guidebook. - Fanconi Anemia Research Fund

112
Fanconi Anemia: Guidelines for Diagnosis and Management
The radial deviation deformity is treated by a combination
of non-operative and operative management that
begins shortly after birth. The initial treatment for the
absent radius is stretching, both by the therapist and
the caregiver. Stretching is usually recommended every
diaper change and is important to the overall success of
treatment. Fabrication of a splint is difficult in the newborn
with a shortened forearm, and is usually delayed
until the forearm is long enough to accommodate a
splint (Figure 10). Splints are used to maintain the hand
in a straight alignment. If no treatment is rendered, the
hand will develop a fixed perpendicular relationship to
the forearm.
Surgical treatment for Types II, III, and IV deficiencies
involves placing the wrist on top of the ulna, which
is the only substantial bone within the forearm. The
procedure is known as a “centralization” or “radialization.”
Centralization remains the principal procedure
to realign the carpus onto the distal ulna. Contraindications
for surgical intervention are mild deformity with
adequate support for the hand (Type 1) and an elbow
extension contracture that prevents the hand from
reaching the mouth. In these children, the radial deviation
of the wrist facilitates hand to mouth function and
straightening would further impair this motion. Another
contraindication to centralization is adults who have
adjusted to their deformity.
The procedure is typically performed at about one year
of age, and the initial correction is impressive. Unfortunately,
the ability to maintain the correction and prevent
recurrence has not been completely solved. Centralization
is performed by release of the tight aberrant radial
musculotendinous units and anomalous contracted
fibrous bands to allow passive correction of the over
the end of the ulna (Figure 11A and B).