Background:
Ehlers-Danlos syndrome (EDS) is a heterogeneous collection of connective tissue disorders characterized by varying degrees of skin hyperextensibility, joint hypermobility, and tissue fragility. Surgical treatment of EDS patients is complicated by the extreme fragility of their vessels and tissues. The purpose of this case report is to present the management of an EDS patient with debilitating low-back pain.
Methods:
A 52-year-old woman with a clinical diagnosis of EDS presented with degenerative disc disease at L4-5 that had not been alleviated by previous microdiscectomies. The clinical course, decision-making process, and treatment are discussed in this case report.
Results:
The patient was referred for genetic evaluation, which classified her with type III EDS, or hypermobility type. We presented the patient with the risks and benefits of fusion versus artificial disc replacement (ADR), particularly with regard to her EDS diagnosis of the hypermobility subtype. Given the patient's lack of extreme spinal hypermobility on examination and the absence of clear contraindications regarding ADR in type III EDS, the decision was made to proceed with ADR. There were no surgical complications, and the patient's low-back pain and radicular symptoms resolved with no evidence of implant migration or hypermobility at 1 year postoperatively.
Conclusions:
In this case report, the referral to a geneticist and consultation with a vascular surgeon were integral steps in the decision to proceed with surgery. Although the clarified diagnosis of type III EDS did not eliminate the potential risk for vascular compromise during surgery, it placed the patient at lower risk than patients with other subtypes of EDS. Similarly, her lack of extreme hypermobility made us more comfortable with pursuing ADR. Although we emphasize extreme caution when considering surgical treatment, this case report suggests that some patients with less severe forms of EDS may be able to successfully undergo anterior spine surgery, including ADR.