The perinatal management of gastroschisis

Gastroschisis is increasing in frequency and is becoming a common condition. It is now invariably detected antenatally and although the long-term outcome in the majority of cases is excellent, the existence of both fetal and postnatal complications has led to variations in practice to try to optimise outcome. This article reviews the evidence for some of these variations where such evidence exists and provides a contemporary view of best practice where it does not.     

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.     

Prenatal diagnosis and management of gastroschisis and omphalocele

Prenatal diagnosis of gastroschisis (GS) and omphalocele (OC) has allowed improved antenatal and perinatal management. It was the aim of this study to compare prenatal findings and assess fetal outcome. Twenty-four fetus with GS and 33 with OC were diagnosed prenatally. Maternal serum alpha-protein (MSAFP), sonographic (US), and perinatal data were analyzed. The average maternal age for GS was younger than for OC (24 vs. 30 years). The median MSAFP values were 7.7 multiples of median for GS and 3.6 for OC. The initial US diagnosis was made at 22 weeks for GS and at 19 weeks for OC. A pathological karyotype was observed in 4% of the fetuses with GS and 33% with OC. The mortality of fetuses with OC was twice as high as that of those with GS. Accurate prenatal diagnosis of GS and OC as well as their differentiation are of crucial importance for both counselling of the parents and the resulting antenatal consequences, and provide the basis for optimal interdisciplinary co-operation at a perinatal tertiary-care center.     

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.     

The umbilicus in gastroschisis: aesthetic considerations

Preservation of the umbilical cord attachment (UCA) in gastroschisis (GS) is still not routine practice. In a prospective series of 36 children with GS, it was always possible to preserve the UCA, even in those undergoing a temporary silo and delayed closure. Reconstruction by umbilical cord capping left no additional scar and achieved a normal abdominal wall. Mild cellulitis in 3 infants resolved on antibiotics, and an initial umbilical weakness in 7 did not require additional surgery. We conclude that preservation of the UCA should be an integral part of surgical technique for all infants with GS. Reconstruction by umbilical cord capping alone achieves an unscarred abdominal wall with an umbilicus of normal shape and position.     

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。     

Elective preterm section for improved primary repair of gastroschisis

For the first time in some 36 years of experience with gastroschisis. the author has encountered in short order both his first and second cases of gastroschisis without any thickening of the eviscerated intestine. Both infants, whose gastroschisis had been detected by maternal alphafetoprotein and ultrasound studies, were delivered by elective preterm cesarean section and were repaired easily with closure of the abdomen in layers. It is suggested that the bowel thickening and rigidity in gastroschisis may result from the trauma of labor and delivery and that elective preterm section, as soon as lung maturity can be demonstrated, may substantially improve the ease of primary repair in gastroschisis.     

The effect of mode of delivery on outcome in fetuses with gastroschisis

The postoperative course in neonates with gastroschisis after successful surgical repair is complicated by prolonged ileus. Elective cesarean section (CS) has been advocated to limit damage to the bowel due to labor and compression in the narrow birth canal and to prevent retrograde infection. We reviewed the records of 58 infants with gastroschisis treated between 1977 and 1993; 12 were delivered by CS for obstetrical reasons and 46 were delivered vaginally (V). Mortality was higher in the CS group (V = 1/46, CS = 4/12). Both groups differed significantly with respect to birth weight and gestational age; gestational age, however, had no influence on morbidity and mortality. Primary contamination was significantly lower in the CS group (V = 29/42, CS = 3/12). However, no difference was found regarding infectious complications. No complications could be attributed directly to the mode of delivery. Since these data do not show any significant advantages of CS on morbidity and mortality, we do not recommend CS for fetuses with gastroschisis diagnosed prenatally.     

自体脐带片修补先天性腹裂

目的　报告用新生儿自体保留的脐带做成脐带片 ,修补先天性腹裂的临床观察。方法　自 1995年 5月～ 2 0 0 0年 6月 ,用自体脐带片修补新生儿腹裂 6例 ,患儿入院后立即清洗消毒 ,根据保留脐带的大小分别获得 3.0～ 3.5cm× 5 .0～ 8.0cm大小的脐带片 ,保留左侧基部 ,右侧脐片与裂开的腹壁边缘间断缝合。结果　 1例术后第 4周发生肠粘连、肠梗阻 ,家长放弃再手术而死亡 ,5例治愈出院。随访 4例 ,随访时间 3个月～ 3年 ,3例上皮覆盖后无切口疝 ,1例遗留小型腹壁疝 ,待Ⅱ期修补。患儿营养发育良好。结论　自体脐带片是患儿自身的生物活性组织 ,无毒性 ,无抗原性 ,并具有一定的弹性 ,修补腹裂后 ,能有效的减轻腹腔压力 ,并且取材容易     

Intraoperative vascular assessment for estimation of risk in primary closure of omphalocele and gastroschisis

Surgical repair of anterior abdominal wall defects is often complicated by a discrepancy between the eviscerated organs and the intra-abdominal space available. Primary closure of the abdominal wall may result in increased intra-abdominal pressure and consecutive circulatory impairment. We report the results of a retrospective and consecutive prospective study evaluating the influence of different witameters on mortality and morbidity in children with gastroschitis and omphalocele. Both studies demonstrated that real-time sonographic investigations, intraoperative Doppler duplex sonography, and colour-coded Doppler sonography provide the oppurtunity to collect objective intraoperative data. Our data indicate that intraoperative vascular assessment facilitates the discrimination between infants who benefit from primary closure and those in whom a staged repair is the treatment of choice.     

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。     

Infections in gastroschisis: organisms and factors

This study aimed to define the incidence, causative organisms and predisposing factors leading to infection related morbidity in newborns with gastroschisis. All gastroschisis patients admitted over the 5-year period (1999-2004) were retrospectively reviewed. Surveillance samples, wound, blood, urine and fecal cultures were analyzed. Duration of total parenteral nutrition, antibiotic therapy, feeding regimes and demographic data were also analyzed. Multiple logistic regression was employed using the SPSS system and p < 0.05 was considered as significant. Seventy-two neonates were identified with 53% having abnormal gut carriage mostly due to Enterobacter and Klebsiella. Wound infection occurred in 20% of cases. Abnormal gut carriage predisposed to the development of wound infection. Line sepsis occurred in 21% of neonates. Endogenous coagulase negative Staphylococcus caused 74% of septic episodes. There was no correlation between abnormal gut carriage and the development of line sepsis. Overall survival was 96%. The cause of infections in gastroschisis patients appears to be multifactorial. A multidisciplinary team can play an important role in reducing the incidence of infections. Strict aseptic protocols and auditing practice can be the invaluable tools in decreasing morbidity rates.     

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。     

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.     

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.     

Neonatal haemochromatosis associated with gastroschisis

We describe, to our knowledge, the first case of progressive neonatal liver failure due to neonatal haemochromatosis (NH) occurring in an infant with a gastroschisis and review the literature regarding these two conditions. A 1,665 g male infant with antenatally diagnosed gastroschisis was born with a severe coagulopathy, anaemia, thrombocytopenia, hypoglycaemia and jaundice. He developed progressive liver failure, complicated by necrotising enterocolitis. Serum ferritin was elevated at 1,459 μg/L. He died on day 40 and a limited post-mortem examination confirmed significant hepatic siderosis with fibrosis and cholestasis, and siderosis of the pancreas. Although no genetic aetiology for gastroschisis has been identified, an occasional inherited tendency has been observed. There is also evidence to support an autosomal recessive inheritance in NH.     

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.     

Late follow-up in patients with gastroschisis

During a 16-year period, 60 neonates with gastroschisis were treated at the Department of Pediatric Surgery in Graz; 6 died and 54 (90%) survived. A questionnaire was sent to 45 patients, who were called for a medical examination; 31 patients (69%) came to follow-up, 26 of whom reported minor abdominal problems that could be related to gastroesophageal reflux (GER). Fifteen agreed to 24-h esophageal pH monitoring and/or upper gastrointestinal series; in 7 pathological GER could be demonstrated. Manometric studies in 6 patients revealed a motility disorder of the esophagus. Only 4 children were concerned by a disfiguring scar or the absence of a navel. Heigtt and weight were within normal limits and the children had developed normally.     

Vanishing gut in infants with gastroschisis

Infants born with gastroschisis (GS) in association with intestinal atresia are well described, however, atresia to the extent where none or very little of the midgut remains has rarely been reported. In this paper we present one infant born with a GS defect in combination with total loss of the midgut and another with a tight GS defect with atresia of the proximal and distal midgut at the regions where the bowel had gone through the abdominal wall. On examining these and other case reports in the literature, we realised that there is a GS spectrum, at one end with simple, localised atresia with the rest of the gut exhibiting minimal vascular compromise and at the other a very small or no defect with absence of the entire midgut. Accepted: 12 January 1999     

非麻醉下床边应用免缝Silo袋处理新生儿腹裂

目的 介绍非麻醉状态下于床边应用免缝Silo袋分期处理无法Ⅰ期关闭的新生儿腹裂。方法 2004年3月和7月．收治2例新生儿腹裂，分别出生后2h和4h，1例肠管污染严重，另1例合并有肠旋转不良和胎粪性肠梗阻，2例均在清醒状态下于NICU床边应用免缝Silo袋Ⅰ期处理无法回纳腹腔的肠管，2例分别于第6d和第7d在肠管完全回纳腹腔后至手术室全麻下行腹壁关闭脐部成形术，其中例2同期行Ladd’s术和肠内胎粪清除术。结果 2例患儿术后恢复好，脐部Ⅰ期愈合，分别随访5个月和2个月，生长发育好。结论非麻醉状态下床边应用免缝Silo袋分期处理无法Ⅰ期回纳的新生儿腹裂是一简便安全的方法，并可推广至产房早期处理腹裂患儿，将有效减少患儿肠管的继发性水肿和炎症，提高生存率。