Abstract:Symbrachydactyly is literally defined as a combination of short fingers with syndactyly. Blauth and Gekeler described four types of symbrachydactyly, ranging from simple shortness of middle phalanges to complete absence of digital rays. In type I (short finger) function is quite normal and syndactyly release is usually the only procedure needed. In type II (cleft hand), presence of a thumb and at least one ulnar finger allows pinch function. Surgical treatment, when needed, consists in separation of webbed fin… Show more
“…La préhension est normale et une chirurgie d'allongement des phalanges distales par une distraction osseuse [11] ne se justifiait donc pas. Au demeurant, nous pensons comme Leroux et al [12] que l'axe de la prise en charge doit être tourné vers l'avenir fonctionnel de la main, bien plus que sur l'avenir esthétique.…”
“…La préhension est normale et une chirurgie d'allongement des phalanges distales par une distraction osseuse [11] ne se justifiait donc pas. Au demeurant, nous pensons comme Leroux et al [12] que l'axe de la prise en charge doit être tourné vers l'avenir fonctionnel de la main, bien plus que sur l'avenir esthétique.…”
“…They are subdivided into transverse, intercalary, and longitudinal (preaxial, postaxial, and mesoaxial) deficiencies. Amniotic band syndrome (also known as constriction band syndrome) is the main differential diagnosis, but some authors [5][6][7][8][9][10][11] have proposed including this syndrome in the failure of formation deficiencies.…”
Congenital upper limb malformations represent complex pathologies because of their varied clinical presentations, imaging features, and etiologies. They can be divided into (1) failure of formation with transverse, intercalary, and longitudinal (preaxial, postaxial, and mesoaxial) deficiencies, (2) failure of differentiation with synostoses, carpal coalitions, syndactylies, and symphalangism, (3) duplication with ulnar dimelia and polydactylies, and (4) brachydactylies. Congenital Madelung's deformity, clinodactyly, camptodactyly, and Kirner's deformity are usually included in these malformations. Despite advances in molecular diagnosis, a good knowledge of clinical and imaging features as well as special consideration of other skeletal or nonskeletal abnormalities are essential to eventually diagnose an embryo fetopathy (maternal valproate treatment, constriction band syndrome), a genetic disorder (trisomy 21 or Down syndrome, Turner's syndrome, Holt-Oram syndrome), or a nongenetic syndrome (vertebral, anal, cardiac, tracheal, esophageal, renal, limb association, Poland's syndrome). Genetic counseling for a child presenting with a congenital upper limb malformation is of great value, both for the treating team and the parents, and imaging is often required. The latter is still largely supported by conventional radiography, both for diagnosis and functional prognosis, but ultrasound and magnetic resonance imaging will be great tools in the near future to better evaluate these conditions.
This article, presented in three sections, review the most common upper limb malformations and their treatments. In this section two there's a discussion about failure of formation and duplication of the parts. The bibliography is continuous since section one.
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