Purpose. To define the orthopaedic problems associated with pseudoachondroplasia (PSACH) and their functional impact. Methods. We reviewed the medical records of 12 consecutive patients presenting to our unit. Radiographic analysis of deformity included assessment of mechanical axis and dysplasia at hip, knee and ankle measured by acetabular index (AI), Reimer's migration percentage (MP), neck-shaft angle, distal lateral femoral (aDFLA) and proximal (mMPTA) and distal tibial angles. The paediatric/adolescent PODCI questionnaires and the SF36 were used to assess quality of life issues. Results. 12 patients (9 female) were reviewed at median age 18yrs (range 12-43yrs). Most symptoms related to walking tolerance, joint discomfort and deformity: 9 patients had genu varum, 7 tibial torsion, 2 patella instability and 3 significant low back pain. All patients had hip dysplasia. 10 had medial displacement of the mechanical axis, with a mean mDFLA 105 deg (88 -128) and mMPTA 75deg (51-90). 2 patients have been treated only with growth hormone; 10 patients have undergone a total of 9 distal femoral, 19 proximal tibial and 2 supramalleolar osteotomies. 6 procedures were performed using an external fixator. 7 limb segments have been treated by guided growth and in all these cases alignment has improved. One patient has had bilateral hip arthroplasties (age 29), a second patient has had bilateral patellectomies. These 10 patients have undergone a mean 3.8 operative procedures on a mean 2.4 occasions. Patients scored less well than their peer groups in all domains of the PODCI assessment. All have maintained some independent mobility. Conclusions. PSACH is a severe skeletal dysplasia with deformity at all levels of the lower limb affecting patient satisfaction and quality of life. Knee deformities are those which most frequently require surgical intervention. Significance. The genetic defect in PSACH differs from that in achondroplasia, joint degeneration is more common and maintenance of limb alignment is essential

The aim of this study was to determine whether an osteoplasty of the femoral neck performed at the same time as an intertrochanteric Imhäuser osteotomy led to an improved functional outcome or increased morbidity. A total of 20 hips in 19 patients (12 left, 8 right, 13 male, 6 female), who underwent an Imhäuser intertrochanteric osteotomy following a slipped capital femoral epiphysis were assessed over an eight-year period. A total of 13 hips in 13 patients had an osteoplasty of the femoral neck at the same time. The remaining six patients (seven hips) had intertrochanteric osteotomy alone. The mean age was 15.3 years (13 to 20) with a mean follow-up of 57.8 months (15 to 117); 19 of the slips were severe (Southwick grade III) and one was moderate (grade II), with a mean slip angle of 65.3° (50° to 80°); 17 of the slips were stable and three unstable at initial presentation. The mean Non-Arthritic Hip Scores (NAHS) in patients who underwent osteoplasty was 91.7 (76.3 to 100) and the mean NAHS in patients who did not undergo osteoplasty was 76.6 (41.3 to 100) (p = 0.056). Two patients required a subsequent arthroplasty and neither of these patients had an osteoplasty. No hips developed osteonecrosis or chondrolysis, and there was no increase in complications related to the osteoplasty. We recommend that for patients with a slipped upper femoral epiphysis undergoing an intertrochanteric osteotomy, the addition of an osteoplasty of the femoral neck should be considered.

Cite this article: Bone Joint J 2014;96-B:1119–23.

We reviewed three infants with destructive osteomyelitis involving the proximal tibial epiphysis at a follow-up of eight to 22 years. All cases showed early radiographic destructive changes in the medial or lateral aspects of the epiphysis and metaphysis. Despite the ominous early appearance of the epiphysis, all cases showed spontaneous re-ossification of the epiphysis with restoration of the tibial condyle and preservation of joint congruity. The patients, however, developed a valgus or varus deformity which was treated satisfactorily with one to three proximal tibial osteotomies. The potential for regeneration of the epiphysis following infantile osteomyelitis of the proximal tibia suggests these cases should be treated expectantly with regard to joint congruity.