Primary (congenital) encephalocele
- Tadanori Tomita, MD
Tadanori Tomita, MD
- Yeager Professor and Division Head of Pediatric Neurosurgery,
- Director, Falk Brain Tumor Center
- Ann & Robert H. Lurie Children’s Hospital of Chicago;
- Professor and Vice-Chair, Department of Neurosurgery,
- Northwestern University Feinberg School of Medicine
- Hideki Ogiwara, MD, PhD
Hideki Ogiwara, MD, PhD
- Assistant Chief, Division of Neurosurgery
- National Center for Child Health and Development, Tokyo, Japan
- Section Editors
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
- Leonard E Weisman, MD
Leonard E Weisman, MD
- Section Editor — Neonatology
- Professor of Pediatrics
- Baylor College of Medicine
An encephalocele is a protrusion of the brain and/or meninges through a defect in the skull (cranium bifidum) that is "closed" or covered with skin. Encephalocele is one of the three most common neural tube defects (NTDs). The epidemiology, clinical features, diagnosis, and management of encephaloceles are discussed in this topic review.
The other main types of NTDs are anencephaly and myelomeningocele, which are discussed in separate topic reviews:
●Anencephaly, which is an open NTD as the affected region of the cranial neural tube is exposed to the body surface. It is a severe defect, and is not compatible with survival. (See "Anencephaly".)
●Myelomeningocele, which is characterized by a cleft in the vertebral column, with a corresponding defect in the skin so that the meninges and spinal cord are exposed. (See "Pathophysiology and clinical manifestations of myelomeningocele (spina bifida)" and "Overview of the management of myelomeningocele (spina bifida)".)
In this topic review we will use the term "encephalocele" to describe lesions that include brain and/or meninges. Some authors use the more general term "cephalocele," and reserve the term "encephalocele" for lesions that include brain and "meningocele" for those that include only meninges .To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- Primary encephalocele
- Secondary encephalocele
- CLINICAL FEATURES
- ASSOCIATED ANOMALIES
- Prenatal diagnosis
- Differential diagnosis
- Sincipital encephaloceles
- Basal encephaloceles
- Occipital encephaloceles
- SUMMARY AND RECOMMENDATIONS