Johannes Roth1, Michal Inbar-Feigenberg2, Julian Raiman3, Marg Bisch4, Pranesh Chakraborty5, John Mitchell6, Luca Di Geso7. 1. Division of Pediatric Dermatology and Rheumatology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. Electronic address: jroth@cheo.on.ca. 2. Division of Clinical and Metabolic Genetics, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 3. Department of Inherited Metabolic Disease, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, UK. 4. Division of Pediatric Dermatology and Rheumatology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. 5. Newborn Screening Ontario and Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. 6. Department of Pediatrics, McGill University, Montreal, Quebec, Canada. 7. Ospedale Provinciale Madonna del Soccorso, Department of Internal Medicine, San Benedetto del Tronto, Marche, Italy.
Abstract
INTRODUCTION: Musculoskeletal findings in MPS can progress after enzyme replacement. Our aim was to examine synovial recesses, tendons, retinacula and pulleys using ultrasonography for structural and inflammatory changes. MATERIAL AND METHODS: The wrist, metacarpophalangeal (MCP), proximal and distal interphalangeal (PIP and DIP) joints, the finger flexor tendons and the knee including entheses of quadriceps and patella tendons were assessed clinically. Ultrasonography of the various synovial recesses of the wrist as well as the extensor retinaculum, carpal tunnel, MCP, PIP and DIP joints of the second finger, extensor and flexor tendons, A1-5 pulleys and the knee joint including relevant entheses followed. Significance of differences between patient values and available normative data were assessed using t-tests. RESULTS: Ultrasonography showed significant abnormal intraarticular material in the wrist without a clear distribution to synovial recesses and without effusions. Doppler signals were found in a perisynovial distribution and not intrasynovial as expected in in inflammatory arthritis. Findings were similar in the knee but not the fingers. Flexor and extensor tendons were also mostly normal in their structure but significant thickening of retinaculae and the flexor tendon pulleys was seen (p<0.0001 compared to normal). CONCLUSION: MPS I patients showed intraarticular deposition of abnormal material in the wrist and knee but not in the finger joints where significant thickening of retinaculae/pulleys controlling tendon position was dominant. No ultrasound findings of inflammatory pathology were demonstrated but rather a secondary reaction to abnormal deposition and direct damage of GAG.
INTRODUCTION: Musculoskeletal findings in MPS can progress after enzyme replacement. Our aim was to examine synovial recesses, tendons, retinacula and pulleys using ultrasonography for structural and inflammatory changes. MATERIAL AND METHODS: The wrist, metacarpophalangeal (MCP), proximal and distal interphalangeal (PIP and DIP) joints, the finger flexor tendons and the knee including entheses of quadriceps and patella tendons were assessed clinically. Ultrasonography of the various synovial recesses of the wrist as well as the extensor retinaculum, carpal tunnel, MCP, PIP and DIP joints of the second finger, extensor and flexor tendons, A1-5 pulleys and the knee joint including relevant entheses followed. Significance of differences between patient values and available normative data were assessed using t-tests. RESULTS: Ultrasonography showed significant abnormal intraarticular material in the wrist without a clear distribution to synovial recesses and without effusions. Doppler signals were found in a perisynovial distribution and not intrasynovial as expected in in inflammatory arthritis. Findings were similar in the knee but not the fingers. Flexor and extensor tendons were also mostly normal in their structure but significant thickening of retinaculae and the flexor tendon pulleys was seen (p<0.0001 compared to normal). CONCLUSION: MPS I patients showed intraarticular deposition of abnormal material in the wrist and knee but not in the finger joints where significant thickening of retinaculae/pulleys controlling tendon position was dominant. No ultrasound findings of inflammatory pathology were demonstrated but rather a secondary reaction to abnormal deposition and direct damage of GAG.
Authors: Yian Khai Lau; Sun H Peck; Toren Arginteanu; Meilun Wu; Megan Lin; Eileen M Shore; Peter S Klein; Margret L Casal; Lachlan J Smith Journal: Bone Date: 2021-10-22 Impact factor: 4.398