Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia

• Published in: Journal of neurosurgery. Pediatrics Vol. 11; no. 2; p. 154
• Main Authors: Sweeney, Kieron J, Caird, John, Sattar, Muhammad Taufiq, Allcutt, David, Crimmins, Darach
• Format: Journal Article
• Language: English
• Published: United States 01.02.2013
• Subjects: Analysis of Variance; Arnold-Chiari Malformation - diagnosis; Arnold-Chiari Malformation - diagnostic imaging; Arnold-Chiari Malformation - pathology; Cerebellum - diagnostic imaging; Cerebellum - pathology; Choristoma - diagnosis; Choristoma - diagnostic imaging; Cranial Fossa, Posterior - pathology; Cranial Fossa, Posterior - surgery; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Ireland; Linear Models; Magnetic Resonance Imaging; Male; Meningomyelocele - diagnosis; Meningomyelocele - diagnostic imaging; Meningomyelocele - pathology; Organ Size; Radiography; Spinal Cord - diagnostic imaging; Spinal Cord - pathology; Syringomyelia - diagnosis; Syringomyelia - diagnostic imaging
• ISSN: 1933-0715
• DOI: 10.3171/2012.10.PEDS12177

McLone and Knepper's unified theory of Chiari malformation Type II (CM-II) describes how the loss of CSF via the open posterior neuropore fails to create adequate distending pressure for the developing rhomboencephalic vesicle. The authors of the present article describe the relationship between the posterior fossa volume and intracranial cerebellar volume as being related to the distance from the obex of the fourth ventricle to the myelomeningocele lesion using a common mathematical model, the Hagen-Poiseuille law. All newborns who required closure of a myelomeningocele at the authors' institution between 2008 and 2011 and who were between 4 weeks premature and 2 months, corrected gestational age, at the time of MRI were included in this study. Volumes and measurements were obtained from axial and sagittal T2-weighted MR images of the brain and spine. A total of 56 newborn infants met the inclusion criteria. There was a direct linear relationship between both posterior fossa volume and cerebellar volume and the spinal level of the myelomeningocele lesion (p = 0.0012 and p = 0.0041, respectively). There was a negative linear relationship between the cerebellar descent, the spinal level of the lesion, and posterior fossa volume and cerebellar volume. These relationships strengthen in patients with no syringomyelia and are not significant in those groups with syringomyelia. The results of a 1-way ANOVA for the 3 groups did not reach significance. Using a linear equation derived from the Hagen-Poiseuille law that describes pressure in the fourth ventricle as being directly related to the length of the central canal from the obex to the myelomeningocele lesion, the authors were able to explain the directly observed linear relation between posterior fossa volume, intracranial cerebellar volume, and cerebellar descent to the level of the spinal lesion. As this model assumes a uniform radius of the central canal they were able to validate this model when they observed a strengthening in relationships in the no syringomyelia group and statistically insignificant correlations in the groups with syringomyelia. They therefore propose that the spinal level of the lesion is one of the major determinants of posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia.