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AJR 2004; 183:183-188
© American Roentgen Ray Society


Original Report

Evaluation of Angioarchitecture of Pulmonary Sequestration in Pediatric Patients Using 3D MDCT Angiography

Edward Y. Lee1, Marilyn J. Siegel1, Lina M. Sierra1 and Robert P. Foglia2

1 Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S Kingshighway Blvd., St. Louis, MO 63110.
2 Department of Pediatric Surgery, St. Louis Children's Hospital, Washington University School of Medicine, One Children's Pl., St. Louis, MO 63110.

Received November 20, 2003; accepted after revision January 12, 2004.

 
Address correspondence to M. J. Siegel (siegelm{at}mir.wustl.edu).


Abstract
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
OBJECTIVE. The goal of this study was to show the ability of 3D MDCT angiography to display the arterial and venous vascular anatomy of pulmonary sequestration in children.

CONCLUSION. MDCT angiography with 3D rendering shows the anomalous feeding artery and the draining veins that allow a diagnosis of pulmonary sequestration. These features may prove useful in distinguishing intra- and extralobar sequestration and in surgical planning.


Introduction
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Pulmonary sequestration is a congenital malformation defined by dysplastic and nonfunctioning pulmonary tissue lacking a normal connection with the tracheobronchial tree and the pulmonary arteries [13]. In both the intra- and extralobar forms, the arterial supply is usually from the descending aorta, and in approximately 20% of cases, it may originate from the infradiaphragmatic aorta. In addition, there are reports of an arterial supply from other systemic arteries and even from the coronary arteries [46]. The venous drainage of the two types of sequestration differs. In the intralobar type, venous drainage is typically into the inferior pulmonary vein. In extralobar sequestration, venous drainage is usually via the azygous vein, although unusual drainage routes, such as drainage into the portal vein, have been reported [7].

Helical CT has proven to be of considerable value in the diagnosis of arterial feeders and parenchymal changes in sequestration [812]. The 3D capability of MDCT is particularly valuable in depicting the relationship of the aberrant artery to adjacent structures [9]. Thus, the definite diagnosis of pulmonary sequestration has become possible with CT angiography, obviating more invasive angiography.

The treatment of choice for sequestration is surgical excision. Because the vascular communications are variable, imaging studies are integral to the evaluation of patients with sequestration. Although it has been suggested that identification of venous drainage is not of primary importance for treatment planning and that only information related to the presence and location of the aberrant artery is essential [9], in our experience, knowledge of venous drainage can be helpful in planning the surgical procedure and in communicating with the child's parents.

Helical CT usually shows the anomalous arteries associated with pulmonary sequestration. Although the use of 3D MDCT imaging to show venous drainage associated with sequestration has been described in adults [8], to our knowledge, its usefulness in defining the venous anatomy in the pediatric population has not been established. The purpose of this study was to show the ability of 3D MDCT angiography to reveal the venous and the arterial angioarchitecture of pulmonary sequestration. This information may be helpful in distinguishing intra- and extralobar sequestrations and in surgical planning.


Subjects and Methods
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Patient Population
Between April 2002 and May 2003, five pediatric patients (three girls and two boys) with the diagnosis of sequestration were identified and constituted the study population. They ranged in age from 6 days to 9 years (mean age, 2 years). The diagnosis was suspected in two patients because of recurrent episodes of pneumonia. Three patients were asymptomatic, and the diagnosis was suspected on the basis of prenatal sonography (n = 1) or chest radiography (n = 2), which showed a left lower lobe mass. The local institutional review board approved the review of the CT studies.

Four pediatric patients, who ranged in age from 6 days to 6 months, were sedated with orally administered chloral hydrate (50–100 mg per kilogram of body weight; maximum dose, 2,000 mg). One 9-year-old patient cooperated for the CT examination without sedation.

Imaging Technique
CT was performed on either a Somatom Plus-4 scanner or Sensation 16 scanner (Siemens Medical Solutions). All patients received nonionic IV contrast material (320 mg I/mL) at a dose of 2 mL per kilogram of body weight. Scan initiation was 12–15 sec after the start of the IV contrast injection in the four younger patients. An automated tracking system with a density of 100 H in the ascending aorta was used to initiate scanning in the older patient.

On the 4-MDCT scanner, we used a collimator width of 2.5 mm and a table speed of 15–20 mm per rotation. On the 16-MDCT scanner, we used a collimator width of 1.5 mm and a table speed of 36 mm per rotation. All examinations were performed with low–radiation dose techniques. Studies were performed at 30 mAs in the four younger patients and at 50 mAs in the older patient. An 80-kV dose was used for all five patients.

Scanning extended from just below the level of the thoracic inlet to just above the level of the renal arteries. CT scans were acquired during quiet respiration in sedated patients and during a single breath-hold in the 9-year-old patient. All images were processed with standard soft-tissue settings (e.g., 400–450 H width; 40–50 H level) and lung window settings (e.g., –160 to –1,800 width; –450 to –550 H level).

Imaging Review
Three-dimensional volume-rendered and maximum-intensity-projection MR images were obtained using a freestanding workstation. All reconstructions were performed by a radiologist experienced in 3D postprocessing techniques, and reconstruction time for each patient was approximately 30 min. The volumes of interest were selected from the axial source to include the aorta and its branches, the pulmonary arteries, the pulmonary veins, and the superior vena cava. Images were reconstructed at a 3-mm slice thickness on the 4-MDCT scanner and at a 2-mm slice thickness on the 16-MDCT scanner. Patient identifying information was removed before review by two observers.

The axial images and the 3D reconstructions were reviewed by two radiologists, and the arterial supply and venous drainage to and from the sequestration were recorded. The axial images were interpreted first. The order of image review was randomized for the 3D interpretation to decrease recall of patient diagnosis. The observers reviewed the reconstructed 3D images in real time and thus could optimize depiction of the course of the vessels.


Results
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Surgical and Pathologic Findings
Surgical and pathologic findings were available for four patients with pulmonary sequestration. In all four patients, the sequestered lungs were located in the left lower hemithorax. There were three intralobar sequestrations and one extralobar sequestration. In all intralobar sequestrations, the anomalous arterial supply was from the thoracic aorta, and the venous drainage was to the left atrium. The sequestered lungs were contained in the pleural investment of the normal lung. On gross examination, the sequestered lungs appeared solid. Microscopically, the pulmonary parenchyma in one patient was a deep reddish brown color and permeated by thick-walled vessels without evidence of inflammation. Two intralobar sequestrations showed changes of consolidation and organizing pneumonia. Additional findings in the adjacent lung in one patient included chronic interstitial pneumonia and pulmonary edema. No intralobar sequestration showed features of cystic adenomatoid malformation on microscopy.

In the one patient with extralobar sequestration, the arterial supply was from the celiac artery. Venous drainage was into a congenital cystic adenomatoid malformation in the right lower lobe. On gross examination, the sequestered lung was contained in its own pleura. On microscopy, sections from the sequestration in the left lung showed changes of mild chronic interstitial inflammation and histologic features of type II cystic adenomatoid malformation. Sections from the right lung showed a congenital cystic adenomatoid type II malformation. The microscopic findings included numerous columnar epithelial-lined cysts (< 2.0 cm). No tissue continuity was found between the left lower lobe sequestration and the right lower lobe cystic adenomatoid malformation. The one patient without surgical confirmation most likely had an extralobar sequestration because the venous drainage was into the portal vein. Arterial supply was from the thoracic aorta.

MDCT Findings
The sequestered lungs appeared as enhancing solid masses in two of the three intralobar sequestrations and in the two extralobar sequestrations on soft-tissue and lung window settings. In one patient with an infected intralobar sequestration, CT showed cystic changes in the sequestration and enhancement of surrounding lung parenchyma.

Table 1 summarizes the ability of axial and 3D MDCT images to depict the vascular anatomy of sequestration in pediatric patients. Axial images and 3D reconstructions allowed identification of the arterial supply to the lesion and its course in all patients (Figs. 1A, 1B, 1C and 2A, 2B, 2C, 2D). In one patient, the arterial supply was more obvious on 3D reconstructions (Fig. 3A, 3B, 3C, 3D). The arteries had a transverse or slightly oblique course from the aorta to the sequestration.


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TABLE 1 Ability of Axial and 3D MDCT Images to Display Vascular Anatomy of Sequestration in Pediatric Patients

 


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Fig. 1A. 6-month-old girl who presented with recurrent left lower lobe pneumonia. At surgery, patient was found to have intralobar sequestration with anomalous arterial vessel arising from thoracic aorta and anomalous venous drainage into left atrium. Axial CT image shows anomalous arterial supply (arrow) to sequestered lung (S), arising from thoracic aorta (A).

 


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Fig. 1B. 6-month-old girl who presented with recurrent left lower lobe pneumonia. At surgery, patient was found to have intralobar sequestration with anomalous arterial vessel arising from thoracic aorta and anomalous venous drainage into left atrium. Axial CT image obtained at level higher than A shows that vein cannot be clearly separated from surrounding inflammation.

 


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Fig. 1C. 6-month-old girl who presented with recurrent left lower lobe pneumonia. At surgery, patient was found to have intralobar sequestration with anomalous arterial vessel arising from thoracic aorta and anomalous venous drainage into left atrium. Posterior 3D volume-rendered image shows anomalous arterial supply (arrow) from descending aorta to sequestered lung (S) and anomalous venous drainage (arrowhead) to left atrium.

 


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Fig. 2A. 6-month-old boy with abnormal findings on chest radiograph. At surgery, patient was found to have extralobar sequestration with anomalous arterial vessel arising from celiac artery and anomalous venous drainage into right atrium. Axial CT image shows anomalous arterial vessel (arrowhead) arising from celiac artery. A = abdominal aorta.

 


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Fig. 2B. 6-month-old boy with abnormal findings on chest radiograph. At surgery, patient was found to have extralobar sequestration with anomalous arterial vessel arising from celiac artery and anomalous venous drainage into right atrium. Axial CT image shows anomalous vein (arrowheads) arising from sequestered lung (S) and crossing midline to right hemithorax.

 


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Fig. 2C. 6-month-old boy with abnormal findings on chest radiograph. At surgery, patient was found to have extralobar sequestration with anomalous arterial vessel arising from celiac artery and anomalous venous drainage into right atrium. Axial lung window CT image shows anomalous vein (arrowheads) arising from sequestrated lung (S) and crossing midline to right hemithorax via cystic adenomatoid malformation (arrows).

 


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Fig. 2D. 6-month-old boy with abnormal findings on chest radiograph. At surgery, patient was found to have extralobar sequestration with anomalous arterial vessel arising from celiac artery and anomalous venous drainage into right atrium. Three-dimensional volume-rendered image obtained from anteroinferior view shows course of anomalous artery (curved arrow) arising from celiac artery and anomalous vein (straight arrows) crossing midline. Association of vein and cystic adenomatoid malformation is seen better on lung window image (C). RA = right atrium, S = sequestered lung.

 


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Fig. 3A. 6-day-old girl with abnormal findings on prenatal sonography, which showed mass in left lower hemithorax. Suspected extralobar sequestration was based on CT evidence of venous drainage into portal vein. Axial CT image shows small vessel (arrowhead) arising from aorta (A).

 


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Fig. 3B. 6-day-old girl with abnormal findings on prenatal sonography, which showed mass in left lower hemithorax. Suspected extralobar sequestration was based on CT evidence of venous drainage into portal vein. Axial CT image obtained through upper abdomen shows tiny vein (arrowhead) adjacent to left portal vein (curved arrow). This small vein was recognized as vein draining from sequestration only in retrospect.

 


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Fig. 3C. 6-day-old girl with abnormal findings on prenatal sonography, which showed mass in left lower hemithorax. Suspected extralobar sequestration was based on CT evidence of venous drainage into portal vein. Coronal maximum-intensity-projection CT image shows small vein (arrows) arising from sequestered lung (S) draining into portal vein (arrowhead).

 


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Fig. 3D. 6-day-old girl with abnormal findings on prenatal sonography, which showed mass in left lower hemithorax. Suspected extralobar sequestration was based on CT evidence of venous drainage into portal vein. Coronal 3D volume-rendered image shows small anomalous artery arising from abdominal aorta (curved arrow) and also clearly shows anomalous vein (straight arrow) draining into portal vein (arrowheads). S = sequestered lung.

 

The presence and course of the anomalous draining vein were identified on axial views in the patient with surgically proven extralobar sequestration (Fig. 2A, 2B, 2C, 2D). In this patient, the vein from the sequestration in the left lower hemithorax crossed the midline and drained into a congenital cystic adenomatoid malformation in the right lower lobe. In the three patients with intralobar sequestrations, the veins draining from the sequestrations could not be identified on axial images. In the neonate with suspected extralobar sequestration, the drainage into the portal vein was not recognized prospectively on axial images, but only in retrospect after review of the 3D MDCT images (Fig. 3A, 3B, 3C, 3D). In all patients, the draining veins were identified on the 3D reconstructions. With the exception of the patient in whom the vein crossed transversely from the left to right hemithorax, the course of the veins was nearly perpendicular to the imaging plane. The veins, therefore, were more difficult to identify on axial images, especially given the associated inflammatory changes.


Discussion
Top
Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 
Our CT protocol and the use of 3D reconstructions provided unique insights into the vascular architecture of pulmonary sequestration in our five pediatric patients. Although the artery was seen on axial images in all patients, in one patient, 3D images allowed definite confirmation of the artery. These results compare favorably with those of Franco et al. [8], who reported that axial images were adequate to provide the diagnosis of pulmonary sequestration, but 3D reconstructions added information about anatomic relationships in the z-axis.

Other investigators have concluded that the venous drainage of pulmonary sequestration may not be clearly shown on CT [9, 11]. We believe that the CT angiography techniques that we have reported allow display of the venous anatomy. In our opinion, as well as that of others [1], knowledge of the anatomy of the venous drainage is potentially useful before surgical resection. Most intralobar sequestrations require lobectomy or at least a segmentectomy of the involved lung. In contrast, with an extralobar pulmonary sequestration in which venous drainage is often into the azygous vein, the anomaly can be removed without resection of the normal lung tissue. If it can be determined preoperatively whether a sequestration is intra- or extralobar on the basis of the venous drainage, an assessment can be made regarding the possible need for lung resection and can be conveyed to the child's parents. In four of our five patients with sequestration, CT angiograms with 3D images provided the clearest visualization of the detailed anatomy of the vein.

The draining vein of pulmonary sequestration is particularly amenable to 3D reconstruction because it is often oriented in the z-axis. Unlike the aberrant artery which usually has an axial course or minimally oblique course through the image plane to reach the sequestration, the vein courses obliquely to reach the left atrium or systemic veins such as the azygous system. The ability to rotate the 3D volume-rendered display in any orientation in real time is helpful in discerning the relationships of the pulmonary veins [13, 14].

One of the obvious disadvantages of MDCT is the radiation exposure, especially in pediatric patients. Our study has shown that a single phase contrast injection can show the location and course of both the artery and the vein. The use of a single phase of contrast material is critical to reduce radiation exposure. In addition, we used relatively low-exposure factors (30–50 mAs and 80 kV) and achieved superb diagnostic-quality images.

Although it involves exposure to ionizing radiation, CT angiography has obviated catheter angiography, which has a substantially higher radiation exposure. MRI is an attractive alternative to CT, but it requires relatively prolonged sedation times in infants and younger children. The shortened imaging time of CT compared with that of MRI decreases the duration of sedation. In addition, MRI cannot accurately evaluate lung parenchyma. Sonography is the other alternative to CT, but it requires a favorable acoustic window and its use is generally limited to evaluating the neonatal chest.

In conclusion, we believe that low-dose 3D CT angiography can provide useful information about the arterial and venous angioarchitecture in pulmonary sequestration in children. Although axial images are diagnostic for evaluation of arterial feeders, 3D images enhance the visualization of small and tortuous venous vessels and, in some cases, the arterial feeders. This information can allow more confident diagnosis and treatment.


Acknowledgments
 
We thank Sanjeev Bhalla for technical assistance with Figure 3D.


References
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Abstract
Introduction
Subjects and Methods
Results
Discussion
References
 

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  3. Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: radiologic-pathologic correlation. RadioGraphics2002; 22 [suppl]:S25 –S43
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  9. Frush DP, Donnelly LF. Pulmonary sequestration spectrum: a new spin with helical CT. AJR1997; 169:679 –682[Free Full Text]
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