Secondary Logo

Journal Logo


Case Reports: A Case of Paget's Disease Treated by Distraction Osteogenesis

Nakase, Takanobu, MD, PhD*; Ohzono, Kenji, MD, PhD*; Araki, Nobuhito, MD, PhD; Hiroshima, Kazuo, MD, PhD*

Author Information
Clinical Orthopaedics and Related Research®: October 2006 - Volume 451 - Issue - p 279-282
doi: 10.1097/
  • Free


Paget's disease is a localized disorder marked by increased bone turnover.10 It usually is associated with deformities of the affected bone and degeneration (osteoarthrosis) in adjacent joints.4,7 Patients with severe osteoarthrosis may be candidates for arthroplasty.3,12,13 However, the complexity of the deformity sometimes makes insertion of a prosthesis difficult, and component malpositioning may lead to early prosthetic failure.3,11-13 Therefore, deformity correction before joint arthroplasty is advantageous for treatment of osteoarthrosis.

Corrective osteotomy is a good alternative to long-bone reconstruction, leading to possible prosthetic insertion and restoration of physiologic alignment of the lower limb. However, patients with Paget's disease sometimes have a limb-length discrepancy because of a deformed and shortened bone. Distraction osteogenesis has been advocated by some authors,1,5 because it enables simultaneous correction of the deformity and shortening.

We present the case of a patient with Paget's disease in which the deformity and the limb-length discrepancy were treated by distraction osteogenesis.

Case Report

A 57-year-old woman was diagnosed with Paget's disease by bone biopsy done when she was 54 years old. She had severe deformities with shortening of her right femur. Her serum alkaline phosphatase level was elevated and she had diabetes mellitus. The patient took bisphosphonate orally. Deformity in the distal femur and limb-length discrepancy gradually developed (Fig 1). The patient limped and experienced pain in the adjacent knee and lower back. At the time of surgery, she had a 3-cm limb-length discrepancy. She also had a diaphyseal deformity that was 10° varus, 47° procurvatum, and 85° external rotation, with mechanical axis deviation 0.5 cm medial to the center of the knee (Fig 1A-B).

Fig 1A
Fig 1A:
C. (A) An AP radiograph taken before surgery shows a 3.0-cm limb-length discrepancy. (B) A preoperative lateral radiograph of a right distal femur shows the typical appearance of Paget's disease and a 47°-procurvatum deformity. (C) A frontal view of a patient shows the deformity and shortening of the right lower limb.

We applied a Taylor Spatial Frame (Smith & Nephew, Memphis, TN) to the femur and performed a percutaneous osteotomy with a 2-cm skin incision approximately 20 cm proximal to the physis. The frame was constructed using two rings (a 205-mm ⅔ proximal ring and a 180-mm full distal ring) and six struts. The proximal ring was fixed with three half-screws (6 mm diameter) and one smooth Ilizarov wire (1.8 mm diameter) as a half-wire. The distalring was fixed with three half-screws (6 mm diameter) and four smooth Ilizarov wires (1.8-mm diameter; one wire was used as a transfixation wire and three wires were used as half-wires). We performed the percutaneous osteotomy using a previously described method.14 The total operation time was 146 minutes and total blood loss during the operation was 100 cc. After 7 days, frame adjustments were started and performed daily, which resulted in improvement of the deformity (10° in the coronal plane, 47° in the sagittal plane, and 25° in the axial plane) and limb-length discrepancy (Fig 2) based on the total residual program. The sizes of several struts were changed during the program. Just after completion of the lengthening procedure of 51 days, two supplemental straight rods were added between the two rings (one in the anterior and the other in the posterior portion) and weightbearing was permitted. Thirty days later, the two rods were removed. Bone union was evaluated based on the radiographic findings showing enough consolidation on the anteroposterior (AP) and lateral views. Bone union was achieved after a fixation period of 132 days (Fig 3A-B). The radiographs showed a formation of normal bone in the distraction gap. No orthosis was applied. The amount of lengthening was 3.5 cm at the anterior portion of the distraction gap. Pin-tract infections sometimes occurred during distraction, and the patient had a 10°-decrease in knee flexion, but no other complications were apparent. The patient continued to take bisphosphonate orally throughout the treatment period. At the final followup 9 months after surgery, the patient reported feeling more comfortable when walking than before surgery because of improvement of the limb-length discrepancy (Fig 3C). Her low back pain was relieved, but she reported persistent mild pain in the right knee.

Fig 2A
Fig 2A:
B. (A) An AP radiograph of the right distal femur taken at the end of the distraction phase shows 3.0-cm distraction gap (arrow). (B) A lateral radiograph of the right distal femur taken at end of the distraction phase shows correction of the 47°-procurvatum deformity. The white arrow is pointing to the distraction gap.
Fig 3A
Fig 3A:
C. (A) An AP radiograph of the right lower limb was obtained 3 months after frame removal. The radiograph shows bony union and improvement of the limb-length discrepancy. (B) A lateral radiograph of the right femur taken 3 month after frame removal shows correction of the deformity and consolidation of the lengthened callus (arrow). (C) A frontal view of the patient taken at the final followup shows improvement of the limb-length discrepancy and deformity.


Using distraction osteogenesis, we reconstructed a complex and severe deformity with shortening of the affected femur anticipating a future arthroplasty in the adjacent joint. After surgery, her discomfort with gait decreased. The pain in her joint also was less, but not eliminated. If this patient becomes a candidate for an arthroplasty in the future, correction of the deformity will make it easier to insert an intramedullary guide, possibly preventing prosthetic malposition and early prosthetic failure. Additionally, the limb-length discrepancy was normalized by distraction osteogenesis, leading to improvement of gait disturbance and low back pain.

One of the interesting findings shown in the current case is that lengthening callus formed in the distraction gap looked like normal regenerate bone, although lengthening was performed at the pathologic bone. Such a finding suggests the possibility of treating this disease by distraction osetogenesis. A similar phenomenon was reported in a patient with Ollier's disease.6

The degree of the deformity of our patient (47° in the sagittal plane) was much more severe than any in the reported cases of patients with Paget's disease.8,11 Our patient had a complex deformity that we corrected using a Taylor Spatial Frame. Distraction callus was visible at the end of the lengthening period, and consolidation of the callus was achieved within a reasonable time. Blood loss was less and operation time was shorter than those in the previous report.11 Pin-tract infection was a major complication experienced by our patient during the distraction period. This complication may have originated from or been exacerbated by the patient's preexisting diabetes mellitus. Fortunately, no other complications were apparent throughout the treatment period. Loosening and migration of the half-pins or wires that were inserted into the pathologic bone were not observed. However, an increased incidence of complications in patients with underlying bone disorders who have lengthening has been reported.9

Many deformities in patients with Paget's disease are complex. Conventional correction using internal fixation is limited in degree and accuracy. A recently developed external fixator, the Taylor Spatial Frame, is a modified version of the Ilizarov external fixator.2 The mechanism of deformity correction of the device is computer based, which enables the surgeon to perform an easy correction procedure for complex deformities. The device may be a powerful tool for treatment of long-bone deformities in patients with Paget's disease.

Our results suggest distraction osteogenesis may be considered for treating patients with Paget's disease who have severe, complex deformities requiring correction.


1. Aronson J. Limb-lengthening, skeletal reconstruction, and bone transport with the Ilizarov method. J Bone Joint Surg Am. 1997;79: 1243-1258.
2. Feldman DS, Shin SS, Madan S, Koval KJ. Correction of tibial malunion and nonunion with six-axis analysis deformity correction using the Taylor Spatial Frame. J Orthop Trauma. 2003;17:549-554.
3. Gabel GT, Rand JA, Sim FH. Total knee arthroplasty for osteoarthrosis in patients who have Paget disease of bone at the knee. J Bone Joint Surg Am. 1991;73:739-744.
4. Hadjipavlou AG, Gaitanis IN, Kontakis GM. Paget's disease of the bone and its management. J Bone Joint Surg Br. 2002;84:160-169.
5. Herbert AJ, Herzenberg JE, Paley D. A review for pediatricians on limb lengthening and the Ilizarov method. Curr Opin Pediatr. 1995;7:98-105.
6. Jesus-Garcia R, Bongiovanni J, Korukian M, Boatto H, Seixas M, Laredo J. Use of the Ilizarov external fixator in the treatment of patients with Ollier's disease. Clin Orthop Relat Res. 2001;382: 82-86.
7. Kaplan FS, Singer FR. Paget's disease of bone: pathophysiology, diagnosis, and management. J Am Acad Orthop Surg. 1995;3:336- 344.
8. Louette L, Lammens J, Fabry G. The Ilizarov external fixator for treatment of deformities in Paget's disease. Clin Orthop Relat Res. 1996;323:298-303.
9. Naudie D, Hamdy R, Fassier F, Duhaime M. Complications of limb-lengthening in children who have an underlying bone disorder. J Bone Joint Surg Am. 1998;80:18-24.
10. Paget J. On a form of chronic inflammation of bones (osteitis deformans). Clin Orthop Relat Res. 1966;49:3-16.
11. Parvizi J, Frankle MA, Tiegs RD, Sim FH. Corrective osteotomy for deformity in Paget disease. J Bone Joint Surg Am. 2003;85:697-702.
12. Roper BA. Paget's disease at the hip with osteoarthrosis: results of intertrochanteric osteotomy. J Bone Joint Surg Br. 1971;53:660- 662.
13. Schai PA, Scott RD, Younger AS. Total knee arthroplasty in Paget's disease: technical problems and results. Orthopedics. 1999;22: 21-25.
14. Yasui N, Nakase T, Kawabata H, Shibata T, Helland P, Ochi T. A technique of percutaneous multidrilling osteotomy for limb lengthening and deformity correction. J Orthop Sci. 2000;5:104-107.
© 2006 Lippincott Williams & Wilkins, Inc.