Effect of performing daily activities while standing on the quantity and quality of the thigh muscles in adults with severe cerebral palsy: a cross-sectional study.

[Purpose] To observe the effect of daily standing, as indicated by gross motor function, on the quantity and quality of the thigh muscles in adults with severe cerebral palsy and to obtain data to determine an appropriate intervention that will improve their quality of life. [Participants and Methods] Thirty-three adults with severe cerebral palsy participated in the study. We assessed the gross motor function using the GMFM-66-IS. We then evaluated the quadriceps muscle thickness and the rectus femoris muscle echo intensity using ultrasonography. We divided the participants into the standing and non-standing groups and then examined the correlations of the GMFM-66-IS score to muscle thickness and echo intensity. We calculated the difference in mean muscle thickness and echo intensity between the two groups using an independent t-test. [Results] Significant positive correlations were found between the GMFM-66-IS score and muscle thickness and echo intensity. In the group-specific analysis, no significant correlation was found between echo intensity and the GMFM-66-IS score in either group. Muscle thickness and echo intensity were greater in the participants of the standing group. [Conclusion] Daily standing, as indicated by gross motor function, affected muscle thickness and echo intensity. Quantitative and qualitative data might need to be evaluated when assessing the muscles of adults with severe cerebral palsy using ultrasonography.

Significant Improvement After Surgery for a Symptomatic Osteoblastoma in a Patient with Camurati-Engelmann Disease: Case Report and Literature Review.

Camurati-Engelmann disease (CED) is a rare, progressive diaphyseal dysplasia characterized as diaphyseal hyperostosis and sclerosis of the long bones. Corticosteroids, bisphosphonates, and losartan have been reported to be effective systemic medications used to reduce CED symptoms. There are no reports of osteoblastoma in patients with CED, and osteoblastoma in the distal radius is rare. We present a patient diagnosed with CED, based on radiological and histological examinations, at 11 years old. At 22 years old, she experienced severe pain in her right forearm and was treated with bisphosphonate, losartan, and prednisolone; however, the pain continued. An expansive and sclerotic lesion at the distal radius was observed on radiography. A follow-up plain radiograph indicated that the lesion was growing. Fluorodeoxyglucose positron emission tomography revealed solitary, intense radiotracer uptake, and a biopsy and surgical resection were performed due to suspected malignancy. Pathologic analysis showed anastomosing bony trabeculae rimmed by osteoblasts observed in a loose fibrovascular stroma. The lesion was diagnosed as an osteoblastoma. Following bone excision and artificial bone grafting, the patient's severe pain almost completely disappeared. At final follow-up, no evidence of osteoblastoma recurrence was noted. To our knowledge, this is the first case report of osteoblastoma arising in a patient with CED. Bone excision and artificial bone grafting may be a treatment option for local symptomatic osteoblastoma in patients with CED.

Acute correction using focal dome osteotomy for deformity about knee joint.

BACKGROUND: Correction of deformities about knee joint may prevent or delay the onset of osteoarthritis or mitigate its effects. Accurate correction of such deformities without production of secondary deformities depends on precise localization and quantification of the deformities. METHODS: We corrected deformities around the knee using acute correction with focal dome osteotomy in 21 segments (15 patients). Five segments underwent limb lengthening postoperatively. RESULTS: The mean correction angle was 16.0 degrees . We were able to correct all segments. In the five lengthening cases, the mean external fixation index (EFI) was 70.9 days/cm, which is much higher than the generally reported EFI. There were eight complications, all but one of which occurred in lengthening cases. CONCLUSIONS: We believe that acute correction with focal dome osteotomy is very useful for cases of alignment correction, but is not indicated for cases of alignment correction with lengthening, due to a high risk of complications related to poor callus formation.

Deformity correction for vitamin D-resistant hypophosphatemic rickets of adults.

We performed correction for bowing deformity of the lower extremities due to vitamin D-resistant hypophosphatemic rickets of three adults, six segments. The operative method was gradual correction and lengthening using distraction osteogenesis by Ilizarov external fixator or Heidelberg external fixator. The orders of the corrections were simultaneous correction of the bilateral femur for one patient, simultaneous correction of the ipsilateral leg for one patient, and diagonal correction of the bilateral leg for one patient. The mean correction angle was 30.5 degrees. The mean external fixation period was 146 days. Each orders of the corrections had its merits and demerits. All patients obtained a physiological alignment and good bone formation by taking Vitamin D and oral phosphate supplements even an adult patient. All the patients had articular pain, such as hip, knee, and ankle, however, these pains healed up. All the patients were satisfied with the outcomes at the time of the final follow-up interview in terms of their cosmetic improvement. Distraction osteogenesis for bowing deformity of the lower extremities due to vitamin D-resistant hypophosphatemic rickets was very effective method and could be applied to adult patients. However, the order of the corrections should be considered carefully depending on each patient.

Treatment of lower limb deformities and limb-length discrepancies with the external fixator in Ollier's disease.

BACKGROUND: In this study, we addressed two questions on the treatment for Ollier's disease: (1) how much callus formation occurs when an osteotomy is performed intralesionally and (2) how is the stability of the wires and half-pins that are inserted intralesionally. METHODS: Four children with Ollier's disease underwent treatment of 12 lower limb segments using distraction osteogenesis until completion of their growth. All osteotomies were performed at the centers of the deformities, resulting in a total of seven osteotomies performed intralesionally. RESULTS: Full correction of the deformity and full restoration of length were achieved in all cases, but a residual limb-length discrepancy of <10 mm remained. The mean external fixation index in the intralesional distraction osteogenesis group was 39.7 days/cm versus 30.8 days/cm in the extralesional distraction osteogenesis group. Conversion from abnormal cartilage to normal regenerate bone was seen in only one segment. Although approximately two-thirds of the wires and half-pins were inserted intralesionally, in all but one case (in which an iatrogenic fracture occurred) the wires and half-pins were well stabilized throughout the external fixation period. CONCLUSIONS: Although deformity and limb-length discrepancies due to Ollier's disease were successfully resolved by distraction osteogenesis, enchondroma may arise in distracted calluses when osteotomized intralesionally. However, the stability of the external fixator was sufficient to lengthen limbs and correct deformities even when wires and half-pins were inserted intralesionally.

Correction and lengthening for deformities of the forearm in multiple cartilaginous exostoses.

BACKGROUND: Multiple cartilaginous exostoses cause various deformities of the epiphysis. In exostoses of the ulna, the ulna is shortened and the radius acquires varus deformity, which may lead to dislocation of the radial head. In this study, we present the results of exostoses resection, with correction and lengthening with external fixators for functional and cosmetic improvement, and prevention of radial head dislocation. METHODS: We retrospectively reviewed seven forearms of seven patients who had deformities of the forearm associated with multiple cartilaginous exostoses. One patient had dislocation of the radial head. Operative technique was excision of osteochondromas from the distal ulna, correction of the radius, and ulnar lengthening with external fixation up to 5 mm plus variance. We evaluated radiographs and the range of pronation and supination. Furthermore, we conducted a follow-up of ulnar length after the operation. RESULTS: Dislocation of the radial head of one patient was naturally reduced without any operative intervention. At the most recent follow-up, six of the seven patients showed full improvement in pronation-supination. Ulnar shortening recurred with skeletal growth of four skeletally immature patients; however, it did not recur in one skeletally mature patient. Overlength of 5 mm was negated by the recurrence of ulnar shortening about 1.5 years after the operation. CONCLUSIONS: We treated seven forearms of seven patients by excision of osteochondromas, correction of radii, and gradual lengthening of ulnas with external fixators. The results of the procedure were satisfactory, especially for function of the elbow and wrist. However, we must consider the possible recurrence of ulnar shortening within about 1.5 years during skeletal growth periods in immature patients.

Deformity correction and lengthening of lower legs with an external fixator.

Deformities combined with shortening in 34 lower limb segments of 28 patients were treated with an Ilizarov external fixator or a Taylor spatial frame at the same level as the osteotomy. We compared an acute correction group (A) with a gradual correction group (G) in patients undergoing deformity correction followed by lengthening. We retrospectively examined the amount of deformity correction, length gained, distraction index, maturation index, and external fixation index in both groups. The mean age of subjects was 12.9 years for A, 17.9 years for G. The mean deformity correction was 17.8 degrees for A, 25.1 degrees for G. Mean lengthening was 5.5 cm for A, 5.0 cm for G. Mean distraction index was 16.4 days/cm for A, 10.6 days/cm for G (P<0.05). Mean maturation index was 40.5 days/cm for A, 29.5 days/cm for G (P=0.081). Mean external fixation index was 58.6 days/cm for A, 42.5 days/cm for G (P<0.05). The distraction index and external fixation index differences between the two groups were statistically significant. Gradual correction may represent a better approach than acute correction with the use of external fixators to treat deformity combined with shortening.

Distraction osteogenesis after irradiation in a rabbit model.

BACKGROUND: The present study was performed to investigate the effects of preoperative irradiation on distraction osteogenesis, as little is known about how preoperative irradiation delays distraction osteogenesis. METHODS: A single dose of irradiation was applied to the right rear legs of rabbits. This was followed by tibial lengthening at a rate of 0.5 mm/day, which was continued for 4 weeks. Bone regeneration was examined radiographically and histologically. RESULTS: In the irradiation group, the radiographs showed little regeneration during the elongation phase. During the maturation phase, the callus appeared slowly, and its formation was spotty. Furthermore, regeneration was not completed until the fourth week of the maturation period. Histological examination at the end of distraction showed a gap in the distraction consisting of loose connective tissue, with part of the fibrous tissue oriented longitudinally. Four weeks after completion of distraction, the major part of the radiolucent region consisted of cartilage. The spotty osteogenesis was identified as enchondral ossification. Immunohistochemical examination of the regeneration area revealed that the blood vessels were extremely localized, and that the level of expression of vascular endothelial growth factor (VEGF) in the osteoblasts was high. Microangiography showed that vascularization at the distracted sites was poor. Distraction osteogenesis was decreased markedly by preoperative irradiation in terms of both rate and process. The results suggested that most of the osteoprogenitor cells were damaged immediately after irradiation. The high level of VEGF in the osteoblasts and the enchondral ossification also suggested a hypoxic state in the distracted region. CONCLUSIONS: Preoperative irradiation interferes with distraction osteogenesis by inducing a state of poor angiogenesis.

Tibial lengthening over an intramedullary nail.

BACKGROUND: Long-term application of an external fixator to treat leg-length discrepancy and short stature often causes complications, such as pin-tract infection or loss of range of motion at the knee or ankle (or both). Prolonged fixator use also interferes with the activities of daily living. To minimize such problems, we have combined intramedullary nailing with external fixation. Using this technique, the external fixator can be removed more quickly after completing the lengthening. METHODS: We combined intramedullary nailing with lengthening in 13 tibias (8 patients) and then compared these cases with 17 standard tibial lengthenings (16 patients) using an external fixator alone. In both groups we excluded patients who had a history of previous bone infection, open fracture, immature bone, soft tissue compromise, antineoplastic chemotherapy, or bone deformity of a severity that required gradual deformity correction. We also excluded cases with lengthening of less than 3 cm. RESULTS: The mean external fixation index differed significantly between the two groups, but the consolidation index did not. Mean operating time for lengthening combined with intramedullary nail placement was approximately 60 min longer than for standard lengthening without nail placement; intraoperative blood loss was not greater in the nailing group. Complications related to the external fixator were far fewer in the combined intramedullary nailing and lengthening group compared with the control group, and callus formation was satisfactory for both groups. CONCLUSIONS: A combination of intramedullary nailing and external fixation produces callus formation as good as that obtained by the standard Ilizarov method of lengthening. Furthermore, this combined procedure decreases the external fixation time and is associated with fewer complications.

Correction of juxtaarticular deformities in children using the Ilizarov apparatus.

BACKGROUND: Deformity correction using external fixation was performed for various disorders in children. We reviewed 18 children who underwent juxtaarticular deformity correction using the Ilizarov apparatus with either a transverse or focal dome osteotomy. The amount of deformity correction, external fixation time, external fixation index, length gained, and incidence of complications were examined. METHODS: A series of 27 operations were performed in lower limb segments on 10 femurs and 17 tibias in 10 boys and 8 girls. The mean age at operation was 12 years (5-18 years). Deformity corrections were performed using a transverse osteotomy in 16 segments and a focal dome osteotomy in 11. RESULTS: The average deformity corrected was 19 degrees (6 degrees-31 degrees). Acute correction was done in 14 segments and gradual correction in 13 segments. The mean lengthening was 4.2 cm in 12 segments (2-8 cm). The mean external fixation time was 161 days (78-352 days). In acute corrections, the external fixation time with a focal dome osteotomy (101 days) tended to be shorter than with a transverse osteotomy (142 days). CONCLUSIONS: Accurate limb alignment was obtained for all cases. A focal dome osteotomy by maximizing the area of bony contact was more effective than a transverse osteotomy for acute deformity correction.

Reconstruction with distraction osteogenesis for juxta-articular nonunions with bone loss.

BACKGROUND: Nonunions of a juxta-articular lesion with bone loss, which represent a challenging therapeutic problem, were treated using external fixation and distraction osteogenesis. METHODS: Seven juxta-articular nonunions (five septic and two aseptic) were treated. The location of the nonunion was the distal femur in four patients, the proximal tibia in one patient, and the distal tibia in two patients. All of them were located within 5 cm from the affected joints. Preoperative limb shortening was present in six cases, averaging 2.9 cm (range, 1-7 cm). The reconstructive procedure consisted of refreshment of the nonunion site, deformity correction, stabilization by external fixation, and lengthening to eliminate leg length discrepancy or to fill the defect. Shortening-distraction was applied to six patients and bone transport to one patient for reconstruction. Intramedullary nailing to reduce the duration of external fixation was simultaneously performed in two cases. All the patients had at least 1 year of follow-up evaluation. RESULTS: Osseous union without angular deformity or leg length discrepancy greater than 1 cm was achieved in all patients. The mean amount of lengthening was 5.8 cm (range, 2.2-10.0 cm). The mean external fixation period was 219 days (range, 98-317 days), and the mean external fixation index was 34.4 days/cm (range, 24.5-47.6 days/cm). All patients reported excellent pain reduction. There were no recurrences of infection in five patients with prior history of osteomyelitis. The functional results were categorized as excellent in two, good in three, and fair in two. CONCLUSION: Despite the length of postoperative external fixation, distraction osteogenesis can be a valuable alternative for the treatment of juxta-articular nonunions.

Osteofibrous dysplasia treated with distraction osteogenesis: a report of two cases.

We treated two patients with osteofibrous dysplasia of the tibia using en bloc marginal excision of the lesion and bone transport, a distraction osteogenesis procedure. In one patient, we had performed curettage procedures twice, each time followed by local recurrence and deformity. In contrast, with marginal excision and bone transport using an Ilizarov apparatus, the disease healed. In the other patient, we performed marginal excision and bone transport as the first procedure, which resulted in a healed lesion. In patients with osteofibrous dysplasia requiring treatment by an open method, curettage frequently is followed by local recurrence and deformity. We now prefer marginal excision of the lesion to avoid long-term failures, as in our first case. Distraction osteogenesis has proven useful for restoring structure and function in the affected limb.

Effects of timing of low-intensity pulsed ultrasound on distraction osteogenesis.

We investigated the effects of low-intensity pulsed ultrasound (LIPUS; 30 mW/cm2 spatial and temporal average) on the timing of LIPUS treatment in distraction osteogenesis. Lengthening of the right tibia was performed in 75 male Japanese white rabbits using unilateral fixators (waiting period, 7 days; distraction rate, 1.5 mm/day; distraction period, 7 days). Rabbits were divided into four groups according to the timing of the LIPUS treatment. Control group had no stimulation. Waiting group was treated with a daily 20-min session of LIPUS during a 7-day latency period. Lengthening group was treated during the lengthening period. Maturation group was treated for the first 7 days after completion of distraction. We evaluated the distraction site by radiography and histology every week for 4 weeks. Bone mineral density (BMD) and mechanical strength were tested and microfocus X-ray computed tomography was performed on specimens 2 weeks after completion of distraction. The lengthening group had greater BMD and mechanical strength than the other groups, bone regeneration was enhanced more in the maturation group than in the control or waiting groups. Histologically, endochondral bone formation in the lengthening and the maturation groups occurred earlier than in the control or waiting groups. These results suggest the LIPUS effect is mediated via endochondral pathways. We concluded that LIPUS stimulates bone formation in distraction osteogenesis and is most effective during the lengthening phase.

Bone transport with frozen devitalized bone: an experimental study using rabbits and a clinical application.

We evaluated the feasibility of bone transport with frozen devitalized bone in the tibia of 20 adult female rabbits. A 1.5-cm segmental defect was created, followed by proximal tibial transverse osteotomy to remove a 2.5-cm segment to be transported after devitalization with liquid nitrogen. The proximal and distal tibia and the devitalized autogenous bone each were fixed with two half-pins. After 2 weeks, transport of the devitalized segment was initiated at 1 mm/day. In a control group, callus formed at proximal and distal osteotomy sites, and distracted callus gradually maturated. The docking site fused and the medullary canal reappeared by 8 weeks after completing distraction. In the frozen-bone group, bone formation proceeded from the proximal tibial end, and the distraction callus slowly matured. The transported segment remained nonviable until revascularization proceeded from its periphery, evident 8 weeks after completion of distraction. Docking sites fused well without infection at pin sites. There was no sign of infection around the pin sites of the devitalized bone. We finally describe similar successful treatment of a 13-year-old girl with tibial deformity resulting from osteofibrous dysplasia who was treated successfully with this procedure. Therefore, bone transport using frozen devitalized bone can regenerate living bone. This experimental model represents the development of a new reconstruction technique of bone transport with devitalized bone.

Successful management of complications from distraction osteogenesis after osteosarcoma resection: a case report.

We report a complicated case of osteosarcoma of the proximal tibia. A 15-year-old boy was referred to us and underwent distraction osteogenesis reconstruction. We administered preoperative chemotherapy for five cycles. Clinical response was determined to be complete by radiography. Marginal excision was then performed with preservation of the proximal tibial epiphysis. Metaphyseal reconstruction (type 2) was performed with distraction osteogenesis. Postoperative chemotherapy also was administered for five cycles. Two years later, the patient developed a deep infection. He underwent curettage and a pedicle peroneal flap transfer, which did not cure the infection. Infected tissues were excised, and shortening-distraction was carried out with the Ilizarov frame. The infection was cured; however, a leg length discrepancy and deformity resulted from frame instability. Four years after the initial operation, the patient fractured his reconstructed leg in a traffic accident. We performed osteosynthesis, deformity correction, and lengthening with the Ilizarov method. We were able to correct the defects using distraction osteogenesis, eventually restoring normal function. Epiphyseal preservation and reconstruction by distraction osteogenesis can provide an excellent outcome, resulting in a stable reconstruction that functionally restores the native limb. Distraction osteogenesis avoids some complications but may involve others, which require detection and appropriate management.

Gradual closed correction of equinus contracture using the Ilizarov apparatus.

We treated 16 patients with equinus contracture using the Ilizarov method without open soft tissue release. No hinge was used in seven patients; instead, their ankle joint was used as the hinge (natural hinge; unconstrained construct). In the remaining nine patients, medial and lateral hinges connecting the tibial and foot rings were placed at the center of the talar dome (rotating hinge; constrained construction). We assessed the results in terms of the extent of dorsiflexion, its improvement, and complications. There was little difference between the results in the natural hinge group and the rotating hinge group. The natural hinge system is therefore the method of choice for treating equinus contracture because it is less invasive and simpler. However, particular care is needed during correction to avoid complications such as anterior subluxation of the talus and joint space narrowing. When complications do occur, intervention should be immediate.

Ankle arthrodesis combined with tibial lengthening using the Ilizarov apparatus.

We report our experience using the Ilizarov method to perform combined ankle arthrodesis and tibial lengthening in six patients (mean age 47 years; range 25-66 years). The average distraction length was 4.1 cm (range 1.1-6.8 cm), and the mean period of follow-up was 36 months (range 26-44 months). Three patients had active infection of the ankle. Four patients had undergone previous surgery, two of which were primary ankle arthrodeses. We performed compression-distraction in three patients and bone transport in three. In the compression-distraction group, the mean length gained was 1.9 cm, the mean external fixation index (EFI) was 144 days/cm, and the mean external fixation time was 246 days. In the bone transport group, the mean length gained was 6.2 cm, the mean EFI was 35.4 days/cm, and the mean external fixation time was 233 days. All cases achieved a good clinical result with a solid ankle arthrodesis and no infection, deformity, or need for additional support. The Ilizarov method may be practically applied for ankle arthrodesis, especially in complicated cases. The EFI and external fixation time tended to increase for patients with a length gain of 3 cm or less.