Limb Body Wall Complex Associated with Placenta Accreta: A Mere Coincidence or a Sign of an Etiopathogenic Link? / Limb body wall complex associada à placenta acreta: uma mera coincidência ou um indício de um elo etiopatogênico?

Abstract A case was reported of a fetus with the anomaly of limb body wall complex associated with placenta accreta. To date, only one account of this condition has been published in the world literature. Due to the low frequency of both complications, the hypothesis has been raised that this association may have happened not by mere coincidence, but rather by a possible common etiopathogenic mechanism. For the first time, a study proposes the existence of a possible etiopathogenic connection between the anomaly of limb body wall complex and hypoxic disorders caused by inadequate placentation in previous uterine scarring.

Resumo Foi relatado um caso de feto com anomalia de limb body wall complex associada a uma placenta acreta. Até o presente, apenas uma descrição com essa condição foi publicada na literatura mundial. Devido à baixa frequência das duas complicações, foi levantada a hipótese de que essa associação possa ter ocorrido não por umamera coincidência,mas por um possível mecanismo etiopatogênico comum. Pela primeira vez, um estudo propõe a existência de uma possível possível ligação etiopatogênica entre a anomalia de limb body wall complex e os transtornos hipóxicos causados pela placentação inadequada em cicatriz uterina prévia.

Significance of fibrotic bands in utero ‑ Amniotic band sequence with limb body wall complex: A rare case of fetal autopsy.

Amniotic band sequence (ABS) includes a wide spectrum of abnormalities resulting from entrapment of various fetal parts from a disrupted amnion, ranging from a mere constriction ring affecting a finger to a fatal form called limb body wall complex (LBWC). Reported cases of ABS with LBWC are very few. The spectrum of anomalies depends on which part gets entrapped and at what point of gestation. Hence, the clinical presentation can be extremely variable. Early detection of such cases using sonology is really challenging due to the small size of the fibrotic bands. Here, we present a case of amniotic band syndrome with LBWC in a fetus at 24 weeks of gestation, which was referred for an autopsy. The fetus also showed scoliosis, gastroschisis, lumbosacral meningocele, congenital talipes equinovarus, and cleft palate, thus having features of placenta cranial and placenta abdominal phenotype which is very rare.

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.

Limb body wall complex or body stalk complex or cyllosomas: a case report.

Limb body wall complex (LBWC) is also called Body stalk complex and Cyllosomas. We present this rare congenital malformation complex highlighting the importance of early sonographic imaging findings in LBWC along with differentiation from other anterior abdominal wall defects. Limb body wall complex / Body stalk anomaly refers to a rare complicated polymalformative fetal malformation syndrome of uncertain etiology and results in head, heart, lung, diaphragm, kidney or gonadal abnormalities. LBWC was first described by Van Allen et al; in (1987). The two of the three following anomalies must be present to establish the diagnosis: 1. Exencephaly / Encephalocele with facial clefts, 2. Thoraco-Abdominoschisis / ventral body wall defects and 3. Limb defects. LBWC arises as a result of early amnion disruptions or error in embryonic development. If all components of the syndrome are present, the condition is lethal. LBWC is invariably fatal and incompatible with life. No case of postnatal survival is reported so far. Serum alpha-fetoprotein measurement and ultrasonography examination is the key to the prenatal diagnosis and followed by medical termination of pregnancy. It presents two distinct phenotypes described by Russo et al (1993) and later Cusi et al in (1996), according to the foetoplacental relationships: 1. Placento-cranial and 2.Placento-abdominal types. Among the 168 live births at S.V.S. Medical College & hospital Mahabubnagar (INDIA) during the period of 2010-2011 we came across an aborted female fetus. It was weighing 1800gms, 30 weeks of gestation diagnosed by antenatal ultrasonography as ventral body wall defect. It was associated with ompholocele, severe scoliosis and limb defects. Its confirmation of the diagnosis of Limb body wall complex with Placento-abdominal type was done by postmortem fetography.

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 Limb-Body Wall Complex Diagnosed by Prenatal Ultrasonography / 대한산부인과학회잡지

Limb-body wall complex, also known as the short umbilical cord syndrome or the body stalk anomaly, 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. Ultrasonographic detection of abdominoschisis, scoliosis, abnormalities of the lower extremities, a single umbilical artery and a short umbilical cord is important for the prenatal diagnosis. This complex should be distinguished from other body wall defects including omphalocele and gastroschisis since the prognosis for limb-body wall complex is uniformly poor. We experienced a case of limb-body wall complex in a fetus of intrauterine pregnancy at 15 weeks who was terminated because of ultrasonographic demonstration of a large abdominal defect with eventration of abdominal organs, short umbilical cord and kyphoscoliosis. Thus, we report a case with brief review of the literatures.

A Case of Limb-Body Wall Complex / 대한주산의학회잡지

Limb-Body Wall complex is a complicated with the essential features of neural-tube defects, body-wall disruption, and limb abnormalities. This complex should be distinguished from other body-wall defects including omphalocele and gastroschisis since the prognosis for limb-body wall is uniformly poor. We have experienced one case of limb-body wall complex in a 22-year-old primigravida, which is presented with a brief review of the literature.