51 : 361

CYANOSIS
Selected References
Allen MC. Assessment of gestational age and neuromaturation. Ment Retard Dev Disabil Res
Rev. 200S;11:21—33.
Allen MC. Risk assessment and neurodevelopmental outcomes. In: Gleason CA, Devaskar
SU, eds. Averys Diseases of the Newborn. Philadelphia: Saunders/Elsevier, 2012:920-935.
Allen MC, Cristofalo EA, Kim C. Outcomes of preterm infants: morbidity replaces mortality.
Clin Perinatol. 2011;38:441—4S4.
Behrman RE, Butler AS, eds. Preterm Birth: Causes, Consequences, and Prevention. Committee
on UnderStonding Premature Birth and Assuring Healthy Outcome5. Washington, DC:
National Academies Press; 2007.
Donohue PK, Boss RD, Shepard J, Graham E, Allen MC. Intervention at the border of viability:
perspective over a decade. Arch Pediatr Adole5c Med. 2009;163:902-906.
Graham EM, Ruis KA, Hartman AL, Northington FJ, Fox HE. A systematic review of the
role of intrapartum hypoxia-ischemia in the causation of neonatal encephalopathy.
Am J Gbstet Gynecol. 2008;199:587—S95.
Miguel-Verges F, Woods SL, Aucott SW, Boss RD, Sulpar LJ, Donohue PK. Prenatal consul-
tation with a neonatologist for congenital anomalies: parental perceptions. Pediatrics.
2009;124:eS73—e579.
Raz S, Debastos AK, Newman JB, Batton D. Intrauterine growth and neuropsychological per-
formance in very low birth weight preschoolers. J Int Neurop5yChol Soc. 2012;18:200-211.
Shankaran S, Lester BM, Das A, et a1. Impact of maternal substance use during pregnancy on
childhood outcome. .$rmin hetal Neonatal Med. 2007;12:143—150.

51CÿähOSÏS

I. Problem. During a physical examination, an infant appears blue. Cyanosis can be

caused by a rise in deoxygenated hemoglobin (more common) or an abnormal
hemoglobin disorder.
II. Immediate questions
A. Does the infant have respiratory distress? If the infant has increased respiratory
effort with increased rate, retractions, and nasal flaring, respiratory disease should
be high on the list of differential diagnoses. Cyanotic heart disease usually presents
without respiratory symptoms (“happy blue baby”) but can have effortless
tachypnea (rapid respiratory rate without retractions). Blood disorders usually
present without respiratory or cardiac symptoms.
B. Does the infant have a murmur? A murmur usually implies heart disease, but
in infants with congenital heart malformations, <50"% have a murmur in the
newborn period. Transposition of the great vessels can present without a
murmur (-60%). Muffled heart sounds can indicate pericardial effusions or
pneumopericardium.
c. Was the infant cyanotic at birth? Infants with transposition of the great vessels
and tricuspid atresia can present almost immediately at birth with cyanosis. In the
perinatal period, infants with truncus arteriosus, total anomalous pulmonary venous
return, and tetralogy of Fallot can present with cyanosis.
D. Is the cyanosis continuous, intermittent, cyclical, sudden in onset, or occurring
only with feeding or crying? Intermittent cyanosis is more common with neu-
rologic disorders; these infants may have apneic spells alternating with periods of
normal breathing. Cyclical cyanosis can occur with nasal obstruction. Continuous
362 NEONATOLOGY

cyanosis is more commonly associated with intrinsic lung disease or heart disease.
Cyanosis with feeding can occur with esophageal atresia and severe
gastroesophageal reflux. Sudden onset of cyanosis may occur with an air leak, such
as pneumothorax. Cyanosis that disappears with crying may mean choanal atresia.
Cyanosis only with crying can occur in infants with tetralogy of Fallot. Cyanotic
spells with no or minimal coughing can occur with pertussis. Crying may improve
cyanosis in respiratory disease and worsen it in cardiac disease.
E. Is the pulse oximeter normal and the infant blue? The pulse oximeter measures
oxygen saturation of hemoglobin that is available to bind oxygen. If there is
abnormal hemoglobin, it will not be measured. If you see a normal pulse oximeter
in a cyanotic infant, think methemoglobinemia.
F. Has the baby had the recommended pulse oximetry screening for congenital
heart disease? This has been recommended by the American Academy of Pediatrics
(AAP) in all newborns (see page 43) as a useful method for screening for critical
congenital cyanotic heart disease.
G. Is there differential cyanosis (DC) ? Differential cyanosis is when there is cyanosis
of the upper or lower part of the body only, and it usually signifies serious heart
disease. The prerequisite for this to happen is the presence of a right-to-left
shunt through the patent ductus arteriosus (PDA). To diagnose this, oxygen
saturation should be measured in the preductal (right hand is preferred since it
accurately reflects preductal value) and postductal (foot). There are 2 different
types of dif- ferential cyanosis.
1. Pink upper half of the body, cyanosis lower part of body (more common).
(Oxygen saturation is greater in the right hand than in the foot.) It occurs with
severe coarctation of the aorta or interrupted aortic arch. It can also occur with
persistent pulmonary hypertension (PPH) with right-to-left shunting through the
ductus arteriosus.
2. Cyanosis in the upper half of the body, pink lower part of the body. This
type is very rare (reversed differential cyanosis [RDC] ) and occurs when
oxygen saturation is lower in the right hand than in the foot. This is seen
in complete transposition of the great arteries with shunt through the PDA
with persistent pulmonary hypertension or aortic interruption/coarctation. It
can also be seen in an infant with supracardiac total anomalous pulmonary
venous connection (TAPVC) to the superior vena cava with a shunt through
the PDA.
H. What is the prenatal and delivery history? Did the mother have a prenatal
sonogram? It may show a cardiac anomaly. An infant of a diabetic mother has
an increased risk of hypoglycemia, TTN, polycythemia, respiratory distress
syndrome, and heart disease (TGA). Infection, such as that which can occur
with premature rupture of membranes, may cause shock and hypotension with
resultant cyanosis. Coxsackie B viral infections cause myocarditis in newborn
infants. Amniotic fluid abnormalities, such as oligohydramnios (associated with
pulmonary hypoplasia) or polyhydramnios (associated with esophageal atresia),
may suggest a cause for the cyanosis. Cesarean section is associated with
increased respiratory distress, transient tachypnea of the newborn (TTN), and
persistent pulmonary hyperten- sion of the newborn (PPHN). Pregnancy-
induced hypertension can be associated with intrauterine growth restriction
(IUGR), polycythemia, and hypoglycemia. Congenital infections can lead to
cardiac abnormalities. Advanced maternal age can be associated with birth
defects such as Down syndrome and Turner syndrome, which include heart
defects. Certain perinatal conditions increase the incidence of congenital
heart disease.
1. Medications used by the mother can cause an increase in congenital heart
disease. Anticonvulsants, lithium, indomethacin, nonsteroidal anti-inflammatory
drugs (NSAIDs), ibuprofen, sulfasalazine, thalidomide, trimethoprim, sulfon-
amide, vitamin A, selective serotonin reuptake inhibitors (SSRIs), marijuana,
alcohol, cigarette smoking, cocaine, and exposure to organic solvents.
51 : 363
CYANOSIS
2. Maternal illnesses that increase the risk of congenital heart disease. Untreated
phenylketonuria (PKU), maternal pregestational diabetes, febrile illness during
the first trimester, influenza, maternal rubella, epilepsy, and maternal
1upus/con- nective tissue disease.
3. Maternal congenital heart disease and/or congenital heart disease in a first-
degree relative. Increased incidence of heart disease in the child.
III. Differential diagnosis. Cyanosis becomes visible when there is >3—5 g/dL of deoxy-
genated hemoglobin/dL. The degree of cyanosis depends on both oxygen saturation
and hemoglobin concentration. Cyanosis can be a sign of severe cardiac, respiratory,
or neurologic compromise. The most common etiology of cyanosis in a newborn
infant is respiratory. Cyanosis can also be caused by a reduced blood oxygen—
carrying capacity secondary to an abnormal form of hemoglobin, such as
methemoglobinemia. Cyanosis may not be apparent in a severely anemic infant or
may be difficult to see in a darkly pigmented newborn. The causes of cyanosis can be
classified as arising from respiratory, cardiac, central nervous system (CNS), or other
disorders.
A. Respiratory diseases. Include primary pulmonary diseases, airway obstruction,
and extrinsic compression of the lungs and congenital defects. Pulmonary diseases
are the most common cause of cyanosis in the newborn.
1. Primary pulmonary diseases. Respiratory distress syndrome (RDS), TTN,
aspira- tion syndromes, pneumonia, bronchopulmonary dysplasia/chronic lung
disease (SPD/CLD), pulmonary interstitial emphysema (PIE), pulmonary
hemorrhage.
2. Airway obstruction. Mucous plug, Pierre Robin syndrome, choanal atresia, vocal
cord paralysis, macroglossia, atelectasis, and others.
3. External compression of the lungs. Any air leak syndrome, pleural effusion, and
others.
4. Congenital defects. Congenital diaphragmatic hernia, pulmonary hypoplasia,
cystic adenomatoid malformation, lobar emphysema, and others.
B. Infections. Sepsis is the second most common cause of cyanosis in infants. Sepsis
causes increased oxygen utilization, which results in cyanosis. Meningitis can also
present with cyanosis.
C. Hypotension and shock. This can be secondary to sepsis, cardiogenic, neurogenic,
or hypovolemic, and all can present with cyanosis. (See Chapter 65.)
D. Cardiac diseases. The majority of congenital heart diseases that present in the first
couple of weeks of life are ductal-dependent cardiac lesions.
1. All cyanotic heart diseases which include the 5 T’s. Transposition of the great
arteries is the most common cyanotic congenital heart disease in newborns.
a. Transposition of the great arteries.
b. T’otal anomalous pulmonary venous return.
c. Tricuspid atresia.
d. Tetralogy of Fallot.
e. Truncus arteriosus.
f. Sixth T (“tons of others”/”terrible T’s”) includes all the others: severe pul-
monic stenosis, double outlet right ventricle, pulmonary atresia with intact
ventricular septum/or with ventricular septal defect (VSD), variations on
single ventricle, Ebstein anomaly of the tricuspid valve, hypoplastic heart
syndrome with intact foramen ovale (no mixing at the atrial level), and
others.
2. Persistent pulmonary hypertension of the newborn (PPHN). In PPHN, infants do
not transition from fetal to newborn circulation. Pulmonary hypertension
causes right-to-left shunting of blood, a decrease in pulmonary blood flow, and
cyanosis.
3. Severe congestive heart failure. This can occur from cardiomyopathies (infant
of diabetic mother [IDM], inborn errors of metabolism, genetic or
neuromuscular disease), myocarditis (bacterial or viral), congenital cardiac
disease, sepsis, peri- natal asphyxia, and sustained tachyarrhythmias.
4. Pneumopericardium or pericardial effusion.
364 NEONATOLOGY

5. Other congenital anomalies such as those associated with cardiac

malformations: Turner syndrome, Noonan syndrome, etc. Pulmonary
arteriovenous malforma- tion is a rare cause of cyanosis in the newborn.
E. Central nervous system diseases. CNS disorders can cause apnea, seizures, and
decreased respiratory effort.
1. Infectious. Bacterial or viral CNS infection (meningitis, encephalitis).
2. Seizures. Infection, metabolic, CNS injury, genetic syndrome, congenital
disorder, primary seizure disorder.
3. Hypoxic ischemic encephalopathy (HIE).
4. Hemorrhage. Periventricular/intraventricular hemorrhage, subdural hemor-
rhage, subarachnoid hemorrhage, intracerebellar hemorrhage, infarction.
5. Congenital disorders. Congenital hydrocephalus, spinal muscle atrophy, con-
genital central hypoventilation syndrome.
6. Drug toxicity (opioid toxicity).
F. Neuromuscular disorders. Werdnig-Hoffman disease, Pompe disease, Barth syn-
drome, Duchenne or Becker muscular dystrophy, limb-girdle muscular dystrophy,
congenital myopathy, neonatal myasthenia gravis, phrenic nerve injury, and con-
genital myotonic dystrophy.
G. Hematologic disorders. A hemoglobin disorder can interfere with the transport of
oxygen and cause cyanosis.
1. Methemoglobineinia (normal arterial0 2). Because the arterial blood is brown
in color, it gives off a bluish hue in the skin of Caucasian people. It can be con-
genital (familial) or secondary to a toxin (medications such as eutectic mixture
of lidocaine and prilocaine [EMLA] , sulfonamides, others) or environmental
substances.
2. Polycythemia/hyperviscosity syndrome (normal art0erial 2). Infants with this
can present with peripheral cyanosis, tachypnea, congestive heart failure (CHF),
and cardiomegaly. Cyanosis is detectable at a higher value of Sao 2. Polycythemia
can cause pulmonary hypertension.
3. Severe anemia from hemorrhage or bleeding disorders.
H. Metabolic abnormalities can present with apnea and cyanosis
1. Drug withdrawal.
2. Hypoglycemia, hypermagnesemia, severe metabolic acidosis.
3. Inborn errors of metabolism.
4. Rarely abnormalities of calcium, potassium, and phosphorus can cause hypoxia
and cyanosis. Calcium and potassium abnormalities can cause cardiac arrhythmias.
I. Other disorders
1. Apnea and bradycardia
2. Hypothermia
3. Hypoadrenalism/hypopituitarism
4. Abdominal distension with elevation of the diaphragm
5. Respiratory depression secondary to maternal medications (eg, magnesium sul-
fate and narcotics) or sedation
J. Pseudocyanosis. Caused by fluorescent lighting.
IV. Database. Obtain a prenatal and delivery history (see Section II.H).
A. Physical examination
1. Assess the infant for central, peripheral, acrocyanosis, versus differential
cyanosis
a. Central cyanosis. Skin, lips, and tongue appear blue. This indicates
generalized cyanosis. This is caused by reduced arterial oxygen saturation.
b. Peripheral cyanosis. Skin is bluish but the oral mucous membranes are pink.
Seen in methemoglobinemia, caused by a normal arterial oxygen saturation
and increased oxygen extraction.
c. Acrocyanosis. Hands and feet are blue but nothing else. This can be present
in normal infants in the first 24-48 hours. It is caused by immature vascular
tone
51 : 365
CYANOSIS
or vasoconstriction secondary to a cold environment. Less commonly it may
indicate poor tissue perfusion or a decrease in cardiac output.
d. Circumoral cyanosis. Blue appearance around the mouth. There is a venous
plexus around the mouth that gets engorged during feeding. Usually a
normal finding, it can be an expression of peripheral cyanosis.
e. Poor peripheral perfusion with cyanosis. Seen in sepsis, hypoglycemia, dehy-
dration, and hypoadrenalism.
f. Differential cyanosis. Cyanosis of the upper or lower part of the body only
(see Section II.G).
2. Assess the heart. Check for any murmurs and for heart rate and blood pressure.
Increased second heart sound intensity can be seen in pulmonary hypertension.
Single second heart sound can be seen with transposition of the great vessels,
aortic atresia, truncus arteriosus, pulmonary atresia, and conditions with
pulmo- nary hypertension. Remember not all infants with congenital heart
disease have a murmur. (Transposition of the great vessels can have no
detectable murmur.) Muffled heart sounds can signify pneumopericardium or
pericardial effusion. A displaced cardiac impulse may mean dextrocardia or
dextroposition. Not all infants with murmurs have congenital heart disease.
3. Assess the respiratory system. Is there retraction, nasal flaring, or grunting?
Retractions are usually minimal in heart disease. Check the nasal passage for
choanal atresia. Infants with pulmonary disease will have tachypnea and distressed
breathing, whereas infants with cardiac disease do not.
4. Assess the abdomen. Check for an enlarged liver. The liver can be enlarged in
CHF and hyperexpansion of the lungs. A scaphoid abdomen may suggest a dia-
phragmatic hernia. Hepatomegaly can indicate high venous pressure.
5. Check the pulses. In coarctation of the aorta, the femoral pulses are decreased.
In patent ductus arteriosus, the pulses are bounding.
6. Consider neurologic problems. Check for apnea and periodic breathing, which
may be associated with immaturity of the nervous system. Observe the infant
for seizures, which can cause cyanosis if the infant is not breathing during
seizures.
7. Assess for multiple malformations on the examination. These may suggest
under- lying heart or pulmonary defects (“CHARGE” or “VATER/VACTERk’
anomalies).
B. Laboratory studies
1. Arterial blood gas measurements on room air. If the patient is not hypoxic,
it suggests methemoglobinemia, polycythemia, or CNS disease. If the patient is
hypoxic, perform the hyperoxia test, described later. Pulse oximetry can be used
to check arterial saturation but is not a good indicator of central cyanosis. An
increased CO 2 can indicate pulmonary, PPHN, or CNS disorders. Metabolic aci-
dosis can indicate sepsis, severe hypoxemia, or shock. A low or normal CO 2 can
indicate cardiac disease.
2. Complete blood count (CBC) with differential. This may reveal an infectious
process. A central hematocrit of >65% confirms polycythemia.
3. Sepsis workup. Blood culture and C-reactive protein (CRP), urine culture, and
lumbar puncture (LP) if indicated.
4. Serum glucose level. To detect hypoglycemia.
5. Methemoglobin level. If the infant has methemoglobinemia, the blood will not
turn red when exposed to air. It will have a chocolate hue. To confirm the
diagnosis, the laboratory should perform a spectrophotometric determination.
c. Imaging and other studies
1. Transillumination of the chest. (See Chapter 40.) Should be done on an
emergent basis if pneumothorax is suspected.
2. Chest radiograph. If normal, it suggests a CNS disease or other cause for the
cya- nosis (see Section III). It can verify lung disease, air leak, or diaphragmatic
hernia. It can also help diagnose heart disease by evaluating the heart size and
pulmonary vascularity. The heart size may be normal or enlarged in hypoglycemia,
polycythemia,
366 NEONATOLOGY

shock, and sepsis. In cardiac lesions with cyanosis and increased pulmonary
blood flow there will be cardiomegaly. Decreased pulmonary vascular markings
rep- resent decreased blood flow through the pulmonary circulation and can be
seen in tetralogy of Fallot, pulmonary atresia/stenosis, truncus arteriosus, and
Ebstein anomaly. Increased pulmonary arterial markings can be seen in truncus
arte- riosus, single ventricle, and transposition of the great arteries. Increased
venous markings can be seen in hypoplastic left heart syndrome and total
anomalous pulmonary venous return. Shape of the heart can be important:
a. Boot-shaped heart. Tetralogy of Fallot, tricuspid atresia
b. Egg-shaped heart (“egg on a string”). Transposition of the great arteries
c. Large globular heart. Ebstein anomaly
d. Dextrocardia/mesocardia. Congenital heart disease
e. “Snowman” or “figure 8.” Total anomalous pulmonary venous return
3. Hyperoxia test. Because of intracardiac right-to-left shunting, the infant with
cyanotic congenital heart disease in contrast to the infant with pulmonary disease
is unable to raise the arterial saturation. Measure arterial oxygen on room air. Then
place the infant on 100’ko oxygen for 10-20 minutes. Then remeasure arterial oxy-
gen. It is best not to use pulse oximetry since it may not give an accurate result.
Nofe: A value of >150 mm Hg does not always rule out cyanotic heart disease.
Diagnosis of cardiac disease can be delayed from a misleading hyperoxia test and
has been
reported (pulmonary disease with cardiac disease, infracardiac total anomalous
pulmonary venous connection with Pao 2 >250 mm Hg). Echocardiogram should
be done if unsure.
a. Normal infant. Pao2 >300.
b. Pulmonary disease. Pao2 >1S0 mm Hg. In an infant with severe pulmonary
disease, the arterial oxygen saturation may not increase significantly.
c. Cardiac disease. Pao2 <50-70 mm Hg. In cyanotic heart disease the Pao 2 most
likely will not increase significantly (usually <100 mm Hg and often <70 mm
Hg).
The hyperoxia test can also help in differentiating the different types of heart
disease. Infants with transposition of the great arteries or severe pulmonary
outflow obstruction will usually have a Pao2 <50 mm Hg. Infants with disease
with both right-to-left and left-to-right shunting (truncus arteriosus, TAPVC
without obstruction, hypoplastic left heart syndrome, single ventricle with
PDA) can have an increase in Pao2 but rarely >150 mm Hg.
d. PPHN. In infants with PPHN, it may or may not increase significantly. If the
Pao2 increases to <20-30 mm Hg, PPHN should be considered.
e. Neurologic disease. Pao2 >150 mm Hg.
f. Methemoglobinemia. Pao2 >200 mm Hg but pulse oximetry remains low.
4. Right-to-left shunt test. Done to rule out PPHN. Best way to do this is with pulse
oximetry. Place 2 pulse oximeters on the infant (one preductal on the right
hand, one postductal on either foot). If the simultaneous difference is >5"%
between preductal and postductal oxygen saturations, it is indicative of a right-
to-left shunt. One can also draw a simultaneous sample of blood from the right
radial artery (preductal) and the descending aorta or the left radial artery
(postductal). If there is a difference of >10—15 mm Hg (preductal more than
postductal), the shunt is significant.
5. Hyperventilation test. Hyper ventilating the infant for 10 minutes (lowering
Paco2 and increasing the pH) will result in a marked improvement in
oxygenation (>30 mm Hg increase in Pao2) in PPHN. This may help differentiate
the infant
with PPHN from that with cyanotic congenital heart disease (little or no
response in CHD).
6. Electrocardiography (ECG). Usually normal in patients with methemoglobin-
emia or hypoglycemia. With polycythemia, pulmonary hypertension, or primary
lung disease, the ECG is normal but may show right ventricular hypertrophy.
The ECG is usually nondiagnostic because of the normal neonatal right-axis
deviation
51 : 367
CYANOSIS
and dominant right wave in the right chest leads. It is very helpful in identifying
patients with tricuspid atresia; it will show left-axis deviation and left ventricular
hypertrophy. ECG can be normal in transposition of the great vessels.
7. Echocardiography. Should be performed immediately if cardiac disease is sus-
pected or if the diagnosis is unclear. It is the gold standard and definitive diag-
nostic test for congenital heart disease. It can confirm pulmonary hypertension.
8. Computed tomography (CT) and CT angiography. May help identify
anomalies of the pulmonary venous return.
9. Ultrasonography of the head. Performed to rule out periventricular/intraven-
tricular hemorrhage.
10. Polysomnographic recording. Helps diagnose apnea and its type.
11. Electroencephalogram (EEG). If seizure is suspected.
V. Plan
A. General management. Act quickly and accomplish many of the diagnostic tasks at
once. Perform resuscitation (ABCs) if necessary and provide respiratory support,
volume resuscitation if necessary, and antibiotics as indicated. Inotropic support
and correcting metabolic acidosis are essential.
1. Perform a rapid physical examination. What is the blood pressure? Other vital
signs? Transilluminate the chest (see Chapter 40). If a tension pneumothorax is
present, rapid needle decompression may be needed.
2. Order immediate studies. For example, blood gas levels, CIRC, and chest radio-
graph. Consider echocardiography.
3. Perform the hyperoxia test. See Section IV.C.3.
B. Specific management
1. Lung disease. (See the appropriate disease chapter.) Respiratory depression
caused by narcotics can be treated with naloxone (Narcan) (see Chapter 148 for
dosing).
2. Air leak (pneumothorax). See Chapter 70.
3. Congenital defects. Surgery is indicated for diaphragmatic hernia.
4. Cardiac disease. Prostaglandin E, (PGE,) is indicated for any clinical condition
in which blood flow must be maintained through the ductus arteriosus to sustain
pulmonary or systemic circulation until surgery can be performed.
a. Give PGE, to increase pulmonary blood flow for pulmonary atresia/steno-
sis, tricuspid atresia, tetralogy of Fallot, and Ebstein anomaly of the tricuspid
valve. Other ways of improving pulmonary blood flow are with supplemental
oxygen, maintaining a respiratory alkalosis, sildenafil, and inhaled nitric
oxide.
b. Give PGE, to increase systemic blood flow for hypoplastic left heart syn-
drome, coarctation of the aorta, critical aortic stenosis, aortic arch
interruption.
c. Give PGE to improve mixing in transposition of the great arteries.
d. PGE, is not recommended in respiratory distress syndrome, PPHN, total
anomalous venous return with obstruction (PGE 1 may minimize obstruction
but does not help clinically), and dominant left-to-right shunt (patent ductus
arteriosus, truncus arteriosus, or ventricular septal defect).
e. If the diagnosis is uncertain, a trial of PGE, can be given over 30 minutes
in an effort to improve blood gas values.
f. Other management. D-transposition of the great arteries requires urgent
balloon atrial septostomy under echocardiogram in the nursery if hypoxia or
acidosis occurs. TAPVR, transposition of the great arteries with VSD, and
trun- cus arteriosus require further cardiac evaluation and possible surgery.
5. PPHN. See Chapter 120.
6. CNS disorders. Treat the underlying disease.
7. Methemoglobinemia. Treat the infant with methylene blue only if the methemo-
globin level is markedly increased and the infant is in cardiopulmonary distress
(tachypnea and tachycardia). Administer intravenously 1 mg/kg of a 1"% solution
of methylene blue in normal saline. The cyanosis should clear within 1—2 hours.
8. Shock. See Chapter 63.