Perioperative outcomes in minimally-invasive versus open surgery in infants undergoing repair of congenital anomalies.

BACKGROUND: This study compared perioperative outcomes among infants undergoing repair of congenital anomalies using minimally invasive (MIS) versus open surgical approaches. METHODS: The ACS NSQIP Pediatric (2013-2018) was queried for patients undergoing repair of any of the following 9 congenital anomalies: congenital lung lesion (LL), mediastinal mass (MM), congenital malrotation (CM), anorectal malformation (ARM), Hirschsprung disease (HD), congenital diaphragmatic hernia (CDH), tracheoesophageal fistula (TEF), hepatobiliary anomalies (HB), and intestinal atresia (IA). Inverse probability of treatment weights (IPTW) derived from propensity scores were utilized to estimate risk-adjusted association between surgical approach and 30-day outcomes. RESULTS: 12,871 patients undergoing congenital anomaly repair were included (10,343 open; 2528 MIS). After IPTW, MIS was associated with longer operative time (difference; 95% CI) (16 min; 9-23) and anesthesia time (13 min; 6-21), but less postoperative ventilation days (-1.0 days; -1.4- -0.6) and shorter postoperative length of stay (-1.4 days; -2.4- -0.3). MIS repairs had decreased risk of any surgical complication (risk difference: -6.6%; -9.2- -4.0), including hematologic complications (-7.3%; -8.9- -5.8). There was no significant difference in risk of complication when hematologic complications were excluded (RD -2.3% [-4.7%, 0.1%]). There were no significant differences in the risk of unplanned reoperation (0.4%; -1.5-2.2) or unplanned readmission (0.2%; -1.2-1.5). CONCLUSIONS: MIS repair of congenital anomalies is associated with improved perioperative outcomes when compared to open. Additional studies are needed to compare long-term functional and disease-specific outcomes. MINI-ABSTRACT: In this propensity-weighted multi-institutional analysis of nine congenital anomalies, minimally invasive surgical repair was associated with improved 30-day outcomes when compared to open surgical repair. LEVEL OF EVIDENCE: III.

Benchmarking utilization, length of stay, and complications following minimally invasive repair of major congenital anomalies.

INTRODUCTION: Infants with newborn congenital anomalies are increasingly undergoing minimally invasive surgical (MIS) repair. Currently available data on outcomes are limited. This study provides national estimates for length of stay and 30-day complications following MIS for congenital anomalies. METHODS: Using the ACS-NSQIP Pediatric (2013-2018), a retrospective analysis of MIS for congenital anomalies was performed. MIS repairs for the following diagnoses were included: pyloric stenosis (PS), congenital lung lesion (LL), mediastinal mass (MM), congenital malrotation (CM), anorectal malformation (ARM), Hirschsprung's disease (HD), congenital diaphragmatic hernia (CDH), tracheoesophageal fistula (TEF), biliary atresia/choledochal cyst (HB), and intestinal atresia (IA). Postoperative LOS (pLOS) and complication rates were examined using multivariable analysis for risk factors after categorizing surgeries by complexity of care related to congenital anomaly: Simple (PS), Complex Group 1 (LL, MM, CM, and ARM), and Complex Group 2 (HD, CDH, TEF, HB, and IA). RESULTS: Across 10 anomalies, 8,326 repairs were performed using an MIS approach. Procedure-specific median postoperative LOS (75th-percentile, 90th-percentile) for PS was 1 day (1, 3); LL was 3 (4, 7); MM was 2 (3, 5); CM was 4 (7, 14); ARM was 3 (5, 8); HD was 5 (8, 12); CDH was 8 (18, 31); HB was 5 (8, 12); TEF was 20 (31, 53); and IA was 17 (25, 40). The overall surgical complication rates (95% CI) were: PS, 5.1% (4.7%-5.6%); LL, 14.2% (12.3-16.4); MM, 8.4% (6.4-11.0); CM, 14.6% (11.9-17.9); ARM, 12.0% (7.1-19.5); HD, 22.1% (19.5-25.0); CDH, 21.1% (17.1-25.6); HB, 20.6% (13.7-29.7); TEF, 36% (27.5-45.5); and IA, 28.6% (19.3-40.1). Risk factors for increased pLOS and complications varied by procedure category and included patient-level and admission characteristics. CONCLUSION: This study provides national benchmarks and risk factors for expected postoperative LOS and 30-day complications following MIS for congenital anomalies.

Variability in the management of adhesive small bowel obstruction in children.

BACKGROUND: This study assessed inter-hospital variability in operative-vs-nonoperative management of pediatric adhesive small bowel obstruction (ASBO). METHODS: A multi-institutional retrospective study was performed examining patients 1-21 years-of-age presenting with ASBO from 2010 to 2019 utilizing the Pediatric Health Information System. Multivariable mixed-effects logistic regression was performed assessing inter-hospital variability in operative-vs-nonoperative management of ASBO. RESULTS: Among 6410 pediatric ASBO admissions identified at 46 hospitals, 3,239 (50.5%) underwent surgery during that admission. The hospital-specific rate of surgery ranged from 35.3% (95%CI: 28.5-42.6%) to 74.7% (66.3-81.6%) in the unadjusted model (p < 0.001), and from 35.1% (26.3-45.1%) to 73.9% (66.7-79.9%) in the adjusted model (p < 0.001). Factors associated with operative management for ASBO included admission to a surgical service (OR 2.8 [95%CI: 2.4-3.2], p < 0.001), congenital intestinal and/or rotational anomaly (OR 2.5 [2.1-3.1], p < 0.001), diagnostic workup including advanced abdominal imaging (OR 1.7 [1.5-1.9], p < 0.001), non-emergent admission status (OR 1.5 [1.3-1.8], p < 0.001), and increasing number of complex chronic comorbidities (OR 1.3 [1.2-1.4], p < 0.001). Factors associated with nonoperative management for ASBO included increased hospital-specific annual ASBO volume (OR 0.98 [95%CI: 0.97-0.99], p = 0.002), older age (OR 0.97 [0.96-0.98], p < 0.001), public insurance (OR 0.87 [0.78-0.96], p = 0.008), and presence of coinciding non-intestinal congenital anomalies, neurologic/neuromuscular disease, and/or medical technology dependence (OR 0.57 [95%CI: 0.47-0.68], p < 0.001). CONCLUSIONS: Rates of surgical intervention for ASBO vary significantly across tertiary children's hospitals in the United States. The variability was independent of patient and hospital characteristics and is likely due to practice variation. LEVEL OF EVIDENCE: III.

Room for Improvement: The Trephination Procedure for Pediatric Patients with Pilonidal Disease.

BACKGROUND: Pilonidal disease is common amongst adolescent males and females and often leads to recurrent symptoms and life-altering morbidity. The traditional surgical approach includes wide excision of the involved area and carries a high rate of postoperative morbidity. A minimally invasive surgical approach using trephines was described by Gips in 2008 and has since been widely adopted by many surgeons. The aim of this study was to explore outcomes of the trephination procedure for pediatric and adolescent patients by evaluating postoperative wound healing and disease recurrence. MATERIALS AND METHODS: A retrospective cohort study for patients that underwent the trephination procedure as part of standard of care for the treatment of pilonidal disease from November 1, 2019-November 1, 2020 was performed. Patient demographics, presenting characteristics, and previous treatment history were identified. Outcome measures included healing time, recurrent disease, and need for reoperation. RESULTS: A total of 19 patients underwent the trephination procedure at a mean age of 16.4 years of age. An average of 3.8 pits were excised and there were no reported intraoperative complications. Following trephination, 26.3% of patients were healed at 30-day's, with just over 40% showing complete healing by 6-months. The recurrence rate was 16.1% at 6-months and approximately 15% of patients required a second surgery. CONCLUSION: Early results for trephination at our institution show a high rate of healing complications and frequent reoperation. Future research is needed to establish the role of the trephination procedure in the context of defining the best practices for treating this challenging disease.

Variability in age at Kasai portoenterostomy for biliary atresia across US children's hospitals.

BACKGROUND/PURPOSE: The purpose of this study was to assess variability in age at Kasai portoenterostomy (KP) in infants with biliary atresia (BA) across children's hospitals in the United States. STUDY DESIGN: A multi-institutional retrospective study was performed examining infants with BA undergoing KP within 6 months of birth from 2016-2019, utilizing the Pediatric Health Information System (PHIS). Multivariable negative binomial mixed effects regression was performed for age at KP, and inter-hospital variability was examined. RESULTS: Across 46 hospitals, 470 infants with BA underwent KP at a median age of 57 days (IQR 42-72), with 212 (45.1%) undergoing KP at ≥60 days of age. There was significant inter-hospital variability in age at KP ranging from 38 days (95% CI: 31d, 47d) to 76 days (95% CI: 63d, 91d) (p<0.0001). Factors associated with later KP were black or African-American race, urgent/emergent admission, and treatment at a hospital in the Pacific-West region. Predictors of earlier KP included later year, history of neonatal comorbidity, and admission to an intensive care service (all p<0.05). CONCLUSION: There is significant variability in the age at KP in infants with BA across children's hospitals in the United States. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: III.

Principles in treating pediatric patients with pilonidal disease - An expert perspective.

Pilonidal disease is common amongst adolescent males and females and often leads to recurrent symptoms and life-altering morbidity. Traditionally, surgery included wide excision of the involved area with primary closure. Post-operative complication rates were high and recurrence of disease common, leading to a search for alternative approaches for treating pilonidal disease. A minimally invasive (trephination) approach was described by Gips in 2008 and has since been adopted by many surgeons. Although the trephination procedure is less morbid than total excision, the risk of wound complications is not insignificant and post-operative recurrence has been reported in more than 10% of patients. The lack of a clear advantage for any single treatment strategy has led a wide variation in provider approach. To standardize the care for pediatric patients with pilonidal disease, a dedicated clinic was created at our institution in 2018. The aim of this paper is to describe an approach to treating pediatric patients with pilonidal disease that has been established through the experience of treating hundreds of adolescent males and females per year. Given the impact on quality of life for those that are affected by pilonidal disease, it is important that future research be directed toward discovery of the best practices for treating this challenging disease.

Relationships Between Hospital and Surgeon Operative Volumes and Surgical Outcomes in Hirschsprung's Disease.

BACKGROUND: The impact of surgical volume on outcomes in Hirschsprung's disease (HD) remains uninvestigated. We aimed to determine whether higher surgeon and hospital-level HD operative volumes are associated with improved surgical outcomes following primary surgery for neonatal HD. MATERIALS AND METHODS: Neonates who underwent either an ostomy or pull-through (PT) procedure for HD before 60 d of life and a PT procedure by age 1 y were identified in the Pediatric Health Information System (PHIS). Index admissions from January 2000 to September 2012 across 41 tertiary childrens hospitals were included. Surgeon and hospital-level HD operative volume were defined as the average annual number of PT procedures performed for HD in the 2 y preceding each included case. We examined the relationship between operative volumes and all-cause readmission, readmission for Hirschsprung's associated enterocolitis (HAEC), and rates of reoperation within 30 d and 2 y. RESULTS: A total of 1268 infants were included. There were 218 patients (17.2%) readmitted to the hospital within 30 d and 540 (42.6%) within 2 y. A total of 119 patients (9.4%) had HAEC-related readmission within 30 d, and 271 (21.4%) had HAEC-related readmission within 2 y. A total of 57 patients (4.5%) had a reoperation within 30 d and 129 (10.2%) within 2 y. In risk-adjusted analyses, there were no significant associations between either surgeon or hospital HD operative volumes and readmission/reoperation rates within 30 d or 2 y. CONCLUSIONS: Neither surgeon nor hospital PT volumes were significantly associated with readmission or reoperation rates for infants with Hirschsprung's disease. Future work is needed to evaluate whether operative volumes are associated with functional outcomes following PT for HD.

Utilization of Augmentation Cystoplasty for Myelomeningocele Patients Remained Stable Over the Past Decade.

OBJECTIVE: To understand the utilization of augmentation cystoplasty (AC) for the management of neurogenic bladder in children with myelomeningocele (MMC). AC carries considerable risk. To mitigate this, recent studies have emphasized alternatives to AC, but it is unknown if these interventions have resulted in fewer ACs being performed. Our goal was to evaluate the use of AC in MMC patients over the past decade. We hypothesized that the use of AC had decreased and that the age at AC increased. METHODS: Using the Pediatric Health Information System, we studied patients with MMC who had an AC between January 2009 and December 2018. International Classification of Disease procedure codes were used to identify AC procedures. We quantified trends in AC utilization by estimating the annual proportion of MMC admissions with an AC. We also assessed trends in patient age at the time of AC. RESULTS: Proportion of AC per MMC admissions across the study period was 4.8%. There was no significant annual trend in the overall number of ACs performed each quarter over the past decade nor was there any change in the estimated annual incidence rate ratio of AC (1.01, 95% confidence interval 0.96, 1.05; P =.75). The estimated annual change in patient age at procedure remained relatively constant over the study period (-0.03 years, 95% confidence interval -0.13, 0.07; P = .51). CONCLUSION: Practice patterns for the utilization of AC in MMC did not change significantly over the past decade despite prominent voices in the literature emphasizing alternative interventions in this patient population.

Treating Center Volume and Congenital Diaphragmatic Hernia Outcomes in California.

OBJECTIVE: To examined outcomes for infants born with congenital diaphragmatic hernias (CDH), according to specific treatment center volume indicators. STUDY DESIGN: A population-based retrospective cohort study was conducted involving neonatal intensive care units in California. Multivariable analysis was used to examine the outcomes of infants with CDH including mortality, total days on ventilation, and respiratory support at discharge. Significant covariables of interest included treatment center surgical and overall neonatal intensive care unit volumes. RESULTS: There were 728 infants in the overall CDH cohort, and 541 infants (74%) in the lower risk subcohort according to a severity-weighted congenital malformation score and never requiring extracorporeal membrane oxygenation. The overall cohort mortality was 28.3% (n = 206), and 19.8% (n = 107) for the subcohort. For the lower risk subcohort, the adjusted odds of mortality were significantly lower at treatment centers with higher CDH repair volume (OR, 0.41; 95% CI, 0.23-0.75; P = .003), ventilator days were significantly lower at centers with higher thoracic surgery volume (OR, 0.56; 9 5% CI, 0.33-0.95; P = .03), and respiratory support at discharge trended lower at centers with higher neonatal intensive care unit admission volumes (OR, 0.51; 9 5% CI, 0.26-1.02; P = .06). CONCLUSIONS: Overall and surgery-specific institutional experience significantly contribute to optimized outcomes for infants with CDH. These data and follow-on studies may help inform the ongoing debate over the optimal care setting and relevant quality indicators for newborn infants with major surgical anomalies.

Learning health systems and the future of clinical research.

Pediatric surgeons are collectively passionate about prioritizing the healthcare needs of children. We contend that this passion is deeply ingrained in how we drive clinical care and influence scientific discovery. Thus, the future of clinical research in our field will be deeply embedded in our history as a "patient-centric" profession. Service to pediatric patients requires an understanding of their needs and expectations, and designing research that acknowledges both. In this article we detail how future pragmatic clinical research will look in the evolving and learning health system.

The disproportionate cost of operation and congenital anomalies in infancy.

BACKGROUND: Congenital anomalies are the leading cause of infant death and pediatric hospitalization, but existing estimates of the associated costs of health care are either cross-sectional surveys or economic projections. We sought to determine the percent of total hospital health care expenditures attributable to major anomalies requiring surgery within the first year of life. METHODS: Utilizing comprehensive California statewide data from 2008 to 2012, cohorts of infants undergoing major surgery, with birth defects and with surgical anomalies, were constructed alongside a referent group of newborns with no anomalies or operations. Cost-to-charge and physician fee ratios were used to estimate hospital and professional costs, respectively. For each cohort, costs were broken down according to admission, birth episode, and first year of life, with additional stratifications by birth weight, gestational age, and organ system. RESULTS: In total, 68,126 of 2,205,070 infants (3.1%) underwent major surgery (n = 32,614) or had a diagnosis of a severe congenital anomaly (n = 57,793). These accounted for $7.7 billion of the $18.9 billion (40.7%) of the total health care costs/expenditures of the first-year-of-life hospitalizations, $7.0 billion (48.6%) of the costs for infants with comparatively long birth episodes, and $5.2 billion (54.7%) of the total neonatal intensive care unit admission costs. Infants with surgical anomalies (n = 21,264) totaled $4.1 billion (21.7%) at $80,872 per infant. Cardiovascular and gastrointestinal diseases accounted for most admission costs secondary to major surgery or congenital anomalies. CONCLUSION: In a population-based cohort of infant births compared with other critically ill neonates, surgical congenital anomalies are disproportionately costly within the United States health care system. The care of these infants, half of whom are covered by Medi-Cal or Medicaid, stands as a particular focus in an age of reform of health care payments.

The Effect of Level of Care on Gastroschisis Outcomes.

OBJECTIVE: To examine the relationship between level of care in neonatal intensive care units (NICUs) and outcomes for newborns with gastroschisis. STUDY DESIGN: A retrospective cohort study was conducted at 130 California Perinatal Quality Care Collaborative NICUs from 2008 to 2014. All gastroschisis births were examined according to American Academy of Pediatrics NICU level of care at the birth hospital. Multivariate analyses examined odds of mortality, duration of mechanical ventilation, and duration of stay. RESULTS: For 1588 newborns with gastroschisis, the adjusted odds of death were higher for those born into a center with a level IIA/B NICU (OR, 6.66; P = .004), a level IIIA NICU (OR, 5.95; P = .008), or a level IIIB NICU (OR, 5.85; P = .002), when compared with level IIIC centers. The odds of having more days on ventilation were significantly higher for births at IIA/B and IIIB centers (OR, 2.05 [P < .001] and OR, 1.91 [P < .001], respectively). The odds of having longer duration of stay were significantly higher at IIA/B and IIIB centers (OR, 1.71 [P < .004]; OR, 1.77 [P < .001]). CONCLUSIONS: NICU level of care was associated with significant disparities in odds of mortality for newborns with gastroschisis.

Infant, maternal, and geographic factors influencing gastroschisis related mortality in Zimbabwe.

INTRODUCTION: Survival for infants with gastroschisis in developed countries has improved dramatically in recent decades with reported mortality rates of 4-7%. Conversely, mortality rates for gastroschisis in sub-Saharan Africa remain as great as 60% in contemporary series. This study describes the burden of gastroschisis at the major pediatric hospital in Zimbabwe with the goal of identifying modifiable factors influencing gastroschisis-related infant mortality. METHODS: We performed a retrospective cohort study of all cases of gastroschisis admitted to Harare Children's Hospital in 2013. Univariate and multivariate analyses were performed to describe infant, maternal, and geographic factors influencing survival. RESULTS: A total of 5,585 neonatal unit admissions were identified including 95 (1.7%) infants born with gastroschisis. Gastroschisis-related mortality was 84% (n = 80). Of infants with gastroschisis, 96% (n = 91) were born outside Harare Hospital, 82% (n = 78) were born outside Harare Province, and 23% (n = 25) were home births. The unadjusted odds of survival for these neonates with gastroschisis were decreased for low birth weight infants (<2,500 grams; odds ratio [OR], 0.15; 95% CI, 0.05-0.51), preterm births (<37 weeks gestational age; OR, 0.06; 95% CI, 0.01-0.50), and for those born to teenage mothers (<20 years of age; OR, 0.05; 95% CI, 0.01-0.46). There was also a trend toward decreased odds of survival for home births (OR, 0.16; 95% CI, 0.02-1.34) and for those born outside Harare Province (OR, 0.35; 95% CI, 0.10-1.22). CONCLUSION: Gastroschisis-related infant mortality in Zimbabwe is associated with well-known risk factors, including low birth weight, prematurity, and teenage mothers. However, modifiable factors identified in this study signify potential opportunities for developing innovative approaches to perinatal care in such a resource-constrained environment.

The prevalence and costs of defensive medicine among orthopaedic trauma surgeons: a national survey study.

OBJECTIVES: Defensive medicine includes medical practices that exonerate physicians from liability without benefit to patients. The national prevalence of defensive medicine in orthopaedic trauma surgery has not been investigated. METHODS: In September 2010, 2000 orthopaedic surgeons randomly chosen from the American Academy of Orthopaedic Surgeons registry received invitations to answer a survey on defensive medicine. Among these surgeons, 1214 (61%) completed the survey and 222 (18.5%) identified themselves as nonmilitary orthopaedic traumatologists. Cost analysis was performed using Centers for Medicare and Medicaid data at the 2011 current procedural terminology code level and then aggregated to reflect the 8 domains of care assessed. RESULTS: For orthopaedic traumatologists, on average 22% of all ordered tests were for defensive reasons (radiography, 19%; computed tomographic scanning, 23%; magnetic resonance imaging, 27%; ultrasound, 42%; referrals, 29%; laboratory tests, 23%; and biopsies, 16%). Defensive hospital admissions averaged 9% each month. Orthopaedic traumatologists reported fewer referrals to specialists compared with non-trauma orthopaedists (P = 0.02), with no significant difference in overall monthly defensive expenditures.Using 2011 current procedural terminology code reimbursement data, defensive medicine costs per respondent were calculated to be approximately $7800 monthly or $94,000/y, which is 20% of each physician's spending. Given the approximately 2724 orthopaedic trauma surgeons in practice in the United States according to the 2010 American Academy of Orthopaedic Surgeons Census, the national cost of defensive medicine for orthopaedic trauma surgery is estimated to be $256.3 million annually. CONCLUSIONS: Defensive medicine among orthopaedic trauma surgeons is a significant factor in health care costs and of marginal benefit to patients. Policies aimed at managing liability risk may be useful in containing such practices.