Carcinoid tumors in children and adolescents: risk for second malignancies.

BACKGROUND: Carcinoid tumors (CTs) are rare in the pediatric population and are generally noted as an incidental finding on histopathologic examination. Cure is usually achieved with wide surgical excision. Second primary malignancies (SPM) of the gastrointestinal tract after CTs have been reported in 13% to 33% of affected adults. The risk of SPM appears highest after small bowel or appendiceal CTs and usually presents within 7 years from diagnosis. PURPOSE: The purpose of this study was to investigate the natural history of CTs in pediatric patients treated at a major children's hospital and to determine whether children and adolescents with primary CTs developed SPM during routine long-term follow-up. METHODS: We conducted a retrospective review of the medical records of children and adolescents with CTs diagnosed at Nationwide Children's Hospital, Columbus, Ohio between 1945 and 2012. RESULTS: Thirty-two patients with CT were identified, representing 0.48% of all malignancies diagnosed at Nationwide Children's Hospital. Mean age at presentation was 13 years (range, 8 to 20 y). The majority were appendiceal (87.5%) followed by bronchial (9.4%). Most of the appendiceal tumors presented with clinical appendicitis (25/28). Three had incidental appendectomies at the time of a planned abdominal surgery for other reasons. Four patients with appendiceal CTs had invasive features. All patients with appendiceal and bronchial CTs were successfully treated by complete surgical excision and were free of disease at an average of 7 years from diagnosis. None of our patients developed SPM during the period of observation (median 84 mo; range, 12 to 156 mo). CONCLUSIONS: In this single-institution retrospective review, survival of children with CT was excellent. No SPMs were observed over the period of follow-up differing from previously reported adult CT series.

Variability in gastroschisis management: a survey of North American pediatric surgery training programs.

BACKGROUND: The optimal surgical management of gastroschisis has yet to be determined. We sought to define the practice patterns in the management of gastroschisis, and to ascertain the degree of variability among and within pediatric surgical training programs. MATERIALS AND METHODS: An electronic survey was sent to all second-year residents in ACGME-accredited pediatric surgery programs in the United States and Canada. The questionnaire evaluated operative strategies, pain control, complications, and adherence to institutional protocols. RESULTS: Of the 38 pediatric surgical training programs, 27 second-year residents (71%) completed the survey. An institutional protocol was utilized in only one program, and 70% reported treatment variability among faculty. Attempted primary closure was the treatment of choice in 76% of centers, and routine silo placement at 24%. The location for routine silo placement was in the neonatal intensive care unit (77%), operating room (22%), and delivery room (1%). General anesthesia was used for all primary closures, while silos were placed using intravenous sedation at 36% of centers. The most frequent silo-related complication was dislodgement, reported by 80%. Other preformed silo complications included the inability to achieve primary fascial closure (27%) and intestinal injury (27%). When entering clinical practice, 74% of trainees stated that they would first attempt primary closure, while 22% favored routine placement of a preformed silo. CONCLUSIONS: Protocol-driven care of infants with gastroschisis is rare in pediatric surgery training centers, leading to great variability in care between institutions, as well as among faculty within single programs. Data-driven protocols may improve care of infants with gastroschisis.

Emerging ethical issues in pediatric surgery.

With the rapid pace of technological advancement and changing political, social, and legal attitudes, physicians face new ethical dilemmas. For pediatric surgeons, these emerging issues affect our relationship with, and the care we provide, to our patients and their families. In this review, we explore issues related to professionalism in pediatric surgery practice, the value of apology, and the risks associated with sleep deprivation. Furthermore, we discuss how the imperative of patient safety presents an opportunity for specialty-driven effort to define standards for the surgical care of children and a responsible process for introducing surgical innovations. Finally, we remind pediatric surgeons of their ethical and professional duty to support clinical research, and advocate the acceptance of community equipoise as sufficient basis for enrolling children in clinical trials.

Reasons for Data-Prompted Site Visits: Field Staff Findings and Review Committee Decisions.

BACKGROUND: A major component of the ACGME's Next Accreditation System (NAS) is the annual review of key performance indicators by each review committee (RC) for all programs under its oversight. The RC may request a site visit that is data-prompted for either a full review of all common and specialty-specific program requirements or a focused review of specific concerns for programs identified as underperforming. OBJECTIVE: The aims of this study were to: (1) identify the reasons that RCs requested data-prompted site visits; (2) describe the findings by accreditation field representatives as reflected in their site visit reports; and (3) summarize the accreditation decisions of RCs that followed the data-prompted site visits (DPSVs). METHODS: RC letters to programs informing them of a DPSV, site visit reports, and RC letters with accreditation decisions were reviewed for all programs having DPSVs from 2015 to 2020. RESULTS: DPSVs were performed in 312 programs, including 59 hospital-based, 122 medical-based, and 131 surgery-based programs; 214 programs had a single DPSV, and 98 programs had repeat DPSV. The most frequent reason that RCs requested a DPSV was noncompliance on the annual ACGME Resident/Fellow Survey. Notification of a DPSV prompted a change in program director in 7% of programs in the single DPSVs group and 57% of programs in the repeat DPSVs group. Surgery-based programs in the single and repeat DPSVs groups were more likely to receive an unfavorable accreditation status. The majority of programs in the single DPSVs group (78%) and repeat DPSVs group (70%) had a status of continued accreditation as of March 2020. CONCLUSIONS: Noncompliance on the Resident/Fellow survey was the most frequent reason that RCs requested a DPSV. The majority of programs in the single and repeat DPSV groups achieved a favorable accreditation status.

A parental educational intervention to facilitate informed consent for emergency operations in children.

BACKGROUND: In contrast to elective surgery, there are little data available on how to facilitate informed consent for emergency surgery. We hypothesized that in parents of children undergoing an emergency operation, portable computer technology would improve their perception of the adequacy of informed consent in the four domains of autonomy, beneficence, content, and assent. MATERIALS AND METHODS: This study is a quasi-experimental time series in which we prospectively compared two methods of preoperative education for parents of children undergoing appendectomy. The control group (N = 45) received standard preoperative discussion, whereas in the intervention group (N = 36) the preoperative education was facilitated by a portable computer presentation. Subjects completed a questionnaire following preoperative education to assess the extent to which informed consent was achieved immediately and at 3-4 weeks. RESULTS: Compared to control, initial ratings for the domains of informed consent tested were significantly higher in the intervention group: autonomy (N = 0.025), beneficence (N = 0.047), assent (N = 0.005), and content (N = 0.003). After 3 weeks, however, the advantage of the intervention group was preserved for the "content" domain, while ratings for "autonomy" significantly declined for both groups. CONCLUSION: A standardized portable computer presentation is an effective means of facilitating preoperative parental education for informed consent prior to emergency surgery in children. However, there is a decline in parental recall and perception of autonomy that is not affected by the strategy chosen for preoperative education.

General surgery versus specialty rotations: a new paradigm in surgery clerkships.

BACKGROUND: The most common 3rd-year surgery clerkship uses general surgery services while limiting the involvement of subspecialty services. A novel surgery clerkship in which students were assigned to either general surgery or subspecialty services for the entire clerkship was tried at a large Midwestern medical school. The purpose of this study was to investigate the outcomes of clerks from subspecialty services and compare them with clerks from general surgery services. METHODS: Outcome measures included scores on the National Board of Medical Examiners Surgery Content Examination, faculty evaluation scores, and residency match results. A multivariate analysis of covariance compared National Board of Medical Examiners test scores and faculty evaluation ratings across service groups. United States Medical Licensing Examination Step 1 scores served as a covariate. RESULTS: Results showed significant differences between groups on faculty evaluation scores (F = 28.03; P

Rare but serious complications of central line insertion.

Discussions on the complications of central venous catheterization in children typically focus on infectious and the more common mechanical complications of pneumothorax, hemothorax, or thrombosis. Rare complications are often more life-threatening, and inexperience may compound the problem. Central venous catheter complications can be broken down into early or late, depending on when they occur. The more serious complications are typically mechanical and occur early, but delayed presentations of pericardial effusions, cardiac tamponade, and pleural effusions may be of equal severity, and delay in diagnosis can be catastrophic. Careful insertion techniques, as well as continued vigilance in the correct position and function of central venous catheters, are imperative to help prevent serious complications.

Colonic diverticulitis in adolescents: an index case and associated syndromes.

Diverticular disease of the colon, a common problem among adults, is diagnosed rarely in children. We report an adolescent patient with sigmoid diverticulitis who required operative treatment. Pediatric patients with the complications of diverticula typically have conditions that result in genetic alterations affecting the components of the colonic wall. Our patient had Williams-Beuren syndrome, although Ehlers-Danlos syndrome, Marfan syndrome, and cystic fibrosis may also be associated with colonic diverticula in adolescence. Pediatric patients with these disorders who experience abdominal pain should be evaluated for the presence of colonic diverticular complications.

Endothelial nitric oxide synthase in human intestine resected for necrotizing enterocolitis.

OBJECTIVE: To determine the expression and function of endothelial nitric oxide synthase (eNOS) in submucosal arterioles harvested from human intestine resected for necrotizing enterocolitis (NEC) or congenital bowel disease. STUDY DESIGN: eNOS expression was determined by using immunohistochemistry. The arteriolar diameter was measured in vitro at pressures of 10 to 40 mm Hg and also in response to the eNOS agonist acetylcholine (ACh), the exogenous nitric oxide (NO) donor S-nitroso-N-acetylpenicillamine, and the smooth muscle relaxant papaverine. Arteriolar release of NO in response to ACh was determined with a Sievers NOAnalyzer. Hemodynamics were also determined at flow rates of 50 and 100 microL/min. RESULTS: eNOS was present in microvessels from both groups, but NEC arterioles failed to demonstrate physiological evidence of eNOS function: they constricted in response to pressure, failed to dilate or generate NO in response to ACh, and failed to dilate in response to flow. However, they dilated in response to exogenous NO and papaverine, indicating functional vascular smooth muscle and vasodilator reserve. CONCLUSION: eNOS-derived NO, a vasodilator in the newborn intestine, did not contribute to vasoregulation in arterioles harvested from intestine resected for NEC. These vessels were constricted; lack of eNOS-derived NO may contribute to this vasoconstriction.

Improvement in Context: Exploring Aims, Improvement Priorities, and Environmental Considerations in a National Sample of Programs Using "Small Data".

BACKGROUND: In 2013, the Accreditation Council for Graduate Medical Education (ACGME) transitioned into a new accreditation system to reduce burden, focus on outcomes, and promote innovation and improvement. One component is a self-study that includes aims, an environmental assessment, and setting improvement priorities. The ACGME initiated voluntary site visits following the self-study. OBJECTIVE: We explored common themes in program aims and assessment of their environment. METHODS: Using grounded theory, inductive and deductive qualitative methods, and truth grounding, we analyzed data from voluntary site visits of 396 core and subspecialty programs between June 2015 and September 2017, with a focus on common themes. RESULTS: We report common themes for aims and the dimensions of the environmental assessment. Themes for strengths include a collegial, supportive learning environment; responsive leaders; and experiences that prepare residents for unsupervised practice. Improvement priorities encompass low learner engagement and "content mismatch" in didactic education, balancing education and service at a time of growing clinical volumes, and improving the utility of assessment systems. Common opportunities encompass collaborations that improve education, involving alumni and harnessing technology to enrich education, while threats include an unsustainable effort for many program leaders, clinical pressures on faculty, and loss of external sites important for education. Linked dimensions of the environmental assessment suggest benefit in a growing focus on learners, and approaches to ensure a humanistic learning environment that allows for growth, self-determination, and inclusion. CONCLUSIONS: The findings highlight actionable themes for the environmental assessment. We discuss implications for programs, institutions, and the ACGME.

Program Strengths and Opportunities for Improvement Identified by Residents During ACGME Site Visits in 5 Surgical Specialties.

Background There is limited information about how residents in surgical specialties view program strengths and opportunities for improvement (OFIs). Objective This study aggregated surgical residents' perspectives on program strengths and OFIs to determine whether there was agreement in perspectives among residents in 5 surgical specialties. Methods Resident consensus lists of program strengths and areas for improvement were aggregated from site visits reports during 2012 and 2013 for obstetrics and gynecology, orthopaedic surgery, otolaryngology, plastic surgery, and surgery programs. Four trained individuals coded each strength or OFI in 1 of 3 categories: (1) factors common to all specialties; (2) program or institutional resources; and (3) factors unique to surgical specialties. Themes were classified as most frequent when listed by residents in more than 20% of the programs and less frequent when listed by residents in less than 20% of the programs. Results This study included a total of 359 programs, representing 27% to 49% of the Accreditation Council for Graduate Medical Education accredited programs in the 5 specialties. The most frequent strengths were progressive autonomy, collegiality, program leadership, and operative volume. Improving research and didactics, increasing faculty teaching and attendance at educational sessions, and increasing the number of nurse practitioners and physician assistants were common OFIs. Conclusions Factors identified as important by surgical residents related to their learning environment, their educational program, and program and institutional support. Across programs in the study, similar attributes were listed as both program strengths and OFIs.

Resident and Faculty Perceptions of Program Strengths and Opportunities for Improvement: Comparison of Site Visit Reports and ACGME Resident Survey Data in 5 Surgical Specialties.

Background Resident and faculty views of program strengths and opportunities for improvement (OFIs) offer insight into how stakeholders assess key elements of the learning environment. Objective This study sought (1) to assess the degree to which residents and faculty in 359 programs in 5 surgical specialties (obstetrics and gynecology, orthopaedic surgery, otolaryngology, plastic surgery, and surgery) were aligned or divergent in their respective views of program strengths and OFIs; and (2) to evaluate whether responses to selected questions on the Accreditation Council for Graduate Medical Education (ACGME) Resident Survey correlated with strengths or OFIs identified by the residents during the site visit. Methods Faculty and resident lists of program strengths and OFIs in site visit reports for 2012 and 2013 were aggregated, analyzed, and compared to responses on the Resident Survey. Results While there was considerable alignment in resident and faculty perceptions of program strengths and OFIs, some attributes were more important to one or the other group. Collegiality was valued highly by both stakeholder groups. Responses to 2 questions on the ACGME Resident Survey were associated with resident-identified OFIs in site visit reports pertaining to aspects of the didactic program and responsiveness to resident suggestions for improvement. Conclusions The findings offer program leadership additional insight into how 2 key stakeholder groups view elements of the learning environment as program strengths or OFIs and may serve as useful focal areas for ongoing improvement activities.

Ethical issues in the management of neonatal surgical anomalies.

This article provides a framework for thinking about three areas in neonatal surgery that contain potential moral and ethical concerns for pediatric surgeons and the parents of a newborn and/or fetus with a surgical anomaly. The utilization of life-sustaining therapy for neonates has made survival possible for many infants with serious birth defects. Sometimes the use of these treatments is problematic in terms of their actual benefit to the infant and the potential for enhancing their future quality of life. Second, the prenatal diagnosis of congential anomalies has made counseling of the prospective parents a routine part of pediatric surgical practice and raises the issue of how best to advise and support a couple whose fetus has a significant birth defect. Finally, pediatric surgeons have a responsibility to their patients and society to provide the highest quality of care. This may involve participation in multi-institutional clinical trials, so that the optimal care of a surgical neonate with a congenital or acquired condition is ascertained by rigorous prospective research evaluation.

Breast disorders in the adolescent patient.

Although breast cancer is rare in childhood and adolescence, breast concerns among patients in this age group are common. Benign proliferative changes and benign masses such as fibroadenomas are the most common entities encountered in the adolescent patient. Evaluation of breast complaints includes a careful history and physical examination. Ultrasonography is the best adjunctive radiologic modality to assess the adolescent breast. Surgical intervention usually is contraindicated in prepubertal patients. In the postpubertal patient, discrete breast masses, which are not suspicious on clinical examination, may be observed. Additionally, FNA and surgical removal are also safe diagnostic and therapeutic alternatives in this patient population.

Difficult central venous access removal: case reports of the use of endovascular snare shearing of endothelialized tetherings.

Although a fibrin sheath occurs in most long-standing central venous catheters, they do not typically interfere with complete removal of the catheter. We present 2 cases of long-standing catheters that could not be removed with simple surgical techniques because of endotheliazation via fibrous attachments to the venous wall. Both catheters were successfully removed using a modified snare technique through the right femoral vein.

Ultrasound prediction of growth abnormalities in fetuses with gastroschisis.

OBJECTIVE: We seek to determine whether (1) mean abdominal circumference (AC) of fetuses with gastroschisis is smaller than published normative values, (2) diagnosis of AC ≤ 2.5th percentile is supported by postnatal diagnosis of small-for-gestational age (SGA) and (3) adverse neonatal outcomes are more common in fetuses affected by gastroschisis with a sonographically measured small AC. METHODS: Retrospective review of pregnancies complicated with gastroschisis between 2000 and 2008. Patient demographics, method of closure, length of stay, use of ventilator support and gastrointestinal complications were compared. RESULTS: Seventy-four fetuses were identified with 368 ultrasound observations. Mean AC of fetuses with gastroschisis fell between the 2.5th and 50th percentile for gestational age. Thirty patients had AC measurements ≤ 2.5th of which 50% were SGA at delivery. Eleven of the 74 fetuses were diagnosed with intrauterine growth restriction (IUGR) and all were SGA. Birth weight was lower in those with a small AC (2104 g vs. 2665 g, p<0.001). There were no other differences in outcomes. CONCLUSION: AC values fell within the normal range of normative curves. Fifty percent of fetuses with small AC were SGA at birth. Neonatal outcomes in patients with small AC are similar to those with a normal AC.