Cornelia de Lange syndrome: A rare case, presented with unilateral pes equinovarus.

Cornelia de Lange syndrome is a genetic disorder with multiple system abnormalities. It is especially characterized by typical facial appearance and hirsutism. Growth and mental retardation, gastrointestinal, cardiovascular, and orthopedic abnormalities are other important features of this syndrome. In this case, we present a rare manifestation of Cornelia de Lange syndrome with a unilateral pes equinovarus deformity without other more specific orthopedic manifestations. Ponseti method's was applied as the initial procedure. Afterwards, complete subtalar release was performed. After four years follow-up, clinical and radiological results were satisfactory. Unilateral pes equinovarus deformity may be a part of this syndrome as well as a sporadic presentation. The discrimination is important for anesthetic procedures and surgical outcomes.

Are the Appropriate Use Criteria for the management of pediatric supracondylar humerus fractures useful in clinical practice?

The aim of this study was to evaluate our treatment modalities in pediatric supracondylar humerus fractures according to the recently published Appropriate Use Criteria (AUC) by the American Academy of Orthopaedic Surgeons. A total of 991 patients with supracondylar humerus fractures were included. After reviewing medical records, 38 different clinical scenarios described in the AUC were observed. Of the 991 patients, 127 were type I, 423 were type II, and 405 were type III fractures according to the Gartland classification. The AUC was appropriate for 100% of type I, only 25 (5.9%) of type II, and 389 (96.0%) of type III fractures. Overall, the total appropriateness rate was 54.5%. We concluded that application of the AUC is useful but burdensome and that AUC provides important guidance especially for rarely encountered urgent clinical scenarios.

How safe is the semi-sterile technique in the percutaneous pinning of supracondylar humerus fractures?

BACKGROUND: The purpose of the present study was to evaluate safety and efficiency of the semi-sterile technique used in recent years in treatment of pediatric supracondylar humeral fractures (SHF). METHODS: Total of 712 patients who were treated for SHF via closed reduction and percutaneous fixation with semi-sterile technique were enrolled in present study. Patients were evaluated for postoperative infection and other complications. Clinical and radiological assessments were also made. RESULTS: It was found that there were 52 (7.3%) pin tract infections, which responded to oral antibiotic administration and pin care without need for early pin removal (before 3 weeks). There were no deep infections. Loss of reduction was observed in 82 patients (11.5%). There were 59 iatrogenic nerve injuries (8.3%), of which 52 (7.3%) were ulnar palsy. Clinically apparent cubitus varus was observed in 29 (4.1%) patients. CONCLUSION: Though semi-sterile technique is an effective treatment in closed percutaneous pinning of SHF, increased pin tract infection risk is a matter of concern.

Debridement, antibiotics, irrigation, and retention (DAIR) of the prosthesis after hip hemiarthroplasty infections. Does it work?

PURPOSE: Debridement, antibiotic, and implant retention (DAIR) is an attractive treatment modality after hip hemiarthroplasty (HA) infections. Data about the success of the procedure after acute onset infections is lacking. The aim of this study was to analyze the clinical outcome and associated risk factors. METHODS: A multicenter, retrospective cohort study was designed, including 39 patients with acute onset prosthetic infection who had undergone debridement and irrigation with prosthesis retention. The primary outcome measure was infection eradication without prosthesis removal. We also analyzed how the success rate was influenced by the length of the interval between implantation of the prosthesis and the beginning of the treatment. RESULTS: The overall success rate was 41%. Sedimentation rate over 60 mm/h and the longer duration (2 weeks) after prosthesis implantation were found as factors negatively influencing the success rate. CONCLUSIONS: Our results indicated limited success to DAIR- treated patients with infected HA. The high failure rate of DAIR treatment after 2 weeks from the implantation should be taken into consideration.

Medial percutaneous hemi-epiphysiodesis improves the valgus tilt of the femoral head in developmental dysplasia of the hip (DDH) type-II avascular necrosis.

BACKGROUND AND PURPOSE: Avascular necrosis (AVN) is a major cause of disability after treatment of developmental dysplasia of the hip (DDH), leading to femoral head deformity, acetabular dysplasia, and osteoarthritis in adult life. Type-II AVN is characterized by retarded growth in the lateral aspect of the physis or by premature lateral fusion, which produces a valgus deformity of the head on the neck of the femur. We investigated the effect of medial percutaneous hemi-epiphysiodesis as a novel technique in the treatment of late-diagnosed type-II AVN. PATIENTS AND METHODS: 9 patients (11 hips) with a diagnosis of type-II AVN who underwent medial percutaneous hemi-epiphysiodesis after the surgical treatment for DDH were included in the study. 10 patients (12 hips) with the same diagnosis but who did not undergo hemi-epiphysodesis were chosen as a control group. Preoperative and postoperative articulotrochanteric distances, head-shaft angles, CE (center-edge) angles, and physeal inclination angles were measured. The treatment group underwent medial hemi-epiphysodesis at a mean age of 8 years. The mean ages of the treatment group and the control group at final follow-up were 14 and 12 years respectively. The mean duration of follow-up was 5.7 years in the treatment group and 8.3 years in the control group. RESULTS: Preoperative articulotrochanteric distance, head-shaft angle, and functional outcome at the final follow-up assessment were similar in the 2 groups. However, preoperative and postoperative CE angles and physeal inclination angles differed significantly in the treatment group (p < 0.05). The final epiphyseal valgus angles were better in the treatment group than in the control group (p = 0.05). The treatment group improved after the operation. INTERPRETATION: Medial percutaneous epiphysiodesis performed through a mini-incision under fluoroscopic control is a worthwhile modality in terms of changing the valgus tilt of the femoral head.

Fixation of intertrochanteric femur fractures using Proximal Femoral Nail Antirotation (PFNA) in the lateral decubitus position without a traction table.

OBJECTIVE: The aim of this study was to evaluate whether intertrochanteric femur fractures can be reduced and nailed properly in the lateral decubitus position using Proximal Femoral Nail Antirotation (PFNA) as a fixation device without the use of a traction table. METHODS: The study included 207 patients (81 male and 126 female; mean age: 75 years, range: 22 to 95 years). According to the Evans classification, there were 7 Type 1, 40 Type 2, 33 Type 3, 38 Type 4, 61 Type 5 and 28 reverse oblique fractures. Radiographs were used to measure the tip-apex distance (TAD), the quadrant of the helical blade according to Cleveland and Bosworth, Ikuta's reduction subgroup, collodiaphyseal angle and reduction gaps postoperatively. RESULTS: Mean follow-up time was 20.4 (range: 6 to 38) months. According to Ikuta's classification, 176 (85%) reduced fractures were of subtype N, 15 (7.2%) subtype P and 16 (7.7%) subtype A. Good or acceptable reduction according to the Herman criteria was obtained in 99% of fractures. Mean TAD was 29.2 millimeters. Mean operation time was 57.2 minutes. Optimal blade position (center-center or inferior-center) was achieved in 53.5% of patients and was in the superior-posterior quadrants in only 2.4% of patients. Cut-out complication occurred in 9 patients (4.3%). CONCLUSION: While the nailing of intertrochanteric fractures in a lateral decubitus position does not provide ideal quadrant placement and TAD, results are encouraging probably due to the excellent stability that is provided by PFNA.

How are outcomes affected by performing a one-stage combined procedure simultaneously in bilateral developmental hip dysplasia?

PURPOSE: The aim of this study was to compare the outcomes of one-stage combined surgical treatment in children of unilateral and bilateral developmental hip dysplasia diagnosed after walking age. METHODS: We reviewed 24 patients who underwent one-stage combined surgery for DDH diagnosed after walking age. Group I consisted of 12 patients with bilateral disease who underwent bilateral operation at one operative setting. Group II consisted of 12 patients with unilateral disease. Pre-operatively the hips were classified according to Tönnis classification. Acetabular dysplasia was evaluated by measuring acetabular index (AI) angles pre-operatively. The acetabular improvements with time regarding AI was noted immediately postoperatively, every six months, and at the final follow-up examination. RESULTS: Mean follow up of the bilateral group I and group II were 54.8 months (range 20-84 months) and 52.6 months (24-80), respectively. There were no statistically significant differences between immediate postoperative and final follow up acetabular index improvement rates in both groups. Also there was no significant difference when both hips were compared in group I. The clinical final outcome of both groups and within group I was similar. CONCLUSIONS: Simultaneous combined surgery is a challenging but worthwhile procedure for late diagnosed patients with bilateral DDH. The short-mid term outcomes of the procedure are encouraging.