Limb Body Wall Complex Placenta-Abdominal Phenotype: Case Report

Abstract

Background: The Limb-Body Wall Complex is a rare congenital anomaly with variations in the clinical presentation spectrum of malformations in the body wall associated with deformities in extremities and viscera.1 It occurs in 0.4 and 3.2 per 100,000 live births globally. The diagnostic is realized with a combination of two out of three criteria: toraco or abdominoquisis, exencefalia/encefalocele with facial hendidures and limb defects. Clinical Case Description: A newborn of 34 weeks of gestation is presented by assessment of the Capurro test. On physical examination, weighing 2480 grams, 43 cm in height, normocephalon with head circumference 32.5 cm, thoracic perimeter 25 cm, at cardiopulmonary auscultation, hypoventilated lungs, Low-pitched audible heart sounds, bradycardiac with omphalocele containing intestinal bowel and a segment of liver with adherence of 20% of its surface to the maternal face of the placenta with absent umbilical cord. Scoliosis and left hemibody pelvis with prominent bone structure are observed. Imperforate anus. Hypoplastic left lower extremities with clubfoot. The newborn died at 20 minutes of life due to heart failure, respiratory failure and multiple malformations. No genetic studies were carried out, nor autopsy due to the lack of economic resources. Conclusion: LBWC is an extremely rare congenital defect of the ventral abdominal wall with a poor prognosis in utero and at birth. In our clinical case, we present the first case of a 34-week-gestation newborn with the Limb-Body Wall complex reported scientific literature in Honduras.