Preoperative evaluation of the aberrant artery in intralobar pulmonary sequestration using multidetector computed tomography angiography

Intralobar pulmonary sequestration is a rare congenital malformation of the lung, and identification of the aberrant artery from the systemic circulation to the sequestered lung is crucial in definitive surgery. Nowadays, various noninvasive imaging modalities such as helical computed tomography, magnetic resonance imaging, and color Doppler sonography have been developed. Among them, multidetector computed tomography angiography, which allows simultaneous imaging of the aberrant artery and venous drainage, has the potential to become the first-line examination in the preoperative assessment of pulmonary sequestration. In this article, we describe 2 cases of intralobar pulmonary sequestration, in which multidetector computed tomography angiography was useful in the preoperative management and surgery was successfully performed.     

The spectrum of pulmonary sequestration

An intralobar sequestration with the unusual anatomical finding of both pulmonary and systemic (azygos) venous drainage is presented in detail. A review of the literature pertaining to pulmonary sequestration revealed a continuum of lung anomalies in which nearly every combination of pulmonary and systemic arterial supply, pulmonary and systemic venous drainage, normal and abnormal pulmonary tissue, gastrointestinal fistula, and defective diaphragm was described. Since no single anatomical mechanism can account for all these anomalies, the spectrum can best be explained at this time as a defect or defects of morphogenesis in the embryonic thorax. The surgeon must be aware of this spectrum of anomalies in order to remain alert to the possibility of unusual blood vessels and gastrointestinal fistula during operation for any cystic or suppurative lesion of the lungs.     

Intralobar pulmonary sequestration

Sequestration is defined as an area of abnormal pulmonary tissue not connected with the bronchial tree, supplied by an aberrant systemic artery and without a normal pulmonary function. Extralobar (ELS) and intralobar (ILS) forms are distinguished. During the year 2002 the authors diagnosed and operated upon two cases of the intralobar form of pulmonary sequestration, and in last 25 years five cases - 4 x ILS and 1 x ELS. Reported are a 35 year old man with relapsing infections of the sequester and a 21 year old woman where the sequestration was accidentally found without clinical symptoms. The focus was localized in both cases in the left lower lobe of the lungs, anomalous supply arteries derived from the thoracic aorta. Venous drainage of the sequester was different - in the man a systemic drainage via the v. azygos, in the woman via the pulmonary veins was found. In one case the diagnosis was made on the basis of angiography and computer tomography, in the other case it was made on the basis of multidetector CT angiography (MDCTA). Both findings were treated by primary surgical intervention lobectomy. The postoperative course was uneventful.     

Pulmonary sequestration presenting as a massive haemoptysis in adult: A case report

IntroductionPulmonary sequestration is a rare congenital lung malformation characterized by non-functioning mass of pulmonary tissue that lacks normal communication with bronchial tree and receives one or more aberrant systemic arterial supply. It usually presents in children with recurrent chest infections. It is uncommon in adult and remains asymptomatic or present with recurrent chest infections, rarely with more severe symptoms like a massive haemoptysis as in seen in our case.Case presentationA 22-year-old male presented with a chief complain of multiple episodes of massive haemoptysis. After evaluation with chest x-ray, CT chest and angiogram, he was diagnosed to have intralobar pulmonary sequestration. He underwent successful thoracotomy and left lower lobectomy. He is asymptomatic after one year of follow up.DiscussionPulmonary sequestration is rare in adult and can present with various symptoms like chest pain, cough, sputum production, recurrent infection and rarely haemoptysis. Intralobar sequestration of left lower lobe is the most common as seen in our case. The diagnosis can be made by Computed Tomogram chest with angiogram. Surgical intervention is the definitive curative treatment. Post-operative outcome is excellent with early patient satisfaction and promising outcome in long term follow-up.ConclusionBronchopulmonary sequestration can rarely present in adults presenting with massive haemoptysis. CT chest is the best modality for diagnosis. Early surgical intervention is definitive treatment with good long term outcome.     

Intralobar pulmonary sequestration supplied by an anomalous aneurysmal artery

Pulmonary sequestration is a relatively rare condition in which a systemic artery supplies blood to an abnormal lung tissue. Pulmonary sequestration with an aneurysmal systemic artery is extremely rare. We describe the case of a 52-year-old man with intralobar pulmonary sequestration supplied by an aneurysmal systemic artery. Because the nomenclature of pulmonary sequestration is still not clear, we propose that type 1 intralobar pulmonary sequestration be called "systemic arterial supply to the normal lung," as named by many professionals, and for this to be distinguished from pulmonary sequestration.     

Intralobar pulmonary sequestration treated by resection of the sequestrated segment

Segmentectomy including the sequestrated segment and lobectomy are generally performed for intralobar pulmonary sequestration. We report a case of intralobar pulmonary sequestration of Pryce type III treated by resection of only the sequestrated segment. A 57-year-old man presented with a 5 x 3 cm mass shadow overlapping a cardiac shadow on the left lower lung field on chest radiograph at medical examination. The mass was not connected with the bronchial tree, and was supplied by an aberrant artery arising from the thoracic descending aorta. The mass was suspected to exist in the normal visceral pleura from chest images. The mass was suspected to be an intralobar pulmonary sequestration from the abovementioned findings, and we performed an operation. At the beginning of the procedure, after dissecting the aberrant artery, only the sequestrated segment was performed.     

Neonatal hyperreninemia and hypertensive heart failure relieved with resection of an intralobar pulmonary sequestration

Intralobar pulmonary sequestrations are congenital lung malformations sometimes known to cause symptoms of heart failure in the neonate because of shunting of blood flow from an anomalous systemic feeding artery into the pulmonary venous system. These are cured by pulmonary lobectomy. We report the case of a 37-weeks' gestational age infant who presented with bilateral pleural effusions perinatally, followed by hypertensive heart failure and hyperreninemia 2 weeks postnatally. Computed tomography revealed a large artery from the infradiaphragmatic aorta proximal to the celiac axis supplying a right lower lobe intralobar pulmonary sequestration and 4 small nonstenosed renal arteries. Subsequent to a right lower lobectomy, the patient recovered with a precipitous drop in plasma renin assay level and was discharged from the neonatal intensive care unit in good condition 12 days postoperatively. This is the first reported case of hyperreninemia treated by lobectomy. We hypothesize that the aberrant feeding vessel resulted in a relative deficiency in renal perfusion via a “steal” effect that was relieved by surgical ligation of the artery.     

Extralobar pulmonary sequestration with an unusual venous drainage to the portal vein: preoperative diagnosis and excision by video-assisted thoracoscopy

Pulmonary sequestration is an uncommon congenital malformation of the lung that can be classified as intralobar or extralobar (ELS). Approximately 90% of ELS occur in the left hemithorax. Approximately 10% of ELS may present below the diaphragm. Both types of sequestrations are characterized by pulmonary tissue that does not communicate with the bronchial tree. The arterial blood supply to 80% ELS is through a direct branch of the thoracic or abdominal aorta, in 15% via another systemic artery and 5% from the pulmonary artery. The venous drainage of ELS is variable, predominantly into the systemic circulation (via the azygos vein, hemiazygos vein, or inferior vena cava). Approximately 25% drain completely or partially through the pulmonary veins. We report an extremely rare case of ELS with unusual venous drainage to the portal vein.     

Intralobar sequestration. A missed diagnosis

Intralobar pulmonary sequestration is an uncommon but distinct clinical entity that may be the unrecognized cause of recurrent pulmonary infections. Between 1967 and 1987, 10 patients, ranging in age from 5 to 39 years, were found to have an intralobar sequestration. Nine patients (90%) had a history of recurrent pulmonary infections, chronic cough, and intermittent fevers. One patient was asymptomatic. Many patients had been treated with antibiotics on numerous occasions. The delay in diagnosis varied between 3 months and 7 years (mean delay, 1.5 years). The chest roentgenogram was abnormal in all patients. The intralobar sequestration was present in the left lower lobe in 7 patients and the right lower lobe in 3 patients. Bronchography was abnormal in 4 patients in whom it was done. Bronchoscopy was performed in 7 patients, but it was only helpful in excluding other diagnoses. Preoperative thoracic arteriography in 9 patients visualized the systemic arterial supply from the thoracic or abdominal aorta to the intralobar sequestration and helped prevent any catastrophic surgical bleeding. A lobectomy was performed in 9 patients and a segmentectomy in 1 patient without morbidity or mortality. In patients with recurrent infections in the same lower lobe, a high index of suspicion for an intralobar sequestration should prompt early diagnostic arteriography and, if confirmed, early operative intervention.     

Anomalous systemic arterial supply to normal basal segments of the left lower lobe.

BACKGROUND: Anomalous arterial supply to the normal basal segments of the lower lobe without sequestration is a rare congenital abnormality, and whether it belongs to the broad spectrum of sequestration disorders remains controversial. METHODS: The cases of all 4 patients who were treated surgically by us were reviewed together with 8 previously reported cases. RESULTS: The anomalous artery originated from the descending thoracic aorta, distributed to the basal segments of the left lower lobe, and drained to a normal inferior pulmonary vein in each case. The anomalous artery was thick and elastic walled. From the review of all 12 cases, male gender, left side, descending thoracic aorta as the aberrant arterial origin, absence of pulmonary blood flow to the basal segments, and normal pulmonary venous drainage were predominant. Despite some differences, the findings seemed closely related to intralobar sequestration. Surgical treatments were lung resection, anastomosis, and ligation of the anomalous artery. CONCLUSIONS: This anomaly is probably one type of sequestration complex. Both aortic and pulmonary arterial angiographic studies are needed to plan the definitive surgical procedure.     

Video-assisted thoracoscopic lobectomy in the treatment of intralobar pulmonary sequestration

Pulmonary sequestration is a congenital malformation characterised by cystic, non-functioning embryonic lung tissue with vascularisation of an abnormal systemic artery. They are classified as intralobar (75%) and extralobar (25%) and are more common in the left lung and lower lobes (60-90%). We report two cases of intralobar pulmonary sequestration located in the lower lobe of the left lung which were subjected to video-assisted thoracoscopic surgery (VATS). Both patients had recurrent infections for which, after performing imaging tests, they were diagnosed with intralobar pulmonary sequestration in the left lower lobe, with an afferent arterial branch to the malformation from the aorta. A lower lobectomy was performed by video-assisted surgery, dividing the aberrant aortic artery with an endostapler. A single thoracic chest tube was placed and removed on postoperative day 2 and the patients were discharged on the same day. In both cases, the pathology examination revealed intralobar pulmonary sequestration. Pulmonary sequestrations are uncommon malformations that can be operated on using minimally invasive techniques, thereby permitting early discharge and a low rate of complications.     

Intralobar pulmonary sequestration with high level of serum CEA; report of a case

We reported a case of intralobar pulmonary sequestration with a high level of the serum CEA. A 53-year-old woman whose chief complaint was cough was admitted to our hospital. Enhanced chest computed tomography (CT) revealed the mass in the left lower lung, lymph-nodes swelling, and the aberrant artery. Magnetic resonance angiography (MRA) conformed the aberrant artery from the descending aorta. The level of serum CEA elevated at 9.6 ng/ml. Left lower lobectomy was performed. A diagnosis of intralobar pulmonary sequestration (Pryce type II) was established in this case. Histopathologically, the peribronchial epithelial cells in pulmonary sequestration showed weak positive for anti-CEA monoclonal antibody. Postoperative course was uneventful and the serum CEA level was 3.5 ng/ml in the normal range at the postoperative 17th day.     

An uncommon cause of massive haemothorax and treatment under cardiopulmonary bypass

Pulmonary sequestration is defined as nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree and that has a systemic arterial blood supply. Herein, we aimed to present a case of a 34-year-old male patient who had massive left-sided haemothorax on admission due to a giant intralobar pulmonary sequestration. An emergent repair was performed under cardiopulmonary bypass with axillofemoral cannulation.     

Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation

BACKGROUND: Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation. METHODS: An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed. RESULTS: In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally. CONCLUSIONS: The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.     

Late revelation of a subphrenic extralobar pulmonary sequestration as a suprarenal mass

Pulmonary sequestrations are some rare congenital anomalies. The incidence was estimated of 0.15% to 1.7%. They are characterized by a mass of non functioning pulmonary tissue that has no communication to the normal bronchial tree. The vascularisation is supplied by systemic arteries. They are classified further as intralobar and extralobar types. Extralobar sequestration, so-called accessory lung, is separated from the normal lung. We present a rare case of subphrenic extralobar pulmonary sequestration in a 57 years old patient. The lesion was initially presented as a non-typical suprarenal mass discovered on CT scan. The approach by laparatomy permitted the resection and the definitive diagnosis.     

The multiple facets of pulmonary sequestration

PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.     

Thoracoscopic surgery for intralobar pulmonary sequestration.

A 47-year-old male with intralobar pulmonary sequestration successfully treated by thoracoscopic surgery was reported. Preoperative selective angiography revealed two aberrant arteries originating from the left infraphrenic artery. Under thoracoscopy, adhesive tissues around the sequestered lung were dissected, aberrant arteries were divided and the sequestered lung was resected. We considered that preoperative detailed analysis of the aberrant artery was very important to safely perform thoracoscopic procedures for pulmonary sequestration.     

Thoracoscopic resection of pulmonary sequestration with carbon dioxide insufflation and indocyanine green

We encountered a rare case of pulmonary sequestration supplied from the right renal artery, which was resected by video-assisted thoracic surgery with carbon dioxide insufflation and indocyanine green-guided technique. A 41-year-old woman with intralobar pulmonary sequestration supplied from the right renal artery was referred to our department. At the time of surgery, we used carbon dioxide insufflation to improve the manoeuvrable workspace for shutting off aberrant arteries and indocyanine green fluorescence guidance to differentiate the boundary of the sequestered lung from the normal lung. These procedures helped in the efficient resection of the lesion.     

Intrapulmonary sequestration operated by video-assisted thoracoscopic surgery lobectomy; report of a case

Pulmonary sequestration is a rare malformation of the respiratory tract. We here report an adult case of intralobar pulmonary sequestration with aberrant artery of the right lower lobe. A 20-year-old man admitted to our hospital with a chest discomfort. Chest computed tomography (CT), angiography, and scintigraphy showed abnormal findings of the right lower lobe with an aberrant artery. Under the diagnosis of pulmonary sequestration, video-assisted thoracoscopic surgery (VATS) lobectomy was performed. VATS is useful for the operation of pulmonary sequestration.     

Hemoptysis and massive hemothorax as presentation of intralobar sequestration

Intralobar sequestration is a rare abnormality usually diagnosed at later age after a history of recurrent pulmonary infections. We present a case of a 55-year-old man in whom both hemoptysis and massive hemothorax were the initial presenting symptoms. This report shows that intralobar sequestration can have a dramatic course of disease, and for this reason resection of the sequestered tissue should be considered in all patients.