Utility of 3-dimensional modeling in prenatally diagnosed large fetal neck mass.

INTRODUCTION: Prenatally diagnosed large fetal neck mass requires multidisciplinary consultation and evaluation of perinatal treatment options. The decision to perform Ex-utero Intrapartum Treatment (EXIT) is based on risk-benefit assessment for both the infant and mother. Though fetal ultrasound and MRI assist with operative planning, a three-dimensional anatomic model offers improved anatomic visualization and prenatal patient counseling. CASE PRESENTATION: Multiple surveillance ultrasound exams between gestational weeks 16 and 32, plus fetal MRI at 29/3 weeks were performed for fetal evaluation. A 3-dimensional model was printed (Form 3 and 3L, clear resin, Formlabs) incorporating fetal MRI (Ax SSFSE TE 100 DL and Cor SSFSE Brain DL) and using Mimics Medical and 3-matic Medical software (Materialise). A left fetal multicystic neck mass measuring 2.1x1.8x1.5 cm was diagnosed at 16/6 weeks gestation in a G8P2416. Fetal MRI performed at 29/3 weeks showed a large exophytic mixed solid-cystic cervicofacial mass, 10.3 x 9.4 x 10.6 cm arising from the left mandible, concerning for a teratoma. Prior to delivery, the model was used to educate and counsel the family regarding the complex clinical situation and the reasoning for delivery via EXIT followed by mass resection. Additionally, the model demonstrated tracheal narrowing and oropharyngeal compression, supporting airway intervention planning. The fetus was delivered at 32/3 weeks via EXIT to intubation using rigid bronchoscopy. Duration of time on placental support was 12 minutes. On day of life 5, the patient underwent resection of the cervical mass. Pathology revealed an immature teratoma, histologic grade 3 without yolk sac elements, and negative regional lymph nodes. CONCLUSION: Three-dimensional fetal modeling facilitates perinatal airway needs assessment, patient counseling, delivery, and post¬natal management.

Management of twin reversed arterial perfusion (TRAP) sequence with microwave ablation: A case report and review of the literature.

Twin reversed arterial perfusion (TRAP) sequence carries a high mortality risk to the "pump twin." Management involves disrupting blood flow to the acardiac mass. In this case, the pregnant patient presented at 20 weeks 6 days with Stage IIb TRAP Sequence and underwent percutaneous ultrasound-guided microwave ablation (MWA) of the acardiac mass at 21 weeks 0 days. The probe traversed the thorax of the acardiac mass and ablated the confluence of the umbilical vessels. A healthy child was delivered at 33 weeks 5 days gestation. This report demonstrates the utility of MWA in TRAP sequence and describes a novel approach.

Indications, Resource Allocation, and Outcomes Associated with Ex-Utero Intrapartum Treatment Procedures: A North American Fetal Therapy Network Survey.

INTRODUCTION: Neonates with cardiorespiratory compromise at delivery are at substantial risk of hypoxic neurologic injury and death. Though mitigation strategies such as ex-utero intrapartum treatment (EXIT) exist, the competing interests of neonatal beneficence, maternal non-maleficence, and just distribution of resources require consideration. Due to the rarity of these entities, there are few systematic data to guide evidence-based standards. This multi-institutional, interdisciplinary approach aims to elucidate the current scope of diagnoses that might be considered for such treatments and examine if treatment allocation and/or outcomes could be improved. METHODS: After IRB approval, a survey investigating diagnoses appropriate for EXIT consultation and procedure, variables within each diagnosis, occurrence of maternal and neonatal adverse outcomes, and instances of suboptimal resource allocation in the last decade was sent to all North American Fetal Treatment Network center representatives. One response was recorded per center. RESULTS: We received a 91% response rate and all but one center offer EXIT. Most centers (34/40, 85%) performed 1-5 EXIT consultations per year and 17/40 (42.5%) centers performed 1-5 EXIT procedures in the last 10 years. The diagnoses with the highest degree of agreement between centers surveyed to justify consultation for EXIT are head and neck mass (100%), congenital high airway obstruction (90%), and craniofacial skeletal conditions (82.5%). Maternal adverse outcomes were noted in 7.5% of centers while neonatal adverse outcomes in 27.5%. A large percentage of centers report cases of suboptimal selection for risk mitigation procedures and several centers experienced adverse neonatal and maternal outcomes. CONCLUSION: This study captures the scope of EXIT indications and is the first to demonstrate the mismatch in resource allocation for this population. Further, it reports on attributable adverse outcomes. Given suboptimal allocation and adverse outcomes, further examination of indications, outcomes, and resource use is justified to drive evidence-based protocols.

High Mobility Group Box 1 Release by Cholangiocytes Governs Biliary Atresia Pathogenesis and Correlates With Increases in Afflicted Infants.

BACKGROUND AND AIMS: Biliary atresia (BA) is a devastating cholangiopathy of infancy. Upon diagnosis, surgical reconstruction by Kasai hepatoportoenterostomy (HPE) restores biliary drainage in a subset of patients, but most patients develop fibrosis and progress to end-stage liver disease requiring liver transplantation for survival. In the murine model of BA, rhesus rotavirus (RRV) infection of newborn pups results in a cholangiopathy paralleling that of human BA. High-mobility group box 1 (HMGB1) is an important member of the danger-associated molecular patterns capable of mediating inflammation during infection-associated responses. In this study, we investigated the role of HMGB1 in BA pathogenesis. APPROACH AND RESULTS: In cholangiocytes, RRV induced the expression and release of HMGB1 through the p38 mitogen-activated protein kinase signaling pathway, and inhibition of p38 blocked HMGB1 release. Treatment of cholangiocytes with ethyl pyruvate suppressed the release of HMGB1. Administration of glycyrrhizin in vivo decreased symptoms and increased survival in the murine model of BA. HMGB1 levels were measured in serum obtained from infants with BA enrolled in the PROBE and START studies conducted by the Childhood Liver Disease Research Network. High HMGB1 levels were found in a subset of patients at the time of HPE. These patients had higher bilirubin levels 3 months post-HPE and a lower survival of their native liver at 2 years. CONCLUSIONS: These results suggest that HMGB1 plays a role in virus induced BA pathogenesis and could be a target for therapeutic interventions in a subset of patients with BA and high HMGB1.

Rotavirus Reassortant-Induced Murine Model of Liver Fibrosis Parallels Human Biliary Atresia.

BACKGROUND AND AIMS: Biliary atresia (BA) is a devastating neonatal cholangiopathy that progresses to fibrosis and end-stage liver disease by 2 years of age. Portoenterostomy may reestablish biliary drainage, but, despite drainage, virtually all afflicted patients develop fibrosis and progress to end-stage liver disease requiring liver transplantation for survival. APPROACH AND RESULTS: In the murine model of BA, rhesus rotavirus (RRV) infection of newborn pups results in a cholangiopathy paralleling human BA and has been used to study mechanistic aspects of the disease. Unfortunately, nearly all RRV-infected pups succumb by day of life 14. Thus, in this study we generated an RRV-TUCH rotavirus reassortant (designated as TR(VP2,VP4) ) that when injected into newborn mice causes an obstructive jaundice phenotype with lower mortality rates. Of the mice that survived, 63% developed Ishak stage 3-5 fibrosis with histopathological signs of inflammation/fibrosis and bile duct obstruction. CONCLUSIONS: This model of rotavirus-induced neonatal fibrosis will provide an opportunity to study disease pathogenesis and has potential to be used in preclinical studies with an objective to identify therapeutic targets that may alter the course of BA.

A Point Mutation in the Rhesus Rotavirus VP4 Protein Generated through a Rotavirus Reverse Genetics System Attenuates Biliary Atresia in the Murine Model.

Rotavirus infection is one of the most common causes of diarrheal illness in humans. In neonatal mice, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammatory obstruction of the extrahepatic biliary tract and intrahepatic bile ducts. We previously showed that the amino acid arginine (R) within the sequence SRL (amino acids 445 to 447) in the RRV VP4 protein is required for viral binding and entry into biliary epithelial cells. To determine if this single amino acid (R) influences the pathogenicity of the virus, we generated a recombinant virus with a single amino acid mutation at this site through a reverse genetics system. We demonstrated that the RRV mutant (RRVVP4-R446G) produced less symptomatology and replicated to lower titers both in vivo and in vitro than those seen with wild-type RRV, with reduced binding in cholangiocytes. Our results demonstrate that a single amino acid change in the RRV VP4 gene influences cholangiocyte tropism and reduces pathogenicity in mice.IMPORTANCE Rotavirus is the leading cause of diarrhea in humans. Rhesus rotavirus (RRV) can also lead to biliary atresia (a neonatal human disease) in mice. We developed a reverse genetics system to create a mutant of RRV (RRVVP4-R446G) with a single amino acid change in the VP4 protein compared to that of wild-type RRV. In vitro, the mutant virus had reduced binding and infectivity in cholangiocytes. In vivo, it produced fewer symptoms and lower mortality in neonatal mice, resulting in an attenuated form of biliary atresia.

The SRL peptide of rhesus rotavirus VP4 protein governs cholangiocyte infection and the murine model of biliary atresia.

Biliary atresia (BA) is a neonatal obstructive cholangiopathy that progresses to end-stage liver disease, often requiring transplantation. The murine model of BA, employing rhesus rotavirus (RRV), parallels human disease and has been used to elucidate mechanistic aspects of a virus induced biliary cholangiopathy. We previously reported that the RRV VP4 gene plays an integral role in activating the immune system and induction of BA. Using rotavirus binding and blocking assays, this study elucidated how RRV VP4 protein governs cholangiocyte susceptibility to infection both in vitro and in vivo in the murine model of BA. We identified the amino acid sequence on VP4 and its cholangiocyte binding protein, finding that the sequence is specific to those rotavirus strains that cause obstructive cholangiopathy. Pretreatment of murine and human cholangiocytes with this VP4-derived peptide (TRTRVSRLY) significantly reduced the ability of RRV to bind and infect cells. However, the peptide did not block cholangiocyte binding of TUCH and Ro1845, strains that do not induce murine BA. The SRL sequence within TRTRVSRLY is required for cholangiocyte binding and viral replication. The cholangiocyte membrane protein bound by SRL was found to be Hsc70. Inhibition of Hsc70 by small interfering RNAs reduced RRV's ability to infect cholangiocytes. This virus-cholangiocyte interaction is also seen in vivo in the murine model of BA, where inoculation of mice with TRTRVSRLY peptide significantly reduced symptoms and mortality in RRV-injected mice. CONCLUSION: The tripeptide SRL on RRV VP4 binds to the cholangiocyte membrane protein Hsc70, defining a novel binding site governing VP4 attachment. Investigations are underway to determine the cellular response to this interaction to understand how it contributes to the pathogenesis of BA. (Hepatology 2017;65:1278-1292).

Rhesus rotavirus VP4 sequence-specific activation of mononuclear cells is associated with cholangiopathy in murine biliary atresia.

Biliary atresia (BA), a neonatal obstructive cholangiopathy, remains the most common indication for pediatric liver transplantation in the United States. In the murine model of BA, Rhesus rotavirus (RRV) VP4 surface protein determines biliary duct tropism. In this study, we investigated how VP4 governs induction of murine BA. Newborn mice were injected with 16 strains of rotavirus and observed for clinical symptoms of BA and mortality. Cholangiograms were performed to confirm bile duct obstruction. Livers and bile ducts were harvested 7 days postinfection for virus titers and histology. Flow cytometry assessed mononuclear cell activation in harvested cell populations from the liver. Cytotoxic NK cell activity was determined by the ability of NK cells to kill noninfected cholangiocytes. Of the 16 strains investigated, the 6 with the highest homology to the RRV VP4 (>87%) were capable of infecting bile ducts in vivo. Although the strain Ro1845 replicated to a titer similar to RRV in vivo, it caused no symptoms or mortality. A Ro1845 reassortant containing the RRV VP4 induced all BA symptoms, with a mortality rate of 89%. Flow cytometry revealed that NK cell activation was significantly increased in the disease-inducing strains and these NK cells demonstrated a significantly higher percentage of cytotoxicity against noninfected cholangiocytes. Rotavirus strains with >87% homology to RRV's VP4 were capable of infecting murine bile ducts in vivo. Development of murine BA was mediated by RRV VP4-specific activation of mononuclear cells, independent of viral titers.

Perinatal Airway Management Mandibular Anomalies: A National Inpatient Cohort Analysis.

OBJECTIVE: To characterize incidence of mandibular anomalies (MAs) and compare gestational age, airway interventions, and complications among individuals with MA phenotypes (isolated retrognathia, isolated micrognathia, syndromic micrognathia, micrognathia plus cleft palate/cleft lip and palate, agnathia/micrognathia plus cervical auricle/otocephaly, and agnathia/micrognathia plus microstomia) and unaffected individuals. METHODS: The Healthcare Cost and Utilization Project Kids' Inpatient Database was used to collect data over a 20-year period beginning in 2000. Interventions were classified as perinatal when performed on day of life (DOL) 0 or 1 and subsequent when performed during the birth hospitalization after DOL 1. Hypoxic complications included cardiac arrest, birth asphyxia, hypoxic-ischemic encephalopathy, anoxic brain damage, intraventricular hemorrhage or cerebral infarction. Descriptive statistics are reported, and the Rao-Scott chi-square test compared groups. RESULTS: MAs affected 119 per 100,000 birth visits. Preterm delivery was more frequent for all MA phenotypes. Individuals with MA phenotypes are more likely to require medical attention (airway intervention on DOL 0 or 1 OR no airway intervention received but patient sustained hypoxic complication/mortality): 16.2%-70.7% vs. 3.8%, p < 0.01. Despite receipt of airway interventions at a higher rate, collectively individuals with MAs who received an airway intervention on DOL 0 or 1 have a mildly elevated risk of hypoxic complication or mortality (32.4% vs. 26.4%, p < 0.01). CONCLUSIONS: Preterm birth is more common, however, does not account for the elevated rate of airway intervention. Individuals with MAs require higher rates of medical attention, and current airway management paradigms are insufficient to prevent complications and mortality. LEVEL OF EVIDENCE: III Laryngoscope, 2024.

Perinatal airway management in neonatal goiter: A healthcare cost and utilization project (HCUP) kids' inpatient database analysis.

INTRODUCTION: Fetal goiter is a rare congenital disorder that can present with life-threatening neonatal airway obstruction. Lifesaving and function-preserving airway management strategies are available, but routine delivery affords a limited window for intervention. Accordingly, fetal goiter is reported among the most common indications for ex-utero intrapartum treatment (EXIT). While EXIT prolongs the window for airway intervention to benefit the neonate, it elevates the risk to the pregnant person and requires extensive resources; therefore, data to guide ideal treatment selection are essential. This study aims to compare perinatal airway interventions between individuals with a birth hospitalization discharge diagnosis (BHDD) of goiter and the general population. MATERIALS AND METHODS: Individuals with and without BHDD of goiter were identified in the Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Database from 2000 to 2019. The frequency of airway interventions on day of life (DOL) 0 or 1 were compared using the Rao-Scott chi-square test. Additionally, gestational age, type of intervention, complications, mortality, birth weight, and length of stay were examined for the goiter cohort. RESULTS: Two-hundred eighty-seven weighted cases of goiter were identified in the study period. The population was 61 % male, 55 % White, and median birthweight was 3.3 kg. The median length of stay was 4.3 days, and average total charges were $42,332. Airway intervention on DOL 0 or 1 was performed in 16.9 % of individuals with goiter compared to 1.6 % in neonates without goiter (p < 0.001). Interventions in the goiter cohort included endotracheal intubation in 16 % of cases, laryngoscopy/bronchoscopy in 1-5% of cases, and tracheostomy in <1 % of cases. Fewer than 1 % of individuals undergoing intubation additionally had mass decompression/resection on DOL 0 or 1. No neonates received extracorporeal membrane oxygenation cannulation or cardiopulmonary resuscitation. Hypoxic encephalopathy occurred in <1 % of cases, among which endotracheal intubation was the only airway intervention performed. There were no mortalities among neonates with goiter. CONCLUSION: Individuals with BHDD of goiter receive significantly higher rates of perinatal airway intervention. In most cases, endoscopic interventions alone were sufficient to avoid hypoxic neurological complications. These findings contribute to data to aid in clinical counseling and empower patients to make informed decisions according to their values and treatment goals.

The Current Pediatric Surgery Job Market: A Perspective of Recent Fellowship Graduates.

PURPOSE: This study describes the job market from the perspective of recent pediatric surgery graduates. METHODS: An anonymous survey was circulated to the 137 pediatric surgeons who graduated from fellowships 2019-2021. RESULTS: The survey response rate was 49%. The majority of respondents were women (52%), Caucasian (72%), and had a median student debt burden of $225,000. Considering job opportunities, respondents strongly emphasized camaraderie (93%), mentorship (93%), case mix (85%), geography (67%), faculty reputation (62%), spousal employment (57%), compensation (51%), and call frequency (45%). 30% were satisfied with the employment opportunities available, and 21% felt strongly prepared to negotiate for their first job. All respondents were able to secure a job. Most jobs were university-based (70%) or hospital employed (18%) positions where surgeons covered median of two hospitals. 49% wanted protected research time, and 12% of respondents were able to secure substantial, protected research time. The median compensation for university-based jobs was $12,583 below the median AAMC benchmark for assistant professors for the corresponding year of graduation. CONCLUSION: These data highlight the ongoing need for assessment of the pediatric surgery workforce and for professional societies and training programs to further assist graduating fellows in preparing to negotiate their first job. TYPE OF STUDY: Survey LEVEL OF EVIDENCE: Level V.

Recurrent ileocolic intussusception with appendiceal lead point in a 2-year-old child: an etiology to be aware of on ultrasound.

Ileocolic intussusception is a differential consideration in young pediatric patients presenting with acute abdominal pain. Appendiceal intussusception is an uncommon variant of ileocolic intussusception where the appendix is contained within the intussusception, which can be challenging to diagnose preoperatively. In this case report, we present a 25-month-old female presenting with intermittent, diffuse abdominal pain. Initial ultrasound evaluation demonstrated ileocolic intussusception, which was successfully reduced by air enema. The patient experienced recurrent symptoms and had several recurrent episodes of ileocolic intussusception with the appendix contained within the intussusceptum. After the fifth recurrence, the patient underwent surgical intervention. The inflamed appendix was discovered to be inverted within the cecum, resulting in a lead point for intussusception of the terminal ileum. The patient underwent laparoscopic reduction of the ileocolic intussusception and appendectomy. Few cases of appendiceal and concurrent ileocolic intussusception are described in the literature. It is important that radiologists and surgeons be aware of this entity when evaluating pediatric ileocolic intussusception, particularly at the time of ultrasound and air enema, in order to ensure appropriate management and prevent complications.

The management of pediatric renovascular hypertension: a single center experience and review of the literature.

INTRODUCTION: Renal artery occlusive disease is poorly characterized in children; treatments include medications, endovascular techniques, and surgery. We aimed to describe the course of renovascular hypertension (RVH), its treatments and outcomes. METHODS: We performed literature review and retrospective review (1993-2014) of children with renovascular hypertension at our institution. Response to treatment was defined by National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents at most-recent follow-up. RESULTS: We identified 39 patients with RVH. 54% (n=21) were male, with mean age of 6.93 ± 5.27 years. Most underwent endovascular treatment (n=17), with medication alone (n=12) and surgery (n=10) less commonly utilized. Endovascular treatment resulted in 18% cure, 65% improvement and 18% failure; surgery resulted in 30% cure, 50% improvement and 20% failure. Medication alone resulted in 0% cure, 75% improvement and 25% failure. 24% with endovascular treatment required secondary endovascular intervention; 18% required secondary surgery. 20% of patients who underwent initial surgery required reoperation for re-stenosis. Mean follow-up was 52.2 ± 58.4 months. CONCLUSIONS: RVH treatment in children includes medications, surgical or endovascular approaches, with all resulting in combined 79% improvement in or cure rates. A multidisciplinary approach and individualized patient management are critical to optimize outcomes. TYPE OF STUDY: Retrospective comparative study LEVEL OF EVIDENCE: Level III.

Extracorporeal Membrane Oxygenation in a Patient with Biliary Atresia: Case and Review of Extracorporeal Life Support Organization Data.

Biliary atresia is a newborn cholangiopathy that may lead to portopulmonary hypertension and cirrhosis-induced cardiomyopathy while awaiting liver transplantation. Extracorporeal life support and hepatic toxin filtration are life-saving interventions that provide cardiopulmonary support and hepatic dialysis to allow resolution of a child's illness. We utilized a combination of these extreme measures to bridge an infant with biliary atresia to transplantation. We reviewed cases of extracorporeal life support utilization in transplantation recipients in the Extracorporeal Life Support Organization database and determined that ours was the only use of pretransplant extracorporeal life support in biliary atresia.

Surgical management and surveillance of pediatric appendiceal carcinoid tumor.

PURPOSE: Appendiceal carcinoid tumors are rare neuroendocrine neoplasms. The aim of this study was to determine if postoperative oncologic follow-up was necessary for this tumor. METHODS: A retrospective review was performed of patients with appendiceal carcinoid 2000-2015. RESULTS: 8382 patients underwent appendectomy 2000-2015. 30 (0.3%) had appendiceal carcinoid. 70% were female (n=21) with an average age of 13.5±2.8 years (range 8-18). Most presented with abdominal pain (n=29, 97%). 20% (n=6) had appendiceal perforation. Mean tumor size was 5.4±4mm (range microscopic - 15mm) with most at the appendiceal tip (n=18, 60%). No node infiltration was found, although 10% (n=3) had perineural and 3% (n=1) had lymphovascular invasion. Five were transmural (17%). Most patients were referred to oncology (n=19, 63%) for staging and surveillance including ultrasonography (n=11, 65%), MRI (n=7, 41%), and CT (n=6, 35%). The majority (79%, n=15) underwent serial 5-HIAA testing. All surveillance was found to be normal, and no patients required further treatment. Mean follow-up was 36±34 months, with 58% (n=11) continuing surveillance. Medical charges ranged $8500-$44,000. No recurrences have been identified. CONCLUSION: Appendectomy is an adequate treatment for pediatric appendiceal carcinoid <16 mm despite presence of histological risk factors. More aggressive surgery and extensive oncologic follow up are of limited value. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Retrospective comparative study.

Long-term morbidity after staging laparotomy for Hodgkin lymphoma.

BACKGROUND: A large cohort of Hodgkin lymphoma (HL) survivors exist. With patients transitioning from pediatric to adult care, practitioners should be aware of potential complications. The aim of this study was to describe the long-term complications of patients who had staging laparotomy for the treatment of HL. METHODS: After institutional review board approval, a retrospective review of hospital records at our institution was performed. Data extracted included demographics, treatment course and long-term postoperative complications. RESULTS: 24 patients with HL underwent staging laparotomy from 1971 to 1994 with median follow-up of 27.9years. Six (33%) had intraabdominal disease. Three patients (17%) required four repeat laparotomies for bowel obstruction. Of these, one had radiation to the inguinal region for local control, one had mantle radiation. Five patients developed a second malignancy. There were no documented cases of postsplenectomy sepsis. Other late effects that were unlikely related to surgery included pulmonary fibrosis (4), heart failure (2), hypothyroidism (4), and dysphagia (3). One patient died of metastatic adenocarcinoma. CONCLUSIONS: Long-term follow-up of patients who underwent staging laparotomy for HL revealed an increased incidence of repeat laparotomy and secondary malignancy. This underscores the importance of a high index of suspicion and screening in this population. LEVEL OF EVIDENCE: Level III.

Cystic Biliary Atresia and Choledochal Cysts Are Distinct Histopathologic Entities.

Cystic biliary atresia (CBA), a rare cystic expansion of atretic extrahepatic bile ducts in young infants, overlaps in age at presentation and imaging features with early choledochal cysts (CC). Treatment and prognosis differ; histologic differences are unsettled. We compared 10 patients with CBA, 1975 to 2015, to an age-similar cohort of 13 infants, and to older patients who had surgery for CC. Operative details, imaging, and clinical courses were correlated to pathologic specimens. Immunostains for smooth muscle actin and myosin heavy chain were used to evaluate cyst walls and atretic segments. CBA cysts typically lacked epithelium and inflammation; cyst walls had an inner, dense cicatricial layer associated with myofibroblastic (MF) hyperplasia that often delaminated producing a grossly visible inner cyst wall. Seven proximal biliary remnants in CBA featured circumferential peribiliary MF hyperplasia/fibrosis with little or no inflammation, similar to isolated BA. Extrahepatic atresia was usually both proximal and distal to the cyst. Features in 10/13 CC from infants and 8/8 CC in older patients had mostly preserved uninjured epithelium and no subepithelial cicatrix. Mural smooth muscle (absent in CBA) was present to some extent in CC at all ages. Unexpectedly, focal MF hyperplasia and laminar sclerosis was present in a few CC in infants, resembling CBA. CBA and infant CC are distinct histologic entities that occasionally overlap. CBA bile duct injury mimics non-CBA. Cystification is an aberrant manifestation of stromal proliferation in BA. The current management approach assuming CBA and CC in infants are 2 separate disease processes is supported but caution is advised.

Quality assessment of lymph node sampling in rhabdomyosarcoma: A surveillance, epidemiology, and end results (SEER) program study.

BACKGROUND: Lymph node sampling is integral in the management of extremity and paratesticular rhabdomyosarcoma (RMS). The aim of this study was to determine overall surgical compliance with treatment protocols and impact of nodal sampling outcomes in these tumors. METHODS: A query of the surveillance, epidemiology, and end results program (SEER) database was performed from 2003 to 2008 for patients <19years of age with RMS. Data obtained included demographics, five-year survival and rate of nodal sampling. Analysis was performed utilizing chi-squared, Kaplan-Meier and hazard ratio modeling. RESULTS: Of 537 patients with extremity RMS, nodal sampling was performed in 25.7% (n=138). This lack of nodal sampling had a negative outcome on survival (p=0.004). Sixty five patients with paratesticular RMS aged greater than 10 were identified and also displayed low rates of lymph node sampling (47.7%, n=31). For paratesticular patients, a similar increase in survival was seen in patients who underwent nodal evaluation (p=0.024). CONCLUSION: Lymph node sampling is the standard of care in RMS. However, surgical compliance with treatment protocols is poor. Nodal evaluation correlated significantly with overall survival. These findings suggest a need for improved education among surgeons and oncologists regarding the need lymph node assessment in pediatric oncology patients. Evidence rating/classification: Prognosis study, Level III.

The presentation and management of choledochocele (type III choledochal cyst): A 40-year systematic review of the literature.

BACKGROUND: Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly. METHODS: PubMed and Cochrane databases were accessed 1975-2015 using terms "choledochocele" or "choledochal cyst". Studies reviewed that met the following criteria: English language, published 1975-2015 with human subjects. RESULTS: 325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews. 13 pediatric case reports of choledochocele exist, with abdominal pain being the most common symptom (n=11). The most frequent diagnostic and treatment modalities were ultrasound (n=10), and endoscopic sphincterotomy (n=5). No malignancies were reported. 58 adult case reports exist, with the most common presenting symptom being abdominal pain (n=54). Ultrasound was the frequently employed diagnostic modality (n=32). Open procedures were performed more often (n=30). Malignant lesions were identified in 5. In 42 institutional reviews, the frequency of choledochocele was 0.7%. Of those for whom treatment was reported, 69% underwent endoscopic sphincterotomy. CONCLUSION: Choledochocele is a rare malformation. Similarities exist between pediatric and adult patients, but malignancy has only been reported in adults. An algorithm based on patient age, cyst size, lining and amenability to endoscopic resection may be considered as a treatment strategy for this uncommon condition.