1976
JANUARY,
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BRONCHOPULMONARY
FOREGUT
A UNIFYING By
KENNETH
B.
HUGH
MALFORMATIONS*
ETIOLOGICAL
HEITHOFF,
M.D.,
CONCEPT
SHASHIKANT
J. WILLIAMS, M.D., CHARLES JOHN CARTER, M.D., PETER and WILLIAM BRENNOM,
MINNEAPOLIS
AND
ST.
PAUL,
MINNESOTA,
M.
SANE,
J. JARVIS, KANE, M.D., M.D.
AND
OAKLAND,
M.D.,
M.D.,
CALIFORNIA
ABSTRACT:
Two and
cases
of
another
congenital
27 cases
bronchopulmonary
reviewed
and
the
foregut
findings
malformation
analyzed.
entire right lung are the most common locations distal portion of the esophagus or cardioesophageal site of communication (83 percent). The majority
The
left
are lower
of pulmonary junction was of the patients
reported
lobe
and
the
involvement. The the most common (6o percent) pre-
sented in the first eight months of life, and the incidence in females was nearly twice that in males. Chronic cough, recurrent pneumonias and respiratory distress were the most common clinical findings, whereas two patients were totally asymptomatic. The esophagogram was the single most useful diagnostic procedure (82 percent). The microscopic structure of the congenital fistula resembled esophagus, bronchus or
both.
prior
Surgical
treatment
was
to corrective
surgery,
whereas
curative
in most
the
instances.
postoperative
Some
deaths
deaths
in most
occurred
instances
were
related
to severe associated congenital anomalies. We believe a common embryologic pathogenesis leads to the formation of bronchopulmonary foregut malformations. These bronchopulmonary formations include intralobar and extralobar sequestrations, pulmonary
of a variety foregut malsequestra-
tion
communica-
with
tion, cysts.
patent,
or
esophageal
involuted-partial
or gastric
or
diverticula,
complete-gastroesophageal
and
esophageal
or
PATENT,
congenital communications between accessory lung tissue and esophagus or stomach are extremely rare and only 24 such instances are recorded in the literature.4’7’9’’#{176}”2’4”6’’7’2026’29’35’37’38 The purpose of this article is not only to report two such patients we recently had a chance to examine, but also to emphasize that this rare anomaly provides evidence for an interesting pathogenesis monary
and
plausible theory of the gamut of
foregut
malformations
REPORT CASE
a
Presented
American From
in
part
Roentgen the
Children’s
at Ray
the
5eventeenth
Society, Hospitals
San in
Annual Francisco,
Minneapolis,
and
St.
Paul
and
cough
was
initially
coid
material
old male
Children’s
weight
gain,
chronic
cough
but The emesis.
was with
low
intermittent
since
productive
currently
infant,
Hospital
birth.
The
of stringy
mu-
hacking
and
was
cough frequently led to The child fed poorly and
slowly. The feedings did not produce any coughing or gagging episodes. The physical examination revealed a pale, irritable, dyspneic infant weighing 4 kg, tern;
37.6#{176}C, the blood
sounds
decreased the
Society
September Paul,
CASES
a ten week
St.
of slow
72/DIm Breath
California,
OF
B.K.,
fever
perature
of
duplication
to
nonproductive. gagging and
in-
Meeting
history
grade
cluding intralobar and extralobar sequestrations with or without communication with the alimentary tract, foregut duplication cysts, and esophageal diverticula. *
I.
admitted
of common bronchopul(BPFM)
bronchogenic
Minnesota,
46
for 24-27,
but Pediatric
pulse 160/mm, pressure was
over no
the rales
Radiology
1974.
and
Oakland,
California.
right nor and
not
hemithorax rhonchi
the
respiration recorded.
Annual
were could
Meeting
be of
the
126,
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VOL.
No.
Bronchopulmonary
I
Foregut
Malformations
47
heard. A grade 2/6 systolic ejection murmur, heard best at the apex, radiated to the back. Electrocardiography showed biventricular hypertrophy. The liver and spleen were palpable 4 cm below the costal margins. A chest roentgenogram (Fig. i ) demonstrated almost
complete
thorax
with
ment
at
small, throughout
1.
extending
Case
FIG. at
hemi-
seg-
hemithorax
Case
T3-T4
vanced
cylindrical
portion
of
the bronchus bronchiectasis
the
exception
costophrenic
are
I. (A)
level
the
seen
and
right
lower
seen
on
A large
is seen.
bronchus saccular
lung. the
Multiple
this
throughout
view of
of a small
angle.
Esophagogram. to a large
esoph-
Frontal opacification
complete with
the right
at
the proximal
roentgenogram.
almost
bronchograms
2.
from
Chest
.
right
segment air
right
of a triangular
base of the right lung. Multiple, radiolucencies were scattered this opacity. An esophagogram demonstrated a large, esophagobron-
demonstrating the
of the
exception
the round
(Fig. 2A) chial fistula
FIG.
opacification
the
supplying
opacity.
esophagobronchial the opaque
bronchiectasis
This
esophagogram.
bronchus A
fistula extends from the proximal esophagus portion in the right hemithorax. Moderately adis present. (B) Bronchogram. A bronchus supplies a small did not communicate with, and was distinctly separate from fair amount of contrast medium has alveolarized, and no
Heithoff
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48
et al.
1976
JANUARY,
3. Case I. (A) Levogram p ring right atrial ii -A large aberrant systemic artery from the innominate artery at its division into the right common carotid and subclavian arteries supplies the BPFM (large arrow). BPFM drains into the azygos vein which leads to its dense opacification (small arrow). (B) Pulmonary arteriogram following right atrial injection. A small branch of the right pulmonary artery supplies the BPFM (arrow). The other branch of the right pulmonary artery supplies the normal right lung which is compressed in the medial and basilar portion of the right hemithorax.
agus the the
at the T 3-
level side supplying hemithorax.
right right
to a large bronchus on the opaque portion in Moderately advanced
cylindrical and saccular observed in this bronchial opacification of the trachea
bronchiectasis was segment. Since no and the bronchus
supplying the aerated portion of the right lung was observed, a bronchogram was performed. This demonstrated a very small bronchus on
the
right
side
supplying
field. This bronchus and was distinctly
seen
on
the
(Fig.
a normal
vessel
artery
at
carotid
and
vessel
and
its
2B).
The
lung
was
free
bronchogram
trachea
and
left
.1j
p.
of “
also
bronchial
Si
FIG.
demonstrated arising
division
from into
subclavian a small
lower
and
system. A levoangiocardiogram aberrant
right
communicate with from the bronchus
esophagogram
bronchiectasis
revealed
the
did not separate
the the
of
innominate
right
arteries branch
a large
(Fig. the
right
common
3M.
This pulmo-
,
4.
tract.
Case
S
IS
;‘
S.
i. Microscopic
Hollow
arrow
findings showing
esophageal stratified squamous brush bordered pseudostratified epithelium of the respiratory indicates a large ring of hyaline
of transition
the fistulous of
the
epithelium into ciliated columnar tract. Dark arrow cartilage.
126,
nary
artery
pulmon Downloaded from www.ajronline.org by 114.135.140.96 on 10/13/15 from IP address 114.135.140.96. Copyright ARRS. For personal use only; all rights reserved
No.
VOL.
Bronchopulmonary
I
(Fig.
ary foregut
veins then
entered drained
At
surgery,
Foregut
3B) supplied the large bronchom alformation . The draining
into a large common into the dilated azygos the
vein vein.
malformation
Malformations CASE
which
der
consisted
of
and
oped
lung.
showed
the
smaller
apical
portion
tended
laterally
lobe
of these
of the and
the
right
hemithorax.
sisting
of
at
crowded into an tion and expanded right hemithorax
formation. 1.5 cm
entire
least
The in length
scopically the the esophageal end it resembled
chest
anteriorly,
occupied
of the
two
cavity while
and the
normal
aspect lung,
separate
ex-
larger
lobes,
conwas
the
left
esophagobronchial fistula was and o. cm in diameter. Micro-
fourth
esophagus
end, whereas at the bronchus (Fig. 4).
bronchial
at
After
omphalocele,
respiratory
the
distress
roentgenogram
the
to Children’s
in Oakland at the age ofthe urinary blad-
right
and
on the
hemithorax
surgical
infant
devel-
died.
day
of birth
densely
opacified
with a single air bronchogram at the right base. The heart and mediastinal shadows were displaced to the right side. There was an unexplained opacity in the medial basal portion of image.
resembled
admitted
omphalocele.
of the
severe
inferior and anteromedial secimmediately to fill the entire upon resection of the mal-
fistula
was Center exstrophy
ruptured
A chest
occupied
posteromedial The
two
lobes
B.G.D.
Medical days with
correction
two, dark consolidated lobes with a separate pleural investment from the remaining normal The
II.
Hospital ofthree
49
hemithorax
Several
vertebral of
the
column right ribs
simulating
segmentation first
(Fig.
into mal
the proximal fistulous tract
the
gastroesophageal
into
both
the right
the
cardiac
anomalies
were
seen,
with
second,
sM.
Injection
esophagus arising
along and
junction
hemithorax
fusion
third
with
of gastrografin showed
from
of the
with
an
the right and
and
abnor-
side of
branching
the
opacity
,.
small
air bronchogram
at the right base. Heart and mediastinal structures are shifted to the right side, opacity in the medial basilar portion of the left hemithorax. (B) Injection of gastroesophagus. An abnormal fistulous tract arises from the right side of the distal segment and then branches into the right hemithorax and basilar portion of the left hemithorax. in the cervical, thoracic, and the upper lumbar spine along with congenital bridging of the
with an unexplained grafin in the proximal of
the
esophagus
Hemivertebrae first four ribs
on
the
right
side
are
seen.
Heithoff
50
in the
duplication
of the
III
TABLE
Age
colon. o-8
DISCUSSION
Number
of Patients
months
16
8 months
Twenty-six instances of bronchopulmonary foregut malformations with patent gastroesophageal communication have been recorded, including the present report. One of the authors (S.M.S.) has examined three more patients from Children’s Hospital of Pittsburgh who are being reported as a part of a separate article.36 Their clinical and roentgenologic findings are included here for the completeness of the statistical analysis. Pulmonary Involvement (Table I). In ten of the 29 cases, the entire right lung was involved; in one stillborn infant, both right and left lungs were totally sequestered. The left lower lobe was involved in eleven, right lower lobe in four, both right lower lobe and left lower lobe in one, left upper lobe in one, and our first patient was the only one to have right upper lobe involvement. Site of Gastrointestinal Communication ( Table ii). The lower esophagus or cardio-
TABLE
Pulmonary
Involvement
Number
of
Patients
Left
right lower
Right Both
*
ii
lobe
lungs and left upper
Right Case
10
lobe
lower
Right Left
lung
4 I
lower
lobes
upper
I
lobe*
I.
TABLE
Site Lower
of Communication esophagus
esophageal Fundus
and junction
of stomach
Mid-esophagus Upper esophagus* *
Case
I
lobe
i.
Ii Number
of Patients
cardio24 3 I I
6 5
IV
TABLE
Sex
Number
Females Males Not
15
9
known
esophageal junction was by far the most common site of communication-24 of 29 cases. The fundus of the stomach communicated in three instances. In one, it involved the mid-esophagus, while in our first case, the upper portion of the esophagus communicated with BPFM. Age (Table III). Age at the time diagnosis is recorded in 27 cases, the other two being anatomic specimens. Sixteen of these have presented with clinical symptoms in the eight
months
of life.
Five
patients who had symptoms week of life had involvement right
Entire
to 18 years than 18 years
More
first
I
I976
JANUARY,
basilar portion of the left hemithorax SB). The infant, in addition, showed
(Fig.
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et al.
lung;
the
symptoms
of the
seven
in the first of the entire in these
patients
were often related to other severe congenital anomalies. Six other patients presented with clinical signs and symptoms in the pediatric group (18 years), whereas the remaining five presented in adult life (i8 to 48 years). Sex (Table Iv). The incidence in females was nearly twice that in males. Fifteen patients were female, whereas nine were male. In five reported cases, sex was not recorded. Clinical Signs and Symptoms. Chronic cough, often related to feeding, recurrent pneumonias, and respiratory distress were the most common clinical signs and symptoms. Infrequent findings were regurgitation
infants
of food,
having
hemoptysis,
other
and
congenital
anemia.
anomalies
The
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VOL.
126,
No.
Bronchopulmonary
I
Foregut
had clinical signs and symptoms related to those anomalies. Only two patients were asymptomatic. Blood Supply. The arterial supply was from the abdominal aorta in nine; from the pulmonary artery in eight; and the thoracic aorta in five. Single examples of innominate, left gastric, and left adrenal artery supply to a BPFM were recorded. Our first case was the only patient to have both systemic
(innominate)
and
pulmonary
ar-
tery supply to a BPFM simultaneously. Venous drainage was pulmonary in eleven and systemic in ten. DIAGNOSIS
An diagnostic
Esop/lagogram investigation.
was
the
most
Of
the
useful inwas perdiagnostic 22
stances where the esophagogram formed, the examination was in i8. Bronchography was performed in seven cases and was not diagnostic in any. In three, bronchiectasis was seen, while in four, displacement of the normal bronchi was observed. Preoperative ilngiography was performed in only three patients. It is, however, to be recommended as an aid to diagnosis and as a valuable guide in planning surgery, as it shows the number, caliber, and location of abnormal vessels, lessening the possibility of hemorrhagic complications due to madvertent damage to them at surgery.
Malformations
lung, and ten of the i8 other patients with lobar involvement showed other congenital anomalies. These anomalies tended to be more varied and severe in those patients with total sequestration of the right lung. The most frequent anomalies were diaphragmatic
STUDIES
Microscopic Structure of the Fistula. Microscopic findings have been recorded in i 8 patients. In eight, transition of the esophageal structure into a bronchial structure was observed. In four, the mucosal lining was stratified squamous epithelium, whereas in five, bronchial stratified ciliated columnar epithelium was seen. In one, gastnic mucosa lined the fistulous tract. Associated Anomalies. Vertebral, gastrointestinal, genitourinary, and cardiac congenital anomalies were frequently associated with BPFM. Nine of the eleven patients with total involvement of the right
hernia
(five),
vertebral
and
rib
anomalies (six), and congenital heart lesions (four). In all five instances, the diaphragmatic hernia was on the left side and was associated with left lower lobe esophagobronchial
communication.
In
four
of the
six cases in which vertebral and rib anomalies were observed, the entire right lung was involved. Other significant but rare anomalies were esophageal atresia and tracheo-esophageal fistula, duodenal stenosis, duplication of the colon, omphalocele, annular
pancreas,
cleft palate, Follow-up two patients are
alive
doing
were severe
died
four
gery
due
Three
posterior
urethral
valves,
and Turner’s syndrome. and Mortality Figures. Twentyhad resection of BPFM: i6
and
deaths ciated
well.
Five
postoperative
directly attributable to congenital anomalies.
months
following
to
infants
died
tract
prior AND
assoOne
successful
respiratory
ETIOLOGY
sur-
infection.
to surgery.
EMBRYOGENESIS
No unanimity of opinion exists with regard to the origin of pulmonary sequestrations. Several theories have been discussed at length in the literature,5’6’8”4”8’30’32’34 and
PATHOLOGIC
5’
these
some these
have
of the theories
intralobar
been
well
summarized
in
recent articles.’4”8’32 Most of postulate that extralobar and sequestrations
are
two
entirely
different entities, but current evidence cates that the two overlap considerably, and share a common embryogenesis.’4”6 Normally,
age
(Fig.
the
ventral
bud
goes
which buds
at
five
6) a small
wall on
seven
lateral furrow laryngotracheal progressively
of the
to form
bifurcates by
weeks
lung
of
bud
embryonic
develops
pharynx.
This
a laryngotracheal
into weeks.
two
mdi-
primitive
Simultaneously
from
lung tube
lung a
appears on each side of the ridge. The furrows become deeper and extend craniad to
Heithoff
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52
Five Weeks
Seven
et al.
tration remains in free communication with the gastrointestinal tract through a muscular, epithelial lined tube-thus forming the type of BPFM under discussion (Fig. 7). The isolation of most cases of bronchopulmonary foregut malformations from the gastrointestinal tract can be attributed to involution of the embryologic pedicle. Such a hypothesis is given great credence by the discovery of intermediate stages of this
Weeks
involutionary 6. Normal
FIG.
join
laryngotracheal
separating from the
tube
the dor-
esophagus.
The theory which most adequately plains the embryogenesis of foregut formations is that of a supernumerary
cxmallung
gut
bud
loca-
gastric
which
tion along
of
arises
a lung with the
accessory
caudal bud and growing
lung
life
onic
is
it
lung,
bud
before
pleura, tion.
On
lung
bud
has the
already adjacent
its
own
it
the
becomes and
forms
it
is
di fference
determines
the
from
invested
with
an
whether
and
not
characteristics
that
try to distinguish lobar sequestation supply, venous Several intra-
one
reported.’8’27’28
In logic
those pedicle
patients does
such
in
the
is
so-called may
not
such
diverticula.
fibrotic stalk
which
may
The
Five
fore-
gastroesophageal may
esophagus
stomach
or like
an
from epiphrenic
Weeks
Seven
whom involute,
Weeks
reasonably
the embryothe seques-
esophagus
esophaqus supernurnary
Jung bud supernuznzy
#{231}
#{188}\
L7
FIG.
7.
J’
Bronchopulmonary
Se-
foregut
pancreas,
and diverticula
the the
when
other
tre-
classical
occurring have
been
of the
occurs with
cysts,
between intraand extraon the basis of blood drainage, location, etc.
not
a
has
within
as ectopic
duplication
of
cases of simultaneously and extralobar sequestrations
been
occasionally
diverticulum.6”4”5
there ofthese
co-exists
fundus
extralobar sequestrations to have distinct characterin our cases as well as those
described
malformation
a sequestration
extralobar.
overlap
or
association
sequestration,
questration
from
and
mendous
in
involution
in simple
either
or
previously
duplication
and
Complete
anomalies, a
Intralobar have been
said fact,
seques-
diverticulum
to be incorporated
embryogenesis
is intralobar
In
as
found
sequestration
interesting
gut
extralobar
timing in
the
be associated with a small notch on the outer wall of the esophagus at the site of prior embryonic communication. A more complicated and embryologically
pleura
separate
been
or esophageal
found
normal
when grows
a
results
of
such
has
pedicle.2’6
embry-
adjacent
later,
Thus,
which
in
formed, lung,
qualitative
in
malformations
with
an intralobar sequestrahand, if this accessory
develops
sequestion.
early
in which
connected to the gastrointestinal fibrous strand. Examples of these forms, which most clearly mdigenesis of these malformations, reported.5”6”8 A variety of fore-
diverticula
caudally If this
development
invested
pleura,
normal
migrates esophagus.
arises
the
becomes the other
and
to the
process
tration is tract by a incomplete cate the have been
development.
thereby
eventually,
ventral sal
lung
1976
JANUARY,
lung
bud
(BPFM)
foregut
malformation.
arise
the
126,
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VOL.
No.
Bronchopulmonary
I
Foregut
Malformations
53
Figure 8, A and B, demonstrates a small bronchopulmonary foregu t malformation of the right lower lung attached to the right
lateral
margin
of the
thin membranous stalk. artery are present within with a small esophageal
8.
FIG. An
(4)
Gross
extralobar
to the (E) by a thin
tamed
duplication the
vein
duplication
drawing.
(5), atof the esophagus (P), which con-
margin pedicle
and
artery
(D)
are
communicated
sequestration.
a
sequestration
right lateral membranous
a small
by
(B) line
and
pulmonary
tached
phageal
specimen
esophagus
A small vein and this stalk along duplication cyst.
and
a small
demonstrated. with the bronchus
(Courtesy
of
Dr.
eso-
This of
31 41
01
R. Telander.)
Spectrum
B.PSFSMS
wit/2
lZIe’StZdtlOfl
‘
S
:5cm
‘SdtP.’t
57211fl/CdtIOfl
S
‘-2.
FThioas
3 Vessels
>a..u.Msa:
I
.5, -
stalk only
Diverticuiwn -
Intralobar
Fibrous
S
stalk
ad
sequestration EXtfdlobdr sequestration
__________
Sb-..
Diverticuiwn 5, 5
L7-
-
.
:i
5’ -5.’
S
::
Duplication
cyst
#{149},..
.
sq’
f
S5)5
j,S
FIG.9.
Diagrammatic
representation
of the
spectrum
of bronchopulmonary
foregut
malformations.
Heithoff
54
The
lumen
of
municated
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This in
of
genesis
the
we feel
the
tance
bronchus
of
spectrum
foregut
of
in
embryologi distinct
congenital
indicates
origin
9.
ni cation
of the
a common
foregut
indebted the
to Dr.
E. A. Boyden and
the
alies is hypothesis
so
and
J. B.,
12.
the
associated
complex that rarely cover all variations.”
3.
M. Total Thorax,
BENEKE,
9,
1905,
4.
5.
6.
can
diverticulum,
lower
accessory
gobronchial
fistula.
Brit.
H.,
EPPINGER,
16.
pulmonary
pp.
280.
X-Ray
i8.
Kronk-
Path.,
ofpulmonary
1902,
sequestra-
Chicago,
Year
1941,31,
392.
G. E., and LIEB0w, fistula and broncho-
sequestrations:
Ann.
sequestration
IwAl,
report of case, 1962, 155,
Surg.,
re215-
of
fistula
right
lung.
with
Ann.
Surg.,
599.
159,
K.,
G., M.,
SHINDO,
MORIM0T0,
R.
NEUDA,
tion, with pathology.
TAJIMA,
J.,
KosuDo,
Intralobar
special Am.
H.,
HAJIKANDO,
and
pulmonary
reference Rev. Resp.
Yo-
sequestra-
to developmental Dis., I973,
107,
911. 19.
1960,
S. H. Malforma-
GIRAY,
Path.,
E. A. Bronchoesophageal
HANNA,
H.,
sequestra-
Diagnosis. Inc.,
Arch.
ofliterature.
20.
W.,
KAFKA,
and
extrapulmonary 7. Dis. Child.,
KEELEY,
J. L., and
origin Ann.
sequestration. 1960,35,
der
KLEBS,
22.
Cor.-6l.f. Bohem., 1874,2, LANE, S., BURKO, H., and diology,
23.
LEWIS,
1971,
E. J.,
sequestration 24.
Louw,
J.
monary
oesophagus
foregu 101,
and with
Surg., H., and
esophagus.
Lunge.
Aerzthl.
111-121. SCOTT, t
H. Congenital
malformation.
Ra-
29 1-292.
MURRAY,
R. E.
Pulmonary
bronchoesophageal 1958,3, CHYWES,
sequestration
and
from
871.
Missbildungen
21.
bronchopulmonary
A. E. Anomalous
bronchus 152,
intra A.M.A.
51.
SCHAIRER,
of right main Surg., 1960,
E.
V. Simultaneous
BEKO,
and Am.
:7. Pediat. Publishers,
Allg.
220.
23,311-315.
Pediatric
faces
M. A., LINDSKOG, A. A. Esophagobronchial view
323-329.
J.
Ergebn.
HALASZ,
pulmonary
E. A. Bronchogenic
Medical
W.
SCHAUENSTEIN,
G. E., and oflungs.
1964,
cysts and theory of in tralobar sequestration : new embryologic data. 7. Thoracic Surg., 1958,35, 604. BOYDEN, E. A., BILL, A. H., JR., and CREIGHTON, S. A. Presumptive origin ofleft lower accessory lung from esophageal diverticulum. Surgery,
Book
43,
I956,
419.
tions
17.
A. A. Intralobar enteric sequestration of lung containing aberrant pancreas. 7. Thoracic & Cardiovasc. Surg., 1961, 4!, 3 14-317.
CAFFEY,
and
Zungen.
B. Many
FELSON,
1413-I
BESKIN,
BOYDEN,
lobe, and oesopha-
7. Surg.,
GRUENFELD,
one
P. Verhandl. d. deutsch. path. Geseisch., 202. Cited by Gruenfeld and Giray.15
1962,52,
7.
unilateral 1968,
associated
15.
anom-
J. R., and AZZONI, A. A. Intralobar pulmonary sequestration and intralobar enteric sequestration associ ated wi th vertebral anomalies. 7. Thoracic & Cardiovasc. Surg., 1967, 53, 471 tion.
lung
14.
for
ARCOMANO,
BATES,
A. W.
FAWCETT,
of
Seminars in Roentgenol., 1972, 7, 3-16. GANS, S. L., and Poi-rs, W. J. Anomalous lobe of lung arising from esophagus. 7. Thoracic Surg., 1951, 2!, 3 13-318. GERLE, R., JARETZKI, A., BERNE, A. S., and ASHLEY, C. A. Congenital bronchopulmonary -foregut malformation, pulmonary sequestration communicating with gastrointestinal tract. New England 7. Med., 1968, 278,
REFERENCES
2.
sequestration
wi th foregut diverticulum (oesophagobronchial fistula) and aberrant artery. Brit. 7. Surg., 1959, 46, 582-586. DAVIDSON, J. S. Case of congenital oesophageal
total
I.
0. G., and
DODGE,
heiten der 8, 267-285.
prints,
and offering constructive criticism. He concurred with the Unifying Concept of Bronchopulmonary Foregut Malformations presented here but emphasized the fact that “the development of the bronchopulsegments
II.
13.
manuscript
monary
ofdiaphragm.
tion.
M. Sane, M.D. Department of Radiology Children’s Health Center 2525 Chicago Avenue, South Minneapolis, Minnesota 55404
are
with
417-421.
precursor;
Shashikant
We
10.
two
their attachment to the esophagus by the fibrous stalk indicated that the precursor arose from the gastrointestinal tract.
reviewing
DAS,
defect
R. J. Case of hernia through 7. Anat., 1917,
GLADSTONE,
associated
64-96.
Intralobar
frank
between
anomalies from
9.
dia-
The
lungs
congenital
embryo-
described
Figure
commu
C
accep-
E. A., and
COCKAYNE,
52,
bronchopulmo-
malformations
grammatically
the
1976
JANUARY,
accessory
missing
for
common
8.
comof
the
necessary
theory
the
cyst
represents
evidence
the of
nary
duplication
with
BPFM. link
the
et al.
fistula.
575. S. Extralobar communicating
associated
with
pulwith
strangulated
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126,
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I
congenital
diaphragmatic
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A. A., and WYLIE, R. H. Congenital communication between esophagus and isolated ectopic pulmonary tissue. 7. Thoracic & Cardiovasc. Surg., 1968, 55, 672. NIKAIDAH, H., and SWENSON, 0. Ectopic origin of
main
Cardiovasc.
in
Surg.,
W. W.
PARK,
pulmonalis. 28.
bronchus
pneumonectomy
1971,
Intralobar
64,
9.
Thoracic
62, 1962,
J.,
P., ALEXANDER, D. Pulmonary ing classic intralobar
and &
31.
32.
33.
151-160.
34.
of lingula
4!,
378.
M.,
and coexist-
KHADEMI,
sequestration:
GROFF,
:i.
esophagus: case 7. Thoracic &
sequestration
Dis. Chest,
PENDSE,
child.
from neonate.
extralobar
Cardiovasc.
types Surg.,
35.
in
1972,
Operativer Ductus
E.
LEVINSKY,
Verschluss Oesopho-Bronchialis
L.,
and eines
J.
GADLICKA,
angeborenen bei
einer
36. 37.
E.
REKTORZIK,
mit Kongenitaler Lun gencytose und pluriglandularer Insuflizienz: Nanosomi und Geroderma bedignt durch Anovarismus. Schweiz Ztschr. Tul’erk., 1958, 55, 92-105. 0RYCE, D. M., SELLORS, I. H., and BLAIR, L. L. Intralobar sequestration of lung associated
Ueber
Surg.,
artery.
7.
Brit.
Accessorische
Lungen-
THOMSON,
51,
WARNER, CLELAND,
and A.M.A.
1956,31,
354-358.
R. Personal communication. N. B., and AQUINO, I. Anomalous of right main-stem bronchus. Surgery,
TELANDER,
1962,
38.
pulmonary 18-29.
lappen. Wochenblatt ZKK GesellschaJt Aertze (Wien)., 186I, 17, 4-6. Cited by Halacz. SANE, S., and GIRDANY, B. Cysts and neoplasma in infant lung. Seminars in Roentgenol., 1972, 7, I22-I48. SCHEIDEGGER, S. Frankfurt Ztschr. Path., 1936, 49, 362. Cited by Gruenfeld and Giray.’5 SMITH, R. A. Theory of origin of intralobar sequestration oflung. Thorax, 1956, ii, 10. ST. RAYMOND, A. H., JR., HARDY, J. D., and R0BBINS, S. G. Lower accessory lung communicating with esophagus and associated with congenital diaphragmatic hernia. 7.
origin
Frau
55
abnormal 1948,35,
Thoracic
127.
POLAK,
Malformations with Surg.,
MOSCARELLA,
of right
27.
Brit. 7. Surg.,
hernia.
102-105.
1962,50,
25.
Foregut
668-676.
F. S., MCGRAW, R., and MEYER, agenesis Am.
with
7. Dis.
heart Child.,
C.,
H.,
PETERSON,
B.
Lung
ectopia
dextrorotation. 1961,
JO!,
514.
JANUARY,
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BRONCHOPULMONARY
FOREGUT
A UNIFYING By
KENNETH
B.
HUGH
MALFORMATIONS*
ETIOLOGICAL
HEITHOFF,
M.D.,
CONCEPT
SHASHIKANT
J. WILLIAMS, M.D., CHARLES JOHN CARTER, M.D., PETER and WILLIAM BRENNOM,
MINNEAPOLIS
AND
ST.
PAUL,
MINNESOTA,
M.
SANE,
J. JARVIS, KANE, M.D., M.D.
AND
OAKLAND,
M.D.,
M.D.,
CALIFORNIA
ABSTRACT:
Two and
cases
of
another
congenital
27 cases
bronchopulmonary
reviewed
and
the
foregut
findings
malformation
analyzed.
entire right lung are the most common locations distal portion of the esophagus or cardioesophageal site of communication (83 percent). The majority
The
left
are lower
of pulmonary junction was of the patients
reported
lobe
and
the
involvement. The the most common (6o percent) pre-
sented in the first eight months of life, and the incidence in females was nearly twice that in males. Chronic cough, recurrent pneumonias and respiratory distress were the most common clinical findings, whereas two patients were totally asymptomatic. The esophagogram was the single most useful diagnostic procedure (82 percent). The microscopic structure of the congenital fistula resembled esophagus, bronchus or
both.
prior
Surgical
treatment
was
to corrective
surgery,
whereas
curative
in most
the
instances.
postoperative
Some
deaths
deaths
in most
occurred
instances
were
related
to severe associated congenital anomalies. We believe a common embryologic pathogenesis leads to the formation of bronchopulmonary foregut malformations. These bronchopulmonary formations include intralobar and extralobar sequestrations, pulmonary
of a variety foregut malsequestra-
tion
communica-
with
tion, cysts.
patent,
or
esophageal
involuted-partial
or gastric
or
diverticula,
complete-gastroesophageal
and
esophageal
or
PATENT,
congenital communications between accessory lung tissue and esophagus or stomach are extremely rare and only 24 such instances are recorded in the literature.4’7’9’’#{176}”2’4”6’’7’2026’29’35’37’38 The purpose of this article is not only to report two such patients we recently had a chance to examine, but also to emphasize that this rare anomaly provides evidence for an interesting pathogenesis monary
and
plausible theory of the gamut of
foregut
malformations
REPORT CASE
a
Presented
American From
in
part
Roentgen the
Children’s
at Ray
the
5eventeenth
Society, Hospitals
San in
Annual Francisco,
Minneapolis,
and
St.
Paul
and
cough
was
initially
coid
material
old male
Children’s
weight
gain,
chronic
cough
but The emesis.
was with
low
intermittent
since
productive
currently
infant,
Hospital
birth.
The
of stringy
mu-
hacking
and
was
cough frequently led to The child fed poorly and
slowly. The feedings did not produce any coughing or gagging episodes. The physical examination revealed a pale, irritable, dyspneic infant weighing 4 kg, tern;
37.6#{176}C, the blood
sounds
decreased the
Society
September Paul,
CASES
a ten week
St.
of slow
72/DIm Breath
California,
OF
B.K.,
fever
perature
of
duplication
to
nonproductive. gagging and
in-
Meeting
history
grade
cluding intralobar and extralobar sequestrations with or without communication with the alimentary tract, foregut duplication cysts, and esophageal diverticula. *
I.
admitted
of common bronchopul(BPFM)
bronchogenic
Minnesota,
46
for 24-27,
but Pediatric
pulse 160/mm, pressure was
over no
the rales
Radiology
1974.
and
Oakland,
California.
right nor and
not
hemithorax rhonchi
the
respiration recorded.
Annual
were could
Meeting
be of
the
126,
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VOL.
No.
Bronchopulmonary
I
Foregut
Malformations
47
heard. A grade 2/6 systolic ejection murmur, heard best at the apex, radiated to the back. Electrocardiography showed biventricular hypertrophy. The liver and spleen were palpable 4 cm below the costal margins. A chest roentgenogram (Fig. i ) demonstrated almost
complete
thorax
with
ment
at
small, throughout
1.
extending
Case
FIG. at
hemi-
seg-
hemithorax
Case
T3-T4
vanced
cylindrical
portion
of
the bronchus bronchiectasis
the
exception
costophrenic
are
I. (A)
level
the
seen
and
right
lower
seen
on
A large
is seen.
bronchus saccular
lung. the
Multiple
this
throughout
view of
of a small
angle.
Esophagogram. to a large
esoph-
Frontal opacification
complete with
the right
at
the proximal
roentgenogram.
almost
bronchograms
2.
from
Chest
.
right
segment air
right
of a triangular
base of the right lung. Multiple, radiolucencies were scattered this opacity. An esophagogram demonstrated a large, esophagobron-
demonstrating the
of the
exception
the round
(Fig. 2A) chial fistula
FIG.
opacification
the
supplying
opacity.
esophagobronchial the opaque
bronchiectasis
This
esophagogram.
bronchus A
fistula extends from the proximal esophagus portion in the right hemithorax. Moderately adis present. (B) Bronchogram. A bronchus supplies a small did not communicate with, and was distinctly separate from fair amount of contrast medium has alveolarized, and no
Heithoff
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48
et al.
1976
JANUARY,
3. Case I. (A) Levogram p ring right atrial ii -A large aberrant systemic artery from the innominate artery at its division into the right common carotid and subclavian arteries supplies the BPFM (large arrow). BPFM drains into the azygos vein which leads to its dense opacification (small arrow). (B) Pulmonary arteriogram following right atrial injection. A small branch of the right pulmonary artery supplies the BPFM (arrow). The other branch of the right pulmonary artery supplies the normal right lung which is compressed in the medial and basilar portion of the right hemithorax.
agus the the
at the T 3-
level side supplying hemithorax.
right right
to a large bronchus on the opaque portion in Moderately advanced
cylindrical and saccular observed in this bronchial opacification of the trachea
bronchiectasis was segment. Since no and the bronchus
supplying the aerated portion of the right lung was observed, a bronchogram was performed. This demonstrated a very small bronchus on
the
right
side
supplying
field. This bronchus and was distinctly
seen
on
the
(Fig.
a normal
vessel
artery
at
carotid
and
vessel
and
its
2B).
The
lung
was
free
bronchogram
trachea
and
left
.1j
p.
of “
also
bronchial
Si
FIG.
demonstrated arising
division
from into
subclavian a small
lower
and
system. A levoangiocardiogram aberrant
right
communicate with from the bronchus
esophagogram
bronchiectasis
revealed
the
did not separate
the the
of
innominate
right
arteries branch
a large
(Fig. the
right
common
3M.
This pulmo-
,
4.
tract.
Case
S
IS
;‘
S.
i. Microscopic
Hollow
arrow
findings showing
esophageal stratified squamous brush bordered pseudostratified epithelium of the respiratory indicates a large ring of hyaline
of transition
the fistulous of
the
epithelium into ciliated columnar tract. Dark arrow cartilage.
126,
nary
artery
pulmon Downloaded from www.ajronline.org by 114.135.140.96 on 10/13/15 from IP address 114.135.140.96. Copyright ARRS. For personal use only; all rights reserved
No.
VOL.
Bronchopulmonary
I
(Fig.
ary foregut
veins then
entered drained
At
surgery,
Foregut
3B) supplied the large bronchom alformation . The draining
into a large common into the dilated azygos the
vein vein.
malformation
Malformations CASE
which
der
consisted
of
and
oped
lung.
showed
the
smaller
apical
portion
tended
laterally
lobe
of these
of the and
the
right
hemithorax.
sisting
of
at
crowded into an tion and expanded right hemithorax
formation. 1.5 cm
entire
least
The in length
scopically the the esophageal end it resembled
chest
anteriorly,
occupied
of the
two
cavity while
and the
normal
aspect lung,
separate
ex-
larger
lobes,
conwas
the
left
esophagobronchial fistula was and o. cm in diameter. Micro-
fourth
esophagus
end, whereas at the bronchus (Fig. 4).
bronchial
at
After
omphalocele,
respiratory
the
distress
roentgenogram
the
to Children’s
in Oakland at the age ofthe urinary blad-
right
and
on the
hemithorax
surgical
infant
devel-
died.
day
of birth
densely
opacified
with a single air bronchogram at the right base. The heart and mediastinal shadows were displaced to the right side. There was an unexplained opacity in the medial basal portion of image.
resembled
admitted
omphalocele.
of the
severe
inferior and anteromedial secimmediately to fill the entire upon resection of the mal-
fistula
was Center exstrophy
ruptured
A chest
occupied
posteromedial The
two
lobes
B.G.D.
Medical days with
correction
two, dark consolidated lobes with a separate pleural investment from the remaining normal The
II.
Hospital ofthree
49
hemithorax
Several
vertebral of
the
column right ribs
simulating
segmentation first
(Fig.
into mal
the proximal fistulous tract
the
gastroesophageal
into
both
the right
the
cardiac
anomalies
were
seen,
with
second,
sM.
Injection
esophagus arising
along and
junction
hemithorax
fusion
third
with
of gastrografin showed
from
of the
with
an
the right and
and
abnor-
side of
branching
the
opacity
,.
small
air bronchogram
at the right base. Heart and mediastinal structures are shifted to the right side, opacity in the medial basilar portion of the left hemithorax. (B) Injection of gastroesophagus. An abnormal fistulous tract arises from the right side of the distal segment and then branches into the right hemithorax and basilar portion of the left hemithorax. in the cervical, thoracic, and the upper lumbar spine along with congenital bridging of the
with an unexplained grafin in the proximal of
the
esophagus
Hemivertebrae first four ribs
on
the
right
side
are
seen.
Heithoff
50
in the
duplication
of the
III
TABLE
Age
colon. o-8
DISCUSSION
Number
of Patients
months
16
8 months
Twenty-six instances of bronchopulmonary foregut malformations with patent gastroesophageal communication have been recorded, including the present report. One of the authors (S.M.S.) has examined three more patients from Children’s Hospital of Pittsburgh who are being reported as a part of a separate article.36 Their clinical and roentgenologic findings are included here for the completeness of the statistical analysis. Pulmonary Involvement (Table I). In ten of the 29 cases, the entire right lung was involved; in one stillborn infant, both right and left lungs were totally sequestered. The left lower lobe was involved in eleven, right lower lobe in four, both right lower lobe and left lower lobe in one, left upper lobe in one, and our first patient was the only one to have right upper lobe involvement. Site of Gastrointestinal Communication ( Table ii). The lower esophagus or cardio-
TABLE
Pulmonary
Involvement
Number
of
Patients
Left
right lower
Right Both
*
ii
lobe
lungs and left upper
Right Case
10
lobe
lower
Right Left
lung
4 I
lower
lobes
upper
I
lobe*
I.
TABLE
Site Lower
of Communication esophagus
esophageal Fundus
and junction
of stomach
Mid-esophagus Upper esophagus* *
Case
I
lobe
i.
Ii Number
of Patients
cardio24 3 I I
6 5
IV
TABLE
Sex
Number
Females Males Not
15
9
known
esophageal junction was by far the most common site of communication-24 of 29 cases. The fundus of the stomach communicated in three instances. In one, it involved the mid-esophagus, while in our first case, the upper portion of the esophagus communicated with BPFM. Age (Table III). Age at the time diagnosis is recorded in 27 cases, the other two being anatomic specimens. Sixteen of these have presented with clinical symptoms in the eight
months
of life.
Five
patients who had symptoms week of life had involvement right
Entire
to 18 years than 18 years
More
first
I
I976
JANUARY,
basilar portion of the left hemithorax SB). The infant, in addition, showed
(Fig.
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et al.
lung;
the
symptoms
of the
seven
in the first of the entire in these
patients
were often related to other severe congenital anomalies. Six other patients presented with clinical signs and symptoms in the pediatric group (18 years), whereas the remaining five presented in adult life (i8 to 48 years). Sex (Table Iv). The incidence in females was nearly twice that in males. Fifteen patients were female, whereas nine were male. In five reported cases, sex was not recorded. Clinical Signs and Symptoms. Chronic cough, often related to feeding, recurrent pneumonias, and respiratory distress were the most common clinical signs and symptoms. Infrequent findings were regurgitation
infants
of food,
having
hemoptysis,
other
and
congenital
anemia.
anomalies
The
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VOL.
126,
No.
Bronchopulmonary
I
Foregut
had clinical signs and symptoms related to those anomalies. Only two patients were asymptomatic. Blood Supply. The arterial supply was from the abdominal aorta in nine; from the pulmonary artery in eight; and the thoracic aorta in five. Single examples of innominate, left gastric, and left adrenal artery supply to a BPFM were recorded. Our first case was the only patient to have both systemic
(innominate)
and
pulmonary
ar-
tery supply to a BPFM simultaneously. Venous drainage was pulmonary in eleven and systemic in ten. DIAGNOSIS
An diagnostic
Esop/lagogram investigation.
was
the
most
Of
the
useful inwas perdiagnostic 22
stances where the esophagogram formed, the examination was in i8. Bronchography was performed in seven cases and was not diagnostic in any. In three, bronchiectasis was seen, while in four, displacement of the normal bronchi was observed. Preoperative ilngiography was performed in only three patients. It is, however, to be recommended as an aid to diagnosis and as a valuable guide in planning surgery, as it shows the number, caliber, and location of abnormal vessels, lessening the possibility of hemorrhagic complications due to madvertent damage to them at surgery.
Malformations
lung, and ten of the i8 other patients with lobar involvement showed other congenital anomalies. These anomalies tended to be more varied and severe in those patients with total sequestration of the right lung. The most frequent anomalies were diaphragmatic
STUDIES
Microscopic Structure of the Fistula. Microscopic findings have been recorded in i 8 patients. In eight, transition of the esophageal structure into a bronchial structure was observed. In four, the mucosal lining was stratified squamous epithelium, whereas in five, bronchial stratified ciliated columnar epithelium was seen. In one, gastnic mucosa lined the fistulous tract. Associated Anomalies. Vertebral, gastrointestinal, genitourinary, and cardiac congenital anomalies were frequently associated with BPFM. Nine of the eleven patients with total involvement of the right
hernia
(five),
vertebral
and
rib
anomalies (six), and congenital heart lesions (four). In all five instances, the diaphragmatic hernia was on the left side and was associated with left lower lobe esophagobronchial
communication.
In
four
of the
six cases in which vertebral and rib anomalies were observed, the entire right lung was involved. Other significant but rare anomalies were esophageal atresia and tracheo-esophageal fistula, duodenal stenosis, duplication of the colon, omphalocele, annular
pancreas,
cleft palate, Follow-up two patients are
alive
doing
were severe
died
four
gery
due
Three
posterior
urethral
valves,
and Turner’s syndrome. and Mortality Figures. Twentyhad resection of BPFM: i6
and
deaths ciated
well.
Five
postoperative
directly attributable to congenital anomalies.
months
following
to
infants
died
tract
prior AND
assoOne
successful
respiratory
ETIOLOGY
sur-
infection.
to surgery.
EMBRYOGENESIS
No unanimity of opinion exists with regard to the origin of pulmonary sequestrations. Several theories have been discussed at length in the literature,5’6’8”4”8’30’32’34 and
PATHOLOGIC
5’
these
some these
have
of the theories
intralobar
been
well
summarized
in
recent articles.’4”8’32 Most of postulate that extralobar and sequestrations
are
two
entirely
different entities, but current evidence cates that the two overlap considerably, and share a common embryogenesis.’4”6 Normally,
age
(Fig.
the
ventral
bud
goes
which buds
at
five
6) a small
wall on
seven
lateral furrow laryngotracheal progressively
of the
to form
bifurcates by
weeks
lung
of
bud
embryonic
develops
pharynx.
This
a laryngotracheal
into weeks.
two
mdi-
primitive
Simultaneously
from
lung tube
lung a
appears on each side of the ridge. The furrows become deeper and extend craniad to
Heithoff
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52
Five Weeks
Seven
et al.
tration remains in free communication with the gastrointestinal tract through a muscular, epithelial lined tube-thus forming the type of BPFM under discussion (Fig. 7). The isolation of most cases of bronchopulmonary foregut malformations from the gastrointestinal tract can be attributed to involution of the embryologic pedicle. Such a hypothesis is given great credence by the discovery of intermediate stages of this
Weeks
involutionary 6. Normal
FIG.
join
laryngotracheal
separating from the
tube
the dor-
esophagus.
The theory which most adequately plains the embryogenesis of foregut formations is that of a supernumerary
cxmallung
gut
bud
loca-
gastric
which
tion along
of
arises
a lung with the
accessory
caudal bud and growing
lung
life
onic
is
it
lung,
bud
before
pleura, tion.
On
lung
bud
has the
already adjacent
its
own
it
the
becomes and
forms
it
is
di fference
determines
the
from
invested
with
an
whether
and
not
characteristics
that
try to distinguish lobar sequestation supply, venous Several intra-
one
reported.’8’27’28
In logic
those pedicle
patients does
such
in
the
is
so-called may
not
such
diverticula.
fibrotic stalk
which
may
The
Five
fore-
gastroesophageal may
esophagus
stomach
or like
an
from epiphrenic
Weeks
Seven
whom involute,
Weeks
reasonably
the embryothe seques-
esophagus
esophaqus supernurnary
Jung bud supernuznzy
#{231}
#{188}\
L7
FIG.
7.
J’
Bronchopulmonary
Se-
foregut
pancreas,
and diverticula
the the
when
other
tre-
classical
occurring have
been
of the
occurs with
cysts,
between intraand extraon the basis of blood drainage, location, etc.
not
a
has
within
as ectopic
duplication
of
cases of simultaneously and extralobar sequestrations
been
occasionally
diverticulum.6”4”5
there ofthese
co-exists
fundus
extralobar sequestrations to have distinct characterin our cases as well as those
described
malformation
a sequestration
extralobar.
overlap
or
association
sequestration,
questration
from
and
mendous
in
involution
in simple
either
or
previously
duplication
and
Complete
anomalies, a
Intralobar have been
said fact,
seques-
diverticulum
to be incorporated
embryogenesis
is intralobar
In
as
found
sequestration
interesting
gut
extralobar
timing in
the
be associated with a small notch on the outer wall of the esophagus at the site of prior embryonic communication. A more complicated and embryologically
pleura
separate
been
or esophageal
found
normal
when grows
a
results
of
such
has
pedicle.2’6
embry-
adjacent
later,
Thus,
which
in
formed, lung,
qualitative
in
malformations
with
an intralobar sequestrahand, if this accessory
develops
sequestion.
early
in which
connected to the gastrointestinal fibrous strand. Examples of these forms, which most clearly mdigenesis of these malformations, reported.5”6”8 A variety of fore-
diverticula
caudally If this
development
invested
pleura,
normal
migrates esophagus.
arises
the
becomes the other
and
to the
process
tration is tract by a incomplete cate the have been
development.
thereby
eventually,
ventral sal
lung
1976
JANUARY,
lung
bud
(BPFM)
foregut
malformation.
arise
the
126,
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VOL.
No.
Bronchopulmonary
I
Foregut
Malformations
53
Figure 8, A and B, demonstrates a small bronchopulmonary foregu t malformation of the right lower lung attached to the right
lateral
margin
of the
thin membranous stalk. artery are present within with a small esophageal
8.
FIG. An
(4)
Gross
extralobar
to the (E) by a thin
tamed
duplication the
vein
duplication
drawing.
(5), atof the esophagus (P), which con-
margin pedicle
and
artery
(D)
are
communicated
sequestration.
a
sequestration
right lateral membranous
a small
by
(B) line
and
pulmonary
tached
phageal
specimen
esophagus
A small vein and this stalk along duplication cyst.
and
a small
demonstrated. with the bronchus
(Courtesy
of
Dr.
eso-
This of
31 41
01
R. Telander.)
Spectrum
B.PSFSMS
wit/2
lZIe’StZdtlOfl
‘
S
:5cm
‘SdtP.’t
57211fl/CdtIOfl
S
‘-2.
FThioas
3 Vessels
>a..u.Msa:
I
.5, -
stalk only
Diverticuiwn -
Intralobar
Fibrous
S
stalk
ad
sequestration EXtfdlobdr sequestration
__________
Sb-..
Diverticuiwn 5, 5
L7-
-
.
:i
5’ -5.’
S
::
Duplication
cyst
#{149},..
.
sq’
f
S5)5
j,S
FIG.9.
Diagrammatic
representation
of the
spectrum
of bronchopulmonary
foregut
malformations.
Heithoff
54
The
lumen
of
municated
Downloaded from www.ajronline.org by 114.135.140.96 on 10/13/15 from IP address 114.135.140.96. Copyright ARRS. For personal use only; all rights reserved
This in
of
genesis
the
we feel
the
tance
bronchus
of
spectrum
foregut
of
in
embryologi distinct
congenital
indicates
origin
9.
ni cation
of the
a common
foregut
indebted the
to Dr.
E. A. Boyden and
the
alies is hypothesis
so
and
J. B.,
12.
the
associated
complex that rarely cover all variations.”
3.
M. Total Thorax,
BENEKE,
9,
1905,
4.
5.
6.
can
diverticulum,
lower
accessory
gobronchial
fistula.
Brit.
H.,
EPPINGER,
16.
pulmonary
pp.
280.
X-Ray
i8.
Kronk-
Path.,
ofpulmonary
1902,
sequestra-
Chicago,
Year
1941,31,
392.
G. E., and LIEB0w, fistula and broncho-
sequestrations:
Ann.
sequestration
IwAl,
report of case, 1962, 155,
Surg.,
re215-
of
fistula
right
lung.
with
Ann.
Surg.,
599.
159,
K.,
G., M.,
SHINDO,
MORIM0T0,
R.
NEUDA,
tion, with pathology.
TAJIMA,
J.,
KosuDo,
Intralobar
special Am.
H.,
HAJIKANDO,
and
pulmonary
reference Rev. Resp.
Yo-
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107,
911. 19.
1960,
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HANNA,
H.,
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20.
W.,
KAFKA,
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extrapulmonary 7. Dis. Child.,
KEELEY,
J. L., and
origin Ann.
sequestration. 1960,35,
der
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22.
Cor.-6l.f. Bohem., 1874,2, LANE, S., BURKO, H., and diology,
23.
LEWIS,
1971,
E. J.,
sequestration 24.
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J.
monary
oesophagus
foregu 101,
and with
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esophagus.
Lunge.
Aerzthl.
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H. Congenital
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MURRAY,
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Pulmonary
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SCHAIRER,
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CAFFEY,
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B. Many
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1413-I
BESKIN,
BOYDEN,
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JANUARY,
accessory
missing
for
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nary
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