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Holoprosencephaly (HPE) is a complex congenital brain malformation characterized by failure of the forebrain to bifurcate into two hemispheres, a process normally completed by the fifth week of gestation. Modern high-resolution brain magnetic resonance imaging (MRI) has allowed detailed analysis of the cortical, white matter, and deep gray structural anomalies in HPE in living humans. This has led to better classification of types of HPE, identification of newer subtypes, and understanding of the pathogenesis. Currently, there are four generally accepted subtypes of HPE: alobar, semilobar, lobar, and middle interhemispheric variant. These subtypes are defined primarily by the degree and region of neocortical nonseparation. Rather than there being four discrete subtypes of HPE, we believe that there is a continuum of midline neocortical nonseparation resulting in a spectrum disorder. Many patients with HPE fall within the border zone between the neighboring subtypes. In addition, there are patients with very mild HPE, where the nonseparation is restricted to the preoptic (suprachiasmic) area. In addition to the neocortex, other midline structures such as the thalami, hypothalamic nuclei, and basal ganglia are often nonseparated in HPE. The cortical and subcortical involvements in HPE are thought to occur due to a disruption in the ventral patterning process during development. The severity of the abnormalities in these structures determines the severity of the neurodevelopmental outcome and associated sequelae., (2010 Wiley-Liss, Inc.)

Current and advanced structural and functional neuroimaging techniques are presented along with guidelines for utilization and principles of imaging diagnosis in fetal and neonatal central nervous system abnormalities. Pattern of injury, timing issues, and differential diagnosis are addressed with emphasis on neurovascular disease. Ultrasonography and computed tomography provide relatively rapid and important screening information regarding gross macrostructural abnormalities. However, current and advanced MRI techniques often provide more definitive macrostructural, microstructural, and functional imaging information.

Unlabelled: BACKGROUND. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management., Materials and Methods: A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2-11 years; mean = 5.2 years)., Results: Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5-7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2-11 years). There has been one death. The majority of tumors were pilocytic astrocytomas., Conclusion: Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.

A curvature abnormality may be the initial or major presenting feature in a child with disease of the spinal column or spinal neuraxis. A simplified classification of common spinal curvature abnormalities of childhood include idiopathic, congenital/dysraphic, skeletal dysplasia, neurofibromatosis, and painful. The great majority of childhood scoliosis falls into the idiopathic category. Atypical clinical or radiographic features in a presumed idiopathic scoliosis may indicate an otherwise occult tumor or hydrosyringomyelia, or may be a consequence of increasing curvature with disk protrusion, nerve impingement, or cord attenuation. Neuroimaging beyond plain films is commonly necessary for atypical idiopathic scoliosis and for the other categories of scoliosis listed.

Vascular anomalies of the head and neck are common lesions of childhood. The vascular anomalies can be divided into hemangiomas and vascular malformations. Each of these lesions has a characteristic imaging appearance. Correctly classifying the anomaly is essential in directing the treatment of these lesions.

Disorders involving the temporal bone in children may be associated with considerable morbidity. Hearing loss occurring during childhood may impair cognitive development and should therefore be diagnosed and treated as soon as possible. Thin section high resolution CT and MR imaging of the temporal bone provide critical information about a variety of developmental, inflammatory, traumatic, and neoplastic disorders, and are used for diagnostic purposes and to help determine therapeutic options.

This article focuses on the neuroendocrine disorders of childhood. The commonly associated neuroradiologic abnormalities of the hypothalamic-pituitary axis are covered in detail.