Left-sided gastroschisis with meromelia of the limbs: A rare association

Gastroschisis is a malformation of the anterior abdominal wall that presents as herniation of abdominal contents through a paramedian abdominal defect, usually to the right side of the midline. The defect involves all the layers of the abdominal wall and there is no membranous covering. In newborns with isolated gastroschisis, the overall prognosis is very good. In rare cases, gastroschisis is associated with other congenital malformations, making the prognosis worse. We herein report a rare case of left-sided gastroschisis associated with meromelia of the limbs.     

Active enlargement of the abdominal cavity--a new method for earlier closure of giant omphalocele and gastroschisis.

The authors present their own modification of surgical staged repair of large abdominal wall defects (AWD). 21 newborns with gastroschisis (GSCH) and 8 with omphalocele (OC) are presented. In all patients the protruding viscera were placed in a prosthetic sac with a semi-permeable lining. The sac was suspended using a system with active external traction with a force amounting to 30 - 40 % of the baby's weight. All babies tolerated the applied method well. A progressive stretching of the abdominal wall and enlargement of the abdominal cavity enabled the defect to be closed between the second and sixth postoperative day. In comparison to the classic method, the presented modification of staged repair of AWD makes an earlier reduction of the viscera into the abdomen possible and does not seem to be associated with a higher risk of mechanical or infectious complications.     

Advances in treatment of congenital gastroschisis based on personal examinations

The aim of the study was the definition of standard approach to a newborn with gastroschisis. The rules of treatment introduced in 1992 enabled the closure of the abdominal wall primarily in all newborns and made it possible to decrease the mortality considerably. This was achieved despite the fact that we usually did not have influence on time and method of delivery or quality of transportation to our department. In the Department of Pediatric Surgery of Institute Mother and Child, in the years 1992-1997, twenty three newborns with gastroschisis were admitted. The standard approach in all patients was the following: insertion of the catheters to the small bowel through the appenddicocaecostomy and to the large bowel through the rectum, intraoperative irrigation of the meconium (mucosolvan), forceful stretching of the abdominal wall, returning of extraabdominal viscera under control of central venous pressure or middle airway pressure (anesthesiologic control). In the postoperative period all patients routinely had controlled ventilation, administration of analgesic drugs, catecholamines, antibiotics and total parenteral nutrition. Intestinal catheters were gradually removed every day for irrigations of all levels. Oral nutrition was started after the return of effective peristalsis. From 23 treated newborns 16 are alive. 7 children died, 4 of them were resuscitated before operation because of severe general condition. Two patients died because of sepsis in the second month of life, one because of SIDS (sudden infant death syndrome) in the third week of life. Sixteen children are followed up. The development of all children is normal.     

Major abdominal wall defects in the low- and middle-income setting: current status and priorities

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30–100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.     

Review of the evidence on the closure of abdominal wall defects

Infants with congenital abdominal wall defects pose an interesting and challenging management issue for surgeons. We attempt to review the literature to define the current treatment modalities and their application in practice. In gastroschisis, the overall strategies for repair include immediate closure or delayed operative repair. The best level of data for gastroschisis is grade C and appears to support that there is no major difference in survival between immediate closure or delayed repair. In patients with omphalocele, the management techniques are more varied consisting of immediate closure, staged closure or delayed closure after epithelialization. The literature is less clear on when to use one technique over the other, consisting of mostly grade D and E data. In patients with omphalocele, a registry to collect information on patients with larger defects may help determine which of the management strategies is optimal.     

生物组织补片修补先天性腹裂9例

目的：总结采用同种异体脱细胞生物组织补片整复、修补先天性腹裂的治疗经验。方法回顾性的分析自2010年以来采用同种异体脱细胞生物组织补片修补的新生儿先天性腹裂9例患儿的临床资料。患儿入院后尽快送入手术室,以温生理盐水及温碘伏仔细冲洗并还纳脱出的腹腔内容物后,将生物组织补片与除皮肤外的腹壁创口边缘全层间断缝合。术后送SICU监护。3例患儿未行呼吸机支持治疗,6例患儿分别行呼吸机支持治疗1～2d。结果9例患儿术后恢复顺利,切口愈合好,无红肿渗液,痊愈出院。随访6月～3年,其中7例腹壁缺损已完全愈合,2例留有小型腹壁疝,待二次手术修补,患儿营养发育与同龄儿无明显差别。结论腹裂手术的原则是回纳疝出脏器,关闭腹壁筋膜及皮肤。但由于腹壁缺损大,需要采取分期手术或延迟手术。生物组织补片其细胞毒性小,生物相容性好,无明显致敏性及排斥反应,修补腹裂后,能有效的减轻腹腔压力,可以一期完成手术,适用于先天性腹裂的治疗。     

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。     

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。     

Gastroschisis: primary closure is possible in most newborns

For more than 15 years, we have practiced a policy of primary repair in cases of gastroschisis. Regardless of the appearance of the intestine and the size of the abdominal cavity, a primary repair has always been considered the method of choice. Twenty-five cases treated consecutively at our departments from February 1984 to April 1990 were investigated. A majority of the infants were preterm and of low birth weight. A majority also had severe bowel abnormalities. In 1 case a delayed repair with a silo had to be performed. In 2 others a small fascial defect had to be temporarily covered with a silastic patch, but in the remaining 22 cases (88%) a primary repair could be carried out without significant morbidity. Two infants died, 1 from severe neonatal asphyxia and cerebral hemorrhage and 1 from liver failure associated with a short-bowel syndrome (the only silo case). It is our belief that primary closure is possible and should be considered in most newborns. Correspondence to: L. Olsen     

Treatment of congenital abdominal wall defects —a 25-year review of 132 patients

The outcome data of 132 patients treated at the Department of Pediatric Surgery in Mainz during the last 25 years were reviewed. Prenatal diagnosis of abdominal wall defects (AWDs) and associated malformations led to increasing selection of the patient population. The aim of primary closure of the abdominal wall can be achieved more frequently in gastroschisis (GS) than omphalocele (OC), while the postoperative course is more complicated and of longer duration in GS. Delayed or secondary closure extended the hospitalization period but had no negative effects on the outcome. Reoperations or planned secondary operations were performed in 23 patients with GS and 14 with OC. Early mortality was 15/55 for OC and 21/77 for GS over the period of 25 years. In recent years, a drastic reduction in mortality has occurred, and mortality is now mainly due to additional malformations. Further development and quality of life are not significantly reduced after survival of an isolated AWD. Malpositioning of parenchymatous organs after closure of AWDs has to be considered during pregnancy and abdominal operations.     

The outcome of newborns with abdominal wall defects according to the method of abdominal closure: the experience of a single center

Recent reports suggest that the technique of abdominal closure in neonates with anterior abdominal wall defects (AWD) correlates with the outcome. The aim of this study is to analyze factors related to mortality and morbidity, according to the technique of abdominal closure of these neonates. Retrospective analysis of charts from 76 consecutive neonates with AWD treated in a single institution. They were divided according to the type of abdominal wall closure: group I: primary closure, group II: silo followed by primary closure and group III: silo followed by polypropylene mesh. Outcome was analyzed separately for neonates with gastroschisis and omphalocele. There were 13 deaths (17.1%). Mortality for neonates with isolated defects was 9.6%. Mortality rate was similar in all groups for either neonates with gastroschisis or omphalocele. Postoperative complications were not significantly different among groups except for a prolonged time of hospitalization in group III. Mortality rate is not correlated with the type of abdominal closure. Neonates with primary closure or with other methods of abdominal wall closure had similar rate of postoperative complications. Neonates with mesh closure of the abdomen have prolonged hospitalization. The use of a polypropylene mesh is a good alternative for neonates whose primary closure or closure after silo placement is not possible.     

A comparative investigation of the bowel wall in gastroschisis and omphalocele: relation to postoperative complications

In gastroschisis the protruding bowel is generally covered by a fibrous coating as a result of prolonged exposure to amniotic fluid but no such coating is found on the exposed bowel loops in a ruptured omphalocele. In patients with gastroschisis, the postoperative course is often complicated by periods of hypoperistalsis. Based on studies of animal models of gastroschisis, various authors have suggested that the postoperative complications might be due to structural defects of the bowel wall. In our own experimental investigation of gastroschisis using chick embryos, we found no evidence of a structural defect of the bowel wall. With a view to clinical substantiation of our experimental findings, we conducted a comparative investigation of patients material, involving 10 cases of gastroschisis, 5 cases of ruptured omphalocele, and 9 cases of intact omphalocele. The investigation revealed an entirely normal structure of the bowel wall in all 10 cases of gastroschisis. On the serosa we noted an inflammation infiltrate rich in collagen fibers, frequently containing squamous cells (vernix) and lanugo. In the cases of ruptured omphalocele, the bowel wall specimens revealed a subacute inflammatory reaction. No major abnormalities were found in the bowel wall of patients with an intact omphalocele. Normal development of the autonomic nervous system was found in all patient material. Severe ischemic changes of the bowel wall were found in 4 of the gastroschisis cases and these were the 4 patients who suffered from postoperative hypoperistalsis. Thus the complications occurring during the postoperative phase of gastroschisis are most probably due to ischemic bowel damage.     

Familial occurrence of gastroschisis. Four new cases and review of the literature.

In two unrelated families, there was familial occurrence of gastroschisis. In one family, a boy and girl were affected and there was a family history of stillbirth, abortion, prematurity, and esophageal obstruction. In the second family, two boys were affected and there was a family history of spontaneous abortion, inguinal hernia, and umbilical hernia. The recurrence of gastroschisis, generally considered a sporadic congenital effect, suggests that the condition may be genetic in nature. Furthermore, the pedigree of one of the families suggests that gastroschisis may be a severe expression of umbilical hernia or other abdominal wall defects. Autosomal dominant inheritance with variable expressivity or multifactorial inheritance may explain the occurrence of gastroschisis in the two families. Thus, a family history of abdominal wall defects may increase the risk for gastroschisis.     

Gastroschisis: neonatal features and somatic development

Thirty-nine newborns were treated for gastroschisis at the Children's Hospital, Helsinki University Central Hospital from 1964 to 1980. Sixteen infants died during the first hospital admission. Of the 23 survivors, 11 were two years or older, and these 11 children were subjected to a follow-up examination. The incidence of the lesion increased markedly during the period of observation. Dysmaturity (birth weight at or below the 10th percentile of normal intrauterine growth) accompanied gastroschisis in 17 cases, and a low birth weight (below 2,500 g) in 23 cases. The mortality of gastroschisis was clearly and constantly decreasing, being 54% for the first decade, 23% for the last 5 years, and 41% for the total series. The age of the mothers at parturition, 20.9 years, was significantly (p less than 0.001) lower than the Finnish average. The extensive clinical and laboratory analysis of the 11 oldest children with completed reconstruction of the abdominal wall indicated good physical condition and, specifically, normal absorptive function of the intestine.     

小肠黏膜下层生物补片在腹裂修补术中的应用

目的 探讨小肠黏膜下层生物补片在腹裂修补术中的作用.方法 2010年6月至2015年5月,我们对20例腹裂患儿采用生物补片进行修复,其中12例采用小肠黏膜下层(SIS)生物补片一期修补腹壁缺损(SIS组),8例采用脱细胞真皮生物补片一期修补腹壁缺损(真皮组),与2006年6月至2010年5月收治的14例未用任何组织替代物、强行一期修补的腹裂患儿(对照组)的临床资料进行比较,观察胎龄、出生体重、出生至手术时间、缺损大小、暴露于腹腔外脏器情况、术后机械通气情况、术后开始进食时间、住院时间、切口感染、腹壁疝的发生率等指标.结果 三组患儿平均胎龄、出生体重、出生至手术时间、缺损大小、腹腔脏器脱出情况之间差异无统计学意义;SIS组12例患儿中仅2例患儿术后需机械通气,平均通气时间24 h,真皮组2例患儿需机械通气,平均通气时间19h,对照组10例患儿需机械通气,平均机械通气时间39 h,补片组机械通气的必要性和通气时长显著低于对照组,补片组中SIS组和真皮组机械通气的必要性和通气时长差异无统计学意义;SIS组和真皮组术后的开始进食时间分别是(186.5±37.7)h、(173.3±41.5)h,显著少于对照组开始进食时间(256.1±41.8)h;SIS组和真皮组的住院时间分别是(16.2±3.0)d、(15.1±2.2)d,显著少于对照组的住院时间(19.4±3.6)d;SIS组术后无切口感染发生,有2例术后3个月发生切口疝,1年后自行愈合,无需再次手术修补;真皮组术后3例发生切口感染、排异反应,经伤口换药、去除补片后瘢痕愈合,有2例术后5个月发生切口疝,2例约1年后逐渐自行愈合,无需再次手术修补;对照组5例发生切口感染,经换药后好转,3例切口裂开,蝶形胶布固定换药后瘢痕愈合,5例术后3个月发生切口疝,3例1年后逐渐愈合,2例2年后未愈合施行手术修补.结论 对腹壁缺损大、腹腔容积小的腹裂患儿可以采用一期补片修复的方式,以减少分期手术率、术后机械通气,降低术后腹腔压力,有利于肠道功能恢复;小肠黏膜下层(SIS)和脱细胞真皮组织补片均能用于修复腹壁缺损,但小肠黏膜下层在生物相容性、抗感染等方面优于脱细胞真皮组织.     

Intra-abdominal inverted umblical cord in gastroschisis: a unique ultrasound finding

A relatively new surgical technique allows for sutureless closure of a gastroschisis defect. Immediately after birth, a long umbilical cord stump is temporarily inverted into the abdominal cavity and later retracted and used to close the abdominal wall defect. Knowledge of this entity is important since the inverted umbilical cord simulates an intra-abdominal mass on cross-sectional imaging. While this procedure is well described in the surgical literature, the imaging features of inverted umbilical cord have yet to be reported. The case presented here highlights the sonographic imaging findings of the umbilical cord during the intestinal decompression phase of sutureless repair of gastroschisis.     

Use of a plastic hemoderivative bag in the treatment of gastroschisis

The authors report their experience with the use of a polyvinyl chloride (PVC) bag for blood-derivative transfer as a prosthesis for the creation of a silo for surgical treatment of gastroschisis (GS) in seven newborn infants. The bag is sterile, impermeable to micro-organisms, transparent, flexible, resistant, internally smooth, does not adhere to the bowel loops, readily available, and inexpensive, properties that make it an excellent alternative as a prosthesis for staged surgical treatment of congenital anomalies of the abdominal wall such as GS and omphalocele. The importance of a multidisciplinary team for the care of newborns with GS is also emphasized for a good postoperative outcome. Accepted: 5 January 1999     

Gastroschisis: etiology,diagnosis, delivery options,and care

Gastroschisis, one of the more common congenital abdominal wall defects, results in herniation of fetal abdominal viscera into the amniotic cavity. This article discusses theories about gastroschisis etiology, in utero diagnostic tools, delivery options, and postdelivery care. Included are detailed considerations regarding immediate interventions after delivery to support the infant's thermal and fluid management needs and to protect the exposed bowel. Surgical options and postoperative care issues and complications are reviewed, as are respiratory distress and vena cava compression from increased abdominal pressure, nutritional support, and interventions related to the prevention of infection. Giving birth to an infant with gastroschisis is an upsetting experience for parents. Evidence suggests, however, that with today's advances in neonatal care and nutrition and with meticulous attention, the survival rate for infants born with gastroschisis can be excellent.     

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.     

Anatomy and embryology of abdominal wall defects

Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele.