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Section 11 — Gastrointestinal & Surgical Verify against local policy v1.0 · July 2026 Baylor Ed. 33 cross-checked Sept 2026

Chapter 11.7 — Congenital Diaphragmatic Hernia (CDH)

Do not bag-mask ventilate: immediate intubation and gastric decompression, gentle lung-protective ventilation for pulmonary hypoplasia and hypertension, physiological stabilization, and surgery only after stabilization — built on West Midlands Neonatal Guidelines 2025–28 and the CDH EURO Consortium consensus

Educational guideline — verify locally. Avoid bag-mask ventilation in a known/suspected CDH (it inflates the intrathoracic bowel and worsens lung compression) — intubate and place a large gastric tube. Ventilation, iNO, and surgical timing must follow local/CDH-center policy. Does not replace attending/surgical judgment.
BEDSIDE ACTION BOX — At Delivery

1. Overview

Overview

Congenital diaphragmatic hernia is a defect in the diaphragm (most commonly left-sided, posterolateral — Bochdalek) allowing abdominal viscera into the chest. The clinical problem is not the hernia itself but the associated pulmonary hypoplasia and pulmonary hypertension, which determine survival. Modern care is built on gentle, lung-protective ventilation, meticulous management of pulmonary hypertension and hemodynamics, and delayed surgery after physiological stabilization — with the recognition that avoiding bag-mask ventilation and lung injury at birth is critical.

Why This Topic Matters

Two early actions change outcome: intubating instead of bag-masking, and ventilating gently. Over-ventilation and aggressive resuscitation cause pneumothorax and worsen pulmonary hypertension. Surgery is not the emergency — stabilizing the physiology is.

2. Who This Guideline Applies To

Scope
  • Neonates with antenatally diagnosed or postnatally suspected congenital diaphragmatic hernia.
  • Delivery-room and NICU teams stabilizing CDH and coordinating surgical transfer.
  • Cross-references: delivery-room stabilization of unique populations (1.2), PPHN (6.3), hypotension/hemodynamics (3.6), ventilation (6.7), and congenital heart disease (3.3).

3. Key Definitions

TermDefinition
CDHDiaphragmatic defect allowing abdominal viscera into the chest (usually left posterolateral — Bochdalek).
Pulmonary hypoplasiaUnderdeveloped lungs (bilateral) from compression — the key determinant of survival.
Permissive hypercapniaAccepting higher CO₂ to avoid ventilator-induced lung injury.
Gentle ventilationLow-pressure, lung-protective strategy targeting adequate (not normal) gas exchange.

4. Pathophysiology

Hypoplasia + Hypertension
  • Herniated viscera compress the developing lungs → bilateral pulmonary hypoplasia (reduced alveoli and vessels).
  • Abnormal, muscularized pulmonary vasculature → pulmonary hypertension (PPHN) with right-to-left shunting and labile hypoxemia.
  • Associated anomalies (cardiac, chromosomal) worsen prognosis — screen.
  • Ventilator-induced lung injury and pneumothorax further compromise the small lungs — hence gentle ventilation.

5. Delivery-Room Management

Intubate, Decompress, Don't Bag-Mask
  • Avoid bag-mask ventilation; intubate early and ventilate through the tube.
  • Place a large gastric tube on continuous suction to decompress the gut and reduce lung compression.
  • Gentle ventilation from the outset; target pre-ductal saturations in the accepted range; avoid high pressures.
  • Establish reliable access; support blood pressure/perfusion; minimal handling; transfer to a CDH center.

6. Gentle, Lung-Protective Ventilation

Protect the Small Lungs
  • Use the lowest pressures that achieve adequate oxygenation and permissive hypercapnia with an acceptable pH (see 6.7); avoid over-distension/barotrauma.
  • Target pre-ductal saturations in the accepted range; tolerate lower post-ductal saturations.
  • Consider HFOV for infants failing conventional gentle ventilation (per CDH-center protocol).
  • Watch for pneumothorax (sudden deterioration — see 6.6).

7. Management Algorithm

1
At birth: intubate + gastric decompression
Do NOT bag-mask; intubate; large gastric tube on suction; gentle ventilation.
2
Gentle ventilation + monitoring
Low pressures, permissive hypercapnia, pre-ductal saturation targeting; pre/post-ductal SpO₂; watch for pneumothorax.
3
Manage PPHN & hemodynamics
Optimize oxygenation/recruitment; support systemic BP/perfusion; inhaled nitric oxide and other pulmonary vasodilators per protocol (see 6.3).
4
Screen & investigate
Echocardiography (PPHN, cardiac anomalies), genetics; confirm CDH side/contents; assess severity.
5
Operate after stabilization
Surgery is not emergent — repair the defect once physiologically stable; ECMO in selected refractory cases at ECMO centers.
6
⚠ Do-not-miss
Bag-mask ventilation worsening the picture; pneumothorax from over-ventilation; severe PPHN; and associated cardiac/chromosomal anomalies.

8. Pulmonary Hypertension & Hemodynamics

Central to Survival
  • Optimize lung recruitment and oxygenation; support systemic blood pressure to reduce right-to-left shunting (see 3.6).
  • Inhaled nitric oxide and other pulmonary vasodilators per CDH-center protocol; response can be variable in CDH.
  • Echocardiography to assess pulmonary pressures and cardiac function; individualize therapy.
  • Consider ECMO for refractory hypoxemic respiratory failure/PPHN at an ECMO center (per criteria).

8+. Baylor Ed. 33 CDH Protocol: Delivery Room to ECMO Criteria

Before birth and in the delivery room
  • Prenatal risk: fetal ultrasound and MRI grade severity by side of the defect, total fetal lung volume, liver herniation and observed-to-expected lung-to-head ratio. A major cardiac anomaly, genetic syndrome or other anomalies increase mortality. These drive counselling (strong recommendation, moderate quality).
  • At delivery (strong recommendation, low quality):
    • Intubate immediately — no bag-mask ventilation — and be careful not to deliver high PIP by bag, T-piece or ventilator.
    • Place a pre-ductal saturation probe at once and a 10 Fr Replogle tube on suction.
    • Establish peripheral IV access in the delivery room; leave umbilical lines and other non-urgent procedures for the NICU (line placement follows a unit algorithm).
    • After fetal tracheal occlusion (FETO), strongly consider a cuffed tube.
  • Oxygen: start ventilation in 100% FiO₂ and decide on pre-ductal saturations: >70% for the first 10 minutes, >80% for the first 2 hours, then >85%. Use the oxygenation index for management decisions. A trial of iNO may be considered by OI, but benefit in CDH is unproven.
Conventional start (Table 16-15, AC + VG)SettingHFOV (Table 16-14)Setting
PEEP5–6MAP13, or 2 above the conventional MAP; maximum 17
Tidal volume4–5 mL/kgFrequency10 Hz
Backup rate40Ti0.3 (33%)
Ti0.3 sΔPEnough for a perceptible chest "jiggle"
Pmax3–5 above measured PIP, maximum 28Switch to HFOV if target PCO₂ cannot be reached on conventional ventilation with PIP ≥28 (weak recommendation); then raise MAP (to 17) and ΔP to reach the goals
FiO₂To pre-ductal SpO₂ >80%
Goals and adjustments
  • Targets: pH ≥7.20 with lactate ≤3 mmol/L; PCO₂ 50–70; PO₂ 40–90; pre-ductal SpO₂ >80% in the first 2 hours, >85% thereafter.
  • Gentle ventilation — volume-targeted, low tidal volume, higher rate — because the hypoplastic lungs injure easily. If pre-ductal SpO₂ is below target or pH is <7.20 and not slowly improving, raise Vt by 0.5 mL/kg up to a working PIP of 28 (strong recommendation, low quality).
Sedation, fluids and nutrition
  • Sedate ventilated CDH infants to ease ventilation and limit PH, but at the lowest effective dose, preserving spontaneous breathing (strong, low quality). Avoid neuromuscular blockade except in infants on very high support.
  • Morphine rather than fentanyl if ECMO is possible, because fentanyl adheres to the circuit tubing. Give 0.05 mg/kg IV bolus on admission, then 0.01 mg/kg/h, titrating by no more than 0.02 mg/kg/h every 30 minutes. Add midazolam 0.05 mg/kg IV every 4–6 h as needed, or an infusion of 0.06 mg/kg/h in term infants if that is not enough — CDH sedation is the exception to the general advice against benzodiazepine sedation below 44 weeks PMA. Watch heart rate, blood pressure (additive hypotension) and respiratory drive.
  • Fluids: limit initial intake to 65 mL/kg/day (strong, very low quality), with maintenance at 40–50 mL/kg/day using concentrated dextrose for adequate glucose delivery. Start starter PN and lipid as glucose needs allow.
  • Lipid choice: before repair and on ECMO, avoid SMOFlipid (its n-3 content may increase bleeding) and give Intralipid 1 g/kg/day to prevent EFAD. After repair and/or ECMO, switch to SMOFlipid, keeping it above 2.5 g/kg/day; involve dietitians if a lower lipid dose is needed for more than 48 hours (see chapter 7.2).
  • Circulation: avoid repeated fluid boluses and aggressive volume; use vasopressors for hypotension, guided by the echo phenotype below.
Hypotension in CDH (Table 16-17)RV function intactRV function impairedRV function severely impaired
RV afterload / contractility+ / ++++ / ±++++ / − − − −
LV preload / LV systolic functionNormal or − / normal or −Normal or − / − −− / − − − −
Septum; TR jet; TAPSENormal; normal; normalFlat; normal or +; +Bowing into LV; ++; ++
Vasoactive choiceEpinephrineVasopressinConsider norepinephrine or ECMO

Avoid iNO if there is LV dysfunction and/or small left-sided structures. Consider milrinone for biventricular dysfunction. Baylor's TAPSE row reads "N, +, ++" across worsening RV function, but TAPSE falls as RV function worsens — read that row with caution.

Additional ECMO indications in CDH (Table 16-16)
  • OI >40 on two separate measurements.
  • PO₂ persistently <40 mmHg, or lactate rising above 3.0.
  • MAP on HFOV >17 cmH₂O.
  • Pre-ductal SpO₂ <85% with pH <7.15.
Hypotension ladder, lines, surfactant, repair and discharge
  • Pressor ladder for non-specific hypotension:
    • Start epinephrine 0.02 mcg/kg/min.
    • At ≥0.03 mcg/kg/min, add hydrocortisone 1 mg/kg IV every 8 h.
    • Titrate epinephrine to a maximum of 0.05, then add vasopressin 0.01 mcg/kg/min, increasing to 0.04. Reduce the dose if fluid retention, oliguria or hyponatraemia appear.
    • Still hypotensive on both at maximum: add norepinephrine 0.02 mcg/kg/min and consider ECMO.
    Rising pressor needs should always prompt an echo. Hypotension in CDH is multifactorial — assess volume status, cardiac position, filling and function (see Table 16-17 above).
  • On admission: confirm tube and line positions by chest and abdominal film, and monitor pre- and post-ductal SpO₂. Place a UAC and a low-lying UVC for reliable central access (strong recommendation, low quality), aiming for a PICC within 12–24 h when stable. Get a STAT head ultrasound if ECMO is likely, and an echo once stable (often the next day). Cluster care and minimize handling and noise, since the pulmonary circulation stays reactive.
  • Surfactant is not routine in the term non-FETO infant at birth (strong, low quality). It may be considered after FETO or for delivery below 37 weeks (strong, weak quality). Do not use NIRS readings to direct management (strong, low quality).
  • ECMO should be considered when saturation or gas targets cannot be met on maximal HFOV (weak, low quality). The decision rests with the CDH programme (ECMO neonatologist plus paediatric surgeon). Most infants who need ECMO have their repair on ECMO (see chapter 6.3).
  • Repair without ECMO when physiologically stable: FiO₂ <0.5, pre-ductal SpO₂ 85–95%, normal blood pressure for gestation, lactate <3 mmol/L, urine output >2 mL/kg/h.
  • Before discharge: monitor every CDH infant for pulmonary hypertension. After ECMO, obtain a brain MRI, neurodevelopmental assessment and follow-up, and a hearing assessment. Refer to a combined pulmonary hypertension and surgical outcomes clinic.

9. Surgical Repair Timing

Delayed, After Stabilization
  • Repair is not an emergency — it is performed after the infant is physiologically stable (improved pulmonary hypertension and gas exchange).
  • Approach (primary vs patch; open vs minimally invasive) is surgeon-led and depends on defect size.
  • Continue gentle ventilation and PPHN management perioperatively; expect a period of instability.

10. Monitoring

ParameterWhenAction
Pre/post-ductal SpO₂, gasesContinuousGentle ventilation; detect PPHN; permissive hypercapnia.
Blood pressure/perfusionContinuousSupport systemic BP; reduce shunting.
EchocardiographySerialAssess PPHN/cardiac function; guide therapy.
Chest signs/CXROn deteriorationDetect pneumothorax.
Readiness for surgeryOngoingOperate after stabilization.

11. Precautions

Safety Cautions
  • Do not bag-mask ventilate a known/suspected CDH — intubate and decompress the stomach.
  • Ventilate gently (low pressures, permissive hypercapnia) — over-ventilation causes pneumothorax and worsens PPHN.
  • Do not rush to surgery — stabilize first; surgery is not the emergency.
  • Manage PPHN and support systemic blood pressure; consider ECMO for refractory failure at an ECMO center.
  • Screen for associated cardiac/chromosomal anomalies.

12. Escalation & Family Support

Family-Centered Communication
  • "Your baby has a hole in the diaphragm, so some tummy organs are in the chest and the lungs are small and the blood pressure in the lungs is high. Our first job is to support the lungs gently and stabilize your baby."
  • "The operation to fix the diaphragm comes later, once your baby is stable — the breathing and lung-blood-pressure problems are what we treat first."

13. Key Pearls

High-Value Clinical Pearls
  • Do NOT bag-mask ventilate CDH — intubate and place a large gastric tube on suction.
  • The problem is pulmonary hypoplasia + pulmonary hypertension, not the hernia itself.
  • Use gentle, lung-protective ventilation with permissive hypercapnia; target pre-ductal saturations.
  • Manage PPHN and support systemic blood pressure; consider ECMO for refractory failure.
  • Surgery is delayed until physiological stabilization — it is not the emergency.
  • Clues: scaphoid abdomen, mediastinal shift, reduced breath sounds; screen cardiac/chromosomal anomalies.

14. Common Mistakes to Avoid

MistakeWhy it harmsBetter practice
Bag-mask ventilating CDH.Distends bowel; compresses lungs.Intubate; gastric decompression.
Aggressive ventilation.Pneumothorax; worse PPHN.Gentle, permissive hypercapnia.
Rushing to surgery.Operating on an unstable infant.Stabilize first; delayed repair.
Ignoring hemodynamics.Worsens shunting/PPHN.Support BP; treat PPHN.
Missing associated anomalies.Worse prognosis unrecognized.Echo + genetics.

15. Board-Style High-Yield Summary

Key Takeaways
  • CDH (usually left Bochdalek): the lethal problems are pulmonary hypoplasia and pulmonary hypertension, not the hernia itself.
  • At birth: do NOT bag-mask ventilate — intubate and place a large gastric tube on continuous suction.
  • Ventilate gently (low pressures, permissive hypercapnia); target pre-ductal saturations; watch for pneumothorax.
  • Manage PPHN (iNO/vasodilators per protocol) and support systemic blood pressure; ECMO for refractory failure.
  • Surgery is delayed until physiological stabilization — it is not the emergency.
  • Clues: scaphoid abdomen, mediastinal shift, reduced breath sounds; screen cardiac/chromosomal anomalies.

16. References

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