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

  • Paediatric Neuroradiology
  • Published:
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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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Authors and Affiliations

  1. Polo scienze delle immagini, di laboratorio ed infettivologiche, Area diagnostica per immagini, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, L.go F. Vito 1, 00168, Roma, Italia

    Rosalinda Calandrelli, Marco Panfili, Gabriella D’Apolito, Alessandro Pedicelli & Cesare Colosimo

  2. Polo scienze della salute della donna e del bambino, Area salute del bambino, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, L.go F. Vito 1, 00168, Roma, Italia

    Giuseppe Zampino

  3. Polo scienze dell’invecchiamento, neurologiche, ortopediche e della testa-collo, Area neuroscienze, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, L.go F. Vito 1, 00168, Roma, Italia

    Fabio Pilato

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  1. Rosalinda Calandrelli
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Correspondence to Rosalinda Calandrelli.

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No funding was received for this study.

Conflict of interest

CC consults for Bracco Diagnostics Inc. and Bayer HealthCare.

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All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Calandrelli, R., Panfili, M., D’Apolito, G. et al. Quantitative approach to the posterior cranial fossa and craniocervical junction in asymptomatic children with achondroplasia. Neuroradiology 59, 1031–1041 (2017). https://doi.org/10.1007/s00234-017-1887-y

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  • DOI: https://doi.org/10.1007/s00234-017-1887-y

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