[Pentalogy of Cantrell: six new cases].
Cantrell's pentalogy is a rare multiple malformation syndrome consisting of supraumbilical abdominal wall defect, sternal defect, pericardial defect, anterior diaphragmatic defect and heart malformation.


OBJECTIVE
Prenatal ultrasound findings and malformations described during autopsy of the Cantrell's pentalogy cases diagnosed between January 1991 and June 2009 in our institute were reviewed. A literature research was conducted to analyze the prevalence and prenatal detection rate of the five previously described malformations and ectopia cordis in the Cantrell's pentalogy cases.


RESULTS
Six cases of Cantrell's pentalogy were diagnosed during the study period in our department. Sonography detected multiple malformations in 3 cases, and isolated malformation in 3 cases. Analyzing the data of 49 Cantrell's pentalogy cases altogether showed that, beside abdominal wall defect and ectopia cordis which had the highest prenatal detection rate (83% and 91% with a prevalence of 94% and 69%, respectively), sternal defect and anterior diaphragmatic hernia were also present in a large number of the cases (80% and 73% respectively).


CONCLUSIONS
Sonographic identification of the sternal defect or diaphragmatic hernia may help to differentiate Cantrell's pentalogy from malformations part of the syndrome but occurring as isolated defects.
Pete
Barbara
B
Semmelweis Egyetem, Altalános Orvostudományi Kar I. Szülészeti és Nogyógyászati Klinika, Budapest. barbipete@hotmail.com
Hajdú
Júlia
J
Harmath
Agnes
A
Csapó
Zsolt
Z
Papp
Csaba
C
Szigeti
Zsanett
Z
Rigó
János
J
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000000981:diagnostic imaging; D000029:Abortion, Legal; D001344:Autopsy; D054083:Ectopia Cordis / Q000175:diagnosis; D005260:Female; D020139:Gastroschisis / Q000175:diagnosis; D006330:Heart Defects, Congenital / Q000175:diagnosis; D006548:Hernia, Diaphragmatic / Q000175:diagnosis; D006801:Humans; D008297:Male; D011296:Prenatal Diagnosis; D016216:Ultrasonography, Prenatal