Development and validation of a WHO ICF compliant hand and upper limb assessment tool for children: The Reach Out questionnaire.

BACKGROUND: Current tools for evaluating hand and upper limb function in children do not represent all domains of the World Health Organization International Classification of Disability, Functioning and Health (ICF) framework and may not capture an accurate progression or regression of function over time. PURPOSE: Based on this framework, we have developed an assessment tool (Reach Out) to evaluate function in children aged from 2 to 16 years following consultation with an advisory panel of specialists. STUDY DESIGN: Primary clinical study. METHODS: Construct validity along with test-retest reliability, inter-rater reliability and sensitivity to change have all been analyzed to validate the Reach Out assessment tool. RESULTS: The assessment tool has been validated in a total of 231 patients. Significant construct validity of 0.64 (P < .00001, 95% confidence interval = 0.56-0.71, n = 231) for both age groups and diagnostic groups was observed. The Reach Out questionnaire was internally consistent with a Cronbach's Alpha of > 0.8 for most domains in most age groups. Test re-retest scores showed that the questionnaire was reliable with most domains of the questionnaire achieving high scores of reliability (P ≤ .03). We also received positive feedback from participants and parents. CONCLUSIONS: The use of this new tool will help identify both progression and regression of function, allowing a more tailored and holistic approach to treatment in children with conditions affecting the hand and upper limb through the incorporation of International Classification of Disability, Functioning and Health domains. This tool is quicker to complete and can be applied to a wide range of ages and diagnostic groups compared to previous assessment tools.

Subjective and Objective Assessment of Hand Function in Mucopolysaccharidosis IVa Patients.

INTRODUCTION: Morquio A is classically described as a skeletal dysplasia, but the impact of the abnormalities on the upper limb has not been described. AIM: The aim of our study is to assess the level of subjective and objective functions of the hand and upper limb in Morquio A patients in order to advance understanding as this may change future management for this complex group of patients. METHOD: We have evaluated a series of ten patients with Morquio syndrome who are currently part of the enzyme replacement therapy trials in our unit. To establish functional levels, we developed a questionnaire based on the principles of the World Health Organization International Classification of Functioning, Disability and Health (WHO ICF). Furthermore, we measured patients' grip function and range of motion. RESULTS: All patients showed hypermobility and limited control of the wrist. Their grip strength was reduced and they showed higher difficulty levels for activities involving strength. It is feasible that the wrist abnormalities may be the major cause of functional loss for Morquio patients. CONCLUSION: Our findings suggest the wrist itself represents an area that we should focus on to be able to improve the patients' functional abilities and hence their level of independence on day-to-day basis.

Early tendon transfer after wide resection of an undifferentiated sarcoma of the forearm in a neonate: a case report.

A 1-month-old female baby was diagnosed with an undifferentiated sarcoma of her forearm after incisional biopsy. She received five cycles of chemotherapy before a wide resection of her extensor muscles including the radial nerve was undertaken, resulting in a complete drop of wrist and fingers. At this stage, primary tendon transfer of the brachioradialis to extensor carpi radial brevis muscle was performed, which proved to be not sufficient. Five months after resection of the tumour, restoration of the extensor function was achieved by flexor tendon transfers to provide wrist, finger and thumb extension. After 2 years, the patient did not show any signs of recurrence and she has a good wrist, finger and thumb extension and flexion. The diagnosis of an undifferentiated sarcoma is one per exclusion. Treatment includes chemotherapy and complete resection of the tumour with a wide margin. We discuss the diagnosis of undifferentiated sarcoma as well as early restoration of hand function in neonates.