Espina bífida / Spinal dysraphism

Trastorno también llamado mielodisplasia, donde existe un desarrollo anormal de los huesos de la columna, de la médula espinal, del tejido nervioso circundante y de las meninges; y que ocurre aproximadamente entre los días 26 a 28 de la gestación. Se describen su etiología, clasificación, aspectos del diagnóstico prenatal, pronóstico y tratamiento, y prevención.

Rehabilitation of patients with spina bifida.

Patients with spina bifida present with multifarious problems requiring multi-disciplinary approach for their rehabilitation. 172 patients of spina bifida attending the Department of Physical Medicine and Rehabilitation at A.I.I.M.S., New Delhi, were studied retrospectively to ascertain the problems posed by them while presenting for rehabilitation. Male:female ratio was 1.23:1. Lumbo-sacral lesion comprised 41.3% followed by lumbar (26.1%) and sacral (22.1%). Common spinal deformities observed were Kyphoscoliosis (62.2%) and increased lumbar lordosis (19.2%). Clinically, active signs and symptoms of hydrocephalus were found in 47.7% associated with mental subnormality in 22.1%. Varying degrees of weakness of the muscles of lower limbs were detected in 98.3% cases, spasticity was found in 10.5% and ataxia in 2.9%. Improvement of muscle power was noticed in 45.3% during first three years and deterioration in 10.5% due to various complications. Foot deformities were commonest amongst deformities. Mobility was affected in 55.8% while 62.2% could attain independence. Bladder symptoms were present in 75% cases and bowel symptoms in 26.7%. Anxiety and guilt amongst parents was high in the families. Main hindering factors in vocational rehabilitation were bladder control, ambulation and pressure sores.

Imaging in hydrocephalus and spinal dysraphism.

Hydrocephalus and spinal dysraphism are two commonly encountered clinical entities where imaging plays a vital role. Sonography, Computed Tomography and Magnetic Resonance Imaging are the modalities used for the evaluation of hydrocephalus. Ultrasound is useful in small infants with open fontanella, is non-invasive and can be performed at cribside. However, it has limitation in identifying the etiology. CT is the most cost effective modality in the evaluation of hydrocephalus and gives detailed anatomical information. MRI offers the added advantage of multiplanar display and can also assess stenosis/patency of CSF pathways, using phase contrast cine techniques. MRI is the modality of choice for evaluating spinal dysraphism and gives excellent information regarding contents of back mass, spinal cord status and associated anomalies like Chiari malformation. Myelography coupled with CT should be reserved for equivocal cases or in centres where MRI is not available.

Spina bifida.

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.