Outcomes in gastroschisis: expectations in the postnatal period for simple vs complex gastroschisis.

Objective: To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling.

Study Design: A retrospective study of 394 neonates with gastroschisis at 11 children's hospitals from January 2013 to March 2017 was performed. Analysis by Fisher's exact tests and Wilcoxon rank sum tests were performed.

Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis.

Purpose: The impact of thoracoscopic surgery on outcomes in children with congenital lung malformations (CLM) remains controversial. The purpose of this study was to determine the effect of operative approach on perioperative outcomes in infants undergoing lobectomy for an asymptomatic CLM.

Methods: After IRB approval, a retrospective cohort study was conducted on 506 children with a CLM resected at one of eleven children's hospitals over a seven-year period.

Immature teratoma in an adolescent with Proteus syndrome: A novel association.

Proteus syndrome (PS) is a complex disorder characterized by variable clinical findings of overgrowth and tumor susceptibility. This report presents the first known association between PS and an ovarian germ cell tumor in an adolescent with immature teratoma. A review of the diagnosis of PS and associated tumors is included.

Pleuropulmonary Blastoma in Pediatric Lung Lesions.

Background: Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy.

Methods: A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children's hospitals in the United States.

Does Use of a Feeding Protocol Change Outcomes in Gastroschisis? A Report from the Midwest Pediatric Surgery Consortium.

Introduction:  Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol.

Clinical outcomes following implementation of a management bundle for esophageal atresia with distal tracheoesophageal fistula.

Background/purpose: This study evaluated compliance with a multi-institutional quality improvement management protocol for Type-C esophageal atresia with distal tracheoesophageal fistula (EA/TEF).

Methods: Compliance and outcomes before and after implementation of a perioperative protocol bundle for infants undergoing Type-C EA/TEF repair were compared across 11 children's hospitals from 1/2016-1/2019. Bundle components included elimination of prosthetic material between tracheal and esophageal suture lines during repair, not leaving a transanastomotic tube at the conclusion of repair (NO-TUBE), obtaining an esophagram by postoperative-day-5, and discontinuing prophylactic antibiotics 24 h postoperatively.

Sutureless vs sutured abdominal wall closure for gastroschisis: Operative characteristics and early outcomes from the Midwest Pediatric Surgery Consortium.

Purpose: To report outcomes of sutured and sutureless closure for gastroschisis across a large multi-institutional cohort.

Methods: A retrospective study of infants with uncomplicated gastroschisis at 11 children's from 2014 to 2016 was performed. Outcomes of sutured and sutureless abdominal wall closure were compared.

Changing the Paradigm for Management of Pediatric Primary Spontaneous Pneumothorax: A Simple Aspiration Test Predicts Need for Operation.

Purpose: Chest tube (CT) management for pediatric primary spontaneous pneumothorax (PSP) is associated with long hospital stays and high recurrence rates. To streamline management, we explored simple aspiration as a test to predict need for surgery.

Methods: A multi-institution, prospective pilot study of patients with first presentation for PSP at 9 children's hospitals was performed.

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium.

Purpose: The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium.

Methods: After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015).

Results: Fifty-three (10.

Development of a multi-institutional registry for children with operative congenital lung malformations.

Introduction: The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection.

Methods: After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative.

Results: Eleven children's hospitals contributed 506 patients.

Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution.

Purpose:  The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB).

Materials And Methods:  An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed.

Proximal duodenal obstruction associated with compression from a replaced right hepatic artery.

Replaced right hepatic artery is a well-described normal anatomical variant, not previously associated with acute or chronic abdominal symptoms or long-term sequelae. We report a 15-year-old girl presenting with nearly a decade of symptoms secondary to external compression of the proximal duodenum by the ventral passage of a replaced right hepatic artery. Surgical bypass of the duodenal obstruction significantly improved her symptoms.

High-affinity Dkk1 receptor Kremen1 is internalized by clathrin-mediated endocytosis.

Kremens are high-affinity receptors for Dickkopf 1 (Dkk1) and regulate the Wnt/β-catenin signaling pathway by down-regulating the low-density lipoprotein receptor-related protein 6 (LRP6). Dkk1 competes with Wnt for binding to LRP6; binding of Dkk1 inhibits canonical signaling through formation of a ternary complex with Kremen. The majority of down-regulated clathrin-mediated endocytic receptors contain short conserved regions that recognize tyrosine or dileucine sorting motifs.

Operative considerations in pediatric retroperitoneal teratomas--a review.

Background: Retroperitoneal teratomas account for 5% of teratomas and occur predominantly in infants. The resection of these tumors has been variously reported as easy or difficult. This report presents a series of retroperitoneal teratomas and reviews the literature focusing on the relevant operative considerations in this tumor.

Laparoscopic versus open distal pancreatectomy in the management of traumatic pancreatic disruption.

Purpose: Traumatic pancreatic transection is uncommon. The role of laparoscopy in the setting of this injury has not been well described.

Patients And Methods: Six large-volume pediatric trauma centers contributed patients <18 years of age who underwent a distal pancreatectomy for traumatic pancreatic transection from 2000 to 2010.

Current status of pediatric intestinal failure, rehabilitation, and transplantation: summary of a colloquium.

An international symposium convened September 9-11, 2010, in Chicago to present the state of the art and science of the multidisciplinary care of intestinal failure in children. Medical and surgical management of the child with intestinal failure was presented with a focus on the importance of multidisciplinary intestinal failure management. Issues of timing of referral and benefit risk analysis for intestine "rehabilitation" and transplant were presented.

The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival.

Objective: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes.

Methods: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis.

Embryonic mouse blood flow and oxygen correlate with early pancreatic differentiation.

The mammalian embryo represents a fundamental paradox in biology. Its location within the uterus, especially early during development when embryonic cardiovascular development and placental blood flow are not well-established, leads to an obligate hypoxic environment. Despite this hypoxia, the embryonic cells are able to undergo remarkable growth, morphogenesis, and differentiation.

Late presentation of congenital diaphragmatic hernia in patients with cystic fibrosis.

Late presentation of congenital diaphragmatic hernia (CDH) is unusual, especially in patients with cystic fibrosis (CF). To our knowledge, cases of CDH in CF patients and the combined effects on lung function have not been previously described. Here we report two cases of late presenting CDH in CF patients and describe effects on lung function.

Induction of fibroblast growth factor 10 (FGF10) in the ileal crypt epithelium after massive small bowel resection suggests a role for FGF10 in gut adaptation.

We have previously reported that fibroblast growth factor 10 (FGF10) is crucial for the survival and proliferation of progenitor cells during embryonic gastrointestinal development. We sought to characterize the potential role of FGF10 signaling in the adaptive response following small bowel resection. Adult wild-type and Fgf10(LacZ) mice underwent 50% small bowel resection (SBR) or sham operation.

Primary gastric tumors of infancy and childhood: 54-year experience at a single institution.

Background/purpose: Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature.

Interdisciplinary management of pediatric intestinal failure: a 10-year review of rehabilitation and transplantation.

Management of children with intestinal failure is optimized by interdisciplinary coordination of parenteral and enteral nutrition support, medical management of associated complications, surgical lengthening procedures, and intestinal transplantation. Three hundred eighty-nine pediatric patients have been referred to our center for interdisciplinary assessment of intestinal failure since 1996 (median age=1 year; range 1 day-28.8 years).

Fibroblast growth factor 10 is required for survival and proliferation but not differentiation of intestinal epithelial progenitor cells during murine colon development.

Epithelial-mesenchymal interactions that govern the development of the colon from the primitive gastrointestinal tract are still unclear. In this study, we determine the temporal-spatial expression pattern of Fibroblast growth factor 10 (Fgf10), a key developmental gene, in the colon at different developmental stages. We found that Fgf10 is expressed in the mesenchyme of the distal colon, while its main receptor Fgfr2-IIIb is expressed throughout the entire intestinal epithelium.

The fibroblast growth factor pathway serves a regulatory role in proliferation and apoptosis in the pathogenesis of intestinal atresia.

Background/purpose: Intestinal atresia occurs in 1:5000 live births and is a neonatal challenge. Fibroblast growth factor receptor 2b (Fgfr2b) is a critical developmental regulator of proliferation and apoptosis in multiple organ systems including the gastrointestinal tract (GIT). Fgfr2b invalidation results in an autosomal recessive intestinal atresia phenotype.

Treatment of infantile choriocarcinoma of the liver.

Primary choriocarcinoma of the liver is an extremely rare childhood malignancy frequently associated with clinical instability at initial presentation. It often mimics other benign and malignant childhood liver tumors. Prompt diagnosis and initiation of treatment are necessary to attain a successful outcome.