(Chest. 2001;120:298-301.)
© 2001
American College of Chest Physicians
A 53-Year-Old Man With Hemoptysis*
Satinder P. Singh, MD and
Hrudaya Nath, MD, FCCP
*
From the University of Alabama at Birmingham (Dr. Nath), and the Veterans Administration Medical Center (Dr. Singh), Birmingham, AL.
Correspondence to: Satinder P. Singh, MD, University of Alabama at Birmingham, 619 South 19th St, Birmingham, AL 35233; e-mail: lhogan{at}uabmc.edu
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Introduction
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A 53
-year-old black man presented with recurrent episodes of hemoptysis,
with 1.5 cups of bright red blood expectorated over a 2-day period. He
denied having fever, chest pain, vomiting, night sweats, shortness of
breath, or dizziness. He had had a dry cough for several months before
admission to the hospital. He also had lost 40 lb from dieting in the
past several years. He was being treated for hypertension and
gastroesophageal reflux disease. He had a 30-year history of tobacco
use, occasional alcohol use, and no IV drug abuse. Clinical examination
revealed a pleasant black man not in acute distress. Vital signs were
stable: pulse rate, 79 beats/min; BP, 154/92 mm Hg; and respiratory
rate, 21 breaths/min. There was no clubbing or lymphadenopathy. Chest
examination revealed scattered crackles at both lung bases. The rest of
the physical examination was unremarkable. Laboratory investigation
showed a hemoglobin level of 14.2 g/dL, a WBC count of
6.0 x 103/µL, and a platelet count of 277 x
103/µL. Prothrombin time was 24.2 s, and
international normalized ratio was 0.88. Fiberoptic bronchoscopy
revealed active oozing of blood from the left lower lobe bronchus with
mildly edematous airways.
A chest radiograph (Fig 1
) obtained at the time of admission demonstrated a vague airspace
opacity in the left lower lobe. Contrast-enhanced CT of the chest was
performed. A dilated left lower lobe pulmonary vein was seen draining
into the left atrium (Fig 2
, top). An enhancing round vascular structure was also seen
anterolateral to the descending thoracic aorta (Fig 2
, top).
Ill-defined airspace opacities in the posterior basal segment of left
lower lobe (Fig 2
, bottom) were thought to represent focal
pulmonary hemorrhage. To evaluate the vascular anomaly, a pulmonary
angiogram was obtained, which demonstrated the absence of pulmonary
artery to the posterior basal segment of the left lower lobe (Fig 3
). There was no evidence of thrombosis, arteriovenous malformation, or
pulmonary varix. The patient experienced three more episodes of
significant hemoptysis while he was in the hospital. He underwent
urgent left thoracotomy.
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Figure 2.. Top: Contrast-enhanced CT
(soft-tissue window) at the level of lower lobe demonstrates an ovoid,
enhancing, dilated left inferior pulmonary vein (arrow). Another round,
enhancing structure next to the descending aorta was suspected to be an
aberrant systemic artery supplying the left lower lobe.
Bottom: A CT image (lung window) 1 cm below that image,
demonstrates ill-defined airspace opacity and increased vascularity in
the posterior basal segment of the left lower lobe.
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Figure 3.. Selective left pulmonary arteriogram in the right
anterior oblique position demonstrates absence of arterial supply to
the posterior basal segment of left lower lobe (asterisk). All the
visualized pulmonary arterial branches are normal in appearance. There
was no evidence of pulmonary thrombosis (acute or chronic), or
arteriovenous malformation.
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What is the diagnosis?
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Diagnosis: Aberrant systemic artery supplying the left lower lobe
without sequestration (Pryce’s type 1 anomaly)
CT demonstrated an increased number and size of pulmonary vessels
in the left lower lobe compared to the right side (Fig 2
,
bottom). A vascular anomaly, either a complex arteriovenous
malformation or anomalous systemic artery supplying the left lower
lobe, was suspected. An arteriovenous malformation was considered less
likely because only a dilated pulmonary vein is seen, without an
accompanying large pulmonary artery.
Based on the CT and pulmonary angiographic findings, the presumptive
diagnosis was sequestration of the lung. Since an abnormal arterial
supply from the thoracic aorta was identified on CT, an aortogram was
not obtained.
At surgery, the left lower lobe was discolored. The superior and
inferior pulmonary veins were normal. A large, 2- to 3-cm, hard,
calcified, aberrant artery was seen arising from the distal descending
thoracic aorta supplying the posterior segment of the left lower lobe.
There was a normal pleural lining of the lung. Left lower lobectomy was
performed. Microscopic examination of the aberrant systemic artery
demonstrated an elastic artery with extensive calcific atherosclerosis
and fibrosis. In the excised lobe, there was extensive intimal
thickening and fibrosis of the pulmonary arteries and arterioles
consistent with pulmonary hypertension. The lung parenchyma showed
accentuation of septae, focal anthracosis, and massive recent as well
as old intra-alveolar hemorrhage. Multiple resected hilar nodes showed
sinus histiocytosis and anthracosis. No cancerous tissue was seen in
the lung parenchyma or the resected nodes.
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Discussion
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The term sequestration was coined by
Pryce1
in 1946 to describe a disconnected bronchopulmonary
segment or cyst with an anomalous systemic artery supplying the
involved lung. There are many variants of sequestration that do not
strictly meet these criteria. Sade et al2
in 1974
suggested the term spectrum of pulmonary sequestration to
include the diverse group of pulmonary abnormalities that appear to be
related and may have a common embryogenesis. This spectrum includes
normal vessels supplying abnormal lung at one end and abnormal vessels
supplying normal lung at the other.3
Pulmonary sequestration is an uncommon congenital malformation. Two
types are commonly described: intralobar sequestration, which describes
the sequestered lung contained within the normal visceral pleura; and
extralobar sequestration, in which the abnormal lung is completely
separate and enclosed in its own pleural sac. The majority (75%) of
cases are of the intralobar sequestration variety. Pryce,1
in his original article, described three types of intralobar
abnormalities based on the distribution of the aberrant artery. In type
1 (our patient), the anomalous artery supplies functional normal lung
tissue, which communicated with the tracheobronchial tree. In type 2,
the systemic artery supplies both normal lung as well as nonfunctional,
noncommunicating lung tissue. In type 3, the anomalous artery supplies
lung that is isolated from the tracheobronchial tree.1
More than 50% of patients with intralobar sequestration become
symptomatic after the age of 20 years.4
The common
symptoms include cough, sputum production, and recurrent pulmonary
infections. Hemoptysis is also a common presenting sign. Chest pain,
asthma, and pleuritic pain are less common presentations. A small
number (15%) of patients may be asymptomatic when the lesion is
discovered.4
The less common extralobar sequestration
frequently presents in the neonates and early childhood with
respiratory distress, cyanosis, and feeding problems. A majority of
patients with extralobar sequestration have other congenital
abnormalities, including esophagobronchial diverticulum, diaphragmatic
hernia, skeletal deformities, cardiovascular defects, and renal
anomalies.5
6
Systemic arterial supply to normal lung is a rare but well-recognized
variant of the sequestration spectrum.7
The aberrant
systemic artery usually arises from the descending thoracic aorta. The
basal segments of left lower lobe are more frequently involved, with no
radiographic abnormality in the lung parenchyma. Drainage of the
involved lung is usually accomplished via the inferior pulmonary vein
into the left atrium. Although the etiology of systemic arterial supply
to the normal lung is unknown, the most likely explanation is that one
or more of the intersegmental arteries from the dorsal aorta retain
their original embryonic connection between the aorta and the pulmonary
parenchyma.8
These patients may be asymptomatic. However,
left ventricular enlargement and congestive heart failure from
left-to-right shunt may eventually develop. Hemoptysis, as in our
patient, is a rare but recognized presenting feature.3
4
It results from rupture of the abnormal pulmonary vessels in the
affected lung, caused by the pulmonary hypertension, resulting from
transmission of systemic pressure to the affected lung. Focal pulmonary
arterial hypertension occurs since the pulmonary arterial branches of
the sequestered lung do not communicate with the rest of the pulmonary
vasculature. Cardiac catheterization often does not reveal pulmonary
hypertension unless longstanding left ventricular failure occurs
from large left-to-right shunt. The term pseudosequestration
has been used to describe the combination of systemic arterial supply
to lung with normal bronchial connections but with coexistent
chronic/recurrent pulmonary infection.9
In these cases,
the chronically inflamed lung and pleura recruit the normally occurring
systemic arteries supplying the lung and chest wall (bronchial,
intercostal, diaphragmatic, and pulmonary ligament arteries), which
result in a systemic arterial blood supply to the inflamed lung tissue.
However, these cases are distinct from the anomaly in our patient,
in which one or more discrete abnormal anterior branches from
descending aorta supply the affected lung. In the former condition
(pseudosequestration), the systemic arterial supply is from
hypervascularization of normal anterior branches of the chest wall
(eg, intercostal and internal mammary arteries).
A chest radiograph may demonstrate a homogeneous opacity in the lung
base in uncomplicated intralobar sequestration. Complicated
sequestration may present as a cystic lesion that may contain air-fluid
levels. Traditionally, diagnosis of pulmonary sequestration is made
with aortography. The classic findings are the anomalous systemic
arterial supply and anomalous venous drainage, depending on the type of
sequestration. The pulmonary arteriogram may be useful in demonstrating
the absence of pulmonary blood supply to an area of lung,9
as in our case. CT is an useful imaging study, especially for
evaluation of a patient with hemoptysis. It can show lung parenchymal
changes and the aberrant vessels supplying the sequestration. The
anomalous systemic artery is visualized in up to 80% of cases after
contrast administration and may be seen in cross-section (as in our
case) or as an enhancing linear structure adjacent to the aorta in the
inferior pulmonary ligament.10
11
12
Use of dynamic scanning
technique, thin-section CT, and helical CT substantially improves the
delineation of the origin and course of the anomalous systemic
artery.10
12
MRI can demonstrate the anomalous vessels and
the parenchymal abnormalities in multiple planes. Sonography has been
used in the diagnostic evaluation of pulmonary sequestration both
in utero and in infants.13
Preoperative diagnosis of sequestration is helpful by forewarning the
surgeon of the anomalous vascularization of the lung, preventing fatal
outcome due to exsanguination from accidental division of the systemic
artery.
Received for publication May 2, 2000.
Accepted for publication August 10, 2000.
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References
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Pryce, DM (1946) Lower accessory pulmonary with intralobar sequestration of lung: report of seven cases. J Pathol 58,457-467[CrossRef]
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Sade, RM, Clouse, M, Ellis, FH, Jr (1974) The spectrum of pulmonary sequestration. Ann Thorac Surg 18,644-658[Medline]
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Felker, RE, Tonkin, ILD (1990) Imaging of pulmonary sequestration. AJR Am J Roentgenol 154,241-249[Free Full Text]
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Savic, B, Birtel, FJ, Tholen, W, et al (1979) Lung sequestration: report of seven cases and review of 540 published cases. Thorax 34,96-101[ISI][Medline]
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DePardes, CG, Pierce, WS, Johnson, DG, et al (1970) Pulmonary sequestration in infants and children: a 20-year experience and review of the literature. J Pediatr Surg 5,136-147[CrossRef][ISI][Medline]
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Ferris, EJ, Smith, PL, Mirza, FH, et al (1981) Intralobar pulmonary sequestration: value of aortography and pulmonary arteriography. Cardiovasc Intervent Radiol 4,17-23[ISI][Medline]
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Kirks, DR, Kane, PE, Free, EA, et al (1976) Systemic arterial supply to normal basilar segments of the left lower lobe. AJR Am J Roentgenol 126,817-821[Abstract]
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Hessel, EA, II, Boyden, EA, Stamm, SJ, et al (1970) High systemic origin of sole artery to basal segments of the left lung: findings, surgical treatment, and embryologic interpretation. Surgery 67,624-632[ISI][Medline]
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Livingston, DR, Mehta, AC, O’Donovan, PB, et al (1986) An angiographic dilemma: bronchopulmonary sequestration versus pseudosequestration; case reports. Angiology 37,896-903
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Ikezoe, J, Murayama, S, Godwin, JD, et al (1990) Bronchopulmonary sequestration: CT assessment. Radiology 176,375-379[Abstract/Free Full Text]
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Rappaport, DC, Herman, SJ, Weisbrod, GL (1994) Congenital bronchopulmonary diseases in adults: CT findings. AJR Am J Roentgenol 162,1295-1299[Abstract/Free Full Text]
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Miller, PA, Williamson, BRJ, Minor, GR, et al (1982) Pulmonary sequestration: visualization of the feeding artery by CT. J Comput Assist Tomogr 6,828-830[ISI][Medline]
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May, DA, Barth, RA, Yeager, S, et al (1993) Perinatal and postnatal chest sonography. Radiol Clin North Am 31,499-516[ISI][Medline]
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Introduction
What is the diagnosis?
