Judicious DICER1 testing and surveillance imaging facilitates early diagnosis and cure of pleuropulmonary blastoma.

Pleuropulmonary blastoma (PPB) and Sertoli-Leydig cell tumor (SLCT) are both associated with germline mutations in DICER1. In this brief report, a maternal history of SLCT led to identification of a deleterious DICER1 mutation in the patient and her asymptomatic infant. Radiographic screening revealed a large Type I PPB, which was completely resected. Identification of DICER1 mutation carriers and imaging of children at risk for PPB may allow detection of PPB in its earliest and most curable form, leading to increased likelihood of surgical cure and decreased risks of treatment-related late effects.

Management of the undescended testis in children: An American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee Systematic Review.

PURPOSE: Management of undescended testes (UDT) has evolved over the last decade. While urologic societies in the United States and Europe have established some guidelines for care, management by North American pediatric surgeons remains variable. The aim of this systematic review is to evaluate the published evidence regarding the treatment of (UDT) in children. METHODS: A comprehensive search strategy and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were utilized to identify, review, and report salient articles. Five principal questions were asked regarding imaging standards, medical treatment, surgical technique, timing of operation, and outcomes. A literature search was performed from 2005 to 2020. RESULTS: A total of 825 articles were identified in the initial search, and 260 were included in the final review. CONCLUSIONS: Pre-operative imaging and hormonal therapy are generally not recommended except in specific circumstances. Testicular growth and potential for fertility improves when orchiopexy is performed before one year of age. For a palpable testis, a single incision approach is preferred over a two-incision orchiopexy. Laparoscopic orchiopexy is associated with a slightly lower testicular atrophy rate but a higher rate of long-term testicular retraction. One and two-stage Fowler-Stephens orchiopexy have similar rates of testicular atrophy and retraction. There is a higher relative risk of testicular cancer in UDT which may be lessened by pre-pubertal orchiopexy.

Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

The effects of early anesthesia on neurodevelopment: A systematic review.

BACKGROUND: There is growing concern regarding the impact of general anesthesia on neurodevelopment in children. Pre-clinical animal studies have linked anesthetic exposure to abnormal central nervous system development, but it is unclear whether these results translate into humans. The purpose of this systematic review from the American Pediatric Surgical Association (APSA) Outcomes and Evidence-Based Practice (OEBP) Committee was to review, summarize, and evaluate the evidence regarding the neurodevelopmental impact of general anesthesia on children and identify factors that may affect the risk of neurotoxicity. METHODS: Medline, Cochrane, Embase, Web of Science, and Scopus databases were queried for articles published up to and including December 2017 using the search terms "general anesthesia and neurodevelopment" as well as specific anesthetic agents. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to screen manuscripts for inclusion in the review. A consensus statement of recommendations in response to each study question was synthesized based upon the best available evidence. RESULTS: In total, 493 titles were initially identified, with 56 articles selected for full analysis and 44 included for review. Based on currently available developmental assessment tools, a single exposure to general anesthesia does not appear to have a significant effect on general neurodevelopment, although prolonged or multiple anesthetic exposures may have some adverse effects. Exposure to general anesthesia may affect different domains of development at different ages. Regional anesthetic techniques with the addition of dexmedetomidine and/or some intravenous agents may mitigate the risks of neurotoxicity. This approach may be performed safely in some patients and can be considered as an option in selected short procedures. CONCLUSION: There is no conclusive evidence that a single short anesthetic in infancy has a detectable neurodevelopmental effect. Data do not support waiting until later in childhood to perform general anesthesia for single short procedures. With the complexities and nuances of different anesthetic methods, patients and procedures, the planning and execution of anesthesia for the pediatric patient is generally best accomplished by an anesthesiologist, ideally a pediatric anesthesiologist. TYPE OF STUDY: Systematic review of level 1-4 studies. LEVEL OF EVIDENCE: Level 1-4 (mainly level 3-4).

Management of intussusception in children: A systematic review.

OBJECTIVE: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to develop recommendations for the management of ileocolic intussusception in children. METHODS: The ClinicalTrials.gov, Embase, PubMed, and Scopus databases were queried for literature from January 1988 through December 2018. Search terms were designed to address the following topics in intussusception: prophylactic antibiotic use, repeated enema reductions, outpatient management, and use of minimally invasive techniques for children with intussusception. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Consensus recommendations were derived based on the best available evidence. RESULTS: A total of 83 articles were analyzed and included for review. Prophylactic antibiotic use does not decrease complications after radiologic reduction. Repeated enema reductions may be attempted when clinically appropriate. Patients can be safely observed in the emergency department following enema reduction of ileocolic intussusception, avoiding hospital admission. Laparoscopic reduction is often successful. CONCLUSIONS: Regarding intussusception in hemodynamically stable children without critical illness, pre-reduction antibiotics are unnecessary, non-operative outpatient management should be maximized, and minimally invasive techniques may be used to avoid laparotomy. LEVEL OF EVIDENCE: Level 3-5 (mainly level 3-4) TYPE OF STUDY: Systematic Review of level 1-4 studies.

Safety culture among pediatric surgeons: A national survey of attitudes and perceptions of patient safety.

PURPOSE: Improving the culture of safety within health care is an essential component of preventing errors and improving overall health care quality. The purpose of this study was to characterize the attitudes and perceptions of patient safety among pediatric surgeons. METHODS: We conducted a cross-sectional online survey of American Pediatric Surgery Association members. Survey items assessed surgeons' knowledge, attitudes, and perceptions of patient safety. We performed descriptive statistics and evaluated associations between respondent characteristics and survey responses. RESULTS: Response rate was 38% (353/928). Surgeons in academic practice (96% vs 83% private, P=0.01) and in leadership positions (98% vs 92%, P=0.03) were more likely to feel actively engaged in patient safety initiatives. Surgeons in private practice were less likely to feel safe having their own children undergo surgery at their institution (80% vs 96% academic, P<0.005). CONCLUSION: Pediatric surgeons have disparate attitudes and perceptions of patient safety within their hospitals. Significant variation exists based on surgeon characteristics. These findings underscore the need to identify barriers to surgeon engagement and develop educational initiatives to empower surgeons as leaders in improving patient safety culture. LEVEL OF EVIDENCE: V.

Laparoscopic duodenal atresia repair using surgical U-clips: a novel technique.

Laparoscopic repair of duodenal atresia has been reported. Reports to date have indicated use of standard laparoscopic suturing and knot tying. Unfortunately, there has been a high leak rate associated with the technique. We report our technique of using U-clips for the duodenoduodenostomy, thus limiting trauma to the duodenum during the anastomosis and less risk for postoperative leakage.

Laparoscopic evaluation for contralateral patent processus vaginalis in children with unilateral inguinal hernia.

BACKGROUND: Management of the contralateral inguinal region in children with a unilateral inguinal hernia remains controversial. The role of laparoscopy in evaluation for contralateral patent processus vaginalis remains unclear. We report the results of an investigation of 1676 consecutive children younger than 10 years of age who underwent unilateral inguinal hernia repair between May 1992 and January 2003. MATERIALS AND METHODS: Bilateral inguinal hernias were noted preoperatively in 194 of the total of 1870 patients, leaving 1676 patients in the study group. In all patients, the contralateral inguinal region was examined under general anesthesia and the operating surgeon noted whether or not a contralateral patent processus vaginalis was suspected. All patients then underwent attempted laparoscopic evaluation of the contralateral inguinal region at the time of unilateral inguinal hernia repair. RESULTS: Laparoscopy was successful in 1603 cases (95.6%) but 73 patients (4.4%) had hernia sacs that were too thin to allow insertion of a 3-mm cannula and 2.7-mm 70-degree telescope. A contralateral patent processus vaginalis was identified in 643 of the laparoscopically examined children (40.1%). At the examination, it was predicted that 446 of the 1603 patients would have a contralateral patent processus vaginalis. Laparoscopy confirmed the presence of a contralateral patent processus vaginalis in 192 (43.0%) of the children predicted to have a contralateral patent processus vaginalis. A contralateral patent processus vaginalis was not suspected from the examination under anesthesia in 1157 of the examined children, and the absence of a contralateral patent processus vaginalis was confirmed by laparoscopy in 706 of these children (61.0%); however, a contralateral patent processus vaginalis was found in 451 (39.0%) of this group. CONCLUSION: We conclude that examination under anesthesia is a poor predictor for the presence or absence of a contralateral patent processus vaginalis. Laparoscopy can reliably evaluate the contralateral inguinal region and is the best method to evaluate for the presence of a contralateral patent processus vaginalis.

Laparoscopic excision of a giant splenic vascular tumor.

The splenic vascular tumor referred to as a hemangioma is rare and typically presents as a small asymptomatic lesion. We report a case of a giant splenic cyst in a 13-year-old boy with abdominal distension. He underwent laparoscopic excision of the splenic cyst without complication. Pathology revealed a vascular tumor. At 15 months of follow-up, he continued to be asymptomatic, and abdominal ultrasound showed no recurrence of his disease. Laparoscopic excision of giant splenic cysts is a viable option in children, allowing for preservation of normal splenic tissue.

Epidural vs patient-controlled analgesia for postoperative pain after pectus excavatum repair: a prospective, randomized trial.

PURPOSE: Management of postoperative pain is a challenge after the minimally invasive repair of pectus excavatum. Pain is usually managed by either a thoracic epidural or patient-controlled analgesia with intravenous narcotics. We conducted a prospective, randomized trial to evaluate the relative merits of these 2 pain management strategies. METHODS: After obtaining permission/assent (Institutional Review Board no. 06 08 128), patients were randomized to either epidural or patient-controlled analgesia with fixed protocols for each arm. The primary outcome variable was length of stay with a power of .8 and α of .05. RESULTS: One hundred ten patients were enrolled. There was no difference in length of stay between the 2 arms. A longer operative time, more calls to anesthesia, and greater hospital charges were found in the epidural group. Pain scores favored epidural for the few days and favored patient-controlled analgesia thereafter. The epidural catheter could not be placed or was removed within 24 hours in 12 patients (22%). CONCLUSIONS: There is longer operating room time, increase in calls to anesthesia, and greater hospital charges with epidural analgesia after repair of pectus excavatum. Pain scores favor the epidural approach early in the postoperative course and patient-controlled analgesia later.

The utility of computed tomography in the management of patients with spontaneous pneumothorax.

BACKGROUND: Spontaneous pneumothorax may result from rupture of subpleural blebs. Computed tomography (CT) has been used to identify blebs to serve as an indication for thoracoscopy. We reviewed our experience with spontaneous pneumothorax to assess the utility of CT in these patients. METHODS: A retrospective review was conducted of all patients who underwent an operation for spontaneous pneumothorax from January 1999 to October 2009. All procedures were performed thoracoscopically. RESULTS: We identified 39 pneumothoraces in 34 patients who underwent evaluation and a procedure for spontaneous pneumothorax. Mean age was 16.1 years (range, 10-23 years), with an average of 1.7 spontaneous pneumothoraces before operation (range, 1-4). Preoperative chest CT scans were obtained in 26 cases. Blebs were demonstrated on 8 CT scans. The presence of blebs was confirmed at operation in all 8 patients. Of the 18 negative scans, 14 (77.8%) were found to have blebs intraoperatively, 7 of these patients were initially managed nonoperatively and developed recurrence. The sensitivity of CT for identifying blebs was 36%. CONCLUSIONS: Chest CT does not appear to be precise in the identification of pleural blebs and a negative examination does not predict freedom from recurrence. Operative decisions should be based on clinical judgment without the use of preoperative CT.

Laparoscopic adrenalectomy in children: a multicenter experience.

INTRODUCTION: Laparoscopic adrenalectomy is now being recognized as the standard approach for adrenalectomy for benign lesions in adults. The published experience in children and adolescents has been limited to sporadic small case series. Therefore, we conducted a large multicenter review of children who have undergone laparoscopic adrenalectomy. METHODS: After Institutional Review Board's approval, a retrospective review was conducted on all patients who have undergone laparoscopic adrenalectomy at 12 institutions over the past 10 years. Operative times included unilateral adrenalectomy without concomitant procedures. RESULTS: About 140 patients were identified (70 males [50%]). Laterality included 76 (54.3%) left-sided lesions, 59 (42.1%) right, and 5 (3.6%) bilateral. Mean operative time was 130.2 ± 63.5 minutes (range 43-406 minutes). The most common pathology was neuroblastoma in 39 cases (27.9%), of which 23 (59.0%) had undergone preoperative chemotherapy. Other common pathology included 30 pheochromocytomas (21.4%), 22 ganglioneuromas (15.7%), and 20 adenomas (14.3%). There were 13 conversions to an open operation (9.9%). Most conversions were because of tumor adherence to surrounding organs, and tumor size was not different in converted cases (P=.97). A blood transfusion was required in 2 cases. The only postoperative complication was renal infarction after resection of a large neuroblastoma that required skeletonization of the renal vessels. At a median follow-up of 18 months, there was only one local recurrence, which was in a patient with a pheochromocytoma. CONCLUSIONS: The laparoscopic approach can be applied for adrenalectomy in children for a wide variety of conditions regardless of age with a 90% chance of completing the operation without conversion. The risk for significant blood loss or complications is low, and it should be considered the preferred approach for the majority of adrenal lesions in children.

Giant ossifying malignant thymoma in a child.

We present the first reported case of an ossifying pediatric thymoma. Our patient was diagnosed with a massive thymoma replacing the whole of the left thoracic cavity. Percutaneous biopsy was attempted 3 times followed by an open incisional biopsy and adjuvant chemotherapy. Complete resection required a median sternotomy and a "trap door" thoracotomy after the tumor failed to respond to chemotherapy. Histology confirmed World Health Organization type B1 lymphocyte-rich thymoma, Masaoka stage I, with extensive osseous metaplasia.

Does an upper gastrointestinal study change operative management for gastroesophageal reflux?

BACKGROUND: In patients with gastroesophageal reflux disease, an upper gastrointestinal (UGI) contrast study is often the initial study performed for those patients being considered for fundoplication. The accuracy of UGI for diagnosing reflux is known to be poor, but there are no data on how often this study influences management. Therefore, we reviewed our experience in patients undergoing fundoplication to quantify the impact of the UGI. METHODS: A retrospective analysis of our most recent 7-year experience with patients undergoing fundoplication was performed. Results of the diagnostic tests and operative course were recorded. RESULTS: From January 2000 to June 2007, 843 patients underwent fundoplication. An UGI study was obtained in 656 patients. A pH study was also performed in 379 of these patients who had an UGI. The sensitivity of the UGI for reflux compared with pH study was 30.8%. An abnormality besides gastroesophageal reflux disease or hiatal hernia that impacted the operative plan was found on the UGI in 30 patients (4.5%). The most common anomaly was malrotation, which was found in 26 patients (4.0%). Malrotation was confirmed in 16 patients and ruled out in 6 patients during fundoplication, and 4 patients had undergone a previous Ladd procedure. Esophageal dilation was performed in 5 patients with the fundoplication for a stricture found on the UGI. Pyloroplasty was performed with the fundoplication in 2 patients, and 1 patient underwent exploration of the duodenum for possible obstruction. CONCLUSIONS: The UGI study is a poor study for accurately delineating which patients have pathologic reflux. However, it reveals a finding that may influence management in approximately 4% of cases.

Management of pediatric acute appendicitis in the computed tomographic era.

BACKGROUND/PURPOSE: The treatment options for complicated appendicitis in children continue to evolve. Optimal management of complicated appendicitis relies on an accurate preoperative diagnosis. We examined the accuracy of our preoperative diagnosis including computed tomography (CT) and the influence on the management of children with perforated and nonperforated appendicitis. METHODS: Following IRB approval, a 6-year review of all patients that underwent an appendectomy for suspected appendicitis was performed. Treatments included immediate operations and initial nonoperative management (antibiotic therapy +/- percutaneous drainage of abscess). Appendicitis was confirmed by histological examination. RESULTS: One thousand seventy-eight patients underwent appendectomy for suspected appendicitis. Preoperative CT scans were performed in 697 (64.7%) patients: 615 (88.2%) positive for appendicitis; 42 (6.0%) negative; and 40 (5.7%) equivocal. One hundred seventy-three (28.1%) positive CT scans further suggested perforation. Initial nonoperative management was initiated in 39 (22.5%) cases of suspected perforated appendicitis with abscess. The positive-predictive value (PPV) for suspected acute appendicitis based on history and physical examination alone was 90.8%. The PPV for positive CT scan for acute appendicitis was 96.4% with a PPV of 91.9% for positive CT scan for perforated appendicitis. CONCLUSIONS: The correct preoperative diagnosis of appendicitis appears statistically more accurate with CT scan compared to history and physical examination alone (PPV 96.4% versus 90.8%, P = 0.045). For those with clinically suspicious complicated appendicitis, CT evaluation may direct therapy toward initial nonoperative management. The efficacy of this regimen warrants further investigation.

Abdominal complications related to type of repair for congenital diaphragmatic hernia.

BACKGROUND: Literature on congenital diaphragmatic hernia (CDH) over the past few decades has focused on prognostic factors and management of pulmonary hypertension/hypoplasia. Larger diaphragmatic defects may require patch closure, reported by some authors to be associated with poorer outcomes. In this study, we evaluate the impact synthetic material has on the need for subsequent abdominal operations, particularly recurrence and small bowel obstruction (SBO). METHODS: After obtaining IRB approval, all patients undergoing repair of congenital diaphragmatic from January, 1994 to December, 2004 were investigated. Records from primary and subsequent admissions were reviewed to identify those patients who underwent major procedures after repair of the diaphragmatic defect. Subsequent abdominal operations in these series were recurrent CDH repair, exploration for SBO and fundoplication. Patients who died prior to hospital discharge were excluded. Statistical comparisons were made using Fisher's exact test: significance was defined as P<0.05. RESULTS: During the study period, there were 81 survivors from CDH repair, 24 with a synthetic patch, and 57 without. Those with a patch repair had a significantly increased risk of recurrence, small bowel obstruction, and subsequent operation (Table 1). Eleven patients had nonabsorbable mesh patches, and 13 were repaired with absorbable (Surgisis-Gold; Cook Technology Inc., West Lafayette, IN). While there were no differences in recurrence between these two groups, four patients (31%) with Surgisis developed SBO compared with one patient (9%) repaired with a nonabsorbable synthetic. CONCLUSIONS: Incidence of SBO and recurrent CDH in patients with a patch was higher than those who underwent primary repair. There may also be a difference in the rate of subsequent SBO depending on the type of mesh used. A prospective trial is under way at our institution to help define this issue.

Use of esophagocrural sutures and minimal esophageal dissection reduces the incidence of postoperative transmigration of laparoscopic Nissen fundoplication wrap.

OBJECTIVES: Herniation of the fundoplication wrap through the esophageal hiatus is a common reason for surgical failure in children who have undergone laparoscopic Nissen fundoplication. Extensive mobilization of the gastroesophageal junction in combination with decreased adhesions after laparoscopy may contribute to the development of this complication. In an attempt to decrease the incidence of wrap migration, we changed our technique to minimal mobilization of the intraabdominal esophagus and to placement of esophageal-crural sutures. In this study, we investigate the impact of these modifications on outcome. METHODS: A retrospective analysis was performed on all patients undergoing laparoscopic fundoplication by the senior author (GWH) from January 2000 through December 2004. Those undergoing operation with extensive esophageal mobilization and without esophagocrural sutures (January 2000 to March 2002) (group I) were compared with those in whom there was minimal esophageal dissection with placement of these esophagocrural sutures (April 2002 to December 2004) (group II). RESULTS: Two hundred forty-nine patients underwent laparoscopic Nissen fundoplication during the study period. One hundred thirty patients were in group I, and 119 patients were in group II. The rate of transmigration decreased from 12% in group I to 5% in group II (P = .072). The relative risk of transmigration with extensive esophageal mobilization and without the esophagocrural sutures was 2.29. CONCLUSIONS: This retrospective study has shown that placement of esophagocrural sutures and minimization of the dissection around the esophagus results in a more than 2-fold reduction in the risk of wrap transmigration after laparoscopic Nissen fundoplication.

VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature.

The VACTERL complex refers to anomalies of the bony spinal column (V), atresias in the gastrointestinal tract (A), congenital heart lesions (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R) and limb lesions (L). The incidence of each of these components has not been precisely quantified in the recent literature and the full array of anomalies within each systemic class of the VACTERL complex has not been well described. Therefore, we reviewed our most recent 20-year experience of patients born with esophageal atresia to comprehensively delineate and accurately describe the type and incidence of associated lesions. A retrospective review was then conducted on all patients diagnosed with esophageal atresia between 1985 and 2005. Patient demographics recorded included gestational age, weight and gender. The specific types of lesions were carefully cataloged. The outcome measure recorded was survival. One hundred and twelve patients were diagnosed with esophageal atresia were identified during the study period. The gestational age range was 28-41 weeks with an average of 36.5 weeks. Average birth weight was 2,557 g (range 1,107-3,890). A male predominance was seen with 62 males and 50 females. The overall survival was 92.9%. The categorical breakdown of anomalies were vertebral (24.1%), atresia (14.3%), cardiac (32.1%), tracheoesophageal fistula (95.5%), urinary (17.0%), skeletal (16.1%) and other (10.8%). VACTERL anomalies are common in patients with esophageal atresia, however, they appear to have little impact on overall survival.