The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg Am

Shriners Hospitals for Children, Tampa, Florida, United States
The Journal Of Hand Surgery (Impact Factor: 1.67). 12/1999; 24(6):1145-55. DOI: 10.1053/jhsu.1999.1145
Source: PubMed


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.

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    • "The inclusion criteria were children age 4–17 years and unilateral or bilateral RLD Bayne type II-IV [6,20] (Table 1). Thirty-one children in the medical registers fulfilled the inclusion criteria. "
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    ABSTRACT: 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. 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. 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. 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.
    Full-text · Article · Mar 2013 · BMC Musculoskeletal Disorders
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    • "Cases with a hypoplastic (or "miniature") radius are considered type II, partial absence of the radius constitutes type III, and type IV represents cases where there is a complete absence of the radius. James et al.5) has since modified the classification scheme, adding type N and 0 to describe isolated thumb and carpal anomalies in cases with a distal radius of normal length. Type III and IV RLD are considered to be the most common forms; these cases also tend to be associated with the greatest amount of radial deviation of the wrist.6) "
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    ABSTRACT: Radial longitudinal deficiency, also known as radial club hand, is a congenital deformity of the upper extremity which can present with a spectrum of upper limb deficiencies. The typical hand and forearm deformity in such cases consists of significant forearm shortening, radial deviation of the wrist and hypoplasia or absence of a thumb. Treatment goals focus on the creation of stable centralized and functionally hand, maintenance of a mobile and stable wrist and preservation of longitudinal forearm growth. Historically centralization procedures have been the most common treatment method for this condition; unfortunately centralization procedures are associated with a high recurrence rate and have the potential for injury to the distal ulnar physis resulting in a further decrease in forearm growth. Here we advocate for the use of a vascularized second metatarsophalangeal joint transfer for stabilization of the carpus and prevention of recurrent radial deformity and subluxation of the wrist. This technique was originally described by the senior author in 1992 and he has subsequently been performed in 24 cases with an average of 11-year follow-up. In this paper we present an overview of the technique and review the expected outcomes for this method of treatment of radial longitudinal deficiency.
    Full-text · Article · Mar 2012 · Clinics in orthopedic surgery

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