Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

BackgroundTotal splenectomy (TS) and partial splenectomy (PS) are used for children with congenital hemolytic anemia (CHA), although the long‐term outcomes of these procedures are poorly defined. This report describes long‐term outcomes of children with CHA requiring TS or PS.ProcedureWe collected data from children ages 2‐17 with hereditary spherocytosis (HS) or sickle cell disease (SCD) requiring TS or PS from 1996 to 2016 from 14 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a prospective, observational patient registry. We summarized hematologic outcomes, clinical outcomes, and adverse events to 5 years after surgery. Hematologic outcomes were compared using mixed effects modeling.ResultsOver the study period, 110 children with HS and 97 children with SCD underwent TS or PS. From preoperatively compared to postoperatively, children with HS increased their mean hemoglobin level by 3.4 g/dL, decreased their mean reticulocyte percentage by 6.7%, and decreased their mean bilirubin by 2.4 mg/dL. Hematologic improvements and improved clinical outcomes were sustained over 5 years of follow‐up. For children with SCD, there was no change in hemoglobin after PS or TS following surgery, although all clinical outcomes were improved. Over 5 years, there was one child with HS and five children with SCD who developed postsplenectomy sepsis.ConclusionsFor children with HS, there are excellent long‐term hematologic and clinical outcomes following either PS or TS. Although hemoglobin levels do not change after TS or PS in SCD, the long‐term clinical outcomes for children with SCD are favorable.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/155920/1/pbc28290.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/155920/2/pbc28290_am.pd

Finishing the euchromatic sequence of the human genome

The sequence of the human genome encodes the genetic instructions for human physiology, as well as rich information about human evolution. In 2001, the International Human Genome Sequencing Consortium reported a draft sequence of the euchromatic portion of the human genome. Since then, the international collaboration has worked to convert this draft into a genome sequence with high accuracy and nearly complete coverage. Here, we report the result of this finishing process. The current genome sequence (Build 35) contains 2.85 billion nucleotides interrupted by only 341 gaps. It covers ∼99% of the euchromatic genome and is accurate to an error rate of ∼1 event per 100,000 bases. Many of the remaining euchromatic gaps are associated with segmental duplications and will require focused work with new methods. The near-complete sequence, the first for a vertebrate, greatly improves the precision of biological analyses of the human genome including studies of gene number, birth and death. Notably, the human enome seems to encode only 20,000-25,000 protein-coding genes. The genome sequence reported here should serve as a firm foundation for biomedical research in the decades ahead

Postoperative respiratory complications in SARS-CoV-2 positive pediatric patients across 20 United States hospitals: A cohort study

Introduction:Data examining rates of postoperative complications among SARS-CoV-2 positive children are limited. The purpose of this study was to evaluate the impact of symptomatic and asymptomatic SARS-CoV-2 positive status on postoperative respiratory outcomes for children. Methods:This retrospective cohort study included SARS-CoV-2 positive pediatric patients across 20 hospitals who underwent general anesthesia from March to October 2020. The primary outcome was frequency of postoperative respiratory complications, including: high-flow nasal cannula/non invasive ventilation, reintubation, pneumonia, Extracorporeal Membrane Oxygenation (ECMO), and 30-day respiratory-related readmissions or emergency department (ED) visits. Univariate analyses were used to evaluate associations between patient and procedure characteristics and stratified analyses by symptoms were performed examining incidence of complications. Results:Of 266 SARS-CoV-2 positive patients, 163 (61.7%) were male, and the median age was 10 years (interquartile range 4-14). The majority of procedures were emergent or urgent (n = 214, 80.5%). The most common procedures were appendectomies (n = 78, 29.3%) and fracture repairs (n = 40,15.0%). 13 patients (4.9%) had preoperative symptoms including cough or dyspnea. 26 patients (9.8%) had postoperative respiratory complications, including 15 requiring high-flow oxygen, 8 with pneumonia, 4 requiring non invasive ventilation, 3 respiratory ED visits, and 2 respiratory readmissions. Respiratory complications were more common among symptomatic patients than asymptomatic patients (30.8% vs. 8.7%, p = 0.01). Higher ASA class and comorbidities were also associated with postoperative respiratory complications. Conclusions:Postoperative respiratory complications are less common in asymptomatic versus symptomatic SARS-COV-2 positive children. Relaxation of COVID-19-related restrictions for time-sensitive, non urgent procedures in selected asymptomatic patients may be reasonably considered. Additionally, further research is needed to evaluate the costs and benefits of routine testing for asymptomatic patients. Level of evidence:Iii, Respiratory complications

A Rare Case of Contralateral Diaphragm Paralysis following Birth Injury with Brachial Plexus Palsy: A Case Report and Review of the Literature

Clinical History. A 4.4 kg male was born to a 25-year-old, G2P1, nondiabetic woman at 39 and 5/7 weeks. Delivery was complicated by shoulder dystocia requiring forceps-assisted vaginal delivery, resulting in left arm Erb’s palsy secondary to left brachial plexus injury. He was born with low muscle tone and bradycardia and subsequently required intubation for poor respiratory effort. He was extubated on day one of life but continued to be tachypneic and have borderline oxygen saturation, requiring intensive care. Chest radiographs demonstrated a progressive clearing of his lung fields, consistent with presumptively diagnosed meconium aspiration. However, a persistent elevation of the right hemidiaphragm was noted, and his tachypnea and increased work of breathing continued. Focused ultrasound of the diaphragm was performed, confirming decreased motion of the right hemidiaphragm. Following a multidisciplinary discussion, thoracoscopic right diaphragm plication was performed on the 33rd day of life. He was extubated postoperatively and subsequently weaned to room air with a notable decrease in tachypnea over 48 hours. He was discharged on postoperative day 12 and continues to thrive at 6 months of age without respiratory embarrassment. Purpose. Ipsilateral phrenic nerve injury with diaphragm paralysis from shoulder dystocia during vaginal delivery is a recognized phenomenon. Herein, we present a case of contralateral diaphragm paralysis in order to draw attention to the clinician that this discordance is possible. Key Points. According to Raimbault et al., clinical management of newborns who experience birth injury is a multidisciplinary effort. According to Fitting and Grassino, though most cases of phrenic nerve injuries are ipsilateral to shoulder dystocia brachial plexus palsy, contralateral occurrence is possible and should be considered. According to Waters, diaphragm plication is a safe and effective operation

Timing of pediatric pyloromyotomy on hospital length of stay

Introduction: Pyloromyotomy timing is predicated upon correction of electrolyte abnormalities. Among infants who presented with normal electrolytes, we hypothesized that pyloromyotomy the evening of presentation, rather than waiting until morning, would confer shorter length of stay (LOS). Methods: This single-center retrospective cohort study included patients who underwent pyloromyotomy from 2012 to 2021. Exposure was time of operation with nighttime considered between the times of 17:00 and 06:59 and daytime between 07:00 and 16:59. A 2:1 daytime to nighttime match was performed among patients who presented with normal electrolytes with Fisher's Exact and Student's t-test for comparisons. Results: Of 520 patients, 15 (3%) underwent pyloromyotomy overnight and were matched to 30 daytime patients. There were no differences in median age (33 days (interquartile range [IQR] 29–44) vs 32 days (IQR 25–44)), male sex (15 (100%) vs 28 (93.3%), or history of prematurity (0 (0%) vs 2 (6.7%)) for nighttime compared to daytime, respectively. Operative outcomes including conversion to open, duodenal perforation, incomplete myotomy, or surgical site infection did not differ between the groups. While the nighttime group had a significantly shorter time from presentation to operating room (OR) than the daytime group (5.3 vs 15.9 h), there were no significant differences in total LOS (45.7 vs 57.3 h, p = 0.13). Conclusion: For infants with hypertrophic pyloric stenosis who present with normal electrolytes, it is safe to offer operation same-day or following a night of hydration. There was no evidence of improved hospital utilization for patients undergoing pyloromyotomy the night of presentation

A mixed-method approach to generate and deliver rapid-cycle evaluation feedback: Lessons learned from a multicenter implementation trial in pediatric surgery

Background: Rapid-cycle feedback loops provide timely information and actionable feedback to healthcare organizations to accelerate implementation of interventions. We aimed to (1) describe a mixed-method approach for generating and delivering rapid-cycle feedback and (2) explore key lessons learned while implementing an enhanced recovery protocol (ERP) across 18 pediatric surgery centers. Methods: All centers are members of the Pediatric Surgery Research Collaborative (PedSRC, www.pedsrc.org), participating in the ENhanced Recovery In CHildren Undergoing Surgery (ENRICH-US) trial. To assess implementation efforts, we conducted a mixed-method sequential explanatory study, administering surveys and follow-up interviews with each center's implementation team 6 and 12 months following implementation. Along with detailed notetaking and iterative discussion within our team, we used these data to generate and deliver a center-specific implementation report card to each center. Report cards used a traffic light approach to quickly visualize implementation status (green = excellent; yellow = needs improvement; red = needs significant improvement) and summarized strengths and opportunities at each timepoint. Results: We identified several benefits, challenges, and practical considerations for assessing implementation and using rapid-cycle feedback among pediatric surgery centers. Regarding potential benefits, this approach enabled us to quickly understand variation in implementation and corresponding needs across centers. It allowed us to efficiently provide actionable feedback to centers about implementation. Engaging consistently with center-specific implementation teams also helped facilitate partnerships between centers and the research team. Regarding potential challenges, research teams must still allocate substantial resources to provide feedback rapidly. Additionally, discussions and consensus are needed across team members about the content of center-specific feedback. Practical considerations include carefully balancing timeliness and comprehensiveness when delivering rapid-cycle feedback. In pediatric surgery, moreover, it is essential to actively engage all key stakeholders (including physicians, nurses, patients, caregivers, etc.) and adopt an iterative, reflexive approach in providing feedback. Conclusion: From a methodological perspective, we identified three key lessons: (1) using a rapid, mixed method evaluation approach is feasible in pediatric surgery and (2) can be beneficial, particularly in quickly understanding variation in implementation across centers; however, (3) there is a need to address several methodological challenges and considerations, particularly in balancing the timeliness and comprehensiveness of feedback. Trial registration: NIH National Library of Medicine Clinical Trials. Clinicaltrials.gov Identifier: NCT04060303. Registered August 7, 2019, https://clinicaltrials.gov/ct2/show/NCT04060303</p

Association between COVID-19 related elective surgery cancellations and pediatric inguinal hernia complications: A nationwide multicenter cohort study

Background: Optimal inguinal hernia repair timing remains controversial. It remains unclear how COVID-19 related elective surgery cancellations impacted timing of inguinal hernia repair and whether any delays led to complications. This study aims to determine whether elective surgery cancellations are safe in pediatric inguinal hernia. Methods: This multicenter retrospective cohort study at 14 children\u27s hospitals included patients ≤18 years who underwent inguinal hernia repair between September 13, 2019, through September 13, 2020. Patients were categorized by whether their inguinal hernia repair occurred before or after their hospital\u27s COVID-19 elective surgery cancellation date. Incarceration and emergency department encounters were compared between pre and postcancellation. Results: Of 1,404 patients, 604 (43.0%) underwent inguinal hernia repair during the postcancellation period, 92 (6.6%) experienced incarceration, and 213 (15.2%) had an emergency department encounter. The postcancellation period was not associated with incarceration (odds ratio 1.54; 95% confidence interval 0.88-2.71; P = .13) or emergency department encounters (odds ratio 1.53; 95% confidence interval 0.94-2.48; P = .09) despite longer median times to inguinal hernia repair (precancellation 29 days [interquartile range 13-55 days] versus postcancellation 31 days [interquartile range 14-73 days], P = .01). Infants were more likely to have the emergency department be their index presentation in the postcancellation period (odds ratio 1.69; 95% confidence interval 1.24-2.31; P \u3c .01). Conclusion: Overall, COVID-19 elective surgery cancellations do not appear to increase the likelihood of incarceration or emergency department encounters despite delays in inguinal hernia repair, suggesting that cancellations are safe in children with inguinal hernia. Assessment of elective surgery cancellation safety has important implications for health policy

Postoperative Respiratory Complications in SARS-CoV-2 Positive Pediatric Patients Across 20 United States Hospitals: A Cohort Study

IntroductionData examining rates of postoperative complications among SARS-CoV-2 positive children are limited. The purpose of this study was to evaluate the impact of symptomatic and asymptomatic SARS-CoV-2 positive status on postoperative respiratory outcomes for children.MethodsThis retrospective cohort study included SARS-CoV-2 positive pediatric patients across 20 hospitals who underwent general anesthesia from March to October 2020. The primary outcome was frequency of postoperative respiratory complications, including: high-flow nasal cannula/non invasive ventilation, reintubation, pneumonia, Extracorporeal Membrane Oxygenation (ECMO), and 30-day respiratory-related readmissions or emergency department (ED) visits. Univariate analyses were used to evaluate associations between patient and procedure characteristics and stratified analyses by symptoms were performed examining incidence of complications.ResultsOf 266 SARS-CoV-2 positive patients, 163 (61.7%) were male, and the median age was 10 years (interquartile range 4-14). The majority of procedures were emergent or urgent (n&nbsp;=&nbsp;214, 80.5%). The most common procedures were appendectomies (n&nbsp;=&nbsp;78, 29.3%) and fracture repairs (n&nbsp;=&nbsp;40,15.0%). 13 patients (4.9%) had preoperative symptoms including cough or dyspnea. 26 patients (9.8%) had postoperative respiratory complications, including 15 requiring high-flow oxygen, 8 with pneumonia, 4 requiring non invasive ventilation, 3 respiratory ED visits, and 2 respiratory readmissions. Respiratory complications were more common among symptomatic patients than asymptomatic patients (30.8% vs. 8.7%, p&nbsp;=&nbsp;0.01). Higher ASA class and comorbidities were also associated with postoperative respiratory complications.ConclusionsPostoperative respiratory complications are less common in asymptomatic versus symptomatic SARS-COV-2 positive children. Relaxation of COVID-19-related restrictions for time-sensitive, non urgent procedures in selected asymptomatic patients may be reasonably considered. Additionally, further research is needed to evaluate the costs and benefits of routine testing for asymptomatic patients.Level of evidenceIii, Respiratory complications