E. Odunusi et al., Genu valgum deformity in Hurler syndrome after hematopoietic stem cell transplantation: Correction by surgical intervention, J PED ORTH, 19(2), 1999, pp. 270-274
Hematopoietic stem cell transplantation has increased the survival of patie
nts with Hurler syndrome. Genu valgum occurs frequently in untransplanted p
atients and has been noted in 52% of our patients after stable engraftment.
No deformities spontaneously corrected. We describe the orthopaedic manage
ment of genu valgum in Hurler syndrome. Medial epiphyseal stapling predicta
bly affects angular deformity in these patients. Recurrence of deformities
either after staple dislodgement or surgical removal can occur, and repeate
d stapling may be required. Surgical epiphyseal stapling has a role in the
management of genu valgum in successfully engrafted Hurler patients. We dis
cuss the relationship of this skeletal deformity to other skeletal deformit
ies and alternative therapies for genu valgum.