Objective:To analyze the outcome and its influencing factors of fetal congenital diaphragmatic hernia (FCHD).Methods:To retrospectively analyze the data of fetal diaphragmatic hernia fetuses detected prenatal in Northwest Women and Children's Hospital from January 2015 to December 2019, including gestational age, number of left and right side, lung area to head circumference ratio (LHR), number of liver herniation cases, combined malformations and delivery. The combined malformations, gestational age, birth weight, treatment and outcome of neonates with diaphragmatic hernia after delivery were analyzed.Results:36% of FCHD had combined malformations, and at least 55% (71/128) cases were induced labor. The gestational age of the fetus with LHR<1.4 was earlier than those with LHR≥1.4. The gestational age of the fetus with combined malformations was earlier than those without combined malformations. The gestational age of the fetus with liver herniation is later than those without liver herniation. There was no statistically significant difference in LHR between fetuses with and without combined malformations, and fetuses with and without liver herniation, respectively. Liver herniation is more likely to be combined with other malformations in the fetus. There was no significant difference in the incidence of left and right sides with combined malformations. Of the 40 children delivered, there were seven at high risk and 4 cases of death.Conclusion:Fetal diaphragmatic hernia has high proportion of combined malformations and high rate of induced labor. The cure rate of simple diaphragmatic hernia is high. The assess of fetal prognosis by the LHR and liver herniation should be further improved.

目的 探讨先天性心脏病患者行开胸手术与微创心脏手术的临床价值.方法 回顾性分析本单位2015年01月至2019年12月间确诊罹患先天性心脏病的300例患者临床病案资料作研究样本,依据手术类型差异进行分组,其中200例入开胸组,施行开胸手术;另100例入微创组,施行微创心脏手术.比较两组手术用时、术中出血量、住院时间、主动脉阻断时间、体外循环时间、胸液引流量、切口长度、生存质量评估量表(Spitzer)差异.结果 两组手术用时比较,差异无统计学意义(P＞0.05);微创组术中出血量、住院时间、主动脉阻断时间、体外循环时间、胸液引流量、切口长度均小于开胸组,差异有统计学意义(P＜0.05);两组术前Spitzer各维度评分比较,差异无统计学意义(P＞0.05);术后1周重测,发现微创组健康、精神、支持、活动四项基本维度评分均高于开胸组,差异有统计学意义(P＜0.05).结论 先天性心脏病患者接受微创心脏手术治疗,手术指标更佳,创伤性更小,术后生存质量更高.

Objective:To explore clinical treatment strategies of children with severe congenital diaphragmatic hernia (CDH).Methods:A total of 7 cases of children with severe CDH who were treated in Northwest Women′s and Children′s Hospital from January 2015 to October 2019 were selected as research subjects. Their general clinical data, treatment outcomes, prognosis and postoperative follow-up results were collected and analyzed by retrospective method. This study met the requirements of World Medical Association Declaration of Helsinki revised in 2013.Results:The analysis results of clinical data of 7 children with severe CDH in this study were as follows. ①General clinical data: 4 cases of male children (No.2, 5-7) and 3 cases of female children (No.1, 3-4) respectively. Among them, 1 case (No. 4) was a full-term infant with low birth weight, 1 case (No. 7) was a full-term infant with low birth weight and multisystem malformations, and 5 cases (No.1-3, 5-6) were premature infants with low birth weight. 2 cases (No.2, 7) were infants born with assisted reproductive technology and complicated with chromosomal abnormalities. Delivery mode of pregnant mothers: 5 cases (No. 1-4, 7) were delivered by cesarean section, and 2 cases (No. 5-6) had natural birth. 4 cases of pregnant mothers (No. 1-2, 6-7) had abortion history. ②Treatment effects: except for the No.6 child with 10 scores of Apgar score at 1, 5, and 10 min after birth, the other 6 cases received tracheal intubation and high frequency oscillatory ventilation (HFOV) in the operating room or delivery room, and also received fasting and gastrointestinal decompression therapy. Due to multiple complex malformations, the guardian of No.1 child gave up treatment. The other 6 children were treated with CDH repair surgery, 3 cases (No.4-6) were operated through the left posterolateral thoracic incision, and 3 cases (No. 2-3, 7) were operated through the left upper abdominal incision. ③Prognosis: 4 cases had a poor prognosis during perioperative period, of which 1 case (No. 1) died after giving up treatment before operation, and 3 cases (No. 3-4, 7) died after operation. Among them, except for No.4 child, No.1, 3, and 7 children have complicated congenital heart disease and other system malformations. The other 3 children (No. 2, 5-6) were cured and discharged from hospital after surgery, and they all had premature delivery, low birth weight, and other uncomplicated deformities. Postoperative follow-up results of these 3 cured children showed that 1 case (No. 2) had CDH recurrence in 1 month after operation, and after receiving CDH repair surgery again, the diaphragm was intact and without recurrence of CDH at the end of follow-up. And No. 5-6 children grew well at the end of follow-up.Conclusions:Complication of congenital heart disease may be one of the main factors affecting the prognosis of children with severe CDH. As to children with severe CDH, treatment strategies such as immediate tracheal intubation, HFOV, and delayed surgical repair (DSR) after delivery may be helpful to improve the surgical cure rate of them.

目的 观察先天性巨结肠术后患者感染病原菌的分布及血清基质细胞衍生因子-1(SDF-1)、白细胞介素-8(IL-8)、IL-17水平的变化,并探讨其临床意义.方法 回顾性分析2015年1月至2020年6月西北妇女儿童医院收治的78例先天性巨结肠手术患儿作为研究对象,根据其术后并发感染与否分组,其中感染组33例,未感染组45例.感染组均行病原学检测,统计病原菌分布情况,比较不同感染程度患儿的病原菌构成比;比较感染组和未感染组患儿血清SDF-1、IL-8、IL-17水平,采用Pearson相关系数模型探究血清SDF-1、IL-8、IL-17水平与常规感染标志物[降钙素原(PCT)、C反应蛋白(CRP)]的相关性,采用受试者工作特征(ROC)曲线评价血清各指标对重症感染的诊断价值.结果 感染组患儿共分离44株病原菌,革兰阴性菌占63.64％,革兰阳性菌占31.82％,真菌占4.55％;感染组患儿随感染程度加重,病原菌分布由革兰阳性菌感染为主逐渐转变为革兰阴性菌感染为主,轻度患儿革兰阳性菌株数占42.86％,中度患儿革兰阳性菌株数占35.71％,重度患儿革兰阳性菌株数占21.43％,不同感染程度患儿革兰阳性菌比较差异有统计学意义(P<0.05);感染组和未感染组患儿血清SDF-1[(625.67±70.78)ng/L vs(327.41±35.47)ng/L]、IL-8[(21.14±2.87)pg/mL vs(14.37±1.64)pg/mL]、IL-17[(17.26±4.32)pg/mL vs(5.17±1.94)pg/mL]水平比较,感染组明显高于未感染组,差异均有统计学意义(P<0.05);先天性巨结肠术后感染患儿血清SDF-1、IL-8、IL-17水平与血清PCT、CRP水平呈正相关(P<0.05);血清SDF-1、IL-8、IL-17联合诊断重度感染的曲线下面积(AUC)为0.854,大于各指标单一诊断的AUC(0.817、0.796、0.713),联合诊断的最佳敏感度为90.00％,特异度为73.91％.结论 先天性巨结肠术后感染病原菌以革兰阴性菌为主,感染患儿血清SDF-1、IL-8、IL-17水平明显升高,各指标联合检测可为临床诊断重度感染提供重要参考.

目的 研究单孔腹腔镜疝裳高位结扎术治疗腹股沟斜疝的手术效果.方法 选取2017年2月至2020年2月我院收治的腹股沟斜疝患儿180例,按照手术方法不同分为2组,其中90例采用小切口疝囊高位结扎术为小切口组,90例采用单孔腹腔镜疝囊高位结扎术为腹腔镜组.比较两组围术期指标(术中出血量、手术时间、下床活动时间、排气时间、住院时间)、术后6h、12h、24 h、48 h疼痛程度[表情-肢体动作-行为-哭闹-安慰性(FLACC)评分]、术前、术后24 h应激反应[白细胞计数(WBC)、血清C反应蛋白(CRP)、白细胞介素-10(IL-10)]、并发症发生率及复发率.结果 腹腔镜组术中出血量小于小切口组,手术时间、下床活动时间、排气时间、住院时间短于小切口组;术后6h、12h、24h、48 h腹腔镜组FLACC评分低于小切口组;术后24 h腹腔镜组WBC、血清CRP、IL-10水平低于小切口组;腹腔镜组并发症发生率低于小切口组,复发率低于小切口组,差异均有统计学意义(P＜0.05).结论 单孔腹腔镜疝囊高位结扎术治疗腹股沟斜疝能减小术中出血量,缩短恢复时间,减轻疼痛程度及应激反应,降低并发症发生率及复发率.

目的 总结腹腔镜鞘状突高位结扎治疗腹阴囊型鞘膜积液的临床经验.方法 回顾性分析腹阴囊型鞘膜积液的临床表现、超声所见、术中所见及术后效果.结果 6例腹阴囊鞘膜积液患儿,5例术前诊断精索鞘膜积液.查体腹股沟至阴囊体积大、张力高囊性包块,透光阳性.超声探查睾丸后上方包块并延伸至腹腔,积液量多,有时可见分隔.囊性包块均自内环口处脱出.2例先将腹腔包块还纳至鞘突管,再行鞘状突高位结扎,最后经阴囊抽出积液.4例先穿刺针刺破腹腔内囊性包块,将囊液排尽,囊膜还纳至鞘突管后,行鞘状突高位结扎.术后随访10个月至24个月,临床及超声检查,均无再次出现症状.结论 腹腔镜鞘状突高位结扎治疗疗腹阴囊型鞘膜积液效果确切.

SALL4 is a zinc finger transcription factor that exerts its physiological role during embryo-fetal development. Analyses of SALL4 expression have shown its oncogenic role in precursor B-cell lymphoblastic lymphoma, acute and chronic myeloid leukemia, gastrointestinal, breast, and lung cancers. The aim of this study was to determine the immunohistochemical profile of SALL4 in pediatric yolk sac tumors (YSTs). Immunohistochemistry detection of SALL4 was performed in 22 cases of pediatric YSTs and 10 mature teratomas. The percentage of tumor cells stained was scored as 0, 1+ (1–30% cells), 2+ (31–60%), 3+ (61–90%), and 4+ (> 90%). To compare its sensitivity and specificity with Glypican-3 and α-fetoprotein (AFP), we also stained tumors from these cases for Glypican-3 and AFP. In contrast to AFP and glypican-3, SALL4 staining in more than 90% of the tumor cells was seen in all 22 pediatric YSTs (100% sensitivity) (P < 0.001 for both SALL4 vs. AFP and SALL4 vs. glypican-3). SALL4 is a sensitive marker for pediatric YSTs and it can be used to distinguish them from mature teratomas. SALL4 is likely to become a new and valuable biomarker for the diagnosis of pediatric YST.

目的 比较胸腔镜手术与传统开放性手术治疗新生儿食管闭锁的临床效果.方法 选择2015年1月至2018年12月西北妇女儿童医院收治的70例食管闭锁新生儿为研究对象,所有患儿行手术治疗,根据手术方法将患儿分为胸腔镜手术组(n=36)和开放性手术组(n=34),比较2组患儿手术时间、术中出血量、住院时间及术后并发症发生率,分别于术前及术后第3天检测患儿血清促胃动素(MTL)、胆囊收缩素(CCK)和促胃液素(GAS)水平.结果 胸腔镜手术组患儿手术时间、住院时间显著短于开放性手术组,术中出血量显著少于开放性手术组(P＜0.05).2组患儿术前血清MTL、CCK及GAS水平比较差异无统计学意义(P＞0.05);与术前比较,2组患儿术后第3天血清MTL、CCK水平显著降低,血清GAS水平显著升高(P＜0.05);术后第3天,胸腔镜手术组患儿血清MTL、CCK水平显著高于开放性手术组,血清GAS水平显著低于开放性手术组(P＜0.05).胸腔镜手术组患儿术后食管吻合口狭窄、食管吻合口瘘、进食困难、胃食管反流、肺部感染发生率分别为13.89％ (5/36)、11.11％ (4/36)、11.11％ (4/36)、13.89％ (5/36)、16.67％ (6/36),开放性手术组患儿术后食管吻合口狭窄、食管吻合口瘘、进食困难、胃食管反流、肺部感染发生率分别为17.65％ (6/34)、20.59％ (7/34)、41.18％ (14/34)、35.29％ (12/34)、47.06％ (16/34);2组患儿食管吻合口狭窄及食管吻合口瘘发生率比较差异无统计学意义(P＞0.05);胸腔镜手术组患儿进食困难、胃食管反流及肺部感染发生率显著低于开放性手术组(P＜0.05).结论 胸腔镜手术治疗新生儿食管闭锁具有手术微创小、术中出血量少、术后恢复快、对消化功能影响小、术后并发症发生率低等优势.

胸膜肺母细胞瘤(pleuropulmonary blastoma,PPB)是儿童较罕见的恶性胸部肿瘤,因临床与影像学表现无特异性,常易误诊为先天性肺气管发育畸形、纵膈肿瘤等[1-4].该病的误诊率高,诊治困难,预后不良.2014年8月至2018年1月西北妇女儿童医院收治4例PPB患儿,本文旨在总结其临床表现及诊治经验,以提高医生对该病的认识和诊治能力.

OBJECTIVE:To investigate the effect of modified Bianchi (single incision in the midline of the scrotum) orchiopexy (MBO) versus that of traditional surgery in the treatment of median or low cryptorchidism. METHODS:Eighty-two children with median or low cryptorchidism were treated from February 2013 to February 2014, 46 (53 testes) by MBO and the other 36 by the traditional method of inguinal incision (control, 40 testes). Comparisons were made in the operation time and postoperative complications between the two surgical strategies. RESULTS:The mean operation time was significantly shorter in the MBO group than in the control (［25±6］ vs ［35±4］ min, P<0.05). No testicular atrophy, hernias or hydrocele was found in either group during the 1－2 years of follow-up. Testis retraction was observed in 3 cases in the MBO group as compared with 2 in the control (P>0.05). The incision scar was obvious in all the controls, with 1 case of postoperative inguinal hematoma, but almost invisible in all the MBO cases. CONCLUSIONS:Modified Bianchi orchiopexy is superior to traditional surgery in the treatment of median or low cryptorchidism for its advantages of short operation time, few complications, and satisfactory appearance of the healed incision.

患儿女,6个月,体质量5.4kg。因先天性心脏病于外院就诊,诊断:右心室双出口,室间隔缺损,房间隔缺损,肺动脉高压。术前X线胸片提示心影增大,双肺纹理增浓,符合先心病表现(图1)。在全麻体外循环下行右心室双出口矫治+房间隔缺损修补术,过程顺利,术后16 h脱离呼吸机。术后第4天突然出现呼吸急促、发绀,X线胸片提示胸腔左

纵隔肿瘤是小儿胸部较常见肿瘤，加强围术期患儿护理，不仅协助医生顺利完成患儿治疗，还可以促进患儿恢复，减少术后并发症发生。我院于2000 年1 月至2013 年6 月收治的纵膈肿瘤患儿44 例，现分析报告如下。

BACKGROUND:This study was undertaken to evaluate the new method for the reconstruction of the pulmonary artery in arterial switch operation (ASO).METHODS:A total of 108 consecutive infants with congenital heart disease were treated with ASO in our department between January 2004 and June 2012. The new pulmonary arterial root was reconstructed with a fresh autologuos pericardium which was clipped pants-like with continuous mattress suture of 6-0 Prolene thread. Patients were reexamined consecutively at 3 and 6 months and 1, 2 and 6 years after discharge. The pulmonary arterial blood velocity was measured by continuous Doppler during systole. The pulmonary flow of healthy children of same age was also measured in the control group. Simplified Bernoulli formula was used to calculate the pressure gradient via the pulmonary artery for determining whether there was pulmonary stenosis.RESULTS:In this series, 96 infants survived after the surgery and 88 were followed up with a mean peirod of (22±4) months. No pulmonary stenosis was detected with the simplified Bernoulli formula.CONCLUSION:No pulmonary stenosis was detected with the simplified Bernoulli formula.

患儿男,9岁8个月,体质量29 kg.X线胸片发现左下肺肿块影3年,未予进一步诊治.因咳嗽再次入当地医院,X线胸片提示肺内肿块影仍存在.查体:心率90次/min,呼吸20次/min.发育良好,口唇及甲床轻度发绀.双肺呼吸音粗,左下肺呼吸音略低,未闻及罗音.律齐,心前区未闻及杂音.安静、大气吸入血氧饱和度(SPO2) 0.84.X线胸片提示双肺纹理增多,左下肺肿块影.心电图、超声心动检查未见明显异常.胸部CT平扫可见类圆形、结节状软组织块影,密度较均匀,边界清,测其CT值为50Hu.增强CT肺窗可见肺叶内类圆形软组织密度影,与心脏分界尚清楚,增强后可见肺内肿块明显均匀强化,CT值为327 Hu.经血管影像重建,见左肺动脉发出分支通向该肿块,并最终回流至左肺静脉(图1).MRI检查见类圆形肿块内流空,一粗大的起源于左肺动脉的供血血管及粗大回流至左肺静脉的引流静脉(图2)。

心包积液是小儿先天性心脏病术后常见的并发症,低心排、心功能不全、心包切开综合征、切口或吻合处渗出均可引起心包积液[14],大量心包积液可造成心包压塞,甚至心搏骤停等严重后果[2,4].笔者总结2007年至2012年小儿先心术后心包积液及心包压塞的护理经验,现报道如下.

Neonatal congenital diaphragmatic hernia (CDH) is a complex anomaly, the clinical course of which depends on the timing and duration of herniation and the amount of viscera herniated. Acute gastrointestinal complications of neonatal CDH are mainly caused by the incarceration of the stomach and intestines[1], which are significantly reduced by prenatal diagnosis and appropriate neonatal treatment. Neonatal gastric perforation associated with CDH is an unusual surgical emergency, the symptoms of which may be quite nonspecific[1], and the radiographic findings may show pleural effusion and massive hydroperitoneum[2, 3]. Rapid diagnosis and appropriate treatment are essential. We present a 4-day-old neonate with gastric perforation and CDH, who was treated successfully. A 2.85 kg male infant was born at term by Cesarean section. The US at 25 weeks was normal. Milk feeding was accepted 2 hours after birth. He presented with recurrent non-bilious vomiting after meals during the first 3 days. Stools were passed during this period. On day 4 of life he had tachypnea and abdominal distension and greenish vomiting and his condition deteriorated rapidly. Then he was admitted to our hospital. On physical examination there were marked abdominal distension and decreased bowel sounds. The auscultation revealed decreased air entry on the lower left side of the chest. A nasogastric tube was passed and it drained bilious aspirate. A supine thoracoabdominal radiography showed an opacified left lower lung field resembling pleural effusion and the presence of gas in the left lower chest with shifting of the mediastinum to the right and a diffuse ground-glass opacity with paucity of bowel gas (Fig. 1). An erect thoracoabdominal radiography showed changes similar to the supine radiography except for the presence of gas fluid level in the left hemithorax (Fig. 1). Subsequent US showed left hydrothorax and an abundant volume of peritoneal fluid. Oral feeding stopped and he was intubated soon after admission. Prompt laparotomy was performed, which revealed the posterolateral diaphragmatic hernia, with a hernia orifice measuring 4×3 cm and consisting a large part of the stomach. A perforation, 4 cm in diameter, was located at the back wall of greater curve of the stomach. An abundant amount of yellow fluid was identified in the peritoneal cavity. The defect of the diaphragm was closed primarily and the perforation was repaired by two-layer closure. He was extubated on the seventh postoperative day and discharged from hospital 35 days after admission. He recovered well after seven months of follow up. Fig. 1 Left: Supine radiography showing an opacified left lower lung field with a cyst and a diffuse ground-glass opacity with paucity of bowel gas; Right: Erect radiography showing gas fluid level in the left hemithorax. There have been only a few case reports of neonatal CDH presenting with gastric perforation[1–3]. Two cases were discovered before birth[2, 3]. Our case highlights the clinical course and radiography of neonatal gastric perforation associated with CDH, which are important in the diagnosis. There was one similar case observed after birth, symptoms of which were gastrointestinal rather than respiratory in origin[1]. In our case, the symptom of CDH before gastric perforation was recurrent non-bilious womiting rather than tachypnea, which is quite nonspecific, and the clinical course such as tachypnea, abdominal distension, greenish vomiting and rapid deterioration of the neonate came with gastric perforation. The radiography of neonatal CDH often shows an opacified hemithorax with mass effect and contralateral shift of the mediastinum. Bowel gas is lacking in the abdomen and can be identified in the chest[4]. In our case, the radiography showed an intrathoracic cyst rather than loops of bowel in the chest because the hernia consisted only a large part of the stomach. Pleural effusions and massive ascites indicated gastrointestinal complication associated with CDH, which is similar to some other cases[2, 3]. Our case suggests that congenital diaphragmatic hernia should also be suspected in the neonate presenting with recurrent womiting. When massive ascites and pleural effusions are noted in association with CDH, gastrointestinal perforation should be considered. Early diagnosis and fluid resuscitation and prompt surgical treatment are imperative.

Objective To summarize the clinical manifestations of delayed diaphragmatic hernia in children and to analyze the influencing factors of operation time limit.Methods A retrospective analysis was conducted on the age , symptoms, signs, auxiliary examinations, comorbidities , intraoperative findings of lung development and operation time limit of 47 children with delayed diaphragmatic hernia treated in the period of January 2006 to June 2013 in Xi’an Children’s Hospital.Results There were 25 cases with respiratory symptoms as the main manifestation , accounting for 53%, and 14 patients with digestive symptoms as the main manifestation ( 30%) .Eight cases were diagnosed by physical examination but they were asymptomatic when not taking examination .There were 22 cases diagnosed with chest X-ray and 18 cases with chest CT .Clinical symptoms , combined diseases and misdiagnosis before operation were related factors of whether emergency operation was needed (The Fisher exact statistical conclusion:P value was 0.010,0.010 and 0.011, respectively, all P<0.05).Conclusion The clinical manifestations of delayed diaphragmatic hernia are various .Related examinations should be taken positively and the patients ’ condition should be analyzed from all aspects for early diagnosis .

患儿,女,9岁11个月,因"咳嗽伴气促并进行性加重半个月"入院.入院检查:精神软,口唇发绀,呼吸急促,吸气相呈轻度三凹症,右侧胸廓饱满,呼吸活动度减弱,听诊呼吸音减轻,叩诊呈实音,心音中,律齐,未闻及明显杂音,腹软,无压痛,肝脾肋下未及,神经系统(一).辅助检查:血常规及超敏反应蛋白正常.胸片:右侧肺野大片状密度增高影.CT报告提示:右侧胸腔巨大混杂密度软组织肿块,大小约12.6 cm×12.6 cm×22.2 cm,以大量多房囊性低密度灶为主,其内可见点状、条索状高密度灶,及斑片状脂肪影.心脏受压明显左偏,右侧膈面显示不清,增强扫描肿块分割可见强化,囊性部分强化不明显,肝脏受压.心电图:窦性心律,肢体导联低电压,T波改变.入院诊断:右纵膈巨大畸胎瘤,纵膈疝.

肺缺如( pulmonary agenesis,PA)是临床非常罕见的畸形,是指气管隆起以下无气道、血管组织及肺实质存在.今就收治的4例单侧肺缺如患儿,结合国内外文献报道,进行综合分析、讨论,以提高对本病的认识.

永存左上腔静脉(persistent left superior vena cava,PLSVC)是先心病患儿的常见血管畸形[1,3],但是永存左上腔合并右上腔静脉(right superior vena cava,RSVC)缺如非常罕见,约占先天性心血管畸形的0.07％～0.34％[2,4].右侧上腔静脉缺如一般没有临床症状,常在检查或手术巾被发现[2,5,7]. 我们介绍1例患有先天性房间隔缺损,合并右侧上腔静脉缺如、永存左上腔静脉、右上肺静脉异位引流的手术治疗.