PubMed · 10293906
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M Wagner. 1989-07-21. New technology for critically ill newborns needs solid planning.. https://pubmed.ncbi.nlm.nih.gov/10293906/
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The outcome of congenital diaphragmatic hernia (CDH) differs for different stages of the fetus or infant's life (i.e., antenatal, immediate postnatal, and postoperative). Assessing combined data from nonrandomized studies is technically difficult. Following recognized methods of reviewing such trials, we aimed to review the available literature on the outcome of CDH to provide a guide to clinicians when counselling parents who have a fetus/infant with this condition. Thirty-five studies reporting data for CDH from 1985 to March 1998 were identified using a high sensitive search strategy, hand-searching journals, and reviewing references of relevant studies. These were systematically reviewed. The median overall mortality was 58% (interquartile range (IQR), 43-65%) for babies diagnosed in utero, 48% (IQR, 35-55%) if born alive, and 33% (IQR, 18-54%) postoperatively. Diagnosis before 25 weeks of gestation is not a uniformly bad prognostic indicator (median mortality, 60%). Outcome was worse for those fetuses with other anomalies (median mortality, 93%). The median percentage mortality for all infants born alive and treated in extracorporeal membrane oxygenation (ECMO) centers was 34% (IQR, 26-47%). Median percentage mortality for all ECMO-treated infants was 44% (IQR, 35-50%). Different treatment strategies may have a variable impact on outcome. These figures, together with local data, may help in parental counselling on prognosis for fetuses/infants with CDH.
The management of a critical airway in infants and toddlers with congenital tracheal stenosis (CTS) continues to be an enormous challenge to the surgeon. Until recently, this condition often proved fatal. Improvements in surgical techniques, anesthetic management, and postoperative critical care have resulted in successful outcomes in children not long ago considered untreatable. However, issues such as the best operative approach and the optimal perioperative management are still unresolved. The diagnosis of CTS, often delayed, must be considered in any infant with stridor, wheezing, cyanosis, or recurrent episodes of pneumonia. Associated anomalies are the rule, including frequently vascular rings and rarely pulmonary agenesis. These defects can be repaired with conventional ventilatory support under cardiopulmonary bypass, or using extracorporeal membrane oxygenation (ECMO). We report our experience in which ECMO was used to support two patients with CTS during the perioperative period. ECMO proved to be both safe and practical, allowing unrushed, precise repair of the tracheal stenosis and providing brief postoperative support. Perioperative outcomes were excellent, although one of our patients died months after the repair. A review of the literature and our experience in which ECMO was used to provide cardiopulmonary support during repair of CTS showed uniformly successful perioperative outcomes.
PURPOSE: The aim of this study was to describe in detail the perinatal developmental profile of the pulmonary vasculature in congenital diaphragmatic hernia (CDH) and to examine the potential beneficial effects of extracorporeal membrane oxygenation (ECMO) on the vascular morphology. Additionally the authors aimed to identify the differences in pulmonary vascular morphology among CDH cases according to the primary cause of death: either extreme lung hypoplasia (LH) or persistent pulmonary hypertension (PPH). METHODS: The authors studied autopsy sections from 30 high-risk CDH cases with respect to the pulmonary arteries in relation to gestational age (GA) and ECMO treatment. They were grouped into CDH-I: 20 cases with GA greater than 34 weeks who were not subjected to ECMO and CDH-II: 10 cases with GA greater than 34 weeks, who were subjected to ECMO for an average time of 237 hours. Five age-matched neonates who died from placental insufficiency or birth asphyxia without evidence of lung hypoplasia served as controls (CON). Medial and adventitial thicknesses of pulmonary arteries were measured in lung sections stained with Elastic van Gieson by 2 investigators blinded for the clinical data. Immunohistological staining with anti-alpha-smooth muscle actin (alpha-SMA) was performed to confirm the precise location of the arterial media before morphometry. CDH cases were subgrouped and compared according to the primary cause of death. Unpaired Student t test was used for statistics, with significant P value < or =.05. RESULTS: In CDH newborns, a significant increase in medial, adventitial, and total wall thickness was found in pulmonary arteries with an external diameter of less than 200 microm as compared with age-matched controls (P<.004, .0001, and .0009, respectively). ECMO-treated CDH newborns showed a significantly thinner arterial adventitia than CDH patients who did not receive this treatment (P<.0001), approaching normal values. However, the medial thickness remained increased. Morphometrically, no significant differences in CDH cases between patients dying of PPH or severe LH could be determined. CONCLUSIONS: (1) In CDH, there is failure of the normal arterial remodeling processes occurring in the perinatal period. (2) Pulmonary vascular morphology in CDH does not differ between the groups with lung hypoplasia or persistent pulmonary hypertension as primary cause of death. (3) Adventitial thinning of these arteries might be one of the mechanisms by which ECMO alters PPH in CDH cases.