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Congenital malformations of the trachea include a variety of conditions that cause respiratory distress in neonates and infants. A number of anomalies are self-limiting while others are life-threatening and require immediate therapy. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent congenital tracheal malformations are: tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft and tracheal agenesis. The management of congenital tracheal malformations is complex and requires an individualized approach delivered by a multidiscipilinary team within centralized units with the necessary expertise.

Background: We previously demonstrated that tracheobronchial innervation, originated from the vagus nerve and hence of neural crest origin, is deficient in rats with experimental congenital diaphragmatic hernia (CDH). The present study examines the development of this innervation during fetal life in an attempt to understand the nature of these deficiencies., Materials and Methods: Pregnant rats were given either 100 mg nitrofen or vehicle on E 9.5. Embryos were recovered on E15 and E18. Control and nitrofen/CDH pups (n = 10 each) were studied on each of these days and compared with our previous results on E21. Whole mount preparations of tracheas stained for anti-protein gene product 9.5 (PGP 9.5) and smooth muscle contractile alpha-actin were examined under confocal microscopy for the morphology of intrinsic neural network. Sections of tracheas were immunostained with anti-low-affinity neurotrophin receptor (p75(NTR)), neural cell marker PGP 9.5, and anti-glial cell marker S100 antibodies. The proportions of sectional areas occupied by neural and glial structures were measured in the proximal and distal trachea. PGP 9.5 protein, and mRNA expressions were determined. Mann-Whitney tests with a threshold of significance of P < 0.05 were used for comparison., Results: Positive staining for p75(NTR) confirmed the neural crest origin of tracheal neural cells. The neural network appeared less organized on E15, and it was less dense on E18 in nitrofen-exposed embryos than in controls. The proportions of section surface occupied by neural elements were similar in both groups on E15, but that of glial tissue was significantly increased in nitrofen-exposed embryos. On E18, the relative neural surface was significantly reduced in CDH embryos in contrast with increased glial tissue surface. On E21 the proportion of neural tissue was reduced only in the distal trachea. The expression of PGP 9.5 protein was decreased in CDH fetuses on E18 and E21. In contrast, PGP 9.5 mRNA levels were increased in CDH fetuses on E18 and E21., Conclusions: The development of intrinsic innervation of the trachea in rats with CDH is abnormal with reduction of neural tissue accompanied by increase of glial tissue that could represent a response to neural damage. The significance of increased PGP 9.5 mRNA levels is unclear.

The purpose of this review is to capture the current state-of-the art of the technical aspects of multiplanar and three-dimensional (3D) images and their thoracic applications. Planimetric and volumetric analysis resulting from volumetric data acquisitions obviates the limitations of segmented transverse images. Among the 3D reconstruction techniques currently available, the most recently introduced technique, i. e., volume rendering, has to be evaluated in comparison with 3D shaded surface display and maximum or minimum intensity projection. Slabs are useful in detecting and localizing micronodular or microtubular patterns and in analyzing mild forms of uneven attenuation of the lungs. Three-dimensional angiography is helpful in the pretherapeutic evaluation and posttreatment follow-up of pulmonary arteriovenous malformations, in the comprehension of the postoperative reorientation of the pulmonary vessels, in the surgical planning of pulmonary tumors, and in the diagnosis of marginated thrombi. The systemic supply to the lung and superior vena cava syndromes are also relevant to these techniques. In acquired or congenital tracheobronchial diseases including stenosis, extraluminal air and complex airway anatomy, multiplanar and 3D reformations have a complementary role to both transaxial images and endoscopy. New developments are also expected in various topics such as 3D conformal radiation therapy, planning of intraluminal bronchoscopic therapy, virtual endoscopy, and functional imaging of the bronchial tree. Miscellaneous clinical applications are promising in the analysis of diaphragmatic morphology and pathophysiology, in the volumetric quantification of the lung parenchyma, and in the vascular components of the thoracic outlet syndromes.

Congenital abnormalities of the bronchi have been classically described with chest X-rays, conventional tomography, bronchography, CT and MR imaging. Recently, the capacity of spiral CT to explore a complete volume with no gap and excellent multiplanar reformations has been emphasized. The contribution of this technique to the analysis of congenital anomalies of tracheobronchial branching patterns encountered in adult patients is illustrated. Agenesis, aplasia, and hypoplasia are discussed, followed by bronchial atresia and abnormalities of bronchial divisions. In most cases spiral CT permits a full and correct evaluation of the malformation as well as its associated anomalies. It appears therefore to be the preferable technique for studying such anomalies of the tracheobronchial tree. Moreover, knowledge of CT aspects of the main congenital bronchial abnormalities along with complete visualization of the tracheobronchial tree will probably lead to detection of more incidental anomalies.