Abstract：
To identify MRI-based quantitative craniofacial variables linked to airways narrowing and obstructive sleep apnea (OSA) development in children with achondroplasia.
We evaluated skull base and midface MRI in two cohorts of children affected by achondroplasia, with (group 1) or without OSA (group 2). 3DFSPGR-T1weighted images were used to assess airways volume (nasopharynx, oropharynx, and laryngopharynx), jugular foramina (JF) and hypoglossal foramina (HF) areas, foramen magnum area, cervical cord area, and maxillary retrusion (SNA angle).
Nineteen out of 27 children with achondroplasia exhibited different degrees of obstructive respiratory impairment (n.4 mild, n.8 moderate, n.7 severe), while 8 children did not show OSA. Each group was compared with age-matched controls without neuroimaging abnormalities. Both groups showed reduced nasopharynx volume, JF areas, and SNA angle, while group 1 showed also reduced oropharynx volume, ratio of FM/cervical cord areas, and HF areas (p < 0.05). A positive correlation between nasopharynx volume and SNA angle was found in both groups, while a positive correlation among upper airways volume, JF and HF areas was found only in group 1. No correlation between upper airways volume and OSA severity was found.
In children with achondroplasia, multifaced craniofacial abnormalities contribute to airways volume reduction predisposing to sleep disordered breathing. MRI-based quantitative assessment allows the appraisal of craniofacial variables linked to the development of sleep-disordered breathing such as FM stenosis, jugular and hypoglossal foramina stenosis, and retruded maxillary position and may be a valuable tool for clinical surveillance.
We evaluated skull base and midface MRI in two cohorts of children affected by achondroplasia, with (group 1) or without OSA (group 2). 3DFSPGR-T1weighted images were used to assess airways volume (nasopharynx, oropharynx, and laryngopharynx), jugular foramina (JF) and hypoglossal foramina (HF) areas, foramen magnum area, cervical cord area, and maxillary retrusion (SNA angle).
Nineteen out of 27 children with achondroplasia exhibited different degrees of obstructive respiratory impairment (n.4 mild, n.8 moderate, n.7 severe), while 8 children did not show OSA. Each group was compared with age-matched controls without neuroimaging abnormalities. Both groups showed reduced nasopharynx volume, JF areas, and SNA angle, while group 1 showed also reduced oropharynx volume, ratio of FM/cervical cord areas, and HF areas (p < 0.05). A positive correlation between nasopharynx volume and SNA angle was found in both groups, while a positive correlation among upper airways volume, JF and HF areas was found only in group 1. No correlation between upper airways volume and OSA severity was found.
In children with achondroplasia, multifaced craniofacial abnormalities contribute to airways volume reduction predisposing to sleep disordered breathing. MRI-based quantitative assessment allows the appraisal of craniofacial variables linked to the development of sleep-disordered breathing such as FM stenosis, jugular and hypoglossal foramina stenosis, and retruded maxillary position and may be a valuable tool for clinical surveillance.
摘要：
为了确定与软骨发育不全儿童气道狭窄和阻塞性睡眠呼吸暂停（OSA）发展相关的基于MRI的定量颅面变量。
我们评估了两组受软骨发育不全影响的儿童的颅底和中面MRI，有（组1）或没有OSA（组2）。3DFSPGR-T1加权图像用于评估气道容积(鼻咽，口咽,和喉咽），颈静脉孔（JF）和舌下孔（HF）区域，大孔区,颈索区，和上颌后缩（SNA角度）。
27例软骨发育不全患儿中，有19例表现出不同程度的阻塞性呼吸损害（第4例，轻度，n.8中度，n.7严重)，而8名儿童未出现OSA。将每组与年龄匹配的无神经影像学异常的对照组进行比较。两组均显示鼻咽体积减少，JF地区,和SNA角度，而第1组也显示口咽体积减少，FM/颈索面积的比率，和HF区域(p<0.05)。两组鼻咽体积与SNA角度呈正相关,虽然上呼吸道容积之间呈正相关，仅在第1组中发现了JF和HF区域。上呼吸道容积与OSA严重程度无相关性。
在患有软骨发育不全的儿童中，多面颅面部异常有助于气道容量减少，易导致睡眠呼吸紊乱。基于MRI的定量评估可以评估与睡眠呼吸紊乱（如FM狭窄）发展相关的颅面变量。颈静脉和舌下神经孔狭窄,上颌骨的位置，可能是临床监测的一个有价值的工具。
我们评估了两组受软骨发育不全影响的儿童的颅底和中面MRI，有（组1）或没有OSA（组2）。3DFSPGR-T1加权图像用于评估气道容积(鼻咽，口咽,和喉咽），颈静脉孔（JF）和舌下孔（HF）区域，大孔区,颈索区，和上颌后缩（SNA角度）。
27例软骨发育不全患儿中，有19例表现出不同程度的阻塞性呼吸损害（第4例，轻度，n.8中度，n.7严重)，而8名儿童未出现OSA。将每组与年龄匹配的无神经影像学异常的对照组进行比较。两组均显示鼻咽体积减少，JF地区,和SNA角度，而第1组也显示口咽体积减少，FM/颈索面积的比率，和HF区域(p<0.05)。两组鼻咽体积与SNA角度呈正相关,虽然上呼吸道容积之间呈正相关，仅在第1组中发现了JF和HF区域。上呼吸道容积与OSA严重程度无相关性。
在患有软骨发育不全的儿童中，多面颅面部异常有助于气道容量减少，易导致睡眠呼吸紊乱。基于MRI的定量评估可以评估与睡眠呼吸紊乱（如FM狭窄）发展相关的颅面变量。颈静脉和舌下神经孔狭窄,上颌骨的位置，可能是临床监测的一个有价值的工具。
