004). Kyphosis had a moderate negative correlation with FVC (r = -0.43; p = 0.03). Chest wall deformity had a strong negative correlation with FEV1/FVC (r = -0.61; p = 0.001). The magnitude of the thoracic curve (mean 55.2°; 28° to 92°) had a significant moderate negative correlation with TLC (r = -0.45; p = 0.04).

In MFS, three factors correlate with decreased pulmonary function measures hypokyphosis, increasing chest wall deformity and increasing coronal curve magnitude. Hypokyphosis and increased chest wall deformity correlated with diminished FEV1/FVC; increasing thoracic spinal curvature with diminished TLC. Further analysis with a larger cohort will help better define the relationship between these deformities and pulmonary function in this unique population.

IV.

IV.

Unicameral bone cysts (UBCs) are most often found when accompanied by a pathological fracture. In these cases, the doctor must determine the optimal timing for the surgery. The purpose of this study was to evaluate the outcome of immediate surgery as compared with delayed surgery in paediatric pathological fractures due to UBCs.

This retrospective study assessed the medical records of 65 patients between January 2012 and September 2016. Group A included 34 patients who underwent immediate surgery, including curettage, demineralized bone matrix and fixation with elastic stable intramedullary nailing. Group B included 31 patients who underwent the same surgery several months later. The outcome evaluations included the radiological changes, brace fixation time, cyst healing time, at the first-, third- and sixth month, and final visit.

The mean brace fixation time was 26.3 days (sd 5.7) for group A and 53.8 days (sd .1) for group B (p = 0.012). According to radiological evaluations, 3/34 patients in group A and 5/31 in group B had recurrence after the first surgery. A second surgery was performed in all cases of recurrence, and all fractures were healed at the last visit. There was no significant difference in the recurrence rate between the two groups (p = 0.4631), and healing times were also similar (p = 0.6033).

Both the immediate and delayed surgery were safe for the treatment of UBCs with fractures. We suggest immediate surgery for shorter fixation time and early activity.

IV.

IV.

Chondrosarcomas typically present in adults during the fifth to seventh decades and are rare in young patients. The biological behaviour and oncological outcomes may be different in children and adolescents.

We retrospectively evaluated the outcomes of all patients with chondrosarcoma of bone who were younger than 18 years of age at the time of diagnosis and were treated at our centre between 1995 and 2018.

The 15 consecutive patients studied included nine male and six female cases, with a mean age at diagnosis of 13 years (7 to 17). The median follow-up was 117 months (30 to 277). The tumours were primary and secondary in ten and five patients, respectively. The tumours were central in 13 and surface in two patients. The tumour locations were the humerus in five, digits in five, femur in three, radius in one and pelvis in one patient. The histological grades were grade I in seven, grade II in seven and grade III in one patient. The surgical treatments were limb salvage in ten patients and ray amputation in five patients. The surgical margins were wide in eight, marginal in two and intralesional in five patients. All the patients were alive and continuously free of disease at the time of the last follow-up. No patient developed metastases or local recurrence.

Chondrosarcoma of bone in children and adolescent patients has a very good prognosis and is less aggressive compared with published outcomes in older patients.

IV.

IV.

Langenskiöld described a reconstructive soft-tissue procedure for irreducible lateral congenital patellar dislocations. Proteases inhibitor Paley further detailed the technique in the surgical management of congenital femoral deficiency. The aim of this study was to evaluate the outcomes of patients with congenital, chronic and recurrent patellar dislocations treated with the modified Langenskiöld procedure.

This is a retrospective case series. Between 2011 and 2018, 18 knees in 13 patients (mean age 15.8 years (sd 4.4; 12 to 29.9), nine female) with diagnoses of recurrent (six patients, eight knees), chronic (four patients, six knees) and congenital (three patients, four knees) patellar dislocations were treated with the modified Langenskiöld procedure.

There were no recurrent lateral dislocations in the congenital or recurrent groups. One of the patients in the congenital group had an overcorrection with some medial patellar maltracking but until this time has not required any further surgery. In the chronic group two of the six knees developed further dislocations; these were both on the same patient, who had no dislocations until one year after surgery. Mean Kujala score was 83.7 (sd 17; 47 to 100) for all groups. In spite of preoperative knee flexion contractures of up to 30° in three patients (six knees), all patients had full extension postoperatively. Eight patients reported being satisfied with their outcome, one was somewhat satisfied, two were very dissatisfied, and two did not respond.

The modified Langenskiöld reconstruction provides a powerful correction for challenging cases of congenital and recurrent patellar dislocations. Re-dislocation as well as overcorrection can occasionally occur.

Level IV.

Level IV.

The aim of this study was to evaluate the success of guided growth by temporal hemiepiphysiodesis of the lateral proximal tibia as a first line treatment option for Blount disease.

This was a retrospective multicentre study conducted in five centres, covering data on 55 limbs in 45 patients, with an average follow-up of 24.5 months following plate insertion. Preoperative alignment analysis was compared with three measurements taken postoperatively. The normalization of the mechanical medial proximal tibia angle (mMPTA) was defined as the primary outcome measure.

Mean age at surgery was 9.5 years. Average preoperative mMPTA was 77°. On average, at 24.5 months post-surgery, mMPTA was 86.33°, while 43/55 limbs (78.18%) have achieved normalization (mMPTA 85° to 90°). Average rate of correction was 1° per month. When grouping the children as infantile (11 limbs), juvenile (12 limbs) and adolescent (32 limbs), operated on before the age of four years, between four and ten years and after the age of ten years, respectively, 63.