Rapunzel Syndrome Complicated by Escherichia coli Sepsis, Bowel Perforation, and Pancreatitis in an 11-year-old Malnourished Female.

The most common presenting symptoms of Rapunzel syndrome include abdominal pain (37%), nausea and vomiting (33.3%), obstruction (25.9%), and peritonitis (18.3%). Less commonly, patients may present with weight loss (7.4%) or intussusception (7.4%). Exceedingly rare complications of Rapunzel syndrome include gastric ulceration, obstructive jaundice, and acute pancreatitis as well as other malabsorptive-related complications including protein-losing enteropathy, iron deficiency, and megaloblastic anemia. This report details the case of an 11-year-old female with Rapunzel syndrome complicated by sepsis, a rare complication reported in only 2% of patients.

Early resuscitation and management of severe pediatric burns.

Pediatric burns are a leading cause of injury and mortality in children in the United States. Prompt resuscitation and management is vital to survival in severe pediatric burns. Although management principles are similar to their adult counterparts, children have unique pathophysiologic responses to burn injury thus an understanding of the differences in fluid resuscitation requirements, airway management, burn and wound care is essential to optimize their outcomes.

Does the incidence of thoracic aortic injury warrant the routine use of chest computed tomography in children?

BACKGROUND: Thoracic aortic injury is a potentially life-threatening injury associated with rapid deceleration mechanisms. Diagnosis is made by chest computed tomography (CT), which is associated with a risk of radiation-induced malignancy. We sought to determine the incidence of aortic injuries in the pediatric population to weigh against the risk of CT imaging. METHODS: The Pediatric Health Information Systems was queried for children ≤18 years with discharge diagnosis code of thoracic aortic injury (901.0) between December 2004 and 2014. Data abstracted included patient age, gender, diagnosis and procedure codes, and discharge disposition, where available. We also queried for imaging codes to determine what type of chest imaging the child received. RESULTS: Between December 2004 and 2014, 311,850 children were admitted to Pediatric Health Information Systems hospitals with traumatic injury. Of these patients, 46 (0.015%) were coded with a thoracic aortic injury and an accompanying E-code. Twenty-seven patients (58.7%) were male, and the median age was 13 years. The most common mechanism of injury was motor vehicle collision (63%, n = 29). Eighteen hospitals (41.9%) had no patients with a thoracic aortic injury in the 10-year period. In children with a thoracic aortic injury, the mortality rate was 11% (n = 5) and 22 (47.8%) underwent a chest CT during their hospitalization. Forty percent (124,909) of all trauma patients underwent chest CT, with a positive rate for aortic injury of 1.8/10,000. The reported estimated cancer risk from a chest CT scan is 25/10,000 for girls and 7.5/10, 000 in boys, greater than the positive CT rate. CONCLUSION: Thoracic aortic injuries are rare in children in the United States. The risk of cancer associated with screening chest CT is greater than the likelihood of identifying an aortic injury. Therefore, screening chest CT scans are unwarranted in injured children. LEVEL OF EVIDENCE: Therapeutic/Care Management, level IV.

Comparing Percutaneous to Open Access for Extracorporeal Membrane Oxygenation in Pediatric Respiratory Failure.

OBJECTIVES: Evaluate trends in method of access (percutaneous cannulation vs open cannulation) for pediatric extracorporeal membrane oxygenation and determine the effects of cannulation method on morbidity and mortality. DESIGN: Retrospective cohort study. SETTING AND SUBJECTS: The Extracorporeal Life Support Organization's registry was queried for pediatric patients on extracorporeal membrane oxygenation for respiratory failure from 2007 to 2015. INVERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 3,501 patients identified, 77.2% underwent open cannulation, with the frequency of open cannulation decreasing over the study period from approximately 80% to 70% (p < 0.001). Percutaneous cannulation patients were more commonly male (24.2% vs 21.5%; p = 0.01), older (average 7.6 vs 4.5 yr; p < 0.001), and heavier (average 33.0 vs 20.2 kg; p < 0.001). Subset analysis of patients on venovenous extracorporeal membrane oxygenation revealed higher rates of mechanical complications due to blood clots (28.9% vs 22.6%; p = 0.003) or cannula problems (18.9% vs 12.7%; p < 0.001), cannula site bleeding (25.3% vs 20.2%; p = 0.01) and increased rates of cannula site repair in the open cannulation cohort. Limb related complications were not significantly different on subset analysis for venovenous extracorporeal membrane oxygenation patients stratified by access site. Logistic regression analysis revealed that method of access was not associated with a difference in mortality. CONCLUSIONS: The proportion of pediatric patients undergoing percutaneous extracorporeal membrane oxygenation cannulation is increasing. Mechanical and physiologic complications occur with both methods of cannulation, but percutaneous cannulation appears safe in this cohort. Further analysis is needed to evaluate long-term outcomes with this technique.

Controversies in extracorporeal membrane oxygenation (ECMO) utilization and congenital diaphragmatic hernia (CDH) repair using a Delphi approach: from the American Pediatric Surgical Association Critical Care Committee (APSA-CCC).

PURPOSE: Review current practices and expert opinions on contraindications to extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH) and contraindications to repair of CDH following initiation of ECMO. METHODS: Modified Delphi method was employed to achieve consensus among members of the American Pediatric Surgical Association Critical Care Committee (APSA-CCC). RESULTS: Overall response rate was 81% including current and former members of the APSA-CCC. An average of 5-15 CDH repairs were reported annually per institution; 26-50% of patients required ECMO. 100% of respondents would not offer ECMO to a patient with a complex or unrepairable cardiac defects or lethal chromosomal abnormality; 94.1% would not in the setting of severe intracranial hemorrhage (ICH). 76.5% and 72.2% of respondents would not offer CDH repair to patients on ECMO with grade III-IV ICH or new diagnosis of lethal genetic or metabolic abnormalities, respectively. There was significant variability in whether or not to repair CDH if unable to wean from ECMO at 4-5 weeks. CONCLUSIONS: Significant variability in practice pattern and opinions exist regarding contraindications to ECMO and when to offer repair of CDH for patients on ECMO. Ongoing work to evaluate outcomes is needed to standardize management and minimize potentially futile interventions. LEVEL OF EVIDENCE: V (expert opinion).

Nutrition Delivery During Pediatric Extracorporeal Membrane Oxygenation Therapy.

BACKGROUND: Macronutrient delivery during pediatric ECMO therapy can be challenging. We examined predictors of nutrient delivery in the first 2 weeks of extracorporeal membrane oxygenation (ECMO) therapy in the pediatric intensive care unit (ICU). METHODS: Details of macronutrient delivery were recorded in children (newborn-18 years of age) who survived 24 hours after cannulation to ECMO over a 3-year period (2012-2015). RESULTS: We analyzed data from 54 consecutive eligible patients, 43% female, with median (interquartile range) ECMO duration of 8.5 (6-24) days, age 0.1 (0, 16) months, ICU length of stay 32 (21, 60) days, and 28-day mortality 13%. Median weight for age z score declined from -0.1 at admission to -1.2 at 30 days (P = 0.013). At least 80% goal energy and protein was delivered in 35 (65%) and 33 (61%) patients, respectively, by day 7; 10% of energy and 11% protein goal was delivered enterally. Parenteral nutrition (PN) was utilized in 47 (87%) patients, initiated by day 1 (1, 3). Enteral nutrition (EN) was successfully delivered in 49 (94%) patients (35% postpyloric), initiated by day 6 (2, 16). Younger age (P = 0.01) and venoarterial mode of ECMO (P = 0.0014) were associated with lower EN delivery. Use of umbilical artery catheters or vasoactive infusions did not impede EN delivery. Late PN delivery was associated with cumulative protein deficits (P = 0.019) and failure to achieve nutrient delivery goals by day 7. CONCLUSIONS: Optimal nutrient delivery was achieved in most patients by day 7, predominantly via PN. Early EN is feasible in low volumes, but PN may be essential to prevent cumulative energy and protein deficits during the first week of ECMO.

Contrast enhanced ultrasound for the evaluation of blunt pediatric abdominal trauma.

INTRODUCTION: Blunt abdominal trauma is a common problem in children. Computed tomography (CT) is the gold standard for imaging in pediatric blunt abdominal trauma, however up to 50% of CTs are normal and CT carries a risk of radiation-induced cancer. Contrast enhanced ultrasound (CEUS) may allow accurate detection of abdominal organ injuries while eliminating exposure to ionizing radiation. METHODS: Children aged 7-18years with a CT-diagnosed abdominal solid organ injury underwent grayscale/power Doppler ultrasound (conventional US) and CEUS within 48h of injury. Two blinded radiologists underwent a brief training in CEUS and then interpreted the CEUS images without patient interaction. Conventional US and CEUS images were compared to CT for the presence of injury and, if present, the injury grade. Patients were monitored for contrast-related adverse reactions. RESULTS: Twenty one injured organs were identified by CT in eighteen children. Conventional US identified the injuries with a sensitivity of 45.2%, which increased to 85.7% using CEUS. The specificity of conventional US was 96.4% and increased to 98.6% using CEUS. The positive predictive value increased from 79.2% to 94.7% and the negative predictive value from 85.3% to 95.8%. Two patients had injuries that were missed by both radiologists on CEUS. In a 100kg, 17year old female, a grade III liver injury was not seen by either radiologist on CEUS. Her accompanying grade I kidney injury was not seen by one of the radiologist on CEUS. The second patient, a 16year old female, had a grade III splenic injury that was missed by both radiologists on CEUS. She also had an adjacent grade II kidney injury that was seen by both. Injuries, when noted, were graded within 1 grade of CT 33/35 times with CEUS. There were no adverse reactions to the contrast. CONCLUSION: CEUS is a promising imaging modality that can detect most abdominal solid organ injuries in children while eliminating exposure to ionizing radiation. A multicenter trial is warranted before widespread use can be recommended. LEVEL OF EVIDENCE: Level II; Diagnostic Prospective Study.

Can we safely decrease intensive care unit admissions for children with high grade isolated solid organ injuries? Using the shock index, pediatric age-adjusted and hematocrit to modify APSA admission guidelines.

BACKGROUND: In 2000, the American Pediatric Surgical Association (APSA) disseminated consensus practice guidelines for the management of blunt liver and splenic injury which included intensive care unit (ICU) admission for children with grade IV injuries. We sought to determine if we could better predict which children with isolated solid organ injuries (SOI) underwent an ICU-level intervention, thus necessitating ICU admission. METHODS: Children with isolated liver, spleen, or kidney injuries admitted to the ICU from November 2003 to August 2015 were identified in our trauma registry, and data were extracted from the medical record. ICU-level interventions were defined as transfusion, vasopressor use, intubation, and operative/procedural intervention. Shock index and pediatric age-adjusted (SIPA) was calculated for all patients. The sensitivity and negative predictive values (NPV) were determined. RESULTS: 133 children met inclusion criteria. 19 (14.3%) required ICU-level intervention, and 114 (85.1%) did not. 95% (n=18) of the intervention group had either an elevated SIPA or a hematocrit <30% on admission compared to 22% (n=25) of patients in the no intervention group. Sensitivity was 95%, and NPV was 99%. CONCLUSIONS: Limiting ICU admission in children with isolated SOI to those with an elevated SIPA or hematocrit <30% would reduce the ICU admission rate by two-thirds while maintaining patient safety. TYPE OF STUDY: Diagnostic study. LEVEL OF EVIDENCE: III.

Head and Cervical Spine Evaluation for the Pediatric Surgeon.

This article is designed to guide pediatric surgeons in the evaluation and stabilization of blunt head and cervical spine injuries in pediatric patients. Trauma remains the number one cause of morbidity and mortality among children, and the incidence of head injuries continues to rise. Cervical spine injuries, on the other hand, are unusual but can be devastating if missed. This article highlights the pathophysiology unique to pediatric head and cervical spine trauma as well as keys to clinical and diagnostic evaluation.

The sensitivity and negative predictive value of a pediatric cervical spine clearance algorithm that minimizes computerized tomography.

BACKGROUND: It is crucial to identify cervical spine injuries while minimizing ionizing radiation. This study analyzes the sensitivity and negative predictive value of a pediatric cervical spine clearance algorithm. METHODS: We performed a retrospective review of all children <21years old who were admitted following blunt trauma and underwent cervical spine clearance utilizing our institution's cervical spine clearance algorithm over a 10-year period. Age, gender, International Classification of Diseases 9th Edition diagnosis codes, presence or absence of cervical collar on arrival, Injury Severity Score, and type of cervical spine imaging obtained were extracted from the trauma registry and electronic medical record. Descriptive statistics were used and the sensitivity and negative predictive value of the algorithm were calculated. RESULTS: Approximately 125,000 children were evaluated in the Emergency Department and 11,331 were admitted. Of the admitted children, 1023 patients arrived in a cervical collar without advanced cervical spine imaging and were evaluated using the cervical spine clearance algorithm. Algorithm sensitivity was 94.4% and the negative predictive value was 99.9%. There was one missed injury, a spinous process tip fracture in a teenager maintained in a collar. CONCLUSIONS: Our algorithm was associated with a low missed injury rate and low CT utilization rate, even in children <3years old. LEVEL OF EVIDENCE: IV.

The lost art of the splenorrhaphy.

BACKGROUND: In the case of the hemodynamically unstable child, splenorrhaphy is preferred to splenectomy to avert postsplenectomy sepsis. However, successful splenorrhaphy requires familiarity with the procedure. We sought to determine how many splenectomies or splenorrhaphies for trauma the average pediatric surgeon can be expected to perform during their career. METHODS: The Pediatric Health Information System (PHIS) Database was queried for patients ≤18years coded with an International Classification of Diseases 9th Edition diagnosis code of a splenic injury from 2004 to 2013. Age, gender, grade of splenic injury, and operations performed were extracted. Numbers of pediatric surgeons per hospital were obtained. RESULTS: 9567 children were identified. 2.1% underwent a splenectomy and 0.8% underwent a splenorrhaphy. The average surgeon performed 0.6 (SD=0.6) splenectomies and 0.2 (SD=0.4) splenorrhaphies for trauma. If these rates remain constant over time, the average surgeon would perform 1.8 (SD =1.7) splenectomies and 0.6 (SD =1.1) splenorrhaphies for trauma over a 30-year surgical career. CONCLUSION: Nonoperative management is associated with a host of benefits, but has resulted in a decrease in the experience level of the pediatric surgeons expected to perform an emergency splenectomy or splenorrhaphy when the unusual occasion arises.