A 37-year follow-up evaluation of multiple-stage femur and tibia lengthening in dyschondroplasia (enchondromatosis) with a net gain of 23.3 centimeters.
This case report presents the rare combination of circumstances of a child with (a) dyschondroplasia (enchondromatosis) limited to all the major epiphyseal plates of one lower extremity, (b) unusual capacity to cooperate, and (c) parents who refused permission to treat the limb by amputation but allowed the child to participate in a long-term clinical experiment. In dyschondroplasia (Ollier's disease), the long bones grow in diameter but not in length. In this condition, it was possible (by fasciotomy, osteotomy, and interposition of wedge blocks or hemicylindrical bone implants (stabilized by an intramedullary rod) to lengthen the leg 23.3 cm in ten stages, during the period between five and 14 years of age. With the additional procedure of epiphyseal arrest of the contralateral distal end of the femur at age 11, the eventual correction of discrepancy in leg length was 25.6 cm. Intraoperative and postoperative skin temperature recordings, toe plethysmography, and electromyography show that there were no deleterious changes in the circulatory or neurological functions of the extremity.
Urist
M R
MR
Department of Surgery, University of California Medical Center, Los Angeles.
D000328:Adult; D001852:Bone Lengthening / Q000379:methods*; D004687:Enchondromatosis / Q000150:complications / Q000601:surgery*; D005260:Female; D005269:Femur / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D007870:Leg Length Inequality / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery*; D008137:Longitudinal Studies; D010009:Osteochondrodysplasias / Q000601:surgery*; D011859:Radiography; D013977:Tibia / Q000000981:diagnostic imaging / Q000601:surgery