Outcomes of congenital diaphragmatic hernia in the modern era of management.

OBJECTIVE: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis. RESULTS: Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. CONCLUSIONS: Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Fulminant pH1N1-09 influenza-associated myocarditis in pediatric patients.

OBJECTIVE: To report an atypical presentation of pH1N1-09 influenza infection in children as fulminant myocarditis and tamponade and the successful treatment with extracorporeal membrane oxygenation. DESIGN: Case report. SETTING: Pediatric cardiac intensive care unit in a quarternary care children's hospital. PATIENTS: Two girls, 5 and 7 yrs of age, infected with pH1N1-09 influenza virus who presented in cardiogenic shock with a pericardial effusion and echocardiographic evidence of tamponade from fulminant myocarditis. INTERVENTIONS: Both patients received a pericardiocentesis. One was managed with multiple, high-dose inotropic agents, whereas the other required institution of extracorporeal membrane oxygenation. MEASUREMENTS AND MAIN RESULTS: Acute respiratory distress syndrome is the major reported clinical manifestation of pH1N1-09 influenza virus infection in hospitalized pediatric patients. In this report we describe two children with confirmed pH1N1-09 influenza infection that required intensive care for fulminant myocarditis. Neither patient had the typical symptoms of influenza-like illness, respiratory compromise, or evidence of pulmonary involvement. One child required extracorporeal membrane oxygenation. Both children survived to hospital discharge. CONCLUSIONS: pH1N1-09 influenza infection can cause fulminant myocarditis in the healthy pediatric population. The clinical presentation may be nonspecific, and the lack of pulmonary symptoms may make diagnosis difficult. Extracorporeal membrane oxygenation support may offer an effective bridge to the recovery of heart function.

The role of fiberoptic endoscopy in the evaluation and management of long gap isolated esophageal atresia.

Accurate measurement of gap length is useful for operative planning in cases of esophageal atresia (EA) without distal fistula. This paper demonstrates how fiberoptic endoscopy of the distal esophagus enables measurement of the gap in the case of isolated EA, and compares other commonly practiced techniques.

Surgical correction of pectus excavatum.

Pectus deformities arise from overgrowth of the cartilagenous portion of the ribs. Traditional resective/open procedures have been almost entirely replaced by video assisted retrosternal bar placement with excellent outcomes. This review considers the comprehensive evaluation and care of a pectus patinet as well as technical details regarding conduct of the surgery.

First employment characteristics for the 2011 pediatric surgery fellowship graduates.

PURPOSE: Information regarding initial employment of graduating pediatric surgery fellows is limited. More complete data could yield benchmarks of initial career environment. METHODS: An anonymous survey was distributed in 2011 to 41 pediatric surgery graduates from all ACGME training programs interrogating details of initial positions and demographics. RESULTS: Thirty-seven of 41 (90%) fellows responded. Male to female ratio was equal. Graduates carried a median debt of $220,000 (range: $0-$850,000). The majority of fellows were married with children. 70% were university/hospital employees, and 68% were unaware of a business plan. Median starting compensation was $354,500 (range: $140,000-$506,000). Starting salary was greatest for >90% clinical obligation appointments (median $427,500 vs. $310,000; p=0.002), independent of geographic location. Compensation had no relationship to private practice vs. hospital/university/military position, coastal vs. inland location, and practice sites number. Median clinical time was 75% and research time 10%. 49% identified a formal mentor. Graduates covered 1-5 different offices (median 1) and 1-5 surgery sites (median 2). 60% were satisfied with their compensation. CONCLUSION: Recent pediatric surgery graduates are engaged mainly in clinical care. Research is not incentivized. Compensation is driven by clinical obligations. Graduates have limited knowledge of the business plan supporting their compensation, nature of malpractice coverage, and commitments to resources including research. Graduates have important fiscal and parenting obligations.

Trends in operative experience of pediatric surgical residents in the United States and Canada.

PURPOSE: Expansion of the number of training programs in pediatric surgery occurred from 2003 through 2010. We sought to determine the effect of program expansion on case volume and distribution of operative experience. METHODS: Public domain data on pediatric surgery resident summary statistics available from the Accreditation Council for Graduate Medical Education (ACGME) from July 2003 through June 2010 were analyzed. Total case volume as primary surgeon or teaching assistant, mean case volume per resident, standard deviation, mode, minimum, and maximum number of cases per resident were evaluated. Mean total cases per resident, minimally invasive laparoscopic and thoracoscopic cases, and requisite cases as defined by the ACGME categories of: tumor, important pediatric surgical, and neonatal cases were analyzed by a Cuzick Wilcoxon-type nonparametric trend statistic using a significance level of 0.05. Skew was assessed by Pearson coefficient with levels of -0.5 to 0.5 defining a parametric distribution. RESULTS: The number of pediatric surgical training residents increased by 42% during the years reported, from 24 to 34. No statistically significant difference was found in the mean number of total cases or requisite cases per resident. The mean volume of minimally invasive procedures increased significantly. Case volume per resident was non-parametrically distributed with increasing positive skew over time. CONCLUSIONS: The increase in number of pediatric surgical resident training positions has not adversely affected overall operative experience or exposure to highly specialized requisite cases, on average. The increasing positive skew of total and index cases, however, suggests that variability between programs in case exposure is increasing over time.

Lung to head ratio in infants with congenital diaphragmatic hernia does not predict long term pulmonary hypertension.

PURPOSE: Lung-to-head ratio (LHR) has been used for antenatal evaluation of infants with congenital diaphragmatic hernia (CDH). We hypothesized that LHR was predictive of acute and chronic pulmonary hypertension in infants with CDH. METHODS: Echocardiograms on all inborn infants with CDH (December 2001-March 2011) were reviewed. Echocardiograms at 1 and 3 months post-repair and most recent follow-up were assessed for presence of pulmonary hypertension (PAH). LHR, gestational age, birth weight, extracorporeal membrane oxygenation (ECMO), and death rate were obtained. Bivariate and multivariate analyses were performed. RESULTS: 106 infants with CDH had LHR obtained at median 28 weeks gestation (median LHR=1.25 [range 0.4-5.3]). Median follow-up was 26.6 months (range 4.6-97.5). The long-term incidence of pulmonary hypertension was 16%. LHR was significantly associated with pulmonary hypertension at one month (p=0.0001) but not at 3 months (p=0.22) or long-term (p=0.54). LHR was predictive of ECMO use (p=0.01) and death (p=0.001). CONCLUSIONS: The overall incidence of PAH in infants with CDH decreases over time. Prenatal LHR predicts PAH at one month but not long-term in infants with CDH. The ability for LHR to predict PAH at one month but not long term may suggest remodeling of the pulmonary vasculature over time.

Developmental outcomes of children with congenital diaphragmatic hernia: a multicenter prospective study.

PURPOSE: To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age. METHODS: This is a multicenter prospective study of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age. RESULTS: BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors. CONCLUSION: CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays.

Congenital diaphragmatic hernia and protective ventilation strategies in pediatric surgery.

Infants affected with congenital diaphragmatic hernias (CDH) suffer from some degree of respiratory insufficiency arising from a combination of pulmonary hypoplasia and pulmonary hypertension. Respiratory care strategies to optimize blood gasses lead to significant barotrauma, increased morbidity, and overuse of extracorporeal membrane oxygenation (ECMO). Newer permissive hypercapnia/spontaneous ventilation protocols geared to accept moderate hypercapnia at lower peak airway pressures have led to improved outcomes. High-frequency oscillatory ventilation can be used in infants who continue to have persistent respiratory distress despite conventional ventilation. ECMO can be used successfully as a resuscitative strategy to minimize further barotrauma in carefully selected patients.

Prenatal lung-head ratio: threshold to predict outcome for congenital diaphragmatic hernia.

OBJECTIVE: The literature suggests that lung-head ratio (LHR) and liver position may inconsistently predict outcome for congenital diaphragmatic hernia (CDH). We reviewed our inborn neonates with isolated left-sided CDH to determine whether these variables predicted survival and to estimate the optimal LHR threshold. METHODS: Prenatal LHR and liver position were obtained from 2002 to 2009. The primary endpoint was survival. RESULTS: LHR was greater in survivors after adjusting for gestational age (median 1.40 versus 0.81; p < 0.001). LHR demonstrated excellent diagnostic discrimination, with area under receiver operating characteristic (ROC) curve 0.93 (95% CI 0.86-0.99). LHR threshold of 1.0 was 83% sensitive and 91% specific in predicting survival. An optimal LHR threshold of 0.85 predicted survival with 95% sensitivity and 64% specificity, reducing false negatives (survivors with low LHR). LHR > 0.85 predicted survival after adjustment for gestational age (OR = 33.6, 95% CI = 5.4-209.5). Liver position did not predict survival. CONCLUSIONS: Prenatal LHR >0.85 predicts survival for infants with isolated left-sided CDH without compromising discrimination of survivors from non-survivors. The diagnostic utility of LHR may be confounded by gestational age at measurement. Stringent LHR threshold may minimize false-negative attribution and improve utility of this measurement as predictor of survival.

Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study.

INTRODUCTION: Experience in thoracoscopic congenital diaphragmatic hernia (CDH) repair has expanded, yet efficacy equal to that of open repair has not been demonstrated. In spite of reports suggesting higher recurrent hernia rates after thoracoscopic repair, this approach has widely been adopted into practice. We report a large, single institutional experience with thoracoscopic CDH repair with special attention to recurrent hernia rates. METHODS: We reviewed the records of neonates with unilateral CDH repaired between January 2006 and February 2010 at Morgan Stanley Children's Hospital. Completely thoracoscopic repairs were compared to open repairs of the same period. In addition, successful thoracoscopic repairs were compared with thoracoscopic repairs that developed recurrence. Data were analyzed by Mann-Whitney U and Fisher exact tests. RESULTS: Thirty-five neonates underwent attempted thoracoscopic repair, with 26 completed. Concurrently, 19 initially open CDH repairs were performed. Preoperatively, patients in the open repair group required more ventilatory support than the thoracoscopic group. Recurrence was higher after thoracoscopic repair (23% vs 0%; P = .032). In comparing successful thoracoscopic repairs to those with recurrence, none of the factors analyzed were predictive of recurrence. CONCLUSIONS: Early recurrence of hernia is higher in thoracoscopic CDH repairs than in open repairs. Technical factors and a steep learning curve for thoracoscopy may account for the higher recurrence rates, but not patient severity of illness. In an already-tenuous patient population, performing the repair thoracoscopically with a higher risk of recurrence may not be advantageous.

University pediatric surgery: benchmarking performance.

The many ways monies enter and leave a university pediatric section are as poorly understood as the value and relationship to the parent hospital or university. This blinded confidential financial performance survey of similar university pediatric surgery sections begins to benchmark performance and define those relationships.

Evaluating cannulation strategies used during second courses of extracorporeal membrane oxygenation in a large cohort of pediatric patients.

PURPOSE: After a successful course of extracorporeal membrane oxygenation (ECMO), patients can deteriorate and a second ECMO course may be contemplated. When a second ECMO course becomes necessary in pediatric patients, survival rates comparable to the first ECMO course are possible. The perceived difficulties involved in recannulation after an initial ECMO course can prevent clinicians from reliably offering a second ECMO run to an eligible pediatric patient. We hypothesized that national ECMO registry data could provide cannulation templates for pediatric patients requiring a second ECMO course. METHODS: We obtained data from the Extracorporeal Life Support Organization registry (1981-2007) on patients 1 to 18 years old who required single-run ECMO (SRE) or multiple-run ECMO (MRE). Primary outcome measures were complications and survival. Cannulation-specific variables were compared using chi(2) methods (Fisher exact, McNemar's). Statistical significance was assumed at P < .05. RESULTS: A total of 3810 (96.8%) children underwent SRE and 127 (3.2%) required MRE. Survival was similar in both groups (49% vs 44%; P = .28). Cannulation data were available in 2539 SRE (67%) and 88 MRE (69%) cases. Compared with SRE, first ECMO courses in MRE patients consisted of fewer cervical (52.3% vs 71.7%; P < .001) but more femoral (20.5% vs 10.7%; P = .01) and central (27.3% vs 17.6%; P = .02) cannulations. In MRE patients, central cannulation was more frequent in second vs first ECMO courses (43.0% vs 27.3%; P = .03). Multiple-run ECMO survival was unaffected by cannulation strategy. Multiple-run ECMO patients with unchanged cannulation sites between first and second ECMO courses had fewer total complications than those requiring new cannulation sites (3.7 vs 5.1; P = .04). CONCLUSIONS: Second ECMO courses in pediatric patients can achieve survival comparable to the first course, but more often require central cannulation. Reusing cannulation sites for a second ECMO course is associated with fewer total complications than cannulating at new sites. These data provide guidance when considering cannulation strategies for second ECMO courses in pediatric patients.

Percutaneous distal perfusion of the lower extremity after femoral cannulation for venoarterial extracorporeal membrane oxygenation in a small child.

Femoral cannulation in pediatric patients requiring extracorporeal membrane oxygenation (ECMO) is commonly associated with distal limb ischemia. Authors have previously reported successful lower limb perfusion using various open techniques to cannulate a distal lower extremity artery at the time of initial ECMO cannulation. These procedures include open femoral artery antegrade cannulation and distal posterior tibial artery retrograde cannulation in older children and adults. Such approaches require ample vessel diameters to accommodate an arteriotomy and catheter insertion and, therefore, are of limited use in smaller children. We hypothesized that after femoral artery cannulation for ECMO, a percutaneous technique of distal limb perfusion might offer unique advantages when treating lower extremity ischemia in small pediatric patients. We report a technique for percutaneous antegrade cannulation in a 4-year-old patient shortly after her primary cannulation for venoarterial ECMO via the femoral artery.

Multivariate model for predicting recurrence in congenital diaphragmatic hernia.

PURPOSE: Risk factors that predispose children with congenital diaphragmatic hernia (CDH) to recurrence remain poorly defined. We report a large series of recurrent CDH and ask whether prenatal patient factors or postnatal treatment variables better predict recurrence. METHODS: Two hundred thirty-eight neonates with unilateral CDH underwent repair from 1990 to 2006. Data were assessed by chi(2) and Mann-Whitney U tests. Multivariate regression identified independent predictors of recurrence. Statistical significance was set at P < .05. RESULTS: We identified 24 recurrences (10%). Median time from repair to recurrence diagnosis was 4.9 months. Patients with recurrence were older (P = .02) and more often required abdominal wall patches at initial repair (P = .01) compared to nonrecurrence patients. Postoperative length of stay (LOS) after initial repair (P < .01) and morbidity (P = .01) were greater in recurrence patients. Use of diaphragm patch at initial repair was greater in patients with recurrence but only approached statistical significance (P = .05). Only 2 variables independently predicted recurrence by multivariate regression as follows: abdominal (not diaphragm) wall patch during initial repair (odds ratio [OR] 3.50; P = .04) and postoperative LOS (OR, 1.012; P = .01). CONCLUSION: Neonates at risk for CDH recurrence are better identified by postnatal treatment variables than by prenatal patient factors. Although age at repair and diaphragm patch use are greater in recurrence patients, the only factors to independently predict recurrence were postoperative LOS and abdominal wall patch use. These data can help optimize follow-up regimens.

Extracorporeal membrane oxygenation for cardiopulmonary failure in pediatric patients: is a second course justified?

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is accepted therapy for cardiorespiratory failure. Even after a successful ECMO course, patient deterioration may occur and a second course of ECMO may be contemplated. Although data regarding second ECMO courses exist in neonates, there are no reports describing second ECMO courses in pediatric patients. We hypothesized that data from a national ECMO registry would be useful in identifying which pediatric patients would be optimal candidates for a second course of ECMO. METHODS: We obtained data from the national Extracorporeal Life Support Organization registry from 1981 to 2007 on all patients 1-18 years old who required single-run ECMO (SRE) or multiple-run ECMO (MRE). Primary outcome measures were complications and survival. Continuous variables were assessed for distribution normality by using a Shaprio-Wilk statistic to guide nonparametric testing. SRE and MRE patients were compared by using chi2 tests (Fisher's exact and McNemar's) to assess differences in categorical variables; continuous data were assessed by using Mann-Whitney U or Wilcoxon signed-rank testing. Two multivariate regression models were constructed to identify independent predictors of survival and complications in MRE patients. Statistical significance was assumed at P < 0.05. RESULTS: A total of 3937 pediatric patients received ECMO for cardiac or respiratory failure. Of them, 3810 (96.8%) children underwent a single course of ECMO, whereas 127 (3.2%) required multiple ECMO runs. Compared with SRE patients, the first ECMO course in MRE patients was notable for higher rates of cardiac ECMO (61% versus 44%, P < 0.001), venoarterial ECMO (88% versus 78%, P = 0.04), and central cannulation (28% versus 17%, P = 0.007). There was no survival difference between MRE and SRE patients (44% versus 49%, P = 0.28). Median time between MRE courses was 9.0 days (interquartile range = 5-20 days). The mean number of complications per MRE patient was higher in the second ECMO run compared with the first (3.93 versus 3.12, P = 0.008). Multivariate regression identified 2 variables as independent predictors of survival in MRE patients: (1) renal complications during first ECMO run (P = 0.04); and (2) total number of complications during second ECMO run (P = 0.005). A separate multivariate analysis identified 3 variables independently predictive of complications in MRE patients: (1) age (P < 0.001); (2) duration of second run (P < 0.001); and (3) total number of complications during first ECMO run (P < 0.001). CONCLUSIONS: ECMO therapy achieves 49% survival in children 1-18 years of age. When a second ECMO course becomes necessary, survival rates comparable to the first ECMO course are possible. Patients developing renal complications during their first ECMO course have worse outcome with a second ECMO course. Patients are at greater risk for complications during a second ECMO course if they experience a high number of first-run complications, are >3 years old, or undergo a prolonged second ECMO course. These data are useful when deciding whether to offer a second ECMO course to an eligible pediatric patient.

Redefining outcomes in right congenital diaphragmatic hernia.

PURPOSE: Available data comparing the management and outcome of right-sided (R-CDH) vs left-sided congenital diaphragmatic hernia (L-CDH) are inconsistent. Large-volume CDH studies are limited by small numbers of R-CDH or are confounded by compilations from multiple institutions with multiple treatment strategies. Consequently, they are underpowered to draw conclusions. To define the behavior and outcomes of R-CDH better, we report the largest single-institution series of R-CDH and ask if factors traditionally linked to poor prognosis in L-CDH were applicable to R-CDH. METHODS: We reviewed a single institution's experience with 267 consecutive evaluable neonates with unilateral CDH repaired from 1990 to 2006, with specific focus on R-CDH. chi(2) tests were performed for disease-related categorical variables. Two-tailed unpaired t tests were used for continuous variables. Factors associated with morbidity and survival were determined by univariate regression. Statistical significance was set at P < .05. RESULTS: Forty right-sided (15%) and 227 (85%) left-sided cases of CDH were identified. Prenatal diagnosis was made in 20 right-sided vs 170 left-sided defects (50% vs 75%, P < .01). Survival was 22 of 40 in R-CDH compared with 175 of 227 in L-CDH (55% vs 77%, P < .01). Extracorporeal membrane oxygenation was required in 16 right-sided and 33 left-sided cases (40% vs 15%, P < .001). A diaphragmatic patch was used in 22 of 29 right-sided compared with 82 of 199 left-sided repairs (76% vs 41%, P < .01); rates of abdominal wall prosthesis were also higher in right-sided hernias (38% vs 19%, P < .05). No differences were detected in right-sided vs left-sided recurrences (14% vs 8%, P = .38), mean time from birth to operation (5.3 vs 4.8 days, P = .80), or presence of cardiac anomalies (15% vs 12%, P = .63). Morbidity persisting beyond 6 months of age was present in 16 of 22 R-CDH survivors compared with 76 of 175 L-CDH survivors (73% vs 43%, P > .05). Among R-CDHs, prenatal diagnosis was the only factor to predict survival by univariate regression (P < .01). Use of a prosthesis in the diaphragm (P < .05) for R-CDH repair correlated with morbidity. CONCLUSION: Although previous reports suggest that associated anomalies, need for extracorporeal membrane oxygenation, and time to repair can influence L-CDH survival, these data do not support extrapolation to R-CDH survival. Right-sided CDH carries a disproportionately high morbidity and mortality. Prenatal diagnosis was the only factor predictive of R-CDH survival. Morbidity may correlate with use of prosthetic material for R-CDH repair. Right-sided CDH is a unique disease that may require a modified antenatal consultation.