Introduction

The “long-term” care and complications related to the population of hydrocephalic and myelomeningocele patients have been infrequently considered. Effective treatment, and even survival, for these patients began in the late 1950s, and was not widespread until the mid 1970s. The first effective hydrocephalic shunt was introduced in 1955. Routine treatment of all myelomeningocele patients did not begin until the late 1970s, after repudiation of “selection” criteria. Only now is the oldest generation of patients approaching their 40s, and the largest cohort of patients is barely into the young adult years. There has been little work published on the long-term medical and care issues for these patients.

It is clear in the care of these patients that the natural history of their disease does not include a decline in function. Caregivers and patients must recognize that a loss of function is either a new problem, such as the development of a syrinx leading to scoliosis, or the morbid sequela of a potentially treatable event, such as an infection leading to brain injury. There is some overlap between these two groups of patients. The majority of myelomeningocele patients also have hydrocephalus, and require management of the problems associated with hydrocephalus in addition to the unique issues involved with myelodysplasia.

This monograph will briefly review the pertinent pathology and initial treatment of these conditions. The more typical problems associated with each diagnosis will be discussed. The evolving longer-term issues affecting these patients will be highlighted.