Upper-Extremity Congenital Anomalies

Deformidades congênitas dos membros superiores: parte I: falhas de formação

“…While the typical form is related to possible fusion of rays, the atypical form is thought to be one of the manifestations of the teratological sequence of brachysyndactyly, occurring through necrosis of the mesenchymal tissue. Thus, (3,22) .…”

“…It may be complete -hand inserted in the trunk, with absence of the upper arm and forearm; proximalabsence or hypoplasia of the upper arm, with forearm and hand inserted in the trunk; or distal -hand inserted in the arm (10) . Phocomelia is very rare and its prevalence increased by 60% with the use of thalidomide during the first trimester of pregnancy (3) . The preferred treatment consists of fitting a prosthesis, and surgery is reserved for facilitating the use of prostheses (3,10) .…”

“…James and Bednar (14) modified this classification, so as to include cases of absence of the thumb with a normal radius (3) . Conservative treatment with placement of a series of orthoses and stretching are recommended from the time of birth, independent of the type of deformity.…”

“…Classically, such deformities are divided into typical and atypical (3) (Table 3). The theories involving the typical and atypical forms of cleft hand differ.…”

“…Of these, around 10% have upper-limb deformities (1)(2)(3) . Some of these deformities occur in isolation, but there are associations with systemic syndromes (4) that may involve blood dyscrasia, cardiopathies, central nervous system (CNS) malformations, digestive tract malformations or delayed neuropsychomotor development.…”