腹裂患儿Silo袋内肠管血供监测的方法探讨

目的介绍一种针对无法一期回纳的严重新生儿腹裂患儿放置silo袋后监测袋内肠管血供的方法及其临床意义。方法对先天性腹裂患儿施行手术时，在Slio袋放置完毕后，将外周血氧饱和度监测仪的条带状探头紧贴硅胶袋壁包绕，监测硅胶袋内肠管血氧饱和度。结果5例无法一期回纳的新生儿腹裂患儿放置免缝Silo袋后成功应用外周血氧饱和度监测仪监测袋内肠管血供情况。结论外周血氧饱和度监测仪可安全应用于对Silo袋内肠管血供的监测，改变了以往仅仅依靠裸眼观察判断袋内肠管血供的欠准确科学的现状，避免了因袋内肠管嵌顿、缺血导致肠管缺血坏死的可能。     

应用免缝SILO袋分期治疗新生儿腹裂临床研究

目的　介绍应用免缝SILO袋分期治疗新生儿腹裂。方法　收集芝加哥大学儿童医院 1998年 1月至2 0 0 2年 7月应用免缝SILO袋分期治疗 15例腹裂患儿。患儿体重 2 1～ 3 5kg ,根据腹壁缺损大小选择环口直径 3～ 5cm的SILO袋。脏器回纳腹腔后择期关闭腹壁。结果　 15例患儿全部顺利分期关闭腹壁缺损。 2例 (13 3% )SILO袋在使用中滑出腹壁 ,立即于床边重新放置SILO袋。SILO袋平均使用天数为 3 7d ,完全经口进食平均为2 2d。 1例患儿伴有小肠闭锁做肠吻合后再安置SILO袋。随访 3个月至 4年 ,15例全部存活。结论　应用免缝SILO袋分期关闭腹壁缺损是一种安全、可靠、有效的方法。     

先天性腹裂外科治疗的麻醉体会

目的总结新生儿先天性腹裂修补术的麻醉体会。方法回顾性分析23例新生儿先天性腹裂修补术的麻醉过程。结果3例采用全麻后Ⅰ期关腹直接复位缝合术，14例先采用非麻醉下免缝Silo袋Ⅰ期免缝手术，5～7d后在气管插管全麻下行Ⅱ期复位缝合术，只放置Silo袋、后期死亡或放弃治疗4例；未经任何治疗自动出院2例。手术患儿中12例麻醉过程较平稳，5例术中血压轻度下降，经加快输血输液等处理后血压回升，无麻醉死亡，均顺利完成手术。术后死亡3例，1例死于多脏器功能衰竭，2例术后肺膨胀不良死于呼吸衰竭。随访时间4个月至4年，患儿均生长发育正常。结论手术是治疗先天性腹裂的主要方法，新生儿先天性腹裂复位修补术的围手术处理关键是麻醉保障，而麻醉的重点是气道管理和保证循环功能稳定。     

一次性无菌腹裂治疗袋应用规范

新生儿腹裂是一种危及生命的严重先天性腹壁畸形,约50%的腹裂患儿需分期修补腹壁缺损。随着国际通用Silo袋的广泛应用,在欧美发达国家腹裂患儿的病死率已降至10%以下。在我国,严重腹裂患儿因缺乏规范有效的治疗模式,部分患儿没有得到及时治疗,导致肠管水肿不断加重;由于缺乏正规的腹裂治疗袋产品,通常采用各种不规范的临时材料应急,导致术后并发症多,早期病死率较高。吴晔明等通过改良国外的Silo技术,开发出具有自主知识产权的腹裂治疗袋,在床边非麻醉下免缝分期治疗新生儿严重腹裂,提出在腹裂治疗袋壁贴敷氧饱和度探头,同时行膀胱测压,实时监测腹裂袋中肠管血供,可及时发现和有效避免腹裂袋中肠管缺血坏死,提高该技术的安全性和可靠性,降低严重腹裂患儿的病死率。该技术在上海交通大学医学院附属新华医院和上海儿童医学中心收治的严重腹裂患儿中已成功应用且趋于成熟,在全国20多家三甲医院得到推广,产生了较好的社会效益。     

先天性腹裂的临床回顾性研究

目的 归纳总结先天性腹裂的治疗效果及体会,展望今后的临床研究方向.方法 回顾上海新华医院、上海儿童医学中心1996年12月至2007年7月收治的23例腹裂患儿,分析其治疗效果,并分别比较低出生体重儿和正常出生体重儿、顺产儿和剖腹产儿的治疗效果.结果 Ⅰ期关腹治疗3例,治愈2例;免缝Silo袋分期修复14例,治愈12例;只放置Silo袋、但后来死亡或放弃治疗4例;未经任何治疗自动出院2例.该病的总治愈率为60.9%(14/23),病死率为13.0%(3/23),放弃治疗者占26.1%(6/23).低出生体重儿和正常出生体重儿、顺产儿和剖腹产儿的治疗效果差异无统计学意义(P＞0.05).结论 腹裂的治愈率仍有待提高.对难以Ⅰ期关腹的腹裂患儿,床边非麻醉下放置免缝Silo袋、Ⅱ期手术修复是一种安全可靠、操作简便的治疗方式.出生体重和分娩方式未对治疗效果造成影响.     

生理盐水袋治愈新生儿腹裂一例

患儿 :男 ,出生后 1ｄ。患儿部分胃及小肠暴露于腹腔外 ,外露肠管粗大与肥厚 ,表面充血、肿胀 ,表面纤维性渗出。术中所见 :脐部稍偏右侧有一直径为6ｃｍ裂口 ,裂口处腹壁全层缺损 ,暴露的肠管表面无任何包膜 ,裂口与脐之间有正常皮肤 ,腹腔明显狭小。诊断为先天性腹裂 ,拟行分期腹裂修补术。选用医用生理盐水袋 ,术前消毒后备用。操作方法 :选用囊袋法修补腹裂。将外露于腹腔外肠管装入生理盐水袋内 ,再将生理盐水袋与裂口周围腹壁全层缝合 ,封闭腹腔。术后悬吊囊袋避免压迫腹腔。从术后第 3ｄ开始 ,逐渐适当紧缩囊袋加压 ,使腹腔逐渐扩大 …     

Ⅰ期无缝合肠管还纳术治疗先天性腹裂

目的 总结Ⅰ期无缝合肠管还纳术治疗先天性腹裂的经验和体会.方法 回顾性分析2007年12月至2011年8月收治的24例先天性腹裂患儿资料,15例采用气管内麻醉下Ⅰ期无缝合肠管还纳术,9例采用人工囊袋分期修复术.将两组患儿的术后进食时间及住院时间进行对比.结果 Ⅰ期无缝合组治愈13例,1例因双肾发育不良放弃,1例因硬肿症死亡;分期组9例全部治愈出院,分期组患儿的术后进食时间及住院时间明显长于Ⅰ期无缝合组.结论 采用气管内麻醉下实施Ⅰ期无缝合肠管还纳修复术,有条件地扩大了Ⅰ期无缝合肠管还纳修复方法治疗的适应证,使更多的先天性腹裂患儿免受二次手术打击,而且口服喂养时间明显提前,缩短了住院时间,也就减少了住院费用,是一种安全可靠且美容的方法.     

先天性腹裂的诊疗体会

目的总结先天性腹裂患儿的治疗经验。方法回顾性分析21例新生儿先天性腹裂患儿的病例资料。19例采用传统全麻插管下I期复位缝合术,2例采用非全麻气管插管下I期免缝手术。结果19例全麻气管插管I期复位缝合术的患儿中,治愈18例,死亡1例,死亡原因为坏死性小肠结肠炎、多器官功能衰竭。2例非全麻气管插管下I期免缝手术患儿均痊愈出院。所有存活患儿均获随访,随访时间3个月至7年,患儿均生长发育正常。结论手术是治疗先天性腹裂的主要方法,产前诊断为产房手术提供了可能,妥善的围手术期处理是手术成功的保障。     

用硅胶袋修复巨型脐膨出与腹裂13例报告

目的总结应用硅胶袋分期修复巨型脐膨出和腹裂的治疗经验。方法回顾性分析2003年以来应用硅胶袋进行分期腹壁修补术的11例患儿病例资料。其中巨型脐膨出3例,腹裂8例。均于气管插管全身麻醉下手术,将无菌硅胶袋与腹壁缺损边缘缝合成囊袋状,包裹膨出的脏器,术后逐渐挤压囊袋至内脏还纳入腹腔后去除囊袋,分层关闭腹壁缺损。脐膨出患儿行脐部成形。全部患儿术后均予呼吸机支持2-24h。结果全组患儿膨出脏器均于术后7-10d还纳入腹腔．此时腹壁无明显张力．术后无腹壁切口裂开及腹腔继发感染,伤口恢复良好,生长发育正常。结论采用无菌硅胶袋替代涤纶补片行腹壁修补术,硅胶袋与腹壁缝合后反应小,费用低,是一种安全、疗效可靠的方法。     

分期修复术治疗腹裂5例

探讨用硅塑囊分期修复术治疗腹裂的临床疗效。方法分析应用硅塑囊分期修复术治疗新生儿腹裂５例的临床塑料。结果５例新生儿腹裂全部存活，术后随诊满意。结论应用硅塑囊分期修复术治疗新生儿腹裂是一种简单，安全有效的方法，有临床应用价值。     

Prenatally diagnosed gastroschisis: a case for preterm delivery?

The successful surgical closure of gastroschisis is invariably followed by a prolonged ileus. Early delivery of infants with prenatally diagnosed gastroschisis has been advocated as a method of limiting the intestinal damage that occurs in utero. This study examines 47 infants with gastroschisis. The mean gestational age of the infants was 37.5 weeks, although there was no policy of induction of premature labour. The mortality for the series was 4% (2 deaths). The mean duration of post-operative ileus was 14.1 days and the mean duration of hospital stay was 38.4 days. There was no correlation between gestational age and duration of ileus or hospital stay. Analysing infants born at 36 weeks' gestation as a separate group also failed to show statistically significant differences for duration of ileus or hospital stay. As gestational age does not correlate with time taken for intestinal function to recover, elective preterm delivery of the infant with prenatally diagnosed gastroschisis is not recommended. Delivery by caesarean section conferred no benefit in this study.     

Gastro-oesophageal reflux in infants under 6 months with cystic fibrosis

AIM: To establish the incidence of pathological gastro-oesophageal reflux (GOR) in newly diagnosed infants with cystic fibrosis and to identify clinical predictors of increased reflux. METHODS: 26 infants with cystic fibrosis less than 6 months of age (14 male, 12 female; mean (SEM) age 2.1 (0.21) months, range 0.8 to 5.6 months) underwent prolonged oesophageal pH monitoring (mean duration 27.1 (0.49) hours; range 21.3 to 30.2 hours). Reflux symptoms, anthropometric variables, pancreatic status, meconium ileus, genotype, and chest x ray findings were correlated with pH monitoring data. RESULTS: Five infants (19.2%) had an abnormal fractional reflux time of greater than 10%, seven (26.9%) of 5-10%, and 14 (53.8%) of below 5%. Infants who presented with frequent vomiting had a significantly higher fractional reflux time than infants who had infrequent or no vomiting. There was no significant association between abnormal chest x rays and pathological GOR. Sex, genotype, nutritional status, meconium ileus, and pancreatic enzyme supplementation were not significantly associated with pathological GOR. CONCLUSIONS: About one in five newly diagnosed infants with cystic fibrosis had pathological GOR. Pathologically increased reflux was present before radiological lung disease was established. Apart from frequent vomiting, no useful clinical predictors of pathological reflux were found.     

Abdominal wall defects in the era of prenatal diagnosis

In order to study the effects of prenatal diagnosis, we reviewed all 141 cases of abdominal wall defects (AWD) seen in our institution since 1980. In the period up to the end of 1994, 65 AWDs were diagnosed at the Department of Obstetric Ultrasound and another 76 infants were born with an AWD, 44 with omphalocele (prenatal diagnosis 29), 21 with a cord hernia (prenatal diagnosis 4), and 76 with gastroschisis (prenatal diagnosis 32). In the prenatal diagnosis group the frequency of cesarean section was 48%, in the postnatal diagnosis group 13%. In the omphalocele group, the pregnancy was terminated in 12 cases and there were 10 intrauterine deaths. A further 10 babies died in the first days of life, so that only 12 came to operation; 6 survived. At least 27 of the 44 infants had other severe anomalies. Omphalocele has a poor prognosis that is not improved by prenatal diagnosis, so that termination is a reasonable option. In the cord hernia group 1 patient died because of a cardiac anomaly; all the others had primary closure without complications. In the gastroschisis group, the pregnancy was terminated in 7 cases and there were 2 intrauterine and 2 neonatal deaths. Sixty-five infants were operated upon immediately after birth, 50 (77%) with primary closure and 15 in two stages. Four died after primary closure (8%) and 4 after a silastic silo (27%). The overall mortality was 12.3%. Postoperative intestinal morbidity was assessed by the period until total enteral nutrition could be established, which was from 8 to 185 days (median 17 days). The unfavorable prognostic factors were intestinal thickening and staged closure. Neither prenatal diagnosis nor mode of delivery could be shown to have any effect on mortality or morbidity.     

床旁激光治疗早产儿视网膜病变的疗效分析

目的：探讨床旁激光治疗早产儿视网膜病变（ROP）的临床效果。方法：回顾性分析2009年3月至2009年8月在我院新生儿重症监护室（NICU）内床旁激光治疗的30例ROP患儿的临床资料。结果：30例ROP患儿中59只眼接受了激光治疗,总体治愈率95%。根据ROP国际分类标准,30例ROP患儿中,Ⅰ区病变有13例（26眼）,Ⅱ区病变有17例（33眼）。Ⅰ区病变组平均出生胎龄、体重及治疗时纠正胎龄均明显低于Ⅱ区病变组,而激光斑点数量明显高于Ⅱ区病变组。Ⅱ区病变组治愈率（100%）明显高于Ⅰ 区病变组（88%）（P<0.05）。结论：NICU床边激光手术治疗Ⅰ区病变和Ⅱ区病变是有效的。与Ⅱ区病变相比,Ⅰ区病变患儿预后相对较差。［中国当代儿科杂志,2010,12（9）：696-699］     

Gastroschisis: determinants of neonatal outcome

This retrospective study elicits information regarding the dependence of neonatal outcome in gastroschisis upon: (1) the mode of delivery, (2) place of birth, (3) time for birth to surgery, (4) method of closure, (5) time from operation to commencement of first enteral feeds. The neonatal intensive care database from five major tertiary centres was used to identify 181 neonates with gastroschisis from 1990 to 2000. There were 8 deaths. There were no significant differences in outcome for infants delivered vaginally (102) versus Caesarean section (79), those born near the tertiary centre (133) as compared to infants born away (48), ones operated within 7 hours (125) compared with those operated after 7 hours (56), with delayed closure (30) versus primary closure (151). Neonates fed within 10 days of operation (85) had significantly lower incidence of sepsis, duration of TPN and hospital stay when compared to those fed after 10 days (96). Early commencement of feeds decreases the incidence of sepsis, duration of total parenteral nutrition (TPN) and hospital stay. Place of delivery, mode of delivery, time to surgery and type of closure do not influence neonatal outcome.     

Staged reduction of gastroschisis: a simple method

 Staged reduction of abdominal contents using a silastic sheet has become standard management in gastroschisis where primary closure is not possible. With the introduction of a pre-made Silastic silo coupled to a spring-loaded ring (Ben Tec, Sacramento, CA), the procedure can be done at the bedside. We present a simple technique utilizing a disposable umbilical-cord clamp that makes reduction a fast, one-physician procedure and present a preoperative step that facilitates tension-free closure of the abdominal fascia. Accepted: 19 November 1999     

Pancreatic function testing in meconium disease in CF: two case reports

We report two infants with cystic fibrosis (CF), presenting with meconium ileus and meconium plug, who had no clinical or biochemical evidence of pancreatic insufficiency during infancy. They underwent pancreatic secretory function testing at 11 and 9 months of age, respectively. Both patients had sufficient lipase and colipase secretion to maintain normal digestion of fat, confirming that meconium disease in CF does not necessarily imply pancreatic insufficiency and the need for enzyme supplementation in infancy. Nonetheless, we documented markedly reduced enzyme secretion in both patients, implying a potential role for the pancreas in the pathogenesis of meconium disease, even when clinical pancreatic insufficiency is absent. In addition, our patient with meconium ileus had a severely limited fluid secretory capacity (10.3% of mean normal values). In contrast, the patient with the milder presentation of meconium plug had a far greater ability to secrete fluid (75% of mean normal), but had poorer pancreatic proteolytic activity. We suggest that impaired fluid secretion may be a very significant factor in the pathogenesis of meconium ileus, and we speculate that an inability to maintain sufficient intraluminal fluid relative to the degree of pancreatic proteolytic deficiency may more adequately explain the risk of occurrence and the severity of intestinal obstruction in CF than either factor alone.     

Non-operative management of gastroschisis: a case-matched study

Non-operative management of gastroschisis also known as plastic closure (PC) has been described as an alternative to conventional primary operative closure (POC) or staged silo closure (SSC). The aim of this study was to compare these techniques in neonates with gastroschisis. A retrospective review of neonates with gastroschisis who underwent PC was undertaken. The minimum follow-up was 1 year. Premature neonates (<35 weeks) and those with intestinal atresia or multiple congenital anomalies were excluded. Frequency matching of PC cases with two control groups with either POC or SSC was performed on 1:1 ratio, based on gestational age and birth weight. Statistical analysis using univariate analysis was performed. Three groups were assembled: PC, POC and SSC (n = 33). Median follow-up was 1,198 days. Groups were comparable with regard to: time to first feed, time to full enteral feeding, frequency of vascular access related infections, ventilation time, NICU LOS (length of stay) and hospital LOS. There was no difference observed in surgical complications. Almost all neonates in the PC group developed an umbilical hernia (83.8%). Umbilical hernias were highly associated with PC compared to the other two groups P = 0.001. To date only one patient has had to have an operative repair of the umbilical hernia in the PC group. Plastic closure is safe and comparable to conventional closure techniques. However, PC is associated with the development of more umbilical hernias. The natural history of these hernias remains to be defined.     

Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus

We set out to determine if the clinical course or genetic profiles of patients with cystic fibrosis who had meconium ileus differed from those of other patients with cystic fibrosis. Since 1950 we have followed 158 patients with meconium ileus among 1175 patients with cystic fibrosis (13.4%). Patients with meconium ileus had lower birth weight (3026 +/- 610 gm) than patients with no meconium ileus (3169 +/- 534 gm; p less than 0.008); the deficit was especially evident in female patients. Survival in the first year of life increased from 55% in those born between 1958 and 1972 to 96% in those born between 1973 and 1987. Since 1973 the median survival of male and female patients with meconium ileus was similar to that in female patients with no meconium ileus (21 years), whereas 78% of males with no meconium ileus survived to this age (p less than 0.0001). Patients with meconium ileus born before 1972 had lower weight and height percentiles at age 13 years compared with patients with no meconium ileus, but this difference was not as apparent in patients born after 1973. There were no differences between the two groups in forced vital capacity, forced expiratory volume in 1 second, or forced expiratory flow in the middle half of forced vital capacity. Patients with meconium ileus acquired Pseudomonas aeruginosa at a younger age than did patients with no meconium ileus (4.20 +/- 4.67 vs 7.18 +/- 5.19 years), but there was no difference in age of acquisition of P. cepacia. In families in which the first child had meconium ileus, 29% of subsequent siblings with cystic fibrosis had meconium ileus, compared with 6% of siblings born to families in which the first child did not have meconium ileus. Allelic frequencies and haplotypic variants for cystic fibrosis chromosomes with respect to DNA markers closely linked to the cystic fibrosis locus were similar in families with cystic fibrosis with meconium ileus and those with no meconium ileus. These findings suggest that patients with cystic fibrosis and those without meconium ileus do not have major intrinsic differences and that the previously poor outlook in patients with meconium ileus has improved greatly.