Spindle cell sarcoma of the chest wall: a pediatric case report.

Chest wall sarcomas are reported to be infrequent among thoracic tumors. The spindle cell subtype makes up a small percentage of this group. These tumors can be asymptomatic or cause symptoms of chest pain and shortness of breath due to the mass effect, which can lead to a delay in diagnosis. A 10-year-old female with a persistent cough, shortness of breath on exertion, and left-sided chest pain presented to the ED. Imaging indicated a chest wall mass filling the left hemithorax with a rightward mediastinal shift. During surgical resection, two tumors were removed, with resection of parts of the latissimus dorsi and serratus anterior. A diagnosis of MGA:NUTM1 spindle cell sarcoma was made pathologically. The patient was successfully treated with surgery and adjuvant chemoradiotherapy. We hope to add to our academic knowledge by presenting the presentation and treatment of SCS in a pediatric patient.

Giant mediastinal teratoma in a young infant: a case report.

Background: Giant mediastinal tumors in the pediatric population can pose unique challenges for resection such as cardiovascular collapse on induction of anesthesia and injury to surrounding structures that may be compressed, displaced, or invaded by the mass. Principles that must be borne in mind during removal of giant mediastinal masses include: appropriate cross-sectional imaging to define extent of mass; airway control during induction of anesthesia; a multidisciplinary collaborative approach including cardiothoracic surgery; preparation for urgent sternotomy; plan for peripheral cannulation to institute cardiopulmonary bypass if needed; preservation of neurovasculature structures during dissection; complete resection whenever possible. While complete resection is desirable and results in an excellent prognosis, it may not be achievable especially if the tumor encases coronary arteries, and it is acceptable to leave small amounts of tumor behind. Case Description: Here we present a case describing surgical management of a giant mediastinal teratoma in a two-month-old female. The patient was found to have a large mediastinal mass during workup for cough and noisy breathing. She underwent preoperative echocardiogram demonstrating normal cardiac function followed by uncomplicated, open resection of the mass. Conclusions: Giant mediastinal tumors give rise to unique challenges for resection in small infants. The principles of airway control, preparation for urgent sternotomy, preparation for peripheral cardiopulmonary bypass cannulation, and preservation of neurovasculature during dissection must be borne in mind.

It's time to deconstruct treatment-failure: A randomized controlled trial of nonoperative management of uncomplicated pediatric appendicitis with antibiotics alone.

BACKGROUND: Published data demonstrate that management of uncomplicated pediatric appendicitis with antibiotics-alone is safe and frequently successful. Randomized controlled trials (RCT) comparing antibiotics-alone to appendectomy are lacking, alongside insight into drivers of failure. We sought to validate the antibiotics-alone approach and identify barriers to success using an RCT design. METHODS: Patients aged 6-17 years with uncomplicated appendicitis were randomized to appendectomy or intravenous piperacillin/tazobactam for 24-48 h followed by 10 days of oral ciprofloxacin/metronidazole. Enrollment required symptoms <48 h, WBC<18, appendiceal diameter <11 mm, and radiographic absence of perforation. Lack of clinical improvement or persistently elevated WBC resulted in appendectomy. Primary outcomes were 1-year success rate of antibiotics-alone and quality-of-life measures. RESULTS: Among 39 children enrolled over 31 months, 20 were randomized to antibiotics-alone and 19 to surgery. At 1 year, 6 nonoperative patients underwent appendectomy (70% success). Four cases were not true antibiotic failures but instead reflected "pragmatic" challenges to executing nonoperative algorithms. Only 2 cases represented recurrent/refractory appendicitis, suggesting a 90% adjusted 1-year success rate. Parental PedsQL™ scores were similar between treatment cohorts (91.3 vs 90.2, P = 0.32). Children treated with antibiotics-alone had faster return to activity (2.0 vs 12 days, P = 0.001) and fewer parental missed work days (0.0 vs 2.5, P = 0.03). CONCLUSIONS: These data corroborate findings from non-randomized studies suggesting 70-90% of uncomplicated pediatric appendicitis can be treated with antibiotics-alone, with fewer disability days. Failures appear multifactorial, often reflecting practical hurdles and not antibiotic limitations. As surgeons consider nonoperative protocols for uncomplicated appendicitis, these data further inform the variability of treatment success. LEVEL OF EVIDENCE: 1; randomized controlled trial.

Increase in Pediatric Perforated Appendicitis in the New York City Metropolitan Region at the Epicenter of the COVID-19 Outbreak.

OBJECTIVE: The aim of the study was to determine whether perforated appendicitis rates in children were influenced by the Coronavirus disease 2019 (COVID-19) surge. BACKGROUND: Disruption of care pathways during a public health crisis may prevent children from obtaining prompt assessment for surgical conditions. Progression of appendicitis to perforation is influenced by timeliness of presentation. In the context of state-mandated controls and public wariness of hospitals, we investigated the impact of the COVID-19 outbreak on perforated appendicitis in children. STUDY DESIGN: We conducted an analysis of all children presenting to 3 hospital sites with acute appendicitis between March 1 and May 7, 2020, corresponding with the peak COVID-19 outbreak in the New York City region. Control variables were collected from the same institutions for the preceding 5 years. The primary outcome measure was appendiceal perforation. RESULTS: Fifty-five children presented with acute appendicitis over 10 weeks. Compared to a 5-year control cohort of 1291 patients, we observed a higher perforation rate (45% vs 27%, odds ratio 2.23, 95% confidence interval 1.29-3.85, P = 0.005) and longer mean duration of symptoms in children with perforations (71 ±â€Š39 vs 47 ±â€Š27 h, P = 0.001) during the COVID-19 period. There were no differences in perforation rates (55% vs 59%, P = 0.99) or median length of stay (1.0 vs 3.0 days, P = 0.58) among children screening positive or negative for SARS-CoV-2. CONCLUSIONS: Children in the epicenter of the COVID-19 outbreak demonstrated higher rates of perforated appendicitis compared to historical controls. Preoperative detection of SARS-CoV-2 was not associated with inferior outcomes. Although children likely avoid much of the morbidity directly linked to COVID-19, disruption to local healthcare delivery systems may negatively impact other aspects of pediatric surgical disease.

Laparoscopic Transabdominal Colopexy for Prolapse of a Newborn End Colostomy: A Novel Technique.

Background: Prolapse can occur in up to 20% of newborn end colostomies and may be associated with significant morbidities that require operative intervention. Prolapse repair is traditionally performed through an open parastomal or laparotomy incision. We report on 2 cases that utilized a novel laparoscopic transabdominal colopexy technique, which offered a minimally invasive solution uniquely suited to neonates and infants while obviating the morbidity of open reoperative surgery. Materials and Methods: Retrospective review of 2 patients at a single center undergoing a laparoscopic transabdominal colopexy for end colostomy prolapse. The primary outcome measure was prolapse recurrence. Secondary outcomes included intraoperative or immediate postoperative complications. Results: Both patients who underwent the laparoscopic transabdominal colopexy procedure had prolapsed end colostomies. There were no intraoperative or immediate postoperative complications. Both patients had no additional episodes of recurrence during the follow-up period. One patient has since had their colostomy reversed without complications. Conclusion: We present our initial results in the utilization of a novel technique for repair of a newborn end colostomy prolapse-laparoscopic transabdominal colopexy. Our technique thus far has demonstrated success in preventing recurrent prolapse through a minimally invasive technique with no significant morbidity.

Simple preoperative radiation safety interventions significantly lower radiation doses during central venous line placement in children.

PURPOSE: The purpose of this study was to reduce radiation exposure during pediatric central venous line (CVL) placement by implementing a radiation safety process including a radiation safety briefing and a job-instruction model with a preradiation time-out. METHODS: We reviewed records of all patients under 21 who underwent CVL placement in the operating room covering 22 months before the intervention through 10 months after 2013-2016. The intervention consisted of a radiation safety briefing by the surgeon to the intraoperative staff before each case and a radiation safety time-out. We measured and analyzed the dose area product (DAP), total radiation time pre- and postintervention, and the use of postprocedural chest radiograph. RESULTS: 100 patients with valid DAP measurements were identified for analysis (59 preintervention, 41 postintervention). Following implementation of the radiation safety process, there was a 79% decrease in median DAP (61.4 vs 13.1 rad*cm2, P < 0.001) and a 73% decrease in the median radiation time (28 vs 7.6 s, P < 0.001). Additionally, there was a significant reduction in use of confirmatory CXR (95% vs 15%, P < 0.01). CONCLUSION: A preoperative radiation safety briefing and a radiation safety time-out supported by a job-instruction model were effective in significantly lowering the absorbed doses of radiation in children undergoing CVL insertion. TYPE OF STUDY: Case-control study. LEVEL OF EVIDENCE: Level III.

Increased capture of pediatric surgical complications utilizing a novel case-log web application to enhance quality improvement.

PURPOSE: Documenting surgical complications is limited by multiple barriers and is not fostered in the electronic health record. Tracking complications is essential for quality improvement (QI) and required for board certification. Current registry platforms do not facilitate meaningful complication reporting. We developed a novel web application that improves accuracy and reduces barriers to documenting complications. METHODS: We deployed a custom web application that allows pediatric surgeons to maintain case logs. The program includes a module for entering complication data in real time. Reminders to enter outcome data occur at key postoperative intervals to optimize recall of events. Between October 1, 2014, and March 31, 2015, frequencies of surgical complications captured by the existing hospital reporting system were compared with data aggregated by our application. RESULTS: 780 cases were captured by the web application, compared with 276 cases registered by the hospital system. We observed an increase in the capture of major complications when compared to the hospital dataset (14 events vs. 4 events). CONCLUSIONS: This web application improved real-time reporting of surgical complications, exceeding the accuracy of administrative datasets. Custom informatics solutions may help reduce barriers to self-reporting of adverse events and improve the data that presently inform pediatric surgical QI. TYPE OF STUDY: Diagnostic study/Retrospective study. LEVEL OF EVIDENCE: Level III - case control study.

Imperforate Anus with Jejunal Atresia Complicated by Intestinal Volvulus: A Case Report.

Anorectal malformations (ARMs) commonly co-occur with other congenital anomalies, particularly VACTERL (vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb, and duodenal) associations. However, this collection of associations is not comprehensive, and other concurrent anomalies may exist that can be missed during the standard work-up of patients with ARMs. We present a rare case of a neonate with a low ARM with concurrent jejuno-ileal atresia that was diagnosed after the correction of the ARM when the patient developed segmental volvulus. This case illustrates the importance of having a high index of suspicion when deviation from a classic presentation occurs.

A novel approach to leveraging electronic health record data to enhance pediatric surgical quality improvement bundle process compliance.

PURPOSE: Quality improvement (QI) bundles have been widely adopted to reduce surgical site infections (SSI). Improvement science suggests when organizations achieve high-reliability to QI processes, outcomes dramatically improve. However, measuring QI process compliance is poorly supported by electronic health record (EHR) systems. We developed a custom EHR tool to facilitate capture of process data for SSI prevention with the aim of increasing bundle compliance and reducing adverse events. METHODS: Ten SSI prevention bundle processes were linked to EHR data elements that were then aggregated into a snapshot display superimposed on weekly case-log reports. The data aggregation and user interface facilitated efficient review of all SSI bundle elements, providing an exact bundle compliance rate without random sampling or chart review. RESULTS: Nine months after implementation of our custom EHR tool, we observed centerline shifts in median SSI bundle compliance (46% to 72%). Additionally, as predicted by high reliability principles, we began to see a trend toward improvement in SSI rates (1.68 to 0.87 per 100 operations), but a discrete centerline shift was not detected. CONCLUSION: Simple informatics solutions can facilitate extraction of QI process data from the EHR without relying on adjunctive systems. Analyses of these data may drive reductions in adverse events. Pediatric surgical departments should consider leveraging the EHR to enhance bundle compliance as they implement QI strategies.

Emergent Airway Management of an Uncooperative Child with a Large Retropharyngeal and Posterior Mediastinal Abscess.

Retropharyngeal abscesses are deep neck space infections that can lead to life-threatening airway emergencies and other catastrophic complications. Retropharyngeal abscesses demand prompt diagnosis and early establishment of a definitive airway when there is airway compromise. This can be difficult in an uncooperative patient. We present the case of a 12-year-old girl with mediastinitis and tracheal compression and anterior displacement from a large retropharyngeal and posterior mediastinal abscess secondary to traumatic esophageal perforation, who received successful awake nasal fiberoptic intubation. Anesthesiologists must be prepared for airway emergencies in uncooperative patients, especially children, but there is controversy concerning the use of sedation.

Managing radiation exposure in children--reexamining the role of ultrasound in the diagnosis of appendicitis.

PURPOSE: To assess the efficacy and accuracy of ultrasonography (US) and selective computed tomography (CT) in the diagnosis of acute appendicitis in children. METHODS: A retrospective review of all ultrasound evaluations for appendicitis from July 1, 2003, to June 30, 2010, was conducted at two urban pediatric centers. Beginning in 2003, a multi-disciplinary diagnostic protocol was implemented to reduce radiation exposure employing US as the initial imaging modality followed by CT for non-diagnostic US studies in patients with an equivocal clinical presentation. The imaging, operative findings, and pathology of 802 patients (365 females, 437 males, age less than 18 years) with suspected appendicitis were reviewed. The sensitivity, specificity, predictive value, and negative appendectomy rate of the protocol were analyzed. A telephone survey was conducted of patients discharged without a diagnosis of appendicitis to evaluate the missed appendicitis rate. RESULTS: Of the 601 pediatric appendectomies performed, a total of 275 (46%) were diagnosed by protocol. The selective protocol had a sensitivity of 94.2%, specificity of 97.5%, positive predictive value of 95.2%, and negative predictive value of 97.0%. The negative appendectomy rate was 1.82%, and the missed appendicitis rate was 0%. No patient discharged after only ultrasound evaluation without undergoing surgery reported missed appendicitis on the survey (41.7% response rate). Protocol use increased from 6.7% to 88.3%. US was the sole imaging modality in 630 of all 802 patients (78.6%). CONCLUSIONS: US followed by selective CT for the diagnosis of acute appendicitis is useful and accurate. This has important implications in the reduction of childhood radiation exposure.

Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study.

INTRODUCTION: Experience in thoracoscopic congenital diaphragmatic hernia (CDH) repair has expanded, yet efficacy equal to that of open repair has not been demonstrated. In spite of reports suggesting higher recurrent hernia rates after thoracoscopic repair, this approach has widely been adopted into practice. We report a large, single institutional experience with thoracoscopic CDH repair with special attention to recurrent hernia rates. METHODS: We reviewed the records of neonates with unilateral CDH repaired between January 2006 and February 2010 at Morgan Stanley Children's Hospital. Completely thoracoscopic repairs were compared to open repairs of the same period. In addition, successful thoracoscopic repairs were compared with thoracoscopic repairs that developed recurrence. Data were analyzed by Mann-Whitney U and Fisher exact tests. RESULTS: Thirty-five neonates underwent attempted thoracoscopic repair, with 26 completed. Concurrently, 19 initially open CDH repairs were performed. Preoperatively, patients in the open repair group required more ventilatory support than the thoracoscopic group. Recurrence was higher after thoracoscopic repair (23% vs 0%; P = .032). In comparing successful thoracoscopic repairs to those with recurrence, none of the factors analyzed were predictive of recurrence. CONCLUSIONS: Early recurrence of hernia is higher in thoracoscopic CDH repairs than in open repairs. Technical factors and a steep learning curve for thoracoscopy may account for the higher recurrence rates, but not patient severity of illness. In an already-tenuous patient population, performing the repair thoracoscopically with a higher risk of recurrence may not be advantageous.

Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease.

PURPOSE: Thoracoscopic lobectomy for congenital cystic lung lesions is an accepted technique in pediatric surgery. Since an increasing number of these lesions are detected prenatally, the safety and efficacy of infant resections have been questioned. We reviewed our experience over a 10-year period to evaluate early resection of these lesions. METHODS: From January 2001 to August 2009, 75 patients under 1 year of age and weighing <10 kg underwent thoracoscopic lobectomy at two institutions. Patients carried the following diagnoses: 52 had congenital cystic adenomatoid malformation, 20 had bronchopulmonary sequestration, and 3 had congenital lobar emphysema. All lesions were confirmed after birth by computed tomography scan. Patient age at operation ranged from 4 days to 11 months and patient weight from 3.1 to 10 kg. RESULTS: Seventy-four of 75 lobectomies were thoracoscopically completed. There were 16 upper lobectomies, 1 middle lobectomy, and 55 lower lobectomies. Operative time ranged from 45 to 225 minutes. Hospital length of stay ranged from 1 to 5 days. A subset of 26 patients had surgery younger than 3 months of age and <5 kg, despite being asymptomatic. Their operative time averaged 90 minutes, and mean length of hospital stay was 1.5 days. CONCLUSION: Thoracoscopic lobectomy is safe for infants <10 kg and avoids the morbidity associated with thoracotomy. Operating early on younger patients may avoid the inflammatory changes associated with both clinically apparent and subclinical infections, even in patients weighing <5 kg. This may make the procedures less technically challenging and may result in lower complication and conversion rates.

Thoracoscopic lobectomy for severe bronchiectasis in children.

BACKGROUND/PURPOSE: Lung resection should be considered for severe, localized bronchiectasis causing recurrent infections in patients with cystic fibrosis (CF) and other diseases. This series represents our experience and results with thoracoscopic lobectomy for the treatment of severe bronchiectasis confined to a single lobe. METHODS: Nineteen thoracoscopic anatomic lobectomies were performed between July 1994 and August 2008. Patient age at the time of surgery ranged from 14 months to 22 years. Left lower lobectomy was the most frequently performed procedure (n = 9). Eight patients suffered from CF, 5 had chronic pneumonia, 2 had chronic aspiration, and 3 had other diagnoses. RESULTS: Mean operative time was 162 minutes (range, 65-300), and no cases required a conversion to thoracotomy. The mean duration of postoperative chest tube drainage was 3.2 days (range, 1-9). The mean postoperative length of stay was 3.6 days (range, 1-12). One patient had a prolonged air leak lasting 9 days, which resolved with the placement of a second chest tube. Another had a small hydropneumothorax, which persisted after chest tube removal but resolved spontaneously. CONCLUSIONS: Thoracoscopic lobectomy for severe lobar bronchiectasis with recurrent infection is technically challenging, but appears to be safe and effective. Avoidance of a thoracotomy, in this group of patients, allows for earlier mobilization, less postoperative pain with cough and chest physiotherapy, and faster recovery. There appear to be significant benefits in these patients with chronic respiratory illnesses.

Catastrophic cardiac injuries encountered during the minimally invasive repair of pectus excavatum.

This paper presents four severe cardiac injuries that occurred in patients who underwent the minimally invasive repair of pectus excavatum (MIRPE). These complications occurred in different clinical settings, namely in a patient with an extremely severe form of pectus, in a patient who had previously undergone an open repair, after a previous open heart surgery, and at the time of bar removal. The purpose of this article is to review the circumstances leading to these cardiac injuries, share what we have learned from these patients, and hopefully help avoid these complications in the future.

Surgical correction of pectus excavatum.

Pectus deformities arise from overgrowth of the cartilagenous portion of the ribs. Traditional resective/open procedures have been almost entirely replaced by video assisted retrosternal bar placement with excellent outcomes. This review considers the comprehensive evaluation and care of a pectus patinet as well as technical details regarding conduct of the surgery.

Challenges to cannulation for extracorporeal support in neonates with right-sided congenital diaphragmatic hernia.

Right-sided diaphragmatic defects represent less than 20% of all congenital diaphragmatic hernias (CDH). Recent data suggest that right CDH (R-CDH) may carry a disproportionately high morbidity as well as increased rates of extracorporeal support when compared with left CDH. Treatment of infants with R-CDH may be further complicated by anatomical distortion unique to right-sided defects. We report 2 cases of azygous vein cannulation in neonates with large isolated R-CDH. Both infants had postnatal deteriorations within 48 hours, met our criteria for extracorporeal membrane oxygenation (ECMO), and underwent venoarterial cannulations through the right neck. In each case, the venous cannula passed directly into the azygous vein and failed to provide adequate ECMO support. Echocardiography confirmed both cases of azygous cannulation. In one child, the right atrium was successfully cannulated after 90 minutes of extensive cannula manipulation. This child survived a 5-day ECMO course and is alive at 22-month follow-up. In the second child, despite prolonged efforts at cannula repositioning, cannulation of the right atrium was not achieved. We did not offer central cannulation because of a rapidly deteriorating clinical course, with expiration in several hours. At autopsy, a dilated azygous vein was evident as a result of inferior vena cava compression by a malpositioned liver. The possibility of azygous vein cannulation may be increased in neonates with R-CDH and has not been previously reported. When evaluating infants with R-CDH for ECMO, clinicians must recognize the possibility of azygous cannulation and its potentially lethal consequences, and should anticipate alternative venous cannulation.

Central pancreatectomy with pancreaticogastrostomy in children.

Children requiring surgical intervention for pancreatic disease may be at risk long term for exocrine insufficiency and glucose intolerance. Pediatric surgeons must balance the need to perform adequate surgical resection while preserving as much normal pancreatic parenchyma as possible. Neoplasms of the middle pancreatic segment with low malignant potential and isolated trauma to the pancreatic body or neck represent 2 conditions where extensive pancreatic resection is unnecessary. Central pancreatectomy for such lesions is well described in adults. Reconstruction of the distal pancreatic remnant is traditionally performed via Roux-en-Y pancreaticojejunostomy. Pancreaticogastrostomy is an alternative approach that has been used to reconstruct the distal pancreas in the adults. Pancreaticogastrostomy offers several technical advantages over pancreaticojejunostomy. Because children may be uniquely susceptible to the long-term consequences of excessive pancreatic resection, 2 cases using this technique of central pancreatectomy with pancreaticogastrostomy are described.