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Incidence
Investigation
Diagnosis of a posterolateral hernia is confi rmed by a chest x-ray [Fig.
5.2]. In left-sided defects, loops of bowel can be seen in the left chest.
The heart is deviated to the right. Little room is left for the lungs,
particularly the left lung which is markedly compressed. Sometimes,
the appearance may be diffi cult to distinguish from basal lung cysts,
in which case a repeat chest x-ray is performed after a nasogastric
tube has been inserted, the tip of which can be seen in the chest.
Alternatively, a barium study will show bowel within the thoracic
cavity when there is a diaphragmatic hernia.
Treatment
Posterolateral (Bochdalek) hernia
Where an antenatal ultrasound examination has identify ed a
diaphragmatic hernia, the best outcomes are achieved if the infant is
transferred to a tertiary paediatric surgical centre prior to birth. This is
because these infants may develop severe pulmonary distress very
quickly after birth, making subsequent transfer diffi cult and
potentially dangerous. Initial treatment involves intensive
cardiorespiratory support and insertion of a nasogastric tube to
prevent bowel dilatation within the chest. Care must be taken to avoid
hyperinfl ation and barotrauma of the small hypoplastic lungs. High-
frequency oscillatory ventilation in
combination with nitric oxide has improved survival rates. Ventilation
with a face mask (‘bagging’) should be avoided as this may force air
into the stomach, increasing its volume at the expense of the already
compromised lungs. Vigorous endotracheal ventilation should also be
avoided because of the risk of causing barotrauma and a tension
pneumothorax, which can lead to the rapid demise of the infant.
Exogenous surfactant provides no specifi c benefi t in newborns with
diaphragmatic hernia. The key to success is careful gentle ventilation
that minimizes injury to the hypoplastic lungs. Sudden deterioration
of the infant’s condition during
initial resuscitation or during transport suggests the development of a
tension pneumothorax, and this may necessitate prompt drainage by
needle aspiration or insertion of an intercostal drain. Fortunately,
strict avoidance of hyperventilation and limited infl ation pressures
have
made this complication rare.n Surgery to return the bowel to the
abdominal cavity and to repair the defect in the diaphragm is
performed when the infant’s condition is stable. This may be
anywhere between 12 h and 7 or more days after birth. In left-sided
defects, a left transverse or subcostal abdominal incision is used. The
management of the infant with severe hypoplastic lungs is diffi cult
and may involve highfrequency oscillation or extracorporeal
membrane oxygenation. The major cause of death remains pulmonary
hypoplasia and pulmonary hypertension. Pulmonary hypertension is
due to the small pulmonary vascular bed and to the changing
resistance of the pulmonary arterioles: it resolves in most patients
with time, provided ventilation does not produce additional lung
injury. Survival rates of about 80% are now being reported.
Diagram of the diaphragm and its attachment to the sternum,
showing the site of an anterior diaphragmatic hernia.
Key Points
Diaphragmatic hernia is diagnosed antenatally or by chest x-ray
in a baby with a barrel chest,scaphoid abdomen and respiratory
distress.
Ventilatory support, especially during transport, should be the
minimum required to prevent deterioration, as hyperinfl ation
with 2_ barotraumas is a signifi cant complication.
Sudden deterioration is usually caused by tension
pneumothorax.
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Case 1
Within minutes of birth, a full-term infant boy develops increasing
respiratory distress and becomes cyanosed. He fails to improve with
upper airway suctioning. The pregnancy was uneventful. He looks
barrel-chested and his abdomen is scaphoid.
Q 1.1 What is the diagnosis?
Q 1.2 What investigation will confi rm the diagnosis?
Q 1.3 What factors determine the outcome in these situations?
Case 2
A newborn infant with a recently diagnosed left-sided congenital
diaphragmatic hernia is about to be transferred to a paediatric surgical
institution by air. He is currently being ventilated through an
endotracheal tube and just maintaining adequate blood gas levels.
Q 2.1 Should his ventilation be increased during transport?
Q 2.2 Should any other manoeuvre be performed to reduce the
likelihood of problems during transport?
Q 2.3 If he suddenly deteriorates, what complication may have
happened?
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