Author(s): Eber E
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Abstract Today, congenital thoracic malformations (CTMs), which comprise a spectrum of anomalies rather than separate entities, are frequently detected on routine antenatal ultrasound. However, with similar appearances of different congenital lung and nonpulmonary lesions, a definitive diagnosis cannot usually be established antenatally with absolute certainty. The natural history of CTMs is extremely variable. Large lesions may cause serious complications in the fetus, necessitating treatment in utero. Such prenatal therapy, however, is only required in a small minority of fetuses. Many lesions decrease in size before birth, and some are no longer detectable by ultrasound or chest radiography in the newborn period. All patients with prenatally detected CTMs require thorough postnatal evaluation, including a chest computed tomographic scan. Postnatally, the clinical appearance of CTMs can vary from immediate respiratory distress at birth to an incidental finding on a chest radiograph at any age. A few patients with large lesions require emergency or urgent surgery in the neonatal period. Furthermore, surgery is the accepted standard of care for all symptomatic lesions. Many children, however, will be asymptomatic at birth and in the neonatal period, and there is controversy as to the management of these newborns. Some authors recommend expectant long-term management of asymptomatic lesions, in particular congenital lobar emphysema and extrapulmonary sequestration. Most authors advocate elective resection of all cystic adenomatoid malformations, bronchogenic cysts, and intrapulmonary sequestrations because of the risk of complications, such as infection, hemorrhage, pneumothorax, sudden respiratory compromise, and malignant transformation. Elective lobectomy appears to be very well tolerated and is the most prevalent surgical method. Video-assisted thoracoscopic surgery was reported to be safe and effective. The timing of surgery in asymptomatic patients is not well delineated, with recommendations ranging from 1 month to 2 years of age. Some authors advocate surgery between 6 and 12 months of age because anesthetic and surgical risks decrease within the first months of life. Long-term prospective studies of CTMs are urgently needed to document their natural history.
This article was published in Semin Respir Crit Care Med
and referenced in Journal of Bioanalysis & Biomedicine