Case report: giant cardiac malignancy in a nine-year-old female.

PURPOSE: To describe the management of a giant cardiac malignancy initially diagnosed as an anterior mediastinal mass. CLINICAL FEATURES: A nine-year-old female with right facial swelling and chronic cough was diagnosed with a large right mediastinal mass. Intermittent ventricular and supraventricular arrhythmias were noted on admission electrocardiograms. Empiric corticosteroid and radiation therapy did not reduce the size of the tumour, and initial tissue biopsies were non-diagnostic. Due to worsening tamponade physiology and persistent arrhythmias, the patient was scheduled for tumour debulking with potential resection. Prior to surgery, a multidisciplinary team was assembled to delineate team member responsibilities and treatment algorithms. The procedure was performed under general anesthesia with spontaneous ventilation preserved during endotracheal intubation and invasive line placement. The team was prepared to provide extracorporeal mechanical support if needed. The child required inotropic and vasoactive medications after transitioning to positive pressure ventilation, but her hemodynamics improved with sternotomy. The lesion was identified as a malignant cardiac clear-cell tumour that was unresectable. Her sternum was left open, as attempted closure led to the re-creation of tamponade physiology. She underwent delayed sternal closure days later. After months of chemotherapy that resulted in significant tumour involution, she underwent successful surgical resection. CONCLUSION: Giant primary cardiac tumours may present similarly to large anterior mediastinal masses. The care of patients with these lesions requires an understanding of the risks associated with mediastinal masses as well as those unique to cardiac tumours. A multidisciplinary approach is critical to providing safe and effective care throughout this process.

The effect of caudal vs intravenous morphine on early extubation and postoperative analgesic requirements for stage 2 and 3 single-ventricle palliation: a double blind randomized trial.

BACKGROUND: High-dose single-shot caudal morphine has been postulated to facilitate early extubation and to lower initial analgesic requirements after staged single-ventricle (SV) palliation. METHODS: With Institutional Review Board approval and written informed parental consent, 64 SV children aged 75-1667 days were randomized to pre-incisional caudal morphine-bupivacaine (100 µg·kg(-1) morphine (concentration 0.1%), mixed with 0.25% bupivacaine with 1 : 200,000 epinephrine, total 1 ml·kg(-1)) and postcardiopulmonary bypass (CPB) intravenous (IV) droperidol (75 µg·kg(-1)) ('active caudal group') or pre-incisional caudal saline (1 ml·kg(-1)) and post-CPB IV morphine (150 µg·kg(-1)) with droperidol (75 µg·kg(-1)) ('active IV group'). Assignment remained concealed from families and the care teams throughout the trial. Early extubation failure rates (primary or reintubation within 24 h), time to first postoperative rescue morphine analgesia, and 12-h postoperative morphine requirements were assessed for extubated patients. RESULTS: Thirty-one (12 stage 2) SV patients received caudal morphine and 32 (15 stage 2) received IV morphine. Extubation failure rates were 6/31 (19%) for caudal and 5/32 (16%) for IV morphine. For successfully extubated patients (n = 54), active caudal treatment significantly delayed the need for postoperative rescue morphine in stage 3 patients (P = 0.02) but not in stage 2 patients (P = 0.189) (Kaplan-Meier survival analysis with LogRank test). The reduction in 12-h postoperative morphine requirements with active caudal treatment did not reach significance (P = 0.085) but morphine requirements were significantly higher for stage 2 compared with stage 3 patients (P < 0.001) (two-way anova in n = 50 extubated patients). CONCLUSIONS: High-dose caudal morphine with bupivacaine delayed the need for rescue morphine analgesia in stage 3 patients. All stage 2 patients required early rescue morphine and had significantly higher postoperative 12-h morphine requirements than stage 3 patients. Early extubation is feasible for the majority of stage 2 and 3 SV patients regardless of analgesic regimen. The study was underpowered to assess differences in extubation failure rates.

Hypersensitivity reactions to aprotinin re-exposure in paediatric surgery.

OBJECTIVE: Hypersensitivity to aprotinin is low (1-3%) but more likely with re-exposure. The manufacturer issued a black box warning which lists aprotinin re-exposure within 1 year of prior exposure as a contraindication. We investigated the temporal relationship between re-exposure interval and hypersensitivity in children. METHODS: With Human Research Review Board approval, charts of all patients exposed to aprotinin during cardiac surgery were reviewed. We extracted data for re-exposure interval and hypersensitivity to skin tests, intravenous test dosing or infusion of the loading dose. We defined systemic hypersensitivity as haemodynamic instability, respiratory symptoms or diffuse skin reaction temporally related to exposure. RESULTS: From March 1994 to June 2007, there were a total of 2333 aprotinin exposures in 1824 patients. A total of 509 re-exposures occurred in 381 patients: 280 in 244 patients with early (within 1 year) re-exposure and 229 in 222 patients with late (after 1 year) re-exposure. Thirteen systemic hypersensitivity reactions occurred in the 509 re-exposures (2.6%): two during skin testing and 11 during the loading dose. Although the incidence of local hypersensitivity was increased with early re-exposure (6/280 or 2.1% vs 0/229, p=0.019), the incidence of the systemic reaction was not different between early and late re-exposures (6/280 or 2.1% (CI 0.8-4.6%) vs 7/229 or 3.1% (CI 1.2-6.2%), p=0.6). Six patients with a previous hypersensitivity reaction had an additional re-exposure to aprotinin; one of these patients had a systemic reaction during the third exposure. CONCLUSION: The incidence and type of hypersensitivity to aprotinin re-exposure in our cohort is consistent with previous reports. Repeat exposure within 1 year did not increase the risk of systemic hypersensitivity.

Near infrared spectroscopy monitoring during pediatric aortic coarctation repair.

BACKGROUND: Near infrared spectroscopy (NIRS) measures regional tissue oxygenation continuously and noninvasively and may allow assessment of changes in regional perfusion in real time. METHODS: We used NIRS monitoring to track real-time changes in regional oxygenation (rSO2) above and below the aortic cross-clamp in patients undergoing aortic coarctation repair and routinely stored these data in an operative electronic data base. This allowed us to analyze the changes in rSO2 during aortic coarctation repair for three pediatric age groups (neonates, infants <1 year, and children >1 year). Two site [cerebral (rSO2-C) and somatic thoracodorsal (rSO2-S)] rSO2 monitoring was performed in patients undergoing aortic coarctation repair. Data for rSO2 were analyzed across sites and age groups before, during and after cross-clamp. RESULTS: Twenty-six patients were available for analysis (11 neonates, 5 infants and 10 children). The regional oxygenation below the cross clamp (rSO2-S) declined significantly in all three age groups, but the decrease in neonates and infants <1 year of age was significantly greater than in the older children. CONCLUSIONS: Monitoring rSO2-S provides real-time trend information of regional oxygenation below the aortic cross-clamp. The decline in rSO2-S during aortic cross-clamp was rapid and large in most neonates and young infants <1 year which suggests impairment of regional perfusion presumably because of a lack of adequate collateral circulation to the monitored regional tissue. In contrast, the rSO2-S changed only to a minor degree in most infants and children >1 year, possibly because they had time to develop a more adequate collateral circulation around incomplete aortic obstruction.

Changes in cerebral and somatic oxygenation during stage 1 palliation of hypoplastic left heart syndrome using continuous regional cerebral perfusion.

OBJECTIVES: Stage 1 palliation of hypoplastic left heart syndrome requires the interruption of whole-body perfusion. Delayed reflow in the cerebral circulation secondary to prolonged elevation in vascular resistance occurs in neonates after deep hypothermic circulatory arrest. We examined relative changes in cerebral and somatic oxygenation with near-infrared spectroscopy while using a modified perfusion strategy that allowed continuous cerebral perfusion. METHODS: Nine neonates undergoing stage 1 palliation for hypoplastic left heart syndrome had regional tissue oxygenation continuously measured by frontal cerebral and thoraco-lumbar (T10-L2) somatic (renal) reflectance oximetry probes (rSO(2), INVOS; Somanetics, Troy, Mich). Surgery was accomplished using cardiopulmonary bypass with whole-body cooling (18 degrees C-20 degrees C) and regional cerebral perfusion through the innominate artery at flow rates guided by estimated minimum flow requirements and measured rSO(2) during reconstruction of the aortic arch. Data were logged at 1-minute intervals and analyzed using repeated measures analysis of variance. RESULTS: A total of 3176 minutes of data were analyzed. Prebypass cerebral rSO(2) was 65.4 +/- 8.9, and somatic rSO(2) was 58.9 +/- 12.4 (P <.001, cerebral vs somatic). During regional cerebral perfusion, cerebral rSO(2) was 80.7 +/- 8.6, and somatic rSO(2) was 41.4 +/- 7.1 (P <.001). Postbypass cerebral rSO(2) was 53.2 +/- 14.9, and somatic rSO(2) was 76.4 +/- 7.7 (P <.001). The risk of cerebral desaturation was significantly increased after cardiopulmonary bypass. CONCLUSIONS: Cerebral oxygenation was maintained during regional cerebral perfusion at prebypass levels with deep hypothermia. However, after rewarming and separation from cardiopulmonary bypass, cerebral oxygenation was lower compared with prebypass or somatic values. These results indicate that cerebrovascular resistance is increased after deep hypothermic cardiopulmonary bypass, even with continuous perfusion techniques, placing the cerebral circulation at risk postoperatively.

Risk reduction in pediatric procedural sedation by application of an American Academy of Pediatrics/American Society of Anesthesiologists process model.

OBJECTIVE: Guidelines for risk reduction during procedural sedation from the American Academy of Pediatrics (AAP) and the American Society of Anesthesiologists (ASA) rely on expert opinion and consensus. In this article, we tested the hypothesis that application of an AAP/ASA-structured model would reduce the risk of sedation-related adverse events. METHODS: Prospectively coded sedation records were abstracted by a hospital quality improvement specialist with practical and administrative experience in pediatric sedation. Process variables included notation of nulla per os (NPO) status, performance of a guided risk assessment, assignment of ASA physical status score, obtaining informed consent, generation of a sedation plan, and assessment of sedation level using a quantitative scoring system. Content variables included adherence to AAP NPO guidelines, ASA class, target sedation level, actual sedation level, age, procedure, and drugs used. Complication risk was assessed by logistic regression and Mantel-Haenszel odds ratios (OR). RESULTS: Complications were identified in 40 of 960 records (4.2%). The complication rate was 34 of 895 (3.8%) with planned conscious sedation and 6 of 65 (9.2%) with planned deep sedation ([DS]; OR: 2.6). Complications were reduced by performance of structured risk assessment (OR: 0.10), adherence to all process guidelines (OR: 0), and avoiding actual DS (OR: 0.4). The only drug associated with higher risk was chloral hydrate (OR: 2.1). Failure to adhere to NPO guidelines did not increase risk in this assessment; however, the adverse event rate was 0 if all process guidelines were followed. CONCLUSIONS: Presedation assessment reduces complications of DS. Repeated assessment of sedation score reduces the risk of inadvertent DS. The data provide direct evidence that AAP/ASA guidelines can reduce the risk of pediatric procedural sedation.