Approach to the Ill-Appearing Newborn in the Pediatric Emergency Department

Yaron Ivan, MD
Director of Resident Education, Pediatric Emergency Medicine, Pediatrix
PEM Site Director UNLVSOM, Pediatric Residency

Child Abuse Medical Director
Sunrise Children's Hospital Emergency Department
3186 S Maryland Pkwy, Las Vegas, NV 89109

Cristina M. Zeretzke-Bien, MD
Division of Emergency Medicine, University of Florida
Pediatric Emergency Medicine
Wolfson’s Children's Hospital, Pediatric Emergency Medicine
Florida College of Emergency Physicians Pediatric Committee, Chair Elect
American College of Emergency Physicians Immediate Past Chair, Pediatric Emergency Medicine Section

Ill-appearing newborns make physicians nervous. These patients tend to deteriorate fast, and often are difficult to evaluate due to a history that is limited by their age and a physical examination that is not always very contributory. During my pediatric emergency medicine (PEM) fellowship training, a systematic approach to those patients mostly based on mentor experience and teaching was useful.

Before considering the differential diagnosis and the initial evaluation I would highly advise taking note of the following two key points:

1. Include the parents by the bedside. In pediatric emergencies, having a parent by the bedside serves two purposes. First, parents witness firsthand that their child is receiving the best possible care and their proximity provides a ready source of medical history when more information is needed.
2. Be sure that an intraosseous (IO) needle is readily available. Intravenous (IV) access often is difficult in the newborn, especially in the setting of acute illness. The IO route often offers the most rapid access for delivery of medication or fluids in the emergent setting.

Five critical conditions to consider in the ill-appearing newborns are the following:

1. Sepsis
2. Congenital adrenal hyperplasia (CAH); adrenal insufficiency
3. Metabolic disease or inborn errors of metabolism (IEM)
4. Congenital heart disease (CHD)
5. Non-accidental trauma (NAT)

After obtaining the history and performing the physical examination, which includes the vital signs, to effectively diagnose and manage these conditions, Obtaining the following tests most often in the following order is recommended:

1. Point-of-care glucose
2. Point-of-care electrolytes
3. Chest x-ray
4. Electrocardiogram (EKG)
5. Computed tomography (CT) brain

With these few tests, the physician can usually get a sense of what the etiology is and perform the next appropriate intervention. The order of the tests above is important because it makes no sense to send a child to CT brain to see if the cause of ill appearance is intracranial bleeding without making sure the sodium, potassium and glucose levels are normal. Any condition the physician can fix should be checked first. A patient with hypoglycemia should not be in the radiology suite getting brain CT while his/her glucose level is abnormally low.

As a PEM physician, it is important to recognize that one cannot definitively care for the patient with intracranial hemorrhage secondary to NAT, or the patient with CHD while in the emergency department. Definitive treatment for those conditions occurs in the operating suite and, hence, requires consultation from surgical service.

To make things even simpler, in addition to considering these conditions in this order, ask the following two key questions.

• What will kill the patient now (or contribute to his/ her deterioration)?
• How do I fix it?

Sepsis

A newborn with sepsis (or concern of sepsis) usually presents with fever or hypothermia, so paying close attention to the vital signs is crucial. One must recognize when the patient presents with fever or hypothermia if the child is truly ill-appearing, local infection probably is not on the differential diagnosis. Under those circumstances, two critical interventions come to mind first.

The first is giving antibiotics early – providing ampicillin and cefotaxime usually covers most invasive bacterial infections in the neonatal period. One can consider ceftriaxone for the child over 21 days of age according to the new American Academy of Pediatrics (AAP) guidelines.

The second is treating septic shock, which includes maintaining cardiac output and peripheral perfusion by giving balanced crystalloid fluids early (up to 40 - 60 mL/kg). This is one of the most important distinctions between taking care of children vs adults. Keeping in mind that the “young healthy heart” can typically tolerate fluid boluses quite well. Bolus therapy given via “push-pull” is rarely wrong under these circumstances. Of course, boluses are titrated to clinical markers of cardiac output, so one must reassess the patient after every intervention.

Congenital Adrenal Insufficiency

This condition is more common than people realize, and its prevalence varies according to ethnicity and geographic area. Thinking about the actual problem comes down to the dysfunctional adrenal gland, which can lead to deficiency of cortisol and aldosterone. The lack of these two crucial hormones may lead to:

• Lack of sodium reabsorption (causing hyponatremia)
• Lack of potassium excretion (causing hyperkalemia)
• Lack of cortisol (leading to hypoglycemia)

Among these, hypoglycemia should be addressed promptly with a glucose bolus following the “Rule of 50.” Simply put, since the resuscitative dose of dextrose for hypoglycemia is 0.5 g/kg, the math works out that the dextrose concentration times the #mL/kg should equal 50. For example, when using D10W, the dose is 5 mL/kg (10x5=50).

Acute hyperkalemia and hyponatremia typically are treated with a 10 to 20 mL/kg normal saline bolus, which corrects the potassium and sodium derangements by:

1. Opposing the hypoperfusion and acidosis and the process of extracellular hydrogen molecules exchanged with potassium.
2. Diluting the extracellular potassium content
3. Correcting the hyponatremia (Normal saline contains 154 meq of Na per liter and usually increases the sodium level.)

Other interventions that come to mind for these children include the following:

• Hypertonic saline if the patient has symptomatic hyponatremia.
• EKG to rule out changes consistent with hyperkalemia and treat the patient appropriately if present.
• Hydrocortisone (typically 25 mg IV in the infant) to compensate for the cortisol deficiency.

Metabolic Disease or Inborn Errors of Metabolism

This is the most challenging diagnosis to make and manage. Simplifying the most important manifestations of inborn errors of metabolism (IEM) is helpful. Hypoglycemia and hyperammonemia are the most common metabolic derangements seen in the context of IEM.

Hypoglycemia is detected early when a point-of-care (POC) glucose measurement is made, and the treatment for hypoglycemia has been discussed.

The treatment for hyperammonemia is challenging in the pediatric emergency department and is focused on removal of the toxic ammonia with sodium phenyl acetate or sodium benzoate. Consultation with an expert in IEM and genetic diseases is critical.

Congenital Heart Disease

Congenital heart disease (CHD) may be difficult to diagnosis early in the evaluation of the ill-appearing infant. In my experience, an EKG and chest x-ray (CXR) (along with a careful physical examination) may help you rule in or rule out CHD. Commonly newborns with CHD will often be in congestive failure and present with moderate to severe tachypnea without respiratory findings that explain the rapid respiratory rate. Typically, infants with CHD and failure present with tachypnea of 60 to 100 breaths per minute without rhinorrhea, cough, or wheezing, and may even otherwise appear well. An EKG and CXR may substitute for a “poor man’s echo” before cardiology is consulted. The CXR may show vascular congestion and cardiomegaly, while the EKG may show evidence of left or right or biventricular strain. Early consultation of the cardiology team is crucial since there are several different congenital diagnoses that are diagnosable via the echocardiogram. As already mentioned, the definitive care of those children is often surgical, which is why the CXR and EKG are not done until the end.

When heart failure is evident on examination and a murmur is heard in the context of diminished or absent femoral pulses, critical coarctation of the aorta is likely. PGE1 (prostaglandin) is indicated in these circumstances that include sudden cardiovascular collapse. This is a treatment that a PEM physician can provide that can “fix” the problem, albeit temporarily.

Non-Accidental Trauma

The diagnosis of non-accidental (intentional) trauma in the infant is unfortunately common. Though most cases of child abuse present with a stable child who may have some bruising or minor injuries (that are not clinically significant but crucial to recognize to provide the child with a safe environment), some infants may present with poor feeding, persistent vomiting somnolence, irritability, or seizures. The most common cause for an abused child to be in extremis is intracranial hemorrhage secondary to head trauma. Hence, CT of the brain is on the list of tools for initial evaluation. As mentioned already, since the definitive management of an infant with intracranial bleeding may be surgical, the goal is to be sure there is no “medical problem” for the patient’s presentation. Medical management of those patients includes hypertonic saline or mannitol as treatment for increased intracranial pressure. Neurologists typically recommend levetiracetam or other antiepileptics for seizure prophylaxis in the context of abusive head trauma.

So next time you encounter an Ill-appearing neonate, remember the diagnosis and the above tests mentioned in the appropriate order to save a life.