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.

A Single Center Experience With Bilateral Wilms Tumor.

BACKGROUND: Wilms tumor (WT) is the most common pediatric renal malignancy and bilateral disease (BWT) occurs in 5% of cases and is associated with poor outcomes. Management of BWT includes chemotherapy and oncologic resection while preserving renal function. Previous literature has demonstrated variable approaches in BWT treatment. The aim of this study was to examine a single institution experience and outcomes with BWT. METHODS: A retrospective chart review was performed for all patients with WT treated at a free-standing tertiary children's hospital between 1998 and 2018. Patients with BWT were identified and treatment courses were compared. Outcomes of interest included need for dialysis post-operatively, need for renal transplantation post-operatively, disease recurrence, and overall survival. RESULTS: Of 120 children with WT, 9 children (6F:3M) of median age 32 months (IQR: 24-50 months) and median weight 13.7 kg (IQR: 10.9-16.2 kg) were diagnosed with and treated for BWT. Pre-operative biopsies were obtained in 4/9 patients, 3 of whom received neoadjuvant chemotherapy and 1 who underwent radical nephrectomy. Of the 5 patients who did not undergo biopsy, 4/5 were treated with neoadjuvant chemotherapy, and 1/5 underwent upfront nephrectomy. Post-operatively, 4/9 children required dialysis, of whom 2 subsequently underwent renal transplantation. Two patients were lost to follow-up, and of the remaining 7 patients, disease recurrence occurred in 5/7 children and overall survival was 71% (n=5). CONCLUSION: Management of BWT varies regarding the use of pre-operative biopsy, neoadjuvant chemotherapy, and extent of disease resection. Further guidelines on treatment protocols may optimize outcomes in children with BWT.

Children with Congenital Central Hypoventilation Syndrome Do Not Wake up to Ventilator Alarms.

PURPOSE: Congenital Central Hypoventilation Syndrome (CCHS) requires lifelong ventilatory support during sleep. Subjects with CCHS are vulnerable to sleep disturbances associated with treatments, monitoring alarms, and care they receive. We hypothesized that sleep would be disrupted in patients  with CCHS due to ventilatory support and other treatments at night. METHODS: An anonymous survey of patients with CCHS, age up to 17 years was conducted through REDCAP. Subjects were recruited in person, by flyer, email, and social media. Data collected included demographics, PHOX2B genotype, ventilatory support, treatments, nursing, and sleep parameters. RESULTS: We received 23 responses (35% female, 8.1 years ± 5.6). PHOX2B genotypes were 20/24 PARM (2), 20/25 PARM (4), 20/26 PARM (2), 20/27 PARM (9), ≥ 20/28 PARM (2), and NPARM (2). Two subjects did not indicate the PHOX2B genotype. 13/23 were ventilated by PPV via tracheostomy, 7 by NIPPV, 2 by diaphragm pacing, and 1 did not indicate. Additional treatments received at night included suctioning (9), aerosol (1), G-tube feeding (2), and none (11). Only 9 received nursing at night. 13 used pulse oximetry for monitoring, and 9 used both pulse oximetry and end tidal CO2 monitor. 17/23 rarely woke up due to ventilator or monitor alarms. 11/23 usually or sometimes woke up at least once a night; only 2/11 woke up due to alarms. 5/17 who rarely woke up to the alarms had night nursing. CONCLUSION: Most subjects with CCHS did not awaken to ventilator or monitoring alarms and a majority of these patients did not have nighttime nursing. (Mathur et al. in Sleep 43(Supplement_1):A333, 2020).

Correction to: Life-threatening cardiac arrhythmias in congenital central hypoventilation syndrome.

The publisher regrets that in the original published version of this article, one of the author's name was incorrectly presented as "Yaniv Bar Cohen". The correct presentation should have been "Yaniv Bar-Cohen" and is now presented correctly in this article.

Life-threatening cardiac arrhythmias in congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome (CCHS) patients are at risk for life-threatening cardiac arrhythmias, and presentation is dependent on their PHOX2B gene mutation. We describe the presentation of life-threatening arrhythmias in our cohort of CCHS patients. We reviewed the records of 72 CCHS patients seen at CHLA from 2004 to 2018. Data collected included demographics, PHOX2B genotype, ventilatory support, clinical symptoms, ambulatory cardiac monitoring results, and presence of cardiac pacemaker. Sixteen of 72 patients had evidence of potential life-threatening cardiac arrhythmias. PHOX2B genotypes were 20/25 polyalanine repeat expansion mutation (PARM), 20/26 PARM, 20/27 PARM, 20/32 PARM, and c.245C > T non-polyalanine repeat mutation. 11/16 patients were ventilated during sleep only. Symptoms included syncope, dizziness, chest pain, tingling in the left arm, and palpitations. 15/16 patients had recorded ambulatory cardiac monitoring. 5/16 patients were symptomatic without significant sinus pauses. 12/16 patients had implantation of cardiac pacemakers. 9/12 had significant sinus pauses on ambulatory monitoring, and 7/12 patients were symptomatic.Conclusion: CCHS patients have potential life-threatening arrhythmias requiring cardiac pacemaker implantation. Many of these patients are symptomatic with significant sinus pauses on ambulatory monitoring. However, some symptomatic patients with no significant pauses on ambulatory monitoring may still require cardiac pacemaker implantation.What is Known:• CCHS patients are at risk for life-threatening sinus pauses and require cardiac pacemaker implantation.What is New:• CCHS patients regardless of PHOX2B genotype are at risk for significant sinus pauses. Many CCHS patients with significant sinus pause on ambulatory cardiac monitoring are symptomatic and most present with syncope. Some symptomatic patients do not have significant sinus pauses but may still require cardiac pacemaker implantation.