Post-tonsillectomy risks in patients with Ehlers-Danlos syndrome

Abstract

Introduction

There is a paucity of data on the connective tissue disorder causing delayed wound healing in children with Ehlers Danlos Syndrome (EDS), potentially affecting the rates of post-tonsillectomy hemorrhage (PTH) following tonsillectomy and adenotonsillectomy (T&A). Understanding the postoperative course of this population could provide better guidance in their postoperative management which formed the basis of our study.

Methods

This was a retrospective chart review in an academic pediatric center that compared the PTH rates between patients with EDS and control children. Relevant patient demographic, past medical history, family history, intraoperative and postoperative information were collected for analyses.

Results

The study comprised of 60 EDS and 72 controlled non-EDS children. No differences in PTH rates between children with and without EDS were found. Significant differences were found between the study groups with increased past medical history for easy bleeding (<0.001), the use of clotting agents (<0.001) and family history of easy bleeding (p = 0.023) in the EDS group. Multivariate models failed to reach statistical significance for these factors.

Discussion

The study failed to show a difference in PTH between the study groups. The association between EDS and concomitant coagulation disorders including von Willebrand (VW) disease and hemophilia did not reach significance but were clinically associated.

Conclusion

EDS children with associated coagulopathies and standard indications for an overnight stay following T&A should be admitted. EDS children without comorbidities could be done as outpatient but the decision should be placed on the otolaryngologist. All EDS patients that have not had hematology laboratory testing or consultation prior to T&A would benefit from such evaluation.