Quantitative approach to the posterior cranial fossa and craniocervical junction in asymptomatic children with achondroplasia
Quantitative approach to the posterior cranial fossa and craniocervical junction in asymptomatic children with achondroplasia
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DOI：
10.1007/s00234-017-1887-y
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发表时间：
2017-10-01
期刊：
影响因子：
2.8
通讯作者：
Colosimo, Cesare
中科院分区：
文献类型：
--
作者：
Calandrelli, Rosalinda;Panfili, Marco;Colosimo, Cesare
我们提出了一种基于磁共振成像(MRI)的定量形态体积测量方法来研究软骨整形患者后颅窝(PCF)和颅颈交界处(CCJ)的变化，探讨其与脑室扩大和髓内压迫的可能联系。应用3DFSPGRT1加权像分析(1)PCF综合征，(2)PCF体积(PCFV)、PCF脑体积(PCFBV)、PCFV/PCFBV比值、小脑体积、脑脊液(CSF)空间体积、IV脑室体积，(3)PCF(斜坡、枕上角、枕部长度、天幕角)和CCJ(枕大孔AP和L1直径)形态测量，(4)FM和颈静脉孔(JF)区测量；(5)幕上脑室体积。所有患者均表现为蝶枕联合软骨融合，8例枕内联合软骨融合，9例CCJ撞击颈髓，仅3例有颈髓病变。与正常对照组比较，斜坡长、外枕长、枕前角、枕上角、小脑体积、幕上脑室系统体积、小脑体积、幕上脑室系统体积显著减小。斜坡长度与幕上脑室容量、枕内软骨提前闭合、枕区呈正相关，而与枕上区呈正相关。我们的分析表明斜坡缩短与脑室扩大有关。另一方面，PCF软骨的过早闭合、枕上骨的形状和生长方向导致FM面积缩小，在一些患者中导致髓质受压。
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.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.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.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.