Myelomeningocele (open spina bifida) - surgical management

Abstract

Myelomeningocele has been recognized since ancient times although written descriptions began not before the 17th century. Among all serious congenital malformations, myelomeningocele is unique that is has a steady and considerable prevalence while being compatible with life. It has a dismal prognosis when left untreated where virtually all die within the first year while aggressive treatment have a profound effect on survival and quality of life. Effective surgical treatment became possible parallel to the treatment of hydrocephalus in the late 1950s. Advent of the shunt systems undoubtedly changed the morbidity and mortality rates due to associated hydrocephalus. Aggressive and effective treatment improved survival rates but also those suffering physical and mental disabilities have increased as well. Ethical and socioeconomic concerns have led to proposal for selective treatment criteria which have raised arguments on medical and ethico-legal rounds. After the swing of the pendulum between early treatment in all affected children and selective treatment of those who fulfilled the criteria for good prognosis, early myelomeningocele repair is practiced widely unless the infant is critically ill.Incidence of myelomeningocele has been decreasing especially in the Western world, partly due to prenatal diagnosis and elective terminations, dietary folate supplementation. Still, it is the most common central nervous system malformation and one of the leading causes of paraplegia, worldwide. Unfortunately, gains in the management of myelomeningocele have been mainly on antenatal diagnosis and prevention while efforts on understanding its cause, mechanisms involved are still tentative. Concerning the surgical management, no revolutionary modification improving outcome has been introduced unlike other fields of neurosurgery.Medical management of a child with myelomeningocele requires a lifelong effort of several disciplines including urology, orthopedics physical and social therapy besides neurosurgery. The initial and probably the most crucial step begin with proper repair of the lesion. The aim of surgery, with its simplest definition should be towards maintaining the medical condition of the newborn. In other words, consequences of an open spinal cord segment with associated malformations have to be avoided with appropriate measures. Comparable to the surgical treatment of any congenital malformation, myelomeningocele repair consist of reversing the failed steps of normal neural tube closure. This requires a thorough understanding of the normal and abnormal embryological sequence of events in formation of the spinal cord. Although the purpose of this chapter is to describe the basic concepts and technique of myelomeningocele repair, contemporary information and progress on epidemiology, and etiology and embryology is presented with discussion of controversial issues regarding the selection process, optimal time for surgery and technical modifications.