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1.

Purpose

Many different prosthetic materials have been used for repair of large posterolateral congenital diaphragmatic hernias (CDH), with high recurrence rates for hernias that cannot be primarily repaired. Since 1993, we have used a composite patch of Gore-Tex/Marlex to repair large CDHs and hypothesized that this repair leads to fewer recurrences.

Methods

This is a retrospective review of 137 consecutive patients with CDH cared for at a single institution from 1993 to 2004. Data collected include timing and method of repair and use of extracorporeal membrane oxygenation. Outcomes include hernia recurrence, complications, and death.

Results

One hundred thirty-seven patients with CDH were analyzed. Repair was not attempted in 12 because of disease severity. Primary repair was accomplished in 79 and 46 required patch repair. Of the 46 patients with patch repairs, 32 required extracorporeal membrane oxygenation, and 18 died before discharge. Of the 28 patch repair survivors, 1 (3.57%) developed a recurrence over a median follow-up of 47 months (range, 2-115 months). Overall survival was 77%.

Conclusions

Gore-Tex/Marlex composite patch repair of large CDHs in this complex patient population results in a lower recurrence rate than has been reported for other types of prosthetic diaphragm repair.  相似文献   

2.

Background:

Minimally invasive surgery (MIS) for the repair of congenital diaphragmatic hernias (CDH) had been described. This report reviews the authors’ experience with MIS repairs of CDH and discusses the technical development of this approach.

Methods:

From 1999 until now, the authors collected data on children who underwent an MIS approach for CDH repair.

Results:

Seventeen children (11 Morgagni and 7 Bochdalek) had undergone an attempt at MIS repair. All Morgagni defects were treated successfully using laparoscopy (mean age, 28 ± 31 months). Mean follow-up was 22 ± 9 months. There was 1 recurrence. Four children with Bochdalek CDH were treated as newborns (range, 3 to 21 days), and 3 had operations later (4, 11, and 32 months). The first repair was attempted initially transabdominally and was converted to a thoracoscopic approach. The rest of the Bochdalek repairs were performed thoracoscopically. Bochdalek repairs via MIS were successful in 3 children (2 older children and 1 neonate). No child had pulmonary hypertension. Two of the 3 Bochdalek patients did well postoperatively (follow-up, 18 +/− 7 months); the last patient experienced recurrence 11 months after repair.

Conclusions:

MIS for CDH is ideal for Morgagni defects. It should be considered for nonnewborns with a Bochdalek CDH. The MIS approach for a newborn with a CDH cannot be recommended because of the high failure rate and frequent rise in Pco2 levels.  相似文献   

3.

Background/purpose

The Canadian Neonatal Network prospectively collects data from 17 pediatric hospitals accounting for 75% of all neonatal intensive care unit beds nationwide. The purpose of this study was to (1) analyze the database to compare actual survival rates of neonates with congenital diaphragmatic hernia (CDH) to predicted outcomes and (2) assess whether institutional CDH volume was associated with improved survival rate.

Methods

Actual survival rates for CDH patients born during a 22-month period were determined from the registry. Predicted survival rates were calculated using the CDH Study Group logistical regression equation. Actual survival rate was compared with predicted using χ2 analysis. Survival rates were stratified by institutional CDH volume and compared using binomial analysis. P value of less than .05 was deemed statistically significant.

Results

Of approximately 20,500 neonatal admissions, 88 cases of CDH were recorded. Seventy-three of 88 (83%) neonates with CDH survived to discharge, whereas the predicted survival rate was 62% (P < .001). Three centers were considered “high-volume” centers with at least 12 CDH diagnoses, and 11 were considered “low-volume” centers. Actual CDH survival was significantly greater at high-versus low-volume centers (90% v 77%; P < .01).

Conclusions

From these prospective data, survival rates of Canadian neonates with CDH are significantly better than predicted by the CDH Study Group equation. Further, high-volume centers in Canada have a significantly higher CDH survival rate than low-volume centers.  相似文献   

4.

Background

Infants with severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) have a high morbidity and mortality. We hypothesized that placement of an abdominal wall silo and staged abdominal wall closure may reduce problems associated with decreased abdominal domain in CDH.

Methods

We performed a retrospective review and identified 7 CDH patients requiring ECMO who had a silastic abdominal wall silo between 2003 and 2006. Variables analyzed included survival, ECMO duration, duration of silo, time to discharge, and long-term outcome.

Results

Predicted mean survival for the entire cohort using the published CDH Study Group equation was 47% (range, 9%-86%). All 7 patients (100%) survived. Extracorporeal membrane oxygenation duration averaged 15 days (range, 5-19 days). Four of the patients (58%) were repaired with a silo on ECMO, and 3 (42%) had their repair after ECMO. The abdominal wall defect was closed at a mean of 21 days (range, 4-41 days). Hospital stay after silo placement averaged 54 days (range, 20-170 days) with no infections or wound complications.

Conclusions

Abdominal wall silo placement in infants with CDH requiring ECMO appears to be an effective strategy for decreased abdominal domain. Further studies are warranted to determine the efficacy of such a strategy for these high-risk CDH patients.  相似文献   

5.

Introduction

Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has been described, but its efficacy and safety have not been validated. The aim was to compare our experience of thoracoscopy with laparotomy repair.

Methods

After ethics approval, we reviewed the notes of neonates with CDH operated in our institution between 2003 and 2008. Two historical groups were compared: infants who underwent laparotomy (2003-2008) or thoracoscopy (2007-2008). Data were compared by t test or Mann-Whitney tests.

Results

Thirty-five children had open repair of CDH, and 13 had thoracoscopic repair. Groups were homogeneous for age and weight. Five (38%) neonates who had thoracoscopy were converted to open for surgical difficulties (n = 4) and O2 desaturation (n = 1). Patch repair was used in 12 (34%) open and 6 (46%) thoracoscopic repairs. End-tidal CO2 was significantly elevated during thoracoscopy, but this was not reflected in arterial CO2 or pH. There were 3 (8%) recurrences after open repair and 2 (25%) after thoracoscopy (P = .19).

Conclusion

Thoracoscopic repair of CDH is feasible. Arterial blood gases should be closely monitored. Despite higher EtCO2, conversion to open was mainly because of difficult repair. A randomized trial is necessary to assess the effect of thoracoscopy on ventilation and recurrences.  相似文献   

6.

Purpose

Recurrence is a well-known complication after patch repair of congenital diaphragmatic hernia (CDH). We propose that a newer, “bioprosthetic” material may lower recurrence rates. The purpose of this study is to compare outcomes of CDH repair with synthetic Gore-Tex (W. L. Gore and Associates, Neward, Del) to the bioprosthetic Permacol (Tissue Science Laboratories Inc, Andover, Mass).

Methods

We performed a retrospective review of 100 consecutive patients with CDH with survival more than 30 days at Children's Medical Center of Dallas (Dallas, Tex) from 1999 to 2007. The incidence and timing of recurrence, as well as comorbidities were assessed.

Results

Primary repair was performed in 63 patients and patch repair in 37, divided between Gore-Tex (29) and Permacol (8). Overall recurrences were as follows: 1 (2%), 8 (28%), and 0 in the primary, Gore-Tex, and Permacol groups, respectively. Median follow-up was 57 months for Gore-Tex and 20 months for Permacol. Median time to recurrence in the Gore-Tex group was 12 months, with no Permacol recurrences. Both the Gore-Tex and Permacol groups had similar comorbidities, including prematurity, congenital heart disease (76% and 63%, respectively), and the need for extracorporeal membrane oxygenation support (38% and 25%).

Conclusion

Our results suggest that Permacol may have lower recurrence rates compared to Gore-Tex and is a promising alternative biologic graft for CDH repair.  相似文献   

7.

Background/Purpose

Congenital diaphragmatic hernia (CDH) is a malformation requiring neonatal surgical repair with in-hospital survival rates above 90%. We examined the long-term functional impact of CDH repair on a cross-sectional cohort of survivors.

Methods

A cohort of 53 CDH families participated in this study. Functional impact was evaluated with parent report of the Functional Status IIR and the Child Health Ratings Inventories General Health Module. Parents also provided a clinical severity score, the child's medical history, and family demographic information. The primary outcome was the effect of medical morbidity on the Functional Status IIR total score.

Results

Congenital diaphragmatic hernia survivors had a median age of 8 years; 50% were in third grade or above. Sixty-six percent had major medical issues at hospital discharge, whereas 48% had current clinical problems. Functional Status IIR total score was strongly correlated with child's clinical severity (r = −0.65; P < .0001) and was lower among children with ongoing medical morbidity, denoting worse functioning (P = .01). Child Health Ratings Inventories General Health Module scores followed a similar pattern.

Conclusions

A subset of long-term CDH survivors continues to have ongoing clinical problems a median of 8 years after surgery, translating to lower functional status. Affected children and their families may benefit from prospective identification and ongoing interventions.  相似文献   

8.
9.

Purpose

Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy.

Methods

We examined deidentified data from the CDH study group (CDHSG) registry from 1995 to 2005 on patients who underwent repair and ECMO therapy (n = 636). We used Cox regression analysis to assess differences in survival between those who underwent repair during and after ECMO.

Results

Five covariates were significantly associated with mortality as follows: timing of repair relative to ECMO (P = .03), defect side (P = .01), ECMO run length (P < .01), need for patch repair (P = .03), birth weight (P < .01), and Apgar score at 5 minutes (P = .03). Birth year, inborn vs transfer status, diaphragmatic agenesis, age at repair, and presence of cardiac or chromosomal abnormalities were not associated with survival. Repair after ECMO therapy was associated with increased survival relative to repair on ECMO (hazard ratio, 1.407; P = .03).

Conclusion

These data suggest that CDH repair after ECMO therapy is associated with improved survival compared to repair on ECMO, despite controlling for factors associated with the severity of CDH.  相似文献   

10.

Objective

To identify factors associated with survival in patients with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO).

Methods

We retrospectively analyzed the data on 3100 patients with CDH in the Congenital Diaphragmatic Hernia Study Group from 82 participating pediatric surgical centers (1995-2004). Covariates considered included prenatal and perinatal clinical information, specifics of surgical repair, and the duration of extracorporeal support.

Result

Nine hundred seven patients from the registry were identified as having been both managed with ECMO and undergone attempted surgical repair. The survival rate for the entire Congenital Diaphragmatic Hernia Study Group registry was 67% and 61% for those receiving ECMO in whom repair was attempted (P < .001). Among ECMO-treated children, survivors had a greater estimated gestational age (38 ± 2 vs 37 ± 2 weeks; P < .01), greater birth weights (3.2 ± 0.5 vs 2.9 ± 0.5 kg; P < .001), were less often prenatally diagnosed (53% vs 63%; P < .01), and were on ECMO for a shorter period of time (9 ± 5 vs 12 ± 5 days; P < .001). In logistic regression models, therapy-related variables, including the duration of ECMO, the nature of diaphragmatic repair, and the type of abdominal closure and certain comorbidities, particularly the presence of a concomitant severe cardiac abnormality, were independently associated with outcome.

Conclusion

Our model identifies a group of pre-surgical and postsurgical parameters that predict survival rate in patients with CDH on ECMO support. This model was derived from the retrospective data from a large database and will need to be prospectively tested.  相似文献   

11.

Purpose

This study was conducted to examine the preoperative factors predictive of subsequent intervention for gastroesophageal reflux (GER) in children with congenital diaphragmatic hernia (CDH).

Methods

We conducted a retrospective cohort study on children who underwent repair of a CDH between January 1, 1995, and December 31, 2002 with follow-up continuing to September 1, 2005. Excluded in the study were children who died during their first admission, or who underwent fundoplication at the time of CDH repair. Univariate and multivariate logistic regressions were performed to examine preoperative factors predictive of subsequent intervention (fundoplication or gastrojejunal tube placement).

Results

Of 86 children, 13 underwent intervention (fundoplication, 10; gastrojejunal tube, 3) for GER. Univariate predictors included the following: right-sided CDH, use of nonconventional ventilation, liver within the chest, and patch closure of the CDH. However, only liver within the chest and patch closure of the CDH were significant predictors in a multiple variable analysis. The positive and negative predictive values of the multivariate model were 69.2% and 87.7%, respectively.

Conclusions

Infants with CDH who have liver within the chest or require patch closure of their hernia are at increased risk for subsequent intervention for GER. These children may represent a subpopulation that would benefit from fundoplication at the time of CDH repair.  相似文献   

12.

Background

Although both laparoscopic and thoracoscopic repair of congenital diaphragmatic hernia (CDH) have been described in the literature, neither appropriate selection criteria nor improved outcomes for minimally invasive repair over open repair have been clearly delineated.

Methods

We reviewed our experience with neonatal CDH repair between 2004 and 2007 to determine clinical parameters that are associated with successful thoracoscopic CDH repair. We compared these patients to a similarly matched cohort of patients who had undergone an open neonatal CDH repair between 1999 and 2003.

Results

From 2004 to 2007, 20 (61%) of 33 patients underwent successful neonatal thoracoscopic CDH repair. Characteristics common to all patients who underwent successful thoracoscopic repair included absence of congenital heart defects, no need for extracorporeal membrane oxygenation, ventilatory peak inspiratory pressure of less than 26 cmH2O, and oxygenation index less than 5 on the day of planned surgery. From 1999 to 2003, 40 patients underwent an open neonatal CDH repair, of which 18 (45%) patients would have matched our selection criteria for thoracoscopic repair. These 2 cohorts were similar in age, estimated gestational age, weight, APGAR scores, and oxygenation index at the time of surgery. The thoracoscopic cohort had statistically and clinically significant quicker return to full enteral feeds, had shorter duration on the ventilator postoperatively, and required less narcotic/sedation postoperatively. Less severe complications occurred in the thoracoscopic cohort. Adjusted total hospital charges were less for the thoracoscopic repair.

Conclusions

Successful thoracoscopic CDH repair can be expected in newborns, which has limited respiratory compromise. Thoracoscopic CDH repair is associated with lower morbidity and quicker recovery than traditional open repair and without increased risk of recurrence or complications.  相似文献   

13.

Background

Timing of repair of congenital diaphragmatic hernia (CDH) in babies that require stabilization on extracorporeal membrane oxygenation (ECMO) remains controversial. Although many centers delay operation until physiologic stabilization has occurred or ECMO is no longer needed, we repair soon after ECMO has been initiated. The purpose of this study is to determine if our approach has achieved acceptable morbidity and mortality.

Methods

Charts of live-born babies with CDH treated at our institution between 1993 and 2007 were retrospectively reviewed. Data were then compared with The Congenital Diaphragmatic Hernia Study Group and Extracorporeal Life Support Organization registries.

Results

Forty-eight (39%) patients required ECMO Thirty-four of these 48 neonates were cannulated before operative repair. Venoarterial ECMO was used exclusively. The mean (SD) time of repair from cannulation was 55 (21) hours. Survival for this subset of patients was 71%. Three patients (8.8%) who underwent repair on ECMO experienced surgical site hemorrhage that required intervention.

Conclusion

Early repair of CDH in neonates on ECMO can be accomplished with acceptable rates of morbidity and mortality.  相似文献   

14.

Purpose

The purpose of the study was to assess the treatment strategies and outcome of right-sided congenital diaphragmatic hernia (R-CDH), particularly extracorporeal membrane oxygenation (ECMO).

Methods

We reviewed the cases of 42 patients treated for R-CDH at our institution from 1991 to 2006. We gathered demographic information, documented ECMO use and the type of surgical repair, and compared outcomes with predicted survival as calculated by the CDH Study Group's equation.

Results

Of the 35 patients included in our statistical analysis (7 were excluded), 12 (34%) were born at our institution, all of whom were prenatally diagnosed with R-CDH. Nineteen patients (54%) required ECMO therapy. Extracorporeal membrane oxygenation was initiated after repair of the R-CDH in 2 patients (11%). Of those patients who went on ECMO before repair, 4 patients (21%) were repaired on ECMO, 9 patients (47%) underwent repair after ECMO, and 4 patients (21%) underwent ECMO but died before their R-CDH could be repaired. Primary repair of the diaphragm was possible in 15 cases (56%), and primary closure of the abdominal incision was possible in 15 of the 23 open repairs (65%). The mean predicted survival for all 35 patients was 63%, whereas 28 (80%) actually survived. Logistic regression showed a significant association between the presence of cardiac defects and mortality (odds ratio = 0.008, P = .014).

Conclusions

Our data suggest that patients with R-CDH have high ECMO utilization and may experience greater relative benefit from ECMO as evidenced by their higher-than-expected overall survival. Extracorporeal membrane oxygenation may be found to have a distinctive role in managing R-CDH. More high-powered series are needed to elucidate differences between R-CDH and left-sided CDH that may dictate alternate forms of management.  相似文献   

15.

Introduction

Pulmonary morbidity in survivors of congenital diaphragmatic hernia (CDH) is caused by hypoplasia, barotraumas, or other reasons. We have previously shown deficient tracheal innervation in rats with CDH. Now we examine whether bronchopulmonary innervation is also abnormal in both infants and rats with CDH.

Material and Methods

Sections of E15, E18, and E21 rat lungs were immunostained for Protein gene product 9.5 and S100 antibodies. Similar immunostaining was performed on tissue from infants dying from CDH (n = 6) and other causes (n = 6) with Neurofilament, S100, and Rearranged during transfection antibodies. Nerve trunks/bronchus were counted, and the proportion of glial and RET-positive cells/bronchial surface was calculated. Glial cell-line derived neurotrophic factor protein and mRNA were measured in rat lungs.

Results

Nerve trunks/bronchus were decreased in infants and rat fetuses with CDH. In contrast, glial and RET-positive cells/bronchial surface were increased in infants and rats with CDH. Both lungs were equally affected. GDNF protein was high, whereas GDNF mRNA was decreased in preterm animals with CDH.

Conclusions

The lungs of infants and rats with CDH have decreased neural components compensated by increased supporting glial cells and persistence high expression of RET and GDNF protein. Because bronchopulmonary innervation controls airway smooth muscle, vessels, and glandular secretions, it is tempting to hypothesize that these deficiencies might play a role in respiratory morbidity in CDH.  相似文献   

16.

Background

Use of exogenous surfactant in congenital diaphragmatic hernia (CDH) patients is routine in many centers. The authors sought to determine the impact of surfactant use in the premature infant with CDH.

Methods

Data on liveborn infants with CDH from participating institutions were collected prospectively. Surfactant use and timing and outcome data were analyzed retrospectively. The authors evaluated the prenatal diagnosis patients as well. The outcome variable was survival to discharge. Odds ratios with confidence intervals were calculated.

Results

Five hundred ten infants less than 37 weeks’ gestation were entered in the CDH registry. Infants with severe anomalies (n = 80) were excluded. Information on surfactant use was available for 424 patients. Infants receiving surfactant (n = 209) had a greater odds of death than infants not receiving surfactant (n = 215, odds ratio, 2.17, 95% CI: 1.5 to 3.2; P < .01). In prenatally diagnosed infants with immediate distress, there was a trend toward worse survival rates among those receiving surfactant at 1 hour (52 patients) versus those that did not (93 patients; odds ratio, 1.93, 95% CI: 0.96 to 3.9; P < .07).

Conclusions

Surfactant, as currently used, is associated with a lower survival rate in preterm infants with CDH. The use of surfactant replacement in premature infants with CDH can be recommended only within the context of a randomized clinical trial.  相似文献   

17.

Purpose

Despite advances in neonatal care of congenital diaphragmatic hernia (CDH), a significant variation exists in the mortality rates reported by individual centers. Center experience (reflected by case volume) may contribute to this variation in outcome. The aim of the study was to determine whether CDH mortality is affected by hospital case volume.

Methods

The CDH cases were abstracted from a disease-specific, 16-hospital, national network. Thirteen hospitals participated in this study. Anonymized hospitals were categorized as either high (>6 cases) or low-volume (≤6 cases) centers (HVC, n = 6; LVC, n = 7) according to the median case number per center. Risk-adjusted (Score for Neonatal Acute Physiology, version II [SNAP-II] score) mortality rates were compared between HVC and LVC.

Results

One hundred twenty-one CDH cases were identified. Overall in-hospital survival was 81%. No significant difference in SNAP-II score was observed between HVC and LVC. Of 97 (15%) infants treated in 6 HVC, 15 (15%) died compared to 8 (33%) of 24 in 7 LVC (P < .05).

Conclusion

Hospital case volume may be partially responsible for mortality rate variation in CDH. This result requires careful analysis, as case volume may merely be a surrogate for other predictive variables.  相似文献   

18.

Background and Purpose

Prenatal corticosteroids have been used in fetuses with congenital diaphragmatic hernia (CDH). We tested the utility of steroids by 2 methods.

Methods

Mothers carrying fetuses with CDH were randomized to 3 weekly doses of betamethasone or placebo starting at 34 weeks. Patients were followed until death or discharge. In a separate cohort study, the CDH Registry was used to compare infants who received prenatal steroids to those who had not.

Results

Thirty-four patients were enrolled at 7 centers, with 32 completing the trial. There were 15 placebo and 17 steroid patients. There was no difference in survival, length of stay, duration of ventilation, or oxygen use at 30 days. For the cohort study, we looked at infants older than 34 weeks who were born after October 2000 when data on prenatal steroids were collected. There were 1093 patients; 390 were evaluable, with 56 receiving steroids. There was no difference in survival, length of stay, ventilator days, or oxygen use at 30 days.

Conclusion

Neither the trial nor the CDH Registry suggest that late prenatal corticosteroids benefit fetuses with CDH. More than 1700 mothers and fetuses would need to be enrolled in a trial to show a 10% improvement in survival. It is unlikely that late steroids offer benefit to most fetuses with CDH.  相似文献   

19.

Background

We report a 15-year experience with renal artery revascularization during abdominal aortic aneurysm (AAA) repair.

Methods

AAA repairs from 1994 to 2009 were reviewed. Postoperative complications, renal function, patency, and survival in patients undergoing renal artery revascularization were evaluated and compared with a control group of patients undergoing juxtarenal AAA repairs not requiring renal artery revascularization.

Results

Sixty patients underwent renal artery revascularization during AAA repair. Transient postoperative renal insufficiency occurred in 20 patients. Temporary hemodialysis was required in 3 patients, with none requiring permanent hemodialysis. There was 1 postoperative death. There was 1 renal artery revascularization failure at 1 month but no other graft failures at 12 months median follow-up evaluation (1-year patency, 97%). In comparison with the control group, transient renal insufficiency and pulmonary complications (33.3% vs 19.8%; P = .042) were more common with renal artery revascularization, with no differences in long-term renal complications or mortality.

Conclusions

Renal artery revascularization can be performed during AAA repair with excellent patency and minimal morbidity.  相似文献   

20.

Background

Congenital diaphragmatic hernia (CDH) is a condition with significant mortality and respiratory morbidity. Long-term neurodevelopmental outcome is not yet well documented.

Methods

The authors examined all cases of CDH at their institution over a 12-month period and performed magnetic resonance imaging (MRI) of the brain postoperatively.

Results

Eight of 10 patients survived to have an MR brain scan. All 8 patients had cerebral injury detected on MRI, with a predominance of white matter and deep nuclear gray matter injury.

Conclusions

Cerebral injury detected with MRI is common in infants with congenital diaphragmatic hernia. The long-term significance of these MRI abnormalities is unknown, although the need for good long-term neurodevelopmental follow-up of infants with CDH is highlighted.  相似文献   

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