CERVICAL MYELOCYSTOCELE WITH CHIARI-II MALFORMATION - MAGNETIC-RESONANCE-IMAGING AND SURGICAL-TREATMENT

BACKGROUND The myelocystocele is a rare clinical entity, and there hav e only been six case reports concerning the cervical myelocystocele, i ncluding ours. METHODS The case of a female neonate with cervical myel ocystocele, who presented with respiratory distress beginning at birth is reported. RESULTS Magnetic resonance (MR) imaging disclosed a cyst ic lesion surrounded by two layers of membrane, which were recognized to be the dural and arachnoid layer and the ependymal lining layer, re spectively, during operation and on histopathologic examination, Hydro myelia at C2-C7 with Chiari II malformation was also observed. After a thorough repair and untethering surgery, the hydromyelia was diminish ed on a follow-up MR examination, The patient's respiratory status nor malized several days postoperatively, and her growth and development w ere within normal limits at the 2-year follow-up. CONCLUSIONS In cases of cervical myelocystocele with Chiari II malformation, untethering t hrough intradural exploration to treat the tethered cord and outer dec ompression of the foramen magnum are needed. MR imaging is the best mo dality for preoperative determination of anatomic relationships. (C) 1 998 by Elsevier Science Inc.