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.

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.

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.

Does routine nasogastric tube placement after an operation for perforated appendicitis make a difference?

BACKGROUND: Divergent opinions exist regarding the routine use of nasogastric (NG) tubes in the postoperative management of patients undergoing abdominal surgery. Empiric use of an NG tube after abdominal surgery is presumed to prevent abdominal distension, vomiting, and ileus, which may complicate the postoperative course. To investigate the validity of this assumption, we compared the postoperative course of patients who underwent appendectomy for perforated appendicitis who subsequently either had or did not have an NG tube placed postoperatively. METHODS: A retrospective chart review of all children operated for perforated appendicitis between 1999 and 2004 was performed. Patients with prolonged hospitalizations were excluded to eliminate bias created by patients with multiple operations and opportunities for NG placement. The use of an NG tube, time to first and to full oral feeds, length of hospitalization, and complications were compared between groups. RESULTS: Patients with NG tubes left in place (N = 105) were compared with those who did not receive an NG tube (N = 54) following appendectomy for perforated appendicitis. Mean time to first oral intake was 3.8 d in those with NG tubes compared with 2.2 d in those without NG tubes (P < 0.001). Similarly, mean time to full feeds was 4.9 d when an NG tube was left compared with 3.4 d in those without tubes (P < 0.001). Mean length of stay was 6.0 d in those with NG tubes compared to 5.6 d in those without (P = 0.002). CONCLUSIONS: The use of NG decompression after an operation for perforated appendicitis does not appear to improve the postoperative course and we recommend that it is not routinely used in this patient population.

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.

Adhesive small bowel obstruction after appendectomy in children: comparison between the laparoscopic and open approach.

INTRODUCTION: Adhesive small bowel obstruction (SBO) is a common postoperative complication. Published data in the pediatric literature characterizing SBO are scant. Furthermore, the relationship between the risk of SBO for a given procedure is not well described. To evaluate these parameters, we reviewed the incidence of SBO after laparoscopic appendectomy (LA) and open appendectomy (OA) performed at our institution. METHODS: With institutional review board approval, all patients that developed SBO after appendectomy for appendicitis from January 1998 to June 2005 were investigated. Hospital records were reviewed to identify the details of their postappendectomy SBO. The incidences of SBO after LA and OA were compared with chi2 analysis using Yates correction. RESULTS: During the study period, 1105 appendectomies were performed: 477 OAs (8 converted to OA during laparoscopy) and 628 LAs. After OA, 7 (6 perforated appendicitis) patients later developed SBO of which 6 required adhesiolysis. In contrast, a patient with perforated appendicitis developed SBO after LA requiring adhesiolysis (P = .01). The mean time from appendectomy to the development of intestinal obstruction for the entire group was 46 +/- 32 days. CONCLUSIONS: The overall risk of SBO after appendectomy in children is low (0.7%) and is significantly related to perforated appendicitis. Small bowel obstruction after LA appears statistically less common than OA. Laparoscopic appendectomy remains our preferred approach for both perforated and nonperforated appendectomy.

The use of fundoplication for prevention of apparent life-threatening events.

OBJECTIVE: Gastroesophageal reflux disease (GERD) is cited by many to be a common cause of apparent life-threatening events (ALTEs). However, there are few reports in the literature regarding the surgical treatment of GERD to prevent a recurrent ALTE. METHODS: A retrospective review of infants undergoing fundoplication between 2000 and 2005 for the prevention of another ALTE was undertaken. Preoperative, operative, and postoperative data as well as follow-up information were collected. RESULTS: During the study period, 81 patients underwent fundoplication after presenting with an ALTE. All but 3 patients (96.3%) had been treated with antireflux medication. Moreover, 71 infants (87.7%) were taking antireflux medication at the time of their ALTE. A significant number of infants (77.8%) were hospitalized with a second ALTE before referral for fundoplication. After fundoplication, only 3 patients (3.7%) experienced a recurrent ALTE during the follow-up period; 2 required a second fundoplication and 1 underwent pyloromyotomy. None of these 3 patients have experienced a recurrent ALTE after the second operation. The median follow-up has been 1738 days. CONCLUSION: Our data suggest that among patients who had an ALTE and are found to have GERD, fundoplication appears to be an effective method for preventing recurrent ALTE.

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.