Congenital tibial pseudarthrosis, changes in treatment protocol.

Ten children with congenital pseudarthrosis of the tibia were treated with resection of pathologic bone, bone grafting, intramedullary rodding, compression with circular frame, simultaneous proximal tibia lengthening, and bone morphologic proteins. Thirteen operations were performed to achieve union. Four patients underwent simultaneous lengthening and four patients received recombinant human bone morphologic protein. Six children required complementary operations for residual ankle and knee valgus, and shortening. Union of the pseudarthrosis was achieved in all cases with lengthening up to 5 cm. This protocol is effective in achieving union of congenital pseudarthrosis of tibia; attention should be paid to the ankle joint to achieve functionality of the limb.

Growth and development of tarsal and metatarsal bones in successfully treated congenital idiopathic clubfoot: early radiographic study.

Fifteen cases of unilateral clubfoot treated according to Ponseti's technique had the talocalcaneal angles on the anteroposterior and lateral views and the size of the talus, calcaneus, I-V metatarsus measured on radiographs of both feet that were taken at a mean age of 15.2 months (range 8-23). The measurements of talocalcaneal angles and size of the talus, calcaneus, and I, II, and III metatarsi were significantly smaller on the affected side, whereas the values for the IV and V metatarsi were similar on both sides. Clubfoot deformity involves all structures of the foot, but intrinsic compressive forces on the small hindfoot bones induce measurable reduction in their size and spatial orientation. This effect is seen early on the medial but not the lateral long tubular forefoot bones.

Primary pyomyositis in children: a retrospective analysis of 11 cases.

This study was undertaken to review our approach to diagnosis and treatment in a series of 11 patients (mean age 8.2 years) with primary pyomyositis, who had neither an underlying disease nor a compromised immune system. Nine of the children had positive blood cultures, Staphylococcus aureus (eight) and Streptococcus group A (one). The sites of infection were iliopsoas (four), obturator (two), hip adductors (two), levator scapula (one), thoracolumbar paraspinal (one) and gastrocnemius (one) muscles. Antibiotic treatment was initially intravenous, followed by oral administration. Of five patients with evidence of abscess formation, three underwent percutaneous drainage, whereas two required open surgical drainage. The infection resolved completely without any sequela in 10 children. One patient who developed acute compartment syndrome showed late signs of osteonecrosis of the tibial shaft segment.

Treatment of severe late-onset Perthes' disease with soft tissue release and articulated hip distraction: revisited at skeletal maturity.

PURPOSE: Late-onset Perthes' disease is diagnosed after 9 years of age. Conservative treatment and conventional surgical techniques have limited ability to reduce the pressure in the joint or change the shape of the femoral head. We used a combination of soft tissue release and joint distraction with a hinged mono-lateral external fixator for these patients. Ten of our patients reached skeletal maturity and were evaluated. METHODS: Clinical assessment included: Harris hip score, hip range-of-motion (ROM), limb length discrepancy, and the Oxford hip questionnaire for pain and function. Radiographic assessment included: Sharp transverse acetabular inclination, the uncoverage percentage, the epiphyseal index before surgery (modified Eyre-Brook), at frame removal, and, at last follow-up, the epiphyseal quotient (of Sjovall) and the Stulberg classification. RESULTS: Our study included eight boys and two girls (mean age at surgery 12.3 years, range 9.4-15.1, mean age at last follow-up 18.1 years, range 15.2-22.8). The mean follow-up was 5.7 years (range 4.3-7.8). The mean Harris hip score was 86.3/100 (range 48.5-96); one patient had <85 points. The hip ROM was slightly limited in most patients, and seven patients had limb shortening between 1-4 cm. The mean Oxford hip questionnaire score was 17.4/60 (range 12-31). The mean Sharp transverse acetabular inclination of the affected side was 42 degrees (range 36-54) compared to 39 degrees for the unaffected side (P = 0.045). The mean uncoverage percentage was 37% (range 27-47) compared to 20% for the unaffected side (P = 0.017). The mean epiphyseal index was 0.71 (range 0.31-0.92) before surgery, 0.79 (range 0.50-0.93) at frame removal (P = 0.012), and 0.72 (range 0.51-0.89) at last follow-up (P = 0.646). The epiphyseal quotient for the eight unilateral cases was 0.72 (range 0.49-0.91), and the Stulberg classification was type III for three cases and type IV for seven. CONCLUSION: Patient satisfaction for function and pain following the combined procedure was good. Radiographic parameters did not change significantly. This should be regarded as a salvage procedure.

Analysis of mesenchymal cells derived from an chondrodysplasia punctuate patient and donors.

Conradi-Hunermann syndrome (CDPX2) is X-linked dominant disorder appeared with aberrant punctuate calcification. The skeletal cells derived from the marrow stroma are active in maintaining the skeletal formation. We obtained mesenchymal stem cells from a patient with CDPX2 and studied the formation of colony forming unit-fibroblast (CFU-F) in vitro in comparison cells obtained from normal donors. Cultured cells were studied morphologically and subjected to gene expression analysis. Marrow stromal cells (MSC)-chondrodysplasia punctuate (CDP) cells from CDPX2 were identified by their mosaic morphology formed three phenotypically distinct types of CFU-F colonies. One type consisted of normal fibroblasts with developed cell body and cellular processes; the second type contained pathological small cells without processes; and the third type comprised of mixed cells. We compared gene expression by the MSC-CDP to cells from normal donors. Transcription factors analyzed proliferation potential were similar in both normal and mixed colonies of MSC-CDP and similar to normal MSCs. The message expression for cytokines and extra cellular matrix (ECM) proteins revealed similar expression for biglycan, osteocalcin, and osteonectin, while IL-6, IL-11, and M-CSF mRNA levels were significantly higher in normal cells than in MSC-CDP. Mixed cells had elevated levels for IL-6 and M-CSF mRNA, but expressed IL-11 at the normal range. The studied genes were expressed at lower levels by the pathological (MSC-CDP) cells compared to normal ones. Hence, MSC-CDP was demonstrated to display abnormal morphology and transcription of several investigated genes. This study further illuminates the basis of the mosaic pattern of mesenchymal cells derived from a patient affected with CDPX2, and their gene expression involvement.

Giant osteofibrous dysplasia (ossifying fibroma) of the tibia: case report and review of treatment modalities.

We present a case of giant osteofibrous dysplasia (OFB) of the proximal tibia with 15 years of follow-up. The tumor recurred after first biopsy and curettage done at the age of 6 years and, again, 4 years later. Following recurrence, the option of amputation was suggested. Staged treatment of curettage, cryosurgery, bone cement as a temporary spacer with internal fixation at age 12 years, followed by bone grafting at age 14 years, led to complete healing. The staged protocol for treatment is proposed as an alternative to more radical solutions. It is suggested to postpone surgical treatment towards skeletal maturity.

Atypical mycobacterial granulomatous epiphyseal osteomyelitis in an immunocompetent child.

Osteomyelitis caused by atypical mycobacteria is rare in children. The majority of affected patients have had some kind of predisposing factor, namely a penetrating injury or surgery, or were immune-compromised. Our experience shows that this diagnosis should be considered in apparently healthy children as well. The use of polymerase chain reaction has now made it possible to identify the pathogen in cases that were previously diagnosed as granulomatous osteomyelitis. We present a case of atypical mycobacterial osteomyelitis affecting the distal femoral epiphysis in an immunocompetent 10-year-old child. The diagnosis in this case was made by the use of the polymerase chain reaction assay.

Perthes-like changes in geleophysic dysplasia.

Geleophysic dysplasia is a rare skeletal dysplasia characterised by 'happy natured' facies, short stature with short limbs, brachydactyly, and joint contractures. This report describes a case of a patient with unilateral Legg-Calvé-Perthes-like changes associated with dysplastic proximal capital femoral epiphysis, typical to geleophysic dysplasia.

Primary subacute epiphyseal osteomyelitis: role of conservative treatment.

Primary subacute epiphyseal osteomyelitis is a rare disease. Owing to its insidious onset, mild symptoms, and inconsistent supportive laboratory data, diagnosis and treatment are usually delayed. The authors report a retrospective review of 16 patients with hematogenous osteomyelitis primarily affecting the epiphysis. In all of these patients an osteolytic lesion developed. In eight patients it was confined to the epiphysis or apophysis alone; in the other patients there was contiguous involvement of the adjacent metaphysis. Complete clinical and radiologic healing was observed in all patients after antibiotic therapy alone. Based on this experience, in view of the controversy in the literature, the authors recommend a conservative treatment policy in the management of both epiphyseal and epiphyseal-metaphyseal subacute osteomyelitis as the treatment of choice. Surgery should be reserved for persistent infection that does not respond to appropriate antibiotic therapy or when bone lesions cannot be distinguished from bone tumors by use of all available imaging modalities.