Improving Neonatal Emergency Care: Critical Concepts
Authors: Tonia J. Brousseau, DO, Pediatric Emergency Medicine Fellow, University of Florida Health Science Center, Shands, Jacksonville; and Ghazala Sharieff, MD, FACEP, FAAP, FAAEM, Associate Clinical Professor, Children’s Hospital and Health Center/ University of California, San Diego; Director of Pediatric Emergency Medicine, Palomar-Pomerado Hospital/ California Emergency Physicians, San Diego, California
Peer Reviewer: Roy M. Vega, MD, FAAP, Attending Physician Pediatric Emergency Medicine, Long Island Jewish Medical Center, New Hyde Park, New York
Infants younger than 28 days (neonates) particularly are challenging to the emergency department (ED) physician. Parental concerns may vary from innocuous rashes to life-threatening emergencies. A difficult delivery may result in subconjunctival hemorrhages, caput succedaneum, or subgaleal hemorrhages, conditions that may not be appreciated by the family until after discharge from the hospital.
The ED physician must be familiar with the normal variants and also the subtle findings that may indicate a more serious underlying problem. The amazing physiologic changes that the newborn accomplishes following delivery may precipitate disaster, if an underlying congenital problem exists.
The astute ED physician is also familiar with particular high-risk times when critical diseases may present, such as cyanotic congenital heart disease, and has a heightened suspicion for these disease processes.
This article provides a comprehensive overview of critical diseases that may present in the newborn period and strategies for their early detection and appropriate management. — The Editor
Introduction
The evaluation and appropriate management of the critically ill neonate (younger than 28 days of age) requires an intimate knowledge of the physiologic changes and life-threatening pathologies that may present during this time period. The innate differences in this fragile population may produce anxiety for the emergency department (ED) physician. Therefore, a broad systematic approach to evaluating the neonate is necessary to provide a comprehensive, yet specific, differential diagnosis for a presenting complaint or symptom. Efficient recognition and prompt management of illness in the neonatal period may be life-saving. Due to early discharge policies from newborn nurseries in recent years, it has become more important for the ED physician to be familiar with the neonate.1
This review categorizes the more common neonatal emergencies and specific management by systems to provide a thorough knowledge base of neonatal emergencies.
The Cardiovascular System
Cyanotic Heart Disease. Cyanotic congenital heart defects that are not detected in the newborn nursery typically will present during the first 2-3 weeks of life when the ductus arteriosus (DA) closes. The neonatal physiology is dynamic because it changes from a low pressure circulatory system as a fetus to a high pressure system after birth. With the neonate’s first breath, oxygen and other mediators stimulate the closure of the DA. Functional closure of the ductus occurs in the first 10-14 hours of life, but anatomic closure may not occur until 2-3 weeks of age due to prematurity, acidosis, and hypoxia.2 The congenital heart defects that classically present with cyanosis commonly are referred to as "the terrible Ts" and are listed in Table 1. Of these defects, tetralogy of fallot, tricuspid atresia, and pulmonary stenosis are associated with a decrease in pulmonary blood flow; whereas total anomalous pulmonary venous return, transposition of the great vessels, and truncus arteriosus are associated with an increase in pulmonary blood flow.
Table 1. Causes of Cyanotic Heart Disease
Presenting with Increased or Decreased
Pulmonary Blood Flow
The immediate goal in evaluation of a cyanotic neonate is to differentiate between cardiac and noncardiac etiologies. The hyperoxia test classically is done by obtaining an arterial blood gas (ABG) measurement, then placing the patient on 100% oxygen for 10 minutes, then repeating the ABG measurement. If the cause of cyanosis is pulmonary, the PaO2 level should increase by 30 torr; if the cause is cardiac, there should be minimal improvement in the PaO2 level. The initial ABG measurement should be obtained with co-oximetry because methemoglobinemia also may cause cyanosis in the neonatal period. A more simple method to complete the hyperoxia test is to provide 100% oxygen and observe the oxygen saturation level on pulse oximetry for an increase of 10% in pulmonary etiologies and minimal change with cyanotic heart disease. A chest radiograph and electrocardiogram (ECG) also should be obtained, but usually are not specific for the diagnosis of congenital heart disease. Although routinely not available in the ED, an echocardiogram is diagnostic. (See Figure 1 for diagrams of the cardiac lesions.)
If the neonate fails to show improved oxygen saturation or PaO2 levels, then a ductal dependent cyanotic heart lesion should be suspected. Prostaglandin E1 (PGE1) should be administered as a bolus of 0.05 mcg/kg IV followed by an infusion of 0.05-0.1 mcg/kg/min IV.3 Age-appropriate airway equipment should be available immediately prior to starting PGE1; a non-dose dependent side effect of PGE1 is apnea that requires intubation and mechanical ventilation. It may take 10-15 minutes for a response to PGE1, and its effect can be recognized by an increase in oxygen saturation level. Supplemental oxygen should be weaned as soon as possible after the patient’s oxygen saturation level has improved. After the patient is stabilized, he should be transported to an appropriate facility for pediatric cardiology and pediatric cardiovascular surgery consultation.
Acyanotic Heart Disease. Clinical decompensation in acyanotic heart diseases also may be a result of DA closure, but the onset is usually gradual with symptoms of congestive heart failure (CHF). Different degrees of obstruction to the left-ventricular outflow tract are present that result in an increase in pulmonary blood flow and a gradual development of heart failure. The classic triad of symptoms for pediatric congestive heart failure is tachypnea, tachycardia, and hepatomegaly. In addition, there may be a history of poor feeding, sweating, color change with feedings, or poor weight gain. Lower extremity edema and jugular venous distention are unlikely findings at this age. The more common causes of acyanotic heart disease are listed in Table 2 and the causes of CHF are listed in Table 3.
Table 2. Causes of Acyanotic
Heart Disease that Present
in the First Month of Life
Table 3. Differential Diagnosis
of Congestive Heart Failure in Neonates
The diagnostic evaluation should include a chest radiograph, ECG, and serum electrolyte levels measurement. Initial management, in addition to airway and breathing support, may include administration of PGE1, but success is less likely because the development of heart failure is gradual and the DA may have been closed already for several days to weeks. The first line in pharmacologic management of congestive heart failure is administration of furosemide 1 mg/kg IV, but other adjuvants include dopamine, dobutamine, and digoxin. Pediatric cardiology consultation should be obtained, and once stabilized the patient should be transported to the appropriate facility.
Dysrhythmias. Supraventricular tachycardia (SVT) in the neonate is defined by a heart rate greater than or equal to 220 bpm. This is the most common symptomatic dysrhythmia in the neonatal period. Presenting symptoms may range from poor feeding and irritability to congestive heart failure and shock. Initial vital signs will reveal the tachycardia. If the patient is stable, then vagal maneuvers may be attempted with ice in a small bag to the neonate’s face with care to avoid obstructing respirations. Other options (e.g., immersing the face in ice water and carotid massage) are not recommended at this age. If these vagal maneuvers are not successful, then adenosine 0.1 mg/kg IV push (maximum of 6 mg IV) may be given; if unresponsive, a second dose of 0.2 mg/kg IV push (maximum of 12 mg IV) may be given. IV access should be as close to the central circulation as possible as adenosine has a very short half-life, and IV adenosine given through an IV in the foot may not be successful. If there is no immediate IV access in the unstable patient, then synchronized cardioversion with 0.5-1.0 joules/kg is recommended.4 However, if the patient has IV access, then a trial of adenosine prior to cardioversion is appropriate. If time allows, a 12-lead ECG should be obtained before, during, and after correction of SVT. The ECG is helpful diagnostically for the pediatric cardiologist, and consultation with a pediatric cardiologist is recommended with arrangement for transportation to the appropriate facility.
The Respiratory System
Respiratory distress is a common presenting symptom with a broad differential diagnosis in the neonatal period. Although the underlying etiology may be pulmonary, other considerations include cardiac disorders, sepsis, meningitis, severe dehydration, metabolic or endocrine abnormalities, gastrointestinal (GI) emergencies, and upper airway abnormalities (e.g., choanal atresia or a hemangioma). The most common cause of cardiac arrest in pediatrics is respiratory failure, therefore, close attention to the respiratory status of the patient is extremely important. As hypoxia develops, the circulatory system responds by a decrease in heart rate (bradycardia) that eventually results in cardiac failure. Initial management is stabilization of the airway, breathing, and circulation (ABCs) followed by evaluation with a chest radiograph, ABG measurement if necessary, and then further laboratory evaluation depending upon the clinical picture.
Bronchiolitis. Bronchiolitis is a respiratory infection caused by several viruses including respiratory syncytial virus (RSV), influenza, parainfluenza, and adenovirus. RSV can present during the neonatal period as mild upper respiratory symptoms, fever, significant respiratory distress, or apnea. Although the neonate is a high-risk group that usually requires admission to the hospital for RSV infections, certain variables (e.g., prematurity, chronic lung disease or bronchopulmonary dysplasia (BPD), and underlying cardiac or pulmonary diseases) contribute to a more severe disease course.5 Currently, infants who were born at more than 32 weeks gestation or who have BPD should receive immune prophylaxis with the monoclonal antibody palivizumab on a monthly basis during the winter months.6 The management is dependent upon the presentation but focuses on the ABCs. Occasionally, neonates with apnea require intubation if the episodes are frequent and require intervention to correct. Nebulized racemic epinephrine may improve respiratory distress and wheezing, but has not been shown to decrease length of stay in the hospital. If there is no noticeable improvement of symptoms within 30 minutes after a nebulizer treatment with racemic epinephrine, then it should be discontinued.7 Although nebulized albuterol often is used and may have some short-term benefit to respiratory symptoms, it also has not been shown to decrease hospitalizations or improve oxygen saturation.8 In addition, the use of corticosteroids has not been shown to change outcome in large studies and remains a controversial issue.9 The work-up is dependent upon the clinical picture but should include a chest radiograph and nasopharyngeal aspirate for diagnosis of RSV bronchiolitis. Neonates who are RSV positive or have respiratory distress or apnea should be admitted to the hospital for observation and monitoring.
Bronchopulmonary Dysplasia (BPD). BPD is a common disease of the premature or very low birth weight neonate that results from lung immaturity and exposure to oxygen and often mechanical ventilation. To establish a baseline, it is important to get a thorough history from the family. These infants often are using oxygen at home and are using bronchodilators, steroids, diuretics, and antihypertensive medications. In addition to the chronic lung disease, this population has a higher incidence of bronchomalacia and tracheomalacia, which makes them high risk for deterioration with a respiratory illness. Death is usually a result of chronic respiratory failure, airway obstruction, apnea, pneumonia, pulmonary hypertension, or cor pulmonale.10 Management includes evaluation of the ABCs (including a tracheostomy if present) and laboratory tests as dictated by the presentation. These infants often are re-hospitalized and may need a higher level of pediatric care in an intensive care unit.10
Congenital Malformations. Occasionally, the cause of the respiratory distress is the result of a congenital malformation. Symptoms of respiratory distress may not develop until after discharge from the newborn nursery or with the first exposure to a respiratory virus. Included in the differential diagnosis are diaphragmatic hernia, tracheoesophageal fistula, cystic adenomatous malformation, and congenital lobar emphysema. A chest radiograph may be diagnostic with a radiolucent area visible in congenital lobar emphysema, cystic adenomatous malformation, and diaphragmatic hernias.11 All malformations presenting with respiratory distress should be approached by stabilization of the ABCs, appropriate consultation with a pediatric surgeon, and transfer to an appropriate facility.
The Neurologic System
Symptoms that indicate an insult or abnormality of the central nervous system (CNS) can be difficult to recognize. The neonate initially may have none or subtle findings (e.g., vomiting, poor feeding, and a decrease in activity), or may present with different degrees of altered mental status. It often requires a high index of suspicion to pick-up these nonspecific historical and physical exam findings. A useful mnemonic to aid with the differential diagnosis of altered mental status is THE MISFITS (Table 4).12
Table 4. Causes of Altered Mental Status
in a Neonate: The MISFITS
Non-accidental Head Trauma (Shaken Baby Syndrome). In 1974, a landmark article by Caffee introduced the concept of the "whiplash shaken baby syndrome."13 (See Figure 2.) This term has evolved, and now is referred to as "shaken baby syndrome" (SBS). The long-term morbidity rate from SBS may be as high as 70%, with a mortality rate as high as 30%.14,15 These infants may have no external signs of trauma. However, a recent study evaluating head trauma in children found that a significant scalp hematoma was associated with an increase risk of intracranial hemorrhage.16 One study found that 31% of abused infants had been seen by a physician after the injury, but the diagnosis of abuse was not recognized.17
Figure 2. Shaken Baby Syndrome
Initial evaluation and management may require stabilization of the ABCs, followed by a close examination looking for any signs of trauma. An initial evaluation of the retina may demonstrate retinal hemorrhages, but the ED is a difficult setting for full visualization of the retina. A recent study demonstrated a 13% false-negative rate by nonopthalmologists in recognizing retinal hemorrhages.18 Laboratory evaluation should be directed by the clinical presentation, but should include a CBC with platelets, PT, and PTT measurements. Suspicion of an infectious etiology requires prompt administration of broad spectrum antibiotics (See section on sepsis). Once stabilized, a computerized tomography (CT) scan of the head should be performed. In addition, a skeletal survey should be completed during the admission to look for new and old fractures. The appropriate child protective agency should be notified when appropriate, and the infant should be admitted or transferred to an appropriate facility.
Seizures. Seizures during the neonatal period may be difficult to recognize due to the immature cortical development. Generalized motor activity is less common; symptoms such as apnea, lip smacking, or abnormal eye movements may be the only indication of seizure activity. Any activity that is suspicious for seizures should prompt a thorough evaluation because seizures are rarely idiopathic during this time period.19 The differential diagnosis of neonatal seizures is listed in Table 5.
Table 5. Etiologies for Neonatal Seizures
Initial management includes stabilization of the ABCs followed by a bedside blood glucose assessment. Hypoglycemia should be corrected with 2-4 cc/kg IV of a 10% dextrose solution.3 Additional blood work should include a serum electrolyte measurement and cultures depending upon the concern for an infectious etiology.
Benzodiazepines are the first line in pharmacologic management, followed by phenobarbital and phenytoin or fosphenytoin. If the seizure still is unresponsive, then consider the use of lidocaine and pyridoxine. (See Table 6 for a list of medications and doses). Other electrolyte abnormalities also should be corrected. Hypocalcemia (< 7 mg/dL) may be corrected with 100-300 mg/kg IV of 10% calcium gluconate and hyponatremia (< 125 mg/dL) with 5-7 cc/kg IV of a 3% saline solution.3 Hyponatremia may be caused by formula overdilution due to improper formula-mixing practices. One study found that 70% of seizures in children younger than 6 months of age without other clinical findings were due to hyponatremia.20 Once the patient is stable, a head CT scan should be completed also. All neonates with seizures should be admitted to the hospital for observation and further evaluation.
Table 6. Pharmaceutical Management
of Neonatal Seizures
Bacterial meningitis also should be considered in the differential diagnosis of neonatal seizures, and broad spectrum antibiotics should be administered. In addition, neonatal herpes also may present with seizures, therefore, the addition of acyclovir 20 mg/kg IV should be considered.21 Both antibiotic and antiviral medications should be started—even if the patient has not been stabilized—before performing a lumbar puncture (LP).
Apnea/Apparent Life-Threatening Event (ALTE). Apnea is a pause in respirations for 20 seconds or longer, or any pause accompanied by bradycardia, pallor, or cyanosis.22 Apparent life-threatening event (ALTE) is a term used to describe an incident that may or may not be true apnea, but is anxiety provoking and fearful for the observers. Examples include choking, reflux, or coughing with a facial color change to red, change in muscular tone, or simply periodic breathing.23 ALTEs have been reported to occur in up to 3% of all children.24 The mortality of patients with apnea of infancy varies in the literature from 2-6%, but infants presenting with apnea during sleep may have up to 10% mortality with the risk of death tripling with two or more recurrences. In more than 50% of ALTEs, no definitive cause can be found.24 Premature infants are at higher risk for apnea and often are discharged from the hospital on apnea monitors and respiratory stimulant medications such as caffeine or theophylline.
Sudden infant death syndrome (SIDS) occurs in about two per 1,000 live births in the United States. SIDS has been defined as the sudden death of an infant younger than 1 year that remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene and review of clinical history.25
The National Institute of Child Health and Development Cooperative Epidemiological Study of SIDS found that only 2-4% of cases had a hospital record of apnea of prematurity, and less than 7% had a history of an ALTE.
Prone sleeping has been found to be a significant risk factor for SIDS. However, even with the implementation of infant supine sleeping campaigns, SIDS still occurs twice as often in African Americans as in Caucasians.26
It may be difficult to obtain a thorough history from an upset observer, but recent upper respiratory symptoms may suggest RSV bronchiolitis as the cause of apnea. The differential diagnosis (Table 7) of an ALTE is broad, and the work-up should include a full sepsis evaluation with administration of IV antibiotics. An ECG also should be obtained to rule out prolonged QT syndrome. The remainder of the evaluation may be tailored to the patient’s presentation and available history. The patient should be admitted for monitoring and observation regardless of how well the baby looks at the time of evaluation.
Table 7. Common Differential
Diagnoses
of Acute Life-
Threatening Events (ALTEs)
The Immune System
Although the evaluation of a patient with fever is controversial at different ages, it remains very clear in those patients younger than or equal to 28 days old. A rectal temperature of 100.4° F is considered a fever in this age group.27 Neonates are by definition immunocompromised patients because they have not developed antibodies to the more common pathogens and have received no immunizations. Delivery through the birth canal exposes the newborn to many pathogens. The most common bacterial organisms usually are colonizing the mother, and therefore, she may not have symptoms. These bacteria include Group B streptococcus (GBS), Escherichia coli, and Listeria monocytogenes. It is important to get a thorough birth history from the mother, including GBS colonization and fever during the delivery. Because of this innate immunocompromised state, each neonate deserves a thorough evaluation.
Sepsis. Fever is a common symptom that prompts a visit to the ED, but is only one of many symptoms that should increase the suspicion of sepsis. Overbundling alone does not raise a rectal temperature into the febrile range, although if the child is overbundled and in a hot car, that condition may raise the infant’s core temperature. Early-onset sepsis usually is seen in the first few days of life and typically is associated with maternal fever, or premature rupture of the membranes. These children classically present with septic shock and neutropenia. Late-onset sepsis occurs after the first week of life, usually is less associated with perinatal risk factors and more likely associated with meningitis. Other common symptoms that may warrant a full sepsis evaluation include hypothermia, poor feeding, a decrease in activity, altered mental status, vomiting, apnea, respiratory distress, irritability, jaundice, rashes, and seizures. Because the initial symptoms frequently are subtle, the ED physician should have a high index of suspicion for sepsis.
A full sepsis work-up should include a CBC, blood culture, urinalysis, urine culture by catheterization or suprapubic tap, chest radiograph, and cerebral spinal fluid (CSF) for cell count, gram stain, and culture (bacterial and viral if indicated), and glucose and protein levels measurement. Hypothermia should be addressed by placing the infant in an infant warmer. Antibiotics should be given as soon as possible; do not wait for the results of the work-up to decide the management.28 Each neonate should be treated with ampicillin 50 mg/kg/dose IV and gentamycin 2.5 mg/kg/dose IV or cefotaxime 50-100 mg/kg IV and admitted to the hospital for observation and culture growth tests. If there is a maternal history or clinical suspicion of herpes, then acyclovir also should be administered.21
Ill-appearing neonates or those with a positive urinalysis, CBC, or cerebrospinal fluid test result are more likely to have a bacterial etiology for the fever. Well-appearing neonates still have a rate of occult bacterial infections that may be as high as 15%.29The Rochester criteria do not apply well to infants younger than 28 days and should not be used in this group of patients.
Omphalitis or cellulitis. A thorough physical exam is just as important as the history and may reveal a source that would change the therapy. For example, an umbilicus with surrounding erythema is suggestive of omphalitis (Figure 3), and the antibiotic coverage should be expanded to include nafcillin 50 mg/kg IV to cover Staphylococcus, which is usually the etiologic agent.21 If there is concern of spread to the peritoneum, then surgical consultation is recommended. The condition should be differentiated from a normal umbilicus or an umbilicle granuloma. Also, the scalp should be inspected because the site from a fetal scalp monitor also may be a source of infection. Regardless of whether an obvious source is present, the full sepsis evaluation should be completed because the presence of one infection does not exclude other infections (e.g., bacteremia, meningitis, or a urinary tract infection).
Figure 3. Omphalitis
The Endocrine System and Genetic Abnormalities
Congenital Adrenal Hyperplasia (CAH). Most neonates with CAH are diagnosed with the state’s newborn screening. In addition, females are recognized early more easily because they typically have ambiguous genitalia. This disorder is most commonly the result of a deficiency in the 21-hydroxylase enzyme.30 The ED physician should be familiar with CAH because patients who go unrecognized may present to the ED in the first 2 weeks of life with shock. The presence of the electrolyte disturbance of hyponatremia and hyperkalemia should increase the suspicion of CAH as the diagnosis. Hypoglycemia is also a common finding in CAH. The hypotension usually is unresponsive to fluids or inotropes because of an overall adrenal insufficiency and requires treatment with steroids (hydrocortisone 25-50 mg/m2 IV). Once stabilized, the patient should be admitted to the appropriate facility.30
Thyrotoxicosis. Neonatal thyrotoxicosis is uncommon, but it occurs in infants born to mothers with Graves disease. The majority of patients are symptomatic at birth and are diagnosed in the newborn nursery, but occasionally there will be delayed onset and they present after discharge. Symptoms include tachycardia, jitteriness, irritability, poor feeding, respiratory distress, hyperthermia, congestive heart failure, and a goiter. Although the symptoms are usually transient, treatment is required with propranolol (1 mg/kg/24 hours in 4 divided doses) and may be titrated to decrease the tachycardia. Propylthiouracil (PTU) (5 mg/kg/24 hours in 4 divided doses) also may be started to help control the hypermetabolic state by inhibiting peripheral conversion of T4 to T3. PTU should be followed by potassium iodide (Lugol’s solution) 1-5 drops orally every 8 hours, which inhibits thyroid hormone release. Corticosteroids also may be helpful in cases that are refractory.31 These patients require admission for observation, pediatric endocrinology consultation, and titration of medications in a monitored setting.
Inborn Errors of Metabolism. Although the diagnosis of an inborn error of metabolism usually is not made in the ED setting, having a high index of suspicion when there is an acute decompensation in a patient and intervening early may affect the long-term morbidity of the neonate. There is a correlation between both the level of hyperammonemia and the length of time it has been elevated and the prognosis.32 The presenting symptoms may be nonspecific but include hypotonia/hypertonia, hypoglycemia, acidosis, seizures, lethargy, coma, body odor, vomiting, and irritability. Laboratory evaluation should include measurement of serum electrolytes, pH, ammonia, lactate, amino acids, and a bedside blood glucose measurement. More than 30% of patients with inborn errors of metabolism present with hyperammonemia.33 In addition, urinesamples should be obtained to check for reducing substances, amino acids, and organic acids. These results may not be available to the ED physician, but they are important to obtain during the acute presentation. Management should include discontinuing all feedings, fluid resuscitation, and consultation with a pediatric geneticist. The diagnostic pathway for inborn errors with both normal or elevated ammonia levels is described in Figure 4. If an inborn error is suspected, then the patient should be admitted for further evaluation.
Figure 4. Diagnostic Pathway for Inborn Errors with Normal
or Elevated Ammonia Levels
The Gastrointestinal System
Regardless of the presenting symptoms or history, any neonate with bilious emesis always is considered a surgical emergency.
Malrotation with Mid-gut Volvulus. Malrotation occurs in 1/500 live births, and volvulus develops in 68-71% of these patients. Although patients may present outside the neonatal period, 50% are diagnosed in the first month of life.34 Symptoms include altered mental status, bilious emesis, abdominal distention, and shock. An upper gastrointestinal contrast series is the preferred diagnostic study with a reported sensitivity of 95% (18/19) and an accuracy of 86% (18/21). Appropriate attention to airway and breathing, in addition to adequate fluid resuscitation, is important while awaiting immediate pediatric surgical consultation. If peritonitis is suspected, then antibiotics also should be initiated.
Necrotizing Enterocolitis (NEC). NEC occurs more commonly in premature infants, but also may present in a full term, previously healthy newborn. Symptoms include lethargy, irritability, feeding intolerance, hematochezia, and abdominal distention. The classical radiographic finding on plain films of the abdomen is pneumatosis intestinalis. Management includes fluid resuscitation, correction of electrolyte levels, broad spectrum antibiotic administration, and surgical consultation with admission to the hospital.35
Toxic Megacolon or Hirschprung Enterocolitis. Hirschprung disease results from a failure of neural crest cells to migrate in the colon resulting in an aganglionic segment of bowel; it occurs in 1/5,000 live births. While there is no ethnic predilection, the male to female ratio is 4:1. The history may include constipation or a delay of initial meconium stool passage of more than 24 hours after birth. When the disease is complicated by enterocolitis, the symptoms are similar to a presentation with NEC, and management also includes fluid resuscitation, electrolyte levels correction, broad spectrum antibiotic administration, and a pediatric surgery consultation.35,36
Hypertrophic Pyloric Stenosis. This condition occurs in 1/250 live births with a male to female ratio of 4:1. Classic symptoms include nonbilious, forceful emesis that eventually becomes projectile and occurs immediately after feeding. The presence of the classic electrolyte disturbance of hypochloremic, hypokalemic, metabolic acidosis is not useful because the diagnosis usually is made prior to developing these abnormalities. On physical exam, an olive’ in the epigastric area may be palpated, and peristaltic waves may be visible. If the patient has had nothing by mouth for 3-4 hours, a nasogastric tube may be placed to look for residual fluid. If there is more than 5 cc aspirate from the nasogastric tube, then the specificity will be 94% and the accuracy 96% for the diagnosis of pyloric stenosis.37 Definitive diagnosis is usually by ultrasound (Figure 5), and although the correction is usually surgical,34 a recent study found that 17 of 19 patients who were treated medically with atropine had resolution of the hypertrophic pyloric stenosis without surgical correction.38 Laparoscopic repair of patients with pyloric stenosis also has been used successfully.39
Figure 5. Pyloric Stenosis
The Hematologic System
Vitamin K Deficiency Bleeding/VKDB (previously hemorrhagic disease of the newborn). With an increase in out-of-hospital births from 5% in 1985 to 9% in 2000, there has been an increase in the number of newborns who may not receive vitamin K at birth.40 Vitamin K deficiency bleeding (VKDB) may present as early, classic, or late onset disease. Early onset is typically in the first 24 hours of life and is diagnosed in the newborn nursery. However, classic onset may present from 1-7 days of life with gastrointestinal bleeding, purpura, hematuria, and oozing from the umbilical stump. Recognition of these symptoms and awareness of this disorder will allow prompt correction with replacement of vitamin K (1 mg IM). Classic onset VKDB rarely causes intracranial hemorrhage. Late onset VKDB presenting between 2 weeks and 6 months of age can be catastrophic, with the more common sites of bleeding being intracranial, gastrointestinal, and cutaneous. Presenting symptoms are primarily neurologic and include seizures, coma, paralysis or poor feeding, and irritability. The mortality of late onset VKDB with intracranial hemorrhage is 33%. Therefore, a thorough birth history is important because early administration of vitamin K in the ED may improve the outcome.41-43
The differential diagnosis of bleeding in the neonatal period includes immune or alloimmune thrombocytopenia, hemophilia, disseminated intravascular coagulation (DIC), severe liver disease, congenital leukemia, marrow aplasia, and intrauterine infections. Regardless of the suspected etiology, each patient should have an initial evaluation that includes a CBC with platelets, PT, PTT, fibrinogen and fibrin-split products measurements, and head CT scan. The patient should be admitted or transferred to the appropriate pediatric critical care setting.
Hyperbilirubinemia (jaundice). Jaundice in the newborn period is a common presentation to the ED. The differential diagnosis for jaundice is dependent upon whether there is an increase in conjugated (direct) or unconjugated (indirect) bilirubin. Although unconjugated hyperbilirubinemia is usually benign and related to physiologic causes, breast feeding jaundice, or breast milk jaundice, there are more severe causes that should not be overlooked. Hemolytic disease of the newborn resulting from blood group (ABO) incompatibility often occurs in the first days of life and often is diagnosed prior to discharge from the hospital. Other etiologies of conjugated hyperbilirubinemia include sepsis, prematurity, G6PD, and polycythemia, Gilbert’s disease, and Crigler-Najjar syndrome. Conjugated hyperbilirubinemia usually is due to an underlying disorder such as biliary atresia, neonatal hepatitis, and alpha-1 antitrypsin deficiency.44 An evaluation should include hemoglobin and hematocrit measurements, total and direct bilirubin measurements, reticulocyte count, and Coombs test. If the neonate presents with an onset of jaundice after eight days of life, a urinalysis and urine culture should be completed because jaundice may be the only presenting symptom of a urinary tract infection.45 Appropriate consultation and admission for further evaluation is recommended.
Conclusion
This review discusses the critical clinical presentations that are representative of the neonatal period. This fragile population deserves special attention when they present to the ED. A thorough knowledge of newborn life-threatening illness, understanding of their specific management, and a systematic approach are imperative.
References
1. Millar KR, Gloor JE, Wellington N. Early neonatal presentations to the pediatric emergency department. Pediatr Emerg Care 2000; 16:145-150.
2. Hammerman C. Patent ductus arteriosus. Clinical relevance of prostaglandins and prostaglandin inhibitors in PDA pathophysiology and treatment. Clin Perinatol 1995;22:457-479.
3. Gunn VL, Nechyba C (eds). The Harriet Lane Handbook, 16th ed. St. Louis:Mosby;2002.
4. Zaritsky AL, Nadkarni VM, Hickey RW, et al, eds. PALS Provider Manual. Dallas, TX: American Academy of Pediatrics and American Heart Association; 2002:186-196. (Pediatric advanced life support course based on consensus by the AHA subcommittee on pediatric resuscitation)
5. Weisman LE. Populations at risk for developing respiratory syncytial virus and risk factors for respiratory syncytial virus severity: Infants with predisposing conditions. Pediatr Inf Dis J 2003;22:S33-S39.
6. American Academy of Pediatrics, Committee on Infectious Diseases and Committee on Fetus and Newborn. Prevention of respiratory syncytial virus infections: Indications for the use of palivizumab and update on the use of RSV-IGIV. Pediatrics 1998;102:1211-1216.
7. Wainwright C, Altamirano L, Cheney J, et al. A multicenter, randomized, double-blind, controlled trial of nebulized epinephrine in infants with acute bronchiolitis. NEJM 2003;349:27-35.
8. Kellner JD, Ohlsson A, et al. Bronchodilators for bronchiolitis. Cochrane Database Sytematic Review 2000;CD001:266.
9. Garrison MM, Christakis DA, Harvey E, et al. Systemic corticosteroids in infant bronchiolitis: A meta-analysis. Pediatrics 2000; 105: E4.
10. Bernstein S, Heimler R, Sasidharan P. Approaching the management of the neonatal intensive care unit graduate through history and physical examination. Pediatr Clin North Am 1998;45;79-105.
11. Devine PC, Malone FD. Non-cardiac thoracic abnormalities. Clin Perinatol 2000; 2:865-899.
12. McCollough M, Sharieff G. Common complaints in the first 30 days of life. Emerg Med Clin North Am 2002;20:27-48.
13. Caffee J. The whiplash shaken infant syndrome: Manual shaking by the extremities with whiplash-induced intracranial and intraocular bleedings, linked with residual permanent brain damage and mental retardation. Pediatrics 1974;54:369-403.
14. Jayawant S, Rawlinson A, Gibbon F, et al. Subdural haemorrhages in infants; population based study. BMJ 1998;317:1558-1561.
15. Haviland J, Russell RI. Outcome after severe non-accidental head injury. Arch Dis Child 1997;77:504-507.
16. Greenes D, et al. Clinical indicators of intracranial injury in head-injured infants. Pediatrics 1999;104:861-867.
17. Jenny C, Hymel KP, Ritzen A, et al. Analysis of missed cases of abusive head trauma. JAMA 1999;281:621-626.
18. Morad Y, Kim YM, Mian M, et al. Nonopthalmologist accuracy in diagnosising retinal hemorrhages in the shaken baby syndrome. J Pediatr 2003;142:431-434.
19. Evans D, Levene M. Neonatal seizures. Arch Dis Child Fetal Neonatol Ed 1998;78:F70-75.
20. Farrar HC, Chande VT, Fitzpatrick DF, et al. Hyponatremia as the cause of seizures in infants: A retrospective analysis of incindence, severity, and clinical predictors. Ann Emerg Med 1995;26:42-48.
21. Pickering LK, ed. Red Book 2003 Report of the Committee on Infectious Diseases. 26th ed. Elk Grove Village, IL: American Academy of Pediatrics;2003:347.
22. National Institutes of Health Consensus Development Conference on Infantile Apnea and Home Monitoring, Sept. 29 - Oct. 1, 1986. Pediatrics 1987;79:292.
23. Steinschneider A, Richmond C, Ramaswamy V, et al. Clinical characteristics of an apparent life-threatening event (ALTE) and the subsequent occurrence of prolonged apnea or prolonged bradycardia. Clin Pediatr 1998;37:223.
24. Brooks JG. Apparent life-threatening events. Pediatr Rev 1996; 17: 257.
25. Willinger M, James LS, Catz C. Defining the sudden infant death syndrome (SIDS): Deliberation of an expert panel convened by the National Institute of Child Health and Human Development. Pediatr Pathol 1991;11:677-684.
26. Hauck F, Moore C, Herman S, et al. The contribution of prone sleeping position to the racial disparity in sudden infant death syndrome: The Chicago Infant Mortality Study. Pediatrics 2002;110:772-780.
27. American College of Emergency Physicians. Clinical policy for the initial approach to children under the age of 2 years presenting with fever. Ann Emerg Med 1993;22:628-637.
28. Baraff L, Al E. Practice guidelines for the management of infants and children 0-36 months of age with fever without source. Pediatrics 1993;92:57-67.
29. Baker MD, Bell L: Unpredictability of serious bacterial illness in febrile infants from birth to 1 month of age. Arch Pediatr Adol Med 1999;153:508
30. Kabbani MD. Congenital adrenal hyperplasia: Epidemiology, management and practical drug treatment. Pediatr Drugs 2001;3: 599-611.
31. Karpman BA, Rappoport B, Filetti S, et al. Treatment of neonatal hyperthyroidism due to Graves’ disease with sodium ipodate. J Clin Endocrinol Metab 1987;64:119.
32. Harris GD, Perkin RM. Inborn errors of metabolism in infants: Recognition, diagnosis, and treatment. Pediatr Emerg Med Rep 2002;7:1-12.
33. Calvo M, Artuch R, Macia E, et al. Diagnostic approach to inborn errors of metabolism in an emergency unit. Pediatr Emerg Care 2000;16: 405-408.
34. Irish MS, Pearl RH, Caty MG, et al. The approach to common diagnoses in infants and children. Pediatr Clin North Am 1998;45: 729-772.
35. Pearl RH, Irish MS, Caty MG, et al. The approach to common abdominal diagnosis in infants and children. Part II. Pediatr Clin North Am 1998;45:1287-1323.
36. Swenson O. Hirshprung disease: A review. Pediatrics 2002;109: 914-917.
37. Mandell G, Wolfson P, Adkins E, et al. Cost-effective imaging approach to the nonbilious vomiting infant. Pediatrics 1999; 103:1198.
38. Kawahara H, Imura K, Nishikawa M, et al. Intravenous atropine treatment in infantile hypertrophic pyloric stenosis. Arch Dis Child 2002;87:71-74.
39. Najmaldin A, Tan HL. Early experience with laparoscopic pyloromyotomy for infantile hypertrophic pyloric stenosis. J Pediatr Surg l995;30:37.
40. Centers for Disease Control and Prevention. National Vital Statistics Report 2002;50:1-102.
41. Van Kries R., Geer F.R., Suttie JW, et al. Assessment of vitamin K status in the newborn infant. J Ped Gastro and Nutr 1993;16:231-238.
42. Bor O, Akgun N, Yakut A, et al. Late hemorrhagic disease of the newborn. Pediatr Int 2000;42:64-66.
43. Wetzel RC, Slater AJ, Dover GJ. Fatal intramuscular bleeding misdiagnosed as suspected nonaccidental injury. Pediatrics 1995;95: 771-773.
44. Agarwal R, Deorari AK. Unconjugated hyperbilirubinemia in newborns: Current perspective. Indian Pediatrics 2002;39:30-42.
45. Garcia FJ, Nager AL. Jaundice as an early diagnostic sign of urinary tract infection in infancy. Pediatrics 2002;109:846-851.
Infants younger than 28 days are challenging to the emergency department physician, who must be familiar with the normal variants and the subtle findings that may indicate a more serious underlying problem. The amazing physiologic changes that the newborn accomplishes following delivery may precipitate disaster if an underlying congenital problem exists. The astute ED physician is also familiar with particular high-risk times when critical diseases may present, such as cyanotic congenital heart disease, and has a heightened suspicion for these disease processes. This article provides a comprehensive overview of critical diseases that may present in the newborn period and strategies for their early detection and appropriate management.
Subscribe Now for Access
You have reached your article limit for the month. We hope you found our articles both enjoyable and insightful. For information on new subscriptions, product trials, alternative billing arrangements or group and site discounts please call 800-688-2421. We look forward to having you as a long-term member of the Relias Media community.