Medication Summary
The medications used most frequently in myelomeningocele are for treatment of neurogenic bladder dysfunction. These medications are used in conjunction with some form of bladder emptying technique to prevent upper urinary tract complications and to facilitate social continence. Among the drugs used are the following:
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Anticholinergics (oxybutynin chloride, hyoscyamine sulfate)
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Tricyclic antidepressants (imipramine hydrochloride; may act through anticholinergic effects)
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Alpha-adrenergic antagonists (terazosin)
Antispasmodic Agents, Urinary
Class Summary
These drugs competitively inhibit the binding of acetylcholine to the muscarinic cholinergic receptor, thereby suppressing involuntary bladder contraction of any etiology. In addition, they increase the volume at the first involuntary bladder contraction, decrease the amplitude of the involuntary bladder contraction, and, possibly, increase bladder capacity.
Oxybutynin chloride (Ditropan, XL, Gelnique, Oxytrol)
Oxybutynin chloride (Ditropan, XL, Gelnique, Oxytrol)
Oxybutynin exerts a direct antispasmodic effect on smooth muscle and inhibits muscarinic action of acetylcholine on smooth muscle. It is used to decrease bladder contractility and reduce detrusor-sphincter dyssynergia. Intravesical instillation of oxybutynin is associated with fewer side effects. A long-acting oral form is also available, for once-daily dosing.
Anticholinergics
Class Summary
Anticholinergics are used to suppress detrusor overactivity.
Hyoscyamine sulfate (Levsin, Levbid, Symax, Anaspaz, HyoMax)
Through parasympatholytic action, hyoscyamine relaxes smooth muscle spasms. It is indicated in the management of lower urinary tract disorders associated with hypermotility.
Tricyclic Antidepressants
Class Summary
Tricyclic antidepressants may act through anticholinergic effects.
Imipramine hydrochloride (Tofranil)
Imipramine has significant anticholinergic activity, as well as some alpha-adrenergic activity. These combined effects may improve bladder-urethral storage function.
Alpha-Adrenergic Antagonists
Class Summary
Alpha-adrenergic receptors are found in the bladder neck and urethra. Alpha-adrenergic antagonists decrease bladder outlet resistance, increase urinary flow rate, and improve bladder emptying.
Terazosin
Terazosin is an alpha 1-adrenergic blocking agent that decreases smooth muscle tone in the bladder neck, leading to reduction of bladder outlet obstruction without affecting bladder contractility. Its major side effects are postural hypotension and syncope, which can be avoided by starting at the lowest dose and increasing slowly. If terazosin therapy is discontinued for several days, restart using the initial dosing regimen.
Doxazosin mesylate (Cardura, Cardura XL)
Doxazosin is a selective inhibitor of alpha1-adrenergic receptors. Blockade of these receptors in the bladder neck decreases outflow resistance.
Alfuzosin (Uroxatral)
Doxazosin is a selective inhibitor of alpha1-adrenergic receptors. Blockade of adrenoreceptors relaxes smooth muscle in the in the bladder neck, which, in turn, decreases outflow resistance.
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The lumbar region of a newborn baby with myelomeningocele. The skin is intact, and the placode-containing remnants of nervous tissue can be observed in the center of the lesion, which is filled with cerebrospinal fluid.
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Myelomeningocele in a newborn.
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Coronal, T1-weighted magnetic resonance imaging (MRI) scans of the brain show a Chiari II malformation. Note the stretching of the brainstem, aqueduct, and fourth ventricle.
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Neonate with a lumbar myelomeningocele with an L5 neurologic level. Note the diaphanous sac filled with cerebrospinal fluid and containing fragile vessels in its membrane. Also, note the neural placode plastered to the dorsal surface of the sac. This patient underwent closure of his back and an untethering of his neural placode. The neural placode was circumnavigated and placed in the neural canal. A dural sleeve was fashioned in a way that reconstructed neural tube geometry.
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Sagittal, T1-weighted magnetic resonance imaging (MRI) scan of a child after closure of his myelomeningocele. Child is aged 7 years. Note the spinal cord ends in the sacral region far below the normal level of T12-L1. It is tethered at the point at which the neural placode was attached to the skin defect during gestation. The MRI scan showed dorsal tethering, and the child complained of back pain and had a new foot deformity on examination. By definition, all children with a myelomeningocele have a tethered cord on MRI, but only about 20% of children require an operation to untether the spinal cord during their first decade of life, during their rapid growth spurts. Thus, the MRI scan must be placed in context of a history and examination consistent with mechanical tethering and a resultant neurologic deterioration.
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Axial, T1-weighted MRI scan of a 15-year-old girl who was born with thoracic myelomeningocele, hydrocephalus, and Arnold-Chiari II syndrome. She was treated with a ventriculoperitoneal shunt. The ventricular system has a characteristic shape, with small frontal and large occipital horns, which are typical in patients with spina bifida. The shunt tube is shown in the right parietal region.
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Approach Considerations
- Bladder Management
- Bowel Management
- Bracing and Orthotics
- Physical Therapy
- Occupational Therapy
- Recreational Therapy
- Myelomeningocele Closure
- Shunting for Hydrocephalus and Syringomyelia
- Chiari Malformation Repair
- Orthopedic Procedures
- Consultations
- Long-Term Monitoring
- Prevention
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- Medication
- Media Gallery
- References
