Bronchopulmonary sequestration is a rare anomaly of the lung which is characterized by the presence of a mass of lung tissue which has no connection with the normal. With bps, a piece of lung tissue develops without being connected to the airways, sometimes. Surgical resection in unsuspected intralobar sequestration can result in fatal hemorrhagic complications. 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. Pdf pulmonary sequestration ps is a rare congenital lung malformation presented as nonfunctioning lung mass. With bps, a piece of lung tissue develops without being connected to the airways, sometimes inside the lung and sometimes outside of it. The rate of hydrops with bronchopulmonary sequestration ranges from 7% 24 to 35% 26. In adults, the typical age at presentation is 2025 years.
Lung sequestration an overview sciencedirect topics. A pulmonary sequestration also known as bronchopulmonary sequestration is a cystic piece of abnormal lung tissue that does not work like normal lung tissue. Anatomically, it is classified into intralobar sequestration ils and extralobar sequestration els based on the. It is a bronchopulmonary foregut malformation with estimated incidence of 0.
Histologically, the parenchymal changes of bronchopulmonary sequestration can easily be. Bronchopulmonary sequestration and pulmonary tuberculosis. Bronchopulmonary sequestration with mr angiographic. Intralobar sequestration is characterized by aberrant formation of nonfunctional lung tissue that has no communication with the bronchial tree and receives.
Jun 20, 2014 extralobar pulmonary sequestrations may be located in intrathoracic or extrathoracic areas. 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. On rare occasions, recurrent or persistent respiratory tract infections can indicate an underlying congenital lung lesion such as bronchopulmonary sequestration bps, which may present. Pulmonary sequestrationdifferences in diagnosis and. We aim to identify the clinical presentation and course of patients diagnosed to have ps during adulthood. Bronchopulmonary sequestration associated with an aneurysm of. Typically, it consists of a systemic arterial supply to an associated anomalous lung segment with various forms of venous drainage. Ankur dave 2014, anesthetic considerations for a patient with intralobar pulmonary sequestration supplied by an aberrant aneurysmal branch. Pulmonary sequestrationdifferences in diagnosis and treatment in a.
Nov 02, 2016 pulmonary sequestration represents approximately 6% of all congenital pulmonary malformations. Bronchopulmonary sequestration bps is a solid lung lesion of nonfunctioning pulmonary tissue, a supernumerary lobe of the lung, which lacks connection to the tracheobronchial. Bronchopulmonary sequestration bps is the second most common congenital lung malformation, with an estimated incidence ranging from 0. Bronchopulmonary sequestrations in a paediatric centre. Utility of sonography in the diagnosis of bronchopulmonary. Bronchopulmonary sequestration bps is a rare congenital malformation of the lower respiratory tract.
Pulmonary sequestration ps is a rare congenital lung malformation. Bronchopulmonary sequestration was discovered in a 36yearold man being treated for pulmonary tuberculosis. 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. Pulmonary sequestration is acystic or solid congenital lung malformation comprised of non functional lung tissue that does not communicate with the normal. Mri demonstrates aberrant artery originating from aorta c. Bilateral pulmonary sequestration in the elderly adult. Thoracoscopic treatment of pulmonary sequestration core. This report of a patient with intralobar pulmonary sequestration demonstrates that this clinical entity can be diagnosed antemortem 114. A pulmonary sequestration, also known as bronchopulmonary sequestration, is a cystic piece of abnormal lung tissue that doesnt function like normal lung tissue. Intralobar bronchopulmonary sequestration in children. The possibility of lifethreatening hemorrhage resulting from failure to control these aberrant vessels has led many. Bronchopulmonary sequestration bps is a lung mass that does not communicate with the tracheobronchial tree or the pulmonary arterial vasculature, and thus does not play a role in oxygenation. 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.
Anatomically, it is classified into intralobar sequestration ils and extralobar sequestration els based on the relationship of the aberrant segmental lung tissue to the pleura. Pulmonary sequestration an overview sciencedirect topics. Chest xray revealed a welldefined opacity in the right lower zone, projected over the medial aspect of the right hemidiaphragm figure 1. Aug 12, 2016 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. Anesthetic considerations for a patient with intralobar. However, ps is usually seen unilaterally but, only rarely, it is bilateral. Using a computerassisted search of mayo clinic medical records, we identified adult patients with ps diagnosed between. Bronchopulmonary sequestration bps is a nonfunctioning bronchopulmonary tissue that is separate from the tracheobronchial tree and receives arterial blood from the systemic circulation.
Diagnosing intralobar pulmonary sequestration archives. Prenatal sonographic markers of the outcome in fetuses with. Bronchopulmonary sequestration is an uncommon pulmonary disorder consisting of a segment of nonfunctioning lung parenchyma that has no communication with the tracheobronchial tree and. Extrathoracic intradiaphragmatic extralobar pulmonary sequestrations are an extremely rare subset of bronchopulmonary sequestrations and there have been very few reported cases until now. Utility of sonography in the diagnosis of bronchopulmonary sequestration by alan e. It is a bronchopulmonary foregut malformation bpfm there are two types. Incidental finding of bronchopulmonary sequestration in a 64. It is a rare abnormality, representing 015%600% of all pulmonary malformations. Intradiaphragmatic extralobar pulmonary sequestration in. Intralobar bronchopulmonary sequestration with bronchial. Uniportal thoracoscopic lobectomy for intralobar pulmonary. Extrathoracic intradiaphragmatic extralobar pulmonary sequestrations are an extremely rare. Bronchopulmonary sequestration, sometimes referred to simply as pulmonary sequestration, is a rare congenital malformation of the lower respiratory tract. Methods bps size and diameter of the feeding artery fa.
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. Pulmonary sequestration is a rare congenital anomaly, and the presence of an aneurysmal feeding vessel is even rarer. Pulmonary sequestration ps is a rare congenital malformation. They usually appear next to the lung or within one part of the lung.
There appears to be a spectrum of sequestration with, at one extreme, an abnormal vessel supplying a. Cardiovascular and respiratory manifestations of pulmonary. Noninvasive imaging of bronchopulmonary sequestration. The abnormal tissue can be microcystic, containing many small cysts, or macrocystic, containing several large cysts. This article discusses the etiology of bps, as well as its pathophysiology, signs and symptoms, imaging studies used to diagnose, and. Diagnosis of pulmonary sequestration using imaging methods. Extralobar pulmonary sequestrations may be located in intrathoracic or extrathoracic areas.
Incidental finding of bronchopulmonary sequestration in a. Repeat xrays demonstrated no change in the opacity. Pulmonary sequestration is a relatively rare entity comprising 0. Bronchopulmonary sequestration is a rare congenital abnormality of the lower respiratory tract, seen mostly in children but often in adults.
We describe a 48yearold korean woman found to have left peridiaphragmatic lesion on computed tomography. Bronchopulmonary sequestration and pulmonary tuberculosis chest. This sequestered tissue is therefore not connected to the normal bronchial airway architecture, and fails to function in, and contribute to, respiration of the organism. Typically, it consists of a systemic arterial supply to an. Most patients with ps are diagnosed because of symptoms due to pulmonary infection or cardiac disease.
Pulmonary sequestration is an uncommon congenital condition for which surgical resection is usually indicated either via open thoracotomy or conventional multiport videoassisted. Her medical history was significant for recurrent right lower lobe pneumonia requiring multiple hospitalizations. 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. Bronchopulmonary sequestration bps is a mass of nonfunctioning lung tissue that does not communicate with the bronchioles, the passages that move air and in out of the lungs. Bronchopulmonary sequestration childrens hospital colorado. It consists of a nonfunctioning mass of lung tissue that receives its vascular supply from the systemic rather than pulmonary circulation, and it lacks. Bronchopulmonary sequestration is a congenital abnormality within the spectrum of bronchopulmo nary foregut malformationslx in which a seques tered portion of lung parenchyma, without normal bronchial communication, has an anomalous systemic fig 2. A axial sonographic image at the level of the sequestration. The sequestration was intralobar in 14 patients and extralobar in 3. The diagnosis of pulmonary sequestration traditionally requires arteriography to identify abnormal systemic vessels feeding the abnormal portion of the lung. Roland brusseau, in a practice of anesthesia for infants and children sixth edition, 2019. The patient was treated successfully by resection of the right lower lobe with the sequestered lung tissue and aneurysm. 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.
The roentgen criteria for establishing this diagnosis are. Definitive diagnosis of pulmonary sequestration requires angiographic visualization of the anomalous feeding and draining vessels. Pdf contemporary management of pulmonary sequestration. This diagnosis was suggested by a murmur heard over the left costovertebral. A 64yearold female was referred for evaluation of an 8. Bronchopulmonary sequestration associated with an aneurysm. 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 the aorta. Bronchopulmonary sequestration bps, sometimes referred to simply as pulmonary sequestration, is a rare congenital abnormality of the lower airway. Extralobar pulmonary sequestration as a cause of recurrent. It consists of a nonfunctioning mass of lung tissue. 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. Bronchopulmonary sequestration bps is a congenital anomaly consisting of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree. The term implies a mass of lung tissue that has no function and lacks. Intrapulmonary sequestrations are the most common form, and 60% of these are found in the posterior basal segment of the left lower lobe.
Wed like to understand how you use our websites in order to improve them. Intralobar pulmonary sequestration with aneurysmal feeding. 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. It is a bronchopulmonary foregut malformation bpfm. Surgical treatment is elective in patients with symptoms, but the management of asymptomatic patients remains controversial. Ct appearance among various noninvasive imaging techniques, ct provides the best display of the parenchymal abnormalities in bronchopulmonary sequestration 24, 6. This diagnosis was suggested by a murmur heard over the left costovertebral angle in a 4 year 7monthold child with a history of recurrent pneumonia, and a persistent mass noted in retrospect on a series of chest xray films.
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. An 11yearold girl presented with abdominal pain and lethargy. Several reports describe preoperative diagnostic difficulties with this pathology. In intralobar pulmonary sequestration the bronchopulmonary tissue is situated within the visceral pleura of a lower lobe of the lung, and venous drainage is. Pulmonary sequestration ps is a form of congenital pulmonary malformation that is generally diagnosed in childhood or adolescence and usually resected when diagnosed. Pulmonary sequestration ucsf fetal treatment center. Bronchopulmonary sequestration is a congenital abnormality of the primitive foregut. Intralobar bronchopulmonary sequestration demonstrated by.
Pulmonary sequestration is vascularized by an aberrant systemic artery, most frequently from the descending thoracic or abdominal aorta. It is a bronchopulmonary foregut malformation with. Abstract purpose to evaluate the prenatal sonographic predictive markers of the outcome in fetuses with bronchopulmonary sequestration bps. Pulmonary sequestration radiology reference article.
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. Ct scan shows the area of consolidation in the hyperaerated abnormal lung. Request pdf bronchopulmonary sequestration bronchopulmonary sequestration is a congenital developmental lung pathology characterized by a supernumerary lung tissue that receives its blood. Pulmonary sequestration, also known as bronchopulmonary sequestration, accessory lung, or bronchopulmonary foregut malformation, represents 0. 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. We describe the endovascular exclusion and surgical resection of an intralobar pulmonary sequestration supplied by an aneurysmal feeding vessel from the aorta. Bronchopulmonary sequestration presenting as a spontaneous. Bronchopulmonary sequestration describes a malformation in which nonfunctioning lung tissue is separated from the normal bronchopulmonary tree and vascularised by an aberrant systemic artery. She was treated with two courses of antibiotics but remained symptomatic with a cough. Pulmonary sequestration ps is a rare malformation consisting of aberrant lung tissue which is not affiliated with the normal bronchial system and is fed by an aberrant artery that derives. Pulmonary sequestration is a rare congenital malformation characterised by a mass of nonfunctioning lung tissue separated from the normal bronchopulmonary tree and vascularised by an aberrant systemic artery. They usually appear next to the lung extrapulmonary, aka extralobar sequestration or within one part of the lung intrapulmonary, aka intralobar sequestration. 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.
In this study, we evaluated the diagnosis and treatment of ps in 31 adult. Bronchopulmonary sequestration was first reported by huber in 1777, and the term sequestration was first introduced by pryce in 1946. We describe a patient with an intralobar bronchopulmonary sequestration of the right lower lobe associated with a true aneurysm of the aberrant artery. Bronchopulmonary sequestration bps is a mass of nonfunctioning pulmonary tissue that lacks an obvious communication with the tracheobronchial tree and receives all or most of its blood supply from anomalous systemic vessels carter, 1959. Diagnosing intralobar pulmonary sequestration archives of. Bronchopulmonary sequestration was diagnosed in 17 patients ranging in age from newborn to 64 years. Bronchopulmonary sequestration bps is a mass of nonfunctioning pulmonary tissue that lacks an obvious communication with the tracheobronchial tree and receives all or most of its blood supply. Prenatal sonographic markers of the outcome in fetuses. The purpose of this article is to report a case of intralobar bronchopulmonary sequestration in a child. Bronchopulmonary sequestration bps is a rare malformation of the lung that occurs before a baby is born. 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. Oct 22, 2012 pulmonary sequestration ps is a rare malformation consisting of aberrant lung tissue which is not affiliated with the normal bronchial system and is fed by an aberrant artery that derives from systemic arteries. Bronchopulmonary sequestration bps is a lung mass that does not communicate with the tracheobronchial tree or the pulmonary arterial vasculature, and thus does not play a role in.
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