Radial Club Hand, a Rare Congenital Abnormality: Report of Two Cases_ Crimson Publishers

Radial Club Hand, a Rare Congenital Abnormality: Report of Two Cases by Ashima Mahajan Crimson Publishers in Orthoplastic Surgery & Orthopedic Care International Journal_ Orthopedic Care International Journal

Abstract
Congenital radial deficiency is an extremely rare congenital anomaly and its presentation may vary from mild hypoplasia to complete absence of radius. Club hand deformities are classified into two main categories radial and ulnar. Ulnar club hand is much less frequent than radial club hand. It is also known as radial club hand or radial dysplasia. We report two cases of rare congenital abnormality which can be helpful to the literature.

Introduction
Congenital radial deficiency also known as radial club hand is a relatively rare congenital anomaly. Patient may present with variable degree of deficiency along the radial (or preaxial) side of the limb and is usually associated with absent thumb, thumb hypoplasia, thin first metacarpal and absent radius [1]. Ulnar club hand is much less frequent than radial club hand and ranges from mild deviation of hand on the ulnar side of forearm to complete absence of ulna. The estimated incidence of CRD is found to be 1 in 30,000 to 1 in 100,000 live births and most of the cases occurs sporadically with no known cause or associated with the syndrome.
Case 1

A 4-year-old male child was brought to the OPD with complaints of cough and fever for 2 days. There was a history of a deformed right upper limb since birth. He was a product of a non-consanguineous marriage and his perinatal history was uneventful (Figure 1). There was no family history of a similar deformity in the past two generations and his developmental history was normal for his age. There was no history of blood transfusion. The physical examination of the child revealed a shortened right forearm which was atrophied as compared to the opposite normal limb and he had a single forearm bone. There was limitation of movements of the right elbow flexion and extension including the rotatory movements of the forearm and the wrist. The systemic examination was normal. Evaluation of the radiographs of both the upper limbs revealed complete absence of the right radius and right first metacarpal bone. Chest X-ray, echocardiogram, hemogram including the platelet counts and ultrasound of the abdomen were normal.

Figure 1: X-ray right forearm with hand revealed complete absence of radius and thumb (type IV congenital radial deficiency).

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