T1 pre-gad T1 pre-gad T2

Diagnosis: Chiari II malformation, cervical encephalocele, teratoma and syrinx

Chiari II malformation is characterized by a small posterior fossa with variable hypoplasia and ectopy of cerebellar tonsils and vermis. Other findings include a so-called Luckenschadel skull, beaking of the tectum, fenestration of the falx, gray matter heterotopia, variable dysgenesis of the corpus callosum, spinal segmentation anomalies, and diastematomyelia. Hydrocephalus and or cord syrinx is present in over 90% . Myelomeningocele is present in 100% of Chiari II malformations. It is believed that Chiari II malformation is secondary to the myelomeningocele since the presence of the myelomeningocele reduces the cerebral spinal fluid pressure necessary to normally form the posterior fossa. Almost all myelomeningoceles associated with Chiari II malformation are in the lumbar spine with a very small minority occurring in the cervical region. Teratoma of the spinal cord is an unusual lesion with most occurring in the sacrococcygeal region.

At surgery in this case, the dura was opened and dysplastic appearing nervous tissue was seen extending into the sac attached to the spinal cord. At pathology, some of the intradural tissue removed was consistent with a mature teratoma which contained glial, neural, peripheral ganglionic, chondroid, and adipose tissue elements. Related Cases

Hamabuchi M, Hasegawa R, Murase T. Teratoma of the spinal cord. A case report with CT scans. J Bone Joint Surg, May 1989; 71(3):p390-2.

Chiari I Dandy-Walker Chiari II