Cirugía en coxa vara por osteogénesis imperfecta. Reporte de dos casos / Coxa vara surgery for osteogenesis imperfecta. A report of two cases

Introducción. La coxa vara es una disminución del ángulo cervicodiafisiario (<110°) y se puede presentar hasta en el 10% de los pacientes con osteogénesis imperfecta (OI), siendo más frecuente en el tipo III. Sus manifestaciones clínicas son alteración en la marcha, acortamiento de la pierna, genu valgo y dolor.Presentación de los casos. Varones de 13 años y 8 años con OI tipo III, signo positivo de Trendelenburg, movilidad articular adecuada y antecedentes de fracturas recurrentes y cirugías previas por deformidades en los que se realizó cirugía para la corrección de coxa vara. Se realizó una evaluación radiográfica y una funcional (escala de Harris) a los 6 y 68 meses de la cirugía, respectivamente.En el caso 1 se logró una corrección de 46° en el ángulo cervicodiafisiario (ángulo inicial: 84°; ángulo final: 130°) y el puntaje en la escala de Harris fue de 70 puntos. En el caso 2, se logró una corrección de 50° (82° vs. 132°), con un puntaje en la escala de Harris de 68 puntos. Conclusiones. la técnica de corrección mediante osteotomía subtrocantérica y la utilización de clavo endomedular y agujas de Kirschner es una opción efectiva para el tratamiento de coxa vara en pacientes con osteogénesis imperfecta

Introduction. Coxa vara is a deformity characterized by a decrease in the neck-shaft angle (<110°) that can occur in up to 10% of patients with osteogenesis imperfecta (OI), being more frequent in type III OI. Its clinical manifestations are gait disturbance, leg shortening, genu valgum, and pain.Case presentation. Male patients aged 13 and 8 years presenting with type III OI, positive Trende-lenburg sign, adequate joint mobility, and a history of recurrent fractures and previous surgeries for deformities, including surgery for coxa vara correction. Radiographic and functional evaluation (Harris scale) were performed 6 and 68 months after surgery, respectively.In the first case, a correction of 46° was obtained (initial angle: 84°; final angle: 130°), as well as a Harris score was 70. In the second case, a correction of 50° (82° vs. 132°) was achieved, with a Harris score of 68 points. Conclusions. Subtrochanteric osteotomy and intramedullary K-wire nailing are effective options for the treatment of coxa vara in patients with osteogenesis imperfecta

Application of interlocking intramedullary nailing in fibrous dysplasia of proximal femur / 中华骨科杂志

Objective:To retrospectively analyze and evaluate the effect of interlocking intramedullary nail in the treatment of proximalfemoral fibrous dysplasia (FD) and to discuss its application techniques and treatment strategies.Methods:The patients with proximal femoral FD who received treatment with interlocking intramedullary nail in our department from April 2014 to August 2018 were retrospectively reviewed. The inclusion criteria included having complete data with more than 3 years follow-up. A total of 31 cases with 32 thighs were included in the present study. Among them, there were 24 males and 7 females. The mean age at operation was 11.4±2.3 years. The mean follow-up duration was 3.9±1.1 years. The surgical methods were osteotomy or in situ interlocking intramedullary nailing, and femoral neck reconstruction nails pass through the epiphyseal plate in some cases. Preoperative, postoperative and final follow-up radiographic changes, including femoral neck shaft angle, proximal femoral diaphyseal deformity, and femoral alignment were compared. Univariate analysis of the influencing factors of postoperative femoral neck shaft angle loss.Results:In this group, 13 patients had coxa varus deformity, and the angle of the neck shaft was 107.3±7.1°, and 23 patients had the bending deformity of the proximal femur, and angled 29.5°±9.1°. 10 cases of coxa varus combined with proximal femoral diaphyseal deformity received double osteotomy and internal fixation, 17cases of simple coxa varus or diaphyseal deformity received single osteotomy and internal fixation, and 5 cases had no significant deformity, underwent in situ intramedullary nailing due to pathological fractures and pain. Postoperative femoral neck shaft angle was 133.2°±5.2°. Complete correction of proximal femoral flexion deformity was obtained and the diaphyseal axis was within the normal range. At the last follow-up, the neck shaft angle of 7 patients (21.9%, 7/32) decreased from 133.6°±3.9° to 125.7°±3.4°. Univariate analysis showed that the proportion of neck shaft angle loss in cases with proximal femoral locking nails passing through the epiphyseal plate was significantly lower than that in the cases without (0 vs. 43.8%, χ 2=6.58, P=0.010). Conclusion:The application of interlocking intramedullary nails in the treatment of proximal femoral fibrous dysplasia can achieve good results, according to the situation of coxa varus or diaphysis bending deformity, single or double osteotomy can be performed. There is a risk of femoral neck shaft angle loss after surgery, which can be avoided by femoral neck reconstruction locking screw fixation crossing epiphyseal plate.

Fixation of Intertrochanteric Valgus Osteotomy with T Plate in Treatment of Developmental Coxa Vara

BACKGROUND: Although the valgus subtrochanteric osteotomy is considered as a standard surgical treatment for coxa vara, there is no consensus on the optimal method of fixation and osteotomy technique. Fixation of the osteotomy has been achieved by various methods including external fixation and internal fixation with pins and cerclage and a variety of plates. The aim of this study is the evaluation of the results of developmental coxa treated by Y intertrochanteric valgus osteotomy fixed with a T-buttress plate compared with other methods of fixation in the literature. METHODS: Eighteen corrective valgus intertrochanteric femoral osteotomies were performed in 18 patients (18 hips) for treatment of unilateral developmental coxa vara deformity and fixed with a T plate. There were 12 males and 6 females. The right hip was affected in 10 patients and the left hip in 8 patients. Clinically, patients were evaluated by Larson hip score. Radiographically, anteroposterior view of the pelvis and frog leg lateral views of the affected hip were taken preoperatively and compared with the findings at the final follow-up. RESULTS: The average follow-up was 29 months (range, 24 to 36 months). Clinical results showed improvement of the mean Larson hip score from 57.8 to 97.0 (p < 0.001). Radiological results showed that all osteotomies were completely united in 2.4 months (range, 2 to 3 months) with the achievement of the planned correction angle. The average correction of Hilgenreiner's epiphyseal angle improved from 78.2° to 27.8° (p < 0.001) at the final follow-up. The femoral neck shaft angle was improved from 93.7° to 129.9° (p < 0.001) at the final follow-up. Shortening of the affected limb was corrected from 2.8 cm to 1.3 cm (p < 0.001) at the last follow-up. No major serious complications were recorded in the present study. CONCLUSIONS: Intertrochanteric valgus osteotomy of the proximal femur fixed with a T plate may be efficient for treatment of developmental coxa vara. With careful planning, it can result in a low complication rate and insignificant or minimal recurrence rate.

Epifisiolistesis capital femoral: consecuencias del diagnóstico tardío: reporte de un caso y revisión de la literatura / Slipped capital femoral epiphysis: consequences of late diagnosis: a case report and literature review

La epifisiolistesis capital femoral es la pérdida de la relación anatómica entre la epífisis y la metáfisis del extremo proximal del fémur, producida por un desplazamiento a través del cartílago de crecimiento. Es la patología de cadera más frecuente de la adolescencia y su origen es multifactorial. El diagnóstico es clínico y se confirma radiológicamente. Es una urgencia traumatológica y su tratamiento debe ser precoz y quirúrgico. Es una lesión que requiere un alto índice de sospecha por parte del médico examinador y no realizar su diagnóstico a tiempo puede tener consecuencias devastadoras. Presentamos el caso de un adolescente, femenino de 12 años, quien sufrió una epifisiolistesis capital femoral, con 6 meses de evolución, no diagnosticada en su fase aguda; mostramos su evolución desde el inicio y su resolución mediante osteotomía de Dunn. El diagnóstico oportuno es la primera y única forma de evitar complicaciones a mediano y largo plazo(AU)

The slipped capital femoral epiphysis syndrome is the loss of the anatomical relationship between the epiphysis and metaphysis of the proximal end of the femoral head caused by a movement across the growth cartilage. Hip is the most common pathology of adolescence and its origin is multifactorial. Diagnosis is clinical and confirmed radiologically. It's an emergency trauma and its treatment should be early and surgical. It is an injury that requires a high index of suspicion by the examining physician and diagnose it early can have devastating consequences. We report the case of a female teenager aged 12, who presented a capital femoral epiphyseal, 6 months of evolution, which was not diagnosed in its acute phase. Introducing its evolution from the beginning and its resolution by Dunn osteotomy. Early diagnosis is the first and only way to avoid complications in the medium and long term(AU)

Coxa Vara with Spondylometaphyseal Dysplasia / 대한정형외과학회잡지

Developmental coxa vara is a rare disease and the symptoms do not appear at birth, but rather, they appear at the age of walking. Clinically, the symptoms include a waddling gait, limb length discrepancy and frequent weariness. Developmental coxa vara is sometimes associated with skeletal dysplasia. Especially, it is associated with spondylometaphyseal dysplsia and the vertebral bodies and long bones are affected. The authors report here on diagnosing and treating this rare disease and we review the relevant literatures.

Analysis about reason of congenital infantile coxa vara being misdiagnosed as infantile dislocation of hip join / 中国矫形外科杂志

[Objective]To analyze reason of congenital infantile coxa vara to be misdiagnosed as infantile dislocation of hip joint.[Method]There were 6 patients(6 hip join)congenital infantile coxa vara being misdiagnosed as infantile dislocation of hip join in 54 patients(73 hip join)and treated by expectant treatment as infantile dislocation of hip join.The curative effect of 6 patients were not good,after serious medical examination,careful reading image data,paying attention to curative effect,6 patients were confirmed to be diagnosed as infantile dislocation of hip join and not infantile dislocation of hip join.[Result]The features for diagaosis of congenital infantile coxa vara were:the big greater trochanter of femur and its position moving up,short spacious neck of femur,spacious medial blank in hip joint,superposed neck of femur and lesser trochanter of femur,praeter propter exponent of acetabulum,acceptable succession of Shenton’s line,enlarged angle of femoral head epiphysis,late center of ossification,incorporated abnormity such as breves femoral bone,bowing of femoral bone,abortive clavicle and bones of cranium,equines.[Conclusion]The second center of ossification of femoral head is appeared 6～9 monthes after birth,and it is difficult to discriminate congenital infantile coxa vara and infantile dislocation of hip join before this.Early to realize symptom,physical sign and character of image of congenital infantile coxa vara is significant for correct diagnosis and can avoid misdiagnosis and mistreatment.

Y-shaped osteotomy for congenital coxa vara / 中国矫形外科杂志

[Objective]To investigate Y-shaped osteotomy and the internal fixation with geese-head steel plates for treatment of congenital coxa vara.[Method]Lesser subtrochanteric and greater laterotrochanteric osteotomy were performed for 7 cases(9 hips)of congenital coxa vara with the Y-shaped osteotomy line.The distal femoral section met the lateral greater trochanteric section,fixed with geese-head steel plates.[Result]The cases were followed up for 1~5 years(averaged 3.6 years).According to Shi Yingqi's evaluation,4 hips were excellent,4 good and 1 fair.[Conclusion]The Y-shaped osteotomy and internal fixation with geese-head steel plates can improve the abnormal structure of upper femur,increase the real length of the affected limb.Therefore,it is an ideal effective method for treatment of congenital coxa vara.

Coxa Vara with Spondylometaphyseal Dysplasia / 대한정형외과학회잡지

Spondylometaphyseal dysplasia(SMD) is very rare disease characterized with platyspondyly and metaphyseal changes in the long bone. Shrot status and later abnormal gait associated with coxa vara are common clinical feature. A 6 years 4 months old boy progressive bilateral coax vara were transferred with abduction limitation of the hip. With the skeletal survey, multiple dysplastic changes of the metaphysic of the long bone including knee, shoulder, ankle, and flatterning of the body of lower thoracic and upper lumbar spine(T11,12,L1) were noted. A corrective valgus osteotomy on the both hip were followed with improvement of the range of motion.

Congenital Coxa Vara: Report of Two Cases / 대한정형외과학회잡지

Congenital coxa vara is an uncommon dysplasia, not evident at brith, which first noticed when the child begins walking. Radiographs of the proximal femur include a decreased neck shaft angle, a wide and vertically aligned physis, an irregular metaphyseal ossification, a shortened femoral neck, a triangular osseous fragment adjacent to the inferior margin of the physis, a normal but osteoporotic femoral head, and secondary degenerative changes of the hip joint in neglected case. Prompt diagnosis and early management can reduce severe deformity and degenerative changes of the hip. Authors had been treated two cases of congenital coxa vara with valgus osteotomy followed by good results.

Congenital Coxa Vara, Acquired Coxa Vara and Valga / 대한정형외과학회잡지

In 5 cases of congenital coxa vara, 7 cases of acquired coxa vara and 4 hips in 3 cases of acquired coxa valga, we performed subtrochanteric osteotomies at Department of Orthopedic Surgery, SeoulNational University Hospital, from December 1980 to February 1984. At a relatively short interim follow-up, following observations were made on the correction of the femoral neck-shaft angle deformities. l. In the congenital coxa vara group, at an average follow-up of 1 year and 3 months, 97.9% of the correction obtained by osteotomy was maintained, based on the roentgenographic measurements of femoral neck-shaft angle. In the acquired coxa vara group, at an average follow-up of 1 year and 2 months, 93.3 % of the correction obtained by osteotomy was maintained. In the acquired coxa valga group, at an average follow-up of 1 year and 5 months, 92.9% of the correction obtained by osteotomy was maintained. 2. At final follow-up, leg length gain averaged 1.26cm in the congenital coxa vara group and 2.23cm in the acquired coxa vara group. An average 0.70cm decrease in leg length was noted in the acquired coxa valga group. 3. Trendelenburg sign, which was positive in all the cases of the congenital and acquired coxa vara group, reverted to negative in all. 4. Slight overcorrection in cases of the acquired coxa vara and undercorrection in cases of the acquired coxa valga, is recommended for later loss of surgically corrected femoral neck-shaft angle. In the congenital coxa vara, it appeared that loss of correction was relatively minor.

A Clinical Study of the Fractures of the Femoral Neck in Children / 대한정형외과학회잡지

Fractures of the femoral neck in children are rare and usually occur as a result of severe trauma. They are one of the most challenging injuries to treat. Their complication rate is high and their anatomic and physiologic characteristics make treatment difficult. The commonly encountered complications are avascular necrosis, nonunion, coxa vara and premature fusion of epiphyseal plate. Treatment of these fractures remains an unsolved problem but accurate reduction held with adequate internal fixation would appear to offer the best chance for succesful results. We report our observations in the treatment of eighteen cases of fracture of the femoral neck in children. Included is a review of references. The results obtained are as follows: 1. Among 18 patients, 11 were boys and 7 were girls. the highest incidence was between the ages of 5 and 7 years. 2. The main causes of fractures were traffic accidents in 10 cases (55.5%), falling from a height, and slipping down. 3. The commonest type of fracture was the cervicotrochanteric type (47.4%). Twelve cases were displaced fractures (66.7 %). 4. The range of Pauwels angle varied from 20° to 70°. 5. The commonly associated injuries were pelvic bone fractures, extremity fractures, skull fractures and soft tissue injuries. 6. Nine cases were treated by skin traction and plaster cast. Five cases ere treated by closed reduction and internal fixation with K-wires or knowles pins. 7. In 13 of the 18 cases, follow-up studies were done for an average period of 18 months. 8. The results were analyzed according to the Ratliffs assessment. Among the 13 cases, 10 cases showed good results, two fair and one poor. 9. All cases treated by closed reduction and internal fixation showed good results without complications. 10. Complications developed in five cases (38.5%), three cases of avascular necrosis and two cases of coxa vara. Among these five cases, one case of avascular necrosis developed as a result of neglect of the fracture and the others as a result of too conservative a treatment. 11. Secondary treatment of trochanteric arthroplasty was required in only one neglected case. This was further by avascular necrosis and the results were poor. 12. As a result, we recommond closed reduction and internal fixation as the most favorable method of treatment for the fracture of the femoral neck in children.

Bilateral Congenital Coxa Vara: Report of one case / 대한정형외과학회잡지

Congenital coxa vara is a rare congenital deformity, seen in infancy and childhood and the ratio of unilateral to bilateral is 3:1. The deformity does not become manifest until after birth and usually not until the age of walking. Pathologically, it consists of a progressively increasing acuteness of the neck-shaft angle;shortnees of the neck; a vertical direction of the epiphyseal plate; an oblique defect of the neck; a greater trochanter extending upward toward the ilium as a besk; a shortened femur; a secondary degenerative changese in the acetabulum due to malposition. We have experienced a case of bilateral congenital coxa vara treated with valgue osteotomy and the case review is presented.