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 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 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 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 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 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 ... 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... hydrocephalus generally not develop papilledema Subgaleal hemorrhage can be an acute life-threatening emergency resulting in acute blood loss and shock Vacuum extraction and coagulopathy, including