METHODS: We describe the surgical management of a girl with dwarfism and congenital spondyloepiphyseal dysplasia, who presented in adolescence with coxa vara and bilateral pseudarthrosis between the femoral neck and the diaphysis, with asymmetric distal migration at both sites and leg length discrepancy. The patient at 16 years underwent valgus osteotomy in situ and femoral neck fixation in the left hip. The right hip was operated 19 months later.
CONCLUSIONS: Hip dysplasia is common in spondyloepiphyseal dysplasia, but a debilitating nontraumatic bilateral pseudarthrosis at the base of the femoral neck has not been previously reported. Valgus corrective osteotomies of the femur in situ led to union and allowed the patient to walk.

The incidence of Slipped capital femoral epiphysis (SCFE) after management of femoral neck fracture in pediatrics is very rare. In this case report, a nine-year-old female child sustained left sided femur neck fracture after a motor car accident. The fracture was fixed by two cannulated screws and healing with mild varus occurred after six weeks. However, progressive slippage of femoral epiphysis was observed. This was treated by subtrochanteric valgus osteotomy and fixed by tension band and Wagner technique. Better radiological and functional outcomes were obtained at the short term follow up.

UNASSIGNED: X-linked hypophosphatemic rachitis (XLHR) is the most common cause of hereditary rickets that can lead to long bone deformities requiring multiple surgical correction procedures. In addition, high rates of fractures are reported in adult XLHR patients. This study aimed to report a case of femoral neck stress fracture in XLHR patient treated with mechanical axis correction. No previous studies demonstrating a combined valgus correction and cephalomedullary nail fixation were identified in the literature.
UNASSIGNED: A 47-year-old male patient with XLHR attended the outpatient clinic with severe left hip pain. X-rays revealed a left proximal femoral varus deformity and a femoral neck stress fracture. After 1 month without improvement of pain, and no radiographic sign of healing, correction of the proximal femoral varus deformity and fixation of the cervical neck fracture was achieved by a cephalomedullary nail. At 8 months follow-up, hip pain relief was achieved with radiographic healing of the femoral neck stress fracture and the proximal femoral osteotomy.
UNASSIGNED: A review of the literature was performed to identify any case report of femoral neck fractures fixation due to coxa vara in an adult. Both coxa vara and XLHR can cause femoral neck stress fracture. This study presented the surgical technique for treating a rare case of femoral neck stress fracture in a XLHR patient with coxa vara. Pain relief and bone healing were achieved by combined deformity correction and fracture fixation with a femoral cephalomedullary nail. The technique for deformity correction and cephalomedullary nail insertion in the patient with coxa vara is shown.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare genetic disease characterized by tetrad camptodactyly, noninflammatory arthropathy, coxa vara deformity, and pericardial effusion. Arthropathy typically affects large joints and presents with joint swelling in the absence of other signs of inflammation. We described the case of a girl affected by CACP syndrome caused by a novel compound heterozygous variant in proteoglycan 4 gene (c.2831_2832insT; c.3892C > T) and associated with temporomandibular involvement. The patient received treatment with intra-articular hyaluronic acid injections, which presented rapid but transient improvements of pain and range of motion. A literature review of previously reported CACP patients has been performed. Of the patients. 69.2% (101 out of 146) were Middle Eastern, and 65.7% (96) were consanguineous. The median age of onset was 24 months (interquartile range of 12-36 months), and median age of diagnosis was 96 months (interquartile range of 48-156 months). Arthropathy was always present, mainly involving hips (95.2%), knees (92.4%), wrists (87.7%), elbows (79.5%), and ankles (57.5%). Camptodactyly and pericardial effusion were described, respectively, in 97.3% (142) and 15.1% (22) of patients. The main radiological findings were coxa vara (95.2%), femoral changes (64.4%), intraosseus cysts (14.4%), and bone erosion (5%). Of the patients, 32.9% (48) had received a previous juvenile idiopathic arthritis diagnosis. CACP syndrome can be easily misdiagnosed with juvenile idiopathic arthritis. A prolonged lack of response to immunosuppressive therapy associated with typical clinical and radiological features should prompt consideration of this rare syndrome.

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The rare case of a 5‑year-old girl with autosomal dominant osteopetrosis type 2, who suffered metaphyseal fractures of the femoral neck on both sides within 6 months is described. On the right side, the diagnosis was made 3 months after the onset of symptoms, so that a coxa vara occurred. The treatment was surgically treated through a valgus osteotomy with fixation of the femoral head with K‑wires. Three months after the operation, the girl complained of a painful restriction of movement on her left side. Radiologically, a metaphyseal femoral neck fracture without coxa vara was diagnosed and in situ fixated with 2 K wires. Two months after the second operation, there was a symmetrical free range of motion of the hips with no symptoms. The metaphyseal femoral neck fracture with verticalization of the growth plate is a serious disease in autosomal dominant osteopetrosis due to the development of a coxa vara, which, if diagnosed at an early stage, can be treated well with in situ fixation. If the coxa vara has already developed, a valgus osteotomy should be performed despite the risk of delayed bone healing.
UNASSIGNED: Es wird der seltene Fall eines 5‑jährigen Mädchens mit autosomal dominant vererbter Osteopetrose Typ 2 beschrieben, die innerhalb eines halben Jahres beidseits metaphysäre Schenkelhalsfrakturen erlitt. Auf der rechten Seite wurde die Diagnose erst mit 3 Monaten Verzögerung nach Auftreten der Symptome gestellt, sodass es bereits zu einer Coxa vara kam. Die Aufrichtung erfolgte operativ durch eine Valgisationsosteotomie mit Fixierung des Hüftkopfes mit Kirschner-Drähten. Drei Monate nach der Operation klagte das Mädchen über eine schmerzhafte Bewegungseinschränkung auf der linken Seite. Radiologisch konnte der Verdacht auf eine metaphysäre Schenkelhalsfraktur aber ohne Coxa vara gestellt werden. Es erfolgte die In-situ-Fixation mit 2 K-Drähten. Bereits 2 Monate nach der zweiten Operation zeigte sich ein symmetrisches freies Bewegungsausmaß der Hüften bei Beschwerdefreiheit. Die metaphysäre Schenkelhalsfraktur mit Vertikalisierung der Wachstumsfuge ist bei autosomal-dominant vererbter Osteopetrose aufgrund der Entwicklung einer Coxa vara eine ernstzunehmende Erkrankung, die, frühzeitig diagnostiziert, gut mit In-situ-Fixation behandelt werden kann. Bei einer bereits entstandenen Coxa vara sollte trotz der Gefahr der verzögerten Knochenheilung eine valgisierende Osteotomie vorgenommen werden.

Focal fibrocartilaginous dysplasia (FFCD) is a rare disease that can cause angular deformities of long bones. The common pathologic finding is a thick fibrotic band extending from epiphysis to metaphysis on one side of the bone. The tethering effect of the fibrotic band around the growth plate is thought to be the main etiology for the development and progression of the deformity. FFCD mostly affects the proximal tibia and the distal femur. The literature contains different treatment options. Here, we present the case of a 20-month-old girl with FFCD on the medial side of the distal femur causing varus deformity. Our treatment protocol included excision of the fibrotic band from the medial side and application of a two-hole plate for guided growth on the lateral side of the distal femur. Deformity correction was achieved rapidly with no complications. A literature review is also presented along with pathologic and magnetic resonance imaging findings.

METHODS: We reported a case of a 25-year-old woman with idiopathic bilateral coxa vara who had initial presentation of hip osteoarthritis. She was later treated with bilateral subtrochanteric valgus osteotomy. A good functional outcome was recorded without nonunion or deformity recurrence. The arthritis of the hips also decelerated.
CONCLUSIONS: Coxa vara first diagnosed in adulthood was relatively uncommon, and the cause in this present case was uncertain. Subtrochanteric valgus osteotomy seemed to be a suitable treatment for this case.

UNASSIGNED: Fibrous dysplasia is a rare benign disorder of the skeletal system characterized by fibro-osseous proliferation with intervening areas of normal or immature bone in the intramedullary region. It can either be a monostotic (involves one bone) or a polyostotic (involves more than one bone) presentation and usually occurs equally in males and females. Deformities such as scoliosis and shepherd\'s crook deformity are frequently encountered in the polyostotic form. We report the management of a rare managed case of bilateral shepherd\'s crook deformity of the proximal femur with impending neck of femur fracture.
UNASSIGNED: A 22-year-old male known case of polyostotic fibrous dysplasia presented with bilateral shepherd\'s crook deformity of the proximal femur. Preoperatively, neck-shaft angle was 15° on the right side and 55° on the left side. The patient had severe pain on the right side due to an impending neck of femur fracture. On the right side, we first did a valgus osteotomy in the subtrochanteric region and fixed with dynamic hip screw, and after 3 months, the left proximal femur valgus osteotomy with dynamic hip screw fixation was done. Postoperatively, we achieved a neck-shaft angle of 140° on the right side and 135° on the left side. 1-year follow-up imaging showed union at both the osteotomy sites. At present, at 12-month post-operative, the patient is walking full weight-bearing without support, no apparent shortening.
UNASSIGNED: Valgus osteotomy is an easy and effective method to correct the shepherd\'s crook deformity and achieve correct mechanical alignment. Valgization of the shepherd crook deformity will prevent the development of a pathological neck of femur fracture.

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