Pulmonary sequestration: diagnosis with color Doppler sonography and a new theory of associated hydrothorax

The diagnosis of pulmonary sequestration has traditionally relied on angiographic demonstration of a systemic artery to the sequestered lung tissue. Rarely, extralobar sequestration can be associated with tension hydrothorax, which in the past has invariably led to fetal hydrops and death. The authors report the cases of three infants who had initially undergone color and spectral Doppler analysis; in two of them, extralobar sequestrations were associated with congenital hydrothorax. All three patients underwent surgical resection and histopathologic evaluation of their sequestrations. On the basis of the findings, the authors believe that torsion of the sequestration occludes the efferent venous and lymphatic channels, initiating the accumulation of pleural fluid and subsequent hydrops through systemic venous obstruction. Color Doppler made possible the identification of minute feeding vessels, obviating further diagnostic studies.     

Echography of pulmonary sequestration

Pulmonary sequestration is a rare congenital disorder in which a portion of normal lung tissue lacks normal connections with the tracheo-bronchial tree and the pulmonary arteries. We report on three cases of left lower lobe extrapleural sequestrations that have been studied with ultrasound since 1980. In the two more recent cases demonstration of the feeding vessel originating directly in the abdominal aorta by real-time ultrasound permitted very rapid non-invasive diagnosis of the malformation. We recommend that a search should be made for such an aberrant vessel with real-time ultrasound whenever a pulmonary sequestration is suspected.     

Surgical treatment of lung sequestration

BACKGROUND/AIM: Pulmonary sequestration is a congenital malformation which consists of afunctional part of the lung, separated of the normal airway, and vascularisated with anomal systemic artery instead of pulmonary artery. There are two kinds of sequestration. Intralobar is surrounded with normal lung and its pleura, and extralobar which has extrapulmonary position and pleura of its own. This anomaly is very rare and appears in 1.1-1.8% of all congenital lung malformations. The illness is revealed either in early childhood whith other life-threatening anomalies or in adulthood and middle age when secondary infection arises. The aim of this paper was to show our own experience in surgical treatment of pulmonary sequestration and to emphasize sequestration as a real differential-diagnostic possibility with patients with recidive bronchopneumonias. METHODS: We retrospectively analyzed medical records for the period from 1967-2007 and found 15 patients with pulmonary sequestration at the average age of 30 years. We pointed out the well known problems with identification of this anomaly, preoperative diagnostic procedures and surgical possibilities of treatment. RESULTS: There were 13 patients with intralobar and two patients with extralobar sequestration. By the use of preoperative angiography, seven patients were found to have intralobar pulmonary sequestration. All intralobar sequestrations were clinically manifested, the most often with recidive bronchopneumonia. Six patients had no preoperative diagnosis of lung sequestration. The most common locality of intralobar sequestration was the left lower lobe (eight patients). We performed nine lobectomies, three sequestrectomies, two segmentectomies and one pneumonectomy. Both extralobar sequestrations were diagnosed intraoperatively. CONCLUSION: Pulmonary sequestration is a rare malformation. Diagnosis is established by angiography. Treatment is exclusively surgical. In the last three years we have had one patient per year. This experience obliges to consider pulmonary sequestration as a real differn tial diagnostic possibility in patients with localised repeated bronchopneumonias.     

Bronchopulmonary sequestration: CT assessment

Computed tomographic (CT) scans of 24 bronchopulmonary sequestrations in 23 patients were reviewed. Seventeen sequestrations were diagnosed at surgery, three at angiography, and four on the basis of radiographic or CT findings combined with appropriate history. Sixteen sequestrations were intralobar, and eight were extralobar; 21 were posterobasal. Seventeen occurred on the left side and seven on the right. Anomalous systemic arterial supply was demonstrated by CT in 16 sequestrations. In the others, a systemic artery was not shown, presumably because of unfavorable orientation or small size of the vessel. The lung abnormalities shown by CT were classified into three types: A = cysts containing air or fluid (n = 8), or soft-tissue masses (n = 2); B = emphysematous lung surrounding cysts, and/or soft-tissue nodules (n = 13); and C = lung hypervascularity (n = 2). In only three cases did the chest radiograph show the emphysematous lung tissue. Such emphysematous lung has rarely before been reported as a CT finding, and lung hypervascularity has not, to the authors' knowledge, been reported. The authors conclude that CT can be helpful in the diagnosis and evaluation of bronchopulmonary sequestration. Characteristic manifestations are (a) a complex lesion containing solid or fluid components combined with emphysematous lung or (b) any basal lesion supplied by a systemic artery.     

3D multidetector CT angiographic evaluation of extralobar pulmonary sequestration with anomalous venous drainage into the left internal mammary vein in a paediatric patient

Pulmonary sequestration is a congenital lung malformation, defined by dysplastic and non-functioning lung tissue lacking normal tracheobronchial connections and accompanied by an anomalous systemic blood supply. Recognition of anomalous arteries and veins in pulmonary sequestration is paramount to making the correct diagnosis. In contrast to intralobar pulmonary sequestration, where anomalous venous drainage is usually into the pulmonary venous system, the pattern of anomalous venous drainage is more varied in extralobar pulmonary sequestration. To the best of our knowledge, anomalous venous drainage to the internal mammary vein in extralobar sequestrations has not been reported. We report an anomalous venous drainage into the internal mammary vein in an extralobar sequestration which was evaluated with 3D multidetector CT angiography.     

Bronchopulmonary foregut malformations. A unifying etiological concept.

Two cases of congenital bronchopulmonary foregut malformation are reported and another 27 cases reviewed and the findings analyzed. The left lower lobe and the entire right lung are the most common locations of pulmonary involvement. The distal portion of the esophagus or cardioesophageal junction was the most common site of communication (83%). The majority of the patients (60%) presented in the first eight months of life, and the incidence in females was nearly twice that in males. Chronic cough, recurrent pneumonias and respiratory distress were the most common clinical findings, whereas two patients were totally asymptomatic. The esophagogram was the single most useful diagnostic procedure (82%). The microscopic structure of the congenital fistula resembled esophagus, bronchus or both. Surgical treatment was curative in most instances. Sime deaths occurred prior to corrective surgery, whereas the postoperative deaths in most instances were related to severe associated congenital anomalies. We believe a common embryologic pathogenesis leads to the formation of a variety of bronchopulmonary foregut malformations. These bronchopulmonary foregut malformations include intralobar and extralobar sequestrations, pulmonary sequestration with patent, or involuted--partial or complete--gastroesophageal communication, esophageal or gastric diverticula, and esophageal or bronchogenic duplication cysts.     

Gynecologic vascular abnormalities: diagnosis with Doppler US

The authors describe the use of duplex and/or color Doppler ultrasonography of the pelvis in three women to demonstrate the presence of venous malformations. One patient with a pulsatile vaginal mass was shown to have an arteriovenous malformation of the vaginal wall. The second patient was shown to have an unsuspected venous angioma in the endometrial cavity. The third patient was shown to have adnexal varices that closely mimicked hydrosalpinx. In the latter two cases, the duplex and color flow capabilities of an endovaginal probe were especially important.     

Small hepatocellular carcinoma: differentiation from adenomatous hyperplastic nodule with color Doppler flow imaging.

Liver nodules smaller than 25 mm in diameter (19 hepatocellular carcinomas [HCCs] and nine adenomatous hyperplastic nodules [AHNs]) were examined with color Doppler flow imaging and hepatic angiography. Angiography revealed a tumor vessel in eight (42%) of the 19 HCCs, while color Doppler flow imaging revealed an arterial pulsating afferent tumor vessel in 10 (53%) of the 19 HCCs but in none of the AHNs. In addition, color Doppler flow imaging revealed a constant-flow efferent tumor vessel continuing to a portal branch in 10 (53%) of the 19 HCCs but in none of the AHNs. In 15 (79%) of the 19 HCCs, a pulsating afferent tumor vessel or a constant-flow efferent tumor vessel or both were observed. Therefore, in this series, color Doppler flow imaging was of value in distinguishing between these two lesions.     

Soft-tissue vascular anomalies: utility of US for diagnosis

PURPOSE: To determine the ultrasonographic (US) features that distinguish soft-tissue hemangioma from vascular malformation and one type of malformation from another. MATERIALS AND METHODS: Eighty-seven vascular anomalies were evaluated by means of US. Lesions were assessed for the presence of solid tissue and abnormal arteries, veins, or cysts. Vessel density, peak flow velocities, and resistive indexes were compared. RESULTS: There were 49 hemangiomas and 38 vascular malformations. A significantly greater proportion of hemangiomas (48 of 49) compared with vascular malformations (zero of 38) consisted of a solid-tissue mass (P < .001). Vessel density was comparable for hemangioma and arteriovenous malformation (AVM) but significantly greater compared with the other vascular malformations (P < .001 in each case). No differences in mean arterial peak velocity were detected between hemangiomas and malformations. Mean venous peak velocity was significantly higher for AVM than for other vascular malformations and hemangioma. Mean resistive index was greater for lymphatic malformation than for hemangioma or AVM. Abnormal veins, arteries and veins, or cysts were univariate predictors for distinguishing between venous, arteriovenous, and lymphatic malformations (P < .001 in all cases). Solid-tissue mass was the only multivariate predictor for differentiating hemangioma from vascular malformation (likelihood ratio test = 109.8, P < .001). CONCLUSION: US can be used to distinguish hemangioma from vascular malformation and detect arterial flow. These distinctions are critical for subsequent management and assessing prognosis.     

Cystic adenomatoid malformation in children: CT histopathological correlation

PURPOSE: This study was performed to assess the accuracy of computed tomography (CT) in classifying the various types of cystic adenomatoid malformation (CAM) of the lung, as described by Stocker et al., taking histopathology as the gold standard. MATERIALS AND METHODS: We retrospectively reviewed six cases of histologically proven CAM. Chest radiography, chest CT and histopathology results were available for all patients. The CT images were reviewed blinded to the histological findings, and attention was paid to the number and size of cysts so as to classify the lesions into the three groups described by Stocker et al. The classification of lesions based on the CT images was then correlated to the histopathological findings. RESULTS: Areas with small-sized cysts (<2 cm) were detected by CT in two patients (33.3%), areas with large cysts (>2 cm) were seen in three cases (50%) whereas in the remaining case, the diagnosis was mixed type I and type II CAM. In one patient with type I CAM, an area of low-density consolidation around the cysts was interpreted as CAM in a context of pulmonary sequestration. The CT classification based on Stocker et al.'s categories was in agreement with the histopathological findings in four cases, whereas in the remaining two cases, the lesions were classed as type I or II on CT and as mixed (type I and II) lesions at histopathology. In one case, the CT classification was correct, but the histopathology revealed the coexistence of pulmonary sequestration. CONCLUSIONS: In our study, there was concordance between CT and histopathology in 66.7% of cases, whereas in 33.3% histopathology revealed areas with mixed grade lesions. CT proved to be accurate in identifying and characterising CAM and provided important information on lesion site and extension.     

Congenital anomalies of the lungs in adults: MR diagnosis

Over an 18-month period, 11 adults with congenital pulmonary anomalies within the sequestration spectrum, identified either surgically or radiographically by CT and/or angiography, were evaluated with MR. These included seven patients with bronchogenic cysts, two with intralobar pulmonary sequestrations, one with scimitar syndrome, and one with bronchial atresia. In each case, MR showed at least some findings indicative of the correct diagnosis. MR proved especially effective in the detection of abnormal fluid collections (nine cases) and the identification of anomalous vessels (three cases). Fluid collections were easily identified in all cases, including four cases with high-density collections on CT (two with bronchogenic cysts and two with intralobar pulmonary sequestration), especially when single-level, multiecho T2-weighted sequences were obtained. Calculated T2 values ranged from 78 to 439 msec (average, 223 +/- 123 msec). Although T2 values were statistically significant for differentiating fluid from muscle (p = .0012) and fat (p = .0046), they were not sufficiently specific to allow precise fluid characterization. Significant limitations were also apparent, such as an inability to detect alterations in the parenchymal architecture of the lungs (three cases) or intrapulmonary vascularity (one case). Despite this, we conclude that MR may be of considerable value in the assessment of congenital pulmonary anomalies and in select cases obviate more invasive diagnostic procedures.     

羊水量与胎儿畸形相关性的研究

目的：应用彩色多普勒超声对20周以上胎儿进行检测，探讨羊水量与胎儿畸形的相关性。方法：回顾性分析1993年以来超声检查的20，200例20周以上的胎儿中，检出羊水量异常404例；分析研究羊水量的多、少、胎儿畸形的部位及单、多发等情况。结果：检出的404例羊水异常，其中羊水过多191例，羊水过少213例，两者的畸形发生率分别为：41％(78／191)；33％(70／213)。结论：彩色多普勒超声对胎儿及其附属物的检查，是其他任何一种影像检查方法不可比拟的。本组资料进一步证明羊水量的异常与胎儿畸形的发生率密切相关。     

Arteriovenous malformations of the kidneys: diagnosis and follow-up with color Doppler sonography in six patients

We performed color Doppler sonography in six patients with arteriovenous malformations of the kidneys. The diagnosis was established by angiography in all patients. Color Doppler sonograms were obtained at a large Doppler frequency-shift range (55 cm/sec of maximal average flow velocity at zero Doppler angle) to depict high-velocity blood flow in the malformation. In all patients, the malformations were seen as focal areas of flow, portrayed as a mixing of lighter colors. These were reflected by a rapid flow rate and marked tortuosity of the vessels. The sonograms showed a small peripheral malformation that was indistinct on selective renal angiography. However, flow in normal vessels grouped in the hilum obscured lighter-color flow of a small central malformation. In three patients who had total or partial ablation of the malformations with alcohol, follow-up color Doppler sonograms showed that the focal areas of flow, represented as mixing of lighter colors, disappeared or became smaller. This study shows that color Doppler sonography is a useful noninvasive procedure for diagnosing arteriovenous malformations of the kidney.     

肺隔离症的影像学诊断

作者报告经手术，病理和临床证实的肺隔离症２３例，其中２０例（８６．９％）术前影橡检查做出正确诊断。描述了本病的影像学诊断方法及其表现，讨论了各种影像方法在诊断本病中的作用。笔者认为，当患者有反复肺部感染病史，Ｘ线平片发现肺下叶后基底段囊状或肿块状阴影，经抗炎治疗长期不吸收，应怀疑本病。Ｂ超，ＣＴ或ＭＲＩ能显示其供血动脉，对诊断非常有帮助，主动脉造影对本病的确诊有特殊价值。最后，作者提出了本病的最佳     

Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology

The Mulliken and Glowacki classification (1982) differentiated vascular anomalies into two groups based on their endothelial characteristics: hemangiomas and vascular malformations. Vascular anomalies are localized defects of the vasculature that affect a limited number of vessels in a restricted area of the body. These defects are secondary to errors in vascular morphogenesis. Depending on the type of vessel involved, the vascular malformation group was subdivided into high-flow (such as arteriovenous malformation and arteriovenous fistula) and low-flow lesions (such as venous and lymphatic malformations). Depending on the type of lesion, the location and degree of involvement and the clinical effect, different types of treatment would be required. For the purpose of this review, we concentrate solely on vascular malformations: the clinical features, genetics, diagnosis, and current treatment options.     

Multidetector CT angiography in pulmonary sequestration

PURPOSE: To evaluate the role of multidetector CT angiography in the diagnosis and preoperative assessment of pulmonary sequestration with angiographic or surgical correlation. METHODS: Eight patients (5 males, 3 females) in the age range of 2 days to 35 years suspected of having pulmonary sequestration on the basis of clinical history and chest radiographs were included in the study. All patients underwent CT angiography (4 or 16 slice) and MPVR, MIP and 3D volume rendered images were generated. The axial images were also reviewed at soft tissue and lung window settings to evaluate the parenchymal changes. RESULTS: CT angiography showed five pulmonary sequestrations on the left and three on the right, located in the basal segments (n = 7) or paravertebral region (n = 1). Aberrant systemic arterial supply was demonstrated in all cases: from the descending thoracic aorta (n = 2); abdominal aorta (n = 3) and celiac axis (n = 3). Venous drainage into inferior pulmonary veins was demonstrated in 4 patients. The angioarchitecture depicted on CT angiography was confirmed on surgery in five patients and by DSA in two patients who subsequently underwent embolization using PVA particles and/or coils. CONCLUSION: The ability of CT angiography to simultaneously image the arterial supply, venous drainage and parenchymal changes in a single examination makes it the imaging modality of choice for the diagnosis and preoperative assessment of pulmonary sequestration.     

Bronchopulmonary and neurenteric forms of foregut anomalies. Imaging for diagnosis and management

Bronchopulmonary foregut malformations encompass a great variety of anomalies that may arise from abnormal differentiation of the respiratory and alimentary tracts, abnormal separation of the two systems, or abnormal development of blood supply, perhaps singly or in combination, during early embryogenesis. The pulmonary and neurenteric forms share the common features of a pulmonary parenchymal opacification and/or intrathoracic mass, with the addition of vertebral anomalies in the instance of the neurenteric malformations. Plain radiographs serve as the starting point for diagnostic evaluation and sometimes are all that is needed; more often, though, the plain film findings suggest which road to follow in further imaging. No single imaging approach can be advocated for all patients. The lesion may be found initially, for example, on prenatal sonography. Ultrasonography also can establish the cystic nature of a mass and may help define vascular supply, although it cannot yet be said to have supplanted angiography's role in evaluating suspected sequestrations. For most intramediastinal or intrapulmonary masses without associated vertebral anomalies, CT will satisfactorily establish the cystic nature and should afford a reasonably confident preoperative diagnosis of bronchogenic cysts or type 1 or 2 cystic adenomatoid malformations. Type 3 cystic adenomatoid malformation, because of its relatively "solid" imaging characteristics, may not be diagnosed confidently by preoperative imaging. The finding of vertebral anomalies associated with a mediastinal mass especially warrants MR imaging to define intraspinal involvement. However, the association of vertebral or rib anomalies with apparent unilateral pulmonary agenesis or hypoplasia arouses suspicion of a bronchopulmonary foregut malformation that communicates with the gastrointestinal tract, and this uncommon situation calls for a barium examination. Radionuclide scintigraphy has an ancillary role in assessing foregut anomalies, but many findings from scintigraphy are relatively nonspecific and anatomic definition is poor. Scintigraphy may yield supportive information in cases of sequestration that have inconclusive findings as determined by plain radiographs and CT.     

肺隔离症的多层螺旋CT诊断

目的：探讨肺隔离症的多层螺旋CT（MSCT）征象,以提高对该疾病的认识及临床诊断。方法回顾性分析经手术及病理证实的肺隔离症患者13例,包括临床表现,多层螺旋CT扫描资料,并复习文献;所有患者均行胸部平扫及双期增强容积扫描。结果13例肺隔离症患者中,12例为叶内型,1例为叶外型。12例叶内型均位于肺下叶内后基底段,9例位于左下肺,3例位于右下肺;1例叶外型位于左膈与下叶之间。13例中1例为增多增粗的异常血管结构,4例为实质性肿块,5例为囊实性肿块,3例为囊性肿块。6例病灶周围可见局限性肺气肿,4例病灶周围见渗出性阴影。13例隔离肺的供血动脉均发自体动脉。结论肺隔离症CT征象典型,容积扫描能对其作出准确诊断,并能为外科手术治疗提供重要参考,多层螺旋CT可以作为诊断肺隔离症的首选方法。     

Spiral CT findings in a case of pulmonary sequestration

Pulmonary sequestration has always been diagnosed by direct demonstration of the vessels tributary to the lesion, usually by angiography. Conventional CT can identify the arterial supply in only two thirds of cases. We report a case of pulmonary sequestration diagnosed using Spiral CT, based on the demonstration of both arterial supply and venous drainage. The capabilities of Spiral CT to detect subtle vessel abnormalities and to yield reliable multiplanar imaging enabled us to show the whole course of both the artery and the vein tributary to the lesion. Axial images were the most useful ones to diagnose pulmonary sequestration; 2D and 3D reconstructions were useful for a detailed and immediate spatial depiction of the parenchymal abnormality and of its vascular pedicle.     

Esophageal communication with a totally sequestrated hypoplastic right lung

Pulmonary sequestrations involving communication with the digestive tractus are known as bronchopulmonary foregut malformations. The authors add a peculiar case report: there was an hypoplastic right lung with a total sequestration from the bronchial tree and communicating with esophagus. This was fed from the pulmonary vasculature without collateral artery formation. The pulmonary vein was normal. Association with a facial dysmorphia and an occipital meningocele is also of interest. The radiological diagnosis is based on the pulmonary X-Ray and the oesophagogram.