Outcomes for pediatric acute lymphoblastic leukemia patients with intestinal perforation.

OBJECTIVES: Intestinal perforation during acute lymphoblastic leukemia (ALL) treatment in children is rare, but represents a severe complication with possible long-term consequences. In this study, we aim to provide an overview of the epidemiology and clinical characteristics of these patients; analyze surgical pathology findings for possible causes; and determine its impact on patients' therapy, nutritional status, and outcome. STUDY DESIGN: Historical chart review from January 2000 to October 2020 of children with ALL and intestinal perforation during therapy diagnosed at a single institution. Data collected included patient demographics, anthropometric measurements, ALL characteristics, diagnosis and surgery of intestinal perforation, pathology, adjustments to treatment plan, and outcome. RESULTS: Of 1840 ALL patients, 13 (0.7%) presented with intestinal perforation during treatment. Perforation occurred during induction phase in 91% of cases. Most patients underwent laparotomy with ostomy creation, and no patient died from the intervention or developed malnutrition. Pathology mainly revealed inflammation at the perforation site. Two samples showed leukemic infiltration and presence of microorganisms. Patients were able to resume ALL therapy in all cases. A total of eight patients (73%) were in first remission at last follow-up, with a median follow-up time of 42 months (interquartile range = 42). CONCLUSION: Early surgical intervention is a successful treatment approach for intestinal perforation in ALL patients. There is a clear predilection for induction phase in the occurrence of intestinal perforation in ALL patients. No specific cause was identified. Patients can receive bridging chemotherapy during surgical recovery and proceed with their treatment without apparent impact on outcome.

Clinical significance of SLN benign capsular nevi in patients with melanoma.

BACKGROUND: Benign capsular nevi (BCN) are not infrequent in sentinel lymph nodes (SLN) of patients with melanoma. Their prognostic significance is unknown and the literature is limited. This study evaluated the clinical significance of incidentally found BCN in these patients. METHODS: A multi-institutional retrospective review of patients undergoing SLN biopsy for cutaneous melanoma between 2000 and 2016. Patients were divided into the following groups: (a) negative SLN and no BCN, (b) negative SLN and presence of BCN, (c) positive SLN seen only on immunohistochemistry (IHC), and (d) positive SLN via hematoxylin and eosin (H&E). Outcomes measured were overall survival and any recurrence. RESULTS: A total of 1253 patients were identified (group 1 = 978, group 2 = 56, group 3 = 32, and group 4 = 187). Fifty-seven percent were male and the mean age was 59.3 years. BCN was identified in 77 patients (6.2%), of which the majority was in the node-negative group (72%). Multivariable analysis showed that BCN was associated with lower recurrence rates, though not statistically significant (hazard ratio [HR] = 0.5; P = .06). IHC- and H&E-positive SLNs were associated with a higher risk of recurrence (HR = 2.4; P = .02 and 2.0, P < .0001, respectively). CONCLUSION: Patients with BCN and negative SLN had lower recurrence rates than patients with negative SLN and no BCN. Our data suggest a possible protective effect against recurrence.

Early Hospital Discharge After Helicopter Transport of Pediatric Trauma Patients: Analysis of Rates of Over and Undertriage.

OBJECTIVES: Helicopter air ambulance (HAA) of pediatric trauma patients is a life-saving intervention. Triage remains a challenge for both scene transport and interhospital transfer of injured children. We aimed to understand whether overtriage or undertriage was a feature of scene or interhospital transfer and how in or out of state transfers affected these rates. METHODS: Children (<18 years) who underwent trauma activation at a level I trauma center between 2011 and 2013 were identified and reviewed. Patients transported by HAA were compared with those transported by ground ambulance (GA). RESULTS: Of 399 pediatric patients (median age, 10.4 years; range, 0.1-17 years; 264 male [66%]), 71 (18%) were transported by HAA. Seventy-two percent of HAA patients went to the intensive care unit or the operating room from the trauma bay or suffered in-hospital mortality (vs 42% GA, P < 0.001). More patients were overtriaged (HAA with injury severity score [ISS] of <15) from interhospital transfers than from the scene (25% vs 3%, P = 0.002). Undertriage (GA with ISS >15) was acceptable at 5% from the scene and 14% from interhospital transfers (P = 0.08). Overtriage of patients with ISS less than 15 to HAA was significantly lower from in-state hospitals (22%) than out-of-state hospitals (45%) (P = 0.02). Undertriage of patients with ISS greater than 15 to GA was also lower from in-state hospitals (20%) versus out-of-state hospitals (38%) (P = 0.03). CONCLUSIONS: Triage of pediatric trauma patients to HAA remains difficult. There remains potential for improvement, particularly as regards interhospital HAA overtriage, but well developed transfer protocols (such in-state protocols) may help.

Magnetic Resonance-guided High-intensity Focused Ultrasound (MRgHIFU) Virtual Treatment Planning for Abdominal Neuroblastoma Utilizing Retrospective Diagnostic 3D CT Images.

Magnetic resonance-guided high-intensity focused ultrasound (MRgHIFU) is a novel treatment for neuroblastoma using ultrasound-induced thermal ablation with real-time MR thermometry. It is unclear which patients would be amenable to MRgHIFU given the retroperitoneal location of many neuroblastomas within the smaller pediatric abdomen. In addition, planning relies on MR scans, which are not routine in the standard pediatric neuroblastoma workup. This study sought to demonstrate that neuroblastomas are targetable with MRgHIFU and available computed tomographic imaging could be utilized for MRgHIFU virtual treatment. Cross-sectional images of 88 pediatric abdominal neuroblastoma patients were retrospectively processed with custom software to be made compatible with the Sonalleve MRgHIFU platform. Targetability measured percent treatment to lesion volume, within adequate safety margins from critical structures. All images were successfully converted into treatment planning files. Median lesion size was 191±195 cm and depth was 29±17 mm. Up to 78 (85%) patients had targetable lesions with a median targetable volume of 15% and ranging up to 79%. Targetability was highest in superficial, right upper quadrant lesions >200 cm, but limited by proximity to bowel and ribs. This study demonstrates the capacity for MRgHIFU to potentially treat the majority of abdominal neuroblastomas and the feasibility of using computed tomographic images for MRgHIFU virtual treatment planning.

Current Approaches to Pediatric Polyposis Syndromes.

Colorectal adenomatous polyposis syndromes encompass a diverse group of disorders with varying modes of inheritance and penetrance. Children may present with overt disease or within screening programs for families at high risk. We provide an overview of the array of pediatric polyposis syndromes, current screening recommendations, and surgical indications and technical considerations. Optimal disease management for these pediatric patients is still evolving and has implications for screening, surveillance, pediatric surgical management, and transition of care gastroenterologic neoplasia physicians and surgeons.

Prehospital blood transfusions in pediatric trauma and nontrauma patients: a single-center review of safety and outcomes.

PURPOSE: Prehospital transfusions are a novel yet increasingly accepted intervention in the adult population as part of remote damage control resuscitation, but prehospital transfusions remain controversial in children. Our purpose was to review our pediatric prehospital transfusion experience over 12 years to describe the safety of prehospital transfusion in appropriately triaged trauma and nontrauma patients. METHODS: Children (<18 years) transfused with packed red blood cells (pRBC) or plasma during transport to a single regional academic medical center between 2002 and 2014 were identified. Admission details, in-hospital clinical course, and outcomes were analyzed. RESULTS: 28 children were transfused during transport; median age was 8.9 ± 7 years and 15 patients were male (54%). Most patients required at least one additional unit of blood products during their hospitalization (79%), and/or required operative intervention (53%), endoscopy (7%), or died during their hospitalization (14%). Comparison of trauma patients (n = 16) and nontrauma patients (n = 12) revealed that nontrauma patients were younger, more anemic, more coagulopathy on admission, and required more ongoing transfusion in the hospital. Trauma patients were more likely to need operative intervention. No patient had a transfusion reaction. CONCLUSION: Remote damage control prehospital transfusions of blood products were safe in this small group of appropriately triaged pediatric patients. Further studies are needed to determine if outcomes are improved and to devise a rigorous protocol for this prehospital intervention for critically ill pediatric patients.

Comparative Study of Liposomal Bupivacaine Versus Paravertebral Block for Pain Control Following Mastectomy with Immediate Tissue Expander Reconstruction.

BACKGROUND: Several approaches to minimize postoperative pain, nausea, and enhance recovery are available for patients undergoing mastectomy with immediate tissue expander (TE) reconstruction. We compared the effectiveness of intraoperative local infiltration of liposomal bupivacaine (LB) to preoperative paravertebral block (PVB). METHODS: We retrospectively reviewed patients who underwent mastectomy with immediate TE reconstruction between May 2012 and October 2014 and compared patients with preoperative ultrasound-guided PVB to those with intraoperative LB infiltration. RESULTS: Fifty-three patients (54.6 %) received LB and 44 received PVB. LB was associated with less opioid use in the recovery room (p < 0.001), fewer patients requiring antiemetics (p = 0.03), and lower day of surgery pain scores (p = 0.008). LB also was associated with longer time to first opioid use (p = 0.04). On multivariable analysis controlling for expander placement location, year of surgery, and axillary lymph node dissection (ALND), the only variable that remained statistically significant was lower opioid use in the recovery room for patients with LB (p = 0.03) and day of surgery pain scores approached significance (p = 0.05). There was no difference in the proportion of patients discharged within 36 h of surgery between the groups. Focusing on first cases of the day (where PVBs are performed in the OR) showed average time to skin incision was 15 min shorter in the LB group (p = 0.004). CONCLUSIONS: Local infiltration of LB in patients undergoing mastectomy with immediate TE reconstruction decreases narcotic requirements in the recovery room, shortens preoperative anesthesiology time, and provides similar, if not better, perioperative pain control compared with PVB.

Frozen section analysis of SLNs in trunk and extremity melanoma has a high false negative rate but can spare some patients a second operation.

OBJECTIVES: The purpose of this study was to evaluate the accuracy of frozen section (FS) analysis of sentinel lymph nodes (SLN) in melanoma. METHODS: Five hundred seventy-one patients underwent FS analysis of SLN between 1/2000 and12/2010. Surgical and pathological characteristics, recurrence, and survival were analyzed. Comparisons were made using χ2 and Fisher's exact t-test. RESULTS: One hundred thirty-three (23%) patients were SLN positive of which 63 (47.4%) were identified on FS. 16/70 SLN metastases not identified on FS (23%) were seen only on immunohistochemistry. FS analysis detected 84% of SLN metastasis >2 mm. SLN FS false negative rate was 53%, positive predictive value 100%, negative predictive value 88%, and overall accuracy 89%. Among patients with a FS positive SLN, 17/63 (27%) had additional positive nodes on CLND, versus 1 of 70 (1.4%) with a positive SLN identified only on permanent section pathology (P < 0.0001). The nodal recurrence rate following a negative SLN biopsy was 5%. CONCLUSIONS: FS analysis for SLNs spared approximately half of patients a second operation. Patients with a positive SLN detected on FS were more likely to have further nodal involvement. In our experience intraoperative pathologic analysis of melanoma SLNs does not impair our ability to detect SLN metastasis or lead to a high rate of false positive results or nodal recurrences. J. Surg. Oncol. 2016;114:879-883. © 2016 2016 Wiley Periodicals, Inc.

A mechanism for the inhibition of DNA-PK-mediated DNA sensing by a virus.

The innate immune system is critical in the response to infection by pathogens and it is activated by pattern recognition receptors (PRRs) binding to pathogen associated molecular patterns (PAMPs). During viral infection, the direct recognition of the viral nucleic acids, such as the genomes of DNA viruses, is very important for activation of innate immunity. Recently, DNA-dependent protein kinase (DNA-PK), a heterotrimeric complex consisting of the Ku70/Ku80 heterodimer and the catalytic subunit DNA-PKcs was identified as a cytoplasmic PRR for DNA that is important for the innate immune response to intracellular DNA and DNA virus infection. Here we show that vaccinia virus (VACV) has evolved to inhibit this function of DNA-PK by expression of a highly conserved protein called C16, which was known to contribute to virulence but by an unknown mechanism. Data presented show that C16 binds directly to the Ku heterodimer and thereby inhibits the innate immune response to DNA in fibroblasts, characterised by the decreased production of cytokines and chemokines. Mechanistically, C16 acts by blocking DNA-PK binding to DNA, which correlates with reduced DNA-PK-dependent DNA sensing. The C-terminal region of C16 is sufficient for binding Ku and this activity is conserved in the variola virus (VARV) orthologue of C16. In contrast, deletion of 5 amino acids in this domain is enough to knockout this function from the attenuated vaccine strain modified vaccinia virus Ankara (MVA). In vivo a VACV mutant lacking C16 induced higher levels of cytokines and chemokines early after infection compared to control viruses, confirming the role of this virulence factor in attenuating the innate immune response. Overall this study describes the inhibition of DNA-PK-dependent DNA sensing by a poxvirus protein, adding to the evidence that DNA-PK is a critical component of innate immunity to DNA viruses.

Postoperative pyoderma gangrenosum (PG): the Mayo Clinic experience of 20 years from 1994 through 2014.

BACKGROUND: Postoperative pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by the development of PG-type lesions within surgical sites. OBJECTIVE: We sought to characterize postoperative PG as a distinct subtype of PG for earlier recognition and prevention of improper therapy. METHODS: We conducted a retrospective chart review of patients with nonperistomal postoperative PG at Mayo Clinic from 1994 to 2014.x RESULTS: Eighteen patients had postoperative PG with an average age of 58 years. Fifteen (83%) were female. Among patients with postoperative PG, 4 (22%) had an associated systemic disease traditionally associated with PG. Sites of postoperative PG included 7 breast (38%), 7 abdomen (38%), 1 back, 1 shoulder, 1 ankle, and 1 scrotum, witxxh breast reconstruction being the most common surgery. The average time to symptoms was 11 days. No patients had a fever. Eight (44%) had documented anemia and 5 (27%) had leukocytosis. Antibiotics and systemic corticosteroids were initiated in 10 (56%) and 14 (83%), respectively. Debridement was done in 11 (61%) patients. LIMITATIONS: Small sample size and retrospective study are limitations. CONCLUSION: Postoperative PG is a rare surgical complication with predilection for the breast and abdomen of females and has less association with systemic disease than idiopathic PG. Early recognition may prevent unnecessary debridements and morbidity.

Paravertebral blocks in patients undergoing mastectomy with or without immediate reconstruction provides improved pain control and decreased postoperative nausea and vomiting.

BACKGROUND: Mastectomy is associated with postoperative nausea and pain. We evaluated whether paravertebral block (PVB) use altered opioid use, antiemetic use, and length of stay in patients undergoing mastectomy. METHODS: We performed a retrospective cohort analysis of all patients who underwent mastectomy with or without PVB from 2008 to 2010. Patient demographics, operative procedure, intraoperative medications, postoperative opioid and antiemetic use, and length of stay were reviewed. Statistical analysis included univariable and multivariable analysis. RESULTS: A total of 605 patients were identified, of whom 526 patients were evaluable. A total of 294 patients underwent mastectomy without PVB (132 bilateral), and 232 patients underwent mastectomy with PVB (148 bilateral). Immediate reconstruction was performed in 203 (39 %) patients. Need for any postoperative antiemetic was less frequent in the PVB group (39 vs. 57 %, p < 0.0001). Day of surgery opioid use was lower in the PVB group than the non-PVB group (mean ± SD 40.1 ± 15.2 vs. 47.6 ± 17.7 morphine equivalents, p < 0.0001). Decreased opioid use was seen in unilateral mastectomy without reconstruction and bilateral mastectomy with and without immediate reconstruction. The proportion of patients discharged within 36 h of surgery was significantly higher in the PVB group (55 vs. 42 %, p = 0.0031). On multivariable analysis controlling for year of surgery, patient age and surgeon, PVB use affected antiemetic use and opioid use but not hospital length of stay. CONCLUSIONS: PVB results in decreased opioid use and decreased need for postoperative antiemetic medication in patients undergoing mastectomy. The greatest benefit is seen in patients undergoing bilateral mastectomy with immediate breast reconstruction.

Locum Tenens and Pediatric Surgery: A Position Statement and Practice Guidelines From the American Pediatric Surgical Association (APSA).

The American Pediatric Surgical Association (APSA) Practice Committee endorsed by the Board of Governors presents a Position Statement on the role of locum tenens in the practice of pediatric surgery. The Practice Committee also presents a set of guidelines for locum tenens practice. These recommendations highlight safe practice and quality care that protects the patient as well as the pediatric surgeon by offering best practice standards, defining optimal resources and establishing parameters by which hospitals and locum tenens agencies should abide. These guidelines are intended to foster discussion and contract negotiation as well as inform decision making for a) pediatric surgeons considering locum tenens opportunities, b) host organizations (hospitals and practices) seeking the coverage of a pediatric surgeon, and c) locum tenens companies vetting both surgeons and hospitals for appropriateness of such coverage. This Position Statement and foundational set of guidelines align with APSA's Vision (all children receive the highest quality surgical care) and Mission (to provide the best surgical care to our patients and families by supporting an inclusive community through education, discovery and advocacy).

Higher rates of regional disease but improved outcomes in pediatric versus adult melanoma.

BACKGROUND: Malignant melanoma is rare in the pediatric population and management is largely extrapolated from adult guidelines. Adult data have shown that immediate completion lymph node dissection (CLND) does not improve overall survival in selected patients with clinically node negative, sentinel lymph node-positive disease. Current nodal management in children is unknown. METHODS: The National Cancer Database (NCDB) was queried for patients with melanoma from 2012-2017 and patients categorized as pediatric (≤18 years, n=962) or adult (n=327,987). Factors associated with CLND in children with positive SLNB were evaluated in multivariable analysis. Kaplan-Meier survival analysis was performed. RESULTS: Compared to adults, children present with thicker primary tumors (T3 or T4 26.5% vs 15.5%, p<0.001), resulting in higher rates of nodal assessment with SLN biopsy or LND (60.2% vs 36.6%, p<0.001) and higher rates of regional nodal disease (35.1% vs 23.4%, p<0.001). Children underwent higher rates of CLND after SLN biopsy (10.4% vs 4.1%) and upfront lymph node dissection (15.2% vs 8.7%). A decreased rate of CLND was noted in 2017 compared to 2012 (odds ratio (OR) 0.16 (p=0.005). CLND was performed more often on multivariable analysis for older pediatric age (>12 years, OR=1.6, p=0.037) and lower extremity primary (OR=0.29, p<0.001). Children with regional nodal disease have improved 3-year overall survival compared to adults (96.5% vs 71.0%, p<0.001). CONCLUSIONS: Children with melanoma have higher rates of nodal disease but better survival than adults. As in adults, there has been a recent increase in close nodal observation rather than CLND for patients with positive SLN. Further study of nodal surveillance for pediatric patients is warranted.

Interhospital variability in localization techniques for small pulmonary nodules in children: A pediatric surgical oncology research collaborative study.

BACKGROUND: Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients. METHODS: A retrospective review was performed on patients < 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques. RESULTS: 225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p < 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p < 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16). CONCLUSIONS: Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time. LEVEL OF EVIDENCE: Retrospective review, Level 3.

Airway Clearance in Tracheomalacia.

Airway clearance is an essential part of airway maintenance to ensure the airway lumen is protected against particulate and infectious insults. The mechanisms involved in airway clearance include intrinsic structural and cellular components that can be impaired or inhibited through developmental defects and surgical interventions. Tracheomalacia is a developmental defect of the airway that can contribute to the mechanical failure to clear the airway. This chapter will review the mechanisms of airway clearance and the processes that can impair this vital process.

Conflict resolution and negotiation in pediatric surgery.

Conflict is pervasive in pediatric surgery - it is experienced during patient care, in the operating room and between colleagues. The ethical principles of autonomy, beneficience, non-maleficience and justice are firmly anchored in any discourse regarding conflict in the setting of healthcare. The authors review key features of conflict in healthcare including the effect on patient outcomes as well as implications for the ability of the surgeon and the medical team to function appropriately and safely when conflicts arise. Although most pediatric surgery fellowship programs have no formal training in conflict management, much has been written about strategies to teach and utilize techniques of conflict mitigation and negotiation. In this article, the authors discuss common areas of conflict in the medical environment and suggest useful tools for the practicing pediatric surgeon to aid in the resolution of conflict.

Surgical Resection After Talimogene Laherparepvec for Melanoma: Persistent Fuorodeoxyglucose Avidity on Positron Emission Tomography Despite No Viable Disease.

BACKGROUND: Talimogene laherparepvec (TVEC) is an injectable attenuated oncolytic herpes simplex virus (HSV-1) used in the treatment of loco regionally metastatic melanoma. Lesions managed by TVEC are generally considered unresectable at time of initiation of intralesional therapy; however, a subset of patients go on to have surgical resection of loco regionally controlled disease. We sought to review our experience with surgical excision of treated lesions to offer an insight into the radiologic correlate, treatment effect, and pathological findings of intralesional TVEC therapy. METHODS: This is a retrospective descriptive case series of patients who underwent TVEC injection at Mayo Clinic, Rochester, MN, between October 2016 and July 2020. Institutional Institutional Review Board approval was obtained. RESULTS: Twenty-one patients underwent intralesional TVEC, met inclusion criteria, and were included in this series. Seven went on to surgical excision of the injected lesions after an initial course of TVEC. Of those 7 patients, 4 had residual melanoma in the specimen on final pathology, while 3 had a complete pathologic response. All 3 patients who had no residual disease on pathology continued to have fluorodeoxyglucose (FDG) avidity on preoperative positron emission tomography scan of the excised lesions. DISCUSSION: Despite ongoing FDG avidity on PET scan, patients who have well-controlled disease and stability over time of the injected lesions may benefit from surgical excision following a course of TVEC. This may render the patient clinically disease free and/or allow them a reprieve from TVEC treatment.

Mutations in modified virus Ankara protein 183 render it a non-functional counterpart of B14, an inhibitor of nuclear factor kappaB activation.

Vaccinia virus (VACV) encodes multiple proteins to evade host innate immunity, including B14, a virulence factor that binds to the inhibitor of kappaB kinase beta (IKKbeta) and blocks nuclear factor kappaB (NF-kappaB) activation. B14 shares 95 % amino acid identity with the 183 protein encoded by modified virus Ankara (MVA), an attenuated VACV strain being developed as a vaccine vector. To evaluate whether the immunogenicity of MVA might be increased by manipulation of MVA immunomodulatory proteins, the MVA counterpart of B14, protein 183, was characterized. Unlike B14, protein 183 was unstable in eukaryotic cells unless proteasome-mediated protein degradation was inhibited. Furthermore, 183 did not inhibit NF-kappaB activation in response to cytokine stimulation, and did not restore the virulence of VACV strain Western Reserve lacking gene B14R. The instability and non-functionality of 183 are probably explained by a deletion of 6 aa within alpha-helix 6 of the B14 crystal structure.

A Systematic Review of Prenatally Diagnosed Intra-abdominal Enteric Duplication Cysts.

INTRODUCTION: With improvements in prenatal ultrasound, more abdominal enteric duplication cysts (EDCs) are diagnosed prenatally. The optimal time for operative intervention is unclear. We aimed to review the evidence supporting the indication and timing of surgery for prenatally diagnosed EDCs. MATERIALS AND METHODS: A systematic review was performed using a defined search strategy identifying articles with inclusion criteria of prenatal diagnosis of histologically confirmed EDCs. Outcomes included (1) indications for resection (symptoms vs. prophylactic) and (2) time from birth to operative intervention. RESULTS: Of 1,590 articles screened, prenatal diagnosis of histologically confirmed EDCs was made in 68 articles, detailing indications and timing for resection for 86 patients. Thirty-five patients (41%) became symptomatic early and were resected promptly at a median of 2 days of age. Indications for intervention included bowel obstruction, volvulus, bleeding, recurrent pain and diagnostic dilemma (concern for cystic neuroblastoma). There were 51 patients (59%) who were resected prophylactically at a median of 90 days of age without interval symptoms. Symptomatic cysts had a larger mean cyst diameter. There was a difference in the anatomic distribution of asymptomatic or symptomatic EDCs-gastric duplications were more frequently asymptomatic while jejunal, proximal ileal and colonic duplications were more frequently symptomatic. CONCLUSION: The optimal approach to EDCs diagnosed prenatally is not supported by strong evidence in the literature. Our systematic review indicates that almost half of the infants diagnosed prenatally become symptomatic early and are resected. In asymptomatic patients, close observation and delayed prophylactic resection in infancy can be considered.

Multifocal hepatoblastoma: What is the risk of recurrent disease in the remnant liver?

PURPOSE: Multifocal hepatoblastoma (HB) is often treated with total hepatectomy and transplantation owing to concerns of surgical resectability, local recurrence, and/or metachronous tumor in the remnant liver. We aimed to review HB patients to determine the risk of local recurrence in multifocal disease. METHODS: We undertook retrospective cohort analysis of all HB patients at a single tertiary referral center between 2001 and 2015. Demographics, diagnostic features, operative details, and outcomes were analyzed. RESULTS: Sixty patients underwent surgical management of HB. 39 had unifocal, and 21 had multifocal disease. Of multifocal patients, 9 underwent liver transplantation, 10 anatomic resections, and 2 nonanatomic resections. Overall, two patients had recurrence in the remnant liver - both from the unifocal group. There were equivalent distant (lung) recurrences between the groups (8% for unifocal versus 14% for multifocal), p = 0.89. At a mean of 75 months of follow-up, overall survival was 97% for unifocal patients and 86% for multifocal patients, p = 0.12. CONCLUSION: Multifocal HB was not associated with increased local recurrence in the setting of R0 resection and chemotherapy. These data do not support the contention that all patients with multifocal HB require a total hepatectomy and transplantation to reduce the incidence of local recurrence and/or metachronous tumor development. LEVEL OF EVIDENCE: Level III - Limited cohort analysis.