Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.007
Shi-kui Chen, S. Xie, Xiangshang Lin, Qian Wang, Xindi Chen
Objective To report one rare pediatric case of esophageal ectopic thymus (EET) and review the relevant literatures. Methods The clinical manifestations, diagnosis and treatment of one child with EET were reviewed. And systematic searches of the relevant literatures were performed for " ectopic thymus" , " children" and " infant" in the databases of China Knowledge Network, Weipu, Wanfang and PubMed. Results One girl was admitted with vomiting and transient suffocation for 24 hours. Ultrasound and computed tomography (CT) indicated a substantial mass at upper esophagus. During operation, tumor was located in esophageal lumen. Final histopathologic diagnosis was consistent with EET. She was discharged at Day 8 postoperatively. During a follow-up period of 3 months, there was no occurrence of vomiting, dysphagia or other discomforts. And upper gastrointestinal radiography indicated that esophageal mucosal folds remained intact and esophagus was not narrowed or dilated. Fifty-three cases of EET were collected from the literature search. Ectopic thymus occurred in neck (n=34) and thyroid (n=18). Most cases could be detected by ultrasound and their clinical symptoms were not obvious. Sometimes a painless mass was present around neck or thyroid. And the complication of EET was rare. Conclusions Ectopic thymus is extremely rare in esophageal lumen and ultrasonic detection is often not feasible. It is necessary to confirm mediastinal thymus preoperatively. Especially for infants and toddlers, radical thymectomy results in immune dysfunction. Esophageal endoscopy and angiography may aid in pinpointing the location of lesion. Key words: Thymus gland; Dystopia; Esophagus
{"title":"A case report of esophageal ectopic thymus in a child and literature review","authors":"Shi-kui Chen, S. Xie, Xiangshang Lin, Qian Wang, Xindi Chen","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.007","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.007","url":null,"abstract":"Objective \u0000To report one rare pediatric case of esophageal ectopic thymus (EET) and review the relevant literatures. \u0000 \u0000 \u0000Methods \u0000The clinical manifestations, diagnosis and treatment of one child with EET were reviewed. And systematic searches of the relevant literatures were performed for \" ectopic thymus\" , \" children\" and \" infant\" in the databases of China Knowledge Network, Weipu, Wanfang and PubMed. \u0000 \u0000 \u0000Results \u0000One girl was admitted with vomiting and transient suffocation for 24 hours. Ultrasound and computed tomography (CT) indicated a substantial mass at upper esophagus. During operation, tumor was located in esophageal lumen. Final histopathologic diagnosis was consistent with EET. She was discharged at Day 8 postoperatively. During a follow-up period of 3 months, there was no occurrence of vomiting, dysphagia or other discomforts. And upper gastrointestinal radiography indicated that esophageal mucosal folds remained intact and esophagus was not narrowed or dilated. Fifty-three cases of EET were collected from the literature search. Ectopic thymus occurred in neck (n=34) and thyroid (n=18). Most cases could be detected by ultrasound and their clinical symptoms were not obvious. Sometimes a painless mass was present around neck or thyroid. And the complication of EET was rare. \u0000 \u0000 \u0000Conclusions \u0000Ectopic thymus is extremely rare in esophageal lumen and ultrasonic detection is often not feasible. It is necessary to confirm mediastinal thymus preoperatively. Especially for infants and toddlers, radical thymectomy results in immune dysfunction. Esophageal endoscopy and angiography may aid in pinpointing the location of lesion. \u0000 \u0000 \u0000Key words: \u0000Thymus gland; Dystopia; Esophagus","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"40 1","pages":"219-223"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76625473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.012
X. Zhan, Daniel Wang, Jianguo Zhu, Jin-pu Peng, Moudong Wu, Feng-ju Lu, Guoqing He, Nini An
Objective To compare the safety and efficacy of super-mini-percutaneous nephrolithotomy (SMP) versus mini-percutaneous nephrolithotomy (MPCNL) in managing renal calculi in children. Methods Medical records were retrospectively reviewed for 40 children with renal calculi. They underwent SMP (n=18) and MPCNL (n=22). In SMP group, there were 14 boys and 4 girls with an age range of (7.90±4.40) years and a stone size of (15.41±5.63) mm; In MPCNL group, there were 14 boys and 8 girls with an age range of (8.62±3.41) years and a stone size of (16.77±3.59) mm. Operative duration, primary stone clearance rate, postoperative complications and other parameters of two groups were statistically analyzed for comparing the characteristics of two operative approaches. Results The time of establishing percutaneous renal channel was shorter in SMP group than that in MPCNL group [(10.75±2.70) vs. (19.68±6.95) min]. And inter-group difference was statistically significant (P=0.016). The first-phase stone clearance, postoperative hemoglobin loss and postoperative complication rate of SMP and MPCNL groups were (83.33±46.72) vs. (101.36±38.91) min, 83.33% vs. 81.81% and (7.59±7.51) vs. (7.75±8.77) g/L. respectively. No significant inter-group difference existed. No blood transfusion was provided. In SMP group, there was Clavien Ⅰ complication (n=1, 5.55%); In MPCNL group (n=5, 22.72%), postoperative fever subsided after aggressive anti-infection treatment. In two groups, there was on onset of Clavien Ⅱ/Ⅲ complications. Ultrasonic follow-ups were conducted for 1-2 years and hydronephrosis or renal atrophy disappeared in both groups. Conclusions Both SMP and MPCNL are safe, effective and mini-invasive for pediatric renal calculi. As compared with MPCNL, SMP has a smaller channel and a thinner mirror body. And SMP is more suitable for infants or toddlers with narrow calyx neck and lower calyx calculi. Key words: Renal calculus; Percutaneous nephrolithotomy; Child
目的比较超微型经皮肾镜取石术(SMP)与微型经皮肾镜取石术(MPCNL)治疗儿童肾结石的安全性和有效性。方法回顾性分析40例小儿肾结石的临床资料。分别行SMP (n=18)和MPCNL (n=22)。SMP组男14例，女4例，年龄(7.90±4.40)岁，结石大小(15.41±5.63)mm;MPCNL组男14例，女8例，年龄(8.62±3.41)岁，结石大小(16.77±3.59)mm。统计分析两组手术时间、原发结石清除率、术后并发症等参数，比较两种手术入路的特点。结果SMP组经皮肾道建立时间明显短于MPCNL组[(10.75±2.70)min vs(19.68±6.95)min]。组间差异有统计学意义(P=0.016)。SMP组和MPCNL组一期结石清除率、术后血红蛋白损失及术后并发症发生率分别为(83.33±46.72)vs(101.36±38.91)min、83.33% vs 81.81%、(7.59±7.51)vs(7.75±8.77)g/L。分别。组间差异不显著。没有输血。SMP组出现ClavienⅠ并发症(n=1, 5.55%);MPCNL组(5例，22.72%)术后发热经积极抗感染治疗后消退。两组均未出现ClavienⅡ/Ⅲ并发症。超声随访1 ~ 2年，两组均无肾积水或肾萎缩。结论SMP和MPCNL治疗小儿肾结石安全、有效、微创。与MPCNL相比，SMP具有更小的通道和更薄的镜体。而SMP更适合于肾萼颈狭窄、下肾盏结石的婴幼儿。关键词:肾结石;经皮肾镜取石术;孩子
{"title":"Comparison of super-mini-percutaneous nephrolithotomy versus mini-percutaneous nephrolithotomy for renal calculi in children","authors":"X. Zhan, Daniel Wang, Jianguo Zhu, Jin-pu Peng, Moudong Wu, Feng-ju Lu, Guoqing He, Nini An","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.012","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.012","url":null,"abstract":"Objective \u0000To compare the safety and efficacy of super-mini-percutaneous nephrolithotomy (SMP) versus mini-percutaneous nephrolithotomy (MPCNL) in managing renal calculi in children. \u0000 \u0000 \u0000Methods \u0000Medical records were retrospectively reviewed for 40 children with renal calculi. They underwent SMP (n=18) and MPCNL (n=22). In SMP group, there were 14 boys and 4 girls with an age range of (7.90±4.40) years and a stone size of (15.41±5.63) mm; In MPCNL group, there were 14 boys and 8 girls with an age range of (8.62±3.41) years and a stone size of (16.77±3.59) mm. Operative duration, primary stone clearance rate, postoperative complications and other parameters of two groups were statistically analyzed for comparing the characteristics of two operative approaches. \u0000 \u0000 \u0000Results \u0000The time of establishing percutaneous renal channel was shorter in SMP group than that in MPCNL group [(10.75±2.70) vs. (19.68±6.95) min]. And inter-group difference was statistically significant (P=0.016). The first-phase stone clearance, postoperative hemoglobin loss and postoperative complication rate of SMP and MPCNL groups were (83.33±46.72) vs. (101.36±38.91) min, 83.33% vs. 81.81% and (7.59±7.51) vs. (7.75±8.77) g/L. respectively. No significant inter-group difference existed. No blood transfusion was provided. In SMP group, there was Clavien Ⅰ complication (n=1, 5.55%); In MPCNL group (n=5, 22.72%), postoperative fever subsided after aggressive anti-infection treatment. In two groups, there was on onset of Clavien Ⅱ/Ⅲ complications. Ultrasonic follow-ups were conducted for 1-2 years and hydronephrosis or renal atrophy disappeared in both groups. \u0000 \u0000 \u0000Conclusions \u0000Both SMP and MPCNL are safe, effective and mini-invasive for pediatric renal calculi. As compared with MPCNL, SMP has a smaller channel and a thinner mirror body. And SMP is more suitable for infants or toddlers with narrow calyx neck and lower calyx calculi. \u0000 \u0000 \u0000Key words: \u0000Renal calculus; Percutaneous nephrolithotomy; Child","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"7 1","pages":"248-251"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81856658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.020
Shuyi Zhao, Tianjing Liu, E. Wang
As an extremely rare disorder of unknown etiology, Gorham-Stout syndrome (GSS) occurs at any age. However, it is frequently found in children and youth. It is characterized by large bone dissolving as a characteristic tumor-like lesion. Most patients often present with multiple osteolysis, swelling, pain, limited mobility and severe pathologic fractures. Due to a low incidence, most reports have focused upon individual cases. Without a definite diagnostic and therapeutic standard, it has limited reference values for clinicians. With a purpose of enhancing its clinical awareness, this review summarized the etiology, pathogenesis, clinical manifestations, imaging findings, pathological features, diagnosis, differential diagnosis, complications, treatments and prognosis of GSS. Key words: Osteolysis, Essential; Child; Lymphatic vessels
{"title":"Research advances of Gorham-Stout syndrome","authors":"Shuyi Zhao, Tianjing Liu, E. Wang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.020","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.020","url":null,"abstract":"As an extremely rare disorder of unknown etiology, Gorham-Stout syndrome (GSS) occurs at any age. However, it is frequently found in children and youth. It is characterized by large bone dissolving as a characteristic tumor-like lesion. Most patients often present with multiple osteolysis, swelling, pain, limited mobility and severe pathologic fractures. Due to a low incidence, most reports have focused upon individual cases. Without a definite diagnostic and therapeutic standard, it has limited reference values for clinicians. With a purpose of enhancing its clinical awareness, this review summarized the etiology, pathogenesis, clinical manifestations, imaging findings, pathological features, diagnosis, differential diagnosis, complications, treatments and prognosis of GSS. \u0000 \u0000Key words: \u0000Osteolysis, Essential; Child; Lymphatic vessels","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"96 1","pages":"280-284"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89103817","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.006
Xiaolei Xie, W. Tan, Fuguang Li, Suhuan Tang
Objective To explore the correlation between fetal cardiovascular malformations and chromosome abnormalities. Methods Retrospective analysis was conducted for clinical data of 72 pregnant women with fetal cardiovascular system dysplasia from January 2013 to August 2018. And all 72 fetuses with abnormal cardiovascular system dysplasia were singletons with termination of pregnancy (n=45), loss of follow-up (n=6) and normal pregnancy (n=21). Results Twenty-two cases of abnormal karyotypes were detected in 72 patient samples with an abnormal rate of 30.6%(22/72), including 18-trisomy (n=10), 13-trisomy (n=3), 21-trisomy (n=2), sex chromosome mosaicism (n=1) and chromosomal structure abnormalities (n=6). As for fetal chromosome aneuploidy, the number of 18-trisomy accounted for 66.7%(10/15). There were single cardiovascular malformations (n=29), abnormal karyotypes (n=3), cardiovascular with other system malformations (n=43) and abnormal karyotypes (n=19). The abnormal karyotypic rate of cardiovascular malformations with other systems was higher than that of single cardiovascular malformations (44.2% vs. 10.3%, P=0.0035). As for fetal single cardiovascular system malformations, the distributions of gestational weeks were as follows: 28 weeks (n=4) & abnormal karyotype (n=1). As for fetal cardiovascular with other systemic malformations, the gestational weeks were 28 weeks (n=3) & abnormal karyotype (n=1). The abnormal karyotypic detection rates of cardiovascular malformation associated with urogenital, craniocerebral, facial neck, skeletal & extremity abnormalities were 100%(3/3), 71.4%(10/14), 58.8%(10/17) and 52.9%(9/17) respectively. However, no abnormal karyotype was detected in 8 cardiovascular cases with concurrent gastrointestinal malformations. Conclusions The number of chromosomes or structural changes is closely correlated with the abnormal development of fetal cardiovascular system. Key words: Fetus; Cardiovascular abnormalities; Chromosomes; Karyotyping
目的探讨胎儿心血管畸形与染色体异常的关系。方法回顾性分析2013年1月至2018年8月72例胎儿心血管系统发育不良孕妇的临床资料。72例异常心血管系统发育不良胎儿均为单胎，终止妊娠(n=45)、失访(n=6)、正常妊娠(n=21)。结果72例患者标本中检出异常核型22例，异常率为30.6%(22/72)，其中18-三体(n=10)、13-三体(n=3)、21-三体(n=2)、性染色体嵌合(n=1)、染色体结构异常(n=6)。胎儿染色体非整倍体，18-三体占66.7%(10/15)。其中单一心血管畸形29例，核型异常3例，心血管合并其他系统畸形43例，核型异常19例。合并其他系统的心血管畸形的核型异常率高于单一心血管畸形(44.2% vs. 10.3%， P=0.0035)。胎儿单一心血管系统畸形的孕周分布为:28周(n=4)，核型异常(n=1)。胎儿心血管合并其他全身性畸形，孕周28周(n=3)，核型异常(n=1)。心血管畸形合并泌尿生殖、颅脑、面颈、骨骼和四肢异常的异常核型检出率分别为100%(3/3)、71.4%(10/14)、58.8%(10/17)和52.9%(9/17)。8例心血管合并胃肠道畸形患者未见异常核型。结论染色体数目或结构变化与胎儿心血管系统发育异常密切相关。关键词:胎儿;心血管异常;染色体;核型分析
{"title":"Correlation between fetal cardiovascular malformations and chromosome abnormalities","authors":"Xiaolei Xie, W. Tan, Fuguang Li, Suhuan Tang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.006","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.006","url":null,"abstract":"Objective \u0000To explore the correlation between fetal cardiovascular malformations and chromosome abnormalities. \u0000 \u0000 \u0000Methods \u0000Retrospective analysis was conducted for clinical data of 72 pregnant women with fetal cardiovascular system dysplasia from January 2013 to August 2018. And all 72 fetuses with abnormal cardiovascular system dysplasia were singletons with termination of pregnancy (n=45), loss of follow-up (n=6) and normal pregnancy (n=21). \u0000 \u0000 \u0000Results \u0000Twenty-two cases of abnormal karyotypes were detected in 72 patient samples with an abnormal rate of 30.6%(22/72), including 18-trisomy (n=10), 13-trisomy (n=3), 21-trisomy (n=2), sex chromosome mosaicism (n=1) and chromosomal structure abnormalities (n=6). As for fetal chromosome aneuploidy, the number of 18-trisomy accounted for 66.7%(10/15). There were single cardiovascular malformations (n=29), abnormal karyotypes (n=3), cardiovascular with other system malformations (n=43) and abnormal karyotypes (n=19). The abnormal karyotypic rate of cardiovascular malformations with other systems was higher than that of single cardiovascular malformations (44.2% vs. 10.3%, P=0.0035). As for fetal single cardiovascular system malformations, the distributions of gestational weeks were as follows: 28 weeks (n=4) & abnormal karyotype (n=1). As for fetal cardiovascular with other systemic malformations, the gestational weeks were 28 weeks (n=3) & abnormal karyotype (n=1). The abnormal karyotypic detection rates of cardiovascular malformation associated with urogenital, craniocerebral, facial neck, skeletal & extremity abnormalities were 100%(3/3), 71.4%(10/14), 58.8%(10/17) and 52.9%(9/17) respectively. However, no abnormal karyotype was detected in 8 cardiovascular cases with concurrent gastrointestinal malformations. \u0000 \u0000 \u0000Conclusions \u0000The number of chromosomes or structural changes is closely correlated with the abnormal development of fetal cardiovascular system. \u0000 \u0000 \u0000Key words: \u0000Fetus; Cardiovascular abnormalities; Chromosomes; Karyotyping","PeriodicalId":10157,"journal":{"name":"中华小儿外科杂志","volume":"26 1","pages":"215-218"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90777634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.008
Yuan-Lung Yang, J. Zhan, Ying-chao Li, Hongxia Ren, Baohong Zhao, Weibing Tang, Heying Yang
Objective To enhance the awareness of biliary hypoplasia (BH) through exploring its diagnosis, treatment and prognosis. Methods For this retrospective study, the clinical data of BH were collected at five domestic hospitals from January 2009 to January 2017. There were 18 boys and 12 girls with an operative age of (83±36) days, a birth weight of (2.75±1.0) kg and a postnatal time of jaundice at (30±6) days. Their birth was term (n=21) and premature (n=9). And the stool was pale yellow stool (n=28) and white (n=2); dark urine (n=19); hepatomegaly & splenomegaly (n=18); mild ascites (n=12). Results All of them were operated. The procedures included cholecystostomy (n=20), cholangiography & biliary flushing (n=8) and non-treatment after cholangiography (n=2). And postoperative medications included antibiotics (n=15), hormone (n=22), hepatic protector & cholagogue (n=30) and gamma globulin (n=9). The follow-up period was 40 to 288 weeks. Jaundice subsided (n=21) and persisted (n=9) within 6 months. And 4/9 had the complications of itchiness (n=3) (1/3 with growth retardation) and anemia (n=1). They were divided into two groups of good prognosis (n=21) and poor prognosis (n=9) according to whether or not jaundice clearing at 6 months post-operation (icteric regression, total bilirubin ≤20 μmol/L), the growth up to standard and presence/absence of complications. Hepatocyte degeneration (n=6 vs. 8) and spot-like necrosis (n=3 vs. 7) were found in good and poor prognosis group. And the difference was statistically significant (P=0.004 & 0.002). Conclusions As a rare disease, BH may be diagnosed by cholangiography and liver biopsy and treated by surgery and postoperative medications. Surgical options include cholecystostomy and flushing. The overall prognosis is excellent. A poor prognosis may be associated with hepatic injury. Key words: Biliary Tract; Dysplasia; Retrospective studies; Multicenter study
目的探讨胆道发育不全的诊断、治疗及预后，提高对胆道发育不全的认识。方法回顾性分析2009年1月至2017年1月国内5家医院BH的临床资料。男18例，女12例，手术年龄(83±36)天，出生体重(2.75±1.0)kg，出生后黄疸时间(30±6)天。他们的出生是足月(n=21)和早产(n=9)。大便呈淡黄色(n=28)，白色(n=2);深色尿(n=19);肝脾肿大(18例);轻度腹水(n=12)。结果全部手术成功。手术包括胆囊造口术(n=20)、胆道造影和胆道冲洗(n=8)和胆道造影后不治疗(n=2)。术后用药包括抗生素(15例)、激素(22例)、护肝催胆剂(30例)、丙种球蛋白(9例)。随访期为40 ~ 288周。6个月内黄疸消退(21例)，持续(9例)。4/9合并发痒(n=3)(1/3合并生长迟缓)和贫血(n=1)。根据术后6个月黄疸是否清除(黄疸消退、总胆红素≤20 μmol/L)、生长是否达标、有无并发症分为预后良好组(21例)和预后不良组(9例)。预后良好组和预后不良组均出现肝细胞变性(n=6 vs. 8)和点状坏死(n=3 vs. 7)。差异有统计学意义(P=0.004 & 0.002)。结论BH是一种罕见的疾病，可通过胆管造影和肝活检诊断，并可通过手术和术后药物治疗。手术选择包括胆囊造口术和冲洗。整体预后良好。预后不良可能与肝损伤有关。关键词:胆道;发育不良;回顾性研究;多中心研究
{"title":"Diagnosis and treatment of biliary hypoplasia: a multicenter survey","authors":"Yuan-Lung Yang, J. Zhan, Ying-chao Li, Hongxia Ren, Baohong Zhao, Weibing Tang, Heying Yang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.008","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.008","url":null,"abstract":"Objective \u0000To enhance the awareness of biliary hypoplasia (BH) through exploring its diagnosis, treatment and prognosis. \u0000 \u0000 \u0000Methods \u0000For this retrospective study, the clinical data of BH were collected at five domestic hospitals from January 2009 to January 2017. There were 18 boys and 12 girls with an operative age of (83±36) days, a birth weight of (2.75±1.0) kg and a postnatal time of jaundice at (30±6) days. Their birth was term (n=21) and premature (n=9). And the stool was pale yellow stool (n=28) and white (n=2); dark urine (n=19); hepatomegaly & splenomegaly (n=18); mild ascites (n=12). \u0000 \u0000 \u0000Results \u0000All of them were operated. The procedures included cholecystostomy (n=20), cholangiography & biliary flushing (n=8) and non-treatment after cholangiography (n=2). And postoperative medications included antibiotics (n=15), hormone (n=22), hepatic protector & cholagogue (n=30) and gamma globulin (n=9). The follow-up period was 40 to 288 weeks. Jaundice subsided (n=21) and persisted (n=9) within 6 months. And 4/9 had the complications of itchiness (n=3) (1/3 with growth retardation) and anemia (n=1). They were divided into two groups of good prognosis (n=21) and poor prognosis (n=9) according to whether or not jaundice clearing at 6 months post-operation (icteric regression, total bilirubin ≤20 μmol/L), the growth up to standard and presence/absence of complications. Hepatocyte degeneration (n=6 vs. 8) and spot-like necrosis (n=3 vs. 7) were found in good and poor prognosis group. And the difference was statistically significant (P=0.004 & 0.002). \u0000 \u0000 \u0000Conclusions \u0000As a rare disease, BH may be diagnosed by cholangiography and liver biopsy and treated by surgery and postoperative medications. Surgical options include cholecystostomy and flushing. The overall prognosis is excellent. A poor prognosis may be associated with hepatic injury. \u0000 \u0000 \u0000Key words: \u0000Biliary Tract; Dysplasia; Retrospective studies; Multicenter study","PeriodicalId":10157,"journal":{"name":"中华小儿外科杂志","volume":"1 1","pages":"224-229"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89643206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.004
C. Tao, D. Tang, Zheming Xu, Long Sun, Z. Gao, Qingjiang Chen, Q. Shu
Objective To summarize the experience of robotic-assistant laparoscopic pyeloplasty (RALP) for ureteropelvic junction obstruction (UPJO) with small pelvis in children. Methods A retrospective analysis was performed on clinical data of 12 UPJO children (small pelvis) undergoing RALP from March 2018 to October 2019. Their clinical presentations, operative approaches and follow-up data were analyzed. There were 7 boys and 5 girls with a mean age of 7(4-15) years. The involved side was left (n=9) and right (n=3). Ultrasound at 1-7 days preoperatively indicated anterioposterior diameter (APD) 3.5 cm during an on set of abdominal pain. And radionuclide examination hinted at different declining levels of renal function and the function of affected kidney ranged from 6% to 43%. Results RALP was performed all successfully without a conversion into open surgery. The pathogenesis was simple stricture or torsion (n=3), polyps (n=3), ectopic vascular compression (n=4), calculus incarceration with infection (n=1) and postcaval ureter (n=1). The average operative duration was 165(115-225) min and the average ureteropelvic anastomosis time 50(40-60) min. There were no obvious intraoperative complications and the mean volume of blood loss was <10 ml. The average follow-up period was 12(2-22) months. The mean preoperative value of APD was (1.87±0.57)cm and the mean postoperative value of APD (1.16±0.82)cm (P<0.05). Conclusions Robot-assisted laparoscopic pyeloplasty may be employed for pediatric UPJO with small renal pelvis (APD<2.5 cm). Such a technique is easy to learn and clinical application prospects are excellent. Key words: Child; Robot assisted laparoscopic surgery; Hydronephrosis; Ureteropelvic junction obstruction
{"title":"Outcomes of robot-assisted laparoscopic pyeloplasty for ureteropelvic junction obstruction with small pelvis in children","authors":"C. Tao, D. Tang, Zheming Xu, Long Sun, Z. Gao, Qingjiang Chen, Q. Shu","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.004","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.004","url":null,"abstract":"Objective \u0000To summarize the experience of robotic-assistant laparoscopic pyeloplasty (RALP) for ureteropelvic junction obstruction (UPJO) with small pelvis in children. \u0000 \u0000 \u0000Methods \u0000A retrospective analysis was performed on clinical data of 12 UPJO children (small pelvis) undergoing RALP from March 2018 to October 2019. Their clinical presentations, operative approaches and follow-up data were analyzed. There were 7 boys and 5 girls with a mean age of 7(4-15) years. The involved side was left (n=9) and right (n=3). Ultrasound at 1-7 days preoperatively indicated anterioposterior diameter (APD) 3.5 cm during an on set of abdominal pain. And radionuclide examination hinted at different declining levels of renal function and the function of affected kidney ranged from 6% to 43%. \u0000 \u0000 \u0000Results \u0000RALP was performed all successfully without a conversion into open surgery. The pathogenesis was simple stricture or torsion (n=3), polyps (n=3), ectopic vascular compression (n=4), calculus incarceration with infection (n=1) and postcaval ureter (n=1). The average operative duration was 165(115-225) min and the average ureteropelvic anastomosis time 50(40-60) min. There were no obvious intraoperative complications and the mean volume of blood loss was <10 ml. The average follow-up period was 12(2-22) months. The mean preoperative value of APD was (1.87±0.57)cm and the mean postoperative value of APD (1.16±0.82)cm (P<0.05). \u0000 \u0000 \u0000Conclusions \u0000Robot-assisted laparoscopic pyeloplasty may be employed for pediatric UPJO with small renal pelvis (APD<2.5 cm). Such a technique is easy to learn and clinical application prospects are excellent. \u0000 \u0000 \u0000Key words: \u0000Child; Robot assisted laparoscopic surgery; Hydronephrosis; Ureteropelvic junction obstruction","PeriodicalId":10157,"journal":{"name":"中华小儿外科杂志","volume":"24 1","pages":"205-209"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82756291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.015
Yiyang Liu, Li-feng Shen, B. Lin, Kai Huang, Qiong Zhang, Chun Zhang, Q. Guo
Objective To explore the efficacy and application value of free great toe fibular flap transplantation for traumatic soft tissue defect of hands in children. Methods From July 2013 to April 2018, 11 children with hand soft tissue defects due to mechanical trauma were treated with free fibular flap. There were 10 boys and 1 girl with an average age of 11.5(5-17) years. The area of transplanted skin flaps was at 3.0 cm×1.5 cm the smallest and at 5.5 cm×2.5 cm the largest. The first dorsal metatarsal arteries (or dorsalis pedis) were anastomosed with radial arteries. Full-thickness inguinal skin was employed for free skin grafting at donor site of flaps. Results The average operative duration was 3.5 hours. One artery and two veins were anastomosed. Postoperative vascular crisis occurred in 1 case. Skin flaps survived after timely surgical exploration. And all flaps survived completely in 11 cases. Necrosis of skin graft occurred in 3 cases and there was subcrustal healing after dressing change. After an average follow-up period of 10 months, skin flaps were satisfactory in appearance, normal in color, excellent elasticity and texture and basically consistent with the surrounding tissues of recipient area. According to Michigan Hand Outcome Questionnaire, the outcomes were " very satisfied" with appearance (n=11), " very satisfied" with the function evaluation of injured fingers (n=7), " satisfied" (n=2), " unsatisfactory" (n=1) and " very unsatisfactory" (n=1). There was no obvious disturbance of toe motor function at donor site of flap. Conclusions On the basis of mastering microvascular anastomosis technique and paying attention to comprehensive managements of perioperative period of children, the transplantation of free great toe fibular flap for repairing soft tissue defect in hands has achieved satisfactory outcomes. The recipient area is aesthetic with concealed donor area and excellent functions of fingers and toes. It is worthy of wider clinical applications. Key words: Surgical flap; Microsurgery; Transplantation; Child; Soft tissue injuries; Hand injuries
目的探讨游离踇趾腓骨皮瓣移植治疗儿童外伤性手部软组织缺损的疗效及应用价值。方法2013年7月~ 2018年4月，对11例因机械损伤导致手部软组织缺损的患儿采用游离腓骨瓣修复。男10例，女1例，平均年龄11.5岁(5-17岁)。移植皮瓣面积最小3.0 cm×1.5 cm，最大5.5 cm×2.5 cm。第一跖背动脉(或足背动脉)与桡动脉吻合。采用全层腹股沟皮肤在皮瓣供区游离植皮。结果平均手术时间3.5 h。一条动脉和两条静脉吻合。术后血管危象1例。皮瓣在及时手术探查后存活。11例皮瓣全部存活。3例移植皮出现坏死，换药后皮下愈合。平均随访10个月，皮瓣外观满意，颜色正常，弹性和质地优良，与受区周围组织基本一致。根据《Michigan Hand Outcome Questionnaire》，对损伤手指的外观评价为“非常满意”(n=11)，对损伤手指的功能评价为“非常满意”(n=7)，“满意”(n=2)，“不满意”(n=1)，“非常不满意”(n=1)。皮瓣供区足部运动功能未见明显改变。结论在掌握微血管吻合技术的基础上，注意儿童围手术期的综合管理，游离踇趾腓骨瓣移植修复手部软组织缺损取得了满意的效果。受区美观，供区隐蔽，手指、脚趾功能良好。值得临床推广应用。关键词:外科皮瓣;显微外科;移植;孩子;软组织损伤;手受伤
{"title":"Transplantation of free great toe fibular flap for repairing traumatic soft tissue defects of hands in children","authors":"Yiyang Liu, Li-feng Shen, B. Lin, Kai Huang, Qiong Zhang, Chun Zhang, Q. Guo","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.015","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.015","url":null,"abstract":"Objective \u0000To explore the efficacy and application value of free great toe fibular flap transplantation for traumatic soft tissue defect of hands in children. \u0000 \u0000 \u0000Methods \u0000From July 2013 to April 2018, 11 children with hand soft tissue defects due to mechanical trauma were treated with free fibular flap. There were 10 boys and 1 girl with an average age of 11.5(5-17) years. The area of transplanted skin flaps was at 3.0 cm×1.5 cm the smallest and at 5.5 cm×2.5 cm the largest. The first dorsal metatarsal arteries (or dorsalis pedis) were anastomosed with radial arteries. Full-thickness inguinal skin was employed for free skin grafting at donor site of flaps. \u0000 \u0000 \u0000Results \u0000The average operative duration was 3.5 hours. One artery and two veins were anastomosed. Postoperative vascular crisis occurred in 1 case. Skin flaps survived after timely surgical exploration. And all flaps survived completely in 11 cases. Necrosis of skin graft occurred in 3 cases and there was subcrustal healing after dressing change. After an average follow-up period of 10 months, skin flaps were satisfactory in appearance, normal in color, excellent elasticity and texture and basically consistent with the surrounding tissues of recipient area. According to Michigan Hand Outcome Questionnaire, the outcomes were \" very satisfied\" with appearance (n=11), \" very satisfied\" with the function evaluation of injured fingers (n=7), \" satisfied\" (n=2), \" unsatisfactory\" (n=1) and \" very unsatisfactory\" (n=1). There was no obvious disturbance of toe motor function at donor site of flap. \u0000 \u0000 \u0000Conclusions \u0000On the basis of mastering microvascular anastomosis technique and paying attention to comprehensive managements of perioperative period of children, the transplantation of free great toe fibular flap for repairing soft tissue defect in hands has achieved satisfactory outcomes. The recipient area is aesthetic with concealed donor area and excellent functions of fingers and toes. It is worthy of wider clinical applications. \u0000 \u0000 \u0000Key words: \u0000Surgical flap; Microsurgery; Transplantation; Child; Soft tissue injuries; Hand injuries","PeriodicalId":10157,"journal":{"name":"中华小儿外科杂志","volume":"14 1","pages":"262-267"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84851172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Objective To explore the clinical characteristics and causes and countermeasures of misdiagnosing mixed gonadal dysgenesis (MGD). Methods From May 2013 to April 2018, clinical data were retrospectively reviewed for 24 MGD children. The average age was 21(10-39) months and the average height 83(71-97) cm. Ten children fell below the average height of the same age group by 2 standard deviations. There were 22 boys and 2 girls. The Prader grade was II (n=3), III (n=15) and IV (n=6). The results of sex hormone determination and sex-related genes were analyzed.Fluorescence in situ hybridization (FISH) for sex chromosome was further tested in 8/10 children with 46, XY chromosome. And gonad specimens were evaluated for histopathology. Results The average level of anti-mullerian hormone (AMH) was 69.42(16.57-189.92) ng/ml. The average testosterone level was 4.93(0.71-8.09) nmol/L after hCG stimulation. WT1 gene mutation was detected in 1 child with a definite diagnosis of Danis-Drash syndrome (DDS). The karyotypes were 45, X/46, XY, 10 cases of 46, XY (n=12, including 8 cases confirmed by FISH as X chimeric XY), 45, X/46, XY/47XYY (n=1) and 45, X/47, XYY/48XYYY (n=1). Forty-eight specimens were classified as dyspastic testis (n=24), undifferentiated gonad tissue (n=1) (previously misdiagnosed as ovary tissue) and fibrous stripe gonads with sex-cord-like structure (n=4). No evidence of malignancy was noticed. Conclusions Ambiguous genitalia is common in MGD children with Muller tube remnant. For suspected MGD children with a 46, XY chromosome, further FISH test for sex chromosome is warranted. And undifferentiated gonad tissue may be found in a streak gonad and ovary tissue is probably mistaken for making a misdiagnosis of ovotesticular DSD. Key words: Gonadal dysgenesis; Sex chromosomes; Histopathology
{"title":"Clinical characteristics and analysis of misdiagnosing mixed gonadal dysgenesis","authors":"Yongchuan Cai, Yu Mao, Jing Fu, L. Dai, Shaoji Chen, Xunjun Wang, Daorui Qin, Jiewen Zheng, Yu Liu, Yunman Tang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.014","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.014","url":null,"abstract":"Objective \u0000To explore the clinical characteristics and causes and countermeasures of misdiagnosing mixed gonadal dysgenesis (MGD). \u0000 \u0000 \u0000Methods \u0000From May 2013 to April 2018, clinical data were retrospectively reviewed for 24 MGD children. The average age was 21(10-39) months and the average height 83(71-97) cm. Ten children fell below the average height of the same age group by 2 standard deviations. There were 22 boys and 2 girls. The Prader grade was II (n=3), III (n=15) and IV (n=6). The results of sex hormone determination and sex-related genes were analyzed.Fluorescence in situ hybridization (FISH) for sex chromosome was further tested in 8/10 children with 46, XY chromosome. And gonad specimens were evaluated for histopathology. \u0000 \u0000 \u0000Results \u0000The average level of anti-mullerian hormone (AMH) was 69.42(16.57-189.92) ng/ml. The average testosterone level was 4.93(0.71-8.09) nmol/L after hCG stimulation. WT1 gene mutation was detected in 1 child with a definite diagnosis of Danis-Drash syndrome (DDS). The karyotypes were 45, X/46, XY, 10 cases of 46, XY (n=12, including 8 cases confirmed by FISH as X chimeric XY), 45, X/46, XY/47XYY (n=1) and 45, X/47, XYY/48XYYY (n=1). Forty-eight specimens were classified as dyspastic testis (n=24), undifferentiated gonad tissue (n=1) (previously misdiagnosed as ovary tissue) and fibrous stripe gonads with sex-cord-like structure (n=4). No evidence of malignancy was noticed. \u0000 \u0000 \u0000Conclusions \u0000Ambiguous genitalia is common in MGD children with Muller tube remnant. For suspected MGD children with a 46, XY chromosome, further FISH test for sex chromosome is warranted. And undifferentiated gonad tissue may be found in a streak gonad and ovary tissue is probably mistaken for making a misdiagnosis of ovotesticular DSD. \u0000 \u0000 \u0000Key words: \u0000Gonadal dysgenesis; Sex chromosomes; Histopathology","PeriodicalId":10157,"journal":{"name":"中华小儿外科杂志","volume":"3 1","pages":"257-261"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78733495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-03-15DOI: 10.3760/CMA.J.ISSN.0253-3006.2020.03.005
Shengchun Yang, Li Ma, M. Zou, W. D. Chen, Techang Liu, Mingjie Zhang, Xinxin Chen
Objective To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations. Methods Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017. There were 7 boys and 7 girls with a mean age of (18.23±28.55 months)(23 days to 9 years). The complications included other cardiac abnormalities (n=10), mesocardia (n=8), right pulmonary hypoplasia (n=7) and collaterall circulation (n=8). Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein (SV). And postoperative survival curves were plotted. Results SVs were dissected from inferior caval vein (IVC) and anastomosed to left artrium (n=9); SVs (2 SVs) were resected together with partial IVC wall and anastomosed to left artrium (n=1); SVs (2 SVs) were resected respectively and anastomosed side-to-side and then anastomosed to left artrium (n=1); SVs were anastomosed to right atrium and interatrium septum be rebuilt (n=2) and SV was connected to artificial conduit and then connected to left atrium (n=1) owing to that SV was too far to left atrium. In the same term of operation, complicated cardiac abnormality was cured. And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%; 1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%, 73% and 73% respectively. One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups. Conclusions Scimitar syndrome may be complicated with other cardiac abnormality, mesocardia and pulmonary hypoplasia, etc. Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium. SV should be anastomosed to right atrium instead of left atrium and interatrium septum. Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively. Key words: Scimitar Syndrome; Congenital heart disease; Cardiac surgical procedures
{"title":"Surgical outcomes of 14 children with scimitar syndrome","authors":"Shengchun Yang, Li Ma, M. Zou, W. D. Chen, Techang Liu, Mingjie Zhang, Xinxin Chen","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.005","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.005","url":null,"abstract":"Objective \u0000To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations. \u0000 \u0000 \u0000Methods \u0000Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017. There were 7 boys and 7 girls with a mean age of (18.23±28.55 months)(23 days to 9 years). The complications included other cardiac abnormalities (n=10), mesocardia (n=8), right pulmonary hypoplasia (n=7) and collaterall circulation (n=8). Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein (SV). And postoperative survival curves were plotted. \u0000 \u0000 \u0000Results \u0000SVs were dissected from inferior caval vein (IVC) and anastomosed to left artrium (n=9); SVs (2 SVs) were resected together with partial IVC wall and anastomosed to left artrium (n=1); SVs (2 SVs) were resected respectively and anastomosed side-to-side and then anastomosed to left artrium (n=1); SVs were anastomosed to right atrium and interatrium septum be rebuilt (n=2) and SV was connected to artificial conduit and then connected to left atrium (n=1) owing to that SV was too far to left atrium. In the same term of operation, complicated cardiac abnormality was cured. And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%; 1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%, 73% and 73% respectively. One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups. \u0000 \u0000 \u0000Conclusions \u0000Scimitar syndrome may be complicated with other cardiac abnormality, mesocardia and pulmonary hypoplasia, etc. Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium. SV should be anastomosed to right atrium instead of left atrium and interatrium septum. Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively. \u0000 \u0000 \u0000Key words: \u0000Scimitar Syndrome; Congenital heart disease; Cardiac surgical procedures","PeriodicalId":10157,"journal":{"name":"中华小儿外科杂志","volume":"50 1","pages":"210-214"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80334748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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