Anomalous unilateral single pulmonary vein: two cases mimicking arteriovenous malformations and a review of the literature

Total anomalous pulmonary venous drainage is a rare congenital anomaly. It usually involves a pulmonary to systemic venous shunt and most cases have a septal defect in order to survive. Anomalous pulmonary venous drainage with pulmonary venous shunting is an extremely rare and entirely benign entity. We present two such cases, in which there was atresia of the left superior pulmonary vein and drainage via a tortuous collateral vein to the left inferior pulmonary vein. This collateral was mistaken on plain film and CT for a pulmonary arteriovenous malformation. Awareness of this anomalous unilateral single pulmonary vein and its radiological appearances may help in avoiding unnecessary pulmonary angiography.     

肝硬化门静脉高压侧枝血管的多层螺旋CT表现

目的用多层螺旋CT(MSCT)统计、分析肝硬化门静脉高压患者的侧枝循环表现。方法收集203例肝硬化门静脉高压患者MSCT上腹部检查的完整资料。结果共发现门静脉侧枝或异常血管612处:食管下段静脉曲张175处,食管旁静脉曲张49处(其中伴假肿瘤征13处),胃底静脉曲张119处,胃冠状静脉曲张105处,胃肾静脉分流34处,脾肾静脉分流15处,椎旁静脉丛曲张16处,脐静脉再通48处,副脐静脉再通22处,脐静脉、副脐静脉同时再通4处,腹壁静脉丛曲张呈蛇头征12处,门静脉海绵样变8处,门静脉右后支瘤样扩张并与下腔静脉分流4处,胃左静脉直接入肝与门静脉左支相连1处。结论MSCT可较好地显示各类门静脉高压侧枝,了解这些异常表现有助于作出正确诊断,避免误诊,对临床选择治疗方法有重要的意义。     

Collateral pathways of the left gastric vein in portal hypertension

Since June 1974, 347 percutaneous transhepatic portal venographic studies were performed on 246 patients with portal hypertension who had had bleeding gastroesophageal varices. Of 234 patients in whom left gastric veins (LGV) (coronary) were demonstrated, 177 (75.6%) had a single LGV and 57 (24.4%) had multiple left gastric veins (21.8% had two LGVs, 2.1% had three LGVs, and 0.5% had five LGVs). Of 193 patients undergoing selective left gastric venography, spontaneous portosystemic communications to the left renal vein were found in 55, to the inferior vena cava in two, to the inferior pulmonary veins in five, to the pericardiophrenic vein in eight, to the right inferior phrenic vein in three, and to the left intercostal veins in one. Interportal communications with the left gastric vein and varices occurred from the left portal vein in 13, from the gastroepiploic vein in one, and from a superior mesenteric vein branch in one. The predominant drainage of esophageal varices was to the azygos vein in 78 of 155 patients, to the hemiazygos vein in 13, and to multiple small unnamed veins in the mediastinum in 57. Opacified varices did not extend above the level of the azygos vein arch in 71 of 130 patients; however, 59 continued cephalad to the azygos arch and drained through more superior veins of the thorax. Knowledge of the anatomy and incidence of each of these portosystemic or interportal venous communications is important to properly treat bleeding esophageal varices by surgery or angiographic embolization.     

肝硬化患者CT门静脉血管成像中门静脉侧支血管的表现

目的 分析肝硬化患者在16排螺旋CT门静脉血管成像(CTPV)上门静脉侧支血管的表现.方法 对36例经临床、肝功能和影像学检查诊断为肝硬化门静脉高压的患者行腹部CTPV检查,经图像后处理,获得门静脉系统及侧支血管三维重建图像.结果 CTPV可以直观地显示门静脉系统及整个门静脉侧支循环系统.36例病例中显示胃左静脉曲张29例(80.6%),食管下段静脉曲张18例(50.0%),胃短/W后静脉曲张15例(41.7%),食管旁静脉曲张9例(25.0%),胃-肾/脾-肾分流8例(22.2%),门静脉海绵样变7例(19.4%),脐静脉与腹壁静脉曲张6例(16.7%),椎旁静脉分流4例(11.1%).结论 CTPV能很好地显示肝硬化患者门脉高压侧支循环的开放部位、范围及严重程度,对判断病情、选择治疗方案及估计预后有重要的临床应用价值.     

Dynamic CT and MRA findings of a case of portopulmonary venous anastomosis (PPVA) in a patient with portal hypertension: a case report and review of the literature

Portopulmonary venous anastomosis (PPVA), which has been rarely reported in conventional CT and MR studies, is an unusual collateral pathway in patients with portal hypertension. It has clinical implications related to right-to-left shunt that are different from the clinical implications related to other more usual portosystemic shunts in portal hypertensive patients. Here, we report the dynamic CT and MRA findings of a case of PPVA in a patient with portal hypertension, directly demonstrating the shunt flow from the paraesophageal varix to the left atrium via the right inferior pulmonary vein.     

Portal vein occlusion with aberrant left gastric vein functioning as a hepatopetal collateral pathway

A rare case of an aberrant left gastric vein functioning as a hepatopetal collateral as a result of portal vein occlusion is presented herein. The portal venous phase of multislice computed tomography clearly demonstrated this anatomic variation and provided a reliable vascular "road map" for percutaneous transhepatic portal venous stent placement. Portal hypertension associated with extensive gastrojejunal varices improved dramatically after stent placement.     

Significance of the site of injection in unexpected right-to-left shunting

Lung perfusion scintigraphy, with Tc-99m MAA injected into a left antecubital vein, revealed extensive uptake in the myocardium, kidneys, spleen, thyroid, and brain. After a right antecubital injection, a normal pulmonary perfusion pattern was found. This finding is specific for right-to-left shunting due to anomalous systemic venous drainage into the left atrium.     

Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients

OBJECTIVE: The purpose of our study is to describe the radiologic findings of adult patients presenting with congenital unilateral pulmonary vein atresia. CONCLUSION: Chest radiography in affected patients typically reveals a small hemithorax and ipsilateral pulmonary artery as well as ipsilateral septal thickening. CT shows, in addition, ground-glass attenuation, the absence of a pulmonary vein connection to the left atrium, and abundant mediastinal venous collateral vessels. MR imaging is helpful in further characterizing the vascular abnormalities. Angiography may help to confirm the diagnosis.     

Patent paraumbilical vein: anatomic and hemodynamic variants and their clinical importance.

Fifty-five of 353 patients with suspected portal hypertension studied with Doppler sonography had a patent paraumbilical vein. Of these 55 patients, 39 had the classic intrahepatic venous circulation found in Cruveilhier-Baumgarten syndrome: hepatopetal flow in all segmental portal veins and hepatofugal flow leaving the liver in a paraumbilical vein in the falciform ligament to join veins of the anterior abdominal wall. Sixteen patients had variants of the classic pattern: Flow in one or more segmental portal veins of the left lobe or the entire liver was hepatofugal. In addition, new venous channels connecting the left portal vein with the extrahepatic paraumbilical vein were found. Assessment of liver function with the Pugh score showed severe impairment in the majority of patients with a patent paraumbilical vein. Patients with the classic intrahepatic circulation had smaller esophageal varices than those with hemodynamic or anatomic variants. The presence of a patent paraumbilical vein did not prevent formation of esophageal varices in the patients studied.     

Normative analysis of pulmonary vein drainage patterns on multidetector CT with measurements of pulmonary vein ostial diameter and distance to first bifurcation

RATIONALE AND OBJECTIVES: To document the frequency of normal and anomalous drainage patterns of the pulmonary veins, and to establish normal values for pulmonary vein ostial diameters, and distance to first bifurcation using multidetector computed tomography, as pertinent to ablation procedures for atrial fibrillation. MATERIALS AND METHODS: Two cardiothoracic radiologists retrospectively reviewed thin-section contrast material-enhanced multidetector computed tomography examinations of the thorax in 200 consecutive patients (38 females and 162 males), age 24-79 years (mean 52.8) referred for imaging before radiofrequency ablation therapy for atrial fibrillation. Pulmonary vein anatomy was based on both the number of venous ostia and the drainage patterns of pulmonary veins. Pulmonary vein ostial diameters and distance to first bifurcation of the four major pulmonary veins (right inferior and superior, left inferior and superior) and any additional pulmonary veins were measured at a workstation using both axial images and multiplanar reconstructions by two experienced cardiothoracic radiologists; the mean pulmonary vein diameter and the shortest distance to first bifurcation of the two measurements are reported. RESULTS: The majority of patients, 82% (164 patients) had four pulmonary veins, with a superior and inferior ostium on the right and a superior and inferior ostium on the left. Of the remainder, 9% (18 patients) had five veins, 4.5% (9 patients) had three veins, 3% (6 patients) had two anomalies each, and 0.5% (1 patient) had three anomalies. The middle lobe pulmonary vein drained into the right superior pulmonary vein in 83.5% of patients, directly into the left atrium in 11% of patients, and into the right inferior pulmonary vein in 5.5% of patients; 6.5% of patients had a single left pulmonary vein ostium. Mean pulmonary vein diameters with 95% confidence intervals at the ostia were as follows: right superior 17.6 (13.64-15.36) mm; left superior 16.6 (16.03-17.08) mm; right inferior 17.1 (16.58-17.55) mm; left inferior 14.8 (14.25-15.27) mm, and independent middle lobe 8.6 (8.27-8.86) mm. Mean distance to first bifurcation with 95% confidence intervals were: right superior 14.5 (17.02-18.23) mm; left superior 17.6 (16.63-18.53) mm; right inferior 7.0 (6.49-7.46) mm; left inferior 13.5 (12.83-14.16) mm, and independent middle lobe 8.4 (7.7-9.17) mm. CONCLUSION: Thin-section thoracic computed tomography demonstrates a greater variability of pulmonary venous drainage than previously described. There is greater variability of the right lung venous drainage compared to the left lung. Eighty-two percent of people have four standard pulmonary veins. There is significant variability in pulmonary vein diameter and distance to first bifurcation.     

Varicose veins of the lung

Varicosity of the pulmonary veins is a rare anomaly of the pulmonary vascular system. The varices do not usually change in size over years, do not cause symptoms and need no therapy. However, raised left atrial pressure can cause increase in the diameter of pulmonary vein varices. A case of lung vein varicosity in the right middle and upper lobe associated with coarctation of the aorta and an anomalous upper-middle lobe vein was observed over a period of 10 years. Increase in left atrial pressure (aortic and relative mitral regurgitation) led to enlargement of the pulmonary veins.     

MDCTP对肝硬化门脉高压侧支循环血管的显示价值

目的:评价16排螺旋CT门静脉血管成像(MDCTP)技术在显示肝硬化门脉高压侧支循环血管方面的应用价值。方法:对38例临床诊断肝硬化门脉高压的患者行上腹部MDCTP检查。采用MIP、MPR、VR等三维重组技术进行图像后处理,获得门静脉系统及侧支循环血管图像。结果:38例中显示食管胃底粘膜下静脉曲张31例,食管旁静脉曲张10例;胃左静脉曲张29例,胃短静脉曲张26例;脐静脉与腹壁静脉曲张7例;脾-肾或胃-肾分流6例;腹膜后分流2例。结论:MDCTP能显示肝硬化患者门-体侧支循环开放部位、范围及程度,有重要的临床应用价值。     

Siebzehnjährige Schülerin mit therapieresistenter Pneumonie

If empiric antibiotic treatment is not successful in a case of suspected pneumonia further investigations are required. We present a case where originally an intralobar, bronchopulmonary sequestration could clearly be diagnosed by a thoracic CT scan. A 3D reconstruction revealed the pathognomonic arterial supply from the thoracic aorta and the venous drainage via pulmonary vein to the left atrium. Therefore, elective resection of the sequestration was indicated.     

A pulmonary venous indentation on the esophagus-a normal variant.

An extrinsic indentation on the anterior left wall of the esophagus, about 4-5 cm below the carina, is a relatively common finding and should be considered a normal variant, although rarely it may be due to an aberrant insertion of a pulmonary vein into the left atrium. The indentation is best seen in a steep left posterior oblique horizontal position. In most cases it is caused by pressure of the left inferior pulmonary vein or a common confluence of the left pulmonary veins near the insertion into the left atrium. Generally, it does not cause any symptoms and should not be mistaken for a mediastinal mass.     

Direct percutaneous access to an omental vein for embolization of Roux-en-Y limb varices in a child

An 11-year-old boy with a history of hepatoblastoma treated with chemotherapy, radiation therapy, and liver transplantation presented with bleeding from Roux-en-Y limb varices. The transhepatic approach for portal intervention posed a risk of liver graft injury. An omental vein that was dilated as a collateral vein due to portal hypertension was found and compressible under ultrasound. The omental vein was percutaneously punctured, and the varices were embolized through a jejunal vein. No complication occurred. Direct percutaneous access to the portal venous system is a useful technique for portal embolization.     

Huge Varicose Inferior Mesenteric Vein: an Unanticipated 99m Tc-labeled Red Blood Cell Scintigraphy Finding

Ectopic varices (EcV) are enlarged portosystemic venous collaterals, which usually develop secondary to portal hypertension (PHT). Mesocaval collateral vessels are unusual pathways to decompress the portal system. Here we report the case of a huge varicose inferior mesenteric vein (IMV) that drained into perirectal collateral veins, demonstrated by ^99mTc-labeled red blood cell (RBC) scintigraphy performed for lower gastrointestinal (GI) bleeding in a 14-year-old girl. This case illustrates the crucial role of ^99mTc-labeled RBC scintigraphy for the diagnosis of rare ectopic lower GI varices.     

MRI of the pulmonary veins: comparison between 3D MR angiography and T1-weighted spin echo

OBJECTIVE: The purpose was to determine the ability of three-dimensional (3D) magnetic resonance (MR) angiography to depict normal pulmonary veins in comparison with spin-echo MR imaging. MATERIALS AND METHODS: MR imaging of 40 patients with cardiovascular disease were reviewed. Patients with known pulmonary venous abnormalities were excluded. Using a standard GE 1.5-T magnet, axial T1-weighted spin-echo 5-mm-thick contiguous slices and 3D MR angiography (contiguous slice thickness of 2.5-3.5 mm, 20-30 c.c. of gadolinium bolus at 1-1.5 c.c./sec, 32-43-second breath-hold, coronal and sagittal plane acquisition) were evaluated retrospectively on separate occasions by two experienced radiologists. Multiplanar imaging projection was used for the identification of pulmonary veins. Each lung was considered to have two drainage veins: a superior vein and an inferior vein. Identification of a pulmonary vein was made by visualizing a connection with the left atrium. RESULTS: 143 pulmonary veins (87.5% +/-5.2) were identified at the level of the left atrium on T1-weighted spin-echo images, and 157 (98.1% +/-1.9) were identified on 3D MR angiography (p<0.01). Overall we identified by T -weighted spin-echo imaging 36 right upper, 38 right lower, 27 left upper, and 38 left lower pulmonary veins. By 3D MR angiography, we identified 38 right upper, 40 right lower, 39 left upper, and 40 left lower pulmonary veins. All four pulmonary veins were detected in 22 patients on spin-echo imaging (55%) and in 37 patients (92.5%) on 3D MR angiography (chi = 3.81, p<0.05). CONCLUSION: A significant difference is demonstrated between 3D MR angiography and spin-echo MR imaging in identifying normal pulmonary veins. MR angiography provides a complete view of normal pulmonary venous anatomy and could be a valuable tool for the assessment of abnormal pulmonary venous drainage.     

门静脉高压静脉曲张的螺旋CT表现

目的评价门静脉高压静脉曲张的螺旋CT表现.材料和方法回顾性分析47例经螺旋CT平扫及双期增强扫描诊断为门静脉高压静脉曲张的CT表现,并对其发生部位作了统计.结果增强扫描门静脉期扭曲扩张的静脉表现为曲线条、团块状或结节状高密度影.其中食管下端粘膜下及食管旁静脉曲张最常见(发生率为61.7%),以下依次为胃周静脉曲张(36.2%),脾门附近静脉曲张(23.4%),脐旁静脉曲张(21.3%),肾及肾上腺静脉曲张(12.8%),腹膜后一椎旁静脉曲张(4.3%),门静脉海绵状变性(4.3%).结论螺旋CT增强扫描门静脉期静脉曲张一目了然,结合其特定的解剖部位,CT能作出定性诊断并判断其程度.     

Unsuccessful lung scan due to major right-to-left shunt through a sinus venosus septal defect

In a patient with a prior history of cerebral abscess and cerebral ischemia, an unsuccessful perfusion lung scan led to a radionuclide angiocardiogram using an arm vein injection. This showed a total right-to-left (R-L) shunt from the superior vena cava (SVC) to the left atrium. Repeat radionuclide study, through a leg vein, demonstrated a moderate R-L shunt and an interpretable lung scan could be obtained. Catheterization and contrast cineangiogram did not provide the exact diagnosis, the preoperative conclusion being anomalous drainage of the SVC into the left atrium, with atrial septal defect (ASD) and partial anomalous pulmonary venous connection to the SVC. The operative diagnosis was high atrial (sinus venosus) septal defect. This example of major but clinically unsuspected R-L shunt emphasizes the value of performing a perfusion lung scan, preferably in conjunction with radionuclide angiocardiography in patients with a prior history of unexplained cerebral abscess or systemic ischemia. Implications of the site of an ASD on quantitation of L-R shunts by radionuclide methods are also discussed.     

多层螺旋CT门静脉成像对门脉高压侧支循环血管的研究

目的:评价多层螺旋CT门静脉成像(MDCTP)在门脉高压侧支循环血管显示中的价值。方法:31例经临床检查确诊的门静脉高压患者行MDCTP检查,采用MIP、MPR、VR等重建技术进行图像后处理,获得门静脉系统及侧支血管图像,两名医师分别判断侧支血管显示情况。结果:MDCTP不仅显示肝内门静脉3~4级分支,还显示了整个门脉侧支血管系统。31例患者中,胃左静脉曲张28例,食管或食管旁静脉曲张27例,脾静脉曲张21例,胃短/胃后静脉曲张7例,脾-肾分流血管1例,脐静脉曲张伴腹壁静脉曲张2例,门静脉栓塞4例,肠系膜上静脉血管闭塞2例。结论:MDCTP能显示门静脉高压侧支血管开放的部位、范围及程度,有助于对门脉高压患者治疗方案的选择,是一种有重要临床价值的无创性门脉检查方法。