Chiari Malformation Type III: A Case Report and Review of Literature

Chiari malformations (CMs) are a group of disorders involving deformities of the posterior fossa and hindbrain. There are seven types of CMs: 0, I, 1.5, II, III, IV, and V. CMIII is a very infrequent disorder characterized by low occipital or superior cervical encephalocele and inferior displacement of the brainstem. Here we present a unique case of CMIII associated with cleft palate, abnormal midbrain, and abnormal corpus callosum. CMIII is a very rare condition, which needs to be reported. This study primarily aims to compare our case to other cases of CMIII. We did not find another case with cleft palate and CMIII. There are only a few cases reported of CMIII. That is why it is vital to report each of these cases. Among reported CMIII cases, our case is unique regarding the co-occurrence of cleft palate. There seems to be an embryological link between these two conditions. However, cleft palate is a relatively common congenital defect, which means that the co-occurrence could be just a coincidence as well. Further research is warranted to broaden the information of this extremely rare syndrome.