P014 Use of prosthetic surgery in Juvenile Idiopathic Arthritis

Background Juvenile Idiopathic Arthritis (JIA) is the most common inflammatory rheumatism in childhood characterized by destructive potential. The involvement of weight-bearing joints constitutes an evolutionary turning point in the course of the disease, which may be responsible for a major functional handicap. In this context, arthroplasty constitutes a therapeutic alternative. Through this study, we aimed to determine the prevalence of the prosthetic surgery and its benefits in children with JIA. Methods This is a retrospective study of JIA patients. The diagnosis of JIA was selected according to ILAR criteria. All the patients included were followed for a minimum of 1 year. The parameters collected were: pain VAS, HAQ, Lequesnealgofunctional index (AFI), disease activity scores and mobility of the operated joint before and 6 months after surgery. Results Forty -four JIA patients were followed over an average period of 14 years (minimum of 1 year). The mean age of disease onset was 9 years [1.5–16 years]. Fifteen cases of coxitis (34%) and 4 cases of destruction of the knees (9%) were noted after an average course of the disease of 8 years [6 months–19 years]. Radiologically, coxofemoral ankylosis is reported in 6 cases, acetabular protrusion in 2 others and total destruction of the knee with misalignment in 2 cases. Eight patients required hip replacement surgery and 2 knee cases. In all cases, the hip prostheses were bilateral. Only one knee prosthesis was placed. The JIA subtype was rheumatoid factor seronegative polyarthritis in 5 cases and enthesitis related arthritis in 3 cases. Six months after surgery, the average AFI dropped from 15.4–4.5; the pain VAS from 73 mm to 50 mm and the HAQ from 2.5–1.06. In the polyarticular JIA, the DAS28 composite score decreased from 4.02–3.8 and in ERA patients the BASDAI decreased from 6.5–4.25 while BASFI remained stable. Recovery of joint mobility was partial in only one case. No immediate or late postoperative complications were noted. Conclusion Prosthetic surgery provided improvement in disease activity and recovery of gait pattern in children with severe JIA. However, the decision of an arthroplasty remains delicate in children, especially since this intervention can compromise the stature growth.

Acetabular Protrusion in a Cohort of Patients with Osteogenesis Imperfecta Evaluated in a Pediatric Hospital

Acetabular protrusion (AP) is present in 33 to 55% of patients with osteogenesis imperfecta (OI). Even though the finding is relatively common, it is poorly described in pediatric patients. The objective of this study was to describe the incidence and associations of AP in pediatric OI patients. We retrospectively and cross-sectionally evaluated clinical histories and radiographic findings of OI patients aged 2 to 19.5 years, recording sex, age, severity, anthropometric measurements, ambulation status, femoral fractures history, and occurrence of orthopaedic surgeries and nephropathy. AP was considered present when the center-edge (CE) angle was more than 35 degrees and the acetabular line crossed the Kohler's line by more than 1 and 3 mm in boys and girls, respectively, and 3 and 6 mm in adult males and females, respectively. The association with risk factors and complications was analyzed through univariate and multivariate logistic regression. A total of 71 children were evaluated. The median age was 8.6 years, and 54.9% of them had moderate to severe forms of OI. In 71.8% of the children, an abnormal CE angle was found, being frequent in mild, moderate, and severe cases. AP was present in 22.5% of all patients and in 41% of children with moderate to severe OI, and was significantly associated with older ages (p = 0.0062) and nonwalking status (p = 0.0093). We found a high prevalence of AP in children with moderate to severe forms of OI, which was present even at younger ages. In addition, we found a significant increase in the number of children with abnormal CE angles even in those with mild forms of OI. The presence of AP was associated with the severity of the OI and age, and in a negative association with the ambulatory status.

Analysis of morphological parameters in pelvic radiography and hip MRI : a practical reporting recommendation

Although numerous criteria have been proposed to define abnormal hip morphology, mostly used in the diagnosis of femoroacetabular impingement, it is not a practical approach to measure all of these parameters in all cases without clinical suspicion. In this study, our aim was to develop an evaluating and reporting standardization for routine hip examinations to define both hip morphology and impingement. A total of 108 patients with routine hip magnetic resonance imaging (MRI) and antero-posterior pelvic radiograph (PR) were included in this retrospective study. Alpha angle (AA), acetabular depth (AD), acetabular protrusion, acetabular anteversion, collo- diaphyseal angle (CDA), lateral center-edge angle (LCEA) and Tönnis angle (TA) were measured. The differences and associations between these parameters were evaluated according to imaging modality or plane, and sex. Although a significant difference has been found between the axial AA and the coronal AA mean values measured on MRI, there was also a strong correlation. Coronal measurements were significantly higher. AA values measured in PR and coronal MRI were comparable. Males had higher AA in both planes as compared to females. There were no significant differences between CDA values in MRI and PR. There was a significant difference and a moderate correlation between AD values in MRI and PR. We suggest that routine reports should include a measurement of AA in two planes, and measurement of CDA in PR or MRI. Due to the difference in AD between MRI and radiography, LCEA or TA may represent better alternatives. Checking for a negative Tönnis sign would represent a practical approach.

Orthopaedic Aspects of Marfan Syndrome: The Experience of a Referral Center for Diagnosis of Rare Diseases

Marfan syndrome is caused by mutations in the fibrillin-1 gene (FBN1). The most important features affect the cardiovascular system, eyes, and skeleton. The aim of this study was to report the most frequent musculoskeletal alterations observed in 146 patients affected by Marfan syndrome. Fifty-four patients (37%) underwent cardiac surgery and 11 of them received emergent surgery for acute aortic dissection. Ectopia lentis was found in 68 patients (47%) whereas myopia above 3D occurred in 46 patients (32%). Musculoskeletal anomalies were observed in all patients with Marfan syndrome. In 88 patients (60.2%), the associated “wrist and thumb sign” was present; in 58 patients (39.7%), pectus carinatum deformity; in 44 patients (30.1%), pectus excavatum; in 49 patients (33.5%), severe flatfoot; in 31 patients (21.2%), hindfoot deformity; in 54 patients (36.9%), reduced US/LS ratio or increased arm span-height ratio; in 37 patients (25.3%), scoliosis or thoracolumbar kyphosis; in 22 patients (15%), reduced elbow extension (170° or less). Acetabular protrusion was ascertained on radiographs in 27 patients (18.4%). Orthopaedic aspects of the disease are very important for an early diagnosis; however, we have not observed definite correlations between the extent of orthopaedic involvement and aortic complications.