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.     

Intralobar Bronchopulmonary Sequestration with Mixed Venous Drainage in a 10-Year-Old Girl

Bronchopulmonary sequestration consists of a mass of abnormal lung tissue that has no normal connection with the bronchial tree and is supplied with blood from an aberrant artery mostly originating in the thoracic aorta. Two forms are recognized: intralobar and extralobar sequestration. The first is localized within the normal visceral pleura and has a venous drainage into the pulmonary system; the latter is localized without the normal lung in its own pleura with venous drainage into the systemic venous system. Intralobar sequestration is the most common form accounting for 75% of the cases. Intralobar sequestration usually presents in adolescence or adulthood with signs of recurrent pneumonia. Extralobar sequestration presents early in life with respiratory distress or feeding difficulties and is frequently associated with other congenital malformations. The diagnosis is confirmed by CT scan of the lungs and magnetic resonance angiography as demonstration of the aberrant vascular supply is essential for the diagnosis. Therapy consists in surgical removal.

We present a case of intralobar sequestration in a 10-year-old girl. The clinical symptomatology was typical. Arterial supply with two aberrant arteries and mixed venous drainage into the pulmonary and systemic systems were particular features.     

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.     

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.     

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.     

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.     

Systemic origin of the sole artery to the basal segments of the left lung without pulmonary sequestration

A sixty-one year-old man with squamous cell carcinoma of the left upper lobe had an aberrant systemic artery to the left basal segments without pulmonary sequestration. Physical examination revealed neither cardiac murmur nor any sign of heart failure, which was at variance with reported cases in the literature. Chest X-ray film showed no abnormal density suggesting sequestrated lung. Bronchogram disclosed obstruction of the left upper lobar bronchus by the tumor and normal segmental bronchi of the lower lobe. Left pulmonary angiogram showed normal arterial distribution of the left upper lobe and the superior segment of the lower lobe, but the basal segmental arteries were not visualized. The aberrant pulmonary artery arising from the descending aorta was visualized by computed tomography. Following dissection of the abnormal vessel pneumonectomy was performed. Pathological examination of the left basal segments revealed prominent atheromatous changes in the aberrant systemic intrapulmonary artery and irreversible obstructive lesions in its tributaries. These arterial lesions in this patient would have precluded plastic operations such as transfer of the origin of the aberrant vessel to the left pulmonary artery even if other circumstances had been favorable for preservation of the left lower lobe.     

A case of pryce type I intrapulmonary sequestration

A twenty-year-old asymptomatic man hospitalized because of a vascular murmur and abnormal shadow in the left lower lung on X-ray film. An aortogram revealed an abnormal artery arising from the descending thoracic aorta and supplying the left basal segment, which had no other pulmonary arteries. Although lung ventilation scintigraphy demonstrated reduced ventilation to the left lower lobe, bronchogram showed an almost normal bronchial tree except that peripheral branches were slightly thin. A clinical diagnosis of Pryce type I intrapulmonary sequestration was made, and left lower lobectomy was performed successfully. We have analyzed 31 cases of Pryce type I intrapulmonary sequestration in Japan. A vascular murmur is often heard, and a chest X-ray usually shows either a mass shadow or increased vascular markings. In most of those cases, an abnormal artery arises from the descending thoracic aorta and it supplies the left basal segment. Because this type of sequestration causes hemoptysis and infections, surgical intervention is indicated.     

A case of unusual branching and aberrant course of the segmental vein (V2) in the upper lobe of the right lung

Two right pulmonary veins emptying into the left atrium is the normal state. We describe a case observed at operation in which three pulmonary veins were present on the right side and emptied into the left atrium; a third vein arised from the dorsal part of the upper lobe and followed an abnormal extrapulmonary course. Right lower lobectomy and mediastinal lymph node dissection were carried out for a 73-year-old male with lung carcinoma. At operation, an aberrant branching vein arising from the upper lobe descended dorsally to the right main bronchus and emptied into the left atrium at the middle point between carina and inferior pulmonary vein. The intermediate bronchus lay between the anomalous vein and superior pulmonary vein. Conventional tomogram, CT scan and pulmonary angiogram showed that pulmonary arteries and bronchi were normal in their pattern of branching and distribution, and that the anomalous vein observed at operation was comprised of V2a and V2b according to the system of naming on Yamashita's. Infrequent variations of pulmonary veins are to be kept in mind to that operation may be performed in safety.     

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.     

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.     

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.     

Congenital bronchopulmonary vascular malformations: clinical application of a simple anatomical approach in 25 cases.

Congenital malformations of the bronchopulmonary airway and related arterial blood supply are a complex group of lesions in which abnormalities of venous drainage and lung parenchyma may coexist. Twenty five cases have been analysed, by a method whereby each anatomical component is separately considered. All 25 patients had abnormalities of the tracheobronchial tree, with no connection to the abnormal segment in nine cases. The aberrant arterial blood supply was single in 16 cases and multiple in nine cases, one patient from the latter group having a mixed pulmonary and systemic arterial supply to a part of the abnormal segment. Seventeen patients had anomalous venous drainage. In nine of these the vein or veins (they were multiple in four cases) drained the major part or the whole of the lung, whereas the aberrant arterial supply was limited to the right lower zone--that is, mismatched anomalous venous drainage. Abnormalities of lung parenchyma included changes within the lesion (for example, cysts, foregut inclusions) and associated abnormalities of surrounding lung (for example, hypoplasia, abnormal lobation). This information, together with the clinical features and haemodynamic data, was found to be essential for decisions on appropriate management. Patients presenting in infancy with haemodynamic disturbance continue to present major management problems (50% mortality), particularly if there are associated congenital heart defects. The role of aberrant systemic artery occlusion or ligation as a first stage procedure is well established in patients with haemodynamic abnormalities. There may be a place for this procedure in selected patients who have no haemodynamic disturbance at presentation.     

Pulmonary vein thrombosis after bilobectomy and development of collateral circulation

Pulmonary vein thrombosis is a rare but potentially life threatening complication following lobectomy or bilobectomy. We present a case of right upper pulmonary vein thrombosis after a middle and lower lobectomy diagnosed at transoesophageal echocardiography. The patient was treated with antibiotics and anticoagulation with good recovery. Pulmonary angiography was performed 35 days after surgery and revealed the venous return of the right lung through the intercostal veins. Despite double venous drainage of the lungs consisting of bronchial and pulmonary veins, pulmonary to systemic collaterals following pulmonary vein thrombosis have not previously been reported. The development of this shunt can prevent gangrene, and surgical resection of the lung segment involved can be avoided.     

A case of aberrant left pulmonary basal aneurysm arising from the descending aorta]

A 65-year-old man was referred to our hospital because of fever and abnormal shadow in the left lung. An aortogram revealed a large artery arising from the descending aorta supplying the left basal segment and, which then flowed into the pulmonary vein. This artery had an aneurysm and 30 mm in diameter. Bronchography showed compression of the left basal branch. A clinical diagnosis of an aberrant left pulmonary basal aneurysm arising from descending aorta was made, and left lower lobectomy was performed, because of the danger apprehension of the aneurysmal rupture. The procedure was successful. There are only 15 case reports of a systemic artery supplying the lung with normal bronchial branch in Japan. Differences of this anomaly from pulmonary sequestration were discussed.     

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.     

Lungs: blood supply,lymphatic drainage and nerve supply

The pulmonary arteries, through their capillary plexus, are entirely concerned with alveolar gaseous exchange, while the nutrient supply of the lung parenchyma is provided by the bronchial arteries. The pulmonary vein tributaries derive partly from the capillaries of the bronchial and the pulmonary arteries. The bronchial veins drain the larger bronchi. The lymphatics of the lungs drain into the nodes lying at the bifurcations of the larger bronchi, then to the tracheobronchial nodes and then into the bronchomediastinal lymph trunk on each side. These usually drain directly into the junction of the internal jugular and subclavian veins on each side, but may drain, on the right, into the right lymph trunk and, on the left, into the thoracic duct. If the subcarinal node is the site of secondary deposits it gives the typical bronchoscopic sign of widening of the carina. The principal function of the sympathetic (T2-4) supply to the lung is bronchodilatation, while the vagus fibres act as stretch receptors.     

An anomalous segmental vein of the left upper lobe of the lung: preoperative identification by three-dimensional computed tomography pulmonary angiography

A number of variations in the pulmonary arteries and veins have been documented, and the information is very important for performing a safe lung resection. This report describes a case of an anomalous segmental vein of the left upper lobe of the lung. The patient was a 75-year old male who was suspected to have lung cancer in the left upper lobe. A contrast-enhanced computed tomography showed a vessel behind the left lower bronchus. A three-dimensional computed tomography angiography demonstrated that it was an anomalous vein for the apicoposterior segment of the left upper lobe of the lung, draining into the left inferior pulmonary vein. The aberrant vein was readily identified during surgery and was divided without injury, and a left upper lobectomy was successfully performed. Aberrant pulmonary veins for the superior segment of the right upper lobe of the lung are rarely observed, and the same kind of anomaly on the left side has not been reported.     

Anomalous right upper lobe venous drainage

Anomalous pulmonary venous return is a rare congenital anomaly mainly involving the right lung and is often associated with congenital intracardiac malformations as atrial septal defect. We report a case of anomalous right upper lobe venous drainage resulting in two right upper lobe veins draining into the azygous vein and into the confluence between superior vena cava and azygous vein, respectively. Preoperative identification of such an aberrant venous drainage is useful for avoiding unexpected intraoperative bleeding.     

Modified Fontan operation for a single ventricle with a nonconfluent pulmonary artery]

A 8-year-old boy with a double inlet right ventricle with a non-confluent pulmonary artery was operated on with a modified Fontan operation. He had right isomerism, right aortic arch, bilateral superior caval veins, and left-sided inferior caval vein. Hepatic veins were separately drained to the right-side atrium. Left Blalock-Taussig shunt and right central shunt operations had been previously performed. Firstly, we had reconstructed the central pulmonary artery with a 16 mm porcine pericardial roll to unify the nonconfluent pulmonary arteries. Secondly, about 2 months after the first operation, we performed a modified Fontan operation. Systemic venous return from the inferior caval vein and the hepatic veins were drained to a reconstructed pericardial roll with an intraatrial Gore-Tex graft, and bilateral superior caval veins were also anastomosed to the roll. The structure of the pulmonary arterial system is one of the most important factors to determine the outcome of a modified Fontan operation. Even if the central pulmonary artery is absent, however, a modified Fontan operation is applicable for the patient whose peripheral pulmonary arteries have enough growth.