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Impact of societal factors and health care delivery systems on gastroschisis outcomes
Accepted Manuscript
Impact of Societal Factors and Health Care Delivery Systems on Gastroschisis Outcomes Jordan S. Taylor MD , Stephen B. Shew MD, FACS, FAAP PII: DOI: Reference:
S1055-8586(18)30071-4 https://doi.org/10.1053/j.sempedsurg.2018.08.010 YSPSU 50772
To appear in:
Seminars in Pediatric Surgery
Please cite this article as: Jordan S. Taylor MD , Stephen B. Shew MD, FACS, FAAP , Impact of Societal Factors and Health Care Delivery Systems on Gastroschisis Outcomes, Seminars in Pediatric Surgery (2018), doi: https://doi.org/10.1053/j.sempedsurg.2018.08.010
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Impact of Societal Factors and Health Care Delivery Systems on Gastroschisis Outcomes Jordan S. Taylor, MD, Stephen B. Shew, MD, FACS, FAAP Department of Surgery, Division of Pediatric Surgery, Lucile Packard Children’s Hospital,
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Stanford University School of Medicine, Stanford, CA, USA
Corresponding author:
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Stephen B. Shew, MD Division of Pediatric Surgery, Stanford University School of Medicine 300 Pasteur Drive Always Building M116 Stanford, CA 94305 USA E-mail address: [email protected] Conflicts of interest: none
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Funding: none
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ABSTRACT Care of infants with gastroschisis is associated with a significant burden on health care delivery systems. Mortality rates in patients with gastroschisis have significantly improved over the past few decades. However, the condition is still associated with significant short-term and
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potentially long-term morbidity. Significant variations in clinical outcomes and resource
utilization may be explained by several factors including provider and hospital experience, level of neonatal intensive care, variations in hospital regionalization of care, and differences in
healthcare delivery systems. Reviewing and assessing these hospital and healthcare system
outcomes for these vulnerable infants.
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related factors are paramount in addressing variations in gastroschisis care and improving
Keywords: Gastroschisis, morbidity, cost, health services research, neonatal intensive
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care
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Introduction Gastroschisis is a birth defect resulting from a failure of normal abdominal wall closure early in gestation. By the time of birth, the majority of the intestines are eviscerated through the abdominal wall defect and the bowel wall has varying degrees of inflammatory thickening. The
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recovery of intestinal function is slower than normal postoperatively, necessitating adequate parenteral nutrition while awaiting the return of bowel function. Intestinal complications such as atresia, necrosis, or perforation may occur. Complicated gastroschisis often leads to multiple additional operations, further delaying hospital discharge and possibly precipitating the need for
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home parenteral nutrition. Therefore, despite the relatively low prevalence (2.32 - 4.49 per
10,000 live births in the United States or approximately 1,850 babies annually), gastroschisis places a significant burden on hospitals and healthcare systems.1-3 It requires multidisciplinary
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subspecialized healthcare from the time of prenatal diagnosis through discharge and can require continued medical care months to years after surgical correction.4, 5
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Morbidity and mortality associated with gastroschisis have drastically improved since the 1970s with the rise in number and capabilities of neonatal intensive care units (NICUs), along
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with refined surgical techniques for closure. Survival has improved to more than 97% in a recent
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large, multi-institutional, North American study.6 As such, comparative outcomes for gastroschisis in high-income countries have now focused more on related morbidity and resource
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utilization (e.g. length of hospital stay). The in-hospital and long-term outcomes are highly variable for these infants, with 10-20% requiring second operations, 7% requiring gastrostomy tubes, at least 20% with cholestasis or gastroesophageal reflux, and up to a third requiring nutritional support at the time of discharge.6-8 Compared to other birth defects, infants with gastroschisis have a longer average hospital length of stay (mean 41 days). In the United States,
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cost of hospital stay is also highly variable, with a mean of $155,629 per patient, and a total of approximately $348 million in 2017 dollars.9, 10 As healthcare systems evolve, greater emphasis is directed toward delivering improved quality medical care with lower and consistent cost (i.e., high value). Best practices mandate
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continual review of outcomes data as well as adaptation of evidence-based medicine to improve care. Fortunately, large, multi-institutional and even multi-national databases can provide
perspective on rare conditions like gastroschisis. In this paper, we examine the effects of hospital and healthcare system factors, such as hospital and NICU experience, regional factors, and
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insurance and societal influences on gastroschisis outcomes (Table 1).
Societal Factors
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Gastroschisis prevalence has been increasing in the US and globally over the last several decades.11 The prevalence within the US was previously reported to be as low as 2.32 per 10,000
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live births, but has increased nearly 30% since the late 1990s.1, 3 The increased burden of disease has not been shared equally. The increase in prevalence has largely effected mothers under 25
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period.3, 12
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years of age, despite a significant (43%) drop in the rate of teen pregnancies over a similar time
Maternal birth place (nativity) has more recently been shown to affect gastroschisis
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outcomes. Foreign-born women have a lower likelihood or risk of delivering infants with gastroschisis compared to US-born women (1.47 vs 4.06 in 10,000 births; OR 0.3-0.5, 95% CI 0.2-0.6).11, 13 Within the US, differences in risk of gastroschisis exist based on rural or urban residence. A California study showed that women living in more rural counties were at an increased risk of having newborns with gastroschisis (OR 1.2-2.2, 95% CI 1.0-3.9).13 Moreover,
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Dubrovsky et al. found regional variations that affected treatment of patients with gastroschisis. Patients born in the South and Northeast US were more likely to be treated at low-volume centers, while those in the West and Midwest were likely to be treated at high volume centers.14 Hospital charges by region also vary widely; the Midwest charges were more than $50,000
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above the national median, whereas the West had charges $65,000 less than the median.15 Maternal factors have been extensively studied for their effect on gastroschisis
prevalence and outcomes. Younger women, particularly those < 20 years of age are at higher risk for fetal gastroschisis (11.45 per 10,000 live births compared to 5.35 per 10,000 live births in 20-
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24 year olds).1 Lower body mass index (< 18.1kg/m2), cigarette smoking or alcohol consumption during pregnancy, and lower socioeconomic status have all been found to put women at increased risk of having babies with gastroschisis in some studies.16
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Race and ethnicity have been reported in numerous studies to have an effect on the prevalence, outcomes, and costs associated with gastroschisis.15 When comparing age-matched
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mothers < 20 years of age, there are differences in prevalence among mothers of different races: 18.1, 16.1, 10.2 per 10,000 live births in white, Hispanic, and black mothers, respectively.3 The
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recent increase in prevalence, though, has affected young (< 20 years old) black mothers more
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than other races, and black neonates are at an increased risk for gastroschisis complicated by atresia (OR 1.74, 95% CI 1.07-2.843).3, 15 The correlations in race-ethnicity to gastroschisis
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prevalence and outcomes are less clear when examining older age groups.16 Hospital charges for gastroschisis were found to be higher in black (additional $50,495) and Hispanic mothers, compared to white mothers.15 Low income and government insurance was found to be a risk for mortality, but associated with lower hospital charges.15 Based on the average income by zip code, infants born to mothers in the first and second income quartiles had an increased odds of
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mortality compared to the fourth quartile (OR 2.52, 95% CI 1.03-6.16; OR 1.36, 95% CI 1.368.23, respectively).15 Hospital charges, however, for those in the lowest quartile for income were more than $50,000 less than those in the highest quartile for income.15
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Hospital Volumes
For many surgical diseases, particularly complex ones, outcomes are reportedly better for patients treated at high volume centers.14, 17 Several studies have examined whether this
relationship is true for gastroschisis. In a California review of 1,500 infants at 55 facilities,
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patients treated at centers with higher volume of gastroschisis repair annually (>9 cases per year) had decreased odds of mortality (OR 0.40, 95% CI 0.21-0.76) compared to those treated at hospitals with fewer than five cases annually.17 Early management and intervention was thought
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to contribute to the observed difference in outcomes. The California data showed shorter hospital stays when patients were treated at high volume centers (43.3 days vs 49 days), though this did
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not maintain statistical significance when accounting for other variables.17 A recently published review of national data, however, does not support the trends seen
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in California. Dubrovsky et al. in 2017 examined the KID from 2003, 2006, 2009, and 2012, and
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found that hospital volume did not have a significant relationship with outcomes in patients with gastroschisis. The study included more than 7,500 patients treated at over 740 hospitals across
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the United States. The authors did not find a significant difference in mortality, but did find that patients had an increased odds for prolonged length of stay (OR 1.59, 95% CI 1.12-2.24) when treated at high-volume centers (>14 cases annually).14 But due to the lack of longitudinal tracking of individual patients in the KID dataset, the limitations of the study include the possibility of lower-level hospitals reporting earlier discharges for patients who were actually
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transferred to other hospitals. Higher volume hospitals were found to have significantly more transfers than lower volume centers (60% vs 12%), which could potentially prolong length of stay in those patients.14 The Canadian Pediatric Surgery Network (CAPSNet) gastroschisis database was analyzed by Baird et al., who found that case volume among the tertiary centers did
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not have an effect on mortality (94.3% vs 97.2% survival, p = 0.17 for low vs high volume centers).18 In their analysis of the more than 400 cases over the 4-year study period, they did not stratify patients by complexity of disease, which may have influenced their findings. When a separate group investigated CAPSNet data for uncomplicated gastroschisis from 2005-2013, they
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found that the best outcomes were realized at medium volume hospitals, defined as 3-8 gastroschisis cases annually.8 However, the outcome advantage was quite modest.
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NICU Designations
While hospital volume for any given condition is often used to judge experience and
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competency in surgical diseases, gastroschisis is a condition primarily managed by pediatric surgeons and neonatologists within NICUs. The American Academy of Pediatrics (AAP) 2004
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(and more recently 2012) policy statement defines the NICU levels of care based on functional
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capabilities, resources, and outcomes.19, 20 NICU level IIIC is the designation for the highest level of neonatal care. One retrospective study examined the effects of NICU designation on
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gastroschisis outcomes. In a review of the California Perinatal Quality Care Collaborative (CPQCC) gastroschisis data from 2008 to 2014, Apfeld et. al examined more than 1,500 records of patients with gastroschisis treated at 129 NICUs. The overall mortality rate was 2.5%, with a median 5 days on ventilator, and a median length of stay of 36 days.21 The majority of patients were born outside level IIIC facilities, with 38.2% born at level IIIB, 10.2% born at IIIA, and
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13% born at level IIA/B centers. When adjusting for confounding factors, the odds of death were higher for patients born at a level IIA/B NICU (OR 6.66; p = 0.004), a IIIA NICU (OR 5.95; p = 0.008), or a level IIIB NICU (OR 5.85; p = 0.002) compared to the highest level, IIIC NICUs.21 Infants born at lower level NICUs also had significantly more days on ventilator (median number
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of days on ventilator at level IIA/B was 9 days) and prolonged length of stay (median 43 days at level IIA/B).21
Apfeld et al. also discuss the likely importance of initial management of patients with gastroschisis. The majority of patients born outside level IIIC NICUs (588/1063 or 55.3%) were
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transferred to a higher level of care within the first 48 hours of life. Despite the rapid transfers, the differences in outcomes (mortality, LOS, days on ventilator) persist based on the hospital and NICU of birth. The CPQCC database also supports the concept that case-specific surgical
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volume improves outcomes. Apfeld et al. found increased mortality risk at centers with the
quartile volume centers.21
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Centralization of Care
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lowest quartile volumes of gastroschisis repair (OR 3.1, 95% CI 1.13-8.49) compared to highest
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The finding of better outcomes at high volume centers has been used as an argument for centralizing or regionalizing care.19 Specifically for gastroschisis, there is evidence to suggest
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that higher volume centers have better outcomes, including decreased mortality.17 There are numerous barriers to centralizing or regionalizing care that need to be examined. Centralizing care requires extensive coordination and consensus in management of gastroschisis and agreed upon criteria for designating centers in a responsible way. Centralizing care for gastroschisis
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could mean delivery at designated centers and/or transferring to designated centers, with the goal of improved outcomes and lower costs. There are very few relevant studies that directly compare centralized vs decentralized systems with respect to their impact on gastroschisis outcomes. One recent study utilized the
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CAPSNet and KID databases to compare gastroschisis outcomes in Canada with outcomes from the US.22 The authors used Canada’s national healthcare system and 16 regional centers as a model for centralized care, in contrast to the decentralized system in the US. They found that mortality for simple gastroschisis was higher in the US compared to Canada (3.4% vs 1.4%, p =
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0.008), though length of stay was shorter in the US (41 vs 45 days, p = 0.004). No difference in the length of stay or mortality was found for patients with complex gastroschisis.22 The US also had significantly higher rates of prematurity and low birth weight (both of which are risk factors
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for worse outcomes), and a Cesarean section deliver rate twice as high as Canada’s.23-25 Notwithstanding the observed differences, the study did not offer strong evidence for
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centralization. Despite Canada’s designation of a limited number of regional pediatric surgical centers, there is still wide variation in the management of gastroschisis at these centers.18
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Furthermore, while the US does not have formal criteria for ‘designated’ gastroschisis centers,
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there is a de facto regionalization, with high rates of transfer to academic centers, level III NICUs, or high-volume centers.22
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Delivery at designated centers Multiple retrospective studies that have tried to examine the effect of birth hospital on
gastroschisis outcomes have produced conflicting results. 26, 27 One large, multi-institutional study utilized the Pediatric Surgery Research Collaborative (PedSRC) from 2008-2013 to compare the outcomes of “inborn” (those born and treated in the same facility) vs “outborn”
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patients with gastroschisis (those who required transfer to separate facility for definitive management).26 Though patients had similar baseline characteristics, inborn infants began enteral feedings earlier and achieved full feeds significantly sooner (27 days vs 31 days, p = 0.0008). Inborn patients were also found to have earlier definitive closure (3.7 days vs 5.4 days,
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p<0.0001).26 On bivariate analysis, inborn patients had significantly shorter length of stay (34 days vs 42 days, p = 0.002), though this fell out of statistical significance on multivariate
analysis. The study argued for patients diagnosed prenatally to be followed and ultimately delivered at a center that has the capabilities to definitively manage gastroschisis.
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The CAPSNet database was also reviewed to examine the same question, whether birth hospital had an effect on gastroschisis outcomes.27 From 2005-2008, there were nearly 400 patients with gastroschisis, with 40.5% of patients being outborn. No difference was appreciated
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between the inborn and outborn cohorts with respect to days of TPN, days until first enteral feed, or length of stay. However on multivariate analysis, outborn status was determined to be an
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independent predictor of complications [OR 1.6 (95% CI 1.09-2.7)], including sepsis, wound dehiscence, and wound infection.27 Many of the level IIIC NICU’s are housed in free-standing
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children’s hospitals without maternity or obstetrics services. Inborn care is not possible in such
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centers.
Transfer to designated centers
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Newborns with gastroschisis are often transferred to other qualified centers even when the birth hospital has potentially adequate surgical and NICU capabilities. Sacks et al. found that more than half of the infants (59.7%) treated at high-volume centers were transferred from midor low-volume centers. In their cohort, transferring patients prior to gastroschisis closure was not associated with increased mortality.17 A recent North American study had similar findings:
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25.7% of their cohort was transferred to another hospital shortly after birth, and transfer status did not significantly affect length of stay or mortality.6 They hypothesized that the benefits of being transferred to higher volume centers outweigh the risks associated with transport. Patients
volume centers with presumed management capabilities.17
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transferred to high-volume centers had improved outcomes compared to those treated at lower
Benefit of transfer to high-volume centers is evident in two different data sources
analyzing nearly all California hospitals.17, 21 However, this finding is not easily apparent when analyzing national data sources that have limited longitudinal tracking of patients’ transfers
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between hospitals.6,14 The Canadian data found an increased risk of necrotizing enterocolitis in gastroschisis patients who were outborn and subsequently transferred to designated centers.8,27 Given these data, it is entirely reasonable, and certainly preferable from a patient and family-
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centered perspective, to deliver babies with a prenatal diagnosis of gastroschisis in tertiary mother-child centers whenever possible. This avoids transfer immediately after birth and
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provides care in an environment with adequate expertise. I Fiscal impact of decentralized gastroschisis care
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As it stands in the US, the care of patients with gastroschisis has a significant financial
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impact on families and hospital systems, with median hospital charges per case ranging from $111,500 to $250,000.9, 15, 28-32 In a review of the 30 most costly pediatric surgical procedures
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performed at 45 hospitals, gastroschisis closure and care had the highest cost variation at the hospital level.30 Despite its relatively low prevalence compared to more common procedures, gastroschisis made up nearly 10% or $34 million of the cumulative cost variation burden in the US from 2014-2015. Cost variation burden is defined as the absolute difference between the median and the individual cost. The review put the median hospital charge at $111,500, but the
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interquartile range was over $48,000.30 Song et al. found the interquartile range to be even higher, with interquartile charges between $139,717 and $384,184.15 The remarkable variability in cost underscores the need for more comparative effectiveness research in gastroschisis outcomes, highlights the lack of consensus in management practices, and may further the
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argument for regionalizing care and/or implementation of collaborative care plans across institutions. Barriers to regionalizing care
While centralizing care to specific regional centers may be potentially beneficial for
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patients and hospital systems, there is insufficient evidence that the practice can significantly improve gastroschisis outcomes. The majority of the evidence regarding healthcare delivery systems and gastroschisis comes from single center retrospective reviews or pooled
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administrative data from various networks. Single center reviews for gastroschisis are often limited by small numbers, or long study periods, during which standards of care often change.33
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The administrative data from healthcare networks often lacks the clinical depth that is needed to make accurate correlations. Furthermore, there is limited evidence that these networks are
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driving improvement, particularly in neonatal surgical diseases.18, 33
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There are other practical barriers to implementing a centralized system of care, at least in the US. There is a lack of widely accepted consensus guidelines for management of
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uncomplicated and complicated gastroschisis, including prenatal and delivery management, initial management strategies, timing of closure, routine use of silos, and feeding practices. There are geographic limitations for the large US population and area. Currently, the majority of patients are treated at lower to medium volume centers.14,17 There are only four centers in California, with a population of approximately 39 million, that are considered high-volume.17
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Furthermore, there is no accepted standard for designating centers as excellent or competent for gastroschisis management. Annual hospital volumes, NICU levels, mortality and morbidity outcomes could be used as surrogates for evaluating centers, though no gastroschisis-specific
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metric exists currently.
Conclusions
While gastroschisis mortality has improved drastically, there are considerable variations in morbidity outcomes and resource utilization based on a multitude of factors. Patient and
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maternal factors have been extensively studied, but little is known about the true effects of
healthcare delivery systems on gastroschisis outcomes. Improving outcomes, particularly for uncommon neonatal surgical disease like gastroschisis will likely require broad-scale adoption of
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evidence-based best practices and implementation of quality improvement programs across multi-institutional networks. There is a reliance on shared, multi-institutional data because of the
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low prevalence of gastroschisis, but the clinical variations in presentation and management are difficult to account for in these large administrative datasets, making identification of best
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practices difficult.
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While not without exception, the majority of the evidence points to an improvement in gastroschisis outcomes when infants are born at, or treated at, higher volume centers and higher
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level NICUs. Regionalizing care may improve outcomes, but there is little definitive proof that official designations are needed or feasible within the US. Potentially independent of strict regionalization, more comparative effectiveness and health services research needs to be done to establish best practice patterns that may allow dissemination and harmonization of gastroschisis care to improve value for all stakeholders.
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Table 1. Gastroschisis outcomes related to healthcare delivery.
Study Author Location
Data source, Quality (A,C, QI)
Years, gastroschisis patients (n)
Study focus
Impact on gastroschisis outcomes from healthcare system variables and comments 15
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CPQCC 129 NICUs A
2008 – 2014 n = 1588
AAP NICU level at birth hospital
Sacks et al. California
OSHPD 55 hospitals A
1999 – 2007 n = 1537
Hospital gastroschisis closure volumes
Fullerton et al. North America
VON 175 hospitals C, QI
2009 – 2014 n = 4420
Predictive factors, patient outcomes
Dubrovsky et al. United States
KID 743 hospitals A
2003, 06, 09, 12 n = 7769
Hospital gastroschisis closure volumes
Youssef et al. Canada
CAPSNet 16 regional centers A, QI
2005 – 2013 n = 700
Predictive factors, hospital volumes
Cameron et al. United States
PHIS 45 Tertiary children’s hospitals A
2014 – 2015 n = 784
CER, interhospital cost variation
Gonzalez et al. United States
PHIS 45 Tertiary children’s hospitals A
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Predictive factors, patient outcomes
CAPSNet/KID 16 regional centers/ 743 hospitals A, QI
2005 – 2013/ 2003, 06, 09, 12 n = 695 / 5216
Centralized vs noncentralized
Allman et al. United States
Pediatrix 351 NICUs C, A
1997 – 2015 n = 6023
Baird et al.
CAPSNet 16
2005 – 2009 n =
Trends in prevalence, demographic risk factors Variation in
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2005 – 2013 n = 4459
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Youssef et al. Canada/United States
↑ odds of mortality, ↑ LOS, ↑ ventilator at lower level NICU ↓ odds of mortality, ↓ LOS (NS) when closure performed at high volume centers (> = 9 cases annually) Limited data regarding healthcare system impact; mode of delivery did not affect mortality, LOS High-volume hospitals receive more transfers, ↑ odds of prolonged LOS, no effect on mortality Volume had no effect on mortality, Moderate-volume centers had ↓ LOS and fewer days on TPN Median hospital charge $114,230, widest range in interhospital cost variation in pediatric surgery Government insurance associated with ↑ LOS, ↑ days on TPN; hospital volume/characteristics have no effect on LOS, days on TPN Mortality rate for simple gastroschisis ↓ in Canada (1.4% vs 3.4%, p < 0.05), NS for complex gastroschisis. ↑ odds of low birth weight, ↓ LOS in US ↓ prevalence from peak in 2008; young maternal age confers ↑ risk NS difference
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Apfeld et al. California
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regional centers A, QI
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delivery practices across regional centers
Lusk et al. California
UCfC 5 University hospitals C
20107 – 2012 n = 191
Institutional variations, closure
Overton et al. United Kingdom
UKOSS; BAPSCASS; BINOCAR 226 maternity units; 28 pediatric surgical units; A, QI PedSRC 6 centers C
2006 – 2007 n = 303
Maternal factors, predictive factors, antenatal management
2008 – 2013 n = 528
Inborn vs outborn
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Inborn patients reach full enteral feeds sooner, ↓ LOS Abbreviations: A (administrative data); C (clinical data); QI (Quality Improvement data); CPQCC (California Perinatal Quality Care Collaborative); AAP (American Academy of Pediatrics); NICU (Neonatal Intensive Care Unit); LOS (length of stay); OSHPD (California Office of Statewide Health Planning and Development); NS (Not Significant, p > 0.05); VON (Vermont Oxford Network); KID (Kids’ Inpatient Database); CAPSNet (Canadian Pediatric Surgery Network); TPN (total parenteral nutrition); PHIS (Pediatric Health Information System); CER (comparative effectiveness research); UCfC (University of California Fetal Consortium); UKOS (UK Obstetric Surveillance System); BAPS-CASS (British Association of Paediatric Surgeons Congenital Anomalies Surveillance System); BINOCAR (British Isles Network of Congenital Anomalies Registers); PedSRC (Pediatric Surgery Research Collaborative)
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Savoie et al. United States
between cesarean and vaginal deliveries on mortality, LOS, days on TPN Wide variation in management, NS difference in measured outcomes (mortality, LOS, ventilator days) Wide variation in antenatal management without clear benefit
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Canada
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Impact of Societal Factors and Health Care Delivery Systems on Gastroschisis Outcomes Jordan S. Taylor MD , Stephen B. Shew MD, FACS, FAAP PII: DOI: Reference:
S1055-8586(18)30071-4 https://doi.org/10.1053/j.sempedsurg.2018.08.010 YSPSU 50772
To appear in:
Seminars in Pediatric Surgery
Please cite this article as: Jordan S. Taylor MD , Stephen B. Shew MD, FACS, FAAP , Impact of Societal Factors and Health Care Delivery Systems on Gastroschisis Outcomes, Seminars in Pediatric Surgery (2018), doi: https://doi.org/10.1053/j.sempedsurg.2018.08.010
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Impact of Societal Factors and Health Care Delivery Systems on Gastroschisis Outcomes Jordan S. Taylor, MD, Stephen B. Shew, MD, FACS, FAAP Department of Surgery, Division of Pediatric Surgery, Lucile Packard Children’s Hospital,
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Stanford University School of Medicine, Stanford, CA, USA
Corresponding author:
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Stephen B. Shew, MD Division of Pediatric Surgery, Stanford University School of Medicine 300 Pasteur Drive Always Building M116 Stanford, CA 94305 USA E-mail address: [email protected] Conflicts of interest: none
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Funding: none
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ABSTRACT Care of infants with gastroschisis is associated with a significant burden on health care delivery systems. Mortality rates in patients with gastroschisis have significantly improved over the past few decades. However, the condition is still associated with significant short-term and
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potentially long-term morbidity. Significant variations in clinical outcomes and resource
utilization may be explained by several factors including provider and hospital experience, level of neonatal intensive care, variations in hospital regionalization of care, and differences in
healthcare delivery systems. Reviewing and assessing these hospital and healthcare system
outcomes for these vulnerable infants.
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related factors are paramount in addressing variations in gastroschisis care and improving
Keywords: Gastroschisis, morbidity, cost, health services research, neonatal intensive
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care
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Introduction Gastroschisis is a birth defect resulting from a failure of normal abdominal wall closure early in gestation. By the time of birth, the majority of the intestines are eviscerated through the abdominal wall defect and the bowel wall has varying degrees of inflammatory thickening. The
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recovery of intestinal function is slower than normal postoperatively, necessitating adequate parenteral nutrition while awaiting the return of bowel function. Intestinal complications such as atresia, necrosis, or perforation may occur. Complicated gastroschisis often leads to multiple additional operations, further delaying hospital discharge and possibly precipitating the need for
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home parenteral nutrition. Therefore, despite the relatively low prevalence (2.32 - 4.49 per
10,000 live births in the United States or approximately 1,850 babies annually), gastroschisis places a significant burden on hospitals and healthcare systems.1-3 It requires multidisciplinary
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subspecialized healthcare from the time of prenatal diagnosis through discharge and can require continued medical care months to years after surgical correction.4, 5
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Morbidity and mortality associated with gastroschisis have drastically improved since the 1970s with the rise in number and capabilities of neonatal intensive care units (NICUs), along
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with refined surgical techniques for closure. Survival has improved to more than 97% in a recent
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large, multi-institutional, North American study.6 As such, comparative outcomes for gastroschisis in high-income countries have now focused more on related morbidity and resource
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utilization (e.g. length of hospital stay). The in-hospital and long-term outcomes are highly variable for these infants, with 10-20% requiring second operations, 7% requiring gastrostomy tubes, at least 20% with cholestasis or gastroesophageal reflux, and up to a third requiring nutritional support at the time of discharge.6-8 Compared to other birth defects, infants with gastroschisis have a longer average hospital length of stay (mean 41 days). In the United States,
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cost of hospital stay is also highly variable, with a mean of $155,629 per patient, and a total of approximately $348 million in 2017 dollars.9, 10 As healthcare systems evolve, greater emphasis is directed toward delivering improved quality medical care with lower and consistent cost (i.e., high value). Best practices mandate
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continual review of outcomes data as well as adaptation of evidence-based medicine to improve care. Fortunately, large, multi-institutional and even multi-national databases can provide
perspective on rare conditions like gastroschisis. In this paper, we examine the effects of hospital and healthcare system factors, such as hospital and NICU experience, regional factors, and
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insurance and societal influences on gastroschisis outcomes (Table 1).
Societal Factors
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Gastroschisis prevalence has been increasing in the US and globally over the last several decades.11 The prevalence within the US was previously reported to be as low as 2.32 per 10,000
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live births, but has increased nearly 30% since the late 1990s.1, 3 The increased burden of disease has not been shared equally. The increase in prevalence has largely effected mothers under 25
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period.3, 12
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years of age, despite a significant (43%) drop in the rate of teen pregnancies over a similar time
Maternal birth place (nativity) has more recently been shown to affect gastroschisis
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outcomes. Foreign-born women have a lower likelihood or risk of delivering infants with gastroschisis compared to US-born women (1.47 vs 4.06 in 10,000 births; OR 0.3-0.5, 95% CI 0.2-0.6).11, 13 Within the US, differences in risk of gastroschisis exist based on rural or urban residence. A California study showed that women living in more rural counties were at an increased risk of having newborns with gastroschisis (OR 1.2-2.2, 95% CI 1.0-3.9).13 Moreover,
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Dubrovsky et al. found regional variations that affected treatment of patients with gastroschisis. Patients born in the South and Northeast US were more likely to be treated at low-volume centers, while those in the West and Midwest were likely to be treated at high volume centers.14 Hospital charges by region also vary widely; the Midwest charges were more than $50,000
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above the national median, whereas the West had charges $65,000 less than the median.15 Maternal factors have been extensively studied for their effect on gastroschisis
prevalence and outcomes. Younger women, particularly those < 20 years of age are at higher risk for fetal gastroschisis (11.45 per 10,000 live births compared to 5.35 per 10,000 live births in 20-
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24 year olds).1 Lower body mass index (< 18.1kg/m2), cigarette smoking or alcohol consumption during pregnancy, and lower socioeconomic status have all been found to put women at increased risk of having babies with gastroschisis in some studies.16
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Race and ethnicity have been reported in numerous studies to have an effect on the prevalence, outcomes, and costs associated with gastroschisis.15 When comparing age-matched
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mothers < 20 years of age, there are differences in prevalence among mothers of different races: 18.1, 16.1, 10.2 per 10,000 live births in white, Hispanic, and black mothers, respectively.3 The
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recent increase in prevalence, though, has affected young (< 20 years old) black mothers more
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than other races, and black neonates are at an increased risk for gastroschisis complicated by atresia (OR 1.74, 95% CI 1.07-2.843).3, 15 The correlations in race-ethnicity to gastroschisis
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prevalence and outcomes are less clear when examining older age groups.16 Hospital charges for gastroschisis were found to be higher in black (additional $50,495) and Hispanic mothers, compared to white mothers.15 Low income and government insurance was found to be a risk for mortality, but associated with lower hospital charges.15 Based on the average income by zip code, infants born to mothers in the first and second income quartiles had an increased odds of
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mortality compared to the fourth quartile (OR 2.52, 95% CI 1.03-6.16; OR 1.36, 95% CI 1.368.23, respectively).15 Hospital charges, however, for those in the lowest quartile for income were more than $50,000 less than those in the highest quartile for income.15
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Hospital Volumes
For many surgical diseases, particularly complex ones, outcomes are reportedly better for patients treated at high volume centers.14, 17 Several studies have examined whether this
relationship is true for gastroschisis. In a California review of 1,500 infants at 55 facilities,
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patients treated at centers with higher volume of gastroschisis repair annually (>9 cases per year) had decreased odds of mortality (OR 0.40, 95% CI 0.21-0.76) compared to those treated at hospitals with fewer than five cases annually.17 Early management and intervention was thought
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to contribute to the observed difference in outcomes. The California data showed shorter hospital stays when patients were treated at high volume centers (43.3 days vs 49 days), though this did
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not maintain statistical significance when accounting for other variables.17 A recently published review of national data, however, does not support the trends seen
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in California. Dubrovsky et al. in 2017 examined the KID from 2003, 2006, 2009, and 2012, and
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found that hospital volume did not have a significant relationship with outcomes in patients with gastroschisis. The study included more than 7,500 patients treated at over 740 hospitals across
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the United States. The authors did not find a significant difference in mortality, but did find that patients had an increased odds for prolonged length of stay (OR 1.59, 95% CI 1.12-2.24) when treated at high-volume centers (>14 cases annually).14 But due to the lack of longitudinal tracking of individual patients in the KID dataset, the limitations of the study include the possibility of lower-level hospitals reporting earlier discharges for patients who were actually
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transferred to other hospitals. Higher volume hospitals were found to have significantly more transfers than lower volume centers (60% vs 12%), which could potentially prolong length of stay in those patients.14 The Canadian Pediatric Surgery Network (CAPSNet) gastroschisis database was analyzed by Baird et al., who found that case volume among the tertiary centers did
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not have an effect on mortality (94.3% vs 97.2% survival, p = 0.17 for low vs high volume centers).18 In their analysis of the more than 400 cases over the 4-year study period, they did not stratify patients by complexity of disease, which may have influenced their findings. When a separate group investigated CAPSNet data for uncomplicated gastroschisis from 2005-2013, they
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found that the best outcomes were realized at medium volume hospitals, defined as 3-8 gastroschisis cases annually.8 However, the outcome advantage was quite modest.
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NICU Designations
While hospital volume for any given condition is often used to judge experience and
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competency in surgical diseases, gastroschisis is a condition primarily managed by pediatric surgeons and neonatologists within NICUs. The American Academy of Pediatrics (AAP) 2004
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(and more recently 2012) policy statement defines the NICU levels of care based on functional
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capabilities, resources, and outcomes.19, 20 NICU level IIIC is the designation for the highest level of neonatal care. One retrospective study examined the effects of NICU designation on
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gastroschisis outcomes. In a review of the California Perinatal Quality Care Collaborative (CPQCC) gastroschisis data from 2008 to 2014, Apfeld et. al examined more than 1,500 records of patients with gastroschisis treated at 129 NICUs. The overall mortality rate was 2.5%, with a median 5 days on ventilator, and a median length of stay of 36 days.21 The majority of patients were born outside level IIIC facilities, with 38.2% born at level IIIB, 10.2% born at IIIA, and
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13% born at level IIA/B centers. When adjusting for confounding factors, the odds of death were higher for patients born at a level IIA/B NICU (OR 6.66; p = 0.004), a IIIA NICU (OR 5.95; p = 0.008), or a level IIIB NICU (OR 5.85; p = 0.002) compared to the highest level, IIIC NICUs.21 Infants born at lower level NICUs also had significantly more days on ventilator (median number
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of days on ventilator at level IIA/B was 9 days) and prolonged length of stay (median 43 days at level IIA/B).21
Apfeld et al. also discuss the likely importance of initial management of patients with gastroschisis. The majority of patients born outside level IIIC NICUs (588/1063 or 55.3%) were
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transferred to a higher level of care within the first 48 hours of life. Despite the rapid transfers, the differences in outcomes (mortality, LOS, days on ventilator) persist based on the hospital and NICU of birth. The CPQCC database also supports the concept that case-specific surgical
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volume improves outcomes. Apfeld et al. found increased mortality risk at centers with the
quartile volume centers.21
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Centralization of Care
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lowest quartile volumes of gastroschisis repair (OR 3.1, 95% CI 1.13-8.49) compared to highest
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The finding of better outcomes at high volume centers has been used as an argument for centralizing or regionalizing care.19 Specifically for gastroschisis, there is evidence to suggest
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that higher volume centers have better outcomes, including decreased mortality.17 There are numerous barriers to centralizing or regionalizing care that need to be examined. Centralizing care requires extensive coordination and consensus in management of gastroschisis and agreed upon criteria for designating centers in a responsible way. Centralizing care for gastroschisis
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could mean delivery at designated centers and/or transferring to designated centers, with the goal of improved outcomes and lower costs. There are very few relevant studies that directly compare centralized vs decentralized systems with respect to their impact on gastroschisis outcomes. One recent study utilized the
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CAPSNet and KID databases to compare gastroschisis outcomes in Canada with outcomes from the US.22 The authors used Canada’s national healthcare system and 16 regional centers as a model for centralized care, in contrast to the decentralized system in the US. They found that mortality for simple gastroschisis was higher in the US compared to Canada (3.4% vs 1.4%, p =
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0.008), though length of stay was shorter in the US (41 vs 45 days, p = 0.004). No difference in the length of stay or mortality was found for patients with complex gastroschisis.22 The US also had significantly higher rates of prematurity and low birth weight (both of which are risk factors
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for worse outcomes), and a Cesarean section deliver rate twice as high as Canada’s.23-25 Notwithstanding the observed differences, the study did not offer strong evidence for
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centralization. Despite Canada’s designation of a limited number of regional pediatric surgical centers, there is still wide variation in the management of gastroschisis at these centers.18
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Furthermore, while the US does not have formal criteria for ‘designated’ gastroschisis centers,
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there is a de facto regionalization, with high rates of transfer to academic centers, level III NICUs, or high-volume centers.22
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Delivery at designated centers Multiple retrospective studies that have tried to examine the effect of birth hospital on
gastroschisis outcomes have produced conflicting results. 26, 27 One large, multi-institutional study utilized the Pediatric Surgery Research Collaborative (PedSRC) from 2008-2013 to compare the outcomes of “inborn” (those born and treated in the same facility) vs “outborn”
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patients with gastroschisis (those who required transfer to separate facility for definitive management).26 Though patients had similar baseline characteristics, inborn infants began enteral feedings earlier and achieved full feeds significantly sooner (27 days vs 31 days, p = 0.0008). Inborn patients were also found to have earlier definitive closure (3.7 days vs 5.4 days,
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p<0.0001).26 On bivariate analysis, inborn patients had significantly shorter length of stay (34 days vs 42 days, p = 0.002), though this fell out of statistical significance on multivariate
analysis. The study argued for patients diagnosed prenatally to be followed and ultimately delivered at a center that has the capabilities to definitively manage gastroschisis.
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The CAPSNet database was also reviewed to examine the same question, whether birth hospital had an effect on gastroschisis outcomes.27 From 2005-2008, there were nearly 400 patients with gastroschisis, with 40.5% of patients being outborn. No difference was appreciated
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between the inborn and outborn cohorts with respect to days of TPN, days until first enteral feed, or length of stay. However on multivariate analysis, outborn status was determined to be an
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independent predictor of complications [OR 1.6 (95% CI 1.09-2.7)], including sepsis, wound dehiscence, and wound infection.27 Many of the level IIIC NICU’s are housed in free-standing
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children’s hospitals without maternity or obstetrics services. Inborn care is not possible in such
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centers.
Transfer to designated centers
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Newborns with gastroschisis are often transferred to other qualified centers even when the birth hospital has potentially adequate surgical and NICU capabilities. Sacks et al. found that more than half of the infants (59.7%) treated at high-volume centers were transferred from midor low-volume centers. In their cohort, transferring patients prior to gastroschisis closure was not associated with increased mortality.17 A recent North American study had similar findings:
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25.7% of their cohort was transferred to another hospital shortly after birth, and transfer status did not significantly affect length of stay or mortality.6 They hypothesized that the benefits of being transferred to higher volume centers outweigh the risks associated with transport. Patients
volume centers with presumed management capabilities.17
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transferred to high-volume centers had improved outcomes compared to those treated at lower
Benefit of transfer to high-volume centers is evident in two different data sources
analyzing nearly all California hospitals.17, 21 However, this finding is not easily apparent when analyzing national data sources that have limited longitudinal tracking of patients’ transfers
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between hospitals.6,14 The Canadian data found an increased risk of necrotizing enterocolitis in gastroschisis patients who were outborn and subsequently transferred to designated centers.8,27 Given these data, it is entirely reasonable, and certainly preferable from a patient and family-
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centered perspective, to deliver babies with a prenatal diagnosis of gastroschisis in tertiary mother-child centers whenever possible. This avoids transfer immediately after birth and
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provides care in an environment with adequate expertise. I Fiscal impact of decentralized gastroschisis care
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As it stands in the US, the care of patients with gastroschisis has a significant financial
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impact on families and hospital systems, with median hospital charges per case ranging from $111,500 to $250,000.9, 15, 28-32 In a review of the 30 most costly pediatric surgical procedures
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performed at 45 hospitals, gastroschisis closure and care had the highest cost variation at the hospital level.30 Despite its relatively low prevalence compared to more common procedures, gastroschisis made up nearly 10% or $34 million of the cumulative cost variation burden in the US from 2014-2015. Cost variation burden is defined as the absolute difference between the median and the individual cost. The review put the median hospital charge at $111,500, but the
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interquartile range was over $48,000.30 Song et al. found the interquartile range to be even higher, with interquartile charges between $139,717 and $384,184.15 The remarkable variability in cost underscores the need for more comparative effectiveness research in gastroschisis outcomes, highlights the lack of consensus in management practices, and may further the
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argument for regionalizing care and/or implementation of collaborative care plans across institutions. Barriers to regionalizing care
While centralizing care to specific regional centers may be potentially beneficial for
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patients and hospital systems, there is insufficient evidence that the practice can significantly improve gastroschisis outcomes. The majority of the evidence regarding healthcare delivery systems and gastroschisis comes from single center retrospective reviews or pooled
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administrative data from various networks. Single center reviews for gastroschisis are often limited by small numbers, or long study periods, during which standards of care often change.33
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The administrative data from healthcare networks often lacks the clinical depth that is needed to make accurate correlations. Furthermore, there is limited evidence that these networks are
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driving improvement, particularly in neonatal surgical diseases.18, 33
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There are other practical barriers to implementing a centralized system of care, at least in the US. There is a lack of widely accepted consensus guidelines for management of
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uncomplicated and complicated gastroschisis, including prenatal and delivery management, initial management strategies, timing of closure, routine use of silos, and feeding practices. There are geographic limitations for the large US population and area. Currently, the majority of patients are treated at lower to medium volume centers.14,17 There are only four centers in California, with a population of approximately 39 million, that are considered high-volume.17
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Furthermore, there is no accepted standard for designating centers as excellent or competent for gastroschisis management. Annual hospital volumes, NICU levels, mortality and morbidity outcomes could be used as surrogates for evaluating centers, though no gastroschisis-specific
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metric exists currently.
Conclusions
While gastroschisis mortality has improved drastically, there are considerable variations in morbidity outcomes and resource utilization based on a multitude of factors. Patient and
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maternal factors have been extensively studied, but little is known about the true effects of
healthcare delivery systems on gastroschisis outcomes. Improving outcomes, particularly for uncommon neonatal surgical disease like gastroschisis will likely require broad-scale adoption of
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evidence-based best practices and implementation of quality improvement programs across multi-institutional networks. There is a reliance on shared, multi-institutional data because of the
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low prevalence of gastroschisis, but the clinical variations in presentation and management are difficult to account for in these large administrative datasets, making identification of best
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practices difficult.
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While not without exception, the majority of the evidence points to an improvement in gastroschisis outcomes when infants are born at, or treated at, higher volume centers and higher
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level NICUs. Regionalizing care may improve outcomes, but there is little definitive proof that official designations are needed or feasible within the US. Potentially independent of strict regionalization, more comparative effectiveness and health services research needs to be done to establish best practice patterns that may allow dissemination and harmonization of gastroschisis care to improve value for all stakeholders.
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Table 1. Gastroschisis outcomes related to healthcare delivery.
Study Author Location
Data source, Quality (A,C, QI)
Years, gastroschisis patients (n)
Study focus
Impact on gastroschisis outcomes from healthcare system variables and comments 15
ACCEPTED MANUSCRIPT
CPQCC 129 NICUs A
2008 – 2014 n = 1588
AAP NICU level at birth hospital
Sacks et al. California
OSHPD 55 hospitals A
1999 – 2007 n = 1537
Hospital gastroschisis closure volumes
Fullerton et al. North America
VON 175 hospitals C, QI
2009 – 2014 n = 4420
Predictive factors, patient outcomes
Dubrovsky et al. United States
KID 743 hospitals A
2003, 06, 09, 12 n = 7769
Hospital gastroschisis closure volumes
Youssef et al. Canada
CAPSNet 16 regional centers A, QI
2005 – 2013 n = 700
Predictive factors, hospital volumes
Cameron et al. United States
PHIS 45 Tertiary children’s hospitals A
2014 – 2015 n = 784
CER, interhospital cost variation
Gonzalez et al. United States
PHIS 45 Tertiary children’s hospitals A
AN US
M
ED
Predictive factors, patient outcomes
CAPSNet/KID 16 regional centers/ 743 hospitals A, QI
2005 – 2013/ 2003, 06, 09, 12 n = 695 / 5216
Centralized vs noncentralized
Allman et al. United States
Pediatrix 351 NICUs C, A
1997 – 2015 n = 6023
Baird et al.
CAPSNet 16
2005 – 2009 n =
Trends in prevalence, demographic risk factors Variation in
CE
PT
2005 – 2013 n = 4459
AC
Youssef et al. Canada/United States
↑ odds of mortality, ↑ LOS, ↑ ventilator at lower level NICU ↓ odds of mortality, ↓ LOS (NS) when closure performed at high volume centers (> = 9 cases annually) Limited data regarding healthcare system impact; mode of delivery did not affect mortality, LOS High-volume hospitals receive more transfers, ↑ odds of prolonged LOS, no effect on mortality Volume had no effect on mortality, Moderate-volume centers had ↓ LOS and fewer days on TPN Median hospital charge $114,230, widest range in interhospital cost variation in pediatric surgery Government insurance associated with ↑ LOS, ↑ days on TPN; hospital volume/characteristics have no effect on LOS, days on TPN Mortality rate for simple gastroschisis ↓ in Canada (1.4% vs 3.4%, p < 0.05), NS for complex gastroschisis. ↑ odds of low birth weight, ↓ LOS in US ↓ prevalence from peak in 2008; young maternal age confers ↑ risk NS difference
CR IP T
Apfeld et al. California
16
ACCEPTED MANUSCRIPT
regional centers A, QI
416
delivery practices across regional centers
Lusk et al. California
UCfC 5 University hospitals C
20107 – 2012 n = 191
Institutional variations, closure
Overton et al. United Kingdom
UKOSS; BAPSCASS; BINOCAR 226 maternity units; 28 pediatric surgical units; A, QI PedSRC 6 centers C
2006 – 2007 n = 303
Maternal factors, predictive factors, antenatal management
2008 – 2013 n = 528
Inborn vs outborn
AN US
Inborn patients reach full enteral feeds sooner, ↓ LOS Abbreviations: A (administrative data); C (clinical data); QI (Quality Improvement data); CPQCC (California Perinatal Quality Care Collaborative); AAP (American Academy of Pediatrics); NICU (Neonatal Intensive Care Unit); LOS (length of stay); OSHPD (California Office of Statewide Health Planning and Development); NS (Not Significant, p > 0.05); VON (Vermont Oxford Network); KID (Kids’ Inpatient Database); CAPSNet (Canadian Pediatric Surgery Network); TPN (total parenteral nutrition); PHIS (Pediatric Health Information System); CER (comparative effectiveness research); UCfC (University of California Fetal Consortium); UKOS (UK Obstetric Surveillance System); BAPS-CASS (British Association of Paediatric Surgeons Congenital Anomalies Surveillance System); BINOCAR (British Isles Network of Congenital Anomalies Registers); PedSRC (Pediatric Surgery Research Collaborative)
AC
CE
PT
ED
M
Savoie et al. United States
between cesarean and vaginal deliveries on mortality, LOS, days on TPN Wide variation in management, NS difference in measured outcomes (mortality, LOS, ventilator days) Wide variation in antenatal management without clear benefit
CR IP T
Canada
17