Improved patient safety with a simplified operating room to pediatric intensive care unit handover tool (PATHQS).

Introduction: Patient handover is a crucial transition requiring a high level of coordination and communication. In the BC Children's Hospital (BCCH) pediatric intensive care unit (PICU), 10 adverse events stemming from issues that should have been addressed at the operating room (OR) to PICU handover were reported into the patient safety learning system (PSLS) within 1 year. We aimed to undertake a quality improvement project to increase adherence to a standardized OR to PICU handover process to 100% within a 6-month time frame. In doing so, the secondary aim was to reduce adverse events by 50% within the same 6-month period. Methods: The model for improvement and a Plan, Do, Study, Act method of quality improvement was used in this project. The adverse events were reviewed to identify root causes. The findings were reviewed by a multidisciplinary inter-departmental group comprised of members from surgery, anesthesia, and intensive care. Issues were batched into themes to address the most problematic parts of handover that were contributing to risk. Intervention: A bedside education campaign was initiated to familiarize the team with an existing handover standard. The project team then formulated a new simplified visual handover tool with the mnemonic "PATHQS" where each letter denoted a step addressing a theme that had been noted in the pre-intervention work as contributing to adverse events. Results: Adherence to standardized handover at 6 months improved from 69% to 92%. This improvement was sustained at 12 months and 3 years after the introduction of PATHQS. In addition, there were zero PSLS events relating to handover at 6 and 12 months, with only one filed by 36 months. Notably, staff self-reporting of safety concerns during handover reduced from 69% to 13% at 6 months and 0% at 3 years. The PATHQS tool created in this work also spread to six other units within the hospital as well as to one adult teaching hospital. Conclusion: A simplified handover tool built collaboratively between departments can improve the quality and adherence of OR to PICU handover and improve patient safety. Simplification makes it adaptable and applicable in many different healthcare settings.

Patch repair is an independent predictor of morbidity and mortality in congenital diaphragmatic hernia.

BACKGROUND: Infants with congenital diaphragmatic hernia (CDH) have variable outcomes. There is a considerable potential benefit in being able to predict perinatally, which infants have severe hypoplasia and are thus more likely to die or survive with significant morbidity. We examine the relationship between a need for patch repair of CDH (PR) and outcome, using a national database. METHODS: Baseline characteristics of patients undergoing PR or non-patch repair (NPR) were compared. Multivariate analysis was performed to determine the association of PR with mortality and morbidity independent of other known predictors. RESULTS: Baseline characteristics of PR and NPR infants were similar although those infants with PR had higher SNAP-II scores. PR was an independent predictor of mortality with an odds ratio of 17.1 (95%CI 2.0-149.2) and was independently associated with secondary outcome measures of morbidity, including the need for oxygen at discharge and the duration of ventilation. CONCLUSIONS: Infants requiring PR have significantly higher mortality and suffer greater morbidity than those undergoing NPR. This association is independent of other known predictors of mortality. Identifying prenatal features associated with this high risk group would be of great clinical value.

Does pre-biopsy contrast enema delay the diagnosis of long segment Hirschsprung's disease?

INTRODUCTION: The diagnosis of long segment Hirschsprung's disease (LSHD) is frequently delayed. Our purpose was to: 1) summarize contrast enema (CE) findings in patients with LSHD, and 2) evaluate the utility of CE by comparing LSHD patients managed with/without pre-biopsy CE. METHODS: All LSHD cases (transition zone [TZ] proximal to the splenic flexure) treated between 1984 and 2009 were stratified according to whether a pre-biopsy CE was done (Group 1) or not (Group 2). CE were reviewed by a single pediatric radiologist, and the original reports were categorized as "helpful", "inconclusive" or "misleading". Group comparisons included elapsed days from admission to diagnostic rectal biopsy/first operation and initial hospitalization length of stay (LOS). RESULTS: 29 patients (16 in Group 1; 13 in Group 2) were identified. CE review revealed TZ in 7/16 (44%); and of these, 6 (86%) underestimated the actual aganglionic segment length. 6/16 (38%) original CE reports were "misleading". Overall, Group 1 patients experienced a significant delay in time to biopsy (p=0.047), first operation (p=0.005), and showed a trend towards prolonged LOS. CONCLUSIONS: Pre-biopsy CE offers little to the diagnosis of LSHD and may contribute to diagnosis/treatment delays. Even if a TZ is recognized in biopsy proven HD, the predicted aganglionic segment length should not guide the operative planning.

Impact of target blood gases on outcome in congenital diaphragmatic hernia (CDH).

INTRODUCTION: Neonatal intensive care unit (NICU) stabilization strategies which normalize physiology according to predetermined blood gas targets may contribute to observed improved survival rates of patients with CDH. The purpose of our study was to compare risk-adjusted outcomes of CDH patients managed with or without blood gas targets established at NICU admission. METHODS: Cases were collected from a national CDH network between May 2005 and November 2007. On NICU admission, the responsible neonatologist was asked to establish target ranges for pH, pCO (2), pO (2), and pre/post-ductal O (2) saturation. The outcomes analyzed were mortality, need for ECMO, days of mechanical ventilation/supplemental oxygen, and length of stay. RESULTS: Of 147 CDH infants, 63 had admission blood gas targets. Severity of illness and gestational age in both groups were comparable (SNAP-II score). Infants with blood gas targets had a significantly lower mortality than those without (Hazard ratio 0.27, p=0.006). CONCLUSIONS: Blood gas targets for the management of infants with CDH are associated with improved survival. Although the willingness to create and use stabilization targets to guide early NICU care may be a surrogate for other factors (experience, staffing, lack of interest), it is clearly associated with improved survival in CDH.

Perinatal predictors of outcome in gastroschisis.

OBJECTIVE: To identify perinatal risk variables predictive of outcome in gastroschisis. STUDY DESIGN: Gastroschisis cases were collected over a 3-year period from a national database. Risk variables evaluated included gestational age (GA), birth weight, time of birth, admission illness severity (score for neonatal acute physiology-II, SNAP-II) score, and abdominal closure type. Mortality and survival outcomes were analyzed. Multivariate analyses were performed. RESULT: In all, 239 infants were survived (96%). SNAP-II score predicted mortality (relative risk (RR)=1.07, 95% confidence interval (CI)=1.0 to 1.1). Length of hospital stay (LOS) and ventilation days were predicted by GA and by SNAP-II score. SNAP-II score predicted total parenteral nutrition (TPN) days (P=0.006). Severe cholestasis (conjugated bilirubin of >10 mg per 100 ml) was inversely related to GA (RR=0.77, 95% CI=0.61 to 0.97) and directly to categorical SNAP-II score (RR=3.4, 95% CI=1.2 to 10.1). Urgent closure predicted fewer TPN days (P=0.003) and shorter LOS (P=0.0002). CONCLUSION: SNAP-II scores significantly predict mortality and survival outcomes. Urgent closure favors fewer TPN days and shorter LOS. Our data refute routine preterm delivery in gastroschisis.

Abdominal compartment syndrome complicating paediatric extracorporeal life support: diagnostic and therapeutic challenges.

We report three paediatric cases, and summarise the reported experience in two others, with cardiorespiratory failure requiring extracorporeal life support for which supportive pump flows could not be maintained due to abdominal compartment syndrome. In two of our patients, the mechanism of abdominal compartment syndrome was massive intra-abdominal fluid extravasation secondary to sepsis, while in the third, the mechanism was post-traumatic intra-abdominal haemorrhage. Although all three children eventually died, decompressive laparotomy and arrest of haemorrhage in the trauma patient restored venous return and enabled technically adequate extracorporeal life support. In two previously reported cases of sepsis with massive fluid resuscitation resulting in abdominal compartment syndrome, one patient died without attempted decompression, while the other patient survived after peritoneal catheter placement restored venous return. Once correctable causes of inadequate venous cannula drainage have been excluded, abdominal compartment syndrome should be considered in any patient on extracorporeal life support with a taut abdomen and reduced venous return. If abdominal compartment syndrome can be proven or is strongly suspected, there may be a role for selective decompressive laparotomy.

Correlation of transcutaneous hepatic near-infrared spectroscopy readings with liver surface readings and perfusion parameters in a piglet endotoxemic shock model.

PURPOSE: To determine whether transcutaneous liver near-infrared spectrophotometry (NIRS) measurements correlate with NIRS measurements taken directly from the liver surface, and invasive blood flow measurements. PROCEDURE: Laparotomy was performed in 12 Yorkshire piglets, and ultrasound blood flow probes were placed on the hepatic artery and portal vein. Intravascular catheters were inserted into the hepatic and portal veins for intermittent blood sampling, and a pulmonary artery catheter was inserted via the jugular vein for cardiac output measurements. NIRS optodes were placed on skin overlying the liver and directly across the right hepatic lobe. Endotoxemic shock was induced by continuous infusion of Escherichia coli lipopolysaccharide O55:B5. Pearson's correlations were calculated between the NIRS readings and the perfusion parameters. FINDINGS: After endotoxemic shock induction, liver blood flow, and oxygen delivery decreased significantly. There were statistically significant correlations between the transcutaneous and liver-surface NIRS readings for oxyhemoglobin, deoxyhemoglobin, and cytochrome c oxidase concentrations. There were similar significant correlations of the transcutaneous oxyhemoglobin with both the mixed venous and hepatic vein saturation, and mixed venous and hepatic vein lactate. CONCLUSIONS: Transcutaneous NIRS readings of the liver, in an endotoxemic shock model, correlate with NIRS readings taking directly from the liver surface, as well as with global and specific organ-perfusion parameters.

Primary reversed gastric tube reconstruction in long gap esophageal atresia.

BACKGROUND/PURPOSE: Esophageal reconstruction in long-gap esophageal atresia (EA) poses a technical challenge with several surgical options. The purpose of this study was to review the authors' experience with the reversed gastric tube (RGT) in esophageal reconstruction. METHODS: This series describes 7 babies with pure EA treated at 2 centers between 1989 and 2001. Data, gathered by retrospective chart review, included clinical details of the esophageal and associated malformations, technique and timing of repair, early and late complications, and long-term follow-up. Institutional review board (IRB) approval of this study has been obtained. RESULTS: Seven babies were included. Associated malformations were present in 4: trisomy 21 in 2 and imperforate anus in 2. After gastrostomy tube placement, patients were treated with gastrostomy tube feedings and continuous upper pouch suction. Median gap length was 5.5 vertebral segments (range, 3 to 9). RGT with a posterior mediastinal esophagogastric anastomosis was performed at median age of 62 days (range, 38 to 131). There were no anastomotic leaks. Three patients had strictures, one required resection. Exclusive oral nourishment was achieved in 5 patients by 6 months of age. At last follow-up (mean, 4.5 years), 6 patients were receiving oral nutrition exclusively, and all were maintaining growth curves. CONCLUSIONS: In long gap EA, early esophageal reconstruction using an RGT can be performed with minimal morbidity and promising short-term results.

The role of prospective randomized clinical trials in pediatric surgery: state of the art?

PURPOSE: This study sought to determine the role of randomized controlled trials (RCT) in the evolution of pediatric surgical practice. METHODS: The authors used a computer-assisted literature search to identify all clinical trials related to pediatric surgery published in the English-language literature from 1966 through 1999. Each article was reviewed in detail for purpose, content, conduct, and quality of the trial. The authors assessed quality with a previously validated instrument (Chalmers Qualitative Assessment). RESULTS: The authors identified 134 RCTs related to pediatric surgery over the past 33 years. This accounts for 0.17% of 80,377 articles published in the field. The areas of surgery studied were analgesia 65 (49%), antibiotics 17 (13%), extracorporeal membrane oxygenation (ECMO) 9 (7%), gastrointestinal, burns, oncology, minimally invasive surgery, vascular access, congenital anomalies, and trauma (each <5%). Only 16 (12%) trials compared 2 surgical therapies, 9 (7%) compared a medical versus a surgical therapy, and 109 (81%) compared 2 medical therapies in surgical patients. Fourteen (10%) RCTs were funded by peer-reviewed agencies. Only 17 (13%) RCTs included a biostatistician as an author or a consultant. Trial design included calculation of sample size and statistical power in 21 (16%) RCTs. Method of randomization was reported in only 51 (38%). The test statistic and observed probability value was reported in 15 (11%). CONCLUSIONS: Clinical trials are used infrequently to answer questions related to pediatric surgery. When RCTs are utilized, they often suffer from poor trial design, inadequate statistical analysis, and incomplete reporting. Pediatric surgery could benefit from increased expertise, funding, and participation in clinical trials.

Fetal therapy with rhIGF-1 in a rabbit model of intrauterine growth retardation.

BACKGROUND: Intrauterine growth retardation (IUGR) may, in part, be due to a deficiency of insulin-like growth factor-1 (IGF-1). The objectives of this study were to determine the relationship between fetal serum IGF-1 levels and fetal and placental size in a rabbit model of IUGR and to compare two techniques of selective, exogenous IGF-1 administration (transamniotic and branch uterine arterial catheter infusion) to growth-retarded fetuses in utero. MATERIALS AND METHODS: Pregnant rabbits (n = 6) had their fetuses harvested near term (31 days) for fetal and placental weighing and serum collection. Growth-retarded fetuses were selectively infused for 7 days with recombinant human IGF-1 (rhIGF-1; 1,440 ng/day) either through a transamniotic catheter (n = 8) or via an adjacent uterine arterial branch catheter (n = 6). Opposite horn runts were sham catheterized, but not infused. At term, the fetal runt pairs and their placentas were harvested and weighed, and their serum was collected. The correlation between fetal and placental weight and endogenous serum IGF-1 was calculated (Pearson coefficient, r), while paired t-tests were used to compare the means between the IGF-1-infused and control groups. RESULTS: There was a significant correlation between fetal (r = 0.4230; P = 0.022) and placental weight (r = 0.4166; P = 0.025) and endogenous serum levels of IGF-1. Transamniotic infusion of rhIGF-1 was associated with an increase in serum IGF-1 level (254 +/- 79 vs 351 +/- 101 ng/ml, P = 0.04) and placental weight (5.4 +/- 2.3 vs 7.1 +/- 3.2 g, P = 0.005), and with a trend toward increased fetal weight between matched fetal runt pairs. Fetal mortality in the uterine arterial catheterized group was 76%, and there was no significant difference in fetal or placental weight or IGF-1 levels between infused and noninfused survivors. CONCLUSIONS: Endogenous fetal serum levels correlate with fetal and placental size in the rabbit IUGR model. Transamniotic administration of rhIGF-1 significantly increases serum IGF-1 levels and placental weight of fetal runts, while uterine vessel catheterization results in prohibitive fetal mortality and does not increase fetal or placental growth or IGF-1 levels.

Omphalocele, colonic atresia, and Hirschsprung's disease: an unusual cluster of malformations in a single patient.

Omphalocele, colonic atresia (CA), and Hirschsprung's disease (HD) are individually rare congenital malformations. An association between CA and HD has been described, but the co-occurrence of all three malformations has not been previously reported. We present an infant born with all three malformations and review the management issues relevant to this case, with an emphasis on the importance of considering co-existent HD in any infant born with CA.

Rational treatment of empyema in children.

HYPOTHESIS: Efficacious and cost-effective treatment of pediatric empyema can be accomplished following a protocol based on its radiographic appearance. Therapeutic modalities include thoracostomy tube drainage (TTD) with or without fibrinolytic therapy (FT) and video-assisted thoracoscopic debridement (VATD). DESIGN: Retrospective case series. SETTING: Tertiary referral center. RESULTS: From 1995 through 1999, 31 children were treated ranging in age from 11 months to 18 years (mean age, 5.1 years). Twenty-seven (87.1%) underwent TTD; of these, 22 (81.5%) received FT with urokinase. The TTD failed in 4 children (14.8%) who required salvage VATD. Primary VATD was performed in another 4 children (12.9%). The mean length of stay was 14.6 days (TTD, 14.1 days; salvage VATD, 20. 0 days; primary VATD, 11.5 days), ranging from 8.0 to 30.0 days. Complications included readmission for fever (2 patients [6.5%]) and gastrointestinal bleeding (1 patient [3.2%]). There were no anaphylactic reactions or bleeding episodes due to urokinase. Two patients (7.4%) treated with TTD and FT developed an air leak that resolved spontaneously. The mean hospital charges were $78,832 (TTD with or without FT, $75,450; salvage VATD, $107,476; primary VATD, $69,634). The procedural charges were highest for salvage VATD. CONCLUSIONS: Most cases of pediatric empyema can be treated by TTD with or without FT. This therapy is safe and effective for children with nascent disease. Primary VATD is preferred in children with advanced disease. Cost-effectiveness could be further improved through better prediction of those patients likely to fail TTD and require salvage VATD. An algorithmic approach based on findings from computed tomography or (better) ultrasonography of the chest may be the best way to make this distinction and rationalize care.

Salvage laparotomy for failure of peritoneal drainage in necrotizing enterocolitis in infants with extremely low birth weight.

BACKGROUND/PURPOSE: Peritoneal drainage is a temporizing procedure for infants with extremely low birth weight (ELBW) who have perforated necrotizing enterocolitis (NEC). "Salvage" laparotomy is advocated when patients worsen after drainage. Some patients have survived with intact gastrointestinal functional after drainage alone. The purpose of this study is to determine if these salvage laparotomies are beneficial. METHODS: The authors reviewed the records of ELBW infants treated at Stanford University with perforated NEC from 1993 through 1998. Data collected included demographic makeup, type of operation, survival rate, postoperative complications, length of stay (LOS), and cost. RESULTS: The authors treated 26 patients, 9 with laparotomy and 17 with peritoneal drainage. The peritoneal drainage group had lower birth weight and more comorbid conditions. Survival rate was similar between laparotomy and drainage: 55.6% versus 41.2%. Four patients in the drainage group underwent salvage laparotomy for perceived clinical deterioration. All of these patients died. The clinical status of patients who had salvage laparotomy and died was similar to those who did not and lived. Seven of 13 patients treated with drainage followed only by supportive care and antibiotics survived. Cost and LOS for patients undergoing salvage laparotomy were much greater than for nonsurviving patients undergoing only peritoneal drainage: 84 +/- 20 days and $660,000 compared with 34 +/- 11 days and $306,000. CONCLUSIONS: Both primary peritoneal drainage and laparotomy should be considered primary therapy for perforated NEC. Patients undergoing peritoneal drainage typically experience clinical deterioration after operation. In this limited experience, salvage laparotomy did not appear beneficial.

Evidence for gene transfer and expression of factor IX in haemophilia B patients treated with an AAV vector.

Pre-clinical studies in mice and haemophilic dogs have shown that introduction of an adeno-associated viral (AAV) vector encoding blood coagulation factor IX (FIX) into skeletal muscle results in sustained expression of F.IX at levels sufficient to correct the haemophilic phenotype. On the basis of these data and additional pre-clinical studies demonstrating an absence of vector-related toxicity, we initiated a clinical study of intramuscular injection of an AAV vector expressing human F.IX in adults with severe haemophilia B. The study has a dose-escalation design, and all patients have now been enrolled in the initial dose cohort (2 x 10(11) vg/kg). Assessment in the first three patients of safety and gene transfer and expression show no evidence of germline transmission of vector sequences or formation of inhibitory antibodies against F.IX. We found that the vector sequences are present in muscle by PCR and Southern-blot analyses of muscle biopsies and we demonstrated expression of F.IX by immunohistochemistry. We observed modest changes in clinical endpoints including circulating levels of F.IX and frequency of FIX protein infusion. The evidence of gene expression at low doses of vector suggests that dose calculations based on animal data may have overestimated the amount of vector required to achieve therapeutic levels in humans, and that the approach offers the possibility of converting severe haemophilia B to a milder form of the disease.

Effects of nitroglycerin and indomethacin on fetal-maternal circulation and on fetal cerebral blood flow and metabolism in sheep.

OBJECTIVE: Our purpose was to evaluate the effects of maternal administration of nitroglycerin and indomethacin on maternal and fetal hemodynamics and on fetal cerebral blood flow and metabolism in sheep. STUDY DESIGN: Invasive vascular and fetal carotid flow monitoring was established in 12 gravid ewes. Isotonic sodium chloride solution, nitroglycerin, and indomethacin were infused maternally, and maternal and fetal heart rate, blood pressure, blood gas values, fetal carotid blood flow, and flow variability were measured. Fetal cerebral uptake of oxygen, glucose, and lactate were calculated. RESULTS: Nitroglycerin infusion caused a significant increase in maternal and fetal heart rate and a significant decrease in maternal and fetal mean arterial pressure at a dosage of 10 microram/kg per minute, without a change in blood gas values. Neither drug had any effect on fetal carotid blood flow, flow variability, or cerebral substrate metabolism. CONCLUSION: Maternal administration of nitroglycerin and indomethacin caused no adverse maternal or fetal circulatory changes and did not alter fetal carotid blood flow or substrate metabolism.

Papillary cystic neoplasm of the pancreas: a report of three pediatric cases and literature review.

BACKGROUND/PURPOSE: Papillary cystic neoplasms are rare pancreatic tumors that typically present in women in their third decade of life. Few cases have been reported in children. METHODS/RESULTS: The authors report on three pediatric patients: a 10-year-old boy, an 11-year-old girl, and a 14-year-old girl. The authors have reviewed the existing literature on papillary cystic neoplasms of the pancreas and suggest that these tumors probably arise early in life, grow slowly, and metastasize infrequently. CONCLUSION: Even when these tumors metastasize, patients seldom die as a result of the malignancy.

Congenital diaphragmatic hernia associated with aortic coarctation.

Congenital diaphragmatic hernia (CDH) may be associated with other anomalies, most frequently cardiovascular in nature. Despite fetal echocardiography, diagnosis of an accompanying cardiac malformation often is not made until after birth and sometimes not until after extracorporeal membrane oxygenation (ECMO) has been instituted. Aortic coarctation associated with CDH may occur as an isolated, surgically correctable malformation or it may be a component of the usually fatal left heart "hypoplasia" or "smallness" syndrome. The authors present two cases of aortic coarctation associated with CDH requiring ECMO that illustrate the management challenges of these coincident diagnosis. In one case, the accompanying coarctation was suspected and required precannulation angiography for confirmation, whereas in the other case, the diagnosis of coarctation was not made until after ECMO cannulation. Depending on its anatomic location and severity, an aortic coarctation associated with life-threatening CDH may limit the physiological efficacy of venoarterial ECMO. Furthermore, arterial cannulation for extracorporeal support requires that flow through the remaining carotid artery be maintained during aortic reconstruction, which may prove difficult for lesions best treated by subclavian flap angioplasty. When the diagnosis of coincident aortic coarctation and CDH is suspected or proven before institution of extracorporeal support, serious consideration should be given to venovenous bypass, because this may provide better postductal oxygenation and facilitate aortic repair with the option of left carotid artery inflow occlusion.

Fetendo-clip: a fetal endoscopic tracheal clip procedure in a human fetus.

Fetal tracheal occlusion accelerates lung growth and corrects the often fatal pulmonary hypoplasia seen in fetuses with congenital diaphragmatic hernia. Fetoscopy presents a unique opportunity to glimpse into the world of the fetus, but its use, until recently, has been limited to diagnostic and simple procedures. Using fetoscopic techniques ("Fetendo"), we now report successful tracheal occlusion in a 30-week-old fetus for the treatment of congenital diaphragmatic hernia.

Use of Doppler ultrasonography in the evaluation of liver blood flow during silo reduction of a giant omphalocele.

Despite the utility of prosthetic silo reduction techniques, surgical treatment of giant omphaloceles containing herniated liver may result in acute compromise of hepatic vascular flow. The authors report a case of an infant with a giant omphalocele containing liver, in whom hepatic vascular compromise was suspected during the course of prosthetic visceral reduction. Doppler ultrasonography performed on the herniated liver through the prosthetic silo demonstrated triphasic vascular flow in the liver as well as normal hepatic venous flow and vena caval flow, and permitted continued gradual visceral reduction and a safe, delayed primary abdominal wall closure on the seventh day of life. Doppler ultrasonography performed through the silo may be an important diagnostic adjunct during the visceral reduction phase of staged abdominal wall closure in infants with giant omphaloceles.