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Serpentine fibula syndrome: expansion of the phenotype with three affected siblings.

We describe three siblings, one of whom has serpentine fibula syndrome (SFS) and has many facial and skeletal features in common with two deceased brothers, making it highly likely that they too had the condition. The karyotype of one of the deceased males was 47,XXY. These are the first affected sibs with SFS, and the first affected males. They all have other abnormalities which have not previously been described as part of the condition, namely congenital heart disease, inguinal herniae (two sibs), intestinal malrotation (two sibs) and coloboma (one sib). Facially they resemble the cases described by ter Haar et al. (1983), who also had congenital heart disease and a skeletal dysplasia though did not have the characteristic bowing of the fibulae. There are also features in common with Hadju-Cheney syndrome.

Abnormalities, Multiple↗

Medial fibula transport with the Ilizarov frame to treat massive tibial bone loss.

UNLABELLED: Massive segmental tibial bone loss from trauma, tumor, or infection is a limb-threatening situation. It is a considerable surgical challenge, especially when associated with extensive skin and soft tissue damage. Amputation was the only solution in the past, but current limb-salvage options include contralateral or ipsilateral microvascularized or free-fibular transfer. However, these methods are not without risks and disadvantages. We report seven patients with massive tibial bone loss treated by gradual medial transport of the ipsilateral fibula using an Ilizarov traction apparatus with olive wires after proximal and distal fibular osteotomies. This method has the advantages of avoiding surgery on the contralateral limb while allowing early weightbearing because of the stability of the Ilizarov frame. Hypertrophy of the transported fibula accompanied by full weightbearing and satisfactory joint motion occurred in all patients. All patients were satisfied with the results, and none thought amputation would have been a better treatment. The minimum followup was 5 years. We think the Ilizarov frame for ipsilateral fibular gradual transport is a reasonable alternative for limb salvage in patients with massive tibial bone loss. LEVEL OF EVIDENCE: Therapeutic Study, Level IV (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Adult↗

Results of free vascularized fibula grafting for allograft nonunion after limb salvage surgery for malignant bone tumors.

The purpose of this study was to assess the results of free vascularized fibula grafting (FVFG) in the treatment of allograft fracture nonunion after limb salvage surgery for malignant bone tumors.A retrospective study was performed on 8 patients who underwent FVFG for allograft fracture nonunions. All had prior tumor resection and allograft reconstruction for osteosarcoma (n = 6) or Ewing sarcoma (n = 2) of the femur (n = 3), tibia (n = 2), humerus (n = 2), or ulna (n = 1). All patients failed an initial course of immobilization; 4 patients failed prior open reduction and internal fixation with autogenous nonvascularized bone grafting. Average age at the time of FVFG was 14 years. Average follow-up was 44 months. The FVFG resulted in successful bony healing in 7 of 8 patients, providing pain relief, limb preservation, and restoration of function. One patient developed an infection requiring fibula removal and staged prosthetic reconstruction. Additional complications requiring further treatment included limb-length discrepancy, additional allograft fracture, and wound infection. The FVFG is an effective treatment option for allograft nonunion after limb salvage surgery because it provides both the mechanical stability and biological stimulus for bony healing. Attention to internal fixation, limb alignment, and microvascular principles is essential to prevent complications and allow for the best functional outcomes.

Adolescent↗

Late stress fracture of a well-incorporated autologous fibula strut graft in the cervical spine: a case report.

Four-level corpectomy for cervical spondylotic myelopathy presents special challenges to successful outcomes. At our institution, where the senior author has performed over 300 multilevel corpectomies with autologous fibula reconstruction, only 11 four-level procedures have been performed. It is important to consider the length of time required for complete revascularization of these long strut grafts as well as unusual postural or gait biomechanics the patient may have. Patients with altered gait or trunk stability who require anterior surgery for cervical spondylotic myelopathy may be best served by concurrent posterior fusion. We report a case in which a patient with cervical spondylotic myelopathy and diplegic cerebral palsy developed a stress fracture in the midportion of his well-incorporated autologous fibula strut graft 1 year after it was placed following four-level corpectomy.

Adult↗

Surgical planning in reconstruction of mandibular defect with fibula free flap: 15 patients.

The authors present a descriptive work on the reconstruction of large oromandibular defects with revascularized fibula free flaps in a group of patients treated by surgical resection of bony and soft tissues of the mandible. The principal objective is preoperative planning for the best morphological, functional, and aesthetic result. The authors report 15 reconstructions of wide oromandibular defects by revascularized fibula free flaps. The average patient age was 42 years. The mean follow-up period was 18 months. Correct surgical planning reduces surgical timing, minimizes the chance of failure during the reconstruction, and contemporaneously increases treatment efficacy and the best functional and aesthetic results.

Adult↗

Oromandibular reconstruction using a fibula osteocutaneous free flap: four different "preplating" techniques.

BACKGROUND: The aim of oromandibular reconstruction following a partially or fully extended resection is to recreate the normal morphology with a mandibular profile as similar as possible to the original and to restore oral function. Oromandibular reconstruction can be performed with fibula osteocutaneous free flaps combined with the preplating technique. The authors report their experience using the preplating technique for oromandibular reconstruction. METHODS: Four different methods can be used to mold the titanium plate that will be used in reconstruction against the existing mandible before resection. The preplating technique was used in 18 patients between 1998 and 2003. In eight patients, the external cortical bone of the mandible was spared by the tumor and the vestibular preplating technique was used. In one patient, the internal cortical bone was unaffected and the lingual preplating technique was used. When the tumor affected both the internal and external cortical bones, the double preplating technique was adopted in eight cases and Luhr's approach was used in one case. RESULTS: A good cosmetic appearance was obtained through the accurate reconstruction of the inferior mandibular border. Mandible continuity was restored, and the masticatory and oral muscles were anchored to the titanium plate, resulting in good occlusal and functional rehabilitation. CONCLUSION: The preplating technique combined with a fibula microvascular free flap is a useful, predictable method for oromandibular reconstruction.

Adolescent↗

Immediate mandibular reconstruction with microsurgical fibula flap transfer following wide resection for ameloblastoma.

Ameloblastoma, the most common odontogenic tumor, is a slow growing, localized tumor of the facial skeleton. Eighty percent of the tumors affect the mandible. Treatment by wide excision is curative in 95% of cases. Reconstruction by bone grafting has a 20% to 30% nonunion rate. We have treated three large ameloblastomae (>6 cm) with fibula flaps, following resection. All patients have healed. With follow-up times of 2 to 3 years, there has been no evidence of recurrence and no difficulties with postoperative evaluation for recurrent disease. We conclude that the fibula flap is a reliable reconstructive operation following resection of large ameloblastomae.

Adult↗

Multiplanar distraction osteogenesis of fibula free flaps used for secondary reconstruction of traumatic maxillary defects.

Traumatic maxillary bone loss, if not treated acutely, is accompanied by contracture of the overlying soft tissue envelope and loss of facial projection in three dimensions. Reconstruction aimed at replacing the bony architecture, expanding the soft tissue envelope and establishing a platform for dental rehabilitation can be accomplished in a staged approach. We present two patients who underwent replacement of missing maxillary segments with a free fibula flap, followed by distraction of the free fibula in three dimensions and eventual dental rehabilitation with osseo-integrated implants.

Adult↗

The fibula in congenital pseudoarthrosis of the tibia: the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

Fibular involvement in congenital pseudoarthrosis of the tibia (CPT) can be either a rare isolated pathology or is in association with the tibial changes. Out of 282 patients with CPT who had complete radiographic work-up, 62% (almost two thirds) demonstrated fibular pathology: 36% had true fibular pseudoarthrosis and the rest, i.e., 26% had fibular hypoplasia or dysplastic fibula. Neurofibromatosis was found in 62% of the patients with fibular pathology. The typical radiological features of tibial pseudoarthrosis are often missing in patients with fibular pathology. It is most probably because fibular changes precede the tibial involvement. In 250 patients with tibial surgical treatment, the highest rate of fusion occurred in patients with fibular involvement compared with those with normal fibula.

Child↗

Congenital pseudarthrosis of the tibia: treatment by transfer of the ipsilateral fibula with vascular pedicle.

We performed five transfers of the ipsilateral fibula in the treatment of congenital pseudarthrosis of the tibia. In all cases, conventional treatment by intramedullary-rod and autogenous-iliac bone grafting had failed at least once. We hoped to avoid the use of contralateral-fibular grafting or other methods that have not yet been proven effective. The ipsilateral fibula was transferred, and its vascular pedicle left intact and secured to both fragments of the tibial pseudarthrosis by cerclage wires. All five transfers have united and hypertrophied with growth. We believe this salvage method is superior to others not only because of the successful results, but also because the intact limb is left undisturbed.

Child↗

Tissue-engineered approach for the treatment of steroid-induced osteonecrosis of the femoral head: transplantation of autologous mesenchymal stem cells cultured with beta-tricalcium phosphate ceramics and free vascularized fibula.

Autologous mesenchymal stem cells (MSCs) cultured with beta-tricalcium phosphate (beta-TCP) ceramics and with a free vascularized fibula were transplanted into three patients with steroid-induced osteonecrosis of the femoral head. The average follow-up period was 34 months and the average patient age at the time of surgery was 28 years old. Fifteen milliliters of bone marrow was obtained from the patients 4 weeks before surgery, and was used for in vitro proliferation of MSCs. beta-TCP granules were immersed in the MSC suspension and the cells were further cultured for 2 weeks. Cultured MSCs/beta-TCP composite granules were implanted into the cavity that remained after curettage of necrotic bone; and finally, a free vascularized fibula was grafted. All hips showed preoperative collapse and radiographic progression was observed in two hips postoperatively. Osteonecrosis did not progress any further and early bone regeneration was observed. This tissue-engineered approach has potentials for the treatment of osteonecrosis. However, our results suggested that the present procedure could not be used for cases with severe preoperative collapse.

Adult↗

Cortical defect of the distal fibula: variant of ossification.

PURPOSE: To elucidate the nature of a normally occurring cortical defect of the distal fibula. MATERIALS AND METHODS: In patients with sprain and nonspecific ankle pain during a 2-year period, the authors reviewed ankle radiographs, computed tomographic (CT) scans, and magnetic resonance (MR) images. Cases were added from our file. Dried skeletons and cadavers were evaluated with radiography for the presence of this cortical defect. RESULTS: Fifteen (1.8%) of 847 radiographs in 739 patients (male, 377; female, 362; age range, 15-84 years) showed a small cortical defect in the anterior aspect at the level of the distal tibiofibular joint. The age range of the subjects with the defect (male, four; female, 11) was 16-66 years. MR imaging in two patients showed a small low-signal-intensity area at the insertion site of the anterior tibiofibular ligament. Evaluation of 148 skeletons (75 subjects) revealed similar cortical defect in two cases (1.4%). CONCLUSION: The cortical defect of the distal fibula, at the insertion site of the anterior tibiofibular ligament, has no clinical significance and should not be confused with neoplasm.

Adolescent↗

A functional analysis of fusion of the tibia and fibula in the rat and mouse.

The distal diaphyseal shafts of the rat tibia and fibula fuse postnatally, a process initiated by the seventh day with the formation of secondary cartilage that is subsequently replaced by endochondral ossification. The histological appearance of this fusion process is described. A homologous process occurs postnatally in the mouse. An analysis of some of the pertinent recent developmental, comparative and paleontological data indicated that extrinsic, biomechanical factors probably played a significant causal role in tibia-fibula fusions. The production of secondary cartilage is evoked by extrinsic forces, and these fusions were analyzed within the present concepts of intrinsic and extrinisic factors in skeletogenesis. It was concluded that the fusions currently reported fit well within the hypothesis of the functional matrix and that, such fusions between skeletal tissues are secondary, compensatory and mechanically obligatory responses to the prior demands of functionally related nonskeletal tissues and organs.

Animals↗

The role of a posteriorly positioned fibula in ankle sprain.

BACKGROUND: Specific anatomic variations of the ankle mortise may predispose people to ankle sprains. HYPOTHESIS: There is a correlation between a higher malleolar index (posteriorly positioned fibula) and incidence of ankle sprain. STUDY DESIGN: Prospective case control study. METHODS: We compared the malleolar index (transverse plane of the talus) on computerized axial tomographic images of 61 patients with ankle sprain with that of 101 normal controls. A positive number for the malleolar index meant that the lateral malleolus was posterior to the plane of the medial malleolus. A negative number meant that the lateral malleolus was actually anterior to the plane of the medial malleolus. RESULTS: The average malleolar index of the patients with ankle sprain was +11.5 degrees with a standard deviation of 7 degrees. Malleolar relationships varied from -6 degrees to +39 degrees, a range of 45 degrees. The average malleolar index in the control group was +5.85 degrees with a standard deviation of 4.9 degrees, which varied from -8 degrees to +16 degrees. However, there was no correlation between recurrence of sprains and malleolar index values. CONCLUSION: Patients with an ankle sprain were more likely to have a posteriorly positioned fibula, possibly predisposing them to ankle sprain.

Adolescent↗

Chronic, painful ankle instability in skeletally immature athletes. Ununited osteochondral fractures of the distal fibula.

We report 10 cases of chronic, painful ankle instability in skeletally immature athletes associated with ununited osteochondral fragments of the distal fibula. All the patients experienced multiple inversion injuries with recurrent pain and instability that failed to improve after a supervised exercise and rehabilitation program. Osteochondral fragments of the distal fibula were visible on all standard radiographs of the affected ankles. Treatment consisted of operative excision of the bony fragment and an anatomic nonaugmented repair of the lateral ligament complex and capsule. All 10 patients were available for long-term followup an average of 6.5 years (+/-2) after surgery; all ankles improved functionally, with no recurrences of instability.

Adolescent↗

Health-related quality of life in patients with transtibial amputation and reconstruction with bone bridging of the distal tibia and fibula.

BACKGROUND: Bone-bridging (arthrodesis of the distal tibia and fibula) at the time of transtibial amputation is a controversial operative technique that is anecdotally reported to improve the weightbearing capacity of the residual limb and to decrease residual limb discomfort. METHODS: Thirty-two consecutive patients with multiple diagnoses had transtibial amputation with a distal tibial-fibular bone-bridge, all done by a single surgeon (MAP). At an average of 16.3 months after surgery all patients completed the Prosthetics Evaluation Questionnaire (PEQ), a validated outcomes instrument specifically created to evaluate quality of life and functional demands in patients with a lower extremity amputations. Their responses were compared with those of 17 preselected, highly functional transtibial amputees from two academic medical centers who previously had transtibial amputations using a traditional non bone-bridge operative technique; their time since amputation averaged 14.7 years. RESULTS: The "nonselected" consecutive patients with a bone-bridged residual limb scored higher (more favorable) in the Ambulation (p = 0.037) and Frustration (p < 0.001) domains of the PEQ and lower (less favorable) in the Appearance (p = 0.025) subscale. Their scores were similar in the other six domains. CONCLUSIONS: Patients of multiple ages with multiple diagnoses who had bone-bridging of the distal tibia and fibula at the time of transtibial amputation had scores on a validated outcomes instrument that were better than or comparable to those of a selected group of highly functional transtibial amputees. The results of this study suggest that bone-bridging at the time of transtibial amputation may enhance patient-perceived functional outcomes.

Adolescent↗

An anatomic study of the short oblique fracture of the distal fibula and ankle stability.

The short oblique fracture of the distal fibula occurring as a stage 2 supination-external rotation injury was investigated in respect to its effect on ankle stability in a series of cadaver dissections. Approximately 25 and 20 degrees of external rotational displacement of the distal fibula and talus, respectively, as well as approximately 1 mm of direct lateral talar shift were noted to be possible with this injury. This degree of rotational or lateral talar displacement was seen to result in tibiotalar joint incongruity. The deltoid ligament effectively prevented talar eversion but not the initial 2 to 3 mm of lateral talar displacement. Ankle stability in respect to medial talar shift was not compromised by removal of the medial malleolus.

Adult↗

The short oblique fracture of the distal fibula without medial injury: an assessment of displacement.

Eighteen patients with ankle injuries presenting as short oblique fractures of the distal fibula with no clinical or radiographic evidence of injury to the medial ankle were studied for fracture displacement. Plain radiographs and computed tomography were used for analysis. All fractures were clinically diagnosed as supination-external rotation stage 2 (SE-II) injuries under the Lauge-Hansen scheme. All exhibited slight displacement on plain radiographs and were treated nonoperatively. Computerized tomography using axial cuts across the fracture site and ankle mortise revealed normal positioning of the talus beneath the tibial plafond, as evidenced by no abnormality of the medial joint space in all patients. In the majority of patients, the relationship between the talus and distal fibula also appeared undisturbed, with fracture displacement being confined to a change in position of the proximal fibular fragment relative to the tibia as compared with the contralateral ankle. In a minority of cases, in addition to the above-described displacement of the proximal fibular fragment, the distal fibular fragment was noted to shift slightly laterally relative to the talus, with mild widening of the lateral joint space. Occult-associated avulsion fractures off the distal tibia were present in 39% of the cases.

Adult↗