Chapter 131: Diaphragmatic Hernia

Bochdalek hernia (posterolateral) · Morgagni hernia · Pulmonary hypoplasia · FETO · Prenatal markers (LHR, O/E TLV) · ECMO · Surgical repair · Prognosis
🫁 Key fact: Bochdalek hernia accounts for 90% of CDH, 80-90% left-sided. Survival ~75% with modern therapy.

🫁 Congenital Diaphragmatic Hernia: Core Concepts

📊 Epidemiology
Incidence 1:2,000-3,000 live births. 85% left-sided. 80-90% Bochdalek (posterolateral). Associated with pulmonary hypoplasia and PPHN.
🧬 Prenatal predictors
Observed/expected lung-to-head ratio (O/E LHR) <25% → severe. Liver herniation → worse prognosis. FETO improves survival.
🩺 Clinical presentation
Respiratory distress at birth, scaphoid abdomen, bowel sounds in chest, mediastinal shift. Delayed presentation possible.
⚕️ Delivery management
Intubate immediately, orogastric tube, avoid bag-mask ventilation. Transfer to tertiary center. ECMO available.
💊 Ventilation strategy
Gentle ventilation (PIP <25), permissive hypercapnia (PaCO2 45-65), avoid high pressures (volutrauma).
📈 Outcomes
Overall survival ~75%. Predictors: O/E LHR, liver position, need for ECMO, associated anomalies. Long-term GERD, neurocognitive deficits, pulmonary issues.
⚡ Associated anomalies: Cardiac (20%), chromosomal (trisomy 13,18,21), genetic syndromes (Fryns, Cornelia de Lange).

🔍 Approach to newborn with suspected congenital diaphragmatic hernia

1
Prenatal diagnosis (US/MRI) – Mediastinal shift, stomach/liver in chest, polyhydramnios. Measure O/E LHR, liver position. Refer to fetal center.
2
Delivery planning – Deliver at tertiary center with ECMO, pediatric surgery, neonatology. Ex utero intrapartum therapy (EXIT) rarely needed.
3
Immediate postnatal management – Intubate (do NOT bag-mask ventilate). Place orogastric tube to continuous suction. Avoid high peak pressures.
4
Confirm diagnosis – Chest X-ray: bowel gas in chest, mediastinal shift, NG tube in thorax. Echocardiogram for cardiac anomalies and PPHN.
5
Stabilization before surgery – Gentle ventilation, permissive hypercapnia, treat PPHN (iNO, HFOV, ECMO if needed). Surgery after stabilization (typically >48-72h).

📋 Stepwise management of congenital diaphragmatic hernia

1
Delivery room – Immediate intubation (ETT), orogastric tube (low continuous suction). Avoid bag-mask ventilation (prevents bowel distension).
2
Respiratory support – Gentle conventional ventilation (PIP <25, PEEP 3-5, rate 30-40). Permissive hypercapnia (PaCO2 up to 65). HFOV for rescue.
3
PPHN management – iNO (20 ppm), milrinone, sedation, treat acidosis. ECMO if OI >40.
4
Hemodynamic support – Inotropes (dobutamine, epinephrine) for RV dysfunction. Avoid dopamine (may increase PVR).
5
Surgical repair – Delay until after stabilization (typically 48h-7d). Primary closure or patch repair (Gore-Tex). Laparoscopic/thoracoscopic in stable infants.
6
Postoperative care – Monitor for recurrence, chylothorax, GERD, pulmonary hypertension. Long-term follow-up for growth, neurodevelopment, hearing, lung function.
📌 FETO (fetoscopic endoluminal tracheal occlusion): For severe CDH (O/E LHR <25%), balloon placed at 27-29 wk, removed at 34 wk. Improves survival from 15% to 40%.

🧠 Rapid reflex prompts – Diaphragmatic Hernia

📌 Most common type of CDH?
Bochdalek hernia (posterolateral, 90%, left-sided 80-90%).
📌 First step in delivery room for known CDH?
Intubate immediately, place orogastric tube, avoid bag-mask ventilation.
📌 Prenatal predictor of poor prognosis?
Liver herniation into thorax and O/E LHR <25%.
📌 FETO procedure: what does it do?
Tracheal occlusion promotes lung growth in severe CDH.
📌 ECMO criteria in CDH?
OI >40 despite optimal medical therapy, or inability to oxygenate/ventilate.
📌 Most common long-term complication after CDH repair?
Gastroesophageal reflux disease (GERD) (>50%).
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