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.

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.

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.