BEDSIDE ACTION BOX — The CDH Delivery
- Intubate immediately — do NOT bag-mask ventilate (positive-pressure mask ventilation distends intrathoracic bowel and worsens lung compression).
- Place a large-bore gastric tube on continuous suction to decompress the gut.
- Use gentle, lung-protective ventilation; permissive hypercapnia; target preductal saturations; avoid barotrauma.
- Manage pulmonary hypertension actively; repair is not an emergency — stabilize first.
- ECMO may be a bridge in refractory cases at capable centers.
1. Overview & Definition
Definition
Congenital diaphragmatic hernia is a developmental defect in the diaphragm — most commonly a left posterolateral (Bochdalek) defect — that lets abdominal viscera herniate into the chest. The core problem is not the hole itself but the pulmonary hypoplasia and pulmonary hypertension that result from the developing lung being compressed.
Why This Topic Matters
Survival hinges on managing lung physiology, not on rushing to close the defect. Getting the delivery-room and stabilization steps right — and resisting the urge to bag-mask or operate early — is what saves these babies.
2. Who This Guideline Applies To
Scope
- Infants with antenatally or postnatally diagnosed CDH.
- Cross-references: persistent pulmonary hypertension (6.3); gentle ventilation (6.7); ECMO criteria; abdominal wall defects (14.3).
3. Pathophysiology
Herniated viscera compress the ipsilateral (and, via mediastinal shift, contralateral) lung during a critical window of development. The result is fewer, smaller alveoli and an abnormal, muscularized pulmonary vasculature — hypoplasia plus a vascular bed primed for pulmonary hypertension. After birth, hypoxia and acidosis drive right-to-left shunting, creating a vicious cycle that must be interrupted with gentle ventilation and pulmonary-hypertension management.
4. Clinical Presentation
At the Bedside
- Respiratory distress from birth, often severe.
- Scaphoid (sunken) abdomen and a barrel-shaped chest.
- Displaced heart sounds (mediastinal shift, usually to the right with left-sided CDH); bowel sounds in the chest.
- Antenatal diagnosis is common; prognosis relates to liver position ("liver up") and lung-to-head ratio.
5. Recommended Workup
What to Check
- Chest/abdominal X-ray: bowel gas in the hemithorax, mediastinal shift, gasless abdomen, tip of the gastric tube in the chest.
- Pre- and post-ductal saturations; blood gas.
- Echocardiogram: pulmonary hypertension, cardiac function, and to exclude associated congenital heart disease.
- Consider genetic evaluation for associated anomalies/syndromes.
6. Stabilization Algorithm
1
At delivery
Immediate intubation; avoid bag-mask ventilation; large-bore gastric tube to suction.
2
Ventilate gently
Lung-protective settings, permissive hypercapnia, target preductal SpO₂; avoid high pressures/volutrauma.
3
Treat pulmonary hypertension
Optimize oxygenation and perfusion; pulmonary vasodilators and inotropic support per protocol; echo-guided.
4
Refractory failure?
Consider ECMO as a bridge at a capable center.
5
Stabilized
Plan surgical repair once physiologically stable — not before.
7. Surgical Repair
Principles
- Repair is delayed until the infant is physiologically stable (stable pulmonary hypertension, adequate oxygenation on gentle support).
- The viscera are reduced into the abdomen and the defect is closed — primary closure for small defects, a patch for large ones.
- Timing and approach (open vs minimally invasive) are individualized by the surgical team.
Other congenital lung lesions (Baylor Ed. 33)
- CDH medical management — delivery room, ventilation, pressors, ECMO criteria and repair readiness — is in chapter 11.7.
- Bronchopulmonary sequestration: non-functioning lung with no airway connection and a systemic arterial supply (usually from the aorta), mostly unilateral and near the left lower lobe; hyperechoic on fetal ultrasound and hard to separate from CPAM. A large arteriovenous shunt can cause high-output failure, hydrops or pulmonary haemorrhage. Extralobar lesions rarely need urgent resection unless the shunt is symptomatic; intralobar lesions are resected electively because of infection risk. Arrange surgical follow-up.
- Congenital pulmonary airway malformation (CPAM): almost always unilateral, one lobe; cystic on ultrasound with no systemic supply on Doppler; fetal MRI helps distinguish sequestration. Microcystic (solid-appearing) lesions carry a worse prognosis. CPAMs usually grow until about 28 weeks, then plateau or involute — follow with serial ultrasound. Hydrops is grave, and hydrops before 32 weeks makes fetal intervention a consideration; a CPAM volume ratio (volume ÷ head circumference) above 2.0 best predicts hydrops and the need for intervention. Deliver large lesions where fetal surgery, HFOV, ECLS and emergency surgery are available. Get a postnatal CT even if the lesion seemed to regress. Resect symptomatic lesions early, and Baylor recommends resecting all CPAMs because of later rhabdomyosarcoma and difficulty excluding pleuropulmonary blastoma. Severe hypoplasia may bring pulmonary hypertension.
- Congenital lobar emphysema: usually one lobe, most often the left upper lobe; causes include bronchial abnormalities, mucus plugs and extrinsic compression, but no obstruction is found in at least half. About 15% have cardiac or vascular anomalies. It presents postnatally with worsening distress and an over-distended lobe on film. Mild cases can be managed with oxygen (resection of asymptomatic lobes is debated, though infection often prompts it); progressive compression requires lobectomy. Positive pressure can abruptly worsen air trapping — the surgeon should be present at induction in case urgent thoracotomy is needed.
8. Monitoring
What to Track
- Pre-/post-ductal saturations, gases, and ventilator requirements.
- Serial echocardiography for pulmonary hypertension and cardiac function.
- Perfusion, urine output, and acid–base balance.
- Post-operative respiratory course and feeding tolerance.
9. Complications
Watch For
- Persistent pulmonary hypertension and chronic lung disease.
- Gastroesophageal reflux and feeding difficulties.
- Recurrent herniation (more common after patch repair).
- Neurodevelopmental impairment and hearing loss; chest-wall/scoliosis issues over time.
10. Escalation & Follow-Up
Escalate When…
- Refractory hypoxemia or pulmonary hypertension (consider ECMO/transfer).
- Hemodynamic instability or suspected associated cardiac disease.
Follow-Up
- Multidisciplinary CDH follow-up: pulmonary, cardiac (pulmonary hypertension), GI/nutrition, neurodevelopment, and hearing surveillance.
Parent Counselling Points
- "Because some of the tummy organs were in the chest, the lung on that side is small and the blood vessels are tense. Our first job is to help the lungs and heart settle."
- "The operation to fix the diaphragm comes after your baby is stable, not straight away."
11. Key Pearls
High-Value Clinical Pearls
- Never bag-mask a known CDH — intubate and decompress the stomach.
- Repair is elective-after-stabilization, not an emergency.
- The disease is lung hypoplasia + pulmonary hypertension, not just a hole in the diaphragm.
- Gentle ventilation and permissive hypercapnia beat aggressive ventilation.
- "Liver up" and a low lung-to-head ratio predict worse outcomes.
12. Common Mistakes to Avoid
Pitfalls & Better Practice
The recurring errors in CDH.
| Mistake | Why it harms | Better practice |
|---|
| Bag-mask ventilation. | Distends intrathoracic bowel, worsens compression. | Intubate immediately; gastric decompression. |
| Rushing to surgery. | Operating on unstable physiology increases mortality. | Stabilize pulmonary hypertension first. |
| Aggressive ventilation for "better gases." | Barotrauma worsens the hypoplastic lung. | Gentle ventilation, permissive hypercapnia. |
| Missing associated cardiac disease. | Alters management and prognosis. | Echocardiography early. |
13. Board-Style High-Yield Summary
Key Takeaways
- CDH (usually left Bochdalek) → pulmonary hypoplasia + pulmonary hypertension.
- Delivery room: intubate, do NOT bag-mask, decompress the stomach.
- Gentle ventilation, permissive hypercapnia, treat pulmonary hypertension; ECMO for refractory cases.
- Repair is delayed until physiologically stable.
- Prognosis: "liver up" and low lung-to-head ratio predict worse outcome; long-term pulmonary, GI, and neurodevelopmental morbidity.
14. References
- 1.Snoek KG, et al. Standardized Postnatal Management of Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus — 2015 Update. Neonatology.
- 2.Puligandla PS, et al. Management of congenital diaphragmatic hernia: a systematic review.
- 3.Baylor College of Medicine, Division of Neonatology. Guidelines for Acute Care of the Neonate, Edition 33, 2025–2026.
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