Abstract | INTRODUCTION: We report about a spina bifida patient with myelomeningocele at the lumbar level L5, extensive Chiari malformation type II with vermal herniation reaching to C6 with downward pontine shift, and a severe hypoplastic cerebellum. Chromosomal diagnostic tests showed no abnormalities. CASE REPORT: The infant experienced severe central apneas successfully treated with oxygen therapy and caffeine medication; functional motor level was established at L5 with sparse anal sphincter function. DISCUSSION: After surgical intervention ( myelomeningocele repair and ventriculoperitoneal shunt placement), these abnormalities significantly improved on radiological imaging; the preoperative hypoplastic, almost undetectable, cerebellum developed to a fair sized cerebellum. Apneas disappeared over time and the patient showed further developmental improvement. Herein, we illustrate and discuss the changes of the cerebellar volume before and after neurosurgical intervention.
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Authors | Annick Kronenburg, Kuo Sen Han, Rob Gooskens, Giuseppe Esposito, Douglas Cochrane, Peter Woerdeman |
Journal | Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
(Childs Nerv Syst)
Vol. 29
Issue 11
Pg. 2131-5
(Nov 2013)
ISSN: 1433-0350 [Electronic] Germany |
PMID | 23686389
(Publication Type: Journal Article)
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Topics |
- Arnold-Chiari Malformation
(pathology, surgery)
- Cerebellum
(abnormalities, pathology, surgery)
- Developmental Disabilities
(pathology, surgery)
- Female
- Gestational Age
- Humans
- Infant, Newborn
- Magnetic Resonance Imaging
- Meningomyelocele
(pathology, surgery)
- Microcephaly
(pathology)
- Nervous System Malformations
(pathology, surgery)
- Neural Tube Defects
(pathology, surgery)
- Neurosurgical Procedures
(methods)
- Spinal Dysraphism
(pathology, surgery)
- Treatment Outcome
- Ultrasonography, Prenatal
- Ventriculoperitoneal Shunt
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