Amyoplasia Congenita In A Premature Baby

Abstract

Aim: Arthrogryposis multiplex congenita is a term used to describe the multiple joint contractures at birth. Amyoplasia congenita in clinic, hands and wrist are in flexion, elbows are in extension, shoulder are in adduction and internal rotation, bilateral pes equinovarus deformity and multiple joint contractures are present. Amyoplasia is the most common sub-group of arthrogryposis multiplex congenita. The pathogenesis of the disease is not known. Early diagnosis, early treatment is important to launch. Herein, we present a 10-month old infant with multiple joint contractures at premature birth with a diagnosis of amyoplasia congenita. Amyoplasia is a rare condition in premature baby. It is the purpose of this report to summarize and update what is known about the etiology, comorbid disorder, and orthopaedic approach of infants with this diagnose.

Keywords

• Arthrogryposis multiplex congenita,amyoplasia,prematurity

Prematüre Bir Bebekte Amyoplazia Konjenita

Abstract

Amaç: Artrogripozis multipleks konjenita; doğumda bulunan çoklu eklem kontraktürlerini tanımlamak için kullanılan bir terimdir. Amyoplazia konjenitanın kliniğinde ise; eller ve bileklerde fleksiyon, dirseklerde ekstansiyon, omuzlarda addüksiyon ve iç rotasyon, bilateral pes ekinovarus deformitesi ve multiple eklem kontraktürleri mevcuttur. Amyoplazia; artrogripozis multipleksin en sık görülen alt grubudur. Hastalığın patogenezi kesin olarak bilinmemektedir. Tanının erken konulması, tedaviye erken başlanılması önemlidir. Burada; premature doğan, multiple eklem kontraktürleri olup amyoplazia konjenita tanısı almış 10 aylık kız hasta sunulmaktadır. Amyoplazia premature bebeklerde nadiren görülür. Bu yazının amacı, tanı alan infantlarda etiyoloji, eşlik eden bozukluklar ve ortopedik yaklaşım hakkında bilinenleri özetlemek ve güncellemektir.

Keywords

• Artrogripozis multipleks konjenita,amyoplazia,prematurite

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