Cervical (non-terminal) myelocystocele associated with rapidly progressive hydrocephalus and Chiari type II...

An infant presented with a rare cervical (non-terminal) myelocystocele as a congenital skin-covered mass located in the midline of the posterior aspect of her neck. Magnetic resonance (MR) imaging and computed tomography showed a cystic mass filled with cerebrospinal fluid in the midline of the posterior aspect of the neck, with a fibrous streak extending from the bottom of the sac to the dorsal surface of the cervical cord. Brain MR imaging also showed a dilated ventricular system and Chiari type II malformation. The patient underwent plastic repair of the lesion, which was diagnosed as myelocystocele. After the surgery, the patient experienced respiratory distress. Ultrasound tomography from the anterior fontanel revealed deterioration of hydrocephalus, so a ventriculoperitoneal shunt was inserted, and the respiratory distress improved. The present case illustrates the possibility of rapidly worsening of hydrocephalus and Chari type II malformation after surgical repair of cervical (non-terminal) myelocystocele.