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947

Case ‘‘:

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*‘

Bronchopulmonary High-Resolution Eric J. Stern,1

W. Richard

Y

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Sequestration: Dynamic, CT Evidence of Air Trapping

Webb,1

Report

Martha

L. Warnock,2

and

Christopher

Ultrafast,

J. Salmon1

lobe with an air-fluid level, and the patient was treated for presumed

Intralobar bronchopulmonary sequestration is a congenital anomaly that consists of nonfunctioning, abnormal lung tissue that is contained within an otherwise normal lung and has an anomalous systemic blood supply [1 -4]. Although intralobar sequestrations lack normal communications with the tracheobronchial tree, they can be ventilated by collateral air drift or through fistulous bronchial communications that may develop after an episode of infection. An air-containing intralobar sequestration can appear on chest radiographs as an area of hyperlucency, with or without associated visible cysts, or can appear as normal lung [5]. The CT appearances of bronchopulmonary sequestration have recently been reviewed [6], and the presence of lucent or low-attenuation areas of “emphysema” have been emphasized. It has been suggested that the emphysema seen in patients with sequestration results from collateral ventilation and air trapping [7]. We recently had an opportunity to test this hypothesis by using dynamic expiratory, ultrafast, highresolution CT (HRCT) in a patient who subsequently had an intralobar pulmonary sequestration confirmed surgically and

necrotizing

pneumonia.

On conventional

CT, obtained

area

nonsegmental

of abnormally

with contiguous lucent lung

1 -cm collimation, a was visible in the

posterior, medial, right lung base, adjacent and inferior to a homogeneous well-circumscribed soft-tissue density, corresponding to the mass visible on the chest radiograph. The lucent lung suggested the presence of one or more thin-walled, air-containing cysts (Fig. 1A). Aortography abdominal

aorta

venous

drainage

showed an anomalous artery arising from the upper and supplying the region of abnormal right lung. The was

not

clearly

shown.

CT (Imatron C-i 00, Imatron Inc., So. San Francisco, was first performed immediately after the patient’s aortogram Ultrafast

obtained,

with

the

angiographic

catheter

still

in

place

within

CA) was the

anomalous artery that was feeding the sequestration, in order to show the venous drainage of the sequestered lung. After the injection of 8 ml of contrast material, 10 CT scans, with 6-mm collimation, each 100-msec long, were obtained in 6 sec, at a single level through the

atnal

showed drainage

chambers.

opacification

The

“venous

phase”

of this

of the left atrium, indicating

CT

angiogram

pulmonary

venous

of the sequestration.

A series of ultrafast high-resolution CT (HRCT) scans (3-mm collimation, high-spatial-frequency reconstruction algorithm) were ob-

pathologically.

tamed

through

the abnormal

right

lower

lobe at 1-cm

intervals

with

patient supine. These showed numerous, moderately thin-walled, air-containing cysts, 0.5 to 1.0 cm in diameter, that were not visible the

Case

Report

A 1 9-year-old

routine

chest

hyperlucent, before,

a chest

woman

radiograph.

had

a 3-cm,

The surrounding

but no air-containing radiograph

rounded

had shown

cysts

soft-tissue

right lower

were

consolidation

mass

AJR 157:947-949,

November

1991 o361-803x/91/1575-0947

a

on conventional CT. Several larger air-containing cysts, up to 3 cm in diameter, were noted adjacent to the smaller cystic spaces.

Last, two series of dynamic, expiratory, ultrafast HRCT scans were obtained through the middle and lower portions of the sequestration in an attempt to show air trapping within the abnormally lucent

lobe appeared

visible.

Five years

in the right lower

Received May 10, 1991; accepted after revision June 18, 1991. 1 Department of Aadiology, University of California, San Francisco, Department of Pathology, University of California, San Francisco,

2

on

San Francisco, San Francisco,

0 American

Roentgen

CA 941 43-0628. CA 94143-0628. Ray Society

Address

reprint

requests

to E. J. Stern.

Downloaded from www.ajronline.org by 122.183.228.146 on 10/07/15 from IP address 122.183.228.146. Copyright ARRS. For personal use only; all rights reserved

948

STERN

ET

AL.

AJA:157,

November

1991

Fig. 1.-A, Conventional CT scan at level of right lung base. A nonsegmental area of hyperlucency is visible. Cystic components of sequestration are poorly seen. B, Dynamic ultrafast high-resolution CT time-density analysis of normal left lung during a forced exhalation. Measurements were acquired from region of interest (ROI) shown. Hounsfield units are on y-axis, time in seconds on x-axis. Note rapid increase in lung density as patient exhales. During exhalation, lung increases in density by approximately 310 H, well within range of normal in patients we have studied. Note that rate of change in density yields a curve that Is convex upward. C, Time-density analysis of multicystic portion of sequestration in right lung, performed during same exhalation and at same level as in B, shows an increase in density as patient exhales, but at a slower rate than ROl in B. Rate of change in density yields a curve that is concave upward, as opposed to convex-upward curve seen in normal lung. Also, increase in lung density (+140 H) is considerably less than in normal left lung. These findings suggest that this area ventilates, but at an abnormally slow rate. D, Time-density analysis from an area of apparently normal lung parenchyma (arrows) adjacent to sequestration also shows a slow and delayed increase in density during exhalation, yielding a curve that is concave upward. Note that this area of lung is qualitatively more lucent than normal anterior right lung. E, Gross pathologic specimen obtained during partial right lower lobectomy shows multicystic honeycomblike lung that represents sequestration. Note normal lung parenchyma (arrow) adjacent to sequestration.

lung.

Ten

scans,

each

with

of 100 rnsec, were obtained in a forced vital capacity maneuver.

a duration

6 sec, as the patient

performed

Time-density

analysis

graphs

and

uation

were

determined

for

values

for each

series

of images.

Selected

and displayed

in the cin#{233}-loop mode

that

during

occurred

tabulated

mean

normal

and

lung

regions

to show

Hounsfield

abnormal also

atten-

lung were

regions

increase

Several

other

areas

of the

seen

in the

sequestration

normal that

contralateral consisted

of small

on

lung. air

no increase

in density

adjacent

to the cystic

the

CT

HACT

findings the

of delayed

and

to be

showed a normal increase

in density

transitional

HRCT,

which

the

suggests

HRCT

Ultrafast done

by using lung

lung

moves

the

had

normal

that

cephalad

diminished

during not

analysis

contralateral through

part

the

exhalation

in density.

obvious

subpleural

cystic

lung, (Figs.

which 1 B-i

on

sequestration.

of the sequestration

scanning

D).

architecture

of the

left lung the

showed

increase

between normal

appeared

inspiration,

bronchovascular

it was

time-density normal

Unexpectedly, which

during

transitional and

exhalation.

sequestration,

obtained

sequestration

This

of

during

scans

region appeared

portion

exhalation.

that was

of lung

normal This

processes

Several areas within the sequestration that contained small cysts showed an increase in attenuation density during exhalation, but this increase was at a delayed rate, and to a lesser degree, than the density

showed

a region dynamic

visualized

the dynamic

cysts

as a control. plane

during

was

Although exhala-

AJA:157,

November

tion,

motion

was

that

the lung

this

to ensure

DYNAMIC

1991

minimal,

and

region

the same. Partial right lower lobectomy pulmonary

Downloaded from www.ajronline.org by 122.183.228.146 on 10/07/15 from IP address 122.183.228.146. Copyright ARRS. For personal use only; all rights reserved

of the x

sequestration specimen.

On

was gross

of which

filled

with

filled

cyst

were

mucin

and

thelium

and

identifiable

which

was performed,

diagnosed

was this

than

1 cm

largest the

rounded

rare

present

represented

lung

viewed

substantially

and an intralobar examination

resected

specimen

(14.0

air-containing

clotted

soft-tissue

cysts,

some

cysts

were

blood.

This

blood-

mass

seen

on

the

the cysts were lined by airway epi-

cartilage

elements.

in the

subpleural gradually

sequestration

No

However,

blended

the

were

was

pathologic

in diameter;

contained

Histologically,

normal

after the

in this part of the specimen.

parenchyma specimen; lung,

less

contained

slice

primarily of thin-walled

the

represented

chest radiograph.

images

on each

inspection,

8.0 x 2.3 cm) consisted

most

the individual

selected

HRCT

region with (Fig.

bronchus

was

some normal lung of

the

the cystic

resected abnormal

1 E).

Discussion It has been suggested that hyperlucent lung parenchyma seen in association with sequestration is the result of air trapping within the sequestration, within large air-containing cysts, or within the adjacent lung [5-7]. In each of these instances, the air trapping presumably occurs because the abnormal lung parenchyma lacks normal bronchial connections and is aerated collaterally [5, 6]. In a recent review, Ikezoe et al. [6] found the presence of lucent or low-attenuation areas of “emphysema” in six of i 6 patients with an intralobar sequestration. Because of the greater contrast and density resolution of CT, the hyperlucent lung associated with sequestrations is better seen on CT scans than on chest radiographs. However, heretofore CT has not clearly shown the relationship of the lucent areas to either cystic regions of the sequestration or to adjacent lung. Ikezoe et al. [6] were unable to show that areas of Iucency were associated with small air-containing cysts, but HRCT was not performed in any of their cases, and cyst walls may not have been visible. In our case, the areas of lucency on CT corresponded to areas of the sequestration that contained both small and large air cysts, and these lucent areas also were seen in apparently normal lung at end exhalation. The cystic portion of the sequestration that we studied showed a delayed and diminished increase in density during exhalation, best shown by using time-density analysis. These findings suggest that communication with the airway occurred in this case, but that an abnormal egress of air, or air trapping, occurred during rapid exhalation; if no communication were present, no change in density or cyst size would be expected during exhalation. Indeed, in several areas of the sequestration, little or no increase in density was measured during exhalation. Because no normal bronchial connection to the sequestration was seen on pathologic examination, we assume that the ability of some parts of the sequestration to ventilate, albeit at a reduced rate, must be because of the presence of anomalous bronchial connections or fistulas.

OF

SEQUESTRATION

949

These features strongly support the hypothesis of air trapping via collateral pathways as the mechanism for the emphysematous parenchyma associated with intralobar pulmonary sequestrations. It is not clear if a previous or ongoing inflammatory process is a prerequisite for the development of these collateral pathways or emphysematous spaces. For example, in smoking-related pulmonary emphysema, a contributing factor to the formation of emphysematous cysts appears to be inflammatory degradation and disruption of elastic fibers in the alveolar walls [8]. Additionally, a region of apparently normal lung adjacent to the sequestration also showed a delayed and diminished increase in density during exhalation. Although we have no pathologic proof, we speculate that this segment of lung, which exhibited abnormal lucency only during exhalation, also must have been trapping air, either because of the mass effect of the sequestration and compression of the segment’s subtending airway or as a manifestation of inherently abnormal lung parenchyma that was transitional between the sequestration and normal lung, detectable only with this technique. The use of ultrafast CT to define the venous drainage of a sequestration has not been previously reported. Although we do not advocate the use of CT angiography in all such cases, the detection of the venous drainage of the sequestration into the left atrium, shown in this case, makes the diagnosis of intralobar sequestration likely. Extralobar sequestrations usually drain via systemic veins into the right atrium and only rarely empty via pulmonary veins. We conclude that dynamic ultrafast HRCT clearly shows the air trapping that is proposed as a mechanism for the emphysematous changes that occur in association with intralobar sequestrations and that this air trapping occurs both in the sequestration and in adjacent normal-appearing parenchyma. Also, HRCT shows the characteristic multicystic component of pulmonary sequestrations better than conventional CT does, thus aiding in the diagnosis.

REFERENCES 1 . Choplin A, Siegel M. Pulmonary sequestration: six unusual presentations. AJR 1980;1 34:695-700 2. Felker A, Tonkin I. Imaging of pulmonary sequestrations. AJR 1990:154 : 241 -249 3. Paul D, Mueller C. Case report: pulmonary sequestration. J Comput Assist Tomogr 1982:6: 163-1 65 4. Wimbish K, Agha F, Brady T. Bilateral pulmonary sequestration: CT appearance. AJR 1983;140:689-690 5. Felson B. Pulmonary sequestration revisited. Med Radiogr Photogr 1988:64:1-28 6. Ikezoe J, Murayarna 5, Godwin JD, Done SL, Verschakelen JA. Bronchopulmonary sequestration: CT assessment. Radiology 1990:176:375-379 7. Culiner M, Wall C. Collateral ventilation in intralobar pulmonary sequestration. Dis Chest 1965:47:118-122 8. Fukuda Y, Basset F, Soler P. Ferrans VJ, Masugi Y, Crystal AG. Intraluminal fibrosis and elastic fiber degradation lead to lung remodeling in pulmonary Langerhans cell granulomatosis (histiocytosis X). Am J Pathol 1990:137:415-424

Bronchopulmonary sequestration: dynamic, ultrafast, high-resolution CT evidence of air trapping.

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