Title: Incomplete Pentalogy of Cantrell with Craniorachischisis – A Rare Case Report

Authors: Dr Anjali Saji, Dr Aparna B Raj, Dr Nithya B G, Dr Nishitha T

 DOI: https://dx.doi.org/10.18535/jmscr/v7i10.160

Abstract

Cantrell’s Pentalogy is a rare congenital malformation consisting of supraumbilical abdominal defect, defect in lower part of sternum, agenesis of anterior portion of diaphragm, ectopiscordis and an intra cardiac defect. Hereby, we are reporting a case of Incomplete Pentalogy of Cantrell with, ectopiacordis, with absent pericardium, gastroschisis and Craniorachischisis.

Keywords: Pentalogy, Cantrell, ectopiacordis, Craniorachischisis.

References

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Corresponding Author

Dr Anjali Saji

Address: PG Ladies Hostel, M.K.C.G Medical College, Berhampur, Odisha, 760004, India