Kimiaki Hashiguchi1, Takato Morioka2, Nobuya Murakami3, Osamu Togao4, Akio Hiwatashi4, Masayuki Ochiai5, Goki Eriguchi6, Junji Kishimoto7, Koji Iihara8. 1. Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. khash@med.kyushu-u.ac.jp. 2. Department of Neurosurgery, Fukuoka Children's Hospital, Fukuoka, Japan. 3. Department of Neurosurgery, Iizuka Hospital, Iizuka, Japan. 4. Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. 5. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. 6. Data Center, Center for Clinical and Translational Research, Kyushu University, Fukuoka, Japan. 7. Department of Research and Development of Next Generation Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan. 8. Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Abstract
PURPOSE: To document long-term morphological changes of Chiari type II malformation (CM-II) following closure of spina bifida manifesta (SBM). METHODS: We retrospectively evaluated postnatal magnetic resonance images of the CM-II and posterior fossa (PF) in 28 consecutive cases. We measured changes in vertebral level and length of the cerebellar peg (CP), cerebrospinal fluid (CSF) spaces anterior and posterior to the cerebrospinal junction, PF area, and the anteroposterior diameters of the foramen magnum (FM) and C1 vertebra. We examined the morphological differences between the cases with and without ventriculoperitoneal (VP) shunting and derived predicted means by nonlinear mixed-effect modeling. RESULTS: At birth, there were significant differences in CP length, PF area, and FM and C1 diameters between those who underwent VP shunting and those who did not. In cases with a CP below C1, VP shunting was required in every case but one. In those with visible CSF space at birth, VP shunts were not required. In 17 of 18 cases with a CP below C1, the vertebral level ascended by mean two vertebral levels (range 0-5 levels) within 4-6 months of delivery. In the remaining case, slowly progressive hydrocephalus and delayed CP descent required VP shunting at 8 months. Predicted mean CP length and FM and C1 diameters were greater in those who underwent VP shunting, but there was no difference in predicted mean PF area. CONCLUSION: The morphology of CM-II and the presence of hydrocephalus influence each other in children who have undergone postnatal SBM repair.
PURPOSE: To document long-term morphological changes of Chiari type II malformation (CM-II) following closure of spina bifida manifesta (SBM). METHODS: We retrospectively evaluated postnatal magnetic resonance images of the CM-II and posterior fossa (PF) in 28 consecutive cases. We measured changes in vertebral level and length of the cerebellar peg (CP), cerebrospinal fluid (CSF) spaces anterior and posterior to the cerebrospinal junction, PF area, and the anteroposterior diameters of the foramen magnum (FM) and C1 vertebra. We examined the morphological differences between the cases with and without ventriculoperitoneal (VP) shunting and derived predicted means by nonlinear mixed-effect modeling. RESULTS: At birth, there were significant differences in CP length, PF area, and FM and C1 diameters between those who underwent VP shunting and those who did not. In cases with a CP below C1, VP shunting was required in every case but one. In those with visible CSF space at birth, VP shunts were not required. In 17 of 18 cases with a CP below C1, the vertebral level ascended by mean two vertebral levels (range 0-5 levels) within 4-6 months of delivery. In the remaining case, slowly progressive hydrocephalus and delayed CP descent required VP shunting at 8 months. Predicted mean CP length and FM and C1 diameters were greater in those who underwent VP shunting, but there was no difference in predicted mean PF area. CONCLUSION: The morphology of CM-II and the presence of hydrocephalus influence each other in children who have undergone postnatal SBM repair.
Authors: N Scott Adzick; Elizabeth A Thom; Catherine Y Spong; John W Brock; Pamela K Burrows; Mark P Johnson; Lori J Howell; Jody A Farrell; Mary E Dabrowiak; Leslie N Sutton; Nalin Gupta; Noel B Tulipan; Mary E D'Alton; Diana L Farmer Journal: N Engl J Med Date: 2011-02-09 Impact factor: 91.245