Surgical repair of massive hemorrhage secondary to button battery ingestion causing aortoesophageal fistula.

BACKGROUND: In pediatrics, foreign body ingestion poses unique challenges. Each case is unique given variability in timing, type, and size of object, compounded by underlying comorbidities and age. In the mid-1990s, mortality and morbidity associated with button battery (BB) ingestion (BBI) emerged corresponding to modification in battery fabrication towards higher voltage, large-diameter lithium cells. AIMS: To describe the case and management of a BBI in a pediatric patient necessitating the use of cardiopulmonary bypass and deep hypothermic circulatory arrest (DHCA). MATERIALS AND METHODS: A 17-month-old female presented with the sudden loss of consciousness at home. Chest X-ray revealed an esophageal foreign body suspicious for BBI. A massive upper gastrointestinal bleeding was temporized with packing. The patient was urgently taken to the operating room for sternotomy, establishment of cardiopulmonary bypass (CPB) with deep hypothermic circulatory arrest (DHCA), removal of BB, repair of the left common carotid artery, esophageal, and tracheal injuries. DISCUSSION: Successful management of BBI requires coordinated care and a multidisciplinary approach. A high degree of clinical suspicion for BBI is imperative to facilitate early aggressive interventions. Lateral and anteroposterior chest films should be obtained in any suspected BBI. CONCLUSION: This case demonstrates the utility of CPB and DHCA where control of bleeding secondary to BBI is not otherwise possible.

Pharyngeal dysfunction associated with early and late onset sleep disordered breathing in children.

OBJECTIVE: To compare the frequency and type of diagnoses associated with pharyngeal dysfunction (PD) in children presenting with early versus late onset sleep disordered breathing (SDB). METHODS: This was a retrospective, cross-sectional study. A consecutive series of children ≤3 years old who underwent management for SDB were retrospectively identified from a prospectively kept surgical database. The early onset group was compared with two separate late onset (≥4years old) groups. Diagnoses associated with PD included gastroesophageal reflux disease (GERD), swallowing dysfunction, prematurity, asthma, and obesity. Distribution of PD diagnoses, airway lesions, syndromic conditions, pulse oximetry scores, and endoscopic pattern of airway obstruction were compared. RESULTS: 73 patients with early onset SDB were identified (51 boys, mean age 2.25 ±â€¯0.64 years, range 1.75-3 years) and compared with two groups of later onset SDB consisting of 75 and 72 patients with mean ages of 7.58 ±â€¯2.40 years and 8.04 ±â€¯3.34 years respectively (range 4-16 years). The early onset SDB group had a higher prevalence of PD diagnoses compared to the later onset group with 35 of 73 patients being diagnosed compared to 41 of 147 children (p = 0.01). Early onset SDB patients were more likely to have GERD or swallowing dysfunction (p < 0.01) while later onset patients more commonly presented with associated asthma or obesity (p < 0.01). There was no statistically significant difference in airway lesions between groups. CONCLUSION: Early-onset SDB is associated with conditions causing PD more often than later-onset SDB. Identifying these conditions and optimizing their management may impact outcomes in treating pediatric SDB.

Fluid Management Practices After Surgery for Congenital Heart Disease: A Worldwide Survey.

OBJECTIVES: To determine common practice for fluid management after cardiac surgery for congenital heart disease among pediatric cardiac intensivists. DESIGN: A survey consisting of 17 questions about fluid management practices after pediatric cardiac surgery. Distribution was done by email, social media, World Federation of Pediatric Intensive and Critical Care Societies website, and World Federation of Pediatric Intensive and Critical Care Societies newsletter using the electronic survey distribution and collection system Research Electronic Data Capture. SETTING: PICUs around the world. SUBJECTS: Pediatric intensivists managing children after surgery for congenital heart disease. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One-hundred eight responses from 18 countries and six continents were received. The most common prescribed fluids for IV maintenance are isotonic solutions, mainly NaCl 0.9% (42%); followed by hypotonic fluids (33%) and balanced crystalloids solutions (14%). The majority of the respondents limit total fluid intake to 50% during the first 24 hours after cardiac surgery. The most frequently used fluid as first choice for resuscitation is NaCl 0.9% (44%), the second most frequent choice are colloids (27%). Furthermore, 64% of respondents switch to a second fluid for ongoing resuscitation, 76% of these choose a colloid. Albumin 5% is the most commonly used colloid (61%). Almost all respondents (96%) agree there is a need for research on this topic. CONCLUSIONS: Our survey demonstrates great variation in fluid management practices, not only for maintenance fluids but also for volume resuscitation. Despite the lack of evidence, colloids are frequently administered. The results highlight the need for further research and evidence-based guidelines on this topic.

Pediatric extracorporeal membrane oxygenation (ECMO): a guide for radiologists.

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment for pediatric patients with respiratory and/or cardiac failure. The ECMO circuit oxygenates and sometimes pumps the blood, effectively replacing lung and/or heart function temporarily. ECMO patients are clinically very complex not only because of their underlying, life-threatening pathology, but also because of the many physiological parameters that must be monitored and adjusted to maintain adequate tissue perfusion and oxygenation. Drainage and reinfusion cannulae connecting the patient to the ECMO circuit are visible on radiograph. These cannulae have different functions, different configurations, different radiographic appearances, and different positions that should be familiar to the interpreting pediatric radiologist. The primary complications of ECMO include hemorrhage, thrombosis and ischemia, as well as equipment failure and cannula malpositioning, all of which may be detected on imaging. In this pictorial essay, we discuss the basics of ECMO function and clinical management, ECMO cannula features and configurations, and the many complications of ECMO from an imaging perspective. Our goal is to educate pediatric radiologists about ECMO imaging, equipping them to properly interpret these studies and to become a useful consultant in ECMO patient care.

Albumin 5% Versus Crystalloids for Fluid Resuscitation in Children After Cardiac Surgery.

OBJECTIVES: To determine the clinical benefit of using colloids versus crystalloids for volume resuscitation in children admitted after cardiac surgery. DESIGN: Retrospective pre-/postintervention cohort study. SETTING: Stollery Children's Hospital tertiary care pediatric cardiac ICU. PATIENTS: Children admitted to the pediatric cardiac ICU after cardiac surgery. INTERVENTIONS: Fluid resuscitation policy change in which crystalloids replaced albumin 5% as the primary fluid strategy for resuscitation after cardiac surgery. MEASUREMENTS AND MAIN RESULTS: Children who underwent cardiac surgery in the 6 months prior to the policy change (5% albumin group) were compared with children admitted during the 6 months after (crystalloid group). Demographic, perioperative, and outcome variables (fluid intake days 1-4 postoperative, vasoactive therapy, blood products, time to negative fluid balance, renal replacement therapies, mechanical ventilation, pediatric cardiac ICU, and length of stay) were collected. Data were analyzed using linear and logistic multivariate analysis. The study included 360 children. There was no association between fluid group and fluid intake (mL/kg) on day 1 postoperatively (coefficient, 2.84; 95% CI, 5.37-11.05; p = 0.497). However, crystalloid group was associated with significantly less fluid intake on day 2 (coefficient, -12.8; 95% CI, -22.0 to -3.65; p = 0.006), day 3 (coefficient, -14.9; 95% CI, -24.3 to -5.57; p = 0.002), and on the first 48 hours postoperative (coefficient, 10.1; 95% CI, -27.9 to -1.29; p = 0.032). Pediatric cardiac ICU stay (coefficient, -1.29; 95% CI, -2.50 to -0.08; p = 0.036) was shorter for the crystalloid group. There were no significant differences in the time to negative balance, need for renal replacement therapy, mechanical ventilation days, hospital stay, or pediatric cardiac ICU survival. CONCLUSIONS: In our study, the use of albumin 5% for resuscitation after cardiac surgery was not associated with less fluid intake but rather the opposite. Albumin administration did not provide measured clinical benefit while exposing children to side effects and generating higher costs to the healthcare system.

Cardiac Surgery-Associated Kidney Injury in Children and Renal Oximetry.

OBJECTIVES: Cardiac surgery-associated acute kidney injury is common in children and associates with negative outcomes. Novel interventions to reduce cardiac surgery-associated acute kidney injury require knowledge of its pathophysiology. States of altered perfusion, oxygen delivery, and energy consumption occur during cardiopulmonary bypass and could protect against or contribute to renal cellular injury and recovery. Near-infrared spectroscopy is noninvasive technology for monitoring regional blood flow and tissue oxygenation. This study evaluated the relationship between renal regional oxygen saturation and cardiac surgery-associated acute kidney injury, using near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass in children. DESIGN: Prospective cohort study. SETTING: Single-center, tertiary care pediatric hospital (Stollery Children's Hospital, Edmonton, AB, Canada). PATIENTS: Children less than or equal to 10 kg undergoing congenital heart disease repair with cardiopulmonary bypass. Heart transplant, preoperative dialysis, sepsis, extracorporeal life support, congenital renal disease, and preoperative nephrotoxins were exclusions. INTERVENTIONS: Renal regional near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass. MEASUREMENTS AND MAIN RESULTS: Outcome measure was cardiac surgery-associated acute kidney injury (defined according to Kidney Disease: Improving Global Outcomes criteria). Regional oxygen saturation was measured continuously using near-infrared spectroscopy (INVOS 5100C Cerebral/Somatic Oximeter; Medronic, Troy, MI) from time of anesthesia to time of transfer to intensive care. Cardiac surgery-associated acute kidney injury occurred in 65%. Lower baseline (precardiopulmonary bypass) regional oxygen saturation was associated with decreased risk of cardiac surgery-associated acute kidney injury (p = 0.01); children with baseline regional oxygen saturation in the highest tertile were 7.14 times more likely to get cardiac surgery- associated acute kidney injury (vs lowest tertile). Area under the curve for ability of baseline regional oxygen saturation to predict cardiac surgery-associated acute kidney injury was 0.73 (95% CI, 0.60-0.85). Children with lower baseline glomerular filtration rate had lower mean renal regional oxygen saturation. CONCLUSIONS: Findings demonstrate that preoperative oxygen supply/demand balance is an important predictor of cardiac surgery-associated acute kidney injury, suggesting lower preoperative (and intraoperative) renal blood flow may be protective. There is not yet a definite link between remote ischemic preconditioning and prevention of cardiac surgery-associated acute kidney injury; however, renal protective effects of sublethal ischemia should continue to be explored.

Predicting early outcomes of liver transplantation in young children: The EARLY study.

AIM: To determine potentially modifiable predictors of early outcomes after liver transplantation in children of age < 3 years. METHODS: This study was a retrospective chart review including all consecutive children of age less than 3-years-old having had a liver transplant done at the Western Canadian referral center from June 2005 to June 2015. Pre-specified potential predictor variables and primary and secondary outcomes were recorded using standard definitions and a case report form. Associations between potential predictor variables and outcomes were determined using univariate and multiple logistic [odds ratio (OR); 95%CI] or linear (effect size, ES; 95%CI) regressions. RESULTS: There were 65 children, of mean age 11.9 (SD 7.1) mo and weight 8.5 (2.1) kg, with biliary-atresia in 40 (62%), who had a living related donor [LRD; 29 (45%)], split/reduced [21 (32%)] or whole liver graft [15 (23%)]. Outcomes after liver transplant included: ventilator-days of 12.5 (14.1); pediatric intensive care unit mortality of 5 (8%); re-operation in 33 (51%), hepatic artery thrombosis (HAT) in 12 (19%), portal vein thrombosis (PVT) in 11 (17%), and any severe complication (HAT, PVT, bile leak, bowel perforation, intraabdominal infection, retransplant, or death) in 32 (49%) patients. Predictors of the prespecified primary outcomes on multiple regression were: (1) HAT: split/reduced (OR 0.06; 0.01, 0.76; P = 0.030) or LRD (OR 0.16; 0.03, 0.95; P = 0.044) vs whole liver graft; and (2) ventilator-days: surgeon (P < 0.05), lowest antithrombin (AT) postoperative day 2-5 (ES -0.24; -0.47, -0.02; P = 0.034), and split/reduced (ES -12.5; -21.8, -3.2; P = 0.009) vs whole-liver graft. Predictors of the pre-specified secondary outcomes on multiple regression were: (1) any thrombosis: LRD (OR 0.10; 0.01, 0.71; P = 0.021) or split/reduced (OR 0.10; 0.01, 0.85; P = 0.034) vs whole liver graft, and lowest AT postoperative day 2-5 (OR 0.93; 0.87, 0.99; P = 0.038); and (2) any severe complication: surgeon (P < 0.05), lowest AT postoperative day 2-5 (OR 0.92; 0.86-0.98; P = 0.016), and split/reduced (OR 0.06; 0.01, 0.78; P = 0.032) vs whole-liver graft. CONCLUSION: In young children, whole liver graft and surgeon was associated with more complications, and higher AT postoperative day 2-5 was associated with fewer complications early after liver transplantation.

Survey of Sedation and Analgesia Practice Among Canadian Pediatric Critical Care Physicians.

BACKGROUND: Despite the fact that almost all critically ill children experience some degree of pain or anxiety, there is a lack of high-quality evidence to inform preferred approaches to sedation, analgesia, and comfort measures in this environment. We conducted this survey to better understand current comfort and sedation practices among Canadian pediatric intensivists. METHODS: The survey was conducted after a literature review and initial focus groups. The survey was then pretested and validated. The final survey was distributed by email to 134 intensivists from 17 PICUs across Canada using the Research Electronic Data Capture system. RESULTS: The response rate was 73% (98/134). The most commonly used sedation scores are Face, Legs, Activity, Cry, and Consolability (42%) and COMFORT (41%). Withdrawal scores are commonly used (65%). In contrast, delirium scores are used by only 16% of the respondents. Only 36% of respondents have routinely used sedation protocols. The majority (66%) do not use noise reduction methods, whereas only 23% of respondents have a protocol to promote day/night cycles. Comfort measures including music, swaddling, soother, television, and sucrose solutions are frequently used. The drugs most commonly used to provide analgesia are morphine and acetaminophen. Midazolam and chloral hydrate were the most frequent sedatives. CONCLUSION: Our survey demonstrates great variation in practice in the management of pain and anxiety in Canadian PICUs. Standardized strategies for sedation, delirium and withdrawal, and sleep promotion are lacking. There is a need for research in this field and the development of evidence-based, pediatric sedation and analgesia guidelines.

Impact and Challenges of a Policy Change to Early Track Extubation in the Operating Room for Fontan.

While policy changes toward early extubation in the operating room (OR) have been commonly seen in palliative surgeries in single ventricle anatomy, no systematic assessment of their impact on patient outcome has been reported. All patients aged 0-17 years admitted to a PICU in a quaternary children's hospital for post-operative management following a primary Fontan procedure between 2005 and 2011 were included. Patients for revision of Fontan or patients admitted to adult Cardiovascular Intensive Care Unit were excluded. Practice policy was changed from routine extubation in the PICU to early extubation in OR in January 2008. Data were compared between the pre-policy-change era (2005-2007) and the post-policy-change era (2008-2011) to assess the impact of the change on patient outcomes. Generalized linear regression (GLM) and interrupted time series (ITS) analysis were used to access the effect of policy change on PICU length of stay and post-operative fluid balance, adjusting for potential confounders using propensity scores. Root cause analysis (RCA) was conducted to describe causes of failed extubation and challenges of this policy change. One hundred twenty-seven children met inclusion criteria. Average body weight was 14.7 kg [standard deviation (SD) 3.9], and age was 3.5 years (SD 1.9). A clear change in extubation practice occurred between the pre- versus post-policy-change eras: 97.5 % were extubated in the PICU in the pre-policy-change era, as compared to 15.0 % in the post-policy-change era. The average PICU length of stay was shortened by 4.1 days from the pre-policy-change era to the post-policy-change era [95 % CI -1.2 to -6.9, p < 0.01] in the GLM, whereas the ITS analysis did not show a statistically significant difference [95 % CI 1.8 to -2.5] (p = 0.23). No statistically significant difference was observed in the fluid balance in the 24 h post-operation in both analyses. Sixteen patients (16/127, 12.6 %) were reintubated mainly for hemodynamic instability in the pre-policy-change era (6/39, 15.4 %) and excessive bleeding or respiratory etiologies in the post-policy-change era (10/88, 11.4 %). Significant reduction of PICU length of stay was achieved without any evidence of worsening of patient outcomes. RCA suggests watchful observations with respect to bleeding and respiratory etiologies are the key to prevent failure of extubation in the current practice.

Development of a Model Identifying Fontan Patients at High Risk for Failed Early Extubation in the Operating Room.

OBJECTIVE: To identify patients at high risk for failed early extubation in the operating room (OR) following the Fontan procedure and generate a predictive model to allow improved clinical decision making. DESIGN: The success of an early-extubation strategy (extubation in the OR) was reviewed in patients aged 0 to 17 years old, undergoing the Fontan procedure between 2008 and 2011. Patients who required reintubation following primary extubation in the OR were compared with those who did not. Logistic regression with a backward variable selection was used to develop a predictive model in two stages: first, using pre-/perioperative predictors and then using postoperative predictors among the first-stage positive. SETTING: Canadian quaternary-care university children's hospital PICU. The treatment policy was changed from the routine extubation in PICU to extubation in the OR in January 2008. RESULTS: A total of 75 patients met our inclusion criteria: 8 patients required reintubation. Patients' average body weight was 14.5 kg (standard deviation [SD] 3.7), average age was 3.5 (SD 1.9) years, and average preoperative transcutaneous arterial saturation was 80.9% (SD 6.8). The first-stage predictive model contained three predictors: concomitant procedure (odds ratio [OR] >999, 95% confidence interval [CI] 15.7-infinity, p < 0.001), total bypassing time (cutoff; ≥99 minutes) (OR >999, 95% CI 6.5-infinity, p < 0.001), and absence of fenestration for pre/operative variables (OR >999, 95% CI 9.5-infinity, p < 0.001). The second-stage model included chest-tube fluid loss (CTFL ≥9.9 mL/kg/first 6 h). Our sequential prediction model had net sensitivity of 87.5% and specificity of 77.6%. CONCLUSION: We produced a predictive model for failed early extubation in Fontan patients. The sensitivity and specificity values are in the range of clinical utility. The model should be validated with an independent sample with a larger sample size.

Does Topical Anesthetic Reduce Pain During Intraosseous Pin Removal in Children? A Randomized Controlled Trial.

BACKGROUND: The purpose of this study was to determine the effectiveness of topical liposomal lidocaine in reducing the pain perceived by children undergoing percutaneous intraosseous pin (PP) removal in the outpatient orthopaedic clinic. METHODS: A triple-blinded, randomized, placebo-controlled clinical trial comparing topical liposomal lidocaine to a placebo was conducted at the Stollery Children's Hospital between September 2008 and February 2011. Subjects undergoing the removal of PP in the orthopaedic outpatient clinic between ages 3 and 16 years were recruited. A computer-generated variable-block randomization scheme was used to determine each subject's group assignment. Pain was recorded just before randomization and immediately after the procedure using the Oucher Scale (for subjects) and a 10-cm Visual Analog Scale (for parents and an observing orthopaedic technician). In a subset of individuals, follow-up telephone calls were made 24 hours postprocedure to inquire about any adverse event from the use of the topical liposomal lidocaine. Data were analyzed using the Student t test. RESULTS: Of a total of 296 recruited subjects, complete data were available on 281 subjects (140 intervention and 141 control). There were no significant differences between the 2 groups with regards to baseline characteristics, including preprocedure pain scores. Although postprocedure pain scores demonstrated an increase in pain in both groups (2.3 points in the treatment group and 2.0 points in the placebo group), no statistically significant difference was seen in postprocedure pain scores between groups (P=0.81). No adverse events were observed or reported. CONCLUSIONS: Topically applied liposomal lidocaine was not effective in reducing pain during this procedure, compared with a placebo. However, this study demonstrates that PP removal is a painful procedure in children. Given the large volume of patients who undergo this procedure and the long-term consequences of experiencing painful procedures in childhood, it is important to find safe and fast-acting methods to decrease procedural pain associated with PP removal.

The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes.

In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children's Hospital.

Cardiac catheterization in children with pulmonary hypertensive vascular disease.

The risks associated with cardiac catheterization in children with pulmonary hypertension (PH) are increased compared with adults. We reviewed retrospectively all clinical data in children with PH [mean pulmonary artery pressure (mean PAp) ≥25 mmHg and pulmonary vascular resistance index (PVRI) ≥3 Wood units m(2)] undergoing cardiac catheterization between 2009 and 2014. Our strategy included a team approach, minimal catheter manipulation and sildenafil administration prior to extubation. Adverse events occurring within 48 h were noted. Seventy-five patients (36 males), median age 4 years (0.3-17) and median weight 14.6 kg (2.6-77 kg), underwent 97 cardiac catheterizations. Diagnoses included idiopathic or heritable pulmonary arterial hypertension (PAH) (29 %), PAH associated with congenital heart disease (52 %), left heart disease (5 %) and lung disease (14 %). Mean PAp was 43 ± 19 mmHg; mean PVRI was 9.7 ± 6 Wood units m(2). There were no deaths or serious arrhythmias. No patient required cardiac massage. Three patients who suffered adverse events had suprasystemic PAp (3/3), heritable PAH (2/3), decreased right ventricular function (3/3), and pulmonary artery capacitance index <1 ml/mmHg/m(2) (3/3) and were treatment naïve (3/3). No patient undergoing follow-up cardiac catheterization suffered a complication. In 45 % of cases, the data acquired from the follow-up cardiac catheterization resulted in an alteration of therapy. Three percent of children with PH undergoing cardiac catheterization suffered adverse events. However, there were no intra or post procedural deaths and no one required cardiac massage or cardioversion. Follow-up cardiac catheterization in patients receiving pulmonary hypertensive targeted therapy is safe and provides useful information.

Neurotoxicity of sedative and analgesia drugs in young infants with congenital heart disease: 4-year follow-up.

OBJECTIVES/AIM: To determine whether sedation/analgesia drugs used before, during, and after infant cardiac surgery are associated with neurocognitive and functional outcomes. BACKGROUND: Some animal models suggest neurotoxic effects of anesthetic drugs on the developing brain; however, potential human effects are unknown. Whether these results can be extrapolated to humans is unknown. METHODS/MATERIALS: Prospective follow-up project of all infants ≤6 weeks old having surgery for congenital heart disease between 04/03 and 12/06. Demographic, perioperative, and sedation/analgesia variables were collected. Outcomes at kindergarten age were Wechsler Preschool and Primary Scale of Intelligence-III, Beery-Buktenica Developmental Test of Visual Motor Integration (VMI-V), and General Adaptive Composite (GAC) of the Adaptive Behavior Assessment System-II. Multivariable linear regression was used to identify predictor variables. RESULTS: From 135 infants who underwent heart surgery, 19 died, 17 were excluded, 8 were lost to follow-up, leaving 91 children for analysis. Multiple linear regression found days on chloral hydrate [3.5 (3.7) days] was associated with lower performance intelligence quotient (PIQ) (Effect size -1.03; 95% CI -1.96, -0.10; P = 0.03), and cumulative dose [54.2 (60.3) mg·kg(-1) ] of benzodiazepines was associated with lower VMI scores (Effect size -0.07; 95% CI -0.12, -0.01; P = 0.026). No other associations were found between sedation/analgesia variables and full-scale IQ, PIQ, Verbal IQ, VMI, or GAC. CONCLUSION: Assessment of this cohort at kindergarten age found a small statistically significant association between days on chloral hydrate and PIQ, and benzodiazepine cumulative dose and lower VMI. No other association between sedation/analgesia drugs and outcomes was found.

Indications and outcomes in children receiving renal replacement therapy in pediatric intensive care.

PURPOSE: We aimed to describe patient characteristics, indications for renal replacement therapy (RRT), and outcomes in children requiring RRT. We hypothesized that fluid overload, not classic blood chemistry indications, would be the most frequent reason for RRT initiation. MATERIALS AND METHODS: A retrospective cohort study of all patients receiving RRT at a single-center quaternary pediatric intensive care unit between January 2004 and December 2008 was conducted. RESULTS: Ninety children received RRT. The median age was 7 months (interquartile range, 1-83). Forty-six percent of patients received peritoneal dialysis, and 54% received continuous renal replacement therapy. The median (interquartile range) PRISM-III score was 14 (8-19). Fifty-seven percent had congenital heart disease, and 32% were on extracorporeal life support. The most common clinical condition associated with acute kidney injury was hemodynamic instability (57%; 95% confidence interval [CI], 46-67), followed by multiorgan dysfunction syndrome (17%; 95% CI, 10-26). The most common indication for RRT initiation was fluid overload (77%; 95% CI, 66-86). Seventy-three percent (95% CI, 62-82) of patients survived to hospital discharge. CONCLUSIONS: Hemodynamic instability and multiorgan dysfunction syndrome are the most common clinical conditions associated with acute kidney injury in our population. In the population studied, the mortality was lower than previously reported in children and much lower than in the adult population.

Mitochondrial DNA depletion syndrome-an unusual reason for interstage attrition after the modified stage 1 Norwood operation.

We describe a patient with a variant of the hypoplastic left heart syndrome who died 16 weeks after a modified stage 1 Norwood from a mitochondrial DNA depletion syndrome.

Upper airway findings in children with Down syndrome on sleep nasopharyngoscopy: case-control study.

OBJECTIVE: To identify the pattern of upper airway endoscopic findings in Down syndrome (DS) children with sleep-disordered breathing (SDB) spectrum. DESIGN AND SETTING: Retrospective case-control study in a tertiary pediatric centre. METHODS: DS children presenting with persistent snoring or SDB who underwent sleep nasopharyngoscopy (SNP) were identified from a prospectively kept surgical database. All SNPs were performed using a uniform intravenous sedation technique. Controls with persistent snoring or SDB from the same database were identified and pair-matched for age, gender, and body mass index (BMI) percentiles. The video recordings of the SNP for all subjects were reviewed. A comparison of the proportions of obstruction (O), mixed (M), and collapse (C) findings in each group was performed. RESULTS: Over a period of 4.5 years, 23 consecutive DS children were identified (7 girls, 16 boys; mean age 7.09 ± 4.37 years). They were matched with 23 controls (mean age 7.6 ± 4.14 years). The DS group exhibited significantly more pharyngeal collapses than the controls (O:C:M, 2:6:15 and 12:0:10, respectively; p < .005). Lingual collapses were also noted more in DS children (11 vs 4), and a more significant number of collapses were seen (p < .004). Whereas nearly equal numbers exhibited tonsillar obstruction, adenoidal obstruction was significantly less in DS children (p < .05). CONCLUSION: Generalized pharyngeal collapse dominates in DS children who present with SDB and is independent of age, gender, and BMI. Adenoidal hypertrophy causing obstruction is much less encountered than in controls.

An evaluation of intraoperative renal support during liver transplantation: a matched cohort study.

BACKGROUND: Intraoperative continuous renal replacement therapy (CRRT) has been utilized during liver transplantation (LT). Our objective was to assess intraoperative CRRT for metabolic control, postoperative complications and outcomes. METHODS: Retrospective matched cohort study. Cases were LT patients receiving intraoperative CRRT. Controls were matched for demographics and Model for End-Stage Liver Disease (MELD) score. Data were extracted on physiology, course and outcomes. RESULTS: 72 patients were included. Despite effort to match by MELD, cases had higher scores (35.4 vs. 29.9, p = 0.01) compared to controls. Preoperatively, cases received more vasopressors (p = 0.006), and more RRT (94.4 vs. 25.7%, p < 0.0001). There was no difference in complications (p = 0.35) or ICU re-admission rate (p = 0.29). Cases were more likely to require postoperative RRT (p < 0.0001). There was no difference in hospital mortality (p = 0.61). CONCLUSIONS: LT patients selected for intraoperative CRRT more commonly have hemodynamic instability and preoperative acute kidney injury requiring RRT. Despite higher illness severity for cases, there were no differences in complications or mortality.

Neurodevelopmental outcome following exposure to sedative and analgesic drugs for complex cardiac surgery in infancy.

OBJECTIVES/AIM: To determine whether sedation/analgesia drugs used before, during, and after infant cardiac surgery are associated with neurodevelopmental outcome. BACKGROUND: Animal models suggest detrimental effects of anesthetic drugs on the developing brain. Whether these results can be extrapolated to human neonates is unclear. METHODS/MATERIALS: This is a prospective follow-up project conducted in Western Canada. In all infants ≤6 weeks of age having surgery for congenital heart disease between April 2003 and December 2006, demographic and perioperative variables were collected prospectively. Sedation/analgesia variables were collected retrospectively. For each drug class (inhalationals, opioids, benzodiazepines, ketamine, and chloral hydrate), we calculated the cumulative dose received during hospitalization, average dose received per day, and cumulative number of days the patient received the drug. The outcomes at 18-24 months were as follows: General Adaptive Composite and motor scaled scores of the Adaptive Behavior Assessment System, significant mental, motor, and vocabulary delay. Multivariable logistic and linear regression was used to analyze the data. RESULTS: One hundred and thirty-five neonates underwent open heart surgery; 19 died, 16 had chromosomal abnormality, and five were lost to follow up, leaving 95 survivors for analysis. Multiple linear regression analysis found no evidence of an association between sedation/analgesia variables and ABAS-GAC score or motor scale score. Multiple logistic regression analysis found no evidence of an association between sedation/analgesia variables and significant mental, motor, or vocabulary delay. CONCLUSION: We found no evidence of an association between dose and duration of sedation/analgesia drugs during the operative and perioperative period and adverse neurodevelopmental outcomes.

Prevalence of laryngomalacia in children presenting with sleep-disordered breathing.

OBJECTIVE: To determine the prevalence of laryngomalacia among children presenting with symptoms of sleep-disordered breathing (SDB). METHOD: A retrospective observational study was conducted at a tertiary care paediatric hospital. All children presenting with SDB during a 55-month period were investigated using sleep nasopharyngoscopy (SNP). Patients who had laryngomalacia were identified. Patients who did not present primarily with SDB, or were not examined with SNP were excluded. Data for analysis was collected from a prospectively kept surgical database and medical records. This included patients' demographics, symptoms (including symptoms in infancy), diagnoses, SNP findings, overnight pulse oximetry findings, and treatment. RESULTS: We identified 358 patients with documented primary diagnosis of SDB and who had undergone SNP. Fourteen of these also had a documented diagnosis of laryngomalacia, giving a prevalence rate of 3.9%. Three children were syndromic, and one had cerebral palsy in addition to SDB and laryngomalacia. Three children were obese, and three children had gastroesophageal reflux disease. Seven cases (50%) had symptoms of snoring and/or swallowing dysfunction and/or stridor in infancy. Twelve patients had adenotonsillar surgery. In eight cases symptoms resolved completely with adenotonsillar surgery only. In total, six patients had a supraglottoplasty. There were three failures to supraglottoplasty. CONCLUSION: The prevalence of laryngomalacia within children presenting with SDB is 3.9%. Our findings support full evaluation of the airway to identify the site of pathology mediating SDB symptoms.