Background
In 2015, the Ehlers-Danlos Syndrome (EDS) Expert Panel met to clarify indications for neurosurgical intervention, which encompassed a variety of defects including cervical instability, Chiari Malformation, and syringomyelia; and recommended a craniocervical fusion with possible evidence of instability or brainstem/cord dysfunction.
Aims
A critical appraisal of the Panel’s report was conducted to evaluate whether recommendations were sufficient to establish clinical practice guidelines.
Methods
The appraisal relied on the AGREE II which includes 23 questions across six domains covering scope and purpose, stakeholders, rigor, clarity, applicability, and independence. Three independent reviewers were selected and after agreement statistics exceeded 0.75 scores were averaged across each domain.
Results
Overall, the Panel’s recommendations received a score of 43.5%, suggesting criteria were insufficient to establish best practice guidelines. The Panel did a good job of describing the sociopolitical context of the problem, but did not address reasons why indications require clarification. While the issue is complex, a large part of the problem centers around the approach to diagnosis, and specifically, limitations of conventional imaging, the lack of standardized craniometrics, and the limited outcome measures for neurological symptoms and functional pathology of the spine. The report was also missing detailed information about each defect, and not all defects subject to current debate were mentioned. The final recommendations also included vague qualifiers and did not discuss the health benefits or risks of the procedure. Recommendations were also not based on a systematic review of the literature, and methods of expert consensus were not described.
Conclusion
Understanding the clinical significance of radiological findings and decision-making regarding surgical candidacy hinge on more rigorous study, and necessarily includes a systematic review of the literature exploring novel diagnostic modalities and metrics. To capture enough studies, the population should be defined by the defect and not limited to EDS patients.