INTRODUCTION Background Within a space of less than a few centimeters, the temporal bone contains highly specialized small anatomic structures that allow for hearing and balance. The temporal bone is composed of five parts (petrous, tympanic, squamosal, mastoid, and zygomatic) and is subdivided as the inner ear, middle ear, and external ear. The inner ear converts mechanical pressure into electrical signal to provide hearing and balance…

INTRODUCTION Background Craniofacial malformations have characteristic osseous and soft tissue features, which lead to visible deformity. In addition, these anomalies can lead to loss of normal function of hearing, swallowing, and vision. These malformations frequently involve combinations of craniofacial locations. Several craniofacial malformations have known genetic causes. The most common genetic abnormality involves the fibroblast growth factor. Imaging CT imaging with 3D reconstruction is the modality…

INTRODUCTION Embryology The ventricles arise from the lumen of the forebrain, midbrain, and hindbrain. At approximately the sixth week of gestation, the lateral ventricles arise as extensions from the anterior-superior aspect of the third ventricle and communicate through the foramen of Monro. The lumen of the hindbrain expands to become the fourth ventricle. The cerebral aqueduct persists in the midbrain and allows communication between the third…

INTRODUCTION Background Trauma is the most common cause of death and a significant cause of morbidity in children. Both accidental and nonaccidental trauma are common in children. Several anatomic differences in younger children should be highlighted to understand why younger children are more susceptible to certain types of injury. First, the skull of young children is thin and pliable and thickens during the first 2 years…

INTRODUCTION Phakomatoses are a group of neurocutaneous disorders that involve structures arising from the embryologic ectoderm, resulting in abnormalities of the skin, nervous system, retina, and globe. There are approximately 30 different phakomatoses; however, the main phakomatoses include neurofibromatosis type 1 (NF-1), neurofibromatosis type 2 (NF-2), tuberous sclerosis, Sturge-Weber syndrome, and Von Hippel-Lindau disease. Table 9.1 highlights the CNS, globe and orbit, skin, and other abnormalities…

INTRODUCTION Background Epilepsy is a chronic seizure condition in which abnormal excessive or uncoordinated neuronal activity leads to abnormal brain function. About 1% of children in the United States have epilepsy. Approximately 66% to 75% of children with epilepsy will become seizure free with anticonvulsant medication. Intractable epilepsy is defined as failure of two or more appropriate antiepileptic drugs and more than one seizure per month…

INTRODUCTION Background Central nervous system (CNS) tumors are the second most common pediatric cancer diagnosed each year, accounting for approximately 25% of childhood cancers. They are responsible for the second most common cause of cancer deaths in children. Survival has slowly improved over the years, and overall survival is now approximately 75%. Supratentorial tumors predominate during the first 2 years of life and late adolescence, while…

INTRODUCTION Background Demyelinating and inflammatory disorders are neuroimmunologic disorders in which there is an exaggerated immune response to the central nervous system (CNS, Fig. 6.1 ). Neuroimmune disorders have become increasingly recognized as more prevalent than was previously understood, and the understanding of these disorders is rapidly changing and continually evolving. Advances in autoantibodies detection, combined with imaging, clinical signs and symptoms, and cerebrospinal fluid (CSF)…

INTRODUCTION Background Central nervous system (CNS) infections can be due to multiple pathogens; however, viral and bacterial infections are the most common ( Table 5.1 ). The CNS injury that results from the infection depends on the timing of infection, pathogen, immune response, and medical treatment. TABLE 5.1 Etiologies of CNS Infections Developmental Stage Pathogenic Agents Congenital (Torch) Viral Cytomegalovirus (CMV) Toxoplasmosis Herpes simplex virus (HSV)…

INTRODUCTION Background Inherited metabolic disorders are challenging to diagnose because they have variable clinical severity and age at presentation, are uncommonly encountered, and can have a range of imaging appearances. The variability in clinical manifestation and imaging appearance relates to the underlying genetic mutation and degree of protein function remaining. Some metabolic disorders can have infantile, juvenile, and adult forms. Many of the metabolic disorders can…

HYPOXIC ISCHEMIC INJURY Key Points Background Hypoxic ischemic injury (HII) typically refers to a combination of a hypoxic and hypoperfusion injury to the fetal or neonatal brain. Clinical manifestations of HII include low Apgar scores (0–3 at 5 minutes and ≤5 at 10 minutes), umbilical cord pH < 7.1, poor cry, weak suck, seizures, diminished movement, absent neonatal reflexes, and hypotonia. Depending on the maturity of…

INTRODUCTION Brain malformations represent a disruption of normal development and can be caused by genetic, infectious, ischemic, and hemorrhagic factors. As seen in Table 2.1 , the time at which the insult occurs during development will determine the malformation. Because the majority of brain malformations occur early in development, the majority of malformations will be present when imaging is performed. Brain malformations are often first encountered…

INTRODUCTION The human brain is a fascinating and essentially miraculous structure with layers upon layers of complexity in its anatomic organization, microscopic connectivity, biochemical workings, and functions. No human can grasp all of the complexity that allows the brain to function, but that doesn’t stop us from trying! Learning about the brain’s development is particularly exciting, daunting, and exhausting. Understanding even just a small fraction of…