MSCT在Apert综合征与Crouzon综合征颅面畸形中的对比研究

发布时间：2018-05-06 20:49

本文选题：Apert综合征 + Crouzon综合征　；参考：《临床放射学杂志》2017年09期

【摘要】：目的应用MSCT比较和分析Apert综合征(AS)与Crouzon综合征(CS)的颅面畸形的特点,获得MSCT的诊断应用价值。方法搜集符合条件的22例患儿,其中AS与CS各11例,所有患儿术前及术后均进行了MSCT检查,并且在工作站进行MPR及VR重组,观察图像包括脑窗、骨窗、软组织窗,对两组之间的眶间距、外侧壁夹角、眼球突出度差异进行统计学比较。结果 AS与CS男女发病率无明显差别,两者都伴颅缝早闭、眼球突出、眼距增宽、面中部凹陷、下颌前突。两组之间比较,AS眼球突出度小于CS,且有统计学差异(P0.05)。AS中位年龄1岁,以冠状缝早闭为主,主要为尖头短头畸形;脑室改变以两侧脑室前角饱满为主,小脑扁桃体形态位置未发现异常,颅内外未见静脉畸形,颈椎畸形以C_(5~6)椎板融合多见。CS中位年龄2岁,以全颅缝早闭为主,主要为尖头小头畸形,部分为舟状头或斜头畸形;脑室改变以幕上脑室或侧脑室不同程度扩大为主,其中5例伴脑积水,11例均伴小脑扁桃体下疝,枕部头皮下均见迂曲增粗引流静脉通过枕骨缺损处与窦汇或横窦相通,颈椎畸形以C_(2~3)椎板融合多见。结论 AS与CS颅面畸形有类似之处,但也有一定的差异,MSCT不仅为其诊断及鉴别诊断提供帮助,更重要的是了解早闭的颅缝及颅内外异常情况,为临床手术方式的选择提供依据,并用来评估术后效果。
[Abstract]:Objective to compare and analyze the characteristics of craniofacial malformation between Apert syndrome (ASS) and Crouzon syndrome (CSS) by MSCT, and to obtain the diagnostic value of MSCT. Methods A total of 22 children with as and CS were collected. All of them were examined by MSCT before and after operation, and MPR and VR were performed on the workstation. The images included brain window, bone window and soft tissue window. The orbital distance, the angle of lateral wall and the degree of exophthalmos between the two groups were compared statistically. Results there was no significant difference between men and women in incidence of as and CS. Both of them were accompanied by early closure of cranial suture, exophthalmos, widening of eye distance, midfacial depression and mandibular protrusion. The degree of eyeball exophthalmos in the two groups was less than that in CSS, and there was statistical difference between the two groups (P 0.05). The median age of as was 1 years old. The main type of premature closure of coronary suture was short head deformity, and the main changes of ventricle were the fullness of anterior horn of both ventricles. No abnormal position of cerebellar tonsil was found, no venous malformation was found outside the brain, and the cervical vertebrae malformation was Cash5 / 6). The median age of CS2 was 2 years old. The main type was the early closure of the whole cranial suture, which was mainly a small head deformity, some of which were navicular head or oblique head malformation. The main changes of ventricle were enlargement of supratentorial ventricle or lateral ventricle, of which 5 cases were accompanied with hydrocephalus and 11 cases had subtonsillar hernia of cerebellum. In occipital subscalp, tortuous and thicker drainage veins were found to communicate with sinus junction or transverse sinus through occipital bone defect. Cervical vertebrae malformation is more common in Cass 2 / 3) laminar fusion. Conclusion there are similarities between as and CS craniofacial malformation, but MSCT is not only helpful for diagnosis and differential diagnosis, but also more important to understand the early closed cranial suture and abnormal condition of craniofacial malformation, so as to provide the basis for the choice of clinical operation methods. And used to evaluate the effect of the operation.
【作者单位】：南京医科大学附属儿童医院放射科;
【分类号】：R725.9;R816.92

【相似文献】