Rarer Expression of Pentalogy of Cantrell with Dandy Walker Malformation and Single Umbilical Artery

International Journal of Research in Health Sciences,2015,3,1,205-208.
Published:January 2015
Type:Case Report
Author(s) affiliations:

Radha Rani K1, Krishnaveni N2, Sarada B3, Obuleswara Prasad P4

1Associate Professor; 2Professor and Head; 3Assistant Professor; 4Senior Resident,  Department of Radio diagnosis, Government Medical College, Anantapuramu, A.P., INDIA.


Pentalogy of Cantrell (Thoraco abdominal ectopia cordis) is a rare congenital malformation syndrome consisting of a specific combination of ventral midline defects involving supraumbilical anterior abdominal wall, anterior diaphragm, diaphragmatic pericardium and sternum which can leads to various congenital anomalies like omphalocele and Ectopia cordis. Less than 90 cases have been reported in the literature. Here we report a case of incomplete Pentalogy of Cantrell with two rarer expressions of this disorder, Dandy walker malformation and 2 vessel cord which was not described in literature till now. Rarity of this entity is reason for presentation of this case report.

Ultrasound: moderate hydrocephalus, empty posterior fossa and non visualization of Cerebellar hemispheres and vermis