Is there a causal relationship between open spinal dysraphism and Chiari II deformity? A study using in utero magnetic resonance imaging of the fetus.

BACKGROUND Chiari II deformity is associated with open spinal dysraphism. A causal relationship has been proposed by McLone and Knepper. This article evaluates that hypothesis. OBJECTIVE To establish the frequency of Chiari II deformity in fetuses with open spinal dysraphism, assess whether meningocele sac neck area and volume influence the severity of posterior fossa changes, and assess whether the severity of associated findings (ventriculomegaly, amount of extracerebral CSF) are associated with Chiari II deformity. METHODS Sixty-five fetuses with open spinal dysraphism were compared with gestationally aged matched "normal" fetuses on ultrafast MR images. Cerebellar vermis and bony posterior fossa surface area were measured on midline sagittal images. Hindbrain herniation was noted if present. In the open spinal dysraphic group, sac neck area and volume were measured. Ventriculomegaly was assessed by linear measurement of the trigone of the lateral ventricle and extracerebral CSF depth was measured maximally over the lateral surface of the cerebral hemispheres. RESULTS Fifteen of 65 fetuses with open spinal dysraphism did not have Chiari II deformity. Neck area and volume of the sac did not correlate with the presence of Chiari II deformity or reduction in bony posterior fossa size. CONCLUSION A relatively high proportion of fetuses with open spinal dysraphism do not have Chiari II deformity in utero. There is a lack of correlation between indicators of spinal dysraphism severity and the extent of the posterior fossa abnormality. This raises some interesting questions about the causality of the Chiari II deformity.