- •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.
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.
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JHT Read for Credit
Quiz: # 918
Record your answers on the Return Answer Form found on the tear-out coupon at the back of this issue or to complete online and use a credit card, go to JHTReadforCredit.com. There is only one best answer for each question.
- # 1.The study design is
- b.case series
- c.case report
- d.prospective cohort
- # 2.Children with HOS often present with
- a.radial longitudinal deficiency
- b.rheumatic fever
- c.ulnar hypoplasia
- d.ulnar nerve deficiency
- # 3.Therapy included
- a.therapeutic taping
- b.constrained induced movement therapy
- c.orthotic use
- d.all of the above
- # 4.Orthotic intervention was begun at
- a.6 weeks old
- b.12 months old
- c.4 months old
- d.6 months old
- # 5.The patient obtained improved alignment and function from a home program and clinical sessions
When submitting to the HTCC for re-certification, please batch your JHT RFC certificates in groups of 3 or more to get full credit.
Published online: March 05, 2021
Accepted: March 1, 2021
Received in revised form: December 17, 2020
Received: June 11, 2020
© 2021 Elsevier Inc. All rights reserved.