Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome

A case in which anomalous systemic venous drainage occurred in association with the hypogenetic lung syndrome (scimitar syndrome) is described. The chest radiograph appearances of the anomalous systemic vein mimicked an anomalous pulmonary or scimitar vein. Angiography demonstrated that the patient also had a small anomalous pulmonary vein draining and a systemic artery supplying, the right lung. As the right lung was hypoplastic, all three features of the hypogenetic lung syndrome were present, in addition to partial anomalous systemic venous drainage.     

Scimitar syndrome: MDCT imaging revisited

Scimitar syndrome refers to a form of anomalous pulmonary venous drainage which implies partial or total failure of the pulmonary veins to reach the left atrium.AimTo emphasize the role of MDCT in the diagnosis of Scimitar syndrome.Patients and methodsWe presented MDCT imaging findings in three patients with Scimitar syndrome.ResultsMDCT revealed anomalous arterial blood supply from abdominal aorta to right lower lung lobe and anomalous venous drainage into infradiaphragmatic IVC, hypoplastic right pulmonary artery and hypoplastic right lung as well as atrial septal defect and bronchial diverticulum.ConclusionMDCT is essential investigation in Scimitar syndrome.     

Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI)

Scimitar syndrome is a rare congenital pulmonary anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava or the right atrium. Very few reports are available that analyze the value of magnetic resonance imaging (MRI) in establishing the diagnosis. We present a case with Scimitar syndrome in which anomalous pulmonary venous return was confirmed by cine MRI.     

Embolization of the Systemic Arterial Supply via a Detachable Silicon Balloon in a Child with Scimitar Syndrome

Scimitar syndrome is a rare congenital disorder. It is characterized by partial or total abnormal venous drainage of the right lung into the inferior vena cava, which is often associated with anomalous systemic arterial supply to the right lung, congenital cardiac anomalies, hypoplasia of the right lung and bronchial anomalies. Symptoms depend on the degree of the shunt and severity of the associated anomalies, which determine the treatment. We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. Successful elective surgery was performed 6 months later, in which right pulmonary veins were directed to the left atrium using a Gore-Tex patch by creating an intra-atrial tunnel. The patient has been symptom-free period during 6 months of follow-up, which supports the idea that recurrent pulmonary infections can be eliminated by embolization of the anomalous arterial supply.     

Horseshoe lung: clinical, pathologic, and radiologic features and a new plain film finding

Horseshoe lung is a rare congenital malformation in which an isthmus of pulmonary parenchyma extends from the right lung base across the midline behind the pericardium and fuses with the base of the left lung. Six cases are presented and eight previously published case reports are reviewed. Eleven of these 14 cases occur in conjunction with scimitar syndrome, the complex of anomalies including hypoplasia of the right lung, anomalous right pulmonary venous return, and anomalous arterial supply to the right lung. Differentiation of scimitar syndrome from horseshoe lung can be made on the plain chest film, if, in addition to the typical radiographic findings of scimitar syndrome, there is evidence of a fine linear density in the medial aspect of the left base representing the lateral extent of the pulmonary isthmus. Recognition of horseshoe lung is important because children present in the first few years of life with significant respiratory symptoms, whereas the scimitar syndrome alone is usually discovered fortuitously after the first decade of life.     

24例镰刀综合征心血管造影征象分析

笔者分析了２４例镰刀综合征心肺血管病变的造影征象及其病理生理意义，并与手术（１４例）和尸解（４例）资料对照。２４例心血管造影示全部或部分右肺静脉异常引流，分别为１７例和７例。异常引流静脉近端局限性狭窄９例，其中２例异常和正常引流静脉间有侧支吻合，右下肺异常体动脉侧支供血１８例。并存各种心脏病变者１８例，马蹄肺６例，右下肺肺隔离症３例，下腔静脉闭锁和狭窄分别为２和１例。右下肺异常体动脉侧支和心脏病变是患者严重症状和肺动脉高压的主要因素。除肺隔离症外，经导管栓塞异常体动脉侧支可使用者明显受益。异常引流静脉狭窄具有限制血液分流的作用。     

Incidental meandering right pulmonary vein,literature review and proposed nomenclature revision

We report a case of an anomalous pulmonary vein on chest X-ray resembling a scimitar sign in an 80-year-old female undergoing investigation of syncope.Multislice computed tomography(CT) with multiplanar reformatting and maximum intensity projections demonstrated an aberrant right inferior pulmonary vein coursing inferomedially towards the diaphragm before turning superiorly and draining normally into the left atrium.The diagnosis of an incidental meandering right pulmonary vein was established.The case is used to review the literature on this rare pulmonary anomaly,including pathogenesis,its relationship with scimitar syndrome and scimitar variant,and diagnosis,with an emphasis on the role modern CT techniques can play in noninvasive diagnosis.A revision to the nomenclature of pulmonary vascular anomalies is proposed to help reduce confusion in the literature.     

Acquired anomalous intrapulmonary venous connection secondary to pulmonary venous stenosis

An unusual case of acquired development of anomalous intrapulmonary venous connection with pulmonary venous stenosis is presented. Appearances on a chest radiograph resembled the "scimitar" sign in a patient with previous surgery for partial anomalous pulmonary venous return. Spiral CT and pulmonary arteriography showed stenosis of the right upper pulmonary vein and an anomalous intrapulmonary venous connection between the right upper pulmonary vein and the right lower pulmonary vein. We consider the slow progression of pulmonary vein stenosis led to anomalous intrapulmonary venous connection as an intrapulmonary collateral.     

Anomalous pulmonary venous connection of entire left lung with intact atrial septum.

Three cases of total unilateral pulmonary venous connection of the left lung with intact atrial septum are described. All patients were acyanotic and had findings of volume overload of the right side of the heart. An anomalous vertical vein in the superior mediastinum and large pulmonary arteries were visible on the plain chest radiograph, similar to what is seen in cases of total anomalous pulmonary venous connection. Selective pulmonary angiography delineated the anomalous connection of the left pulmonary veins and documented the absence of an atrial septal defect.     

Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR

PurposeTo report the anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR.Materials and methodsRetrospective review of the medical records between February 2001 and February 2016 was performed. To identify patients, radiological reports were queried for “Scimitar” or “partial anomalous pulmonary venous return.” Patients with radiological findings of Scimitar syndrome were included. Patients without cross-sectional imaging were excluded. Patients' demographics, radiologic images, and medical notes were reviewed. Two radiologists re-read the available imaging studies. Images were reviewed for Scimitar syndrome confirmation, number and location of Scimitar vein drainage, number of lobes drained by the Scimitar vein, and right pulmonary artery and lung hypoplasia. In addition, the number of pulmonary veins draining into the left atrium, left sided anomalous pulmonary veins, congenital heart disease, aortic arch anomalies, cardiac dextroposition, right ventricular enlargement, pulmonary artery enlargement, and elevated QP:QS ratios were identified. Other associated anomalies including the presence of an anomalous feeding artery and pulmonary sequestration, abnormal lobar pattern, localized bronchiectasis, horseshoe lung, accessory diaphragm, diaphragmatic hernia, vertebral anomalies, and genitourinary tract anomalies were reviewed.ResultsSixteen patients (3 males, 13 females; mean age 39.5 years, range 14 days–72 years) with confirmed Scimitar syndrome on CT and MR imaging were identified. The Scimitar vein drained to the infra-diaphragmatic inferior vena cava (IVC) in ten patients and to the supra-diaphragmatic IVC in six patients. The most common associated anomalies were right ventricle enlargement (93.3%), variant lobar pattern of the right lung (92.9%), enlarged pulmonary arteries (60%), and cardiac dextroposition (50%).ConclusionRecognizing the radiologic characteristics and anatomical associations of Scimitar syndrome is important as features of the primary condition and associated anomalies may have implications in surgical management.     

浅析右肺中叶综合症的X线与临床(附病例报告2例)

目的:确定常规X线正位加侧位及前弓位胸片能否提高右肺中叶综合症的诊断准确性。材料与方法:2例右肺中叶综合症患儿(男女各1例:年龄均为6岁)于临床症状出现后3～15天内经常规X线正位片加摄侧位及前弓位胸片检查。结果:X线正位加摄侧位及前弓位胸片显示,右肺中叶呈带状致密影,并与心影重叠相连,右心膈区出现一尖端向内的三角形高密度阴影。结合临床症状与体征,诊断为右肺中叶综合症,后经正规抗痨治疗,胸片上的阴影连同临床症状与体征一起全部消失。结论:常规X线正位片加摄侧位及前弓位胸片能卓有成效地提高右肺中叶综合症的诊断准确性。     

The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return

An anomalous pulmonary vein draining into the subdiaphragmatic inferior vena cava was initially demonstrated on computed tomographic (CT) scans. The diagnosis of scimitar syndrome was confirmed with digital subtraction angiography. In retrospect, the anomalous vein and dextroposition of the heart were shown on chest radiographs.     

Scimitar syndrome associated with gallbladder duplication

Scimitar syndrome is a rare congenital anomaly associated with the venous drainage of the lung to the inferior vena cava through a systemic vein. Chest radiography of an asymptomatic patient, aged 36, showed a linear opacity extending from the pulmonary hilum to the diaphragm, on the right chest (Scimitar sign). Computerized tomography of the thorax exposed that the cause of that image was Scimitar vein. Abdominal ultrasonography revealed duplication in the gallbladder. The literature holds no reports of an association between Scimitar syndrome and gallbladder duplication. Secondary gastrointestinal malformations can also be found in patients suffering from this syndrome.     

Computed tomography of partial anomalous pulmonary venous connection in adults

OBJECTIVES: To systematically describe the imaging features and clinical correlates of a partial anomalous pulmonary venous connection diagnosed on computed tomography (CT) in adults. METHODS: Twenty-nine adults with a partial anomalous pulmonary venous connection on CT were retrospectively identified. There were 19 women and 10 men, with a mean age of 53 (range: 19-83) years. Four cases were identified by review of 1825 consecutive chest CT reports from July 2000-July 2001, and 25 cases were culled from chest radiology teaching files at 3 institutions. Inclusion criteria were availability of CT images and medical charts. Chest radiographs (25 of 29 cases) were reviewed for mediastinal contour abnormalities, heart size, and pulmonary vascular pattern. Chest CT scans were reviewed for location, size, and drainage site of the anomalous vein; presence or absence of a pulmonary vein in the normal location; cardiac size and configuration; and pulmonary vasculature. Charts were reviewed for evidence of pulmonary and cardiovascular disease, history of congenital heart disease, and results of other cardiac imaging. RESULTS: The prevalence of a partial anomalous pulmonary venous connection was 0.2% (4 of 1825 chest CT reports). Seventy-nine percent (23 of 29 patients) had an anomalous left upper lobe vein connecting to a persistent left vertical vein, only 5% (1 of 23 patients) of whom had a left upper lobe vein in the normal location. Seventeen percent (5 of 29 patients) had an anomalous right upper lobe vein draining into the superior vena cava, 60% (3 of 5 patients) of whom also had a right upper lobe pulmonary vein in the normal location. One patient (3%) had an anomalous right lower lobe vein draining into the suprahepatic inferior vena cava. Chest radiographic findings were abnormal left mediastinal contour in 64% (15 of 25 patients), abnormal right mediastinal contour in 8% (2 of 25 patients), and cardiomegaly in 24% (6 of 25 patients). Computed tomography findings were cardiomegaly in 48% (14 of 29 patients), right atrial enlargement in 31% (9 of 29 patients), right ventricular enlargement in 31% (9 of 29 patients), and pulmonary artery enlargement in 14% (4 of 29 patients). Pulmonary or cardiovascular symptoms were present in 69% (20 of 29 patients), 55% (11 of 20 patients) of whom had specific alternative diagnoses (excluding congestive heart failure and pulmonary hypertension) to explain the symptoms. Only 1 patient (3%) was diagnosed with a secundum atrial septal defect. CONCLUSIONS: A partial anomalous pulmonary venous connection was seen in 0.2% of adults on CT. In contrast to previous series focusing on children, the anomalous vein in adults was most commonly from the left upper lobe, in women, and infrequently associated with atrial septal defects.     

肺类癌致异位促肾上腺皮质激素综合征的影像学特点分析

目的：探讨影像手段在隐匿性小结节型样肺类癌致异位促肾上腺皮质激素综合征（EAS）中的诊断价值。 方法：回顾性分析我院收治并确诊的两例小结节样肺类癌致EAS患者的影像资料,包括：MRI、PET-CT、胸部CT、双侧岩下窦静脉采血、肺结节穿刺活检术;结合患者临床表现和实验室检查,通过文献分析总结此类病例的影像特点。 结果：两例患者均有明显的库欣综合征表现,外周血促肾上腺皮质激素（ACTH）明显升高,皮质醇分泌无昼夜节律。MRI资料提示不能排除垂体微腺瘤,双侧岩下窦静脉采血排除了垂体来源ACTH瘤,因胸部CT均发现类圆形小结节,一例右上肺结节穿刺活检证实肺类癌来源ACTH瘤;另一例左下肺结节选择直接胸腔镜手术切除。手术切除后行病理学检查示一例为低分化类癌,另一例为不典型类癌。 结论：支气管肺部类癌是导致EAS的常见病因之一,如青壮年库欣综合征患者同时发现肺部小结节,MRI或双侧岩下窦静脉取血排除了垂体来源的ACTH瘤,应警惕排除肺部隐匿性异位分泌ACTH的肿瘤,必要时穿刺活检及影像学检查随访,早期发现并手术切除肺部肿瘤。     

Loeffler's syndrome in a child: A rare radiological and histopathological diagnosis

Loffler syndrome is an uncommon, self-limited, benign pulmonary eosinophilia that usually lasts less than a month. Abnormal chest radiography occurs in 95% of patients; however, computed tomography findings are not well described. We present clinical features, radiological, and pathological findings of Loeffler''s syndrome with secondary bacterial pneumonia in a child. He presented with dry cough, hemoptysis 2 times, chest pain for 1 week. Blood tests revealed high C-reactive protein levels and eosinophilia. On the initial computed tomography (CT) scan, a lesion was discovered at the upper edge of the right lung hilum. The lesion developed in size, together with right pleural effusion, on the repeated CT scan. A lung biopsy revealed a substantial number of inflammatory cells, including eosinophils and neutrophils. After ruling all other possibilities, Loffler''s syndrome was confirmed. As a result of antibiotic treatment, favorable outcomes were confirmed by improving clinical symptoms and follow-up chest CT scans. A close combination of pulmonary symptoms, peripheral blood eosinophilia, abnormal chest imaging, and histopathological findings must be taken to confirm the diagnosis of Loeffler''s syndrome.     

Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography

We report two cases of abnormal configuration of the pulmonary venous system of the right lung evaluated with the use of three-dimensional spiral computed tomography (3D-CT). In the first case, an unusual form of scimitar syndrome was detected, consisting of two scimitar veins uniting into a common trunk immediately before their confluence into the inferior vena cava (IVC). The second case concerns a meandering pulmonary vein that followed a circuitous course through the right lung and came in close proximity to the IVC, before draining the entire right lung to the left atrium. In both cases, 3D-CT enabled a non-invasive, detailed anatomic evaluation of the right pulmonary vascular bed.     

Hemoptysis complicating Scimitar Syndrome: from diagnosis to treatment

We report the case of a 24-year-old patient with known scimitar syndrome presenting with hemoptysis. Multidetector CT angiography showed the scimitar vein draining the right lung to the inferior vena cava associated with right lower lobe bronchopulmonary sequestration. The presence of hemoptysis associated with scimitar syndrome is likely secondary to an anomalous systemic arterial blood supply. We treated this patient successfully with transcatheter occlusion of the anomalous feeding arterial vessels.     

Sigmoid megaesophagus with thoracic pseudotumor appearance

A 61-year-old man presented with dysphagia, weight loss and shortness of breath. On examination, he had reduced lung sounds on the right, and obvious neck vein distention. Chest X-ray raised a suspicion of lung tumor with possible superior vena cava syndrome. Subsequent CT scan of chest confirmed the presence of markedly dilated and tortuous esophagus (sigmoid megaesophagus) extending to the right hemithorax and pressing on the trachea. The patient was referred for surgery.     

特发性单侧透明肺的影像诊断

目的分析特发性单侧透明肺的影像诊断。方法本组共29例特发性单侧透明肺。29例均拍胸部正侧位片,其中19例行电视透视,14例行胸部CT扫描,2例做支气管造影,1例行体层摄影,1例行肺动脉、主动脉造影。结果29例患肺透亮度增大,其中左全肺23例,左下叶5例,左全肺并右肺中下叶1例。左肺门及肺血管性纹理细小。21例左侧胸廓轻度塌陷,21例纵隔气管左偏,19例电视透视见纵隔摆动,14例CT扫描见左肺动脉细小,其中主干细小9例,左肺下叶动脉细小5例。12例见右下肺动脉干增粗,5例动脉段延长,7例肺动脉段突出,4例心脏增大,19例支气管扩张。结论影像学检查是特发性单侧透明肺的关键确诊手段。