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Type-I Tibial Hemimelia

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Background: Tibial hemimelia is a rare but disabling condition. Although reconstructive methods have been described, the recommended treatment typically has been amputation at various levels followed by the use of a suitable prosthesis. A new technique known as femoro-fibulo-calcaneal arthrodesis has been developed as a limb-salvage procedure for patients with type-I deficiency who refuse amputation or have no access to good prosthetic care. Methods: Twelve children (18 extremities) with type-I tibial hemimelia were managed surgically. The ages of the patients at the time of surgery ranged from 2 to 14 years. The procedure includes 3 stages: loosening, lengthening, and stabilization. In all patients, the loosening stage involved release of soft-tissue contractures at both ends of the fibula. The lengthening stage involved either supervised lengthening at home with use with use of an external fixator (6 patients) or the use of traction in the hospital (6 patients). In all patients, the stabilization stage was subsequently performed by stabilizing the fibula to the femoral condyles proximally and the talus distally with use of crossed Kirschner wires. Results: All 12 patients returned for follow-up for the first 5 years. All patients were evaluated by author. Two patients who had ipsilateral femoral focal deficiency were subsequently lost to follow-up. The remaining 10 patients were followed for a mean of 10 years (range, 5 to 32 years). All 10 patients were able to walk on their feet during follow-up. None of the patients in the present study had an amputation or needed a prosthesis. There were no major complications. Conclusions: A new procedure, femoro-fibulo-calcaneal arthrodesis, has been proposed for the treatment of type-I tibial hemimelia. The suggested procedure is simple, biological, cost-effective, and dependable. It provides a long-term stable and functional extremity that enables the patient to walk with plantigrade feet with sensation and proprioception. This limb-saving procedure should be considered as an alternative for patients who refuse to lose the limb or for whom good prosthetic care is unavailable. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Ovid Technologies (Wolters Kluwer Health)
Title: Type-I Tibial Hemimelia
Description:
Background: Tibial hemimelia is a rare but disabling condition.
Although reconstructive methods have been described, the recommended treatment typically has been amputation at various levels followed by the use of a suitable prosthesis.
A new technique known as femoro-fibulo-calcaneal arthrodesis has been developed as a limb-salvage procedure for patients with type-I deficiency who refuse amputation or have no access to good prosthetic care.
Methods: Twelve children (18 extremities) with type-I tibial hemimelia were managed surgically.
The ages of the patients at the time of surgery ranged from 2 to 14 years.
The procedure includes 3 stages: loosening, lengthening, and stabilization.
In all patients, the loosening stage involved release of soft-tissue contractures at both ends of the fibula.
The lengthening stage involved either supervised lengthening at home with use with use of an external fixator (6 patients) or the use of traction in the hospital (6 patients).
In all patients, the stabilization stage was subsequently performed by stabilizing the fibula to the femoral condyles proximally and the talus distally with use of crossed Kirschner wires.
Results: All 12 patients returned for follow-up for the first 5 years.
All patients were evaluated by author.
Two patients who had ipsilateral femoral focal deficiency were subsequently lost to follow-up.
The remaining 10 patients were followed for a mean of 10 years (range, 5 to 32 years).
All 10 patients were able to walk on their feet during follow-up.
None of the patients in the present study had an amputation or needed a prosthesis.
There were no major complications.
Conclusions: A new procedure, femoro-fibulo-calcaneal arthrodesis, has been proposed for the treatment of type-I tibial hemimelia.
The suggested procedure is simple, biological, cost-effective, and dependable.
It provides a long-term stable and functional extremity that enables the patient to walk with plantigrade feet with sensation and proprioception.
This limb-saving procedure should be considered as an alternative for patients who refuse to lose the limb or for whom good prosthetic care is unavailable.
Level of Evidence: Therapeutic Level IV.
See Instructions for Authors for a complete description of levels of evidence.

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