Aulana’s insides were compacted because of something she was born without, but a special surgery is giving her stomach some much needed space.

Aulana Hulbert has to walk on her hands to get around. She can’t use her legs.

“She has no spine from the rib cage to the top of her pelvis and because of this her abdominal contents are squeezed,” David W. Polly, Jr., MD, Professor and Chief of Spine Surgery at the University of Minnesota and Chief of Spine Surgery at Twin Cities Shriners Hospital for Children, told Ivanhoe.

It makes it hard for Aulana to eat and control her bladder and bowels, but a rare surgery could help.

“I won’t be squishing my guts anymore,” Aulana told Ivanhoe.

“Previously, the described treatment of his had been to amputate the legs and then to use the leg bones to create a spine,” Dr. Polly said.

However, Dr. Polly is not taking Aulana’s legs.

“We figured out a way to build a spine for this child,” Dr. Polly said.

Donor bone helps connect the spine to the pelvis. Then, one of aulana’s ribs becomes a part of the new spine.

“We cut a segment of the rib, leaving the blood vessels attached, and swing the rib down to give blood supply to the bone to give it a chance to heal. They will all grow together and become her bone over time,” Dr. Polly said.

Now, she has about six more inches of spine.

Aulana still walks on her hands, but the extra bone is making things better for her belly and her ability to sit up without using her hands.

“Also, she’s finding it a little easier to eat now and she has a little better bowel and bladder control,” Dr. Polly explained.

Besides aulana, only one other person in the world has had this procedure. Dr. Polly performed both operations. He tells us Aulana will likely have her spine lengthened one or two more times to give her internal organs the room they need as she grows. While her condition is rare, Dr. Polly says it’s related to maternal diabetes. As diabetes rates increase, he tells us he wouldn’t be surprised if the abnormality becomes more common.

 

BACKGROUND:  Caudal regression syndrome (CRS) is a rare congenital disorder in which the development of the lower half of the body is impaired, often causing the vertebrae of the lower spine to be misshapen or missing. The disorder can also cause malformed legs or feet, small hips, as well as other problems, although the severity of impairment varies between individuals. What causes the syndrome is not yet understood, but there is an association with maternal diabetes, and one out of every 350 infants born to mothers with diabetes has the condition. (Source: www.nlm.nih.gov)

 

SYMPTOMS: Individuals with CRS can have various problems associated with the disease, and the severity of these issues can also be very different between individuals, but some common symptoms include:

 

  • Malformed Kidneys – This can mean a missing kidney, kidneys that are fused together, or duplication of the tubes that carry urine from the kidney to the bladder.
  • Malformed Legs or Feet – CRS patients can have clubbed feet, loss of sensation in the legs, or outward or upward-turning feet. It is also common for the leg bones to be underdeveloped, making walking difficult for some people. 
  • Difficulty with Bladder and Bowel Control – The inability to completely control bladder and bowel function is often due to patients having an abnormal twisting of the large intestine or other malformations in the gastrointestinal tract. (Source:

    www.nlm.nih.gov)

 

NEW TECHNOLOGY:  The first lifesaving surgery in 2012 for this type of problem used to come at the cost of a patient’s lower leg.  In one operation at Birmingham Children’s Hospital, a 5-year-old girl had a 10-centimeter gap in her spine.  She was born with a rare disorder called spinal segmental dysgenesis.  Five bones that made part of her spine were missing.  Her legs were also contorted against her stomach, and she had little feeling in them.  Like Aulana, she was running out of room in her chest and was at risk of her organs being crushed. Surgeons amputated from her knee down, and a section of bone was taken to bridge the gap in her spine. Two metal rods were bolted to the upper spine and the hips in order to provide extra support. The procedure took 13 hours.  (Source: http://www.bbc.co.uk/news/health-20426413)

 

David W. Polly, Jr., MD, Professor and Chief of Spine Surgery at the University of Minnesota and Chief of Spine Surgery at Twin Cities Shriners Hospital for Children, talks about a new advancement in spine surgery.