Assessment of Hindfoot Alignment: Intraoperative Fluoroscopy Versus Standing Radiograph.

Few intraoperative assessments are available for hindfoot alignment. In the current study, we demonstrated the feasibility of hindfoot alignment via intraoperative fluoroscopy. We retrospectively compared measurements of heel alignment obtained via intraoperative fluoroscopy with those acquired using standard radiographs. Two observers compared the heel alignment ratios and angles derived from 100 pairs of images. The effects of age, sex, laterality, and body mass index on the discrepancy between fluoroscopic images and radiographs were analyzed. The heel alignment ratio revealed a strong correlation between standing radiograph and intraoperative fluoroscopy, based on a correlation coefficient of 0.844 (p < .001). The heel alignment angle also showed significant correlation based on a correlation coefficient value of 0.667 (p < .001). None of the demographic factors showed any significant effect on the discrepancy between the 2 sets of images. Our study showed that the heel alignment determined via intraoperative fluoroscopy was comparable to that of a standard standing radiograph without any significant association with demographic factors.

Comparative Analysis of Proteomes and Phosphoproteomes in Patients with Prostate Cancer Using Different Surgical Conditions.

PURPOSE: To establish the standard of procedure in preparing benign and cancerous prostate tissues and evaluate the quality of proteomics and phosphoproteomics during transurethral resection of the prostate (TUR-P) with different surgical conditions. MATERIALS AND METHODS: TUR-P tissue samples from three patients, two diagnosed with prostate cancer and one with benign prostatic hyperplasia, were each analyzed under three different conditions, based on differences in energy values, tissue locations, and surgical techniques. Global- and phosphorylated proteomic profiles of prostate tissues were analyzed by liquid chromatography-tandem mass spectrometry. RESULTS: A total of 6,019 global proteins and 4,280 phosphorylated peptides were identified in the nine tissues. The quantitative distributions of proteins and phosphorylation in tissues from the same patient were not affected by changes in the surgical conditions, but indirect relative comparisons differed among patients. Phosphorylation levels, especially of proteins involved in the androgen receptor pathway, important in prostate cancer, were preserved in each patient. CONCLUSIONS: Proteomic profiles of prostate tissue collected by TUR-P were not significantly affected by energy levels, tissue location, or surgical technique. In addition, since protein denaturation of samples through TUR-P is rarely confirmed in this study, we think that it will be an important guide for tissue samples in castration resistant prostate cancer patients, where it is difficult to obtain tissue. This result is the first report about proteomic and phosphoproteomic results with TUR-P samples in prostate cancer and will be theoretical basis in protein analysis research with prostate cancer tissues.

Reoperation rate, mortality and ambulatory ability after internal fixation versus hemiarthroplasty for unstable intertrochanteric fractures in elderly patients: a study on Korean Hip Fracture Registry.

INTRODUCTION: The treatment of unstable intertrochanteric fracture in elderly patients is challenging and how to treat these patients remains controversial. The purposes of this study were to compare (1) reoperation rate, (2) mortality and (3) the postoperative change of walking ability between patients undergoing internal fixation (IF) and those undergoing bipolar hemiarthroplasty (HA) due to this type of fracture based on the data from the Korean Hip Fracture Registry. MATERIALS AND METHODS: Between July 2014 and June 2016, we extracted 623 unstable intertrochanteric fractures (616 patients aged ≥ 65 years) according to the classification of the Association for the Study of Internal Fixation-American Orthopaedic Trauma Association. Among the 564 patients, 396 were treated with IF (IF group) and 168 with bipolar HA (HA group). We compared the reoperation rate and mortality between IF group and HA group. In patients, who were followed up more than 2 years after the surgery, we compared the postoperative change of walking activity from ambulatory outdoors (Koval's grade 1, 2, 3) to housebound (Koval's grade 4, 5, 6). RESULTS: The rate of reoperation was higher in the IF group (24/396, 6.1%) than in the HA (4/168, 2.4%) (p = 0.046). At the final follow-up, 79 (35.7%) of the 221 IF patients became housebound, whereas 21 (23.3%) of the 90 HA patients became housebound (p = 0.022). CONCLUSION: This study showed HA was associated with lower rate of reoperation and lower decrement rate of walking ability compared to IF in elderly patients with unstable intertrochanteric fractures.

Is subtle cavus foot a risk factor for chronic ankle instability? Comparison of prevalence of subtle cavus foot between chronic ankle instability and control group on the standing lateral radiograph.

BACKGROUND: Subtle cavus foot (SCF) is an entity characterized by mild cavus. However, few studies have examined whether a SCF may be a risk factor for chronic ankle instability (CAI). METHODS: This study included 116 patients who underwent lateral ankle ligament repair (modified Broström operation) for CAI and 105 controls. We used the standing lateral radiograph, so compared calcaneal pitch angle, Meary's angle, heights of the first and fifth metatarsal bases, and fibular positions between groups. Additionally, two observers subjectively rated the standing lateral radiographs for the presence of SCF. RESULTS: There were no significant intergroup differences in any of the radiographic angles. The prevalence of SCF was 20.7% in the CAI group and 18.1% in the control group according to observer 1 versus 21.6% and 28.6% (CAI group and control group, respectively) according to observer 2. There were no significant intergroup differences in the proportion of SCF between the two observers (p=0.105 and 0.211, respectively). CONCLUSION: SCF was not a significant risk factor for CAI when judging by standing lateral radiograph, and the detection of SCF seems to require considerable experience. Thus, care should be taken when determining whether to perform corrective osteotomies when treating CAI patients with SCF. LEVEL OF EVIDENCE: III, case control.

Radiologic Outcomes of Intramedullary Nailing in Infraisthmal Femur-Shaft Fracture with or without Poller Screws.

BACKGROUND: Intramedullary nails have been the treatment of choice for acute femur-shaft fractures in adults. However, the infraisthmal location has a high risk of nonunion and is easy to malalign. This study evaluated radiologic outcomes of initial supportive use of poller screws in combination with antegrade femoral nailing in infraisthmal femur-shaft fracture. METHODS: A total of 49 patients who had undergone antegrade nailing with or without supportive poller-screw insertion for infraisthmal femur-shaft fracture were included in this retrospective cohort study (23 patients with poller screws in group 1 versus 26 patients without poller screws in group 2). Patient demographics including sex, age, classification, mechanism of injury, operative time, poller-screw time, and radiologic outcomes, including union rate, time to union, and number of malunions, were evaluated. RESULTS: Union rate in group 1 (95.6%) was significantly higher than that in group 2 (84.6%) (p = 0.04). Time to union was 19.8 weeks in group 1 and 20.3 weeks in group 2 (p = 0.31). CONCLUSION: Initial supportive insertion of two poller screws after nailing took a mean of 21minutes additionally but could lessen the risk of nonunion significantly in this study. We believe that these findings may have important clinical relevance for the treatment of infraisthmal femur-shaft fracture.

Primary Glomerulonephritis with Unique C4d Deposition and Concurrent Non-infectious Intermediate Uveitis: a Case Report and Literature Review.

C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy. Consequently, C4 GN was suggested as the pathologic diagnosis. Although laser microdissection and mass spectrometry for glomerular deposit and pathologic evaluation of the retinal tissue were not performed, this is the first report of C4 GN in Korea and the first case of coexisting C4 GN and uveitis in the English literature.

Prevalence and clinical significance of hypertrophic labrum in non-dysplastic hips.

BACKGROUND: Hypertrophic labrum in dysplastic hip has been frequently reported and is known as limbus in developmental dysplasia of the hip. However, hypertrophic labrum without hip dysplasia has not been reported to date. The purpose of this study was to define hypertrophic labrum and to investigate the prevalence and clinical significance of an abnormally hypertrophic labrum in non-dysplastic hips. METHODS: Between 2007 and 2014, direct CT arthrography was performed in 464 patients (470 hips) who had chronic groin pain and positive impingement or Faber test. There were 189 male (192 hips) and 275 female (278 hips) patients. The mean age was 46.8 years (range, 16-74 years). The hypertrophic labrum was defined as a labrum wider than two standard deviations away from the mean. Surgical correlation of hypertrophic labrum was obtained in cases with hip arthroscopy by a single surgeon. RESULTS: Seventeen hips (3.6%) were found to have a hypertrophic labrum which had a mean width of 11.2 mm (±1.1) (range, 9.8-14.1 mm). The average width (±SD) of the labrum without hypertrophy was 5.5 mm (±2.4) (range, 2-9.8 mm). Arthroscopic findings of the hypertrophic labrum included fissure and longitudinal tear at the chondrolabral junction and they were associated with chondral abnormality adjacent to the lesion. CONCLUSIONS: Hypertrophic labrum without hip dysplasia is not rare with a high incidence of tears. Care should be taken during arthroscopic access to the hip joint in patients with this morphologic variation as iatrogenic damage can occur more easily. LEVEL OF EVIDENCE: Case Series; Level IV.

Combination chemotherapy with methotrexate and vinblastine for surgically unresectable, aggressive fibromatosis.

OBJECTIVE: To elucidate the clinical benefit and safety of low-dose chemotherapy using methotrexate and vinblastine in patients (mostly adults) with progressive and/or symptomatic fibromatosis. METHODS: Patients were enrolled if they were treated with methotrexate and vinblastine chemotherapy for recurrences after surgical excision or newly diagnosed aggressive fibromatosis that was not amenable to surgical resection at the Korea University Medical Center from May 2008 to February 2016. RESULTS: Twenty-two patients were treated with this regimen, and 21 were eligible for safety and efficacy analysis. Eleven (52%) of 21 patients showed a documented partial response (PR), and 11 showed stable disease (SD) by the end of treatment. All the patients who achieved PR reported a significant reduction in pain and improvement in the function of the affected lesions. Median progression-free survival was not reached at the time of analysis. The most common adverse event was abnormalities of the liver transaminases (overall 84.2%). The most common grade 3 or higher toxicity was neutropenia (36.8%), but no febrile neutropenic event was observed. The elevated levels of transaminases were normalized by reducing the dose of methotrexate or delaying treatment. CONCLUSIONS: Low-dose chemotherapy with methotrexate and vinblastine for 1 year was effective and well tolerated by adult patients with aggressive, recurrent fibromatosis.

Magnetic Resonance Imaging Findings in Small Patella Syndrome.

Small patella syndrome (SPS) is characterized by aplasia or hypoplasia of the patella and pelvic girdle abnormalities, including bilateral absence or delayed ossification of the ischiopubic junction and infra-acetabular axe-cut notches. Here, we report a case of SPS in a 26-year-old female. Magnetic resonance image (MRI) showed a small patella with thick eccentric non-ossified patellar cartilage and femoral trochlear dysplasia with hypoplastic patellar undersurface. To our knowledge, this is the first report of MRI findings in SPS. MRI findings could be clinically relevant because elongation of the medial patellofemoral ligament and trochlear dysplasia with eccentric non-ossified patellar cartilage might lead to patellofemoral maltracking with an osteochondral lesion or acute dislocation or an extensor mechanism injury. Though the patient presented in this case report only had a gastrocnemius injury at the origin site, physicians should carefully examine abnormalities with MRI when an SPS patient has a trauma to the knee.

Two Distinct Isoforms of Matrix Metalloproteinase-2 Are Associated with Human Delayed Kidney Graft Function.

Delayed graft function (DGF) is a frequent complication of renal transplantation, particularly in the setting of transplantation of kidneys derived from deceased donors and expanded-criteria donors. DGF results from tubular epithelial cell injury and has immediate and long term consequences. These include requirement for post-transplantation dialysis, increased incidence of acute rejection, and poorer long-term outcomes. DGF represents one of the clearest clinical examples of renal acute ischemia/reperfusion injury. Experimental studies have demonstrated that ischemia/reperfusion injury induces the synthesis of the full length secreted isoform of matrix metalloproteinase-2 (FL-MMP-2), as well as an intracellular N-terminal truncated MMP-2 isoform (NTT-MMP-2) that initiates an innate immune response. We hypothesized that the two MMP-2 isoforms mediate tubular epithelial cell injury in DGF. Archival renal biopsy sections from 10 protocol biopsy controls and 41 cases with a clinical diagnosis of DGF were analyzed for the extent of tubular injury, expression of the FL-MMP-2 and NTT-MMP-2 isoforms by immunohistochemistry (IHC), in situ hybridization, and qPCR to determine isoform abundance. Differences in transcript abundance were related to tubular injury score. Markers of MMP-2-mediated injury included TUNEL staining and assessment of peritubular capillary density. There was a clear relationship between tubular epithelial cell expression of both FL-MMP-2 and NTT-MMP-2 IHC with the extent of tubular injury. The MMP-2 isoforms were detected in the same tubular segments and were present at sites of tubular injury. qPCR demonstrated highly significant increases in both the FL-MMP-2 and NTT-MMP-2 transcripts. Statistical analysis revealed highly significant associations between FL-MMP-2 and NTT-MMP-2 transcript abundance and the extent of tubular injury, with NTT-MMP-2 having the strongest association. We conclude that two distinct MMP-2 isoforms are associated with tubular injury in DGF and offer novel therapeutic targets for the prevention of this disorder.

Is DBM beneficial for the enhancement of bony consolidation in distraction osteogenesis? A randomized controlled trial.

The aim of the present study was to compare the radiographic and clinical outcomes of DBM injection and conventional treatment during tibial lengthening over an intramedullary nail in adult patients with short stature. Twenty-nine patients were randomized to receive DBM injection (n = 14) or conventional treatment without any injection (n = 15) and evaluated. The outcome was measured on the basis of the pixel value ratio (PVR) in the digital radiographs during the consolidation period; healing index; clinical assessment; and the rate of complications. In the DBM group, the mean PVR of 1 (mineral density of the callus is comparable to the adjacent bone) was reached by 40 weeks in anterior and medial cortices which was significantly different than that in the control group (P = 0.03 for anterior cortex; P = 0.04 for medial cortex). The average healing index in the DBM group was 39.8 ± 5.3 days/cm compared to 44.3 ± 5.8 days/cm in the control group (P = 0.05). There were no significant differences in clinical outcomes (P = 0.23) and functional status (P = 0.47) including complications (P = 0.72) between two groups. In this randomized clinical trial, injection of DBM at the time of initial operation enhanced consolidation of regenerate callus without interfering with clinical outcomes compared to that with conventional treatment.

High mortality and poor morbidity after hip fracture in patients with previous vertebral fractures.

Although vertebral fracture in patients is a predictor of subsequent hip fracture, no study has assessed the mortality and functional outcome in hip fracture patients with previous vertebral fracture. Between September 2009 and December 2012, we evaluated 246 patients over 50-years-of-age diagnosed with femoral neck or intertrochanteric fractures who underwent surgery. The patients were categorized into two groups and two subgroups. Group Ia comprised 150 patients with previous vertebral fracture at the time of hip fracture. Group Ib comprised 96 patients with no vertebral fracture. Group IIa consisted of 76 patients <80-years-of-age with previous vertebral fracture. Group IIb comprised 69 patients <80-years-of-age without previous vertebral fracture. The mortality rate and functional outcome of osteoporotic hip fracture patients with and without vertebral fractures were compared. The cumulative mortality rate at 6 and 12 months post-fracture was 19 and 23 % in Group Ia and 6 and 7 % in Group Ib, respectively. In subgroup analysis, the cumulative mortality rate at 6 and 12 months was 13 and 17 % in Group IIa and 3 and 4 % in Group IIb, respectively. Shut-in patients at the final follow-up included 51 of 103 (49.5 %) patients in Group Ia and 19 of 83 (22.9 %) patients in Group Ib. In subgroup analysis, the shut-in patients included 18 of 58 (31.0 %) patients in Group IIa and 10 of 62 (16.1 %) patients in Group IIb. Previous vertebral fracture was associated with a poor functional outcome and increased mortality in patients with hip fracture.

The significance of serum phosphate level on healing index and its relative effects in skeletally immature and mature patients with hypophosphatemic rickets.

The aim of this study was to find out the ideal cut-off level of phosphate for safe healing when deformity correction and concomitant lengthening are indicated in the two different skeletal maturity groups of patients with rickets. Thirty-nine hypophosphatemic rickets patients were selected for the study and were divided into two groups: 27 skeletally immature (group IM) and 12 skeletally mature (group M). The outcomes were evaluated with respect to the healing index (HI), laboratory findings, and complications with the mean follow-up of 5.1 years (range, 3.1-7.9). The healing index (HI) of group IM was 1.44 month/cm and HI of group M was 1.68 month/cm. The negative correlation between the level of serum phosphate and HI in group M (coefficient=-0.94) was evaluated to be less than the correlation in group IM (coefficient=-0.50), indicating that the HI is more likely to be affected by serum phosphate in group M than in group IM. Preoperative serum phosphate levels of 2.3 mg/dL and 2.6 mg/dL were analyzed to be the cut-off values of group IM and group M, respectively, in which the cut-off points divided the series into two groups having the most significantly different HI.

Bone formation in a rat tibial defect model using carboxymethyl cellulose/BioC/bone morphogenic protein-2 hybrid materials.

The objective of this study was to assess whether carboxymethyl cellulose- (CMC-) based hydrogel containing BioC (biphasic calcium phosphate (BCP); tricalcium phosphate (TCP) : hydroxyapatite (Hap) = 70 : 30) and bone morphogenic protein-2 (BMP-2) led to greater bone formation than CMC-based hydrogel containing BioC without BMP-2. In order to demonstrate bone formation at 4 and 8 weeks, plain radiographs, microcomputed tomography (micro-CT) evaluation, and histological studies were performed after implantation of all hybrid materials on an 8 mm defect of the right tibia in rats. The plain radiographs and micro-CT analyses revealed that CMC/BioC/BMP-2 (0.5 mg) led to much greater mineralization at 4 and 8 weeks than did CMC/BioC or CMC/Bio/BMP-2 (0.1 mg). Likewise, bone formation and bone remodeling studies revealed that CMC/BioC/BMP-2 (0.5 mg) led to a significantly greater amount of bone formation and bone remodeling at 4 and 8 weeks than did CMC/BioC or CMC/BioC/BMP-2 (0.1 mg). Histological studies revealed that mineralized bone tissue was present around the whole circumference of the defect site with CMC/BioC/BMP-2 (0.5 mg) but not with CMC/BioC or CMC/BioC/BMP-2 (0.1 mg) at 4 and 8 weeks. These results suggest that CMC/BioC/BMP-2 hybrid materials induced greater bone formation than CMC/BioC hybrid materials. Thus, CMC/BioC/BMP-2 hybrid materials may be used as an injectable substrate to regenerate bone defects.

Fibrocartilaginous intramedullary bone forming tumor of the distal femur mimicking osteosarcoma.

Fibrocartilaginous dysplasia (FCD) has occasionally led to a misdiagnosis and wrong decision which can significantly alter the outcome of the patients. A 9-yr-old boy presented with pain on his left distal thigh for 6 months without any trauma history. Initial radiographs showed moth eaten both osteolytic and osteosclerotic lesions and biopsy findings showed that the lesion revealed many irregular shaped and sclerotic mature and immature bony trabeculae. Initial diagnostic suggestions were varied from the conventional osteosarcoma to low grade central osteosarcoma or benign intramedullary bone forming lesion, but close observation was done. This study demonstrated a case of unusual fibrocartilaginous intramedullary bone forming tumor mimicking osteosarcoma, so that possible misdiagnosis might be made and unnecessary extensive surgical treatment could be performed. In conclusion, the role of orthopaedic oncologist as a decision maker is very important when the diagnosis is uncertain.

Role of hybrid monolateral fixators in managing humeral length and deformity correction.

BACKGROUND AND PURPOSE: Humeral lengthening and deformity correction are now being done increasingly for various etiologies. Monolateral external fixators have advantages over traditional Ilizarov circular fixators; they are easy to apply, they are less bulky, and they are therefore more convenient for the patient. We assessed the effectiveness of hybrid monolateral lateral fixators in humeral lengthening and deformity correction. METHODS: We retrospectively reviewed 23 patients (40 humeri) with various pathologies who underwent lengthening-with or without deformity correction using monolateral external fixator-between 2003 and 2008. Mean age at the time of the surgery was 14 (10-22) years. The mean follow-up time was 3.4 (1-7) years. RESULTS: The average duration of external fixator use was 8.3 (6-19) months. The mean lengthening achieved was 8.8 (4-11) cm and percentage lengthening was 49% (19-73). The healing index was 28 (13-60) days/cm. The major complications were refracture in 3 humeri and varus angulation of 2 humeri. The minor complications were superficial pin tract infection (6 segments), transient radial nerve palsy (1 segment), and elbow flexion contracture (5 segments). All complications resolved. INTERPRETATION: Hybrid monolateral fixators can be used for humeral lengthening and deformity correction. The advantage over circular fixators is that they are less bulky and patients can perform their day-to-day activities with the fixator in situ.

Subtrochanteric valgus osteotomy with monolateral external fixator in hips for patients with severe cerebral palsy.

Subtrochanteric valgus osteotomy has been used for painful hip joint dislocation in patients with severe cerebral palsy. The goal of this study was to evaluate 11 patients (17 hips) with severe cerebral palsy who had chronically dislocated and painful hips treated with subtrochanteric valgus osteotomy using a monolateral external fixator. A retrospective review was performed of 11 patients (average age, 17.8 years) with severe quadriplegic cerebral palsy with flexion-adduction contractures due to chronically dislocated and painful hips. A subtrochanteric valgus osteotomy with a monolateral fixator was performed in all patients. Patients were analyzed clinicoradiologically, and caregivers were asked about ease of handling, transfers, and perineal care. At an average follow-up of 37 months (range, 14-72 months), all caregivers were satisfied with the surgery and felt that their child was more comfortable and could sit with support for a longer time period and that perineal care, wheelchair mobilization, and transfers were much easier. A total of 11 complications in 7 patients were observed, including pin-tract infections, delayed consolidation, abduction deformity, and hypostatic pneumonia. The complication rate of subtrochanteric valgus osteotomy was comparable with other methods, and this method had the advantage of shorter surgical time, ease of application, no internal implant with lesser chance of infection or heterotopic calcification, and less intraoperative blood loss with less morbidity.

Bone tunnel widening with autogenous bone plugs versus bioabsorbable interference screws for secondary fixation in ACL reconstruction.

BACKGROUND: Use of an autogenous bone plug for the tibial tunnel in anterior cruciate ligament (ACL) reconstruction has been advocated to achieve tendon-to-bone healing. Our hypothesis was that use of an autogenous bone plug, instead of a bioabsorbable interference screw, for secondary fixation of tendon allograft to the proximal part of the tibia would reduce the complication rate and tibial tunnel widening. METHODS: We prospectively reviewed the cases of eighty-one patients (average age, 32.0 years) who had undergone ACL reconstruction with Achilles tendon allograft between 2000 and 2006. A bioabsorbable interference screw was used for the tibial tunnel in forty-one patients (group I). These patients were compared with forty patients in whom autogenous bone from the tibia had been used (group II). The two groups were assessed with use of the International Knee Documentation Committee (IKDC), Lysholm, and Tegner activity scores and with KT2000 arthrometer testing. The cross-sectional area perpendicular to the long axis of the tibial tunnel was calculated digitally with use of magnetic resonance imaging (MRI). RESULTS: No significant differences were seen between the two groups with respect to IKDC, Lysholm, or Tegner activity scores or the results of laxity testing with arthrometry. A total of fourteen complications (34%) occurred in group I. In contrast, six complications (15%) were seen in group II (p = 0.046). The mean cross-sectional area enlargement was 38% in group I and 15% in group II (p = 0.017). CONCLUSIONS: The clinical results associated with bioabsorbable screws and bone plugs were not significantly different. Laxity evaluation demonstrated no significant differences between bioabsorbable screws and bone plugs. Compared with bioabsorbable interference screws, autogenous bone plugs reduced the complication rate and tibial tunnel widening without inducing instability. We believe that an autogenous bone plug for the tibial tunnel is a reasonable option in selected patients.

Congenital pseudarthrosis of the tibia treated with a combination of Ilizarov's technique and intramedullary rodding.

BACKGROUND AND PURPOSE: Ilizarov's technique and intramedullary rodding have often been used individually in congenital pseudarthrosis of the tibia. In this series, we attempted to combine the advantages of both methods while minimizing the complications. METHODS: We reviewed 15 cases of congenital pseudoarthrosis of the tibia (CPT) who were treated with a combination of Ilizarov's apparatus and antegrade intramedullary nailing between 2003 and 2008. The mean age at surgery was 7.5 (3-12) years and the mean limb length discrepancy was 2.5 (1.5-5) cm. At a mean follow-up time of 4.5 (1.6-7.2) years after the index surgery, the patients were evaluated clinically and radiographically for ankle function (AOFAS score) and for malalignment, signs of union, limb length discrepancy, and complications. RESULTS: 14 patients achieved union, in 6 patients primary union and in 8 patients after secondary procedures. The AOFAS score improved from a preoperative mean of 40 (20-57) to 64 (47-75). The main complication was refracture in 1 patient, and non-union in 1 patient. INTERPRETATION: The combination of the Ilizarov technique and conventional antegrade intramedullary nailing was successful in achieving union with few complications, though this should be shown in long-term studies lasting until skeletal maturity.

New proposed clinico-radiologic and molecular criteria in hypochondroplasia: FGFR 3 gene mutations are not the only cause of hypochondroplasia.

We applied a comprehensive set of clinical and radiological criteria for the diagnosis of hypochondroplasia (HCH) in 160 patients with short stature 58 of whom were diagnosed to have HCH. Taking into account the genotypic and phenotypic variations in HCH, we conducted a study with these 58 patients and tested them for mutations in the fibroblast growth factor receptor 3 (FGFR3) and the short stature homeobox (SHOX) gene. We characterized the phenotypes by clinical and radiologic findings. In the patients with HCH, 19 were included in Group I (FGFR3 mutations-mutations of definite significance), and 39 were in Group II (6 SHOX mutations and 33 negative for disease-causing FGFR3 mutations). The clinical findings were similar in two groups regardless of the presence or absence of mutations. More than 95% of the patients had mesomelic proportions. In Group I, the radiological findings of mesomelia of upper and lower limbs and, L1/L4 ratio in anterior-posterior and lateral view were more typical than in Group II. This study proposes comprehensive clinico-radiological criteria for the diagnosis of HCH, which would help in detecting the true incidence of this underdiagnosed condition. The presence of SHOX mutations suggest genotypic-phenotypic overlap between HCH and Leri-Weill dyschondrosteosis, though further investigation is needed to effectively elucidate the importance of these mutations. Also, the 56.9% of HCH patients with negative mutations for FGFR3 suggests that there are other undiscovered gene mutations associated with this phenotypic entity.