Staged reconstruction of radial longitudinal deficiency and thumb aplasia – case report and a review of the literature

Purpose: Presentation of surgical treatment of a girl with type IV radial longitudinal deficiency and type IV thumb hypoplasia as well as review of the most relevant literature published in the previous 25 years. Methods: The treatment consisted of placement of an external fixator and subsequent distraction in the radioulnar and proximodistal axis as the initial procedure followed by radialization according to Buck-Gramcko around the 2nd birthday, 10 weeks after the primary procedure. The last step of the treatment was a pollicization of the index finger performed before the 4th birthday. Results: The surgical treatment resulted with a very favorable outcome – a clinically stable wrist, radiologically well aligned carpus over the ulna and an index pollicised in such a manner that enables adequate opposition to the other fingers. Conclusion: A review of literature in the last 25 years shows that radialization of the ulna with a preceding distraction offers benefits over centralization alone. Distraction of the wrist protects the distal ulnar growth plate and thus promotes growth. Soft tissue release with a bilobar flap can also provide a good outcome as wells as a microvascular joint transfer from the 2nd toe whereas the latter requires advanced microsurgical skills.