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Chapter 5: Hand and Arm Differences in FA 101 Figure 2: One-year-old with a right Type IV thumb deficiency attached to the radial side of the hand, also known as a pouce flouttant (floating thumb). (Courtesy of Shriners Hospital for Children, Philadelphia) Figure 3: Six-year-old with complete absence or Type V deficiency of his right thumb. (Courtesy of Shriners Hospital for Children, Philadelphia)
102 <strong>Fanconi</strong> <strong>Anemia</strong>: Guidelines for Diagnosis and Management child often helps discriminate between these types by pattern of usage. An unstable thumb (Type III-B) is not incorporated into pinch and grasp. Prehension or pinch develops between the index and long digits, and the index finger tends to rotate out of the palm to resemble a thumb position. In equivocal cases, the decision is further complicated by the delayed ossification of the bones at the base of the thumb (trapezium and trapezoid), which do not ossify until four to six years of age. The decision to ablate a hypoplastic thumb without a base is often a difficult process for parents and caregivers. Discussions with the surgeon and conversations with families who have made similar decisions are often helpful. A thumb that is slightly smaller than the normal thumb (Types I, II, and III-A) can be reconstructed, or augmented, by tendon transfers to improve its motion and use. Thumb reconstruction in Types II and III-A requires addressing all deficient elements. The tight web space is corrected by opening the space between the thumb and index finger. The metacarpophalangeal joint instability is corrected by ulnar collateral ligament reconstruction. The deficient thenar muscles are supplanted by a tendon and/or muscle transfer from the ring or small finger to the thumb (Figure 4). This tendon transfer improves active motion and enhances function. There is negligible effect on the donor digit. A thumb without a stable base (Types III-B, IV, and V) is removed, and the index finger moved to the thumb position. The index is moved with its nerves, arteries, tendons, and muscles. This procedure is known as a pollicization. The time to perform pollicization remains controversial, with a trend toward early surgery (6 months to 1 year of age), prior to the normal
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Chapter 9 Matched Sibling Donor Hem
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Chapter 12 Novel Treatment Options
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Chapter 13 Head and Neck Squamous C
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Chapter 14 The Adult Fanconi Anemia
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