Mr imaging findings of haemophilic arthropathy of the elbow in children

Abstract

Haemophilic arthropathy (HA) of the elbow is an uncommon cause of elbow pain and swelling in children and adolescents.HA due to recurrent haemarthrosis is the most common musculoskeletal manifestation of haemophilia and one of the most disabling complications of this disease. Children with severe haemophilia suffer from recurrent and acute joint haemorraghe. The therapy consists of factor VIII or IX replacement infusions (treatment on demand). The aim of this therapy is to keep the deficient factor > 1% of its normal value to convert severe haemophilia into a milder form of the disorder. The importance of starting replacement therapy early has been stressed in recent literature (1-3).The aim of this study is to define the MR imaging features of HA arthropathy of the elbow at initial MR imaging. Materials and methodsA retrospective study was performed in a pediatric tertiary care centre. Institutional ethics approval was obtained.Subjects with documented HA of the elbow were identified using a radiology information system database keyword search of final radiology reports over a 10 year period. Patients with haemophilia presenting with elbow lesions in the setting of acute musculoskeletal trauma were excluded (Fig. 1-5). If the subject had undergone more than one MRI examination, only the first set of MRI images was reviewed. Twelve the first age of life and received factor substitution therapy since.The images were reviewed by two pediatric radiologists. Both reviewing radiologists were not blinded to the original reported findings but used the report only as a means to identify patients for inclusion into the study.The presence of joint effusion and synovial hypertrophy was assessed. Synovial haemosiderin deposition was considered present if blooming artefact was evident on gradient patients were identified meeting inclusion criteria for the study. The mean age was 12.2 years (range 4.1-16.6 years). All patients had been diagnosed with haemophilia within JBR-BTR, 2010, 93: 247-251. Haemophilic arthropathy of the elbow is a rare cause of elbow pain in children and adolescents. The purpose of this study is to determine the MR appearance of the spectrum of lesions found in haemophilic arthropathy of the elbow at initial MR imaging. It is important to be aware of the early changes in this entity, since early diagnosis and treatment of the disease may prevent progressive joint destruction. MR IMAGING FINDINGS OF HAEMOPHILIC ARTHROPATHY OF THE ELBOW IN CHILDREN

A systematic review of MR imaging as a tool for evaluating haemophilic arthropathy in children

“…An additional six articles were identified from bibliographical references. Thus, a total of 32 studies were included in our systematic review (Fig. ).…”

“…Using the QUADAS‐2 criteria, 10 studies were judged to be of adequate quality and 22 studies of inadequate quality . Figure S2 illustrates the distribution of ratings according to the items of the tool.…”

“…Using the QUADAS-2 criteria, 10 studies were judged to be of adequate quality [9,10,22,29,[32][33][34]39,45,52] and 22 studies of inadequate quality [5,7,8,12,28,30,31,[35][36][37][38][40][41][42][43][44][46][47][48][49][50][51]. Figure S2 illustrates the distribution of ratings according to the items of the tool.…”

“…Using the STARD tool, 14 studies (14/32, 43.8%) were judged to be of fair quality [8][9][10]12,22,29,30,37,38,41,45,46,48,51], 11 studies (34.4%) of poor quality [5,7,31,35,40,[42][43][44]47,49,50] and seven studies (21.9%) of excellent quality [28,[32][33][34]36,39,52]. Figure S1 illustrates the distribution of ratings according to the various items.…”

Pediatric Imaging of the Elbow: A Pictorial Review

“…Involvement of the elbow is the most common musculoskeletal manifestation of hemophilia and one of its most disabling complications. 22 It is important to be aware of the changes in hemophilic arthropathy. Early diagnosis and treatment of the disease may prevent development of late-stage disease with progressive joint destruction and ultimately arthroplasty of the elbow.…”