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01-10-2017 | Paediatric Neuroradiology

Quantitative approach to the posterior cranial fossa and craniocervical junction in asymptomatic children with achondroplasia

Authors: Rosalinda Calandrelli, Marco Panfili, Gabriella D’Apolito, Giuseppe Zampino, Alessandro Pedicelli, Fabio Pilato, Cesare Colosimo

Published in: Neuroradiology | Issue 10/2017

Abstract

Purpose

We propose an magnetic resonance imaging (MRI)-based quantitative morphovolumetric approach to the posterior cranial fossa (PCF) and craniocervical junction (CCJ) changes in achondroplastic patients investigating possible associations with ventriculomegaly and medullary compression.

Methods

We analyzed MRI of 13 achondroplastic children not treated by surgery. 3D FSPGR T1-weighted images were used to analyze (1) PCF synchondroses; (2) PCF volume (PCFV), PCF brain volume (PCFBV), PCFV/PCFBV ratio, cerebellar volume, cerebrospinal fluid (CSF) spaces volume, and IV ventricle volume; (3) PCF (clivus, supraocciput, exocciput lengths, tentorial angle) and CCJ (AP and LL diameters of foramen magnum (FM)) morphometry; (4) measurements of FM and jugular foramina (JF) areas; and (5) supratentorial ventricular volume.

Results

All patients showed synostosis of spheno-occipital synchondroses, eight showed synostosis of intra-occipital synchondroses, nine showed CCJ impingement on the cervical cord but only three had cervical myelopathy. Compared to controls, clivus and exocciput lengths, LL and AP diameters of FM, FM area and JF area were significantly reduced, supraocciput length, tentorial angle, PCFV, PCFBV, cerebellar volume, supratentorial ventricular system volume were significantly increased. A correlation was found between clivus length and supratentorial ventricular volume, premature closure of intra-occipital synchondroses and FM area while a trend was found between FM area and supraocciput length.

Conclusion

Our analysis demonstrates a relationship between the shortening of the clivus and the ventriculomegaly. On the other hand the premature closure of PCF synchondroses, the shape, and the growth direction of supraocciput bone contribute to reduce the FM area, causing in some patients medullary compression.

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Title: Quantitative approach to the posterior cranial fossa and craniocervical junction in asymptomatic children with achondroplasia
Authors: Rosalinda Calandrelli
Marco Panfili
Gabriella D’Apolito
Giuseppe Zampino
Alessandro Pedicelli
Fabio Pilato
Cesare Colosimo
Publication date: 01-10-2017
Publisher: Springer Berlin Heidelberg
Published in: Neuroradiology / Issue 10/2017
Print ISSN: 0028-3940
Electronic ISSN: 1432-1920
DOI: https://doi.org/10.1007/s00234-017-1887-y

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Springer Medicine

Quantitative approach to the posterior cranial fossa and craniocervical junction in asymptomatic children with achondroplasia

Abstract

Purpose

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

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