Several congenital anomalies presenting in the newborn period require immediate diagnosis and treatment. Certain conditions constitute medical and surgical emergencies in the delivery room and are best handled by anticipation and a multidisciplinary team approach to improve chances of successful outcomes. Basic principles of resuscitation should be applied in all cases by a team skilled in airway management and circulatory support. Recent advances in prenatal diagnostic technologies facilitate prenatal counseling as well as delivery room strategies and coordination of neonatal care.
The transition from fetus to newborn is the most complex physiologic adaptation that occurs in human life. Inadequate transition from fetal to neonatal life is one of the most common causes of cardiopulmonary insufficiency in the newborn.
Normal airway anatomy and patency are essential for a successful transition to extrauterine life. Bilateral choanal atresia is a life threatening condition. Since newborns are obligatory nose breathers, symptoms appear immediately after birth. It is often associated with other developmental anomalies such as CHARGE (coloboma, heart defects, choanal atresia, retarded growth and development, genito-urinary abnormalities, and ear anomalies), Treacher Collins, and Tessier syndromes.
Micrognathia is usually associated with conditions such as Pierre Robin sequence. At birth, this can be severe enough to cause posterior displacement of the tongue with airway obstruction, and can be an airway emergency. In many cases, prone positioning and placement of an oral airway may relieve symptoms until surgical correction is possible, but in the extreme cases, even in the most experienced hands, intubation can be very challenging. Mandibular distraction osteogenesis has been increasingly used for neonates with this congenital anomaly.
Prenatal diagnosis has transformed the outcome of fetuses with airway obstruction. Thorough evaluation of prenatal imaging allows for categorizing fetuses with airway compromise into those who will require a special mode of delivery and those who can be delivered without any special resources. The ex utero intrapartum treatment (EXIT) approach allows accessing the airway while the fetus is still attached to placental support. This permits the fetal airway to be secured in an elective, controlled manner. Once adequate ventilation has been established, the fetus is taken off placental support. The indications for an EXIT procedure have grown over time and now include a myriad of conditions, including cervical lymphangiomas, cervical teratomas, large congenital lung malformations, and congenital high airway obstruction syndrome (CHAOS).
Acute Hypoxemic Respiratory Failure
Neonatal hypoxemic respiratory failure (HRF) can be due to a variety of etiologies, including prematurity, perinatal insults or disorders of transition, and congenital lung abnormalities. Persistent pulmonary hypertension (PPHN) of the newborn refers to a failure of the normal fall in pulmonary vascular resistance that normally occurs during the first hours and days after birth. While PPHN can occasionally be an isolated problem with known obvious precipitant, it is more commonly associated ...