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Spina bifida is a complex birth defect affecting the central nervous system, leading to varied spinal cord and brain abnormalities. It often results in physical challenges, including limb weakness and orthopedic issues, impacting long-term health.
Area of Science:
- Pediatric Neurosurgery
- Developmental Biology
- Neurology
Background:
- Spina bifida is the most frequent central nervous system birth defect in pediatric neurosurgery.
- It involves developmental abnormalities of the vertebrae, spinal cord, and associated neural structures.
- Severity ranges from minor spinal defects to complex myelomeningocoele with associated brain anomalies.
Purpose of the Study:
- To define spina bifida and its spectrum of central nervous system involvement.
- To outline the common multisystem complications associated with spina bifida.
- To highlight its complexity as a developmental defect compatible with long life.
Main Methods:
- Review of characteristic developmental abnormalities.
- Description of the range of spina bifida manifestations.
- Analysis of associated neurological and musculoskeletal sequelae.
Main Results:
- Spina bifida encompasses the entire central nervous system, with variable severity.
- Common complications include lower extremity weakness, orthopedic problems, pressure sores, and anorectal/urologic dysfunction.
- Associated conditions can include Chiari malformation and hydrocephalus.
Conclusions:
- Spina bifida presents a wide spectrum of neurological and physical impairments.
- Multisystem involvement underscores its complexity.
- It is a significant developmental defect compatible with extended survival.
Abstract:
Spina bifida is the most common central nervous system birth defect encountered by the pediatric neurosurgeon. It is defined by characteristic development abnormalities of the vertebrae and spinal cord and associated changes in the cerebrum, brainstem and peripheral nerves. The expression of spina bifida encompasses the entire central nervous system, ranging in severity from merely an absent spinous process with normal intraspinal structures to the other extreme of myelomeningocoele, Chiari malformation, hydrocephalus, and cortical cytoarchitectural changes. Most children with myelomeningocoele have some degree of weakness of their lower extremities and many have significant orthopaedic problems. As a result of denervation, muscle imbalance ensues and can result in abnormalities at the hip, knee and foot. Anesthesia of various portions of the skin can lead to pressure sores, particularly later in life. Anorectal neuropathy may cause a variety of defecatory dysfunctions. Urologic abnormalities are also common. These multisystem abnormalities associated with spina bifida contribute to its widely accepted identity as the most complex development defect compatible with long life.