title: When Closure Fails: What the Radiologist Needs to Know About the Embryology, Anatomy, and Prenatal Imaging of Ventral Body Wall Defects.
abstract: Ventral body wall defects (VBWDs) are one of the main categories of human congenital malformations, representing a wide and heterogeneous group of defects sharing a common feature, that is, herniation of one or more viscera through a defect in the anterior body wall. Gastroschisis and omphalocele are the 2 most common congenital VBWDs. Other uncommon anomalies include ectopia cordis and pentalogy of Cantrell, limb-body wall complex, and bladder and cloacal exstrophy. Although VBWDs are associated with multiple abnormalities with distinct embryological origins and that may affect virtually any system organs, at least in relation to anterior body wall defects, they are thought (except for omphalocele) to share a common embryologic mechanism, that is, a failure involving the lateral body wall folds responsible for closing the thoracic, abdominal, and pelvic portions of the ventral body wall during the fourth week of development. Additionally, many of the principles of diagnosis and management are similar for these conditions. Fetal ultrasound (US) in prenatal care allows the diagnosis of most of such defects with subsequent opportunities for parental counseling and optimal perinatal management. Fetal magnetic resonance imaging may be an adjunct to US, providing global and detailed anatomical information, assessing the extent of defects, and also helping to confirm the diagnosis in equivocal cases. Prenatal imaging features of VBWDs may be complex and challenging, often requiring from the radiologist a high level of suspicion and familiarity with the imaging patterns. Because an appropriate management is dependent on an accurate diagnosis and assessment of defects, radiologists should be able to recognize and distinguish between the different VBWDs and their associated anomalies. In this article, we review the relevant embryology of VBWDs to facilitate understanding of the pathologic anatomy and diagnostic imaging approach. Features will be illustrated with prenatal US and magnetic resonance imaging and correlated with postnatal and clinical imaging.
authors.affiliation: Department of Radiology, Hospital de Base, São José do Rio Preto Medical School, São Paulo, Brazil. Electronic address: usantor@yahoo.com.br.
authors.forename: Ulysses S
authors.initials: US
authors.lastname: Torres
authors.affiliation: Department of Radiology, Hospital de Base, São José do Rio Preto Medical School, São Paulo, Brazil.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Portela-Oliveira
authors.affiliation: Department of Radiology, Hospital de Base, São José do Rio Preto Medical School, São Paulo, Brazil.
authors.forename: Fernanda Del Campo Braojos
authors.initials: Fdel C
authors.lastname: Braga
authors.affiliation: Clínica de Diagnóstico por Imagem, CDPI, Rio de Janeiro, Brazil.
authors.forename: Heron
authors.initials: H
authors.lastname: Werner
authors.affiliation: Clínica de Diagnóstico por Imagem, CDPI, Rio de Janeiro, Brazil.
authors.forename: Pedro Augusto Nascimento
authors.initials: PA
authors.lastname: Daltro
authors.affiliation: Department of Radiology, Hospital de Base, São José do Rio Preto Medical School, São Paulo, Brazil.
authors.forename: Antônio Soares
authors.initials: AS
authors.lastname: Souza
mesh_terms: D034861:Abdominal Wall / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D001746:Bladder Exstrophy / Q000175:diagnosis* / Q000196:embryology; D003937:Diagnosis, Differential; D054083:Ectopia Cordis / Q000175:diagnosis* / Q000196:embryology; D005260:Female; D020139:Gastroschisis / Q000175:diagnosis / Q000196:embryology; D006554:Hernia, Umbilical / Q000175:diagnosis / Q000196:embryology; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D016216:Ultrasonography, Prenatal / Q000379:methods*