Complex Gastroschisis Research Articles

OC26.01: First trimester screening for preterm birth

Objectives: The morbidity of infants with largely depends on coexisting intestinal anomalies such as intestinal atresia, stenosis, perforation, necrosis or volvulus. We aimed to establish a correlation between prenatal ultrasound findings and postnatal outcome in foetuses with gastroschisis. Methods: A retrospective review of antenatal cases of isolated gastroschisis referred to our centre from 1999 to 2010 was carried out. The following prenatal ultrasound markers were analysed for their correlation with bowel complications: the presence of IUGR ( 6 mm), thickened intestinal wall (>3 mm), stomach dilatation. The postnatal outcome was obtained and the neonates were divided into two groups: 1st group with associated bowel anomalies (‘‘complex’’ group), 2nd group without bowel anomalies (‘‘simple’’ group). The outcome measures were: the time on parenteral nutrition (TPN), the length of hospital stay (LHS), foetal or neonatal death. Results: Over a 12-year period there were 109 cases of gastroschisis followed in our unit. One hundred and five cases were eligible for analysis. Survival rate was 101/105 (96.2%). There were two neonatal deaths and two intrauterine foetal demises. None of the ultrasound markers was predictive of foetal or neonatal death. Fifteen of 103 live-born babies (14.6%) had ‘‘complex’’ gastroschisis. The median TPN in this group was 102 days (range 22–1095) and the median LHS 110 days (range 29–450) compared to median TPN 34 days (range 10–360) and median LHS 42 days (range 20–225) in the simple gastroschisis group (P < 0.05). The only ultrasound marker predictive of ‘‘complex’’ gastroschisis was intraabdominal bowel dilation (OR = 2.8 [95% CI 1.07–7.36] P = 0.037. Conclusions: Infants with ‘‘complex gastroschisis’’ are likely to have substantial morbidity and worse outcome. The only ultrasound marker predictive of ‘‘complex’’ gastroschisis is intra-abdominal bowel dilation. OC25.04 2D sonographic evaluation of the fetal palate

Negative pressure wound therapy in the management of neonates with complex gastroschisis

Negative pressure wound therapy (NPWT) is an accepted form of treatment in managing complex wounds in adults and children. It is not as widely used in the neonatal population due to limited clinical experience. We describe the application of the RENASYS™ system (Smith and Nephew, UK) in delivering NPWT to four neonates with complex gastroschisis, all of whom achieved successful outcomes.

Gastroschisis: a national cohort study to describe contemporary surgical strategies and outcomes

Background Information on adoption of newer surgical strategies for gastroschisis and their outcomes is largely limited to hospital-based studies. The aim of this study was to use a new UK national surveillance system to identify cases and thus to describe the contemporary surgical management and outcomes of gastroschisis. Methods We conducted a national cohort study using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System to identify cases between October 2006 and March 2008. Results All 28 surgical units in the United Kingdom and Ireland participated (100%). Data were received for 95% of notified cases of gastroschisis (n = 393). Three hundred thirty-six infants (85.5%) had simple gastroschisis; 45 infants (11.5%) had complex gastroschisis. For 12 infants (3.0%), the type of gastroschisis could not be categorized. Operative primary closure (n = 170, or 51%) and staged closure after a preformed silo (n = 120, or 36%) were the most commonly used intended techniques for simple gastroschisis. Outcomes for infants with complex gastroschisis were significantly poorer than for simple cases, although all deaths occurred in the simple group. Conclusions This study provides a comprehensive picture of current UK practice in the surgical management of gastroschisis. Further follow-up data will help to elucidate additional prognostic factors and guide future research.

Ward reduction of gastroschisis: risk stratification helps optimise the outcome

Categorization of gastroschisis into low risk (simple) and high risk (complex) has been reported as an important determinant of outcome. The role of risk categorization in choosing the optimal surgical approach is unreported. This study aims to investigate the role of risk categorization in decision making for ward reduction of gastroschisis. Data on a cohort of 52 consecutive neonates with gastroschisis between 2000 and 2009 were reviewed. A clinical pathway based on risk categorization was implemented in 2004, and children with simple gastroschisis underwent ward reduction and those with complex gastroschisis underwent surgical closure. Thirty-three neonates since 2004 were analysed and compared to the 19 born prior to 2004. Of the 33 children with gastroschisis in the study group, 23 were assessed as simple and underwent ward reduction with 96% survival. Ten had complex gastroschisis and underwent varying surgical procedures. Only six out of ten children (60%) with complex gastroschisis survived in spite of multiple surgical attempts. Risk stratification of gastroschisis at birth helps in choosing optimal surgical management. Ward reduction can be successfully and safely performed in all children with simple gastroschisis. Those with complex gastroschisis require conventional surgical treatment.

Does A Multidisciplinary Nutrition Team Improve the Outcomes of Infants With Simple or Complex Gastroschisis?

Does A Multidisciplinary Nutrition Team Improve the Outcomes of Infants With Simple or Complex Gastroschisis?

Risk stratification in gastroschisis: can prenatal evaluation or early postnatal factors predict outcome?

The prenatal or postnatal factors that predict complex gastroschisis in patients (atresia, volvulus, necrotic bowel and bowel perforation) remain controversial. We evaluated the prognostic value of prenatal ultrasonographic parameters and early postnatal factors in predicting clinical outcomes. We analyzed maternal and neonatal records of 46 gastroschisis patients treated from 1998 to 2007. Information regarding demographics, prenatal ultrasound data when available, intrapartum and postnatal course was abstracted from medical records. Outcome variables included survival, ventilator days, TPN days, time to full enteral feeds, complications and length of stay. Univariate or multivariate analysis was used, with P < 0.05 considered as significant. A total of 75% of complex patients were categorized within 1 week of life. Interestingly, prenatal bowel dilation (>17 mm) and thickness (>3 mm) did not correlate with outcome or risk stratification into simple versus complex (P < 0.05). Complex patients had increased morbidity compared to simple patients (sepsis 58 versus 18%; P = 0.021, NEC 42 versus 9%; P = 0.020, short bowel syndrome 58 versus 3%; P = 0.0001, ventilator days 24 versus 10; P = 0.021; TPN days 178 versus 38; P = 0.0001 and days to full feeds 171 versus 31; P = 0.0001; and length of stay 90 versus 39 days, P = 0.0001). Prenatal bowel wall dilation and/or thickness did not predict complex patients or adverse outcome. Complex gastroschisis patients can be identified postnatally and have substantial morbidity.

Outcomes in Neonatal Gastroschisis: An Institutional Experience

We describe outcomes of 70 infants with gastroschisis admitted to our neonatal intensive care unit between 2001 and 2005. Demographic data and outcome measures including discharge or death, simple versus complex (intestinal atresia, perforation, bowel necrosis, or volvulus), length of stay, mechanical ventilation and total parenteral nutrition, commencement of enteral feeds and age at reaching full feeds, and number of episodes of bacteremia were evaluated. In our study, 72% of the patients (n = 58) were simple cases, 28% were complex, and 44% had a positive blood culture. Median length of stay was 43 and 116 days for simple and complex cases, respectively. Median age at commencement of enteral feeds was 19 and 44 days for simple and complex cases, respectively. Six (14%) infants with simple and 8 (50%) with complex gastroschisis required an assisted feeding device (gastrostomy) tube before discharge. We concluded that the type of gastroschisis (simple versus complex) is an important determinant of outcome, including time to start and get to full feeds, duration of parenteral nutrition, length of stay, and survival. Although it is not always possible to determine whether the defect is simple or complex by antenatal scans, this information will be valuable to families with infants with gastroschisis and should be made available as soon as possible.

Intestinal transplantation for short bowel syndrome secondary to gastroschisis

Background/Purpose Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome. Methods A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994. Results Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years. Conclusions Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.

INTESTINAL TRANSPLANTATION FOR SHORT GUT SYNDROME SECONDARY TO GASTROSCHISIS

P605 Aims: Gastraschisis is the most frequent cause of pediatric intestinal transplant. The outcome for infants with gastroschisis has improved with current advantages in prenatal diagnosis, neonatal care and surgical techniques. However, a subset of these patients requires extensive bowel resections and in addition small portion of them develops long-term dependence on total parental nutrition (TPN) as a status of short-bowel syndrome. This study reviews our experience with intestinal transplantation for children who had sustained small intestinal loss as a result of gastroschisis in order to elucidate those factors affecting the eventual outcome of children with short-bowel syndrome secondary to gastroschisis. Methods: A retrospective chart review was performed for children who underwent small bowel transplantation for gastroschisis at the University of Miami between August 1994 and June 2003. Results: Thirty-two transplants were performed in twenty-eight children with gastroschisis during the study period. There were 14 boys and 14 girls with a median age of 23 months (range, 6 months to 8.5 years) at transplant. 14 patients (50%) were less than one year old at the time of transplant. Most patients were born prematurely and the average estimated gestation age was 34.5 weeks (range; 30 to 38 weeks). Mean birth weight was 2192 g. (range; 1235 g to 3012 g). Gastroschisis was prenatally diagnosed in 14 cases. Associated mid-gut anomalies were present in 22 infants (Bowel atresia was seen in 14 cases, Volvulus 3 cases, and/or Bowel ischemia 16 cases). Spontaneous prenatal closure of gastroschisis, which was considered to be a rare anomaly usually associated with bowel atresia and ischemia due to very small abdominal defect, was seen in 9 patients. In 3 patients, necrotizing enterocolitis (NEC) was developed after gastroschisis repair. Nineteen infants needed bowel resection within a week after birth. Most of the patients had complicated course and required multiple abdominal surgery before transplant. Transplantation types included isolated small intestine (n=8), liver and small intestine (n=8) and multivisceral transplants (n=16). Eighteen patients (64.3 %) currently are alive at a median follow-up of 15.7 months (range, 0.5 to 72 months). Short-term survival rate has significantly improved in recent years. Patient survival at 6 months, one year, and 2 years was: 50.0 %, 40.0 %, and 40.0% in the period between 1994 and 2000; 94.4 %, 77.9 %, and 77.9 % in 2001 through present. Conclusions: The outcome for most of the children with simple gastroschisis is quite good without being short bowel syndrome or intestinal failure. However, patients with complex gastroschisis, especially those with associated intestinal anomalies, likely to need extra surgeries in their early days of life and have a significant risk to become short bowel syndrome. Intestinal transplantation can be a life-saving option and provides a satisfactory outcome for children with gastroschisis associated short bowel syndrome who already developed serious TPN-related complications.

Ultrasound diagnosis of fetal anomalies associated with in utero cocaine exposure: further support for cocaine-induced vascular disruption teratogenesis.

The dramatic increase in cocaine use over the past decade has led to a concern about its possible teratogenicity. We have identified 6 structural fetal anomalies which we postulate may have cocaine-induced vascular accidents as the teratogenic mechanism: 2 complex choroid plexus cysts, gastroschisis, meconium peritonitis, urethral stenosis, and radial hypoplasia. Two additional anomalous neonates were born to perinatal cocaine users. Eight of 51 (15.7%) cocaine-exposed perinates exhibited anomalies versus 120 of 2,194 (5.4%) perinates without known cocaine exposure during this time period. This represents a 3-fold relative risk (RR = 2.87, OR = 3.22, chi square = 9.68, p < 0.005) for the cocaine-exposed fetus. Subjects were all identified as cocaine users prior to ultrasound-detected anomaly and ultrasonologists were blinded to maternal drug history. The vascular disruption model as the plausible mechanism for cocaine-associated teratogenesis is supported by the type of anomalies reported. In addition, cocaine use was prospectively determined to have occurred at the critical developmental period in each case.