Author(s)
Taylor Jamil, MD, MPH
Caitlin Blades, MS
Kenny Chan, MD
Affiliation(s)
University of Colorado - Anschutz; Children's Hospital of Colorado;
Abstract:
INTRODUCTION: Ehlers-Danlos syndrome (EDS) is an inherited collagen disorder that, based on the subtype, comes with varying risks of life-threatening hemorrhage. The risk of post-operative adenotonsillectomy (T&A) hemorrhage should be scrutinized in patients with EDS. Better risk assessment will aid in medical optimization prior to surgery, counseling families of surgical risks, and may decrease admission costs in lower-risk EDS patients.
OBJECTIVES: Describe the bleeding risk in patients with EDS compared to patients without EDS in post-T&A hemorrhage.
METHODS: This was a retrospective study of children receiving a T&A with a diagnosis of EDS compared to a random selection of over 25,000 patients without a diagnosis of EDS who received a T&A in the past 10 years at pediatric academic center. Admission criteria via the clinical practice guidelines was analyzed. Bleeding risk in children with EDS was assessed by EDS subtype, prior echocardiograms, electrocardiograms, and laboratory data.
RESULTS: Of 62 patients with EDS and 72 controls, those with EDS were more likely to be female (p=0.033), younger (p=0.051), non-Hispanic (p<0.001), and white (p=0.01). Patients with EDS were more likely to have pre-operative bleeding symptoms (p<0.001), other bleeding disorders or anticoagulation use (p<0.001) and have a family history of bleeding disorders (p=0.023) compared to controls. Patients with EDS had no significant increase in post-tonsillectomy hemorrhage (p=0.118). This was also true when controlling for a personal or family history of bleeding disorders (0.380).
CONCLUSION: Patients with EDS no significant risk of a post-tonsillectomy hemorrhage than children without EDS Patients with EDS have no higher risk for post-tonsillectomy bleeding than controls. Subjects with EDS subtype 4 and other comorbidities should be managed jointly with pediatric sub-specialists and considered for overnight observation.