Pulmonary intralobar sequestration in a patient with cystic fibrosis

We report a case in which pulmonary Intralobar Sequestration (ILS) was an incidental finding at autopsy in an adult with Cystic Fibrosis. Two aberrant arteries from the descending thoracic aorta supplied a bronchial cystic lesion in the right lower lobe. Termination of the segmental bronchus and scar formation proximal to the cyst suggested prior bronchial obliteration. The elastic configuration of the aberrant aortic-derived vessels of the sequestration contrasted sharply with massively hypertrophied, muscular, bronchial arteries which supplied the bronchiectatic upper lobe. Sections of inferior pulmonary ligament were studied in five additional patients with CF but without ILS. Small muscular arteries were consistently noted within the inferior pulmonary ligament. These histologic findings support the concept that the vascular portion of ILS is congenital, whereas the bronchocystic component, in some cases, may be acquired.     

Striated muscle cells in non-neoplastic lung tissue: a clinicopathologic study

This study was performed to study the prevalence, origin, and clinical implication of striated muscle cells in congenital non-neoplastic lung abnormalities. Five cases of striated muscle cell proliferation within congenital non-neoplastic pulmonary abnormalities were identified from a series of 31 (16%) resected specimens obtained at King's College Hospital, London, during the period 1992 to 1998. Lung tissue was also obtained from 48 normal human fetuses and serial sections stained for the presence of striated muscle. A histologic and immunohistochemical study of the clinical cases and the fetal material was performed by using phosphotungstic acid hematoxylin staining and immunostaining for myoglobin and desmin. Striated muscle cells were identified either as a diffuse or a focal proliferation within the lung interstitia of five infants. The congenital lung anomalies were intra-abdominal pulmonary sequestration associated with congenital cystic adenomatoid malformation (CCAM), intrathoracic sequestration again with features of CCAM, an intrathoracic sequestration associated with a congenital diaphragmatic hernia, and 2 Stocker type II intrathoracic CCAMs. Striated muscle cells were not identified in any section of lung tissue derived from the fetal series. Striated muscle cells proliferation in non-neoplastic lung tissue is more common than usually reported. Although the exact origin of such cells is speculative, because it is always detected within pulmonary anomalies, a wide morphogenetic error is likely. The clinical implication of its presence has to be further defined. HUM PATHOL 31:1477-1481.     

Changes in systemic and pulmonary blood flow distribution in normal adult volunteers in response to posture and exercise: a phase contrast magnetic resonance imaging study

Hemodynamics are usually evaluated in the supine position at rest. This is only a snapshot of an individual’s daily activities. This study describes circulatory adaptation, as assessed by magnetic resonance imaging, to changes in position and exercise. Phase contrast magnetic resonance imaging of blood flow within systemic and pulmonary arteries and veins was performed in 24 healthy volunteers at rest in the prone and supine position and with bicycle exercise in the supine position. No change was seen in systemic blood flow when moving from prone to supine. Exercise resulted in an increased percentage of cardiac output towards the lower body. Changes in position resulted in a redistribution of blood flow within the left lung—supine positioning resulted in decreased blood flow to the left lower pulmonary vein. With exercise, both the right and left lower lobes received increased blood flow, while the upper lobes received less.     

Interstitial fluid pressure in the isolated perfused rabbit lung

WIIG, H., OPDAHL, H., NICOLAYSEN, A. & NICOLAYSEN, G. 1985. Interstitial fluid pressure in the isolated perfused rabbit lung. Acta Physiol Scand 125 , 601–607. Received 10 March 1985, accepted 9 May 1985. ISSN 0001–6772. Department of Physiology, University of Bergen and Institute of Physiology, University of Oslo, Norway. Interstitial fluid pressure was measured in nine isolated perfused rabbit lungs with the servonull micropipette method. Bevelled glass micropipettes, with tip diameter 2–6 μm (o.d.) were inserted 2–6 mm into the left lung. At alveolar pressures of 3 to 5 cm H₂O we found mean interstitial fluid pressures of 1.0 (SD 1.0) and 1.6 (SD 1.0) cm H₂O relative to pleural pressure in the upper (n = 19) and lower (n = 21) lobes respectively. The vertical distance between the measuring sites in the upper and lower lobes was about 3 cm. Net filtration caused by elevated left atrial pressure caused practically no change in interstitial fluid pressure. Increased alveolar pressure either increased or decreased interstitial fluid pressure. The measured pressures probably represent interstitial fluid pressure in alveolar junctions or in the interstitium around small pulmonary arteries or veins. We conclude that interstitial fluid pressure in these sites is between alveolar and pleural pressure, and that it is only moderately affected by changes in alveolar pressure. The interstitial compliance appears to be high and there seem to be little or no vertical gradients in interstitial fluid pressure within the lung.     

Pulmonary sequestration with ectopic pancreatic tissue in a Wistar Hannover GALAS rat

A multicystic mass of the lung was found in a male 11-week-old Wistar Hannover GALAS rat. The cystic mass was located in the region of the right caudal lobe and had no direct communication with the tracheobranchial tree. Histologically, the pulmonary mass was composed of variably sized cysts containing mucinous material, cellular debris, erythrocytes, and inflammatory cells. In the septal stroma between the cysts, well-developed elastic and muscular arteries were present and in some areas, ectopic pancreatic tissue was observed. In the rest of the lung lobes, there was no evidence of previous pneumonic events or other respiratory diseases. Based on these findings, the present case was diagnosed as a congenital pulmonary sequestration with ectopic pancreatic tissue.     

Evaluation of the post-shunt status with electron beam computed tomography in cyanotic congenital heart disease

The assessment of the accuracy of Electron Beam Computed Tomography (EBCT) for the follow-up of pulmonary vascular system after the shunt operation in cyanotic congenital heart diseases was purpose of the study. The study group consists of 16 consecutive patients with cyanotic congenital heart disease who had Blalock-Taussig (BT) shunt (n=7), bi- directional cavo-pulmonary shunt (BCPS, n=7) and unifocalization (n=2). EBT images were obtained on systolic phase under EKG gating and after intravenous administration of contrast agent. We evaluated the shunt patency, anatomy of intrapericardial pulmonary artery, parenchymal pulmonary vessels and background lung attenuation for the pulmonary blood flow, and the presence of systemic arterial and venous collaterals. Angiography (n=12) and echocardiography (n=16) were used as the gold standards. EBCT was consistent with angiogram in detecting the shunt patency and in depicting the anatomy of the intrapericardial pulmonary artery. Occlusion of the BT shunts was not detected in 2 patients by echocardiography. Diffuse or focally decreased pulmonary flow on EBCT in 8 patients was consistent with the pulmonary hemodynamics pattern revealed by cardiac catheterization. Uneven attenuation between lobes was related with multifocal supply of pulmonary flow or occlusion of lobar pulmonary arteries. Systemic collateral arteries were observed in 5 at the corresponding site of the decreased pulmonary flow. Systemic venous collaterals seen in all patients following BCPS were eventually draining to the inferior vena cava in 5 and to the left atrium in 2. EBCT provided accurate information of the pulmonary vascular system after shunt and has unique advantage over echocardiography in assessing patency of BT shunt or unifocalization tubes within the pleural space, the estimation of regional difference in pulmonary hemodynamics, and the detection of systemic collateral vessels. Therefore EBCT may provide useful information about the timing of definitive correction and the need for a second shunt or an interventional procedure prior to total repair.     

Pattern of fluid accumulation in NO2-induced pulmonary edema in dogs. A morphometric study.

To ascertain whether the pattern of fluid accumulation could be altered by an agent introduced through the airways, the authors studied the physiology and morphology of 11 dogs exposed to 150-494 ppm.hr NO2 and compared them with 3 new and 5 previously reported control dogs. NO2 caused a partly reversible decrease in systemic arterial pressure and cardiac output, a fall in arterial PO2, and rapid shallow breathing, while pulmonary arterial and wedge pressures remained normal. Post mortem, lower (LL) and middle (ML) lobes were frozen, sections fixed for light microscopy by freeze-substitution, and wet weight/dry weight (W/D) ratios were measured. Alveolar edema was graded, and the distribution of interstitial edema around arteries and veins and within bronchovascular bundles was studied with morphometry: edema ratios (ER) were calculated as area of interstitium/area of vessel or airway. We found that NO2 produced an exposure-dependent increase in lung water (r = 0.73), and that both LL and ML had similar W/D ratios (7.77 and 8.39, respectively) and percent alveolar edema (30% and 34%). Morphometry of interstitial edema showed that the ER for vessels and airways of edematous LL were essentially similar to controls, while those of the ML were markedly increased. It is concluded that NO2 produces exposure-related lung edema and preferential alveolar flooding with probable secondary interstitial accumulation. The discrepancies in interstitial edema between middle and lower lobes may be due to differences in lung volume or in ventilation.     

A histologic study of extralobar pulmonary sequestration and its anomalous artery as a clue to its development

In 1997, an anomalous small mass of soft tissue was found in the left thoracic cavity of a Japanese male cadaver during student dissection practice. It was linked only by branches from the thoracic aorta, hemiazygos vein and greater splanchnic nerve, with no connection to the normal lung by vessels or airway. Examination of cut sections clarified that this anomalous small tissue mass was an extralobar pulmonary sequestration (EPS). Many cases of pulmonary sequestration have been reported from the clinical aspect, but there are few from a purely anatomical viewpoint. In the present case, detailed gross anatomical and histologic observations clarified that the anomalous artery supplying the sequestration resembled an elastic artery. In view of the findings of other reports, the anomalous arteries are divided into two types, the elastic type such as the pulmonary artery and the muscular type such as the bronchial artery. So it is thought that the histologic observation of an anomalous artery in comparison with normal pulmonary and bronchial arteries may offer an important clue to consider in elucidating the origin and development of pulmonary sequestration.     

Extralobar pulmonary sequestration with an associated cyst of mixed bronchogenic and esophageal type--a case report.

We report an unusual case of extralobar pulmonary sequestration (ELS) with an associated cyst of mixed bronchogenic and esophageal type. A 58-year-old woman was incidentally found to have a 6 x 6 x 5 cm sized mass in the right superior mediastinum. The mass consisted of sequestrated pulmonary tissue and an unilocular cyst with a direct communication. The cyst could not be easily classified because it was lined by squamous or respiratory epithelium with two distinct muscle layers and bronchial glands. Bronchial cartilage was present in close proximity to the ELS. This unusual combination of ELS with a foregut cyst might be a part of bronchopulmonary foregut malformation, attributed to a common embryologic pathogenesis.     

Pulmonary sequestration with CCAM type II appearing as adrenal tumor protruding through a Bochdalek hernia

A case of a pulmonary sequestration, which almost exclusively consisted of a congenital cystic adenomatoid malformation type II located subdiaphragmatically in the left retroperitoneal area, is reported. This case, in a 24-year-old male patient, is unique in that it appeared as an adrenal incidentaloma and extended through a Bochdalek hernia into the pleural space. It was discovered upon routine ultrasound screening for hepatocellular carcinoma in a patient with a carrier state for hepatitis B. Diagnosis was established only upon histological analysis of the surgically removed tumor after staining with hematoxylin and eosin as well as surfactant A and B. The location of the tumor may indicate that it was formed by an entrapment of a lung bud by the developing diaphragm. This appearance may give us insight into the formation of such tumors. It also highlights the difficulty of diagnosing subdiaphragmatic retroperitoneal tumors without histological examination.     

Immune Complex Injury of the Lung

Abundant evidence currently exists to suggest that immune complexes play an important role in inflammatory diseases of the lung. Clinically, idiopathic pulmonary fibrosis, eosinophilic granuloma of lung, and systemic lupus erythematosus have been shown to be associated with the presence of immune complexes both in lung and in the serum. Experimentally, there is compelling evidence that acute lung injury can be triggered by the deposition of complexes in vascular walls or by the presence of performed immune complexes instilled into the airways. The observed reactions are, as expected, complement- and neutrophil-dependent. The morphologic changes in lung caused by products of complement activation (C5a and related peptides) depend on whether complement activation occurs within the vasculature or within the airways. Airway activation is associated with intraalveolar accumulations of neutrophils, while intravascular activation leads to intracapillary sequestration of neutrophils. The chronic formation of immune complexes within the vasculature (in the model of “chronic serum sickness”) leads to an interstitial fibrotic reaction and a thickening of basement membranes. Recent studies of intravascularly infused preformed immune complexes indicate a proclivity for certain types of complexes to localize within lung. These “lung-seeking” complexes differ from non-lung-seeking complexes only in the ratio of antigen to antibody. Complement does not seem to alter the tendancy for certain complexes to localize within lung. These studies emphasize the potential importance of immune complexes in lung injury and point out the variety of mechanisms involved in both the localization process and the injury process.     

Sequence of structural changes and elastin peptide release during vascular remodelling in sheep with chronic pulmonary hypertension induced by air embolization.

The progression of structural changes in the pulmonary arterial bed were followed in a model of chronic pulmonary hypertension. Chronically instrumented awake sheep received continuous air embolization for 0 (controls), 1, 4, 8, or 12 days (n = 5-6/group). After the period of embolization, the lungs were removed, the pulmonary arteries were distended with barium-gelatin, and the lungs were fixed via the airways with formal-saline. Quantitative techniques were applied to sections from random blocks from the lungs of each animal. One day of embolization resulted in granulocyte sequestration in the lung interstitium and in small vessels; additionally, intraalveolar and perivascular edema was present. By 4 days, increased medial thickness, appearance of muscle in smaller arteries than normal (e.g., muscular arteries at alveolar duct level: control = 1.2 +/- 1.2%; day 4 = 22.7 +/- 7.7) and reduction in number of barium-filled intraacinar arteries was found. The arterial changes progressed in severity to day 8 and were similar at day 12. Since arterial remodelling involves increased elastin deposition, the concentration of elastin peptides was measured in lung lymph. Increased flux of elastin peptides was apparent from day 2 of embolization and continued to increase to a level 20 x baseline by day 12 (baseline 351 +/- 86 micrograms/15 min; day 12 = 6338 +/- 2999). Comparison of the onset of the structural changes with previous findings shows that the arterial remodelling parallels the onset of sustained pulmonary hypertension. The increase in lung-lymph elastin peptides by day 2 provides evidence that vascular remodelling is initiated before day 4 of embolization. The early sequestration of granulocytes and appearance of edema suggest that these may be part of the trigger to the development of the structural changes.     

Structure,blood supply,and lymphatic vessels of the sheep's visceral pleura

We investigated the morphology of the visceral pleura of 36 sheep, using macroscopic, histologic, and ultrastructural approaches to quantify regional pleural thickness, blood supply, and lymphatic drainage, including the pulmonary ligament and hilar lymphatic distributions. Pleural thickness increased caudally and dorsally, such that the costal pleura of the caudal lobes had a mean minimum pleural thickness of 83 μm. The blood supply to the entire visceral pleura came exclusively from the bronchial arteries. Lymph vessels formed an extensive plexus throughout the serous membrane of all lobes. Trunk lymphatics (> 100 μm diameter) had a density of about 2/cm of pleural length on all lobar surfaces except for the cranial and middle lobes, where their density on the costal surfaces was ≤ 1/cm. Pleural trunk lymphatics coursed to the pulmonary ligaments and to the hilum on their way to regional lymph nodes. At the hilum they anastomose with intrapulmonary lymphatic trunks. The principal lymph nodes to receive pulmonary lymph were the caudal mediastinal node and tracheobronchial nodes. The visceral pleura of sheep is thick, showing considerable regional diversity in morphology.     

系统性硬化症肺部受累的胸部高分辨力CT表现特点分析

目的分析系统性硬化症（SSc）肺部受累时胸部高分辨力计算机体层摄影术（HRCT）表现,提高对SSc肺部损害的认识。方法选择2009年1月~2012年6月明确诊断的45例SSc患者,其中男性9例,女性36例;年龄为20~80岁,平均年龄58.50岁。回顾其胸部HRCT表现,并进行HRCT评分,对其胸部HRCT特点进行总结分析。结果胸部HRCT证实,存在间质性肺疾病患者为32例（71.1%）,其中34.4%患者（11/32）无呼吸系统症状。SSc肺部受累在HRCT图像上以磨玻璃影（81.3%）和网格影（56.3%）最为常见,分布以双下肺（71.9%）及胸膜下分布（81.3%）为主。对两侧的上中下肺野的HRCT评分进行t检验发现双侧病变差异无统计学意义（P〉0.05）,呈对称性分布。对上中下肺野的HRCT评分进行两两比较,下肺野受累最明显,中野次之,上野受累最少（P〈0.05）。在存在肺间质受累的患者中,弥漫型患者的HRCT评分（8.82±5.56）与局限型患者的评分（8.73±5.61）间差异无统计学意义（P〉0.05）。肺外胸部脏器受累包括肺动脉增宽、胸膜病变、心包积液、纵隔淋巴结肿大、食管扩张。肺动脉增宽33.3%（15/45）,弥漫型患者中肺动脉增宽的发生率与局限型患者的发生率差异无统计学意义;有雷诺现象者的肺动脉增宽的发生率高（15/34 vs 0/11;P=0.005）。结论胸部HRCT对SSs的肺、胸膜、食道、肺动脉的评估均有较高价值,其中肺间质受累是最为常见的,对称性分布、双下肺突出的间质性病变为其主要特点。     

Multifocal distribution of pulmonary capillary haemangiomatosis

AIMS: We investigated a case of pulmonary capillary haemangiomatosis, a rare condition, to determine the extent of the pathological changes within the lungs. Systematic histological sampling has not previously been performed in this condition. METHODS AND RESULTS: A 52-year-old woman with a history of ischaemic cardiomyopathy suffered from repeated respiratory infections, which were attributed to chronic pulmonary congestion. She died suddenly of fulminant pulmonary thromboembolism. An autopsy was performed and lung tissue was sampled at multiple sites. Beside passive congestion, the lungs showed well-circumscribed areas containing proliferations of small capillaries infiltrating the pulmonary septa and the walls of otherwise normal blood vessels and bronchi. The most severely affected areas were found to be in the periphery of both lower lobes. A diagnosis of pulmonary capillary haemangiomatosis was made. CONCLUSIONS: This is the first case of pulmonary capillary haemangiomatosis in which systematic histological sampling has been performed. Mapping of lesions disclosed the multifocal distribution of pulmonary capillary haemangiomatosis in this patient.     

Intralobar sequestration of the lungs

The paper deals with 6 cases of intralobar pulmonary sequestration that is a rare developmental defect accompanied by other anomalies and tumors. In 4 cases, the diagnosis was made on the basis of X-ray and bronchologic examinations before surgery, in 2 cases, during the surgery. The morphologic features were found to be cystic formations accompanied by chronic inflammation in the lung tissue, cylindrical brochiectasis along with celomic cyst of the pericardium and microcarcinoid in the cyst wall.     

Massive pleural effusion and hypoplasia of the lung accompanying extralobar pulmonary sequestration

Autopsy of a premature infant who died with severe respiratory insufficiency revealed a large effusion and an extralobar pulmonary sequestration in the left pleural space. The presence of dilated lymphatics in the sequestration suggested that it was the source of the effusion. The left lung was very hypoplastic, apparently at least in part as a result of the effusion.     

Pulmonary lymphatic filling is increased in spontaneously hypertensive rats

Extravascular lung liquid must rely on tissue-space pressure gradients to drive it into the lymphatics because the fluid is outside the lymphatic contractile pumping and valve control. Focal tissue pressure changes could result from muscular contraction in the blood vessel walls. Perivascular lymphatics usually lie within the adventitia of pulmonary blood vessels, and are generally more noticeable in veins than arteries. Spontaneously hypertensive rats have exaggerated focal pulmonary venous muscle (venous sphincters). These muscular tufts are often near initial lymphatics; if their contraction was important for lymph transport, spontaneously hypertensive rats could have more lymphatic filling in the areas of the pulmonary venous sphincters than normotensive rats. Because the focal muscularity is found in pulmonary veins more than arteries, veins may have more focal lymphatic filling than arteries. To test these hypotheses, lung histology and vascular and lymphatic casts of spontaneously hypertensive and normotensive rats were examined. Contracted venous sphincters were found on 108 of 127 veins with lymphatics in the spontaneously hypertensive rats and 5 of 41 in the normotensive rats P<0.01). The spontaneously hypertensive rats had deeper venous contractions and more lymphatic filling around both arteries and veins (P<0.01). In the hypertensive rats, the venous was greater than the arterial lymphatic filling (P<0.01). On the pleural surface, hypertensive rats also had greater lymphatic filling than controls (P<0.01). This anatomic evidence suggests that pulmonary venous sphincters are associated with focal lymphatic filling, and perivascular muscle action might be a component of the pulmonary lymphatic system.     

Repeated Escherichia coli endotoxin-induced pulmonary inflammation causes chronic pulmonary hypertension in sheep. Structural and functional changes

Chronic pulmonary hypertension occurs in several human diseases in which there is evidence of chronic or repeated bouts of pulmonary inflammation. To determine whether prolonged lung inflammation causes persistent pulmonary hypertension Escherichia coli endotoxin was given to seven chronically instrumented awake sheep three times a week for 10 to 14 weeks. Pulmonary artery, left atrial and systemic arterial pressures, cardiac output, arterial blood gases and pH were monitored before starting endotoxin treatment and twice weekly, immediately before endotoxin infusion. Three sheep receiving saline over a similar time period served as controls. Pulmonary vasoreactivity to breathing 12% oxygen and a bolus infusion of an analog of prostaglandin H2 was also assessed. Peripheral lung biopsy tissue was taken at baseline and at periods throughout the experiment to assess pulmonary inflammation. Repeated endotoxin infusions resulted in a significant increase in mean pulmonary artery pressure from the 8th week of treatment and more than a 50% increase from week 10 (baseline = 18.4 cm H2O +/- 1.0 (mean +/- SE); 10 weeks endotoxin = 27.8 +/- 4.3; p less than 0.05). Pulmonary vasoreactivity to both an analog of prostaglandin H2 and 12% oxygen decreased in the period from 4 to 8 weeks of endotoxin treatment. Light microscopic assessment of lung biopsy tissue showed a persistent four-fold increase above baseline in number of peripheral lung granulocytes. Electron microscopy revealed that granulocytes, lymphocytes, and monocytes sequestered in the lungs of these animals, and that structural damage to the endothelium was minimal. Morphometry of lungs obtained at autopsy in which the pulmonary arteries had been distended with barium-gelatin showed extension of muscle into the walls of smaller intra-acinar arteries (than normal) and a reduction in number of filled peripheral arteries. We conclude that repeated infusions of endotoxin into sheep cause persistent lung inflammation, altered pulmonary vasoreactivity, sustained pulmonary hypertension, and some of the structural changes characteristic of this disease. Chronic inflammation may play a role in the pathogenesis of chronic pulmonary hypertension.     

肺癌组织内肺动脉供血活体灌注观察

目的:探讨肺癌组织内肺动脉动态供血情况.方法:选择未作任何化疗栓塞的5例肺癌患者,根据普通X线、CT、MRI及肺叶分段定位,按照肺叶分段原理,结扎肺动脉之前,肺动脉内注入美蓝溶液5～10ml,术后将取出的肺叶作连续厚切片,组织块厚约5 mm.并用数码机连续摄片.结果:肺癌组织呈分叶状,癌巢间肿瘤血管染色密集,呈血湖状,主要集中在肿瘤的中央部,癌叶内亦见点状肿瘤血管分布,但相对较少,肿瘤与正常肺组织间肿瘤血管相对稀疏.结论:通过肿癌组织肺动脉注射美蓝溶液,进一步证实了肺癌组织内肺动脉参与供血.