目的 探讨先天性卵巢囊肿的CT表现与临床特征,提高对该病的认识和诊断水平.方法 回顾性分析24例经手术、病理证实的先天性卵巢囊肿的CT表现及临床特征,总结其共同点及规律.结果 经手术及病理证实24例病例中:卵巢单纯囊肿11例,卵巢囊肿伴蒂扭转10例,卵巢浆液性囊腺瘤伴蒂扭转1例,卵巢囊肿伴出血1例,卵巢自截1例.所有卵巢单纯囊肿均表现为类圆形薄壁囊性肿块.当卵巢囊肿伴发出血、蒂扭转、自截等并发症时,CT可见囊壁增厚、囊壁钙化.此外囊液分层是卵巢囊肿伴出血的典型CT表现,囊肿边缘高密度三角形征是囊肿伴发蒂扭转的特征性CT征象.结论 先天性卵巢囊肿,尤其当伴发出血、蒂扭转、自截时,CT表现具有一定特征性,认识相关CT征象有助于术前准确诊断并识别并发症.

患儿 女,于外院经剖宫产出生,出生体质量 1.785 kg.出生时 Apgar阿普加评分 7～8～9,自主呼吸弱,无哭声,球囊正压通气无明显改善,听诊双肺呼吸极弱,无明显自主呼吸,心率 70 次/min,给予气管插管,声门暴露困难,气管插管中冒出中量透明黏稠羊水样物.遂转至广东省妇幼保健院,行纤维支气管镜检查诊断为先天性气管闭锁.

患者 女,44 岁,间断阴道出血 1 月余.专科查体:宫颈后唇、阴道后穹窿形态消失,触之出血.宫颈液基薄层细胞检测:阴性.盆腔 MRI:宫颈体积增大,宫颈满布大小不一类圆形异常信号影,T1 WI呈低信号(图 1 ),T2 WI呈高信号(图 2 ),扩散加权成像(diffusion weighted imaging,DWI)呈低信号及高信号(图 3),增强扫描未见强化(图4),考虑宫颈黏膜增生伴多发纳氏囊肿.

Objective:To investigate the diagnostic values of prenatal ultrasound and MRI in fetal adrenal lesions and analyze the characteristic imaging features of different diseases.Methods:The images of 22 fetuses (the mean gestational age was 30 weeks and the male to female ratio was 15:7) with adrenal lesions found by prenatal ultrasound in Guangdong Women and Children Hospital from March 2013 to December 2022 were retrospectively analyzed, 14 of them underwent MRI examination. Two sonographers and two radiologists with experiences in diagnosing fetal diseases were used to read the images by double-blind method. When the diagnosis results of the same examination were inconsistent between two doctors, the consensus was reached after discussion.Results:Adrenal neuroblastoma (NB) was diagnosed in 14 of 22 cases; the lesions of 2 cases became smaller and disappeared after prenatal and multiple postnatal follow-up, and were diagnosed as adrenal hematoma combined with imaging findings; 5 cases was diagnosed as intraperitoneal pulmonary sequestration (IEPS) by postoperative pathology; 1 case was adrenal teratoma. Both ultrasound and MRI accurately diagnosed NB, adrenal hematoma, and IEPS, and misdiagnosed teratoma as NB. Adrenal NB might present as cystic, complex, or solid mass with partial calcification, might be bilateral, and might develop intrahepatic metastases during the fetal period. In the initial stage of hemorrhage, adrenal hematoma showed hyperechoic on ultrasound, then gradually reduced to anechoic, and finally gradually calcified or disappeared. Lesion stratification could be seen on MRI, and high signal shadow on T1WI. The ultrasonography of IEPS showed homogeneous solid or complex mass, among which solid mass accounted for the majority, and supplied by a branch of systemic arterial. Homogeneous solid or complex high signal shadow showed in single shot fast spin echo (SSFSE) sequence images, with signal intensity higher than or equal to that of lung tissue, and the lesion closely connected with diaphragm. The teratoma showed mixed echo on ultrasound and complex signal mass on SSFSE sequence, and it was difficult to distinguish fat deficient teratoma from NB.Conclusions:Prenatal ultrasound and MRI have high diagnostic values in fetal adrenal lesions. The two kinds of examinations complement each other and can improve the diagnostic accuracy.

Objective:To compare the prenatal ultrasound (US) and magnetic resonance imaging (MRI) characteristics of Joubert syndrome (JS) to enrich the knowledge on JS and improve its diagnosis.Methods:The prenatal US and MRI characteristics of 14 cases of JS at Guangdong Women and Children Hospital from January 2014 to January 2022 were retrospectively analyzed.Results:Among the 14 cases of JS fetuses, systematic US examination and MRI examination were performed 17 times and 16 times, respectively. A total of 8 cases (57%, 8/14) were diagnosed with JS by both US and MRI, while 6 cases (43%, 6/14) had inconsistent diagnosis results. Among all the 17 ultrasound and 16 MRI examinations, US suggested a diagnosis of JS 7 times (41%, 7/17) and a suspicious diagnosis of JS 4 times (23%, 4/17) while MRI suggested a diagnosis of JS 13 times (81%, 13/16). Regarding the image characteristics of US and MRI, consistent typical molar tooth sign was suggested in 7 cases (50%, 7/14), while inconsistent results were suggested in 7 cases (50%, 7/14); consistent little or no cerebellar vermis was suggested in 10 cases (71%, 10/14), while inconsistent results were suggested in 4 cases (29%, 4/14); consistent aspect ratio of the fourth ventricle >1 was suggested in 12 cases (86%, 12/14), while inconsistent results were suggested in 2 cases (14%, 2/14); consistent closely adjacent cerebellar hemispheres were suggested in 8 cases (57%, 8/14), consistent distinctly separated cerebellar hemispheres were suggested in 2 cases (14%, 2/14), and inconsistent results were suggested in 4 cases (29%, 2/14). Among the 17 ultrasound and 16 MRI examinations, the sign of "midline fissure" was showed 3 times (18%, 3/17) and 13 times (81%, 13/16) by ultrasound and MRI, respectively. Hyperechoic region of the cerebellar hemisphere was shown by US in 12 cases (86%, 12/14), while hyperechoic region was misdiagnosed as vermis in 2 cases (17%, 2/12).Conclusion:Accurate recognition of the prenatal imaging characteristics and pitfalls of JS is helpful to improve the prenatal detection rate of JS.

目的:探讨128层MSCT虚拟仿真内镜技术(VE)对婴幼儿先天性心脏病的诊断价值.方法:回顾性分析67例先天性心脏病患儿128层螺旋CT和经胸壁超声心动图(TTE)资料,并与手术结果作对照.采用5分单盲法评价VE图像质量,比较心房心室和大血管的差异,建立VE飞行路径规划.结果:VE诊断主要心脏结构异常的总符合率51.7％～100％,灵敏度51.7％～100％;MPR总符合率53.3％～100％,灵敏度53.3％～100％;TTE总符合率25.0％～100％,灵敏度25.0％～100％.VE和MP R在室间隔缺损、右心室双出口、大动脉转位、肺动脉狭窄、冠状动脉畸形和血管环的诊断中其总符合率和灵敏度都高于TTE,但诊断房间隔缺损及卵圆孔未闭的总符合率和灵敏度不如TTE.VE在显示结构异常的直观性和三维空间感优于MPR.VE图像质量评分对比:RA与LV、RV、LA及大血管图像评分均存在显著性差异(P=0.000<0.01),LA与RV(P=0.006<0.01)、大血管图像评分存在显著性差异(P=0.002<0.01).大血管VE图像质量最佳,其后为RV及LV,LA、RA图像质量最差.VE飞行路径规划时应将左右心系统分别建立.结论:128层MSCT虚拟仿真内镜技术可准确显示婴幼儿先天性心脏病的心腔和大动脉的异常结构,尤其对室间隔缺损、圆锥动脉干畸形和冠状动脉畸形较TTE更准确.VE有更强的直观性和三维空间感,更接近心脏直视手术视野,有助于手术方案的合理设计,对提高虚拟手术教学和改善医患术前交流有重要意义.

患者 女,32岁.既往体健,孕25周超声提示胎儿头围相当于23周,孕29周复查超声提示胎儿头围相当于26周,腹围相当于27周,股骨与肱骨相当于29周. 孕31周行胎儿头颅MRI检查示:胎儿大脑表面光滑,脑沟浅少,双侧室管膜下囊肿(图1),双侧半卵圆中心脑白质T2信号增高(图2).孕35周复查MRI显示:胎儿大脑脑沟仍浅少(图3),脑回巨大,脑白质T.2信号仍增高(图4),扩散加权成像(diffusion weighted imaging,DWI)显示半卵圆中心信号偏低(图 5),表观扩散系数(apparent diffusion coefficient,ADC)值升高,约 2.20×10-3 mm2/s(正常值约 1.75×10-3 mm2/s),提示脑白质髓鞘化不良.另发现胎儿右肾肾盂轻度扩张(图6).

Rationale: Most congenital soft tissue masses are benign. Ewing's sarcoma (ES) is a highly malignant tumor that commonly occurs in children and adolescents and rarely occurs during the fetal period. Cases of congenital soft tissue ES with magnetic resonance imaging (MRI) findings are scarce. To the best of our knowledge, no previous reports have described the pre- and postnatal MRI findings of ES. Patient concerns: We present a case of congenital soft tissue ES arising in the body wall, which was examined using MRI during the prenatal and neonatal periods. Diagnoses: Malignancy was suspected by diffusion-weighted imaging, which demonstrated restricted diffusion within the mass even during the fetal period. ES was confirmed via histopathological examination after birth. Interventions: The patient initially underwent conservative treatment for suspected hemangioma. Tumorrectomy was undergone after three weeks based on previously dissatisfied therapeutic effects. Outcomes: The patient died of multiple distant metastases despite undergoing postoperative chemotherapy and metastasectomies. Lessons: Fetal or neonatal soft tissue ES may be clinically misdiagnosed as a hemangioma. It is important to suspect this through an imaging approach such as diffusion-weighted imaging.

Objective:To explore the related factors and incidence of liver function damage in children with EB virus related infectious mononucleosis (IM), and to raise the level of understanding on liver function damage.Methods:A total of 101 cases of children diagnosed with IM were collected from our hospital from May 2017 to May 2020, including 63 males and 38 females, with an average age of 3.61 years old. The level of alanine aminotransferase (ALT) >50 U/L and/or aspartate aminotransferase (AST) >40 U/L were defined as abnormal liver function, and the level of ALT ≥100 U/L and/or AST ≥100 U/L were defined as liver function damage. The incidences of abnormal liver function and liver function damage were compared between age and gender, and the hospital stay, thermal spike, and fever time were compared between children with and without liver function damage.Results:There was no statistically significant difference in the incidence of abnormal liver function among different age groups ( χ2=3.093, P=0.213). There was no statistically significant difference in the incidence of liver function damage between the <3 years old group and the 3-6 years old group ( P>0.05); there was a statistically significant difference in the incidence of liver function damage between the <3 years old group and the >6 years old group ( χ2=11.366, P<0.001); there was a statistically significant difference in the incidence of liver function damage between the 3-6 years old group and the >6 year old group ( χ2=10.266, P<0.001). The incidences of abnormal liver function and liver function damage in female IM children were 94.7% (36/38) and 63.1% (24/38), respectively, which were higher than those in male IM children [73.0% (46/63) and 34.9% (22/63)], with statistically significant differences (both P<0.05). There were no statistically significant differences in the thermal spike and fever time between children with liver function damage and children without liver function damage (both P>0.05); the hospital stay of children with liver function damage was (7.23±3.89) d, which was higher than that of children without liver function damage [(5.56±1.93) d], with a statistically significant difference ( P<0.05). Conclusions:Children with IM are facing a status of being in high incidences of abnormal liver function and liver function damage, which is related to age, sex, and hospital stay. It is necessary to strengthen the protection to children with different degrees of liver function damage.

目的 探讨糖皮质激素治疗抗N-甲基-D-天冬氨酸受体脑炎(抗NMDAR脑炎)致硬膜外脂肪增多症(SEL)的临床特征.方法 回顾分析1例抗NMDAR脑炎致SEL患儿的临床资料.检索万方数据库及PubMed数据库,复习相关文献.结果 患儿,女,8岁,表现为右手不自主活动、癫痫样发作伴精神行为异常,脑脊液抗谷氨酸受体抗体IgG 1:32,诊断为抗NMDAR脑炎.患儿先后接受丙种球蛋白、糖皮质激素(泼尼松)、血浆置换及环磷酰胺免疫治疗.随访中发现患儿脑脊液蛋白异常升高,脊髓磁共振(MRI)示脊髓SEL.逐渐减停泼尼松,以合理饮食及运动控制体质量后,复查脊髓MRI明显好转.检索文献,国外有14篇文献报道15例类固醇激素治疗其他疾病致SEL.儿童SEL可出现背痛、无力、急性麻痹等临床症状,也可无症状.结论 外源性类固醇激素治疗抗NMDAR脑炎引起的儿童SEL,可在临床症状出现之前早期诊断,并通过减停激素及控制体质量等治疗完全康复.

肌上皮癌(Myoepithelioma carcinoma,MEC)是主要发生于头颈部大小涎腺的罕见恶性肿瘤,以腮腺最为多见,偶见发生于肺部、乳腺、皮肤、胃等[1-3].原发于鼻腔鼻窦者极为罕见,发病年龄约21～67岁[4].本文报道1例新生儿鼻腔鼻窦MEC,并结合文献对其影像学表现进行总结归纳.

目的 运用受试者工作特征(ROC)曲线分析确定可用于判断孤立性先天性膈疝(CDH)胎儿预后的MRI观察值/预期值胎儿肺容积(o/e FLV)界定值.方法 选取孕18～38周的孤立性CDH胎儿98例.应用新的适用于国内的FLV预期值回归方程来测算孤立性CDH胎儿的MRI o/e FLV值.按产后是否存活分组观察o/e FLV情况.采用ROC曲线分析确定孤立性CDH胎儿产后能否生存的o/e FLV界定值及敏感度、特异度.结果 产后存活组和死亡组的o/e FLV平均值分别为52.59％±15.08％和29.57％±8.52％.ROC曲线分析显示最佳MRI o/e FLV界定值为37.82％,灵敏度为86.1％,特异度为88.5％.结论 MRI o/e FLV界定值的确定为判断孤立性CDH胎儿预后提供了依据,有助于孤立性CDH胎儿的产前咨询与管理.

Objective To investigate the clinical characteristics and risk factors of congenital choledochal cysts (CCC).Methods This retrospective study recruited 52 cases who were antenatally diagnosed with CCC and underwent surgical treatment after birth in Guangdong Women and Children Hospital from January 2013 to August 2018,with complete clinical data.According to the enlargement of cysts during pregnancy,they were divided into two groups:progressive group (≥ 15 mm,22) and stable group (＜15 mm,30).Antenatal and postpartum ultrasound and MRI features of the two groups were analyzed.Clinical manifestations and biochemical examination results before and after operation were compared between the two groups.Other data,including amylase level in cyst fluid during operation,cholangiography findings,liver biopsy results,and post-operation follow-up,were also analyzed.Chi-square test,t (t1) test,and Pearson correlations tests were performed for data analysis.Results (1) The average age of the 52 patients at operation was 46(7-822) d.The cysts of all cases were first detected during 19-21 weeks of gestation.The maximum diameter of the cyst in the progressive group was larger than that in the stable group after 34 weeks of pregnancy [31-34 weeks:(31.1 ±8.4) vs (23.1 ± 6.6) mm,t=3.911;＞34 weeks:(36.1 ± 6.8) vs (27.1 ± 7.3) mm,t=4.557;pre-operation:(51.8± 18.0) vs (34.0± 15.6) mm,t=3.809;all P＜0.01].(2) In the progressive group,the cysts were irregular in shape and enlarged after birth.The common hepatic duct and intrahepatic bile duct were dilated and gradually distended after birth,while the distal end of the common bile duct was narrowed,thus to form a cone-like duct.Deposits could be seen inside the cysts after delivery.Irregular cysts were also presented in the stable group,and five of them had dilatation of common hepatic duct and intrahepatic bile duct after birth.However,no cone-like formation was seen,the distal end of the common bile duct was visible,and deposits in cysts were occasionally found.(3) Twenty-five patients underwent laparotomy,and seven of them showed increased amylase level in cyst fluid including four with 2-5 times above the upper limit of normal value (one in the progressive group and three in the stable group).The other three cases were all in the stable group and their amylase levels in cyst fluid were more than ten times of the upper limit.The level of direct bilirubin in the progressive group was higher than that in the stable group before the operation [18.40(2.50-113.30) vs 8.70(0.00-16.80) μmol/L,u=2.400,P＜0.05].(4) Among the 52 cases,patients with type Ⅰ,Ⅳ and Ⅴ cyst accounted for 71.1％ (37/52),26.9％ (14/52) and 2.0％ (1/52),respectively.All cases were followed up regularly six months to one year after the operation.Liver function and bilirubin became normal and the growth and development of the babies were similar to those of the same age.(5) Different degrees of liver fibrosis and inflammation were shown in 46(88.5％) cases and more severe in older babies among those who underwent surgery in the progressive group.The time at operation was not associated with the severity of liver fibrosis and inflammation in the stable group.Hepatic fibrosis and inflammation were more serious in the progressive group than in the stable group (fibrosis grading:x2=14.260,P=0.006;inflammatory activity grading:x2=9.904,P=0.019).Conclusions Larger diameter (≥ 30 mm) in the initial prenatal examination or a significant increase in cystic diameter (≥ 15 mm) during pregnancy are risk factors for early stenosis or occlusion in the distal end of common bile duct requiring close follow-up after birth.When jaundice or abnormal liver function occur and stool color becomes light,early surgical treatment (one to two months after birth,generally within three months) for CCC is recommended to rule out the anomalous union of the pancreaticobiliary duct and hepatic disorders,as well as the cystic biliary atresia.

目的 探讨产前超声联合磁共振诊断胎儿部分性胼胝体发育不全的价值.方法 收集产前超声联合磁共振诊断为部分性胼胝体发育不全的胎儿15例,对比出生或引产后的检查结果,分析产前超声特征.结果 产前超声直接征象:胼胝体正中矢状切面相应部位缺失12例(12/15,80.0％).间接征象:透明隔腔形态异常(80.0％)、 侧脑室扩张(73.3％)、 大脑纵裂池分离(73.3％)、 脑中线丘脑后方囊性包块(26.7％)、 侧脑室"泪滴状改变"(26.7％)、 胼周动脉走行异常(26.7％),合并颅内或其他系统畸形6例.对比出生或引产后随访结果,诊断准确率:产前超声80.0％,产前磁共振93.3％.结论 超声可以诊断胎儿部分性胼胝体发育不全,更精确的诊断建议结合产前胎儿头颅磁共振检查.

胎儿磁共振成像(magnetic resonance imaging,MRI)是协同于超声的重要影像诊断方法,既可确认超声发现,也可通过额外发现对专业超声检查进行补充,其在胎儿脑发育评估中的应用更为广泛[1].如果指征明确、检查合适且结果解读正确,MRI不仅有助于诊断,而且还是治疗方案制定、分娩计划和咨询的重要依据之一.

Objective To investigate the prenatal ultrasonic characteristics and clinical outcomes of fetal congenital lobar emphysema (CLE). Methods A retrospective analysis was done to demonstrate the prenatal ultra-sonic characteristics of 10 children diagnosed as CLE after birth from January 2011 and May 2014. The clinical out-comes were followed up. Results Ten patients were diagnosed with CLE after birth, whose prenatal ultrasound mani-fest hyperechoic masses in the fetal chests, in which 7 cases occurred in the upper left lobe, 5 cases were found with multiple cystic echo in the masses, 9 cases were detected with compression and shift of the heart. No aortic blood ves-sel that supplied blood to the mass was found in all the cases. All the cases were diagnosed as CLE by CT and patholo-gy. Conclusion Prenatal ultrasound can detect thoracic hyperechoic mass, and the masses locate in the upper lobe and occupy the whole lobe should be more vigilantly suspected the possibility of CLE.

目的 建立大样本量、孕周分布均衡的中国胎儿肺容积(FLV)预期值回归方程.方法 采集457胎胎龄18～39周胸部正常胎儿产前MRI,在单次激发快速自旋回波序列轴位图像上测量FLV.对采集到的样本数据进行回归分析,根据拟合度及检验结果确定回归模型,并得出基于孕周的FLV预期值回归方程.结果 457胎胎儿平均孕周为(29.44±4.61)周,其中孕周≤28周胎儿占43.11％(197/457).基于MRI测量FLV值为9.47～131.48 ml,平均(50.78±23.76)ml.回归分析所得方程为FLV预期值=0.00492.76(g为孕周,R2=0.845,P＜0.001).结论 本研究所得的FLV预期值回归方程简便实用,适用于大多数产前检查,有助于国内开展胎儿肺产前评估相关研究.

目的 探讨胎儿小肠梗阻的产前MRI特点.方法 回顾性分析18例经手术病理证实的胎儿小肠梗阻及其并发症的MRI表现.具体分析产前MRI上扩张肠管的信号特点,以及结直肠显示情况与小肠梗阻发生水平的关系.并就胎粪性假性囊肿等并发症的MRI表现与手术结果作一对照分析.结果 18例胎儿小肠梗阻中,十二指肠梗阻7例、空肠梗阻4例、回肠梗阻7例.其中,十二指肠梗阻和空肠近段梗阻的扩张肠管在T1WI呈低信号,在T2WI呈高信号;空肠中近段梗阻的扩张肠管在T1WI呈等-稍高信号,在T2WI呈高信号;空肠中远段和回肠梗阻的扩张肠管在T1WI呈高信号,在T2WI呈低信号.胎粪性假性囊肿在T1WI呈高信号.可合并囊内液-液分层,以及可见与小肠扩张肠管相连.结论 产前MRI可依据扩张小肠内信号特点及结直肠情况推断小肠梗阻发生的水平.胎粪性假性囊肿有着与其病理相关的特征性的MRI表现.产前MRI在胎儿小肠梗阻诊断上颇具优势,有助于产前咨询与围产期管理.

Objective To explore the diagnostic value of prenatal MRI in fetal Joubcrt syndrome and related disorders (JSRD).Methods Prenatal MRI data of 5 JSRD fetuses were analyzed retrospectively.The ratio of AP diameters of crown to root of molar tooth sign,the ratio of AP diameters of isthmus to interpeduncular fossa,and the ratio of the transverse to AP diameters of the roof of the fourth ventricle on the axial images of the midbrain were measured.Results Molar tooth signs were observed in 5 fetuses on the axial images of midbrain.The cerebellar vermis disappeared,and cleft sign was observed.Enlarged cisterna magna was observed in 3 fetuses.The ratios of AP diameters of crown to root were 0.58-0.90,of AP diameters of isthmus to interpeduncular fossa were 0.84-1.00,and of transverse to AP diameters of the fourth ventricle roof were 0.42-0.60.Conclusion Molar tooth sign and cleft sign were characteristic findings for JSRD on MRI,therefore being helpful to prenatal diagnosis of JSRD.

目的 探讨MRI在不同胎盘植入深度中的诊断价值,分析不同胎盘植入深度在MRI中的特征性表现.方法 回顾性分析384例胎盘植入高风险患者MRI影像资料.由2名高年资影像医师阅片,判断是否存在胎盘植入以及植入深度,同时分析不同胎盘植入深度的MRI影像特征,并与术中临床诊断和/或病理结果进行比较.结果 384例胎盘植入高风险患者中共有137例经术中临床和/或病理诊断为胎盘植入,其中粘连性胎盘植入89例,植入性胎盘植入44例,穿透性胎盘植入4例.MRI准确诊断89例粘连性胎盘植入中的54例(敏感性60.7％,特异性97.0％),44例植入性胎盘植入中的42例(敏感性95.5％,特异性99.4％)以及4例穿透性胎盘植入中的3例(敏感性75％,特异性100％).粘连性胎盘植入部分病例在SSFSE序列图像中仅表现为局部胎盘与子宫肌层界线模糊,邻近胎盘内可见少许低信号影,而部分并无明显异常发现.植入性胎盘植入最具特征的影像表现为胎盘实质内可见条带状低信号影及增粗、迂曲的血管影,部分可见子宫膨出.穿透性胎盘植入包括局部子宫肌层缺失,胎盘内显著增粗、迂曲血管影,膀胱呈幕状改变或胎盘结节状突入膀胱.结论 MRI对植入性和穿透性胎盘植入有较高诊断价值,存在相对特征性的影像表现,对粘连性胎盘植入的诊断敏感性较低.