Madazli Riza, Tüysüz Beyhan, Aksoy Figen, Barbaros Merve, Uludağ Seyfettin, Ocak Vildan
Department of Obstetrics and Gynaecology, Cerrahpasa Medical Faculty, University of Istanbul, Turkey.
Fetal Diagn Ther. 2002 Jan-Feb;17(1):29-33. doi: 10.1159/000048002.
Arthrogryposis multiplex congenita is a general term for congenital multiple joint contractures, the aetiology of which is variable. Prenatal diagnosis is usually based on the detection of diminished fetal movements and joint contractures on ultrasound. There are also reports of early diagnosis of arthrogryposis in the first and early second trimester by detection of subcutaneous oedema. We report another case of arthrogryposis multiplex congenita with increased nuchal translucency and scoliosis diagnosed by ultrasonography at 15 weeks of gestation. The pregnancy was terminated at the request of the parents. Post-mortem examination revealed that it was not associated with fetal myopathy or neuropathy. Multiple joint contractures with increased nuchal translucency without any underlying fetal neurogenic and myogenic pathology may be a distinct form of arthrogryposis multiplex congenita.
先天性多发性关节挛缩症是先天性多关节挛缩的统称,其病因多样。产前诊断通常基于超声检查发现胎儿活动减少和关节挛缩。也有报告称在孕早期和孕中期通过检测皮下水肿可早期诊断关节挛缩症。我们报告了另一例先天性多发性关节挛缩症病例,该病例在妊娠15周时通过超声检查诊断为颈部半透明带增厚和脊柱侧弯。应父母要求终止了妊娠。尸检显示其与胎儿肌病或神经病无关。颈部半透明带增厚且无任何潜在胎儿神经源性和肌源性病变的多关节挛缩可能是先天性多发性关节挛缩症的一种独特形式。