The spectrum of radial longitudinal deficiency: a modified classification.
ABSTRACT The records of 119 patients with 196 extremities with radial longitudinal deficiency seen between 1923 and 1996 were reviewed. We propose a global classification system that includes the spectrum of pathology affecting the radial side of the extremity, including deficiency of the radius, carpal abnormalities, and hypoplastic thumbs. Radial deficiency could be classified for 181 extremities of 104 patients using this classification system. Type N has a normal length radius and a normal carpus with thumb hypoplasia, type O has a normal length radius and radial side carpal abnormalities, type 1 has more than 2 mm shortening of the radius, type 2 has a hypoplastic radius, type 3 has a partial radius with absence of the distal physis, and type 4 has complete absence of the radius. All patients had thumb hypoplasia. Eighty-two percent of extremities with thumb hypoplasia but no deficiency of the radius that were available for carpal bone classification had carpal anomalies, including absence, hypoplasia, and coalitions. All the extremities with type 1 radial deficiency had carpal anomalies. Carpal abnormalities could not be determined for types 2, 3, and 4 deficiency because most had a prior centralization. Proximal radioulnar synostosis or congenital dislocation of the radial head was seen in 44% of extremities with type 1 radial deficiency. This classification includes carpal anomalies and thereby links isolated thumb hypoplasia and deficiency of the radius into one system.
- SourceAvailable from: Anna Gerber Ekblom[show abstract] [hide abstract]
ABSTRACT: 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 (49.6o) and digits (447o) were less than norms. The mean hand forearm angle was 34[degree sign] 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.BMC Musculoskeletal Disorders 03/2013; 14(1):116. · 1.88 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Radial, ulnar, and central deficiencies represent a spectrum of abnormalities in the development of the upper limb. Radial longitudinal deficiency is often associated with abnormalities in other organ systems, such as cardiac and renal, and so requires a comprehensive medical evaluation. On the other hand, ulnar longitudinal deficiency tends to be associated only with other musculoskeletal abnormalities. In all of these conditions, there is a high incidence of ipsilateral thumb abnormalities. Given the importance of the thumb in overall hand function, abnormalities of the thumb often guide treatment for these conditions. Surgical treatment of the wrist and forearm in radial longitudinal deficiency is controversial, as will be outlined in this review.The Journal of hand surgery 05/2013; · 1.33 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: To review the clinical characteristics in a series of 25 patients with VACTERL (vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, cardiac defects, renal and limb anomalies) association who were ascertained for upper limb involvement. The study involved a review of clinical and radiologic data from patients with VACTERL association collected by a hand surgery clinic between 2004 and 2013. Radial axis involvement was found in all 25 patients (100%), with severe thumb function impairment in 79% and complete absence of the radius in roughly 33%. Costovertebral anomalies were the most frequent feature, found in 23 patients (92%). All 3 core features (anal atresia, tracheoesophageal fistula with esophageal atresia, and costovertebral anomalies) were present in only 12% of the patients. Twelve patients (48%) had abnormalities not part of the VACTERL spectrum, showing a specific pattern of non-VACTERL-type malformations, including genitourinary abnormalities (12%), single umbilical artery (8%), and tethered cord (8%). Previously unreported clinical findings were concurrent hypoplasia of both the odontoid process and the coccyx in 2 patients and an isolated sacral dimple in 2 patients. Upper limb involvement in VACTERL association is a specific feature of the radial axis that occurs in monolateral form in approximately 75% of cases and, when bilateral, always occurs in a nonsymmetrical fashion. Odontoid and coccygeal hypoplasia and sacral dimple are newly reported malformations of the VACTERL phenotype.The Journal of pediatrics 11/2013; · 4.02 Impact Factor