Cerebellar Disorders in Children – Chapter 13: Chiari Type II Malformation (ebook)


Chapter 13 – Chiari Type II Malformation

Michael S. Salman

Chiari type II malformation (CM2) occurs almost universally in neonates born with myelomeningocele (MMC), the most common and severe type of spina bifi da (Wagner et al. 2002; Bowman et al. 2009). CM2 is a congenital anomaly that involves the cervical spinal cord, midbrain, and hindbrain (i.e., pons, medulla, and cerebellum) (Harding and Copp 2002; Barkovich 2005). In this chapter, historic aspects and the epidemiology of CM2 are highlighted, then the theories, characteristic features, and the clinical presentation of CM2 are discussed. The following sections deal with investigations, management, and prognosis of CM2. Issues related to the spinal cord lesion in MMC and its effects on lower limbs, tethered cord, renal and bowel functions are beyond the scope of this chapter.

Please note that Chapters 7-22 are also available in Part 4: Malformations.

About the Complete Book:

This clinically orientated text by an international group of experts is the first definitive reference book on disorders of the cerebellum in children. It presents a wealth of practical clinical experience backed up by a strong scientific basis for the information and guidance given. The first part sets out the theoretical underpinnings of cerebellar disorders. This is followed by sections on clinical conditions grouped according to common characteristics such as aetiology and symptomatology. The descriptions of the clinical conditions each systematically cover, as appropriate, epidemiology, prevalence, diagnostic criteria, clinical features (including course and prognosis), pathophysiology, genetics, investigations, differential diagnosis, and management and treatment. This book will be an invaluable resource for all those caring for children affected by cerebellar disorders, including malformations, genetic and metabolic disorders, acquired cerebellar damage, vascular disorders and acute ataxias. This comprehensive reference text on cerebellar disorders in children includes chapters on cerebellar development, prenatal cerebellar imaging, imaging of the posterior fossa, with coverage of a broad range of malformations, genetic and metabolic disorders involving the cerebellum, prenatal cerebellar disruptions (as related to prematurity), vascular disorders, tumors and paraneoplastic syndromes, as well as acute ataxia and trauma to the posterior fossa. Numerous checklists are provided to assist in the differential diagnosis of clinical signs and neuroimaging findings.

Paediatric neurologists, paediatricians, neurologists, developmental paediatricians, neuroimaging specialists, geneticists, neonatologists

Clinics in Developmental Medicine No. 191-192