Non-vascularised fibula grafts for reconstruction of segmental and hemicortical bone defects following meta- /diaphyseal tumour resection at the extremities.

BACKGROUND: The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challenging, and biological treatment options should be applied whenever possible, especially in benign lesions and early stage sarcomas. We aimed to evaluate the results of segmental (SR) and hemicortical reconstructions (HR) at the extremities using non-vascularised fibula grafts. METHODS: We retrospectively enrolled 36 patients who were treated with non-vascularised fibula reconstructions (15 SR, 21 HR) after bone tumour resection (15 malignant, 21 benign). All cases were evaluated regarding consolidation, hypertrophy at the graft-host junctions, and complications; moreover, the functional and oncological results were assessed. The mean follow-up was 8.3 years (2.1-26.6 years). RESULTS: Primary union was achieved in 94% (SR 87%, HR 100%) of patients, and 85% (SR 81%, HR 88%) showed hypertrophy at the graft-host junction. The overall complication rate was 36% with 4 patients (11%) developing local recurrence. There was a significant correlation between the development of mechanical complications (fracture, delayed-/non-union) and a defect size of ≥12 cm (p = 0.013), segmental defects (p = 0.013) and additional required treatment (p = 0.008). The functional outcome was highly satisfactory (mean MSTS score 86%). CONCLUSIONS: Due to encouraging results and advantages (such as their remodelling capacity at the donor site), non-vascularised fibula reconstructions should be considered a valuable alternative treatment option for patients with hemicortical defects or segmental reconstructions of less than 12 cm in which no additional neo-/adjuvant treatment is necessary.

Standardized diagnostic criteria for developmental dysplasia of the hip in early infancy.

BACKGROUND: Clinicians use various criteria to diagnose developmental dysplasia of the hip (DDH) in early infancy, but the importance of these various criteria for a definite diagnosis is controversial. The lack of uniform, widely agreed-on diagnostic criteria for DDH in patients in this age group may result in a delay in diagnosis of some patients. QUESTIONS/PURPOSES: Our purpose was to establish a consensus among pediatric orthopaedic surgeons worldwide regarding the most relevant criteria for diagnosis of DDH in infants younger than 9 weeks. MATERIAL AND METHODS: We identified 212 potential criteria relevant for diagnosing DDH in infants by surveying 467 professionals. We used the Delphi technique to reach a consensus regarding the most important criteria. We then sent the survey to 261 orthopaedic surgeons from 34 countries. RESULTS: The response rate was 75%. Thirty-seven items were identified by surgeons as most relevant to diagnose DDH in patients in this age group. Of these, 10 of 37 (27%) related to patient characteristics and history, 13 of 37 (35%) to clinical examination, 11 of 37 (30%) to ultrasound, and three of 37 (8%) to radiography. A Cronbach alpha of 0.9 for both iterations suggested consensus among the panelists. CONCLUSION: We established a consensus regarding the most relevant criteria for the diagnosis of DDH in early infancy and established their relative importance on an international basis. The highest ranked clinical criteria included the Ortolani/Barlow test, asymmetry in abduction of 20° or greater, breech presentation, leg-length discrepancy, and first-degree relative treated for DDH. LEVEL OF EVIDENCE: Level IV, diagnostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions.

This retrospective study analyses 23 children treated with vertical expandable prosthetic titanium rib (VEPTR) for correction of non-congenital early onset spine deformities. After the index procedure (IP), the device was lengthened at 6-month intervals. The average (av) age at the time of IP was 6.5 years (1.11-10.5). The av follow-up time was 3.6 years (2-5.8). Diagnosis included 1 early onset idiopathic scoliosis, 11 neuromuscular, 2 post-thoracotomy scoliosis, 1 Sprengel deformity, 2 hyperkyphosis, 1 myopathy and 5 syndromic. Surgeries (187) included 23 IPs, av 6.5 (4-10) device expansions per patient (149) and 15 unplanned surgeries. 23 complications (0.13 per surgery) included 10 skin sloughs, 5 implant dislocations, 2 rod breakages and 6 infections. Coronal Cobb angle was av 68 degrees (11 degrees -111 degrees ), at follow-up av 54 degrees (0 degrees -105 degrees). Pelvic obliquity was av 33 degrees (13 degrees -60 degrees ), at follow-up av 16 degrees (0 degrees -42 degrees ). T1 tilt was av 29 degrees (5 degrees -84 degrees ), two remained unchanged, the remainder improved 10 degrees -68 degrees. Sagittal plane: All but two had stable profiles, two hyperkyphosis of 110 degrees /124 degrees improved to 56 degrees /86 degrees. Space available for lung ratio was less than 90% in ten before the IP, improved in nine and deteriorated in one. Originally designed for thoracic insufficiency syndromes related to rib and vertebral anomalies, VEPTR proved to be a valuable alternative to dual growing rods for non-congenital early onset spine deformities. The complication rate was lower, the control of the sagittal plane and the pelvic obliquity was as good, but the correction of the coronal plane deformity was less than growing rods. However, VEPTR's spine-sparing approach might provoke less spontaneous spinal fusion and ease the final correction at maturity.

Coronal plane segmental flexibility in thoracic adolescent idiopathic scoliosis assessed by fulcrum-bending radiographs.

Knowledge about segmental flexibility in adolescent idiopathic scoliosis is crucial for a better biomechanical understanding, particularly for the development of fusionless, growth-guiding techniques. Currently, there is lack of data in this field. The objective of this study was, therefore, to compute segmental flexibility indices (standing angle minus corrected angle/standing angle). We compared segmental disc angles in 76 preoperative sets of standing and fulcrum-bending radiographs of thoracic curves (paired, two-tailed t tests, p < 0.05). The mean standing Cobb angle was 59.7 degrees (range 41.3 degrees -95 degrees ) and the flexibility index of the curve was 48.6% (range 16.6-78.8%). The disc angles showed symmetric periapical distribution with significant decrease (all p values <0.0001) for every cephalad (+) and caudad (-) level change. The periapical levels +1 and -1 wedged at 8.3 degrees and 8.7 degrees (range 3.5 degrees -14.8 degrees ), respectively. All angles were significantly smaller on the-bending views (p values <0.0001). We noted mean periapical flexibility indices of 46% (+1), 49% (-1), 57% (+2) and 81% (-2), which were significantly less (p < 0.001) than for the group of remote levels 105% (+3), 149% (-3), 231% (+4) and 300% (-4). The discal and bony wedging was 60 and 40%, respectively, and mean values 35 degrees and 24 degrees (p < 0.0001). Their relationship with the Cobb angle showed a moderate correlation (r = 0.56 and 0.45). Functional, radiographic analysis of idiopathic thoracic scoliosis revealed significant, homogenous segmental tethering confined to four periapical levels. Future research will aim at in vivo segmental measurements in three planes under defined load to provide in-depth data for novel therapeutic strategies.

Extra-abdominal desmoid tumours - further evidence for the watchful waiting policy.

PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres. METHODS: The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0–40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities. RESULTS: The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0–11.5 years). CONCLUSIONS: Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.

The vertical expandable prosthetic titanium rib implant for the treatment of thoracic insufficiency syndrome associated with congenital and neuromuscular scoliosis in young children.

Expansion thoracoplasty and vertical expandable prosthetic titanium rib (VEPTR; Synthes Spine Co., West Chester, Pennsylvania, USA) implantation is a new method for the treatment of thoracic insufficiency syndrome and congenital spinal deformity in children. The longitudinal rib implant expands the thorax and indirectly corrects spinal deformity, thus allowing spinal, thoracic and probably lung growth. VEPTR has been used since 1989 in San Antonio, USA, and was introduced to Europe in 2002. This paper describes the preliminary experience with the European patients. Fifteen children with progressive scoliosis had a VEPTR implantation at a mean age of 6 years (11 months to 12 years). Nine children had thoracic insufficiency syndrome due to unilateral unsegmented bars (n = 4), absent ribs (n = 1), hemivertebrae (n = 2) or bilateral fused ribs (n = 2). Six children had severe thoracolumbar scoliosis and pelvic obliquity due to neuromuscular scoliosis. After VEPTR implantation, families and patients reported improvement of the thoracic insufficiency syndrome and better sitting abilities in the neuromuscular patients, as well as radical cosmetic improvement. There were three complications (skin breakage, lumbar hook displacement, rib fracture) after performing fifteen primary VEPTR implantations and 13 expansion surgeries in eight patients. Our experience suggests that expansion thoracoplasty and VEPTR implantation is a safe and efficient method for the treatment of thoracic insufficiency syndrome in young children with severe scoliosis.

Pathogenesis and biomechanics of adolescent idiopathic scoliosis (AIS).

Adolescent idiopathic scoliosis is defined as a scoliosis that starts after the age of ten and has no clear underlying disease as a reason for its development. There is, however, a disparity between the growth of the vertebral bodies anteriorly and that of the posterior elements. The vertebral bodies grow faster than the posterior elements, resulting primarily in a lordosis. The diminished dorsal growth impedes the ventrally located vertebral bodies from increasing in height, forcing them to become distorted, i.e., rotate, in order to create space for themselves. This produces a rotational lordosis. The idea of looking at it in this way dates back to Somerville in 1952. Many recent studies have confirmed this idea and have shown that the spinal canal is shorter than the anterior ligament of the vertebral bodies. In a mathematical model of the spine it was demonstrated that-although the vertebral column in humans is still predominantly loaded in an axial direction-certain segments of the human spine (especially the backward inclined segments) are subject to dorsally directed shear loads as well. In addition to the antero-posterior difference in growth, there is also a deformation of the vertebral bodies itself in 3-D. This is probably secondary and not primary effects, but this question is still under discussion. For the treatment of scoliosis, the biomechanical principles of axial and transverse forces are used. The combination of axial and transverse loads is most beneficial for all curves. The axial forces provide most of the corrective bending moment when deformity is severe, while the transverse loads take over the correcting function when deformity is mild. The deformity angle of 53° is the break-even point for the axial and transverse loads. In more severe curves transverse forces become less and less efficient, while axial forces rapidly gain more and more effect.

Virtual reconstruction of pelvic tumor defects based on a gender-specific statistical shape model.

Tumors in the pelvic region cause deformation and destruction of bony structures. Because the original pelvic anatomy cannot be adequately assessed at the tumor site, reconstruction with patient-specific implants is required. A widely used strategy for the reconstructive planning is mirroring of the contralateral side. We analyzed the statistical shape model (SSM)-based reconstruction method and compared it with the mirroring approach. Our approach used a gender-specific pelvic SSM (n = 50 for each gender) to generate implant geometries. The main objectives of this study were to analyze and evaluate the virtual anatomical reconstruction of eight tumor-damaged pelvic bones using the SSM approach. We achieved an overall mean deviation distance of 0.89 mm and 1.26 mm for the reconstruction of the equivalent defect in the healthy hemipelvis. Quantitative comparison with the mirroring method showed that the SSM-based reconstruction method reconstructs the defect with the same clinically acceptable accuracy as the mirroring method. The study demonstrates that the presented model can be a valuable tool for the planning of pelvic reconstructive surgery and implant design.

Growth guidance of angular lower limb deformities using a one-third two-hole tubular plate.

PURPOSE: The eight-plate system for angular deformity correction is well known, reliable and effective at any age during growth. Due to high implant costs, we sought to evaluate the effectiveness and safety of a less expensive alternative. METHODS: Between 2006 and 2011, 41 children with angular deformities were managed using a two-hole one-third tubular plate in cases where an eight plate would normally be indicated. Inclusion criteria in this retrospective study were: genu valgum and genu varum. X-ray documentation was performed before and after surgery and patients were followed clinically every 3 months after surgery. The cost per implant was 361.40 Sfr (Swiss Francs) compared to the eight plate at 737 Sfr. RESULTS: Mean time for correction was 13 months. A mean LDFA/MPTA after correction of 89.9°/86.8° was recorded, as well as a mean correction angle of 6.8°/6.6°. The complication rate was 6.6 % (one superficial wound infection and one insufficient correction in an older child). These results compare favourably with published data on the eight plate. CONCLUSION: The two hole one-third tubular plate seems to be a clinically and also cost effective alternative to the eight plate. Full deformity correction is gained for a fraction of the cost. LEVEL OF EVIDENCE: Level III.

Supracondylar stress fracture of the femur in a child.

We describe the unique case of a supracondylar-supraphyseal femoral stress fracture in a healthy 13-year-old boy. The importance of careful history taking is emphasized particularly in regard to asking about new, repetitive activities. The important differential diagnosis in this anatomical region is neoplastic disease and the use of imaging, particularly MRI scanning will yield useful information but is not definitive. Regular follow-up and repeat imaging must be undertaken where the diagnosis of stress fracture is made so that the diagnosis can be ultimately confirmed with the appearance of a healing fracture. Active management in lower limb stress fractures with immobilization is also emphasized as it is apparent that such fractures often do not follow a benign course. Immobilization will also help ensure compliance to treatment and follow-up, which can sometimes be difficult in this patient population.

Synovial sarcoma in patients under 20 years of age: a multicenter study with a minimum follow-up of 10 years.

PURPOSE: Synovial sarcoma (SS) is an aggressive soft-tissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS. METHODS: We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients (n = 8) was 20.1 years (12.1-27.6) and for nonsurvivors (n = 5) 4.9 years (range: 2.6-9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value. RESULTS: Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5- and 10-year survival rates were 77 and 61%, respectively. The mean time until a local recurrence (n = 5) was 3.2 years (range: 0.7-10.2), while there was a mean time of 2.1 years until the occurrence of late metastases (n = 5; range: 0.8-4.8). A high tumor grade and having a tumor in the trunk were adverse factors in terms of overall, local recurrence-free, and metastasis-free survival. Patients with wide resections or amputations had fewer local recurrences than patients with marginal or intralesional resections. CONCLUSION: Inadequate primary excision of SS results in incomplete excision in the majority of cases. The tumor site, size, and histological grade should be considered when determining a risk-adapted treatment for SS, and wide surgical excision is the surgical intervention of choice. While local recurrence and late metastases appear to occur after a shorter time period in pediatric patients than in adults, in view of the tendency for late recurrence and metastasis with SS, follow-up should be at least 10 years.

The management of Legg-Calvé-Perthes' disease: is there a consensus? : A study of clinical practice preferred by the members of the European Paediatric Orthopaedic Society.

PURPOSE: The aim of the study was to find out whether or not there is consensus among experienced pediatric orthopaedists about the management of certain clinical scenarios in Legg-Calvé-Perthes' disease. METHODS: A questionnaire was sent to all 297 members of the European Paediatric Orthopaedic Society (EPOS) describing four cases of Legg-Calvé-Perthes' disease (LCPD) with two X-rays each and a short description of the clinical scenario. Two of the patients were younger and two were older than six years of age. From both age groups there was one with a good range of motion and an X-ray classified as Herring A or B, while the other patient had a poor range of motion and an X-ray classified as Herring C. EPO members were asked to choose from various treatment options or to describe any other therapy that they would advise in the clinical scenarios. RESULTS: One-hundred and fifty members answered the questionnaire. The participants had an average of 20 years of experience in pediatric orthopaedics. There was a consensus that no surgery should be performed in a young patient with a good range of motion and that there should be no weight relief when older with a good range of motion. Conservative containment treatment (abduction splint, Petrie cast) and arthrodiastasis was suggested in only very few centres. There was a tendency to perform an operation when the patient is older with a poor range of motion and to perform operative treatment only when there were subluxation or head at risk signs. pelvic osteotomies or a combination of pelvic and femoral osteotomies rather than femoral osteotomies alone. Age did not determine the indication for treatment and there was no agreement on the indications for physiotherapy. There was also no consensus on the type of pelvic osteotomy to be used. CONCLUSIONS: The study showed that indications for the treatment of LCPD is based more on the personal experience of the surgeon rather than on scientific data.