Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group

BACKGROUND: Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult. METHODS: The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients. RESULTS: Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours). CONCLUSIONS: The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.     

Cardiac arrest before repair or extracorporeal membrane oxygenation cannulation does not increase the mortality rate associated with congenital diaphragmatic hernia

Despite recent advances in the management of high-risk congenital diaphragmatic hernia (CDH), mortality remains high. Deaths occur later because infants with inadequate pulmonary parenchyma are treated aggressively but eventually succumb to respiratory failure. In an attempt to identify absolute predictors of mortality the authors examined retrospectively their experience with CDH to determine if cardiac arrest before repair or initiation of extracorporeal membrane oxygenation (ECMO) invariably increased mortality. The authors reviewed the charts of 119 infants who had high-risk CDH treated between 1981 and 1994. They were divided into two groups: those that suffered cardiopulmonary arrest (CA, n = 21) before CDH repair or ECMO cannulation; and those that did not (NCA, N = 98). The authors compared mortality rate, ventilatory parameters, duration of, and complications on ECMO, as well as length of hospitalization between groups. Twenty-one infants suffered CA before initiation of ECMO support or CDH repair. Three infants (14%) suffered CA before arrival at our institution; seven (33%) after, and 11 (53%) both before and after arrival. There was no difference between the CA and NCA groups in terms of birth weight, gestational age, race and gender mix, or pregnancy and delivery complications. Five-minute Apgar scores were significantly lower in the CA group compared with the NCA group (4.6 v 5.7, P = .04). The CA group also had significantly worse "best postductal" blood gas and ventilatory parameters. There was no significant difference in length of hospitalization, time from admission to ECMO cannulation or CDH repair, or incidence of complications while on ECMO between the two groups. CA cases were more likely to require ECMO support (76% v 48%, P = .02) and to stay on ECMO for a more prolonged period than NCA cases (5.8 v 3.8 days, P = NS). However, there was no significant difference in overall survival between CA and NCA cases (43% v 51%, P = NS). Cardiopulmonary arrest before repair of CDH or ECMO cannulation is not a univariate independent predictor of mortality and therefore should not preclude these high-risk infants from maximum intensive care therapy, including ECMO cannulation.     

Congenital diaphragmatic hernia: developing a protocolized approach

BACKGROUND/PURPOSE: The purpose of this study was to evaluate the evolving outcome of newborns who have congenital diaphragmatic hernia (CDH) using a protocolized approach to management, which includes extracorporeal membrane oxygenation (ECMO) and to present the details of such a management protocol. METHODS: A retrospective chart review was conducted of the neonatal outcome of near-term (>34 weeks' gestation) newborns with CDH all referred to the Royal Alexandra Hospital either before or after delivery. A protocol was developed that included antenatal assessment, the use of antenatal steroids, planned delivery, use of prophylactic surfactant, pressure limited gentle ventilation, permissive hypercarbia and hypoxia, and venovenous ECMO, if indicated. RESULTS: Sixty-five infants with CDH were treated from February 1989 through August 1996. Twenty-three infants were inborn, 20 of whom were antenatal referrals. Overall, 51 of the 65 infants survived (78%). Thirteen of the 23 inborn infants survived with conservative management, and 10 required ECMO, of whom, eight were long-term survivors. Thirty-eight infants required ECMO, and 26 survived (68%), whereas there were only two deaths among the 27 conservatively treated infants. Eighteen of 20 inborn infants with an antenatal diagnosis survived, compared with 13 of 21 (62%) outborn infants. An antenatal diagnosis before 25 weeks' gestation was associated with a 60% survival rate. Sixty-three percent of infants whose best postductal PaO2 value before ECMO was less than 100 torr survived, and 7 of 11 infants with a best postductal PaO2 value of less than 50 torr before ECMO survived (64%). The average age at surgery progressively increased over time both for infants who did not require ECMO (1.3 days to 5.8 days; P = .01) and for infants who received ECMO (1.9 days to 8.2 days; P = .016). CONCLUSIONS: The use of a protocolized management for infants with CDH has been associated with improving outcome in a population at high risk. The components (either separately or combined) of these protocolized approaches need to be tested in prospective trials to determine their true benefit. In addition, there is a need to evaluate prospectively the outcomes of infants with CDH born in ECMO centers compared with those infants born in other tertiary care neonatal units to determine the most appropriate management of the fetus with CDH.     

Does extracorporeal membrane oxygenation benefit neonates with congenital diaphragmatic hernia? Application of a predictive equation

The overall survival of neonates with congenital diaphragmatic hernia (CDH) remains poor despite the advent of extracorporeal membrane oxygenation (ECMO). Attempts at accurately predicting survival have been largely unsuccessful. The purpose of this study was twofold: (1) to identify independent predictors of survival from a cohort of CDH neonates treated at the authors' institution when ECMO was not available and combine them to form a predictive equation, and (2) to apply the equation prospectively in a cohort of CDH neonates, treated at the same institution when ECMO was available, to determine whether ECMO improves outcome. From the clinical data of 62 CDH neonates treated at the authors' center by the same team of university neonatologists and pediatric surgeons between 1983 and 1993 (before ECMO availability), 15 preoperative and seven operative variables were selected as potential independent predictors. When subjected to multivariate, stepwise logistic regression analysis, four variables were identified as statistically significant (P < .05), independent predictors of survival: (1) ventilatory index (VI), (2) best preoperative PaCO2, (3) birth weight (BW), and (4) Apgar score at 5 minutes. When combined via logistic regression analysis, the following predictive equation was formulated: P (probability of survival to discharge) = [1 + e(x)]-1 where x = 4.9 - 0.68 (Apgar) - 0.0032 (BW) + 0.0063 (VI) + 0.063 (PaCO2). Applying a standard cut-off rate of survival at less than 20%, the equation yielded a sensitivity of 94% and a specificity of 82% in identifying the correct outcome of patients treated with conventional ventilatory management. The overall survival rate was 66%. Since the availability of ECMO at the center, 32 CDH neonates were treated using the same conventional ventilatory treatment and surgical repair by the same university staff. The overall survival rate was 69%. The predictive equation was applied prospectively to all neonates to determine predicted outcome, but was not used to decide the treatment method. Eighteen neonates received conventional therapy alone; 16 of 18 survived (89%). Fifteen of the 16 patients who survived had their outcomes predicted correctly (94%). Fourteen neonates did not respond to conventional therapy and required ECMO; 6 of 14 survived (43%). Six of the eight patients predicted to survive, lived (75%). All six patients predicted to die, died despite the addition of ECMO therapy (100%). The mean hospital cost, per ECMO patient who died, was $277,264.75 +/- $59,500.71 (SE). An odds ratio analysis, using the four independent predictors to standardize for degree of illness, was performed to assess the risk associated with adding ECMO therapy. The result was 1.25 (P = 0.75). Although the cohort was not large enough to eliminate significant beta error, the data strongly suggested no advantage of ECMO. At this center, absolute survival rates for neonates with CDH have not been significantly altered since ECMO has become available (66% v 69%). The authors conclude that the predictive equation remains an accurate measurement of survival at their center even when ECMO is used as a salvage therapy. The method of creating a predictive equation may be applied at any institution to determine the potential outcome of CDH neonates and assess the effect of ECMO, or other salvage therapies, on survival rates.     

Inhaled nitric oxide in congenital hypoplasia of the lungs due to diaphragmatic hernia or oligohydramnios

OBJECTIVE: We determined whether inhaled nitric oxide (NO) could improve systemic oxygenation in human neonates with hypoplastic lungs. METHODS: A multicenter nonrandomized investigation was performed to study the efficacy of short-term NO inhalation. Inhaled NO was administered at 80 ppm to nine neonates without evidence of structural cardiac disease by echocardiography. Lung hypoplasia was due to congenital diaphragmatic hernia (CDH) in eight patients and to oligohydramnios in one patient. A total of 15 trials of NO inhalation were performed in these nine patients. Eight trials in seven patients were performed before extracorporeal membrane oxygenation ((ECMO); one patient had two trials) and seven trials were performed in five patients after decannulation from ECMO (two patients had two trials each). RESULTS: NO inhalation before ECMO did not change postductal PaO2 (42 +/- 3 mmHg vs 42 +/- 4 mmHg), oxygen saturation (SpO2; 89% vs 88%) or oxygenation index (31 +/- 4 cm H2O/torr vs 31 +/- 4 cm H2O/torr) for the group. All patients required ECMO support, which lasted from 5 to 17 days (mean 9). After decannulation from ECMO, NO inhalation increased postductal PaO2 from a median of 56 mm Hg (range 41 to 94) to a median of 113 mm Hg (range 77 to 326), P < .05. It decreased the oxygenation index from a median of 23 cm H2O/torr (range 11 to 7) to a median of 11 cm H2O/torr (range 4 to 21), P < .05. It increased SpO2 from 91% to 96% (P < .05) and pH from 7.48 +/- .03 to 7.50 +/- .03. CONCLUSION: In our patients with hypoplastic lungs, inhaled NO was effective only after ECMO. This could be due to maturational changes such as activating the endogenous surfactant system. Inhaled NO may be effective in neonates with hypoplastic lungs who have recurrent episodes of pulmonary hypertension after ECMO, even if they were previously unresponsive.     

Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery

Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia. The pulmonary vascular bed may be extremely reactive to various stimuli, and in the treatment it is important to avoid pulmonary vasospasm. The strategy in our institution since 1990 has involved a prolonged preoperative stabilization with gentle mechanical ventilation. Pressures have been kept as low as possible, and slight hypercarbia has been accepted. Peak inspiratory pressures exceeding 35 cm H2O have been avoided. Extracorporeal membrane oxygenation (ECMO) has been used according to standard inclusion criteria. Nitric oxide and high-frequency oscillation have been added to the therapeutic modalities during the study period. When the patient was considered stabilized, surgical repair was undertaken after a delay of 24 to 96 hours. In patients on ECMO who could not be decannulated, surgical repair was undertaken while on ECMO. From 1990 through 1995, 52 patients were admitted with a diagnosis of CDH. Forty-three of these were risk group patients presenting with respiratory distress within 6 hours after birth. A total of 48 patients survived (survival rate 92%), and 39 of the risk group patients (survival rate 91%). There were only four hospital deaths, all with contraindications to ECMO. It is suggested that the adopted protocol is beneficial in the treatment of CDH and that the fraction of patients who have pulmonary hypoplasia incompatible with life is smaller than previously believed.     

Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion

BACKGROUND/PURPOSE: Fetuses with congenital diaphragmatic hernia (CDH) who have a "poor prognosis" with postnatal treatment now can be identified on the basis of liver herniation, early diagnosis (before 25 weeks' gestation) and a low lung-to-head ratio (LHR). Because complete in utero repair proved unsuccessful for this group, the strategy of temporary tracheal occlusion was developed to gradually enlarge the hypoplastic fetal lung. The purpose of this study is to compare the outcome of patients in the poor-prognosis group treated by one of three methods: (1) standard postnatal care, (2) fetal tracheal occlusion via open hysterotomy, and (3) the recently developed video-fetoscopic (Fetendo) technique of tracheal occlusion without hysterotomy. METHODS: In the past 3 years, 34 of 86 fetuses with an isolated left CDH met criteria for the poor-prognosis group. Thirteen families chose postnatal treatment at an extracorporeal membrane oxygenation (ECMO) center, 13 underwent open fetal tracheal occlusion, and eight underwent fetoscopic tracheal occlusion. RESULTS: The survival rate was 38% in the group treated by standard postnatal therapy, 15% in the open tracheal occlusion group, and 75% in the Fetendo group. There were less postoperative pulmonary complications noted in mothers who underwent the Fetendo procedure versus the open tracheal occlusion. All but one Fetendo clip patient had a striking physiological response demonstrated by sonographic enlargement of the small left lung that was documented postnatally by plain radiographs and its subjective appearance during repair of the CDH. In contrast, only 5 of the 13 open tracheal occlusion patients demonstrated lung growth. CONCLUSION: Fetuses with a left CDH who have liver herniation and a low LHR are at high risk of neonatal demise and appear to benefit from temporary tracheal occlusion when performed fetoscopically, but not when performed by open fetal surgery.     

Expression of the human dystrophin gene in mdx mouse skeletal muscles after ballistic transfection

OBJECTIVE: This study aimed to determine the prevalence of sensorineural hearing loss (SNHL) in 2-5-year-old survivors with neonatal respiratory failure due to congenital diaphragmatic hernia (CDH) with or without the need for extracorporeal membrane oxygenation (ECMO). STUDY DESIGN: The study design was a prospective, multicenter, longitudinal outcome study of consecutively surviving neonates admitted to a single tertiary intensive care unit. SETTING: The study was conducted at four audiologic departments affiliated with tertiary-level intensive care follow-up programs. PATIENTS: Thirty-seven surviving children receiving neonatal intensive care from February 1989 through January 1995 for neonatal respiratory failure due to CDH were studied. Excluded were 15 children with early death and I child lost to follow-up. INTERVENTION: The initial treatment depended on the severity of neonatal respiratory failure: ECMO-treated (n=31, 20 survivors) (death before ECMO initiation, 2) and non-ECMO treated (n=20, 17 survivors, another survivor lost to follow-up). MAIN OUTCOME MEASURE: Early childhood audiologic test results were measured. RESULTS: Sensorineural hearing loss was found in almost 60% of subjects: ECMO-treated, 12 (60%) of 20; non-ECMO-treated, 10 (59%) of 17. Of the 22 children with SNHL, 16 had mild- to-moderate low-frequency sloping to moderate-to-severe high-frequency loss. Of the remaining, six had severe-to-profound loss at 500 Hz and above. Seventeen children had normal responses to sound as newborns or in infancy. Five children were not tested. Documented progression was found in nine children. Twenty children currently are using amplification, and 2 have had cochlear implantation. CONCLUSIONS: Of children with CDH in this area presenting early with severe neonatal respiratory failure, SNHL developed in 60% by 2-5 years of life. Ongoing monitoring of the hearing status of children with CDH is imperative.     

Airway muscle in infants with congenital diaphragmatic hernia: response to treatment

BACKGROUND/PURPOSE: Airway muscle hyperactivity and chronic lung disease frequently follow congenital diaphragmatic hernia (CDH) treatment. The aim of this study was to compare the quantity of airway muscle and alveolar ductal artery muscle in CDH infants after various treatments. METHODS: Five groups were studied postmortem: CDH, died within 24 hours, without high ventilatory assistance (n = 3); CDH, various extracorporeal membrane oxgenation (ECMO) durations, without high ventilatory assistance (n = 4); CDH, various ECMO durations, with high ventilatory assistance (n = 7); no CDH, without high ventilatory assistance (n = 12); and no CDH, with high ventilatory assistance and bronchopulmonary dysplasia (BPD) (n = 5). Sections from standardized fixed lungs were immunohistochemically stained for alpha-smooth muscle actin. Muscle surrounding conducting airways from small preterminal bronchioles to bronchi was quantitated in both the ipsilateral and contralateral lungs with computerized image analysis. Similarly, muscle mass was quantitated in alveolar ductal arteries. RESULTS: CDH infants with low ventilatory assistance, regardless of postnatal age, had the same quantity of airway muscle as low ventilatory assistance controls. Infants with CDH and prolonged high ventilatory assistance had significantly more muscle throughout the conducting airways, similar to BPD infants without CDH, even though the CDH infants had significantly less exposure to high ventilatory assistance. With both low and high ventilatory assistance, the quantity of muscle in both the ipsilateral and contralateral lungs was similar. In contrast, small acinar arteries in CDH infants have increased muscle mass at birth. This muscle is decreased by ECMO but persists in CDH infants with high ventilatory assistance. CONCLUSIONS: The authors show that postnatally, CDH infants acquire increased muscle quantity throughout the conducting airways, in both the ipsilateral and contralateral lungs, with relatively short exposure to high ventilatory assistance. The normal decrease in acinar arterial mass that occurs postnatally is delayed in CDH infants with high ventilatory assistance.     

Neonatal detection of developmental dysplasia of the hip (DDH)

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

The impact of diaphragm management on prolonged ventilator support after thoracoabdominal aortic repair

PURPOSE: The relationship of the division of the diaphragm during thoracoabdominal aortic repair to prolonged ventilator support has not been studied. The purpose of this study was (1) to determine whether preservation of diaphragm integrity has a significant effect on postoperative ventilator duration and (2) to elucidate other pulmonary risk factors related to thoracoabdominal aortic surgery and to study the relationship of these factors to the intact diaphragm technique. METHODS:Between February 1991 and January 1997, we repaired 397 descending and thoracoabdominal aortic aneurysms. Descending thoracic aneurysms were not included in the study because their repair does not include the diaphragm. A total of 256 patients participated in this study. The diaphragm was divided in 150 patients and left intact in 106 patients. Examined as potential risk factors were patient demographics, history and physical findings, aneurysm extent, urgency of the procedure, acute dissection, cross-clamp time, homologous and autologous blood product consumption, and adjunctive operative techniques. FEV1 also was considered in the 197 patients for whom preoperative spirometry was available. Prolonged mechanical ventilation was defined as ventilator support for >72 hours. Data were analyzed by univariate contingency table and multiple logistic regression methods. RESULTS: Increasing age (odds ratio [OR], 1.02/y; P <.02), current smoking (OR, 2.6; P <.0008), total cross-clamp time (OR, 1.0/min; P <.008), units packed red blood cells transfused (OR, 1.06/unit; P <.008), and division of the diaphragm (OR, 2.03; P <.02) were significant, independent predictors of prolonged ventilation. Sixty-seven percent of patients (71 of 106) whose diaphragms were preserved were extubated in <72 hours compared with 52% of patients (78 of 150) who underwent diaphragm division (OR, 0.53; P <.02). CONCLUSION: Independently of well known pulmonary risk factors, an intact diaphragm during thoracoabdominal aortic repair results in a higher probability of early ventilator weaning.     

A multicenter trial of 6-aminocaproic acid (Amicar) in the prevention of bleeding in infants on ECMO

BACKGROUND/PURPOSE: Intracranial hemorrhage (ICH), is a major source of morbidity and the leading cause of death in neonates treated with extracorporeal membrane oxygenation (ECMO). Anecdotal reports have suggested that epsilon-aminocaproic acid (EACA) can decrease the risk of ICH. The purpose of this study was to evaluate, in a multiinstitutional, prospective, randomized, blinded fashion, the effect of EACA on the incidence of hemorrhagic complications in neonates receiving ECMO. METHODS: All neonates (except congenital diaphragmatic hernia) who met criteria for ECMO at three institutions were eligible for enrollment. EACA (100 mg/kg) or placebo was given at the time of cannulation followed by 25 mg/kg/h for 72 hours. Bleeding complications, transfusion requirements, and thrombotic complications were recorded. Post-ECMO imaging included head ultrasound scan computed tomography (CT) scan, and duplex ultrasound scan of the inferior vena cava and renal vessels. RESULTS: Twenty-nine neonates were enrolled (EACA, 13 and placebo, 16). Five (17.2%) patients had a significant (grade 3 or larger) ICH. There was no statistical difference in the incidence of significant ICH in patients who received EACA (23%) versus placebo (12.5%). Septic patients accounted for all of the ICH in the EACA group. Thrombotic complications (aortic thrombus and SVC syndrome) developed in two patients from the placebo group. There was no difference in thrombotic circuit complications between groups. CONCLUSIONS: Our results suggest that the use of EACA in neonates receiving ECMO is safe but may not decrease the overall incidence of hemorrhagic complications.     

Retention of three endodontic posts cemented with five dental cements

BACKGROUND: Although the use of extracorporeal life support (ECLS) following repair of congenital heart defects in children is increasing, the criteria for ECLS usage in these patients is not well defined. The overall survival of such patients is disappointingly low and may depend on both the indication for support and the time at which ECLS is commenced. METHODS: Between January 1993 and December 1996, 727 children underwent surgery for congenital heart defects at our institution with an overall hospital mortality of 5.8% (42 children). Nine of these children were treated with ECLS postoperatively. There were seven males and two females with a mean age of 7.2 months (range 2 weeks-3 years). Seven children could not be weaned from cardiopulmonary bypass (CPB) in the operating theatre. A further two were treated with ECLS later on during the postoperative period (commenced at 14 and 48 h). Full veno-arterial extra corporeal membrane oxygenation (ECMO) support was used in all children except one in whom a left ventricular assist device (LVAD) was used. RESULTS: The median duration of support was 121 h (range 15-648 h). Four children (44%) were weaned from support and two of these are long-term survivors. Of the seven children in whom ECLS was instituted because of failure to wean from CPB, there was one long term survivor (LVAD support). Of the two patients in whom ECLS was instituted during the post-operative period there is one long-term survivor. CONCLUSIONS: Weaning form ECLS and decannulation in 44% of our patients is comparable to other series of post-cardiotomy patients requiring ECLS. However, full veno-arterial ECMO instituted because of a failure to wean from CPB during corrective surgery is associated with an extremely poor outcome (zero long-term survivors in six patients).     

Relation between levels of essential fatty acids in maternal milk, in maternal blood and in the blood of neonates 1 and 5 days after birth

In six pediatric patients with postoperative low-output-syndrome (LOS), uni-(LVAD) and biventricular (ECMO) assist systems with Biomedicus centrifugal pump were used. ECMO was applied in 5 children aged between 3 days and 16 years, one infant with an age of 16 months was implanted with LVAD. One patient from the ECMO-group and the patient with LVAD-support survived. Analysis of vital organ functions during ECMO and LVAD support are presented and characteristics of both support systems for use in pediatric patients with postcardiotomy LOS based on worldwide experience discussed. In conclusion, both, uni- (LVAD) and biventricular circulatory support (ECMO) using Biomedicus centrifugal pump can be safely applied for management of postcardiotomy LOS even in neonates, however, ECMO support in comparison to LVAD is a more aggressive approach associated with a higher complication rate.     

Pediatric ECMO for pulmonary support: experience from 12 cases

Extracorporeal membrane oxygenation (ECMO), which can be described as treatment with a modified heart-lung machine over a prolonged period of time, is used to support patients with life-threatening but potentially reversible lung failure. ECMO by itself does not cure the patient but gives the lungs a chance to rest while awaiting spontaneous or therapeutic healing. The method is well documented in the neonatal age group. In the non-neonatal age group, however, experience is less extensive. This report of the initial result from our hospital with 12 non-neonatal pediatric cases shows high survival and low morbidity. Nine of the 12 patients were able to be weaned from ECMO (75% survival) and 8 of these 9 patients were long-term survivors. Medium time on the ventilator after discontinuation of ECMO was 4 days. At follow-up, all long-term survivors had no signs of neurological or pulmonary sequelae. These encouraging results point to the fact that ECMO should be considered more often in cases of life-threatening but potentially reversible pulmonary failure.     

Brain damage after aortic arch repair using selective cerebral perfusion

BACKGROUND: Selective cerebral perfusion is one of the most popular methods for cerebral protection during aortic arch repair. However, causes of postoperative brain damage are not fully understood. We analyzed brain damage after aortic arch repair using selective cerebral perfusion for true aortic arch aneurysm in regard to preoperative cerebral infarction and intracranial and extracranial occlusive arterial disease. METHODS: Over a 9-year period, 60 patients with true aortic arch aneurysm underwent aortic arch repair using selective cerebral perfusion. Postoperative brain damage was evaluated in regard to preoperative cerebral infarction detected by computed tomography, magnetic resonance imaging, or both in 50 patients and intracranial and extracranial occlusive arterial disease detected by digital subtraction angiography, magnetic resonance angiography, or both in 35 patients. RESULTS: Seven (12%) of the 60 patients died within 30 days of operation. Postoperative brain damage occurred in 6 (10.5%) (3, coma, and 3, hemiplegia) of 57 patients; 3 patients who died without awakening were excluded. Preoperatively, old cerebral infarction was detected in 9 patients (18%), and silent cerebral infarction (lacunar infarction and leukoaraiosis) was diagnosed in 26 patients (52%). Postoperative brain damage occurred in 3 (33%) of the 9 patients with preoperative cerebral infarction and in 3 (23%) of 13 patients with negative preoperative brain findings; this excludes 2 patients who died without awakening. No patient with silent cerebral infarction had postoperative brain damage. Occlusive arterial disease was detected in 7 patients (20%). The incidence of brain damage in these patients was 71% (5/7), which was significantly greater than that of 4% (1/28) in patients without occlusive arterial disease (p < 0.001). CONCLUSIONS: Silent cerebral infarction may not be a risk factor for postoperative brain damage. Preoperative evaluation of intracranial and extracranial occlusive arterial disease provides important information as to whether a patient might sustain brain damage after aortic arch repair using selective cerebral perfusion.     

Extra-corporeal membrane oxygenation for paediatric respiratory failure

Extracorporeal membrane oxygenation (ECMO) uses modified cardiopulmonary bypass technology to provide prolonged respiratory or cardiorespiratory support for patients of all ages who have failed conventional intensive care management. The use of ECMO for neonatal respiratory failure is now evidence-based following the publication of the randomised UK Collaborative Trial. ECMO use in children remains more controversial, but overall survival of 71% is possible in a group of moribund patients whose mean PaO2/FIO2 ratio of 61 mmHg accurately predicts death in studies of conventional ventilation. Common diagnoses for children requiring ECMO support are pneumonia and the acute respiratory distress syndrome (ARDS).     

Immediate management of posterior urethral disruptions due to pelvic fracture: therapeutic alternatives

PURPOSE: We retrospectively reviewed the results of 3 types of initial management of pelvic fracture urethral disruption in children. MATERIALS AND METHODS: From 1980 to 1994, 35 boys 2 to 15 years old (mean age 8.1) with prostatomembranous urethral disruption were treated, including 17 who also had associated injuries. Immediate treatment included suprapubic cystostomy and delayed urethroplasty in 19 patients (group 1), urethral catheter alignment without traction and concomitant suprapubic cystostomy in 10 (group 2), and primary retropubic anastomotic urethroplasty in 6 (group 3). RESULTS: In all patients in groups 1 and 2 severe urethral obliteration developed. Four group 3 patients (66%) had a stricture at the site of anastomotic repair. After delayed urethroplasty 16 group 1 (84%) and all 10 group 2 patients were continent. However, only 3 group 3 patients (50%) achieved continence. Retrospectively associated bladder neck injury occurred in 5 of the 6 incontinent boys. Erections were observed before and after treatment in all but 3 children. Unstable pelvic ring fractures (type IV) comprised 28% of all pelvic fractures with a high rate of associated injuries. CONCLUSIONS: As described, urethral alignment was not beneficial for avoiding urethral obliteration. Therefore we recommend suprapublic cystostomy as the only form of initial treatment in these cases. Urinary incontinence seems more likely related to associated bladder neck rupture and the severity of pelvic fracture rather than to initial treatment or delayed urethral repair. Consequently, when associated bladder neck injury is present, we advocate immediate surgical repair.     

Frequency, causes, and outcome of home ventilator failure

STUDY OBJECTIVES: The safety of home ventilators has been questioned. We collected data to study the following: frequency of home ventilator failure, apparent causes for the failure or malfunction, and adverse consequences following the failure. STUDY DESIGN: Information on all requests to correct home ventilator failures reported to a home respiratory equipment vendor was collected prospectively between November 1991, and November 1992. PATIENTS: There were 150 ventilator-assisted patients aged 2 to 77 years; 44 were < or = 18 years. They received 841,234 h of home mechanical ventilation (average, 15.4 h/d per ventilator-assisted patient). RESULTS: There were 189 reports of home ventilator failure. Defective equipment or mechanical failure was found in only 39% (73 reports), equivalent to one home ventilator failure for every 1.25 years of continuous use. Other causes of ventilator failure included the following: improper care, damage, or tampering with the ventilator by caregivers (13%), functional equipment improperly used by caregivers (30%), and equipment functional but the patient's condition changed, mimicking ventilator failure (3%). No problem could be identified in 16%. The following actions were required: ventilator replacement (44%), repair of a defective part (6%), replacement of a functioning ventilator for psychological comfort (14%), ventilator adjustments made (21%), caregiver reeducation (7%), caregiver anxiety or distress reduced (3%), and no action required (4%). Hospitalization was required only in two cases (1%). No adverse outcomes, deaths, or serious injuries were associated with home ventilator failure. CONCLUSIONS: We conclude that in 150 patients requiring home mechanical ventilation, ventilator failure occurred relatively infrequently, and there were no adverse outcomes as a result of equipment failure at home. We speculate that equipment failure is not a frequent or serious problem for ventilator-assisted patients treated at home.     

Initial experience with endovascular aneurysm repair: comparison of early results with outcome of conventional open repair

PURPOSE: To determine the safety, effectiveness, and problems encountered with endovascular repair of abdominal aortic aneurysm (AAA). Initial experience with endoluminal stent grafts was examined and compared with outcome for a matched concurrent control group undergoing conventional operative repair of AAA. METHODS: Over a 3-year period, 30 patients underwent attempts at endovascular repair of infrarenal AAA. Of the 28 (93%) successfully implanted endografts, 8 were tube endografts, 8 bifurcated grafts, and 12 aortouniiliac grafts combined with femorofemoral bypass. Most of the procedures were performed in the past year because the availability of bifurcated and aortoiliac endografts markedly expanded the percentage of patients with AAA who might be treated with endoluminal methods. The follow-up period ranged from 1 to 44 months, with a mean value of 11 months. RESULTS: Endovascular procedures demonstrated significant advantages with respect to reduced blood loss (408 versus 1287 ml), use of an intensive care unit (0.1 versus 1.75 days), length of hospitalization (3.9 versus 10.3 days), and quicker recovery (11 versus 47 days). Although the total number of postoperative complications was identical for the two groups, the nature of the complications differed considerably. Local and vascular complications characteristic of endovascular repair could frequently be corrected at the time of the procedure and tended to be less severe than systemic or remote complications, which predominated among the open surgical repair group. On an intent-to-treat basis, 23 (77%) of the 30 AAAs were successfully managed with endoluminal repair. The seven (23%) failures were attributable to two immediate conversions caused by access problems, three persistent endoleaks, one late conversion caused by AAA expansion, and one late rupture. CONCLUSIONS: Although less definitive than those for conventional operations, these early results suggest that endovascular AAA repair offers considerable benefits for appropriate patients. The results justify continued application of this method of AAA repair, particularly in the treatment of older persons at high risk.