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BACKGROUND Pediatric non-central nervous system (CNS) malignant rhabdoid tumors (MRTs) are rare and aggressive malignancies without standard treatment strategies. The National Cancer Database (NCDB) was utilized to describe the incidence, characteristics, treatment strategies, and outcomes in pediatric patients. METHODS Patients

Objective To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development and (3) patient-reported quality of life in children with sarcoma of the chest wall. Summary background data Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. Methods A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008-2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. Results The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (IQR = 1-3), and number of ribs resected did not correlate with margin status (p = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor (HR 2.24, p = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posterior rib resection (HR 8.43; p = 0.003) and increased number of ribs resected (HR 1.78; p = 0.02). Overall, patient-reported quality of life is not impaired following chest wall tumor resection. Conclusions Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.

Minimally invasive nephrectomy is performed routinely for adult renal tumors and for many benign pediatric conditions. Although open radical nephroureterectomy remains the standard of care for Wilms tumor and most pediatric renal malignancies, there are an increasing number of reports of minimally invasive surgery (MIS) for those operations as well. The APSA Cancer Committee performed a systematic review to better understand the risks and benefits of MIS in pediatric patients with renal tumors. Methods The search focused on MIS for renal tumors in children and followed the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) checklist. The initial database search identified 491 published articles, and after progressive review of abstracts and full-length articles, 19 were included in this review. Results There were two direct comparison studies where open surgery and MIS were compared. The remaining studies reported only on minimally invasive nephrectomy. Across all studies, there were a total of 151 patients, 126 of which had Wilms tumor and 10 patients had RCC. 104 patients had MIS, with 47 patients having open surgery. In the two studies in which open surgery and MIS were directly compared, more lymph nodes were harvested during open surgery (median = 2 (MIS) vs 5 (open); mean = 2.47 (MIS) vs 3.8 (open)). Many noncomparison studies reported the harvest of 2 of fewer lymph nodes for Wilms tumor. Several MIS patients were also noted to have intraoperative spill or positive margins. Survival between groups was similar. Conclusions There is a lack of evidence to support MIS for pediatric renal tumors. This review demonstrates that lymph node harvest has been inadequate for MIS pediatric nephrectomy and there appears to be an increased risk for intraoperative spill. Survival data are similar between groups, but follow-up times were inconsistent and patient selection was clearly biased, with only small tumors being selected for MIS. Type of study Review article. Level of evidence III.

Background Minimally invasive surgery has broad applicability to pediatric diseases, including pediatric cancer resection. Neuroblastic tumors of childhood are highly variable in presentation, and so careful selection of appropriate candidates for minimally invasive resection is paramount to achieving safe and durable surgical and oncological outcomes. Methods The American Pediatric Surgical Association Cancer Committee developed questions seeking to better define the role of minimally invasive surgery for neuroblastic tumors. A search using PubMed, Medline, Embase, Web of Science, ProQuest Dissertations, and Clinical Trials was performed for articles published from 1998 to 2018 in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P) guidelines. Results The evidence identified is all retrospective in nature. Minimally invasive surgical resection of neuroblastic tumors is safe for carefully selected smaller (4-6 cm) image defined risk factor (IDRF)-negative abdominal tumors when oncologic principles are followed. Size is a less-well defined criterion for thoracic neuroblastic tumors. Open approaches for both abdominal and thoracic tumors may be preferable in the presence of IDRF's. Conclusion Small tumors without IDRF's are reasonable candidates for minimally invasive resection. Surgical oncologic guidelines should be closely followed. The quality of data supporting this systematic review is poor and highlights the need for refinement in the study of such surgical techniques to improve knowledge and outcomes for patients with neuroblastic tumors. Type of Study Systematic Review. Level of Evidence Level III and Level IV.

OBJECTIVE To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. SUMMARY BACKGROUND DATA Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. METHODS The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients

This article reviews of the current evidence-based treatment standards for children with Wilms tumor. In this article, a summary of recently completed clinical trials by the Children’s Oncology Group are provided, the current diagnostic evaluation and surgical standards are discussed, and the surgical impact on current risk stratification for patients with Wilms tumor is highlighted. LEVEL OF EVIDENCE: This is a review article of previously published and referenced LEVEL 1 studies, but also includes expert opinion LEVEL V, represented by the American Pediatric Surgical Association Cancer Committee.

Background Mesenchymal chondrosarcoma is a rare and aggressive bone tumor with few reports of primary tumor in the chest wall. Case We report a case of a 17‐year‐old male presenting with back pain and a posterior mediastinal mass. Imaging demonstrated what was thought to be a benign chondral tumor. The patient underwent resection which confirmed extraskeletal mesenchymal chondrosarcoma. The patient declined proposed adjuvant chemotherapy and underwent multiple resections for rapid local reoccurrence. He ultimately elected for hospice care. Conclusion The case highlights the importance of close disease monitoring and exploration of treatment options, given lack of established guidelines and consistent tumor features.

Study objective The primary goal of this study was to determine if the addition of clonidine to ropivacaine prolonged periumbilical numbness compared to ropivacaine alone in pediatric patients receiving ultrasound guided rectus sheath nerve blocks for laparoscopic appendectomy. The secondary goals were to evaluate differences in perioperative pain scores, analgesic consumption, sedation, anxiolysis, and hemodynamic effects from clonidine. Design This was a single center, randomized, double-blinded prospective study. Setting This study was conducted within the pediatric operating rooms at the Children’s Hospital of Pittsburgh, a large university-based academic medical center. Patients Fifty pediatric patients (ages 10-17 years old) without pre-existing cognitive impairment, developmental delay or chronic pain undergoing laparoscopic appendectomy during weekday hours were enrolled and randomized to control versus intervention groups. Intervention Ultrasound guided rectus sheath nerve block injections were performed at the beginning of surgery with either ropivacaine 0.5% plus normal saline or ropivacaine 0.5% plus clonidine (2 mcg/kg, maximum of 100 mcg). Measurements The duration of periumbilical numbness, Numeric Pain Rating Scale scores, University of Michigan Sedation Scale, State-Trait Anxiety Inventory for Children, analgesic consumption, heart rate, blood pressure, and mean arterial pressures, were recorded for each patient at several time points in the perioperative setting. Main results There were no significant differences in demographic characteristics between groups. The median duration of periumbilical numbness did not significantly differ between the ropivacaine only and the ropivacaine plus clonidine groups 540.0 minutes [360.0 -1015.0] (median [interquartile range (IQR)]) versus 823.5 minutes [509.5- 1080.0], p = 0.451. There were no significant differences in perioperative analgesic consumption, pain and anxiety scores, PACU sedation, or hemodynamic instability. Conclusions The addition of clonidine did not significantly prolong rectus sheath nerve block duration and was well tolerated in pediatric patients. Perioperative analgesia, hemodynamics, anxiety, and PACU sedation did not differ between groups. Trial registration Clinical Trials NCT02439281

Background/Purpose Thoracoscopic excision of pulmonary nodules is often required for diagnostic or therapeutic purposes, however subpleural and sub-centimeter nodules can be difficult to visualize. Various CT-guided localization techniques have been described, though there is minimal published pediatric data regarding the use of microcoils. We hypothesize that microcoil localization facilitates thoracoscopic resection of pulmonary nodules in children. Methods A multi-institutional retrospective review of children who underwent preoperative CT-guided localization of lung nodules was conducted from 2012 to 2019. A combination of methylene blue dye (MBD), wires, and microcoils were utilized for CT-guided localization. When microcoils were utilized, fluoroscopy assisted in lesion identification and resection. Results Eighteen patients (mean age 13 years, range 2–21 years) underwent thoracoscopic resection of 24 preoperatively localized pulmonary nodules. Mean size and depth of the lesions were 5.5 mm and 10 mm, respectively. Microcoil placement was successful 95% of the time and assisted in lesion localization in 88% of cases. Wire localization was not a durable technique, as 3 of 5 wires became dislodged upon lung isolation. Conclusions Preoperative CT-guided localization with microcoils can assist in fluoroscopic-guided resection of pulmonary nodules in children. This technique avoids the pitfall of wire dislodgement, and provides surgeons an additional technique to localize sub-centimeter, subpleural nodules. Type of Study Retrospective Review. Level of Evidence Level III.

Background To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD). Methods Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected. Results Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival. Conclusion This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.

Background: Image-guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma. Procedure: A multi-institutional retrospective study was performed by the Pediatric Surgical Oncology Research Collaborative on children with neuroblastoma at 12 institutions over a 3-year period. Data collected included demographics, clinical details, biopsy technique, complications, and adequacy of biopsies for cytogenetic markers utilized by the Children's Oncology Group for risk stratification. Results: A total of 243 children were identified with a diagnosis of neuroblastoma: 79 (32.5%) tumor excision at diagnosis, 94 (38.7%) open incisional biopsy (IB), and 70 (28.8%) PCNB. Compared to IB, there was no significant difference in ability to accurately obtain a primary diagnosis by PCNB (95.7% vs 98.9%, P =.314) or determine MYCN copy number (92.4% vs 97.8%, P =.111). The yield for loss of heterozygosity and tumor ploidy was lower with PCNB versus IB (56.1% vs 90.9%, P

Abdominal wall nerve blocks have been gaining popularity for the treatment of perioperative pain in children. Our aim was to compare a technique of surgeon-performed, laparoscopic abdominal wall nerve blocks to anesthesia-placed, ultrasound-guided abdominal wall nerve blocks and the current standard of local wound infiltration.After institutional review board approval was obtained, a retrospective chart review was performed of pediatric patients treated at a single institution during a 2-year period. Statistics were calculated using analysis of variance with post-hoc Bonferonni t tests for pair-wise comparisons.Included in this study were 380 patients who received ultrasound-guided abdominal wall nerve blocks (n = 125), laparoscopic-guided abdominal wall nerve blocks (n = 88), and local wound infiltration (n = 117). Groups were well matched for age, sex, and weight. There was no significant difference in pain scores within the first 8 hours or narcotic usage between groups. Local wound infiltration demonstrated the shortest overall time required to perform (P .0001). Patients who received a surgeon-performed abdominal wall nerve block demonstrated a shorter duration of hospital stay when compared to the other groups (P = .02).Our study has demonstrated that laparoscopic-guided abdominal wall nerve blocks show similar efficacy to ultrasound-guided nerve blocks performed by pain management physicians without increasing time in the operating room.

Objective To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. Background H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. Methods A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary children's hospital from 2002–2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. Results We identified 102 patients (median 9.5 per center, range 1–16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. Conclusions There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. The level of evidence rating Level IV.

OBJECTIVE. The contrast-enhanced ultrasound (CEUS) imaging features of hepatic vascular tumors in infants, including infantile hemangioma (IH) and congenital hemangioma (CH), are not well reported....

This chapter addresses the gastrointestinal complications that can occur in pediatric cardiac intensive care unit. Gastrointestinal complications are not uncommon in the critically ill cardiac patients and are associated with prolong ICU stay and hospital length of stay as well as poor growth and development.

Background Differentiated thyroid carcinomas (DTCs) are rare in young children but represent almost 10% of all malignancies diagnosed in older adolescents. Methods This article reviews the recent literature describing surgical therapeutic approaches to pediatric DTC, associated complications, and long-term recurrence and survival outcomes. Results Similar to adult thyroid cancers, pediatric DTCs are more common in females and are associated with thyroid nodules, family history of thyroid cancer, radiation exposure, iodine deficiency, autoimmune thyroid disease, and genetic syndromes. Management of thyroid cancers in children involves ultrasound imaging, fine needle aspiration, and surgical resection with treatment decisions based on clinical and radiological features, cytology and risk assessment. Conclusions Total thyroidectomy and compartment based resection of clinically involved lymph node basins form the cornerstone of treatment of DTC. There is an evolving literature regarding the use of molecular genetics to inform treatment strategies and the use of targeted therapies to treat iodine refractory and surgically unresectable progressive disease. Type of study Summary review. Level of evidence This is a review article of previously published Level 1–5 articles that includes expert opinion (Level 5).

Elective laparoscopic cholecystectomy (LC) pediatric patients in our institution have historically been admitted for an overnight hospital stay (OHS). The purpose of this study was to implement an ERAS protocol for elective LC in pediatric patients to promote same-day discharge (SDD) while maintaining excellent outcomes.An ERAS protocol for elective LC was implemented encompassing pre-, peri-, and postoperative management. A retrospective review of prospectively collected data from patients before (BI) and after implementation (AI) of the protocol was performed.A total of 250 patients (BI 105, AI 145) were included in the study. The AI group had significantly higher rate of SDD compared to BI (77.2% vs. 1.9%, p 0.01) and significantly decreased opioid use (morphine equivalents mg/kg AI 0.36 vs. BI 0.46, p 0.001). There were also no significant differences in the rate of total 30-day emergency department visits (BI 11.4% vs. AI 9.7%, p = 0.52) or surgery-related 30-day emergency department visits (BI 7.6% vs. AI 8.3%, p = 0.53). Factors that predisposed patients to an OHS after LC included higher ASA, later surgery start times, and longer operative times.The ERAS protocol significantly increased the rate of SDD after elective LC in pediatric patients without an associated increase in emergency department visits or readmissions.III.

Background/Purpose Melanoma is the most common skin cancer in children and often presents in an atypical fashion when compared to adults. The purpose of this review is to present an update on the epidemiology, surgical and medical management and prevention strategies in pediatric melanoma. Methods A comprehensive review of the current literature on the epidemiology, surgical and medical management and prevention of adult and pediatric melanoma was performed by the authors and the results of this review are summarized in the manuscript. Results Most recently, the incidence of melanoma in children has been declining, possibly owing to increased awareness and sun exposure prevention. The mainstay of therapy is surgical resection, often with sentinel lymph node biopsy. A positive sentinel node has prognostic value; however, completion node dissection is no longer recommended in the absence of clinically or radiographically positive nodes. Those with advanced disease also receive adjuvant systemic therapy using increasingly targeted immunologic therapies. Conclusions Sentinel lymph node positive patients no longer require completion lymph node dissection and instead may be followed by ultrasound. However, it is important to note that children have been excluded from most melanoma clinical trials to date, and therefore, recommendations for management are based on existing pediatric retrospective data and extrapolation from adult studies. Level of evidence IV.

10024 Background: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy, predominantly seen in children and young adults. Given its rarity, data are limited. We describe the largest cohort of patients with IMT to date, with an aim to further characterize this poorly understood tumor. Methods: A multi-institutional review was performed at 18 North American hospitals participating in the Pediatric Surgical Oncology Research Collaborative to identify IMT patients ≤39 years, diagnosed from 2000-2018. Descriptive statistics are described as median and interquartile range. Multivariable analysis was used to identify predictors of event free survival (EFS). Results: 182 patients were identified with a median age of 11 years (5-17); 52% were female. 33% of tumors were thoracic, 26% abdominal/pelvic, 20% head/neck, and 14% genitourinary. Common presenting symptoms included pain (29%), respiratory symptoms (24%), weight loss (12%), and a palpable mass (10%). Median tumor size was 3.9 cm (1.9-6.5). Anaplastic lymphoma kinase (ALK) overexpression was identified via immunohistochemistry in 53% of patients tested. One third of patients had abnormal cytogenetics, with 12% of the entire cohort having an ALK mutation. 7% of patients had distant disease at diagnosis. 13% of patients received neoadjuvant therapy including chemotherapy (3%), ALK inhibitor (4%), radiation (0.5%), non-steroidal anti-inflammatories (NSAIDs) (7%), or steroids (2%). Of those who underwent resection with known margin status (n = 158), 66% had complete resection, 20% had microscopic positive margins, and 14% had gross residual disease. Just over 40% of patients had an en bloc resection of involved organs, most commonly lung (26%). 21% of patients received adjuvant therapy, including chemotherapy (3%), ALK inhibitor (9%), radiation (0.5%), NSAIDs (8%), or steroids (5%). 12% of all patients received an ALK inhibitor: 24% neoadjuvant, 62% adjuvant, and 14% without surgery. Median follow-up time was 36 months (14-69). Overall 5-year survival (OS) was 95% and 5-year EFS was 80%. Predictors of recurrence included respiratory symptoms, larger tumor size, or distant disease at diagnosis. Gender, race, age and primary site were not predictive of EFS. Likewise, there was no association of ALK overexpression or ALK mutation with EFS. The presence of gross or microscopic margins following resection was not associated with recurrence. Conclusions: IMT is a rare tumor with favorable OS. Five year recurrence rate was 20%. Presenting with respiratory symptoms, larger tumor size, or metastatic disease was associated with recurrence, while ALK positivity was not. Notably, a positive margin after resection was not associated with increased recurrence, indicating that aggressive attempts at surgical resection that would compromise form or function may not be warranted.

Transanastomatic feeding tube (TAT) use in the repair of tracheoesophageal fistulas (TEF) with or without esophageal atresia (EA) and EA with or without TEF allows for earlier enteral feedings, however, may predispose to esophageal stricture.We review our institutional experience with esophageal atresia repair over a 15-year period from 2000 to 2015 and report on our observed complication rate with emphasis on the surgical approach and use of TATs.We identified 110 TEF repairs. Ninety-six were Type C, 7 were Type A, 4 were Type D, and 3 were Type E (H-Type). TATs were used in 74% of patients. The stricture rate with the TAT approached 56% versus 17% without a TAT (p0.0005). There was no difference in leak rate (p=0.27). Ninety-four TEF repairs were performed via open thoracotomy, and 16 were initially approached thoracoscopically. Six out of 16 that were started thoracoscopically were completed with the minimally invasive approach. Whether the case was started thoracoscopically, completed thoracoscopically, or performed open made no difference in the rate of stricture or anastomotic leak, but we did observe an increase in musculoskeletal complications in the open thoracotomy group (28% vs. 0).Our data suggests that the use of TATs does not protect against anastomotic leak, but may increase stricture rate. Further, the thoracoscopic group showed no difference in the leak or stricture rate and demonstrated less musculoskeletal complications. Confirmation of these findings will require a prospective study.III.

BACKGROUND: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration. METHODS: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period. RESULTS: Sixty-six children, with a median age of 11 years (range 1–17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; P = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% (n = 11) and count recovery in 35% (n = 6). Cancer therapy was delayed in 35% (n = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, P = .05) and longer LOS (29 vs 12 days; P = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications (P CONCLUSIONS: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.

Neuroblastoma is an embryonic cancer arising from neural crest stem cells. This cancer is the most common malignancy in infants and the most common extracranial solid tumor in children. The clinical course may be highly variable with the possibility of spontaneous regression in the youngest patients and increased risk of aggressive disease in older children. Clinical heterogeneity is a consequence of the diverse biologic characteristics that determine patient risk and survival. This review will focus on current progress in neuroblastoma staging, risk stratification, and treatment strategies based on advancing knowledge in tumor biology and genetic characterization. TYPE OF STUDY: Review article. LEVEL OF EVIDENCE: Level II.

Fetus-in-fetu is a rare congenital tumor containing fetal tissue with an unclear etiology, but some believe it to arise from a parasitic monozygotic twin within the host twin. We present the case of a 3-week old term neonate found to have an abdominal mass and undescended right testicle. At laparotomy, a large, circumscribed mass was present in the right upper quadrant. It was found to be associated with the vas deferens and gonadal vessels, appearing to be closely associated with the right-sided cryptorchid testis. Pathology revealed an intratesticular fetus-in-fetu. Fetus-in-fetu is a rare condition where a well-developed mass believed to arise from a monozygotic, parasitic twin, is found within its sibling. First reported by Dr. Meckel in 1800, this is thought to occur in 1 in 500,000 live births with less than 100 cases reported in the literature. Fetus-in-fetu is differentiated from mature teratoma by the presence of an axial skeleton with metameric segmentation and well-differentiated tissues. FIF is a benign disease, however surgical excision continues to be the treatment of choice, in order to confirm diagnosis by pathology and to exclude malignant teratoma. Fetus-in-fetu is a rare of congenital lesion that can present as an abdominal mass. Due to the high prevalence of retroperitoneal location, a FIF should remain on the differential for an infant with an abdominal mass and ipsilateral cryptorchid testis.

Purpose Bladder cancer is the sixth most common cancer in the United States, but is exceedingly rare in young patients, leading to a lack of accepted standards for diagnosis, treatment, and surveillance. We review our institutional experience with bladder urothelial neoplasms in pediatric and young adult patients summarizing presentation, treatment, and outcomes. Methods Surgical pathology records at our institution were searched for cases of urothelial neoplasms among patients ≤25 years of age treated between January 1997 and September 2016. Cases submitted exclusively for pathology review were excluded. Diagnoses were confirmed based on pathologic examination using the 2004 World Health Organization classification system. Results Thirty-four patients were identified with a mean age of 21.1 years (range 8–25 years), and median follow-up was 25.1 months (1–187 months). The male to female ratio was 1.83:1. The most common presenting symptom was hematuria (n=26; 76%). Diagnoses were invasive urothelial carcinoma (n=3), noninvasive urothelial carcinoma (n=24), PUNLMP (n=6), and urothelial papilloma (n=1). Noninvasive lesions were resected by cystoscopy, after which 12% (n=4) experienced complications (grade II or greater). One patient with stage IV invasive disease at diagnosis died, and 2 patients developed recurrences. Of those with noninvasive carcinoma, 29% (n=7) required repeat cystoscopy soon after initial TURBT at outside institutions, and 17% (n=4) had tumors downgraded from high-grade to low-grade after pathology review. Conclusion Hematuria is the most common sign of bladder neoplasia in children and young adults and should be investigated by cystoscopy. The majority of urothelial neoplasms in these patients are noninvasive and can be successfully treated with transurethral resection. Level of evidence Level IV (Retrospective study with no comparison group).

Background Lymphatic mapping to guide sentinel lymph node biopsy (SLNB) typically requires lymphoscintigraphy prior to surgery. In young pediatric patients, this process often requires intubation in the nuclear medicine suite followed by transport to the operating room (OR). Methods We reviewed 14 pediatric cases in which a portable nuclear imaging camera was utilized to perform the entirety of the SLNB in the OR. Results and Conclusion This method, utilizing intraoperative nuclear imaging, helped to confirm removal of the sentinel lymph node in real time, decreased anesthesia time, and avoided transport of a sedated or intubated child. Level of Evidence III.

Background Bilateral rectus sheath blocks have proven to be superior to local anesthetic infiltration for umbilical incisions and have been gaining popularity for the treatment of perioperative pain in children. We aim to develop a technique of surgeon performed rectus sheath blocks under laparoscopic-guidance alone. Methods In phase I, we observed the laparoscopic appearance of a rectus sheath block. The pain management team performed an ultrasound-guided rectus sheath nerve block as we visualized the posterior rectus sheath with the laparoscope. In phase II, after completion of the laparoscopic procedure, we performed a rectus sheath nerve block. Ultrasound was used to identify where the local anesthetic had been injected. Results Nineteen patients were included in this study, accounting for 38 rectus sheath blocks. In phase I, we observed with the laparoscope the delivery of ten ultrasound-guided rectus sheath blocks. In phase II, 28 laparoscopic-guided rectus sheath blocks were completed with immediate ultrasound confirmation of correct placement. Conclusions We have demonstrated that the rectus sheath nerve block can be performed reliably under laparoscopic-guidance alone. The efficacy of the laparoscopic-guided nerve block compared to the ultrasound-guided approach will need further study in a prospective, randomized trial. Type of study Retrospective review. Level of evidence 3.

Biologic therapy for pediatric cancer has gained much enthusiasm over the past two decades. Recent advances in the clinical sequencing of tumors, have also allowed for the identification of targetable mutations. These findings have created a new avenue of biologic agent development and study. As experience with biologic agents builds, so too must the awareness of the surgeon to identify potential complications of these drugs. Complications can result from the direct effect of a biologic on the patient, such as gastrointestinal perforation or secondary spontaneous pneumothorax, or they can be related to the effect a biologic has on another aspect of a patient's care, such as wound complications following surgery. The morbidity caused by these complications must be weighed against any potential benefit of an agent, when determining the role for biologic therapy in the overall cancer treatment strategy.

Congenital diaphragmatic hernia (CDH) is associated with significant mortality due to lung hypoplasia and pulmonary hypertension. The role of embryonic pulmonary innervation in normal lung development and lung maldevelopment in CDH has not been defined. We hypothesize that developmental defects of intrapulmonary innervation, in particular autonomic innervation, occur in CDH. This abnormal embryonic pulmonary innervation may contribute to lung developmental defects and postnatal physiological derangement in CDH. To define patterns of pulmonary innervation in CDH, human CDH and control lung autopsy specimens were stained with the pan-neural marker S-100. To further characterize patterns of overall and autonomic pulmonary innervation during lung development in CDH, the murine nitrofen model of CDH was utilized. Immunostaining for protein gene product 9.5 (a pan-neuronal marker), tyrosine hydroxylase (a sympathetic marker), vesicular acetylcholine transporter (a parasympathetic marker), or VIP (a parasympathetic marker) was performed on lung whole mounts and analyzed via confocal microscopy and three-dimensional reconstruction. Peribronchial and perivascular neuronal staining pattern is less complex in human CDH than control lung. In mice, protein gene product 9.5 staining reveals less complex neuronal branching and decreased neural tissue in nitrofen-treated lungs from embryonic day 12.5 to 16.5 compared with controls. Furthermore, nitrofen-treated embryonic lungs exhibited altered autonomic innervation, with a relative increase in sympathetic nerve staining and a decrease in parasympathetic nerve staining compared with controls. These results suggest a primary defect in pulmonary neural developmental in CDH, resulting in less complex neural innervation and autonomic imbalance. Defective embryonic pulmonary innervation may contribute to lung developmental defects and postnatal physiological derangement in CDH.

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. Recent evidence suggests that embryonic organ differentiation, including pancreatic β-cells, is tightly regulated by oxygen levels. Since a major determinant of oxygen tension in mammalian embryos after implantation is embryonic blood flow, here we used a novel survivable in utero intracardiac injection technique to deliver a vascular tracer to living mouse embryos. Once injected, the embryonic heart could be visualized to continue contracting normally, thereby distributing the tracer specifically only to those regions where embryonic blood was flowing. We found that the embryonic pancreas early in development shows a remarkable paucity of blood flow and that the presence of blood flow correlates with the differentiation state of the developing pancreatic epithelial cells in the region of the blood flow.

OBJECTIVE Glucose-dependent insulinotropic polypeptide (GIP) is a member of a structurally related group of hormones that also includes glucagon, glucagon-like peptides, and secretin. GIP is an incretin, known to modulate glucose-induced insulin secretion. Recent studies have shown that glucagon is necessary for early insulin-positive differentiation, and a similar role for incretins in regulating embryonic insulin-positive differentiation seems probable. Here we studied the role of GIP signaling in insulin-positive differentiation in the embryonic mouse pancreas. RESEARCH DESIGN AND METHODS The ontogeny of the GIP ligand and GIP receptor in the embryonic pancreas was investigated by immunohistochemistry and RT-PCR. GIP signaling was inhibited in cultured embryonic pancreata using morpholine-ring antisense against GIP ligand and receptor, or small interfering RNA (siRNA) for GIP ligand and receptor. Markers of endocrine cells and their progenitors were studied by immunohistochemistry and RT-PCR. RESULTS GIP and GIP receptor mRNA were both detected in the embryonic pancreas by embryonic day 9.5 and then persisted throughout gestation. GIP was generally coexpressed with glucagon by immunostaining. The GIP receptor was typically coexpressed with insulin. Morpholine-ring antisense or siRNA against either GIP ligand or GIP receptor both inhibited the differentiation of insulin-positive cells. Inhibition of GIP or its receptor also led to a decrease in the number of Pdx-1–positive and sox9-positive cells in the cultured embryonic pancreas. The number of Pax6- and Nkx2.2-positive cells, representative of developing pancreatic endocrine cells and β-cells, respectively, was also decreased. CONCLUSIONS GIP signaling may play a role in early embryonic pancreas differentiation to form insulin-positive cells or β-cells.

Background Operative intervention plays an important role in the management of primary liver cancers in children. Recent improvements in diagnostic modalities, pre- and postoperative chemotherapy, and operative technique have all led to improved survival in these patients. Both hepatic resection and orthotopic liver transplantation are effective operations for pediatric liver tumors; which intervention is pursued is based on preoperative extent of disease. This is a review of our institution's experience with operative management of pediatric liver cancer over an 18-year period. Methods A retrospective chart review from 1990 to 2007 identified patients who were ≤18 years old who underwent operative intervention for primary liver cancer. Demographics, type of operation, intraoperative details, pre- and postoperative management, as well as outcomes were recorded for all patients. Results Fifty-four patients underwent 57 operations for primary liver cancer, 30 of whom underwent resection; the remaining 27 underwent orthotopic liver transplantation. The mean age at diagnosis was 41 months. Twenty patients had stage 1 or 2 disease and 34 patients had stage 3 or 4 disease. Forty-eight (89%) patients received preoperative chemotherapy. Postoperative chemotherapy was given to 92% of patients. Mean overall and intensive care unit duration of stay were 18 and 6 days, respectively. About 45% of patients had a postoperative complication, including hepatic artery thrombosis ( n = 8), line sepsis ( n = 6), mild acute rejection ( n = 3), biliary stricture ( n = 2), pneumothorax ( n = 2), incarcerated omentum ( n = 1), Horner's syndrome ( n = 1), and urosepsis ( n = 1). Only 6 patients had a recurrence of their cancer, 5 after liver resection, 3 of whom later received a transplant. There was only 1 recurrence after liver transplantation. There was 1 perioperative mortality from cardiac arrest. Overall survival was 93%. Conclusion Operative intervention plays a critical role in the management of primary liver cancer in the pediatric population. Neoadjuvant chemotherapy can be given if the tumor seems unresectable at diagnosis. If chemotherapy is unable to sufficiently downstage the tumor, orthotopic liver transplantation becomes the patient's best option. Our institution has had considerable experience with both resection and liver transplantation in the treatment of pediatric primary liver cancer, with good long-term outcomes.

Intraoperative ultrasound is an extremely useful adjunct in both open and minimally invasive surgery. Use of ultrasound for central venous access has become standard of care; however, the focus of this chapter is on other uses of ultrasound in the operating room. Intraoperative ultrasound has an important role in oncologic surgery, specifically in identifying the location of tumors and their relationship to surrounding structures, especially in the liver and the pancreas. Identifying the presence and extent of tumor thrombus has incredibly important implications in surgery for Wilms’ tumor. Ultrasound is an excellent tool to identify the location of abscesses. In the operating room, ultrasound can be used to identify the location of deep soft-tissue or intra-abdominal abscess. Similarly, it can identify the presence of pleural fluid, help distinguish between loculated and simple fluid, and guide drainage of a pleural effusion or emypema. When concerns about blood flow arise in the operating room, color Doppler can be used to confirm adequate blood flow. Ultrasound may be helpful to identify a foreign body in the soft tissue that is not seen on X-ray. Ultrasound is an extremely powerful tool that can be used in countless situations in the operating room. It should be looked at as another tool available to the surgeon, and its use is not limited to those discussed in this chapter.

Background/purpose Fibrolamellar hepatocellular carcinoma (FL-HCC) arises in pediatric/adolescent patients without cirrhosis. We retrospectively evaluated the impact of resection, nodal status, metastasis, and PRETEXT stage on overall survival (OS). Methods With IRB approval, we reviewed records of 25 consecutive pediatric patients with FL-HCC treated at our institution from 1981 to 2011. We evaluated associations between OS and PRETEXT stage, nodal involvement, metastasis, and complete resection. Results Median age at diagnosis was 17.1years (range, 11.6–20.5). Median follow-up was 2.74years (range, 5–9.5). Five (28%) patients had PRETEXT stage 1 disease, 10 (56%) had stage 2, 2 (11%) had stage 3, and 2 (11%) had stage 4 disease. On presentation, 17 (68%) patients had N1 disease, and 7 (28%) had parenchymal metastases. Complete resection was achieved in 17 (80.9%) of 21 patients who underwent resection. Five-year OS was 42.6%. Survival was positively associated with complete resection ( P =0.003), negative regional lymph nodes ( P =0.044), and lower PRETEXT stage ( P P =0.05). Conclusions In young patients with FL-HCC, lower PRETEXT stage and complete resection correlated with prolonged survival, while metastatic disease and positive lymph node status were associated with poor prognosis. Thus, we recommend complete resection and regional lymphadenectomy whenever possible.

Traumatic pancreatic transection is uncommon. The role of laparoscopy in the setting of this injury has not been well described.Six large-volume pediatric trauma centers contributed patients18 years of age who underwent a distal pancreatectomy for traumatic pancreatic transection from 2000 to 2010.Twenty-one patients without another indication for emergency laparotomy underwent a distal pancreatectomy for Grade III pancreatic injuries, of which 7 underwent laparoscopic distal pancreatectomy. Mean (±SD) age was 8.6±4.7 years, and 67% were male. There was no difference in the presence of other injuries between the two groups (43% in each group). Computed tomography revealed a transected pancreas in 85% of the laparoscopic patients and 75% of the open group (P=1.0). Mean operative time was 218±101 minutes with laparoscopy compared with 195±111 minutes with the open procedure (P=.7). Median duration of hospitalization was 6 days (range, 6-18 days) in the laparoscopic group compared with 11 days (range, 5-26 days) in the open group (P=0.3). Postoperative morbidity was not different between the two groups (57% versus 21% for laparoscopic versus open, P=.2).Laparoscopy is equivalent to open distal pancreatectomy in children with select traumatic pancreatic injuries.

Purpose: Neuroblastoma is the most common extracranial solid tumor in children. Twenty percent of all neuroblastomas arise in the thorax. This study evaluates the open vs thoracoscopic resection of thoracic neuroblastoma. Methods: A retrospective chart review was conducted from the medical records of all children undergoing resection of a thoracic neuroblastoma from 1990 to 2007 at our institution. We evaluated patients who underwent open vs thoracoscopic resection and compared demographics, pathologic condition, stage, operative details, complications, and outcomes between the 2 groups. Results: A total of 149 cases of neuroblastoma were identified during the study period, 36 (24%) of which had tumor located in the thorax. Thirty-six of these patients underwent 37 operations for primary thoracic neuroblastoma. Open thoracotomy was used in 26 cases with the thoracoscopic approach to resection used in the remaining 11. We observed no differences in patient demographics including mean age, sex, or ethnicity. Tumors in both groups were of similar histologic condition, location, surgical margin, lymph node status, and stage. The length of operation was similar between the 2 groups, but length of stay was shorter in the thoracoscopic group (2.0 days; range, 1-7 days vs 3.5 days; range, 2-8 for the open group; P = .01). Estimated blood loss was also less in the minimally invasive group (median, 10 mL; range, 0-75 mL vs 25 mL; 5-650 mL in the open group; P = .02). Review of outcomes showed no significant difference in complications, recurrence, survival, or disease-free survival between these 2 groups. Conclusions: This retrospective review of thoracic neuroblastoma for an 18-year period shows that thoracoscopic resection is an effective approach to this tumor and offers shorter length of stay and decreased blood loss when compared to open thoracotomy.

Laparoscopic adrenalectomy is well established as the standard technique for all indications of adrenalectomy except adrenal carcinoma; however, some also consider large adrenal masses a relative contraindication. We present a video of a laparoscopic excision of a large ganglioneuroma and right adrenalectomy in a 12-year-old female.Our patient was noted to have a right suprarenal mass on a computed tomography scan following complaints of back and abdominal pain. Upon surgical consultation, she underwent a magnetic resonance imaging, which showed a 7.9 x 4.4 x 5.6 cm heterogeneously enhancing suprarenal lesion that was either arising from or compressing the right adrenal gland. The patient's preoperative work-up included normal urinary metanephrines, alpha-fetoprotein, and beta-HCG. After discussion with the patient and family, the decision was made to proceed with laparoscopic excision of the mass.The patient underwent successful laparoscopic excision of the suprarenal mass and right adrenalectomy and was discharged from the hospital on postoperative day 2. During the procedure, retraction was achieved by using a combination of 5-mm grasping instruments, Endokittner dissectors (Ethicon Endosurgery Cincinnati, OH), and a suction irrigator to provide traction and counter traction. A flexible 5-mm liver retractor (Mediflex; Velmed, Inc., Wexford, PA) was placed directly through the abdominal wall without a trocar in order to elevate the liver from the area of the right adrenal and retroperitoneum. This enabled us to "rotate" the lesion out from behind the vena cava and from along the vertebral bodies. The pathology revealed an 8.5 x 7.0 x 3.0 cm ganglioneuroma, with primarily neural and Schwann cell-type tissue with interspersed large, prominent ganglion cells, and a normal adrenal gland.As demonstrated by our video, large adrenal masses in the pediatric population can be successfully excised laparoscopically with appropriate surgeon comfort and experience.

Iatrogenic splenic injury requiring splenectomy is a well-recognized and potentially serious complication of colon resection. Iatrogenic splenectomy is associated with significant morbidity and mortality, including bleeding and the postsplenectomy sepsis syndrome. Our study aims to compare the incidence of iatrogenic splenectomy in laparoscopic colon resection with that of open colon resection over an 11-year-period at Mount Sinai. A retrospective chart review of all patients undergoing colon resection at Mount Sinai Medical Center during the last 11 years was performed to identify patient demographics, procedure, indication, and outcome. There was a significant difference (P=0.03) in the incidence of iatrogenic splenectomy during open colectomy (13/5477, 0.24%) versus laparoscopic colectomy (0/1911, 0%). All cases complicated by iatrogenic splenectomy involved splenic flexure mobilization. Laparoscopy has many recognized advantages over open procedures, including shorter recovery and length of stay. This retrospective review of our experience at Mount Sinai presents another potential benefit of the laparoscopic approach to colon resection.

This report was designed to discuss the case of a patient taking immunosuppression after transplant who developed squamous-cell carcinoma in a chronic pilonidal cyst and to examine the reported literature on malignant degeneration of pilonidal disease. We performed a comprehensive literature review and examined the medical record of a patient taking immunosuppressive medications after renal transplant who developed squamous-cell carcinoma in a chronic pilonidal cyst located at the superior gluteal cleft. Pilonidal disease is a common condition, and it is important to know that malignancy can arise when the disease is chronic. Immunosuppression and human papillomavirus infection may be predisposing factors to malignant degeneration of pilonidal cysts and may accelerate the transformation.

Because malrotation most commonly presents in infants, treatment recommendations for older children (1 year) have been based on data obtained from small case series. The purpose of this study was to use a large national database to determine the clinical significance of older children presenting with malrotation to develop treatment recommendations for this group.Records of children undergoing a Ladd's procedure were identified in the Kids' Inpatient Database, an administrative database that contains all pediatric discharges from 27 states during 2000. Patient characteristics, associated diagnoses, operations performed, and mortality were evaluated. Discharge weighting was used to obtain a national estimate of the number of children older than 1 year treated for malrotation.Two hundred nineteen older children (1 and18 years) undergoing a Ladd's procedure were identified in the database. One hundred sixty-four (75%) of these patients were admitted for treatment of malrotation, whereas most of the remaining 55 patients (25%) were admitted for another diagnosis and underwent a Ladd's procedure incidental to another abdominal operation. Seventy-five patients underwent a Ladd's procedure during an emergency admission. Thirty-one patients had volvulus or intestinal ischemia, 7 underwent intestinal resection, and 1 patient died. Based on case weightings, it was estimated that 362 older children underwent a Ladd's procedure for symptoms related to malrotation in 2000 in the United States (5.3 cases per million population).These findings provide support for performing a Ladd's procedure in older children with incidentally found malrotation to prevent the rare but potentially devastating complications of this anomaly.

Background The benefit of a prophylactic Ladd’s procedure in older children and adults with malrotation is controversial. The purpose of this study was to determine the role of the Ladd’s procedure in patients with asymptomatic malrotation diagnosed after infancy. Methods A Markov decision analysis was used to compare the quality adjusted life expectancy with and without a Ladd’s procedure among patients with asymptomatic malrotation. Data obtained from the Nationwide Inpatient Sample were used to estimate the age-related probability of emergency surgery or volvulus among patients with malrotation. Estimates of the mortality of elective and emergency surgery, mortality of volvulus, and utilities of each health state were obtained from the literature. Results After infancy, the gain in quality adjusted life expectancy associated with a prophylactic Ladd’s procedure was highest when asymptomatic malrotation was treated at 1 year old and steadily declined until asymptomatic malrotation was treated at 20 years old. An increasing advantage of observation over prophylactic surgery on life expectancy was observed after the second decade of life. A 2-fold increase in mortality risk for an elective Ladd’s procedure decreased the age threshold to 14 years, whereas a 4-fold increase decreased the threshold to 7 years. These results were found to be robust by sensitivity analyses and Monte Carlo simulation. Conclusion A Ladd’s procedure should be considered for children diagnosed with asymptomatic malrotation, particularly those who are younger and with a low risk of postoperative mortality. The rare occurrence of midgut volvulus does not justify performing a prophylactic Ladd’s procedure on most adults with malrotation.

To reduce the risks associated with splenectomy, there has been a trend toward splenic preservation when performing distal pancreatectomy. Although laparoscopy has gained almost universal acceptance, it is still not utilized often for pancreatic surgery in children. In fact, review of the literature shows only a few case series of splenic-preserving laparoscopic distal pancreatectomy, and even less reported experience in the pediatric population. To our knowledge, there has been only one other report of laparoscopic spleen-preserving distal pancreatectomy in the setting of trauma in a child.A 13-year-old boy suffered a handlebar injury to the abdomen secondary to a dirt bike accident. He did not report loss of consciousness but complained of abdominal pain. CT scan revealed a grade II splenic laceration with a transected distal pancreas. He was hemodynamically stable with a large contusion in the left upper quadrant. Laboratory evaluation showed that his hematocrit was stable but was significant for elevated amylase and lipase. During his hospitalization, he developed significant abdominal distension and his amylase and lipase continued to rise. He was taken to the operating room for definitive management of his pancreatic injury 72 hours after his initial injury.In the operating room, a central line was placed so that he could receive total parenteral nutrition postoperatively. The video demonstrates the performance of a laparoscopic spleen-preserving distal pancreatectomy. There was a fair amount of old blood seen in the left upper quadrant and behind the pancreas. There was no evidence of active bleeding. The operation was performed without complication and with preservation of the splenic vessels.The postoperative course was very smooth with the child resuming diet by postoperative day (POD) 4. He was sent home on POD 7. This video demonstrates that in the proper trauma patient, a minimally invasive approach to a spleen-preserving distal pancreatectomy can still be performed.