Congenital upper limb anomalies : studies of epidemiology and hand function

Objectives: This thesis has three interrelated aims: (1) To describe the epidemiology of congenital upper limb anomalies (CULA) in Stockholm County, Sweden, in order to augment the few existing population studies of CULA (paper I); (2) To measure the incidence of different categories of CULA while using and evaluating a recently proposed new classification scheme (Oberg, Manske and Tonkin (OMT) Classification) based on more current knowledge of limb development than the previously used International Federation of Societies for Surgery of the Hand (IFSSH) Classification is based on (paper II); and (3) To investigate the relationship between measurements of body function and structure with both activity and participation in children and adults with radial longitudinal deficiency (RLD) by using the International Classification of Functioning and Health (ICF) framework, in order to shed light on what aspects of physical limb function and structure actually affect individuals’ daily life activity (papers III and IV). Methods: 562 children born with a CULA were identified through registry studies. Incidence and relative frequency of different types of anomalies were calculated. Distribution of gender, affected side, associated non-hand anomalies and occurrence among relatives were investigated (paper I and II). In twenty children (paper III) and 20 adults (paper IV) with RLD, Body function and structure was evaluated by measures of range of motion, grip strength, key pinch, sensibility and radiographic parameters. Activity was evaluated by Box and Blocks test, Assisting Hand Assessment (AHA) and Sollerman Hand Function test and participation by Children Hand-use Experience Questionnaire (CHEQ), Quick-DASH and SF-12. Statistical correlations between assessments of body function and structure, activity and participation were examined. Results: The incidence of CULA in Stockholm, Sweden, 1997 to 2007, was 21.5 per 10,000 live births (paper I). All CULA could be classified using the OMT classification. The largest main category was Malformations (429 cases), followed by Deformations (124 cases), Dysplasias (10 cases) and Syndromes (14 cases) (paper II). In children with RLD (paper III), significant relationships were found between measurements of activity and range of motion of digits as well as between measurements of participation and range of motion of wrist. In adults with RLD (paper IV), significant relationships were found between measurements of activity and grip strength, key pinch and range of motion of elbow and digits. In adults, measurements of participation showed significant relationships with grip strength, forearm length and range of motion of elbow and digits. However, radiographic measurements of radial wrist deviation did not show a significant relationship with measurements of activity or participation in children or in adults with radial longitudinal deficiency. Conclusions: The incidence of CULA in one Swedish region confirms the findings in the only previous comparable total population study. The OMT classification proved useful and accurate and with further refinements can replace the IFSSH classification. In children and adults with RLD, grip strength, key pinch, forearm length and elbow and digital motion seem to be more important for the individual´s levels of activity and participation than the radial angulation of the wrist. The current treatment principle of surgical correction of the angulated wrist could therefore be questioned

Hand function in children with radial longitudinal deficiency

BACKGROUND: In children with hypoplasia or aplasia of the radius (radial longitudinal deficiency) manual activity limitations may be caused by several factors; a short and bowed forearm, radial deviation of the wrist, a non-functional or absent thumb, limited range of motion in the fingers and impaired grip strength. The present study investigates the relation between these variables and activity and participation in children with radial dysplasia. METHODS: Twenty children, age 4-17 years, with radial longitudinal dysplasia Bayne type II-IV were examined with focus on the International Classification of Functioning and Health, version for Children and Youth (ICF-CY) context. Body function/structure was evaluated by measures of range of motion, grip strength, sensibility and radiographic parameters. Activity was examined by Box and Block Test and Assisting Hand Assessment (AHA). Participation was assessed by Children's Hand-use Experience Questionnaire (CHEQ). Statistical correlations between assessments of body function/structure and activity as well as participation were examined. RESULTS: The mean total active motion of wrist (49.6°) and digits (447°) were less than norms. The mean hand forearm angle was 34° radially. Ulnar length ranged from 40 to 80% of age-related norms. Grip strength (mean 2.7 kg) and Box and Block Test (mean 33.8 blocks/minute) were considerably lower than for age-related norms. The mean score for the AHA was 55.9 and for CHEQ Grasp efficiency 69.3. The AHA had significant relationship with the total range of motion of digits (p = 0.042). Self-experienced time of performance (CHEQ Time) had significant relationship with total active motion of wrist (p = 0.043). Hand forearm angle did not show any significant relationship with Box and Block Test, AHA or CHEQ. CONCLUSION: In radial longitudinal deficiency total range of motion of digits and wrist may be of more cardinal importance to the child's activity and participation than the angulation of the wrist

Hand Function in Adults with Radial Longitudinal Deficiency.

Functional impairment in individuals with radial longitudinal deficiency can be influenced by several factors, including a short and bowed forearm, radial deviation of the wrist, a non-functional or absent thumb, limited finger motion, and impaired grip strength, but their relationship with activity and participation in adults with radial deficiency is not known.METHODS: Twenty individuals, eighteen to sixty years of age, who had Bayne type-II to V radial longitudinal deficiency, were examined in the context of the International Classification of Functioning, Disability and Health. Body function and structure were evaluated by measures of range of motion, grip strength, key pinch, sensibility, and radiographic parameters. Activity was evaluated by the Box and Block Test and the Sollerman test, and participation was evaluated by QuickDASH (the short form of the Disabilities of Arm, Shoulder and Hand outcome measure) and by the Medical Outcomes Study 12-Item Short Form Health Survey. Statistical correlations among assessments of body function and structure, activity, and participation were examined.RESULTS: The mean total active motion of the wrist (43°) and mean total active motion of the digits (377°) were less than the norms. The mean radial deviation of the wrist was 31°. The mean grip strength (4.0 kg), key pinch (1.4 kg), and scores for the Box and Block Test (55 blocks per minute) and the Sollerman test on hand function (56 points) were considerably lower than the norms. The mean scores were 18 points for QuickDASH, 51 points for Short Form-12 physical component summary, and 53 points for Short Form-12 mental component summary. Significant relationships were found between the Box and Block Test and grip strength (p = 0.012), key pinch (p < 0.001), and total active motion of digits (p < 0.001); between the Sollerman test and the total active motion of elbow (p < 0.001) and the total active motion of digits (p < 0.001); between the QuickDASH and forearm length (p < 0.001), the total active motion of elbow (p = 0.001), and the total active motion of digits (p < 0.001); between the Short Form-12 physical component summary and grip strength (p = 0.016), forearm length (p < 0.001), total active elbow motion (p < 0.001), and total active digit motion (p < 0.001); and between the Short-Form-12 mental component summary and radial deviation of the wrist (p = 0.019). No significant correlations were found between the radiographic measurement of the radial deviation of the wrist (total forearm angle) and the Box and Block Test (p = 0.244), the Sollerman test (p = 0.775), QuickDASH (p = 0.156), Short Form-12 physical component summary (p = 0.107), or Short Form-12 mental component summary (p = 0.129).CONCLUSIONS: In individuals with radial longitudinal deficiency, grip strength, key pinch, forearm length, and elbow and digital motion seem to be more important for the individual's activity and participation than the radial angulation of the wrist.LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence

Hand function in children with radial longitudinal deficiency

BACKGROUND: In children with hypoplasia or aplasia of the radius (radial longitudinal deficiency) manual activity limitations may be caused by several factors; a short and bowed forearm, radial deviation of the wrist, a non-functional or absent thumb, limited range of motion in the fingers and impaired grip strength. The present study investigates the relation between these variables and activity and participation in children with radial dysplasia. METHODS: Twenty children, age 4-17 years, with radial longitudinal dysplasia Bayne type II-IV were examined with focus on the International Classification of Functioning and Health, version for Children and Youth (ICF-CY) context. Body function/structure was evaluated by measures of range of motion, grip strength, sensibility and radiographic parameters. Activity was examined by Box and Block Test and Assisting Hand Assessment (AHA). Participation was assessed by Children's Hand-use Experience Questionnaire (CHEQ). Statistical correlations between assessments of body function/structure and activity as well as participation were examined. RESULTS: The mean total active motion of wrist (49.6°) and digits (447°) were less than norms. The mean hand forearm angle was 34° radially. Ulnar length ranged from 40 to 80% of age-related norms. Grip strength (mean 2.7 kg) and Box and Block Test (mean 33.8 blocks/minute) were considerably lower than for age-related norms. The mean score for the AHA was 55.9 and for CHEQ Grasp efficiency 69.3. The AHA had significant relationship with the total range of motion of digits (p = 0.042). Self-experienced time of performance (CHEQ Time) had significant relationship with total active motion of wrist (p = 0.043). Hand forearm angle did not show any significant relationship with Box and Block Test, AHA or CHEQ. CONCLUSION: In radial longitudinal deficiency total range of motion of digits and wrist may be of more cardinal importance to the child's activity and participation than the angulation of the wrist