China
Africa
Explore chapters and articles related to this topic
Respiratory Medicine
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Colin Wallis, Helen Spencer, Sam Sonnappa
In pulmonary agenesis, there is no development of the lung beyond the carina. Bilateral pulmonary agenesis occurs at the single respiratory bud stage and is incompatible with life. Unilateral pulmonary agenesis may be associated with complete tracheal rings.
Congenital Disorders of the Larynx, Trachea and Bronchi
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
Agenesis of one main bronchus and its associated lung is not as rare as tracheal agenesis and is compatible with survival, although such children often have coexisting congenital anomalies and are at risk from chest infections because of their much-reduced respiratory reserve. Bilateral bronchial and pulmonary agenesis is extremely rare and is, of course, fatal. Occasionally, localized atresia occurs in a peripheral bronchus, resulting in a distal mucocoele which may need to be resected if it is causing severe compression of the surrounding lung.
Congenital malformations of the lung
Published in Prem Puri, Newborn Surgery, 2017
Shannon M. Koehler, Keith T. Oldham
Postnatally, bilateral pulmonary agenesis is exceedingly rare and is inevitably incompatible with life. Unilateral pulmonary agenesis may be asymptomatic; however, symptomatic patients may pose difficult neonatal management issues, not only from the standpoint of respiratory insufficiency but also because of a high incidence (50%–70%) of associated anomalies.69 Older children may be asymptomatic or demonstrate nonspecific respiratory symptoms including a history of failure to thrive, exercise intolerance, recurrent respiratory infections, and chest asymmetry or scoliosis. A shift in the location of heart tones and absent ipsilateral breath sounds is demonstrable on physical examination. Chest roentgenograms will demonstrate hyperinflation of the contralateral lung and possibly a fluid-filled ipsilateral hemithorax in the setting of marked displacement of the mediastinum.69 If the diagnosis had not yet been confirmed, again, the absence of the ipsilateral mainstem bronchus or the pulmonary artery is a definitive finding, and this can be established by endoscopy, echocardiography, axial imaging, or angiography. The prognosis reported in the literature is quite variable, but there is a worse prognosis for those patients with right-sided unilateral pulmonary agenesis. Kayemba-Kay et al.69 suggest that a normal life can be expected for those patients with left-sided pulmonary agenesis, but this likely depends more on the concurrent congenital anomalies.
Tracheal agenesis with esophageal atresia: an autopsy case report of a variant incompatible with life
Published in Fetal and Pediatric Pathology, 2020
Kritika Krishnamurthy, Jyotsna Kochiyil, Robert J. Poppiti
Floyd et al. classified TA into three types based on severity: Type 1 where the distal trachea is connected to the esophagus by a fistula (13%); Type 2 where the carina is connected to the esophagus by a fistula (65%); and Type 3 where the two main bronchi arise directly from the esophagus (22%) [9]. Though this classification is widely accepted, infrequent variants of TA, such as our case, have been reported that do not fit into these categories. Faro et al. have proposed a more comprehensive classification of TA defining seven subtypes as follows—(1) Faro type A: total pulmonary agenesis, (2) Faro type B: complete TA with a separate origin of the main bronchi from the esophagus, (3) Faro type C: total TA with normal main bronchi fuzing in the midline at the carina; a trachea-esophageal fistula (TEF) may or may not be present, (4) Faro type D: larynx joined by atresic strand to distal trachea with a TEF present, (5) Faro type E: agenesis of the proximal trachea with a normal caudal segment of the distal trachea and a TEF, (6) Faro type F: agenesis of the proximal trachea with a normal caudal segment of the distal trachea and no TEF is present, (7) Faro type G: TA in a short segment [3]. This expanded classification still fails to encompass all the possible variants of TA such as our case.