DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20164620

Pentalogy of cantrell with single umbilical artery: a rare fetal anomaly

Raj Kumar Meena, Veena Meena

Abstract


Pentalogy of cantrell (PC) is a rare syndrome having both specific ventral and dorsal midline defect, and rarely found to be associated with other anomalies. Most cases occur randomly for no apparent reason (sporadically). We report a 20 years old primigravida referred at 17 weeks 4 days of gestational age, for a fetal anomaly scan having an anterior abdominal wall defect in fetus. Ultrasound done, showed single live fetus gestational age of 18 weeks, with fetal heart, great vessels, stomach, and intestinal loops were found to be lying outside thoracic cavity. Fetal autopsy following termination of the pregnancy confirmed the presence of the malformations with single umbilical artery. Occurrence of both ventral and dorsal midline defects suggests that the timing of fetus insult would be between 14 to 18 days after conception when the splanchnic mesoderm (myocardium) and somatic mesoderm (sternum, abdominal wall, diaphragm and pericardium) is dividing.


Keywords


Ectopic cordis, Pentalogy of cantrell, Single umbilical artery

Full Text:

PDF

References


Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 1958;107(5):602-14.

McMahon CJ, Taylor MD, Cassady CI, Olutoye OO, Bezold LI. Diagnosis of pentalogy of Cantrell in the fetus using magnetic resonance imaging and ultrasound. Pediatr Cardiol. 2007;28:172-5.

Grethel EJ, Hornberger LK, Farmer DL. Prenatal and postnatal management of a patient with pentalogy of cantrell and left ventricular aneurysm. Fetal Diagn Ther. 2007;22:269-73.

Carmi R, Boughman JA. Pentalogy of Cantrell and associated midline anomalies: a possible ventral midline developmental field. Am J Med Genet. 1992;42(1):90-5.

Oka T, Shiraishi I, Iwasaki N, Itoi T, Hamaoka K. Usefulness of helical CT angiography and MRI in the diagnosis and treatment of pentalogy of Cantrell. J Pediatr. 2003;142:84.

Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: a case report and review of the syndrome. Pediatrics. 1972;50:778-92.

Fernández MS, López A, Vila JJ, Lluna J, Miranda J. Cantrell’s pentalogy. Report of four cases and their management. Pediatr Surg Int. 1997;12:428-31.