Prenatal diagnosis of pentalogy of Cantrell in three cases, two with craniorachischisis

In this report, we describe 3 cases of pentalogy of Cantrell diagnosed prenatally with sonography. All 3 fetuses had an omphalocele and ectopia cordis; 2 of them also had craniorachischisis, in which the open cranial defect (anencephaly or exencephaly) is continuous with the completely open spine (s...

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Bibliographic Details
Published in:Journal of clinical ultrasound 2005-07, Vol.33 (6), p.308-311
Main Authors: Polat, Ibrahim, Gül, Ahmet, Aslan, Halil, Cebeci, Altan, Ozseker, Bülent, Caglar, Bahar, Ceylan, Yavuz
Format: Article
Language:English
Subjects:
Abdominal Wall - abnormalities
Abdominal Wall - diagnostic imaging
Abnormalities, Multiple - diagnosis
Abortion, Eugenic - methods
Adult
craniorachischisis
Diaphragm - abnormalities
Diaphragm - diagnostic imaging
Female
Heart Defects, Congenital - complications
Heart Defects, Congenital - diagnosis
Hernia, Umbilical - complications
Humans
Magnetic Resonance Imaging - methods
Neural Tube Defects - complications
Neural Tube Defects - diagnosis
Pentalogy of Cantrell
Pericardium - abnormalities
Pericardium - diagnostic imaging
Polyhydramnios
Pregnancy
prenatal diagnosis
Prostaglandins - administration & dosage
Sternum - abnormalities
Sternum - diagnostic imaging
Syndrome
ultrasonography
Ultrasonography, Prenatal - methods
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Summary:In this report, we describe 3 cases of pentalogy of Cantrell diagnosed prenatally with sonography. All 3 fetuses had an omphalocele and ectopia cordis; 2 of them also had craniorachischisis, in which the open cranial defect (anencephaly or exencephaly) is continuous with the completely open spine (spinal dysraphism). The association of the pentalogy of Cantrell with exencephaly is rare, and to our knowledge, the association of this syndrome with craniorachischisis has not been reported previously. In addition, 2 of our cases had clubfoot, and 1 of them also had clubhand, neither of which has been reported previously in association with the pentalogy of Cantrell. Finally, polyhydramnios, which occurred in 2 of our cases, has been reported to occur in association with that syndrome in only 1 previous case. We believe that sonographers should be aware that these anomalies may occur simultaneously with the classic anomalies of the pentalogy of Cantrell. © 2005 Wiley Periodicals, Inc. J Clin Ultrasound 33:308–311, 2005
ISSN:0091-2751
1097-0096
DOI:10.1002/jcu.20134