Cervical myelocystocele with Chiari II malformation: magnetic resonance imaging and surgical treatment.

BACKGROUND

The myelocystocele is a rare clinical entity, and there have only been six case reports concerning the cervical myelocystocele, including ours.

METHODS

The case of a female neonate with cervical myelocystocele, who presented with respiratory distress beginning at birth is reported.

RESULTS

Magnetic resonance (MR) imaging disclosed a cystic lesion surrounded by two layers of membrane, which were recognized to be the dural and arachnoid layer and the ependymal lining layer, respectively, during operation and on histopathologic examination. Hydromyelia at C2-C7 with Chiari II malformation was also observed. After a thorough repair and untethering surgery, the hydromyelia was diminished on a follow-up MR examination. The patient's respiratory status normalized several days postoperatively, and her growth and development were within normal limits at the 2-year follow-up.

CONCLUSIONS

In cases of cervical myelocystocele with Chiari II malformation, untethering through intradural exploration to treat the tethered cord and outer decompression of the foramen magnum are needed. MR imaging is the best modality for preoperative determination of anatomic relationships.