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.     

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.     

Role of steroid administration to reduce inflammation after thoracotomy in a rat surgical stress model

BACKGROUND: Unilateral lung surgical insult can lead to bilateral pulmonary inflammation after transthoracic esophagectomy for esophageal cancer. An inflammatory response of the cytokine network induced by surgical stress followed by neutrophil sequestration is the major mechanism involved in these complications. We examined modulation of the inflammatory cytokine and neutrophil sequestration by preoperative steroid administration in a rat thoracotomy model. METHODS: Adult male rats were subjected to right thoracotomy with pulmonary compression in the surgical stress model. In the another surgical stress model, 10 mg/kg of methylprednisolone (MP) was administered 1 h before the operation intraperitoneally, and peri-operative biological effects of steroid administration to neutrophil sequestration and cytokine-induced neutrophil chemoattractant (CINC) as an inflammatory cytokine were examined. Sham-operated animals were subjected to intubation only. RESULTS: Surgical insult to the unilateral lung increased local CINC concentration followed by an increase in neutrophil sequestration, wet-to-dry weight ratio in bilateral lungs and systemic CINC concentration. Pre-treatment with methylprednisolone reduced CINC concentration as well as neutrophil sequestration and wet-to-dry weight ratio of both lungs. CONCLUSIONS: These data suggest that inflammatory changes induced by the unilateral operative procedure occur in both lungs through local inflammatory response. Pre-operative methylprednisolone administration down-regulates cytokine release and neutrophil sequestration, preventing lung edema, and possibly may act as a prophylactic treatment against acute lung injury caused by a so-called "second attack" theory.     

Video-assisted thoracic surgery for bronchopulmonary sequestration

Bronchopulmonary sequestration is a rare congenital lung anomaly for which surgical resection is the definitive treatment. Open thoracotomy is the conventional approach, yet associated with considerable morbidity. We report one of the largest series of major lung resection for bronchopulmonary sequestration using the video-assisted thoracic surgery (VATS) approach that could reduce such morbidity. Six cases of VATS anatomical lobectomy for intrapulmonary sequestration performed between January 1996 and January 2005 were reviewed. The six patients included two males and four females, with a mean age of 43.3 years (range: 27-64 years). Anatomical lobectomy without conversion to open was achieved in all cases. The mean operating time was 112.8 min (range: 90-140 min), the mean blood loss was 283.3 ml (range: 100-500 ml), and the mean length of post-operative hospital stay was 8.8 days (range: 7-24 days). There was no mortality. Three patients had minor wound infection. The results were comparable patients receiving lung resections for bronchopulmonary sequestration by an open approach. VATS major lung resection for bronchopulmonary sequestration is safe and feasible. Further studies are warranted to define the role of VATS in the management of bronchopulmonary 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.     

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.     

Video-assisted thoracoscopic surgery for ligation of anomalous systemic arterial supply to pulmonary sequestration

A 52-year old female with anomalous systemic arterial supply to pulmonary sequestration was reported. The patient was admitted because of an abnormal lung shadow on chest X-ray film. Computed tomography (CT) showed an anomalous systemic arterial supply to pulmonary sequestration of the left lower lung without lung infection. Video-assisted thoracoscopic surgery for ligation of the anomalous systemic artery was performed. Postoperative course has been uneventful for 14 months after surgery. Blood supply increased to the left lower lung by 3-dimensional CT after surgery. The ligation of anomalous systemic arterial is enough for this disease.     

Primed neutrophils injure rat lung through a platelet-activating factor-dependent mechanism.

Bacterial lipopolysaccharide (LPS) promotes transient lung neutrophil sequestration. These LPS-primed neutrophils, when stimulated by an N-formyl peptide (FNLP), promote lung injury. We hypothesized that LPS-primed, FNLP-stimulated neutrophils promote lung injury through a platelet-activating factor (PAF)-dependent mechanism. Rats were pretreated with either saline or WEB2170, a PAF receptor antagonist (10 mg/kg po). One hour after pretreatment, rats were administered intraperitoneal LPS (salmonella typhimurium lipopolysaccharide, 500 micrograms/kg) followed 6 hr later by intravenous FNLP (250 micrograms/kg infused over 30 min). Two hours after the initiation of FNLP infusion, rats were sacrificed and assays were performed to measure: (1) lung neutrophil sequestration with myeloperoxidase (MPO) activity; (2) circulating neutrophil activation with nitroblue tetrazolium (NBT) staining, and (3) lung microvascular leak with 125I-albumin flux. We found that lung myeloperoxidase, circulating neutrophil NBT staining, and lung 125I-albumin flux were increased (P less than 0.05) in saline-pretreated LPS/FNLP rats, relative to control. While lung MPO remained increased (P less than 0.05) in WEB2170-pretreated LPS/FNLP rats, circulating neutrophil NBT and lung 125I-albumin flux were decreased (P less than 0.05) relative to those in saline-pretreated rats. We conclude that PAF mediates LPS/FNLP-induced neutrophil activation and lung injury, but is independent from lung neutrophil sequestration. Thus, lung neutrophil sequestration does not inevitably produce lung injury. Rather, neutrophils can accumulate in the lung without causing lung injury if neutrophil activation can be blocked.     

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.     

Video-Assisted Thoracic Surgery Lobectomy for Extralobar Pulmonary Sequestration in a Child: Report of a Case

Pulmonary sequestration in infants and children is conventionally treated by resecting the sequestered lung parenchyma (sequestrectomy) or by performing lobectomy through a standard thoracotomy. We performed lobectomy by video-assisted thoracic surgery, using an original tracheal tube that we designed, in a 6-year-old boy with extralobar pulmonary sequestration and bronchiectasis in the left lower lobe.     

Infarction of extralobar pulmonary sequestration after blunt trauma

Pulmonary sequestration is a rare congenital lung malformation that occurs in either an intralobar or extralobar location and is generally asymptomatic. We report an unusual case of a child who presented with infarction of a previously asymptomatic extralobar pulmonary sequestration after blunt trauma, eventually requiring surgical excision.     

Infant case and adult case of scimitar syndrome with lung sequestration

We present 2 cases of Scimitar syndrome with lung sequestration, 1 of which is infant case and the other is adult. A 3-month-old baby was transferred to our hospital because of severe heart failure. Cardiac ultrasonography and angiography revealed atrial septal defect and lung sequestration. Because of critical condition, she underwent emergency operation. After the operation of abnormal lung resection, her general condition improved. We emphasize that the prognosis is influenced by operation timing. A 20-year-old female was admitted for atrial septal defect. We diagnosed Scimitar syndrome and lung sequestration as the result of close examinations. We carried out definitive operations of the closure of atrial septal defect, the correction of partially anomalous pulmonary venous return and the resection of malformed lung. This adult case is popular one of Scimitar syndrome.     

Intralobal sequestration in children--a new concept from the form of bronchial tree in sequestrated lung]

Twenty cases of the intralobal sequestration of the lung in children have been experienced. The age of the patients were from eleven days to fifteen years. Intralobal sequestration was classified into two groups, (central type and peripheral type) by the way of reconstruction of the bronchial tree in the sequestrated lung. In 8 cases of central type, the site of lesion was variable. The bronchial trees of the sequestrated lung were running toward the hilus of the normal lung but the bronchi of the affected area in the normal lung were absent. The aberrant arteries were muscular in histology, and the drainage veins were pulmonary vein. In 12 cases of peripheral type, the lesion was in segment 10 area in every cases. The bronchial trees of the sequestrated lung were running toward the pulmonary ligament where an aberrant artery came from, and B10 of the normal lung were patent. The aberrant arteries were elastic in histology, and the drainage veins were pulmonary and/or azygos vein. It is conceivable that the peripheral type is the real intralobal sequestration and the central type is the mixture of some other diseases.     

Hypertonic immunomodulation is reversible and accompanied by changes in CD11b expression.

BACKGROUND: In a two-hit model of hemorrhagic shock and lipopolysaccharide (LPS), we previously showed that hypertonic saline (HTS) resuscitation reduced lung sequestration of neutrophils and the accompanying injury. This effect was partially attributed to suppressed expression of the surface adhesion molecule CD11b. This study investigates the duration of this protective effect after a single HTS dose and the usefulness of repeated infusions. MATERIAL AND METHODS: The previous two-hit rodent model was used. Neutrophil lung sequestration was measured by bronchoalveolar fluid cell count. CD11b expression was followed by flow cytometry. In vitro studies used isolated human neutrophils. RESULTS: Eighteen hours following resuscitation, the protective effect of HTS was lost. At this time, LPS caused an increase in both neutrophil lung sequestration and CD11b expression, regardless of the resuscitation regimen used. A second infusion of HTS prevented these changes and restored the lung protection observed earlier. In vitro studies showed that the duration of hypertonic pretreatment is an important determinant of cell responsiveness under the isotonic conditions: Four but not 2 h hypertonic exposure was able to prevent upregulation of CD11b induced by LPS added immediately after reestablishing isotonicity. CONCLUSIONS: This study demonstrates that HTS resuscitation lessens lung neutrophil sequestration and CD11b surface expression induced by LPS. This protective effect is transient but can be restored by a second HTS infusion suggesting that maintenance of beneficial effect necessitates repeated HTS addition. The reversibility ensures rapid modulation of neutrophil functions, thereby preventing acute tissue damage without causing long-lasting immunosuppression.     

Soluble tumor necrosis factor receptor prevents post-pump syndrome.

Post-pump syndrome is an acute lung injury following cardiopulmonary bypass (CPB) which is indistinguishable from the adult respiratory distress syndrome (ARDS). Tumor necrosis factor (TNF) is central to the inflammatory process and is capable of triggering the entire pathophysiologic response leading to ARDS. We hypothesized that treatment with a soluble TNF receptor-binding protein (TNFbp) would reduce the increase in serum TNF and prevent acute lung injury in our sequential insult model of ARDS following CPB. Anesthetized pigs were randomized to one of three groups: Control (n = 3), surgical preparation only; CPB + LPS (n = 6), femoral-femoral hypothermic bypass for 1 h followed by infusion of low dose Escherichia coli lipopolysaccharide (LPS; 1 microg/kg); and TNFbp + CPB + LPS (n = 4), pretreatment with intravenous TNFbp (2 mg/kg) followed immediately by both insults. CPB + LPS caused severe lung injury demonstrated by a significant fall in PaO2 and an increase in both intrapulmonary shunt and peak airway pressure as compared to all groups (P < 0.05). These changes were associated with a significant increase in plasma TNF level and pulmonary neutrophil sequestration. TNFbp significantly reduced plasma levels of TNF and prevented the lung injury typically observed with this ARDS model, but did not reduce pulmonary neutrophil sequestration. Thus, elevated serum TNF is not responsible for neutrophil sequestration but does play a role in neutrophil activation which causes lung injury. Prophylactic use of TNFbp in CPB patients may prevent neutrophil activation and reduce the incidence of post-pump ARDS.     

Endovascular treatment of symptomatic pulmonary sequestration

Pulmonary sequestration is a rare congenital malformation whose origin is bronchial and arterial simultaneously and its vascularization comes from an anomalous systemic artery. Its clinical presentation includes recurrent pneumopathy in the same anatomic location of the lung and difficult to resolve or recurrent lung abscess. It is usually treated with antibiotherapy and eventual surgical resection. A 23-year-old woman with history of recurrent respiratory infections and three episodes of hemoptysis was admitted at the hospital. Computed tomography and magnetic resonance imaging confirmed diagnosis of pulmonary sequestration. The angiographic study showed the presence of three inflow arteries arising from the thoracic aorta (T10) and supplying the abnormal lung parenchyma at the base of the left hemithorax. The patient underwent endovascular treatment consisting of exclusion of the inflow vessels with Amplatzer occlusive devices and coils. Subsequent computed tomography angiogram confirmed complete infarction of the sequestration. At 7 months, the patient presented with a new episode of bronchial infection. Repeated angiography showed persistence of intermediate small nutrient branches that were treated with coil embolization. The patient is symptom-free at 41 months after this secondary procedure. Endovascular treatment of pulmonary sequestration, with selective embolization of the inflow arteries, is a very attractive minimally invasive therapeutic option, as compared with conventional surgery, and potentially less prone to associated complications.     

Pulmonary sequestration with tuberculosis confined to the sequestrated lung

Pulmonary sequestration is a relatively rare malformation. The incidence of common pyogenic infection in this anomaly is very high. We describe a non-symptomatic, 19-year-old man who was misdiagnosed with left lower lobe pneumonia, which was treated with antibiotics for nearly one month. Contrast-enhanced computed tomography (CT) with multiplanar reconstruction showed an aberrant artery originated from the left side of the descending aorta, and went through the infiltration of the left lower lobe. The patient underwent surgical removal of the affected lobe. Microscopy demonstrated resected sequestrated lung tissue that was mainly composed of caseous necrosis with Langhans cells. And the tuberculosis was just confined to the sequestrated lung without any other sites of lung tuberculous infection. The patient received subsequent antituberculous chemotherapy after his operation. At 4-month follow-up, his clinical status was excellent. There are few reports of sequestration combined with tuberculosis. This case showed us two things: first, a persistent infiltration or consolidation in a same segment, especially in the lower lobe, reminds us of the possibility of sequestration; and second, even if the diagnosis of sequestrated lung is confirmed, we should consider whether the patient has any other diseases, besides the tuberculosis.     

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.     

Acute lung injury following reperfusion after ischemia in the hind limbs of rats.

In this study, we proposed that oxygen free radicals participate in the acute pulmonary injury that follows limb ischemia/reperfusion. Using an established model of hind limb ischemia, reproducible lung injury occurred after reperfusion. Lung microvascular permeability was measured with 125I-BSA and increased two-fold after 30 minutes of reperfusion. Pulmonary injury was blocked with DMSO, DMTU, allopurinol, indomethacin, and SOD plus catalase. The degree of pulmonary neutrophil sequestration as assessed by tissue myeloperoxidase activity was significantly diminished in animals pretreated with antioxidants. Pretreatment with indomethacin did not attenuate the neutrophil sequestration within the pulmonary parenchyma. These data suggest that increased lung microvascular permeability and neutrophil accumulation occur following hind limb ischemia/reperfusion. Therapeutic interventions with oxygen radical inhibitors blocked this process, while the prostaglandin inhibitor, indomethacin, only reduced lung permeability.