Journal Article

Combined clinic and home-based therapeutic approach for the treatment of bilateral radial deficiency for a young child with Holt-Oram syndrome: A case report

Ragni, Lori B., Kim, Grace, Zlotolow, Dan A., Daluiski, Aaron

2022

Overview

•This case highlights how a combined clinic and home-based therapy approach can allow for long term management of Holt-Oram syndrome.•Caregiver education and understanding of a home exercise program can improve compliance.•This case demonstrates early and consistent orthosis wear can improve passive range of motion.•Modified constraint induced movement therapy may improve hand and digit function pre and postpollicization.•Comprehensive, long-term assessment may be needed to evaluate improvement following surgical procedures for Holt-Oram syndrome. Holt-Oram syndrome (HOS) is a rare, genetic condition characterized by the combination of congenital heart defect and hypoplasia in one or both upper extremities. Children with HOS commonly present with varied joint and limb involvement including radial longitudinal deficiency impacting hand function. Evidence-based guidelines regarding orthotic wear and therapeutic techniques are lacking. The aim of this case report was to present the results of a long-term occupational therapy program for a patient with HOS pre and postpollicization. Case report. A 4-month-old patient with bilateral radial longitudinal deficiencies began outpatient occupational therapy for custom orthosis fabrication and treatment which included long term clinic and home-based intervention. Techniques included passive range of motion, orthosis wear, therapeutic taping, and modified constraint induced movement therapy. Longitudinal assessment of musculoskeletal alignment and functional hand use was performed using goniometry for passive and active range of motion, the Assisting Hand Assessment (AHA), and The Thumb Grasp and Pinch Assessment (T-GAP). Improvement in passive and active range of motion was achieved as well as improved activity level function as measured by the AHA and T-GAP postpollicization and intervention. A combined clinic and home-based therapeutic approach can be effective for children with HOS to improve alignment and function pre and postpollicization to further enhance hand function. Comprehensive, long-term assessment is necessary to fully evaluate and communicate improvement.

• Publisher: Elsevier Inc,Elsevier Limited
• Subject: Abnormalities, Multiple / Age / Alignment / Caregivers / Case reports / Child / Children / Children & youth / Classification / Congenital / Extremities / Fabrication / Fingers & toes / Heart Defects, Congenital - genetics / Heart Septal Defects, Atrial - genetics / Holt-Oram syndrome / Humans / Hypoplasia / Infant / Intervention / Movement / Occupational therapy / Orthopedics / Orthoses / Orthosis / Pediatrics / Protocol / Radial longitudinal deficiency / Range of motion / Therapy / Upper Extremity Deformities, Congenital - therapy / Wear