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Congenital diaphragmatic hernia
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Erin E. Perrone, George B. Mychaliska
The majority of infants with CDH are diagnosed antenatally (68%). Ultrasound may show bowel and/or liver in the chest, the heart and stomach in the same plane, or mediastinal shift (Figure 13.3a–c). Ultrasound can give estimates of disease severity by calculating observed-to-expected lung to head ratio (o/e LHR) with the most severe disease having a calculated o/e LHR <25%. Fetal magnetic resonance imaging (MRI) has also become a useful adjunct to differentiate CDH from lung bud anomalies. Fetal MRI can estimate observed-to-expected total fetal lung volumes (o/e TFLVs), prognosticate survival for prenatal counseling, and evaluate the degree of liver herniation within the chest.
Concurrent Congenital Diaphragmatic Hernia and Extralobar Pulmonary Sequestration
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Kate Gallo, Gillian Alex, Christopher W. Seder
The overall incidence of pulmonary sequestration is small and only represents 0.15%–6.4% of all pulmonary anomalies [3]. Of the two forms of sequestration (intralobar and extralobar), extralobar is the least common representing only 19% of sequestrations [1]. The cause of BPS is not clearly understood, which makes understanding its relationship to other intrathoracic anomalies difficult. There are two working theories that have been proposed to explain the development of EPS. The first theory suggests that the embryonic lung bud detaches and isolates from normal developing lung tissue allowing separate pleura to form [6]. The second theory involves an anomaly in foregut bud formation [6]. Unfortunately, neither of these theories explain the association between CDH and EPS.
Fibroblast Pneumonocyte Factor
Published in Jacques R. Bourbon, Pulmonary Surfactant: Biochemical, Functional, Regulatory, and Clinical Concepts, 2019
As noted in Chapter 10, the appearance of the alveolar type II cell (pneumonocyte) with its ability to synthesize and secrete pulmonary surfactant is the key to the neonate’s adaptation to the appearance of a pulmonary air-liquid interface at the time of birth. Some of the primitive epithelial cells of the embryonic lung bud are “committed” to this phenotype early in development, but once committed they do not fully express the phenotype (“progression”) until much closer to term. Immunological reagents to type II cell markers are being used to trace these phenomena.6 Kusakabe et al.7 microdissected lung rudiments from day 12 to 15 rat embryos and transplanted them into adult rat kidney capsules. Fourteen days later, they were studied morphologically and by immunostaining with a polyclonal antibody specific for surfactant protein A (SP-A) (Chapter 4). Intact rudiments showed branching morphogenesis and epithelial acquisition of the type II cell phenotype as detected by immunofluorescence with the antibody to SP-A. However, when epithelia were dissected free of mesenchyme and implanted alone, branching morphogenesis did not occur, and SP-A expression was only seen in epithelia transplanted on or after day 13. It was concluded that in addition to the role of mesenchyme in directing branching morphogenesis,8 a mesenchymalepithelial interaction prior to day 13 committed the epithelial cells to subsequent expression of the type II cell phenotype.7
Extralobar Pulmonary Sequestration in Adrenal Mimicking Neuroblastoma: A Case Report
Published in Fetal and Pediatric Pathology, 2023
Raktim Mukherjee, Oindrila Das, Subhankar Chakravorty, Suravi Mohanty, Uttara Chatterjee
Extralobar pulmonary sequestration is a cystic malformation composed of bronchopulmonary tissue outside the lungs that is discontinuous from the tracheobronchial tree. Pulmonary sequestration usually consists of nonfunctioning primitive lung tissue that derives blood from systemic circulation. Embryological origin of this condition is thought be due to development of an accessory lung bud from the ventral aspect of primitive foregut. In the majority of cases it is intralobar and is usually asymptomatic. It often presents in adult period with recurrent chest infections. Extralobar sequestration is uncommon and forms 25% of all sequestrated lungs [3]. The usual sites of ELS are costophrenic sulcus, mediastinum, pericardium, areas within or below diaphragm and retroperitoneum. Intra adrenal extralobar pulmonary sequestration has been reported 3 times previously [4, 5, 7]. Of these, 1 was a 40-year-old man and the other 2 were in children aged 2 years and 2 year 9 months [4, 5, 7]. Extralobar sequestration in the neonatal period has been not been reported. The majority (95%) of the extra pulmonary sequestrations present on the left side [5, 8]. Our case presented with right sided ELS, which makes the imaging and clinical diagnosis more difficult. Associated congenital anomalies can be congenital diaphragmatic hernia, congenital heart diseases or as in our case, congenital pulmonary airway malformation type 2 or the small cyst type.
Embryogenesis of Ectopic Bronchogenic Cysts: Keep It Simple
Published in Journal of Investigative Surgery, 2020
Cohn et al. [2] provide the classical theory that bronchogenic cysts are generated as a result of abnormal bronchial budding of the small lung bud given from the ventral wall of the pharynx. Others have proposed an origin from an accessory lung bud [3] or from an accessory bronchus [4]. Besides, it should be understood that the primitive trachea and esophagus are packed in a mass of mesenchyme, which will be the future mediastinum. The mediastinum tissue is continuous with the root of the neck cephalad, and the septum transversum just below; the latter will form the tendinous center of the diaphragm. It follows that a detached bronchogenic cyst can be found in these regions, such as at the bifurcation of the trachea (most common), the posterior mediastinum, the chest wall [4]. The cyst may also abandon the diaphragm to invade the abdominal cavity, and end intraperitoneally or extraperitoneally, thus found in the liver or the adrenal gland [4].
Lung regeneration using amniotic fluid mesenchymal stem cells
Published in Artificial Cells, Nanomedicine, and Biotechnology, 2018
Alireza Azargoon, Babak Negahdari
The lung is derived from the foregut endoderm. A quite number of patterning process cause the gut tube separation and other endodermal organs including pancreas, liver, eventually resulting in the generation of the lung buds and trachea [23,24]. Active reciprocal signalling between nearby mesenchyme and developing multipotent distal tip epithelium are necessary for lung buds stereotypical branching morphogenesis, likewise early differentiation events resulting in different cell lineages. The proximal epithelium was firstly formed from proximal progenitors with the emergence of neuroendocrine, basal, ciliated and secretory cells lining the maturing epithelium (Figure 1 and Table 1) [25]. As branching morphogenesis proceeds, bronchioles ultimately branch into alveoli or millions of terminal air sacs, where gaseous exchange took place after birth. The respiratory epithelium is mainly made of two cells before birth; for gaseous exchange-type-I alveolar epithelial cells, and for secreting surfactants-type-II cells needed to stimulate breathing at birth. Lung maturation accomplishment occurs post-natally. As it matures, the turnover of cell decreases and is very slow in the completely mature adult lung (Table 2).