4. EMBRYOLOGY
• Septum transversum divides pleural and coelomic cavities during fetal
development.
• This separation usually completes at the posterolateral region.
• Commonest location of the hernia – Postero-lateral (Bochdalek Hernia)
• Abdominal contents enter into the thorax
• Poor development of the abdomen – remains scaphoid
5.
6. EMBRYOLOGY
• Both lungs become hypoplastic,decreased bronchial and pulmonary artery
branching.
• Low lung weight,lung volume and DNA content.
• Most common location – LEFT
• Syndromic hernias – Gene mutations (Trisomy 18 and 21)
• Added surfactant deficiency may be present – worsens respiratory
insufficiency
7. PRE-NATAL DIAGNOSIS
• Pre-natal USG – CDH diagnosis (15 weeks of gestation).
• Look for
• 1) Herniated abdominal viscera
• 2) Changes in liver position
• 3) Mediastinal shift
• Index of severity in Left sided CDH – Lung to Head Ratio (LHR)
8. PRE-NATAL DIAGNOSIS
• LHR – Product of length and width of right lung at the level of the cardiac
atria divided by the head circumference(mm).
• LHR of less than 1 – poor prognosis
• LHR of greater than 1.4 – favorable outcome
9. POST DELIVERY DIAGNOSIS
• Chest X-Ray – Loops in thorax – D/D – Bronchopulmonary foregut
malformations.
• Respiratory distress due to
• 1) Pressure effects of the herniated loop
• 2) Pulmonary Hypertension
• 3) Hypoplastic ipsilateral lung
• Mortality around 50%.
10.
11. TREATMENT
• Prompt cardio-respiratory stabilization
• Initial 24 to 48 hours after birth – relatively stable due to high levels of Pao2
and good perfusion – “Honeymoon Period” – progresses to CP failure.
• Earlier immediate surgical intervention.
• Now , as the main cause of the distress is PULMONARY
HYPERTENSION and PULMONARY HYPOPLASIA. So, current
management is managing pulmonary hypertension and minimising baro-
trauma while optimising oxygen delivery (CONTROLLED MECHANICAL
VENTILATION).
12. TREATMENT
• Maintain PaCO2 between 50-60 mmHg;pH >7.25.
• Alternate – High Frequency Oscillatory Ventilation(HFOV)
• Do ECHO – Pul.Htn. And cardiac anomalies.
• ICU – 1) Minimal sedation
• 2) ET suctioning
• 3) Avoidance of Pulm.Htn via hypoxia (Use Inhaled NO) or
(Bicarbonate administration)
13. TREATMENT
• If worsening of Pulm.Htn – right sided heart failure ensues and systemic
perfusion is impaired. – may need I.V fluid titration and ionotropic support.
• If persistently hypoxic – ECMO via venovenous (or) venoatrial access.
• Maintained on ECMO till Pulm.Htn reduces and lung ventilation improves
(usually 7-10 days to several weeks).
• May cause bleeding due to associated anti-coagulation.
14. TREATMENT
• Criteria for using ECMO –
• 1) Normal cardiac anatomy based on ECHO
• 2) No fatal chromosomal anomalies
• 3) Infant would die without an ECMO
• 4) Weight more than 2 kgs
• 5) Gestational age more than 34 weeks.
15. TREATMENT
• Hypercapnia with avoidance of barotrauma – another strategy.
• Patients not on ECMO – surgical repair done once HD status is normal.
• Patients on ECMO – perform early repair on bypass (or) wait until infants
lung has improved and Pulm.Htn subsided.
• Approaches – abdominal (or) transthoracic
• Post op ventilation continued and slow weaning to avoid Pulm.Htn.
19. PATHOPHYSIOLOGY
• Increased intra-abdominal pressure produces a rapid deflation of the lungs
via an open glottis – produces a large pleuroperitoneal pressure gradient
• The tears commonly occur at the weak portions – muscular portions (costal
muscle).
• Types – Circumferential (or) Radial (or) Both
• Right (or) Left
20. CLINICAL FEATURES
• Commonly presents with shock
• Chronic diaphragmatic hernias-
• 1) respiratory – (dyspnoea,exercise intolerance)
• 2) GI – Anorexia,vomiting,diarrhoea,weight loss,pain after food ingestion.
• 3) Non-specific – Depression
21. INVESTIGATIONS
Chest X-ray – loss of diaphragmatic line
loss of cardiac silhouette
dorsal (or) lateral displacement of lung fields
presence of gas (or) barium filled stomach (or) intestines in
thoracic cavity
Pleural effusion
USG Abdomen
