RadiAL APLASIA & TAR SYNDROME
Aplasia.ORG
When our daughter was born with TAR Syndrome and Radial Aplasia in 2020, a flurry of questions emerged, and a long quest for answers and treatment options began. After the successful surgical correction of the effects of her Radial Aplasia – by an Ulnarization, performed by Dr. Dror Paley and his team in 2022 – we decided to share the knowledge we acquired, and our experiences.
This website chronicles our journey and reflects our personal opinions. It is 100% non-commercial, funded by us only, to help affected patients & parents, and to raise awareness about the procedure. We did not receive any compensation or discounts, etc. Disclaimer.
Feel free to reach out to us if you have questions not answered here.
Our Story
Our daughter was born with TAR Syndrome and Radial Aplasia in 2020, which came as a massive surprise to us. Despite undergoing pre-natal checks and more than seven ultrasounds, none of the doctors identified her condition.
Following an unremarkable labor process, we immediately noticed the unusual appearance of her hands. Our journey in search of the best treatment options, educating ourselves about her syndrome, and fighting for her to be able to lead a perfectly normal life began.
After consulting with doctors in Germany and the U.S., we arranged a surgery called Ulnarization, pioneered by Dr. Paley. In 2022, she underwent a successful operation performed by Dr. Paley and Dr. Shannon. Now, she lives her life without any restrictions, engaging in activities like playing, jumping, running, and sharing laughter with her sibling, just like any other child.
Radial Aplasia: Pathology
Radial aplasia, also known as radial dysplasia or radial longitudinal deficiency or radial clubhand, is a medical condition in which the radius bone in the forearm is either absent or underdeveloped. This can result in a shortened forearm, a hand deformity, and a limited range of motion in the affected arm. Radial aplasia is diagnosed prenatally or at birth.
Radial aplasia can occur as an isolated abnormality or as part of a larger syndrome, such as Holt-Oram syndrome, Fanconi anemia, or TAR syndrome. The cause of radial aplasia is poorly understood, but genetic factors often play a role.
Treatment Options
Radial Aplasia is a rare medical condition. Deciding how to treat the resulting radial deviation of the hand – or, in some instances, whether to intervene at all – is challenging because the available options are far from ordinary, (almost) always successful procedures.
Custom Orthoses
To enable corrective surgical procedures for hand positioning in cases of radial aplasia, either the “too short” tissues and tendons causing the characteristic bent hand position need to be stretched beforehand, or during the surgical intervention, the surgeon must shorten the ulna. Otherwise, straightening the hand is not feasible.
Irrespective of the planned intervention type, it’s advantageous to maximize the expansion of tissue and tendons to allow passive movement of the hand toward the neutral position. Currently, many surgeons recommend using night positioning splints to perform initial stretching.
A Selection of Relevant Publications
Deszczyński, Jarosław & Albrewczyński, Tomasz & Shannon, Claire & Paley, Dror. (2021). Radial Club Hand Treated by Paley Ulnarization Generation 3: Is This the New Centralization?. Children. 8. 562. 10.3390/children8070562.
(1) Background: Patients treated with the two previous generations of ulnarization developed a bump related to the ulnar head becoming prominent on the radial side of the hand. To finally remedy this problem, a third generation of ulnarization was developed to keep the ulnar head contained. While still ulnar to the wrist center, the center of the wrist remains ulnar to the ulnar head, with the ulnar head articulating directly with the trapezoid and when present the trapezium. (2) Methods: Between 2019 and 2021, 22 radial club hands in 17 patients were surgically corrected with this modified version of ulnarization. (3) Results: In all 17 patients, the mean HFA (hand–forearm-angle) correction was 68.5° (range 12.2°–88.7°). The mean ulna growth was 1.3 cm per year (range 0.2–2 cm). There were no recurrent radial deviation deformities more than 15° of the HFA. (4) Conclusions: This new version of ulnarization may solve the problem of the ulna growing past the carpus creating a prominent ulnar bump. The results presented are preliminary but promising. Longer-term follow-up is needed to fully evaluate this procedure.
Paley, Dror. (2020). Shortening: The orthopedic theory of relativity. Journal of Limb Lengthening and Reconstruction. 6. 1. 10.4103/2455-3719.288573.
Paley, Dror. (2017). The Paley ulnarization of the carpus with ulnar shortening osteotomy for treatment of radial club hand. SICOT-J. 3. 5. 10.1051/sicotj/2016040.
Recurrent deformity from centralization and radialization led to the development in 1999 of a new technique by the author called ulnarization. This method is performed through a volar approach in a vascular and physeal sparing fashion. It biomechanically balances the muscle forces on the wrist by dorsally transferring the flexor carpi ulnaris (FCU) from a deforming to a corrective force. The previous problems of a prominent bump from the ulnar head and ulnar deviation instability were solved by acutely shortening the diaphysis and by temporarily fixing the station of the carpus to the ulnar head at the level of the scaphoid. This is the first report of this modified Paley ulnarization method, which the author considers a significant improvement over his original procedure..
Shannon, Claire & Paley, Dror. (2020). Extramedullary Internal Limb Lengthening. Techniques in Orthopaedics. 35. 195-200. 10.1097/BTO.0000000000000466.
Paley, Dror. (2018). Limb reconstruction in the early 21 st century: The indications are broader and wider. Journal of Limb Lengthening & Reconstruction. 4. 65. 10.4103/2455-3719.253397.