Priorities in management include:
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First stage, immediate nonoperative care:
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Elbow extension contracture:
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Elbow passive flexion stretching exercises.
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Elbow flexion motion may be improved indirectly by splinting to improve
wrist position.
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Wrist displacement and angulation:
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Angulation of the wrist may be helped considerably by splinting, although
ulnocarpal displacement can only be corrected with surgery.
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Corrective splinting or casting should be undertaken immediately, which
will reduce the extent of soft tissue release needed for correction.
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Splinting/casting for weeks to months. Prolonged splinting will
restrict psychomotor development and for this reason the most common interval
of splinting recommended before surgery is months but not years.
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Screening for associated medical conditions. Systemic and potential
life threatening disorders are associated with radial club hand involving
cardiac, renal, spine and hematopoetic systems and require screening before
any surgery is undertaken.
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Second stage, surgery:
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Early surgery is probably better. There is a trend to proceed with
surgery at or before one year of age, but preoperative corrective splinting
or casting is always indicated. Surgery before age one usually requires
less soft tissue release, less frequent need for carpectomy, and is followed
by more physiologic widening of the distal ulna to support the carpus.
Results of surgery are clearly worse when delayed to the age of three or
beyond.
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Surgical planning should include:
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Elbow capsulotomy and triceps lengthening.
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Osteotomy for ulnar bowing.
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Flap transfer of distal forearm skin from the area where it is most ulnarly
redundant to the area which is most radially deficient.
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Correction of wrist alignment:
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Wrist centralization, but if possible,
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Buck Gramcko's wrist radialization with alignment of the distal ulna on
the radial carpus and transfer of the entire radial muscle mass
to the insertion of the extensor carpi ulnaris muscle, which is itself
shortened by reefing. Profundus tendon transfers for wrist motor reconstruction
would seem to be a good idea, but have been shown to be unreliable.
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Later procedures for syndactyly correction and pollicization.
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