Congenital tracheobronchial stenosis.

Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling.

Imaging findings of bronchial atresia in fetuses, neonates and infants.

Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: 1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a tremendous volume increase of the distal involved lung with secondary hypoplasia of the normal lung, and 2) peripheral, located at the segmental/subsegmental bronchial level, which may present as an isolated lesion or as part of a complex congenital malformation. Prenatal findings are mostly nonspecific. Postnatal exams show overinflated lung areas and focal bronchial dilations. The typical fluid-filled bronchoceles are not always observed in neonates but develop progressively in the first months of life. This pictorial essay describes the spectrum of imaging findings of bronchial atresia in fetuses, neonates and infants.

In Utero Diagnosis of Unilateral Bronchial Atresia: One Pathologically Proven and 2 Presumed Cases With Similar Sonographic Findings at Presentation.

Unilateral bronchial atresia is a rare prenatal diagnosis that can be confused with other congenital lung abnormalities, particularly congenital pulmonary airway malformation. Accurate distinction between these entities is important for appropriate clinical care and prognosis. Familiarity of the key imaging and clinical features of unilateral bronchial atresia in comparison to other fetal lung abnormalities should increase the likelihood of achieving a timely and accurate diagnosis.

Low birth weight for gestation and airway function in infancy: exploring the fetal origins hypothesis.

BACKGROUND: Poor fetal growth has been associated with impaired airway function in adult life, but evidence linking birth weight and airway function in early childhood is sparse. We examined the hypothesis that low birth weight for gestation is associated with impaired airway function shortly after birth and that this is independent of impaired postnatal somatic growth. METHODS: Airway function was measured using the raised volume technique in healthy white infants of low (< or =10th centile) or appropriate (> or =20th centile) birth weight for gestation and was expressed as forced expiratory volume in 0.4 s (FEV0.4), forced vital capacity (FVC), and the maximal expired flow at 25% of forced vital capacity (MEF25). Infant length and weight, maternal height and weight, maternal report of smoking prenatally and postnatally, and parental occupation were recorded. RESULTS: Mothers of low birth weight for gestation infants (n=98) were lighter, shorter, and more likely to smoke and have partners in manual occupations. At 6 weeks their infants remained lighter and shorter than those of appropriate birth weight (n=136). FEV0.4, FVC, and MEF25 were reduced in infants of low birth weight for gestation, in those whose mothers smoked in pregnancy, or who were in manual occupations. After adjusting for relevant maternal and infant characteristics, infants in the low birth weight for gestation group experienced a mean reduction of 11 ml in FEV0.4 (95% CI 4 to 18; p=0.002), of 12 ml in FVC (95% CI 4 to 19; p=0.004), and of 28 ml/s in MEF25 (95% CI 7 to 48; p=0.03). CONCLUSIONS: Airway function is diminished in early postnatal life as a consequence of a complex causal pathway which includes social disadvantage as indicated by maternal social class, smoking and height, birth weight as a proximal and related consequence of these factors, and genetic predisposition to asthma. Further work is needed to establish the relevance of these findings to subsequent airway growth and development in later infancy and early childhood.

Bronchial diseases and lung aeration in children.

Bronchial diseases are common in children, and are usually associated with disturbances of aeration. This article briefly summarizes the embryological development and respiratory physiology pertinent to pediatric bronchial diseases. Current diagnostic imaging tools are discussed, with an emphasis on CT, which can demonstrate bronchial pathology such as bronchial obstruction and bronchiectasis in larger bronchi, as well as indirectly show the peripheral physiologic consequences of bronchial disease, such as alterations in aeration. Computed tomography measurements of lung attenuation may aid in diagnosis in problematic cases. Diseases that affect the pediatric airways at different ages are reviewed. Knowledge of these entities is important for accurate interpretation of imaging studies.

[Concept of defects in the interrupted development of the bronchopulmonary system. II. Dysplasias]. / Kontseptsiia za porotsite pri preustanoveno razvitie na bronkho-pulmonalnata sistema. II. Displazii.

The dysplasias are defects in case of interrupted development to the respiratory section of broncho-pulmonary system with a relatively completed differentiation of the air passages. With the abortive development of pulmonary parenchyma, the bronchialization of the "primitive air spaces" is disturbed - the functional unity between pulmonary parenchyma and air passages is not realized. "The primitive air spaces" remain at an immature stage and are transformed into cyst-like formations. Dysplasias correspond to the so-called cystose lungs and its numerous synonyms. In our opinion, the term "dysplasia of the broncho-pulmonary system" in pathologoanatomical and pathogenetic sense more correctly reflects the discrepancy in organogenesis and histogenesis of the respiratory section of the air passages.