Orthopaedic diagnoses in patients with Klippel-Trenaunay syndrome.

PURPOSE: Klippel-Trenaunay syndrome (KTS) is a rare combined vascular malformation composed of capillary malformation, lymphatic and/or venous malformation and limb overgrowth, which commonly affects the extremities. Due to limb involvement, it is not uncommon for these patients to require referral to an orthopaedic surgeon. Herein we reviewed the prevalence of orthopaedic diagnoses in a large cohort of KTS patients and described the associated surgical interventions. METHODS: Between 1976 and 2012, 410 patients fulfilling strict criteria for KTS were evaluated at a single institution. Patient charts were reviewed for demographic information, details of the clinical evaluation, orthopaedic consultation and surgical interventions. RESULTS: A total of 264 of 410 patients (64%) with confirmed KTS required orthopaedic evaluation. Of these 264 patients, 84% had documented limb-length discrepancy. Other common diagnoses included: angular deformities (10%), scoliosis (9%), osteopenia/osteoporosis (7%), pathological fractures (6%), joint contracture (5%), degenerative joint disease (4%) and limb/joint pain (4%). Of the 264 patients evaluated by orthopaedic surgery, 133 patients (50.4%) underwent 169 surgeries. Surgery was most commonly performed for limb-length discrepancy (62%). Multivariable analysis confirmed an orthopaedic condition was more likely in patients with lymphatic malformation (odds ratio (OR) 3.78; p < 0.001), as well as in those with bone and/or soft-tissue hypertrophy of the lower extremity (OR 7.51; p < 0.001) or foot (OR 3.23; p < 0.001). CONCLUSION: Orthopaedic conditions are common in patients with KTS and approximately 50% require surgical intervention. Those with a lymphatic malformation and/or soft-tissue hypertrophy of the lower extremity are more likely to need surgery. LEVEL OF EVIDENCE: Level IV, Descriptive Case Series.

Management of paediatric humeral shaft fractures and associated nerve palsy.

PURPOSE: The aim of this study is to review the management of all paediatric humerus diaphyseal fractures treated at a single institution over a 20-year period. METHODS: Retrospective review from between 1996 and 2016 identified 96 humerus shaft fractures in paediatric patients (0 to 17 years). After excluding those deceased from inciting trauma, pathological and perinatal fractures, 80 patients remained for analysis. Data collected included age, fracture type, displacement, nerve palsy, treatment, complications and time to union. Radiographs were reviewed at the time of injury and at latest follow-up. RESULTS: Of 80 paediatric humeral diaphyseal fractures, 65 (81%) were treated with immobilization. In all, 15 (19%) fractures were treated with surgical stabilization. Most common indications were fracture displacement, open fractures and to improve mobilization in patients with multiple injuries. Fractures were stabilized with a plate (eight), flexible nails (five), external fixation (one) and percutaneous pinning (one). The operative group, compared with the nonoperative group, was older, had more high-energy mechanisms, more open fractures and increased fracture displacement. All patients in the nonoperative and operative groups went on to union with minimal complications. A nerve palsy was present in five patients (6%)with three of the five involving the radial nerve (4%). All nerve palsies were observed and had full neurological recovery. CONCLUSION: Over a 20-year period nonoperative management of paediatric humerus shaft fractures was successful in the majority of patients. Operative stabilization, when rarely indicated, had a low complication rate and improved radiographic alignment. All nerve injuries fully recovered without surgical intervention. LEVEL OF EVIDENCE: IV.

Colonna arthroplasty with concomitant femoral shortening and rotational osteotomy. Long-term results.

The results of Colonna capsular arthroplasty in twenty-two hips in twenty patients were reviewed. All twenty patients were at least five years old at the time of the operation, which was performed for either complete dislocation or marked subluxation of the hip. None were candidates for reconstructive procedures designed to preserve articular cartilage. The mean age at the time of the Colonna arthroplasty was nine years and three months (range, five years to fifteen years and two months), and the mean duration of follow-up was sixteen years (range, six to thirty-two years). At the most recent follow-up examination, the mean Harris hip score, for the twenty-one hips for which it was available, was 82 points (range, 52 to 98 points), the patients had improved gait, and there was marked improvement in the radiographic appearance of the hip according to the classification system of Severin. Thirteen hips in twelve patients had concomitant femoral shortening and rotational osteotomy at the time of the Colonna arthroplasty, and none of these patients who did not have evidence of avascular necrosis of the capital femoral epiphysis preoperatively had it postoperatively. Three hips that did not have concomitant femoral shortening had evidence of new-onset avascular necrosis after the Colonna arthroplasty. Concomitant femoral shortening and rotational osteotomy allowed the operation to be performed without preoperative traction, dramatically reduced the need for a subsequent rotational femoral osteotomy, and reduced the prevalence of postoperative avascular necrosis of the capital femoral epiphysis.

Femoral shaft fracture treatment in patients age 6 to 16 years.

Traction followed by spica casting, the one method used to treat femoral shaft fractures in children that was used in the past, has given way to a multiplicity of methods today. To evaluate the morbidity and costs of these various methods, 85 fractures in 81 patients age six to 16 years were evaluated. Early spica casting gave excellent results with low complications and low costs. All surgical treatments cost approximately the same: 3 times the cost of early spica casting and equivalent to traction followed by spica casting. Intramedullary flexible rods resulted in quicker healing and return to full weight bearing than did external fixation, which had the highest complication rate. One case of avascular necrosis in an 11-year-old girl treated with a reamed intramedullary rod suggests that this method is best reserved for children at or near skeletal maturity.

Results of total hip arthroplasty for Crowe Type III developmental hip dysplasia.

From 1969 through 1980, 90 hips in 82 patients had cemented total hip arthroplasty for Type III developmental hip dysplasia. Seventy hips were reviewed at an average of 16.6 years (range, 5-23 years) after operation. Aseptic loosening developed in 53% of acetabular cups and 40% of femoral stems. Despite attempts to place acetabular components in the anatomic center, 18 cups (25.7%) were placed outside that area. Using a measurement method to determine the true acetabular region and approximate femoral head center, final acetabular loosening strongly correlated with initial cup placement. Loosening occurred in 15 of 18 cups (83.3%) initially positioned outside of the true acetabular region compared with loosening in 22 of 52 cups (42.3%) initially positioned within the true acetabular region. Acetabular loosening also correlated with initial lateral displacement or initial superior displacement of the hip center from the approximate femoral head center. Initial cup placement medial to the approximate femoral head center was predictive of successful long term acetabular component fixation. The method of acetabular reconstruction did not affect eventual cup loosening. Placement of the hip arthroplasty center of rotation in or near the true acetabular region is recommended.