Every child with radial longitudinal deficiency is unique and therefore is treated individually. While there are general principles in treatment and these principles may be applied to most children, we begin care by working to understand each child and each family.
In a child with radial longitudinal deficiency presenting for evaluation, we consider many factors as we plan treatment. First, the family’s feeling about the anomaly and their desire for intervention is considered. Each family comes from a different place and has different expectations, goals, and desires. In some, there is a family history which will affect the understanding and perhaps provide the most realistic understanding as those families really know how radial longitudinal deficiency affects daily activities, daily tasks, and interactions with peers.
Other factors are considered as well. In young children, we find that the time of diagnosis matters to the family’s comfort with the diagnosis. If a child is diagnosed by prenatal ultrasound, the family will have more time to grow to accept the diagnosis and perform their own research and come to some early conclusions about the role of the hand surgeon. If not diagnosed before birth, more time may be necessary for the family to become comfortable with the diagnosis. The age of the child at the time of the visit to the hand surgeon also matters (for similar reasons) as families can better understand potential function as the child ages and increases hand use. Associated conditions matter including medical conditions (the kidneys and the heart). In TAR syndrome, for example, the platelet count has to be considered before discussing surgery.
As I have written before, in children with radial longitudinal deficiency we have a general plan. Consider this child who is nearing two years of age and travels a great distance to come visit. She has a severe angulation of the wrist with an absent radius (making this a Type IV radial deficiency) and an absent thumb (Type V thumb). This is a quite common presentation in our clinic.
|Radial longitudinal deficiency with marked deviation at the wrist.|
|Radial longitudinal deficiency with absent radius bone and deviation.|
We also check a few other features. First, we assess how correctable the wrist deviation posture is- passive stretching can help but the flexibility matters in considering surgery. This child is mildly flexible but cannot be corrected all the way to neutral (i.e., we cannot straighten her wrist).
|Gentle stretching shows limited correction of the wrist in radial deficiency.|
|Radial longitudinal deficiency– lateral view showing palmar position of the carpus and hand compared to the forearm.|
|Application of a unilateral (one sided) fixator in radial deficiency. The device allows gradual stretching which will make the centralization procedure easier and safer in the future.|
Charles A. Goldfarb
My Bio at Washington University