先天性胆总管囊肿几种胆肠吻合术的指征与评价

先天性胆总管囊肿是小儿较常见的胆道系统发育异常。随其病变的发展 ,梗阻可致胆汁淤滞 ,使肝脏遭受渐进性损害。亦可引起胆道系统感染、胆源性胰腺炎、囊内结石形成以及囊肿癌变等。故临床上一经确诊 ,即应行胆肠吻合胆道重建手术。1.小儿胆肠吻合手术一般原则　先天性胆总管囊肿胆肠吻合重建胆道的良机为在尚未形成肝脏不可逆损害之前 ,通过手术解除胆路梗阻 ,使淤胆肝脏好转 ,并减少并发症的发生。其手术应遵循以下原则。手术关键是恢复胆汁向肠道排泄的通畅性 ,并努力做到或减少消化液反流致上行性胆道感染。新建的胆肠通路 ,应防止吻合…     

信息动态

目的 探讨超声诊断小儿先天性肠旋转不良合并中肠扭转的价值.方法 回顾性分析130例上消化道梗阻手术患儿的临床资料和术前超声检查结果,并与消化道造影检查结果进行对比分析.结果 130例手术患儿中,85例证实为先天性肠旋转不良,其中并中肠扭转74例(87.06％,74/85例),超声诊断73例,漏诊1例,敏感度为98.65％(73/74例);未合并中肠扭转11例,超声均未检出.45例无先天性肠旋转不良,术前超声42例符合,3例误诊.超声诊断总敏感度为85.88％(73/85例),特异度为93.33％(42/45例),准确率为88.46％(115/ 130例),假阳性率为6.67％(3/45例),假阴性率为14.12％(12/85例).超声检查与手术结果一致性较好(Kappa =0.76).85例先天性肠旋转不良患儿中,55例术前同时接受超声及消化道造影检查.46例经手术证实合并中肠扭转,超声诊断符合率97.83％(45/46例).消化道造影具有直接征象的为63.04％(29/46例).超声与消化道造影明确诊断先天性肠旋转不良合并中肠扭转的符合率比较差异有统计学意义(P＜0.01).结论 超声及消化道造影均可诊断小儿先天性肠旋转不良,而超声对并中肠扭转的肠旋转不良诊断价值更高.     

先天性肠闭锁肠电图和肠间质细胞的同步研究

目的 通过肠闭锁患儿肠电图和肠间质细胞的临床研究，探讨肠闭锁患儿手术前后肠动力的改变及与肠间质细胞发育情况的关系。方法 分别对正常新生儿和肠闭锁患儿手术前后进行肠电图检测；对先天性肠闭锁患儿标本，分别选取闭锁盲端远近端肠壁全层组织，采用Envision法免疫组织化学技术，观察肠间质细胞的发育情况。结果 肠闭锁患儿闭锁近端术前肠电图电压高于正常对照组，术后肠电压及术前术后的肠电图频率与对照组接近；在肠闭锁肠壁中，发现肠间质细胞在远近端的分布与正常对照组相比显著减少，肠间质细胞网络被破坏。近端改变范围在8cm左右，远端改变局限在1～2cm内。结论 肠电图(EEG)对肠闭锁的诊断和术后肠功能恢复的判断有一定的临床意义，肠电图表现可反映肠间质细胞的发育情况。     

小儿先天性肥厚性幽门狭窄合并肠旋转不良的临床特点

目的 探讨小儿先天性肥厚性幽门狭窄合并肠旋转不良的临床特点.方法 回顾性分析2003年1月至2009年12月于我科治疗的6例先天性肥厚性幽门狭窄合并肠旋转不良患儿的临床资料.结果 通过胃肠透视及超声检查,4例患儿术中确诊该病并经1次手术解决,2例术前仅诊断为先天性肥厚性幽门狭窄,行幽门肌切开术,再次出现呕吐后行2次手术发现肠旋转不良.所有病例预后良好.结论 先天性肥厚性幽门狭窄合并肠旋转不良较为少见,病因不清,先天性肥厚性幽门]狭窄的症状易掩盖肠旋转不良的临床表现而贻误其诊断.胃肠透视对于诊断该病有重要意义,彩超检查有良好的应用价值,及时的诊断治疗可获得良好的治疗效果.     

先天性肠闭锁352例诊治体会

目的探讨先天性肠闭锁、肠狭窄的临床表现、诊断方法、手术方式以及预后。方法回顾性分析2000年1月至2010年6月作者收治352例先天性肠闭锁、肠狭窄患儿临床资料,闭锁部位：十二指肠84例（24％）,空回肠236例（67％）,结肠32例（9％）。结果352例中,治愈320例,治愈率91％,死亡12例,20例放弃治疗。并发症：吻合口漏14例（4％）,粘连性肠梗阻74例（21％）,切口感染18例（5％）,肺炎32例（9％）。结论先天性肠闭锁、肠狭窄诊断不难,十二指肠闭锁可在产前做出诊断．应根据临床特点选择手术方式。     

肠内营养对梗阻性黄疸幼鼠肠黏膜结构的影响

小儿梗阻性黄疸（OJ）见于先天性胆道闭锁、胆管扩张症等疾病，常伴有局部和全身免疫功能抑制、营养不良和肠屏障功能障碍，进而引起肠道细菌和内毒素移位，甚至肠源性感染。在大鼠胆总管结扎制作的梗阻性黄疸模型中，肠道呈炎症、水肿改变，肠上皮细胞之间间隙形成，肠黏膜上细菌附着数量增加。完全肠外营养时小肠黏膜上皮细胞间的紧密连接降低，肠道内分泌性免疫球蛋白A含量下降，细菌数也增加。     

关于先天性巨结肠类缘病名称的商榷

18 8 6年Ｈｉｒｓｃｈｓｐｒｕｎｇ首次提出先天性巨结肠 (Ｈｉｒｓｃｈｓｐｒｕｎｇ’ｓｄｉｓｅａｓｅ ,ＨＤ)的病因在于直肠先天性狭窄 ,奠定了小儿便秘病因认识史上一个重要的里程碑 ,ＨＤ一直被认为是小儿便秘的首要原因。 1971年瑞士病理学家Ｍｅｉｅｒ Ｒｕｇｅ首次描述结肠神经元性发育异常 (ｎｅｕ ｒｏｎａｌｃｏｌｏｎｉｃｄｙｓｐｌａｓｉａ ,ＮＣＤ )的病理现象 ,从而揭开小儿便秘病因研究的新里程。近 30年来越来越多的研究显示肠神经元异常是一类确有临床意义但又表现复杂的疾病。尽管目前关于肠神经元分布异常的认识仍不足 …     

先天性肠旋转不良的诊治体会

目的总结小儿先天性肠旋转不良及合并畸形的临床表现特点,探讨合适的治疗方法。方法本组病人全部采用Ladd's手术方法,将压迫于十二指肠前方的腹膜带彻底松解,对合并十二指肠狭窄或闭锁者外观不容易看出,则采用从胃管向十二指肠内注入盐水的方法以确定诊断。结果41例中治愈38例,死亡3例,治愈率为92.7%。结论早期诊断、早期治疗、手术中勿遗漏合并畸形是提高治愈率,减少死亡率的关键。     

小儿急性肠套叠肠切肠吻合术42例

目的总结行肠切除肠吻合术的小儿急性肠套叠的诊断和治疗经验。方法对42例行肠切肠吻合术的肠套叠患儿的临床资料进行回顾性分析。结果肠套叠78．57％为复套叠，多为回回结型。大多数术后愈合良好。结论小儿急性肠套叠关键是早期诊断、及时治疗。术中发现肠坏死应尽量减少切除范围，保留回盲部。     

肠重复畸形的诊断和治疗

目的探讨小儿肠重复畸形的诊断和治疗。方法回顾性分析12例小儿肠重复畸形病例的临床资料、辅助检查和手术治疗情况。结果全部病例均治愈。结论肠重复畸形临床表现多种多样,缺乏特异性,腹部B超、同位素扫描、X线、CT检查对诊断有帮助。手术是确诊及治疗的唯一方法。     

先天性肠系膜裂孔疝的诊断和治疗

目的探讨先天性肠系膜裂孔疝的早期诊断和治疗方法。方法回顾性分析本科2005年11月-2007年3月经手术证实的4例患者临床资料，并结合文献资料，总结对先天性肠系膜裂孔疝的诊治体会。结果4例均为术中确诊；既往有腹部手术史1例，术前考虑为黏连性肠梗阻；2例表现为急性肠梗阻行急诊手术，2例反复间歇性腹痛行择期手术。其中1例术前CT检查提示肠系膜裂孔疝可能；术中1例行I期肠切除吻合术，3例行肠管复位加修复缺损术；1例早期发生感染性休克；4例均痊愈出院。结论本病术前诊断困难，腹部CT、肠系膜血管三维重建有助于提高术前诊断率。早期诊断和及时手术是先天性肠系膜裂孔疝治疗的关键。对于反复腹痛而辅助检查不能确诊者，腹腔镜探查即可诊断，又能有效控制疾病发展。     

Neonatal bowel strangulation: Rare presentation of congenital diaphragmatic hernia

We report a case of congenital diaphragmatic hernia (CDH) with perinatal bowel strangulation requiring intestinal resection. Ten hours after birth, the newborn started to be lethargic and developed bilious emesis. X‐ray documented distended loops of bowel with air fluid levels in the abdomen and a gasless, non‐homogeneous opacity of the left hemithorax, a right mediastinal shift and loss of the sharp left hemidiaphram line. On gastrographin enema the left colon was above the adjacent left diaphragm. Emergency surgery was performed at 16 h of age. The entire small bowel appeared reddish and compromised. After 24 h, second‐look laparotomy was performed and only 25 cm of small bowel were viable. The postoperative period was uneventful. Neonatal bowel strangulation in CDH should be taken into account when estimating postnatal morbidity and mortality and, even if CDH treatment is not an emergency procedure, if gastrointestinal symptoms prevail over respiratory symptoms, surgery should be carried out without delay.     

Congenital intestinal fibrosarcoma with rapid recurrence requiring adjuvant chemotherapy

A total of 16 cases of congenital fibrosarcoma have been reported from 1975 to March 2015. Five of the 16 had abnormal fusion between erythroblast transformation specific translocation variant 6 and neurotrophin recptor gene neurotrophic tyrosine kinase, receptor, type 3 (ETV6–NTRK3); in another five out of 16 this was absent, and six were not tested. All were managed by surgical resection but none involved metastasis. Herein we report the case of a newborn baby girl with congenital fibrosarcoma negative for ETV6–NTRK3 gene fusion, who presented with ileal perforation and positive resection margin. She had rapid recurrence with lymph node metastasis treated with postoperative chemotherapy. There was no further recurrence at >3 years of follow up.     

Hirschsprung's Disease: A Viral Etiology?

This study investigates the hypothesis that Hirschspiung's disease (HD), congenital rectal agangliosis, may be etiologically linked to antenatal cytomegalovirus (CMV) infection. Bowel specimens from 72 HD patients, 144 control I infants (Hirschsprung-like symptoms, normal histology), and 36 control 11 infants (deaths from nongastrointestinal causes) were analyzed for CMV genomes by polymerase chain reaction. Positive results were obtained in 6 HD patients (8.8%) and none of the controls. Our findings suggest that antenatal CMV infection, a potentially preventable condition, may be one of the etiological factors in HD.     

Solitary Intestinal Fibromatosis with Perinatal Bowel Obstruction

Three newborn male infants presented with bowel obstruction in the first day of life and at surgery were found to have solitary tumors involving the small or large intestine. Histologic examination in each case showed a transmural infiltrative spindle cell lesion having the morphologic features of fibromatosis. Ultrastructural studies in one case revealed the tumor to be composed of myofibroblasts. The patients are all alive and well without recurrences 26 months to 10 years after surgery. Only 3 previous cases of solitary congenital fibromatosis of the intestinal tract have been reported. Some of the other congenital spindle cell tumors cited in the literature under various names have morphologic and biologic similarities to our cases and may in fact be examples of congenital fibromatosis. The appropriate treatment of this unusual lesion is local excision, and the prognosis is excellent.     

Congenital alveolar capillary dysplasia and associated gastrointestinal anomalies

Congenital alveolar capillary dysplasia is a rare cause of irreversible pulmonary hypertension with 100% mortality. We present three cases of congenital alveolar capillary dysplasia with associated gastrointestinal abnormalities. Three full-term neonates presented with pulmonary hypertension needing ventilatory support by oscillation. Of the three, two neonates subsequently needed extracorporeal membrane oxygenation. Abdominal distension associated with bilious aspirates was the gastrointestinal manifestation. One child had duodenal atresia and anorectal anomaly, one with intestinal malrotation and the other with a rare combination of intestinal malrotaion and total colonic Hirschsprung's disease. All three infants succumbed to pulmonary hypertension at mean age 34 days. The etiopathogenesis and pathology of this condition are discussed with a comprehensive review of the literature.     

Monitoring of Congenital Malformations in Tottori, 1974–1983*

ABSTRACT The prevalence and distribution of the congenital malformed infants and the neurocutaneous syndrome patients who were born in Tottori Prefecture, Japan between 1974 and 1983 were reported. The average prevalence of 581 cases with congenital malformations at birth was 1.21 per 100 births (live and stillbirths), which was similar to other populations in Japan. The prevalence values of cleft lip, cleft palate and cleft palate with cleft lip in Tottori was somewhat higher than in Kanagawa and Osaka districts. Frequencies of tuberous sclerosis, Sturge-Weber syndrome and neurofibromatosis in live births were about 1:22,000,1:65,000 and 1:2,200, respectively. These data in Japanese population were considered to be about the same as those reported in Caucasian populations.