FIGURE 96.12 Mongolian spots. (Courtesy of George A. Datto, III, MD. In: Chung EK,
Atkinson-McEvoy LR, Boom JA, et al., eds. Visual Diagnosis and Treatment in Pediatrics . 2nd
ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2010. With permission.)
Ash-leaf macules are irregular hypopigmented macules, often with an oval or
“ash-leaf” appearance, commonly associated with tuberous sclerosis (TSC), a
condition characterized by benign tumors in multiple organs. While all the
clinical features of TSC may not be apparent in the first year of life, the median
age of presentation is over 6 months of age, most commonly with seizures,
infantile spasms, or several macules. Cardiac rhabdomyoma, often found on
prenatal ultrasound (US), is the second most common presentation. More than
three macules, at least 5 mm in diameter, are part of the criteria for TSC. Should
the clinician suspect TSC in an infant with several criteria, the infant should be
referred for molecular genetic testing ( Fig. 96.14 ).
FIGURE 96.13 Café-au-lait macules. (Reprinted with permission from Fletcher M. Physical
Diagnosis in Neonatology . Philadelphia, PA: Lippincott-Raven Publishers; 1998.)
FIGURE 96.14 Ash-leaf macules. (Courtesy of Ilona J. Frieden, MD. In: Chung EK, AtkinsonMcEvoy LR, Boom JA, et al., eds. Visual Diagnosis and Treatment in Pediatrics . 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2010. With permission.)
NEONATAL HEAD AND NECK EMERGENCIES
KEY POINTS
Neonates may develop hemorrhagic shock from intracranial or
subgaleal bleeding.
Neonates are obligate nasal breathers until 6 weeks of life. Any nasal
obstruction can cause respiratory distress during this period.
RELATED CHAPTERS
Signs and Symptoms
Cyanosis: Chapter 21
Respiratory Distress: Chapter 71
Medical, Surgical, and Trauma Emergencies
ENT Emergencies: Chapter 118
Neurosurgical Emergencies: Chapter 122
Disorders of Head Size, Shape, and Symmetry
CLINICAL PEARLS AND PITFALLS
Overriding sutures are common in babies born via normal vaginal
delivery.
Neonates can herniate despite an open anterior fontanelle, even
without bulging of the anterior fontanelle, if the pathology is confined to
the posterior fossa.
Increase in head circumference accompanied by any sign of increased
intracranial pressure (bulging fontanelle and widened sutures) should
be investigated.
Neonates with hydrocephalus generally do not develop papilledema.
Subgaleal hemorrhage can be an acute life-threatening emergency
resulting in acute blood loss and shock. Vacuum extraction and
coagulopathy, including vitamin K deficiency, may be predisposing
factors.
Current Evidence
Shape, size, and symmetry are factors that must all be considered in the
evaluation of the neonate’s head. Macrocephaly, microcephaly, cranial
asymmetry, and bulging anterior fontanelle are signs of underlying pathology.
Goals of Treatment
Clinicians need to identify generally innocent etiologies from those that are life
threatening. The primary goals should be early recognition of hemorrhagic shock
from intracranial bleeding and increased intracranial pressure. All neonates with
disorders of size and shape of head need a thorough physical examination
including neurologic examination. Those with significant findings will require
head US, computed tomography (CT), or magnetic resonance imaging (MRI). In
any patient with significant findings, neonatology, neurology, and/or neurosurgery
consultation are warranted. Admission to the intensive care unit is necessary in
infants with cardiovascular instability.
Clinical Considerations
Clinical Recognition. The size, shape, and skin discoloration on the head can
first be noted by the clinician on inspection of the baby during physical
examination. Parents are often unaware of the problem unless the head size is
severely enlarged, has prominent skin changes, prominent asymmetry, or the baby
is presenting with other symptoms. Vague symptoms may be noted by the parent
including poor feeding, irritability, cyanotic episodes (apneic episodes), fever,
inability to maintain temperature, or the baby is “just not herself.” Measurement
of the head circumference should be routinely performed for infants presenting
within the first 2 weeks of life.
Triage Considerations. Neonates with bulging or sunken fontanelle associated
with poor feeding, irritability, or temperature instability should be evaluated
promptly for meningitis, sepsis, or shock. Associated hypothermia and ill
appearance imply neonatal sepsis and demands emergent treatment.
Clinical Assessment. Neonatal disorders of head shape, size, and symmetry are
detected by inspection, palpation, and measurement of head circumference. The
head should be examined in the midline position, then on each side. Benign
dermatologic lesions such as seborrheic dermatitis (cradle cap) ( Fig. 96.15 ),
capillary hemangioma, vesicles and ulcers from fetal scalp monitoring electrodes,
caput succedaneum ( Fig. 96.16 ), cephalohematoma ( Fig. 96.17 ) and significant
conditions such as giant congenital nevi, port-wine stain, and tumors may be