Clavicular bone defects managed with free vascularised fibular grafting: evidence to date.

Reconstructive surgery of the clavicle using free vascularised fibula grafting (FVFG) is sometimes required for the management of severe bone loss or non-union. As the procedure is relatively rare, there is no universal agreement on the management and outcome. This systematic review aimed to first, identify the conditions for which FVFG has been applied; second, to gain an understanding of the surgical techniques used; and third, to report outcomes related to bone union, infection eradication, function and complications. A PRISMA strategy was used. Medline, Cochrane Central Register of Controlled Trials, Scopus and EMBASE library databases were interrogated using pre-defined MeSH terms and Boolean operators. Quality of evidence was evaluated based on OCEBM and GRADE systems. Fourteen studies based on 37 patients were identified with a mean follow-up time of 33.3 months. The most common reasons for the procedure were: fracture non-union; tumours requiring resection; post-radiation treatment osteonecrosis and osteomyelitis. The operation approaches were similar, involving graft retrieval, insertion and fixation and vessels chosen for reattachment. The mean clavicular bone defect size was 6.6 cm (± 1.5), prior to FVFG. Bone union occurred in 94.6% with good functional outcomes. Complete infection eradication occurred in those with preceding osteomyelitis. The main complications were broken metalwork, delayed union/non-union and fibular leg paraesthesia (n = 20). The mean re-operation number was 1.6 (range 0-5.0). The study demonstrates that FVFG is well tolerated and has a high success rate. However, patients should be advised about complication development and re-intervention requirement. Interestingly, overall data is sparse with no large cohort groups or randomised trials.

Treatment of Recalcitrant Isolated Congenital Fibular Pseudarthrosis: Fibular Segment Transfer and Tibiofibular Synostosis with Unilateral External Fixator.

Isolated congenital pseudarthrosis of the fibula is a rare entity with a limited number of cases reported in the literature. Treatment is challenging because of recalcitrant nonunion and because no consensus about the best treatment plan exists. We report a case of isolated congenital fibular pseudarthrosis with valgus deformity of the ankle. The patient had a history of two failed operations. We used a novel surgical plan that combined tibiofibular synostosis with fibular segment transfer through a unilateral external fixator. The patient showed good early results with fibular union. We advocate the combination of tibiofibular synostosis and fibular segment transfer to restore the integrity and stability of the ankle in recalcitrant isolated congenital fibular pseudarthrosis cases with a history of failed surgery.

Post-traumatic radioulnar synostosis: a retrospective case series of 10 patients in Kuwait.

BACKGROUND: The development of radioulnar synostosis due to post-traumatic injuries of the elbow or forearm can lead to debilitating outcomes. Several treatment options are available to hinder the progression and prevent recurrence. We used a combination of these treatments in a series of patients and observed the outcomes. METHODS: We conducted a retrospective study of 10 patients with post-traumatic radioulnar synostosis (9 men and 1 woman) who required surgical intervention in a tertiary orthopedic center. All of these patients were subjected to the same treatment combination (preoperative radiotherapy, tissue interposition after heterotopic ossification resection, and adjuvant indomethacin postoperatively). Improvement in range of motion (flexion, extension, and rotation) and the Mayo score was assessed and compared preoperatively and postoperatively via statistical analysis. RESULTS: In comparison to the patients' preoperative state, which ranged from poor to fair, all 10 patients reported excellent Mayo scores after intervention with the triple therapy combination, with a mean Mayo score of 36 ± 10.2 points. Flexion, extension, and rotation improved by mean values of 55.2° ± 38.7°, 50.2° ± 34.0°, and 47.9° ± 40.0°, respectively. There was 1 complication that has subsided on follow-up. CONCLUSION: The triple therapy combination was found to provide good functional and prophylactic results preventing recurrence.

[Risk Factors for the Development of Distal Tibiofibular Synostosis after Unstable Ankle Fractures]. / Rizikové faktory pro vznik distální tibiofibulární synostózy po luxacních zlomeninách hlezna.

PURPOSE OF THE STUDY The study analyses a cohort of patients with surgically treated ankle fractures who developed complete distal tibiofibular synostoses. It focuses on their occurrence and association with the extent of tibiotalar dislocation of the ankle joint on the trauma X-ray and its relation to the choice of surgery. MATERIAL AND METHODS The cohort of a total of 824 patients with type B and C fractures according to Weber classification was followed up for 9 years. The cohort consisted of 403 (48.9%) men and 421 (51.1%) women. The exclusion criteria included associated talus fractures, calcaneus fractures and fractures of the other bones of the foot. The studied data were obtained retrospectively from medical documentation and by evaluation of trauma X-rays and X-rays obtained during the postoperative checks. The ankle fractures were classified based on the Weber classification and the basic epidemiologic data (age and gender), type of fracture and extent of tibiotalar dislocation of ankle fractures on the trauma X-ray were evaluated. Posttraumatic ankle dislocation was divided into tibiotalar dislocation > 10 mm, tibiotalar dislocation < 10 mm and the group with regular ankle joint. When evaluating the treatment method, the cohort was divided into three groups: Group 1 with one-stage osteosynthesis, Group 2 with temporary K-wire transfixation or external fixation and subsequent secondary conversion to internal osteosynthesis, and Group 3 with definitive transfixation or external fixation of the ankle. The results were statistically evaluated using the Pearson s chi-square test, or the Fisher s exact test for low frequencies. A multivariant logistic regression model was created to identify statistically significant factors contributing to the development of synostosis. The results with the p-value < 0.05 were considered statistically significant. RESULTS In the whole cohort, the synostosis of distal tibiofibular joint was observed in a total of 131 (15.9%) patients. In men it was in 85 (21.1%) cases and in women in 46 (10.9%) cases, which was statistically significant (p < 0.0001). There was a statistically significant difference (p = 0.0020) between the mean age in the group of patients with synostosis (54.4 years) and the mean age in the group of patients without synostosis (49.1 years). Complete distal tibiofibular synostoses were found in 78 (12.7%) fractures classified as type B according to the Weber classification and in 53 (25.5%) type C fractures. When taking into account the gender, synostoses occurred more frequently in men in both types of fractures classified based on the Weber classification, only in type C fractures no statistical significance was established (p = 0.3026). Various size of posttraumatic tibiotalar dislocation was present in both types of fractures. The group with less severe type B ankle fractures showed a statistically significant dominance of synostosis development in cases with large tibiotalar dislocation of more than 10 mm (p<0.0001). In the group with type C fractures different results were obtained. The highest frequency of cases with synostosis was reported in the group with dislocation smaller than 10 mm (p = 0.0698). In the entire cohort, 615 (74.6%) one-stage osteosyntheses were performed and synostoses developed in 77 (12.5%) cases. In 165 (20.0%) patients, transfixation with K-wires or external fixation with subsequent conversion to secondary osteosynthesis were used and synostoses were identified in 50 (30.3%) cases (p < 0.0001). The open fractures showed an insignificantly smaller number of synostoses than the closed fractures (p = 0.5902). DISCUSSION Posttraumatic distal tibiofibular synostoses have varied morphology. A number of studies confirmed that they do not affect much the functional status of the ankle, even despite their extensive finding in the area of syndesmosis is evident on the Xray. Etiologically, a certain role in their development is reported to be played by posttraumatic hematoma in case of damage to deep soft and bony structures of the ankle. CONCLUSIONS A higher occurrence of synostoses was observed in male population, older age patients and also in type C fractures according to the Weber classification. Larger tibiotalar dislocation showed statistical significance in the development of synostoses in type B fractures according to the Weber classification, whereas in type C fractures it was not the main factor contributing to the development of synostosis. In cases where one-staged osteosynthesis was performed, the occurrence of synostoses was statistically significantly lower than in secondary osteosynthesis after temporary stabilisation. Key words: ankle fracture, distal tibiofibular synostosis, ankle joint dislocation, Weber classification, acute surgery, delayed surgery.

GDF6 Knockdown in a Family with Multiple Synostosis Syndrome and Speech Impairment.

Multiple synostoses syndrome type 4 (SYNS4; MIM 617898) is an autosomal dominant disorder characterized by carpal-tarsal coalition and otosclerosis-associated hearing loss. SYSN4 has been associated with GDF6 gain-of-function mutations. Here we report a five-generation SYNS4 family with a reduction in GDF6 expression resulting from a chromosomal breakpoint 3' of GDF6. A 30-year medical history of the family indicated bilateral carpal-tarsal coalition in ~50% of affected family members and acquired otosclerosis-associated hearing loss in females only, whereas vertebral fusion was present in all affected family members, most of whom were speech impaired. All vertebral fusions were acquired postnatally in progressive fashion from a very early age. Thinning across the 2nd cervical vertebral interspace (C2-3) in the proband during infancy progressed to block fusion across C2-7 and T3-7 later in life. Carpal-tarsal coalition and pisiform expansion were bilaterally symmetrical within, but varied greatly between, affected family members. This is the first report of SYNS4 in a family with reduced GDF6 expression indicating a prenatal role for GDF6 in regulating development of the joints of the carpals and tarsals, the pisiform, ears, larynx, mouth and face and an overlapping postnatal role in suppression of aberrant ossification and synostosis of the joints of the inner ear (otosclerosis), larynx and vertebrae. RNAseq gene expression analysis indicated >10 fold knockdown of NOMO3, RBMXL1 and NEIL2 in both primary fibroblast cultures and fresh white blood cells. Together these results provide greater insight into the role of GDF6 in skeletal joint development.

Intragenic Deletions in FLNB Are Part of the Mutational Spectrum Causing Spondylocarpotarsal Synostosis Syndrome.

Spondylocarpotarsal synostosis syndrome (SCT) is characterized by vertebral fusions, a disproportionately short stature, and synostosis of carpal and tarsal bones. Pathogenic variants in FLNB, MYH3, and possibly in RFLNA, have been reported to be responsible for this condition. Here, we present two unrelated individuals presenting with features typical of SCT in which Sanger sequencing combined with whole genome sequencing identified novel, homozygous intragenic deletions in FLNB (c.1346-1372_1941+389del and c.3127-353_4223-1836del). Both deletions remove several consecutive exons and are predicted to result in a frameshift. To our knowledge, this is the first time that large structural variants in FLNB have been reported in SCT, and thus our findings add to the classes of variation that can lead to this disorder. These cases highlight the need for copy number sensitive methods to be utilized in order to be comprehensive in the search for a molecular diagnosis in individuals with a clinical diagnosis of SCT.

A novel approach for simultaneous tibiofibular synostosis takedown and peroneus longus ligamentoplasty for posttraumatic tibiofibular synostosis: a case report and review of the literature.

INTRODUCTION: A singular procedure involving both a distal tibiofibular synostosis resection with syndesmosis repair by peroneus longus ligamentoplasty has not been reported in the English literature. We report a case of simultaneous distal tibiofibular synostosis resection and syndesmosis stabilization by peroneus longus ligamentoplasty for the treatment of symptomatic distal tibiofibular synostosis formation, following neglected syndesmosis injury. CASE PRESENTATION: A 42-year-old Caucasian man presented with ankle pain and painful range of motion 20 months following ankle trauma. Distal tibiofibular synostosis was identified, and our patient was successfully treated by simultaneous synostosis takedown and peroneus longus ligamentoplasty for distal tibiofibular syndesmosis repair. CONCLUSIONS: Our experience illustrates that in cases of painful posttraumatic distal tibiofibular synostosis, simultaneous synostosis resection with peroneus longus ligamentoplasty may show good clinical results. LEVEL OF EVIDENCE: 5.

Rare Complication of Distal Radius and Ulnar Styloid Fractures with Percutaneous Fixation: A Case Report on Distal Radioulnar Synostosis.

CASE: A 42-year-old man presented with distal radius fracture. We performed external fixation combined with Kirschner wiring, which was removed 6 weeks postoperatively. After the removal of the implants, the patient could not achieve any pronation-supination, and distal radioulnar synostosis became apparent during the follow-up. The patient underwent distal ulnar osteotomy, and 60° pronation and full supination were achieved. No complications were reported at the 32-month follow-up. CONCLUSION: This is a rare case of radioulnar synostosis after percutaneous fixation surgery for distal radius fracture. The modified Sauve-Kapandji procedure can help restore motion, together with other appropriate postoperative interventions, and provides early mobilization.

Chronic dysphagia caused by Laryngo-vertebral Synostosis after anterior fusion for cervical spine trauma: a case report.

BACKGROUND: Anterior cervical spine surgery is often associated with postoperative dysphagia, but chronic dysphagia caused by laryngo-vertebral synostosis is extremely rare. We report a case of chronic dysphagia caused by synostosis between the cricoid cartilage and cervical spine after anterior surgery for cervical spine trauma. CASE PRESENTATIONS: We present a case of a 39-year-old man who had sustained complex spine trauma at C5-6 associated with complete spinal cord injury at the age of 22; the patient presented with a 5-year history of chronic dysphagia. Computed tomography demonstrated posterior shift of the esophagus as well as calcification of the cricoid cartilage and its fusion to the right anterior tubercle of the C5 vertebra. A barium swallow study demonstrated significant barium aspiration into the airway and no laryngeal elevation. The patient underwent resection of the bony bridge and omohyoid muscle flap insertion. His symptoms ameliorated after surgery. CONCLUSION: Synostosis between the cricoid cartilage and cervical spine may occur associated with cervical spine trauma and causes chronic dysphagia. Resection of the fused part can improve dysphagia caused by this rare condition and omohyoid muscle flap might be a good option to prevent recurrence.

First case of compound heterozygous BHLHA9 variants in mesoaxial synostotic syndactyly with phalangeal reduction.

Mesoaxial synostotic syndactyly with phalangeal reduction (MSSD) is an extremely rare autosomal recessive limb abnormality characterized by the fusion of third and fourth fingers. To date, only homozygous missense and frameshift mutations have been reported in BHLHA9 associated to MSSD. In this study, we report a patient who presented with clinical and radiological features of MSSD. A customized skeletal dysplasia NGS panel revealed the presence of two novel compounds heterozygous variants in BHLHA9: NM_001164405.1: c.[226A>T][269G>C]; p.[(Lys76*)][(Arg90Pro)]. Thus, this is the first case of MSSD in a nonconsanguineous family.

Successful Treatment of Pediatric Posttraumatic Tibiofibular Synostosis by Resection and Peroneal Artery Perforator Adipofascial Flap: A Case Report.

CASE: Pediatric posttraumatic tibiofibular synostosis (PPTFS) is a rare postfracture complication that often leads to growth abnormalities. There are very few reports demonstrating the long-term efficacy of surgical treatment. An 11-year-old boy with PPTFS displayed progressive prominence of the fibular head and shortening of the lateral malleolus subsequent to a fracture suffered at the age of 5 years. He was treated by synostosis resection and a peroneal artery perforator adipofascial flap. Eight years postoperatively, the synostosis had not recurred, and his earlier growth abnormalities were nearly normal. CONCLUSIONS: Peroneal artery perforator adipofascial flap is effective for pregrowth spurt PPTFS.

Union Rates and Reported Range of Motion Are Acceptable After Open Forearm Fractures in Military Combatants.

BACKGROUND: High-energy open forearm fractures are unique injuries frequently complicated by neurovascular and soft tissue injuries. Few studies have evaluated the factors associated with nonunion and loss of motion after these injuries, particularly in the setting of blast injuries. QUESTIONS/PURPOSES: (1) In military service members with high-energy open forearm fractures, what proportion achieved primary or secondary union? (2) What is the pronation-supination arc of motion as stratified by the presence or absence of heterotopic ossification (HO) and synostosis? (3) What are the risks of heterotopic ossification and synostosis? (4) What factors may be associated with forearm fracture nonunion? METHODS: A retrospective study of all open forearm fractures treated at a tertiary military referral center from January 2004 to December 2014 was performed. In all, 76 patients were identified and three were excluded, leaving 73 patients for inclusion. All 73 patients had serial radiographs to assess for HO and union. Only 64 patients had rotational range of motion (ROM) data. All patients returned to the operating room at least once after initial irrigation and débridement to ensure the soft tissue envelope was stable before definitive fixation. The indication for repeat irrigation and débridement was determined by clinical appearance. Patient demographics, fracture and soft tissue injury patterns, surgical treatments, neurovascular status at the time of injury, incidence of infection, heterotopic ossification (defined as the presence of heterotopic bone visible on serial radiographs), radioulnar synostosis, bony status after initial definitive treatment (union, nonunion, or amputation), and forearm rotation at final followup were retrospectively obtained from chart review by someone other than the operating surgeon. Seventy-six open forearm fractures in 76 patients were reviewed; 73 patients were examined for osseous union as three went on to early amputation, and 64 patients had forearm ROM data available for analysis. Union was determined by earliest radiology or orthopaedic staff official dictation stating the fracture was healed. Nonunion was defined as the clinical determination by the orthopaedist for a repeat procedure to achieve bony union. Secondary union was defined as union after reoperation to achieve bony union, and final union was defined as overall percentage of patients who were healed at final followup. Of the patients analyzed for union, 20 had less than 1 year of followup, and of these, none had nonunion. Of the patients analyzed for ROM, eight patients had less than 6 months of followup (range, 84-176 days). Of these, one patient had decreased ROM, none had a synostosis, and the remaining had > 140° of motion. RESULTS: Initial treatment resulted in primary union in 62 of 73 patients (85%); secondary union was achieved in eight of 11 patients (73%); and final union was achieved in 70 of 73 patients (96%). Although pronation-supination arc in patients without HO was 140° ± 35°, a limited pronation-supination arc was primarily associated with synostosis (arc: 40° ± 40°; mean difference from patients without HO: 103° [95% confidence interval {CI}, 77°-129°], p < 0.001); patients with HO but without synostosis had fewer limitations to ROM than those with synostosis (arc: 110° ± 80°, mean difference: 77° [35°-119°], p < 0.001). Heterotopic ossification developed in 40 of 73 patients (55%), including a radioulnar synostosis in 14 patients (19%). Bone loss at the fracture site (relative risk (RR) 6.2; 95% CI, 1.8-21) and healing complicated by infection (RR, 9.9; 95% CI, 4.9-20) were associated with the development of nonunion after initial treatment. Other potential factors such as smoking status, vascular injury, both-bone involvement, need for free flap coverage and blast mechanism were not associated. CONCLUSIONS: Despite a high-energy mechanism of injury and high rate of soft tissue defects, the ultimate probability of fracture union in our series was high with a low infection risk. Nonunions were associated with bone loss and deep infection. Functional motion was achieved in most patients despite increased burden of HO and synostosis compared with civilian populations. However, if synostosis did not develop, HO itself did not appear to interfere with functional ROM. Future investigations may provide improved decision-making tools for timing of fixation and prophylactic means against HO synostosis. LEVEL OF EVIDENCE: Level III, therapeutic study.

Update on leg fractures in paediatric patients.

Leg fractures are common and further increasing in prevalence in paediatric patients. The diagnosis is readily made in most cases. Choosing the best treatment is the main issue. Non-operative treatment is the reference standard for non-displaced or reducible and stable fractures but requires considerable expertise and close monitoring, as well as an immobilisation period that far exceeds 3 months in many cases. Some surgical teams therefore offer elastic stable intra-medullary nailing (ESIN) as an alternative to children who do not want to be immobilised for several months. Internal fixation is required for unstable or irreducible leg fractures. ESIN is often used as the first-line method, based on its very good risk/benefit ratio. For fractures that do not lend themselves to ESIN, optimal stabilisation can be achieved by choosing among the other available options (screw-plate fixation, rigid intra-medullary nailing or external fixation) on a case-by-case basis. Close monitoring during the first few days is crucial to ensure the early detection of compartment syndrome. The other complications and sequelae are non-specific.

Severity of ulnar deficiency and its relationship with lower extremity deficiencies.

To assess the characteristics of ulnar deficiency (UD) and their relationship to lower extremity deficiencies, we retrospectively classified 82 limbs with UD in 62 patients, 55% of whom had femoral, fibular, or combined deficiencies. In general, UD severity classification at one level (elbow, ulna, fingers, thumb/first web space) statistically correlated with similar severity at another. Ours is the first study to show that presence of a lower limb deficiency is associated with less severe UD on the basis of elbow, ulnar, and thumb/first web parameters. This is consistent with the embryological timing of proximal upper extremities developing before the lower extremities.

Major complications after distal biceps tendon repairs: retrospective cohort analysis of 970 cases.

BACKGROUND: The major complication and reoperation rates after distal biceps repair are poorly defined. The purpose of this large retrospective cohort study of distal biceps repairs performed by multiple surgeons within a large orthopedic group was to more clearly define the rates and risk factors of clinically impactful major complications and reoperations. METHODS: All distal biceps tendon repairs performed from January 2005 through April 2017 with a minimum 2-month follow-up were identified using Current Procedural Terminology code 24342. We included 970 patients. The primary outcome measure was the total major complication rate. Reoperations, minor complications, and risk factors were also tracked. RESULTS: Repairs were performed via a single anterior incision in 652 cases and a 2-incision exposure in 318 cases. A 7.5% major complication rate and 4.5% reoperation rate were observed overall. Major complications occurred at the following rates: proximal radioulnar synostosis, 1.0%; heterotopic ossification or loss of range of motion with reoperation, 0.9%; tendon rerupture, 1.6%; deep infection, 0.5%; posterior interosseous nerve palsy, 1.9%; and complex regional pain syndrome, 0.6%. The 2-incision exposure was identified as a significant risk factor for the development of proximal radioulnar synostosis when compared with single-incision repair techniques (P = .0003; odds ratio, 19), occurring in 2.8% of 2-incision exposure cases. Lateral antebrachial cutaneous nerve neuritis or numbness and radial sensory nerve neuritis or numbness were documented more frequently in the postoperative period among patients treated with a single-incision exposure (P < .0001 and P = .034, respectively). CONCLUSIONS: Distal biceps repair is associated with a 7.5% major complication rate and 4.5% reoperation rate. The use of a 2-incision technique for repair increases the risk of radioulnar synostosis.

The reverse Sauvé-Kapandji procedure for the treatment of (posttraumatic) proximal radioulnar synostosis.

Proximal radioulnar synostosis is a rare but highly disabling posttraumatic complication in periarticular elbow injuries. Surgical treatment is an option for functionally limiting proximal radioulnar synostosis; however, the approach can endanger local neurovascular structures, especially if the synostosis affects the level of the bicipital tuberosity. We report two cases of proximal radioulnar synostosis with a preoperative prono-supination range of motion of 0° and 15° treated by a reverse Sauvé-Kapandji procedure resecting a 1-cm section of the radial shaft distal to the bicipital tuberosity and leaving the synostosis in place. An improvement in prono-supination arc of motion of 82.5° was achieved at 2 years of follow-up with no complications associated with the technique. The reverse Sauvé-Kapandji procedure could be an option in the treatment of proximal radioulnar synostosis in selected cases.

Circumferential Adipofascial Graft for Prevention of Recurrence of Posttraumatic Radioulnar Synostosis.

Radioulnar synostosis can cause substantial loss of function, and surgical treatment can be challenging. Recurrence of the contracture related to scar or reformation of the synostosis is problematic. Several techniques have been described for prevention of recurrence. We present a technique utilizing a free wrap around adipofascial graft for interposition and circumferential coverage of the ulna after resection of the heterotopic bone. We believe this technique has the advantages of technical simplicity, secure interposition, and reliable outcomes.