The Journal of Bone and Joint Surgery, Vol 71, Issue 2 278-287, Copyright © 1989 by Journal of Bone and Joint Surgery, Inc
Congenital longitudinal deficiency of the tibia
PL Schoenecker, AM Capelli, EA Millar, MR Sheen, T Haher, MD Aiona and LC Meyer
Shriners Hospital for Crippled Children, St. Louis, Missouri 63131.
Fifty-seven patients (seventy-one limbs) who had congenital longitudinal
deficiency of the tibia (tibial hemimelia) were retrospectively categorized
according to radiographic type (Types 1 through 4, as described by Jones et
al.). At an average follow-up of nine years, fifty-six of fifty-seven
patients walked independently. An ablative surgical procedure was performed
on sixty-one of the seventy-one lower extremities. According to the
classification of Jones et al., fifty-four limbs had a Type-1 (a or b) or
Type-2 deficiency. In twenty-two of these extremities, disarticulation of
the knee was performed; in twenty-five, a Syme amputation; and in one, a
Chopart amputation. The ipsilateral foot was retained in six extremities
that had a severe Type-1 or Type-2 deficiency. Medial transfer of the
fibula (the Brown procedure) generally yielded less than satisfactory
results; in ten of fourteen extremities, one or more additional operations
were needed. Seventeen extremities were classified as having a Type-3 or
Type-4 deficiency; Syme amputation was done in nine and Chopart amputation,
in four. Despite satisfactory reconstruction of the ankle, a Syme
amputation was necessary in most extremities that had a Type-4 deficiency
because a major leg-length discrepancy was projected. In four limbs that
had a Type-3 or Type-4 deficiency, the foot was retained.
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