Study Reveals Similar Spinal Surgery Outcomes in Rett Syndrome and Cerebral Palsy

Study Reveals Similar Spinal Surgery Outcomes in Rett Syndrome and Cerebral Palsy
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Patients with Rett syndrome have similar spinal surgery outcomes to those of people with cerebral palsy (CP), according to new research.

The findings also revealed comparable degrees of spine abnormalities before surgery, as well as similar duration of hospital stays.

The study, “Surgical outcomes in Rett syndrome patients are comparable to cerebral palsy patients,” was published in The Spine Journal and will be presented at the upcoming 35th Annual Meeting of the North American Spine Society, which will be held online.

Rett syndrome and CP patients often need surgery to correct scoliosis, a term that refers to a sideways curvature of the spine.

Although limited, the medical literature of Rett syndrome includes reports of major surgical complications, such as respiratory failure, prolonged intubation, infections, significant blood loss, and prolonged hospital stays.

CP patients frequently are nonverbal and unable to walk. They also often experience respiratory and surgical complications, suggesting that the two patient groups may be similar.

Most surgeons, however, have more experience treating CP patients than people with Rett.

A team of researchers from New York conducted a review of 27 Rett and 78 CP patients who underwent posterior spinal fusion surgery from 2005 to 2018. During this type of surgery, rods, hooks, wires, or screws are attached to the curved part of the backbone to straighten the spine. Small pieces of bone, usually from the pelvic bone, are then put over the spine, where they will grow with the spinal bone to fuse it it into the proper position.

The two groups showed no significant differences in preoperative Cobb angle — a measure of side-to-side spinal curvature — a forward bending of the back called kyphosis, or pelvic misalignment, also known as pelvic obliquity.

Other similarities included the fixation points where bones are fused, time to having breathing tubes removed after surgery (extubation), the rate at which each group experiences complications, and the lengths of ICU or hospital stays. Patients in both groups also spent similar time in surgery

Yet, people with CP had significantly more estimated blood loss, blood transfusions during surgery, and longer anesthesia time. Cobb angle after surgery was significantly higher among CP patients, but kyphosis and pelvic obliquity remained similar.

Significant differences included both pre- and post-surgery coronal decompensation, or a shift in the spine away from midline.

The investigators found no differences between non-ambulatory Rett and CP patients.

“RS patients are comparable to CP patients in terms of surgical, periop [during surgery], and radiographic outcomes,” the team concluded.

Forest Ray received his PhD in systems biology from Columbia University, where he developed tools to match drug side effects to other diseases. He has since worked as a journalist and science writer, covering topics from rare diseases to the intersection between environmental science and social justice. He currently lives in Long Beach, California.
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José holds a PhD in Neuroscience from Universidade of Porto, in Portugal. He has also studied Biochemistry at Universidade do Porto and was a postdoctoral associate at Weill Cornell Medicine, in New York, and at The University of Western Ontario in London, Ontario, Canada. His work has ranged from the association of central cardiovascular and pain control to the neurobiological basis of hypertension, and the molecular pathways driving Alzheimer’s disease.

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Forest Ray received his PhD in systems biology from Columbia University, where he developed tools to match drug side effects to other diseases. He has since worked as a journalist and science writer, covering topics from rare diseases to the intersection between environmental science and social justice. He currently lives in Long Beach, California.
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  • biomarkers
  • spinal surgical outcomes

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