Pediatric emergency medicine trisk 618

Temperature regulation can also be altered in infants with hypoxic–ischemic
injury. In patients presenting with hypoxia–ischemia (e.g., after cardiac arrest),
fever should be aggressively prevented because hyperthermia can worsen
neurologic injury.

COLOR CHANGES AND DERMATOLOGIC FINDINGS
Goals of Emergency Care
The goal is to recognize the difference between benign presentations, such as
acrocyanosis, and life-threatening conditions, such as true cyanosis and pallor.
KEY POINTS
Evaluation of jaundice requires assessment of both direct and indirect
bilirubin.
Acceptable levels of indirect hyperbilirubinemia depend on both
prematurity and postnatal age.
Many neonates with herpes simplex virus (HSV) infection have a
negative maternal history of HSV.
RELATED CHAPTERS
Signs and Symptoms
Cyanosis: Chapter 21
Jaundice: Conjugated Hyperbilirubinemia and Jaundice: Unconjugated
Hyperbilirubinemia: Chapters 44 and 45
Pallor: Chapter 62
Rash: Neonatal: Chapter 69
Septic-Appearing Infant: Chapter 73
Medical, Surgical, and Trauma Emergencies
Cardiac Emergencies: Chapter 86
Dermatologic Urgencies and Emergencies: Chapter 88
The Children’s Hospital of Philadelphia Clinical Pathways


ED Pathway for Evaluation/Treatment of Neonates

Hyperbilirubinemia/Jaundice
URL: />Authors: D. Aronson, MD; J. Lavelle, MD; C. Jacobstein, MD; N.
Boorstein, RN
Posted: October 2009, last revised January 2018

With

ED Clinical Pathway for Evaluation/Treatment of Febrile Young
Infants (0–56 Days Old)
URL: />Authors: R. Scarfone, MD; P. Gala, MD; A. Murray, MD; M.K. Funari,
RN; J. Lavelle, MD; L. Bell, MD; C. Jacobstein, MD
Posted: August 2010, last revised August 2019

Color Changes
Cyanosis
CLINICAL PEARLS AND PITFALLS
Cyanosis may not be visible toward the end of the neonatal period
because of physiologic anemia.
Benign acrocyanosis may involve the perioral region but spares the lips
and mucous membranes.
Diarrheal illness and/or dehydration can lead to acquired
methemoglobinemia.
Current Evidence. Cyanosis refers to a blue tone visible in the skin and mucous
membranes, caused by desaturated or abnormal hemoglobin. The human eye can
detect cyanosis when there is at least 5 g/dL of reduced hemoglobin. Therefore,
hemoglobin oxygen desaturation may be missed if there is anemia. Conversely,
abnormal hemoglobin may be saturated with oxygen yet unable to release to the
tissues, resulting in visible cyanosis. Methemoglobinemia is a classic example of
this situation. Central cyanosis is caused by deoxygenated blood entering the
systemic circulation. This is usually due to CHD, specifically cardiac defects

allowing systemic venous blood to bypass the lungs (right-to-left shunt), but
central cyanosis may also be caused by respiratory compromise or pharmacologic
agents. Acrocyanosis, the transient blue discoloration of the hands and feet in
response to vasomotor instability or a cool environment, is caused by
vasoconstriction of the small arterioles and does not reflect reduced systemic
arterial oxygenation. Mottling is the patchy-colored appearance of the body
surface, resulting from dilation of the superficial veins showing through the thin
neonatal skin.
Goals of Treatment. The goals of ED evaluation of the cyanotic infant include
early recognition of cardiorespiratory pathology or pharmacologic causes.
Clinical Considerations
Clinical Recognition. Proper lighting is important to assess cyanosis in neonates.
Location of cyanosis helps determine its cause. Cyanosis noted in the mucous
membranes, tongue, trunk, and extremities is central. In contrast, acrocyanosis is
limited to hands, feet, and perioral region, with the tongue and rest of skin
remaining pink. This condition may be associated with cool ambient temperature.
Acrocyanosis is benign and may resolve with warming. Local blue discoloration
of a single extremity could be the result of compromised distal circulation. A
local blue hue to skin may also be the result of pigment from blue clothing dye.
Triage Consideration . “Blue babies” should be evaluated promptly for
cardiorespiratory disease. The degree of oxygen desaturation associated with
cyanosis should be documented by pulse oximetry.
Clinical Assessment. When obtaining history, relevant questions include the
following: When was the color change first noted? Is it persistent or intermittent?
For example, choanal atresia will cause cyanosis at rest, which improves with
crying, whereas the cyanosis of congenital cardiac disease will often worsen with
crying because of increased pulmonary vascular resistance. Does the cyanosis
improve with oxygen? Are there other accompanying symptoms? In cyanotic

cardiac disease (i.e., right-to-left shunt), the breath sounds will be normal with
symmetric chest excursion. In contrast an infant with cyanosis due to pulmonary
disease or congestive heart failure will have wheezes or crackles and accessory
chest muscle use. With pulmonary disease or congestive heart failure, the increase
in saturation may be dramatic when the infant receives increased oxygen (see
hyperoxia test, under Section: Neonatal Cardiac Emergencies).


Cyanosis accompanied by mottling in a lethargic neonate with tachycardia
indicates shock. Sepsis, hypovolemia, intra-abdominal surgical emergency, and
metabolic crisis from inborn errors of metabolism (IEM) should be considered in
addition to primary cardiorespiratory problems. Early recognition and volume
resuscitation are critical for treatment.
Methemoglobinemia is characterized by a cyanotic infant without underlying
cardiac or pulmonary disease. The infant can look cyanotic to gray, with an
almost normal-appearing pulse oximetry. Supplemental oxygen will not alter the
color. Methemoglobinemia is confirmed by venous or capillary blood gas or the
persistence of a chocolate-brown color of a blood drop on filter paper. Initial
treatment is first searching and removing the offending agent, which is most often
topical anesthetic agents, aniline dyes, and high levels of nitrate in the water
supplies. Levels of methemoglobinemia above 20% are associated with clinical
symptoms. If methemoglobinemia is greater than 30% of total hemoglobin,
consider a dose of methylene blue, 1 to 2 mg/kg, given over 5 minutes.
Jaundice
CLINICAL PEARLS AND PITFALLS
Jaundice within the first 24 hours of life is pathologic.
Evaluation of jaundice requires assessment of both direct and indirect
bilirubin.
Acceptable levels of indirect hyperbilirubinemia depend on both
prematurity and postnatal age.

Acute bilirubin toxicity results in lethargy and progressive
encephalopathy in the neonate. Untreated, persistent bilirubin
encephalopathy results in kernicterus, a permanent brain injury.
Galactosemia should be considered in infants who have jaundice that
persists beyond 3 weeks of age.
Current Evidence. Jaundice is a yellow appearance of the skin or sclera caused
by elevated bilirubin levels. Bilirubin accumulates with excessive hemolysis,
failure of hemoglobin to conjugate with glucuronic acid in the liver, or inadequate
excretion through the liver canaliculi or bile ducts. Unconjugated bilirubin is
reported as indirect, and indicates excessive red blood cell hemolysis or inability
of the liver to keep pace with conjugation of bilirubin produced by hemolysis.