Pentalogy of Cantrell with Craniorachischisis: MRI and Ultrasonography Findings

Summary

We report a case of pentalogy of Cantrell associated with craniorachischisis diagnosed in utero using a combination of ultrasonography and MRI. Fetus at 14 weeks of gestation had gastroschisis, ectopia cordis and craniorachischisis. Craniorachischisis is a defect in which the open cranial defect (anencephaly or exencephaly) is continuous with the completely open spine (spinal dysraphism). Sonography clearly demonstrates anencephaly or exencephaly, extensive dysraphism and ectopia cordis in the early weeks of gestation. MRI usually does not provide any additional information about anomalies particularly in the first trimester compared to ultrasonography. In our opinion MRI is not necessary for the diagnosis of pentalogy of Cantrell in the early weeks of gestation.