Surgery to correct spinal abnormalities does not appear to pose additional risks for bleeding or postoperative complications related to wound healing in children with Ehlers-Danlos syndrome (EDS), compared with those without EDS having the same procedure, an analysis revealed.
The study, “Complications in Children with Ehlers-Danlos Syndrome following Spine Surgery: Analysis of the Pediatric National Surgery Quality Improvement Program Database,” was published in the journal World Neurosurgery.
EDS is a genetic disease that affects connective tissues throughout the body, resulting in hypermobile joints and fragile skin. Because of the hypermobility of joints, EDS patients are prone to developing spinal deformities.
In patients who do not respond to conservative treatments, corrective surgery is sometimes needed to secure unstable parts of the spine.
Due to the increased fragility of tissue in people with EDS, reports have suggested that these patients may be at a higher risk for bleeding and wound healing complications following surgery. However, given the rarity of EDS, these reports may not represent the actual risk.
To get a clearer picture of the rate of complications arising from spinal surgery in EDS patients, researchers analyzed the American College of Surgeons Pediatric National Surgical Quality Improvement Program database, which contains information from more than 50 pediatric hospitals in the U.S.
The team also analyzed the patients’ general level of disease using the American Society of Anesthesiologists (ASA) classification, cardiac risk factors, and additional abnormalities of the central nervous system.
Of the 369,176 patients in the database, 279 had EDS. Of those, 56 (20%) had undergone a spine procedure. The mean age of these patients at the time of surgery was 11.59 years old, and 54% were female.
For comparison purposes, the 56 EDS patients were matched with 21,434 patients without EDS who underwent the same surgical procedure.
Complications were defined as any deviation from the expected postoperative outcomes within 30 days after surgery, such as problems with bleeding, wound healing, wound disruption, graft failure, and infections.
Of the 56 EDS patients, 37 had some type of spinal fusion surgery (arthrodesis). Some of these patients had large multilevel spinal fusion — with 14 patients having more than 13 levels of vertebrae fused, and 15 a fusion of seven to 12 levels.
Results showed that, despite 41% of the EDS patients requiring blood transfusions, there was no significant difference in transfusions compared with non-EDS patients undergoing the same surgical procedure. The amount of blood transfused was also the same between the two groups.
There were no differences in terms of number of fusion levels, duration of anesthesia, length of operation time, and length of hospital stay (number of days from operation to hospital discharge) between the two groups. There were also no reported cases of neurological complications after surgery.
Among EDS patients, no hospital stay lasted longer than 30 days, and there was no 30-day mortality.
Wound-related complications were also not significantly different between EDS and non-EDS patients, and only one patient contracted pneumonia, which was the only reported infection.
One EDS patient had a pulmonary embolism (blood clot in lung arteries). “However, given that there was only one instance it is unclear if this truly represents an increased risk of pulmonary embolism in this population,” the researchers wrote.
Of note, before surgery, EDS patients had a worse level of general disease, with an ASA classification of three or higher; and a strong association was found between EDS patients and cardiac risk factors — suggesting an increase in surgical risk in EDS patients with these conditions.
“Overall, these results suggest that with the usual standard of perioperative care, spine surgery in pediatric EDS patients does not carry additional risk over the general populations for bleeding or postoperative complications related to wound healing,” the researchers concluded.
“Future research is needed to address whether the data presented here is truly generalizable to all subtypes of EDS, and whether complications appear in long-term follow up,” they added.