Myelomeningoceles in Children Homepage

 

Authors

Jeffrey Campbell, M.D.

Ricardo Gepp, M.D.

Timothy George, M.D.

Christopher Baggott, M.D.

Solomon Ondoma, M.D.

Section Editors

Bermans Iskandar, M.D.

Graham Fieggen, M.D.

Senior Editor

Ann Ritter, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

Open neural tube defects include anencephaly cranially and myelomeningocele caudally. Spinal dysraphism refers to incomplete closure of one or more vertebral arches. While a deficit in primary neurulation leads to anencephaly or myelomeningocele, which fall into the category of open spinal dysraphism, failure in secondary neurulation or disjunction leads to closed spinal dysraphic anomalies.

The incidence of open neural tube defects cannot be known. However, the prevalence of myelomeningocele is approximately 18 per 100,000 live births in the United States. The success of maternal periconceptional folate supplementation has shifted the paradigm from treating neural tube defects to preventing neural tube defects. Despite the falling prevalence in industrialized countries, myelomeningocele remains a serious public health problem, particularly in developing countries.

Because life expectancy has increased considerably within the last 60 years, care for adolescents and adults with myelomeningocele has become a new challenge. Comprehensive, life-long care from orthopedists, urologists, physiatrists, physical therapists, occupational therapists, and neurosurgeons is essential and is often provided at multidisciplinary clinics.

Key Points

• Life-long management required: Life-long multidisciplinary care is essential to maximize quality of life, given the incidence of secondary complications and potential for failure of some of the perinatal treatments.
• Myelomeningoceles remain a significant health problem: In spite of the decreased prevalence of myelomeningocele in developed countries over the past three decades, myelomeningocele remains a common and costly disease in developing countries.
• Hydrocephalus: Hydrocephalus is common (~80%) in children born with myelomeningoceles.
• Fetal closure may be of benefit: Intrauterine closure of myelomeningocele has been shown to reduce the incidence of Chiari II malformation and hydrocephalus. Risk-benefit assessments of this technique may lead to changes in practice, but this procedure is still a management under experimental investigation.