Surgical Considerations and Postoperative Care of the Newborn
Robert J. Touloukian
Major advances in the care of the newborn have improved the survival and quality of life dramatically for babies born with a major congenital anomaly or acquired condition requiring emergency surgery during the first month of life. In most centers, the overall survival rate approaches 85% to 90%. The modern era of neonatal surgery can be traced to the early 1960s, with the establishment of tertiary-care neonatal special-care units, innovative surgical techniques, modern ventilatory management, intravenous nutrition, and the multimodal treatment of sepsis and shock.
Perinatology, with its emphasis on the diagnosis and treatment of high-risk fetuses having congenital anomalies, has paralleled the development of the high-risk obstetric service, thereby reducing the need for infant transport from community to tertiary-care centers. Improved radiologic modalities, including ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI), have revolutionized our ability to perform a noninvasive evaluation of a sick newborn. Certain fetal intervention procedures have become safe, and even hysterotomy with open corrective operation has resulted in fetal salvage.
Most surgical procedures in newborns are carried out by pediatric surgeons who are general surgeons with an additional 2 years of training obtained in one of 36 approved training centers in the United States and Canada (2003). This training encompasses the fields of neonatal and general surgery, head and neck trauma, transplantation, burns, endoscopy, gynecology, and urology. Diaphragmatic hernia, esophageal atresia, Hirschsprung disease, intestinal atresia, imperforate anus, omphalocele, and gastroschisis are certain “index” neonatal conditions for which exceptional technical skill and judgment must be learned by pediatric surgical trainees. Because each condition occurs no more than once per 3,000 live births, regionalization of care is required to achieve sufficient surgical expertise within the limited period of training. Currently, more than 600 active board-certified pediatric surgeons practice in the United States and Canada, the majority of whom are affiliated with a tertiary-care neonatal center.
The interface between neonatology and pediatric surgery begins with an initial evaluation of the infant and continues through the postoperative period. As in all other fields of pediatrics, good communication is needed. Because an infant’s condition must be monitored closely, neonatologists and primary nurses are essential partners of the surgeon. Surgeons, however, remain the responsible physicians for the overall care of surgical patients, determining the timing of operation, the need for supplementary evaluations, the progression of feeding schedules, antibiotic usage, and the like. That this relationship must be both collaborative and collegial is essential.
PRENATAL DIAGNOSIS
Prenatal diagnosis has revolutionized the surgical care of the newborn with a correctable anomaly (Table 30.1). Screening ultrasonographic examination and determination of the maternal serum alpha1-fetoprotein provide the first level of evaluation by the obstetrician, who then refers the mother for perinatology and pediatric surgical consultation at a center for high-risk patients. At this time, the surgeon and parents discuss the diagnosis and proposed treatment, further diagnostic testing (e.g., amniocentesis for chromosome analysis), and the corrective surgery required, along with its risks and potential benefits. Furthermore, the family should have a clear understanding of the potential for long-term physical disability or neurologic impairment in their infant. Hand-drawn illustrations, the use of medical textbooks, and clinical examples taken from the surgeon’s own experience may help to clarify many important questions about the fate of the fetus. More recently, the Internet has been the source of information for many parents.
TABLE 30.1. PRENATAL DETECTION AND OVERALL SURVIVAL OF COMMON NEONATAL SURGICAL CONDITIONS | |||||||||||||||||||||||||||||||||||||||||
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The advisability of terminating a pregnancy sometimes is raised if the diagnosis of a noncorrectable anomaly is made
before the twenty-fourth week of gestation. Specific indications for termination would include chromosome abnormalities incompatible with normal life (omphalocele with trisomy 13 or 18), severe neurologic impairment (e.g., encephalocele or anencephaly), or hazards to maternal health, but the need for corrective surgery alone is not deemed an appropriate reason to terminate a pregnancy. Delivery in a tertiary-care center obviates the need for infant transport and shortens the time from birth to surgical correction. This is mandatory for babies with congenital diaphragmatic hernia, abdominal wall defects, or intestinal obstruction when emergency surgery is required within hours of birth. Recent reports have cautioned against unnecessary cesarean section in the absence of evidence of fetal distress.
before the twenty-fourth week of gestation. Specific indications for termination would include chromosome abnormalities incompatible with normal life (omphalocele with trisomy 13 or 18), severe neurologic impairment (e.g., encephalocele or anencephaly), or hazards to maternal health, but the need for corrective surgery alone is not deemed an appropriate reason to terminate a pregnancy. Delivery in a tertiary-care center obviates the need for infant transport and shortens the time from birth to surgical correction. This is mandatory for babies with congenital diaphragmatic hernia, abdominal wall defects, or intestinal obstruction when emergency surgery is required within hours of birth. Recent reports have cautioned against unnecessary cesarean section in the absence of evidence of fetal distress.
INFANT TRANSPORT
Surgical conditions that are diagnosed postnatally require emergency transport of the neonate to a tertiary-care center. Esophageal atresia, Hirschsprung disease, and other forms of lower intestinal obstruction, including anorectal anomalies, are not detected routinely by prenatal ultrasonography but are potentially life-threatening within the first 12 to 24 hours of life. Despite the increasing number of babies with congenital diaphragmatic hernia diagnosed by routine prenatal screening, some of these infants’ disorders are not detected until respiratory distress develops shortly after birth. The indication encountered most frequently for emergency transport (from a level 2 nursery) is the baby who has “suspect” necrotizing enterocolitis and develops peritonitis after medical management is begun. For these reasons, a tertiary-care center must provide the capability for prompt transport by ambulance, helicopter, or other aircraft, depending on the distance from the referring hospital to the neonatal center.
The transport team consists of a cadre of neonatal nurses with advanced training in resuscitation, endotracheal intubation, institution of a peripheral intravenous line, umbilical artery catheterization, and insertion of a nasogastric or chest tube. The risks associated with specific anomalies and the means by which to prevent further injury or clinical deterioration should be understood. Babies born with a gastroschisis have rapid conductive heat and evaporative fluid loss. The exposed viscera must be covered with warm, moist saline sponges and plastic wrap to prevent additional fluid loss and inadvertent twisting of the mesentery or bowel. Early intervention with intravenous glucose solution is life-saving for a newborn who has omphalocele and macroglossia (Beckwith syndrome), becomes hypoglycemic, and could have brain-damaging seizures.
PREOPERATIVE PREPARATION
Several special considerations render the surgical care of the neonate unique. Box 30.1 lists general concerns in the surgical care of newborns.
Temperature Regulation
The premature neonate’s ratio of skin surface area to lean body mass is higher than that in an older infant or child. In the neonate, increased metabolic demands and the absence of a normal thermogenic shivering mechanism produce peripheral vasodilatation, resulting in rapid surface cooling and systemic hypothermia. This concern is particularly valid both in the stressed preterm infant, whose nutritional reserves are minimal, and when body cooling also leads to increased metabolism, which raises oxygen requirements. As heat loss continues, peripheral vasoconstriction occurs, with shunting of arterialized blood, increased lactic acid load, and eventually a profound metabolic acidosis.
BOX 30.1 General Considerations in the Surgical Care of the Newborn
Temperature regulation
Respiratory support
Cardiovascular status
Radiographic evaluation
Vascular access, fluid replacement
Antibiotic management
Catheters
Additional diagnostic tests
Informed consent
Hypothermia is minimized by examining the infant under a radiant heat warmer and by exposing the infant only for short periods when it is essential. Particularly concerning is the infant with gastroschisis or omphalocele, because evaporative fluid loss compounds the hypothermia. The use of a warming mattress and supplementary radiant heating lamps are additional helpful measures to prevent hypothermia. Once the initial evaluation of the infant is complete, a humidified and warmed isolation incubator (Isolette) provides the best substitute for the uterus.
Respiratory Support
Careful attention must be given to the airway and lungs because hypoxemia, hypercapnia, and oxygen desaturation often occur in the face the of extrinsic compression of the airway or because of the pulmonary aspiration of salivary or gastrointestinal secretions. This complication may develop rapidly in babies with cervical cystic hygroma involving the floor of the mouth and paratracheal tissues, those with such thoracic problems as congenital diaphragmatic hernia, or those with esophageal atresia with tracheoesophageal fistula. Abdominal distention and upward displacement of the diaphragm also may restrict ventilation in babies with neonatal intestinal obstruction. This problem is particularly common with more distal obstruction, such as meconium ileus, and in babies with a large ovarian or duplication cyst or hydronephrotic kidney. Careful monitoring of respiratory status is essential to detect apneic spells. These risks are magnified in a preterm infant because of the intrinsic tendency to irregular respiratory patterns. Any tendency toward desaturation mandates continuous monitoring, use of supplementary oxygen, and (often) institution of nasal continuous positive pressure or placement of an endotracheal tube with intermittent mandatory ventilation. A primary example of the importance of careful respiratory monitoring is seen in a newborn with congenital diaphragmatic hernia with adequate ventilation at birth. Because of the tendency toward an accumulation of air in the herniated intrathoracic stomach or bowel, the contralateral lung rapidly becomes compromised. In these patients, a combination of peripheral oxygen saturation and arterial blood gas determinations is important to detect hypoxia or a rising carbon dioxide level. A mixed respiratory and metabolic acidosis is a common occurrence.
Cardiovascular Status
Many infants with cyanotic congenital heart disease, such as aortic stenosis or hypoplastic left heart syndrome, appear to be
perfectly stable at birth and begin to show evidence of deoxygenation after the ductus arteriosus closes 24 to 48 hours after birth. Increased resistance in the pulmonary vascular bed, such as occurs in infants with pulmonary hypoplasia and congenital diaphragmatic hernia, tends to keep the ductus patent and cause right-to-left shunting of deoxygenated blood into the systemic circulation. The infant with a noncardiac anomaly also may have increased pulmonary vascular resistance secondary to hypoxia, acidosis, hypothermia, and hypovolemia. Unnecessary delays in treating newborns with intestinal obstruction predispose such infants to pulmonary aspiration that will aggravate any underlying tendency to increased pulmonary vascular resistance.
perfectly stable at birth and begin to show evidence of deoxygenation after the ductus arteriosus closes 24 to 48 hours after birth. Increased resistance in the pulmonary vascular bed, such as occurs in infants with pulmonary hypoplasia and congenital diaphragmatic hernia, tends to keep the ductus patent and cause right-to-left shunting of deoxygenated blood into the systemic circulation. The infant with a noncardiac anomaly also may have increased pulmonary vascular resistance secondary to hypoxia, acidosis, hypothermia, and hypovolemia. Unnecessary delays in treating newborns with intestinal obstruction predispose such infants to pulmonary aspiration that will aggravate any underlying tendency to increased pulmonary vascular resistance.
Radiographic Evaluation
At least one set of radiographic studies is obtained in any baby needing an abdominal or thoracic operation. Additional studies, including ultrasonography, CT, and MRI also may be useful.
The plain chest roentgenogram is diagnostic of the great majority of surgically correctable thoracic problems, including diaphragmatic hernia (Fig. 30.1), tension disturbances secondary to pneumothorax or hydrothorax, and even those lesions intrinsic to the lungs, such as lobar emphysema or congenital adenomatoid malformation. Caution must be exercised in distinguishing suspected congenital pulmonary cysts from a diaphragmatic hernia by obtaining an ultrasonography of the diaphragm or even by gastrointestinal contrast studies. Ventilation perfusion scans will give valuable information about the possibility of pulmonary hypoplasia in patients with diaphragmatic eventration, a condition that may be either congenital or secondary to brachial plexus birth injury.
Plain abdominal roentgenograms obtained in multiple views (i.e., supine, prone, and oblique) remain the gold standard in evaluating babies for intestinal obstruction, perforation, or a mass lesion. The oblique view is our choice for detecting free air with necrotizing enterocolitis. When such roentgenograms are repeated at 6-hour intervals, perforation with free air over the liver may be recognized before peritonitis is established and the baby becomes septic. Visualizing the number, distribution, and size of the dilated intestinal loops is very helpful in determining the site of obstruction. A diagnosis of duodenal stenosis can be facilitated by having the radiologist inject 30 or 40 cc of air through the nasogastric tube and then obtaining a prone view of the abdomen to best visualize a “double bubble.” When the etiology of duodenal obstruction remains uncertain, barium is introduced through the nasogastric tube to assess the location of the duodenum and thereby rule out malrotation, which can be associated with midgut volvulus. With more distal obstruction, the differential diagnosis among meconium ileus, ileal stenosis, meconium plug syndrome, and Hirschsprung disease can be confusing. Because plain films are nondiagnostic, a barium (or water-soluble contrast) enema should be carried out on an emergency basis. In many instances, the enema is therapeutic, but a submucosal biopsy must be obtained if Hirschsprung disease remains a diagnostic possibility.