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。     

Frequency of anomalies and hospital outcomes in infants with gastroschisis and omphalocele

Background

Gastroschisis and omphalocele are the most common anterior abdominal wall defects affecting infants. There are few large cohort studies describing the frequency of associated anomalies in infants with these 2 conditions. We describe associated anomalies and outcomes in infants with these defects using a large, multi-center clinical database.

Methods

We identified all infants with gastroschisis or omphalocele from a prospectively collected database of infants discharged from 348 neonatal intensive care units in North America from 1997 to 2012. Maternal and patient demographic data, associated anomalies, and outcome data were compared between infants with gastroschisis and omphalocele.

Results

A total of 4687 infants with gastroschisis and 1448 infants with omphalocele were identified. Infants with omphalocele were more likely to be diagnosed with at least 1 other anomaly compared with infants with gastroschisis (35% vs. 8%, p < 0.001). Infants with omphalocele were more likely to develop pulmonary hypertension compared with those with gastroschisis (odds ratio [OR] 7.78; 95% confidence interval 5.81, 10.41) and had higher overall mortality (OR 6.81 [5.33, 8.71]).

Conclusion

Infants with omphalocele were more likely to have other anomalies, be diagnosed with pulmonary hypertension, and have higher mortality than infants with gastroschisis.     

Benefit of preformed silos in the management of gastroschisis

Gastroschisis is traditionally managed by primary closure (PC) or delayed closure after surgical silo placement. Bedside insertion of preformed silos (PFS) and delayed closure has become more widespread, although its benefits remain unclear. To identify differences in outcome of infants managed with PFS compared with traditional closure (TC) techniques. Single-centre retrospective review of 53 consecutive neonates admitted between February 2000 and January 2006. Data expressed as median (range). Non-parametric statistical analysis used with P < 0.05 regarded as significant. Forty infants underwent TC and 13 had PFS and delayed closure. Median ventilation time in both groups was 4 days (P = 0.19) however this was achieved with higher mean airway pressures (MAPs) (day 0, 10 (5–16) versus 8 (5–10) cmH₂O; P = 0.02) and inspired oxygen (40 (21–100) versus 30 (21–60)%; P = 0.03) in TC group. Urine output on day-1 of life was significantly higher in PFS group (1.1 (0.16–3.07) versus 0.45 (0–2.8) ml/kg/h; P = 0.02). Inotrope support was required in 17/40 (43%) of TC versus 0/13 (0%) in PFS (P < 0.01). After exclusion of infants with short bowel syndrome and/or intestinal atresia (n = 9), there was a shorter time to full enteral feeds in the TC group (22 (12–36) versus 27 (17–45); P = 0.07), although there was no difference in the period of parenteral nutrition (PN) (P = 0.1) or overall hospital stay (P = 0.34). No deaths or episodes of necrotizing enterocolitis occurred. The use of PFS for gastroschisis closure is associated with a reduction in pulmonary barotrauma, better tissue perfusion and improved early renal function, consistent with a reduction in abdominal compartment syndrome.     

The pathogenesis of omphalocele and gastroschisis

The embryology of gastroschisis and omphalocele remains a matter of speculation. Most authors still assume that they represent separate entities with a different pathology and embryology. In contrast, others feel that gastrochisis is simply the end-result of a ruptured omphalocele. Reviewing the current literature on the normal and abnormal embryology of the anterior abdominal wall, it becomes obvious that appropriate embryological knowledge of these processes is still missing. Animal models are not at hand that would allow clear definitions of morphological changes unique to either malformation. Nevertheless, our own observations of the pathological anatomy of these anomalies lead us to believe that the abdominal wall defects are the result of disturbed development of the embryonic umbilicus. This includes gastroschisis, which is more likely a ruptured small omphalocele than a developmental entity of its own. In our view, the common ventral abdominal wall defects fall into two main categories: (1) large omphaloceles; and (2) small omphaloceles, with gastroschisis as a subentity of this lesion.     

Use of amniotic grafts in the repair of gastroschisis

This article describes the use of amniotic grafts (AG) in the repair of large abdominal wall defects in newborns with gastroschisis. From 1988 to 1995, 22 newborns with gastroschisis underwent surgical repair. In 12 primary closure (PC) was performed; in 10 the abdominal wall defect was covered with an AG. A double layer of AG was used and the graft was additionally covered with a silastic silo in 8 cases. The overall mortality was 14%; 3 children died from necrotizing enterocolitis or sepsis after AG. However, the difference in mortality of newborns with PC versus AG was not statistically significant. Patient characteristics, the postoperative courses, nd the frequency of complications were similar after PC and AG. It is concluded that AG has no negative impact on the postoperative course and yields a low overall mortality. In our opinion there are several advantages in using the AG technique: it is an autoplastic material that is readily available without costs, reefing and removal is unnecessary, and there is a potentially low rate of adhesions.     

Use of the pulse oximeter in the assessment of mesenteric circulation in a case of gastroschisis

Pulse oximetry of the bowel mesentery proved useful in the assessment of the adequacy of circulation to the congested and oedematous bowel in a case of gastroschisis.     

先天性腹裂治疗方式20年系统评价

目的客观呈现20年来一期手术关腹和Silo技术分期修复治疗的先天性腹裂患儿存活情况。方法检索相关数据库,对两种方法治疗的腹裂患儿的存活率进行Meta分析,比较其差异。结果Meta分析显示,1988—2007年一期关腹组术后存活率高于Silo分期修复组,敏感性分析结果与之一致。亚组分析显示,1988。1997年两组存活率差异无统计学意义,1998。2007年一期关腹组存活率高于Silo分期修复组。结论1988—2007年一期手术关腹患儿术后存活率高于Silo技术分期修复。由于病例分组存在选择偏倚,难以得出一期关腹效果优于Silo技术分期修复的结论。     

Use of pulse oximeter placed on a gastroschisis silo to monitor intestinal oxygen saturation

The use of a silo for temporary coverage of exposed viscera for newborns with gastroschisis has allowed gradual reduction of the externalized intestine into the abdominal cavity. However, there has not been an easy way to monitor blood perfusion to the intestine within the silo other than with visual examination. In addition, visual examination of bowel through the silo is sometimes difficult for medical staff due to serositis and peel over the bowel. We have adopted an approach to monitor oxygen saturation of silo-contained intestine by placing a pulse oximeter sensor on the surface of the transparent silo to detect intestinal ischemia. Pulse oximeter sensors were applied on both a patient’s distal extremity and the silo on five consecutive patients who were born with gastroschisis. The sensor was left on the silo during the entire period of gradual reduction. Perfusion index, pulse and oxygen saturation were observed and checked against the sensor placed on a peripheral extremity. The silo-placed pulse oximeter and peripheral pulse oximeter sensors showed a similar pulse and oxygen saturation throughout the reduction period in all five patients. In general, perfusion index was higher from the silo pulse oximeter compared to the peripheral pulse oximeter reading. A pulse oximeter can be used to monitor intestinal oxygen saturation contained within a silo and help modulate the rate of manual reduction of intestine.     

大鼠腹裂模型肠管损伤的研究

目的研究先天性腹裂的肠管受损害情况，探讨该病术后并发症的原因。方法利用大鼠腹裂模型，运用组织学、生化学和免疫组织化学方法，分析腹裂胎鼠肠管的组织结构，DNA和蛋白质，细胞增生和凋亡等方面的改变。结果共获得腹裂胎鼠16只，对照胎鼠21只。与对照组相比，腹裂鼠肠管变短、充血水肿、粘连，肠壁表面纤维覆盖，壁内胶原沉积，DNA总量下降，蛋白质总量基本不变，细胞增生率下降，凋亡率上升。结论腹裂的肠管损伤是多方面的，是术后肠管运动和吸收功能异常的原因，大鼠的腹裂模型是对先天性腹裂的病因、病理等方面研究的合适工具。     

Ruptured exomphalos and gastroschisis: a retrospective analysis of morbidity and mortality in Nigerian children

The survival of infants with major abdominal-wall defects (AWD) has improved over the years in developed countries. In Zaria, northern Nigeria, survival from intact exomphalos (EX), has improved with the adoption of non-operative management. Ruptured EX (REX) and gastroschisis (GS), however, remain problematic. This is a report of the mortality in REX and GS in a retrospective review of 16 infants with REX and 14 with GS managed over 10 years at the Ahmadu Bello University Teaching Hospital, Zaria. The median age at presentation was 3 days and 24 h for REX and GS, respectively; 29 of the 30 patients were delivered at home. Two patients with REX and 4 with GS had associated anomalies involving mostly the gastrointestinal tract. Bowel or omental strangulation occurred in 13 patients, resulting in gangrene in 8. Fascial closure was achieved in 20 patients, skin closure only in 4, and in 4 improvised silo coverage was used, the latter associated with high infection rate. Neonatal intensive care units (NICU) and total parenteral nutrition (TPN) were not available. The overall mortality was 18.6% (gastroschisis 10, ruptured exomphalos 8, 11 from sepsis and 7 due to respiratory embarrassment). The management of these AWDs thus continues to be problematic in our environment, and mortality remains high. Provision of more modern supportive facilities (NICU and TPN) may improve the survival in our and similar environments. Accepted: 14 May 1999     

Reduction and local care of the artificial sac in gastroschisis and large omphalocoele — a new technique

A simple and safe technique of gradual reduction of a large gastroschisis or omphalocoele, using a tongue depressor strapped to the top of the sac and hanging from the incubator to keep the sac in an upright position, is described. Silver sulphadiazine was used as an antiseptic dressing. Offprint requests to: K. C. Tan     

Intestinal damage in gastroschisis is independent of the size of the abdominal defect

The intestinal damage in gastroschisis (GS) has been attributed to a narrow abdominal wall defect (AWD), among other causes, but this causal effect is difficult to prove in humans. The present experimental study was done to ascertain the damaging effect of clinically extrapolable mild and moderate constriction at the AWD on the intestine of fetuses with GS. AWDs of two different sizes were carried out in the fetal rabbit model: small-ring GS (1.5× bowel diameter, SRG) and large-ring GS (3× bowel diameter, LRG); a group of unoperated littermates served as controls. Fetal body weight, intestinal length and weight, bowel diameter and wall thickness, and histology were checked 7 days later. No statistical difference was found in body weight and bowel diameter among the groups. Intestinal length, weight, and wall thickness were significantly different in the GS groups compared to the controls, but no difference was found between the GS groups. Histology did not show venous stasis, ischemic lesions, or differences in the degree of edema between groups SRG and LRG. Mesothelial hyperplasia was seen in both GS groups. The intestinal changes in length, weight, diameter, wall thickness, and histology in GS should thus not be attributed to the diameter of the AWD. Accepted: 14 April 2000     

Influence of birthplace on gastroschisis outcomes in a state in the southeastern region of Brazil

ObjectiveTo characterize the influence of birthplace on outcomes of patients with gastroschisis admitted to three hospitals in a state in Brazil’s southeastern region, according to condition inborn (born in a reference center) or outborn (born outside the reference center).MethodsRetrospective multicenter cohort study of patients with gastroschisis. The sample size utilized was of patients admitted in three hospitals with a diagnosis of gastroschisis ICD 10 Q79.3 between January 2000 to December 2018. Patients were divided into two groups, inborn and outborn. Characteristics of prenatal, perinatal and postoperative were compared using statistical tests. The level of significance adopted was P-value < 0.05.ResultsIn total, 144 cases of gastroschisis were investigated. The outborn patients group had higher rates of absence of antenatal diagnosis (p = 0.001), vaginal delivery (p = 0.001), longer time between birth and abdominal wall closure surgery (p = 0.001), to silo removal (p = 0.001), to first enteral feeding (p = 0.008), for weaning from mechanical ventilation (p = 0.034), used less peripherally inserted central catheter (PICC) and required more venous dissections (p = 0.001), and lower mean of serum sodium (p = 0.015). There were no differences in mortality rates and length of hospital stay between the inborn and outborn groups.ConclusionAlthough outborn patients with gastroschisis were less likely to have an antenatal diagnosis and were more prone to a longer time to undergo surgical and feeding procedures, and to spend more time in mechanical ventilation, these disadvantages seemed not to reflect on the death rate and the length of hospital stay of patients from this group.     

Local dexamethasone improves the intestinal lesions of gastroschisis in chick embryos

Background and aims. Eviscerated bowel in gastroschisis (Gx) undergoes changes that lead to dysfunctions and create management difficulties. This study tests the hypothesis that exposure of the eviscerated bowel of chick embryos with Gx to dexamethasone might have beneficial effects on the parietal lesions.Methods. Gx was created in chick embryos on incubation day 15 and either dexamethasone (0.047 mg in 0.24 ml) or 0.075% saline were instilled into the amnio-allantoic chamber on day 17. The chicks were recovered near hatching (day 19) and eviscerated and non-eviscerated portions of the intestines were recovered, weighed and processed for HE and synaptophysin staining or for total DNA and protein measurements. Total mural and serosal layer thickness were determined and intramural ganglia were counted. ANOVA was used for comparison among groups with significance level set at p<0.05.Results. Chicks with Gx and Gx + saline controls had reduced body weight and tibial length in comparison with controls. The eviscerated bowel was heavier with marked wall thickening at the expense of all layers but particularly of the serosa. They had decreased total intestinal DNA with normal protein and decreased intramural ganglion density. In contrast, chicks from the Gx + dexamethasone group had normal body weight and tibial length, near-normal intestinal wall thickness with slightly increased serosal width, near-normal intestinal DNA content and normal density of intramural ganglia.Conclusion. Local dexamethasone had beneficial effects on the eviscerated bowel of chicks with Gx as judged by decreased wall thickening, normalization of total intestinal DNA and richer neural population. Late gestational exposure to steroids could represent another alternative for preventing intestinal lesions in Gx.     

The pivotal role of the surgeon in the results achieved in gastroschisis

A single neonatal surgical unit treated 42 cases of gastroschisis over a 12-year period (1981–1993). The surgical management of each case was individualised, but every attempt was made to perform a primary repair when possible, based on the premise that this strategy gave the best outcome. The eviscerated intestine was evaluated with the patient under general anaesthesia. Serosal peel was not removed and intestinal atresias were not repaired. Gangrenous intestine was resected. The contents of the bowel were emptied proximally via a large naso-gastric tube and distally via the anus with warm saline lavage. The anterior abdominal wall was stretched and then reduction of the prolapse attempted. Following maximal enlargement of the peritoneal cavity, it was left to the operator to decide whether primary repair was possible and, indeed, permissible in each instance. Staged repairs necessitated the use of silastic pouches. Respiratory and intestinal insufficiency were managed by intermittent positive-pressure ventilation and total parenteral nutrition (TPN). Over one-half of the cases (24 of 42) were under 2.5 kg at birth. Intra-uterine growth retardation was unusual. Ten babies were delivered for obstetrical indications by Caesarean section: 50% were pre-term and in 4 pre-natal diagnosis of a ventral abdominal wall anomaly had been made. The transmural defects were all sited at the umbilicus and were to the right of a consolidated cord in 41 instances. Midgut necrosis due to torsion was encountered in 1 case; 3 further cases with intestinal atresia occurred. Primary closure was obtained in 30 (71%) of the cases reviewed. A prosthetic pouch was used in 12 patients for on everage 10 days in 10 uncomplicated cases. The average length of time in days of tertiary care given to 25 uncomplicated cases treated by primary fascial closure was: ventilatory support 4; intensive care treatment 8; and nutritional source TPN 20. There were 5 deaths (12%): 1 was unpreventable due to prenatal intestinal infarction; 2 were due to abdominal compartment syndrome with renal failure, and, intestinal ischaemia complicating primary and planned staged repairs; 1 caused by intestinal infarction due to torsion of bowel in a pouch; and 1 due to invasive infection. The role played by the strategy taken by the surgeon in the management of gastroschisis is crucial to the outcome. The creation of a compartment-like syndrome produced uncorrectable complications in this series of cases in both primary and staged abdominal wall closures. Minor degrees of this complication proved to be reversible in some patients, which was the reason for the wait-and-see attitude adopted in the management of this problem, often with fatal outcome. Where intra-peritoneal pressure monitoring is not used, the operating surgeon relies on unscientific observations for decision-making at the operating table. The time from birth to operation in 25 of the reviewed cases was on average 5 1/2 h. Of this group, 20 were outborn babies. This is unsatisfactory, but as shown by this review, even in the absence of prenatal management, which should ensure prompt repair, satisfactory results are still possible.     

The impact of iatrogenic gastroschisis on pulmonary maturation in the fetal rabbit models of congenital diaphragmatic hernia

Purpose  The aim of this study was to analyze the effect of iatrogenic gastroschisis on pulmonary hypoplasia in fetal rabbits with congenital diaphragmatic hernia (CDH). Materials and methods  A total of 30 pregnant rabbits received fetal surgery on gestational day 23. A left diaphragmatic hernia was created in one end fetus (DH group) of each rabbit, and the other end fetus of the same rabbit received sham thoracotomy as control (CR group). Another 19 pregnant rabbits underwent partial resection of the diaphragm in both end fetuses on gestational day 23, and then artificial gastroschisis was performed on one end fetus (GS group) on gestational day 26, while the other end remained as control (CGS group). The fetuses were harvested on gestational day 30. The histological and morphometric evaluation of lungs and livers of the end fetuses in each group was conducted. Results  In the DH group, the lungs were hypoplastic with a decrease in the total lung weight to body weight ratio, and remarkable thickening in alveolar septa. The lung vessels showed significantly thicker arterial walls when compared with those from control fetuses. The pathological finding in the CGS group was similar to that of the DH group. The thickness of the alveolar septa and of the pulmonary arterial walls showed no significant difference among the GS group, DH group and the CGS group. The ratio of liver weight to body weight increased notably in the GS group, DH group and CGS group compared with that in the CR group. Conclusions  In the fetal rabbit models of CDH, pulmonary hypoplasia is the most significant pathological feature. Iatrogenic gastroschisis does not improve pulmonary maturation due to the active growth of the liver that herniates into the thoracic cavity.     

The effect on the intestines of continuous release of methylene blue from a drug delivery system: an experimental study in a chick embryo gastroschisis model

Increased small bowel nitric oxide synthase (NOS) activity has been suspected as a cause of postnatal intestinal dysmotility in gastroschisis. The effect of continuous delivery of methylene blue loaded polymer (MBLP) hydroxy-propyl methyl cellulose-ethyl cellulose (HPEC—MC) and daily injection of methylene blue (MB) on the intestinal damage (ID) was evaluated using a chick embryo gastroschisis model. Fourteen-day-old fertilized chick eggs were divided into five groups. In the control (C) group, no intervention was performed. In the sham (S) group, the allantoic and amniotic membranes were opened to create a common cavity that resembles the amniotic cavity in human. In the gastroschisis only (GO) group, a defect in the abdominal wall of the embryo was made, and intestinal loops were exteriorized following connection of amniotic and allantoic cavities. In the gastroschisis plus methylene blue (G+MB) group, gastroschisis was created and MB administered into the amnioallantoic cavity (AAC) by daily injections for 5 days. In the gastroschisis plus methylene blue loaded polymer (G+MBLP) group, MBLP was placed into AAC after gastroschisis was created. At the end of the 19th day of incubation, intestinal morphological changes were investigated macroscopically and microscopically. Although the survival rates were decreased in the chick embryos with creation of gastroschisis compared with C and S groups (p<0.001), the survival rates were increased in G+MBLP group (76.92%) when compared with the GO group (41%) (p<0.001). Because of multiple intervention of embryos, higher mortality was observed in the G-MB group (75.61%). Macroscopic and microscopic scores of ID and mean intestinal wall thickness were significantly higher in the GO group when compared with C, S, G+MB, and G+MBLP groups (p<0.001). The mean score of intestinal ganglia morphology was significantly increased and the total number of ganglion cells was significantly decreased in the GO group when compared with C, S, G+MB, and G+MBLP groups (p<0.001). It is possible to decrease intrauterine intestinal morphological changes in gastroschisis by inhibiting NOS. As a first preliminary study, we believe that use of MBLP may be an alternative for fetal treatment by eliminating the harmful effects of multiple interventions or amniotic fluid exchanges.     

The role of labor in gastroschisis bowel thickening and prevention by elective pre-term and pre-labor cesarean section

Twelve consecutive cases of gastrochisis seen during the past 3 years are reported. Four were managed by elective pre-term and pre-labor cesarean section as soon as lung maturity was established by biochemical means and 8 by delivery after the onset of labor, 4 by vaginal delivery and 4 by cesarean section. There was no intestinal thickening or peel in any of the 4 infants born by elective pre-term and pre-labor section. All were quickly and easily repaired (the last 3 with umbilicus preservation) with no associated atresias or stenosis and with minimal length of hospitalization. The 8 cases delivered after the onset of labor all had marked instestinal thickening, 2 (25%) had severe and extensive intestinal necrosis or atresias (apple-peel), and 1 died (12.5%). All had prolonged and complicated hospital stays with multiple operations. The routine use of elective pre-term and pre-labor section in all cases of gastroschisis diagnosed prenatally by -fetoprotein (AFP) screening and ultrasound is strongly recommended, as are routine AFP screening and ultrasound studies during pregnancy.     

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。     

The influence of delay in closure of the abdominal wall on outcome in gastroschisis

 To evaluate the effect of a delay in closure of the abdominal wall (AWC) on outcome in the management of gastroschisis, a retrospective analysis of 91 babies admitted over a 7-year period (1992–1998) to a single neonatal surgical unit with a diagnosis of gastroschisis was carried out. Antenatal diagnosis was made in 89 (98%) cases. Surgical intervention occurred in 90 babies at a median of 4 h (standard error 0.345, range 0.5–17) post-delivery. In 72 (80%) cases primary closure of the abdominal defect was achieved, with a silo fashioned in the remaining 18 (20%). One infant died prior to AWC. The median time to full oral feeding was 22 days (2.96, 5–160), and to discharge 28 days (4.03, 11–183). There was no correlation between time to AWC and any measured outcome parameter. There was no significant difference in mortality in those patients having closure before 6 h. Thus, no correlation between time to AWC and outcome was demonstrated. This would suggest that the time taken to optimally resuscitate a newborn infant prior to surgical closure does not have an adverse influence upon outcome and is to be recommended. Accepted: 31 January 2000