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Evaluation and Management of the Critically Ill

Neonate in the Emergency Department


U. Olivia Kim, MD ⁎, David C. Brousseau, MD MS†, G. Ganesh Konduri, MD ⁎

The care of a critically ill neonate in the emergency department setting presents a special
challenge for practitioners who do not routinely encounter compromised neonates. This
review will provide guidelines for the initial stabilization of these infants as well as identify
differential diagnoses that should be considered. As with any critically ill patient, achieving
physiologic stability of the neonate is the first priority. However, in addition to maintaining
the airway, breathing, and circulation, thermoneutrality must be achieved for a successful
outcome. Obtaining the history and initiating diagnostic evaluations should occur during or
shortly after the course of stabilization to promptly initiate disease-specific therapies. The
mnemonic NEO SECRETS may help focus the care and promote early identification of the
cause of the infant’s deterioration.
Clin Ped Emerg Med 9:140-148 C 2008 Elsevier Inc. All rights reserved.

KEYWORDS neonatal emergency care, neonatal emergencies

R apid evaluation and stabilization of a critically ill


neonate is a required skill in the emergency depart-
ment (ED). Neonates can acutely decompensate from a
group B streptococcus [GBS] screens, and blood type)
medications, complications during pregnancy and maternal
illnesses. A birth history should follow and includes the
variety of causes. However, neonates have a limited gestational age at delivery, indication for delivery (maternal
repertoire of responses to stress and the presenting signs pregnancy induced hypertension, fetal distress, etc), the
can be nonspecific. Therefore, a logical and multisystem route of delivery including any instrumentation, complica-
approach to the evaluation and management of common tions during delivery, birth weight, and resuscitation
causes is essential to stabilizing these infants. The required. The immediate neonatal course should also be
following review summarizes our approach to a critically reviewed for difficulties in transitioning to extrauterine life
ill newborn in the setting of a busy ED. Although history, such as respiratory distress, hypoglycemia, feeding diffi-
physical examination, and laboratory evaluation are culties, jaundice, and length of hospital stay. The mother
described in sequence, it is understood that emergent may remember the infant's discharge weight and this
cardiopulmonary stabilization takes precedence over should be asked.
detailed evaluation of the neonate for underlying causes. The neonate's medical history consists of the time from
A rapid appraisal of the newborn for some of the more discharge from the birth hospital to the time of presenta-
common diagnoses is essential though, for institution of tion to the ED. Details on the infant's oral intake including
specific therapies. the amount of water used to mix formula or any additives
used should be obtained. The infant's urine output as well
as stooling habits should also be assessed. If available,
History ⁎Division of Neonatology, Department of Pediatrics, Medical College of
Initial evaluation of the critically ill neonate may be focused Wisconsin, Milwaukee, WI.
on immediate needs but should be orderly so as not to †Division of Emergency Medicine, Department of Pediatrics, Medical
overlook significant signs or symptoms. The elements of a College of Wisconsin, Milwaukee, WI.
Reprint requests and correspondence: U. Olivia Kim, MD, Children's
neonatal history should begin with a maternal history, such Corporate Center, Division of Neonatology, Suite C410, PO Box 1997,
as maternal antenatal laboratory tests (status of rubella, Milwaukee, WI 53201. (E-mails: okim@mcw.edu,dbrousse@mcw.edu,
hepatitis B, syphilis, human immunodeficiency virus, gkonduri@mcw.edu)

140 1522-8401/$ – see front matter C 2008 Elsevier Inc. All rights reserved.
doi:10.1016/j.cpem.2008.06.003
Evaluation and management of the critically ill neonate 141

information from the first well child check should be malformation. Fixed and dilated pupils with a persistent
obtained, either from the mother or the primary care oculocephalic response may indicate lidocaine toxicity,
provider's office. The neonate's examination and growth especially if there is a recent history of a circumcision or
parameters from the last examination should be reviewed other medical procedure. The mouth, neck, shoulders, and
and compared with the weight obtained in the ED. Results arms should be assessed. Brachial pulses should be
of the state mandated newborn screening test results may palpated and compared with femoral pulses to rule out a
be obtained from the primary physician's office, hospital of significant coarctation of the aorta. Examination of the
birth, or directly from the state newborn screening chest may reveal a pronounced cardiac murmur, unequal
program. These test results are important in the evaluation breath sounds, or significant subcostal retractions. A
of certain metabolic and endocrine causes of a neonate's distended, tender, and tympanic abdomen should prompt
acute illness as discussed later in this review. surgical consultation and a focused diagnostic search. The
genitalia should be inspected. Testicular torsion or
incarcerated inguinal hernias may be found. Ambiguous
Physical Examination genitalia in a female infant or a hyperpigmented scrotum in
The physical examination of the neonate begins with a male infant may suggest congenital adrenal hyperplasia
inspection and an overall observation of the neonate's (CAH). Bloody stools may suggest gastrointestinal necro-
condition. It is important to remember that the presenting sis. All extremities should be palpated to ensure that bony
symptoms and signs can be nonspecific and common to a structures are intact. A neurologic examination including
variety of diseases including sepsis, heart failure, and evaluation of the primitive neonatal reflexes (root, suck,
metabolic diseases. Therefore, it is important to consider a gag, Moro) should be done.
list of common disorders that present with immediate The history, physical examination, and initial stabiliza-
deterioration in the neonatal period. The overall appear- tion usually occur concurrently in a deteriorating infant
ance of the infant is often more significant in assessing the and may easily be interrupted. Hence, care must be taken
severity of the illness. A flexed posture with spontaneous to ensure a thorough physical examination is completed on
movements and pink perfusion is reassuring. A flaccid all critically ill infants.
posture with decreased spontaneous movements, cyanosis,
and respiratory distress signifies a gravely ill neonate in
need of immediate attention. Review of vital signs on Initial Stabilization
admission may also provide an indication of distress. As in all pediatric patients, stabilization prioritizes securing
Axillary temperatures for neonates vary widely but are the airway, then establishing breathing and maintaining
generally thought to be between 36.2°C and 37.7°C [1]. adequate circulation. Endotracheal intubation and fluid
Heart rates between 110 and 160 beats per minute are resuscitation are usually required in critically ill neonates,
normal for healthy term infants and may vary during sleep and these procedures, if indicated, should not be delayed
or active awake states [2]. Respiratory rates between 40 while waiting for diagnostic evaluation. Establishing
and 60 breaths per minute are also normal for a neonate optimal ventilation and oxygenation is often sufficient to
[3]. Normal systolic blood pressures during the first month improve both respiratory and cardiac insufficiency; how-
of life range from 65 to 75 mm Hg and diastolic pressures ever, intravenous (IV) fluids and resuscitation may be
from 40 to 50 mm Hg [4]. An easy rule of thumb is that the required in the gravely ill neonate. An initial bolus of
mean blood pressure should approximate the estimated 10 mL/kg of isotonic fluid (0.9 NS) should be given over 10
gestational age in weeks at the time of measurement. For to 15 minutes and repeated if necessary. If the umbilical
example, a 2 week old former 38 week gestation infant's stump is still present, this fluid may be given through an
mean blood pressure should be around 40 mm Hg. umbilical venous catheter [5].
Incorrect blood pressure readings may be obtained if the If the physical examination identifies poor peripheral
sphygmomanometer cuff is too small or large. Four perfusion and cyanosis with or without a cardiac murmur,
extremity blood pressures should be obtained on all a continuous infusion of prostaglandins (PGE1, 0.01-
hemodynamically unstable neonates because discrepancies 0.05 μg/kg per minute) should be promptly initiated and
between upper and lower limb blood pressures may pediatric cardiology consulted. If abdominal distension or
indicate a ductal dependent coarctation of the aorta. discoloration is noted on physical examination, a surgical
The remainder of the physical examination should abdomen must be considered. Bilious emesis and/or
follow and may suggest the source of illness. Inspection, abdominal distention in the neonate should be treated as
palpation, and auscultation of the infant should occur from a surgical emergency and should be promptly evaluated
head to toe. The head should be palpated and carefully with appropriate diagnostic tests and surgical consultation.
inspected for trauma in the encephalopathic neonate. The Neonates with enteric emergencies often require additional
fontanelle should be auscultated to assess for a cranial fluid resuscitation as well as decompression of the
bruit, especially if the neonate is hemodynamically gastrointestinal tract with a large bore repogle tube. Two-
unstable as this may be a sign of a cerebral arterio-venous view abdominal radiographs should be done to assess the
142 U.O. Kim et al.

bowel gas pattern and rule out perforation of a viscus. estimation and ionized calcium measurements should also
Pediatric surgery should be notified, and transfer to an be considered. Serum calcium, magnesium, phosphorus,
appropriate facility should be initiated if necessary. blood ammonia, lactate and pyruvate, and cerebrospinal
Measures must be taken to promote thermoneutrality fluid studies should be performed if the neonate demon-
because this is essential for successful stabilization of a strated central nervous system signs such as seizures and
critically ill neonate. In vivo thermoregulation is an active encephalopathy. Empiric use of IV broad-spectrum anti-
process that requires oxygen, glucose, corticosteroids, and biotics is usually initiated in the ill appearing neonate after
catecholamines [6]. A critically ill neonate who is hypoxic, the initial blood work is obtained.
hypoglycemic, or adrenally suppressed will be unable to
maintain a normal body temperature and will become
hypothermic. Prolonged hypothermia may result in cold Differential Diagnoses
stress that can affect all organ systems. The cold stressed Once the neonate has recovered from the initial decom-
infant may present with peripheral vasoconstriction to pensation, efforts can be directed toward determining the
maintain heat, respiratory distress to increase oxygenation, etiology of the infant's condition. Working through the
metabolic acidosis due to hypoglycemia, and anaerobic differential diagnosis in a critically ill neonate can be
metabolism if hypoxia persists, which further worsens the exhaustive and time-consuming. Commonly considered
respiratory distress [7]. This vicious cycle of hypothermia and causes for a neonate's catastrophic deterioration are
hypoxia will impede the response to resuscitation measures. infection, congenital heart disease, and metabolic disorders
For the cold infant, heat should be provided along with (Table 1). However, there are other etiologies for critical
initial stabilization measures. Warmth can be initiated with illness in the neonate that must be considered as well. The
a radiant warmer bed or overhead heat lamps. Although following mnemonic, “NEO SECRETS,” may help focus
other methods such as chemical heating pads have been the care and promote early identification of the cause of the
used, radiant heat is the most common and efficient in infant's deterioration (Table 2).
rewarming an infant [7]. In an ED setting, the radiant
warmer also allows unrestricted access to the neonate for Inborn Errors of Metabolism
medical procedures. However, its use in the cold infant Most neonates with inborn errors of metabolism become
must be done with caution. Rapid warming can result in symptomatic after oral feeds and ingestion of the
apnea, hypotension, and shock [8]. To prevent overheating offending agent; therefore, most present between days 2
of the neonate, use the servo-control option on the radiant to 7 of life and may be erroneously diagnosed as having
warmer, set the skin temperature to 36.5°C, and apply the sepsis. Early suspicion of an inborn error of metabolism is
skin probe to the anterior abdomen of the neonate. Oxygen essential for prompt diagnosis and the removal of the
consumption will increase as the infant's core temperature harmful metabolite.
returns to normal. If the infant remains hypoxic because of Affected neonates commonly demonstrate poor feeding
respiratory or cardiac insufficiencies, rewarming may with associated poor suck. Irritability, vomiting and failure
worsen tissue hypoxia. These complications must be to thrive, hepatosplenomegaly, jaundice, and abnormal
monitored for, and if seen, the rewarming process should
Table 1 Most common causes for catastrophic illness in the
be slowed by resetting the servo-control skin temperature
neonate.
to 1°C above the neonate's core temperature. Once the
neonate achieves that temperature, the servo skin tem- Ductal-Dependent
perature is increased by 1°C again until the neonate attains Congenital
a core temperature of 36.5°C. Care should be taken to Infectious Heart Disease
avoid hyperthermia in neonates with suspected hypoxic Diseases (Require PGE1) Metabolic Disorder
ischemic encephalopathy because elevated core tempera- GBS Left-sided lesions: Organic acidemias
ture can exacerbate neurologic injury in these cases. Gram-negative Coarctation of Hyperammonemias
bacilli the aorta
Herpes simplex Interrupted aortic Fatty acid oxidation
Laboratory Evaluation virus arch defects
Enterovirus Aortic stenosis
Laboratory evaluation of a critically ill neonate can be
Respiratory Hypoplastic left
directed toward a specific etiology if suspected. In absence
syncytial virus heart syndrome
of a clear etiology, laboratory samples are also frequently Right-sided lesions:
obtained to help determine diagnosis and management. Pulmonary atresia
Appropriate routine laboratory work in a critically ill or stenosis
neonate consists of a complete blood count with a Tetralogy of Fallot
differential, blood and urine cultures, urinalysis, electro- Tricuspid atresia
lytes including blood urea nitrogen and creatinine, For the purposes of this review article, this list is a brief outline of
glucose, and an arterial blood gas. Bedside blood glucose common diagnoses and is not meant to be comprehensive.
Evaluation and management of the critically ill neonate 143

Table 2 A mnemonic for the differential diagnoses of a all enteral feeds, initiation of IV fluids, and correction of
critically ill neonate: NEO SECRETS. associated acid-base and electrolyte abnormalities.
N iNborn errors of metabolism
E Electrolyte abnormalities
Electrolyte Abnormalities
O Overdose (toxin, poison) Electrolyte abnormalities in the critically ill neonate are
S Seizures commonly the result of an underlying process rather than
E Enteric emergencies the initiating factor. However, delayed management may
C Cardiac abnormalities hinder any resuscitative efforts and may contribute to the
R Recipe (formula, herbs, additives) neonate's ultimate demise. Water and electrolyte metabo-
E Endocrine crisis lism are closely associated, and the management of one
T Trauma may affect the other.
S Sepsis
Water balance in the neonate is primarily controlled by
antidiuretic hormone whose action results in the uptake of
urine odors may be found. If the disease progresses water from the distal renal tubules as well as the cortical and
unchecked, apnea, lethargy, a comatose state, and death medullary collecting ducts. This conservation of water is
may occur, and this deterioration can occur quickly [9]. triggered by contraction of the intravascular volume and/or
Metabolic acidosis, persistent hypoglycemia, or a sugges- an increase in the serum osmolality. However, the renal
tive family history should increase the suspicion for an response to antidiuretic hormone is diminished in neo-
inborn error of metabolism. Prompt consultation with a nates, thereby placing the critically ill neonate at high risk
neonatologist or medical specialist in genetics or inborn for imbalance [10]. Water requirements increase with
errors of metabolism is imperative. In addition to the initial advancing age. Neonates require 60 to 80 mL/kg per day in
stabilization blood work previously mentioned, directed the first 2 days of life, 100 to 150 mL/kg per day during days
laboratory evaluation includes serum amino acids, ammo- 2 through 7, and 120 to 180 mL/kg per day thereafter until
nia, pyruvate, lactate, urine organic acids, and urinalysis 28 days of life.
for ketones (Figure 1). If a confirmed serum ammonia level For the dehydrated neonate, fluid resuscitation requires
is more than 200 μmol/L, immediate transfer to an the replacement of the fluid deficit in addition to
intensive care unit with the ability to perform neonatal maintenance water requirements. The percentage of
dialysis must be arranged. dehydration is simply calculated as follows: fluid deficit
Specific treatments are indicated based on the final diagnosis (L) = pre-illness weight (kg) − illness weight (kg) [11]. For
and with the guidance of the medical specialist in genetics or the neonate, the birth weight, hospital discharge weight, or
inborn errors of metabolism. Immediate general measures will the 2-week office visit weight should be available for
include the elimination of the inciting metabolite by stopping comparison with the weight in the ED.

Figure 1 Approach to the neonate with a suspected inborn error of metabolism. FAO, fatty acid oxidation; RTA,
renal tubular acidosis.
144 U.O. Kim et al.

Sodium balance in the neonate is primarily determined causes include autosomal recessive polycystic kidney
by the nephron. The fractional excretion of sodium is disease, renal dysplasias, and renal tubular acidosis.
inversely proportional to gestational age. Although most These are usually accompanied by significant alterations
healthy term infants have a mature basal sodium balance in blood urea nitrogen and creatinine.
with a fractional excretion of sodium of less than 1%, The clinical signs and diagnostic evaluation of sodium
certain conditions, including hypoxia and respiratory and potassium abnormalities are shown in Table 3. The
distress, can result in higher urinary losses of sodium and management of these derangements is the same: calculate
sodium imbalance [12]. Sodium supplementation in the the deficit and replace the losses while avoiding rapid
first 24 hours of life is not necessary because hyponatremia correction. If the neonate is seizing because of hypona-
at this time is due to excess free water and not sodium tremia, 4 to 6 mL/kg of 3% NaCl IV may be given rapidly
deficiency. During the remainder of the first week, neonates without increased risk of central pontine myelinosis [13].
require 2 to 4 mEq/kg per day of sodium; this increases to 3 Hyperkalemia with electrocardiogram changes also
to 5 mEq/kg per day during the remainder of the first month requires additional measures: calcium gluconate 10% 100
of life. Similarly, potassium supplementation should be mg/kg IV over 3 to 5 minutes, NaHCO3 (1-2 mEq/kg IV
started on the second day of life. Potassium requirements in over 5-10 minutes), and insulin (0.1 U/kg/hr) IV infusion.
the first week of life are 1 to 2 mEq/kg per day and 2 to
3 mEq/kg per day for the remaining month of life. Overdose
Hyponatremia, hyperkalemia, and metabolic acidosis Neonatal toxicology is very different from pediatric
may also be caused by congenital renal anomalies that are toxicology. Accidental ingestion is common in toddlers
associated with progressive renal failure. The common but rare in neonates. Their immature pharmacokinetics

Table 3 Clinical presentation and diagnostic workup guidelines for common electrolyte abnormalities in a critically ill newborn.
Signs and Symptoms Diagnostic Workup
Hyponatremia (Na b130 mEq/L)
+
Edema If zK and shock, r/o CAH
Vomiting If AUOP and Aserum osmolality, r/o SIADH
Muscle weakness or renal defect
Seizures and/or coma If zUOP and dehydration, r/o improper mixing
of formula
Hypernatremia (Na+ >150 mEq/L) Lethargy or irritability If zUOP and Aurine specific gravity, r/o renal
Seizures decreased tone
Seizures, concentration defects
If AUOP and zspecific gravity, r/o breastfeeding
failure, improper formula mixing
Hypokalemia (K+ b3 mEq/L) Weakness If zurine K, r/o renal losses
Ileus If hypertensive, r/o excess mineralocorticoids
Hypertension or renin
ECG changes: zP wave amplitude, If normotensive, r/o RTA, diuretics,
inverted T waves GI losses or malnutrition
Hyperkalemia (K+ N 6 mEq/L) Weakness If Aurine K+ and z17-OHP, r/o CAH
Tetany If AUOP, r/o renal failure
ECG changes: peaked T waves, If zchloride and metabolic acidosis,
wide QRS, ST segment depression, V-fib r/o type IV RTA
R/o metabolic acidosis because this can
cause factitious hyperkalemia
Hypocalcemia (total serum Clonic seizures, jerking or tetany If AMg+ correct Mg first
Ca2+ b7 mg/dL, ionized Laryngospasm, stridor, weak cry If zserum Phos, r/o high Phos formula or cow’s
Ca2+ b1.1 mmol/L) Prolonged QT interval milk ingestion, APTH
If zserum Phos, zurine Ca, r/o renal failure
If APTH, APhos, zAP, r/o Vit D abnormality
If zPTH, APhos, zAP, r/o rickets
If no thymus on CXR, r/o DiGeorge syndrome
Hypoglycemia Lethargy Often symptom of other illness
(serum glucose b40 mg/dL) Seizures (sepsis, asphyxia, shock, metabolic disorder)
If zinsulin Ainsulin-like growth factor 1,
r/o hyperinsulinemia
r/o indicates rule out; UOP, urine output; SIADH, syndrome of inappropriate antidiuretic hormone; DTRs, deep tendon reflexes, RTA, renal tubular
acidosis; ECG, electrocardiogram; V-fib, ventricular fibrillation; GI, gastrointestinal; 17-OHP, 17-hydroxyprogesterone; Ca, calcium; Mg,
magnesium; Phos, phosphorus; PTH, parathyroid hormone; AP, alkaline phosphatase; Vit D, vitamin D; K, potassium; CAH, congenital adrenal
hyperplasia; Na, sodium; CXR, chest x-ray.
Evaluation and management of the critically ill neonate 145

makes them more susceptible to toxins. Unique neonatal metabolism is also regulated by several different hormones
features include increased skin surface area-to-body weight including glucagon and insulin. Surges of epinephrine and
ratio, immature hepatic and renal function resulting in norepinephrine after birth contribute to the mobilization of
impaired drug metabolism and excretion, and decreased hepatic glycogen [17], gluconeogenesis, and maintenance
plasma protein binding. All these factors allow drug of plasma glucose concentration. Hepatic glucose produc-
toxicity at lower doses [14,15]. The routes of neonatal tion depends on the amount of glycogen stores, gluconeo-
poisoning also differ from those of toddlers and include genic precursors, gluconeogenic and glycogenolytic
dermal exposure to toxins in addition to oral ingestion of systems, and an intact endocrine system to coordinate
contaminated breast milk. Dermal routes of exposure can these processes. Abnormalities in glucose metabolism
occur if rubbing alcohol (isopropyl alcohol or ethanol) is reflects either exposure to irregular maternal glucose
used for antiseptic cord care or in baths for fever reduction. metabolism or intrinsic metabolic problems in the neonate
Systemic absorption of large amounts of topical diphenhy- [18]. Large for gestational age, which may be associated
dramine may also occur. Oral overdoses of diphenhydra- with diabetes mellitus in the mother, and small for
mine have been reported in cases of caregivers giving the gestational age infants are at increased risk of hypoglycemia.
antihistamine to sedate a fussy infant. Homeopathic colic The clinical presentation and causes of hypocalcemia
remedies such as Gripe Water may contain sodium and hypoglycemia are detailed in Table 3. Similar to
bicarbonate and ethanol and can cause toxicity if given electrolyte abnormalities, treatment of hypocalcemia and
in large quantities [16]. hypoglycemia include supplementation of the deficient
Ingestion of contaminated breast milk is another route for agent. Symptomatic hypocalcemia, which may present as
toxin exposure for the neonate. Morphine, methyl mercury, seizures, requires an IV bolus of 100 to 200 mg/kg of 10%
and pesticides have all been implicated in cases of acute calcium gluconate followed by repeat boluses every
neonatal deterioration and a complete maternal history 6 hours and/or a continuous calcium infusion. After the
including medications as well as complementary alternative acute period, some neonates may need vitamin D
medicines should be obtained for breast-fed infants. supplementation and a low-phosphorus formula such as
Similac PM 60/40 (Mead Johnson, Evansville, Indiana).
Seizures Likewise, symptomatic hypoglycemia, which may also
Neonatal seizures commonly manifest as subtle motor present as seizures, requires a 2 mL/kg IV mini-bolus of
automatisms such as lip smacking, bicycling movements of D10W to rapidly return the blood glucose level to normal
the legs, tongue thrusting, apnea, and staring spells. The without overshooting the desired glucose concentration
classic tonic-clonic movements are not typical in neonates, [19]. This is then followed by a continuous glucose
which may lure caretakers to overlook this diagnosis. The infusion. Determination of the glucose infusion rate (GIR)
underlying etiology and treatment of the clinical seizures is determined by the following equation: GIR (mg/kg per
will dictate the outcome and ultimate neurologic develop- minute) = [IV rate (mL/h) × dextrose concentration (g/dL) ×
ment. Causes for neonatal seizures may include subar- 0.167] ÷ weight (kg). Most neonates will be euglycemic with
achnoid or subdural hemorrhages, intracranial infections, a GIR of 5 to 8 mg/kg per minute. Infants requiring a higher
electrolyte abnormalities, and drug withdrawal. Less GIR may have hyperinsulinemia. D25W boluses should
common causes are pyridoxine deficiency and local be avoided.
anesthetic toxicity. Congenital adrenal hyperplasia describes a group of
The diagnosis and management of seizures related to disorders with an inherited defect in an enzyme required
hyponatremia, hypocalcemia, and hypoglycemia are for the adrenocortical synthesis of cortisol from cholesterol
discussed in greater detail in the “Electrolytes” and [20]. The lack of glucocorticoids, and occasionally
“Endocrine” sections of this review. After electrolyte mineralocorticoids, in conjunction with the accumulation
abnormalities are corrected and seizures continue, of premetabolites (commonly testosterone) result in the
phenobarbital is the drug of choice, and 20 mg/kg IV clinical manifestations of the affected neonate. The most
should be given to halt seizure activity. common is 21-hydroxylase deficiency resulting in the
inability to convert progesterone to aldosterone or cortisol
and causing an accumulation of testosterone. Female
Endocrine Crisis infants classically present with ambiguous genitalia with
Endocrine emergencies such as hypoglycemia and hypo- varying degrees of virilization. However, a male infant may
calcemia are relatively common in the neonatal period. only have a hyperpigmented scrotum and no other
Other disorders such as CAH and thyrotoxicosis are not physical abnormalities and can only be recognized by
routinely encountered in the ED setting but must be appropriate laboratory evaluation and results of the
considered in critically ill neonates. Calcium metabolism is newborn screen if available. Affected infants classically
tightly regulated and involves the concerted effects of have severe salt-wasting and hyperkalemia requiring
parathyroid hormone, vitamin D, and calcitonin on the aggressive fluid resuscitation and exogenous glucocorti-
targeted organs: intestines, kidneys, and bone. Glucose coids and or mineralocorticoids. Some infants may be
146 U.O. Kim et al.

hypertensive. If CAH is suspected, early consultation with immediate evaluation by a pediatric cardiologist should be
a pediatric endocrinologist is recommended. requested. Lesions with pulmonary overcirculation (atrial
Another cause of neonatal endocrine emergencies is or ventricular septal defects, atrioventricular canal, truncus
thyrotoxicosis. Although rare, affected neonates can arteriosus, and partial anomalous pulmonary venous
present from birth to 6 weeks of age with tachycardia, return) can present with evidence of congestive heart
tremors, sweating, and irritability [21]. The most common failure and respiratory distress; however, the deterioration
cause of neonatal thyrotoxicosis is maternal Graves in these infants is not as dramatic as with ductal dependent
disease, which results in transplacental passage of thyr- lesions. Congestive heart failure may also be due to
oid-stimulating hormone receptor stimulating antibodies supraventricular tachycardia and is suspected when the
[22]. A complete history will also reveal the classic infant's heart rate is more than 220 bpm. A pediatric
complaint of failure to thrive despite hyperphagia [20]. If cardiologist should be consulted as soon as congenital
neonatal thyrotoxicosis is suspected, early consultation heart disease is suspected.
with a pediatric endocrinologist is recommended.
Recipe
Cardiac Abnormalities Incorrect mixing of formula may contribute to electrolyte
A decompensating neonate with a yet undiagnosed imbalances in the neonate ranging from severe hyperna-
congenital cardiac defect is perhaps the most daunting tremia (not enough water added) to severe hyponatremia
patient for many emergency physicians. Most physicians and water intoxication (not enough formula added) (see
remember the 5 classic cyanotic congenital heart defects: “Electrolyte Abnormalities”). A thorough history of the
truncus arteriosus, transposition of the great arteries, mixing of formula is essential.
tricuspid atresia, tetralogy of Fallot, and total anomalous The recent trend of complementary and alternative
pulmonary venous return. However, most of these medicine has promoted the use of “natural” foods
patients become symptomatic during the first days of including organic foods as well as the use of supplemental
life and are most often diagnosed within the immediate vitamins and homeopathic dietary additives. Nestle has
newborn period. released the first commercial infant formula with added
The emergency physician must be armed with a probiotics (bifidobacteria) called “Good Start Supreme®
differential diagnosis of cardiac lesions that present after with Natural Cultures” to “help support baby's healthy
the immediate newborn period to provide appropriate immune system.” Although studies have yet to show a
therapy for an affected neonate. Ductal dependent lesions significant adverse effect of the commercially added
usually present in the first 2 weeks of life with severe probiotics (Lactobacillus, Bifidobacterium) [23-25], con-
hemodynamic compromise. The ductus arteriosus is cern is raised regarding unregulated addition of probiotics
usually patent in these infants in the immediate newborn to expressed breast milk or formula by well-meaning care
period, and these infants can be asymptomatic at the time takers. Because these additives are considered dietary
of discharge from the birth hospital. They remain well- supplements, there is no requirement to demonstrate
appearing until they develop a dramatic hemodynamic safety, purity, or potency before marketing probiotics [26].
collapse as the ductus arteriosus begins to close. Hence,
neonates presenting with sudden appearance of shock Enteric Emergencies
require an immediate evaluation for ductal dependent Gastroschisis, omphalocele, tracheo-esophageal fistula,
congenital cardiac defects and should receive aggressive IV imperforate anus, meconium ileus, bowel atresias, and
fluid resuscitation and prostaglandins to maintain the significant congenital diaphragmatic hernias are often
patency of the ductus arteriosus while awaiting evaluation diagnosed and addressed shortly after birth. Hence, they
from the cardiac specialists. The ductal dependent lesions will not be included in the following discussion. Common
are usually of 2 types: left-sided and right-sided obstructive enteric emergencies in neonates who were otherwise
lesions. Left-sided obstructive lesions (aortic stenosis, asymptomatic at home are discussed below.
coarctation of the aorta, interrupted aortic arch, hypoplas- The suspicion of a surgical abdomen can be raised even
tic left heart syndrome) present with signs of severe with a limited history and physical exam. A history of
systemic hypoperfusion with pallor, mottling, decreased or feeding intolerance or bilious emesis with physical findings
absent pulses, severe metabolic acidosis, and cardiomegaly of a tender, distended abdomen with discoloration of the
with pulmonary congestion on chest radiograph (CXR). overlying skin should prompt immediate consultation with
Right-sided obstructive lesions (pulmonary atresia, severe a surgeon skilled in neonatal care.
pulmonary stenosis, tetralogy of Fallot, tricuspid atresia) A true surgical emergency is the neonate with
present with severe cyanosis, metabolic acidosis, and malrotation with or without midgut volvulus. A contrast
decreased perfusion of the lung fields on CXR. Prostaglan- study, usually an upper GI, is required to rule out
din infusion (PGE1 at a rate of 0.01-0.05 μg/kg per minute) malrotation. Visualization of a dilated duodenum,
should be immediately started when neonates present with abnormal positioned duodenojejunal junction with
these signs, with or without a cardiac murmur, and an right-sided jejunal loops, and a malpositioned cecum
Evaluation and management of the critically ill neonate 147

on a contrast study confirms malrotation of the intestines sepsis is a well-known cause of neonatal collapse. Common
[27]. If a “corkscrew” appearance of the jejunum is seen, microbes of concern are GBS, Listeria monocytogenes,
midgut volvulus is present. Time is of the essence Escherichia coli, and Staphylococcus aureus. Maternal
because intestinal necrosis can rapidly occur if there is a history of GBS status and penicillin prophylaxis at the
delay in making this diagnosis. Definitive surgical time of delivery may be helpful but late-onset GBS is
treatment consists of an exploratory laparotomy and commonly community acquired. It is important to note
Ladd's band procedure. that intrapartum and early neonatal antibiotic treatment
If an neonate is previously premature, is an infant of a does not prevent late-onset GBS meningitis, pneumonia, or
cocaine-using mother, or has a history of surgical closure sepsis. Maternal history of a cervical cerclage during
of an abdominal wall defect, necrotizing enterocolitis pregnancy may increase the risk for gram-negative sepsis
must be considered. Abdominal radiographs may reveal in the neonate, whereas a history of brown-colored
intestinal dilatation and thickened bowel walls. The amniotic fluid (which may be mistaken as meconium-
presence of pneumatosis intestinalis or portal venous stained fluid) may suggest Listeria.
gas is pathognomonic for necrotizing enterocolitis. If Prompt initiation of broad-spectrum IV antibiotics such
there is no free air or evidence of peritonitis, medical as ampicillin and gentamycin or ampicillin and cefotax-
management should consist of IV antibiotics and bowel ime is essential. Ampicillin is effective against GBS,
rest. If free air or peritonitis is found, surgical interven- Listeria, and S aureus, whereas gentamycin is effective
tion is indicated. against most gram-negative bacilli. If ampicillin and
Hirschsprung disease should be considered in the cefotaxime are used, remember that several strains of β-
neonate with a history of failure to pass meconium or lactamase–producing E coli have become resistant to
infrequent stools and abdominal distension. A rectal biopsy these drugs [29] and the threshold to add or change
is diagnostic and demonstrates the absence of ganglion cefotaxime to gentamycin in the neonate with suspected
cells. If there is rapid progression of abdominal distension gram-negative sepsis must be low.
and profuse vomiting, toxic megacolon must be assumed Viral infections include herpes simplex virus and
and emergent surgical treatment with a diverting colost- enterovirus and can also compromise a neonate. Dis-
omy may be necessary. seminated neonatal herpes simplex virus infection is
clinically indistinguishable from sepsis and should be
Trauma (After the Perinatal Period) considered in the differential of sepsis. A history of
Neonates are not immune from accidental injuries such as maternal genital lesions or respiratory symptoms may be
motor vehicle accidents, falls, and the antics of overzealous helpful but are often negative. If sepsis is highly
older siblings. Unfortunately, they may also be the victims suspected, consider a viral infection as well. In addition
of adult anger, frustration, and anxiety. An obtunded, to blood cultures, send surface cultures and blood or
encephalopathic neonate may have a metabolic disorder or cerebrospinal fluid for polymerase chain reaction assays
sepsis, but trauma must also be considered. Intracranial for the suspected virus. Acyclovir should be started in
pathology includes hemorrhage, edema, contusional tears, septic-appearing neonates with signs of encephalopathy
and diffuse axonal injury [28]. and/or disseminated intravascular coagulopathy. Because
Other organs can be involved in trauma. The muscu- pleconaril is no longer available for the treatment of
loskeletal system can also be affected as evidenced by neonates with enteroviral infections, supportive treatment
fractures of varying degrees, but bony injury alone is rarely must be maximized. A detailed review of neonatal sepsis
a cause of acute clinical deterioration of a neonate. is presented later in the article on “Evaluation and
However, if associated with a pneumothorax or liver management of a newborn with sepsis.”
laceration, emergent resuscitation may be required. Blunt
abdominal trauma can result in adrenal hemorrhage, Summary
which can lead to hypovolemia and shock. Hence, radio- Neonatal symptoms of illness are limited and nonspecific,
graphic imaging and ultrasonography play a crucial role in requiring the treating physician to be astute and complete
the evaluation of a critically ill neonate who has been the in the care and management of these patients. Regardless of
victim of a trauma. Pediatric neurosurgeons and pediatric the diagnosis, the management of the critically ill neonate
general surgeons should be consulted early in the manage- is still dependent on a rapid assessment and support of
ment of an injured neonate to help provide prompt surgical airway, breathing, and circulation. Although the history
intervention if needed and collaborate with the medical and physical examination may help narrow the differential
team in maximizing the infant's outcome. diagnosis, beginning resuscitation and ensuring thermo-
neutrality are essential for the successful management of
Sepsis any critically ill neonate. Laboratory evaluation can be
Sepsis is intentionally discussed last so as to give all other directed toward a specific diagnosis or can be broad
potential diagnoses careful consideration before attributing enough to allow for the identification of a diagnosis or
a neonate's deterioration to sepsis. Nonetheless, neonatal tracking changes in the status of the neonate. Although the
148 U.O. Kim et al.

differential diagnosis is extensive, a systematic approach to 13. Sarniak AP, Meert K, Hackbarth R, et al. Management of hypona-
initial resuscitation, followed by application of the “NEO tremic seizures in children with hypertonic saline: a safe and effective
strategy. Crit Care Med 1991;19:758-62.
SECRETS” mnemonic will allow the treating physician to
14. Mendenhall AK, Eichenfield LF. Back to basics: caring for the
be focused and able to implement time-efficient interven- newborn's skin. Contemp Pediatr 2001;17:98-114.
tions that will lead to the best possible outcome for the 15. Spray A, Siegfried E. Dermatologic toxicology in children. Pediatr
critically ill neonate. Ann 2001;30:197-202.
16. Blumenthal I. The gripe water story. J R Soc Med 2000;93:172-4.
17. Kawai Y, Arinze IJ. Activation of glycogenolysis in neonatal liver. J Biol
References Chem 1981;256:853-8.
1. Ludwig-Beyer P, Heuther P. Pain, temperature, sleep, and sensory 18. Kalhan SC, Parimi PS. Disorders of carbohydrate metabolism. In:
function. In: Huether S, McCance K, editors. Understanding Fanaroff AA, Martin RJ, editors. Neonatal-perinatal medicine: diseases
pathophysiology. St. Louis, MO: Mosby-Year Book; 1996. p. 319-45. of the fetus and infant. 7th ed., vol 2. Philadelphia, PA: Mosby; 2002.
2. Fletcher MA. General information. Physical diagnosis in neonatology. p. 1351-76.
Philadelphia, PA: Lippincott, Williams & Wilkins; 1998. p. 3-28. 19. McGowan JE. Neonatal hypoglycemia. Pediatr Rev 1999;20:e6-e15.
3. Southgate WM, Pittard III WB. Classification & physical examination 20. Levine LS. Congenital adrenal hyperplasia. Pediatr Rev 2000;21:
of the newborn. In: Klaus MH, Fanaroff AA, editors. Care of the high- 159-67.
risk neonate. 5th ed. Philadelphia, PA: Saunders; 2001. p. 117. 21. Foley Jr TP. Maternally transferred thyroid diseases in the infant:
4. Zubrow AB, Hulman S, Kushner H, et al. Determinants of blood recognition and treatment. In: Bercu BB, Shulman DI, editors.
pressure in infants admitted to neonatal intensive care units: a Advances in perinatal thyroidology. New York, NY: Plenum Press;
prospective multicenter study. Philadelphia Neonatal Blood Pressure 1991. p. 209-26.
Study Group. J Perinatol 1995;15:470. 22. LaFranchi S. Evaluation and management of neonatal Grave's disease;
5. Kattwinkel J, editor. Textbook of neonatal resuscitation. 5th ed. Elk 2008. Up to Date. Available at: http://patients.uptodate.com/topic.
Grove Village, IL: American Academy of Pediatrics; 2006. p. 6-4, 6-5. asp?file=pediendo/5108. Accessed 7-25-08.
6. Noerr B. Keeping the newborn warm: understanding thermoregula- 23. Puccio G, Cajozzo C, Meli F, et al. Clinical evaluation of a new starter
tion. Mother Baby J 1997;2:6-12. formula for infants containing live Bifidobacterium logum BL999 and
7. Hackman PS. Recognizing and understanding the cold-stressed term prebiotics. Nutrition 2007;23:1-8.
infant. Neonatal Netw 2001;20:35-41. 24. Saavedra JM, Abi-Hanna A, Moore N, et al. Long-term consumption of
8. Perlstein PH, Edwards NK, Sutherland JM. Apnea in premature infant formulas containing live probiotic bacteria: tolerance and
infants and incubator air temperature changes. N Engl J Med 1970; safety. Am J Clin Nutr 2004;79:261-7.
282:461. 25. Boehm G, Jelinek J, Stahl B, et al. Prebiotics in infant formulas. J Clin
9. Zinn AB. Inborn errors of metabolism. In: Fanaroff AA, Martin RJ, Gastroenterol 2004;38:s76-9.
editors. Neonatal-perinatal medicine: diseases of the fetus and infant. 26. Boyle RJ, Robins-Browne RM, Tang MLK. Probiotic use in clinical
7th ed., Vol. 2. Philadelphia, PA: Mosby; 2002. p. 1468-516. practice: what are the risks? Am J Clin Nutr 2000;83:1256-64.
10. McCance RA, Naylar NJ, Widdowson EM. The renal response of 27. Ryckman FC. Selected anomalies and intestinal obstruction. In:
infants to a large dose of water. Arch Dis Child 1954;29:104. Fanaroff AA, Martin RJ, editors. Neonatal-perinatal medicine: diseases
11. Stone B. Fluids and electrolytes. In: Robertson J, Shilkofski N, editors. of the fetus and infant. 7th ed., vol 2. Philadelphia, PA: Mosby; 2002.
The Harriet Lane handbook: a manual for pediatric house officers. p. 1276-307.
17th ed. Philadelphia, PA: Mosby; 2005. p. 285. 28. David TJ. Shaken baby (shaken impact) syndrome: non-accidental
12. Lorenz J, Kleinman LI, Ahmed G, et al. Phases of fluid and electrolyte head injury in infancy. J Royal Soc Med 1999;92:556-61.
homeostasis in the extremely low birth weight infant. Pediatrics 1995; 29. Paterson DL, Bonomo RA. Extended-spectrum b-lactamases: a clinical
96:484-9. update. Clin Microbiol Rev 2005;18:657-86.