Use of magnets in pediatric surgery.

Since the 1970s, magnets have been progressively harnessed for use in minimally invasive treatment of pediatric surgical disease. In particular, multiple magnetic devices have been developed for treating esophageal atresia, pectus excavatum and scoliosis. These devices, which can be placed via small incisions or under endoscopic or fluoroscopic guidance, provide the added benefit of sparing patients multiple large, invasive procedures, and allowing for gradual correction of congenital anomalies over days to months, depending on the disease. In the following text, we detail the current landscape of magnetic devices used by pediatric surgeons, illustrate their use through clinical cases, and review the available body of literature with respect their outcomes and complications.

Sleeve gastrectomy for the treatment of adolescent obesity in children aged 13 and under: a retrospective study.

BACKGROUND: Sleeve gastrectomy (SG) induces weight reduction and improves metabolic co-morbidities in children with severe obesity but remains underutilized, especially for young adolescents and preadolescents. OBJECTIVE: We hypothesized there would be no differences in weight loss or co-morbidity resolution at 1 year post-SG in children who underwent SG at 13 years or younger compared to children who underwent SG at 17-18 years old. SETTING: Academic medical center, United States. METHODS: Medical records of children who underwent laparoscopic SG at a quaternary academic center from September 2014 to October 2022 were reviewed. A cohort of 15 patients, ≤13 years of age, was compared to a matched cohort of 15 older adolescent patients. Preoperative characteristics and postoperative outcomes were collected. RESULTS: Both cohorts had similar baseline characteristics. Median preoperative body mass index (BMI) was 51.8 kg/m2 for the ≤13 cohort compared to 50.9 kg/m2 in the older cohort (P = .87). Time to postoperative enteral feeds and length of stay were similar between both groups, and there were no 30-day readmissions or immediate postoperative complications. Median percentage excess BMI loss at 1 year postoperation was 54% (IQR, 25.5%-94.5%) for the ≤13 cohort compared with 44% (IQR, 34.0%-51.0%) for the older cohort (P = .34). Two of 11 patients were lost to follow-up in the younger group compared to 4 of 15 in the older group (P = .61). Both groups demonstrated significant improvement in metabolic syndrome co-morbidities after SG. CONCLUSION: SG in younger children is associated with successful postsurgical outcomes compared with adolescents, with effective weight loss and improvement of obesity-related metabolic co-morbidities.

Outcomes after metabolic and bariatric surgery in preteens versus teens using the Metabolic and Bariatric Surgery Accreditation and Quality Improvement Program database and center-specific data.

OBJECTIVE: The American Academy of Pediatrics (AAP) recently released clinical guidelines for the treatment of childhood obesity, including surgery being appropriate for children 13 years of age and older. The use of this age cut-off was due to a lack of data for children younger than 13. To address this knowledge gap, the Metabolic and Bariatric Surgery Accreditation and Quality Improvement Program (MBSAQIP) database was queried to compare outcomes in preteens to teens after bariatric surgery hypothesizing that there would be no difference in outcomes between the two groups. METHODS: Patients from the MSAQIP database (2016-2021) were identified and divided into groups <13 years and 13-18 years and were matched using propensity scores based on race, sex, and preoperative BMI. Outcomes were compared including change in BMI, complication rates, 30-day readmission or reoperation, and mortality. Additionally, the centers responsible for the bulk of the preteen patient entries queried their center-specific databases to evaluate weight loss over time. RESULTS: A total of 4755 patients were identified, 47 of whom were <13 years of age. Preteens had similar sex distribution (66% vs. 75% female), were more likely to be Black (27.7% vs. 18.3%) or Hispanic (21.3% vs. 7.6%) race, and weighed less (274 ± 58 vs. 293 ± 85 lb, p = 0.01), but they had similar BMI (46.9 ± 7 vs. 47 ± 13 kg/m2 ) as their teen counterparts. Preteens were more likely to suffer from sleep apnea (34% vs. 19%, p < 0.01) and insulin-dependent type 2 diabetes (10.6% vs. 1.8%, p < 0.01). There were no complications in the preteens compared to teens (0% vs. 0.5%), and they did not undergo any unplanned readmissions (0% vs. 2.9%) or reoperations (0% vs. 0.8%) within 30 days of surgery. There were also no mortalities reported in preteens (0% vs. 0.1%). The risk-adjusted decrease in BMI between preteens and teens was also comparable at 30 days (4.2 [95% CI: 3.0-5.4] vs. 4.6 [95% CI: 4.4-4.7], p = 0.6). Decrease in BMI in preteens was 7 ± 3 kg/m2 at 3 months and 9 ± 4 kg/m2 at 12 months after surgery, which represented a percentage BMI change of 16 ± 7 and 20 ± 8, respectively. CONCLUSIONS: This study demonstrates that bariatric surgery in preteens is safe and efficacious when performed at specialized centers, and that age criteria may not be required. The AAP and others are encouraged to include age cut-offs in their guidelines for children with obesity and bariatric surgery only when data are available to support their inclusion.

Lessons Learned From the First-In-Human Compassionate Use of Connect-EA™ in Ten Patients With Esophageal Atresia.

INTRODUCTION: Delayed primary repair of esophageal atresia in patients with high-risk physiologic and anatomic comorbidities remains a daunting challenge with an increased risk for peri-operative morbidity and mortality via conventional repair. The Connect-EA device facilitates the endoscopic creation of a secure esophageal anastomosis. This follow-up study reports our long-term outcomes with the novel esophageal magnetic compression anastomosis (EMCA) Connect-EA device for EA repair, as well as lessons learned from the ten first-in-human cases. We propose an algorithm to maximize the advantages of the device for EA repair. METHODS: Under compassionate use approval, from June 2019 to December 2022, ten patients with prohibitive surgical or medical risk factors underwent attempted EMCA with this device. All patients underwent prior gastrostomy, tracheoesophageal fistula ligation (if necessary), and demonstrated pouch apposition prior to EMCA. RESULTS: Successful device deployment and EMCA formation were achieved in nine patients (90%). Mean time to anastomosis formation was 8 days (range 5-14) and the device was retrieved endoscopically in five (56%) cases. At median follow-up of 22 months (range 4-45), seven patients (78%) are tolerating oral nutrition. Balloon dilations (median 4, range 1-11) were performed either prophylactically for radiographic asymptomatic anastomotic narrowing (n = 7, 78%) or to treat clinically-significant anastomotic narrowing (n = 2, 22%) with no ongoing dilations at 3-month follow up post-repair. CONCLUSION: EMCA with the Connect-EA device is a safe and feasible minimally-invasive alterative for EA repair in high-risk surgical patients. Promising post-operative outcomes warrant further Phase I investigation. LEVEL OF EVIDENCE: IV, Case series of novel operative technique without comparison group.

Time to resolution of iodine-123 metaiodobenzylguanidine (123 I-MIBG) avidity and local control outcomes for high-risk neuroblastoma following radiation therapy.

INTRODUCTION: 123 I-MIBG scan is used in neuroblastoma (NB) to monitor treatment response. Time to resolution of 123 I-MIBG avidity after radiation therapy (RT) is unknown. We sought to determine time to resolution of 123 I-MIBG avidity after RT and local failure (LF) rate. METHODS: We performed a retrospective review of children with high-risk NB who underwent 123 I-MIBG scans pre- and post-RT from 2003 to 2019. Time from RT to resolution of 123 I-MIBG activity was analysed. LF and cumulative incidence of local progression (CILP) after RT stratified by site, presence of residual disease and use of boost RT were determined. RESULTS: Forty-two patients with median age 3.9 years (1.9-4.7 years) were included, with median follow-up time 3.9 years (1.4-6.9). Eighty-six lesions were treated with RT to median dose of 21.6 Gy. Eighteen of 86 lesions were evaluable for time to resolution of MIBG avidity after RT, with median resolution time of 78 days (36-208). No LF occurred among 26 patients who received RT to primary sites after GTR, versus 4/12 (25%) patients treated with residual primary disease. 2-year CILP was 19% (12% primary disease 25% metastatic disease (P = 0.18)). 2-year CILP for non-residual primary, residual primary, non-residual metastatic and residual metastatic lesions was 0%, 42%, 11% and 30% respectively (P = 0.01) and for boosted and non-boosted residual lesions was 29% and 35% (P = 0.44). CONCLUSION: Median time to MIBG resolution after RT was 78 days. Primary lesions without residual disease had excellent local control. LF rate was higher after RT for residual disease, with no benefit for boost RT.

Trends in Adolescent Bariatric Procedures: a 15-Year Analysis of the National Inpatient Survey.

BACKGROUND: Adolescents with severe obesity achieve superior health outcomes following metabolic and bariatric surgery (MBS) than medical treatment alone. Surgery results in significant and sustained decrease in BMI and reduces associated comorbidity. We characterized the changing demographics of adolescents who had MBS over a 15-year time period. METHODS: Using ICD-9 and ICD-10 codes, the 2005-2019 National Inpatient Sample database was queried for patients < 20 years who underwent MBS. National trends, socio-demographics, and hospital resource utilization were analyzed. RESULTS: Between 2005 and 2019, there were 16,381 pediatric hospitalizations for MBS. The annual MBS procedures increased from 839 cases in 2005 to 1785 in 2019. There was a significant shift away from laparoscopic gastric bypass (LGB) to sleeve gastrectomy (SG). Initially, LGB represented 67% of cases with no SG. In 2019, SG constituted 85% of MBS procedures, while LGB comprised only 14%. Patients were predominantly female (76%) and White (56%). Over time, there were considerable increases in patients with public insurance (12 to 46%) and Hispanics (11 to 30%). There was also a shift away from urban, non-teaching hospitals to urban, teaching hospitals. The average length of stay (LOS) decreased, while mean charges remained similar. CONCLUSION: Our study demonstrates a gradual increase in the utilization of MBS among adolescents and a significant shift to SG. We observed a shift in MBS cases to teaching institutions, along with an increase in Hispanics and patients with public insurance. However, MBS remains underutilized, and effort should be made to increase early referral of adolescents for MBS evaluation.

The novel application of an emerging device for salvage of primary repair in high-risk complex esophageal atresia.

INTRODUCTION: Preservation of native esophagus is a tenet of esophageal atresia (EA) repair. However, techniques for delayed primary anastomosis are severely limited for surgically and medically complex patients at high-risk for operative repair. We report our initial experience with the novel application of the Connect-EA, an esophageal magnetic compression anastomosis device, for salvage of primary repair in 2 high-risk complex EA patients. Compassionate use was approved by the FDA and treating institutions. OPERATIVE TECHNIQUE: Two approaches using the Connect-EA are described - a totally endoscopic approach and a novel hybrid operative approach. To our knowledge, this is the first successful use of a hybrid operative approach with an esophageal magnetic compression device. OUTCOMES: Salvage of delayed primary anastomosis was successful in both patients. The totally endoscopic approach significantly reduced operative time and avoided repeat high-risk operation. The hybrid operative approach salvaged delayed primary anastomosis and avoided cervical esophagostomy. CONCLUSION: The Connect-EA is a novel intervention to achieve delayed primary esophageal repair in complex EA patients with high-risk tissue characteristics and multi-system comorbidities that limit operative repair. We propose a clinical algorithm for use of the totally endoscopic approach and hybrid operative approach for use of the Connect-EA in high-risk complex EA patients.

Effect of telehealth implementation on an adolescent metabolic and bariatric surgery program.

BACKGROUND: Pediatric severe obesity is a worldwide health concern. Treatment with metabolic and bariatric surgery can reduce morbidity and mortality. The COVID-19 pandemic not only has had a significant effect on rates of pediatric obesity but also has necessitated a rapid transition to virtual medicine. OBJECTIVE: We aimed to identify and examine adolescent metabolic and bariatric surgery patient participation rates through our program's virtual telehealth programming as compared with prepandemic traditional in-person clinic appointments. SETTING: This study took place at an academic pediatric quaternary care center. METHODS: We evaluated 92 adolescent patients with a total of 2442 unique encounters between January 2018 and July 2021. RESULTS: The rate of attendance was found to be greater for telehealth visits (83.1%) than for in-person appointments (70.5%) for all clinics regardless of appointment type (preoperative versus postoperative). Cancellation rates were lower for telehealth visits (9.9%) than for in-person appointments (22.5%). CONCLUSION: This study provides evidence that telehealth can be implemented successfully in an adolescent metabolic and bariatric surgery program and can improve attendance rates for all provider and appointment subtypes.

Local Control Outcomes Using Stereotactic Body Radiation Therapy or Surgical Resection for Metastatic Sarcoma.

PURPOSE: Traditional management of metastatic sarcoma primarily relies on systemic therapy, with surgery often used for tumor control. We analyzed the rates of recurrence, overall survival, and treatment complications in patients undergoing either surgical resection or stereotactic body radiation therapy (SBRT) for metastatic sarcoma of the bone and/or soft tissue. METHODS AND MATERIALS: The records of patients with metastatic sarcoma between 2009 and 2020 were reviewed. Local recurrence (LR) was defined as tumor growth or recurrence at the tumor site. Cumulative LR incidence was analyzed accounting for the competing risk of death, and groups were compared using the Gray test. Overall survival (OS) was assessed using the Kaplan-Meier method and log-rank test. Hazard ratios were determined using the Cox proportional hazards model. RESULTS: A total of 525 metastatic lesions in 217 patients were analyzed. The mean age of patients was 57 years (range, 4-88 years). The lung was the predominant site treated (50%), followed by intra-abdominal (13%) and soft tissue (11%). Two-year cumulative incidences of LR for surgery and SBRT were 14.8% (95% confidence interval [CI], 11.6%-18.5%) and 1.7% (95% CI, 0.1%-8.2%), respectively (P = .003). Local recurrence occurred in 72 (16.5%) of 437 tumors treated with surgery and 2 (2.3%) of 88 tumors treated with SBRT. The adjusted hazard ratio for LR of lesions treated surgically was 11.5 (P = .026) when controlling for tumor size and tumor site. Median OS was 29.8 months (95% CI, 25.6-40.9 months). There were 47 surgical complications among a total of 275 procedures (18%). Of 58 radiation treatment courses, radiation-related toxic effects were reported during the treatment of 7 lesions (12%), and none were higher than grade 2. CONCLUSIONS: We observed excellent local control among patients selected for treatment with SBRT for metastatic sarcoma, with no evidence of an increase in LR after SBRT compared with surgical management. Further investigation is necessary to better define the most appropriate local control strategies for metastatic sarcoma.

Leiomyomatosis in an Infant With a SUFU Splice Site Variant: Case Report.

Heterozygous loss-of-function variants in the suppressor of fused protein gene (SUFU) can result in Gorlin syndrome, which is characterized by an increased frequency of basal cell carcinoma, medulloblastoma, odontogenic keratocysts, as well as other tumors. We describe a case of a 5-month-old female who presented with multiple intra-abdominal leiomyomata and was found to have a likely pathogenic splice site variant in the SUFU gene. This is the first reported case of leiomyomatosis secondary to a pathogenic SUFU variant in an infant and may represent an early, atypical presentation of Gorlin syndrome.

Ten-year experience with laparoscopic pedicled omental flap for cerebral revascularization in patients with Moyamoya disease.

BACKGROUND: The omental flap has numerous extraperitoneal applications in reconstruction and revascularization given its favorable immunologic and angiogenic properties. In patients with Moyamoya disease, cerebral revascularization using a pedicled omental flap has proven to be a viable option following direct revascularization procedures. Historically, harvesting omentum involved laparotomy with the associated risk of complications; herein we describe outcomes from a 10-year experience of laparoscopic harvesting of pedicled omental flap for cerebral revascularization in Moyamoya patients. METHODS: A retrospective chart review was performed of all patients with Moyamoya disease who underwent laparoscopic omental cerebral transposition between 2011 and 2021. Intraoperative and postoperative complications, length of stay (LOS), and outcomes at follow-up were analyzed. RESULTS: Twenty-one patients underwent the procedure during the study period. Three intraoperative complications occurred (one segmental transverse colectomy for mesenteric injury, one converted to omental free flap, and one requiring micro anastomosis). Average overall LOS was 6 ± 6 days, with 3 ± 3.5 days in the ICU (mean±SD). Following discharge, complications included epigastric incisional hernia at the graft fascial exit site, recurrent neck pain at subcutaneous tunneling site, and partial scalp necrosis. One patient required subsequent direct bypass seven months after the initial procedure owing to the progression of the disease. All other patients had partial or complete resolution of symptoms. CONCLUSION: Our retrospective observational study indicates that laparoscopic pedicled omental flap mobilization and transposition is a safe and effective method of indirect cerebral revascularization in patients with Moyamoya disease. LEVEL OF EVIDENCE: N/A.

Sutureless closure for the management of gastroschisis.

Gastroschisis is a common congenital anomaly in which the midgut fails to return to the abdominal cavity resulting in exposed intestines, which are not covered by a membrane in a neonate. The incidence of gastroschisis has been increasing worldwide resulting in an evolving medical and surgical management. Gastroschisis can be either simple or complicated. Complicated gastroschisis occurs when gastroschisis is associated with gastrointestinal conditions such as intestinal atresia, volvulus, stenosis or perforation. In this instance, the mortality and morbidity of patients significantly increases. Initial management of gastroschisis requires a multi-modal, interdisciplinary approach in order to successfully care for a neonate. Patients should be managed in a neonatal intensive care unit under the care of intensivists, respiratory therapists and pediatric surgeons. Temperature regulation, hydration and protection of the bowel are of the utmost priorities. Surgical management of gastroschisis focuses on reduction of the bowel and closure of the abdominal wall defect. Initially, the defect was closed primarily with suture, however, more recently, a sutureless closure has become prevalent. This, in conjunction with use of a silo, has led to a shift from the operating room and general anesthesia to the bedside. This article aims to discuss the presentation, diagnosis and management of gastroschisis.

Adult residual rectourethral fistula and diverticulum presenting decades after imperforate anus repair: a case report.

BACKGROUND: This report describes a rare surgical case of an intraabdominal mass in a middle-aged patient 40 years after imperforate anus repair. CASE PRESENTATION: A 44-year-old Latino male with history of repaired anorectal malformation presented with recurrent urinary tract infections and rectal prolapse with bothersome bleeding and fecal incontinence. During his preoperative evaluation, he was initially diagnosed with a prostatic utricle cyst on the basis of magnetic resonance imaging findings, which demonstrated a cystic, thick-walled mass with low signal contents that extended inferiorly to insert into the distal prostatic urethra. However, at the time of surgical resection, the thick-walled structure contained an old, firm fecaloma. The final pathology report described findings consistent with colonic tissue, suggesting a retained remnant of the original fistula and diverticulum. CONCLUSIONS: Although rare, persistent rectourethral fistula tracts and rectal diverticula after imperforate anus repair can cause symptoms decades later, requiring surgical intervention. This is an important diagnostic consideration for any adult patient with history of imperforate anus.

Outcomes of Bariatric Surgery in Older Versus Younger Adolescents.

OBJECTIVES: In this report, we compare weight loss, comorbidity resolution, nutritional abnormalities, and quality of life between younger and older adolescents after metabolic and bariatric surgery. METHODS: From March 2007 to December 2011, 242 adolescents (≤19 years of age) who underwent bariatric surgery at 5 clinical centers in the United States were enrolled in the prospective, multicenter, long-term outcome study Teen-Longitudinal Assessment of Bariatric Surgery. Outcome data from younger (13-15 years; n = 66) and older (16-19 years; n = 162) study participants were compared. Outcomes included percent BMI change, comorbidity outcomes (hypertension, dyslipidemia, and type 2 diabetes mellitus), nutritional abnormalities, and quality of life over 5 years post surgery. RESULTS: Baseline characteristics, except for age, between the 2 cohorts were similar. No significant differences in frequency of remission of hypertension (P = .84) or dyslipidemia (P = .74) were observed between age groups. Remission of type 2 diabetes mellitus was high in both groups, although statistically higher in older adolescents (relative risk 0.86; P = .046). Weight loss and quality of life were similar in the 2 age groups. Younger adolescents were less likely to develop elevated transferrin (prevalence ratio 0.52; P = .048) and low vitamin D levels (prevalence ratio 0.8; P = .034). CONCLUSIONS: The differences in outcome of metabolic and bariatric surgery between younger and older adolescents were few. These data suggest that younger adolescents with severe obesity should not be denied consideration for surgical therapy on the basis of age alone and that providers should consider adolescents of all ages for surgical therapy for obesity when clinically indicated.

Obstetric and neonatal outcomes in pregnancies complicated by fetal lung masses: does final histology matter?

PURPOSE: Fetal lung masses complicate approximately 1 in 2000 live births. Our aim was to determine whether obstetric and neonatal outcomes differ by final fetal lung mass histology. MATERIALS AND METHODS: A review of all pregnancies complicated by a prenatally diagnosed fetal lung mass between 2009 and 2017 at a single academic center was conducted. All cases included in the final analysis underwent surgical resection and histology diagnosis was determined by a trained pathologist. Clinical data were obtained from review of stored electronic medical records which contained linked maternal and neonatal records. Imaging records included both prenatal ultrasound and magnetic resonance imaging. Fisher's exact test was used for categorical variables and the Kruskal-Wallis test was used for continuous variables. The level of significance was p<.05. RESULTS: Of 61 pregnancies complicated by fetal lung mass during the study period, 45 cases underwent both prenatal care and postnatal resection. Final histology revealed 10 cases of congenital pulmonary airway malformation (CPAM) type 1, nine cases of CPAM type 2, and 16 cases of bronchopulmonary sequestration. There was no difference in initial, maximal, or final CPAM volume ratio between groups, with median final CPAM volume ratio of 0.6 for CPAM type 1, 0.7 for CPAM type 2, and 0.3 for bronchopulmonary sequestration (p = .12). There were no differences in any of the maternal or obstetric outcomes including gestational age at delivery and mode of delivery between the groups. The primary outcome of neonatal respiratory distress was not statistically different between groups (p = .66). Median neonatal length of stay following delivery ranged from 3 to 4 days, and time to postnatal resection was similar as well, with a median of 126 days for CPAM type 1, 122 days for CPAM type 2, and 132 days for bronchopulmonary sequestration (p = .76). CONCLUSIONS: In our cohort, there was no significant association between histologic lung mass subtypes and any obstetric or neonatal morbidity including respiratory distress.

Epigenetic Targeting of TERT-Associated Gene Expression Signature in Human Neuroblastoma with TERT Overexpression.

Neuroblastoma is a deadly pediatric solid tumor with infrequent recurrent somatic mutations. Particularly, the pathophysiology of tumors without MYCN amplification remains poorly defined. Utilizing an unbiased approach, we performed gene set enrichment analysis of RNA-sequencing data from 498 patients with neuroblastoma and revealed a differentially overexpressed gene signature in MYCN nonamplified neuroblastomas with telomerase reverse transcriptase (TERT) gene overexpression and coordinated activation of oncogenic signaling pathways, including E2Fs, Wnt, Myc, and the DNA repair pathway. Promoter rearrangement of the TERT gene juxtaposes the coding sequence to strong enhancer elements, leading to TERT overexpression and poor prognosis in neuroblastoma, but TERT-associated oncogenic signaling remains unclear. ChIP-seq analysis of the human CLB-GA neuroblastoma cells harboring TERT rearrangement uncovered genome-wide chromatin co-occupancy of Brd4 and H3K27Ac and robust enrichment of H3K36me3 in TERT and multiple TERT-associated genes. Brd4 and cyclin-dependent kinases (CDK) had critical regulatory roles in the expression and chromatin activation of TERT and multiple TERT-associated genes. Epigenetically targeting Brd4 or CDKs with their respective inhibitors suppressed the expression of TERT and multiple TERT-associated genes in neuroblastoma with TERT overexpression or MYCN amplification. ChIP-seq and ChIP-qPCR provided evidence that the CDK inhibitor directly inhibited Brd4 recruitment to activate chromatin globally. Therefore, inhibiting Brd4 and CDK concurrently with AZD5153 and dinaciclib would be most effective in tumor growth suppression, which we demonstrated in neuroblastoma cell lines, primary human cells, and xenografts. In summary, we describe a unique mechanism in neuroblastoma with TERT overexpression and an epigenetically targeted novel therapeutic strategy. SIGNIFICANCE: Epigenetically cotargeting Brd4 and Cdks suppresses human neuroblastoma with TERT overexpression by inhibiting the TERT-associated gene expression networks.