title: Holocord hydrosyringomyelia with terminal myelocystocele revealed by constructive interference in steady-state MR imaging.
abstract: We report an operated case with terminal myelocystocele and holocord hydrosyringomyelia (syrinx). The patient exhibited a subcutaneous mass at the right lumbosacral region with multiple anomalies including scoliosis and hyperdactylia of the right foot and systemic disease such as hydronephroureter. Neurological examination revealed palsy of the left diaphragm, and left upper and right lower limbs. Constructive interference in steady-state (CISS) images demonstrated a terminal myelocystocele with a huge syrinx extending to the medulla. The curvilinear reconstruction of the CISS sequence depicted continuous cystocele and whole syrinx. At day 37, sac excision and untethering of the spinal cord were performed. The caudal part of the central canal was opened to the subarachnoid space (terminal ventriculostomy), resulting in a slight improvement of limb palsy and shrinkage of the holocord syrinx. CISS imaging is useful to detect the complicated pathology, and terminal ventriculostomy should be performed to improve associated syrinx.
authors.affiliation: Departments of Neurosurgery, Pediatrics and Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Fukuoka, Japan. khash@med.kyushu-u.ac.jp
authors.forename: Kimiaki
authors.initials: K
authors.lastname: Hashiguchi
authors.forename: Takato
authors.initials: T
authors.lastname: Morioka
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Samura
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Yoshida
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Miyagi
authors.forename: Shinji
authors.initials: S
authors.lastname: Nagata
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kokubo
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshiura
authors.forename: Tomio
authors.initials: T
authors.lastname: Sasaki
mesh_terms: D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008161:Lumbosacral Region / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008591:Meningomyelocele / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D013595:Syringomyelia / Q000150:complications / Q000175:diagnosis* / Q000601:surgery