AJR 2000; 175:993-995
© American Roentgen Ray Society
Coil Embolization of Pulmonary Sequestration in Two Infants
A Safe Alternative to Surgery
Kür
ad Tokel1,
Faith Boyvat2 and
Birgül Varan1
1
Department of Pediatric Cardiology, Bakent
University Faculty of Medicine, 12. sokak 7/7,
Bahçelievler 06490, Ankara, Turkey.
2
Department of Radiology, Bakent University
Faculty of Medicine, Bahçelievler 06490,
Ankara, Turkey.
Received November 4, 1999;
accepted after revision March 8, 2000.
Address correspondence to B. Varan.
Introduction
Pulmonary sequestration describes a rare congenital mass of nonfunctional
pulmonary tissue that lacks a normal connection with the bronchial tree or the
pulmonary arteries [1,
2]. Intralobar and extralobar
forms of this condition are seen. Intralobar sequestration occurs in the
normal lung parenchyma and is located in the visceral pleura. Extralobar
sequestration makes up 25% of pulmonary sequestration cases and has its own
distinct pleural covering [1,
2]. Pulmonary sequestration has
conventionally been treated by surgical removal of the tissue. We report two
infants with extralobar pulmonary sequestration who were successfully treated
with coil embolization.
Case Reports
Case 1
A 6-month-old male infant was admitted to the hospital with a cough and
difficulty feeding because of exhaustion. On physical examination, the
patient's weight was 4650 g and his height was 59 cm, both below the fifth
percentile for his age. A grade I/VI systolic ejection murmur was audible at
the third intercostal space, to the right of the sternum
[3]. No hepatomegaly was noted.
Telecardiography showed cardiomegaly and increased pulmonary vascular markings
but no consolidation. Echocardiography revealed enlargement of the right
atrium, right ventricle, and pulmonary artery. Color Doppler sonography showed
turbulent flow in the inferior vena cava. Cardiac catheterization had been
done 1 month earlier at another center, and the child was referred to our
hospital for repair of a secundum-type atrial septal defect associated with
pulmonary hypertension. The infant's mean pulmonary artery pressure was 32 mm
Hg and his pulmonary-to-systemic blood flow ratio (Qp/Qs) was 2.18. Previous
angiography had given the impression of an arteriovenous malformation. After
sedation with midazolam and local anesthesia, the patient was recatheterized
via the arterial route with a 5-French sheath. Angiography revealed a large
feeding artery originating from the proximal abdominal aorta
(Fig. 1A). The anomalous vessel
had two branches and was supplying blood to a localized region of the right
lower lobe, with venous drainage to the right atrium.
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Fig. 1A. —6-month-old male infant with extralobar pulmonary sequestration in
right lung. Aortogram shows large feeding artery originating from proximal
abdominal aorta and supplying blood to localized region of lower lobe of right
lung.
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We made the diagnosis of extralobar pulmonary sequestration, atrial septal
defect, and pulmonary hypertension. We performed embolization of the feeding
artery with polyvinyl alcohol and two coils, each 5 mm in diameter and in
length, (Gianturco; Cook, Bjaeverskov, Denmark). Angiography after
embolization indicated almost complete occlusion
(Fig. 1B), and the patient was
discharged the next day. We performed follow-up catheterization 2.5 months
later, at which time we found insignificant flow in the feeding artery, a mean
pulmonary artery pressure of 36 mm Hg, and a Qp/Qs of 3.1. Six months later,
the infant's weight was 7900 g and he was feeding normally. At this writing,
he is awaiting repair of his atrial septal defect.
Case 2
An 11-month-old female infant was referred to our hospital after cardiac
catherization at another center for ligation of a patent ductus arteriosus.
She had a history of respiratory distress, recurrent respiratory infections,
excessive sweating, and feeding problems. Her weight and height were 6000 g
and 67 cm, respectively, both below the fifth percentile for her age. On
auscultation, a continuous murmur was audible just beneath the left clavicle.
Coarse rales were heard in both lung fields. Telecardiography showed mild
cardiomegaly. The left atrium and left ventricle were both enlarged on
echocardiography. Suprasternal and high parasternal examination revealed a
ductal ampulla, but typical Doppler flow pattern and flow with color Doppler
imaging could not be shown. The patient was recatheterized from the right
femoral artery with a 5-French sheath. We observed a large left-to-right shunt
in the proximal inferior vena cava and in the lower portion of the right
atrium but no shunt at the level of the pulmonary artery (Qp/Qs, 3.2). The
patient's mean pulmonary artery pressure was 23 mm Hg. Angiography showed no
ductal shunt, but two large feeding arteries were visible. These arteries
originated from the abdominal aorta and had venous drainage to the inferior
vena cava and the right atrium (Figs.
2A and
2B).
Our diagnosis was extralobar pulmonary sequestration in the right lower
lobe and "silent" patent ductus arteriosus. We embolized the
feeding arteries using polyvinyl alcohol and two coils (5 x 5 and 5
x 3 mm) (Gianturco: Cook) for each artery. Control angiography showed a
mild shunt in one of the feeding arteries, so a third coil (4 x 3 mm)
was used to stop the residual flow. Angiography after embolization confirmed
complete occlusion of the two arteries
(Fig. 2C).
Two months later, the infant's body weight had increased from 6000 to 7350
g, and she had no respiratory problems. Follow-up catheterization was done for
coil embolization of the ductus arteriosus. Arterial access was gained with a
4-French sheath, but we could not exchange this with a 5-French sheath. The
patient's Qp/Qs was 1.9. An oximetric study revealed a left-to-right shunt at
the pulmonary artery level. Insignificant shunting was noted from each feeding
artery. At 2.5 years old, she weighed 13 kg and had no signs of congestive
heart failure. Cardiac catheterization was made and coil embolization of her
patent ductus arteriosus was attempted. The length of the ductus was too short
to keep the coil; the coil tended to slide to the left pulmonary artery on
several trials so we drew it back. Aortography revealed no flow in the feeding
arteries. Surgical ligation of her patent ductus arteriosus was done.
Discussion
Intralobar and extralobar forms of pulmonary sequestration are known to
occur alone or simultaneously
[1]. Blood supply to the tissue
comes from an anomalous systemic artery or from arteries that originate from a
systemic artery, usually the thoracic or abdominal aorta. Venous drainage
commonly occurs via the pulmonary veins, but occasionally via the azygos,
hemiazygos, or portal vein. The extensive review by DeParades et al.
[2] noted that venous drainage
of extralobar pulmonary sequestration occurred mostly via the hemiazygos vein
and that the pulmonary veins were not involved in their patients. Venous
return via the pulmonary veins was common in patients with intralobar
pulmonary sequestration.
Our patients are rare examples of extralobar pulmonary sequestration
presenting with congestive heart failure, pulmonary hypertension,
malnutrition, and growth retardation. Both patients attracted medical
attention because of their cardiac defects: atrial septal defect in one and
patent ductus arteriosus in the other. Neither of the patient's
telecardiograms showed pulmonary consolidation. The clinical improvement in
both patients after the procedure is clear evidence of the adverse effect of
abnormal arterial flow on their cardiac and nutrition status.
Patients with pulmonary sequestration have conventionally undergone
surgical removal of the abnormal tissue mass. Coil embolization is a new and a
less invasive technique than surgical treatment. Fuhrman et al.
[4] described the use of coil
embolization in infants and children for congenital thoracic vascular
anomalies such as pulmonary arteriovenous malformations, aorticopulmonary
collaterals in cases of tetralogy of Fallot, and patent ductus arteriosus. The
indications for coil embolization are various. The method can be used to
eliminate right-to-left shunting of blood in arteriovenous fistulas, to
protect lungs from excessive intraoperative bronchial blood flow, to eliminate
left ventricular volume overload, to prevent collateral blood flow, and to
occlude unwanted shunts [4].
Reidy et al. [5] reported eight
therapeutic embolization procedures in seven patients with congenital heart
disease.
Specific to pulmonary sequestration, arterial embolization offers a less
invasive method of occluding the anomalous artery in selected patients. Perry
et al. [6] and Rothman and Tong
[7] reported the successful
treatment of four pulmonary sequestration cases with coil embolization, but no
long-term results were available. Park et al.
[8] used coil embolization to
treat extralobar pulmonary sequestration in a 6-day-old male neonate with
nonimmune hydrops caused by congestive heart failure. By 6 months old, the
patient's growth and developmental status were normal. Similarly, we observed
clinical improvement in our patients, and we did not encounter any
complications with the procedure or during follow-up. Other authors have
reported problems such as femoral artery thrombosis at the puncture site
(which recanalized after systemic urokinase treatment)
[8], inadvertent embolization
of the pulmonary arteries or the aorta
[6,
7], and fever
[7].
The body of experience with the embolization technique for pulmonary
sequestration is gradually expanding. Documented successes like those of our
patients and others suggest that this is a safe alternative method to surgery,
especially for infants with extralobar pulmonary sequestration who present
with congestive heart failure.
References
-
Fakhoury KF, Seilheimer DK. Pulmonology. In: Garson A, Bricker JT,
Fisher DJ, Neish SR, eds. The science and practice of pediatric
cardiology, 2nd ed., Baltimore: Williams & Wilkins,
1998: 2813-2832
-
DeParades CG, Pierce WS, Johnson DG, Waldhausen JA. Pulmonary
sequestration in infants and children: a 20-year experience and review of the
literature. J Pediatr Surg
1970;5:136
-147[Medline]
-
O'Rourke RA, Braunwalk E. Physical examination of the
cardiovascular system. In: Fauci AS, Braunwalk E, Isselbacher KJ, et al., eds.
Harrison's principles of internal medicine, 14th ed.,
vol. 1. Maidenhead, Berkshire, United Kingdom:
McGraw-Hill International, 1998:1231
-1237
-
Fuhrman BP, Bass JL, Castaneda-Zuniga W, Amplatz K, Lock JE. Coil
embolization of congenital thoracic vascular anomalies in infants and
children. Circulation
1984;70:285
-289[Abstract/Free Full Text]
-
Reidy JF, Jones ODH, Tynan MJ, Baker EJ, Joseph MC. Embolisation
procedures in congenital heart disease. Br Heart J
1985;54:184
-192[Abstract/Free Full Text]
-
Perry SB, Radtke W, Fellows KE, Keane JF, Lock JE. Coil
embolization to occlude aortopulmonary collateral vessels and shunts in
patients with congenital heart disease. J Am Coll
Cardiol 1989;13:100
-108[Abstract]
-
Rothman A, Tong AD. Percutaneous coil embolization of superfluous
vascular connections in patients with congenital heart disease. Am
Heart J 1993;126:206
-213[Medline]
-
Park ST, Yoon CH, Sung K, et al. Pulmonary sequestration in a
newborn infant: treatment with arterial embolization. J Vasc Interv
Radiol 1998;9:648
-650[Medline]
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Introduction
Case Reports
