Category: Clinical Sciences/Health Conditions
Congenital upper limb deficiency results from failure of formation or abnormal shaping of the upper limb during gestation. Children with congenital upper limb deficiency adapt to achieve functional milestones, with or without surgical or non-surgical intervention. All interventions at different stages of life are guided towards the optimum functional gain. There is no study available showing rehabilitation approach in ulnar deficiency involving both upper limbs presented at adulthood. This case report documents functional improvement in a 15-year-old male presented with a bilateral ulnar longitudinal deficiency with bilateral elbow flexion deformity managed with Joshi’s External Stabilization System (JESS) with a patient-centered rehabilitation approach.
Patient-centered goals were established, targeting activities of daily living (ADL), by using the Goal Attainment Scale (GAS) with the involvement of the patient and family. JESS application was performed for the correction of elbow flexion deformity in a stage-wise manner. Elbow range was improved by sequential and gradual distraction to improve reach out of upper extremity. The reconditioning and therapeutic exercises were implemented with subsequent ADL training. The outcome measurement was done using the GAS calculation sheet (https://www.kcl.ac.uk/cicelysaunders/resources/tools/gas).
Active elbow range was improved by 200 on left side 250 on the right side. Satisfactory improvement was achieved in activities of daily living. The targeted activities were activities of self-care (bathing, toileting, lower body dressing, eating) and instrumented activities of daily living (writing, routine clean up tasks, use of the mobile phone). The baseline and outcome GAS scores were 36.4 and 53.4 respectively. The change in GAS of 17.0 confirms better than expected result.
Patients presenting at different stages of life with congenital limb deficiency should be treated with patient-centred goal approach. JESS can be used effectively to achieve these goals and rehabilitation.
Ameya Joshi– Senior Resident, All India Institute of Physical Medicine & Rehabilitation, Mumbai
Anil Gaur– Director, All India Institute of Physical Medicine and Rehabilitation, Mumbai.
Amit Mhambre– Head of the Department PMR, All India Institute of Physical Medicine and Rehabilitation, Mumbai.