Eighteen years of paediatric extracorporeal membrane oxygenation and ventricular assist devices: insight regarding late outcomes.

BACKGROUND: We reviewed all patients who were supported with extracorporeal membrane oxygenation and/or ventricular assist device at our institution in order to describe diagnostic characteristics and assess mortality. METHODS: A retrospective cohort study was performed including all patients supported with extracorporeal membrane oxygenation and/or ventricular assist device from our first case (8 October, 1998) through 25 July, 2016. The primary outcome of interest was mortality, which was modelled by the Kaplan-Meier method. RESULTS: A total of 223 patients underwent 241 extracorporeal membrane oxygenation runs. Median support time was 4.0 days, ranging from 0.04 to 55.8 days, with a mean of 6.4±7.0 days. Mean (±SD) age at initiation was 727.4 days (±146.9 days). Indications for extracorporeal membrane oxygenation were stratified by primary indication: cardiac extracorporeal membrane oxygenation (n=175; 72.6%) or respiratory extracorporeal membrane oxygenation (n=66; 27.4%). The most frequent diagnosis for cardiac extracorporeal membrane oxygenation patients was hypoplastic left heart syndrome or hypoplastic left heart syndrome-related malformation (n=55 patients with HLHS who underwent 64 extracorporeal membrane oxygenation runs). For respiratory extracorporeal membrane oxygenation, the most frequent diagnosis was congenital diaphragmatic hernia (n=22). A total of 24 patients underwent 26 ventricular assist device runs. Median support time was 7 days, ranging from 0 to 75 days, with a mean of 15.3±18.8 days. Mean age at initiation of ventricular assist device was 2530.8±660.2 days (6.93±1.81 years). Cardiomyopathy/myocarditis was the most frequent indication for ventricular assist device placement (n=14; 53.8%). Survival to discharge was 42.2% for extracorporeal membrane oxygenation patients and 54.2% for ventricular assist device patients. Kaplan-Meier 1-year survival was as follows: all patients, 41.0%; extracorporeal membrane oxygenation patients, 41.0%; and ventricular assist device patients, 43.2%. Kaplan-Meier 5-year survival was as follows: all patients, 39.7%; extracorporeal membrane oxygenation patients, 39.7%; and ventricular assist device patients, 43.2%. CONCLUSIONS: This single-institutional 18-year review documents the differential probability of survival for various sub-groups of patients who require support with extracorporeal membrane oxygenation or ventricular assist device. The indication for mechanical circulatory support, underlying diagnosis, age, and setting in which cannulation occurs may affect survival after extracorporeal membrane oxygenation and ventricular assist device. The Kaplan-Meier analyses in this study demonstrate that patients who survive to hospital discharge have an excellent chance of longer-term survival.

Appendiceal carcinoids in children--management and outcomes.

BACKGROUND: Appendiceal carcinoid tumors are very rare in children, and management has been guided by adult presentations and outcomes. Here, we present our experience with pediatric appendiceal carcinoid tumors. METHODS: We undertook a retrospective review of all cases of appendiceal carcinoids in children over a 20-y period. Data regarding clinical presentation, diagnosis, pathology, follow-up, and outcomes were collected and analyzed. RESULTS: We identified 13 cases of appendiceal carcinoids. All cases were diagnosed after appendectomy for presumed appendicitis (nine acute and four interval;), with no patient having carcinoid syndrome. Mean age at diagnosis was 13.7 y, and all but one case was female. Tumor size ranged from microscopic foci of tumor cells to 2.1 cm (mean, 0.93 cm). Seven cases had invasion of the mesoappendix, three of which underwent a subsequent right hemicolectomy. The patient with the largest tumor (2.1 cm) had evidence of lymphatic invasion with three nodes positive for tumor after right colectomy. No patient had elevation of 5-hydroxyindoleacetic acid or serum chromogranin A, and surveillance computed tomographic scans did not reveal any liver metastases. CONCLUSIONS: This is a relatively large series of carcinoids of the appendix in children. We found no evidence of carcinoid syndrome or metastatic disease in these cases other than lymphatic. The need for a secondary colectomy is perhaps questionable.

Management of electrical and chemical burns in children.

BACKGROUND: Pediatric electrical and chemical burns are rare injuries, and the care of these patients varies significantly. We reviewed our experience in management of electrical and chemical burns to analyze the clinical course, management, and outcomes. METHODS: A retrospective review was conducted on children with chemical and electrical burns presenting to two large regional pediatric burn centers over a 10-y period (2002-2012). Clinical data including patient demographics, nature of burns, management, and outcomes were collected and analyzed. RESULTS: There were 50 cases, 25 chemical and electrical burns each. Overall, the mean±standard deviation age was 6.2±5.6 y, and the mean total body surface area burn was 4.3±3.2%. Chemical burns were larger, had less depth, and shorter length of stay, whereas electrical burns were smaller, deeper, and had a longer length of stay. Two chemical burns and six electrical burns required grafting. Twelve percent of electrical burns required rehabilitation, and 20% required compression garments for hypertrophic scars. Six percent required late surgeries. CONCLUSIONS: Pediatric electric and chemical burns are rare and require specialized care. Graft rates are not high but are mostly noted in electrical burns.

Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific treatment algorithm.

OBJECTIVES: To assess the impact of varying approaches to congenital diaphragmatic hernia (CDH) repair timing on survival and need for ECMO when controlled for anatomic and physiologic disease severity in a large consecutive series of patients with CDH. BACKGROUND: Our publication of 60 consecutive patients with CDH in 1999 showed that survival was significantly improved by limiting lung inflation pressures and eliminating hyperventilation. METHODS: We retrospectively reviewed 268 consecutive patients with CDH, combining 208 new patients with the 60 previously reported. Management and ventilator strategy were highly consistent throughout. Varying approaches to surgical timing were applied as the series matured. RESULTS: Patients with anatomically less severe left liver-down CDH had significantly increased need for ECMO if repaired in the first 48 hours, whereas patients with more severe left liver-up CDH survived at a higher rate when repair was performed before ECMO. Overall survival of 268 patients was 78%. Survival was 88% for those without lethal associated anomalies. Of these, 99% of left liver-down CDH survived, 91% of right CDH survived, and 76% of left liver-up CDH survived. CONCLUSIONS: This study shows that patients with anatomically less severe CDH benefit from delayed surgery whereas patients with anatomically more severe CDH may benefit from a more aggressive surgical approach. These findings show that patients respond differently across the CDH anatomic severity spectrum and lay the foundation for the development of risk-specific treatment protocols for patients with CDH.

Advances in pulmonary management and weaning from ECLS.

ECMO for neonatal and pediatric respiratory failure provides gas exchange to allow lung recovery from reversible pulmonary ailments. This is a comprehensive discussion on the various strategies and advances utilized by pediatric ECLS specialists today. ECMO patients require continual monitoring, serial gasses and radiographs, near-infrared spectroscopy (NIRS - to monitor oxygen delivery to regional tissue beds), and more quality ECLS directed care. As the foundation to lung recovery, good EMCO closely monitors ECLS flow rates, sweep gasses, and membrane lung function. Mixed venous oxygen saturation (Sv02) greater than 65% indicates good oxygen delivery and sweep gas adjustments maintain PaCO2 of 40-45 mm Hg. Lung recovery ventilatory settings do not fully rest the lungs but maintain normal or nontoxic pressure and oxygen levels. Neonatal recovery settings are PIP (cm H20) of 15-20, PEEP of 5-10, ventilator rate of 12-20 and an inspiratory time of 0.5-1 s, and FiO2 of 0.3-0.5. Pediatric recovery settings are PIP (cm H20) < 25, PEEP of 5-15, ventilator rate of 10-20 and an inspiratory time of 0.8-1 s, and FiO2 of <0.5. Some studies demonstrate a higher recovery PEEP level decreases duration of ECMO, but do not demonstrate a mortality difference. Multiple adjunctive therapies such as surfactant, routine pulmonary clearance and respiratory physiotherapy, iNO, prone positioning, bronchoscopy, POCUS, CT imaging, and extubation or "awake ECLS" can significantly affect pulmonary recovery. Patience is necessary as lung recovery may take weeks or even months on the nontoxic settings. On these settings, dynamic recovery will be revealed by improvement in tidal volume, minute ventilation and radiographic pulmonary aeration, prompting discussion about weaning. When this pulmonary compliance recovery becomes evident, decreasing ECLS flow while also decreasing circuit FiO2 and/or sweep gas are common components to ECMO weaning strategies.

"Pulling the plug"--management of meconium plug syndrome in neonates.

BACKGROUND: The significance of meconium plug syndrome (MPS) is unclear but has been associated with Hirschsprung's disease and magnesium tocolysis. We reviewed our experience to attempt to identify any potential association with these conditions and to review our outcomes. METHODS: Using the International Classification of Diseases, Ninth revision, code for meconium obstruction, patient charts were identified during the 1998-2008 period. A total of 61 cases of MPS were found, after excluding 7 of meconium ileus. Data regarding the hospital course and outcomes were collected and analyzed. RESULTS: Approximately 30% of patients had spontaneous resolution of the meconium plug without any treatment. Of those patients requiring treatment, contrast barium enema was used, with 97% success. Only 2 patients required surgical intervention owing to worsening distension and subsequent peritonitis. When we stratified the patients according to gestational age of >36 and <36 wk, contrast barium enemas were performed 2.2 ± 1.8 versus 8.6 ± 7.8 d after birth (P = 0.003), respectively, and the lower gestational age patients had a longer length of stay. Contrast barium enema was still successful in 94% of patients with a gestational age of <36 wk. Magnesium tocolysis was noted in 16% of the cases, and Hirschsprung's disease was only found in 3.2% of patients. CONCLUSIONS: Patients with MPS have excellent outcomes, independent of gestational age. Contrast barium enema remains the initial diagnostic and treatment of choice for patients with MPS. Also, although previous reports have shown a link between magnesium tocolysis and Hirschsprung's disease with MPS, our experience suggests otherwise.

The usefulness of the upper gastrointestinal series in the pediatric patient before anti-reflux procedure or gastrostomy tube placement.

OBJECTIVE: Most children undergo an upper gastrointestinal study (UGI) before an anti-reflux (AR) procedure or gastrostomy tube placement (GT). Anatomic abnormalities detected by UGI are uncommon and we hypothesize that the value of routine preoperative use of this test is limited. METHODS: Five hundred and seventy-two patients who underwent either an AR or GT over a 10-y period at our institution were reviewed. Data including patient demographics, indications for surgery, preoperative testing, and type of operation were collected. RESULTS: Of the 572 cases, an UGI was performed in approximately 71%. The results were interpreted as normal in 63%, and abnormal in 37%. The most common abnormality noted was gastroesophageal reflux in approximately 80%, followed by an anatomic abnormality in 6%, most of which were expected. Of 36 anatomic abnormalities noted, only four were unexpected in the total cohort. In addition to an UGI, half of the subjects received additional evaluations that included pH probes and gastric emptying studies (GES). In these studies, 56% of pH probes and 45% of GES had findings in which reflux was noted. Compared with UGIs, these tests were significantly more likely to identify reflux in patients. CONCLUSION: These results suggest that the utility of an UGI before AR or GT procedures is low. Anatomic abnormalities were rare and changed clinical management in a total of four cases. A prospective trial would help to further validate these findings and help identify patients who would benefit from an UGI.

A perioperative bivalirudin anticoagulation protocol for neonates with congenital diaphragmatic hernia on extracorporeal membrane oxygenation.

INTRODUCTION: There is little published data regarding bivalirudin anticoagulation for surgical neonates on extracorporeal membrane oxygenation (ECMO). This study described our perioperative anticoagulation protocol and evaluated the relationship of bivalirudin dose to activated partial thrombin time (aPTT) and thromboelastography reaction time (TEG-R) monitoring assays. MATERIALS AND METHODS: Neonates with congenital diaphragmatic hernia (CDH) on ECMO and single-agent bivalirudin anticoagulation at our institution from 2016 to 2018 were included. Bivalirudin infusion rates, laboratory results, transfusions, and clinical events during the initial (cannulation to repair) and postoperative (up to 60 h post-repair) periods were recorded. RESULTS: Forty-two neonates met inclusion criteria. Bivalirudin was started at 0.16 mg/kg/h and titrated in 10-20% increments to target aPTT of 70-85 s and TEG-R of 9-12 min. All patients achieved target anticoagulation levels within the first 12 h on doses ranging from 0.12-0.36 mg/kg/h. Postoperatively, bivalirudin increased to median 0.16 (range 0.08-0.40), 0.22 (0.08-0.60), and 0.39 (0.08-0.80) mg/kg/h by 6, 24, and 60 h, respectively. On multivariable regression, no significant association of aPTT (p = 0.09) or TEG-R (p = 0.22) with bivalirudin dose was seen. Hemoglobin decrease ≥2 g/dL in 24 h occurred in 39%, but there were no reoperations, deaths, or circuit changes for thrombosis. CONCLUSIONS: This standardized perioperative bivalirudin protocol achieved target anticoagulation level quickly. Postoperative bleeding was managed without significant morbidity. Consistent dose-response relationships between bivalirudin and aPTT or TEG-R were not seen, but gradually increasing doses were needed to maintain therapeutic anticoagulation.

Gastroschisis with jejunal and colonic atresia, and isolated colonic atresia in dichorionic, diamniotic twins.

Despite the increasing incidence of gastroschisis, the cause remains unknown. Genetic factors may contribute to bowel anomalies as demonstrated by cases of gastroschisis in twins and siblings, and other types of bowel anomalies in twins. Atresia of the colon represents one of the rarest causes of neonatal intestinal obstruction. We present the first case of dichorionic, diamniotic male twins in which there was gastroschisis with jejunal and colonic atresia in Twin A and isolated colonic atresia in Twin B.

Stainless steel crown aspiration during sedation in pediatric dentistry.

Foreign body aspiration (FBA) causes death in more than 300 children every year in the United States. Morbidity and mortality are increased in children due to narrow airways and immature protective mechanisms. Factors to consider in pediatric dentistry are: (1) the patient's age and behavior; (2) presence and extent of disability; (3) local anesthesia; (4) body positioning; and (5) loose teeth. FBA requires prompt recognition and early treatment to minimize potentially serious and sometimes fatal consequences. The purpose of this case report was to describe the aspiration of a stainless steel crown in a 5-year-old boy during conscious sedation. It also discusses how a prompt and accurate diagnosis, early referral, and immediate treatment helped prevent serious complications.

Extracorporeal Membrane Oxygenation (ECMO) Risk Stratification in Newborns with Congenital Diaphragmatic Hernia (CDH).

BACKGROUND: A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model. METHODS: The study population was obtained from CDH Study Group registry for the period between 2007 and 2016. Only centers offering ECMO were included. The cohort was restricted to ECMO candidates and then divided into derivation and validation sets. Using all relevant perinatal predictors in the registry, univariate analysis was performed for the composite outcome of ECMO use or death without ECMO use. The model was derived using the derivation cohort with multivariable logistic regression and automatic stepwise forward selection (P < 0.05 for qualifying variables), and a c-statistic was obtained. The model was then tested on the validation cohort. Sample reuse validation and bootstrap validation were performed. The validated model was then tested for accuracy on CDH subgroups. RESULTS: There were 1992 patients in the derivation cohort. Four significant variables were identified in the final ECMO risk model: 1-min and 5-min Apgar scores and highest and lowest post-ductal partial pressure of CO2 during the first 24 h of life. The model c-statistic was 0.824 which was confirmed with cross-validation and bootstrap optimism correction. The validation cohort c-statistic was 0.823 (N = 993). The model had good discrimination for left and right CDH, inborn and outborn patients, patients born before and after 2011, and high and low volume centers. The model performed significantly better for postnatally diagnosed patients. CONCLUSIONS: This study represents proof-of-concept that a risk model can accurately estimate the probability of ECMO use in CDH newborns. This stratification could assist centers as a metric for assessment of ECMO usage and outcomes. Refinement and prospective validation of this model should be carried out prior to clinical application. LEVEL OF EVIDENCE: 3.

The use of Seprafilm in pediatric surgical patients.

Hyaluronate carboxymethylcellulose (Seprafilm; Genzyme Corp., Cambridge, MA) has been demonstrated to be safe and efficacious for the prevention of abdominal and pelvic adhesions; however, information is scarce regarding its use in children and adolescents. We wanted to characterize our experience with Seprafilm in a pediatric surgical population. After Institutional Review Board approval, we searched our hospital and pediatric surgery database for cases in which Seprafilm was used and retrospectively reviewed medical records for demographic and clinical information. Between July 2000 and October 2005, 18 patients had Seprafilm placed. Patients ranged in age from 0.25 to 18 years. Seven patients had not undergone any previous operations. Of patients having undergone prior surgery, the most common preceding intervention was a Ladd's procedure. Seprafilm was applied in a variety of scenarios, the most frequently being adhesiolysis for bowel obstruction. No patient required reoperation for obstruction. Two patients underwent subsequent operations for unrelated reasons; one was noted to have no significant adhesions, but the other had extensive adhesions. No patient demonstrated signs or symptoms of small bowel obstruction, intraperitoneal abscess, or localized inflammatory reaction to Seprafilm. Seprafilm was successfully used in our pediatric surgical population without complications.

ECMO in CDH: Is there a role?

Despite wide use and decades of experience, survival of congenital diaphragmatic hernia (CDH) patients treated with extra-corporeal membrane oxygenation (ECMO), as reported by the extra-corporeal life support organization (ELSO), remains unchanged at 50%. High-survival rates both with and without utilizing ECMO have been reported, fueling questions about the utility of ECMO support in this difficult population. This review looks at data from the Congenital Diaphragmatic Hernia Study Group and individual center reports, to evaluate the role of ECMO in CDH, focusing on defining the patients most likely to benefit, and discussing how those benefits can best be achieved. These data show that ECMO improves survival in those CDH patients who are most severely affected, but potential complications of ECMO delivery outweigh benefit in patients with less severely affected. Improved results can be expected by minimizing ECMO complications, and by improving rates of CDH repair in patients that require ECMO.

Improved Survival in Left Liver-Up Congenital Diaphragmatic Hernia by Early Repair Before Extracorporeal Membrane Oxygenation: Optimization of Patient Selection by Multivariate Risk Modeling.

BACKGROUND: Delayed repair of congenital diaphragmatic hernia (CDH) for days or longer has become standard, allowing improved stabilization for many, but potentially complicating treatment in severely affected infants who require extracorporeal membrane oxygenation (ECMO) and arrive unrepaired. Survival in left liver-up CDH, the most severe anatomic subset, averages 45% in published studies, with deaths often occurring in patients who failed to improve on ECMO and are repaired late, or not at all. Reliable early prediction of ECMO risk in these patients could identify the best candidates for repair before ECMO. We sought to predict ECMO risk in left liver-up CDH, and to further evaluate survival stratified by surgical timing in these patients. STUDY DESIGN: We reviewed 298 single-center, consecutive CDH patients, focusing on 87 inborn left liver-up patients without associated lethal anomalies. Multivariate analysis using anatomic and physiologic markers of severity was performed to define associations with need for ECMO. RESULTS: Sixty of 87 ECMO-eligible inborn left liver-up CDH patients required ECMO (69%). Of these, 20 of 21 (95%) repaired in the first 60 hours and before ECMO survived; whereas 13 of 20 (65%) who had repair delayed and arrived to ECMO unrepaired survived (p = 0.018). Lung-to-head ratio, Apgar scores, Congenital Diaphragmatic Hernia Study Group-predicted survival, pH, PCO2, and PO2 at 1 hour of life all correlated strongly with risk for ECMO. Accurate multivariate models to predict ECMO (area under the receiver operating characteristic curve [AUC] 0.91 and 0.91) were successfully developed. CONCLUSIONS: Early repair of left liver-up CDH before ECMO results in improved survival. Multivariate models can accurately assess risk for ECMO at 1 hour of life, permitting stratification of CDH surgical timing to maximize survival potential while minimizing risk.

Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: Whom should we treat?

PURPOSE: Centers that care for newborns with congenital diaphragmatic hernia (CDH) may impose selection criteria for offering or limiting aggressive support in those patients most severely affected. The purpose of this study was to analyze outcomes in newborns with highly severe CDH uniformly treated for survival. METHODS: We reviewed 172 consecutive inborn patients without associated lethal anomalies treated at a single institution with a dedicated CDH program. Survival, respiratory outcome, and time to discharge in the most severe 10% (or fewer) of patients based on the physiologic measures of 5-minute Apgar, CDH Study Group (CDHSG) predicted survival, need for ECMO in the first 6 hours, and need for ECMO in the first 3 hours of life were studied. We also identified patients with best PaCO2 greater than 100 and best pH less than 7.0. A multivariate model (AUC-0.92) predicting mortality was also used to define the most severe 10%. RESULTS: Of 172 consecutive inborn patients, 18 had a 5-minute Apgar of 3 or less, and 11 survived (61%), 10 had a 5-minute Apgar of 2 or less, and 6 survived (60%), and 6 had a 5-minute Apgar of 1 or less, and 4 survived (67%). Seventeen had a CDHSG predicted survival less than 25%, and 9 survived (53%). Thirteen of 172 required ECMO for rescue in the first 6 hours of life, and 9 survived (69%), including 7 in the first 3 hours, and 5 survived (71%). Despite focused resuscitation in the delivery room and high levels of ventilatory support, 22 patients had a best PCO2 greater than 100 and best pH less than 7.0 for 1 hour or longer. Twelve of these 22 survived to discharge (55%). Of 17 defined by multivariate predictive modeling as the most severe, 8 survived (47%) with zero of the 3 ECMO ineligible prematures surviving. Of the 16 (10%) most severe ECMO-eligible patients, 10 of 16 survived (63%). All survivors were discharged home on no ventilatory support greater than nasal cannula oxygen. CONCLUSION: In newborn CDH patients without lethal associated anomalies, accepted measures of physiologic severity failed to predict mortality. Survival met or exceeded 50% even in the most severe 10% as defined by these measures. These data support the practice of treating each patient for survival regardless of the physiologic severity in the first hours of life, and selection criteria for not offering ECMO should be reevaluated where practiced.

Delayed emergency myelopoiesis following polymicrobial sepsis in neonates.

Neonates have increased susceptibility to infection, which leads to increased mortality. Whether or not this as a result of implicit deficits in neonatal innate immune function or recapitulation of innate immune effector cell populations following infection is unknown. Here, we examine the process of emergency myelopoiesis whereby the host repopulates peripheral myeloid cells lost following the initial infectious insult. As early inflammatory responses are often dependent upon NF-κB and type I IFN signaling, we also examined whether the absence of MyD88, TRIF or MyD88 and TRIF signaling altered the myelopoietic response in neonates to polymicrobial sepsis. Following neonatal polymicrobial septic challenge, hematopoietic stem cell (HSC) expansion in bone marrow and the spleen were both attenuated and delayed in neonates compared with adults. Similar reductions in other precursors were observed in neonates. Similar to adult studies, the expansion of progenitor stem cell populations was also seen in the absence of MyD88 and/or TRIF signaling. Overall, neonates have impaired emergency myelopoiesis in response to sepsis compared with young adults. Despite reports that this expansion may be related to TLR signaling, our data suggest that other factors may be important, as TRIF(-/-) and MyD88(-/-) neonatal HSCs are still able to expand in response to polymicrobial neonatal sepsis.

Small watercraft injuries in children.

The speed and use of small watercraft have increased dramatically in recent years. We report our experience with pediatric trauma resulting from small watercraft accidents. We conducted a retrospective chart review including all children admitted with injuries sustained in small watercraft accidents. Sixteen children were included; nine were injured in jet ski accidents and seven in accidents involving other craft. Jet ski accidents tended to result in more serious injuries (closed-head injuries, hollow and solid viscus injuries, chest trauma, spinal injuries leading to paralysis, and death) than those sustained in accidents with small boats. Skin and soft-tissue injuries and long-bone fractures were the most frequent injuries following accidents with other small boats. Six of eight children (75%) injured on jet skis required operative interventions. Only three of seven (43%) children in other watercraft accidents required surgery (P < 0.05 jet ski vs other watercraft). When compared with children injured in accidents involving small boats those involved in jet ski accidents tended to have more serious injuries and require operative intervention more frequently. A high index of suspicion for serious injuries must be maintained when evaluating children with this mechanism of injury.

Twenty years of progress in congenital diaphragmatic hernia at the University of Florida.

Over the past 20 years the clinical paradigms underlying the care of children with congenital diaphragmatic hernia (CDH) have undergone profound changes. The purpose of this work is to provide an historic review of research and clinical studies related to CDH at the University of Florida (UF) and Shands Children's Hospital during the chairmanship of Edward M. Copeland, III, M.D. and to present our current clinical results. During Dr Copeland's tenure survival for newborns symptomatic with CDH treated at UF/Shands Children's Hospital has improved from less than 20 per cent to 85 per cent. Clinical observations have suggested and research studies at UF using a fetal lamb model have confirmed that fetal distress can occur late in gestation, which may predispose infants with CDH to pulmonary hypertension. However, our patient experience has confirmed that the most significant cause of mortality in human infants is not pulmonary hypertension but iatrogenic injury to their hypoplastic lungs. Strict avoidance of barotrauma in these babies has been the most important clinical advance during these two decades. Significant clinical and research problems remain including defining optimal prenatal care, management of complications during the first few weeks of life, and development of strategies to accelerate lung growth. Dramatic improvements in survival have resulted in children who manifest a number of clinical problems that were not evident when most of these patients died early in infancy. Our experience at Shand's Children's Hospital/UF indicates that feeding problems, respiratory infections, and management of subtle or overt neurologic complications may become major issues for some of these survivors and their families.

Feasibility of laparoscopic pyloromyotomy under spinal anesthesia.

BACKGROUND: Most agents used for GA are considered to be neurotoxins and affect developing brains in experimental models, leading to a push for spinal anesthesia (SA). There are no reports of laparoscopic pyloromyotomy (LP) performed under SA. We present our experience with LP and SA and discuss feasibility. METHODS: A retrospective analysis was performed on a consecutive series of patients who underwent an LP. An 'intent to treat' analysis was utilized, and GA was compared to SA. Data regarding patient characteristics, operative intervention, complications, and postoperative course were collected. RESULTS: Twelve cases had attempted SA for the LP, 9 were successful. During the same time, 12 cases underwent LP under GA. We found no difference for length of procedure, time to the first feed, or the postoperative LOS. The time to leave the OR after conclusion of the procedure was significantly shorter for the SA group (14min vs. 28min, p<0.001). There were no complications from the SA, however three cases had to be converted to GA. CONCLUSIONS: It is feasible and safe to perform laparoscopic pyloromyotomy under spinal anesthesia. Given the increasing concern over the use of GA in infants, consideration may be given to use of SA for LP.

Extracorporeal life support in patients with congenital diaphragmatic hernia: how long should we treat?

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and, despite advances, extracorporeal life support (ie, extracorporeal membrane oxygenation [ECMO]) is commonly required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. STUDY DESIGN: We conducted a single-institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO and define how long patients should be supported. RESULTS: Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar score at 1 minute, Apgar score at 5 minutes, and Congenital Diaphragmatic Hernia Study Group predicted survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. Lung-to-head ratio also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. CONCLUSIONS: In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO can take 4 weeks or longer, and might require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations <4 weeks should be avoided.