title: Pentalogy of Cantrell with Unilateral Kidney Evisceration: A Case Report and Review of Literature.
abstract: Pentalogy of Cantrell (PC) is a congenital malformation syndrome characterized by midline thoracoabdominal wall defect resulting from defective development in the septum transversum. Major hallmarks of this rare anomaly are omphalocele and ectopic heart. In most cases, the diagnosis is made by two-dimensional ultrasound in second trimester. The prognosis of PC relies on the presence and severity of cardiac anomalies but in most cases outcome is fatal. To the best of our knowledge, the presence of kidneys into the omphalocele in Cantrells' pentalogy has not been reported yet. In this article, we report a case of PC associated with unilateral kidney evisceration.
authors.affiliation: a University Department of Obstetrics and Gynecology.
authors.forename: Vedran
authors.initials: V
authors.lastname: Madžarac
authors.affiliation: a University Department of Obstetrics and Gynecology.
authors.forename: Ratko
authors.initials: R
authors.lastname: Matijević
authors.affiliation: b University Department of Pathology , University Hospital Merkur , Zagreb , Croatia.
authors.forename: Anita
authors.initials: A
authors.lastname: Škrtić
authors.affiliation: a University Department of Obstetrics and Gynecology.
authors.forename: Željko
authors.initials: Ž
authors.lastname: Duić
authors.affiliation: a University Department of Obstetrics and Gynecology.
authors.forename: Nikola
authors.initials: N
authors.lastname: Fistonić
authors.affiliation: a University Department of Obstetrics and Gynecology.
authors.forename: Jasenka Zmijanac
authors.initials: JZ
authors.lastname: Partl
mesh_terms: D034861:Abdominal Wall / Q000473:pathology; D000328:Adult; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000473:pathology*; D006554:Hernia, Umbilical / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007674:Kidney Diseases / Q000473:pathology*; D058502:Pentalogy of Cantrell / Q000175:diagnosis / Q000473:pathology*; D011296:Prenatal Diagnosis / Q000379:methods; D011379:Prognosis; D016216:Ultrasonography, Prenatal
keywords: ectopic heart; kidney evisceration; pentalogy of Cantrell