Tuesday, March 18, 2014

Spina bifida

Via Ann Althouse linking to a 
 a John Mellencamp interview

"They had to cut my head off basically, I have scar from ear to ear. That was my first experience." "You think circumcision's bad? Wait till they cut your fucking head off! But my grandmother told me for my entire life — everyday, 'John, you're the luckiest boy in the world.' And when you hear that everyday from somebody you actually start to believe it, which made me adventurous, which made me not afraid to try things, which made me not care about stopping myself from doing things that people said you shouldn't be doing." That's John Mellencamp, answering "What adventure changed your life?," the first question in an excellent interview.

the Spina bifida usually is fatal but at the lower spine. I assume he is talking about the problem of hydrocephalus. 

usually these are shunted with a tube that allows the fluid to drain into the blood stream or abdomen where it is absorbed.

 but the operation that slits you "from ear to ear" is the Arnold chiari malformation.

Surgical repair of myelomeningocele

Several surgical centers have attempted to lessen the effect of Arnold-Chiari II malformation, neurologic impairment, and need for shunting through the use of in utero surgical repair of the myelomeningocele. The first such repair was performed in 1997, after several animal models demonstrated a benefit from the procedure. Studies of the outcomes of these surgeries have been somewhat promising, although still inconclusive.[4, 5, 6]
In the Arnold Chiari malformation, the stem of the brain is pushed down into the hole at the base of the spine...it not only can stop the fluid from draining but can put pressure on the brainstem that regulates your breathing etc...there are several types. LINK

Type II is usually accompanied by a myelomeningocele—a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord and its protective membrane to protrude through a sac-like opening in the back. A myelomeningocele usually results in partial or complete paralysis of the area below the spinal opening. The term Arnold-Chiari malformation (named after two pioneering researchers) is specific to Type II malformations.

I haven't had any patients with meningomyelocoel that had this type surgery, but you do see this with other congenital problems, and so one of my patients with achondroplasia (a form of dwarfism) had this surgery.

What other disorders can occur with Chiari malformations?
   Chiari malformations can occur with other conditions. Some are:
     * hydrocephalus (excessive fluid in the brain)
     * spina bifida (opening of the spine usually associated with an
       abnormality of the spinal cord)
     * syringomyelia (excessive fluid in the spinal cord, leading to a
       cavity detectable by MRI).
     * other conditions, including some inherited conditions like
       achondroplasia (a type of dwarfism), Hajdu-Cheney syndrome,
       Albright hereditary osteodystrophy (pseudohypoparathyroidism), or
       other rarer syndromes.

 He had the hole in the base of his skull made larger. Article here.

this article has photos.

no, it's outside my area of expertise.

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