Prenatal diagnosis of a case of pentalogy of cantrell with. All structured data from the file and property namespaces is available under the creative commons cc0 license. Successful atrial septal defect transcatheter closure in a. Cantrell syndrome is a very rare congenital disease associating five features. Figures 2 we studied 21 patients who presented with a diagnosis of pentalogy of cantrell. Although the pathogenesis of this syndrome is not well understood. Pentology of cantrell pc is a rare syndrome with unknown etiology presenting with pentad of midline, upper abdominal wall disorder, defect of lower sternum, anterior diaphragmatic defect, pericardial abnormality and congenital abnormalities of the heart as explained by toyama.
Pentalogy of cantrell pc is a rare congenital anomaly involving defects in the anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, and lower sternum, and other congenita. The first case was diagnosed prenatally at 15 weeks gestation, the earliest intrauterine finding in the literature. In this paper, we report a case of partial cantrells syndrome with left ventricular diverticulum, triatrial situs solitus, ventricular. Congenital heart disease, abdominal wall defect, pentalogy of cantrell. To summarize the experience and feasibility of radical onestage surgical repair of pentalogy of cantrell in. Read embryology, sternal clefts, ectopia cordis, and cantrells pentalogy, seminars in pediatric surgery on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Left ventricle diverticulum with partial cantrells syndrome. Pentalogy of cantrell is a rare syndrome consisting of a spectrum of five structural ventral midline defects, with the combination of an omphalocele and ectopia cordis being the hallmark of the disorder.
Pentalogy of cantrell is characterized by a combination of birth defects. Embryology, sternal clefts, ectopia cordis, and cantrells. Remote work advice from the largest allremote company. Cardiac malformations in patients with pentalogy of. Cooccurrence of severe goltzgorlin syndrome and pentalogy of. Files are available under licenses specified on their description page. Pentalogy of cantrell pc is a rare congenital anomaly involving defects in the anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, and lower sternum, and other congenital intracardiac abnormalities. Pentalogy of cantrell poc is a lethal multiple congenital anomalies syndrome, characterized by the presence of 5 major malformations. Left ventricular diverticulum is present in 2050 % of cases 3, 4. Misoprostol is a synthetic analogue of prostaglandin e1 and is used for effects such as uterotonic abortifacient. Pentalogy of cantrell pc is an extremely rare multiple congenital anomaly syndrome, characterized by the presence of five major malformations.
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