Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex).

OEIS is an extremely rare constellation of malformations, which includes omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect. We report here autopsy findings in a case of OEIS complex, which apart from the major anomalies of the complex had bilateral club foot that is, congenital talipes equinovarus, right hydroureter, and body stalk anomaly. The umbilical cord was absent, and the umbilical vessels were embedded in an amniotic sheet, which connected the skin margin of the anterior body wall defect to the placenta, this feature being the hallmark of limb body wall complex (LBWC). This case further supports the view that OEIS and LBWC represent a continuous spectrum of abnormalities rather than separate conditions and may share a common etiology and pathogenetic mechanism as proposed by some authors.

A Case of Limb-body Wall Complex / 대한산부인과학회잡지

Limb-body wall complex, also known short umbilical cord syndrome and body stalk anomaly, is a complex set of disruptive abnormalities consisting of failure of ventral body wall to close, short umbilical cord, disruption of lateral body wall, distinctive scoliosis of spine, limb defects, facial defects and cranial defects. This disorder has similar findings to amnioitic band syndrome. but limb-body wall complex appears severe scoliosis of spine, herniation of internal organs, absent or short umbilical cord. Recently, we present a case of limb-body wall complex which found at 32+1 weeks. A case was diagnosed with ultrasonography, so we report a case of limb-body wall complex with brief review of literature.

A Case of Short Umbilical Cord Sundrome / 대한산부인과학회잡지

Short umbilical cord syndrome, also known as the limb-body wall malformation complex and the body stalk anomaly, is a poorly defined sporadic group of congenital anomaly charaterized by a complex set of disruptive abnormalities having in common the failured closure of the ventral body wall. This disorder is charaterized by a short or absent umbilical cord and disruption of the lateral body wall, spine, limbs, face, and cranium, isolated or in combination. Recently, we present a case of short umbilical cord syndrome which found in a term baby, so we report a case of short umbilical cord syndrome with brief review of literature.

Body Stalk Anomaly: Analysis of 10 Autopsy Cases

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.

Body stalk anomaly: a case report

A case is presented of an amnionic rupture sequence which led to massive fetal ventral herniation and lordoscoliosis. Characteristic ultrasonographic findings of an omphalocele, fetal attachment to the placenta, and the absence of free-floating umbilical cord were observed.