Tags

Type your tag names separated by a space and hit enter

Costello syndrome: Analysis of the posterior cranial fossa in children with posterior fossa crowding.
Neuroradiol J. 2015 Jun; 28(3):254-8.NJ

Abstract

This study aimed to assess changes in the posterior cranial fossa (PCF) to shed light on the mechanism of cerebellar herniation in children with Costello syndrome (CS) and posterior fossa crowding. We performed a morphovolumetric PCF analysis on brain magnetic resonance imaging (MRI) in seven children with CS (mean age 31 ± 16 months) comparing the MRI scans with those of seven age-matched healthy subjects.PCF volume (PCFV), PCF brain volume (PCFBV) and cerebellar volume (CeV) were assessed on axial T2-weighted MRI. Morphometric parameters (diameters of the foramen magnum, tentorial angle, basiocciput, supraocciput, basisphenoid and exocciput lengths) were measured on sagittal T1-weighted MRI. The volume of the cerebrospinal fluid (CSF) spaces was calculated as PCFV minus PCFBV.Five out of seven CS children showed tonsillar herniation in the upper cervical canal; no child had hydrocephalus but three out of seven children showed ventriculomegaly. In addition, the PCFV/PCFBV ratio, PCFV, CSF spaces volume, basiocciput, basisphenoid and exocciput lengths and latero-lateral and antero-posterior diameters of the foramen magnum were significantly reduced, whereas no significant changes were found in supraocciput length, PCFBV, CeV or hindbrain volume. The volumetric reduction of the PCF due to bony posterior fossa hypoplasia is a predisposing factor for developing cerebellar tonsillar herniation through the foramen magnum in children with CS. The altered anatomy of the foramen magnum and upward expansion of the PCF secondary to an increased tentorial slope serves to explain the possible mechanism of cerebellar herniation in patients with CS.

Authors+Show Affiliations

Institute of Radiology, Università Cattolica Sacro Cuore, Rome, Italy rosalinda.calandrelli@rm.unicatt.it.Institute of Radiology, Università Cattolica Sacro Cuore, Rome, Italy.Institute of Radiology, Università Cattolica Sacro Cuore, Rome, Italy.Department of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.Institute of Radiology, Università Cattolica Sacro Cuore, Rome, Italy.Institute of Radiology, Università Cattolica Sacro Cuore, Rome, Italy.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

26246091

Citation

Calandrelli, Rosalinda, et al. "Costello Syndrome: Analysis of the Posterior Cranial Fossa in Children With Posterior Fossa Crowding." The Neuroradiology Journal, vol. 28, no. 3, 2015, pp. 254-8.
Calandrelli R, D'Apolito G, Marco P, et al. Costello syndrome: Analysis of the posterior cranial fossa in children with posterior fossa crowding. Neuroradiol J. 2015;28(3):254-8.
Calandrelli, R., D'Apolito, G., Marco, P., Zampino, G., Tartaglione, T., & Colosimo, C. (2015). Costello syndrome: Analysis of the posterior cranial fossa in children with posterior fossa crowding. The Neuroradiology Journal, 28(3), 254-8. https://doi.org/10.1177/1971400915592549
Calandrelli R, et al. Costello Syndrome: Analysis of the Posterior Cranial Fossa in Children With Posterior Fossa Crowding. Neuroradiol J. 2015;28(3):254-8. PubMed PMID: 26246091.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Costello syndrome: Analysis of the posterior cranial fossa in children with posterior fossa crowding. AU - Calandrelli,Rosalinda, AU - D'Apolito,Gabriella, AU - Marco,Panfili, AU - Zampino,Giuseppe, AU - Tartaglione,Tommaso, AU - Colosimo,Cesare, PY - 2015/8/7/entrez PY - 2015/8/8/pubmed PY - 2015/10/31/medline KW - Chiari anomaly KW - Magnetic resonance imaging KW - posterior cranial fossa SP - 254 EP - 8 JF - The neuroradiology journal JO - Neuroradiol J VL - 28 IS - 3 N2 - This study aimed to assess changes in the posterior cranial fossa (PCF) to shed light on the mechanism of cerebellar herniation in children with Costello syndrome (CS) and posterior fossa crowding. We performed a morphovolumetric PCF analysis on brain magnetic resonance imaging (MRI) in seven children with CS (mean age 31 ± 16 months) comparing the MRI scans with those of seven age-matched healthy subjects.PCF volume (PCFV), PCF brain volume (PCFBV) and cerebellar volume (CeV) were assessed on axial T2-weighted MRI. Morphometric parameters (diameters of the foramen magnum, tentorial angle, basiocciput, supraocciput, basisphenoid and exocciput lengths) were measured on sagittal T1-weighted MRI. The volume of the cerebrospinal fluid (CSF) spaces was calculated as PCFV minus PCFBV.Five out of seven CS children showed tonsillar herniation in the upper cervical canal; no child had hydrocephalus but three out of seven children showed ventriculomegaly. In addition, the PCFV/PCFBV ratio, PCFV, CSF spaces volume, basiocciput, basisphenoid and exocciput lengths and latero-lateral and antero-posterior diameters of the foramen magnum were significantly reduced, whereas no significant changes were found in supraocciput length, PCFBV, CeV or hindbrain volume. The volumetric reduction of the PCF due to bony posterior fossa hypoplasia is a predisposing factor for developing cerebellar tonsillar herniation through the foramen magnum in children with CS. The altered anatomy of the foramen magnum and upward expansion of the PCF secondary to an increased tentorial slope serves to explain the possible mechanism of cerebellar herniation in patients with CS. SN - 1971-4009 UR - https://www.unboundmedicine.com/medline/citation/26246091/Costello_syndrome:_Analysis_of_the_posterior_cranial_fossa_in_children_with_posterior_fossa_crowding_ L2 - https://journals.sagepub.com/doi/10.1177/1971400915592549?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -