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Pulmonary Sequestration

Last Updated: July 26, 2006
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Synonyms and related keywords: lung sequestration, bronchopulmonary sequestration, extrapulmonary sequestration, intrapulmonary sequestration, lung bud, sequestrum lung, broncho-pulmonary-foregut malformation, scimitar syndrome, horseshoe lung, cystic arteriovenous fistula, cystic arteriovenous malformation, extralobar sequestration, ELS, intralobar sequestration, ILS

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Author: Ali Nawaz Khan, MBBS, LRCP, FRCS, FRCP, FRCR, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia

Coauthor(s): Margaret Aird, MBChB, FRCR, Consulting Staff, Department of Radiology, Wythenshawe Hospital; Apam Chiphang, MBBS, Staff Physician, Department of Radiology, North Manchester General Hospital; David Iain Hodgson, MBChB, FRCS, Special Registrar, North West Regional Rotation, North Manchester General Hospital; Lalam Radhesh Krishna, MBBS, MRCS, Specialist Registrar, Department of Radiology, North Manchester General Hospital; Sumaira Macdonald, MBChB, MRCP, FRCR, PhD, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute; Tufail Ahmed Patankar, MBBS, DMRD, DMRE, FRCR, Consultant Neuroradiologist and Interventional Neuroradiologist, Department of Neuroradiology, Royal Preston Hospital; Nigel Thomas, MBBS, Vice-Chair, Manchester (North) Research Ethics Committee; Honorary Lecturer, Visiting Professor, University of Salford

Ali Nawaz Khan, MBBS, LRCP, FRCS, FRCP, FRCR, is a member of the following medical societies: American Institute of Ultrasound in Medicine, Radiological Society of North America, Royal College of Physicians, Royal College of Physicians and Surgeons of the USA, Royal College of Radiologists, and Royal College of Surgeons of England

Editor(s): S Bruce Greenberg, MD, Professor of Radiology, University of Arkansas for Medical Sciences; Consulting Staff, Department of Radiology, Arkansas Children's Hospital; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Marc S Keller, MD, Chairman, Professor, Departments of Radiology and Pediatrics, Department of Medical Imaging, Alfred I DuPont Hospital for Children; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; and Charles S White, MD, Vice Chairman for Clinical Affairs, Professor of Radiology, University of Maryland School of Medicine, Director of Thoracic Radiology, Department of Radiology, University of Maryland Medical Center

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Background: Pulmonary sequestration is an embryonic mass of lung tissue that has no identifiable bronchial communication and that receives its blood supply from one or more anomalous systemic arteries. Multiple feeding vessels may be present. This congenital anomaly can be classified as extralobar sequestration (ELS) or intralobar sequestration (ILS).

Many patients with ELS present in infancy with respiratory distress and chronic cough; some lesions are diagnosed coincidentally. Intrapulmonary sequestration is usually diagnosed later in childhood or adulthood when the patient presents with infection.

Pathophysiology: Bronchopulmonary sequestrations are classified as either extralobar or intralobar.

ELSs are masses composed of nonfunctioning primitive pulmonary parenchymal tissue that have no connection to the tracheobronchial tree. They consist of uniformly dilated bronchioles, alveolar ducts, and alveoli, which form the bulk of the lesion, although bronchial structures may be absent. The interstitium is composed of delicate connective tissue, which varies in thickness according to the patient's age.

This sequestration is called extralobar because the mass lies outside of the normal investment of visceral pleura; it also may lie outside of the thorax in a subdiaphragmatic position in as many as 10% of patients.

The arterial supply is predominantly via systemic arteries (95%) rather than pulmonary arteries (5%); the systemic arteries are commonly branches of the thoracic aorta or the abdominal aorta (80%). In rare cases, the supply may be from anomalous vessels arising from the splenic, gastric, subclavian, and intercostal vessels. Venous drainage also occurs most commonly via the systemic veins (75%), for example, the inferior vena cava (IVC) or azygos or portal veins rather than pulmonary veins (25%).

ELS is widely accepted to be congenital in origin. In normal embryologic development of the lung, the primitive bronchial tree develops as a ventral diverticulum of the foregut at 3 weeks' gestation, the bud then elongates and bifurcates into right and left lung buds at 26 days and then into lobes at 5-8 weeks' gestation.

The accepted theory concerning how an ELS arises is that an accessory lung bud develops from the ventral aspect of the primitive foregut. The accessory lung bud migrates caudally with the foregut and receives its blood supply from the splanchnic plexus, as does the foregut. If the bud arises after the pleurae have developed, it is not incorporated within the lung visceral pleura, and an ELS is formed. Caudal movement explains the lower lobe predomination and the presence of subdiaphragmatic ELS.

The theory that ELS is congenital is supported by the early age of presentation in infants and the association with other congenital abnormalities in as many as 65% of patients. The most common association is with diaphragmatic hernias (20%); others include congenital cystic adenomatoid malformation (CCAM), bronchogenic cysts, and foregut malformations. In addition to a foregut communication, associated anomalies are common and include diaphragmatic hernias, cardiovascular malformations, bronchogenic cysts, pectus excavatum, and other lung anomalies.

Similar to ELS, ILS is also a nonfunctioning area of pulmonary parenchyma and usually is not in communication with the tracheobronchial tree; however, it may contain air via the pores of Kohn or a connection to normal small bronchi. ILS is incorporated within the normal visceral pleura of the lung, unlike ELS. Also unlike ELS, an ILS, when discovered, usually contains dense fibrous parenchyma, which has replaced the normal pulmonary tissue as the result of chronic inflammation and fibrosis. Multiple cysts are present that contain viscid fluid or gelatinous material; the pleura is thickened by adhesions to mediastinal and diaphragmatic parietal pleura. Remnants of bronchi and bronchioles are replaced by fibrous connective tissue containing inflammatory infiltrates, as are alveolar ducts and alveoli.

The arterial supply is systemic in origin and arrives via the descending thoracic aorta (73%), the abdominal aorta or celiac axis artery (21%), and the intercostal arteries (4%). In 95% of patients, venous drainage occurs via the pulmonary veins; in 5% of patients, venous drainage occurs via the IVC, the superior vena cava (SVC), the azygos systems, or the intercostal veins.

The origin of ILS has been described in the past as congenital and is explained by the accessory lung bud theory. The accessory lung bud was believed to arise prior to development of lung visceral pleura, and thus, it was included within the pleura. The theory explains the systemic arterial supply but not the pulmonary venous drainage. In contrast to ELS, ILS is not commonly associated with other congenital anomalies. The lack of association with other congenital anomalies, coupled with the difference in patients' ages at presentation and the associated infective and fibrotic changes revealed on histologic analysis, has led to the theory that ILS may primarily be an acquired postinflammatory process.

The current widely held theory is that ILS is acquired after one or more episodes of necrotizing pneumonia result in obliterative bronchitis and obstruction of a lower lobe bronchus. This phase is followed by interruption of the pulmonary arterial supply to the infected lung parenchyma and hypertrophy of the systemic arterial supply from the thoracic aorta within the inferior pulmonary ligaments. The diaphragmatic pleural supply involves the celiac axis aorta and abdominal aorta, and these vessels also may be recruited. Venous drainage remains via the pulmonary veins.

Most ILSs are likely to be acquired; however, some ILSs may still be congenital in origin, since reports of neonatal ILS, bilateral ILS, and coexistent ILS/ELS exist. Other evidence that some ILSs may be congenital in origin is the association with other congenital anomalies in 6-12% of patients.

Several variants to the pulmonary sequestration spectrum are believed to exist, supporting a congenital etiology. These include scimitar syndrome, horseshoe lung, cystic adenomatoid lung, and pulmonary arteriovenous malformations.

Macroscopically, ELSs are usually single lesions sized 0.5-15 cm (most are 3-6 cm). ELSs are usually pyramidal or ovoid masses that are gray-white to pink and covered by smooth-to-fine wrinkled pleurae. In ELSs that communicate with the foregut, a thin-to-thick hollow stalk joins the ELS sequestration to the esophagus or, more rarely, to the stomach. Microscopically, ELSs have uniformly dilated bronchioles, alveolar ducts, and alveoli. A well-formed bronchus can be identified in approximately 50% of specimens.

Macroscopically, ILS lesions typically have thickened pleura covered with adhesions between adjacent structures. The cut surface of an ILS shows fibrous parenchyma or multiple cysts ranging from a few millimeters to larger than 5 cm in diameter. The cysts are typically filled with viscid yellow or white fluid. Microscopically, the pulmonary parenchyma is replaced by chronic inflammatory tissue.

Frequency:

  • In the US: Bronchopulmonary sequestration accounts for as many as 6.4% of all congenital pulmonary malformations and 1.1-1.8% of all pulmonary resections. ILS accounts for 75-86% of sequestrations, and ELS accounts for 14-25%.

    ELS is seen predominantly on the left side (90%), and it has been described in both the thorax and abdomen (as many as 10% of patients). The most common site is between the lower lobe and the diaphragm (63-77%), but lesions have been described in the upper and middle zones of the thorax.

    ILS is seen almost exclusively in the lower lobes (98%) and predominantly on the left side (60%); bilateral involvement is rare.

  • Internationally: International frequency of pulmonary sequestration is believed to the same as that in the United States.

Mortality/Morbidity:

  • ELS can be complicated by infection if bronchial and GI tract connections are present with associated morbidity. If resection is performed prior to infection, mortality and morbidity rates are exceedingly low and the prognosis is good.
  • Patients with ILS can present with massive spontaneous hemorrhage, which is potentially fatal but exceedingly rare. Other complications and causes of morbidity include chronic infection and fibrosis. Resection has low mortality and morbidity rates.

Race: No evidence has demonstrated any racial predilection.

Sex: In ELS, males are affected approximately 4 times more often than females. ILS shows no sex predilection.

Age: Most patients with ELS present when they are younger than 1 year, and 61% present when they are younger than 6 months. Some pulmonary sequestrations are detected in utero. In 10% of cases, patients are asymptomatic at the time of diagnosis.

ILS appears in older patients, with more than 50% occurring after adolescence. A first presentation rare in patients older than 50 years. Symptoms in neonates and infants are rare, and 15% of ILSs are asymptomatic at diagnosis.

Anatomy: In ELS, the systemic arterial supply is typically via the thoracic aorta or the abdominal aorta (>80%); however, the arterial supply can be via pulmonary (5%), subclavian, splenic, gastric, and intercostal (15%) arteries. Venous drainage is usually via the azygos or hemiazygous veins or the IVC (>80%), although the subclavian and portal veins are rarer options. In approximately 25% of patients, venous drainage is at least partially via the pulmonary veins.

In ILS, the systemic arterial supply is via the descending thoracic aorta (72%), abdominal aorta, celiac axis or splenic artery (21%), and intercostal artery (3.7%) and rarely via the subclavian, internal thoracic, and pericardiophrenic arteries. In approximately 16% of patients, more than one systemic artery is present. Most venous drainage (95%) is via the pulmonary veins.

Clinical Details:

Extralobar sequestration

Although children with ELS can present at any age, 60% present in the first 6 months of life. Many lesions are diagnosed coincidentally during imaging investigations for surgery or for associated congenital anomalies. Although the lung anomaly is usually not detected antenatally, maternal polyhydramnios, fetal ascites, and hydrothorax may indicate the diagnosis. On the first day of life, patients not uncommonly present with dyspnea, cyanosis, and feeding difficulties, although children can present at any age.

Feeding difficulties are usually related to a communication between the ELS and the GI tract. In addition, patients with ELS may present with recurrent chest infections, similar to patients with ILS. Symptoms can occur as a result of other associated anomalies, which are present in 40-60% of patients and range from the relatively innocuous accessory spleen to severe cardiovascular malformations, including truncus arteriosus and total anomalous pulmonary drainage.

In addition, reports describe myocardial ischemia in the left coronary artery caused by vasospastic angina and coronary stealing from the coronary circulation by an anomalous vessel arising from the anterior arterial branch from the left circumflex artery. Diaphragmatic hernias with concomitant pulmonary hypoplasia affect approximately 20% of patients. Pulmonary sequestration associated with bronchopleural fistulae, malrotation of the intestines, and a Meckel diverticulum has been reported in the same patient.

Intralobar sequestration

Patients with ILS presented significantly more often with an infection than patients with ELS (91% vs 14%, respectively). Adult patients had significantly more respiratory infections than pediatric patients (67% vs 31%); as a result, greater numbers of lobectomies were performed in adults (Al-Bassam, 1999).

Symptoms may occur from associated anomalies in approximately 11% of patients with ILS compared with 60% of patients with ELS, 40% of patients with congenital lobar emphysema, and 25% of patients with CCAM. The most common anomalies associated with ILS are esophagobronchial fistulae and diverticula, implying the presence of a bronchopulmonary foregut malformation. Anomalies of the chest wall are not uncommon but may be acquired as a result of chronic lung infection. Physical examination may reveal signs of pulmonary consolidation. Rarely, auscultation may identify a bruit or continuous murmur over the sequestered lung segment from a large systemic blood supply. Murmur in the sequestered lung segment may occur in either ILS or ELS.

Preferred Examination:

  • Chest radiographs can provide a reasonable diagnostic clue to pulmonary sequestration. A mass in the posterobasal segment of the lung in young patients with recurrent localized pulmonary infections is suggestive of ILS. When such a lesion resolves incompletely with appropriate medical treatment, an underlying sequestration should be considered.

  • Bronchography (CT or radiographic) may be helpful in excluding other diagnoses.

  • CT scans have 90% accuracy in the diagnosis of pulmonary sequestration.

  • Arteriography (conventional or CT angiography [CTA]) is helpful in differentiating the lesion from other abnormalities of the lung, such as pulmonary arteriovenous fistulae, but the CT scans should be correlated with clinical presentation and chest radiographs.

  • MRI and magnetic resonance angiography (MRA) can provide information similar to that on CT scans.

  • Ultrasonography is noninvasive and safe, making its use ideal in prenatal and postnatal settings. Diagnosis can be achieved as early as the second trimester. Color flow and duplex Doppler ultrasound can elegantly depict the ectopic blood supply and drainage.

  • Radionuclide angiography is another noninvasive technique that may demonstrate the systemic arterial blood supply to the sequestration, thus establishing the diagnosis.

Limitations of Techniques:

  • Use of conventional radiographs must be avoided in women who are pregnant. Distinguishing ELS from ILS by using plain radiographs is difficult, and differentiation is important in selecting the mode of treatment. Infradiaphragmatic ELS is difficult to detect on plain radiographs.

  • Bronchography is invasive, and its findings are nonspecific.

  • Diagnosis with arteriography is based on the demonstration of systemic arterial blood supply to the lung sequestration, but arteriography is invasive, and its findings are nonspecific. The same findings have been described in other lung anomalies and in healthy lung segments.

  • Use of CT scanners requires a high radiation dose; therefore, performing CT in pregnant women and in infants should be avoided if possible. Infants may require sedation or general anesthesia.

  • MRI has limited availability, it is an expensive tool, and it may require the use of heavy sedation or general anesthesia in young patients.

  • Ultrasonographic findings are nonspecific in the antenatal and neonatal periods, and the differential diagnosis is wide in these findings. Ultrasonography has limited value in adults with both ILS and ELS.

  • The experience with nuclear medicine techniques is limited and mostly anecdotal, and the technique requires the use of ionizing radiation.
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Bronchiectasis
Bronchogenic Cyst
Emphysema
Empyema
Lung, Arteriovenous Malformation
Lung, Carcinoid
Lung, Metastases
Neuroblastoma
Pneumonia, Atypical Bacterial
Pneumonia, Neonatal
Pneumonia, Typical Bacterial
Solitary Pulmonary Nodule


Other Problems to be Considered:

Scimitar syndrome is a congenital anomaly in which an anomalous pulmonary vein drains into the IVC or its junction at the right atrium. An association exists with hypoplasia of the right lung, hypoplasia of the right pulmonary artery, and an anomalous systemic vascular supply to the lung.

CCAM is a lung parenchymal abnormality resulting from overgrowth of bronchioles. The blood supply to the abnormal lung segment is normal, but, rarely, an aberrant systemic blood supply may be present.

Horseshoe lung shares some of the characteristics of scimitar syndrome. The abnormality consists of an isthmus of lung tissue that joins the lung bases across the midline behind the pericardium. The blood supply to the horseshoe lung may be anomalous.

Pulmonary arteriovenous fistula/malformation consists of an abnormal pulmonary artery and venous connection but normal underlying lung parenchyma.

Solid mass in the differential diagnosis for ILS

Bronchogenic carcinoma
Bronchial adenoma
Metastatic lung neoplasms
Lung abscess
Empyema
Bochdalek hernia
Neurogenic tumor
Meningocele
Pleural tumor
Extramedullary hematopoiesis

Cystic mass in the differential diagnosis for ILS

Bronchial atresia
Congenital lobar emphysema
Bronchiectasis
Cystic adenomatoid malformation
Intrapulmonary bronchogenic cyst
Arteriovenous malformation
Lung abscess
Necrotizing pneumonia
Cavitating neoplasm
Cavitating infarct
Fungal pneumonia
Tuberculous pneumonia
Bronchogenic foregut cyst
Diaphragmatic eventration
Pericardial cyst
Empyema
Emphysema

Extralobar sequestration

Neuroblastoma
Teratoma
Foregut duplication
Mesoblastic nephroma
Adrenal hemorrhage

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Findings:

Degree of Confidence: Chest radiographic findings are usually distinctly abnormal in most patients, and these can provide reasonable diagnostic clues of pulmonary sequestration. An indolent process in the posterobasal segment of the lower lobe in a young person with recurrent localized pulmonary infections is suggestive of ILS.

Distinguishing ELS from ILS is difficult by using plain radiographic findings. Extralobar lesions are solid more often and are associated with elevation of the ipsilateral diaphragm. On the contrary, intralobar lesions appear more cystlike, and air is present if a pulmonary communication exists. The opacity of the sequestration increases with infection. When such a lesion resolves incompletely with appropriate medical treatment, an underlying sequestration should be considered.

Lack of filling or lack of demonstration of a communication on bronchography images of the tracheobronchial tree through a normally located bronchus is a characteristic finding that can help to make the diagnosis with reasonable certainty in conjunction with other clinical and radiologic findings. Bronchography or CT may be helpful in excluding other diagnoses. The occasional presence of contrast material in the cystic area during bronchography may suggest the confusing diagnosis of cystic bronchiectasis.

False Positives/Negatives: Pulmonary sequestration in asymptomatic individuals may be confused with a bronchogenic cyst, CCAM, Bochdalek hernia, and mediastinal or pulmonary neoplasm. In symptomatic individuals, the differential diagnosis includes pneumatoceles, pneumonia, bronchiectasis, and lung abscess. Lesions considered in the differential diagnosis for infradiaphragmatic lesions include neuroblastoma, teratoma, adrenal hemorrhage, and mesoblastic nephroma and foregut duplication.

The occasional presence of contrast material in the cystic area during bronchography may suggest the confusing diagnosis of cystic bronchiectasis. Bronchographic findings may be misleading because the failure of the contrast agent to enter the bronchus is not pathognomonic of a sequestrated lung segment and may occur as a result of a foreign body, mucous plug, or bronchial atresia.

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Bronchiectasis

Bronchogenic Cyst

Emphysema

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Lung, Arteriovenous Malformation

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Lung, Metastases

Neuroblastoma

Pneumonia, Atypical Bacterial

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Pneumonia, Typical Bacterial

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  CT SCAN Section 5 of 12   Click here to go to the previous section in this topic Click here to go to the top of this page Click here to go to the next section in this topic
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Findings: The role of CT is to define vascular anatomy and to provide supporting evidence that opacities depicted on chest radiographs or antenatal sonograms may be sequestrations.

Simple sectional CT studies can reveal the anatomic position of an abnormality and may contribute to knowledge of vascular supply in only two thirds of patients. With the advent of volumetric slip-ring scanning (either spiral or multisection), the vascular supply and venous drainage of both ILS and ELS lesions can be defined with a much higher degree of certainty. Volumetric slip-ring CT scans can provide information regarding the morphologic structure and attenuation values of any focus. Powerful computing with 3-dimensional (3D) reconstruction provides excellent spatial resolution and definition of the spatial relationships of structures, which can obviate invasive angiographic procedures.

Sequestration may be either ILS (75%) or ELS (25%).

  • In ELS, 80% of sequestrations lie between the lower lobe and the diaphragm. Lesions are usually located in the region of the posterior basal segments of the lower lobes. Left-sided lesions are more common than right-sided lesions. The mass may be closely associated with the esophagus, and fistulae may develop.

  • In ILS, sequestrations occur within pulmonary visceral pleurae and do not communicate with the bronchial tree. ILS is seen in males and females in equal numbers. Lesions of ILS may be solid, fluid, hemorrhagic, or mucous containing. Cystic or emphysematous elements may be present, and adjacent atelectasis often exists. Most lesions appear hypervascular because of an abundant systemic vascularization. Super-added infection may lead to some consolidation in adjacent segments, and a chronic inflammatory process may induce localized reactive neovascularization. Mucoid impaction of a bronchus surrounded by hyperinflated lung is believed to be characteristic of ILS.

The position of the lesion and its persistence in a relatively young individual raises the index of suspicion that the underlying pathology may be the result of a sequestered segment. Demonstration of a dominant feeding vessel, usually from the aorta or its major vessels, and venous drainage to the pulmonary veins suggests the diagnosis. Alternative venous drainage patterns in ILS include a route directly into the left atrium via the azygos or hemiazygos systems, into intercostals veins, or into the IVC or SVC.

The finding of alternative venous drainage patterns separates pulmonary sequestration from other diagnoses, such as infection and tumor, round atelectasis, Bochdalek hernia, and pulmonary infarction. Enlargement of the associated abnormal feeding vessels is a constant feature, and the azygos vein also is frequently enlarged. Multiple supply arteries are found in 15% of sequestrations; 73% of sequestrations develop blood vessels leading off the abdominal aorta, and 18% develop blood vessels leading off the thoracic aorta. Rare documented origins include the ascending aorta and the arch, subclavian, innominate, celiac, right coronary, and circumflex arteries.

Lesions of ELS may occur above or below the diaphragm, sometimes in the retroperitoneum. In ELS, 8% of lesions are subdiaphragmatic and can mimic masses arising in various organs, such as the adrenal gland. Most ELS (80%) occurs in males. Venous drainage occurs via the systemic circulation. ELS frequently is associated with other congenital extrapulmonary anomalies.

CT technique for optimal depiction of lesions by using state-of-the-art volumetric scanning requires a fast intravenous (IV) contrast injection rate and appropriate volume and delay based upon size. Multiplanar and 3D MIP reconstructions are helpful.

Degree of Confidence: Accuracy of diagnosis of a pulmonary sequestration is approximately 90% if a lesion is depicted in a typical site, if it has solid and cystic components, and if it is associated with emphysema and an abnormal blood supply and venous drainage into either the pulmonary veins or the systemic veins.

False Positives/Negatives: Lung abscess, congenital adenomatoid cystic malformation, lung tumor, round atelectasis, Bochdalek hernia, and pulmonary infarction may mimic pulmonary sequestration. If an aberrant blood supply and drainage cannot be demonstrated, a false-negative examination may occur.
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Findings: Contrast-enhanced MRA or even conventional T1-weighted spin-echo (SE) images may help in the diagnosis of pulmonary sequestration by demonstrating a systemic blood supply, particularly from the aorta, to a basal lung mass. In addition, MRA may demonstrate venous drainage of the mass and may obviate more invasive investigations.

Degree of Confidence: MRI and MRA can provide information similar to that of CT without the need for ionizing radiation; however, MRI is less accessible, takes longer to perform, is subject to motion artifacts, and requires sedation in infants and small children.

False Positives/Negatives: Sufficient experience has not been accumulated in the use of MRI in the diagnosis of pulmonary sequestration. Demonstration of aberrant blood supply to the sequestrated segment is pivotal to the diagnosis; therefore, meticulous technique is necessary because respiratory and cardiac motion may theoretically degrade the images. Moreover, a systemic artery supplying lung tissue is not pathognomonic of sequestration, since anomalous systemic arterial supply to normal segments of the lung is a rare but well-recognized congenital anomaly.

As in any imaging technique, MRI findings must be interpreted in the light of the clinical presentation and the ultrasonographic and chest radiographic findings.
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Findings: Lesions of ILS appear as solid intrathoracic masses that may contain small cystic areas secondary to multiple fluid-filled bronchi. The left lower lobe is the most common site. Appearances are nonspecific and can be complex solid homogeneous or inhomogeneous lesions and echogenic or cystic, depending on the histologic components in the lesion. These findings are suggestive of a number of possibilities in the pulmonary sequestration spectrum.

Demonstration of a systemic arterial supply and left atrial venous drainage by using color flow and duplex sonography establishes the diagnosis. Sonographic demonstration of a vascular supply may be difficult, and the failure to depict the supply does not exclude the diagnosis. The arterial supply is most commonly derived from the descending aorta, but it can arise from the celiac, splenic, intercostal, subclavian, internal thoracic, or pericardiophrenic arteries, in descending order of frequency. In ILS, 16% of lesions can have multiple blood supplies.

Prenatal diagnosis of retroperitoneal ELS is not rare, accounting for 2-5% of all lung sequestrations.

Analysis of ultrasound-guided fine needle biopsy specimens of respiratory epithelium confirms the diagnosis of extrapulmonary ELS, but most of the time, surgical resection follows imaging evaluation.

Ultrasonography is useful in prenatal diagnosis of pulmonary sequestration and its complications, in assessing progression, and in forming a prognosis, which in turn is important for appropriate parental counseling and fetal therapy. Serial prenatal sonograms are necessary in patients in whom pulmonary sequestration is suspected to search for poor prognostic factors such as increasing mediastinal shift and increasing size of the sequestration. In fetuses with chest masses, 8% have additional structural abnormalities and an abnormal karyotype.

In ELS, 65% of patients have associated anomalies, such as an accessory spleen, congenital heart disease, or a diaphragmatic hernia. In patients with ELS, complications can include tension hydrothorax, polyhydramnios, and hydrops fetalis. Ultrasonography can demonstrate absent or reversed diastolic flow in a torsed vascular pedicle, which is believed to cause complications in patients with ELS. In patients with ILS, prenatal complications are unlikely because the sequestrated segment is well anchored in the thorax and unable to undergo torsion.

Degree of Confidence: Ultrasonography is important in the diagnosis of pulmonary sequestration. Ultrasonography is noninvasive and safe; these features make its use ideal in the prenatal and postnatal periods. The basal location of most of these lesions provides an excellent acoustic window for sonography. Diagnosis can be made as soon as the early second trimester. Demonstration of a systemic arterial supply and pulmonary venous drainage by using color flow and duplex sonography establishes the diagnosis.

False Positives/Negatives: Mimics of ILS include congenital diaphragmatic hernia, CCAM, tracheobronchial atresia, cystic mediastinal teratoma, and bronchogenic and enteric cysts. Absence of peristalsis and presence of an intact diaphragm excludes diaphragmatic hernia. If CCAM is microcytic type 3, it can be sonographically indistinguishable from pulmonary sequestration. If bronchial communication occurs after infection in patients with ILS, highly echogenic reverberation artifacts caused by air may be seen.

In the retroperitoneal location, mimics of ELS include neuroblastoma, adrenal hemorrhage, teratoma, and lymphangioma. One ELS is diagnosed for every 2.5 neuroblastomas. A neuroblastoma is characterized by poorly defined margins and low or mixed echogenicity with foci of calcification. Neuroblastoma is more often cystic, right sided, and seen in the third trimester; ELS is more often echogenic, left sided, and possibly seen as early as the second trimester. Adrenal hemorrhage typically has cystic components and involves the adrenal gland, either wholly or in part. Differentiating adrenal hemorrhage from pulmonary sequestration may be a function of time rather than initial appearances. Teratomas and lymphangiomas occur considerably less frequently.

On antenatal and neonatal sonograms, an extralobar pulmonary sequestration may mimic a neuroblastoma. The differential diagnosis of an infradiaphragmatic extralobar sequestration includes neuroblastoma, teratoma, adrenal hemorrhage, and mesoblastic nephroma and foregut duplication cysts.
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Findings: A single case of ILS has been described in which xenon-131 study was used to further the diagnosis. In a 29-year-old male patient, dynamic single-photon emission computed tomography (SPECT) with Xe-133 gas showed the retention of the radionuclide within a hyperlucent lung mass that was demonstrated on CT scans. Left lower lobectomy subsequently was performed and showed no fistulous communication between an anomalous and the normal bronchial trees, but noncontiguous incompletely developed visceral pleura was demonstrated between the sequestered segment and the adjacent normally ventilated lung. Thus, retention of the radionuclide supports the role of intralobar collateral air drift and airtrapping in producing secondary changes of a focal hyperlucent lung area within the lung segment in ILS.

Radionuclide angiography has been used to delineate the systemic blood supply to a sequestered lung segment. In a series of 5 patients, radionuclide angiograms obtained with technetium-99m macroaggregated albumin and 99mTc pertechnetate were obtained. Perfusion lung scans showed segmental perfusion defects in the lower lobes at the site of the sequestered lung segments. Radionuclide angiography revealed abnormal systemic blood flow through the descending aorta to the left lower lobe. Subtraction scans were obtained from the 2 images in different phases in the aortic phase on the radionuclide angiograms; these images revealed abnormal systemic blood flow through the descending aorta more clearly than images from conventional radionuclide angiography.

Fourier-phase analysis of first-pass data was used to evaluate blood flow to cystic lung masses in 2 children in whom lung sequestration was suspected. The analysis did not depend on the location of the mass and demonstrates the location of a systemic rather than a pulmonary arterial blood supply to the mass.

Degree of Confidence: Ventilatory abnormalities in ILS and the adjacent lung have recently been suggested by the results of CT attenuation analysis with ultrafast electron-beam CT in a patient with pathologically proven emphysematous changes. However, it has also been shown that regional lung attenuation analysis, as assessed using CT scans, reflects not only aeration but also coincidental hemodynamic change. Dynamic Xe-133 SPECT is more accurate and sensitive for detection of regional ventilatory abnormalities associated with airtrapping and for estimation of regional Xe-133 clearance.

Radionuclide angiography is noninvasive and can reveal a systemic arterial system, rather than pulmonary circulation, as the source of supply to the lung sequestration.

Fourier-phase analysis of first-pass data also allows noninvasive detection of pulmonary sequestration, although experience with this is limited.

False Positives/Negatives: Scimitar syndrome may be indistinguishable from pulmonary sequestration on radionuclide angiography.
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Findings: The blood supply of 75% of pulmonary sequestrations is derived from the thoracic or abdominal aorta. The remaining 25% of sequestrations receive their blood flow from the subclavian, intercostal, pulmonary, pericardiophrenic, innominate, internal mammary, celiac, splenic, or renal arteries. The arterial supply typically enters the lung via the pulmonary ligament if the artery originates above the diaphragm. Arteries originating below the diaphragm reach the sequestration by piercing the diaphragm or via the aortic or esophageal hiatus. In the rare instance of sequestration in an upper lobe, arterial supply from the internal thoracic artery has been reported. If aortography is unrevealing, a coronary source should be included in the preoperative search.

The arterial supply usually is composed of a single vessel that is disproportionately large. This vessel is typically 0.5-2.0 cm in diameter, and multiple arteries are present in 15-20% of cases in which the arteries are 3 mm or smaller in diameter. Venous drainage occurs most often via the pulmonary vein in ILS, establishing a left-to-right shunt, and the drainage occurs via bronchial or other systemic veins in ELS. Occasionally, drainage is solely to the azygos or hemiazygos system. In rare cases, drainage is to the intercostal, innominate, or portal veins. Dual venous drainage to both pulmonary and systemic veins is the most uncommon situation.

Degree of Confidence: The definitive diagnosis is made by using angiography (conventional, CT, or MR angiography) that delineates the feeding vessel to the sequestration along with its venous system. Aortograms and pulmonary angiograms may be needed in some patients in whom pulmonary sequestration is suspected.

False Positives/Negatives: Arteriography is helpful in differentiating pulmonary sequestration from other abnormalities of the lung, such as pulmonary arteriovenous fistulae. However, demonstration of a systemic artery supplying lung tissue is not pathognomonic of sequestration, since a congenital anomalous systemic arterial supply to normal segments of the lung is rare but well known. Arteriography must be interpreted along with clinical and chest radiographic findings.
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Intervention: Transarterial embolization of the aberrant arterial supply to an ILS may be used as a definitive procedure or as a preoperative procedure to minimize the risk of vascular complications during resection. In one series of 16 children with pulmonary sequestrations treated with endovascular embolization of the feeding systemic artery, embolization alone cured the sequestration in 10 children. Some patients may have significant arteriovenous shunting through the lesion, which can benefit from a transarterial coil-spring or particulate embolization.

Laparoscopic approaches have been used in the resection of an abdominal ELS. Advances in imaging have made the distinction of sequestrations from other suprarenal masses, including neuroblastomas, possible in most cases. However, resection provides absolute tissue diagnosis and remains the treatment of choice. Laparoscopic resection offers the benefit of minimally invasive therapy in addition to providing tissue for confirmation. If resection is performed prior to infection, the mortality and morbidity rates are exceedingly low, and the prognosis is good.

Fetal hydrops is a common complication in patients with ELS and is associated with a high perinatal mortality rate and severe respiratory problems at birth. In a 27-week-old fetus with this condition, an injection of 1 mL of pure alcohol and pleuroamniotic shunting achieved resolution of the hydrops. The pregnancy reached maturation and a full-term healthy neonate was delivered who did not require postnatal intervention.

Medical/Legal Pitfalls:

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Caption: Picture 1. Intralobar pulmonary sequestration. Midtrimester fetal sonogram shows a triangular echogenic mass astride the left diaphragm. Color Doppler sonogram (not shown) showed an ectopic blood supply to the mass arising from the infradiaphragmatic aorta.
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Caption: Picture 2. Intralobar pulmonary sequestration. Midtrimester fetal sonogram shows a complex mass with a cystic component astride the left diaphragm. Color Doppler sonogram (not shown) failed to demonstrate an ectopic blood supply to the mass. At thoracotomy after birth, an intralobar sequestration was confirmed. Note that identical appearances may occur with congenital cystic adenomatoid malformation.
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Caption: Picture 3. Intralobar pulmonary sequestration. Chest radiograph in an 8-year-old patient who presented with signs of an acute chest infection showed a left lower lobe consolidation (not shown). After appropriate medical treatment, the child improved clinically, but opacity in the left lower lobe persisted. A sequestrated lung segment was suspected because of a history of several previous respiratory infections from age 3 years and up. Aortogram shows contrast material injected within the upper abdominal aorta. An anomalous artery is arising from the infradiaphragmatic portion of the aorta and is supplying a supradiaphragmatic mass in the left lower lobe (arrow).
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Caption: Picture 4. Intralobar pulmonary sequestration. An 8-year-old patient presented with signs of an acute lung infection. The venous phase of aortogram shows pulmonary venous drainage into the left atrium (arrow) (same patient as in Image 3).
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Caption: Picture 5. Intralobar pulmonary sequestration. Radiographic subtraction of Image 4 shows pulmonary venous drainage.
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Caption: Picture 6. Extralobar pulmonary sequestration. A solid mass in the posterior mediastinum in a 55-year-old patient who smokes. A bronchogenic neoplasm was suspected.
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Caption: Picture 7. Extralobar pulmonary sequestration. CT scan in a 55-year-old patient who smokes shows a nonspecific mass in the posterior mediastinum (arrow). At thoracotomy, the mass was seen to be attached to the paravertebral region by a feeding artery originating from the descending thoracic aorta. Histologic examination confirmed an extralobar sequestration.
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Caption: Picture 8. Supine chest radiograph in an infant shows a large opacity at the left costophrenic angle, which can be followed upward to below the diaphragm (see also Images 9-11).
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Caption: Picture 9. Color Doppler sonogram on the same patient as in Picture 8 shows an infradiaphragmatic artery arising from the aorta and a vein draining back into the hemiazygos vein.
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Caption: Picture 10. Contrast-enhanced portal venous phase transaxial CT scan through the liver and base of the left lung shows a large mass of mixed attenuation with arterial supply from the aorta and enlarged hemiazygos vein from venous return (same patient as in Images 8-9, and 11).
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Caption: Picture 11. Contrast-enhanced portal venous phase transaxial CT scan through the liver and base of the left lung shows a large mass of mixed attenuation with arterial supply from the aorta and enlarged hemiazygos vein from venous return (same patient as in Images 8-10). At surgery, an extralobar sequestration was confirmed.
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Pulmonary Sequestration excerpt