Journal of Pathology and Translational Medicine

Case Report

Limb-Body Wall Malformation Complex: Two autopsy cases and its pathogenesis.: Geun Shin Lyu, Nam Hoon Kim, Eun Kyung Hong, Jung Dal Lee; Korean J Pathol. 1993;27(6):638-644.

Abstract PDF: 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.

Original Article

Body Stalk Anomaly: Analysis of 10 Autopsy Cases.: Seung Sook Lee, Je G Chi; Korean J Pathol. 1993;27(3):235-242.

Abstract PDF: Body stalk anomaly represents an extreme maldevelopment of embryonic body folding and is characterized by absence of the umbilicus and umbilical cord. The failure of complete obliteration of the extraembryonic coelom is responsible for the absence of the umbilical cord formation and the wide-based insertio of the amnioperitoneal membrane onto the placental chorionic plate. We have analyzed 10 autopsy cases of various midline anomalies of the body that could best be classified into body stalk anomaly. All cases were either stillborns or dead immediately after birth. The pregnancy was interrupted due to this anomaly in 6 cases, and their gestational ages varied from 17 weeks to 37 weeks. The affected fetuses were characterized bt absent or vestigial umbilical cord, and ruptured amnion with direct amnioperitoneal connection without the mediation of the umbilical cord. Exomphalos with abdominal wall defect and serve scoliosis were characteristic components of this anomaly, that provided important clues in differentiating other similar anomalies. Other associated anomalies included neural tube defect, intestinal atresia, genitourinary and skeletal defects, pulmonary hypoplasia, single umbilical artery and narrow-spaced chest and abdomen, etc. These findings strongly suggest that anomaly of body stalk represents mechanical teratogenesis due to early amnion repture and subsequent effect, and should be categorized into amniotic band disruption syndrome.

Case Report

Amniotic Band Syndrome: An autopsy case report.: Hye Seon Ahn, Gil Ro Han, Jin Hee Sohn, Jung Il Suh; Korean J Pathol. 1989;23(4):482-486.

Abstract PDF: We report an autopsy case of amniotic band syndrome exhibiting microcephaly, asymmetric encephalocele, microphthalmia, nasal deformity, cleft lip and palate accompanied by left maxillary and zygomatic bone deformities. The amniotic membrane of the placenta was also attached to the herniated brain. The twenty-year-old primigravid mother had no history of taking drug, irradiation, infection or trauma before or during pregnancy.

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