本文選題：Apert綜合征 + Crouzon綜合征　； 參考：《臨床放射學(xué)雜志》2017年09期

【摘要】：目的應(yīng)用MSCT比較和分析Apert綜合征(AS)與Crouzon綜合征(CS)的顱面畸形的特點(diǎn),獲得MSCT的診斷應(yīng)用價(jià)值。方法搜集符合條件的22例患兒,其中AS與CS各11例,所有患兒術(shù)前及術(shù)后均進(jìn)行了MSCT檢查,并且在工作站進(jìn)行MPR及VR重組,觀察圖像包括腦窗、骨窗、軟組織窗,對(duì)兩組之間的眶間距、外側(cè)壁夾角、眼球突出度差異進(jìn)行統(tǒng)計(jì)學(xué)比較。結(jié)果 AS與CS男女發(fā)病率無明顯差別,兩者都伴顱縫早閉、眼球突出、眼距增寬、面中部凹陷、下頜前突。兩組之間比較,AS眼球突出度小于CS,且有統(tǒng)計(jì)學(xué)差異(P0.05)。AS中位年齡1歲,以冠狀縫早閉為主,主要為尖頭短頭畸形;腦室改變以兩側(cè)腦室前角飽滿為主,小腦扁桃體形態(tài)位置未發(fā)現(xiàn)異常,顱內(nèi)外未見靜脈畸形,頸椎畸形以C_(5~6)椎板融合多見。CS中位年齡2歲,以全顱縫早閉為主,主要為尖頭小頭畸形,部分為舟狀頭或斜頭畸形;腦室改變以幕上腦室或側(cè)腦室不同程度擴(kuò)大為主,其中5例伴腦積水,11例均伴小腦扁桃體下疝,枕部頭皮下均見迂曲增粗引流靜脈通過枕骨缺損處與竇匯或橫竇相通,頸椎畸形以C_(2~3)椎板融合多見。結(jié)論 AS與CS顱面畸形有類似之處,但也有一定的差異,MSCT不僅為其診斷及鑒別診斷提供幫助,更重要的是了解早閉的顱縫及顱內(nèi)外異常情況,為臨床手術(shù)方式的選擇提供依據(jù),并用來評(píng)估術(shù)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.
【作者單位】： 南京醫(yī)科大學(xué)附屬兒童醫(yī)院放射科;
【分類號(hào)】：R725.9;R816.92

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