Transcript Retinoids Induce Apoptosis and Release of Basic FGF from

Blue Spells in “Well Babies”
N. Ambalavanan MD
Assistant Professor,
Division of Neonatology
University of Alabama at Birmingham
Aug 2005
What are “blue spells”?
 Sudden
onset of:
– Central cyanosis
• with respiratory distress
• without distress
– Apnea and / or Limpness
• with cyanosis
• without cyanosis
– Choking
What is normal in well babies?

Transient central cyanosis during crying or
straining, resolving rapidly when the baby is quiet
 Periodic breathing, with pauses of up to 15 seconds,
with no cyanosis or bradycardia
 Occasional GE reflux (“spitting up”)
 Bradycardia on deep pharyngeal suctioning
 Acrocyanosis and/ or dark lips (with pink tongue)
 Changes in tone with sleep state
Central cyanosis in neonates

Oxygen saturation of <85% may present as central
cyanosis, if deoxygenated Hb> 4-5g / dL
 Not all hypoxemia presents as cyanosis:
– PaO2 is in the 35-50 mm Hg range (SpO2 85-90%)
– Hb too low (e.g. Hb 10, 80% SpO2)

Not all cyanosis due to hypoxemia
– methemoglobinemia

Focus: Pulmonary or non-pulmonary etiology?
Cyanosis: Pulmonary or non-pulmonary?
 Tachypnea
and/or increased depth of breathing
may be seen with both pulmonary and nonpulmonary causes
 Retractions are more often seen with lung
disease
 Slow breathing may be seen with nonpulmonary causes (esp. CNS causes)
Pulmonary causes of cyanosis

Most common:
– Parenchymal lung disease most common
• TTN
• RDS
• pneumonia
• meconium aspiration
• malformations (CCAM, CLE etc)
– Rare causes
• CDH, spontaneous pneumothorax, airway obstruction
(e.g. Pierre-Robin syndrome, laryngeal web, vascular
rings and slings)
Non-pulmonary cyanosis + Rapid breathing

Cardiac abnormality
– eg. TGA, TOF, TAPVR, Truncus A, Tricuspid Atr, AV
Canal, HLH
– mixing lesions / decreased PBF / decreased CO

Persistent pulmonary hypertension (PPHN)
 Septicemia
 Metabolic and blood disorders
– hypoglycemia / CAH / hypothermia / Inborn error of
metabolism / Methemoglobinemia

Early shock
Non-pulmonary cyanosis + slow breathing

CNS
– Sedation due to maternal or neonatal drugs (MgSO4,
opiates)
– Meningitis
– Perinatal asphyxia
– CNS trauma due to difficult delivery
– CNS malformation
– Congenital neuromuscular disease (e.g. congenital
myotonia, myasthenia)
Inborn errors of metabolism (IEMs)

IEMs may present
– before birth, at birth, during first 2-3 days,
– as sudden death (or) as deterioration following normal birth
and delivery
 Mom may develop HELLP if fetus has LCHAD deficiency
 Perinatal asphyxia common misdiagnosis (esp if congenital
lactic acidoses or pyridoxine dependency)
 Sudden death may occur with fatty acid oxidation defects
[Leonard & Morris: Lancet 2000; 356: 583-87]
Inborn errors of metabolism (IEMs)

Seizures/Apnea
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Pyridoxine dependency
Peroxisomal disorders
Molybdenum cofactor deficiency
Non-ketotic hyperglycinemia
Congenital lactic acidosis
Severe hypotonia
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Peroxisomal disorders
Non-ketotic hyperglycinemia
Congenital lactic acidoses
Carbohydrate deficient glycoprotein syndromes
Inborn errors of metabolism (IEMs)

Cardiomyopathy and arrhythmias may occur with LCFA
oxidation or respiratory chain defects
 Neurologic deterioration may occur with organic
acidemias, urea-cycle defects, MSUD, FAO defects,
congenital lactic acidoses etc
 Persistent metabolic acidosis with normal tissue perfusion
may occur with organic acidemia or congenital lactic
acidosis
 Mild respiratory alkalosis in non-ventilated babies may
signify hyperammonemia, esp if irritability and stridor also
present
Management
 History
– Exact details of episode, antecedent factors, and
how episode resolved
– Obstetric history - anesthesia, MgSO4, GBS,
PROM, antibiotics, HSV
– Delivery history - need for resuscitation, Apgar
scores, medications used
– Family history of similar spells, inborn errors of
metabolism
Management
 Physical examination
– Cyanosis / Pallor / Perfusion / Temperature / O2 Saturation
– Respiration - apnea / periodic / fast / slow
– G / F / R / Stridor (r/o choanal atresia, laryngomalacia)
– Heart rate - regular / arrhythmia
– BP - four limb
– Auscultation: S1 / S2 / Murmurs / Lung sounds
– Abdominal organomegaly
– CNS - Tone / Reflexes / Symmetry / AF / Abnormal
movements
Management
 Investigations to consider
– Sepsis screen (CBC, BlCx, CSF, CXR)
– Cardiac screen (ECHO, EKG, 100% O2 test, pre- and postductal SpO2)
– CAH screen (Electrolytes)
– MetHb screen (ABG for MetHb, blood exp to room air)
– CNS screen (Cranial USG, CSF, EEG, CT/MRI)
– IEM screen (at time of sepsis screen)
• ABG (pH, HCO3), Glucose, Electrolytes, Anion Gap,
LFTs, Ammonia, Pediatrix newborn screen, urine sugars
and ketones
Management
 Therapy
– directed towards possible cause
– general supportive neonatal intensive care
important
• Airway, Ventilation, Perfusion, IV access
– O2 / mechanical ventilation if pulmonary disease
– Antimicrobials if suspicion of sepsis / meningitis
– PGE1 if suspicion of ductal dependent cardiac
disease
Management of IEMs
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Stop nutrient triggering disorder e.g. protein, galactose
Give high-energy intake
NICU care to correct tissue perfusion, dehydration, acidosis
Hyperammonemia Rx with Na benzoate, Na phenylbutyrate,
arginine
Dialysis
Insulin to control hyperglycemia and reduce catabolism
Vitamins e.g Biotin, B6, B12
Specific therapy e.g. carnitine, glycine
Home monitors?

Do not monitor for transient choking, reflux, apnea,
bradycardia or cyanosis that was not life threatening
 Do not monitor asymptomatic premies, all sibs of
SIDS infants, infants of drug-using moms
 Monitoring can be done if:
– event truly life-threatening (required CPR or vigorous
stimulation) with no identifiable cause or if untreatable
cause (e.g severe CNS hypoventilation)
– Sibling of two or more infants who died of SIDS

Monitors not an answer for most infants