The spectrum of arthrogryposis in 33 chinese children

Brain Dev. 1997 Apr;19(3):187-96. doi: 10.1016/s0387-7604(96)00557-8.

Abstract

The clinical profile of 33 children (19 boys, 14 girls) with multiple congenital contractures has been studied. The majority (54%) belong to arthrogryposis multiplex congenita with a static clinical course. Children were classified into three groups: group I (limb involvement only; n = 21) having arthrogryposis multiplex congenita (n = 18), distal arthrogryposis syndrome (n = 2) and Streeter syndrome (n = 1); group II (limb involvement with other malformation or anomalies; n = 7) having congenital contractural arachnodactyly (n = 3), Larsen syndrome (n = 1), multiple pterygium syndrome (n = 1), craniocarpotarsal dystrophy (n = 1), and Schwartz Jampel syndrome (n = 1); and group III (limb involvement with central nervous system dysfunction or mental retardation; n = 5) having myotonia dystrophica (n = 2), congenital muscular dystrophy (n = 1), foetal alcohol syndrome (n = 1) and Pena-Shokeir syndrome (n = 1). Three children died, one each of arthrogryposis multiplex congenita, congenital contractural arachnodactyly and myotonia dystrophica. The majority had a good prognosis with independent function and mobility.

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Adolescent
  • Adult
  • Amniotic Band Syndrome / complications
  • Amniotic Band Syndrome / diagnosis
  • Arthrogryposis / classification*
  • Arthrogryposis / complications
  • Arthrogryposis / mortality
  • Asian People*
  • Child
  • Child, Preschool
  • Female
  • Fetal Alcohol Spectrum Disorders / complications
  • Fetal Alcohol Spectrum Disorders / diagnosis
  • Humans
  • Infant
  • Infant, Newborn
  • Intellectual Disability / complications
  • Intellectual Disability / diagnosis
  • Male
  • Muscular Dystrophies / complications
  • Muscular Dystrophies / diagnosis
  • Myotonia / complications
  • Myotonia / diagnosis
  • Osteochondrodysplasias / complications
  • Osteochondrodysplasias / diagnosis
  • Prognosis