Parents Demand and Teenager Refuses Epidural Anesthesia.

A 15-year-old girl is scheduled to undergo an upper lobectomy to debulk metastatic Ewing sarcoma. The anesthesiologist recommended placement of a thoracic epidural catheter to provide postoperative analgesia. The patient did not want a needle to be placed near her spine. She was terrified that the procedure would be painful and that it might paralyze her. Although the anesthesiologist reassured her that sedation and local anesthesia would make the procedure comfortable, she remained vehemently opposed to the epidural procedure. The parents spoke privately to the anesthesiologist and asked for placement of the epidural after she was asleep. They firmly believed that this would provide optimal postoperative analgesia and thus would be in her best interest. Experts discuss the pros and cons of siding with the patient or parents.

Legal and Ethical Considerations for Requiring Consent for Apnea Testing in Brain Death Determination.

The past decade has witnessed escalating legal and ethical challenges to the diagnosis of death by neurologic criteria (DNC). The legal tactic of demanding consent for the apnea test, if successful, can halt the DNC. However, US law is currently unsettled and inconsistent in this matter. Consent has been required in several trial cases in Montana and Kansas but not in Virginia and Nevada. In this paper, we analyze and evaluate the legal and ethical bases for requiring consent before apnea testing and defend such a requirement by appealing to ethical and legal principles of informed consent and battery and the right to refuse medical treatment. We conclude by considering and rebutting two major objections to a consent requirement for apnea testing: (1) a justice-based objection to allocate scarce resources fairly and (2) a social utility objection that halting the diagnosis of brain death will reduce the number of organ donors.

Blood pressure in adults with short stature skeletal dysplasias.

Hypertension, compounded by obesity, contributes to cardiovascular disease and mortality. Data describing hypertension prevalence in adults with short stature skeletal dysplasias are lacking, perhaps due to poor fit of typical adult blood pressure cuffs on rhizomelic or contracted upper extremities. Through health screening research, blood pressure was measured in short stature adults attending support group meetings and skeletal dysplasia clinics. Blood pressure was measured with a commercially available, narrower adult cuff on the upper and/or lower segment of the arm. Height, weight, age, gender, diagnosis, exercise, and medications were collected. Subjects were classified as normotensive, prehypertensive, or hypertensive for group analysis; no individual clinical diagnoses were made. In 403 short stature adults, 42% were hypertensive (systolic >140, diastolic >90 OR taking antihypertensive medications). For every BMI unit and 1 kg weight increase in males, there was a 9% and an 8% increase, respectively, in the odds of hypertension versus normotension. In females, the increase was 10% and 6%, respectively. In those with achondroplasia, the most common short stature dysplasia, males (n = 106) had 10% greater odds of hypertension versus normotension for every BMI unit and kilogram increase. In females with achondroplasia (n = 128), the odds of hypertension versus normotension was 8% greater for each BMI unit and 7% for each additional kilogram. These data suggest a high population prevalence of hypertension among short stature adults. Blood pressure must be monitored as part of routine medical care, and measuring at the forearm may be the only viable clinical option in rhizomelic short stature adults with elbow contractures.

Susceptibility weighted imaging in infants with staged embolization of vein of Galen aneurysmal malformations.

BACKGROUND AND PURPOSE: The vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation with a higher morbidity and mortality, especially in neonates. Ultrasound, CT and MR are usually used in diagnosis and treatment monitoring of these disorders. In this current study, we aim to examine utility of SWI in evaluation of treatment response in infants with VGAM. MATERIALS AND METHODS: We performed a retrospective chart analysis of children with VGAM in our institution between January 2008 and December 2016. Inclusion criteria included; confirmed VGAM on DSA; available SWI sequence at baseline and at follow up after at least a single embolization session; age at initial MR of 18 years or younger. Signal intensity and Angioarchitecture of VGAM and cerebral veins on SWI, as well as hydrocephalus and clinical outcome were evaluated. RESULTS: Of 11 patients identified with VGAM in our institution, 5 children (3 males and 2 females) satisfied the inclusion criteria. The average age at initial MR was 29 days (range 1-120). Fourteen MRI were available for review. All children had VGAM of mural type. Intramedullary veins were dilated and SWI-hypointense in all children, while subependymal and sulcal veins were dilated and SWI-hypointense in 4 patients on initial MRI. On the first available follow up MRI, cerebral veins have mostly normalized in 4 children and remained mostly dilated and SWI-hypointense in 1 child; even after complete treatment of the VGAM. CONCLUSION: Our preliminary findings show that SWI seems to offer a beneficial non-invasive tool in evaluating passive venous congestion patterns in pediatric patients with VGAM. It remains to be determined in larger studies, the clinical significance of these SWI changes.

Membrane Oxygenator Use With Biventricular Assist Device: Facilitation of Support and Lung Recovery.

We present the use of a low-resistance membrane oxygenator (Quadrox D, Maquet) in series with a pulsatile right ventricular assist device (Berlin Heart EXCOR, Berlin Heart) in a patient with biventricular support who required high-frequency oscillatory ventilation (HFOV), due to refractory acute respiratory distress syndrome associated with Cytomegalovirus pneumonia. The high mean airway pressure associated with the use of HFOV resulted in a significant negative impact on left ventricular assist device (LVAD) filling that led to a combined respiratory and metabolic acidosis and the need for vasopressor support. Oxygenator placement enabled transition to conventional ventilation and the discontinuation of vasopressor support. This case demonstrates the feasibility and safety of the use of this lung support system in patients requiring ventricular assist device (VAD) support.

PICU Up!: Impact of a Quality Improvement Intervention to Promote Early Mobilization in Critically Ill Children.

OBJECTIVE: To determine the safety and feasibility of an early mobilization program in a PICU. DESIGN: Observational, pre-post design. SETTING: PICU in a tertiary academic hospital in the United States. PATIENTS: Critically ill pediatric patients admitted to the PICU. INTERVENTION: This quality improvement project involved a usual-care baseline phase, followed by a quality improvement phase that implemented a multicomponent, interdisciplinary, and tiered activity plan to promote early mobilization of critically ill children. MEASUREMENTS AND MAIN RESULTS: Data were collected and analyzed from July to August 2014 (preimplementation phase) and July to August 2015 (postimplementation). The study sample included 200 children 1 day through 17 years old who were admitted to the PICU and had a length of stay of at least 3 days. PICU Up! implementation led to an increase in occupational therapy consultations (44% vs 59%; p = 0.034) and physical therapy consultations (54% vs 66%; p = 0.08) by PICU day 3. The median number of mobilizations per patient by PICU day 3 increased from 3 to 6 (p < 0.001). More children engaged in mobilization activities after the PICU Up! intervention by PICU day 3, including active bed positioning (p < 0.001), and ambulation (p = 0.04). No adverse events occurred as a result of early mobilization activities. The most commonly reported barriers to early mobilization after PICU Up! implementation was availability of appropriate equipment. The program was positively received by PICU staff. CONCLUSIONS: Implementation of a structured and stratified early mobilization program in the PICU was feasible and resulted in no adverse events. PICU Up! increased physical therapy and occupational therapy involvement in the children's care and increased early mobilization activities, including ambulation. A bundled intervention to create a healing environment in the PICU with structured activity may have benefits for short- and long-term outcomes of critically ill children.

Resistive Index Variability in Anterior Cerebral Artery Measurements During Daily Transcranial Duplex Sonography: A Predictor of Cerebrovascular Complications in Infants Undergoing Extracorporeal Membrane Oxygenation?

OBJECTIVES: The purpose of this study was to determine the value of resistive index (RI) variability in predicting cerebrovascular complications during extracorporeal membrane oxygenation (ECMO). METHODS: This retrospective study included 36 infants treated by ECMO. The RI was measured on daily transfontanellar duplex sonography, obtained first without fontanel compression and then after gentle compression with the transducer. The age at ECMO cannulation, sex, gestational age at birth, method of delivery, indication, and type and duration of ECMO were recorded. RESULTS: There was a statistically significant difference in RI variability in infants who developed cerebrovascular complications as opposed to those who did not (P = .002). Resistive index variability of 10% or greater on any day was associated with an increased risk for cerebrovascular complications (P = .0482; χ2 = 3.9). Variability in the first 5 days was significantly higher than on following days (P < .0001). The age at ECMO cannulation showed a significant difference, with mean ± SD values of 1.1 ± 0.9 days in the complications group and 2.7 ± 2.2 days in the no-complications group (P = .043). CONCLUSIONS: Resistive index variability of 10% or greater on any day had a statistically significant risk of cerebrovascular complication development. Extracorporeal membrane oxygenation cannulation at younger than 3 days conferred an increased risk of cerebrovascular complications.

The heat is on... thermal epiglottitis as a late presentation of airway steam injury.

BACKGROUND: Thermal epiglottitis is a rare but potentially life-threatening disease. Diagnosis requires a thorough history and high clinical level of suspicion, particularly in children. Thermal epiglottitis from steam inhalation can have a slow onset without oropharyngeal signs of thermal injury, findings that can hide the clinical diagnosis. OBJECTIVE: Our aim was to review the pathophysiology and clinical presentation of thermal epiglottitis and the challenges involved in diagnosis and management of this form of atypical epiglottitis. CASE REPORT: We describe the case of a 22-month-old male presenting to the pediatric emergency department after a scald burn from steam and boiling water resulting in 12% body surface area burns to his chin, chest, and shoulder, with no obvious oropharyngeal or neck injuries. At the time of presentation, he was afebrile and well appearing. Six hours after the injury, he was sitting in the "tripod position," drooling, with pooled saliva in his mouth and inspiratory stridor. Intubation in the operating room using conventional direct laryngoscopy was not successful and he was intubated using an operative endoscope. Laryngoscopy demonstrated thermal epiglottitis. A tracheostomy was performed to secure the airway, and he was admitted to the pediatric intensive care unit. He was discharged home and decannulated 4 weeks later, when airway endoscopy showed complete recovery with normal airway structures. CONCLUSION: A thorough history and physical examination together with a high level of suspicion and aggressive, collaborative airway management is vital in preventing catastrophic airway obstruction in atypical forms of epiglottitis.

Variability in anticoagulation management of patients on extracorporeal membrane oxygenation: an international survey.

OBJECTIVE: The objective of this study was to determine current practices of anticoagulation in patients on extracorporeal membrane oxygenation. DESIGN: Internet-based cross-sectional survey distributed between November 2010 and May 2011. SETTING: Extracorporeal Life Support Organization-registered extracorporeal membrane oxygenation centers internationally. PARTICIPANTS: : Extracorporeal membrane oxy genation medical directors and coordinators. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 121 responses from extracorporeal membrane oxy genation medical directors and coordinators at 187 Extracorporeal Life Support Organization centers with access to the survey. Eighty-four of 117 (72%) respondents reported having a written institutional extracorporeal membrane oxy genation protocol for both anticoagulation and blood product management at their institutions. Sixty-nine of 117 (59%) respondents reported use of tip-to-tip or partially heparin-bonded circuits. Unfractionated heparin was used at all centers; only 8% of respondents indicated use of alternative anticoagulation medications in the six months prior to the survey. The preferred method of anticoagulation monitoring was the serial measurement of activated clotting time, as reported by 97% of respondents. In this survey, 82% of respondents reported antithrombin III testing, 65% reported anti-factor Xa testing, and 43% reported use of thromboelastography during extracorporeal membrane oxy genation. Goal ranges for these three tests and interventions triggered by out-of-range values were found to be variable. CONCLUSIONS: Extracorporeal membrane oxy genation anticoagulation management policies vary widely by center. The majority of extracorporeal membrane oxy genation programs employ activated clotting time as the preferred anticoagulation monitoring tool. The coagulation system is also monitored using more specific markers such as antithrombin III, anti-factor Xa, and thromboelastography by a large number of centers. Future research is needed to elucidate optimal anticoagulation management and improve outcomes.

Food and Drug Administration approval for medications used in the pediatric intensive care unit: a continuing conundrum.

OBJECTIVES: Many drugs used in the pediatric intensive care unit are administered "off label," i.e., they have been neither thoroughly tested for efficacy and safety nor approved for use in children. The U.S. Congress has enacted legislation to promote standards and requirements for Food and Drug Administration labeling for drugs used in pediatrics. Nevertheless, we hypothesized that most medications used in our pediatric intensive care unit were not Food and Drug Administration approved for use in pediatric patients. DESIGN: A list of medications dispensed in the pediatric intensive care unit from January through February 2008 was obtained from our pharmacy database. We then determined whether each medication had been granted Food and Drug Administration approval for use in children. Medications were divided into the following categories: not approved for use in any pediatric age group, approved for use in limited age groups only, and approved for use in all pediatric age groups. SETTING: A pediatric intensive care unit at a tertiary care hospital with 26 beds and 1,500 admissions per year. MEASUREMENTS AND MAIN RESULTS: In the 2-month period, 248 different medications were dispensed with a total of 49,707 medication orders. Sixty (24.2%) of the medications dispensed were not Food and Drug Administration approved for use in any pediatric age group, 106 (42.7%) were approved for use in limited age groups, and 82 (33%) were approved for use in all pediatric age groups. Eleven of the 25 most frequently dispensed medications were approved for use in limited age groups, but none of them was used for the indication or age group for which they were approved. CONCLUSIONS: Despite the efforts of Congress, 67% of medications prescribed and administered in the pediatric intensive care unit did not have Food and Drug Administration approval or had only limited approval, underscoring the need for the medical community to demand oversight and research to improve drug labeling for our patient population.

Unrecognized burden of methicillin-resistant Staphylococcus aureus and vancomycin-resistant Enterococcus carriage in the pediatric intensive care unit.

Routinely, children's hospitals use data from clinical cultures to estimate the burden of methicillin-resistant Staphylococcus aureus (MRSA) and vancomycin-resistant Enterococcus (VRE) carriage. In our pediatric intensive care unit, a program of weekly surveillance cultures uncovered an unrecognized reservoir of MRSA and VRE carriers. This weekly surveillance enabled more accurate estimates of the incidence rates of MRSA and VRE carriage and led to an increased number of isolation-days for patients.

Reduction of catheter-associated bloodstream infections in pediatric patients: experimentation and reality.

OBJECTIVE: Few data exist on successes at reducing pediatric catheter-associated bloodstream infections (CA-BSI). The objective was to eradicate CA-BSI with a multifaceted pediatric-relevant intervention proven effective in adult patients. DESIGN: Prospective cohort of pediatric intensive care (PICU) patients with historical controls. SETTING: Multidisciplinary PICU. PATIENTS/PARTICIPANTS: PICU patients with intervention targeting PICU providers. INTERVENTIONS: Multifaceted intervention involving preintervention staff surveys, provider educational program, creation of central catheter procedure cart, guideline-supported central catheter insertion checklist, nursing staff empowerment to stop procedures that breached guidelines, and real-time data feedback to PICU leadership. MEASUREMENTS AND MAIN RESULTS: We measured rate of CA-BSI per 1000 catheter days from August 2001 through September 2006. Reliable use of evidence-based best practices for insertion of central catheters in our PICU was associated with a statistically and clinically significant decrease in our CA-BSI rate for 24 months postintervention (p < .05). During a portion of this postintervention period, we experienced a dramatic increase in our CA-BSI rate that was ultimately found to be due to the introduction of a new positive displacement mechanical valve intravenous port in April 2004. After removal of this positive displacement mechanical valve, our CA-BSI rate dropped from 5.2 +/- 4.5 CA-BSI per 1000 central catheter days to a rate of 3.0 +/- 1.9 CA-BSI per 1000 central catheter days. Chart review of postintervention CA-BSI cases revealed that these patients acquired CA-BSI weeks after both PICU admission and after insertion of the most recent central catheter. CONCLUSIONS: Our data show that improving practices for insertion of central catheters leads to a reduction of CA-BSI among pediatric patients but not elimination of CA-BSI. More research is needed to identify best practices for maintenance of central catheters for children. In addition, our experience shows that even despite good interventions to control CA-BSI, institutions must remain vigilant to factors such as new technology with apparent advantages but short track records of use.

Do not attempt resuscitation orders in pediatrics.

Do-not-attempt resuscitation orders are becoming more common in pediatrics, particularly as programs for hospice and palliative care in children develop. Concomitantly, there arises the need to decide when it is appropriate to use these technologies. It is at this point that the skills of relationship building, listening, and empathic concern become indispensable.

Lung isolation in a child with unilateral necrotizing Clostridium perfringens pneumonia.

OBJECTIVE: To describe lung isolation and the selective application of continuous positive airway pressure using an endobronchial blocker in a patient with sickle cell disease and unilateral necrotizing Clostridium perfringens pneumonia. DESIGN: Case report. SETTING: Pediatric intensive care unit. PATIENT: A 12-yr-old male with sickle cell disease developed persistent necrotizing pneumonia of the left lung following exchange transfusion for acute chest syndrome and hyper-hemolytic syndrome. INTERVENTIONS: An endobronchial blocker was placed into the left main stem bronchus for lung isolation and application of continuous positive airway pressure to the left lung for 48 hrs. MEASUREMENTS AND MAIN RESULTS: After 14 days of persistent atelectasis of the left lung despite thorascopic decortication and multiple bronchoscopies, our patient had substantial lung aeration within 48 hrs of continuous positive airway pressure applied via the endobronchial blocker. Lung resection was avoided and the patient was successfully extubated 2 days after removal of the blocker. CONCLUSIONS: This case report demonstrates a therapeutic application of prolonged lung isolation and differential ventilation in a patient with an airway too small for commercially available double-lumen endotracheal tubes. The apparent success of this intervention suggests the feasibility of selective ventilation in pediatric patients and highlights a novel application of the bronchial blocker.

The physician's role in discussing organ donation with families.

Federal Conditions of Participation from the Health Care Financing Administration (now the Centers for Medicare and Medicaid Services) introduced in 1998 require that all families be presented the option of organ and tissue donation when death is imminent. The perception that physicians were being excluded from participating in this process led to a resolution at the American Medical Association House of Delegates meeting in December 1999, calling on the American Medical Association Council on Scientific Affairs to review the Conditions of Participation "to ensure that there is no prohibition of physician involvement in the organ donation process..." The number of organs procured for transplantation in the United States is insufficient to meet needs. Families' hospital experiences significantly affect their decisions to donate organs. Discussing severe brain injury, brain death, and organ donation after brain death with families is a specialized form of end-of-life decision-making and care in the intensive care unit; however, the knowledge, skills, and attitudes necessary for physicians and nurses to promote good end-of-life decision-making are widely variable. The federal Conditions of Participation require that those making requests of families for organ donation receive specific training. They do not prohibit physician involvement in initiating organ donation requests, provided these individuals are properly trained. Physicians have an important role in caring for patients and families in these circumstances, and the care they provide is enhanced through training, attention to the special issues involved, and collaboration with organ procurement organization personnel.