International Pediatrics/Vol. 16/No. 1/2001
Spinal dysraphism refers to a group of congenital anormalies that is characterized by a failure of fusion of midline structures and it is frequently referred to as open or closed. Open lesion occur in early stage and are called spina bifida aperta or myelomeningocele. The closed defect, spinal bifida occulta, is covered by skin and may not be detected until later in life.
Neonates with a tethered spinal cord from spinal dysraphism may have a normal neurological exam. However, if the condition id undetected and untreated, these children, as they grow, may develop neurological deficits, orthopedic changes, urinary dysfunction or pain in the lower back or leg. It is postulated that the mechanical distortion of the spinal cord, as the child grows or during normal daily activities, contribute to the clinical picture.
occasionally the question arises that if the patient is neurologically intact and has a tethered spinal cord, is surgical intervention necessary? Mclone has shown that once motor deficits occur, opproximately 45% of patient return to normal following and untethering procedure. If the patient has developed urinary incontinence, then only 12% of those patients will become continent following untethering. It is accepted that once the diagnosis of a tethered spinal cord is made untethering is recommended prior to the development of the inevitable deterioration that is seen in the unoperaed patient.
Lipomyelomeningocele and diastematomyelia require more extensive surgical procedure. The goal of surgery in the former is to resect as much of the fatty mass as possible and free up the attachment of the fatty tissue, which has tethered the dura and the spinal cord. Technological advances, such as the CO2 laser and improved illumination and magnification, have increased surgical precision and reduced the surgical trauma.
Because most children with myelomeningocele also have hydrocephalus, treatment may include the implantation of a shunt, a tube that allows the excess cerebrospinal fluid to drain to other parts of the body.
Tethered cord syndrome is frequently seen in our neurological department as urological problems present as the initial signs or symptoms for this syndrome. Lumbosacral lipoma can be responsible for progressive neurological and urological defects. Surgery for untethering these structures may prevent late deterioration. Pierre-Kahn et al. routinely and systematically performed an operation even in the absence of neurological defects. Untethering surgery may help decompress and spare functional neural tissue and prevent retethering of the spinal cord. In our patients, the operation included untethering, removal of tumors, duroplasty, laminectomy. Although it delayed the release of our patients, effects reversed the loss of neurological function and thus prevented deterioration of renal function.
Tethered cord syndrome is a progressively worsening disorder. It may cause irreversible changes of neurological and urological complication if the diagnosis is delayed. Then early diagnosis and early operation of tethered cord syndrome is important. Since surgical intervention did not completely cure this condition during adulthood, it would be helpful to study ways to detect this disease early during childhood.