Pulmonary sequestration in older child and in adults.

Pulmonary sequestration is often found in children with recurrent pneumonia, but is not rare in adults. During the last 6 years (1985-1990), 6 patients with pulmonary sequestration were surgically treated. One 40-year-old patient had an extralobar sequestration, and 5 patients ranging in age from 14 to 26 years had an intralobar sequestration. The patient with extralobar sequestration was asymptomatic. In cases of intralobar sequestration, 4 patients were symptomatic. All 6 patients had an abnormal chest roentgenogram. Preoperative arteriography revealed anomalous blood supply came from a systemic artery in 4 patients. All cases with intralobar sequestration had a lower lobectomy, and one case with extralobar sequestration had an extirpation. Even in cases without any symptoms, there was the possibility of a microscopic infection in the sequestrated lesion with the occurrence of later symptoms. Therefore, patients diagnosed with pulmonary sequestration should be considered for surgical resection.     

Unique extralobar sequestration with atypical location and aberrant vessels

We report a unique extralobar pulmonary sequestration in the upper thoracic region with 3 aberrant vessels connecting to the right subclavian artery, right superior pulmonary vein, and right pulmonary artery in a 20-year-old man. The sequestered lung was completely excised using a video-assisted thoracoscopic approach.     

Extralobar pulmonary sequestration with aberrant lobulation of the lower lobe; report of a case

An adult case of extralobar pulmonary sequestration with aberrant lobulation of the right lower lobe is reported. A 32-year-old woman admitted to our hospital with a right intrathoracic tumor in the chest computed tomography (CT). Chest CT showed a mass lesion on the right diaphragm of a diameter 5 cm and abnormal fissure of the right lower lobe. Under the diagnosis of pulmonary sequestration without clearly thick vessels, video-assisted thoracoscopic surgery was performed. The mass existed between the right diaphragm and posterior lower lobe with a thin stalk, and the lower lobe had aberrant lobulation. The resected mass was histopathologically diagnosed as extralobar pulmonary sequestration.     

Malignant spindle cell tumour (fibrosarcoma) in sequestration of lung—a case report

Pulmonary sequestration is an uncommon congenital anomaly of respiratory system and usually diagnosed in childhood but can present at any at age. It frequently presents as a mediastinal mass with symptoms from vascular shunting, or associated anatomic defects. This case report describes an adult who presented with chest pain due to extralobar sequestration of right lung extending into the mediastinum. Per-operatively it was found to be an extra pulmonary mass lesion with a peduncle attached to right lung suggestive of extralobar sequestration. Histopathological examination of the specimen showed it to be spindle cell tumor (fibrosarcoma) in sequestrated lung. The patient had an uneventful recovery and is under regular follow up without any further problems.     

Pulmonary sequestration complicated by anomalies of pulmonary venous return

Five anomalies of pulmonary venous drainage were seen among 12 children operated for lung sequestration. In two children, venous drainage from the sequestrated lobe and the rest of the right lung was via a single channel into the inferior vena cava ("scimitar syndrome"). In one of these children, the sequestrated lobe was resected and repair of the scimitar syndrome was delayed; in the second patient, the anomalous pulmonary venous drainage was not recognized preoperatively and the vein was ligated, resulting in acute hemorrhagic infarction of the right lung and death of the patient. Three patients had less severe anomalies of pulmonary venous drainage. We recommend very careful evaluation of patients with lung sequestration with special reference to pulmonary venous drainage.     

Pulmonary sequestration.

Pulmonary sequestration is a congenital anomaly in which an aberrant systemic artery arising from the thoracic or upper abdominal aorta supplies part of the lungs, usually the lower lobe. The sequestered lung may be anatomically distinct from the remainder of the lobe (extralobar), or may be included in the substance of the lobe, in which case it may or may not have bronchial communication with the rest of the bronchial tree. The patients present, often in the first two decades of life, with recurrent and severe bronchopulmonary infections. Associated anomalies are present, especially in the extralobar variety. Nine cases of sequestration are reviewed, stressing significant clinical, radiological, and arteriographic findings. Preoperative demonstration of the anomalous vessel by aortography has contributed significantly to the planning and safety of the surgical procedure, which was generally a lower lobectomy. Eight out of nine patients survived the procedure.     

A unique congenital mediastinal malformation

We report a case of a 35-year-old patient presenting with a unique asymptomatic malformation associating extralobar pulmonary sequestration communicating with a bronchogenic cyst of the esophageal wall via the aortopulmonary window, dextroisomerism, and complete agenesia of the left pericardium. Despite computed tomography (CT) scan and magnetic resonance imaging (MRI), the diagnosis could not be established before left thoracotomy. The sequestrated lobe and bronchogenic cyst were then successfully resected.     

133. Arteriovenöses pulmonales Aneurysma im extralobär separierten Lungenlappen mit ungewöhnlich großem Links-Links-Shunt

Zusammenfassung Bei einem 13jährigen Knaben wurde ein mit einem L-LShunt von 40% des GKMV einhergehendes arteriovenöses, pulmonales Aneurysma, in einem separierten Lungenlappen des rechten Unterfeldes angetroffen, das von einem transdiaphragmal aus der Aorta abdominalis aufsteigenden Arterienast gespeist wurde. Gefäßanatomie und Hämodynamik wurden durch Farbstoff-Verdünnungsmethode, Cineangiokardiographie und transfemorale Aortographie abgeklärt. Die Therapie bestand in der Eastirpation des aneurysmatragenden, accemorischen Lungenanteils. Die Beobachtung ergänzt die bekannten Formen der Lungenseparation um einen vasculär-aneurysmatischen Typ.

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Arteriovenous pulmonary aneurysm localized in an extralobar sequestrated pulmonary lobe with an unusually large left-left shunt

Summary An arteriovenous pulmonary aneurysm in an eatralobar sequestrated pulmonary lobe of the lower right side causing a shunt volume of 40% of the systemic circulation volume was found in a 13-year-old boy in whom an atypically localized machinery murmur was detected. The aneurysm was fed by an arterial branch ascending from the abdominal aorta and passing across the diaphragm. Diagnosis was made by a dye-dilution method, cineangiocardiography and transfemoral aortography. The sequestrated lung lobe was resected. Our observation adds a vascular aneurysmatic type to the other known forms of pulmonary sequestration.

     

Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral chest: common origin?

Extralobar pulmonary sequestration (ELS) and congenital cystic adenomatoid malformation have been reported to coexist in several variations. This suggests a common embryologic origin. A 6-month-old boy presented with a history of recurrent pneumonias. The patient was diagnosed with a right lower lobe congenital cystic adenomatoid malformation (CCAM) and a left lower lobe ELS/CCAM. The diagnosis was made with the aid of a multidetector computed tomography (CT). Three-dimensional CT reconstruction showed the presence of a right lower lobe CCAM, a left lower ELS with an aberrant arterial supply from the celiac axis, and possible venous drainage into the right CCAM. The patient underwent a right thoracotomy. Intraoperatively, the lesions were discovered to be connected by a band of tissue. The right lower lobe CCAM and the left ELS were removed from the right chest. Histologic analysis confirmed the presence of a CCAM within the right lower lobe. The ELS had involvement of a type II CCAM within the sequestration. The connection between the right CCAM and left ELS/CCAM showed an anomalous conducting airway, anomalous vein, and anomalous artery connecting the 2 lesions. The authors present the first case of a CCAM connected to an ELS/CCAM in the contralateral hemithorax. The unique anatomic configuration of these lesions suggests a common embryologic origin of ELS and CCAM.     

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.     

Extralobar pulmonary sequestration

Extralobar sequestration of pulmonary tissues is relatively rare and seldom produces symptoms. An incidental finding is described of extralobar sequestration of the lung in a Nigerian child, presenting as an anterior mediastinal polycystic mass. The mass was attached to the right pulmonary artery by a fibrovascular pedicle which contained a small elastic artery, veins, and nerve bundles. Sequestrated lobes are usually situated in the posterior basal lobes of the lungs and may be associated with other congenital abnormalities, but the case reported here differs in these respects. Death was due to enterocolitis unrelated to the congenital abnormality.     

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.     

Extralobar pulmonary sequestration showing high CA 19-19 levels

We report a case of 40 year-old woman with left thoracic pain who was diagnosed as having a cystic mass located posteriorly at the basis of the left pleural cavity. The preoperative serum CA 19-9 level was increased (2 900 IU/mL). Further investigations did not find neoplastic lesions in the gastrointestinal tract. The intraoperative finding of an anomalous systemic arterial supply to the mass suggested the diagnosis of extralobar sequestration, confirmed at the histopathological examination. The association between pulmonary sequestration and increased tumor markers levels is overlooked in western literature, but it is often reported by many Japanese authors. This case report would underline the practical usefulness for preoperative diagnosis of pulmonary sequestration, when the CT-scan does not demonstrated an anomalous systemic vessel. The common embryogenic origin of both respiratory and digestive apparatus can explain the increased levels of tumor markers such as CA 19-9 and carcinoembryonic antigen in bronchogenic cyst, intestinal duplication and pulmonary sequestration.     

A case of intralobar pulmonary sequestration associated with abnormal connection between the aberrant artery and the pulmonary artery

The patient was a 5-year-old girl and had a history of recurrent pneumonia. Angiogram demonstrated that the aberrant arteries arising from descending aorta communicated with left pulmonary A10 where the multi-lobulated cystic lesion was seen by chest X-P. The right heart catheterization revealed the step-up of oxygen saturation in distal part of left pulmonary artery A10. Diagnosis was the intralobar pulmonary sequestration with the aberrant arteries communicating with pulmonary artery. The connection between aberrant arteries and pulmonary artery in this case might be caused by the chronic inflammation in the sequestrated lung. We found 4 case reports in which the communication between aberrant arteries and pulmonary artery was evident.     

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.     

Video-assisted thoracic resection for intralobar pulmonary sequestration

We present a case in which video-assisted thoracic resection for intralobar pulmonary sequestration (ILPS) was successfully performed. A 36-year-old woman had repeated pneumonia. Chest computed tomography (CT) showed a round mass in the right lower lobe of the lung. Subsequent three-dimensional CT revealed that a large anomalous artery arising from the descending thoracic aorta was distributing to the posterior basal segment containing the lesion and was draining into the inferior pulmonary vein. The patient was diagnosed with ILPS and underwent surgery. The anomalous artery was divided, and the sequestered segment was completely resected by video-assisted thoracic surgery (VATS). We think that VATS resection for ILPS is feasible and is a major therapeutic option as noninvasive surgery.     

Pulmonary sequestration associated with asymptomatic aspergillosis.

Intralobar pulmonary sequestration associated with asymptomatic aspergillosis is a rare case. We describe the case of a 65-year-old woman with intrapulmonary sequestration, anomalous systemic arterial supply to the left lower lobe and aspergillosis who underwent left lower lobectomy and ligation of an anomalous artery by Video-Assisted Thoracoscopic surgery (VATS). Pathological examination showed the parenchymal distortion and chronic inflammation. Aspergillus were found in the cyst. VATS lobectomy for intralobar pulmonary sequestration is a safe and valid procedure.     

An incidental and uncommon pulmonary sequestration with an uncommon feeding artery

INTRODUCTIONPulmonary sequestration is a rare congenital anomaly and most intralobar sequestrations were located in lower lobes. There is little information on middle lobe intralobar sequestration.PRESENTATION OF CASEA 44 year-old man with right middle lobe syndrome was referred for surgical management. He underwent video-assisted thoracoscopic right middle lobectomy and his postoperative course was uneventful. Pathology is notable for an incidental intralobar sequestration.DISCUSSIONOur case was unique in that the location of incidental pulmonary sequestration is uncommon and in that its feeding artery was uncommonly located in the fissure.CONCLUSIONWe report an extremely rare case of right middle lobe intralobar sequestration with a feeding artery in the fissure.     

Successful repair of a coarctation complex with an anomalous right subclavian artery in an infant

A 46-day-old infant weighing 2250 g with the coarctation of the aorta, ventricular septal defect, PDA, the anomalous right subclavian artery, and the persistent left superior vena cava initially underwent by the reversed subclavian flap aortoplasty with the anomalous right subclavian artery and pulmonary arterial banding. The reversed subclavian flap aortoplasty was useful for the coarctation complex with the anomalous right subclavian artery. Two months later he gained weight to 2500 g, and then VSD closure and debanding of PAB was performed successfully. This two-staged operation was recommended for the poor risk coarctation complex with the anomalous subclavian artery.     

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.