Congenital Upper Limb Anomalies 185from operative interventions are generally poor such as camptodactyly (Watts
and Hooper, 2006). Other conditions that have yet to declare the degree of
their structural or functional impairment are likely best to monitor and manage
conservatively. Conservative management allows a child to accommodate to a
disorder and develop alternative ways to function, which may be problematic
if operative intervention is desired in the future. When conservative
management fails to correct or slow the progression of a condition, patients
and caregivers turn to operative intervention.
Operative Intervention
If the patient and family opt for reconstructive surgery to address the
CULA, three main factors influence decision-making for the operative plan:
function, appearance, and timing.
Hand function requires a stable shoulder, upper arm and forearm. Within
the hand, a mobile thumb is needed to accomplish opposition, radial digits for
fine pinching, and ulnar digits for grasping. Adequate digit length, stability,
and mobility are needed to optimize hand function. In a complicated preaxial
polydactyly with 7 digits and near mirror image duplication, one of the extra
digits should be pollicized (shortening with partial rotation) to form a thumb
and the remaining polydactylous digits removed (Figure 3). Depending on the
CULA, it may not be possible to attain normal function, particularly with a
complex anomaly. Prioritization of functional objectives for reconstruction
will depend on the complexity of the abnormality, the handedness of the child,
and the function of the other upper limb. Cultural expectations must also be
considered. For example, if the forearm must be fixed in a stable position to
optimize hand function, having the hand fixed in supination would allow a
child to hold their own bowl when eating, whereas in another culture
maintaining the hand in a neutral position would accommodate more customs.
The appearance of a noticeable abnormality such as distal thumb
duplication (Wassel type III) can be a source of considerable concern for the
child and parents, even if it allows significant function. Moreover, loss of a
structure is often less noticeable than an abnormal structure. Pollicization of
the index finger in a hand with thumb aplasia produces a reasonable thumb
equivalent with three additional digits, an outcome that passes as normal to the
casual observer. In some complex cases, such as symbracydactyly,
reconstruction to form a normal appearing hand is not possible. In these cases,
adding a toe transfer to improve function may be seen as further disfiguring