@ It is a disease with high mortality (30-60%) and the most important factor determining mortality is lung hypoplasia.
@ Pulmonary hypertension and pulmonary hypoplasia must be reduced before surgery in these patients.
@ Congenital diaphragmatic hernias occur from three points.
@ Posterolateral (Bochdalek) hernias are the most common (85-90%) and approximately 90% are on the left posterolateral.
@ Retrosternal (Morgagni) hernias are the second most common.
@ The third type is hiatal hernias.
@ The phrenic nerves provide the motor and sensory innervation of the diaphragm.
@ Pulmonary hamartoma and congenital cysts, diaphragmatic eventration, hemopneumothorax should be considered in the differential diagnosis.
Congenital posterolateral diaphragmatic hernia (Bochdalek hernia)
• It is 1/1000 in stillbirths, 1/2000-5000 in live births, and it is 2 times more common in girls.
• 30% are stillborn due to other major additional anomalies.
• 10-38% have a hernia sac (pleura-peritoneal membrane).
• 85-90% is on the left side.
• Small intestines are the most common intra- abdominal organ passing from the defect to the thoracic cavity. Then the spleen, stomach, colon and left lobe of the liver pass respectively.
• In right-sided hernias, mainly the liver fills the thoracic cavity.
• The most important factor determining the prognosis is the degree of pulmonary hypoplasia and whether pulmonary hypertension will return due to this.
• There is NO RELATIONSHIP between the size of the defect and the amount of organ passing into the thoracic cavity.
• Lung pressure increases after birth as swallowed air will cause distention (VENTILATION WITH MASK).
• Pulmonary hypertension and persistent fetal circulation are often present.
• Additional anomalies have an important role on mortality and major additional anomalies are 3 times more common in right hernias.
• Anencephaly, meningomyelocele, hydrocephalus, NTD (neural tube defects), cardiac anomalies, esophageal atresia, omphalocele, cleft palate and rotation anomalies.
• They are the most common accompanying cardiac anomalies. The main ones are; heart hypoplasia, aortic coarctation, ASD, VSD, vascular ring.
• Ultrasonography is frequently used in antenatal diagnosis and intrauterine surgery can be performed.
• Trisomy 13, 18, 21 and 12p tetranomy are common.
• The most prominent symptom is respiratory distress that occurs shortly after birth.
The baby is dyspneic, tachypneic and cyanotic.
• The heart apex beat is displaced.
• Abdomen is collapsed, breathing and heart sounds are deep. Bowel sounds are heard in the thoracic cavity.
• The hemithorax on the side of the hernia does not participate in breathing and lung sounds are not heard on that side.
• On the X-ray that includes the lungs and abdominal cavity (babygram); Gas-filled intestinal loops, mediastinal shift, and suppressed lung tissue are seen on the hernia side.
• Barium X-ray (from the rectum) and computed tomography are useful in diagnosis.
• While left hernias are mostly repaired by laparotomy, thoracotomy is preferred in right hernias because of the liver.
• Pulmonary hypertension must be controlled before surgery.
• The average survival rate after surgery is 60%, and babies who have respiratory distress in the first 48 hours have a low chance of survival.
Retrosternal hernia (Parasternal hernia/Morgagni hernia)
• It is a component of Cantrell's pentology.
• Almost all of them have a hernia sac.
• Colon and omentum often enter the sac.
• The most common accompanying anomaly is congenital heart diseases.
• In addition, gastrointestinal system anomalies, omphalocele, trisomy 21 and Down syndrome may accompany.
• It is significantly associated with mental retardation.
• The majority are asymptomatic and the diagnosis is made by the presence of gas behind the sternum on routine chest X-ray.
• It does not give many symptoms in the neonatal period, if it does, congenital heart diseases should be considered.
• Older children have recurrent upper respiratory tract infections, cough, vomiting and epigastric restlessness.
• On the chest X-ray, there are liquid-gas shadows of the intestine behind the sternum. Lateral X-ray, contrast-enhanced X-ray and computed tomography are also helpful in diagnosis.
• Treatment is surgical correction with a mid-umbilical incision.
• The most important complication after surgery is pneumopericardium.
Diaphragm Eventration
0 Eventration is the upward doming of the diaphragm in the thoracic direction, with an intact but poorly developed muscular layer.
0 It is due to insufficient development of muscles.
0 All or part of the diaphragm is higher than normal.
0 It is usually unilateral and is more common on the left side.
0 Those due to phrenic nerve injury are more common on the right side.
0 There are paradoxical diaphragmatic movements and respiratory distress. In inspiration, the diaphragm rises instead of descending, and at the same time, the mediastinum is pushed to the opposite side.
0 In severe cases, lung hypoplasia may accompany.
0 Congenital eventration, congenital diaphragmatic hernia, mass in the lung or mediastinum are important in the differential diagnosis.
0 Diagnosis AP and lateral chest X-ray, fluroscopy and ultrasonography.
0 If there is respiratory distress, diaphragmatic plication is performed.