Kurt F. Heiss

Reversal of mortality for congenital diaphragmatic hernia with ECMO.

Extracorporeal Membrane Oxygenation (ECMO) has been available to neonates with respiratory failure at the University of Michigan School of Medicine since June 1981. In order to evaluate the impact of this type of pulmonary support, a retrospective analysis of 50 neonates with posterolateral congenital diaphragmatic hernia (CDH) who were symptomatic during the first hour of life and were treated between June 1974 and December 1987 was carried out. The patients were divided into two groups, those treated before June 1981 (16 patients) and those treated after June 1981 (34 patients). Overall survival improved from 50% (eight of 16 patients) during the pre-ECMO era to 76% (26 of 34 patients) during the post-ECMO period (p = 0.06). During the period after June 1981, 21 neonates were unresponsive to conventional therapy and were therefore considered for ECMO. Failure of conventional therapy was defined as acute clinical deterioration with an expected mortality of greater than 80% based on an objective formula previously reported. Six patients were excluded on the basis of specific contraindications to ECMO. Thirteen of 15 infants (87%) supported with ECMO survived. Three patients treated before 1981 met criteria for ECMO; all three died while receiving treatment using conventional therapy. These survival differences are significant (p less than 0.01). In addition, the survival of 87% for the infants treated with ECMO versus the expected mortality of greater than 80% for these same patients when treated with conventional therapy is highly significant (p less than 0.005). Based on this data, ECMO appears to be a successful, reliable, and safe method of respiratory support for selected, critically ill infants with CDH.

American College of Surgeons National Surgical Quality Improvement Program Pediatric: A beta phase report

PURPOSE The American College of Surgeons (ACS) National Surgical Quality Improvement Program Pediatric (NSQIP-P) expanded to beta phase testing with the enrollment of 29 institutions. Data collection and analysis were aimed at program refinement and development of risk-adjusted models for inter-institutional comparisons. METHODS Data from the first full year of beta-phase NSQIP-P were analyzed. Patient accrual used ACS-NSQIP methodology tailored to pediatric specialties. Preliminary risk adjusted modeling for all pediatric and neonatal operations and pediatric (excluding neonatal) abdominal operations was performed for all cause morbidity (other than death) and surgical site infections (SSI) using hierarchical logistic regression methodology and eight predictor variables. Results were expressed as odds ratios with 95% confidence intervals. RESULTS During calendar year 2010, 29 institutions enrolled 37,141 patients. 1644 total CPT codes were entered, of which 456 accounted for 90% of the cases. 450 codes were entered only once (1.2% of cases). For all cases, overall mortality was 0.25%, overall morbidity 7.9%, and the SSI rate 1.8%. For neonatal cases, mortality was 2.39%, morbidity 18.7%, and the SSI rate 3%. For the all operations model, risk-adjusted morbidity institutional odds ratios ranged 0.48-2.63, with 9/29 hospitals categorized as low outliers and 9/29 high outliers, while risk-adjusted SSI institutional odds ratios ranged 0.36-2.04, with 2/29 hospitals low outliers and 7/29 high outliers. CONCLUSION This report represents the first risk-adjusted hospital-level comparison of surgical outcomes in infants and children using NSQIP-P data. Programmatic and analytic modifications will improve the impact of this program as it moves into full implementation. These results indicate that NSQIP-P has the potential to serve as a model for determining risk-adjusted outcomes in the neonatal and pediatric population with the goal of developing quality improvement initiatives for the surgical care of children.

Severe myocardial dysfunction during extracorporeal membrane oxygenation.

Of the 102 neonates with respiratory failure supported with extracorporeal membrane oxygenation (ECMO) at this institution between 1984 and 1987, 8 patients developed severe myocardial dysfunction that was noted shortly after onset of bypass. The neonates in the cardiac dysfunction group were more hypoxic (average PaO2 = 26 +/- 8 mm Hg v 41 +/- 19 mm Hg, P less than .01) in the immediate pre-ECMO period. Seventy-five percent were unstable hemodynamically (6 hypotensive, 3 bradycardic, 2 sustained cardiac arrest, 4 required epinephrine pressor support). On ECMO, 5 of the 8 neonates developed an ischemic cardiomyopathy that lasted for less than 24 hours and resolved without therapeutic intervention. In the other 3 cases, prolonged periods of dysfunction were noted and afterload reduction through administration of tolazoline or hydralazine was beneficial. These 8 patients serve to demonstrate the reversible nature of postischemic cardiac dysfunction in patients on ECMO and in the neonatal population in general.

Antireflux Surgery Outcomes in Pediatric Gastroesophageal Reflux Disease

OBJECTIVES:Antireflux surgery is performed frequently in children with gastroesophageal reflux disease (GERD). Few comparative studies exist which assess the indications for and short- or long-term outcome of open Nissen fundoplication (ONF) and laparoscopic Nissen fundoplication (LNF) for pediatric GERD. We investigated the frequency of reoperation and factors that might influence its occurrence.METHODS:We performed a retrospective, follow up cohort study of all children ≤5 years, who underwent LNF or ONF at our institution from January 1, 1997 to December 31, 2002, where five pediatric surgeons perform fundoplication. Mean follow up time was 36.2 months. The following information was obtained: surgical indications, hospital course data, and long-term surgical outcomes. Data were analyzed using univariate and multiple logistic regressions.RESULTS:Overall, 456 (150 [32.9%] ONF vs. 306 [67.1%] LNF) cases were analyzed. Reoperation was performed in 55 (12.06%), LNF 43 (14.05%), and ONF 12 (8%). The mean interim to reoperation for LNF was 11 months compared to 17 months for ONF (P = 0.007). The reoperation rate at 12 and 24 months were 10.5%, 13.4% and 4%, 6.7% respectively, when LNF was compared to ONF (P = 0.01). The multivariate analysis showed that initial LNF and prematurity were the main predictors for reoperation.CONCLUSIONS:The majority of reoperations for both LNF and ONF occurred in the first year after initial operation; LNF had a significantly higher reoperation rate than ONF. The probability of reoperation for LNF and ONF increases with the presence of comorbidities, especially prematurity and chronic respiratory conditions.

Efficacy of venovenous extracorporeal membrane oxygenation for neonates with respiratory and circulatory compromise

We report a 12-month experience at Egleston Childrens Hospital in Atlanta, Ga., with a protocol under which venovenous extracorporeal membrane oxygenation (ECMO) was used instead of venoarterial ECMO. Fifty-five newborn infants were referred for ECMO, four of whom had disqualifying conditions (all four died). Thirty-one infants were supported without recourse to ECMO, one of whom died. Of the 20 remaining patients, three were placed on a venoarterial ECMO regimen because of our early uncertainty about the efficacy of venovenous ECMO or because of technical constraints. All other patients (n = 17), including three with congenital diaphragmatic hernia, were supported with venovenous perfusion. No patient begun on a venovenous ECMO regimen required conversion to venoarterial bypass. Before ECMO, venovenous patients required an average dopamine dose of 16 micrograms/kg per minute and an average dobutamine dose of 6 micrograms/kg per minute. Of 15 patients studied before ECMO, three had significantly impaired contractility, and all had evidence of pulmonary hypertension on an echocardiogram. Mean blood pressure did not change while heart rate fell from 172 to 146 beats/min during the first 2 hours of ECMO and vasoactive drug doses were reduced. Of the 17 venovenous ECMO patients, 15 (88%) survived. We conclude that neonatal patients with severe hypoxia and substantial circulatory compromise can be effectively supported by venovenous ECMO in most cases.

Thrombosis of the portal venous system after splenectomy for pediatric hematologic disease

Splenic, portal, or mesenteric venous thrombosis after splenectomy for hematologic disease has not been reported in the pediatric literature. It is a rare complication associated with significant morbidity and mortality in adult reports. Between 1981 and 1991, 3 patients (13-year-old boy with hereditary elliptocytosis [HE], 13-year-old boy with thalassemia intermedia [TI], and 18-year-old girl with idiopathic thrombocytopenic purpura [ITP]) presented with abdominal pain, nausea, with or without fever, at 4, 11, and 13 days postsplenectomy, respectively. Abdominal Doppler ultrasound (US) and/or computed tomography (CT) showed: (1) an intraluminal filling defect with partial obstruction to flow in the right branch of the portal vein with the remaining vessels patent (HE); (2) splenic vein thrombosis with complete occlusion of the main portal vein and proximal superior mesenteric vein (TI); and (3) complete thrombosis of the splenic vein, proximal superior mesenteric vein and portal vein (including central radicles), with retrogastric collateralization (ITP). Subsequent imaging showed either complete resolution of vascular obstruction on no treatment (patient 1), or portal venous cavernomatous transformation with hepatofugal flow after 6 months of systemic anticoagulation (patients 2 and 3), and all 3 patients are currently asymptomatic. Postoperative sonographic evaluation of a consecutive series of pediatric splenectomies for hematologic disease (n = 16), was performed at a median of 51 days (range, 3 to 124). This demonstrated one case of asymptomatic left portal venous thrombosis with subsequent recanalization in the absence of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Pure esophageal atresia: Outlook in the 1990s

Eleven newborns with pure esophageal atresia were treated between 1980 and 1989 inclusive; there were six girls and five boys. Their gestational age ranged from 31 to 40 weeks (average, 37 weeks) and weight from 1.1 to 3.0 kg (average, 2.2). The only associated anomalies were Downs syndrome, respiratory distress syndrome, and patent ductus arteriosus. All babies received an immediate gastrostomy. Several radiologic studies were done to see if the distance between the two esophageal pouches was decreasing. Dilatations of the upper pouch were carried out in two patients. After a wait of 1 to 7 months (average, 3 1/2) a primary anastomosis was attempted; the weight of six babies doubled during this time. Eight neonates had a primary repair (two were aided by a circular myotomy). Two had a staged gastric tube constructed, and one baby had a gastric pull-up procedure. Three of the infants with a primary anastomosis required a subsequent antireflux operation, and one needed her anastomosis resected 16 months later. Ten of these 11 newborns are alive and well; one of the gastric tube children died from an adhesive small bowel obstruction at age four years.(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal life support for support of children with malignancy and respiratory or cardiac failure : The extracorporeal life support experience

Background: Extracorporeal life support (ECLS) is a means of respiratory and hemodynamic support for patients failing conventional therapies. Children with cancer who develop complications during therapy may require ECLS. Methods: The extracorporeal life support organization (ELSO) registry was queried for all patients younger than 21 years with an International Classification of Diseases, Ninth Revision diagnosis of neoplasm. ELSO centers were also asked whether patients with neoplasms should be offered ECLS. Results: From 1994 to 2007, 107 children met inclusion criteria: 73 with hematologic malignancies and 34 with solid tumors. The median age was 3.71 years. Patients had a total of 112 ECLS runs (five patients had two runs). Patients required ECLS primarily for pulmonary support (n = 86). Median duration of ECLS was 6.1 days. Overall, 62 of 107 patients (58%) died while on ECLS because of irreversible organ failure (n = 37), diagnosis incompatible with life (n = 11), hemorrhage (n = 6), and family request (n = 8). Forty-five of 107 patients (42%) survived ECLS. During the remaining hospitalization, eight patients died resulting in 37 of 107 surviving to hospital discharge (35%). Risk factors for death included lower Po2 before ECLS, higher oxygen index, higher positive end-expiratory pressure, and development of renal or cardiopulmonary complications (p < 0.05). No differences in outcomes were noted in patients with either hematologic malignancies or solid tumors. One hundred eighteen of 133 eligible ECLS centers responded to the questionnaire for an 89% response rate. Among those who responded, 112 of 133 would consider placing a child (95%) with a neoplasm on ECLS. Conclusion: Children with cancer and respiratory failure can be offered ECLS with a reasonable expectation for survival. The opinions of the ELSO center suggest that decisions to offer ECLS to a child with malignancy should be made on a case by case basis, with prognosis of the malignancy being an important factor.

Risk-Adjusted Hospital Outcomes for Children’s Surgery

BACKGROUND The American College of Surgeons National Surgical Quality Improvement Program-Pediatric was initiated in 2008 to drive quality improvement in children’s surgery. Low mortality and morbidity in previous analyses limited differentiation of hospital performance. METHODS: Participating institutions included children’s units within general hospitals and free-standing children’s hospitals. Cases selected by Current Procedural Terminology codes encompassed procedures within pediatric general, otolaryngologic, orthopedic, urologic, plastic, neurologic, thoracic, and gynecologic surgery. Trained personnel abstracted demographic, surgical profile, preoperative, intraoperative, and postoperative variables. Incorporating procedure-specific risk, hierarchical models for 30-day mortality and morbidities were developed with significant predictors identified by stepwise logistic regression. Reliability was estimated to assess the balance of information versus error within models. RESULTS: In 2011, 46 281 patients from 43 hospitals were accrued; 1467 codes were aggregated into 226 groupings. Overall mortality was 0.3%, composite morbidity 5.8%, and surgical site infection (SSI) 1.8%. Hierarchical models revealed outlier hospitals with above or below expected performance for composite morbidity in the entire cohort, pediatric abdominal subgroup, and spine subgroup; SSI in the entire cohort and pediatric abdominal subgroup; and urinary tract infection in the entire cohort. Based on reliability estimates, mortality discriminates performance poorly due to very low event rate; however, reliable model construction for composite morbidity and SSI that differentiate institutions is feasible. CONCLUSIONS: The National Surgical Quality Improvement Program-Pediatric expansion has yielded risk-adjusted models to differentiate hospital performance in composite and specific morbidities. However, mortality has low utility as a children’s surgery performance indicator. Programmatic improvements have resulted in actionable data.

Malignant risk in juvenile polyposis coli: Increasing documentation in the pediatric age group☆

The presence of juvenile polyps with resulting bleeding and abdominal pain has traditionally been considered a benign, self-limiting process which would resolve with age. The dictum that these polyps were usually solitary, were found predominantly in the rectosigmoid area, and were without malignant potential has been reconsidered in recent years with the increased use of colonoscopy. Several case reports in both adults and children have documented the presence of adenomatous changes in this syndrome. We report 3 cases of children, ages 3, 11, and 11 who were found to have adenomatous polyps in the midst of fields of juvenile polyps on evaluation for rectal bleeding. All three were treated definitively with endorectal pull-through. Two of these patients had atypia on histological evaluation, one of which was severe. We recommend a more aggressive approach to patients found to have multiple juvenile polyps on barium enema, including colonoscopic biopsies at several sites to determine the presence of adenomatous changes, with colectomy and endorectal pull-through should these be found.