Genu Valgum Deformity in Hurler Syndrome After Hematopoietic Stem Cell Transplantation: Correction by Surgical Intervention

Abstract

Hematopoietic stem cell transplantation has increased the survival of patients with Hurler syndrome. Genu valgum occurs frequently in untransplanted patients and has been noted in 52% of our patients after stable engraftment. No deformities spontaneously corrected. We describe the orthopaedic management of genu valgum in Hurler syndrome. Medial epiphyseal stapling predictably affects angular deformity in these patients. Recurrence of deformities either after staple dislodgement or surgical removal can occur, and repeated stapling may be required. Surgical epiphyseal stapling has a role in the management of genu valgum in successfully engrafted Hurler patients. We discuss the relationship of this skeletal deformity to other skeletal deformities and alternative therapies for genu valgum.