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To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs.Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.

Introduction Von Hippel-Lindau disease (VHL) is a rare cause of hereditary bilateral Pheochromocytomas (PHEO). Traditionally, treatment has been total adrenalectomy due to a lifetime risk of developing new tumors. Limited data exists on the surgical management of bilateral PHEO in children with VHL. We reviewed our experience with laparoscopic partial adrenalectomy for bilateral PHEO. Methods A retrospective review was performed of patients undergoing adrenalectomy for PHEO in children with VHL from 2004 to 2019. Results Eight children with VHL diagnosed with bilateral PHEO underwent 16 adrenalectomies (10 synchronous, 5 metachronous, 1 for recurrence). Median age at diagnosis was 13 [range 8–17] years with a median tumor size of 2.3 [range 0.5–7.7] cm. Of 16 adrenalectomies, all were performed laparoscopically, 14 were partial adrenalectomies; 2 patients required a contralateral total adrenalectomy due to size and diffuse multinodularity. There were no postoperative complications. No patients required corticosteroid replacement at the end of the study period. Two patients had new ipsilateral tumors identified after a median follow up of 5 [range 4–6] years with one undergoing repeat partial adrenalectomy. There were no mortalities in the study period. Conclusion Partial adrenalectomy for bilateral PHEO in patients with VHL is safe and does not compromise outcomes. When technically feasible, laparoscopic partial adrenalectomy should be considered as a primary surgical approach for children with VHL. Level of Evidence Level IV - Case series with no comparison group

Gallbladder duplication is an uncommon congenital anomaly with an estimated incidence of 1 in 4000 patients. Here we present a child who underwent a duodenal duplication cyst marsupialization who continued experiencing chronic abdominal pain one year after the initial procedure. She had an incidentally discovered duplicated gallbladder on ultrasound and an ejection fraction of 2% on hepatobiliary iminodiacetic acid (HIDA) scanning. Preoperative magnetic resonance cholangiopancreatography (MRCP) revealed two gallbladders with individual cystic ducts. She underwent an uneventful laparoscopic double cholecystectomy for biliary dyskinesia and subsequently had resolution of her chronic abdominal pain. Keywords: Duplicated gallbladder, Biliary dyskinesia, Pediatric, Cholecystectomy, Duodenal duplication

Purpose The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms. Methods A retrospective review of girls 2–21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated. Results Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. AFP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively). Conclusions Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population. Type of Study Retrospective Cohort Review. Level of Evidence Level III.

Hypocalcemia occurs frequently after a total thyroidectomy in pediatric patients. Four hour postoperative PTH monitoring predicts the need for calcium supplementation in the adult thyroidectomy population. We evaluated the role of the 4 h postoperative PTH level in determining the need for calcium supplementation after thyroidectomy in the pediatric population.This is a retrospective review of children undergoing total thyroidectomy by a single pediatric surgeon from July 2011 through July 2018. Intact PTH obtained four hours postoperatively determined the need for calcium supplementation for patients beginning in November 2014 onward. Serum total calcium levels were monitored concurrently with serum intact PTH levels. Serum calcium levels were followed in our Multispecialty Pediatric Endocrine Surgery clinic within the month following thyroidectomy.From July 2011 through July 2018, there were a total of 56 total thyroidectomies at our institution. Prior to November 2014, all pediatric total thyroidectomies received calcium supplementation per our institutional protocol. Based on ionized calcium levels, 26.3% (5/19) of children developed hypocalcemia. From November 2014 to July 2018, 37 pediatric patients required total thyroidectomies. 29.7% (11/37) had low 4-h postoperative PTH levels. 72.7% (8/11) patients with low 4-h postoperative PTH levels had corresponding postoperative day 1 total calcium levels less than 8.5 or ionized calcium levels less than 1.12, and five children (45.5%) developed symptomatic hypocalcemia. 70% (26/37) of children had normal 4-h postop PTH levels, with only 5 (19%) ever developing hypocalcemia. No patients with a normal postop PTH level developed symptomatic hypocalcemia or required IV calcium repletion. A single 4-h postoperative PTH10 pg/dl for identifying hypocalcemia has a sensitivity of 81% and specificity of 91%, with AUC 0.81.The 4-h postoperative serum PTH level can help determine the need for calcium supplementation in pediatric patients undergoing total thyroidectomy, thereby reducing unnecessary calcium supplementation and serial lab draws to monitor for postoperative hypocalcemia.Level II.

Purpose The purpose of this study was to evaluate the efficacy and risk profile of esophageal stents in the management of complicated pediatric esophageal disease. Methods An IRB-approved, single-center, retrospective review was performed on all pediatric patients (n = 13) who underwent esophageal stent placement (2005–2017). Demographic, perioperative, and outcome data were analyzed (p Results Forty-one stents were placed due to recalcitrant strictures (n = 36), perforations (n = 2), and/or fistulae (n = 3). Median age at initial stent placement was 23.8 months (range, 50 days to 16 years), and median stent duration was 36 days (range, 3–335). The recurrence rate for strictures after initial stent removal was 100%. Four (31%) children subsequently underwent definitive operative repair. There were 5 deaths, including 2 related to stent placement. Seventy-one percent of stents were associated with an adverse event, most commonly intraluminal migration (56%). Younger children experienced an increased risk for airway compression and retching (p = 0.010). Conclusion These data suggest that stents are associated with high complication rates and are not effective as definitive therapy for recalcitrant strictures in children. Although there may be a temporizing role for stents in selected patients, further refinements in stent technologies are needed to help manage this difficult patient population. Level of evidence Level IV

Background Laparoscopic intraoperative cholangiogram (IOC) with common bile duct exploration (CBDE) and endoscopic retrograde cholangiopancreatography (ERCP) are two therapeutic techniques for choledocholithiasis. The preferred technique is unclear. Materials and methods We identified subjects who underwent laparoscopic cholecystectomy (LC) and IOC/CBDE or ERCP from July 1, 2006, to December 31, 2016. We retrospectively reviewed 81 patients (≤ 18 y) who received these interventions for suspected choledocholithiasis. Main outcomes analyzed were success of intervention and complications. Results Of the 81 patients, 21 ERCPs and three endoscopic ultrasounds (EUSs) were performed before LC. Eighteen of 21 (85.7%) patients had stones or sludge cleared by ERCP, whereas 3 (14.3%) had normal common bile ducts without evidence of stones. Five of 24 (20.8%) had significant post-ERCP complications. Seven of 24 (29.2%) had more than one admission. Sixty of 81 patients underwent LC with IOC ± CBDE. Twenty one of 60 (36.2%) were found to have abnormal IOC. Eight of 15 (53.3%) attempted laparoscopic CBDE were successful. Eleven of 21 (52.4%) patients with abnormal IOC had post-LC ERCP (10) and EUS (1). Patients admitted to the Pediatric Surgery service were more likely to undergo LC first than ERCP/EUS (OR 3.46, 95% CI 1.26 to 9.45, P = 0.016). Patients undergoing LC first had a shorter length of stay (mean LOS 5.13 d versus 4.07, median 5.0 versus 3.0 d, P-value Conclusions Successful and safe laparoscopic treatment of choledocholithiasis is possible in the pediatric patient. A laparoscopic-first approach to suspected choledocholithiasis may reduce the number of procedures needed in this patient population.