Pediatric and Young Adult Image-Guided Percutaneous Bone Biopsy-A New Standard of Care?

BACKGROUND: There are no consensus guidelines regarding the use of percutaneous needle biopsy for the diagnosis of soft tissue and bone tumors. The aim of this study was to understand the efficacy of image-guided percutaneous biopsy for pediatric patients with soft tissue and bony masses, the role of intraoperative image guidance, and diagnostic accuracy. PATIENTS AND METHODS: A retrospective institutional chart review was performed on patients who underwent percutaneous biopsy of soft tissue or bone tumors between 2007 and 2017. Data collected included preoperative imaging, type of biopsy, demographics, insurance status, number of samples taken, and pathologic results. RESULTS: One hundred forty-one children and young adults underwent 169 biopsies. Female patients received 48.2% of biopsies. The mean age was 14.3 ± 7.0 years. Core needle biopsies made up 89.4% of procedures, while 10.6% were fine needle aspirate. The mean number of samples per patient was 3.6 ± 2.5. All patients had imaging guidance, with computed tomography used in 44.7% of patients, 9.9% using fluoroscopy, 7.1% using ultrasound for guidance, and 53 (37.6%) patients had more than one modality. Diagnostic specimens were obtained in 97.9% of biopsies. The most common overall pathology was osteoid osteoma. The most common malignant tumors were osteosarcoma and Ewing's sarcoma. CONCLUSION: Image-guided percutaneous biopsy is a safe and effective method of obtaining accurate tissue samples in children and young adults with soft tissue or bone masses. LEVEL OF EVIDENCE: Level 4-Study of diagnostic test.

Disparities in care of pediatric, adolescent, and young adult patients with solid tumors: A systematic review.

BACKGROUND: Numerous studies have demonstrated a variety of social inequalities within pediatric and young adult patients with solid tumors. This systematic review examines and consolidates the existing literature regarding disparities in pediatric and young adult solid tumor oncology. PROCEDURE: A MeSH search was performed on the following databases: MEDLINE, PubMed, OvidSP Cochrane, Central, Embase, Cinhal, and Scopus. The systematic review was performed using Rayyan QCRI. RESULTS: Total 387 articles were found on the initial search, and 34 articles were included in final review. Twenty-seven studies addressed racial and ethnic disparities; 23 addressed socioeconomic disparities. Patients with Hispanic ethnicity, Black race, and lower socioeconomic status were more likely to present at later stages, have differences in treatments and higher mortality rates. CONCLUSION: This qualitative systematic review identified both racial and socioeconomic disparities in pediatric cancer patients across a variety of solid tumor types. Patients with Hispanic ethnicity, Black race, and lower socioeconomic status are associated with disparities in stage at presentation, treatment, and outcome. Characterization of existing disparities provides the evidence necessary to support changes at a systemic level.

Thoracoscopy vs thoracotomy for the management of metastatic osteosarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

Complete surgical resection of pulmonary metastatic disease in patients with osteosarcoma is crucial to long-term survival. Open thoracotomy allows palpation of nodules not identified on imaging but the impact on survival is unknown. The objective of this study was to compare overall survival (OS) and pulmonary disease-free survival (DFS) in children who underwent thoracotomy vs thoracoscopic surgery for pulmonary metastasectomy. A multi-institutional collaborative group retrospectively reviewed 202 pediatric patients with osteosarcoma who underwent pulmonary metastasectomy by thoracotomy (n = 154) or thoracoscopy (n = 48). Results were analyzed by Kaplan-Meier survival estimates and multivariate Cox proportional hazard regression models. With median follow-up of 45 months, 135 (67.5%) patients had a pulmonary relapse and 95 (47%) patients were deceased. Kaplan-Meier analysis showed no significant difference in 5-year pulmonary DFS (25% vs 38%; P = .18) or OS (49% vs 42%, P = .37) between the surgical approaches of thoracotomy and thoracoscopy. In Cox regression analysis controlling for other factors impacting outcome, there was a significantly increased risk of mortality (HR 2.11; P = .027; 95% CI 1.09-4.09) but not pulmonary recurrence (HR 0.96; P = .90; 95% CI 0.52-1.79) with a thoracoscopic approach. However, in the subset analysis limited to patients with oligometastatic disease, thoracoscopy had no increased risk of mortality (HR 1.16; P = .62; 0.64-2.11). In conclusion, patients with metastatic osteosarcoma and limited pulmonary disease burden demonstrate comparable outcomes after thoracotomy and thoracoscopy for metastasectomy. While significant selection bias in these surgical cohorts limits the generalizability of the conclusions, clinical equipoise for a randomized clinical trial in patients with oligometastatic disease is supported.

Long-term follow-up of surgical outcomes for patients with Wilms tumor and neuroblastoma.

BACKGROUND: There are minimal data on long-term surgical outcomes of patients who have undergone resection for Wilms tumor (WT) and neuroblastoma (NB). METHODS: A retrospective review of patients in a long-term survivor clinic between the years 1967 and 2016 in a pediatric tertiary care hospital (>5 years posttreatment) was performed. RESULTS: Eighty-six survivors of WT and 86 survivors of NB who had ongoing follow-up in the survivors' clinic were identified. The median age at diagnosis was 2.5 years (range, 0.4-15.7 years) with a mean follow-up of 22.3 years (±10.4 years) for WT. The median age at diagnosis for patients with NB was 0.9 years (range, 0.1-8.6 months); mean follow-up of 21.7 years (±7.9 years). Twelve patients with WT (14.0%) had at least 1 repeat laparotomy, 11.1% for bowel obstruction, at a median of 3 months from initial surgery. Twelve patients (14.0%) with NB required laparotomy and 8.1% for bowel obstruction, at a median of 12 years after initial surgery. The incidence of hypertension in patients with WT who had undergone nephrectomy was not outside of population norms. Patients who underwent thoracotomy for a NB have a higher incidence of scoliosis and Horner syndrome. CONCLUSIONS: Small bowel obstruction requiring laparotomy is significantly higher than the literature norms for both tumor patient populations and typically occurs in the early postoperative period for patients with WT and remotely in patients with NB. The long-term surgical complications of patients who underwent resection for NB and WT clearly merit follow-up and patient education within multidisciplinary long-term survivorship clinics.

Can a children's hospital still cut it? Comparing outcomes of pediatric, adolescent and young adult patients undergoing thoracic surgery for lung metastases.

BACKGROUND: Adolescents and young adults (AYAs) with cancer have unique medical challenges compared with younger children and older adults. Dedicated centers have been established to deliver cancer therapy to the AYA population; many of these programs are located in pediatric hospitals. Outcomes of AYA patients on pediatric protocols are generally superior to those on adult protocols. Little is understood about the impact of care within a pediatric environment for surgical care of young adults. METHODS: A retrospective institutional review was performed of patients undergoing thoracic metastectomy between 2012 and 2017. Demographics, procedural factors, cost, and outcomes were analyzed. Patients were divided into two groups: > 18 and <18 years. RESULTS: Ninety-one procedures were performed: 61.5% (n = 56) were in patients <18 years old and 38.5% (n = 35) were > 18 years old. The median age was 6.5 years for <18 years old and 28 years for > 18 years old. Older patients had a significantly longer operative time on thoracoscopic cases; 91 versus 63 minutes. Fifty percent of the > 18 group had > 1 lesion resected compared with one lesion resected in 80.8% in <18 years old. No significant differences were found between the two groups in the duration of chest tube or length of stay. The AYA group demonstrated more "adult type" comorbidities. CONCLUSION: AYA patients have unique developmental and emotional challenges. Surgical intervention in this special population of patients cared for within a pediatric environment shows no significant difference in outcome compared with pediatric patients undergoing the same procedure. AYA patients with "adult type" comorbidities can safely undergo multidisciplinary care including surgery within a pediatric environment without the need to fragment care.

Association of Age at Duodenal Atresia Repair With Outcomes: A Pediatric NSQIP Analysis.

PURPOSE: Neonates with duodenal atresia (DA) are often born prematurely and undergo repair soon after birth, while others are delayed to allow for growth until closer to term corrected gestational age (cGA). Premature infants have been demonstrated to experience worse outcomes, but it is unclear whether delaying surgery mitigates the increased morbidity. This study evaluates the association of timing of DA repair with postoperative morbidity. METHODS: We retrospectively evaluated neonates undergoing DA repair from the National Surgical Quality Improvement Program-Pediatric database (2015-2020). A multivariable regression analyzed factors associated with composite morbidity, including cGA and age in days of life (DOL) at surgery. A propensity score matched analysis was completed in premature neonates born at ≤35 weeks gestation to compare outcomes at similar birth gestational ages (bGA) and birth weight who underwent early (<7 DOL) versus delayed (≥7 DOL) repair. RESULTS: 809 neonates were included with a median bGA of 36 weeks (IQR 34-38), birth weight of 2.46 kg (IQR 1.96-2.95), and DOL at surgery of 2 (IQR 1-5). Infants born ≤35 weeks represented 35.23% of the cohort. On multivariable analysis, increasing cGA at surgery was associated with decreased morbidity (OR: 0.91, CI [0.84, 0.99]), and increasing DOL at surgery was associated with increased morbidity (OR: 1.02, CI [1.00, 1.04]). On propensity score matched analysis, delayed repairs were associated with increased postoperative ventilation (6 days vs. 2 days, p < 0.05); however, there were no differences in composite or surgical morbidity between early and delayed repairs. CONCLUSIONS: Morbidity after DA repair in neonates ≤35 weeks cGA is primarily driven by non-surgical causes, but delaying surgery does not appear to mitigate the risks associated with prematurity. It seems reasonable to consider repair in neonates around 33-34 weeks gestation without prohibitive risk factors. Optimal timing of DA repair requires a delicate balance between these factors. LEVEL OF EVIDENCE: Level III. TYPE OF STUDY: Retrospective Cohort Study.

Concurrent Cholecystectomy Does Not Increase Splenectomy Morbidity in Patients With Hemolytic Anemia: A Pediatric NSQIP Analysis.

PURPOSE: Children undergoing splenectomy for hemolytic anemia often have cholelithiasis, which may or may not be symptomatic. It is unclear whether concurrent cholecystectomy increases length of stay or morbidity after splenectomy. The purpose of this study was to compare morbidity among children undergoing laparoscopic splenectomy alone versus splenectomy with concurrent cholecystectomy in patients with hemolytic anemia. METHODS: We retrospectively evaluated children with hemolytic anemia undergoing non-traumatic laparoscopic splenectomy in the National Surgical Quality Improvement Program-Pediatric database (2012-2020). Outcomes were compared for patients undergoing splenectomy alone (n = 1010) versus splenectomy with cholecystectomy (n = 371). Pearson's Chi-square and Student's t-tests were utilized as appropriate. Propensity score-matching was completed, controlling for eight demographic and clinical variables. RESULTS: 1381 patients were identified, 73.1% undergoing splenectomy alone and 26.9% splenectomy with cholecystectomy. Splenectomy with cholecystectomy patients were older (10.9 years vs. 8.4 years, p < 0.01), more likely to have hereditary spherocytosis (56.1% vs. 40.8%, p < 0.01), less likely to have sickle cell disease (12.1% vs. 33.5%, p < 0.01), more likely ASA class 1 or 2 (49.3% vs. 42.1%, p < 0.01), and had similar preoperative hematocrit levels (29.6 vs. 29.3, p = 0.33). The splenectomy with cholecystectomy group was less likely to receive preoperative blood transfusions (13.5% vs. 25.4%, p < 0.01). There were 360 pairs selected on propensity score-matching, and splenectomy with cholecystectomy was associated with increased operative time (182 min vs. 145 min, p < 0.01) and decreased occurrences of a postoperative transfusion (4.2% vs. 8.9%, p = 0.01). Length of stay after surgery (2.5 days vs. 2.3 days, p = 0.13), composite morbidity (3.9% vs. 3.4%, p = 0.69), and 30-day readmission rates (3.3% vs. 7.4%, p = 0.08) were all similar. CONCLUSIONS: Splenectomy with cholecystectomy is associated with similar postoperative morbidity, length of stay and readmission rates compared to splenectomy alone. These data support the safety of concurrent cholecystectomy with splenectomy for children with cholelithiasis in the setting of hemolytic anemia. TYPE OF STUDY: Retrospective Cohort Study. LEVEL OF EVIDENCE: Level III.

Geographic barriers to children's surgical care: A systematic review of existing evidence.

BACKGROUND: Ensuring that children have access to timely and appropriate surgical care is a vital component of comprehensive pediatric care. This study systematically reviews the existing evidence related to geographic barriers in children's surgery. METHODS: Medline and Scopus databases were searched for any English language studies that examined associations between geographic burden (rural residence or distance to care) and a quantifiable outcome within pediatric surgical subspecialties. Two independent reviewers extracted data from each study. RESULTS: From 6331 studies screened, 22 studies met inclusion criteria. Most studies were retrospective analyses and conducted in the U.S. or Canada (14 and three studies, respectively); five were conducted outside North America. In transplant surgery (seven studies), greater distance from a transplant center was associated with higher waitlist mortality prior to kidney and liver transplantation, although graft outcomes were generally similar. In congenital cardiac surgery (five studies), greater travel was associated with higher neonatal mortality and older age at surgery but not with post-operative outcomes. In general surgery (eight studies), rural residence was associated with increased rates of perforated appendicitis, higher frequency of negative appendectomy, and increased length of stay after appendectomy. In orthopedic surgery (one study), rurality was associated with decreased post-operative satisfaction. No evidence for disparate outcomes based upon distance or rurality was identified in neurosurgery (one study). CONCLUSIONS: Substantial evidence suggests that geographic barriers impact the receipt of surgical care among children, particularly with regard to transplantation, congenital cardiac surgery, and appendicitis.

Management of visceral vascular anomalies.

Visceral vascular anomalies are common in patients with vascular malformations in other parts of the body and can include lymphatic, venous, and arteriovenous malformations. Depending on the organ or organs involved they may present differently and pose different treatment challenges. Defining the malformation and understanding its extent is paramount in devising management regimens. Medical, interventional, and surgical therapies are often required in combination to treat these complex lesions. There are new and promising advances in the development of therapeutic agents targeting the PI3K/AKT/mTOR pathway. Due to the complex nature of these lesions a coordinated, multi-disciplinary approach is necessary to manage and mitigate symptoms and complications of this diverse group of vascular malformations.

APSA 5.0: Saving even more lifetimes the Jay and Margie Grosfeld presidential symposium.

In light of APSA's 50th Anniversary, the typical Presidential Address was transformed into a "symposium" consisting of talks on the maturation of our organization to APSA 5.0 and the issues and opportunities related to its internal and external environment, especially as they apply to our pediatric surgical patients. Speakers included the President and experts in the fields of diversity, as well as inequity and poverty in the United States.

The Spectrum of Large Adrenal Masses: A Case Series.

Background: Adrenocortical carcinoma (ACC) is a rare malignancy that is challenging to diagnose and has important implications for surgeons who approach this disease. Despite its rarity, it must always be in the differential diagnosis when investigating and treating large adrenal masses. We aim to demonstrate the complexities of this disease through a review of five recent patients at a single tertiary care center. Methods: A series of five patients are described, each of whom presented to a single institution as referrals for "large adrenal mass" in the past sixteen months. Their pre-operative diagnosis, radiographic findings, the operative approach and the pathology results were examined. Results: The first patient had a 12 cm high grade adrenocortical carcinoma. The second patient had pathology consistent with a 9 cm, high grade ACC. The third had a liposarcoma. The fourth patient had a myelolipoma. The fifth was diagnosed with a benign process. Conclusion: Although adrenocortical carcinoma is an uncommon cancer, it has significant implications for the patient's prognosis and ultimately, their treatment algorithm. Therefore, when evaluating large adrenal masses, surgeons must remain vigilant of the possibility of adrenocortical carcinoma.

Non-Obstetric Vulvar/Paravaginal Hematoma in an Adolescent Girl with Scleroderma: An Opportunity for Intimate Partner Violence Intervention.

Description A 15-year-old female presented to the emergency department with swelling and pain in her left labial region as well as urinary retention after intercourse. This was the patient's first time having sexual intercourse and the patient stated that her boyfriend "kneed" her in the labia. A CT scan of the pelvis revealed a large vulvar/external hematoma measuring 6 × 10 × 7 cm which extended into the vaginal vault. This case is the first of a vulvar hematoma reported in a pediatric patient with scleroderma. This case was complicated by the fact that our patient claimed her boyfriend intentionally "kneed" her in the labia, thereby calling sexual abuse into question. Discerning between childhood connective tissue disorders and abuse injuries can be difficult, especially in genital trauma. The treatment team suspected early on that this was a case of intimate partner assault based on the severity of the injury alone and continued when she presented again to the emergency department with concerns for abuse. Sexual violence should be high on the differential in children with connective tissue disorders who present with vulvar or paravaginal hematomas. In our opinion, these injuries warrant a thorough investigation by a child abuse specialist, child protective services and law enforcement.

Venous malformations.

Venous malformations include a spectrum of slow-flow malformations that together are the most common forms of vascular anomalies. Care of these patients requires a multi-disciplinary approach. Goals of care are to ameliorate symptoms and to preserve function. Use of therapeutic compression garments remains the mainstay of therapy. There are new and promising therapies over the last few years that will be invaluable tools for optimal care of this complex patient population. Advances in medical therapy through inhibition of the mTOR/PI3K/AKT pathway with Sirolimus and more proximal targeted drugs along with advances in sclerotherapy techniques are promising for the long-term improvement and amelioration of symptoms in patients with venous malformations.

Pediatric golf cart trauma: Not par for the course.

INTRODUCTION: Golf cart trauma in southeast Georgia represents a significant source of morbidity in the pediatric population. We believe these events are related to the introduction of new state legislation that allows local authorities to govern golf cart operation. METHODS: We performed a retrospective review from 2010 to 2016 of children involved in golf cart traumas (n = 46). We recorded age, gender, Glasgow Coma Scale score (GCS), Injury Severity Score (ISS), location of event, and patient position during event. Outcomes included injury type and length of stay (LOS). RESULTS: The most common position in a golf cart was a passenger (52.2%). Events varied regionally and correlated with stringency of local legislation. Skull fractures afflicted 48% (n = 22) of children and traumatic brain injuries (TBIs) were noted in 35% (n = 17) of patients. TBIs (LOS = 4.6 days, p = 0.006) and abdominal injuries (LOS = 8.5 days, p = 0.017) lengthened mean hospital stay. Increasing ISS was associated with an increased probability of sustaining a TBI (OR 1.295, p = 0.004). Younger children were more likely to sustain a skull fracture (OR 1.170, p = 0.034) while older children incurred more orthopedic injuries (OR 1.217, p = 0.045). CONCLUSION: Skull fractures and TBIs are common following pediatric golf cart trauma. Georgia's varying municipality legislation likely contributes to the growing frequency of this trend. LEVEL OF EVIDENCE: Retrospective study, IV.

Genetic predisposition to cancer: Surveillance and intervention.

Cancer is one of the leading causes of early mortality for children and adolescents. Identifiable genetic cancer predisposition conditions account for a growing proportion of pediatric and adolescent cancer, likely due to increasing knowledge about various predisposition conditions, more widespread cancer genetic counseling, and available diagnostics. Greater awareness, data-driven surgical intervention and clinical surveillance can help facilitate cancer prevention and early detection at cancer stages more amenable to cure. An extensive literature review of published studies and expert opinion with consensus guidelines are reviewed. Specific syndromes where genetics, imaging and surgical intervention are utilized to benefit affected patients and families are presented. In many tumor predisposition syndromes, the underlying genetic diagnosis is made concurrently, or after, malignancy is identified. Improved recognition of underlying predispositions, along with appropriate surgical interventions and imaging surveillance should lead to increased patient survival.