Surgical Management of Shoulder and Knee Instability in Patients with Ehlers-Danlos Syndrome: Joint Hypermobility Syndrome

Abstract

Ehlers-Danlos Syndrome (EDS) is a hereditary disorder of the connective tissue, which has been classified into numerous subtypes over the years. EDS is generally characterized by hyperextensible skin, hypermobile joints, and tissue fragility. According to the 2017 International Classification of EDS, 13 subtypes of EDS have been recognized. The majority of genes involved in EDS are either collagen-encoding genes or genes encoding collagen-modifying enzymes. Orthopedic surgeons most commonly encounter patients with the hypermobile type EDS (hEDS), who present with signs and symptoms of hypermobility and/or instability in one or more joints. Patients with joint hypermobility syndrome (JHS) might also present with similar symptomatology. This article will focus on the surgical management of patients with knee or shoulder abnormalities related to hEDS/JHS.

Proximal tibiofibular joint (PTFJ) dislocation due to Ehlers-Danlos syndrome: posterolateral open-wedge high tibial osteotomy combined with medial closed-wedge distal femoral osteotomy can correct the severe valgus deformity with a markedly increased tibial posterior slope

“…Although there have been reports of dislocation of the hip, patella, shoulder, elbow joints, etc, in patients with EDS,1–3 a case of dislocation of the PTFJ associated with EDS has never been reported. Traumatic PTFJ dislocation in adulthood, although rare,13 provides little useful information about the effects of PTFJ dislocation in skeletally immature knees on bony growth and/or bony deformities.…”

“…Ehlers-Danlos syndrome (EDS) is a hereditary disorder that leads to systemic connective tissue fragility, such as in the skin, joints and blood vessel walls, with joint hypermobility, leading to systemic joint dislocations and subsequent osteoarthritis. Although various types of joint dislocations have been reported, [1][2][3] there are no previous reports on proximal tibiofibular joint (PTFJ) dislocations caused by EDS. Here, we report a case of EDS with PTFJ dislocation that led to a severe valgus-aligned knee with posterolateral dysplasia of the lateral tibial condyle.…”

Double-bundle anterior cruciate ligament reconstruction using autologous hamstring tendon hybrid grafts in a patient with hypermobile Ehlers-Danlos Syndrome: A case report

“…The hypermobile EDS (hEDS) has been described in those with musculoskeletal disorders including joint hypermobility, joint subluxations/dislocations, and ligament and tendon rupture [1,4]. There were some reports regarding joint laxity and pain in the patient with hEDS [5,6].…”

“…According to previous studies, the treatment of knee injury in patients with hEDS can be challenging [5,6].…”