Myelomeningocele: neglected aspects
1Adolescent Urology, University College, 235 Euston Road, London, UK. bjui@ucl.ac.uk
Insights
Myelomeningocele is the primary cause of neurogenic bladder in children. Early management and preparation are crucial for independence, as adult complications like heart and lung disease are common.
Area of Science:
- Pediatric Urology
- Neuroscience
- Rehabilitation Medicine
Background:
- Myelomeningocele is the leading cause of neurogenic bladder in children, impacting long-term survival and quality of life.
- Adults with myelomeningocele face significant health challenges, including cardiovascular and pulmonary diseases, with mortality rates increasing by age 35.
Purpose of the Study:
- To outline the long-term implications of neurogenic bladder due to myelomeningocele in children.
- To emphasize the critical need for early intervention and comprehensive preparation for adult independence.
Main Methods:
- Review of long-term outcomes and complications associated with myelomeningocele.
- Analysis of factors influencing adult independence, including mobility, continence, education, and employment.
- Assessment of reproductive health and risks in affected individuals.
Main Results:
- Progressive physical deterioration, increased wheelchair dependency, and surgical risks are common in adults, particularly those with thoracic lesions.
- Early establishment of bladder management and comprehensive childhood preparation are vital for adult independence and quality of life.
- While sexual function and fertility can be affected, many achieve partnerships, with a high risk of neural tube defects in offspring requiring folic acid prophylaxis.
Conclusions:
- Effective management of neurogenic bladder in childhood is paramount for improving long-term outcomes and enabling independence in adulthood.
- Holistic preparation encompassing physical, educational, and social aspects is essential for individuals with myelomeningocele.
- Reproductive health requires careful consideration, with proactive measures to mitigate risks for future generations.
Abstract:
The commonest cause of neurogenic bladder in children is myelomeningocele. Survival of children is much improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate with age, especially in those with thoracic lesions. Those who walk in childhood have a 20-50% chance of becoming wheelchair dependent as adults. Immobility, poor respiratory reserve, obesity, latex allergy and worsening kyphoscoliosis contribute to the increased risks of surgery. It is essential that safe and manageable urine drainage is established in childhood: the bladder never improves with time, and surgical reconstruction becomes progressively more difficult. Independence in adult life will only be possible with intense preparation in childhood. Children must be allowed to join in with family chores and events. Education, both academic and practical, must be encouraged. Skills such as driving, shopping and birth control must be taught. However, even with the best support, less than 40% will have gainful employment. Children who are continent and have lesions below L2 are likely to have normal sexual function. Sexual activity in adolescents, especially in those with hydrocephalus, is limited (but not absent). However, by adult life, about two thirds will have established a regular partnership. All females and those males who are naturally potent are likely to be fertile. There is a high risk of neural tube defects in their offspring unless the female partner takes prophylactic folic acid for 3 months before pregnancy and for first trimester.
