Surgical management of pes planus in children with hypermobile Ehlers-Danlos: A case report and review of literature

Abstract

Ehlers-Danlos Syndrome (EDS) is a genetic condition that affects your body's connective tissues. There are many subtypes to the disease, the most relevant to the lower extremity is hypermobile EDS. In the lower extremity patients with hypermobile EDS present with abnormal mobility to all lower extremity joint articulations leading to the development of an often extreme and progressive form of pes planus. The degree of deformity and lack of intrinsic soft tissue stability leads to pain in the foot/ankle and compounds problems in the more proximal joint articulations. To our knowledge there has not been literature published regarding specifically the surgical management of hypermobile EDS in the lower extremity. Here we report, a case of an 11-year-old female with pes planus that was initially treated with standard joint sparing treatments including soft tissue procedures and extra-articular rearfoot osteotomies to address patient's pes planus. The procedures were performed with relatively few post-operative complications; however, the patient's deformity had reoccurred 5 years after the initial operation following the initial surgeries. It was discovered that the initial procedures were failing, and the arch had subsequently collapsed. After some research, the diagnosis of hypermobile EDS was made with the use of the Beighton scoring scale and other criteria as defined by the Ehlers-Danlos society. The patient then underwent a robust arthrodesis to help address the collapsing arch. This case was the beginning of the senior authors journey and long-term prospective cohort study on the surgical management of pes planus in children with hypermobile Ehlers-Danlos.