Jan 25, 2022
Today we drained an arachnoid cyst in the OR, and it was so beautiful. It’s like a swimming pool in the brain, and at the bottom we could see the internal carotid, the MCA, the optic nerve. Truly amazing.
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Today we drained an arachnoid cyst in the OR, and it was so beautiful. It’s like a swimming pool in the brain, and at the bottom we could see the internal carotid, the MCA, the optic nerve. Truly amazing.
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Individuals with spina bifida and/or Chiari malformation may develop a syrinx (collection of fluid in the spinal cord). The mechanism is thought to be blockage of CSF flow in the subarachonid space at the level of the foramen magnum --> venous congestion --> spinal cord edema --> collection of fluid in the potential space at the center of the spinal cord (syringomelia) or off-center in the cord (hydromelia). Image comes from an anesthesiology paper, Anand, et alia, "Anesthetic management of a patient with syringomyelia and Arnold-Chiari malformation type I with autonomic dysfunction," Anaesth Pain & Intensive Care 2015;19(3):408-411.
So I had orientation for the neurosurgery elective today, and they were talking about how the elective is a little more chill and said “we’ll torture you on the Sub-I haha.” Don’t really think it was a joke though.
Prognosis in spina bifida
The lower the spinal lesion, the better the prognosis. According to Medscape, "Approximately 75% of children with myelomeningocele have an IQ higher than 80. Among those whose intelligence is normal, 60% are learning disabled, with the most common feature being a nonverbal learning disability. Particular deficits are seen in mathematics, sequencing, visual perceptual skills, and problem solving. Prevalence of attention problems has been estimated to be 30-40%."
Source: Mark Forster, "Spina Bifida," Medscape (21 Sept. 2018).
Did you know?
Men and women with spina bifida can still have satisfying sex lives and their own families.
The lower the spinal level of the lesion (especially T12 or lower), the more likely the person can orgasm. Men with a lesion between T11-L2 typically can have neither reflexive nor psychogenic erections, but they can still benefit from erectile dysfunction drugs, a vacuum devise, or an internal prosthesis.
Women are typically fertile, and they should take 4g of folic acid for 1-3 months before getting pregnant and at least through the first trimester. Men may be infertile due to repeat UTIs. Or they may have fertile sperm but need assisted reproduction.
If one parent has spina bifida, there is a 3-5% chance of a child having it as well.
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Q. How is neurogenic bladder treated?
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A. The usual treatment for neurogenic bladder is clean intermittent catheterization (CIC, used by 90% of patients), typically four times a day. This may be augmented by anticholingeric medications to relax the detrusor muscle so it can hold more volume under less pressure and reduce urge incontinence; alpha-adrenergic agonists to tighten the sphincter muscle and reduce stress incontinence; and/or surgical procedures to increase the capacity of the bladder or to make catheterization easier.
Credit: J.F. Reybard, "Catheters," Credit: Wellcome Collection. Attribution 4.0 International (CC BY 4.0).
Mortality in spina bifida
Did you know? The leading cause of death in spina bifida used to be end-stage renal disease due to obstructive nephropathy due to neurogenic bladder. Now that bowel and bladder regimens are part of the care of individuals with spina bifida, the leading causes of death are sepsis secondary to wounds or urinary tract infections, followed by shunt malfunctions, central sleep apnea, and--increasingly, as this population survives into old age--cancer and other "natural causes."
Let's talk about bowel regimens. Individuals with high lesions tend to have better bowel function, which depends on an intact intestinal reflex; the lower the lesion, the more likely it will interrupt the lumbar and sacral plexus nerves required for gut peristalsis. For instance, anyone with lack of rectal tone will have trouble holding in an enema to make it maximally effective. So some people will get a stoma to do Mallone Anterograde Continence Enemas (MACE) from above. Or else they use a large-volume cone enema. Finally, consider using a "Squatty Potty" or similar "toilet stool" to encourage anatomical positioning and easier flow.
Pro-Tip: Did you know that the "Squatty Potty" is officially known as a "Defecation Posture Modification Device"?
Disclaimer: #TeachingRounds has not independently verified claims made about Squatty Potty or related products. We received no compensation of any kind for mentioning it and encourage you to do your own research