Advanced therapy in thoracic surgery - part 4 pot

most likely to present in the perinatal period as a result of
displacement of normal lung and impaired respiration. If
peripheral lesions cause airway obstruction, postobstruc-
tive infections or lobar hyperinflation may develop.
When airway blockage is more central, significant hyper-
inflation, mediastinal shift, and cardiopulmonary com-
promise may occur. A more unusual presentation in
which the cyst exists within the airway wall may present
with signs and symptoms similar to an airway foreign
body; if such a cyst is proximal and enlarges rapidly due
to mucus production, inflammation, or hemorrhage,
acute airway obstruction may occur. This condition may
be hard to diagnose with routine imaging studies as there
is no predominant mass. Bronchoscopy may be the only
means for diagnosis and treatment of this situation. For
the majority of patients, however, symptoms are less
severe and less acute; presentation is usually as an older
infant or child with complaints of infection or dysphagia.
Symptoms of cough, wheezing, fever, or hemoptysis
prompt chest roentgenogram, which may demonstrate
an unusual opacity or lucency, postobstructive emphyse-
matous changes, or air–fluid level within the cyst
(Figure 13-3). A chest radiograph that suggests a mass
should be followed by CT scan to verify and localize the
mass, determine its resectability, and to eliminate any
nonoperative diagnoses such as pneumonias, simple lung
abscesses, and certain lymphangiectasis. CT scan should
demonstrate a cystic structure with a nonenhancing wall;
it may have an air–fluid level (Figures 13-4 and 13-5). A
finding of segmental emphysematous change may
warrant bronchoscopy to rule out an airway foreign body

(especially in the age group in which aspiration is
common) or extrinsic airway compression. It may be
difficult to differentiate between bronchogenic cyst and
pulmonary abscess, but an indolent clinical course
coupled with persistent radiograph findings in the face of
abatement of clinical symptoms suggests bronchogenic
cyst over infectious etiologies. Those with persistent
evidence of systemic infection and pulmonary symptoms
may have infection alone or in combination with under-
lying bronchogenic cysts and would likely benefit from
resection with respect to either the infection or cyst.
Patients who present with dysphagia should undergo
esophagogram, which will sometimes reveal evidence of
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FIGURE 13-4. Bronchogenic cyst: Computed tomography scan with 2 cm right-sided nonenhancing mass representing a paratracheal bron-
chogenic cyst.
FIGURE 13-3. Bronchogenic cyst: Plain radiograph demonstrating a
large right-sided bronchogenic cyst.
difficult to differentiate a bronchogenic cyst from other
congenital cystic malformations. These other diagnoses
may have associated anomalies, which need to be investi-
gated and addressed prior to cyst excision. Cysts should be
followed by ultrasound prenatally and if a large cyst causes
in utero compromise from mediastinal displacement or
hydrops, in utero decompression can be achieved via aspi-
ration or thoracoamniotic shunt placement.
7
These cysts

do not routinely expand rapidly in utero, so ultrasound
monitoring does not have to be done as frequently as for
lesions such as CCAM or pleural effusions.
There is some debate, but most pediatric surgeons
believe for several reasons that asymptomatic children
should undergo resection of their bronchogenic cyst in the
absence of prohibitive medical comorbidities and risk
factors that may rarely warrant observation. Cysts increase
in size over time due to mucous production and therefore
will ultimately become symptomatic; resection before
infectious complications develop is preferable, especially
since this may decrease inflammation and make resection
technically easier. There is also a malignant potential. Both
rhabdomyosarcoma and adenocarcinoma have developed
within bronchogenic cysts.
8–10
Although malignancy is a
rare occurrence, the natural history is difficult to evaluate
as most lesions are resected in children. A well-
differentiated papillary adenocarcinoma in a retroperi-
toneal bronchogenic cyst was found at laparotomy for
abdominal pain in a 55-year-old woman.
8
Therefore, in
asymptomatic adults, because of the malignant potential
and presence of the lesion since birth, bronchogenic cysts
should be addressed at the time of their discovery. The
advent of sophisticated techniques provides an avenue for
surveillance and in some situations may justify conserva-
tive treatment of adults with small, stable, asymptomatic

cysts that are not prone to connection with the tracheo-
bronchial tree.
11
Cyst fluid and sometimes cyst wall can be
sampled via percutaneous or transbronchial needle aspira-
tion. Recurrence after aspiration, malignant cells, cyst
growth, symptoms, an air–fluid level, or an intraparenchy-
mal lesion warrants complete surgical resection. When
evaluating the benefits of resection in adults, the patient’s
projected longevity and comorbid medical issues should
be assessed in conjunction with the proposed surgical
procedure (less invasive bronchoscopy or thoracoscopy
versus mediansternotomy or thoracotomy) and the proba-
bility of developing a malignancy.
Pulmonary Sequestration
Pulmonary sequestrations were first described by Pryce
in 1946. They are composed of nonfunctional embryonic
lung with absent or abnormal communication to the
tracheobronchial tree and a predominantly systemic
vascular supply (Figure 13-6). Several theories exist
regarding their development. Sequestrations could result
from traction applied to developing lung tissue by elon-
gating systemic arterial channels, which would explain
the ultimate anatomical relationship between the
pulmonary tissue and systemic blood supply. Seques-
trations may also represent other congenital lung anom-
alies such as CCAMs or bronchogenic cysts that develop
an aberrant blood supply. Alternatively, sequestrations
may develop from accessory lung buds, which have
systemic blood supply separate from the normal

pulmonary vasculature. The arterial supply to the seques-
tration usually emanates from below the diaphragm and
is almost always a branch from the abdominal aorta.
Sequestrations that develop before the completion of
visceral pleura formation exist within normal lung
parenchyma and are called intralobar; extralobar seques-
trations develop after pleura formation of the normal
lung and have their own pleura (Figure 13-7).
Pulmonary sequestration accounts for about 30% of
bronchopulmonary-foregut anomalies.
4
There is no
strong gender preference, although some reports describe
a male preference (1.5:1). No known causative genetic
defect exists, although at least one report involves two
male siblings with sequestrations.
12
Associated congenital
anomalies occur and with higher frequency in extralobar
sequestration; as the bronchopulmonary tree and foregut
are closely linked in development, many associated
anomalies are other congenital lung bud or upper
gastrointestinal defects. Sequestration may be found in
conjunction with bronchogenic cysts, esophageal cysts,
CCAM, and tracheoesophageal fistulas. Sequestrations
are most often unilobar and involve the lower lobes but
can involve an entire lung
13
or rarely both lungs.
Classification into intralobar and extralobar subtypes is

not only useful from a pathologic standpoint, but also
has clinical application, as each variety has different
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167
FIGURE 13-6. Pulmonary sequestration: 50ϫ magnification;
hematoxylin/eosin stain.
of sequestrations with gastrointestinal communication
reported that 43% were evident by 7 days of life, 30%
within the first year, 17% by age 18 years, and 10% as
adults.
17
Most commonly, however, children present by
age 10 years with chronic cough, recurrent pneumonia,
or its complications. Pulmonary abscess can cause
erosion of vessels, and because of their systemic arterial
pressure, hemoptysis or hemothorax can be massive;
even fatal hemoptysis has been reported. Sequestrations
are discovered incidentally in about 15% of cases on
imaging for unrelated issues or during surgery to correct
other congenital defects such as diaphragmatic hernia.
15
Diagnostic imaging should start with plain chest
radiograph, which may demonstrate consolidation (since
extralobar sequestrations are not connected to the
tracheobronchial tree and are not aerated) or mass effect;
sometimes there will be cysts or evidence of lung abscess
(Figure 13-8). The diagnosis of sequestration can be
confirmed by documenting the aberrant blood supply
with ultrasound, MRI, or CT (Figures 13-9 and 13-10). It

is important to image below the diaphragm since most
arterial supply is from the abdominal aorta. Invasive
studies such as aortography are unnecessary; even MRI
and CT are not routinely necessary if plain film and
ultrasound provide enough evidence of a thoracic mass
with infradiaphragmatic aortic blood supply. Ultrasound
is noninvasive and excellent for vascular evaluation; it
also does not require the same duration and degree of
stillness as magnetic resonance scanning. In young chil-
dren, advanced imaging may require general anesthesia
as well as contrast material; therefore, more complex tests
should be used only if additional information is needed
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169
FIGURE 13-9. Pulmonary sequestration: A, Ultrasound identifying the aberrant systemic blood supply originating from the aorta and entering
the mass. B, Duplex demonstrates the arterial waveform in this vessel.
FIGURE 13-8. Pulmonary sequestration: Plain radiograph with hyper-
lucent area demonstrating a right-sided pulmonary sequestration.
without esophageal atresia. These usually have systemic
arterial supply but may also have atretic pulmonary
blood supply. Venous drainage may be pulmonary,
azygous, or portal. These cases should undergo resection.
In the cases where lobar or segmental airways connect to
the esophagus, the corresponding airway is absent in
conjunction with the tracheobronchial tree.
17
Type IV
sequestrations (5%) are described as intralobar seques-
trations in which part of the tracheobronchial tree

connects to the esophagus. These have systemic blood
supply and they should be resected.
The outcome of children with pulmonary sequestra-
tion is excellent if it is isolated or if the prognosis for any
associated congenital conditions is good. The survival for
surgical resection has reached almost 100%, even for
pneumonectomy; the main complications are postopera-
tive pulmonary infection or leak from gastrointestinal
communication. Improvements in imaging and under-
standing of the vascular abnormalities associated with
sequestrations make exsanguination from an aberrant
artery preventable. Reflux is also reported to occur
frequently in those who have had sequestrations with
connection to the gastrointestinal tract at the gastro-
esophageal junction. Complications occur infrequently
and are treatable. The major morbidity comes from
pulmonary hypoplasia or associated congenital anom-
alies, especially cardiac defects or diaphragmatic hernia
associated pulmonary hypoplasia. A 75% mortality is
reported in the 15% of sequestrations with associated
conditions.
20
Congenital Lobar Emphysema
CLE refers to a hyperinflated segment of histologically
normal lung. It occurs rarely and is more common in
male than female babies (3:1). In 20% of patients, asso-
ciated cardiac, rib, and renal anomalies exist. Most often,
a single, upper lobe is affected, specifically the left upper
lobe; multiple lobes can be involved and 20% are bilat-
eral. Lobar emphysema is caused by expiratory collapse

of the airway and air trapping. Air can also enter the
parenchyma through pores of Kohn with adjacent lung
and thereby keep an area hyperinflated even if the
airway is obstructed on inspiration. Constant hyperin-
flation leads to septal destruction and large emphysema-
tous air sacs. Physiologically, CLE causes symptoms by
displacing normal lung, creating mediastinal shift, and
decreasing venous return. CLE can be due to not only
intrinsic obstruction (foreign body, mucus, or endo-
bronchial lesion) but also extrinsic causes of compres-
sion such as masses, enlarged cardiac chambers (15%),
lymphadenopathy, or vascular rings, and these etiologies
should be entertained during differential diagnosis espe-
cially in a patient presenting after the newborn period.
Intrinsic causes, which are more likely to present in
infancy, include hypoplasia (Figure 13-11) of airway
cartilage (documented in about 35% but perhaps as
prevalent as 70%), excess bronchial mucosa, mucous
plugging, airway torsion, alveolar fibrosis, alveolar septal
destruction, and polyalveolar lobes. Polyalveolar lobes,
which may account for up to 30% of congenital hyperin-
flation syndromes, demonstrate increased numbers of
alveoli that accumulate air.
21
In up to 50% of cases, the
exact cause of hyperinflation is not identified.
The timing and acuity of presentation depends on the
degree of hyperinflation, rate at which it develops,
displacement of normal lung, and mediastinal shift.
Presentation can be dramatic, for example, with expira-

tory obstruction causing a sudden increase in hyperin-
flation with mediastinal shift and cardiopulmonary
collapse. This is most characteristic of CLE from airway
hypoplasia with collapse or intrinsic airway obstruction
as an etiology. With parenchymal causes of emphysema
such as septal destruction or polyalveolar lobes, hyperin-
flation is from gradual accumulation; therefore, sudden
expansion due to expiratory insufficiency does not lead
to sudden mediastinal shift phenomena. If the area
involved is large, infants may present in the perinatal
period when their lungs become aerated and hyperinfla-
tion develops. Respiratory distress can be severe if the
hyperinflation is sudden, causing mediastinal shift,
which exacerbates instability by diminishing the expan-
sion of the contralateral lung and limiting venous return
and cardiac output. Infants usually present with respira-
tory distress or even cyanotic spells within 1 to 2 months
of age. Children who present in the newborn period are
more likely to have symptoms that worsen quickly and
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171
FIGURE 13-11. Congenital lobar emphysema (CLE): Pathology speci-
men of airway from surgically resected CLE. The airway is collapsed
and cartilage, which should have a uniform circumferential distribu-
tion, has a sparse and irregular pattern. 50ϫ magnification;
hematoxylin/eosin stain.
require surgery early on. Fifty percent of babies that
undergo resection for CLE are diagnosed within several
days of life; the other half may not present for months.

Less symptomatic infants and children have a more
insidious presentation manifested by failure to thrive,
mild tachypnea, unequal breath sounds, shifted position
of maximal cardiac impulse, expiratory wheezing,
hyperresonance, or tracheal deviation. The presentation
can take several months while the degree of collapse
reaches a point at which air trapping occurs. Crying
spells can significantly exacerbate hyperinflation and
mediastinal shift.
Prenatal diagnosis is rare because the lungs are not
aerated; therefore, the diagnosis is usually made with
plain chest film in the newborn period or upon presenta-
tion with clinical signs. Chest radiograph will demon-
strate a hyperlucent area, surrounding atelectasis,
ipsilateral diaphragmatic flattening, and possibly medi-
astinal and tracheal deviation (Figure 13-12) The lobes
are involved in the following distribution: left upper lobe
40%, right middle lobe 35%, right upper lobe 20%, and
bilateral 1%.
22
Differentiation via radiograph from
primary cysts or tension pneumothorax may be difficult
but one will see faint outlines of pulmonary vasculature
within the affected CLE. Additional imaging is usually
unnecessary to diagnose CLE but may be helpful in find-
ing the etiology or differentiating CLE from atypical
appearing CCAM or pneumothorax if diagnosis is
unclear on plain film. Extrinsic causes of airway obstruc-
tion such as a thoracic mass or vascular ring should be
investigated using MRI or CT, especially in older patients

in whom these etiologies are more likely (Figure 13-13).
Bronchoscopy should be performed if suspicion of a
foreign body or endobronchial lesion is high. Echo-
cardiography to identify associated cardiac conditions
and ultrasound to look for renal anomalies will be posi-
tive in 20%. Although not routinely necessary, a
ventilation–perfusion scan, if performed, will show
delayed uptake and washout of xenon and decreased
blood flow in the emphysematous portion. Pulmonary
function tests (they would show slightly decreased flow)
are not necessary for preoperative workup of isolated CLE
since the remainder of the lung has normal function.
There is no associated lung hypoplasia because the hyper-
inflation develops after delivery and does not cause in
utero compression of the developing lung. Occasionally,
on CT or ventilation–perfusion scan, additional areas of
hyperinflation become evident; these, however, can be
seen and addressed at the time of thoracotomy.
The treatment strategy for CLE is resection of the
affected lobe (Figure 13-14). One must also eliminate any
possible extrinsic or intrinsic cause for obstruction and
hyperinflation that can masquerade as CLE; if this leads
to resolution of air trapping, the diagnosis was not
primary CLE and lobar resection be avoided.
Occasionally, a mass or vascular ring causing compres-
sion will be apparent as the cause of bronchiolar collapse
and can be removed. In addition, rarely, things causing
endobronchial obstruction may be amenable to endo-
scopic removal. For this reason, bronchoscopy should be
performed prior to resection to identify any potentially

treatable lesions. Since at least one-half the cases of CLE
have no known etiology and many others have hypoplas-
tic airway cartilage as a cause, sparing a lobe is rarely
possible. Complete resection of the lobe and any addi-
tional segments involved is curative and should be
prompt. Some feel that babies with mediastinal shift
regardless of symptoms should be kept under medical
observation until surgical correction. If there is evidence
of longstanding stable disease without symptoms
(delayed referral perhaps), these children have low likeli-
hood of sudden decompensation and can remain at
home until surgery, which should occur within the next
couple of weeks. Symptoms, unpredictable course, or
unpredictable compliance are indications for admission
and surgery as soon as any necessary preoperative treat-
ment is completed. Evaluation for associated cardiac
172
/ Advanced Therapy in Thoracic Surgery
FIGURE 13-12. Congenital lobar emphysema (CLE): Plain radiograph
showing a left-sided CLE with hyperinflation, mediastinal shift, and
displacement of the cardiac silhouette towards the right hemithorax.
Lung markings are present throughout the left hemithorax and no
collapsed lung edge is evident which differentiates this from a
tension pneumothorax.
cells; occasional elements of skeletal muscle can be found
suggesting a hamartomatous condition. The most
commonly used classification for CCAM divides the
malformations into three types based on the cyst charac-
teristics.
24

Type I is macrocystic disease and represents 60
to 70% of CCAM; 50% of type I lesions occur on the left,
35% occur on the right, and between 2 and 14% are
bilateral.
25,26
This type of CCAM is composed of single or
multiple cysts (usually between one and four) that are
more than 2 cm. The cyst walls may include smooth
muscle cells and are lined with ciliated pseudostratified
columnar epithelium. Type II CCAM, which occurs 20%
of the time, is commonly called mixed disease and
contains adenomatoid material mixed with small and
medium-sized cysts of about 1 cm. In this case, alveolar
type tissue is found among the cysts that are lined with
ciliated cuboidal or columnar epithelium. Congenital
anomalies (which occur in up to 70% of type II CCAM)
and prematurity (up to 75%)
23
are almost exclusively
associated with this type of CCAM, accounting for its
poorest prognosis. Type III disease occurs 10% of the
time and is referred to as microcystic CCAM. This
subtype presents more often in boys. Its appearance on
ultrasound is echogenic due to solid components, and
this firm mass tends to involve an entire lobe. It is com-
posed of mainly adenomatoid bronchioalveolar-like
tissue with minimal amounts of small cysts but there is
no normal lung. Some authors classify CCAM into two
types.
27

The macrocystic type has single or multiple fluid
filled cysts greater than 5 mm, and the microcystic type
has solid components in combination with cysts less that
5 mm.
As with other chest occupying lesions that lead to
compression or lung hypoplasia, the most common
symptom is respiratory distress. In situations where
mediastinal displacement has occurred, decreased venous
return can lead to cardiac collapse and further dimin-
ished function of the contralateral lung. In utero, this
mass effect leads to lung hypoplasia and to hydrops;
compression of the esophagus can also lead to polyhy-
dramnios. The severity of symptoms depends on the
degree of hypoplasia. In the newborn period severe respi-
ratory and cardiovascular symptoms develop from lung
hypoplasia and mediastinal shift. In older children,
recurrent infection is the most common symptom. With
longstanding disease, bronchioalveolar cancer and rhab-
dosarcoma have been reported.
28
Some infants with
prenatal diagnosis are asymptomatic at delivery and may
even have radiologic regression or disappearance of the
mass. Most have varying degrees of respiratory symp-
toms at birth. Most diagnoses are made before age 6
months, and 60% are made within the first month. One-
half of CCAMs will present in the newborn period either
with symptoms or as a result of prenatal screening; also,
type II CCAM babies will often present with prematurity.
The 50% of infants that present after the newborn period

fare much better with their milder CCAMs. These chil-
dren may not have a lesion that causes respiratory
distress at rest, but during times of stress, such as with
upper respiratory infections, increased activity, or some-
times feeding, they will become symptomatic. At these
times they may present with tachypnea or wheezing, and
on auscultation may have decreased breath sounds over
the affected lobe. Infection can also occur with cough
and fever, and a pattern of recurrent infections usually
leads to chest roentgenogram. Failure to thrive is another
presentation prompting a search for pulmonary afflic-
tions. Sometimes the diagnosis is made incidentally as
patients undergo imaging for other reasons. The chest
radiograph may reveal the characteristic cystic mass or
an infiltrate. It may take recurrent infiltrates in the same
lobe to trigger studies such as CT to diagnose an underly-
ing CCAM.
CCAM is the most commonly diagnosed congenital
lung anomaly because its cystic appearance makes it
readily apparent on prenatal ultrasound. Prenatal diag-
nosis is therefore common and CCAMs are detectable on
ultrasound by 12 weeks’ gestation. Ultrasound typically
demonstrates a macrocystic, microcystic, or solid lesion
and may show displacement of the heart or diaphragm.
Polyhydramnios is seen in 65% of cases and is ominous.
Hydrops and ascites can be seen in up to 45%. The diag-
nosis in babies after delivery is usually discovered after
the infant has plain chest film for respiratory distress or
unequal breath sounds; up to 15% of babies are diag-
nosed after age 6 months. The chest radiograph will have

a mass that may appear typically macrocystic or may
appear solid in nature if there is microcystic disease
Surgical Management of Congenital Lesions of the Lung
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175
FIGURE 13-15. Congenital cystic adenomatoid malformation:
Dysplastic overgrown bronchioles of congenital cystic adenomatoid
malformation. 12.5ϫ magnification; hematoxylin/eosin stain.
(Figure 13-16). The appearance of CCAM on plain chest
film may be deceptive; a frequent mimic of CCAM is
congenital diaphragmatic hernia, which has a very differ-
ent treatment algorithm. Placement and visualization of
a nasogastric tube in the chest differentiates the two
thoracic anomalies. Infants with prenatal diagnosis of
CCAM should have confirmation of its persistence, as
there are reports that some CCAMs seen on prenatal
sonography are no longer apparent on postnatal imaging.
Examination with ultrasound or CT helps to assess the
amount of involved lung and to differentiate CCAM
from mediastinal and other primary lung lesions such as
sequestration, bronchogenic cyst, diaphragmatic hernia,
or lymphangiectasis. In the newborn period, an ultra-
sound to confirm that the mass is a parenchymal CCAM
may be the only necessary study (Figure 13-17). It will
also indicate whether the lesion is microcystic or macro-
cystic (Figure 13-18). Although uncommon, vascular
abnormalities can occur with cystic adenomatoid malfor-
mations and one should look for this on ultrasound. As
the age at presentation increases, chest CT becomes more
important and effective at deciphering the growing

differential of infectious etiologies, malignancies, and
foreign bodies. Many surgeons prefer CT imaging
176
/ Advanced Therapy in Thoracic Surgery
FIGURE 13-16. Congenital cystic adenomatoid malformation (CCAM): Plain radiograph demonstrating multiple cysts in a left-sided CCAM. There
is minimal mediastinal displacement. Multiple cysts can appear similar to a congenital diaphragmatic hernia.
FIGURE 13-17. Congenital cystic adenomatoid malformation, multi-
cystic: Ultrasound. Bottom ridge represents the vertebral column. The
left side of the image is cephalad.
FIGURE 13-18. Congenital cystic adenomatoid malformation (CCAM):
Ultrasound. The vertebral column can be seen traversing the image
and a large hypoechoic area represents the normal lung. The two
small hypoechoic areas are the CCAM, and the echodense area
bordering the left side of the image is the liver.
because of the axial images and their own familiarity
with interpreting this test (Figure 13-19).
The treatment for CCAM even in asymptomatic
patients is surgical resection before the development of
infectious or malignant complications (Figures 13-20
and 13-21). Babies with prenatal diagnoses should be
cared for at a tertiary medical center and followed with
ultrasound every 2 weeks to watch for the development
of hydrops. Hydrops is the most ominous sign of poor
outcome, and affected infants have severe lung hypopla-
sia; in the past almost all babies with hydrops died (up to
70% in utero and 90% that were delivered). Hydrops also
precipitates preeclampsia, and premature delivery further
exacerbates the situation with lung disease of prematu-
rity.Fetal echocardiography should be performed to
identify any concomitant cardiac defects and help

provide a prognosis. Today, parents have the following
options: postnatal care, fetal intervention, or termination
for moribund cases. In considering fetal procedures and
their risks, it is important to know that even in cases with
hydrops, regression of the CCAM can occur in up to 15%
of cases. Also, with modern resuscitation, ventilatory
techniques, prepartum steroids, and extra corporeal
membrane oxygenation (ECMO), fetuses without
hydrops or other lethal anomalies have almost 100%
survival and fetal intervention cannot be routinely
recommended. For this reason, consideration of fetal
intervention can effectively be restricted to those with
early development of hydrops. Even before the advent of
open fetal surgery and fetoscopic techniques, fetal inter-
vention has been available via cyst aspiration and thora-
coamniotic shunting and these techniques have been
reported as early as 1988.
29
Cyst aspiration requires repeat
drainage as the thoracic cavity rapidly fills with fluid to
occupy the space created by the cyst aspiration
27
;this is
therefore not a good long-term solution. Thoracoamni-
otic shunting has been successful in a small number of
hydropic patients with macrocystic disease who have a
dominant cyst amenable to drainage; however, shunt
dislodgement or occlusion can be problematic. In a
report of thoracoamniotic shunt use in six fetuses with
hydrops and macroscopic disease with a predominant

large cyst, five of six patients survived and the one death
was due to premature rupture of membranes followed by
precipitous delivery. This underscores the point that even
seemingly less invasive fetal procedures disturb the
intrauterine environment and are not without significant
risk of premature delivery.
30
Fetal thoracotomy with
surgical resection of the CCAM continues to evolve. A
review of 134 fetuses with CCAM over a 15-year period
confirms the principle that fetal procedures be limited to
those with hydrops. Fourteen of the fetuses underwent
Surgical Management of Congenital Lesions of the Lung
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177
FIGURE 13-20. Congenital cystic adenomatoid malformation:
Intraoperative photo.
FIGURE 13-21. Congenital cystic adenomatoid malformation: Specimen.
FIGURE 13-19. Congenital cystic adenomatoid malformation, left-
sided: Computed tomography scan.
elective abortion and the remaining 120 were analyzed.
One hundred and one fetuses were managed expectantly
with delivery and any surgical intervention done postna-
tally; every hydropic fetus died or expired shortly after
delivery, and all 76 nonhydrops babies survived, although
4 required ECMO support. This further verifies that
nonhydrops fetuses have good prognosis, but hydrops is
lethal without prenatal intervention. In the same study,
19 fetuses underwent fetal procedures; 6 fetuses were
discussed above regarding thoracoamniotic shunting.

The remaining 13 fetuses underwent thoracotomy and
lobectomy between gestational weeks 21 and 29 done
through a hysterotomy. Survival of the hydrops fetuses
after open fetal surgery was 62% and hydrops resolved
within 2 weeks, mediastinal shift was gone after 3 weeks,
and there was evidence of substantial lung growth. All 5
of 13 deaths were attributable to inability to maintain the
pregnancy either during the procedure or in the periop-
erative period.
27
Preterm labor following fetal surgery and
pulmonary hypoplasia continue to be responsible for the
morbidity and mortality in this technique. These data
demonstrate that without hydrops the risk of fetal inter-
vention outweighs the benefit and these infants should be
treated after delivery with an attempt to carry the fetus to
term.Other authors have confirmed these data.
30–33
In
cases of the “mirror” syndrome of maternal hyperdy-
namic state or after placentomegaly develops, there were
no survivors of fetal interventions due to inability to
sustain the pregnancies. Therefore, fetal intervention is
not indicated and in these situations or in the face of
concomitant lethal anomalies, some families have opted
for termination. If hydrops develops after 32 weeks or if
the biophysical profile of an otherwise viable fetus deteri-
orates (greater than 24 weeks’ gestation without other
lethal conditions) early delivery is indicated. Early deliv-
ery occurs in up to 50% of CCAMs and is often the result

of preterm labor that cannot be treated. Steroids should
be given to enhance lung maturity if the fetus is less than
32 weeks’ gestation. The majority of these preterm
infants will require immediate intubation and resuscita-
tion. Severely premature infants (less than 29 weeks) will
have not only CCAM-related lung hypoplasia, but also
lung disease of prematurity to add to their respiratory
compromise, and a significant number of these infants
will be dependent on such high ventilator settings to
sustain marginal respiratory parameters that they will
not tolerate thoracotomy. If there is any indication that
the CCAM and its mass effect on respiratory mechanics
are the predominant cause of respiratory failure, resec-
tion should be undertaken immediately and the condi-
tion may improve; however, this is not always clear and if
the pulmonary insufficiency is mostly related to lung
hypoplasia or prematurity, resection will not bring swift
improvement, and positioning or retraction necessary for
surgery may be more than the baby can tolerate. In addi-
tion, as with in utero CCAMs, these lesions may decrease
in size after birth. In very premature, low birth weight
babies whose primary lung disease or pulmonary hyper-
tension is the main cause of respiratory failure, resection
should be postponed until the infant is fit for thoraco-
tomy under general anesthesia from a respiratory stand-
point. In some premature infants clinical reasoning
becomes somewhat circular, with resection precluded
due to lung disease of prematurity and slow improve-
ment in pulmonary status in part potentially due to the
remaining mass. There are some maneuvers to improve

respiratory mechanics while waiting for surgery. Placing
the child with the symptomatic side down may decrease
distension on the affected side by limiting aeration and
jet or high-frequency ventilation minimizes hyperinfla-
tion and decreases traumatic high airway pressures in
premature lungs. Associated life-threatening conditions,
especially cardiac conditions, must be addressed, and the
congestive cardiac failure must be aggressively treated
with inotropes and diuresis. Unfortunately some babies
have such significant hypoplasia that they do not
improve. ECMO can be used to support infants with
severe pulmonary hypoplasia, and surgical resection of
the CCAM can even be performed while on ECMO. The
infants must weigh more than 2 kg to utilize ECMO and
must be fully heparinized. Inability to wean from bypass
by approximately 2 weeks indicates pulmonary hypopla-
sia so severe that the infant is unsalvageable and bypass
support is withdrawn.
Te rm or near-term infants with a prenatal diagnosis of
CCAM should undergo chest radiograph because there
may be regression or even disappearance of the mass. If
the mass is not evident on postnatal plain film, ultra-
sound or CT should be done; if there is still no evidence
of a mass, patients are followed with serial ultrasound.
Evidence on future exams of a mass warrants excision.
Those with a clear lesion on imaging but mild or absent
symptoms can undergo resection electively if no mediasti-
nal shift develops over the first 48 hours. Mediastinal
shift, tachypnea, oxygen requirement, or ventilator depen-
dence should prompt resection as soon as preoperative

preparation can be completed and more urgently if symp-
toms dictate. Cardiac lesions should be anticipated and
sought with echocardiogram, especially with type II
lesions, in which associated congenital anomalies are
common. Children and adults with asymptomatic or inci-
dentally noted CCAM should undergo resection electively
not only because there is documented risk of malig-
nancy,
28
but also because infectious complications that
would likely develop can thereby be avoided. Technically,
resection of CCAM usually requires lobectomy, but in up
178
/ Advanced Therapy in Thoracic Surgery
to 15% pneumonectomy may be required. This is more
common in type III CCAM as the more diffuse and
microscopic nature may make it difficult to separate from
surrounding normal lung and because type III disease has
a propensity to involve the entire lung. Very rarely,
segmental resection of the CCAM is possible along with
its tributary tracheobronchial tree. The airways and
vessels follow a normal anatomic configuration, although
there may be additional systemic arterial supply similar to
that in sequestration. Significant anesthetic difficulties
regarding airway deviation or hyperinflation are rare and
mild compared with CLE, but they must be anticipated.
Pulmonary hypoplasia and lung disease of prematu-
rity are the most important factors in determining
outcome. There is a tremendous range in survival
(11–95%)

25
and fetal hydrops portends the poorest prog-
nosis. Uncomplicated macrocystic (type I) CCAM has
survival rates of 70 to 95%. Types II and III CCAMs have
the poorest prognosis (< 50% survival
23
) due to frequent
associated cardiac defects, severe lung hypoplasia, the
incidence of prematurity in type II lesions, and the
increased incidence of diffuse lung involvement in type
III. In addition, type II CCAM is more frequently associ-
ated with in utero hydrops and polyhydramnios, which
both independently worsen prognosis. Medical comor-
bidities not only contribute independent physiological
complications but also increase anesthetic risk and some-
times postpone surgical resection. Fetal hydrops is seen
in up to 45% of fetuses with CCAM and is a grave prog-
nostic sign; in 68% of these there will be fetal demise,
and of the remaining 32% that are delivered, 89%
succumb, making the outcome with hydrops almost
universally fatal. Polyhydramnios itself is associated with
50% mortality.
25
Of all CCAMs diagnosed in the prenatal
or newborn period, up to 60% will die because this
includes the significant number of cases with fetal
hydrops or polyhydramnios with in utero death and
those with associated anomalies that die. However, of the
infants that survive to delivery, since the remaining group
has very low incidence of hydrops and is comprised

mainly of the favorable CCAM subtypes, survival of 80 to
100% has been reported.
4,34
Infants without hydrops,
associated severe prematurity, or associated anomalies
have a survival of up to 90%. Children who have delayed
presentation with infection fare the best and have essen-
tially 100% survival. The advent of fetal surgery shows
promise in improving the survival for cases of fetal
hydrops in which demise was previously almost certain,
and the survival for fetuses with hydrops may be as high
as 74% with fetal intervention.
27
Unfortunately, intraop-
erative contractions, maternal hyperdynamic state, and
preterm delivery still lead to fetal demise up to 25% of
the time.
27
Cases without evidence of hydrops have a
more favorable prognosis, and regression of the lesion
occurs in up to 15%; all nonhydropic fetuses analyzed in
the literature survived with postnatal treatment, and fetal
surgery cannot be routinely recommended for these cases
at this point. The ability to predict more accurately which
fetuses will have significant symptoms after birth and,
more importantly, development of techniques to prevent
surgically induced preterm delivery may expand the indi-
cations for this modality.
Pleural Effusion
Fluid in the pleural space occurs in 1 in 10,000 newborns

and has a male predilection (2:1). There are no known
associated teratogens but effusions have been associated
with a variety of other anomalies and genetic syndromes
including Down syndrome, Turner syndrome, Caffey
hyperostosis, Opitz-Frias syndrome, congenital
pulmonary lymphangiectasis, esophageal atresia, and
extralobar sequestration.
35,36
Congenital effusions may be
primary (chyle) or secondary (nonimmune hydrops or
hypoalbuminemia), and most are found on the right side
although effusions can occasionally be bilateral. Primary
chylous effusions are of unclear etiology, although some
derangement in either increased production or decreased
absorption must be involved. Reduced lymphatic
drainage may come from inadequate communication
between pulmonary lymphatic channels and their main
thoracic drainage; this may be a result of congenital
absence, birth injury, or obstructing mediastinal mass.
Lymphatic abnormalities are not usually found in other
organs with the exception of rare associated lymphang-
iectasis.
Congenital effusions can become very large and cause
ipsilateral lung hypoplasia due to in utero pulmonary
displacement. Situations in which the effusion causes
mediastinal shift may lead to hydrops by impairing
venous return and cardiac output. Many infants will have
prenatal diagnosis and will have respiratory distress at
birth. Most other infants present within the first several
days of life with varying degrees of respiratory distress.

Infants with mild symptoms present with decreased
breath sounds on the affected side, and plain chest film
quickly identifies the effusion as an opacified hemithorax
and caudad displacement of the ipsilateral diaphragm.
Primary pleural effusions may demonstrate downward
displacement of the ipsilateral diaphragm but secondary
effusions will not and this helps to distinguish between
the two. The appearance of the pleural fluid may by
deceiving, as chylous effusions may appear clear if the
patient has no enteral fat intake. A cell count demon-
strating more than 60% lymphocytes supports chylotho-
rax; triglycerides are usually greater than 200 mg/dL,
Surgical Management of Congenital Lesions of the Lung
/
179
specific gravity is usually greater than 1.012, protein
levels are about 40 g/L, and glucose has serum levels.
37
Pleural effusions can be detected on prenatal ultra-
sound by 16 weeks’ gestation; their discovery should
prompt investigation of fetal heart function (as an etiol-
ogy for secondary effusion or for associated anomalies),
thoracic masses, and chromosomal abnormalities, and
serial ultrasound should be performed every 2 weeks to
watch for development of hydrops. If there is evidence of
hydrops, aspiration should be performed as often as
necessary to control the hydrops. Pleuroamniotic (thora-
coamniotic) shunt placement may obviate multiple aspi-
rations. These shunts often become occluded or
dislodged, so continual sonographic monitoring and

drain replacement must be done to assure proper
drainage. Early delivery is not recommended unless
refractory hydrops develops or if gestation is greater than
32 weeks. Pleural effusions may also resolve over time in
utero especially in cases of small unilateral collections,
but this is uncommon. For large primary effusions with-
out hydrops in which respiratory compromise at delivery
is a concern, aspiration shortly before delivery should be
done. Unless there has been prenatal drainage, most
infants with sizeable pleural effusion will need to be intu-
bated and one should also be prepared to perform emer-
gent thoracentesis. Intubation should be the standard
with respiratory distress, bilateral effusions, or for any
premature baby with effusions. Infants who are relatively
asymptomatic (mild tachypnea, minimal oxygen re-
quirements) should be followed with serial chest
roentgenogram; resolution of mild effusions may occur
as intrapleural pressures change or with treatment of
associated conditions (ie, diuresis and inotropic
support). Worsening of symptoms (ventilator depen-
dence, significant tachypnea, mediastinal shift, or cardio-
vascular compromise) in patients who do not respond to
medical treatment mandates drainage. Initially, this may
be done via aspiration, which may need to be repeated
every few days. If multiple aspirations become necessary,
a drainage tube can be placed. Secondary effusions will
subside with treatment of their causative conditions.
Primary, chylous effusions may be more recalcitrant.
Chylothorax due to surgical injury heals quite readily
with decompression and dietary measures; therefore, we

infer that lymphatic injuries resulting from birth trauma
may respond similarly. If there is a mass obstructing
lymphatic drainage, its removal will enhance normal
drainage and the effusion should resolve. Cases without
known etiology or those in which congenital lymphatic
defects are suspected may be the most difficult to treat.
An elemental diet can be given orally as long as improve-
ment in the effusion is noted and protein losses are not
causing malnutrition. Persistent accumulation requires
parenteral nutrition to minimize lymph volume and to
support the protein losses in the effusion. It is essential to
remember that T cells and thereby immunity are also
decreased because of these lymphatic losses. These
management strategies are usually successful but if
drainage does not improve for more than 2 weeks, surgi-
cal ligation of the thoracic duct proximal and distal to
the leak should be performed
38
and can be accomplished
via open thoracotomy or thoracoscopic techniques. Pre-
operatively, a fat-laden meal may increase drainage and
help identify the leak; alternatively, 1% isosulfan blue dye
can be used. Preoperative lymphoscintigraphy in such
small patients is usually nonspecific and rarely helpful.
Surgical ligation is usually successful; the addition of
fibrin glue may fortify the repair but is unproven. There
are reports of microvascular lymphatic repair in thoracic
duct injuries; however, there are no reports documenting
efficacy of repair in the miniscule lymphatics specific to
congenital chylothorax or in the neonatal age group. In

the event of surgical failure, an unidentifiable duct leak,
or a diffuse leak, pleuroperitoneal shunting can be done
for decompression.
As with many congenital thoracic problems, the
outcome is dependent on associated factors such as fetal
hydrops, prematurity, cardiac disease, pulmonary hypo-
plasia, and associated genetic syndromes. The outlook
after in utero diagnosis is poor, with 50% mortality.
Hydrops carries the gravest prognosis with 95% mortal-
ity; babies with polyhydramnios (42%) also have bad
outcome and bilateral cases often suffer fetal demise. The
infants that survive to term represent cases with favorable
prognosis without hydrops, bilaterality, or polyhydram-
nios. The overall survival, therefore, for babies born with
congenital pleural effusions is 75%, and babies born
without associated anomalies have an 85% survival.
Congenital Pulmonary
Lymphangiectasis
Congenital pulmonary lymphangiectasis or lymphang-
iectasia is a condition in which dilated lymphatics occupy
the bronchovascular space, the pleura, and the interlobu-
lar septa. During the fifth month of gestation, pulmonary
interstitial connective tissue fails to diminish and
lymphatic channels become occluded; this leads to the
formation of diffusely dilated cystic lymphatics.
39
The
condition is very rare and found twice as often in boys as
girls. It can occur in conjunction with lymphedema and
there have been associations with congenital cardiac

diseases and Noonan, Ullrich-Turner, and Down
syndromes.
40
Conditions which increase lymphatic
volume and circulation exacerbate lymphatic dilation.
Primary congenital lymphangiectasis can be distin-
180
/ Advanced Therapy in Thoracic Surgery
guished from a secondary form of the disease by its
presentation in the neonatal age group and the dismal
clinical course. Many fetuses are stillborn, and any
infants that come to term present with severe respiratory
failure and pleural effusion. Plain chest film demon-
strates effusion with diffuse granular parenchyma,
prominent interstitial markings, and variable hyperinfla-
tion in one or both lungs.
41
Aspiration of pleural fluid
will yield lymph and sometimes biopsy is necessary to
confirm the diagnosis. Treatment is supportive with
drainage of pleural effusions, low-fat high-protein diet,
and medium-chain triglyceride supplementation aimed
at limiting the volume of lymphatic flow and thereby the
distension of the lymphatics. Heart and lung transplanta-
tion has been performed but has not shown encouraging
results with respect to this disease entity.
39
Unfortunately,
despite these measures, outcome is often fatal early on
due to the significant pulmonary hypoplasia.

Pulmonary Lymphangiomatosis
Pulmonary lymphangiomatosis is a condition in which
there exist multiple lymphangiomas within the lung
parenchyma as well as concomitant lymphatic disorders
in up to 75% of patients.
39
The exact developmental
abnormality that leads to the appearance of these lym-
phangiomas is unknown, but there is increased prolifera-
tion of interconnected lymphatic channels within the
mediastinum and lung parenchyma with surrounding
parenchyma that is normal in architecture. Histologically,
there may be isolated lymphangiomas or a more diffuse
pattern that, although invasive in appearance, consists of
mature cells and is benign. The condition is very rare and
the occurrence is equal between sexes. Lesions are bilat-
eral and generally located in the mediastinum, pleura,
chest wall, parenchyma, or bone. Lymphangiomatosis
usually presents late in childhood with symptoms of
wheezing or respiratory distress. It can also manifest as
chylous pleural effusions, chylopericardium, chylous
ascites, chyloptysis, protein losses, lymphopenia, and
even disseminated intravascular coagulopathy. Chest
radiograph will demonstrate bilateral interstitial infil-
trates, pleural effusions, and pericardial effusions, and
pulmonary function tests may show both an obstructive
and restrictive pattern. On CT imaging, the pulmonary
septa are thickened and the mediastinum and perihilar
regions can be encased. Lymphangiography will reveal
many lymphangiomata associated with the thoracic duct,

lung, and bone. The combination of chylothorax and
lytic bone lesions is suggestive of pulmonary lymphan-
giomatosis.
39
Biopsy is diagnostic and special endothelial
stains such as factor VIII–related antigen and CD31 vari-
eties help identify this lesion.
39
Over time these lesions
continue to grow relentlessly and cause compression
phenomena. Treatment is palliative and again methods to
decrease lymphatic volume and chylous effusions should
be employed. Surgical resection is plagued by high recur-
rence, as complete excision without sacrifice of essential
structures is infrequently possible. Sclerotherapy with
doxycycline is often employed to palliate the effusions
and attempt to minimize protein losses. This difficult-to-
treat condition is mostly fatal.
Pulmonary Hemangiomatosis
Pulmonary hemangiomatosis is a locally invasive but
benign vascular tumor of the lung parenchyma. Capillary
proliferation of thin-walled vessels in the pulmonary
interstitium is characteristic on histopathological review.
The condition is very rare with few reported cases.
Patients present with respiratory distress or pulmonary
hypertension, and there can be an associated consump-
tive coagulopathy (Kasabach-Merritt syndrome) or
hemolytic anemia. Treatment is supportive including
pulmonary toilet, treatment of concurrent infections,
and nutritional optimization. Additional modalities that

have been employed in attempts to eradicate the disease
or halt its progression include steroids, cytotoxic agents,
radiation, laser ablation, embolization or resection of
localized disease, and cryotherapy. Unfortunately, none
have had great results and come at the expense of many
significant side effects or invasive procedures. Most
recently, interferon-␣ has been used with encouraging
results; although there can be hemodynamic effects in the
first 48 hours, side effects are otherwise minimal.
42
The
mechanism of interferon action is probably as an antian-
giogenesis agent via inhibition of proliferating smooth
muscle, endothelium, and fibroblasts. Lung transplanta-
tion has been employed in a few cases that progressed to
unrecoverable pulmonary failure.
43
Previously this condi-
tion was considered universally fatal, but the advent of
antiangiogenesis therapies and lung transplantation for
unresponsive cases may warrant cautious optimism.
Primary Pulmonary Hypoplasia and
Agenesis
Pulmonary hypoplasia exists in varying degrees and may
be primary or secondary; in its most severe form it is
called pulmonary agenesis and there is an absence of
parenchymal and bronchial structures on one side.
Secondary pulmonary hypoplasia is caused by conditions
that mechanically restrict lung development by occupy-
ing the thoracic cavity, by limiting development of the

thorax (thoracic dystrophies), or by impeding amniotic
fluid return (renal impairment or neuromuscular dys-
Surgical Management of Congenital Lesions of the Lung
/
181
function of diaphragmatic excursion). These lesions are
as common as the above-mentioned etiologies. Primary
hypoplasia and agenesis are very rare and thought to be
due to failure at various stages of development in
bronchial budding from the trachea. There does not
seem to be a gender preference. A multitude of associated
congenital anomalies have been associated with primary
pulmonary hypoplasia and agenesis, namely intracardiac
defects, esophageal atresia, and genitourinary anomalies.
Primary hypoplasia may be unilateral or bilateral and
both sides are affected equally; agenesis only slightly
more often affects the left side and is unilateral although
it may be associated with contralateral hypoplasia. In the
cases of agenesis, up to 50% have associated anomalies
and in most cases patients with concomitant congenital
defects have a right-sided agenesis. Agenesis is extremely
rare,with a worldwide experience of only several
hundred cases.
Some infants are asymptomatic and diagnosed via
work-up of another congenital condition or on routine
physical exam that demonstrates decreased breath
sounds on the affected side with possible mediastinal and
tracheal displacement. Older children may also have
evidence of chest asymmetry or scoliosis. Some present
with an antenatal diagnosis or as a result of incidental

findings on unrelated imaging studies. Patients who
present with respiratory failure do so in a continuum of
severity from exercise induced dyspnea or wheezing, to
marked tachypnea and cyanosis. For unclear reasons,
infectious complications represent the predominant
presentation, although there is no anatomic basis for
postobstructive phenomena. It is postulated that the
airway has decreased clearance mechanisms.
2
The diag-
nosis is apparent on plain film and shows an empty
hemithorax with contralateral lung hypertrophy displac-
ing the mediastinum to the affected side so that it occu-
pies some of the empty hemithorax. Vertebral anomalies
are also common.
44
This can be differentiated from a
tension pneumothorax as the mediastinal structures are
deviated toward the hypoplastic side instead of being
pushed to the contralateral side by air pressure. Since it is
not possible to discern exactly the anatomy of the airway
on plain film, it is possible that severe atelectasis from a
variety of causes, especially main stem occlusion, could
appear similarly. Confirmation that the bronchus is
absent must be obtained via bronchoscopy, which is the
most sensitive test. Ultrasound, CT, or MRI will also
provide this information but are unnecessary as bron-
choscopy is mandatory and conclusive in any child who
can tolerate the procedure. In the event that there is
unacceptable anesthetic or surgical risk (ie, respiratory

compromise from infection, coagulopathy, or associated
cardiac disease), imaging studies that obviate general
anesthesia may be used as confirmatory studies.
Historically, bronchography was performed with contrast
but posed significant risk to the contralateral lung. Its use
has been obviated by the techniques discussed above.
Although the treatment of pulmonary agenesis will not
be altered by these confirmatory studies, it is important
to treat any other causes of lung collapse that are revealed
and in this way bronchoscopy may be therapeutic as well.
Patients who undergo echocardiography or cardiac
catheterization for cardiac disease will demonstrate a
pathognomonic absence of the ipsilateral pulmonary
artery.
The treatment of primary hypoplasia and agenesis
centers on exceptional pulmonary toilet and prevention
of infection in the solitary lung. Infections of a single
lung are life threatening and must be immediately and
aggressively treated with antibiotics, chest physiotherapy,
nutritional support, and appropriate bronchodilators.
Surgical intervention is generally limited to broncho-
scopic confirmation of the agenesis (a normal trachea
and contralateral bronchus will be seen without evidence
of the ipsilateral structures), maintenance of the contra
lateral bronchus, or correction of the many associated
anomalies. The aim is to limit or consolidate procedures
to minimize general anesthesia and to optimize lung
performance. The greatest difficulty is with procedures
that necessitate displacement of the solitary lung as in
tracheoseophageal fistula repair. Cardiopulmonary

bypass can be used for support although these proce-
dures can usually be accomplished by an experienced
team without the use of bypass. Any alternative to
intrathoracic procedures should obviously be considered
and an example with respect to tracheoesophageal fistula
ligation would be transabdominal occlusion of the
gastroesophageal junction via ligature with concomitant
gastrostomy (D. J. Y. Dunn, personal communication).
Although most patients who have hypoplasia related to
thoracic dystrophy die due to insurmountable congenital
conditions, a few may benefit from thoracoplasty to
distract and enlarge the thorax. The use of lung trans-
plantation is not reported for pulmonary agenesis.
2
In the past, up to one-half of the children born with
pulmonary agenesis died before age 5 years from compli-
cations of pulmonary infection or associated ano-
malies
4,45
; the increased incidence of additional congenital
defects in right-sided agenesis compounds the difficulty.
Recently, with the advent of more powerful antibiotics,
sophisticated modes of ventilation, and improvements in
the treatment of cardiac disease, survival seems to be
improving.
182
/ Advanced Therapy in Thoracic Surgery
Alveolar Capillary Dysplasia
Alveolar capillary dysplasia occurs when there is aberrant
development of pulmonary capillaries coupled with

abnormal pulmonary lobules. The pneumocytes and
vessels are separated by an increased distance, and there
may be a misalignment or absence of pulmonary veins
within the pulmonary intralobular septa. Special stains
for CD34 and collagen type IV also support this diagno-
sis. Although there has been one report of a familial case,
it is most often sporadic.
46
Its occurrence is very rare and
uniformly fatal although not necessarily immediately.
Infants have severe persistent pulmonary hypertension.
Definitive diagnosis of alveolar capillary dysplasia is
made upon histopathologic review of the lung biopsy,
and its utility is in providing prognostic information for
the family, as there is no successful treatment. Infants are
usually treated as if they have primary pulmonary hyper-
tension before the diagnosis of alveolar capillary dyspla-
sia is established. This is done using standard measures of
alkalinization, sophisticated ventilatory modes, nitric
oxide, and even ECMO. Although intravenous prostacy-
clin and inhaled nitric oxide have both been shown to
decrease pulmonary vascular hypertension and increase
oxygen saturation, no known modality of treatment has
been shown to effect survival in alveolar capillary dyspla-
sia.
47
The treatment is usually pursued until the infant
succumbs to refractory pulmonary hypertension, and
only after postmortem examination is the diagnosis
made. Alternatively, in a child showing no improvement

despite maximal therapy, if a biopsy is performed and
establishes a diagnosis of alveolar capillary dysplasia the
family can be counseled and care terminated due to the
known fatal outcome of this disease.
Pulmonary Arteriovenous Fistula
Pulmonary arteriovenous fistulas consist of single or
multiple fistulas between pulmonary arterial and venous
channels. There are two types of congenital pulmonary
arteriovenous fistulas. The capillary form is associated
with Rendu-Osler-Weber syndrome in 60% of cases and
consists of multiple capillary telangiectasias; it is inher-
ited in an autosomal dominant pattern and has variable
penetrance. The cavernous type involves one or more
branches of the pulmonary artery feeding a cavernous
angioma.
48
Pulmonary fistulas are extremely rare and less
than 20 case reports of newborn cases exist in the litera-
ture.
48
Only 15% of cases are diagnosed in infancy, and
patients present with a continuum of respiratory distress
symptoms. These range from mild symptoms to club-
bing, polycythemia, cyanosis, and sometimes cardiac fail-
ure from intrapulmonary shunting. The severity of
symptoms depends upon the size and number of vessels
involved in the fistula process. Peripheral arteriolar
involvement has inconsequential respiratory and hemo-
dynamic effect whereas with more central involvement of
larger vessels or more numerous involvement of small

vessels, symptoms may be life threatening. Multiple
episodes of pulmonary hemorrhage are common in the
capillary form. Infectious cerebral issues have also been
described and are thought secondary to emboli that
escape the natural alveolar filter of the lung by traversing
the fistulas. The diagnosis is made from a combination of
clinical and radiographic tests. Arterial blood gases will
reveal varying degrees of right-to-left shunting and venti-
lation perfusion studies will be abnormal. Increased
vascular markings or an interstitial infiltrate are charac-
teristic but nonspecific findings on plain chest film. A CT
scan revealing a confluence of vessels and high attenua-
tion in the lung parenchyma is suggestive of arteriovenous
fistulas, and this can be confirmed via echocardiography,
nuclear medicine study, or pulmonary angiogram. In
echocardiography with agitated microbubbles, the
bubbles to which the capillary alveolar filter is a natural
barrier travel through the fistulas and into the left heart.
Albumin labeled with radioactive tracer similarly is
normally caught in this natural filter and appears in the
lung parenchyma; appearance in the systemic circulation
indicates a shunt is present and the area within the par-
enchyma without tracer demonstrates where the shunt
has bypassed the normal pulmonary deposition. Pul-
monary angiography demonstrating a capillary network
of abnormally shaped vessels not only confirms the diag-
nosis but is also an avenue for treatment via emboliza-
tion. Embolization is very successful for focal lesions and
results immediately in physiologic improvement of arter-
ial blood oxygen saturation.

48
Situations involving diffuse
disease or embolization failures or situations in which
new fistulas open after successful embolization are best
treated with resection, which is curative. The use of inter-
feron has been proposed to control the growth and
proliferation of these arteriovenous fistulas, but there is
no evidence yet that it is successful. Interferon may have
application in situations in which disease too diffuse for
surgical resection.
Conclusions
Congenital lung bud anomalies represent a diverse group
of conditions related by their common developmental
lineage. Their often parallel embryological maturation
leads to similarities among these entities as well as their
occasional synchronous presentation. The physiological
significance of space-occupying fetal thoracic lesions is
their impairment of the developing lung and their ability
to impair cardiac performance through mediastinal shift.
Surgical Management of Congenital Lesions of the Lung
/
183
Surgical resection of congenital lung lesions can be cura-
tive, but outcome is ultimately dependent upon the
underlying pulmonary capacity and associated congeni-
tal anomalies. Fetal surgery has become a feasible alterna-
tive in the treatment of infants with some congenital
anomalies. At the present time it can only be recom-
mended for treatment of congenital lung lesions causing
fetal hydrops in fetuses without evidence of concomitant

lethal conditions. Current investigation within this field
seeks to identify the optimal timing and optimal candi-
dates for intervention, and its success depends upon the
ability to control preterm labor.
Acknowledgments
The authors thank Dr. Andrew Campbell, Chief of
Ultrasound at St. Christopher’s Hospital for Children
and Professor of Pediatric Radiology at Drexel University
College of Medicine, for images of congenital lung
malformations from his personal teaching files and for
his suggestions regarding diagnostic imaging of these
anomalies.
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Surgical Management of Congenital Lesions of the Lung
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185
186
CHAPTER 14
VASCULAR
RINGS AND S
LINGS
RICHARD G. OHYE,
MD
LAURIE C. WILD
, RN, MSN
KHALED H. MUTABAGANI
, MD, PHD
ERIC J. DEVANEY
, MD

EDWARD
L. BOVE, MD
Anomalies of the aortic arch and pulmonary artery have
long been recognized. The first report of a vascular ring
dates back to 1737 with the description of a double aortic
arch by Hommel. Subsequently, in 1794, Bayford linked
posterior esophageal compression and dysphagia with an
aberrant right subclavian artery. He termed the anomaly
lusus naturae or “prank of nature” and the related symp-
toms as dysphagia lusoria.The term “vascular ring” is
credited to Robert Gross, who in 1945 performed the first
surgical repair of a double aortic arch in a 1-year-old
child with tracheal compression. In 1947, Gross also
performed the first innominate artery suspension for
innominate artery compression syndrome.
Pulmonary artery sling was first described by
Glaevecke and Doehle in 1897 based on a post-mortem
finding in a 7-month-old infant with severe respiratory
compromise. Potts reported the first division and reim-
plantation of an aberrant pulmonary artery in 1954.
Embryology
By the end of the fourth week of embryonic develop-
ment, the six aortic or branchial arches have formed
between the dorsal aortae and ventral roots. Subsequent
involution and migration of the arches results in the
anatomically normal or abnormal development of the
aorta and its branches. The majority of the first, second,
and fifth arches regress. The third arch forms the com-
mon carotid artery and proximal internal carotid artery.
The right fourth arch forms the proximal right subcla-

vian artery. The left fourth arch contributes to the
portion of the aortic arch from left carotid to left subcla-
vian arteries. The proximal portion of the right sixth arch
becomes the proximal portion of the right pulmonary
artery, while the distal segment involutes. Similarly, the
proximal left sixth arch contributes to the proximal left
pulmonary artery, and the distal sixth arch becomes the
ductus arteriosum (Figure 14-1).
The pulmonary artery is formed from two vascular
precursors, as well as through a combination of angio-
genesis, the de novo development of new blood vessels,
and vasculogenesis, the budding and migration of exist-
ing vessels. As stated above, the proximal pulmonary
arteries are based upon the sixth arches, whereas the
primitive lung buds initially derive their blood supply
from the splanchnic plexus. Ultimately, these two
segments of the pulmonary artery join and the vascular
network with the lung parenchyma forms (Figure 14-2).
FIGURE 14-1. Normal aortic arch development. AO = aorta; DA =
ductus arteriosum; LCCA = left common carotid artery; LECA = left
external carotid artery; LICA = left internal carotid artery; LSCA = left
subclavian artery; PA = pulmonary artery; RCCA = right common
carotid artery; RSCA = right subclavian artery.
With the transition to solid food, dysphagia becomes
more apparent. Occasionally, older children may present
with symptoms only of dysphagia.
The presentation of a patient with an incomplete
vascular ring is variable. Children with innominate artery
compression usually present within the first one to two
years of life with respiratory symptoms. Although, aber-

rant right subclavian artery is the most common arch
abnormality, occurring in approximately 0.5 to 1% of the
population, it rarely causes symptoms. Classically, when
symptoms do occur, they present in the seventh and
eighth decade, as the aberrant vessel becomes ectatic and
calcified, causing dysphagia lusoria due to impingement
of the artery on the posterior esophagus. An aberrant
right subclavian can cause symptoms in children when
they are of an abnormally large caliber or when associ-
ated with tracheomalacia, as in the one patient in our
series requiring early intervention. However, since an
aberrant right subclavian is rarely the cause of respira-
tory symptoms in children, it is imperative to rule out the
other more frequent etiologies.
Children with pulmonary artery slings generally
present with respiratory symptoms within the first few
weeks to months of life. As with complete rings, respira-
tory symptoms may include stridor, nonproductive
cough, apnea, or frequent respiratory infections and may
mimic other conditions leading to misdiagnosis.
Diagnosis
The methods for diagnosing a vascular ring are variable
because of the variability in presentation and the spec-
trum of diagnostic tests available. A child with a
presumptive diagnosis of asthma or tracheomalacia may
be referred to a pulmonologist and a diagnosis of vascu-
lar ring made or suspected initially by chest radiograph
and bronchoscopy. In some situations, the diagnosis is
made by echocardiography during evaluation for concur-
rent cardiac defects. Regardless, the diagnosis generally

begins with a chest radiograph. Confirmatory studies
may include barium esophagography, computed tomog-
raphy (CT), magnetic resonance imaging (MRI), bron-
choscopy, and echocardiography. Tracheograms and
cardiac catheterizations, which have been used exten-
sively in the past, are rarely currently indicated.
Chest Radiograph
The initial evaluation of a child suspected of having a
vascular ring should include an anteroposterior and
lateral chest radiograph. The study may show unilateral
or bilateral hyperinflation, atelectasis, or infiltrate. The
anteroposterior view will generally reveal the side of the
aortic arch and descending aorta and may suggest a
double aortic arch. The lateral film may reveal tracheal
compression. With a pulmonary artery sling, there may
be anterior bowing of the right main stem bronchus,or
leftward deviation of the distal trachea and carina.
Barium Esophagography
Historically the diagnostic study of choice, barium
esophagography remains a very useful modality. In many
cases, the clinical history and barium esophagography are
sufficient for surgical intervention.
6
The patterns of
external compression on the column of barium are often
characteristic of the various arch anomalies. The findings
on barium esophagography may also be subtle and can
be dependent upon operator experience. The radiologist
should therefore be made aware of the suspicion of a
vascular ring to improve the sensitivity of the examina-

tion. A double aortic arch will show impressions on both
sides of the esophagus on the anteroposterior view. The
right arch is generally higher than the left (Figure 14-3).
The lateral projection reveals posterior compression of
the esophagus (Figure 14-4). A right aortic arch with
aberrant left subclavian will also show posterior com-
pression of the aorta on the lateral view of a barium
188
/ Advanced Therapy in Thoracic Surgery
FIGURE 14-3. Anteroposterior projection of a barium esophagogram
in a patient with a double aortic arch. The arrows indicate the typical
impression of the higher right and lower left aortic arches.
190
/ Advanced Therapy in Thoracic Surgery
Bronchoscopy
Many patients presenting with respiratory symptoms
undergo a bronchoscopy prior to the diagnosis of vascu-
lar anomaly. A double aortic arch and a right aortic arch
with retroesophageal left ligamentum can reveal external
compression of the trachea. Bronchoscopy can be very
useful in diagnosing innominate artery compression
syndrome as there is a specific finding of pulsatile
compression of the anterior trachea one to two centime-
ters above the carina. Compression of the pulsatile mass
with the bronchoscope resulting in loss of the right
upper extremity pulse confirms the diagnosis. The lumen
must be reduced by more than 70 to 80% for the innomi-
nate artery to be the sole etiology of the respiratory
symptoms. Bronchoscopy is mandatory in cases of
pulmonary artery sling to evaluate the patient for the

presence or extent of associated tracheal stenosis.
Bronchoscopy is also useful to rule out other potential
causes of respiratory symptoms, such as tracheomalacia,
subglottic stenosis, or foreign body. As with any patient
with a potentially compromised airway, appropriate care
should be taken during bronchoscopic evaluation.
Echocardiography
Two-dimensional and Doppler echocardiography are also
useful in diagnosing vascular anomalies. Both surface
and transesophageal techniques have been employed to
define the anatomy of the vascular ring, as well as to
demonstrate other associated cardiac lesions. At our
institution, we have found the echocardiogram to be a
useful adjunct. However, significant operator depen-
dence, the inability to assess atretic segments and sur-
rounding structures such as the airways, and the ready
availability of other studies has generally relegated
echocardiography to being a secondary modality.
Treatment
Complete Vascular Rings
double aortic arch
A double aortic arch occurs when the distal portion of
the right dorsal aorta fails to regress (Figure 14-8). The
two arches form a complete ring, encircling the trachea
FIGURE 14-7. Coronal view of a magnetic resonance image in a
patient with an aberrant right subclavian artery showing the artery
(white arrow) originating from the descending aorta and coursing
upward from left to right.
FIGURE 14-8. Formation of a double aortic arch. AO = aorta; DA =
ductus arteriosum; LCCA = left common carotid artery; Lig. = ligamen-

tum arteriosum; LSCA = left subclavian artery; PA = pulmonary artery;
RCCA = right common carotid artery; RSCA = right subclavian artery.
FIGURE 14-6. Coronal view of a magnetic resonance image in a
patient with a double aortic arch showing the two arches (white
arrows) surrounding the trachea and esophagus (black arrow).
and esophagus. The right arch is dominant in the major-
ity of the cases, followed by left dominant, with codomi-
nant arches being the least common (see Table 30–1).
The left and right carotid and subclavian arteries gener-
ally arise from their respective arches. The ligamentum
arteriosum and descending aorta usually remain on the
left.
The approach to repair of a double aortic arch is via a
left posterolateral thoracotomy. The procedure can easily
be accomplished through a limited, muscle-sparing inci-
sion through the third or fourth intercostal space. The
lung is retracted anteriorly and inferiorly, exposing the
posterior mediastinum. The vagus and phrenic nerves are
identified and preserved. The pleura is incised from the
descending aorta up through the left subclavian artery
and retracted with stay sutures. The descending aorta, left
subclavian artery, left arch, right arch, and esophagus are
all positively identified (Figure 14-9). The ligamentum or
ductus arteriosum is circumferentially dissected, doubly
ligated, and divided, with care not to injure the recurrent
laryngeal nerve. The nondominant arch is then divided
between two vascular clamps at the point where brachio-
cephalic flow is optimally preserved. If there is concern
regarding the location for division, the arches can be
temporarily occluded at various points, while monitoring

pulse and blood pressure in each limb. If there is an
atretic segment, the division is done at the point of the
atresia. The ends of the divided arch are oversewn with
two layers of polypropylene suture. Dissection around
the esophagus and trachea in the regions of the ligamen-
tum or ductus and nondominant arch allows for retrac-
tion of the vascular structures and lysis of any residual
obstructing adhesions.
right aortic arch with left ligamentum
arteriosum
There are three anatomic variations for a right arch with
a left ligamentum, depending embryologically on the
segment of the left fourth arch or left dorsal aorta that
regresses. This variability determines where the ligamen-
tum arteriosum and left subclavian artery arise. If the left
fourth arch regresses between the aorta and left subcla-
vian, a right aortic arch with aberrant left subclavian
artery results. The ligamentum arteriosum is retro-
esophageal, bridging the left pulmonary artery and aber-
rant left subclavian, forming a complete vascular ring
(Figure 14-10). If the left fourth arch regresses after the
origin of the left subclavian artery but before the arch
reaches the dorsal aorta to communicate with the left
sixth arch (which becomes the ductus arteriosum), there
is mirror-image branching. The retroesophageal ligamen-
tum arteriosum arises directly from the descending aorta
or from a Kommerell’s diverticulum off of the descend-
ing aorta, forming the complete ring (Figure 14-11). If
Vascular Rings and Slings
/

191
FIGURE 14-9. Intraoperative view of a double aortic arch. DAo =
descending aorta; L = left; R = right.
FIGURE 14-10. Formation of a right aortic arch with aberrant left
subclavian and retroesophageal left ligamentum arteriosum. AO =
aorta; DA = ductus arteriosum; LCCA = left common carotid artery; Lig.
= ligamentum arteriosum; LSCA = left subclavian artery; PA =
pulmonary artery; RCCA = right common carotid artery; RSCA = right
subclavian artery.
FIGURE 14-11. Formation of a right aortic arch with mirror-image
branching and retroesophageal left ligamentum arteriosum. AO =
aorta; DA = ductus arteriosum; LCCA = left common carotid artery; Lig.
= ligamentum arteriosum; LSCA = left subclavian artery; PA =
pulmonary artery; RCCA = right common carotid artery; RSCA = right
subclavian artery.
communication is maintained between the left fourth
and sixth arches, there is mirror-image branching with
the ligamentum arising from the anterior, mirror-image
left subclavian and a ring is not formed (Figure 14-12).
The surgical approach for a right aortic arch with
retroesophageal left ligamentum arteriosum is the same
as for a double arch. After positively identifying the
descending aorta, pulmonary artery, and aortic arch, the
ligamentum is localized (Figure 14-13). The origin of the
ligamentum may be directly from the descending aorta,
from a Kommerell’s diverticulum off of the descending
aorta, or from an aberrant left subclavian artery. The
ligamentum is dissected free, ligated in case of patency,
and divided with care to avoid injuring the recurrent
laryngeal nerve. Any adhesions around the esophagus

and trachea are lysed. Rarely, the Kommerell’s diverticu-
lum has been reported to cause compression even after
division of the ligamentum. As such, it may be prudent
to resect or suspend the diverticulum posteriorly to the
prevertebral fascia, if it is particularly prominent.
Incomplete Vascular Rings
innominate artery compression
In innominate artery compression syndrome, the aortic
arch and ligamentum are in their normal leftward posi-
tion. However, the innominate artery arises partially or
totally to the left of midline (Figure 14-14). As the artery
courses from left to right anterior to the trachea, it causes
tracheal compression. The symptoms of innominate
artery compression may be mild to severe. With mild
symptoms and minimal tracheal compression on bron-
choscopy, children can be observed expectantly as the
symptoms may resolve with growth. Indications for
surgery include apnea, severe respiratory distress, signifi-
cant stridor, or recurrent respiratory tract infection.
Several approaches for the correction of innominate
artery compression syndrome have been described. These
include simple division, division with reimplantation
into the right side of the ascending aorta, and suspension
to the overlying sternum. Suspension is currently the
most widely used technique. This method avoids the
risks of cerebral and right arm ischemia. In addition, by
not performing a circumferential dissection of the
innominate artery, the suspension of the vessel will also
pull up on the anterior trachea. While Dr. Gross first
described the procedure of suspension through a left

anterolateral thoracotomy, adequate exposure is easily
obtained through a limited left anterior, right anterior, or
right inframammary anterolateral thoracotomy. Once the
innominate artery is exposed, no dissection of the artery
is undertaken. Pledged polypropylene sutures are passed
partial thickness through both the innominate artery and
192
/ Advanced Therapy in Thoracic Surgery
FIGURE 14-12. Formation of a right aortic arch with mirror-image
branching and left ligamentum arteriosum, which does not form a
vascular ring. AO = aorta; DA = ductus arteriosum; LCCA = left
common carotid artery; Lig. = ligamentum arteriosum; LSCA = left
subclavian artery; PA = pulmonary artery; RCCA = right common
carotid artery; RSCA = right subclavian artery.
FIGURE 14-13. Intraoperative view of a right aortic arch with aber-
rant left subclavian and patent left retroesophageal ductus arteriosum
rather than the typical ligamentum. L = left.
FIGURE 14-14. Embryological origin of innominate artery compression
syndrome. AO = aorta; LCCA = left common carotid artery; LSCA = left
subclavian artery; PA = pulmonary artery.
Vascular Rings and Slings
/
193
the aorta at the origin of the innominate. Temporary
distraction on the sutures under bronchoscopic guidance
aids in the optimal placement of the sutures in the vessels
and overlying sternum. Once satisfactory reestablishment
of tracheal patency is confirmed by bronchoscopy, the
sutures are brought through the sternum and secured.
Rarely is it necessary to divide and reimplant the innomi-

nate at a more appropriate location on the arch, except
under such extenuating circumstances as concurrent
pectus excavatum.
left aortic arch with aberrant right
subclavian artery
An aberrant right subclavian artery occurs when there is
regression of the right fourth arch between the right
common carotid and right subclavian arteries
(Figure 14-15). The right subclavian then arises from the
leftward descending aorta, laying posterior to the esoph-
agus as it crosses from left to right. Although the artery
can compress the esophagus posteriorly, it is rarely the
cause of symptoms in children. Surgical treatment
involves simple division via a left posterolateral thoraco-
tomy.
Pulmonary Artery Sling
Normally, the right and left sixth aortic arches contribute
to the proximal portions of their respective pulmonary
arteries. If the proximal left sixth arch involutes and the
bud from the left lung migrates rightward to meet the
right pulmonary artery, a pulmonary artery sling is
formed (Figure 14-16). Pulmonary artery slings are asso-
ciated with complete tracheal rings and tracheal stenosis
in 30 to 40% of patients.
8
Origin of the right upper lobe
bronchus from the trachea has been reported in frequent
association with pulmonary artery sling.
3
Initial attempts at the repair of a pulmonary artery

sling involved reimplantation after division of the left
pulmonary artery (LPA) and translocation of the trachea
without cardiopulmonary bypass. These early reports
had a high incidence of LPA thrombosis. This has led
some authors to advocate division of the trachea and
translocation of the LPA. This approach would seem
sensible if the trachea were being divided in the course of
tracheal reconstruction. However, due to the incidence of
airway complications with tracheal reconstruction, it is
difficult to justify dividing a normal trachea. In addition,
anterior compression of the reconstructed trachea has
been reported when the LPA is not relocated onto the
main pulmonary artery.
9
More recently, several authors
have once again advocated the reimplantation of the LPA
with excellent results.
9,10
The procedure is done via a
median sternotomy on cardiopulmonary bypass to
insure optimal visualization of the repair. Aortic cross-
clamping is not necessary. The origin of the LPA from the
right pulmonary artery (RPA) is located and the LPA
mobilized from behind the trachea (Figure 14-17). After
the institution of cardiopulmonary bypass, the LPA is
divided off of the RPA and the proximal end oversewn.
The LPA is then translocated anterior to the trachea and
reimplanted into the main pulmonary artery.
Any necessary reconstruction of the trachea is done
concurrently with bronchoscopic assistance. Many tech-

niques for tracheal reconstruction have been described,
the most common of which are resection with primary
reanastomosis and sliding tracheoplasty for short
segment stenosis and rib cartilage or pericardial patch for
long areas of narrowing. Primary reanastomosis and slid-
ing tracheoplasty have the benefit of not requiring any
patch material but are not suitable for long-segment
stenosis. Rib cartilage has the advantage of structural
rigidity during spontaneous negative pressure ventila-
tion. However, in the very small stenotic neonatal airway,
particularly when there is early take-off of the right
upper lobe bronchus directly from the trachea further
diminishing the size of the distal trachea, a pericardial
FIGURE 14-15. Formation of a left aortic arch with aberrant right
subclavian artery. LCCA = left common carotid artery; LSCA = left
subclavian artery; RCCA = right common carotid artery; RSCA = right
subclavian artery.
FIGURE 14-16. Formation of a pulmonary artery sling. LPA = left
pulmonary artery; RPA = right pulmonary artery.
Vascular Rings and Slings
/
195
Postoperative Management
For most vascular rings and pulmonary artery slings not
requiring tracheal reconstruction, postoperative manage-
ment is routine, with early extubation and short inpa-
tient hospitalization. Occasionally, postobstructive
atelectasis or pneumonia may require prolonged intuba-
tion for ventilatory support and management of secre-
tions and antibiotic therapy. The parents should also be

cautioned that, even in cases of a simple vascular ring,
the respiratory symptoms frequently do not resolve for 3
to 6 months or more.
When concurrent tracheal reconstruction has been
performed, the patient is kept intubated, sedated, and
paralyzed if necessary, with the endotracheal tube and
positive-pressure ventilation serving to stent open the
repair for 1 week. Prior to attempted extubation, bron-
choscopy is performed to assess the repair and identify
any need for removal of granulation tissue or secretions.
Occasionally, tracheostomy with or without long-term
positive-pressure ventilation is required. If significant
portions of the trachea have been resected, hyperexten-
sion of the neck is avoided to prevent traction on the
anastomosis. This may be accomplished by placing a
heavy suture from the chin to the upper chest for the first
week postoperatively.
Results
Mortality for the repair of a vascular ring is 0.5 to 7.6%,
with improved survival occurring in more recent series.
1–4
Most deaths are related to other cardiac defects or respi-
ratory infection and failure. Historically, pulmonary
artery sling has been associated with LPA thrombosis in
up to 90% and death in 50%. However, Backer and
colleagues recently reported a series of 16 patients
repaired utilizing LPA division and reimplantation, all of
whom also required tracheal reconstruction.
10
There were

no operative mortalities, one late death due to respiratory
complications, and 100% LPA patency. The major source
of morbidity, as well as mortality, in this and other series
is related to tracheal reconstruction.
9,10
References
1. van Son JAM, Julsrud PR, Hagler DJ, et al. Surgical treat-
ment of vascular rings: the Mayo clinic experience. Mayo
Clin Proc 1993;68:1056–63.
2. Woods RK, Sharp RJ, Holcomb GW III, et al. Vascular
anomalies and tracheoesophageal compression: a single
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196
CHAPTER
15
THORACIC OUTLET
SYNDROME
HAROLD C. U
RSCHEL JR, MD
, FACP, FACS
, FACC, LLD (HON
), DS (
HON)
A
MIT N. PATEL
, MD, MS
Thoracic outlet syndrome is the compression of the
subclavian vessels and brachial plexus at the superior
aperture of the thorax. It has been previously designated
according to etiologies such as scalenus anticus, costo-

clavicular, hyperabduction, cervical rib, and first rib
syndromes. The various syndromes are similar, and the
specific compression mechanism is often difficult to
identify; however, the first rib seems to be a common
denominator against which most compressive factors
operate.
1–3
The symptoms are neurologic, vascular, or mixed,
depending on which component is compressed.
Occasionally, the pain is atypical in distribution and
severity and is experienced predominantly in the chest
wall and parascapular area, simulating angina pectoris.
4
Diagnosis of nerve compression can be objectively
verified by determining the ulnar nerve conduction
velocity.
5
In vascular compression, the diagnosis is usually
established clinically, rarely requiring the use of angiog-
raphy.
The ulnar nerve conduction velocity (UNCV) test has
widened the clinical recognition of this syndrome and
has improved diagnosis, selection of treatment, and
assessment of therapeutic results.
6,7
Physiotherapy to improve posture, strengthen shoul-
der girdle muscles, and stretch neck muscles is used
initially in most cases of thoracic outlet syndrome and is
often successful in cases of mild compression.
8

Surgical
treatment involves removal of the first rib, usually
through the transaxillary approach, and is reserved for
cases of severe compression that have not responded to
medical therapy.
Anatomic Considerations
The subclavian vessels and brachial plexus traverse the
cervicoaxillary canal to reach the upper extremity. The
outer border of the first rib divides this canal into a proxi-
mal division composed of the scalene triangle and the
space bounded by the clavicle and the first rib—the costo-
clavicular space. The distal division comprises the axilla.
The proximal division is the most critical for neurovascular
compression. It is bound superiorly by the clavicle and the
subclavius muscle; inferiorly by the first rib; anteromedially
by the border of the sternum, the clavipectoral fascia, and
the costocoracoid ligament; and posterolaterally by the
scalenus medius muscle and the long thoracic nerve. The
scalenus anticus, inserting on the scalene tubercle of the
first rib, divides the costoclavicular space into two compart-
ments: an anterior compartment containing the subclavian
vein and a posterior compartment containing the subcla-
vian artery and brachial plexus. The axilla, which is the
outer division of the cervicoaxillary canal, with its underly-
ing structures including the pectoralis minor muscle, the
coracoid process, and the head of the humerus, is also an
area of potential compression (Figure 15-1).
Compression Factors
A variety of factors may cause compression of the
neurovascular bundle at the thoracic outlet. The basic

factor, which was pointed out by Rosati and Lord, is
deranged anatomy to which congenital, traumatic, and
atherosclerotic factors may contribute.
8
Bony abnormali-
ties are present in approximately 30% of patients, either
as cervical rib, bifid first rib, fusion of first and second
ribs, clavicular deformities, or previous thoracoplasty
(Table 15-1).
Hyperabduction Test
When the arm is hyperabducted to 180°, the components
of the neurovascular bundle are pulled around the
pectoralis minor tendon, the coracoid process, and the
head of the humerus. If the radial pulse is decreased,
compression should be suspected.
9
Arm Claudication Test
The shoulders are drawn up and backward. The arms are
raised to the horizontal position with the elbows flexed
90°. With exercises of the hands, numbness or pain is
experienced in the hands and forearms if compression is
present.
Signs and Symptoms
The symptoms of thoracic outlet syndrome depend on
whether the nerves or blood vessels, or both, are
compressed at the thoracic outlet. Symptoms of nerve
compression that most frequently occur are pain and
paresthesia, present in about 95% of patients, and motor
weakness, in approximately 10%. Pain and paresthesia
are segmental in 75% of cases, 90% occurring in the

ulnar nerve distribution.
11
Pain is usually insidious in
onset and commonly involves the neck, shoulder, arm,
and hand. In some patients, the pain is atypical, involving
the anterior chest wall or the parascapular area, and is
termed pseudoangina because it simulates angina
pectoris. These patients have normal coronary arteri-
ograms and decreased ulnar nerve conduction velocities,
strongly suggesting the diagnosis of thoracic outlet
syndrome. The usual shoulder, arm, and hand symptoms
that might have provided the clue for the diagnosis of
thoracic outlet syndrome are initially either absent or
minimal compared with the severity of the chest pain.
Without a high index of suspicion, the diagnosis of
thoracic outlet syndrome is frequently overlooked, and
many of these patients become “pseudo–cardiac cripples”
without an appropriate diagnosis or develop severe
psychological depression when told that their coronary
arteries are normal and that they have no significant
cause for their pain.
The two distinct groups of patients with pseudoang-
ina are as follows. Group I patients have symptoms and
clinical findings suggesting angina pectoris but have
normal coronary arteriograms and significant depression
of UNCV. Group II patients have both significant coro-
nary artery disease, as evidenced by 75% or greater
stenosis in one or more of the major coronary arteries on
coronary arteriography, and thoracic outlet syndrome, as
evidenced by depression of the ulnar nerve conduction

velocity. A high index of suspicion of thoracic outlet
disease in such individuals must be maintained so that
the appropriate methods of diagnosis and management
can be exercised. Objective laboratory tests that are
important for differentiating these two groups of patients
include electrocardiogram, exercise stress tests, coronary
arteriograms, electromyogram, UNCV, cine-
esophagogram, and radiographs of the chest.
4
To understand the symptomatic overlap between
coronary artery disease and this atypical manifestation of
the thoracic outlet syndrome, that is, pseudoangina, it is
necessary to review the neuroanatomy, innervation, and
pain pathways of the arm, chest wall, and heart.
At least two types of pain pathways are present in the
arm—the commonly acknowledged C5 to T1 cutaneous
“more superficial” fibers, and the T2 to T5 afferent spinal
fibers, which travel with the sympathetic nerves and
transmit “deeper” painful stimuli from the ulnar median
and parascapular distribution, as reported by Kuntz.
12
The cell bodies of the two types of afferent neurons are
situated in the dorsal root ganglia of the corresponding
spinal segments. They synapse in the dorsal gray matter
of the spinal cord and the axons of the second order
neurons, cross the midline, and ascend in the spinothala-
mic tract to the brain.
Compression of the “superficial” C8 to T1 cutaneous
afferent fibers elicits stimuli that are transmitted to the
brain and recognized as integumentary pain or paresthe-

sias in the ulnar nerve distribution. In contrast, compres-
sion of the predominantly “deeper” sensory fibers elicits
impulses that are interpreted by the brain as deep pain
originating in the arm or referred to the chest wall.
The pseudoangina experienced in thoracic outlet
compression shares with angina pectoris the same
dermatomal distribution in that the heart, arm, and chest
wall have afferent fibers convergent on T2 to T5 spinal
cord segments and cell bodies that are located in the
corresponding dorsal root ganglia. Referred pain to the
chest wall is a component in both pseudoangina and
angina pectoris. Due to somatic pain being more
common than visceral pain, the brain interprets activity
arriving in a given pathway as a pain stimulus in a partic-
ular somatic area.
Two theories attempt to explain the mechanism of
referred pain from the heart or arm stimuli to chest wall.
The convergence theory holds that somatic and visceral
afferents converge on the same spinothalamic neurons;
when the same pathway is stimulated by activity in visceral
afferents, the signal reaching the brain is the same and the
pain is projected to the somatic area. The facilitation effect
theory holds that because of subliminal fringe effects,
incoming impulses from visceral structures, such as the
heart, lower the threshold of spinothalamic neurons
receiving afferents from somatic areas, so minor activity in
the pain pathways from the somatic areas—activity that
198
/ Advanced Therapy in Thoracic Surgery