It is more common in infants with low birth weight lbw. Extralobar pulmonary sequestration simulates posterior neurogenic. 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. The alveoli that are present tend to not be mature enough to function normal. Both forms derive their blood supply from the systemic circulation. Abstract bronchopulmonary sequestration was diagnosed in 17 patients ranging in age from new born to 64 years. Bronchopulmonary foregut malformations bpfm are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. 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. Nov 02, 2016 pulmonary sequestration represents approximately 6% of all congenital pulmonary malformations. Pryces traction theory is generally felt to be the most accurate explanation of this pathology.
Pathology demonstrated extralobar bronchopulmonary sequestration with a separate foregut malformation most consistent with bronchogenic cyst fig. 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. Several methods of treatment have been described since the first publication. The widespread use of antenatal ultrasound examination has resulted in an increase in the prenatal diagnosis of cpam 3,4. Green, col, all mc, usa abstract we believe the broad clinical, embryological, and radiological spectrum of pulmonary sequestration has not been adequately emphasized. Pulmonary sequestration ucsf fetal treatment center. Intralobar pulmonary sequestration masquerading as lung. Jun 22, 2018 he was diagnosed with an infected intralobar bronchopulmonary sequestration and underwent videoassisted thoracoscopic wedge resection. Jan 30, 2019 intraabdominal extralobar pulmonary sequestration ieps is a rare type of pulmonary sequestration ps. Ankur dave 2014, anesthetic considerations for a patient with intralobar pulmonary sequestration supplied by an aberrant aneurysmal branch.
Imaging in bronchopulmonary sequestration abbey 2009. Ps is a spectrum of bronchopulmonary foregut anomalies, characterized overall as non. Bronchopulmonary sequestration bps is most of the times simply referred to as pulmonary sequestration and is estimated to comprise 0. Pulmonary sequestration was first described in 1861 by rektorzik as an embryonic mass of lung tissue that has no identifiable bronchial communication and receives its own blood supply from an anomalous systemic artery with an origin at either the thoracic or abdominal aorta. Ccam and bronchopulmonary sequestration are congenital lung masses that are traditionally described as having distinct embryology, pathology and natural history. It is comprised of nonfunctioning primitive lung tissue, having no communication with the tracheobronchial tree and contains a systemic arterial supply. Bronchopulmonary sequestration childrens hospital colorado. Extralobar pulmonary sequestration with absence of pericardium and atrial septal defect in a woman zhenhuan tian1, yuncan zhou2 and hongsheng liu1 abstract background. This study evaluated the accuracy of postnatal computed tomography ct imaging in the identification of congenital bronchopulmonary. Pediatric pulmonary sequestration clinical presentation. Extralobar pulmonary sequestration elps are segmentsof lung tissue accessory lungs with a distinct pleural investment and complete anatomic and physiologic seprartion from the normal lung. Although rare, it is the most common congenital lung lesion. Bronchopulmonary sequestration presenting as a spontaneous.
Sagittal image a of the chest of a 24week fetus referred for a congenital pulmonaryairway malformation shows a homogeneoussolid lung lesion asterisk in the region of the left lower lobe. Bronchopulmonary sequestration was discovered in a 36yearold man being treated for pulmonary tuberculosis. Bronchopulmonary sequestration bps is a rare malformation of the lung that occurs before a baby is born. Pulmonary sequestration causing congestive heart failure in infancy. Sequestrations intralobar and extrapulmonary lobar author. Pulmonary sequestration, also known as bronchopulmonary sequestration, accessory lung, or bronchopulmonary foregut malformation, represents 0. Management of congenital cystic adenomatoid malformation and. The immature infant will breathe some level of oxygen, and even room air represents a hyperoxic exposure. Bronchopulmonary sequestration was first reported by huber in 1777, and the term sequestration was first introduced by pryce in 1946. 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. This arterial supply typically originates from the descending aorta and occasionally from the intercostal, celiac or splenic arteries, while venous drainage. Bronchopulmonary sequestration is a rare malformation of the lower respiratory tract. 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 pulmonary sequestration masquerading as.
A pulmonary sequestration also known as bronchopulmonary sequestration is a cystic piece of abnormal lung tissue that does not work like normal lung tissue. Bronchopulmonary sequestration bps is a rare developmental disorder in which part of the lung is disconnected from the main airway and receives aberrant arterial inflow from the aorta instead of from the pulmonary arteries. Intralobar pulmonary sequestration ips, right lower lobe, lobectomy, histopathology. Pulmonary sequestration is a continuum of lung anomalies for which no single embryonic hypothesis is yet available. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. Blood supply is usually by anomalous systemic artery with venous drainage into the azygos or hemizygos system the abnormal tissue may be located either above or below the.
Pathology of bronchopulmonary dysplasia jacqueline j. Conventionally, bronchopulmonary sequestration has been categorized into intralobar and. Pulmonary sequestration associated with increased serum tumo. Bronchopulmonary malformations refer to a spectrum of pulmonary developmental anomalies, including congenital pulmonary airway malformation cpam, previously known as congenital cystic adenomatoid malformation ccam, bronchopulmonary sequestration bps, hybrid lesion and congenital lung overinflation clo.
Diagnosis of pulmonary sequestration by magnetic resonance imaging. Bronchopulmonary sequestration bps is a rare congenital malformation of the lower respiratory tract. Usefulness of chest ct in the diagnosis of pulmonary. Bronchopulmonary sequestrations constitute approximately 0. Histologically, the parenchymal changes of bronchopulmonary sequestration can easily be. The authors conclude that ct can be helpful in the diagnosis and evaluation of bronchopulmonary sequestration. Intralobar sequestration accounts for 75% of pulmonary sequestrations. Prenatal diagnosis of fetal intraabdominal extralobar. Pulmonary sequestration a broad spectrum of bronchopulmonary foregut abnormalities george l. Pathology of the lung excluding pulmonary tuberculosis. Sequestration is a rare congenital abnormality, representing 0. Observations in patients with intralobar sequestration with or without an aberrant systemic artery were made on a 3dimensional reconstruction of bronchoalveolar components and afferent and efferent vessels.
Pulmonary sequestration is a congenital anomaly resulting in nonfunctioning lung tissue in either the extralobar or intralobar position. Lung sequestration an overview sciencedirect topics. 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. Pulmonary sequestration an overview sciencedirect topics. Noninvasive imaging of bronchopulmonary sequestration. Bronchopulmonary sequestration is a benign, rare lung abnormality. A report to rein force the need to excise the hernia sac.
Complication of mechanical ventilation and oxygen therapy for treatment of neonatal respiratory distress syndrome infants almost always bronchopulmonary sequestration is a rare congenital abnormality of the lower respiratory tract, seen mostly in children but often in adults. Classically identified due to recurrent infections or bronchiectasis. They usually appear next to the lung or within one part of the lung. 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.
Ct appearance among various noninvasive imaging techniques, ct provides the best display of the parenchymal abnormalities in bronchopulmonary sequestration 24, 6. Metastatic oesophegeal adenocarcinoma to an intralobar bronchopulmonary sequestration. A 40yearold man presented with acute onset of left flank pain for 4 hours. Pdf pulmonary sequestration ps is a rare congenital lung malformation presented as nonfunctioning lung mass, abnormally supplied by an. The most plausible theory is the formation of an accessory supernumerary lung bud below the normal lung bud. Bronchopulmonary dysplasia is the end result of lung injury in a developmentally immature host, so developmental similarities should exist between the animal model and the human disease. 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. Relationship to intralobar pulmonary sequestration. Thoracic ct showing a lesion on right lower lobe with. Pulmonary sequestration radiology reference article. Extralobar pulmonary sequestration with absence of. The term implies a mass of lung tissue that has no function and. 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. Pulmonary sequestration and related congenital bronchopulmonary vascular malformations.
With bps, a piece of lung tissue develops without being connected to the airways, sometimes inside the lung and sometimes outside of it. Bronchopulmonary sequestration with massive pleural. Ccam is considered to represent a hamartomatous change in the tertiary bronchioles, and has an incidence of 1 in 25,000 to 35,000 live births. Histopathology confirmed the diagnosis of intralobar sequestration ils. It is a bronchopulmonary foregut malformation bpfm. Intralobular sequestration can be considered a variant of els. The term implies a mass of lung tissue that has no function and lacks normal communication with the rest of the tracheobronchial tree. Congenital cystic adenomatoid malformation and bronchopulmonary sequestration are congenital lung tumors that are classically described as having distinct embryology, pathology, and natural history. Intrathoracic extralobar bronchopulmonary sequestration. 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. Bronchopulmonary sequestration is a rare congenital abnormality of the lower respiratory tract, seen mostly in children but often in adults. Introduction bronchopulmonary sequestration bps or sequestration is a rare congenital abnormality of the lower respiratory tract.
Bronchopulmonary sequestration and pulmonary tuberculosis. Coalson, phd over the past three decades, advances in prenatal and neonatal intensive care have contributed to marked improvements in survival rates for extremely immature infants born during the canalicular phase of lung development at 24 to 26 weeks, a time when alveolar. The rate of hydrops with bronchopulmonary sequestration ranges from 7% 24 to 35% 26. Pulmonary sequestration is a very rare anomaly in the pediatric age group, especially intralobar variety. Congenital pulmonary airway malformation cpam is a developmental malformation of the lower respiratory tract. Pulmonary sequestration is a rare congenital anomaly of a mass of lung tissue, which can have cystic changes and is a very important differential. Lung nontumor cystic disease congenital anomalies bronchopulmonary dysplasia. Pdf contemporary management of pulmonary sequestration. They usually appear next to the lung extrapulmonary, aka extralobar sequestration or within one part of the lung intrapulmonary, aka intralobar sequestration. Fetal pleuroamniotic shunting for bronchopulmonary.
Large pulmonary sequestrations can present in the newborn with potentially fatal respiratory distress. Histopathology examination confirmed a diagnosis of pulmonary sequestration. Bps belongs to the group of foregut malformations and is. Pulmonary sequestrations may be defined as intralobular or extralobular, depending on their location. 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. Characteristic manifestations are a a complex lesion containing solid or fluid components combined with emphysematous lung or b any basal lesion supplied by a systemic artery. Extralobar sequestration is a rare congenital malformation of lung tissue, which. 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.
Ccam and bronchopulmonary sequestration bps are major categories of congenital pulmonary malformations. Bronchopulmonary sequestration is an uncommon pulmonary disorder consisting of a segment of nonfunctioning lung parenchyma that has no communication with the tracheobronchial tree and receives its blood supply via an aberrant systemic artery 1,2,3,4. Left side congenital diaphragmatic hernia with sac and. 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. Bronchopulmonary sequestration bps, sometimes referred to simply as pulmonary sequestration, is a rare congenital abnormality of the lower airway.
The abnormal tissue can be microcystic, containing many small cysts, or macrocystic, containing several large cysts. The postoperative course was uncomplicated, and the infant was discharged home on the second postoperative day. Bps belongs to the group of foregut malformations and is often accompanied by other defects. 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. Histologically, lung parenchyma in the sequestered segment displays evidence of lymphocytic inflammation and. Sections of the mass showed lung parenchymal tissue comprising dilated.
The diagnosis of lung carcinoma could not be confirmed on the basis of the histopathology report. Abstract broncho pulmonary sequestration is a rare anomaly which represents 0. A sequestrated lung presenting as lung abscess a rare case. A pulmonary sequestration, also known as bronchopulmonary sequestration, is a cystic piece of abnormal lung tissue that doesnt function like normal lung tissue. The preoperative diagnosis is usually not a pulmonary sequestration in most of the patients. Anesthetic considerations for a patient with intralobar. Some authors propose a greater male prevalence this may be the case for the. Pulmonary sequestration the annals of thoracic surgery.
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