Comparative Clinicopathologic and Genomic Analysis of Hepatocellular Neoplasm, Not Otherwise Specified, and Hepatoblastoma.

Accurate diagnosis and treatment of hepatocellular neoplasm, not otherwise specified (HCN-NOS), poses significant challenges. Our study aimed to investigate the clinicopathologic and genomic similarities and differences between HCN-NOS and hepatoblastoma (HB) to guide diagnostic and treatment strategies. The clinicopathologic characteristics of 16 patients with HCN-NOS and 23 patients with HB were compared. Molecular studies, including the OncoKids DNA- and RNA-based next-generation sequencing panel, chromosomal microarray, and targeted Sanger sequencing analyses of CTNNB1 and TERT promoters, were employed. We found that patients with HCN-NOS were older (P < .001) and more frequently classified as high risk (P < .01), yet they showed no significant differences in alpha fetoprotein levels or survival outcomes compared with those with HB. HCN-NOS and HB had a comparable frequency of sequence variants, with CTNNB1 mutations being predominant in both groups. Notably, TERT promoter mutations (37.5%) and rare clinically significant variants (BRAF, NRAS, and KMT2D) were exclusive to HCN-NOS. HCN-NOS demonstrated a higher prevalence of gains in 1q, encompassing the MDM4 locus (17/17 vs 11/24; P < .001), as well as loss/loss of heterozygosity (LOH) of 1p (11/17 vs 6/24; P < .05) and chromosome 11 (7/17 vs 1/24; P < .01) when compared with HB. Furthermore, the recurrent loss/LOH of chromosomes 3, 4p, 9, 15q, and Y was only observed in HCN-NOS. However, no significant differences were noted in gains of chromosomes 2, 8, and 20, or loss/LOH of 4q and 11p between the 2 groups. Notably, no clinically significant gene fusions were detected in either group. In conclusion, our study reveals that HCN-NOS exhibits high-risk clinicopathologic features and greater structural complexity compared with HB. However, patients with HCN-NOS exhibit comparable alpha fetoprotein levels at diagnosis, CTNNB1 mutation rates, and survival outcomes when subjected to aggressive treatment, as compared with those with HB. These findings have the potential to enhance diagnostic accuracy and inform more effective treatments for HCN-NOS.

Intercostal Nerve Cryoablation is Associated with Reduced Opioid Use in Pediatric Oncology Patients.

INTRODUCTION: Intercostal nerve cryoablation reduces postoperative pain in adults undergoing thoracotomy and children undergoing pectus excavatum repair. We hypothesize that cryoablation is associated with decreased post-thoracotomy pain and opioid use in pediatric oncology patients. METHODS: A single-center retrospective cohort study was performed for oncology patients who underwent thoracotomy from January 1, 2017 to May 31, 2021. Outcomes included postoperative opioid use measured in morphine milligram equivalents per kilogram (MME/kg), pain scores (scale 0-10), and opioid prescription at discharge. Univariable analysis compared patients who received cryoablation to patients who did not receive cryoablation. Multivariable regression analysis controlling for age and prior thoracotomy evaluated associations between cryoablation and postoperative pain. RESULTS: Overall, 32 patients (19 males:13 females) underwent thoracotomy with 16 who underwent >1 thoracotomy resulting in 53 thoracotomies included for analysis. Cryoablation was used in 14 of 53 (26.4%) thoracotomies. Throughout the postoperative hospitalization, patients receiving cryoablation during thoracotomy consumed less opioids compared to patients who did not receive cryoablation (median 0.38 MME/kg, interquartile range [IQR] 0.20-1.15 versus median 1.47 MME/kg, IQR 0.71-4.02, P < 0.01). Maximum pain scores were lower in cryoablation patients (median 6, IQR 5-8) than noncryoablation patients (median 8, IQR 6-10), with a significant difference observed on postoperative day 4 (P = 0.01). Cryoablation patients were also less frequently prescribed opioids at discharge (21.4% versus 58.97%, P = 0.02). Multivariable regression demonstrated that cryoablation was associated with 2.59 MME/kg less opioid use (95% confidence interval -4.56 to -0.63) and decreased likelihood of opioid prescription at discharge (adjusted odds ratio 0.14, 95% confidence interval 0.03-0.67). CONCLUSIONS: Cryoablation is significantly associated with decreased post-thoracotomy pain and opioid use in pediatric cancer patients and should be considered in postoperative pain regimens.

Epidural Analgesia Is Associated With Reduced Inpatient Opioid Consumption and Length of Stay After Wilms Tumor Resection.

INTRODUCTION: Wilms' tumor (WT) is the most common renal malignancy in children and requires an extensive laparotomy for resection. Epidural analgesia (EA) is commonly used in postoperative pain management, but previous literature suggests it may prolong length of stay (LOS). We hypothesized that EA is associated with prolonged LOS but decreased postoperative opioid use in children undergoing WT resection. MATERIALS AND METHODS: A retrospective chart review was performed for all WT patients who underwent nephrectomy between January 1, 1998, and December 31, 2018, at a tertiary children's hospital. Patients with incomplete records, bilateral WT, caval or cardiac tumor extension, or intubation postoperatively were excluded. Outcomes included postoperative opioid consumption measured in oral morphine equivalents per kilogram, receipt of opioid prescription at discharge, and postoperative LOS. Mann-Whitney and multivariable regression analyses were performed. RESULTS: Overall, 46/77 children undergoing WT resection received EA. Children with EA used significantly less inpatient opioids than children without EA (median 1.0 vs. 3.3 oral morphine equivalents per kilogram; P < 0.001). Comparing patients with EA to patients without, there was no significant difference in opioid discharge prescriptions (57% vs. 39%; P = 0.13) or postoperative LOS (median 5 d vs. 6 d; P = 0.10). Controlling for age and disease stage, EA was associated with shorter LOS by multivariable regression (coefficient -0.73, 95% confidence interval: -1.4, -0.05; P = 0.04). CONCLUSIONS: EA is associated with decreased opioid use in children without an associated increase in postoperative LOS following WT resection. EA should be considered as part of multimodal pain management for children undergoing WT resection.

Outcomes of Central Hepatectomy for Pediatric Liver Tumors.

BACKGROUND: Central hepatectomy (CH) is an uncommon surgical technique that is an option for resection of centrally located tumors, with the advantage of sparing normal hepatic parenchyma. Few studies have described outcomes in children undergoing CH. MATERIALS AND METHODS: An IRB-approved, retrospective chart review of patients who underwent CH at Children's Hospital Los Angeles between 2005 and 2016 was performed. Data included patient demographics, peri-operative factors, and post-operative outcomes. The IRB approved waiver of consent. RESULTS: Eight patients (4F:4M) with median age of 1.9 Y underwent CH: 7 patients for HB and 1 patient for focal nodular hyperplasia. Two of the seven HB patients had metastatic disease at diagnosis. Six of the seven HB patients received a median of 4 rounds (3-7 rounds) of pre-operative chemotherapy. The median operative time was 197.5 Min (143-394 Min) with median blood loss of 175 mL (100-1200 mL). Complications included a bile fluid collection requiring aspiration. Seven patients had negative margins on pathology. One patient with a positive margin successfully completed therapy, without recurrent disease. All patients survived to follow-up, with a median follow-up duration of 1.1 Y (0.1-12.1 Y). Two patients developed recurrent disease requiring formal hepatic lobectomy and orthotopic liver transplantation. These patients had negative pathologic margins, with tumor within 1 mm of resection margins. CONCLUSION: CH is an effective alternative to extended hepatectomy for patients with centrally located liver tumors and is associated with good clinical and pathologic outcomes.

Non-Operative Management is More Cost-Effective than Immediate Operation in Perforated Appendicitis Patients with Seven or More Days of Symptoms.

BACKGROUND: Management of perforated appendicitis in children remains controversial. Nonoperative (NO) and immediate operative (IO) strategies are used with varying outcomes. We hypothesized that IO intervention for patients with perforated appendicitis would be more cost-effective than NO management. METHODS: A retrospective chart review of all patients with appendicitis from 2012 to 2015 was performed. Patients with perforated appendicitis were defined by evidence of perforation on imaging. We excluded patients who presented with sepsis, organ failure, and ventriculoperitoneal shunts. NO management was determined by surgeon preference. Univariate and multivariate analyses were performed. RESULTS: IO was performed on 145 patients with perforated appendicitis, whereas 83 were treated with NO management. Compared to IO patients, NO patients incurred higher overall costs, greater length of stay, more readmissions, complications, peripherally inserted central venous catheter lines, interventional radiology drains, and unplanned clinic and emergency department visits (P < 0.0001 for all). Multivariate analysis adjusting for age, days of symptoms, admission C-reactive protein and white blood cell count revealed that NO management was independently associated with increased costs (OR 1.35, 1.12-1.62, 95% CI). Cost curves demonstrated that total cost for IO surpasses that of NO management when patients present with greater than 6.3 d of symptoms (P = 0.01). CONCLUSIONS: Our data suggest that IO is more cost-effective than NO management for patients with perforated appendicitis who present with less than 6.3 d of symptoms, after which point, NO management is more cost-effective. LEVEL OF EVIDENCE: IV.

Hepatocellular malignant neoplasm, NOS: a clinicopathological study of 11 cases from a single institution.

AIMS: The primary aim of this study is to characterize hepatocellular malignant neoplasm, NOS (HEMNOS), a new provisional entity describing a subset of paediatric hepatocellular tumours, which have histological features of neither typical hepatoblastoma (HB) nor hepatocellular carcinoma (HCC). METHODS AND RESULTS: The clinicopathological features of 11 patients with HEMNOS were analysed retrospectively. The median age and serum alpha-fetoprotein level at diagnosis was 7 years and 182 000 ng/ml, respectively. Ten patients presented with pretreatment extent of disease (PRETEXT) stages III/IV multifocal tumours, eight with major vascular involvement, three with lung metastases and three with extrahepatic extension. The original pathology diagnoses were: HB in seven patients, HCC in two and HEMNOS in two. Our pathology review of pre-chemotherapy specimens showed that six tumours had equivocal/overlapping histological features of HB and HCC, four had predominant HB histology along with focal HCC-like histology and one had HB histology. Seven of nine post-chemotherapy resection specimens showed predominant HCC-like histology. Beta-catenin, glypican 3 and spalt-like transcription factor 4 immunostaining showed that all the tumours had a mixed HB/HCC immunophenotype. Telomerase reverse transcriptase immunostaining showed nuclear staining in nine of the 11 tumours. All patients received chemotherapy and achieved gross total primary tumour resection. Nine of the 11 patients were treated with established HB chemotherapy regimens. After a median follow-up of 6.1 years (range: 1.2-11.8 years), all patients were in remission. CONCLUSIONS: HEMNOS is a subtype of HB with focal HCC-like histology, a high-risk clinical profile but favourable outcome following chemotherapy and complete tumour resection.

The role of simultaneous abdominal surgery and wound classification in ventriculoperitoneal shunt complication.

PURPOSE: To evaluate whether simultaneous abdominal surgery or wound contamination at the time of ventriculoperitoneal (VP) shunt placement are associated with increased shunt complications. METHODS: Pediatric patients who underwent VP shunt placement were identified using the National Surgical Quality Improvement Program Pediatric database. VP shunt complication rates were compared between patients who underwent simultaneous abdominal surgeries at the time of VP shunt placement vs those who did not and between those with clean/clean-contaminated and contaminated/dirty wound classifications. Adjusted analysis was performed using 1:5 case-control matching. RESULTS: Among 2715 patients who underwent VP shunt placement, 21 had simultaneous abdominal procedures and were matched with 105 control patients. No significant difference was found in overall (34.3 vs 14.3%, p = 0.07), infectious (8.6 vs 4.8%, p = 1.000), or non-infectious (25.7 vs 9.5%, p = 0.156) shunt complications in the simultaneous vs non-simultaneous group, respectively. In a separate analysis of wound classification, 12 patients with contaminated/dirty wounds were matched with 60 patients with clean/clean-contaminated wounds. The rates of shunt infections for clean/clean-contaminated and contaminated/dirty cases were 10.0 and 16.7%, respectively (p = 0.613). CONCLUSION: In our matched case-control study, neither simultaneous abdominal surgery nor wound contamination at the time of VP shunt placement demonstrated significant increased risk of 30-day post-operative complication.

The clinical management and outcomes of cervical neuroblastic tumors.

BACKGROUND: Although patients with peripheral neuroblastoma (NB; pelvic and thoracic) typically have better outcomes and less aggressive disease compared with patients with abdominal disease, little has been published with regard to the management and outcomes of patients with cervical NB. Herein, we sought to determine the characteristics of cervical neuroblastic tumors and the effect of extent of resection on survival and outcomes. METHODS: We performed a retrospective review of 325 children with neuroblastic tumors at Children's Hospital Los Angeles over a 15-y period (January 1990-February 2015). Data collected from the medical record included location of tumor, age at diagnosis, age at resection, extent of resection, chemotherapy course, International Neuroblastoma Staging System stage, histologic International Neuroblastoma Pathology Classification, and MYCN amplification, a poor prognostic marker. Outcome variables included postoperative complications and overall survival. RESULTS: Twelve patients (3.6%) were found to have cervical neuroblastic tumors (nine NBs, one ganglioneuroblastoma, and two ganglioneuromas). All had favorable histology, and none (0/12) had MYCN amplification. Of the NB patients, four of nine patients underwent resection, whereas the other five underwent biopsy followed by chemotherapy or observation alone. Of the 12 total patients, six underwent gross total resection, four (67%) of which developed complications. At a median follow-up of 4.4 y, there were no recurrences or deaths. CONCLUSIONS: Cervical neuroblastic tumors represent favorable lesions with good outcomes similar to other peripheral neuroblastic tumors. In our study, survival was excellent regardless of extent of tumor resection. Based on our data, we recommend a minimally aggressive surgical approach in managing children with cervical neuroblastic tumors.

Management of Complicated Gastric Bezoars in Children and Adolescents.

BACKGROUND: Gastric bezoars in children are infrequent. Most are trichobezoars. Surgical intervention is sometimes necessary. OBJECTIVES: To describe the clinical findings and radiological workup, as well as treatment and outcome of patients with complicated gastric bezoars who underwent surgery in our institution. METHODS: We conducted a retrospective review of all cases of surgery for gastric bezoars performed in our institution between 2000 and 2010. Data collected included gender and age of the patients, composition and extent of the bezoar, presenting signs and symptoms, imaging studies used, performance of endoscopy, and surgical approach. Outcome was measured by the presence of postoperative complications. RESULTS: We identified seven patients with gastric bezoars who underwent surgery. All were females aged 4-19 years. Six had trichobezoars and one had a mass composed of latex gloves. Presenting symptoms included abdominal pain, vomiting, weight loss, and halitosis. All patients had a palpable epigastric mass. A large variety of imaging modalities was used. Endoscopic removal was attempted in three patients and the laparoscopic approach in one patient, but both routes failed. All patients eventually underwent laparotomy with gastrotomy and recovered without complications. CONCLUSIONS: The presence of gastric bezoars should be suspected in any child with unexplained abdominal pain, vomiting, weight loss, or halitosis, or with a palpable abdominal mass, especially in girls. A variety of imaging modalities can aid in diagnosis. Endoscopic removal might be attempted, although failure of this approach is frequent, necessitating surgical intervention, preferably laparotomy and gastrotomy, which has an excellent outcome.

On-Q ® pain pump versus epidural for postoperative analgesia in children.

PURPOSE: The On-Q(®) pain pump provides a continuous infusion of local anesthesia for management of postoperative pain. The objective of this study was to assess the efficacy and outcomes of the On-Q(®) pump compared to continuous epidural in children postoperatively. METHODS: We performed a retrospective review of patients in our hospital who received a postoperative epidural or On-Q(®) pump from 2005 to 2008. Patients were sub-categorized by incision type. RESULTS: Seventy patients received epidural and 66 On-Q(®). On-Q(®) therapy was longer by 1 day (p < 0.0001), but did not affect postoperative length of stay. Patients with On-Q(®) pumps had a decreased rate of Foley catheter placement (p = 0.002) and shorter duration of catheter use by more than a day (p < 0.001). Moderate to severe pain was similar in the two groups on postoperative days 0-5. Supplemental narcotic use was higher in the On-Q(®) group only on postoperative day 1 (p = 0.005) and in patients with midline and transverse abdominal incisions. No differences were seen in time to ambulation or recovery of postoperative ileus. CONCLUSION: The On-Q(®) pain pump is an effective method for postoperative pain control, without the inherent risks of epidural catheters.

Survival outcomes and surgical morbidity based on surgical approach to pulmonary metastasectomy in pediatric, adolescent and young adult patients with osteosarcoma.

BACKGROUND: Thoracotomy is considered the standard surgical approach for the management of pulmonary metastases in osteosarcoma (OST). Several studies have identified the advantages of a thoracoscopic approach, however, the clinical significance of thoracotomy compared to thoracoscopy is yet to be evaluated in a randomized trial. AIMS: The primary aim was to determine the survival outcomes in OST patients based on surgical approach for pulmonary metastasectomy (PM) and secondary aim was to assess the post-operative morbidities of OST PM through various surgical approaches. MATERIALS AND METHODS: We conducted a single institution retrospective study to compare survival outcomes and surgical morbidity according to the surgical approach of the management of pulmonary metastases in patients with OST. RESULTS: Sixty-one patients with OST underwent PM. Twenty-one patients were metastatic at diagnosis and underwent PM during primary treatment; nine had thoracotomy, six thoracoscopy, and six combined thoracoscopy with thoracotomy (CTT). Forty-three patients with first pulmonary relapse or progression underwent PM; 18 had thoracotomy, 16 thoracoscopy and nine CTT. There was no difference in survival between surgical approaches. There were significantly more postoperative morbidities associated with thoracotomy for initial PM (pain and postoperative chest tube placement), and for PM at first relapse (pneumothoraces, pain, Foley catheter use and prolonged hospitalizations). CONCLUSION: Our study demonstrates that patients with OST pulmonary metastases have comparable poor outcomes despite varying surgical approaches for PM. There were significantly more postoperative morbidities associated with thoracotomy for PM. Surgical bias and other competing risks could not be assessed given the limitations of a retrospective study and may be addressed in a prospective trial evaluating surgical approach for PM in OST.

Alpha-fetoprotein levels correlate with the pathologic grade and surgical outcomes of pediatric retroperitoneal teratomas.

INTRODUCTION: Retroperitoneal teratomas (RTs) are rare neoplasms that comprise 2-5% of all pediatric teratomas and 10% of all pediatric retroperitoneal tumors. Although alpha-fetoprotein (AFP) is a recognized tumor marker for immature teratomas, its prognostic value in neonates and children with RTs is unknown due to physiologic elevation of this marker. The aim of this study was to determine whether AFP level correlates with pathologic grade of pediatric RT and patient outcome. METHODS: A retrospective analysis of all RTs seen at our institution over a 20-year period was performed. Thirteen patients with RTs were identified; their AFP levels were correlated with tumor grade, patient age, and outcome. RESULTS: Twelve patients were less than 1 year of age at diagnosis. There were nine patients with immature, and four with mature RTs. All patients with mature RTs had AFP levels within the normal range, while eight out of nine patients with immature RTs had elevated AFP levels. A statistically significant elevation in AFP was noted in grade III RTs compared with mature RTs (P = 0.036), regardless of age. Major complications including vascular injury, sepsis, and death were more prevalent in high-grade RTs. CONCLUSION: We conclude that AFP levels correlate with tumor immaturity and predict operative morbidity even in patients less than 2 months of age. Despite the increased morbidity associated with surgical excision of high-grade RTs, complete resection results in long-term disease-free survival in the majority of infants with this rare pediatric neoplasm.

Association of Escherichia coli O157:H7 with necrotizing enterocolitis in a full-term infant.

Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency of the neonate. NEC is predominantly seen in premature infants; however, in rare instances it can affect full-term infants as well. Although the pathogenesis of NEC remains elusive, it is well established that bacterial colonization is required for development of this disease. In this report, we present a case of a full-term infant, who developed a very aggressive form of NEC and was found to have Escherichia coli (E. coli) O157:H7 both in stool and blood cultures. Unfortunately, despite aggressive surgical and intensive care management, this infant suffered pan-intestinal necrosis and expired. We were not able to establish the route of transmission. To our knowledge, this is the first report of the association of E. coli O157:H7 with NEC.

Symptomatic catheter-associated thrombosis in pediatric trauma patients: Choose your access wisely.

BACKGROUND: Traumatic injury and the presence of a central venous catheter are 2 of the strongest risk factors for venous thromboembolism in children. The purpose of this study was to determine the incidence of symptomatic, catheter-associated thrombosis in critically injured children. We hypothesized that femoral venous catheters are associated with a greater rate of thrombotic complications when compared with all other central venous access points. METHODS: We reviewed a retrospective cohort (2006-2016) of injured children (≤18 years) admitted to a pediatric intensive care unit with central access placed ≤7 days from admission. Symptomatic, catheter-associated thrombosis was determined by radiographic evidence. Poisson regression was used to compare the incidence of catheter-associated thrombosis per 1,000 catheter days between femoral and nonfemoral catheters. All comparisons were 2-tailed with α = 0.05. RESULTS: We examined 209 pediatric trauma patients with central access (65% femoral, 19% subclavian, 11% arm vein, and 5% internal jugular). Femoral catheters were removed earlier (median [interquartile range] 4 [2-7] vs 8 [3-12] days, P < .001) and were larger in diameter (5 Fr [4-7] vs 4 Fr [4-4], P < .001) when compared with all other catheters. Catheter-associated thrombosis was more frequent in femoral versus nonfemoral catheters (18.4 vs 3.5 per 1,000 catheter days, P = .01). CONCLUSION: Femoral venous catheters are associated with a greater incidence of symptomatic, catheter-associated thrombosis in pediatric trauma patients. When central venous access is indicated for injured children, the femoral site should be avoided. If a femoral venous catheter is necessary, use of a smaller catheter should be considered.

Potential survival benefit with repair of congenital diaphragmatic hernia (CDH) after extracorporeal membrane oxygenation (ECMO) in select patients: Study by ELSO CDH Interest Group.

PURPOSE: Studying the timing of repair in CDH is prone to confounding factors, including variability in disease severity and management. We hypothesized that delaying repair until post-ECMO would confer a survival benefit. METHODS: Neonates who underwent CDH repair were identified within the ELSO Registry. Patients were then divided into on-ECMO versus post-ECMO repair. Patients were 1:1 matched for severity based on pre-ECMO covariates using the propensity score (PS) for the timing of repair. Outcomes examined included mortality and severe neurologic injury (SNI). RESULTS: After matching, 2,224 infants were included. On-ECMO repair was associated with greater than 3-fold higher odds of mortality (OR 3.41, 95% CI: 2.84-4.09, p<0.01). The odds of SNI was also higher for on-ECMO repair (OR 1.49, 95% CI: 1.13-1.96, p<0.01). A sensitivity analysis was performed by including the length of ECMO as an additional matching variable. On-ECMO repair was still associated with higher odds of mortality (OR 2.38, 95% CI: 1.96-2.89, p<0.01). Results for SNI were similar but were no longer statistically significant (OR 1.33, 95% CI: 0.99-1.79, p=0.06). CONCLUSIONS: Of the infants who can be liberated from ECMO and undergo CDH repair, there is a potential survival benefit for delaying CDH repair until after decannulation. TYPE OF STUDY: Treatment Study LEVEL OF EVIDENCE: III.

Thoracoscopic repair of neonatal diaphragmatic hernia.

INTRODUCTION: The use of minimally invasive surgery (MIS) in the neonatal population is increasing. Thoracoscopic intervention for congenital diaphragmatic hernia (CDH) is no exception. In this report, we describe our initial experience with thoracoscopic repair of left-sided diaphragmatic defects in neonates. MATERIALS AND METHODS: We performed retrospective chart reviews on all neonates who underwent thoracoscopic repair of CDH between November 2004 and January 2008. Neonates that underwent thoracoscopic repair were physiologically stable with resolved pulmonary hypertension and minimal to moderate ventilatory support. They had no associated cardiac anomalies. RESULTS: We identified 15 neonates with CDH who underwent thoracoscopic repair during the study period. Ten neonates underwent primary repair of the diaphragmatic defect. Five neonates with large defects required closure with a synthetic patch, which was placed thoracoscopically. The average operating room time was 134 minutes. There were no instances of intraoperative respiratory or cardiac instability. Three patients had a recurrence. One recurrence was seen after thoracoscopic patch repair. Two recurrences occurred following primary repair of left diaphragmatic hernias. There were no deaths. Follow-up has been 4-40 months. CONCLUSIONS: Neonatal MIS for CDH should be limited to stable patients. The ideal candidate is the newborn without associated anomalies, not requiring extracorporeal membrane oxygenation, on minimal ventilatory support, and without evidence of pulmonary hypertension. It is technically possible to perform thoracoscopic repair with a patch.

The Use of Fetal Bronchoscopy in the Diagnosis and Management of a Suspected Obstructive Lung Mass.

Etiologies of fetal lung anomalies include congenital pulmonary airway malformation (CPAM), intra- or extralobar pulmonary sequestration, congenital high airway obstruction syndrome (CHAOS), bronchogenic cyst, and bronchial atresia. Fetal tracheobronchoscopy has been reported both as a diagnostic and therapeutic procedure in the setting of severe congenital lung lesions. In this case report, prenatal imaging of a fetus with a large chest mass was suspicious for an obstructive bronchial lesion. The absence of visible normal lung tissue on the right side and mass effect on the left side raised the concern for pulmonary hypoplasia. After antenatal betamethasone and a period observation, hydropic changes developed. Fetal tracheobronchoscopy was then performed in an effort to identify and decompress the suspected obstructive bronchial lesion. Other than release of bronchial debris, no anatomical abnormalities were visualized. However, the right lung lesion and mediastinal shift both decreased after the fetal bronchoscopy. The newborn underwent postnatal resection of a CPAM Type II and is doing well. We hypothesize that fetal tracheobronchoscopy provided the following potential diagnostic and therapeutic benefits: (1) exclusion of an obstructive bronchial lesion; (2) disimpaction of bronchial debris from the saline lavage that we posit may have contributed to the rapid reduction in CPAM size.

Preoperative Educational Intervention Decreases Unplanned Gastrostomy-Related Health Care Utilization.

Apprehension in taking independent care of children with medical devices may lead to unnecessary visits to the ED and/or acute clinic (AC). To address these concerns, our institution implemented a gastrostomy tube (GT) class in 2011 for caretakers. We hypothesized that inappropriate GT-related ED/AC visits would be lower in preoperatively educated caregivers. We performed a retrospective cohort study of all patients aged 0 to 18 who received GT (surgical or percutaneous) at our institution between 2006 and 2015 (n = 1340). Class attendance (trained vs untrained) and unscheduled GT-related ED/AC visits one year after GT placement were reviewed. Gastrostomy-related ED/AC visits were classified as appropriate (hospital-based intervention) or inappropriate (site care and education/reassurance). Occurrence of ED/AC visits was compared between trained and untrained cohorts. We found that 59 per cent of patients had an unscheduled GT-related ED/AC visit within one year of placement. The trained cohort had 27 per cent less unplanned ED/AC visits within one year (mean 1.21 (SD 1.82) vs untrained 1.65 (2.24), P < 0.001). On multivariate analysis, GT education independently decreased one-year GT-related health care utilization (Odds Ratio 0.75, 95% Confidence Interval 0.59-0.95). Formal education seems to decrease GT-related health care utilization within one year of placement and should be integrated into a comprehensive care plan to improve caregiver self-efficacy.

Outcomes of infants with congenital diaphragmatic hernia treated with venovenous versus venoarterial extracorporeal membrane oxygenation: A propensity score approach.

PURPOSE: Previous studies comparing extracorporeal membrane oxygenation (ECMO) modality for congenital diaphragmatic hernia (CDH) have not accounted for confounding by indication. We therefore hypothesized that using a propensity score (PS) approach to account for selection bias may identify outcome differences based on ECMO modality for infants with CDH. METHODS: We utilized ELSO Registry data (2000-2016). Patients with CDH were divided to either venoarterial (VA) or venovenous (VV) ECMO. Patients were matched by PS to control for nonrandom treatment assignment. Subgroup analyses were conducted based on timing of CDH repair relative to ECMO. Primary analysis was the "intent-to-treat" cohort based on the initial ECMO mode. Mortality was the primary outcome, and severe neurologic injury (SNI) was a secondary outcome. RESULTS: PS matching (3:1) identified 3304 infants (VA = 2470, VV = 834). In the main group, mortality was not different between VA and VV ECMO (OR = 1.01, 95% CI: 0.86-1.18) and there was no difference in SNI between VA and VV (OR = 0.80; 95% CI: 0.63-1.01). For the pre-ECMO CDH repair subgroup, 175 VA cases were matched to 70 VV. In these neonates, mortality was higher for VV compared to VA (OR = 2.10, 95% CI: 1.19-3.69), without any difference in SNI (OR = 1.48; 95% CI: 0.59-3.71). For the subgroup that did not have pre-ECMO CDH repair, 2030 VA cases were matched to 683 VV cases. In this subgroup, VV was associated with 27% lower risk of SNI relative to VA (OR = 0.73, 95% CI: 0.56-0.95) without any difference in mortality (OR = 0.94, 95% CI: 0.79-1.11). CONCLUSION: This study revalidates that ECMO mode does not significantly affect mortality or SNI in infants with CDH. In the subset of infants who require pre-ECMO CDH repair, VA favors survival, whereas, in the subgroup of infants that did not have pre-ECMO CDH repair, VV favors lower rates of SNI. We conclude that neither mode appears consistently superior across all situations, and clinical judgment should remain a multifactorial decision. LEVEL OF EVIDENCE: Level III.

The biological prosthesis is a viable option for abdominal wall reconstruction in pediatric high risk defects.

BACKGROUND: Our aim was to explore the indications for and outcome of biological prostheses to repair high risk abdominal wall defects in children. METHODS: A retrospective chart review was performed of all cases of abdominal wall reconstruction in a single institution between 2007 and 2015. Demographic and clinical variables, technique and complications were described and compared between prosthesis types. RESULTS: A total of 23 patients underwent abdominal wall reconstruction using a biological prosthesis including 17 neonates. The main indication was gastroschisis (17 patients) followed by ruptured omphalocele and miscellaneous conditions. Alloderm™ was most commonly used followed by Surgisis™, Strattice™, Flex-HD™ and Permacol™. In 22 cases wounds were contaminated or infected. Open bowel/stomas were present in 9 cases. Skin was not closed in 11 cases. Post-operative complication rate was 30% and hernia recurrence rate was 17% after a mean follow-up time of 16 months. CONCLUSIONS: The use of a biological prosthesis may offer advantages over a synthetic mesh in pediatric high risk abdominal wall defects. The surgeon should be ready to consider its use in selected cases.