Occurrence of radial club hand in children with different syndromes

Abstract

Aim. Clinical analysis of congenital radial club hand as part of some genetic syndromes as well as the optimization of methods of non-surgical and surgical treatment of patients with this pathology. Material and Methods. From 2007 to 2014, we conducted a survey of 170 children with congenital radial club hand. Among them, 32 patients were diagnosed (18.8%) with different syndromes. We assessed the degree to which the radius was underdeveloped among this group of patients as well as the management features of patients according to various comorbidities. Results. The assessment identified Holt-Oram syndrome in 17 children (nine boys and eight girls; 53.1%) and TAR-syndrome in nine children (four boys and five girls; 28.1%). VACTERL syndrome was detected in four male patients (12.5%) and Nagera syndrome was observed in two children (one boy and one girl; 6.25%). Surgical treatment of radial club hand in patients with genetic syndromes is the same as that of the patients with isolated congenital radial club hand: a single- or two-stage correction of the hand relative to the ulna with subsequent reconstruction of the rays of the hand. The duration of treatment of such patients did not significantly differ compared to the patients with isolated congenital radial club hand. Conclusion. Congenital radial club hand, identified as part of genetic syndromes, requires a comprehensive examination to diagnose comorbidities, observation, and treatment by specialists to determine the optimal age for surgical correction of the existing strain of the upper limb.