Congenital cystic adenomatoid malformation of the lung (CCAM) and Bronchopulmonary sequestration (BPS): Prenatal diagnosis, pre- and postnatal interventions, and early- and long-term outcome (14 years clinical experience with 60 patients) - a podcast by Ludwig-Maximilians-Universität München

DEUTSCHE ZUSAMMENFASSUNG siehe PDF-Datei S. 7 (Teil I) und S. 111 (Teil II). ///

ENGLISH ABSTRACT:
BACKGROUND AND PURPOSE: Fetal cystic lung lesions, such as congenital cystic adenomatoid malformation of the lung (CCAM) and bronchopulmonary sequestration (BPS), were once considered rare and of poor prognosis. The advances in prenatal imaging and fetal therapeutic intervention techniques have contributed to a revision of this opinion. However, hardly any long-term investigation exists and prenatal predictive factors are still controversially debated with a lack of detail. This complicates adequate counseling of expectant parents and patients management. The goal of this research was four-fold: (1) to study the long-term outcome of children with prenatally detected congenital cystic lung lesions, (2) to compare the long-term outcome with the early outcome of the disease, (3) to determine prenatal ultrasonographic features which are prognostic for the outcomes, and (4) to compare the findings of this study with the data obtained by an extended statistical analysis of all major series of patients published in the literature.
PATIENTS AND METHODS: This prospective cohort study, a long-term follow-up study, was conducted on 60 fetuses prenatally diagnosed with cystic lung lesions and ultrasonographically evaluated from 1988 through 2002 at the University of California, San Francisco (UCSF) Medical Center and at the University Hospital Großhadern of the Ludwig-Maximilians-University (LMU) Munich, Germany. Based on a follow-up questionnaire (see appendix of PDF-file for copy) with 39 questions, sent to the patients’ parents up to 14 years later, and a review of the patients’ medical records, clinical data were extracted about present and past respiratory and non-respiratory symptoms, therapeutic interventions, and imaging studies. Respiratory difficulties such as the requirement of ventilatory support or oxygen, recurrent respiratory infections, asthma, and/or limited physical endurance were categorized into four severity groups. The study patients were classified into five surgical intervention groups: (1) termination of pregnancy, (2) invasive fetal treatment, (3) neonatal surgery, (4) childhood surgery, and (5) no surgical intervention. Postoperative early and late complications were investigated. The outcome was grouped into (i) early neonatal outcome, (ii) interim childhood outcome, and (iii) final outcome. The age at improvement was evaluated. To identify prenatal predictors for the outcome, the children’s prenatal ultrasound data were retrospectively reviewed. Data about the size, (Stocker) type, and location of the lung lesion and the development of a hydrops fetalis, mediastinal shift, ascites, pleural and pericardial effusions, skin edema, placental thickening, polyhydramnios, and the fetal growth were extracted. All quantifiable parameters were categorized into four severity groups. The size of the mass was categorized into (i) small, (ii) moderate, (iii) large, or (iv) very large. The predictive value of each prenatal parameter examined in this study was compared with predictions published in the literature. All study data were analyzed using the Pearson's chi-square test, t-tests of the means, analysis of variance (ANOVA), and bivariate correlation. The Committee of Human Research approved the study (see appendix of PDF-file for approval letter).
RESULTS: The survival rate in this study of 60 fetuses with cystic lung lesions was 94% (51 of 54 cases) to the exclusion of 4 terminated pregnancies and 2 deceased, untreated fetal treatment candidates. Three patients died after birth despite invasive fetal treatment due to advanced hydrops fetalis and delayed open fetal surgery. Of the 51 surviving children, 11 (22%) children had invasive fetal treatment, 12 (24%) were operated on as neonates, 15 (29%) were operated on in childhood, and 13 (25%) had no surgery. Invasive fetal treatment had a success rate of 79% (11/14) with

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