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The Korean Journal of Pathology 1993;27(6): 638-644.
Limb-Body Wall Malformation Complex: Two autopsy cases and its pathogenesis.
Geun Shin Lyu, Nam Hoon Kim, Eun Kyung Hong, Jung Dal Lee
Department of Pathology, Hanyang University Medical School, Seoul, Korea.
Limb-body wall malformation complex(LBWC), also know as the amniotic band syndrome, is a poorly defined, sporadic group of congenital anomaly characterized by a collection of protean fetal malformation, deformation and disruption. Accurate diagnosis is often difficult because of its variable presentation pattern and the absence of exactly same case. We report two autopsy cases. One revealed body wall, cardiac, and craniofacial anomalies with anencephaly, and amniotic adhesive band attached to craniofacial defect of the fetal part without evidence of amniotic rupture. The other exibited abdominal wall defect with omphalocele, visceral, postural, and limb anomalies together with neural tube defect in the lumbosacral region. The pathogenesis of this syndrome was discussed in detail.
Key Words: Limb-body wall malformation complex; Amniotic band syndrome; Pathogenesis