Bone · 2026
Practical guidance for clinicians to optimise orthopaedic outcomes in achondroplasia: International Achondroplasia Forum guiding principles.
СтатьяНазвание и аннотация: Перевод в очереди
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Kunkel Philip, Vleggeert-Lankamp Carmen, Riganti Simone, Leiva-Gea Antonio, Boero Silvio, Fredwall Svein, Mohnike Klaus, Mortier Geert, Okada Keita, Sessa Marco, Irving Melita, International Achondroplasia Forum
Аннотация
BACKGROUND: Achondroplasia is associated with multisystem impairments that may require surgical interventions associated with high complication rates. The orthopaedic treatment landscape in achondroplasia varies globally, with some countries recommending surgical intervention more commonly. Achondroplasia-specific concerns related to preparation and conduct of surgical procedures necessitate careful consideration of treatment options in consultation with a multidisciplinary care team. The International Achondroplasia Forum convened a workshop in October 2024 to develop guiding principles for orthopaedic outcomes in achondroplasia, specifically spinal deformity, symptomatic spinal stenosis (and related neurological sequelae), upper and lower limb shortening/deformity (and associated functional limitations), chronic pain, and health-related quality of life, alongside achondroplasia-specific perioperative risk mitigation and post-surgical rehabilitation.
RESULTS: Six guiding principles were agreed at the workshop with the aim of standardising and optimising orthopaedic care for people with achondroplasia. All principles were approved (votes in favour: 93%-100%) with a high level of agreement (range: 8.43-9.33). The International Achondroplasia Forum principles highlight the importance of a multidisciplinary team to guide treatment decisions and the need for essential procedures to be conducted at specialised institutions by clinicians experienced in the treatment of achondroplasia. Approaches to orthopaedic management include educating parents and caregivers; brace therapy, if necessary, to prevent or slow the development of thoraco-lumbar kyphosis; and weight management to minimise pain, dysfunction, and risk of complications. Pharmacological treatment is currently limited to vosoritide.
CONCLUSIONS: These guiding principles aim to improve outcomes for people with achondroplasia by reducing treatment heterogeneity and providing recommendations to caregivers. Consultation with an experienced and specialised multidisciplinary team, emphasis on non-surgical approaches to address orthopaedic symptoms and the conduct of essential surgical procedures only in specialised centres form the core of the guiding principles.
RESULTS: Six guiding principles were agreed at the workshop with the aim of standardising and optimising orthopaedic care for people with achondroplasia. All principles were approved (votes in favour: 93%-100%) with a high level of agreement (range: 8.43-9.33). The International Achondroplasia Forum principles highlight the importance of a multidisciplinary team to guide treatment decisions and the need for essential procedures to be conducted at specialised institutions by clinicians experienced in the treatment of achondroplasia. Approaches to orthopaedic management include educating parents and caregivers; brace therapy, if necessary, to prevent or slow the development of thoraco-lumbar kyphosis; and weight management to minimise pain, dysfunction, and risk of complications. Pharmacological treatment is currently limited to vosoritide.
CONCLUSIONS: These guiding principles aim to improve outcomes for people with achondroplasia by reducing treatment heterogeneity and providing recommendations to caregivers. Consultation with an experienced and specialised multidisciplinary team, emphasis on non-surgical approaches to address orthopaedic symptoms and the conduct of essential surgical procedures only in specialised centres form the core of the guiding principles.