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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Pulmonary sequestration tends to present in childhood and is more common in males. The extralobar subtype usually presents earlier than intralobar sequestration. The anomaly is a segment of lung with no communication to the pulmonary arteries or bronchial tree.
Intralobar Pulmonary Sequestration with Aberrant Venous Drainage
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Julia Coughlin, Christopher W. Seder
Chest X-ray and CT scan are noninvasive modalities that aid in the diagnosis of pulmonary sequestration. In the current case, a chest X-ray demonstrated the typical findings of ILS: pneumonia in the medial basal posterior left lower lobe. However, the contrast-enhanced CT scan demonstrated an arterial and venous blood supply pattern generally seen in ELS, making the diagnosis unclear. Savic et al. conducted an analysis of the characteristics of intralobar and extralobar sequestrations and found more variation in the venous return of ELS than ILS. Over 95% of ILS cases demonstrated venous return via the pulmonary vein. In the remaining few cases, venous return was supplied by the azygous (1%), hemiazygous (1%), inferior vena cava (1%), superior vena cava (0.75%), and intercostal vein (0.75%) [2]. Although the majority of ELS drained via the superior vena cava, 52 of the 133 ELS cases were drained by alternative routes, such as the hemiazygous vein (15%), pulmonary vein (8%), inferior vena cava (4%), azygous vein (3%), portal vein (3%), intercostal vein (0.75%), and superior renal vein (0.75%) [2]. Thus, although imaging serves as helpful diagnostic tool, only surgical exploration allows the establishment of an accurate diagnosis.
The thorax
Published in Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague, Paediatric Surgical Diagnosis, 2018
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague
Pulmonary sequestration occurs where lung tissue is separated from the tracheobronchial tree and pulmonary vessels. It is a congenital malformation comprising nonfunctioning lung tissue with an abnormal or absent bronchial communication and a blood supply from anomalous systemic arteries, which usually arise from the aorta. The abnormal lung tissue may be incorporated within normal lung (intralobar sequestration) additional to the normal pulmonary segments. When the abnormal pulmonary tissue has its own pleural covering, it is called extralobar sequestration. It may lie below, within or above the diaphragm and receives a blood supply directly from the aorta. The pulmonary structures (bronchial and alveolar elements with mucus-secreting epithelium) without normal communications may develop static accumulations of secretions, which are prone to infection. The vessels may form significant arteriovenous shunts, especially in the first year of life. In older children, sequestration becomes apparent when there are persistent radiological changes after respiratory infection. A correct diagnosis before thoracoscopy or thoracotomy enables the abnormal systemic artery that supplies the sequestered segment to be identified more easily at surgery.
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.
Congenital Pulmonary Airway Malformation – 19-Year Experience from a Tertiary Care Center in India
Published in Fetal and Pediatric Pathology, 2019
Hema Kini, Saraswathy Sreeram, Saumya Shukla, Sadashiva Rao, Kausalya Sahu, Deepa Adiga, Pooja Suresh
In the 10 postnatal cases, four were not detected in the antenatal scan. Two cases were thought to be lung abscess and pneumonia because the children presented with high-grade fever (Table 1, Cases 6 and 8, respectively). CPAM types 1 and 2 were detected on histopathological examination. Superimposed acute inflammation was seen, however, microbiological culture were not obtained. Patients were started on broad-spectrum antibiotics. One of the cases had an intra-operative diagnosis of extralobar sequestration (Table 1, case 9). CPAM type 2 was incidentally detected on microscopy. Pulmonary sequestration with CPAM has been reported [9]. In another case, a congenital diaphragmatic hernia was suspected and the specimen was sent to prove eventration and look for skeletal muscle (Table 1, case 7). CPAM type 2 was a surprise diagnosis. The patient also had an associated TEF.
Management of severe hemoptysis
Published in Expert Review of Respiratory Medicine, 2018
Antoine Parrot, Sebastian Tavolaro, Guillaume Voiriot, Antony Canellas, Jalal Assouad, Jacques Cadranel, Muriel Fartoukh
In terms of the etiology of bleeding, MDCTA is the relevant procedure because it can diagnose the cause as bronchiectasis, sequelae of tuberculosis, or neoplastic disease. One must be careful of the pitfalls of alveolar flooding. Localized opacification that appears to be of tissue and is surrounded by ground glass may be a tumor opacity or a result of alveolar flooding. Nor can MDCTA distinguish between an intraluminal thrombus and a small tumor. In more rare cases, it reveals congenital abnormalities like pulmonary sequestration or agenesis of the pulmonary artery. For determining the cause of bleeding, MDCTA performs significantly better than bedside examinations like history-taking, chest radiography, and FOB [41,42]. The etiology is important to investigate, since cryptogenic hemoptysis is never at the root of a pulmonary mechanism, whereas some etiologies like cancer and infection (such as bacterial lung disease and tuberculosis) may display a dual mechanism that causes hemoptysis [19,22].