CONGENITAL DIAPHRAGMATIC HERNIA
During fetal development, the gut can herniate into the thorax through
one of three possible dia-phragmatic defects: the left or right posterolateral
foramen of Bochdalek or the anterior foramen of Morgagni. The reported
incidence of diaphrag-matic hernia is 1 in 3000–5000 live births. Left-sided
herniation is the most common type (90%). Hallmarks of diaphragmatic
herniation include hypoxia, a scaphoid abdomen, and evidence of bowel in the
thorax by auscultation or radiogra-phy. Congenital diaphragmatic hernia is
often diagnosed antenatally during a routine obstetric ultrasound examination.
A reduction in alveoli and bronchioli (pulmonary hypoplasia) and mal-rotation
of the intestines are almost always pres-ent. The ipsilateral lung is
particularly impaired and the herniated gut can compress and retard the
maturation of both lungs. Diaphragmatic hernia is often accompanied by marked
pulmonary hyper-tension and is associated with 40–50% mortality.
Cardiopulmonary compromise is primarily due to pulmonary hypoplasia and
pulmonary hyperten-sion rather than to the mass effect of the herniated
viscera.
Treatment is aimed at immediate stabilization with sedation, paralysis,
and moderate hyperven-tilation. Pressure-limited ventilation is used. Some
centers employ permissive hypercapnia (postductal Paco2< 65 mm Hg) and accept mild
hypoxemia (preductal Spo2> 85%) in an effort to reduce
pul-monary barotrauma. High-frequency oscillatory ventilation (HFOV) can
improve ventilation and oxygenation with less barotrauma. Inhaled nitric oxide
may be used to lower pulmonary artery pres-sures but does not appear to improve
survival. If the pulmonary hypertension stabilizes and there is little
right-to-left shunting, early surgical repair may be undertaken. If the patient
fails to stabilize, extracorporeal membrane oxygenation (ECMO) may be
undertaken. When initiated in the critical care unit in a neonate, venoarterial
ECMO usu-ally involves pumping blood from the jugular vein through a membrane
oxygenator and countercur-rent heat exchanger before returning it to
ipsilat-eral carotid artery. Timing of the repair following ECMO is
controversial. Treatment with prena-tal intrauterine surgery has not been shown
to improve outcomes.
Gastric distention must be minimized by placement of a nasogastric tube
and avoidance of high levels of positive-pressure ventilation. The neonate is
preoxy-genated and intubated awake, or without the aid of muscle relaxants.
Anesthesia is maintained with low concentrations of volatile agents or opioids,
muscle relaxants, and air as tolerated. Hypoxia and expan-sion of air in the
bowel contraindicate the use of nitrous oxide. If possible, peak inspiratory
airway pressures should be less than 30 cm H2O. A
sud-den fall in lung compliance, blood pressure, or oxygenation may signal a
contralateral (usually right-sided) pneumothorax and necessitate place-ment of
a chest tube. Arterial blood gases are pref-erably monitored by
sampling a preductal artery if an umbilical artery catheter is not already in
place. Surgical repair is performed via a subcostal incision of the affected side;
the bowel is reduced into the abdomen and the diaphragm is closed. Aggressive
attempts at expansion of the ipsilateral lung fol-lowing surgical decompression
are detrimental. Postoperative prognosis parallels the extent of pul-monary
hypoplasia and the presence of other con-genital defects.
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