Closing gastroschisis: The good, the bad, and the not-so ugly.

PURPOSE: The diagnosis of "closing" or "closed gastroschisis" is made when bowel is incarcerated within a closed or nearly closed ring of fascia, usually with associated bowel atresia. It has been described as having a high morbidity and mortality. METHODS: A retrospective review of closing gastroschisis cases (n = 53) at six children's hospitals between 2000 and 2016 was completed after IRB approval. RESULTS: A new classification system for this disease was developed to represent the spectrum of the disease: Type A (15%): ischemic bowel that is constricted at the ring but without atresia; Type B (51%): intestinal atresia with a mass of ischemic, but viable, external bowel (owing to constriction at the ring); Type C (26%): closing ring with nonviable external bowel +/- atresia; and Type D (8%): completely closed defect with either a nubbin of exposed tissue or no external bowel. Overall, 87% of infants survived, and long-term data are provided for each type. CONCLUSIONS: This new classification system better captures the spectrum of disease and describes the expected long-term results for counseling. Unless the external bowel in a closing gastroschisis is clearly necrotic, it should be reduced and evaluated later. Survival was found to be much better than previously reported. TYPE OF STUDY: Retrospective case series with no comparison group. LEVEL OF EVIDENCE: Level IV.

Outcome differences between simple and complex gastroschisis.

BACKGROUND: We reviewed differences of the outcome of newborn with simple and complex gastroschisis treated at our institution over the past fifteen years. METHODS: A retrospective cohort study was performed on all infants with gastroschisis treated at the Institute for mother and child health care, Belgrade, between 2001 and 2015 (n=70). Premature infants (<34 weeks of gestation) and babies with birth weight less than 1500 g were excluded (n=5). We compared outcomes in infants with simple gastroschisis and those with complex gastroschisis. An outcome analysis was performed for birth weight, gestational age, gender, mode of defect closure, presence of intestinal necrosis or perforation, reoperation, duration of mechanical ventilation and total parenteral nutrition, presence of bowel pseudoopstraction, sepsis, total duration of hospital stay, mortality rates. RESULTS: Of 65 patients, 15 (23,07%) had complex gastroschisis, including atresia 5 patients (33,3%), ischemic complication 9 patients (60%) and one patient with closing gastroschisis (6,66%). Sixty eight percent underwent primary closure.There was difference between the simple and the complex gastroschisis in duration of mechanical ventilation (P= ,000003), total parenteral nutrition (P= ,000019), bowel pseudoobstruction (P= ,00067), reoperation (P= ,00122), sepsis (P= ,0043), hospital stay (P= ,000198). In the simple gastroschisis group 92% of patients survived to discharge. In the complex gastroschisis group 3 patients died in hospital (P= ,338). CONCLUSIONS: More research should be focused to predict complex gastroschisis and to improve prenatal diagnosis and postnatal management, without a significant increase in morbidity and mortality. KEY WORDS: Complex, Gastroschisis, Outcomes, Simple, Vanishing.

Time trends in prevalence of gastroschisis in Texas, 1999 to 2011: Subgroup analyses by maternal and infant characteristics.

BACKGROUND: The prevalence of gastroschisis, a birth defect of the abdominal wall, has been increasing in several areas around the world. Suggested risk factors for gastroschisis include maternal age, race/ethnicity, nativity, body mass index, and socioeconomic status. METHODS: Data for cases of gastroschisis and live births were taken from the Texas Birth Defects Registry and Texas vital records for deliveries between 1999 and 2011. Prevalence by subgroups over time was calculated for: infant sex, maternal age, race/ethnicity, nativity, education, parity, plurality, body mass index, and payer type. Stratified, adjusted, and interaction analyses of the trends were conducted using Poisson regression. A joinpoint trend analysis was also conducted for each subgroup. RESULTS: A total of 2,549 gastroschisis cases and 4,970,979 live births were delivered in 1999 to 2011, for an overall prevalence of 5.13 cases per 10,000 live births (95% confidence interval = 4.93-5.33). On average, the prevalence increased 4.8% each year; this overall time trend was statistically significant (p-value < 0.0001). The time trend remained significant after adjusting for all variables, except payer type. The stratified analysis showed the increasing time trend was significant in many of the subgroups. However, there were no significant differences in the magnitude of the time trends between subgroups according to the interaction analysis. CONCLUSION: This study confirms the increasing prevalence of gastroschisis over the time period 1999 to 2011 in Texas. The results suggest that no population subgroups are experiencing a significantly different increase in gastroschisis prevalence over time than others.

Complex gastroschisis is a different entity to simple gastroschisis affecting morbidity and mortality-a systematic review and meta-analysis.

BACKGROUND: Comparison of the outcome of newborns with simple (sGS) and complex gastroschisis (cGS: gastroschisis with atresia, necrosis, perforation or volvulus). MATERIALS AND METHODS: We conducted a systematic database search, quality assessment and meta-analyzed relevant articles which evaluated the mortality and morbidity of newborns with cGS versus sGS. Risk ratios (RR) with 95% confidence interval (CI) were reported for categorical data, and the mean difference (MD) was calculated for continuous data. Pooled estimates of RR and MD were computed using generic inverse variance and a random-effects model. RESULTS: Of 19 identified reports, 13 eligible studies were included. The mortality of infants with cGS (16.67%) was significantly higher than sGS (2.18%, RR: 5.39 [2.42, 12.01], p<0.0001). Significantly different outcome was found for the following parameters: Infants with cGS are started on enteral feedings later and they take longer to full enteral feedings with a subsequent longer duration of parenteral nutrition. Their risk of sepsis, short bowel syndrome and necrotizing enterocolitis is higher. They stay longer in hospital and are more likely to be sent home with enteral tube feedings and parenteral nutrition. DISCUSSION: Occurring in 17% of infants born with gastroschisis, complex gastroschisis is associated with a significantly increased morbidity and mortality. More research should be focused on this special subgroup of patients, not only on postnatal management, but additionally directing efforts to improve diagnosing and predicting complex gastroschisis prenatally as well as implement any probable fetal intervention to alleviate its disastrous outcome.

Novel exomphalos genetic mouse model: the importance of accurate phenotypic classification.

BACKGROUND: Rodent models of abdominal wall defects (AWD) may provide insight into the pathophysiology of these conditions including gut dysfunction in gastroschisis, or pulmonary hypoplasia in exomphalos. Previously, a Scribble mutant mouse model (circletail) was reported to exhibit gastroschisis. We further characterise this AWD in Scribble knockout mice. METHOD: Homozygous Scrib knockout mice were obtained from heterozygote matings. Fetuses were collected at E17.5-18.5 with intact amniotic membranes. Three mutants and two control fetuses were imaged by in amnio micro-MRI. Remaining fetuses were dissected, photographed and gut length/weight measured. Ileal specimens were stained for interstitial cells of Cajal (ICC), imaged using confocal microscopy and ICC quantified. RESULTS: 127 fetuses were collected, 15 (12%) exhibited AWD. Microdissection revealed 3 mutants had characteristic exomphalos phenotype with membrane-covered gut/liver herniation into the umbilical cord. A further 12 exhibited extensive AWD, with eviscerated abdominal organs and thin covering membrane (intact or ruptured). Micro-MRI confirmed these phenotypes. Gut was shorter and heavier in AWD group compared to controls but morphology/number of ICC was not different. DISCUSSION: The Scribble knockout fetus exhibits exomphalos (intact and ruptured), in contrast to the original published phenotype of gastroschisis. Detailed dissection of fetuses is essential ensuring accurate phenotyping and result reporting.

A proposed classification for the spectrum of vanishing gastroschisis.

Infants born with gastroschisis in association with intestinal atresia are well described. We are the proposing the classification of vanishing gastroschisis. In this series of six cases, at one end of the spectrum is an infant having gastroschisis with a much narrower defect on the right side of umbilicus. The ischemic bowel loops were connected to bowel inside the abdomen by a fibrous band compressing the exposed bowel mesentery. On the other end of spectrum, an infant having extensive bowel atresia and complete closure of abdominal wall defect (gastroschisis) detected on antenatal ultrasound. These cases should raise awareness of this devastating complication in prenatal management of gastroschisis.

[Closing gastroschisis: a distinct entity with high morbidity and mortality]. / Closing Gastroschisis: eine Sonderform der Gastroschisis mit hoher Morbidität und Mortalität.

PURPOSE: We correlate severe bowel damage in gastroschisis to the rare intrauterine event of narrowing of the abdominal wall around the protruding intestines. We describe this "closing gastroschisis" as a distinct entity. Prenatal ultrasound findings as gastric or bowel dilation were compared to the postnatal findings in order to find markers for an early in utero diagnosis of closing gastroschisis. Early diagnosis could prompt timely delivery to save the compromised bowel and avoid short gut syndrome. MATERIALS AND METHODS: We documented the pre- and postnatal course of our patients with gastroschisis from 2007 to 2009.  Closing gastroschisis was suspected antenatally and confirmed postnatally. We identified 5 out of 18 patients showing closure of the abdominal wall with varying degrees of bowel damage. Prenatal ultrasound findings were correlated to the postnatally confirmed extent of intestinal damage. RESULTS: We could not find consistent ultrasound markers for prenatal diagnosis of closing gastroschisis. In prenatal ultrasound three patients presented significant gastric dilation and then experienced severe courses postnatally due to segmental gut necrosis. One of these three died and the other two developed short gut syndrome. In one case progressive intraabdominal loop dilation with simultaneous shrinking of the extraabdominal loops occurred corresponding to closing gastroschisis with segmental midgut necrosis. CONCLUSION: Closing gastroschisis must be seen as a special form of gastroschisis. Extended intestinal damage is often life-threatening. In longitudinal observation dynamics of fetal ultrasound findings can lead to the diagnosis of closing gastroschisis. Progressive intraabdominal loop dilation is always highly suspicious and must lead to close follow-up and timely delivery.

Risk stratification of 4344 patients with gastroschisis into simple and complex categories.

BACKGROUND: Gastroschisis is a congenital full-thickness abdominal wall defect characterized by the protrusion of intraabdominal organs outside the abdominal domain that requires surgical management in the early neonatal period. The goal of this study was to validate a previous risk stratification classification of infants born with this defect. METHODS: A retrospective analysis of a nonoverlapping combination of the databases National Inpatient Sample and Kids' Inpatient Database (1988-2003) was performed. These combined databases contain information from nearly 93 million discharges in the United States. Infants with gastroschisis were identified by an International Classification of Diseases, Ninth Revision procedure code of 54.71 (repair of gastroschisis) and an age at admission of less than 8 days. Infants were divided into simple and complex categories based on the absence or presence of intestinal atresia, stenosis, perforation, necrosis, or volvulus. Variables of sex, race, geographic region, coexisting diagnoses, hospital type and charges adjusted to 2005 dollars, length of stay, inpatient mortality, and complications were collected. Comparison between the 2 groups was performed using Pearson chi2 for categorical outcomes and the Kruskal-Wallis test for non-normally distributed continuous variables. RESULTS: A total of 4344 infants with gastroschisis were identified and divided into simple and complex categories. Simple gastroschisis represented 89.1% (n = 3870) of the group, whereas 10.9% (n = 474) had complex disease. Simple and complex patients differed in coexisting cardiac disease (8.3% vs 11.8%, P = .01), hospital type (78.7% vs 84.1% treated at urban teaching centers, P < .01), median length of stay (28 vs 67 days, P < .01), median inflation-adjusted hospital charges ($90,788 vs $197,871; P < .01), and inpatient mortality (2.9% vs 8.7%, P < .01). Gastrointestinal (14.4% vs 83.5%, P < .01), respiratory (2.6% vs 4.6%, P = .01), and infectious disease complications (24.3% vs 45.4%, P < .01) also differed between the groups. CONCLUSIONS: These data use the largest data set to date to validate the risk stratification of infants with gastroschisis. This analysis improves the characterization and understanding of clinical subsets of infants in whom this congenital condition is diagnosed.

Left-sided gastroschisis.

Gastroschisis is a periumbilical, abdominal wall defect arising to the right of the umbilicus. We describe the clinical course of a left-sided gastroschisis in a term female born at 39 weeks' gestation. To our knowledge, there have been only 14 left-sided abdominal wall defects reported in the literature. We discuss our case and review the literature to try to determine if there is any clinic difference between right- versus left-sided lesions to make recommendations as to management.

Ascertainment of gastroschisis using the ICD-9-CM surgical procedure code.

OBJECTIVE: To determine the effectiveness of using the ICD-9-CM procedure code 54.71 for case ascertainment of gastroschisis. METHODS: Using procedure code 54.71, we queried a statewide hospital discharge database to identify all cases coded as undergoing surgical repair of gastroschisis. Each retrieved case was verified as having gastroschisis by review of the hospital record. All gastroschisis cases were then matched to the Florida Birth Defect Registry (FBDR) dataset. This registry uses a passive system of multiple data sources and employs the ICD-9-CM diagnostic code 756.79 to identify gastroschisis and other abdominal wall defects. RESULTS: Of 93 cases identified by using code 54.71, 92 were confirmed by record review to have gastroschisis. The FBDR identified 87 of the 92 cases (95%). The FBDR missed three of the remaining five cases because of linkage difficulties between inconsistent data elements in the respective data files. The other two cases were not identified by the FBDR because the source database (AHCA discharge) truncates the entry of ICD-9-CM diagnostic codes when more than 10 of them are listed in the medical record. CONCLUSIONS: Use of the surgical procedure code was demonstrated to be superior to the diagnostic code as a method for identification of gastroschisis cases. The same approach may be useful in the detection of malformations other than gastroschisis.

Abdominal wall defects in Denmark, 1970-89.

In the last two to three decades, increasing rates of gastroschisis but not of omphalocele have been reported from different parts of the world. The present study represents a register containing 469 children born with abdominal wall defects based on data retrieved from 20 birth cohorts (1970-89) in three nationwide registries. A tentative estimate of the completeness as regards identification of liveborn and stillborn infants is a minimum of 95% and 90% respectively. All cases were reclassified to 166 cases of gastroschisis, 258 of omphalocele and 16 of gross abdominal wall defect. The average point prevalence at birth of gastroschisis was 1.33 per 10 000 live and stillbirths. During the first decade, an increase in prevalence occurred culminating in 1976, followed by a decrease reaching its initial value in 1983 and then a new increase. Overall, no significant linear trend could be demonstrated for the entire period. The average point prevalence at birth for omphalocele was 2.07 and for gross abdominal wall defect 0.12 per 10 000 live and stillbirths with no significant change in the period. The geographical distribution of gastroschisis and omphalocele showed no difference per county.

[Diagnostic prenatal ultrasonography of malformations of the fetal anterior abdominal wall]. / Diagnostic échographique anténatal des malformations de la paroi anterieure du foetus.

Abdominal wall defects include a broad spectrum of structural malformations with variable severity and prognosis. The purpose of prenatal ultrasound examination is to correctly diagnose and classify these malformations according to their localization (particularly their relation to the umbilical cord insertion), their contents, their size and associated malformations or karyotypic abnormalities. Based on this examination, two groups can be distinguished: gastroschisis or omphalocele (when the latter is isolated, in particular without karyotypic abnormalities) which can be surgically corrected at birth, and for which predictive criteria of outcome must be evaluated (vitality of herniated bowel, size and contents of omphalocele); severe malformations (ectopia cordis, cloacal exstrophy, Beckwith-Wiedemann syndrome, short umbilical cord, amniotic band syndrome) for which pregnancy termination could be proposed.