Comparing Healthcare Needs in Extremely Low Birth Weight Infants With NEC and Spontaneous Intestinal Perforation.

BACKGROUND: Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) affect 6-8% of extremely low birth weight (ELBW) infants. SIP has lower mortality than NEC, but with similar short-term morbidity in length of stay, growth failure, and supplemental oxygen requirements. Comparative long-term neurodevelopmental outcomes have not been clarified. METHODS: Data were prospectively collected from 59 North American neonatal units, regarding ELBW infants (401-1000 g or 22-27 weeks gestational age) born between 2011 and 2018 and evaluated again at 16-26 months corrected age. Outcomes were collected from infants with laparotomy-confirmed NEC, laparotomy-confirmed SIP, and those without NEC or SIP. The primary outcome was severe neurodevelopmental disability. Secondary outcomes were weight <10th percentile, medical readmission, post-discharge surgery and medical support at home. Adjusted risk ratios (ARR) were calculated. RESULTS: Of 13,673 ELBW infants, 6391 (47%) were followed including 93 of 232 (40%) with NEC and 100 of 235 (42%) with SIP. There were no statistically significant differences in adjusted risk of any outcomes when directly comparing NEC to SIP (ARR 2.35; 95% CI 0.89, 6.26). However, infants with NEC had greater risk of severe neurodevelopmental disability (ARR 1.43; 1.09-1.86), rehospitalization (ARR 1.46; 1.17-1.82), and post-discharge surgery (ARR 1.82; 1.48-2.23) compared to infants without NEC or SIP. Infants with SIP only had greater risk of post-discharge surgery (ARR 1.64; 1.34-2.00) compared to infants without NEC or SIP. CONCLUSIONS: ELBW infants with NEC had significantly increased risk of severe neurodevelopmental disability and post-discharge healthcare needs, consistent with prior literature. We now know infants with SIP also have increased healthcare needs. LEVELS OF EVIDENCE: Level II.

Predictors of Mortality in Very Low Birth Weight Neonates With Congenital Diaphragmatic Hernia.

BACKGROUND: Limited data exists regarding the mortality of very low birth weight (VLBW) neonates with congenital diaphragmatic hernia (CDH). This study aims to quantify and determine predictors of mortality in VLBW neonates with CDH. METHODS: This analysis of 829 U.S. NICUs included VLBW [birth weight ≤1500g] neonates, born 2011-2021 with and without CDH. The primary outcome was in-hospital mortality. A generalized estimating equation regression model determined the adjusted risk ratio (ARR) of mortality. RESULTS: Of 426,140 VLBW neonates, 535 had CDH. In neonates with CDH, 48.4% had an additional congenital anomaly vs 5.5% without. In-hospital mortality for neonates with CDH was 70.4% vs 12.6% without. Of those with CDH, 73.3% died by day of life 3. Of VLBW neonates with CDH, 38% were repaired. A subgroup analysis was performed on 60% of VLBW neonates who underwent delivery room intubation or mechanical ventilation, as an indicator of active treatment. Mortality in this group was 62.7% for neonates with CDH vs 16.4% without. Higher Apgars at 1 min and repair of CDH were associated with lower mortality (ARR 0.91; 95%CI 0.87,0.96 and ARR 0.28; 0.21,0.39). The presence of additional congenital anomalies was associated with higher mortality (ARR 1.14; 1.01,1.30). CONCLUSION: These benchmark data reveal that VLBW neonates with CDH have an extremely high mortality. Almost half of the cohort have an additional congenital anomaly which significantly increases the risk of death. This study may be utilized by providers and families to better understand the guarded prognosis of VLBW neonates with CDH. TYPE OF STUDY: Level II. LEVEL OF EVIDENCE: Level II.

Factors associated with D-lactic acidosis in pediatric intestinal failure: A case-control study.

BACKGROUND: D-lactic acidosis (DLA) is a serious complication of short bowel syndrome (SBS) in children with intestinal failure (IF). Malabsorbed carbohydrates are metabolized by bacteria in the intestine to D-lactate which can lead to metabolic acidosis and neurologic symptoms. METHODS: A retrospective chart review was performed in children ≤18 years old with SBS who had one of the following criteria: unexplained metabolic acidosis, neurologic signs or symptoms, history of antibiotic therapy for small bowel bacterial overgrowth, or high clinical suspicion of DLA. Cases had serum D-lactate concentration >0.25 mmol/L; controls with concentrations ≤0.25 mmol/L. RESULTS: Of forty-six children, median age was 3.16 (interquartile range (IQR): 1.98, 5.82) years, and median residual bowel length was 40 (IQR: 25, 59) cm. There were 23 cases and 23 controls. Univariate analysis showed that cases had significantly lower median bicarbonate (19 vs. 24 mEq/L, p = 0.001), higher anion gap (17 vs. 14 mEq/L, p < 0.001) and were less likely to be receiving parenteral nutrition, compared with children without DLA. Multivariable analysis identified midgut volvulus, history of intestinal lengthening procedure, and anion gap as significant independent risk factors. Midgut volvulus was the strongest independent factor associated with DLA (adjusted odds ratio = 17.1, 95% CI: 2.21, 133, p = 0.007). CONCLUSION: DLA is an important complication of pediatric IF due to SBS. Patients with IF, particularly those with history of midgut volvulus, having undergone intestinal lengthening, or with anion gap acidosis, should be closely monitored for DLA.

Association between neurodevelopmental outcomes and concomitant presence of NEC and IVH in extremely low birth weight infants.

OBJECTIVE: To quantify the association between necrotizing enterocolitis (NEC) and neurodevelopmental disability (NDI) in extremely low birth weight (ELBW) infants with intraventricular hemorrhage (IVH). STUDY DESIGN: ELBW survivors born 2011-2017 and evaluated at 16-26 months corrected age in the Vermont Oxford Network (VON) ELBW Follow-Up Project were included. Logistic regression determined the adjusted relative risk (aRR) of severe NDI in medical or surgical NEC compared to no NEC, stratified by severity of IVH. RESULTS: Follow-up evaluation occurred in 5870 ELBW survivors. Compared to no NEC, medical NEC had no impact on NDI, regardless of IVH status. Surgical NEC increased risk of NDI in patients with no IVH (aRR 1.69; 95% CI 1.36-2.09), mild IVH (aRR 1.36;0.97-1.92), and severe IVH (aRR 1.35;1.13-1.60). CONCLUSIONS: ELBW infants with surgical NEC carry increased risk of neurodevelopmental disability within each IVH severity stratum. These data describe the additive insult of surgical NEC and IVH on neurodevelopment, informing prognostic discussions and highlighting the need for preventative interventions.

Factors Associated With Chronic Intestinal Inflammation Resembling Inflammatory Bowel Disease in Pediatric Intestinal Failure: A Matched Case-Control Study.

BACKGROUND AND AIMS: There is a subset of intestinal failure patients with associated chronic intestinal inflammation resembling inflammatory bowel disease. This study aimed to evaluate factors associated with chronic intestinal inflammation in pediatric intestinal failure. METHODS: This was a single-center retrospective case-control study of children <18 years old with intestinal failure. Cases were defined by abnormal amounts of chronic intestinal inflammation on biopsies. Children with diversion colitis, eosinophilic colitis, or isolated anastomotic ulceration were excluded. Cases were matched 1:2 to intestinal failure controls based on sex, etiology of intestinal failure, and duration of intestinal failure. Multivariable conditional logistic regression was used to compare clinical factors between cases and controls, accounting for clustering within matched sets. A subgroup analysis was performed assessing factors associated with escalation of anti-inflammatory therapy. RESULTS: Thirty cases were identified and matched to 60 controls. On univariate analysis, longer parenteral nutrition (PN) duration (1677 vs 834 days, P = 0.03), current PN use (33.3% vs 20.0%, P = 0.037), and culture-proven bacterial overgrowth (53.3% vs 31.7%, P = 0.05) were associated with chronic intestinal inflammation. On multivariable analysis, no variable reached statistical significance. On subgroup analysis, duration of intestinal failure, location of inflammation, and worst degree of inflammation on histology were associated with escalation of therapy. CONCLUSIONS: PN dependence and intestinal dysbiosis are associated with chronic intestinal inflammation in children with intestinal failure. Severity of inflammation is associated with escalation of therapy. Further analysis is needed to assess these associations and the efficacy of treatments in this population.

Impact of concomitant necrotizing enterocolitis on mortality in very low birth weight infants with intraventricular hemorrhage.

OBJECTIVE: To evaluate the impact of necrotizing enterocolitis (NEC) on mortality in very low birth weight (VLBW) infants with intraventricular hemorrhage (IVH). STUDY DESIGN: Data were collected on VLBW infants born 2014-2018 at Vermont Oxford Network (VON) centers. NEC and IVH were categorized by severity. Adjusted risk ratios (ARR) for in-hospital mortality were calculated. RESULTS: This study included 187 187 VLBW infants. Both medical and surgical NEC increased mortality risk compared to those without NEC. Stratification by IVH severity modified this effect (no IVH: ARR 3.04 (95%CI 2.74-3.38) for medical NEC and 4.17 (3.84-4.52) for surgical NEC; mild IVH: ARR 2.14 (1.88-2.44) for medical NEC and 2.49 (2.24-2.78) for surgical NEC; severe IVH: ARR 1.14 (1.03-1.26) for medical NEC and 1.10 (1.02-1.18) for surgical NEC). CONCLUSION: The relative impact of NEC on mortality decreased as IVH severity increased. Given the frequent coexistence of NEC and IVH, these data inform multidisciplinary management of these complex patients.

High Rate of Venous Thromboembolism in Severe Pediatric Intestinal Failure.

OBJECTIVE: To quantify the rate of venous thromboembolism (VTE) in patients with pediatric intestinal failure and identify associated risk factors. STUDY DESIGN: We performed a retrospective cohort study in pediatric patients (<21 years old) with severe pediatric intestinal failure (≥90 consecutive days of parenteral nutrition) secondary to short bowel syndrome who were treated from 2014 to 2021 at an interdisciplinary intestinal rehabilitation program. The primary outcome was the incidence of VTE. Multivariable regression was performed to identify independent clinical predictors of VTE. RESULTS: A total of 263 patients (59.7% male) met the criteria for inclusion. The cumulative incidence of VTE was 28.1%, with a rate of 0.32 VTEs per 1000 catheter-days. On univariate analysis, the number of catheter days, number of catheters, and history of central line-associated blood stream infection were associated with VTE. On multivariable logistic regression, a higher number of catheters was an independent risk factor for VTE (aOR, 1.17; 95% CI, 1.06-1.29). Additionally, earlier gestational age was a risk factor for VTE such that every week decrease in gestational age conferred a 9% increased risk of VTE (aOR, 1.09; 95% CI, 1.02-1.16). CONCLUSIONS: In this retrospective study, 28.1% of patients with severe pediatric intestinal failure developed VTE; the number of catheters and early gestational age were noted to be independent risk factors for VTE. This high incidence of VTE highlights the need to investigate VTE in pediatric intestinal failure prospectively, including the potential benefit of prophylactic anticoagulation.

Pediatric trauma mortality in India and the United States: A comparison and risk-adjusted analysis.

BACKGROUND: There is a paucity of research comparing pediatric risk-adjusted trauma mortality between high-income and low- and middle-income countries. This limits identification of populations and injury patterns for targeted interventions. We aim to compare independent predictors of pediatric trauma mortality between India and the United States (US). METHODS: A retrospective cohort study was conducted for pediatric patients (age <18 years) in India's Towards Improved Trauma Care Outcomes (TITCO) project database and the US National Trauma Data Bank (NTDB) from 2013 to 2015. Demographic, injury, physiologic, anatomic and outcome data were analyzed. Multivariable regressions were used to determine independent predictors of mortality. RESULTS: 126,678 pediatric trauma patients were included (India 3,373; US 123,305). Pediatric patients in India were on average significantly younger, with a higher median injury severity score (ISS), had lower systolic blood pressure, and suffered a higher case fatality rate (13.0% vs. 1.0%). When controlling for demographic, mechanism, physiologic, and anatomic injury characteristics, sustaining an injury in India was the strongest predictor of mortality (OR 22.70, 95% CI 18.70-27.56). On subgroup analysis, the highest relative odds of mortality in India was seen in children with lower injury and physiologic severity. CONCLUSIONS: Risk-adjusted pediatric trauma-related mortality is significantly higher in India compared to the US. The comparative odds of mortality are highest among children with lower injury and physiologic severity. This suggests that low-cost targeted interventions focused on standard timely trauma care, protocols, training and early imaging could improve pediatric injury mortality in India. TYPE OF STUDY: Retrospective Prognosis Study LEVEL OF EVIDENCE: II.

Autoimmune Thyroiditis and Risk of Malignancy in Children with Thyroid Nodules.

Background: It is uncertain whether the presence of autoimmune thyroiditis (AIT) increases the risk of thyroid cancer in children with thyroid nodules. This study evaluated the association between AIT and thyroid cancer in pediatric patients with thyroid nodules. Methods: A cross-sectional study was performed of pediatric patients (<19 years old) with a thyroid nodule (≥1 cm) who underwent fine-needle aspiration in an academic pediatric thyroid center. AIT was defined by the presence of thyroid autoantibodies or diffusely heterogeneous sonographic echotexture. The primary outcome was diagnosis of thyroid cancer. The association of AIT with thyroid cancer was evaluated with univariable and multivariable logistic regression. Associations of AIT with subject and nodule characteristics were also assessed. Results: Four hundred fifty-eight thyroid nodules in 385 patients (81% female) were evaluated at a median age of 15.5 years (interquartile range 13.5-17.0). Thyroid cancer was present in 108 nodules (24%). AIT was present in 95 subjects (25%) and was independently associated with an increased risk of thyroid cancer (multivariable odds ratio [OR] 2.19, 95% confidence interval [CI] 1.32-3.62). Thyroid cancer was also independently associated with younger age, nodule size, and solitary nodules, but was not associated with serum thyrotropin concentration. AIT was not associated with the likelihood of subjects undergoing thyroid surgery (p = 0.17). AIT was less commonly associated with follicular thyroid carcinoma than with papillary thyroid carcinoma (OR 0.22, CI 0.05-1.06). Among papillary thyroid carcinomas, AIT was strongly associated with the diffuse sclerosing variant (OR 4.74, CI 1.33-16.9). AIT was not associated with the extent of local, regional, or distant disease at thyroid cancer diagnosis. Conclusions: AIT is independently associated with an increased risk of thyroid cancer in children with thyroid nodules. These findings suggest that the evaluation of thyroid autoantibodies and thyroid echotexture may inform thyroid cancer risk assessment and surgical decision-making in children with thyroid nodules.

Antibiotic Therapy for Culture-Proven Bacterial Overgrowth in Children With Intestinal Failure Results in Improved Symptoms and Growth.

OBJECTIVES: To evaluate symptoms, enteral tolerance, growth, and antibiotic regimens in pediatric intestinal failure (IF) patients after treated with antibiotic therapy for small bowel bacterial overgrowth (SBBO). METHODS: Single-center retrospective review of children 0-18 years with IF with endoscopic cultures demonstrating >10 5 CFU/mL from 2010 to 2017. Symptoms, enteral tolerance, growth, and antibiotic regimens were evaluated at the time of endoscopy and 6 months later. RESULTS: Of 505 patients followed in our intestinal rehabilitation program, 104 underwent upper gastrointestinal endoscopy and 78 had positive duodenal cultures. Clinical data pre- and post-endoscopy were available for 56 patients. Compared to baseline, in the 6 months following targeted antibiotic treatment, children showed significant improvement in emesis or feeding intolerance (58.9% vs 23.2%, P < 0.001), abdominal pain (16.1% vs 7.1%, P = 0.02), high stool output (42.9% vs 19.6%, P = 0.002), and gross GI bleeding (19.6% vs 3.6%, P = 0.003). Mean BMI-for-age z scores increased significantly (-0.03 ± 0.94 vs 0.27 ± 0.82, P = 0.03); however, height-for-age z scores, weight-for-age z scores, and percent of calories from enteral intake were not significantly different after therapy. Antibiotic regimens remained highly variable. CONCLUSIONS: Children with IF and culture-positive SBBO showed significant improvement in symptoms and BMI-for-age z scores after duodenal culture with subsequent targeted antibiotic therapy. Longer follow-up may be needed to detect improvements in linear growth and percent of calories from enteral feeds. Antibiotic regimens remain highly variable. Long-term consequences of chronic antimicrobial therapy, including antimicrobial resistance, remain unknown. Prospective studies focused on standardizing duodenal sampling technique, correlating culture and pathology data, and evaluating antibiotic resistance patterns are needed.

Current outcomes of infants with esophageal atresia and tracheoesophageal fistula: A multicenter analysis.

OBJECTIVE: This study aims to quantify mortality rates and hospital lengths of stay (LOS) in neonates with esophageal atresia and tracheoesophageal fistula (EA/TEF), and to characterize the effects of birth weight (BW) and associated congenital anomalies upon these. METHODS: Data regarding patients with EA/TEF were prospectively collected (2013-2019) at 298 North American centers. The primary outcome was mortality and secondary outcome was LOS.  Risk factors affecting mortality and LOS were assessed. RESULTS: EA/TEF was diagnosed in 3290 infants with a median BW of 2476 g (IQR 1897,2970). In-hospital mortality was 12.7%. Mortality was inversely correlated with BW. After adjustment, the risk of mortality decreased by approximately 11% with every 100 g increase in BW. A significant congenital anomaly other than EA/TEF was diagnosed in 37.9% of patients. Risk of mortality increased in patients with associated congenital anomalies, most notably in those with a severe cardiac anomaly. Lower BW was associated with an increased mean LOS among survivors. Similar to mortality risk, additional anomalies were associated with prolonged LOS. CONCLUSIONS: This study demonstrates an in-hospital mortality of over 10%. Both increased mortality and prolonged LOS are highly associated with lower birth weight and the presence of concomitant congenital anomalies.

Puberty and growth in patients with pediatric intestinal failure.

BACKGROUND: Pediatric intestinal failure (PIF) affects nutrition, metabolism, and endocrine development, but its downstream impact on puberty is unknown. METHODS: A retrospective review was performed of patients age >8 years with PIF managed at an intestinal rehabilitation program. Outcomes of interest were peak height velocity (PHV), age at PHV, and age at pubertal onset (Tanner stage 2). Outcomes were stratified by sex and compared to established norms. RESULTS: Of 110 patients with PIF, 54.5% were male. Compared to the CDC 50th percentile, PHV in PIF patients was similar for females (8.09±2.36 vs. 7.37 cm/yr;p = 0.23) but significantly higher for males (9.27±2.56 vs. 7.91 cm/yr;p = 0.038). Age at PHV in PIF patients was significantly younger for both males (12.31±2.14 vs. 13.38 years;p = 0.049) and females (10.70±1.06 vs. 11.71 years;p = 0.001). PIF patients reached pubertal onset earlier than published norms; this was significant for males (12.41±1.80 vs. 13.44 years;p = 0.014), but not for females (10.45±1.81 vs. -11.15 years;p = 0.13). The mean height-for-age Z-score was -1.2, with 20% of patients having a Z-score less than -2. CONCLUSIONS: Pubertal onset and growth are neither delayed nor diminished in patients with PIF. The high incidence of short stature, however, highlights the importance of optimizing prepubertal linear growth to attain full height potential. TYPE OF STUDY: Prognosis study (Retrospective cohort study).

Morbidity associated with laparotomy-confirmed spontaneous intestinal perforation: A prospective multicenter analysis.

BACKGROUND: Differences in morbidities between spontaneous intestinal perforation (SIP) and necrotizing enterocolitis (NEC) are unknown. METHODS: Prospectively collected multicenter data regarding very low birth weight (VLBW) infants 2015-2019 were analyzed. Diagnosis of SIP or NEC was laparotomy-confirmed in all patients. Multivariable regression modeling was used to assess adjusted length of stay (LOS; primary outcome) and adjusted risk ratios (ARR) for weight <10th percentile at discharge, and supplemental oxygen requirement at discharge. RESULTS: Of 201,300 VLBW infants at 790 hospitals, 1523 had SIP and 2601 had NEC. Adjusted LOS was similar for SIP and NEC (92 vs 88 days, p = 0.08561), but significantly higher than seen without SIP or NEC (68 days, p<0.0001). The risk of growth morbidity at discharge was similar between SIP and NEC (74.2% vs 75.3%; ARR:1.00;0.94,1.06), but higher than infants without SIP or NEC (47.7%; ARR:0.50;0.47,0.53). Infants with NEC were less likely to require supplemental oxygen at discharge than infants with SIP (24.4% vs 34.9%; ARR:0.80; 0.71,0.89). CONCLUSIONS: Although mortality is known to be lower in VLBW infants with SIP than NEC, this study highlights the similarly high morbidity experienced by both groups of infants. These benchmark data can help align counseling of families with expected outcomes. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Prognosis study (Cohort Study).

Long-term assessment of bilirubin and transaminase trends in pediatric intestinal failure patients during the era of hepatoprotective parenteral nutrition.

PURPOSE: This study aimed to characterize the relationship between hepatoprotective parenteral nutrition (PN) dependence and long-term serum liver tests in children with intestinal failure (IF). METHODS: A retrospective review was performed of children with severe IF (> 90 consecutive days of PN) who were followed from 2012 to 2019 at a multidisciplinary intestinal rehabilitation program. Patients were stratified into three groups based on level of PN dependence at most recent follow up: EN (achieved enteral autonomy), mixed (parenteral and enteral nutrition), and PN (> 75% of caloric intake from PN). PN at any point for this cohort was hepatoprotective, defined as soy-based lipids < 1.5 g/kg/day, combination (soy, medium chain fatty acid, olive and fish oil) lipid emulsion, or fish oil-based lipid emulsion. Kaplan-Meier analysis and a generalized estimating equation (GEE) model were utilized to estimate time to normalization and trends, respectively, of two serum markers of liver health: direct bilirubin (DB) and alanine aminotransferase (ALT). RESULTS: The study included 123 patients (67 EN, 32 mixed, 24 PN). Median follow up time was 4 years. Based on the Kaplan Meier curve, 100% of EN and mixed group patients achieved normal DB levels by 3 years, while 32% of the PN group had elevated DB levels (Fig. 1). At 5 years, 16% of EN patients had elevated ALT levels compared to 73% of PN patients (p < 0.001, Fig. 2). The PN group's ALT levels were 1.76-fold above normal at 3 years (95%CI 1.48-2.03) and 1.65-fold above normal at 5 years (95%CI 1.33-1.97, Fig. 3). CONCLUSIONS: While serum bilirubin levels tend to normalize, long-term PN dependence in the era of hepatoprotective PN is associated with a persistent transaminase elevation in an overwhelming majority of patients. These data support continued vigilant monitoring of liver health in children with intestinal failure. LEVEL OF EVIDENCE: III.

Evaluation of Anemia and Nutritional Status on Children Undergoing Resection of Primary Liver Tumors.

INTRODUCTION: Complete tumor resection of primary malignant liver tumors offers the best chance of survival. However, many of these children may experience anemia and failure to thrive. This study analyzes the association of preoperative anemia and nutritional support with outcomes in children undergoing major resection of primary malignant liver tumors. METHODS: Using the National Surgical Quality Improvement Program Pediatric database from 2012 to 2015, children undergoing major liver resections for primary malignant hepatic tumors were selected. Patient demographics, comorbidities, and 30-d outcomes were compared with respect to the presence of preoperative anemia and the need for nutritional support. Outcomes included 30-d postoperative complications, perioperative blood transfusions, and hospital readmissions. Propensity score matching was performed to control for significant confounders. RESULTS: One hundred ten children were included, 76 (69.1%) with preoperative anemia, and 36 (32.7%) receiving nutritional support. Anemia was associated with preoperative chemotherapy (P = 0.02) and steroids (P = 0.03). Nutritional support was associated with cardiac (P = 0.01), respiratory (P < 0.01), neurologic (P < 0.01), and hematologic comorbidities (P = 0.02). There were 20 (18.2%) postoperative complications and 6 (5.5%) hospital readmissions. After propensity score matching, there was no difference in complications between anemic and nonanemic patients (P = 0.13). Preoperative nutritional support was associated with an increased rate of complications (P < 0.01). Neither anemia (P = 1.00) nor nutritional support (P = 0.49) were associated with readmissions. CONCLUSIONS: The need for nutritional support is common in children undergoing resection of primary malignant hepatic tumors. Anemia was not significantly associated with postoperative complications. In this study, nutritional support was associated with an increased risk of postoperative complications. The need for nutritional support may warrant special attention to the patient's overall conditioning during operative planning.

Analysis of risk factors for morbidity in children undergoing the Kasai procedure for biliary atresia.

OBJECTIVE: To evaluate the perioperative risk factors for 30-day complications of the Kasai procedure in a large, cross-institutional, modern dataset. STUDY DESIGN: The 2012-2015 National Surgical Quality Improvement Program Pediatric database was used to identify patients undergoing the Kasai procedure. Patients' characteristics were compared by perioperative blood transfusions and 30-day outcomes, including complications, reoperations, and readmissions. Multivariable logistic regression was used to identify risk factors predictive of outcomes. Propensity matching was performed for perioperative blood transfusions to evaluate its effect on outcomes. RESULTS: 190 children were included with average age of 62 days. Major cardiac risk factors were seen in 6.3%. Perioperative blood transfusions occurred in 32.1%. The 30-day post-operative complication rate was 15.8%, reoperation 6.8%, and readmission 15.3%. After multivariate analysis, perioperative blood transfusions (OR 3.94; p < 0.01) and major cardiac risk factors (OR 7.82; p < 0.01) were found to increase the risk of a complication. Perioperative blood transfusion (OR 4.71; p = 0.01) was associated with an increased risk of reoperation. Readmission risk was increased by prematurity (OR 3.88; p = 0.04) and 30-day complication event (OR 4.09; p = 0.01). After propensity matching, perioperative blood transfusion was associated with an increase in complications (p < 0.01) and length of stay (p < 0.01). CONCLUSION: Major cardiac risk factors and perioperative blood transfusions increase the risk of post-operative complications in children undergoing the Kasai procedure. Further research is warranted in the perioperative use of blood transfusions in this population. LEVEL OF EVIDENCE: IV.