BACKGROUND:In many pediatric malignant germ cell tumor (MGCT) protocols, patients who do not achieve a complete response (CR; normal tumor markers, no radiological/histological residual) after three cisplatin-etoposide-bleomycin (PEb) cycles receive two to three "consolidation" cycles of the same regimen. Evidence for this practice is limited, and historical outcomes for non‑CR patients remain poor. METHODS:We retrospectively reviewed patients diagnosed with intermediate-risk MGCT, enrolled in AGCT0132, between 2003 and 2011. All patients received three cycles of PEb and underwent response assessment at the end of induction. Patients not in CR were prescribed three additional cycles of PEb as consolidation. We compared event-free survival (EFS) and overall survival (OS) for patients who did and did not receive consolidation. RESULTS:Among 210 patients enrolled, two patients were excluded from analyses: one without complete information, and one had rapid progressive disease (PD) and did not complete induction therapy. Out of 208 patients, 193 had a CR after three cycles of induction chemotherapy, and their post-induction 4-year EFS and OS were 93% and 99%. Fifteen patients were not in CR at the end of the first three cycles. Twelve received consolidated chemotherapy as mandated per protocol, and their 4-year EFS and OS were 61% and 65%, respectively. Three patients were deemed to have progressive disease at the end of induction and received second-line therapy. CONCLUSION:Children with MGCTs who did not achieve CR after the first three cycles of chemotherapy had an inferior outcome compared to those with a CR, despite receiving additional cycles of PEb chemotherapy. We conclude that consolidation is of unclear benefit. We suggest that pediatric MGCT patients who fail to achieve a CR after standard induction chemotherapy may receive a salvage regimen with different agents rather than consolidation with more of the same chemotherapy.

Introduction Spigelian hernias represent only 0.1 - 0.2% of all abdominal wall hernias and are infrequently encountered in pediatric patients. Limited literature surrounding pediatric Spigelian hernias exists, and there is no uniform or optimal surgical repair technique. Case presentation A term female with unremarkable prenatal history presented a left-sided Spigelian hernia at birth that contained loops of bowel, by physical examination and ultrasound. The hernia was easily reducible, so she was discharged with a plan for an elective repair at a later point. At 8 months of age, she underwent an abdominal wall ultrasound that showed a defect of 6 cm in length. She underwent elective repair at 9 months of age due to parental preference in the setting of an enlarging defect size. The hernia repair was done through a combination of laparoscopic and open techniques. The laparoscopy part, which consisted in one port placed in the umbilicus for a camera, enabled visualization of the suspected hernia site, confirm the proper approximation of the lateral and medial borders of the hernia, and helped avoid any intra-abdominal injuries during the repair. The repair itself was completed in an open manner with interrupted sutures in a top to bottom approach, in a transverse orientation. The patient recovered well from the operation. At 1 year of follow up she has shown no signs of recurrence. Conclusion Congenital pediatric Spigelian hernias can be successfully repaired using a combination of laparoscopy and open approach. The addition of a laparoscopy allows proper visualization of the intra-abdominal side of the repair.

Children, adolescents and young adults with testicular germ cell tumors require appropriate surgical care to insure excellent outcomes. This article presents the most critical elements, and their basis in evidence, for surgery in this population. Specifically, the importance of inguinal radical orchiectomy for malignant tumors, partial orchiectomy for prepubertal tumors and normal serum tumor markers, and the appropriate use of post-chemotherapy retroperitoneal lymph node dissection in those with residual retroperitoneal masses.

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1,2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function. Herein we outline the critical elements of tumor resection and management of sacrococcygeal germ cell tumors with a focus on the technical aspects of this tumor across a range of presentations.

Performance of the appropriate operation is highly important to ensure that any patient with a suspected ovarian germ cell tumor receives optimal therapy that prioritizes cure while simultaneoulsy minimizing risk of short and long-term toxicities of treatment. The following critical elements of any operative procedure performed for a suspected pediatric or adolescent ovarian germ cell tumor are reviewed: 1. Complete resection of the tumor via ipsilateral oophorectomy while avoiding tumor rupture and spillage, and 2. Performance of complete intraperitoneal staging at the time of initial tumor resection.

PURPOSE:Partial, or subtotal, splenectomy (PS) has become an accepted alternative to total splenectomy (TS) for management of hematologic disorders in children, but little is known about its long-term outcomes. Here, we present our institutional experience with partial splenectomy, to determine rate of subsequent TS or cholecystectomy and identify if any factors affected this need.METHODS:All patients who underwent partial splenectomy at a single tertiary children's hospital were retrospectively reviewed from 2002 through 2019 after IRB approval. Primary outcome of interest was rate of reoperation to completion splenectomy (CS) and rate of cholecystectomy. Secondary outcome were positive predictor(s) for these subsequent procedures.RESULTS:Twenty-four patients underwent PS, at median age 6.0 years, with preoperative spleen size of 12.7 cm by ultrasound. At median follow up time of 8.0 years, 29% of all patients and 24% of hereditary spherocytosis (HS) patients underwent completion splenectomy at median 34 months and 45 months, respectively. Amongst HS patients who did not have a cholecystectomy with or prior to PS, 39% underwent a delayed cholecystectomy following PS. There were no significant differences in age at index procedure, preoperative splenic volume, weight of splenic specimen removed, transfusion requirements, preoperative or postoperative hematologic parameters (including hemoglobin, hematocrit, total bilirubin, and reticulocyte count) amongst patients of all diagnoses and HS only who underwent PS alone compared to those who went on to CS. There were no cases of OPSS or deaths.CONCLUSION:Partial splenectomy is a safe alternative to total splenectomy in children with hematologic disease with theoretical decreased susceptibility to OPSS. However, families should be counseled of a 29% chance of reoperation to completion splenectomy, and, in HS patients, a 39% chance of delayed cholecystectomy if not performed prior to or with PS. Further studies are needed to understand predictors of these outcomes.

Hafezi, Niloufar MD; Pecoraro, Anthony MD; Landman, Matthew P. MD, FACS; Colgate, Cameron MS; Rescorla, Frederick J. MD, FACS Author Information

STUDY OBJECTIVE:Pediatric ovarian neoplasms with imaging appearance suggestive of teratoma are often presumed to have low risk of malignancy. We assessed the pre-operative imaging appearance of pediatric malignant ovarian germ cell tumors (MOGCT) and the presence of associated teratoma in a series of MOGCT.DESIGN:Retrospective review of clinical and pathology data.SETTING:Multicenter trial for extracranial malignant germ cell tumors in young female individuals by the Children's Oncology Group (COG study AGCT0132) that included yolk sac tumor, embryonal carcinoma and choriocarcinoma.PARTICIPANTS:Female individuals 0-20 years of age at enrollment with ovarian primary nonseminomatous malignant germ cell tumors.INTERVENTIONS:Review of data forms, including prospectively collected surgical checklist documenting imaging characteristics of the tumor, and review of pathology reports.MAIN OUTCOME MEASURES:Description of imaging appearance and frequency of mixed histology with benign teratoma elements.RESULTS:A total of 138 female individuals (11 months to 20 years of age) had primary ovarian tumors. Imaging appearance and pathology information were available for 133 patients. Among the 133 patients, tumor appearance was solid (10.5%), solid with calcification (3.0%), mixed cystic and solid (58.7%), mixed cystic and solid with calcification (24.8%), and unknown (3.0%). In all, 54% had elements of teratoma in addition to malignant histology.CONCLUSION:Mixed cystic and solid appearance with or without calcification was seen in 83.5% of pediatric ovarian malignant germ cell tumors. Associated benign teratoma was common. The presence of a mixed cystic and solid appearance on preoperative imaging should not dissuade the surgeon from obtaining preoperative serum markers and undertaking complete surgical staging.

418 Background: Patients with clinical stage I (CS I: cN0M0) germ cell tumors (GCT) exhibit favorable oncologic outcomes. While prognostic features can help inform treatment in adults with CS I GCT, we lack reliable means to predict relapse among pediatric patients. We sought to identify predictors of relapse in children with CS I GCT. Methods: We performed a pooled post hoc analysis on pediatric CS I GCT patients enrolled in 3 prospective trials: INT-0097 (phase II), INT-0106 (phase III), and AGCT0132 (phase III). Pathology was centrally reviewed. Patient demographics, pT stage, serum tumor markers, margin status, histology, relapse, and survival were compiled. Cox regression analyses were used to identify predictors of outcomes. Results: 88 patients were identified with histologic data available. Most patients were pT1-2 stage. Yolk sac tumor was present in 75%, while 16% had embryonal carcinoma, and 9% had choriocarcinoma. When evaluable, lymphovascular invasion (LVI) was present in 36/66 (55%) of patients. Over a median follow-up of 5.0 years, no patients died and 24 patients (27%) relapsed (median relapse-free survival not reached). Predictors of relapse included presence of choriocarcinoma (HR 4.3, p=0.004), embryonal carcinoma (HR 3.8, p=0.002), pT3 stage (HR 6.9, p=0.027), and age >12 years (HR 3.1, p=0.011). LVI (HR 2.4, p=0.072), serum tumor markers, and dominant tumor size did not reach significance. Pediatric CS I GCT patients exhibit remarkable 5-year survival. Conclusions: Using combined data from multiple prospective trials, our study identifies clinicopathologic features that predict relapse and potentially inform personalized treatment for these patients.

INTRODUCTION AND OBJECTIVE: Patients with clinical stage I (CS I: cN0M0) germ cell tumors (GCT) exhibit favorable oncologic outcomes. While prognostic features can help inform treatment in adults with CS I GCT, we lack reliable means to predict relapse among pediatric patients. We sought to identify predictors of relapse in children with CS I GCT. METHODS: We performed a pooled post hoc analysis on pediatric CS I GCT patients enrolled in 3 prospective trials: INT-0097 (phase II), INT-0106 (phase III), and AGCT0132 (phase III). Pathology was centrally reviewed. Patient demographics, pT stage, serum tumor markers, margin status, histology, relapse, and survival were compiled. Cox regression analyses were used to identify predictors of outcomes. RESULTS: 88 patients were identified with histologic data available. Most patients were pT1-2 stage. Yolk sac tumor was present in 75%, while 16% had embryonal carcinoma, and 9% had choriocarcinoma. When evaluable, lymphovascular invasion (LVI) was present in 36/66 (55%) of patients. Over a median follow-up of 5.0 years, no patients died and 24 patients (27%) relapsed (median relapse-free survival not reached). Predictors of relapse included presence of choriocarcinoma (HR 4.3, p=0.004), embryonal carcinoma (HR 3.8, p=0.002), pT3 stage (HR 6.9, p=0.027), and age >12 years (HR 3.1, p=0.011). LVI (HR 2.4, p=0.072), serum tumor markers, and dominant tumor size did not reach significance. Pediatric CS I GCT patients exhibit remarkable 5-year survival. CONCLUSIONS: Using combined data from multiple prospective trials, our study identifies clinicopathologic features that predict relapse and potentially inform personalized treatment for these patients. Source of Funding: None

Background: Long-gap esophageal atresia (LGEA) precludes immediate primary repair. When delayed primary esophagoesophagostomy (DPE) is not feasible, a reverse gastric tube (RGT) is a potential salvage option. The purpose of this study was to determine if DPE and RGT had both similar short-term and long-term outcomes. Methods: A retrospective review of all EA patients from 1994 to 2016 was undertaken. Data were stratified by surgical management (DPE versus RGT). Baseline demographics, operative information, postoperative management, and complications were analyzed. Descriptive statistics were used and P-values <0.05 were considered statistically significant. Results: Two hundred and eighteen patients with EA were treated during this period; 37/218 (17%) had LGEA. Mean gap length was 3.3 +/- 1.2 cm. Thirty-three patients underwent some form of repair, all of which were managed initially with a gastrostomy tube feeds. Twenty-five patients underwent DPE with 89% of these never requiring revision, and 86% having excellent function with long-term follow-up. In eight patients, esophageal length was never adequate for DPE; therefore, six were reconstructed with RGT, and two underwent gastric transposition. There were no significant differences in complications, revisions, ventilator days, overall length of stay, weight percentiles, or conduit function between children undergoing RGT compared with DPE at a mean follow-up of 5.5 years. Conclusions: Surgical treatment of LGEA is complex, and controversy exists regarding the optimal repair method when DPE is not feasible. In this series, DPE after gastrostomy tube feeds often allowed for sufficient esophageal lengthening with satisfactory long-term esophageal function. However, when adequate length for DPE was not attainable, these data suggest that RGT is a viable conduit with favorable postoperative outcomes. (C) 2020 Elsevier Inc. All rights reserved.

Rao, Raghavendra MD; Turk, Farheen NA; Landman, Matthew MD, FACS; Rescorla, Frederick J. MD, FACS Author Information

Purpose: The purpose of this study was to develop a pre-operative risk assessment tool for childhood and adolescent ovarian malignancy, in order to guide operative management of pediatric ovarian masses. Methods: We conducted a retrospective analysis of patients <18 years old who underwent ovarian surgery at two quaternary care pediatric centers over 4 years (1/1/13-12/31/16). Probability of malignancy was estimated based on imaging characteristics (simple cyst, heterogeneous, or solid), maximal diameter, and tumor markers (a-fetoprotein, beta-human chorionic gonadotropin). Results: Among 188 children with ovarian masses, 11% had malignancies. For simple cysts, there were no malignancies (0/24, 95% CI = 0-17%). Among solid lesions, 44% (15/34, 95% CI = 28-62%) were malignant. Among marker-elevated heterogeneous masses, 40% (2/5, 95% CI = 12-77%) were malignant. Conversely, small (<= 10 cm) and large (>10 cm) marker-negative heterogeneous lesions had malignancy proportions of 0% (0/39, 95% CI = 0-11%) and 5% (2/40, 95% CI = 1-18%), respectively. Conclusions: Given the malignancy estimates identified from these multi-institutional data, we recommend an attempt at ovarian-sparing resection for simple cysts or tumor marker-negative heterogeneous lesions <= 10 cm. Oophorectomy is recommended for solid masses or heterogeneous lesions with elevated markers. Finally, large (>10 cm) heterogeneous masses with non-elevated markers warrant a careful discussion of ovarian-sparing techniques. Complete surgical staging is mandatory regardless of operative procedure. Type of Study: Study of Diagnostic Test. (C) 2019 Elsevier Inc. All rights reserved.

Background: Ileal pouch-anal anastomosis (IPAA) is the standard surgical reconstruction for patients with familial adenomatous polyposis (FAP) and ulcerative colitis (UC) who undergo total proctocolectomy (TPC). Although patients receive the same reconstruction, their postoperative complications can differ. We hypothesize that indication for TPC and other preoperative clinical factors are associated with differences in postoperative outcomes following IPAA. Methods: A retrospective cohort of pediatric patients who underwent proctocolectomy with IPAA from 1996 to 2016 was identified. Preoperative, operative, and postoperative clinical variables were collected. Univariate analyses were performed to evaluate for relevant postoperative clinical differences. Results: Seventy-nine patients, 17 with FAP and 62 with UC, were identified. FAP patients spent a mean of 1125 +/- 1011 d between initial diagnosis and first surgery compared to 585 +/- 706 d by UC patients (P = 0.038). FAP patients took a mean of 57 +/- 38 d to complete TPC with IPAA compared to UC patients at 177 +/- 121 d (P < 0.001). FAP and UC patients did not differ in mean number of bowel movements at their 6-mo postoperative visit (4.7 +/- 2.1 versus 5.6 +/- 1.9, respectively [P = 0.134]). FAP patients were less likely to experience pouchitis (P = 0.009), pouch failure (P < 0.001), and psychiatric symptoms (P = 0.019) but more likely to experience bowel obstruction (P = 0.002). Conclusions: IPAA is a safe, restorative treatment for FAP and UC patients after TPC. Based on diagnosis and preoperative course, there are differences in morbidity in IPAA patients. Clinical data such as these will allow surgeons to help families anticipate their child's preoperative and postoperative courses and to maximize successful postoperative outcomes. (C) 2018 Elsevier Inc. All rights reserved.

Background: Esophageal achalasia is an uncommon condition in children. Although many interventions exist for the management of this disorder, esophageal (Heller) myotomy offers one of the most durable treatments. Our institution sought to review patients undergoing Heller myotomy concentrating on preoperative clinical factors that might predict postoperative outcomes. Materials and methods: All patients from January 1, 2007, to December 31, 2016, who underwent surgical treatment for achalasia at our tertiary pediatric hospital were identified and included in the study cohort. Electronic medical records for these patients were reviewed for clinical presentation variables, nonsurgical preoperative treatment, surgical approach, clinical response to surgery, need for postoperative treatment for ongoing symptoms, and high-resolution manometry (HRM) data. Results: Twenty-six patients were included in the study, and all underwent myotomy with partial fundoplication (median age: 14.4 y [interquartile range 11.6-15.5]). At a median follow-up of 9.75 mo (interquartile range 3.5-21 mo), 16 (61.5%) patients reported good resolution of their dysphagia symptoms with surgery alone. Two patients (7.7%) had perforation of the gastrointestinal tract requiring surgical intervention. Eight patients (30.8%) required additional treatment for achalasia, with 5 (19.2%) of these undergoing additional surgery or endoscopic treatment. Patients who had preoperative dilation did not have good resolution of their dysphagia (n = 2; P = 0.037). Two of four patients undergoing postoperative dilation had preoperative dilation. None of these patients underwent preoperative manometry. There was a statistically significant difference in the ages of patients who required postoperative intervention and those who did not (14.1 versus 15.2 y old, respectively; P = 0.043). In patients who reported improvement of gastroesophageal reflux disease/reflux type symptoms after Heller myotomy, lower esophageal residual pressure (29.1 versus 18.7 mmHg; P = 0.018) on preoperative HRM was significantly higher than in those who did not report improvement after surgery. Higher upper esophageal mean pressure (66.6 versus 47.8 mmHg; P = 0.05) also predicted good gastroesophageal reflux disease/reflux symptom response in a similar manner. Conclusions: Current analysis suggests that preoperative dilation should be used cautiously and older patients may have a better response to surgery without need for postoperative treatment. In addition, preoperative HRM can aid in counseling patients in the risk of ongoing symptoms after surgery and may aid in determining if a fundoplication should be completed at the index procedure. Further research is needed to delineate these factors. Level of evidence: Level III. (C) 2018 Elsevier Inc. All rights reserved.

We present a case of extensive gastric pneumatosis with portal venous air due to superior mesenteric artery syndrome in an underweight, developmentally normal seventeen-year-old boy. Despite his alarming imaging findings, his abdominal exam was benign and he was successfully treated nonoperatively.

Purpose The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB). Materials and Methods An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed. Results A total of 51 patients with a pathologic diagnosis of CPAM (n = 45; 88.2%) or PPB (n = 6; 11.8%) underwent surgical resection. Among patients treated for PPB, one death occurred approximately 13 months after diagnosis. Although four patients with PPB (four out of six; 66.7%) had radiographic indicators highly suggestive of malignancy prior to surgery, two had a preoperative diagnosis of CPAM (two out of six; 33.3%). Twenty-four patients (24 out of 45; 53.3%) with CPAM underwent resection after developing symptoms and 21 (21 out of 45; 46.7%) were symptomatic at the time of surgery. Mann-Whitney's tests revealed a statistically significant difference in postoperative length of stay (median: 6 vs. 3 days; p < 0.001) and days with thoracostomy tube in place (median 3 vs. 2 days; p = 0.003) for symptomatic versus asymptomatic patients, respectively. Conclusion CPAM patients appear to recover faster from surgery, if performed before the onset of symptoms. There may be a benefit to waiting until at least 3 months of age to complete resection in the asymptomatic patient. A low threshold for resection should be maintained in patients where delineating CPAM from PPB is difficult.

Purpose: Rectal prolapse is a commonly occurring and usually self-limited process in children. Surgical management is indicated for failures of conservative management. However, the optimal approach is unknown. The purpose of this study is to determine the efficacy of sclerotherapy for the management of rectal prolapse. Methods: This was a retrospective review of children <18 years with rectal prolapse who underwent sclerotherapy, predominantly with peanut oil (91%), between 1998 and 2015. Patients with imperforate anus or cloaca abnormalities, Hirschprung disease, or prior pull-through procedures were excluded. Results: Fifty-seven patients were included with a median age of 4.9 years (interquartile range (IQR) 3.2-9.2) and median follow-up of 52 months (IQR 8-91). Twenty patients (n =20/57; 35%) recurred at a median of 1.6 months (IQR 0.8-3.6). Only 3 patients experienced recurrence after 4 months. Nine of the patients who recurred (n =9/20; 45%) were re-treated with sclerotherapy. This was successful in 5 patients (n =5/9; 56%). Two patients (n=2/20; 10%) experienced a mucosal recurrence which resolved with conservative management. Forty-four patients were thus cured with sclerotherapy alone (n=44/57; 77%). No patients undergoing sclerotherapy had an adverse event. Thirteen patients (n=13/20; 65%) underwent rectopexy after failing at least one treatment of sclerotherapy. Three of these patients (n=3/13; 23%) recurred following rectopexy and required an additional operation. Conclusions: Injection sclerotherapy for children with rectal prolapse resulted in a durable cure of prolapse in most children. Patients who recur following sclerotherapy tend to recur within 4 months. Another attempt at sclerotherapy following recurrence is reasonable and was successful half of the time. Sclerotherapy should be the preferred initial treatment for rectal prolapse in children and for the initial treatment of recurrence. Type of study: Treatment Study. (C) 2017 Elsevier Inc. All rights reserved.