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Ulnar dysmelia

J A Ogden, H K Watson, W Bohne

    The Journal of Bone and Joint Surgery. American Volume
    |June 1, 1976
    PubMed
    Summary
    This summary is machine-generated.

    Surgical resection of the fibrocartilaginous anlage in infants with ulnar aplasia can reduce forearm deformities. Deferring the one-bone-forearm procedure may also prevent complications in these complex limb abnormalities.

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    Area of Science:

    • Orthopedics
    • Pediatric Surgery
    • Developmental Biology

    Background:

    • Ulnar hypoplasia and aplasia are complex postaxial forearm and hand abnormalities.
    • Three types of ulnar deformity were identified: hypoplasia, partial aplasia, and total aplasia.
    • A fibrocartilaginous anlage may represent the 'absent' ulna segment.

    Purpose of the Study:

    • To review cases of ulnar hypoplasia and aplasia.
    • To evaluate the role of the fibrocartilaginous anlage in deformity progression.
    • To recommend surgical timing for managing these congenital abnormalities.

    Main Methods:

    • Retrospective review of eleven patients with ulnar hypoplasia/aplasia.
    • Radiographic assessment of ulnar deformities and associated structures.

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  • Analysis of surgical outcomes following anlage resection.

    • Main Results:

    • The fibrocartilaginous anlage can tether structures, causing in utero and postnatal deformities like wrist deviation and radial head dislocation.
    • Surgical resection of the anlage in the first two years of life appeared to reduce angular growth deformities.
    • Complications associated with the one-bone-forearm operation may be reduced by deferring the procedure.

    Conclusions:

    • Early resection of the ulnar fibrocartilaginous anlage is recommended to mitigate progressive deformities.
    • Delayed surgical intervention for the one-bone-forearm procedure is advised.
    • Understanding the role of the anlage is crucial for managing these complex limb abnormalities.