Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children With Chest Wall Sarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

OBJECTIVE: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall. SUMMARY OF BACKGROUND DATA: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. METHODS: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. RESULTS: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status ( P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor(HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs resected (HR 1.78; P = 0.02). Overall, patient-reported quality of life is not impaired after chest wall tumor resection. CONCLUSIONS: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.

Patterns of opioid use in dialysis access procedures.

BACKGROUND: Overprescription of opioids after surgical procedures is recognized as an important contributor to opioid misuse. Dialysis access procedures are commonly performed outpatient operations with few data or guidelines to inform prescription pain management practices. We sought to characterize opioid pain medication use after dialysis access surgery to promote a conservative approach to postoperative opioid prescriptions. METHODS: We performed a retrospective review of patients who underwent surgical dialysis access procedures from August 2018 through January 2019. Patient-reported opioid use information was captured in a brief questionnaire administered during routine follow-up appointments or phone calls and recorded in the electronic medical record. The procedure, type of intraoperative anesthesia or analgesia, postoperative prescription provided, and patient factors (including age, sex, dialysis type, history of chronic pain, and preoperative opioid or benzodiazepine use) were recorded. All procedures were classified by type (arteriovenous fistula or graft with a short incision [AVF-S], arteriovenous fistula or graft with a long incision [AVF-L], or peritoneal dialysis [PD] catheter), and descriptive statistics were performed using R (R Foundation for Statistical Computing, Vienna, Austria). RESULTS: Eighty-six patients underwent dialysis access procedures in the study time frame, of whom 63 were administered the pain questionnaire and 58 quantified opioid use; 85% of patients received a prescription, but 31% took no opioids and 71% used opioids for ≤2 days. Interquartile ranges (25th-75th percentile) of prescription and consumption quantities for patients who underwent AVF-L procedures were 10 to 28 pills and 2.5 to 20 pills; for patients who underwent AVF-S, quantities were 4.0 to 8.4 pills and 0 to 4.3 pills; and PD quantities were 10 pills and 3.3 to 9 pills. Thirty-one patients (53%) reported receiving more pain medication than they used, which resulted in a median of 8 excess pills per patient with an unused pill interquartile range of 0 to 22 pills for AVF-L procedures, 0 to 4.2 pills for AVF-S procedures, and 1.3 to 6.7 pills for PD procedures. Patients who were prescribed oxycodone or had a repeated operation had significantly increased opioid use. CONCLUSIONS: This investigation of opioid use after surgical dialysis access procedures suggests that most patients use relatively few opioid pills after surgery, which translates into overprescription and leftover medication for >50% of patients. A conservative approach to postoperative prescription guidelines using lower prescription quantities would encourage opioid-related risk reduction while providing adequate postoperative analgesia. Recommended quantities for postoperative prescriptions were generated using the 80th percentile consumed and were 0 to 6 pills for brachiobasilic or brachiocephalic fistulas, 0 to 5 pills for basilic vein transposition, 0 to 5 pills for radiocephalic AVF, 0 to 15 pills for upper arm grafts, and 0 to 10 pills for PD catheter placement.

Rapid polyclonal desensitization with antibodies to IgE and FcεRIα.

BACKGROUND: Rapid desensitization, a procedure in which persons allergic to an antigen are treated at short intervals with increasing doses of that antigen until they tolerate a large dose, is an effective, but risky, way to induce temporary tolerance. OBJECTIVE: We wanted to determine whether this approach can be adapted to suppress all IgE-mediated allergies in mice by injecting serially increasing doses of monoclonal antibodies (mAbs) to IgE or FcεRIα. METHODS: Active and passive models of antigen- and anti-IgE mAb-induced IgE-mediated anaphylaxis were used. Mice were desensitized with serially increasing doses of anti-IgE mAb, anti-FcεRIα mAb, or antigen. Development of shock (hypothermia), histamine and mast cell protease release, cytokine secretion, calcium flux, and changes in cell number and FcεRI and IgE expression were evaluated. RESULTS: Rapid desensitization with anti-IgE mAb suppressed IgE-mediated immediate hypersensitivity; however, some mice developed mild anaphylaxis during desensitization. Rapid desensitization with anti-FcεRIα mAb that only binds FcεRI that is not occupied by IgE suppressed both active and passive IgE-mediated anaphylaxis without inducing disease. It quickly, but temporarily, suppressed IgE-mediated anaphylaxis by decreasing mast cell signaling through FcεRI, then slowly induced longer lasting mast cell unresponsiveness by removing membrane FcεRI. Rapid desensitization with anti-FcεRIα mAb was safer and longer lasting than rapid desensitization with antigen. CONCLUSION: A rapid desensitization approach with anti-FcεRIα mAb safely desensitizes mice to IgE-mediated anaphylaxis by inducing mast cell anergy and later removing all mast cell IgE. Rapid desensitization with an anti-human FcεRIα mAb may be able to prevent human IgE-mediated anaphylaxis.

Rapid desensitization of mice with anti-FcγRIIb/FcγRIII mAb safely prevents IgG-mediated anaphylaxis.

BACKGROUND: Stimulatory IgG receptors (FcγRs) on bone marrow-derived cells contribute to the pathogenesis of several autoimmune and inflammatory disorders. Monoclonal antibodies that block FcγRs might suppress these diseases, but they can induce anaphylaxis. OBJECTIVE: We wanted to determine whether a rapid desensitization approach can safely suppress IgG/FcγR-mediated anaphylaxis. METHODS: Mice were injected with serially increasing doses of 2.4G2, a rat mAb that blocks the inhibitory FcγR, FcγRIIb, and the stimulatory receptor, FcγRIII. Rectal temperature was used to detect the development of anaphylaxis. Passive and active IgG-mediated anaphylaxis were evaluated in mice that had been rapidly desensitized with 2.4G2 or mock-desensitized in mice in which monocyte/macrophages, basophils, or neutrophils had been depleted or desensitized and in mice in which FcγRI, FcγRIII, and/or FcγRIV had been deleted or blocked. RESULTS: Rapid desensitization with 2.4G2 prevented 2.4G2-induced shock and completely suppressed IgG-mediated anaphylaxis. Rapid desensitization of ovalbumin-sensitized mice with 2.4G2 was safer and more effective than rapid desensitization with ovalbumin. 2.4G2 treatment completely blocked FcγRIII and removed most FcγRI and FcγRIV from nucleated peripheral blood cells. Because IgG(2a)-mediated anaphylaxis was partially FcγRI and FcγRIV dependent, the effects of 2.4G2 on FcγRI and FcγRIV were probably crucial for its complete inhibition of IgG(2a)-mediated anaphylaxis. IgG(2a)-mediated anaphylaxis was partially inhibited by depletion or desensitization of monocyte/macrophages, basophils, or neutrophils. CONCLUSION: IgG-mediated anaphylaxis can be induced by ligation of FcγRI, FcγRIII, or FcγRIV on monocycte/macrophages, basophils, or neutrophils and can be safely suppressed by rapid desensitization with anti-FcγRII/RIII mAb. A similar approach may safely suppress other FcγR-dependent immunopathology.

Critical elements of pediatric neuroblastoma surgery.

Neuroblastoma (NB) is the most common solid extracranial malignancy of childhood with an incidence of 1 per 100,000 in the United States compromising approximately 10 % of childhood cancer. Unfortunately, patients with high-risk NG continue to have long-term survival less than 50 %. Both Children's Oncology Group and the International Society of Paediatric Oncology have demonstrated the important role of surgery in the treatment of high-risk NB. Herein, we compose the results of an extensive literature review as well as expert opinion from leaders in pediatric surgical oncology, to present the critical elements of effective surgery for high-risk neuroblastoma.

Outcomes and cost of medical and surgical treatments of pilonidal disease: A single institution's 10-year review.

Background: Pilonidal disease is a chronic inflammatory skin disorder typically located in the gluteal cleft. Treatment varies from antibiotic therapy to extensive surgical resection and reconstruction; however, complications and recurrence are common. To understand risk factors, outcomes, and costs associated with various treatments, we performed a retrospective chart review of all patients treated for pilonidal disease at a single health care system from 2008 to 2018. Methods: Patients with an ICD diagnosis code associated with pilonidal disease were identified. Charts were reviewed for demographic, clinical, and cost information related to pilonidal disease encounters. Data were analyzed for risk of recurrence by Cox proportional hazards regression and economic burden by Wilcoxon signed-rank test. Results: During the study time frame, 513 patients were diagnosed with pilonidal disease. Primary treatment included 108 patients (21%) with wide excision, 167 (32%) with antibiotics alone, 79 (15%) with incision and drainage, and 109 (21%) with incision and drainage plus antibiotics. The rate of recurrence following antibiotic therapy, incision and drainage, or wide excision was 36.7%, 35.9%, and 21.3%, respectively. Sex, body mass index, obesity, or hidradenitis suppurativa was not associated with recurrence; however, smokers who underwent incision and drainage had a higher risk of recurrence (P < .0001). The median cost of each primary treatment was $3,093 for excision, $607 for incision and drainage, $281 antibiotics alone, and $686 for incision and drainage plus antibiotics. Conclusion: Pilonidal disease presents with a high degree of heterogeneity and is often managed primarily with antibiotics, incision and drainage, or surgical excision. Risk of recurrence was less in patients who underwent wide excision; however, these patients had higher overall cost compared to patients that had nonoperative management. Level of evidence: Level III.

Interhospital variability in localization techniques for small pulmonary nodules in children: A pediatric surgical oncology research collaborative study.

BACKGROUND: Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients. METHODS: A retrospective review was performed on patients < 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques. RESULTS: 225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p < 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p < 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16). CONCLUSIONS: Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time. LEVEL OF EVIDENCE: Retrospective review, Level 3.

Case report: a step-wise management of concurrent presentation of congenital single lung and aberrant right subclavian artery in an infant girl.

INTRODUCTION: Congenital single lung (CSL) is a rare condition, and symptomatic patients often present with respiratory distress or recurrent respiratory infection due to mediastinal shift causing vascular or airway compression. Aberrant right subclavian artery (ARSA) is another rare congenital anomality that can lead to tracheal or esophageal compressions. There is only one other case of concurrent presentation of CSL and ARSA reported, which presented unique challenge in surgical management of our patient. Here we present a step-wise, multidisciplinary approach to manage symptomatic CSL and ARSA. CASE PRESENTATION: An infant girl with a prenatal diagnosis of CSL developed worsening stridor and several episodes of respiratory illnesses at 11 months old. Cross-sectional imaging and bronchoscopic evaluation showed moderate to severe distal tracheomalacia with anterior and posterior tracheal compression resulting from severe mediastinal rotation secondary to right-sided CSL. It was determined that her tracheal compression was mainly caused by her aortic arch wrapping around the trachea, with possible additional posterior compression of the esophagus by the ARSA. She first underwent intrathoracic tissue expander placement, which resulted in immediate improvement of tracheal compression. Two days later, she developed symptoms of dysphagia lusoria due to increased posterior compression of her esophagus by the ARSA. She underwent transposition of ARSA to the right common carotid with immediate resolution of dysphagia lusoria. As the patient grew, additional saline was added to the tissue expander due to recurrence in compressive symptoms. CONCLUSIONS: Concurrent presentation of CSL and ARSA is extremely rare. Asymptomatic CSL and ARSA do not require surgical interventions. However, if symptomatic, it is crucial to involve a multidisciplinary team for surgical planning and to take a step-wise approach as we were able to recognize and address both tracheomalacia and dysphagia lusoria in our patient promptly.

A novel box for aerosol and droplet guarding and evacuation in respiratory infection (BADGER) for COVID-19 and future outbreaks.

The coronavirus disease 2019 (COVID-19) pandemic caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has infected millions and killed more than 1.7 million people worldwide as of December 2020. Healthcare providers are at increased risk of infection when caring for patients with COVID-19. The mechanism of transmission of SARS-CoV-2 is beginning to emerge as airborne spread in addition to direct droplet and indirect contact as main routes of transmission. Here, we report on the design, construction, and testing of the BADGER (Box for Aerosol and Droplet Guarding and Evacuation in Respiratory Infection), an affordable, scalable device that contains droplets and aerosol particles, thus minimizing the risk of infection to healthcare providers. A semi-sealed environment is created inside the BADGER, which is placed over the head of the patient and maintains at least 12-air changes per hour using in-wall vacuum suction. Multiple hand-ports enable healthcare providers to perform essential tasks on a patient's airway and head. Overall, the BADGER has the potential to contain large droplets and small airborne particles as demonstrated by simulated qualitative and quantitative assessments to provide an additional layer of protection for healthcare providers treating COVID-19 and future respiratory contagions.

Predicting the Safe Use of Deceased After Circulatory Death Liver Allografts in Primary Sclerosing Cholangitis.

OBJECTIVES: The use of deceased after circulatory death liver allografts in patients with primary sclerosing cholangitis is controversial, given the increased risk of graft complications in patients with primary sclerosing cholangitis. We hypothesized that transplant of deceased after circulatory death livers into recipients with primary sclerosing cholangitis when appropriately selected using the UK deceased after circulatory death scoring system is not associated with increased graft failure and mortality. MATERIALS AND METHODS: We analyzed 99 229 transplants (between January 2001 and December 2018) from the Organ Procurement and Transplantation Network database. Deceased after circulatory death transplants were stratified by the UK scoring system as low risk or high risk. We identified 3958 patients with primary sclerosing cholangitis who received deceased after brain death transplant and 95 patients with primary sclerosing cholangitis who received deceased after circulatory death transplant. RESULTS: As expected, 5-year graft survival was lower in the circulatory death recipient group (69.0% vs 78.4%; P = .02). However, 5-year graft survival was significantly lower in the high-risk versus low-risk UK scoring system group (60.0% vs 75.4%; P = .02), with rate in the low-risk group similar to the brain death recipient group (78.4% vs 75.4%; P = .52). On multivariate analysis, the high-risk group had significantly increased risk of graft loss (hazard ratio of 1.92; P = .01). However, the low-risk group had equivalent graft survival to the brain death recipient group (hazard ratio of 1.23; P = .31). CONCLUSIONS: Graft failure was higher in patients with primary sclerosing cholangitis who received livers from deceased after circulatory death donors; however, the risk of graft loss was abrogated using appropriately matched donor and recipient combinations.

Appendectomy Versus Observation for Appendicitis in Neutropenic Children With Cancer.

BACKGROUND: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration. METHODS: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period. RESULTS: Sixty-six children, with a median age of 11 years (range 1-17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; P = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% (n = 11) and count recovery in 35% (n = 6). Cancer therapy was delayed in 35% (n = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, P = .05) and longer LOS (29 vs 12 days; P = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications (P < .01). CONCLUSIONS: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.

Suture fistula revisited for long gap esophageal atresia.

BACKGROUND: Long gap esophageal atresia presents a challenge to pediatric surgeons due to the variability in surgical management when primary anastomosis is not feasible. Mechanical traction procedures enhance tissue growth that allows early anastomosis, before delayed primary closure (DPC) would be attempted to allow for rapid oral feeding, or when operative discoveries require flexibility of the surgical plan. The Suture Fistula procedure, first published by Alan Shafer and Tirone David in 1974, is a simple, effective, but underutilized single-stage procedure which results in spontaneous fistulization of approximated, non-anastomosed esophageal segments using tension sutures. METHODS: A retrospective chart review was performed of patients who underwent the Suture Fistula procedure at a single institution since 1992. A literature review of all published case series of patients who underwent this procedure was also performed. RESULTS: There were 5 case series found with a total of 24 patients, and three new cases presented. Patients were noted to have spontaneous fistulization with gastrostomy tube feed reflux noted in the upper esophagus or mouth on average of post-operative day 14, which occurred in over 85% of patients. While nearly all patients required esophageal dilation and anti-reflux procedures, the morbidity of the procedure, including esophageal leak, is very low, and similar to the Foker or Kimura procedures, which have been more popular despite their surgical complexity. CONCLUSION: We propose the Suture Fistula technique to be a simpler, more effective, and safe alternative to other mechanical traction suture procedures in cases where primary anastomosis is not feasible. LEVEL OF EVIDENCE: Level IV.

Improvement in hearing loss over time in Cornelia de Lange syndrome.

OBJECTIVES: Patients with Cornelia de Lange Syndrome (CdLS) are reported to have conductive (CHL) and sensorineural hearing loss (SNHL), but there is little information pertaining to the progression of hearing loss over time. The goal of this study was to examine the prevalence of CHL and SNHL in adults and children with CdLS and look for changes in SNHL over time. METHODS: Retrospective chart review of patients with CdLS presenting to a CdLS clinic was conducted. Also, a written survey of clinical concerns was collected from additional patients/families seen in the clinic and through the Cornelia de Lange Foundation. RESULTS: Seventy-eight patients (50% female) were included in the chart review. Mean age was 16.8 ± 11.4 years (range-0.6-50 years) and mean age at diagnosis of hearing loss was 4.6 ± 10.6 years (n = 26). Five patients (6.4%) had severe to profound SNHL that improved with time, including 2 who had complete normalization of audiogram results. Thirty-five families/patients completed the clinical survey, and 45.5% of the families reported a noticeable improvement of hearing over time. CONCLUSIONS: Conductive hearing loss and SNHL are common in CdLS. More than 50% of the patients seen in an adult CdLS clinic reported improvement in hearing loss over time, and a subset of patients had an improvement in SNHL. In light of these findings, we recommend longitudinal evaluations of hearing loss in these patients with both auditory brainstem response and otoacoustic emissions testing if SNHL is identified.