Abstract
Congenital lateral ray deficiency is considered to be a manifestation of fibular hemimelia. However, we have noted patients with absent lateral ray but stable knee and ankle joints, and named this condition terminal hemimelia of the lower extremity. This study was undertaken to further define this group of patients and to compare these patients with fibular hemimelia patients. Four boys and one girl of mean age six years two months were in the terminal hemimelic group and four boys and three girls of mean age eight years seven months in the fibular hemimelic group at the final evaluation. Clinical features commonly observed in the fibular hemimelia such as knee valgus, knee instability, tibial bowing, ball and socket ankle, ankle instability, tarsal coalition, leg length inequality were compared between both groups. Terminal hemimelia of the lower extremity was the same as fibular hemimelia in clinical features below the ankle joint. However, terminal hemimela was found to be milder than fibular hemimelia in terms of limb shortening. The clinical features above the ankle joint were different between both groups. Knees and ankles were stable, and gait disturbance were rarely noticed in patients with terminal hemimelia of the lower extremity.
Résumé
Le déficit congénital du rayon externe doit être considéré comme une manifestation de l’ectromélie longitudinale externe. Cependant, nous avons noté chez certains patients présentant une absence du rayon latéral un genou et une cheville stables. Nous l’avons appelé dans ces conditions hémimélie terminale de l’extrémité inférieure. Cette étude a pour but de définir ce groupe de patients et de comparer ces patients avec l’ectromélie longitudinale externe classique. 4 garçons et une fille, d’âge moyen 6 ans et deux mois présentant une hémimélie terminale et 4 garçons et trois filles, d’âge moyen de 8 ans et sept mois présentant une ectromélie longitudinale externe ont été étudiés. Etude clinique : les éléments cliniques habituels dans l’ectromélie longitudinale externe tel que l’instabilité, le valgus du genou, la courbure tibiale, la cheville en dôme avec instabilité et la synostose du tarse postérieur ainsi que l’inégalité de longueur ont été comparés dans les deux groupes, l’hémimélie terminale de l’extrémité inférieure est identique à l’ectromélie longitudinale externe en ce qui concerne la cheville, cependant, l’hémimélie terminale entraîne un raccourcissement du membre moins important que l’ectromélie longitudinale externe. Les éléments cliniques, au niveau de la cheville, sont différents dans les deux groupes. Le genou et la cheville sont stables. Il y a peu de trouble de la marche habituellement chez ces patients.
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An erratum to this article can be found at http://dx.doi.org/10.1007/s00264-007-0401-2
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Baek, G.H., Kim, J.K., Chung, M.S. et al. Terminal hemimelia of the lower extremity: absent lateral ray and a normal fibula. International Orthopaedics (SICO 32, 263–267 (2008). https://doi.org/10.1007/s00264-006-0293-6
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DOI: https://doi.org/10.1007/s00264-006-0293-6