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 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.     

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.     

Robot-assisted segmental resection for intralobar pulmonary sequestration

IntroductionPulmonary sequestration is a rare congenital malformation found most frequently as intralobar sequestration in the left lower lobe. Complete surgical resection is considered the treatment of choice.PresentationWe present the case of a 29- year-old woman with intralobar pulmonary sequestration (ILS) diagnosed on chest CT. The sequestration was located in the left lower basal segments (segments 9 and 10) and was treated successfully by robot-assisted segmental resection without complication.DiscussionRecently, robot- assisted thoracoscopic lobar resections started to be performed for ILS. The sublobar, segmental resection are reserved mainly for the resection of pulmonary nodules. We report a first case of robot-assisted anatomical segmental resection for ILS.ConclusionWe highlight the role of robotic technology offering three-dimensional view and excellent dexterity enhancing the surgical performance and getting the surgical procedure more precise and safer. This could be useful especially in case of challenging sublobar resections.     

Localised fibrous mesothelioma arising in an intralobar pulmonary sequestration.

A localised fibrous mesothelioma arising from an intralobar lung sequestration occurred in a 64 year old Turkish woman. This appears to be the first report of a mesothelioma occurring within a pulmonary sequestration.     

Pulmonary sequestration children: is the thoracoscopic approach a good option?

Background The thoracoscopic approach for pulmonary sequestration in children seems to be feasible and reproducible. To date, there have been no studies comparing the thoracoscopic approach with the conventional technique in this indication. Therefore, the aim of this study was to compare these two approachs.Methods We performed a retrospective study in patients treated surgically for a prenatal diagnosis of pulmonary sequestration between 2000 and 2003. Three or five ports were used, and single-lung ventilation was required. The clinical, operative, and postoperative data were compared with those for patients operated on during the same period via thoracotomy. For statistical purposes, the children who required conversion to the open proecedure were grouped with those who underwent thoracotomy. The following data were compared: age, and weight, operating time, duration of postoperative drainage via chest tube, duration of postoperative narcotic use, pain scores on the 1^st and 2^nd days after the procedure, and length of hospital stay. The Mann-Whitney test was used for statistical analysis.Results Five infants were operated on via a thoracoscopic approach. Two required conversion to an open procedure. There were four intralobar sequestrations (ILS) and one extralobar sequestration (ELS). The mean age at surgery was 6.2 months (range, 4–9). The mean operating time, excluding the patients who were converted to open, was 155 min (range, 120–190). In the thoracotomy group, there were one ELS and one ILS. Two groups were defined: the exclusively laparoscopy group (EL) (n = 3) and the thoracotomy and conversion group (TC) (n = 4). The duration of postoperative drainage, duration of postoperative narcotic use, and hospital stay seemed to be shorter in the group EL. These times, respectively, were 3.4 vs 2.75 days, 4.1 vs 3.3 days, and 7.5 vs 4.3 days = (p = 0.03). There were two complications in the TC group (one wound abcess and one pleural leak that required a chest tube).Conclusion The thoracoscopic approach for pulmonary sequestration is feasible. It seems to reduce the duration of postoperative drainage, postoperative narcotic use, hospital and stay. These results need to be confirmed by a prospective and more detailed study.     

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.     

Combined endovascular and surgical approaches to treat intralobar pulmonary sequestration: a case report

Pulmonary sequestration is a congenital abnormality of a non-functional pulmonary mass with anomalous systemic arterial supply. Surgical resection is the gold standard treatment, but it carries a risk of life-threatening haemorrhage from accidental injury of the anomalous artery. Endovascular embolisation has been introduced as a safe alternative, but does not eliminate the possibility of symptom recurrence. We report a case of a 61-year old woman with intralobar pulmonary sequestration treated with a combination of endovascular coil embolisation and surgical resection.     

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.     

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.     

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 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.     

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.     

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.     

Bilateral intralobar pulmonary sequestration: therapeutic implications

Bilateral intralobar pulmonary sequestration within the lower lobes was suggested by computed tomography and confirmed by arteriography in a 27-year-old woman. Although one side was asymptomatic, both sequestrated areas were resected using staged bilateral thoracotomies. Histopathological examination showed massive inflammation in the symptomatic as well as the asymptomatic sequestrum. To our knowledge, this is the sixth reported case of bilateral intralobar pulmonary sequestration and the third for which bilateral resection has been performed.     

Distribution,diagnosis, and treatment of pulmonary sequestration: Report of 208 cases

ObjectiveThis study was performed to explore the clinical features, typing, distribution, and treatment of pulmonary sequestration (PS), with the aim of improving the awareness and treatment of this condition.MethodsClinical data regarding surgical procedures, outcomes, and prognosis of 208 pediatric patients with PS who were treated in our center from January 2005 to October 2017 were retrospectively analyzed.ResultsPS was confirmed by ultrasonography, enhanced computed tomography (CT), and/or magnetic resonance imaging (MRI) before surgery, and the surgeries were smoothly performed in all 208 patients (138 males, 70 females; age, 1 month to 14 years; mean age, 19.70 ± 48.82 months). The operative time ranged from 10 to 230 min (mean, 70 ± 48.75 min), and the intraoperative blood loss volume ranged from 1 to 200 ml (mean, 5 ± 18 ml). PS was located in multiple sites of the thoracic cavity and was also found in some rare locations such as the neck and abdomen. The feeding arteries of the PS mainly arose from the thoracic aorta or abdominal aorta, and a few of them originated from other vessels in the systemic circulation. The venous drainage differed between intralobar and extralobar PS: in patients with intralobar PS, the venous drainage was mainly via the pulmonary veins, especially the lower pulmonary veins; in patients with extralobar PS, the venous drainage was via the azygos vein and hemiazygos vein or reached the right atrium via the vena cava. The infection rate in children with intralobar sequestration was 71.17% (79/111), and that in children with extralobar sequestration was 31.37% (16/51).ConclusionPS has increasingly been detected by prenatal ultrasonography, and enhanced CT and MRI are the main techniques for diagnosing PS. Once confirmed, PS should be surgically resected. We choose an age of 6 to 12 months for surgical resection. Minimally invasive video-assisted thoracic surgery has many advantages in the treatment of PS and can be the treatment of choice for this condition.Type of studyTreatment Study.Level of evidenceLevel III.     

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.     

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.     

Intralobar pulmonary sequestration

For the period of 21 years the intralobar pulmonary sequestration was revealed in 6 patients aging from 11 to 47 years old. In all the observations of the intralobar sequestration the only affection of lower pulmonary lobes was noted. In 4 patients the symptomless course of the disease was present, while in 2 it was manifested by the recurrent pneumonia. The correct diagnosis before the operation was established in one patient only. All the patients were operated on, in 2 two aberrant vessels to the sequestrated pulmonary region were revealed and in all the patients--bronchogenic cyst in the affected region.