Congenital Diaphragmatic Hernia (Case 33)

Chapter 74 Congenital Diaphragmatic Hernia (Case 33)



Christiana R. Farkouh, MD, MPH




Case


A newborn term infant presents with cyanosis and respiratory distress at birth. Initial chest radiograph demonstrates translucencies of various sizes in the left hemithorax, and the cardiac silhouette is deviated into the right hemithorax.




Differential Diagnosis









Congenital diaphragmatic hernia (CDH) Congenital cystic adenomatoid malformation (CCAM) Bronchopulmonary sequestration (BPS)


Speaking Intelligently


If the diagnosis is known before birth, a thorough prenatal evaluation should ensue to determine if other congenital abnormalities are present that may represent a known genetic defect/chromosomal anomaly or syndrome. Subsequently, these infants should be delivered in institutions that can provide maximal cardiorespiratory support in the form of extracorporeal membrane oxygenation (ECMO) because these infants can exhibit severe cardiorespiratory instability and pulmonary hypertension. Immediately after birth I intubate the airway and place a Replogle tube into the stomach with continuous suction to minimize gastric and intestinal distention with air. These interventions serve to secure lung expansion and minimize intestinal distention. Because the diagnosis is not always known before birth, I strongly recommend obtaining a chest radiograph immediately when a newborn infant presents with respiratory distress. Because of the clinical finding of diminished breath sounds on the side of the thorax where abdominal organs have herniated, the possibility of a pneumothorax exists and may subsequently lead to placement of a chest tube or needle thoracotomy before obtaining radiographic evidence of a pneumothorax. Such an intervention will not only be of no clinical benefit, but may be more injurious if any of the abdominal viscera are punctured.



Patient Care



Clinical Thinking



• Once the infant is born, immediately intubate the trachea and place a Replogle catheter into the stomach with continuous low wall suction.

• Proper positioning of the infant with the affected side down also serves to support full expansion of the contralateral lung.

• Intravenous and arterial access is then obtained typically via umbilical vessels. However, umbilical venous catheters may be difficult to position, especially if the liver is also herniated into the chest.

• CDH is not a surgical emergency. Stabilizing the infant’s respiratory status with either mechanical ventilation or with the use of ECMO is the priority.

• If ECMO appears likely given the degree of lung disease, serious discussions with the family are warranted, given the risks of ECMO itself and the concern for prolonged respiratory difficulties after repair is accomplished.

• In addition to the structural defect in the diaphragm that results in migration of abdominal contents into the chest, which impedes lung development on the affected side, the contralateral lung tissue is affected as well because of the shift in the mediastinal structures into the opposite chest.

• Some patients may also suffer from some degree of pulmonary hypoplasia on both sides of the lung as a result of interference in the normal growth and development of the lung.

• Because of the possible need for ECMO, these infants should all receive a head ultrasound, and echocardiogram should also be performed to visualize the cardiac structure, exclude any congenital heart defect, and ascertain overall cardiac function.


History



• Because CDH is readily identifiable on prenatal ultrasound, the majority of these infants are identified prenatally.

• History may be limited to the pregnancy history, prenatal ultrasounds, and even possibly prenatal magnetic resonance imaging (MRI).

• When not identified prenatally, these infants suffer respiratory distress immediately after birth, at which time the defect is identified on routine CXR.

• CXR demonstrates abdominal contents (stomach, bowel, and/or liver) in the thorax.


Physical Examination



• Vital signs: Tachypnea, respiratory distress, and hypoxia (cyanosis).

• The hallmark finding in infants with CDH is a scaphoid abdomen due to the abdominal contents being in the chest.

• Diminished breath sounds on the affected side and heart sounds may be better auscultated in the midline or more laterally depending on the side of the defect. A left CDH may have better heart sounds in the midline or right side of the sternum.

• The actual size of the defect in the diaphragm cannot be ascertained until in the operating room.

• Identify if other congenital anomalies are apparent on examination.


Tests for Consideration



• Arterial blood gas $156
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Jul 18, 2016 | Posted by in PEDIATRICS | Comments Off on Congenital Diaphragmatic Hernia (Case 33)

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