A Multi-Institutional Study Comparing Stoma Location in Neonates With Intestinal Perforation.

INTRODUCTION: Neonates with intestinal perforation often require laparotomy and intestinal stoma creation, with the stoma placed in either the laparotomy incision or a separate site. We aimed to investigate if stoma location is associated with risk of postoperative wound complications. METHODS: A multi-institutional retrospective review was performed for neonates ≤3 mo who underwent emergent laparotomy and intestinal stoma creation for intestinal perforation between January 1, 2009 and April 1, 2021. Patients were stratified by stoma location (laparotomy incision versus separate site). Outcomes included wound infection/dehiscence, stoma irritation, retraction, stricture, and prolapse. Multivariable regression identified factors associated with postoperative wound complications, controlling for gestational age, age and weight at surgery, and diagnosis. RESULTS: Overall, 79 neonates of median gestational age 28.8 wk (interquartile range [IQR]: 26.0-34.2 wk), median age 5 d (IQR: 2-11 d) and median weight 1.4 kg (IQR: 0.9-2.42 kg) had perforated bowel from necrotizing enterocolitis (40.5%), focal intestinal perforation (31.6%), or other etiologies (27.8%). Stomas were placed in the laparotomy incision for 41 (51.9%) patients and separate sites in 38 (48.1%) patients. Wound infection/dehiscence occurred in 7 (17.1%) neonates with laparotomy stomas and 5 (13.2%) neonates with separate site stomas (P = 0.63). There were no significant differences in peristomal irritation, stoma retraction, or stoma stricture between the two groups. On multivariable regression, separate site stomas were associated with increased likelihood of prolapse (odds ratio 6.54; 95% confidence interval: 1.14-37.5). CONCLUSIONS: Stoma incorporation within the laparotomy incision is not associated with wound complications. Separate site stomas may be associated with prolapse. Patient factors should be considered when planning stoma location in neonates undergoing surgery for intestinal perforation.

Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.

BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare malformation in which splanchnic venous flow bypasses the liver. CPSS is associated with other congenital anomalies and syndromes and can be associated with life-threatening complications. CPSS and their management remain underreported in the literature. Here, we review the clinical characteristics, management, and outcomes of a cohort of children and young adults with CPSS from two pediatric centers. METHODS: Cases of CPSS from Cincinnati Children's Hospital Medical Center and C.S. Mott Children's Hospital were reviewed to define CPSS anatomy, associated anomalies, complications, interventions, and outcomes. The imaging features and histopathology of liver lesions were characterized in detail. RESULTS: A total of 11 cases were identified. Median age was 10 years (range 0-26); 8 (73%) cases were female. Associated anomalies included six patients with heterotaxy (55%), five patients with congenital heart disease (45%), three patients with Turner syndrome (27%), and two patients with omphalocele, exstrophy, imperforate anus, spinal defects (OEIS) complex (18%). Eight (73%) cases had hyperammonemia ± encephalopathy. A 4-month-old presented with hepatopulmonary syndrome, and 12-year-old presented with pulmonary hypertension. Eight patients (73%) had liver lesions including five with premalignant adenomas and three with well-differentiated hepatocellular carcinoma (HCC). Four children underwent successful CPSS occlusion/ligation. Three children underwent liver transplant (2) or resection (1) for HCC without recurrence at extended follow-up. CONCLUSIONS: CPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.

Laparoscopic donor nephrectomy for the pediatric recipient population: Risk factors for adverse outcomes.

Kidney transplantation is the optimal treatment of ESRD in children. Some studies have reported inferior outcomes in recipients of LDN allografts who are ≤ 5 yr of age. We performed a retrospective review of pediatric recipient outcomes of 110 LDN allografts at our institution and examined predictors of adverse outcomes. Subgroup analysis was performed by dividing recipients into three age categories: 0-5 yr, 6-17 yr, and ≥ 18 yr. There was no significant difference between incidences of DGF or ARE between groups. Kaplan-Meier analysis demonstrated 100% allograft survival in 0- to 5-yr-old recipients, nearly reaching statistical significance (p = 0.07) for outcome superior to that of the two older age groups. Pretransplant HD was associated with increased risk of DGF (p = 0.05). Significant risk factors for ARE were recipient weight >15 kg (p = 0.033) and multiple renal arteries (p = 0.047). Previous ARE was associated with an increased risk of allograft failure (p = 0.02). LDN is not associated with increased risk of DGF, ARE, or allograft failure in the youngest recipients. These findings support an aggressive pursuit of preemptive transplantation even in the youngest pediatric allograft recipients.

Management of hepatoblastoma: an update.

PURPOSE OF REVIEW: To summarize the current standards and guidelines for the diagnosis and management of hepatoblastoma, a rare pediatric liver tumor. RECENT FINDINGS: Hepatoblastoma is the most common malignant liver tumor in childhood. International collaborative efforts have led to uniform implementation of the pretreatment extent of disease (PRETEXT) staging system as a means to establish consensus classification and assess upfront resectability. Additionally, current histopathological classification, in light of more advanced molecular profiling and immunohistochemical techniques and integration of tumor biomarkers into risk stratification, is reviewed. Multimodal therapy is composed of chemotherapy and surgical intervention. Achievement of complete surgical resection plays a key role in successful treatment for hepatoblastoma. Overall, outcomes have greatly improved over the past four decades because of advances in chemotherapeutic agents and administration protocols as well as innovations of surgical approach, including the use of vascular exclusion, ultrasonic dissection techniques, and liver transplantation. Challenges remain in management of high-risk patients as well as patients with recurrent or metastatic disease. SUMMARY: Eventually, a more individualized approach to treating the different types of the heterogeneous spectrum of hepatoblastoma, in terms of different chemotherapeutic protocols and timing as well as type and extent of surgery, may become the basis of successful treatment in the more complex or advanced types of hepatoblastoma.

Role of myeloid differentiation factor 88 in Rhesus rotavirus-induced biliary atresia.

BACKGROUND: Biliary atresia (BA) is a unique neonatal disease resulting from inflammatory and fibrosing obstruction of the extrahepatic biliary tree. Previous studies have demonstrated the critical role of innate immunity and the Th1 response to activated inflammatory cells and overexpressed cytokines in the pathogenesis of BA. Myeloid differentiation factor 88 (MyD88) is a critical adaptor molecule that has been shown to play a crucial role in immunity. We investigated the role of MyD88 in the inflammatory response and development of cholangiopathy in murine BA. METHODS: MyD88 knockout (MyD88(-/-)) and wild-type (WT) BALB/c pups were injected with Rhesus rotavirus or saline on day 1 of life. The mice were monitored for clinical symptoms of BA, including jaundice, acholic stools, bilirubinuria, and death. The liver and extrahepatic bile ducts were harvested for histologic evaluation and the quantification of viral content, determination of cytokine expression, and detection of inflammatory cells. RESULTS: Rhesus rotavirus infection produced symptoms in 100% of both MyD88(-/-) and WT pups, with survival of 18% of WT and 0% of MyD88(-/-) mice. Histologic analysis demonstrated bile duct obstruction in both MyD88(-/-) and WT mice. Viral titers obtained 7 d after infection and expression of interferon-γ and tumor necrosis factor-α at day 3, 5, 8, and 12 after infection revealed no significant differences between the WT and MyD88(-/-) mice. Flow cytometry demonstrated similar levels of activated CD8+ T cells and natural killer cells. CONCLUSIONS: The pathogenesis of murine BA is independent of the MyD88 signaling inflammatory pathway, suggesting alternative mechanisms are crucial in the induction of the model.

Critical elements of pediatric liver cancer surgery.

The appropriate management of pediatric liver malignancies, primarily hepatoblastoma and hepatocellular carcinoma, requires an in depth understanding of contemporary preoperative risk stratification, experience with advanced hepatobiliary surgery, and a good relationship with one's local or regional liver transplant center. While chemotherapy regimens have become more effective, operative indications more well-defined, and overall survival improved, the complexity of liver surgery in small children provides ample opportunity for protocol violation, inadequate resection, and iatrogenic morbidity. These guidelines represent the distillation of contemporary literature and expert opinion as a means to provide a framework for preoperative planning and intraoperative decision-making for the pediatric surgeon.

Pediatric and adult trauma centers differ in evaluation, treatment, and outcomes for severely injured adolescents.

BACKGROUND/PURPOSE: This study aims to investigate differences in imaging, procedure utilization, and clinical outcomes of severely injured adolescents treated at adult versus pediatric trauma centers. METHODS: The National Trauma Data Bank was queried retrospectively for adolescents, 15-19years old, with a length of stay (LOS) >1day and Injury Severity Score (ISS) >25 treated at adult (ATC) or pediatric (PTC) Level 1 trauma centers from 2007 to 2011. Patient demographics and utilization of imaging and procedures were analyzed. Univariate and multivariate regression analysis was used to compare outcomes. RESULTS: Of 12,861 adolescents, 51% were treated at ATC. Older age and more nonwhites were seen at ATC (p<0.01). Imaging and invasive procedures were more common at ATC (p<0.01). Shorter LOS (p=0.03) and higher home discharge rates (p<0.01) were seen at PTC. ISS and mortality did not differ. Age, race, ATC care (all p<0.01), and admission systolic blood pressure (SBP) (p=0.03) were predictors of CT utilization. ISS, SBP, and race (p<0.01) were risk factors for overall mortality; SBP (p=0.03) and ISS (p<0.01) predicted death from penetrating injury. CONCLUSIONS: Severely injured adolescents experience improved outcomes and decreased imaging and invasive procedures without additional mortality risk when treated at PTC. PTC is an appropriate destination for severely injured adolescents.

Teen trauma without the drama: outcomes of adolescents treated at Ohio adult versus pediatric trauma centers.

BACKGROUND: The optimal treatment facility for adolescent trauma patients is controversial. We sought to investigate risk-adjusted outcomes of adolescents treated at adult-only trauma centers (ATCs) versus pediatric-only trauma centers (PTCs) in a state system with legislated American College of Surgeons-verified institutions to determine ideal prehospital referral patterns. METHODS: The Ohio Trauma Registry was queried for patients 15 years to 19 years with a length of stay (LOS) greater than 1 day at ATC (Level 1) or PTC (Levels 1 and 2) from 2008 to 2012. Race, sex, emergency department vital signs, Injury Severity Score (ISS), computed tomography, and ultrasound imaging were reviewed. Outcomes by mechanism of injury included ventilator days, intensive care unit LOS, hospital LOS, and mortality. Statistical analysis was performed using χ test, t test, and Wilcoxon rank-sum test. Propensity score-based risk adjustment matching was used to compare groups (propensity score within 0.01, ISS within 5). RESULTS: Of 5,793 adolescents examined, (84% blunt, 16% penetrating) 66% were treated at an ATC. In unmatched comparisons, age, ISS, vital signs, and mortality differed significantly between centers (p < 0.01). For adolescents with blunt injury, more males (71.6% vs. 66.3%, p < 0.01) and nonwhites (19.2% vs. 15.8%, p < 0.01) were seen at PTCs. For penetrating trauma, more males (88.6% vs. 50.8%, p < 0.01) and nonwhites (66.4% vs. 34.3%, p < 0.01) were admitted to ATCs. In 873 propensity-matched pairs for blunt trauma and 95 propensity-matched pairs of penetrating injuries, no differences were seen in a priori outcomes. Imaging (blunt, head computed tomography and abdominal ultrasound, p < 0.01; penetrating, abdominal ultrasound, p = 0.02) was more common at ATCs. CONCLUSION: Major outcome differences for injured adolescents do not exist between ATCs and PTCs, regardless of injury pattern. Imaging remains more prevalent at ATCs. In a state system with mandatory American College of Surgeons-verified centers, injury patterns need not dictate triage decisions for adolescents. LEVEL OF EVIDENCE: Epidemiologic study, level III.