Development and validation of a risk stratification index to predict death in gastroschisis

doi:10.1016/j.jpedsurg.2007.01.028

Journal of Pediatric Surgery

Volume 42, Issue 6, June 2007, Pages 950-956

https://doi.org/10.1016/j.jpedsurg.2007.01.028 Get rights and content

Abstract

Background

Gastroschisis is a rare congenital anomaly, the improved surgical management of which has contributed to a survival rate greater than 90%. Development of an accurate risk stratification system to help identify the subset of patients at greatest risk for death may lead to further improvements in outcome.

Methods

Infants with gastroschisis were identified from 16 years of the National Inpatient Sample database and the Kids' Inpatient Database using the International Classification of Diseases, Ninth Revision, Clinical Modification procedure code 54.71 (repair of gastroschisis) and an age of less than 8 days. Logistic regression analysis determined which coexisting diagnoses were significantly associated with death. Odds ratios from the logistic regression model were simplified and used as weighting factors to create an additive index. The index was validated using the 2003 Kids' Inpatient Database data set.

Results

Intestinal atresia, necrotizing enterocolitis, rare cardiac anomalies, and lung hypoplasia were strongly associated with death and used to create a scoring system with a potential range of 0 to 10. Every point increase on the scale of gastroschisis risk stratification index is associated with a 95% relative increase in the likelihood of death.

Conclusion

We have developed a novel index, which is superior to previous classification systems in identifying patients with gastroschisis who are at highest risk for death.

Section snippets

Methods

An exemption from institutional review board review was obtained from the Johns Hopkins Medicine Institutional Review Board.

Results

In total, we identified 4310 patients with an ICD-9-CM procedure code “repair of gastroschisis” and an age of admission of less than 8 days. Of these, 3330 were identified from 1988 through 2002 (creation data set), and 980 were identified from 2003 (validation data set). Overall inpatient mortality was 3.5% (149/4310).

Univariable and multivariate logistic regression were applied to the creation data set, and 4 covariates that are diagnosed at or soon after birth were identified as highly

Discussion

Risk stratification schemes in medicine are often developed by expert panels. These scaling systems, however, are often nonlinear and may not be validated when applied to large data sets of patients [26]. Developing prediction scales and risk stratification schemes by converting risk factors identified through logistic regression modeling may be a better approach and has already been applied to screening for child abuse and predicting the risk of death after carotid endarterectomy [27], [29],

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Cited by (39)

Assessment of gastroschisis risk factors in Egypt
2020, Journal of Pediatric Surgery
Mortality in infants born with gastroschisis (GS) in low-to-middle-income countries (LMICs) is high. This study aimed to assess factors which might affect outcome in Egypt in order to improve survival.
A prospective study over a 15-month duration was completed. Variables assessed covered patient, maternal, antenatal, treatment, and complications. The Gastroschisis Prognostic Score (GPS) was used to predict outcome. A validated questionnaire was used to assess socioeconomic status. The main outcome was mortality.
Twenty-four cases were studied. Median gestational age was 37 (26–40) weeks, and 9 (38%) were preterm. Mortality occurred in 15 (62%) infants. Median transfer time was 8 (1.5–35) hours, and 64% survived if transferred before 8 h. Median maternal age was 20 (16–27) years. All families were of a low or very-low socioeconomic level. Only 25% had antenatal scans. Most cases were simple GS, and only 3 (12.5%) were complex GS. Median length of stay was 14 (1–52) days, TPN duration was 12 (0–49) days, and days to full feeds was 5 (3–11) days. The GPS score ranged from 0 to 6 in the studied cases and negatively correlated with outcome (r_S = − 0.98; p = 0.03).
The mortality of GS in Egypt is very high, mainly due to sepsis and prematurity. Young maternal age and poor socioeconomic status are linked to GS. The GPS is a good indicator of morbidity and mortality in a LMIC setting. Survival improved with better resuscitation and strict management protocols. More effort is needed to improve antenatal detection, and transfer time should be ideally below 8 h.
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Newborn Abdominal Wall Defects
2020, Pediatric Gastrointestinal and Liver Disease, Sixth Edition
Newborn abdominal wall defects present significant challenges to the pediatric surgeon. This chapter discusses the two most common abdominal wall defects—gastroschisis and omphalocele. These two defects are distinct entities with important differences in epidemiology, etiology, and clinical presentation. The chapter highlights important anatomical considerations and associated anomalies in gastroschisis and omphalocele, as well as principles of prenatal, postnatal and surgical management. Outcomes of infants with gastroschisis and omphalocele are additionally discussed.
Predicting Morbidity and Mortality in Neonates Born With Gastroschisis
2020, Journal of Surgical Research
Gastroschisis is an increasingly common congenital abdominal wall defect. Due to advances in neonatal critical care and early surgical management, mortality from gastroschisis and associated complications has decreased to less than 10% in most series. However, it has been recognized that the outcome of gastroschisis has a spectrum and that the disorder affects a heterogeneous cohort of neonates. The goal of this study is to predict morbidity and mortality in neonates with gastroschisis using clinically relevant variables.
A multicenter, retrospective observational study of neonates born with gastroschisis was conducted. Neonatal characteristics and outcomes were collected and compared. Prediction of morbidity and mortality was performed using multivariate clinical models.
Five hundred and sixty-six neonates with gastroschisis were identified. Overall survival was 95%. Median hospital length of stay was 37 d. Sepsis was diagnosed in 107 neonates. Days on parenteral nutrition and mechanical ventilation were considerable with a median of 27 and 5 d, respectively. Complex gastroschisis (atresia, perforation, volvulus), preterm delivery (<37 wk), and very low birth weight (<1500 g) were associated with worse clinical outcomes including increased sepsis, short bowel syndrome, parenteral nutrition days, and length of stay. The composite metric of birth weight, Apgar score at 5 min, and complex gastroschisis was able to successfully predict mortality (area under the curve, 0.81).
Clinical variables can be used in gastroschisis to distinguish those who will survive from nonsurvivors. Although these findings need to be validated in other large multicenter data sets, this prognostic score may aid practitioners in the identification and management of at-risk neonates.
Risk stratification and outcome determinants in gastroschisis
2018, Seminars in Pediatric Surgery
Citation Excerpt :
Several risk or illness stratification systems have been applied to the gastroschisis population. Arnold et al, analyzed 4344 infants registered in the National Inpatient Sample Database and the Kid's Inpatient Database to identify GS infants at highest risk for death.47 The clinical variables of intestinal atresia, necrotizing enterocolitis, rare cardiac anomalies and lung hypoplasia were most associated with mortality and appeared to accurately segregate simple from complex GS patients.
Selection of outcome determinants and risk stratification are necessary to identify patients at higher risk for morbidity and mortality. This facilitates human and material resource allocation and allows for improved family counseling. While several different factors, including prenatal ultrasonographic bowel features, the timing and mode of delivery, and the features of bowel injury have been investigated in gastroschisis, there is still significant debate as to which of these best predicts outcome. This article reviews the different outcome predictors and risk prognostication schemata currently available in the literature to help guide clinicians caring for infants with gastroschisis.
Factors Associated with Timing and Adverse Outcomes in Patients with Biliary Atresia Undergoing Kasai Hepatoportoenterostomy
2018, Journal of Pediatrics
To assess factors associated with timing of hepatoportoenterostomy (HPE) and adverse perioperative outcomes in patients with biliary atresia in the US.
We examined hospitalizations in infants aged <1 year using the National Inpatient Sample database for 2000-2011. We identified cases using the International Classification of Diseases, Ninth Revision, Clinical Modification codes for biliary atresia and HPE. Multivariable logistic regression models were used to examine association between select factors and age at HPE, as well as adverse perioperative outcomes.
Our analysis of 1243 biliary atresia hospitalizations showed that only 37.7% of patients had HPE in the first 60 days of life. Patients who underwent HPE after 60 days of age were uninsured, were more likely to be black (aOR, 4.22; 95% CI, 1.49-11.95), less likely to be admitted at a teaching hospital (aOR, 0.27; 95% CI 0.10-0.79), and less likely to have a concomitant congenital malformation (aOR, 0.49; 95% CI 0.25-0.98). Patients with delayed age at HPE incurred significantly higher hospital costs ($57 914 vs $34 074; P = .026). Delayed age at HPE and weekend admission were independently associated with increased odds of adverse perioperative outcome (aOR, 1.09; 95% CI, 1.01-3.02 and 3.98; 95% CI, 1.67-9.46, respectively).
Current outcomes in patients with biliary atresia in the United States are suboptimal and result in higher costs. The specific factors associated with delayed care are further evidence that universal health care and screening are needed for all infants, along with systematic referral of potential patients with biliary atresia to specialized health centers.
Vanishing gastroschisis: Good outcome after a 10-year follow-up
2018, Journal of Pediatric Surgery Case Reports
Gastroschisis (GS) is a full-thickness abdominal wall defect with prolapse of the bowel and sporadically other viscera. The abdominal wall itself may close around the viscera determining entry/exit level intestinal atresia and ischemia or variable midgut infarction, a phenomenon described as vanishing GS (VGS). We report on an infant harboring VGS born to a 35-year-old Gravida 2, Para 0, affected with seizures controlled by anti-convulsive therapy. The infant is well after ten years of follow-up. Guidelines and directives have not been officially issued at international level yet. We suggest that close antenatal monitoring may prevent severe bowel loss in some cases pondering it as the best approach. To the best of our knowledge, after multiple surgical interventions, most patients with closed GS should have a favorable outcome.

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^☆: Dr Arnold is supported with a K12 grant (1K12 RR017627) from July 1, 2005, through June 30, 2007.

: Papers presented at the 58th Annual Meeting of the Section on Surgery of the American Academy of Pediatrics.

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AAP papersDevelopment and validation of a risk stratification index to predict death in gastroschisis☆,

Abstract

Background

Methods

Results

Conclusion

Section snippets

Methods

Results

Discussion

J Pediatr Surg

J Pediatr Surg

Obstet Gynecol

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Vasc Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Lancet

Gastroschisis with case report

Va Med Mon

AAP papers
Development and validation of a risk stratification index to predict death in gastroschisis☆,