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The respiratory system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Many congenital abnormalities, such as tracheal agenesis, are rare. Tracheo-oesophageal fistula, which allows food to travel from the oesophagus into the main airways, causes choking and recurrent infections. Lung cysts may be congenital and persist into adult life. These include foregut cysts, pulmonary sequestration, congenital lobar emphysema and congenital cystic pulmonary adenomatoid malformation (CPAM) (Figure 8.2). Pulmonary hypoplasia is characterized by the underdevelopment of one or both lungs and is often due to poor lung expansion or compression of the lungs in-utero (e.g. with oligohydramnios or congenital diaphragmatic hernia respectively), and has a poor prognosis.
Cardiology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Tracheal transplantation is beginning to be introduced to treat conditions such as tracheal agenesis or stenosis. The procedure is different to heart and lung transplantation in that a decellularised graft is prepared ex vivo with the recipient patient’s own stem cells and growth factors to produce a new trachea. As a result immunsuppression is not required as the tissue transplanted is recipient compatible.
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.
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
Tracheal agenesis (TA) is an infrequent congenital anomaly that presents with respiratory distress and difficult intubation. TA associated with an esophageal atresia is a infrequent variant of TA that is not included in the current classification systems. Here in we present a variant of type G tracheal atresia complicated by a Vogt III/Gross B esophageal atresia with a proximal TEF. In this case, the proximal trachea and esophagus end in a blind loop with no distal communication, making this variant unamenable to current treatment and incompatible with life.