{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T13:43:00Z","timestamp":1788183780573,"version":"build-2803163510"},"reference-count":26,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2019,10,6]],"date-time":"2019-10-06T00:00:00Z","timestamp":1570320000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Pentalogy of Cantrell (PC) is a malformation characterized by defects in the ventral abdominal wall, lower sternum, diaphragmatic pericardium, anterior diaphragm associated with omphalocele, thoraco-abdominal ectopia cordis, diaphragmatic hernia, and intracardiac abnormalities. PC is stratified as complete or incomplete and we present both the complete and incomplete forms.<\/jats:p>","DOI":"10.3390\/children6100109","type":"journal-article","created":{"date-parts":[[2019,10,7]],"date-time":"2019-10-07T03:34:01Z","timestamp":1570419241000},"page":"109","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Complete and Incomplete Pentalogy of Cantrell"],"prefix":"10.3390","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9666-6883","authenticated-orcid":false,"given":"Ranjit I.","family":"Kylat","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Arizona, College of Medicine, PO BOX 245073, 1501 N Campbell Avenue, Tucson, AZ 85724, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,10,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1002\/ajmg.1320420118","article-title":"Pentalogy of Cantrell and associated midline anomalies: A possible ventral midline developmental field","volume":"42","author":"Carmi","year":"1992","journal-title":"Am. J. Med. Genet."},{"key":"ref_2","first-page":"602","article-title":"A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart","volume":"107","author":"Cantrell","year":"1958","journal-title":"Surg. Gynecol. Obstet."},{"key":"ref_3","first-page":"778","article-title":"Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome","volume":"50","author":"Toyama","year":"1972","journal-title":"Pediatrics"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"201","DOI":"10.3389\/fped.2018.00201","article-title":"Cantrell syndrome-A rare complex congenital anomaly: A case report and literature review","volume":"6","author":"Gozar","year":"2018","journal-title":"Front. Pediatr."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1053\/j.sult.2015.01.001","article-title":"When closure fails: What the radiologist needs to know about the embryology, anatomy, and prenatal imaging of ventral body wall defects","volume":"36","author":"Torres","year":"2015","journal-title":"Semin. Ultrasound. CT MR"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1053\/j.sempedsurg.2008.03.004","article-title":"Embryology, sternal clefts, ectopia cordis, and Cantrell\u2019s pentalogy","volume":"17","author":"Engum","year":"2008","journal-title":"Semin. Pediatr. Surg."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"E129","DOI":"10.1111\/chd.12101","article-title":"Pentalogy of Cantrell with ectopia cordis totalis, total anomalous pulmonary venous connection, and tetralogy of Fallot: A case report and review of the literature","volume":"9","author":"Restrepo","year":"2014","journal-title":"Congenit. Heart Dis."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1111\/j.1747-0803.2009.00375.x","article-title":"Pentalogy of Cantrell with left ventricular diverticulum: A case report and review of literature","volume":"5","author":"Singh","year":"2010","journal-title":"Congenit. Heart Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1177\/2150135110390528","article-title":"Pentalogy of Cantrell: Forty-two years of experience in the Hospital Infantil de Mexico Federico Gomez","volume":"2","year":"2011","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1007\/s00246-013-0706-4","article-title":"Spectrum of Cantrell\u2019s pentalogy: Case series from a single tertiary care center and review of the literature","volume":"34","author":"Mallula","year":"2013","journal-title":"Pediatr. Cardiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e205","DOI":"10.1016\/j.athoracsur.2019.02.050","article-title":"Congenital left ventricular diverticulum in Pentalogy of Cantrell: Puzzle solved on dual source CT","volume":"108","author":"Ojha","year":"2019","journal-title":"Ann. Thorac. Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1186\/1752-1947-7-287","article-title":"Duplication of the ALDH1A2 gene in association with pentalogy of Cantrell: A case report","volume":"7","author":"Steiner","year":"2013","journal-title":"J. Med. Case Rep."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1002\/humu.21505","article-title":"Mutation update for the PORCN gene","volume":"32","author":"Lombardi","year":"2011","journal-title":"Hum. Mutat."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1002\/ajmg.1320490416","article-title":"Linkage localization of the thoraco-abdominal syndrome (TAS) gene to Xq25-26","volume":"49","author":"Parvari","year":"1994","journal-title":"Am. J. Med. Genet."},{"key":"ref_15","unstructured":"MacTaggart, B., Bowen, C., Markowitz, M., Chong, J., Bamshad, M., Ma, X., and Adelstein, R.S. (2016, January 14). Using whole-exome sequencing to identify genetic variants in patients diagnosed with pentalogy of Cantrell. Proceedings of the 30th Annual Showcase of NIH Intramural Research, Bethesda, MD, USA."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"431","DOI":"10.3109\/15513815.2011.587500","article-title":"Craniorachischisis with a variant of pentalogy of Cantrell, with lung extrophy","volume":"30","author":"Atis","year":"2011","journal-title":"Fetal Pediatr. Pathol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1002\/ajmg.a.33895","article-title":"Co-occurrence of severe Goltz-Gorlin syndrome and pentalogy of Cantrell\u2014Case report and review of the literature","volume":"155","author":"Smigiel","year":"2011","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"43","DOI":"10.3109\/15513815.2015.1122123","article-title":"Pentalogy of Cantrell with unilateral kidney evisceration: A case report and review of literature","volume":"35","author":"Partl","year":"2016","journal-title":"Fetal Pediatr. Pathol."},{"key":"ref_19","first-page":"911","article-title":"Multidisciplinary approach of assessing malformed fetuses exemplified in a rare case of pentalogy of Cantrell associated with craniorachischisis, pulmonary extrophy and right-sided aortic arch with aberrant brachiocephalic artery","volume":"59","author":"Albu","year":"2018","journal-title":"Rom. J. Morphol. Embryol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1007\/s00247-017-3914-x","article-title":"Fetal anterior abdominal wall defects: Prenatal imaging by magnetic resonance imaging","volume":"48","author":"Victoria","year":"2018","journal-title":"Pediatr. Radiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1080\/15513815.2018.1556367","article-title":"Prenatal diagnosis and management of ectopia cordis: Varied presentation spectrum","volume":"38","author":"Erturk","year":"2019","journal-title":"Fetal Pediatr. Pathol."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Naburi, H., Assenga, E., Patel, S., Massawe, A., and Manji, K. (2015). Class II pentalogy of Cantrell. BMC Res. Notes, 29.","DOI":"10.1186\/s13104-015-1293-7"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s00431-007-0578-9","article-title":"Pentalogy of Cantrell: Two patients and a review to determine prognostic factors for optimal approach","volume":"167","author":"Moonen","year":"2008","journal-title":"Eur. J. Pediatr."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/S0003-4975(00)01388-6","article-title":"Ectopia cordis and other midline defects","volume":"70","author":"Morales","year":"2000","journal-title":"Ann. Thorac. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"77","DOI":"10.5999\/aps.2014.41.1.77","article-title":"Repair of inferior sternal cleft using bilateral sternal bar turnover flaps in a patient with pentalogy of Cantrell","volume":"41","author":"Chia","year":"2014","journal-title":"Arch. Plast. Surg."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e1567","DOI":"10.1097\/GOX.0000000000001567","article-title":"Reconstruction of congenital sternal clefts: Surgical experience and literature review","volume":"5","author":"Alshomer","year":"2017","journal-title":"Plast. Reconstr. Surg. Glob. Open"}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/6\/10\/109\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:27:56Z","timestamp":1760189276000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/6\/10\/109"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,6]]},"references-count":26,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2019,10]]}},"alternative-id":["children6100109"],"URL":"https:\/\/doi.org\/10.3390\/children6100109","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,6]]}}}