One-Bone Forearm Procedure for Severe Recalcitrant Forearm Deformities in Masada IIb Hereditary Multiple Exostoses.

Introduction: Hereditary multiple exostoses (HME) Masada IIB has traditionally been treated by gradual ulnar lengthening with questionable efficacy in reducing the dislocated head. One-bone forearm (OBF) has been used as a reconstructive procedure in forearm deformities with very scarce literature for HME. The study aims to report short-term results of OBF as a definitive procedure for severe forearm deformities in Masada IIB patients with respect to clinical and radiological parameters. Materials and Methods: Four patients with HME Masada IIb were included in this retrospective study. All patients complained of forearm and wrist deformity with an abnormal bony protrusion restricting elbow motion. Indications for OBF were ulnar shortening > 3 cm, dysplastic proximal radius with convex radial head and restricted prono-supination. All patients were examined pre-operatively and post-operatively clinically and radiographically using the Peterson's outcome score. Results: The average age was 13 years (12-14 years). Pre-operative ulnar shortening, carpal slip percentage, and radial articular angle was 3.4 cm, 79.5%, and 47.5°, respectively. All radial heads were dislocated with convex articular surface restricting elbow extension and forearm prono-supination. At the latest follow-up, the mean elbow flexion was 110° with forearm in 10° supination. The mean carpal slip percentage, radial articular angle, and Peterson functional grade was 15%, 22.5°, and 8 points, respectively. The mean follow-up period was 30.25 months with no recurrence. Conclusion: We recommend one bone forearm as a definitive procedure in HME Masada IIB patients with severe forearm deformities with ulnar shortening > 3 cm and dysplastic proximal radius with a dislocated radial head, for faster return to function.

Supracondylar Humerus Fractures: Classification Based Treatment Algorithms.

Supracondylar humerus fractures are the most common fractures around the elbow in children between 4 and 10 years of age. The treatment of supracondylar humerus fractures can vary from conservative treatment to operative treatment depending on the fracture type. All around the world, the most commonly used classification system is the Wilkins-modified Gartland classification of supracondylar humerus fractures. Currently, the decision to operate or conserve the fracture is taken on basis of this classification system. Non-operative treatment for type I fractures and operative treatment for type III fractures have been well-established in literature. The management of type II supracondylar humerus fracture creates confusion in the minds of numerous orthopaedic surgeons around the world. We have tried addressing this using a classification-based treatment algorithm. Other classification systems like the AO classification, Lagrange and Rigault classification and Bahk classification with special reference to special fracture patterns that require attention and pre-op planning have also been mentioned. It is important to understand that operative management of each supracondylar humerus fracture is unique as regards fixation method and it is important to consider the fracture pattern before internal fixation.

Short Term Results of a New Comprehensive Protocol for the Management of Congenital Pseudarthrosis of the Tibia.

BACKGROUND: Treatment of Congenital Psuedarthrosis of Tibia (CPT) often poses significant challenges due to difficulty in achieving union and subsequent complications like refractures, implant failures, etc. Our new comprehensive protocol is aimed at achieving crossunion between the tibia and fibula. AIMS AND OBJECTIVES: The aim of the present study is to evaluate the short-term results of our new protocol and to compare the results with our previously used techniques. MATERIALS AND METHODS: 10 patients with mean age 2.35 years (1 to 6.5 years) who were treated by our new comprehensive protocol were included in Group A, and 11 patients with mean age 2 years (1 to 5.5 years) who primarily underwent intramedullary rodding with bone graft were included in Group B. The new comprehensive protocol consisted of pre-operative Zolendronate infusion, surgery consisting of intramedullary fixation of tibia supplemented with Ilizarov ring fixator and bone grafting aimed at achieving tibia-fibula cross-union. Retrospective evaluation of serial radiographs was performed and outcomes with respect to union and subsequent complications were analysed. RESULTS: 10/10 (100%) patients in Group A united, whereas union was achieved in only 8/11 (72%) patients in Group B. The index surgery was successful in achieving union in all 10 patients in Group A, whereas in Group B 2.25 (1 to 4) surgeries were needed to achieve union. The time to union was significantly shorter in Group A (4.68 months) as compared to Group B (30.88 months). The cross sectional area of union was significantly greater in Group A (3.82 cm2) as compared to Group B (1.18 cm2). One patient in Group A needed a subsequent corrective osteotomy for tibial valgus, and one patient underwent tibia lengthening; whereas in Group B, two patients needed corrective osteotomes for residual malaligments. CONCLUSION: Our study demonstrates that the new comprehensive protocol is extremely effective for achieving sound union in Congenital Pseudarthrosis of Tibia.

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.

Physeal growth arrest after tibial lengthening in achondroplasia: 23 children followed to skeletal maturity.

BACKGROUND AND PURPOSE: Bilateral tibial lengthening has become one of the standard treatments for upper segment-lower segment disproportion and to improve quality of life in achondroplasia. We determined the effect of tibial lengthening on the tibial physis and compared tibial growth that occurred at the physis with that in non-operated patients with achondroplasia. METHODS: We performed a retrospective analysis of serial radiographs until skeletal maturity in 23 achondroplasia patients who underwent bilateral tibial lengthening before skeletal maturity (lengthening group L) and 12 achondroplasia patients of similar height and age who did not undergo tibial lengthening (control group C). The mean amount of lengthening of tibia in group L was 9.2 cm (lengthening percentage: 60%) and the mean age at the time of lengthening was 8.2 years. The mean duration of follow-up was 9.8 years. RESULTS: Skeletal maturity (fusion of physis) occurred at 15.2 years in group L and at 16.0 years in group C. The actual length of tibia (without distraction) at skeletal maturity was 238 mm in group L and 277 mm in group C (p = 0.03). The mean growth rates showed a decrease in group L relative to group C from about 2 years after surgery. Physeal closure was most pronounced on the anterolateral proximal tibial physis, with relative preservation of the distal physis. INTERPRETATION: Our findings indicate that physeal growth rate can be disturbed after tibial lengthening in achondroplasia, and a close watch should be kept for such an occurrence-especially when lengthening of more than 50% is attempted.

Fibula-related complications during bilateral tibial lengthening: 60 patients followed for mean 5 years.

BACKGROUND AND PURPOSE: Complications related to the fibula during distraction osteogenesis could cause malalignment. Most published studies have analyzed only migration of the fibula during lengthening, with few studies examining the effects of fibular complications. PATIENTS AND METHODS: We retrospectively reviewed 120 segments (in 60 patients) between 2002 and 2009. All patients underwent bilateral tibial lengthening of more than 5 cm. The mean follow-up time was 4.9 (2.5-6.9) years. RESULTS: The average lengthening percentage was 34% (21-65). The ratio of mean fibular length to tibial length was 1.05 (0.91-1.11) preoperatively and 0.83 (0.65-0.95) postoperatively. The mean proximal fibular migration (PFM) was 15 (4-31) mm and mean distal fibular migration (DFM) was 9.7 (0-24) mm. Premature consolidation occurred in 10 segments, nonunion occurred in 12, and angulation of fibula occurred in 8 segments after lengthening. Valgus deformities of the knee occurred in 10 segments. INTERPRETATION: PFM induced valgus deformity of the knee, and premature consolidation of the fibula was associated with the distal migration of the proximal fibula. These mechanical malalignments could sometimes be serious enough to warrant surgical correction. Thus, during lengthening repeated radiographic examinations of the fibula are necessary to avoid complications.

Habitual anterior dislocation of the hip in cerebral palsy: a case report.

Habitual anterior dislocation of the hip joint is extremely rare. We believe this is the first case reported of a habitual anterior dislocation of the hip occurring in a child with cerebral palsy. The dislocation was painless, voluntary and used to occur every time the child would extend and externally rotate the limb. Operative intervention in the form of a varus derotation femoral osteotomy and anterior capsulorraphy was planned, but the caregivers of the patient were unwilling for the same. Hence, the patient was given an adduction and internal rotation brace and was counselled not to perform the activity needed to dislocate the hip. This case report calls attention to a unique problem previously not reported in cerebral palsy.

Is bilateral lower limb lengthening appropriate for achondroplasia?: midterm analysis of the complications and quality of life.

BACKGROUND: Use of the Ilizarov technique for limb lengthening in patients with achondroplasia is controversial, with a high risk of complications balancing cosmetic gains. Although several articles have described the complications of this procedure and satisfaction of patients after surgery, it remains unclear whether lengthening improves the quality of life (QOL) of these patients. QUESTIONS/PURPOSES: We asked whether bilateral lower limb lengthenings with deformity correction in patients with achondroplasia would improve QOL and investigated the correlation between complication rate and QOL. PATIENTS AND METHODS: We retrospectively reviewed 22 patients (average age, 12.7 years) diagnosed with achondroplasia who underwent bilateral lower limb lengthenings between 2002 and 2005. These patients were compared with 22 patients with achondroplasia for whom limb lengthening was not performed. The two groups were assessed using the American Academy of Orthopaedic Surgeons (AAOS) lower limb, SF-36, and Rosenberg self-esteem scores. Minimum followup was 4.5 years (range, 4.5-6.9 years). RESULTS: Among the lengthening group, the average gain in length was 10.21 ± 2.39 cm for the femur and 9.13 ± 2.12 cm for the tibia. A total of 123 complications occurred in these 88 segments. The surgical group had higher Rosenberg self-esteem scores than the nonsurgical group although there were no differences in the AAOS and the SF-36 scores. The self-esteem scores decreased with the increase in the number of complications. CONCLUSIONS: Our data suggest that despite frequent complications, bilateral lower limb lengthening increases patients' QOL. We believe lengthening is a reasonable option in selected patients. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.