After she had suffered severe headaches for years, the family of six-year-old Allie Barone took her to a specialist in July only for doctors to tell them that their little girl’s skull was too small for her brain.
An MRI revealed that Allie had Type I Chiari malformation, a condition which affects one percent of the population and means the base of her skull was too small to accommodate her growing brain.
Allie, from Clinton, New York faced potential paralysis as her spinal fluid could not flow freely into her skull and so her mother checked her into the Children’s National Medical Center in Washington D.C. for a series of dangerous neurological procedures.
Allie Barone after one of her recent surgeries at the Children’s National Medical Center in Washington D.C.
‘Her brain stem was compressed and squished through the opening of the base of her skull, where the skull meets the spinal cord; it’s supposed to be wide open,’ said Allie’s mother Stephanie Barone.
‘When the spinal fluid couldn’t get into her brain, that’s why she had the headaches.’
Checking into the hospital on August 1st, Allie and her family hoped that the team at the hospital would cure her of the crippling headaches which had plagued her and would be made worse by sudden movements such as running or sneezing.
‘Essentially, what happens, the cerebellum tonsils are pushing through the bottom of the skull and potentially putting pressure on the spinal cord and the tissue of the spinal cord,’ said Dr. Robert Keating, professor and chief of neurosurgery at Children’s National Medical Center.
Allie underwent four surgeries on the back of her skull and neck that left her in hospital for almost a month but ended succesfully
Doctors used beaded stitches to bind Allie’s neck and skull back together after three previous attempts to stop spinal fluid leaking failed
During the first procedure that Allie went through Dr. Keating cut into her neck and removed bone that was obstructing the access of the spinal fluid using ultrasound – the whole process took him four hours.
In a demoralising setback for the family though their hopes were dashed when Allie began leaking spinal fluid from the initial incision four days after the first procedure – so Dr. Keating took her back into surgery.
‘Now that we had opened the covering and put a patch on, it was leaking through the patch,’ said Dr. Keating.
What are Chiari Malformations?
- Around one percent of the U.S. population has a Chiari malformation and increasingly adults in their 20s and 30s are being diagnosed after complaining of headaches and pressure build ups at the base of their skulls.
- Chiari malformations are caused by structural defects in the brain and spinal cord, according to the National Institute of Neurological Disorders and Stroke, or can occur if too much spinal fluid is drained from the back in cases of an injury.
- Not all Type I Chiari malformations present with symptoms and it is the least serious form.
- Type II usually involves a form of spina bifida; Type III is the most dangerous and causes severe neurological defects
- Type IV patients are born horribly disfigured and may have parts of the cerebellum missing and parts of the skull and spinal cord may be visible to the naked eye.
‘Our goal was to go in and reinforce the stitches, and at the same time, place a tube in her lower back so we could divert the spinal fluid and let the neck heal.’
For the next few days Allie’s condition improved before another set-beck sent the little into surgery for the third time as her spinal fluid began to leak again.
‘The feeling is, ‘Why are we having issues?” said Keating.
‘The answer was, she had hydrocephalus, or too much water in the brain. We needed to put a shunt in to remove the excess water.’
Once that procedure was completed, Stephanie and the Barones thought that they would be able to bring their daughter home.
However, as they were signing the discharge papers, Allie bent down to complete a drawing, her favourite pastime and she noticed her neck was leaking fluid again.
‘She’d look down at her drawing, and then look up; and it would drip down the back of her neck,’ said Barone.
‘She just said, ‘It’s dripping again, Mommy;’ and we both knew it meant we weren’t leaving the next morning, and that she needed another surgery.
‘It was a heartbreaking hour for both of us, and then turned out to be what finally helped the doctors find the problem and get us home.’
However, Dr. Keating handed over the fourth surgery to a colleague who had the breakthrough of performing the procedure on Allie with her sitting up.
This allowed doctors to notice that her second vertebrae was abnormally shaped and was acting like a knife, scraping the dura (the outer layer of three membranes that surround the brain and spinal cord).
‘It was another abnormality that no one knew about,’ said Barone.
Allie Barone (left) her mother Stephanie (centre) and her sister pose for a picture posted to Stephanie’s Facebook account
‘She started school (last week) and she has more energy than ever. She’s like a whole new kid.’
With her head and neck held together with beaded stitches, Allie is due back in Washington D.C. for a checkup at the end of September when they will be removed.
After that the sprightly six-year-old is looking forward to returning to her favourite activity – skiing with her little sister.
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