Chiari malformation type III treated with primary closure

Chiari malformations represent various clinical and anatomic processes that entail varying degrees of involvement of the rhombencephalon (hindbrain). The type III Chiari malformation is distinctly rare. Chiari described only one case of his type III deformity, which is indicative of its rarity. We report a case of Chiari malformation type III, a female child who presented to us at the age of 1 month with a swelling at the back of the neck since birth. MRI revealed a Chiari type III malformation with a high cervical meningoencephalocele along with herniation of cerebellar tissue through the bony defect in the posterior elements of the C2 vertebra. At surgery, the meningoencephalocele was excised along with the gliotic cerebellar tissue, and a posterior fossa decompression along with augmentation duraplasty was done. The postoperative period was uneventful. The case and relevant literature is reviewed.