation Strength of recommendation* Level of evidence†
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.
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-EO
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.
  • 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.
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).

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