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Embryology, Anatomy, and Neonatal Lung Pathophysiology

Overview The respiratory system consists of conducting and gas exchange components, with the bifurcating airways and accompanying pulmonary arteries (PAs) bringing air and blood to peripheral capillary-lined airspaces for gas exchange. In understanding and evaluating pulmonary pathologic processes, it is useful to view the lung as consisting of multiple sets of branching trees including airways, arterial and venous pulmonary vasculature, systemic vasculature, and lymphatics. This chapter…

Trauma

The mortality rate of spine-related trauma in children is higher than in adults and is estimated at 25% to 32%. Fortunately, children with incomplete neurologic lesions fare better than adults, with up to 90% having partial recovery and 60% having full recovery. Etiology Motor vehicle accidents (MVAs), falls, and sports injuries are the most common causes of pediatric spinal trauma. Young athletes are at higher risk…

Vascular Lesions

Vascular lesions of the spine are uncommon in children. Vascular lesions are subdivided into fast-flow lesions, such as arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs), and slow-flow lesions, such as venous (formerly “cavernous”) malformations. Rarely, children suffer spinal cord infarcts. Several syndromes are associated with spinal cord AVMs and AVFs, including hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu), neurofibromatosis, and Parkes–Weber, Cobb, and CLOVES (congenital, lipomatous, overgrowth, vascular malformations,…

Spinal Tumor and Tumor-like Conditions

Spinal Neoplasms Overview. Spinal tumors occur predominantly in young or middle-aged adults and are rare in children. The most common presentation is vague pain, gait disturbance, change in spinal curvature, motor weakness, and bowel and bladder dysfunction. In this chapter, general concepts regarding spinal neoplasms are discussed, followed by anatomic categories of lesions. Categories include neoplasms located in the intramedullary, intradural-extramedullary, or extradural compartments. Imaging. The…

Infections of the Spine and Spinal Cord

Spinal infections in children occur uncommonly. Etiologies include direct and indirect (hematogenous) inoculation by bacterial, viral, parasitic, and fungal agents. Direct bacterial infectious inoculation may be as a result of trauma, instrumentation, or via a preexisting congenital sinus tract or area of spinal dysraphism (i.e., meningocele). Lyme disease ( Fig. 44.1 ), caused by a Borrelia burgdorferi -infected tick bite, is also a cause of spinal…

Congenital Abnormalities of the Spine

Embryology and Developmental Anatomy Spine formation begins early in gestation, commencing at the end of the second gestational week with formation of the Hensen node and continuing into the beginning of the third week with the appearance of the neural plate during gastrulation. The notochordal process forms at day 16 or 17, with transient communication of the amnion through the notochordal canal to the yolk sac…

Prenatal Imaging

Vast improvements have been made in the diagnosis and therapy of fetal anomalies as a result of advances in imaging and surgical technology. Nowhere is this more applicable than in the fetal spine. Ultrasonography is the primary imaging modality for fetal evaluation and helps distinguish normal from abnormal spine development and provides valuable information about spinal anomalies. Magnetic resonance imaging (MRI) is complementary to ultrasonography, as…

Spinal Cord Imaging Techniques

Pediatric spinal cord imaging relies heavily on ultrasound (US) in infants under 6 months of age and magnetic resonance imaging (MRI) thereafter. Other imaging modalities, such as radiography, computed tomography (CT), and nuclear scintigraphy, have a limited role but may provide useful information in cases with specific questions. Plain Radiography Plain radiographic spine series include frontal and lateral radiographs of the cervical, thoracic, or lumbar region.…

Embryology, Anatomy, and Normal Findings

Embryology The spinal cord forms in three stages beginning in the third gestational week. The first stage, neurulation, involves progression from neural plate to neural tube. The notochord becomes the nucleus pulposus of the intervertebral disks. The second stage, canalization, involves formation of cysts within the caudal cell mass that coalesce and fuse to the distal neural tube forming the primitive spinal cord. The third stage,…

Trauma

Incidence and Etiology Head trauma and resulting traumatic brain injury (TBI) are among the most common causes of morbidity and mortality in children. Head trauma alone is responsible for 50% of deaths in children ages 1 to 14 years. Approximately 475,000 cases of TBI occur annually in children less than 14 years in the United States. Half of the cases occur in children under 5 years.…

Neuroimaging in Pediatric Epilepsy

Overview Epilepsy is a common pediatric neurologic disorder. In North America, the overall incidence of epilepsy is approximately 50/100,000 per year, highest for children below 5 years of age and the elderly. Children are at higher risk for developing epilepsy than adults. Atypical, idiopathic, and focal epilepsy as well as epileptic syndromes require evaluation with magnetic resonance imaging (MRI). Approximately 30% of children with epilepsy become…

Pediatric Stroke

Stroke is a leading cause of disability and death in children. Three distinct time periods can be used to categorize stroke in the pediatric population: fetal (14 weeks of gestation until the onset of labor), perinatal/neonatal (between late third trimester and the first month of life), and childhood (beyond the first month of life until adulthood). The true incidence of fetal stroke is unknown, particularly because…

Cerebrovascular Disorders

Stroke is defined as a neurologic deficit persisting for more than 24 hours and may be caused by cerebral ischemia or intracranial hemorrhage. The annual incidence of stroke in children ranges from 2 to 13 cases per 100,000 patients and is among the top 10 causes of death in childhood. Among children diagnosed with stroke, more than 40% will have major neurologic sequelae and more than…

Pediatric Brain Neoplasms

Overview Brain tumors are the most common solid pediatric tumors and are the leading cause of death in children from solid tumors. The incidence rate of all childhood primary brain and central nervous system (CNS) tumors is estimated to be 5.47 cases per 100,000 person-years. An estimated 4830 new cases of childhood primary malignant and nonmalignant brain and other CNS tumors are expected to be diagnosed…

Infection and Inflammation

Brain Infections Bacterial, viral, fungal, and parasitic organisms are all causative factors in central neurologic system (CNS) infection. Brain infection manifests as encephalitis, cerebritis, and meningitis. Encephalitis refers to diffuse infection of the brain parenchyma, whereas cerebritis is due to a more focal parenchymal infection. Meningitis refers to infection of the pia, arachnoid, and dural membranes as well as the cerebrospinal fluid (CSF). Ventriculitis is often…

Inherited Metabolic and Neurodegenerative Disorders

Inherited Metabolic Brain Disorders Inherited metabolic brain disorders produce changes in brain metabolism and structure as a result of genetic mutations. Clinically, children with metabolic brain disorders often present with nonspecific symptoms, such as hypotonia, seizures, and developmental delay, which often makes diagnosis difficult. A combination of neurologic symptoms with and without visceral manifestation and age at onset of symptoms often are important factors indicating an…

Hydrocephalus

Hydrocephalus means “water in the brain.” It is the morphologic end result of many different processes related to enlarging ventricles with compression on brain parenchyma and subarachnoid spaces leading to raised intracranial pressure (ICP). The active enlargement of cerebrospinal fluid (CSF) space in the ventricles eventually leads to loss of brain tissue. By convention, ventriculomegaly associated with increased ICP is termed hydrocephalus . It is crucial…

Congenital Brain Malformations

Advances in gene sequencing and genotype-phenotype profiling have furthered understanding of many brain malformations. Some malformations previously considered disparate in terms of gestational timing and etiology are now considered phenotypic variants of common pathways while others previously considered variants of a specific malformation are now known to result from mutations affecting disparate molecular pathways (e.g., the lissencephalies). Given the large number of congenital human brain malformations,…

Neonatal Brain Injury

A significant number of deaths and permanent neurologic disabilities occur during the first month of life, the neonatal period. During the first month of life, term infants and particularly preterm neonates are vulnerable to a wide range of adverse events that may cause brain injury. Imaging is used not only to diagnose and understand neonatal brain injury but also to predict the neurodevelopmental outcome. Because of…

Prenatal Imaging

Central nervous system (CNS) anomalies occur in 1.4 to 1.6 per 1,000 live births and 3% to 6% of still births. Whereas some anomalies can be detected as early as the first trimester (such as anencephaly), others may not develop or become apparent until later in gestation. Ultrasound is the initial imaging modality used for the assessment of fetal CNS anomalies. When ultrasound is carefully performed…