The Hand in Distal Arthrogryposis.

Area of Science:

• Orthopedics
• Genetics
• Pediatric Rehabilitation

Background:

• Distal arthrogryposis (DA) is the second most common arthrogryposis type, primarily affecting hands and feet.
• It is predominantly inherited in an autosomal-dominant pattern.
• Key hand deformities include camptodactyly, thumb-in-palm, and windblown hand deformities, significantly impacting function.

Purpose of the Study:

• To provide an updated review of distal arthrogryposis, focusing on hand deformities.
• To discuss current clinical features and treatment strategies.
• To highlight the need for standardized, evidence-based treatment approaches.

Main Methods:

• Literature review of current research on distal arthrogryposis.
• Analysis of clinical features and common hand deformities.
• Synthesis of treatment principles, including nonoperative and operative interventions.

Main Results:

• Treatment should be individualized and multidisciplinary.
• Nonoperative management is often the initial approach.
• Operative treatment focuses on soft-tissue release, muscle rebalancing, and potential bony correction.

Conclusions:

• Early treatment of distal arthrogryposis hand deformities is associated with better outcomes.
• A lack of consensus and scarce reported cases necessitate further long-term, multicenter studies.
• Standardization of treatment protocols is needed to optimize functional results for patients with distal arthrogryposis.