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Certain medical and surgical conditions presenting at delivery
or during the neonatal period require prompt diagnosis and timely treatment
to effect a good outcome. Recent advances in prenatal diagnostic
technology, including ultrasound techniques, fetal echocardiography,
and fetal MRI imaging, can facilitate prenatal counseling as well
as plans for delivery room and neonatal management. However, these
techniques do not substitute for a careful newborn assessment and
postnatal imaging to confirm and expand upon any prenatal diagnostic
studies. Coordination of care is essential, because these conditions
typically require the involvement of surgical and medical subspecialty
providers. The neonatologist or pediatrician should ensure intervention
is timely and in the patient’s best interest, and should
facilitate appropriate communication with family.
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The successful transition to extrauterine life depends upon patent,
structurally intact airways and specific physiologic shifts in pulmonary
vascular resistance and parenchymal water transport. Upper airway
obstruction is usually evident at or shortly after birth by increased
work of breathing, diminished air entry, poor perfusion, and in
extreme cases, cyanosis. Craniofacial anomalies can affect nasopharyngeal
patency. Choanal atresia usually involves the posterior nasal septum
of one or both choanae and can be anticipated by inability to pass
a 5-Fr. catheter into the posterior pharynx. Micrognathia associated
with conditions such as Pierre Robin syndrome may be sufficiently
severe to cause posterior displacement of the tongue and airway
obstruction. Similarly, severe macroglossia seen in association
with Beckwith-Wiedemann syndrome or subglossal cysts such as cannulae can
cause functional obstruction of the proximal airway. In many instances
of proximal airway obstruction, prone positioning and placement
of an oral airway may relieve symptoms until surgical correction
is possible. Severe cases may require tracheostomy. Antenatal diagnostic
studies may facilitate delivery room management. Several centers
report successful treatment of upper airway obstruction prior to
delivery with the infant still attached to the placental circulation.1,2
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Anomalous development of the larynx and proximal trachea may
present with symptoms ranging from subtle stridor to acute catastrophic
respiratory distress. Inspiratory stridor is most often associated
with laryngomalacia due to redundant aryepiglottic folds or an enlarged,
floppy epiglottis. Despite the stridor, respiratory compromise is
less common. If feeding problems or respiratory distress persist,
surgical laryngoplasty often yields good results. Unilateral vocal
cord paralysis usually presents with stridor, while bilateral cord
paralysis often presents as acute airway obstruction with severe
respiratory distress requiring urgent intervention.
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Unusual anomalies of the upper airway include laryngeal cysts,
tracheal stenosis, and laryngotracheal clefts. The extent of symptoms
and extent of intervention are functions of the severity of the
defect. Tracheal atresia with complete high airway obstruction syndrome
(CHAOS) is not compatible with survival unless diagnosed during
the prenatal period. Extended survival beyond the delivery room
has been reported in patients treated with tracheostomy at the time
of delivery.3
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Extrinsic compression of conducting airways can occur with congenital
tumors of the neck or mediastinum. Cystic hygromas and cervical
teratomas are most often implicated. ...