Bronchopulmonary sequestration was first described in 1946 by pryce as an abnormal artery arising from the aorta and supplying a bronchopulmonary mass or cyst that is dissociated from the normal bronchial tree. Pulmonary sequestration ps is a rare disease, of unknown aetiology, representing 0. Bronchopulmonary sequestration is a pulmonary anomaly of nonfunctioning bronchopulmonary tissue that lacks a normal bronchial connection and is characterized by a systemic arterial blood supply. Bronchopulmonary sequestration bps is a congenital anomaly consisting of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its blood supply from the systemic rather than the pulmonary circulation. A pulmonary sequestration also known as bronchopulmonary sequestration is a cystic piece of abnormal lung tissue that does not work like normal lung tissue. A single mass containing a esophageal duplication cyst, cpam, extralobar sequestration, and benign teratoma has been reported. Ankur dave 2014, anesthetic considerations for a patient with intralobar pulmonary sequestration supplied by an aberrant aneurysmal branch. It is characterized by a mass of pulmonary tissue that becomes separated from the normal bronchopulmonary tree and supplied by one or more aberrant systemic arteries 1,2. Pulmonary sequestration is a cystic or solid mass composed of nonfunctioning primitive tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. Broncopulmonary sequestration is a rare congenital abnormality and may be intralobar or extralobar.
Pulmonary sequestration is a rare congenital present from birth malformation where nonfunctioning lung tissue is separated from the rest of the lung and supplied with blood from an unusual source, often an artery from systemic circulation. Pulmonary sequestration radiology reference article. A ct scan of the thorax with intravenous contrast showed a. Arterial supply is from an aberrant systemic artery, usually a branch. Case report bilateral intralobar pulmonary sequestration a case report reuben grech md, mrcsed email. They usually appear next to the lung extrapulmonary, aka extralobar sequestration or within one part of the lung intrapulmonary, aka intralobar sequestration. They usually appear next to the lung or within one part of the lung. Some authors propose a greater male prevalence this may be the case for the. Pediatric pulmonary sequestration clinical presentation. We described the case of a 36yearold turkish female with an extralobar pulmonary sequestration who suffered from chest and back pain for five years without any evidence of pulmonary infection. Pulmonary sequestration ps is a rare congenital malformation. Pulmonary sequestration a broad spectrum of bronchopulmonary foregut abnormalities george l. Bronchopulmonary sequestration bps is a solid lung lesion of nonfunctioning pulmonary tissue, a supernumerary lobe of the lung, which lacks connection to the tracheobronchial tree and receives its blood supply from an aberrant systemic feeding artery, originating commonly from the descending aorta. Bronchopulmonary sequestration is an uncommon congenital lung malformation.
A 46yearold woman with a history of pulmonary infection was referred to our department for imaging. Bronchopulmonary sequestration with massive pleural. Anesthetic considerations for a patient with intralobar. This arterial supply typically originates from the descending aorta and occasionally from the intercostal, celiac or splenic arteries, while venous. Our pdf merger allows you to quickly combine multiple pdf files into one single pdf document, in just a few clicks. Intralobar sequestration of lung associated with an. Pulmonary sequestration also known as accessory lung is a cystic or solid mass of nonfunctioning primitive segmental lung tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. Diagnosis of pulmonary sequestration using imaging methods. It consists of a nonfunctioning mass of normal lung tissue that lacks normal communication with the tracheobronchial tree, and that receives its arterial blood supply from the systemic circulation. Intralobar bronchopulmonary sequestration is a congenital pulmonary disease in which a portion of an otherwise normal lobe has no connection with the bronchial tree or pulmonary arterial circulation, and receives its arterial supply from anomalous branches of. Intralobar pulmonary sequestration in a 46yearold woman.
It is a rare abnormality, representing 015%600% of all pulmonary malformations. The diagnosis of pulmonary sequestration traditionally requires arteriography to identify abnormal systemic vessels feeding the abnormal portion of the lung. The authors describe a case of pulmonary sequestration with calcification in the walls. Case report bilateral intralobar pulmonary sequestration a. Bronchopulmonary sequestration bps is a rare congenital malformation of the lower respiratory tract.
Bronchopulmonary sequestration describes a malformation in which nonfunctioning lung tissue is separated from the normal bronchopulmonary tree and vascularised by an aberrant systemic artery. With bps, a piece of lung tissue develops without being connected to the airways, sometimes inside the lung and sometimes outside of it. As in our patient, the sequestration was located in the right lower lobe figure 1. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and that receives its arterial blood supply from the systemic circulation 1. Green, col, all mc, usa abstract we believe the broad clinical, embryological, and radiological spectrum of pulmonary sequestration has not been adequately emphasized. Usefulness of chest ct in the diagnosis of pulmonary. The past medical history included several hospitalizations for fever and radiographic evidence of pulmonary consolidation in the left lower lobe figure 1a and 1b. First you need to drag and drop the pdf files for merging to the box above in bulk or one by one.
Pulmonary sequestration, also called accessory lung, refers to the aberrant formation of segmental lung tissue that has no connection with the bronchial tree or pulmonary arteries. A case report 185 conclusion pulmonary sequestration is a congenital lesion in which a systemic artery supplies a cystic portion of the lung in which its bronchus. Surgical treatment is elective in patients with symptoms, but the management of. Bronchopulmonary sequestration is an area of lung parenchyma that is devoid of normal connection to the trachebronchial tree.
Soda pdf merge tool allows you to combine pdf files in seconds. Conventional computed tomographic ct scanning is, however, at a disadvantage because of its inability to obtain. A 40yearold man presented with acute onset of left flank pain for 4 hours. Most often the mass is supplied by an anomalous systemic artery and has its own bronchial system, which usually does not commu nicate with the normal bronchial tree. Noninvasive imaging techniques have recently been used to replace arteriography.
The rate of hydrops with bronchopulmonary sequestration ranges from 7% 24 to 35% 26. It is a bronchopulmonary foregut malformation bpfm. Pulmonary sequestration ps is a congenital lung disease characterized by nonfunctioning pulmonary tissue that lacks normal communication with the bronchial tree and is supplied by a nonpulmonary systemic artery. Typically, it consists of a systemic arterial supply to an associated anomalous lung segment with various forms of venous drainage. This free online tool allows to combine multiple pdf or image files into a single pdf document. Bronchopulmonary sequestration bps is a rare malformation of the respiratory tract consisting of a mass of bronchopulmonary tissue that is separate from the tracheobronchial tree and fed by a separate systemic artery. The reduction in size or disappearance of an inutero bronchopulmonary sequestration is thought to be due to torsion andor clotting of the vascular pedicle, or decompression into the normally expanding lung 15,29. The feeding vessel most commonly originates from the aorta, although other origins, such as the. We aim to identify the clinical presentation and course of patients diagnosed to have ps during adulthood. Pdf merge combine pdf files free tool to merge pdf online.
This sequestered tissue is therefore not connected to the normal bronchial airway architecture, and fails to function in, and contribute to, respiration of the. Aberrant arterial supply of pulmonary sequestration. Several reports describe preoperative diagnostic difficulties with this pathology. Approximately 75% of bronchopulmonary sequestrations are. Bronchopulmonary sequestration bps, sometimes referred to simply as pulmonary sequestration, is a rare congenital abnormality of the lower airway. A chest xray showed an area of opacity behind the cardiac silhouette in the lower area of the left hemithorax. A free and open source software to merge, split, rotate and extract pages from pdf files. Using a computerassisted search of mayo clinic medical records, we identified adult patients. A pulmonary sequestration is a medical condition wherein a piece of tissue that ultimately develops into lung tissue is not attached to the pulmonary arterial blood supply, as is the case in normally developing lung.
Bronchopulmonary sequestration is a rare congenital abnormality of the lower respiratory tract, seen mostly in children but often in adults. Intralobar bronchopulmonary sequestration in children. Pulmonary sequestration is a relatively rare entity comprising 0. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and that receives its arterial blood supply from the systemic circulation. Bronchopulmonary sequestration was first reported by huber in 1777, and the term sequestration was first introduced by pryce in 1946. Bronchopulmonary sequestration bps is the second most common congenital lung malformation, with an estimated incidence ranging from 0.
The term implies a mass of lung tissue that has no function and lacks normal communication with the rest of the tracheobronchial tree. Coil embolization of intralobar pulmonary sequestration. Pulmonary sequestration ps is a form of congenital pulmonary malformation that is generally diagnosed in childhood or adolescence and usually resected when diagnosed. Despite antibiotic treatment, the pulmonary opacity had not completely resolved. A pulmonary sequestration, also known as bronchopulmonary sequestration, is a cystic piece of abnormal lung tissue that doesnt function like normal lung tissue. Pulmonary sequestration ucsf fetal treatment center. Calcification in pulmonary sequestration radiology. The roentgen criteria for establishing this diagnosis are. Intralobar bronchopulmonary sequestration with bronchial. Bronchopulmonary sequestration bps is a rare malformation of the lung that occurs before a baby is born. Surgical resection in unsuspected intralobar sequestration can result in fatal hemorrhagic complications.
It is a bronchopulmonary foregut malformation with estimated incidence of 0. Bronchopulmonary sequestrations in a paediatric centre. Congenital cystic adenomatoid malformation and bronchopulmonary sequestration are congenital lung tumors that are classically described as having distinct embryology, pathology, and natural history. Pulmonary sequestrations may be defined as intralobular or extralobular, depending on their location. A discrete foregut duplication cyst, cpam, and extralobar pulmonary sequestration occurring in the same patient are rarely encountered in the literature. Pdf imaging in bronchopulmonary sequestration roman. The abnormal tissue can be microcystic, containing many small cysts, or macrocystic, containing several large cysts. Lung sequestration an overview sciencedirect topics.