OCTOBER, 1966

INFANTILE LOBAR EMPHYSEMA*

REPORT OF TWO CASES WITH UNUSUAL
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ROENTGENOGRAPHIC MANIFESTATION
By E. A. FRANKEN, JR., M.1).,t and ISABELLE BCEHL, \I.l)4
INDIANAPOLIS, INDIANA

I iliatioll
IflallY
tile
areas
bronchi,
tamed COD pale,
bronchioles, and
eosinophil
alveoli
ic
iii

Ill a-
re#{231}pgizedcause of rp tory_distress
terial, indicating the presence of protein-con-
in infancy in which rompt recognition foiJ
taming fluid. An occasional squame was seen
lowe y surgical extirpation o tie in-
in tiliS illatenial. No abnormality of tile carti-
lages was noted. In many foci, the alveoli were
Th n ia nosis of iiiTii#{128}ile distended and there was rupture of tile septa,
loban emphysema is made - am roent- producing large, cyst-like spaces.
genognams o the chest. The characteristic
CASE II. D.S., a 2 day old white female, was
referred to Indiana University Medical Center
with a history of cyanosis and dvspnea of 24

aEfhis institution in tile recent past, the hours duration.

Physical examination showed
a dyspneic and cyanotic child with decreased
roentgen manifestations were atypical in 2;
breath sounds and increased resonance in the
these 2 cases form tile basis of this report.
right chest. Initial chest roentgenogram (Eig.
REIORT OF CASES 3) demonstrated a shift of the heart and medi-
astinum to the left, with a wedge-shaped density
CASE I. D.M., a 2 day old white male, was in the right lower lung field. The remainder of
admitted to Indiana University Medical Center the right lung was characterized by a hazy den-
with a history of dyspnea and cyanosis since
sity obscuring the vascular markings. Hernia-
birth. The right hemithorax was hyperresonant
compared to the left side, and breath sounds
were decreased on the right. Admission roent-
genogran of the chest (Fig. i) showed the me-
diastinunl and heart to be displaced to the left,
and there was separation of the ribs on the
right. A wedge-shaped density was noted in the
mid-portion of the right lower lung field. The
major portion of tile right lung had a hazy ap-
pearance with poor definition of the vascular
markings. On tile lateral roefltgeflOgranl, her-
niation of rigilt lung across the anterior me-
diastinum was seen (Fig. 2). A diagnosis of in-
fantile lobar emphysema was made, and after
12 hours of observation, thoracotomy was
performed. The right middle lobe herniated
through tile surgical inCiSiOn, and it was noted
to occupy most of the pleural cavity, compress-
ing the upper and lower lobes. A right middle FIG. I. Case i. Pressure effects of the distended
lobectorny was performed, following which the right middle lobe are evident, with collapse of the
patient made an uneventful recovery. right lower lobe and shift of heart and mediastinum
On gross examination of the right middle lobe, to the left. The right middle lobe fills the right
no abnormality of the bronchi was noted. The hemithorax and shows a hazy density- with oblitera-
lung was subcrepitant. On microscopic exam- tion of the normal vascular markings.

* From the i)epartments of Radiology t and Patho!ogy, Indiana University Medical Center, Indianapolis, Indiana.

354
 \OL. 98, No. 2 lIlfalltile Lol)ar Empilvsellla 355

I
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1-in. 2. Case 1. Ilerniation of the distended lobe

through tile anterior mediastinum is seen.

tion of the right lung through the anterior

mediastinum was noted on the lateral roent-
genogram (Fig. 4). Experience with Case i
prompted the diagnosis of infantile lobar em-
phvsenla. At the time of thoracotomy, 8 hours
after admission, the right upper lobe was found
to be markedly emphvsematous, compressing FIG. 4. Case II. The emphysematous right upper
the adjacent lobes, and occupying almost the lobe herniates through the anterior mediastinum
on the lateral roentgenogram.

entire pleural cavity. The surgeon commented

that considerable fluid was present in the dis-
tended lobe. After lobectomy, the patients
postoperative course was complicated by inter-
mittent atelectasis of portions of the lower
lobes, but she eventually recovered completely.
On gross examination, the right upper lobe
was noncrepitant, and clear yellow fluid exuded
from the cut surface. No al)normalitv of the
bronchi was seen. Microscopic examination re-
vealed areas of emphysema interspersed with
small foci of atelectasis. In the emphysematous
areas, the alveoli were markedly distended and
in many places the alveolar septa were dis-
rupted, forming cyst-like spaces. The alveoli
and bronchioles contained eosinophilic material
with many squames, indicating the presence of
protein-containing fluid (Fig. , A and B).
11G. 3. Case ii. The distended right upper lobe r-
duces collapse of the middle and lower lobes and DISCUSS ION
shift of the mediastinum to the left. The poor defi-
nition of vascular markings and hazy density- are Infantile lobar emphsema is an occa-
seen in tile emphvsematotzs right upper lobe, which sional cause of respiratory distress in in-
tills tile right hemi thorax. fancy; 26 cases have been encountered in a
 356 E. A. Franken, Jr. and Isabelle Buehl OCTOBER, 1966

P..
i.-4. I
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-.
t#{149}. p#{149}
- t.

FiG. . Case 11. (A) Emphysema with rupture of alveolar walls and protein-containing material in the
broncilioles and alveoli (X2o magnification). (B) Note the emphysema, squames, and protein-containing
fluid in the alveoli (X2oo magnification).

10 year period in one large pediatric in- rnucosa. Several cases have shown 0111) al-
stitution.5 About olle-ilalf of the patients ve(iTiiibrosis, and this is tilougllt by some
present with tile signs of respiratory dis- to be the etiology of the emphysema.2 In
tress in the neonatal period. The remainder many instances, no specific anatomic ab-
are seen in the first to fourth month of life, normality is found,7 as in the 2 cases re-
and in these patients, symptoms are less ported here.
severe. In the neonate, dvspnea with on Differential diagnosis includes congenital
without c\anosis IS the presenting feature. andinerrstvsrs,ttatao-
Iil_empi1yl3lIllOst always ipvQiyes a mi#{243}idThtiaatelectasis with com-
single lobe, usually the ni ht on left u en pensator r em hysema oUiFoes,
on t e ri lt middle lobe.
xamination o 1 esected lobe reveals matic hernia.
distention of the alveoli with ru tune of al- T#{228}fmeiit of infantile lobar emphysema
veolar wa s an cyst- i e formation. The consists of surgical removal of the involved
etio5 lobe. Surgery should not l)e delayed in
thought to e uetoyaiytypeQLth those patients presenting in the neonatal
strli#{244}nMthei5f#{246}ichi. The bronchial ob- period, as deterioration in the clinical status
of the patient is frequent, and conservative
ductus arteriosus, aberrant vesseLand en- treatment without lobectonlv is usually
larged heart or lv mph npcisJntniisic fatal.
bionchial btriition may be caused by a The typical roentgenognaphic Ill ani festa-

defiienc fbFonchitTciT#{228}Ie, bron- tions of infantile lobar emphysema have

chial stenosis, and redi#{241}idIUThronchial been described in the literature;346 they
 VOL. 98, No. 2 Infantile Lobar Emphysema 357

can be divided into signs of a distended lobe SUMMARY

and signs of pressure effects on adjoining Recognition of infantile lobar emphy-

viscera. The involved lobe is enlarged, at- sema as the cause of respiratory distress in
tenuation ofV#{228}c#{252}I#{227}Yiifrkih Tireent, infancy is primarily a problem of roentgen
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an yper ucencv is noted. Pressure phe- diagnosis. Roentgen examination of the

chest reveals a distended lobe compressing
p- adjacent lung and displacing the heart and
posite 51 e, epression o the diaphragm, mediastinum to the opposite side. Two
of cases of infantile lobar emphysema are re-
lung in o t e anterior mediastinum. he 2 ported in which the emphysematous lobe
pres- was fluid-filled, causing a hazy density in
sureeffects, wit shifiEof the mediastinum the involved area on the chest roentgeno-
andcoflapse of gram. The origin of the fluid is not deter-
and notewortj.yJeate-i#{128},4h*t-iti-eadrcase mined, but aspiration of amniotic fluid is
the1I5is not radiolucent, bu-t rather a considered as a possibility. The roentgen
hazygensity was recognizedwithjoor def- diagnosis of infantile lobar emphysema
inition of the vascular markings. This is should be based on signs of overdistention,
believed to 15due toHiiidti#{241}iulation in not hyperlucency of the lobe.

the fluid in these lobes is speculative. The E. A. Franken, Jr., M.D.

Department of Radiology
presence of a large number of squames in
Indiana University Medical Center
Case H suggested aspiration of amniotic 1100W. Michigan
fluid at the time of delivery. In Case i only Indianapolis, Indiana
an occasional squame was seen, and this is
not unusual in a newborn infant. Delayed We acknowledge the assistance of G. J.
absorption of the alveolar fluid found in Kurlander, M.D., in preparation of this paper.
fetal lungs is another possibility. Recently,
Allen et al. reported a similar case in which REFERENCES
they noted dilated lymphatic channels in
T. ALLEN, R. P., TAYLOR, R. L., and REIQUAM,
septa of the lung. The microscopic slides in C. W. Congenital lobar emphysema with di-
our cases did not show these lymphatics. lated septal lymphatics. Radiology, 1966, 86,
It is of some interest that of the cases 929-93 I.

we have personally encountered, the 2 who 2. BOLANDE, R. B., SCHENIDER, A. F., and BOGGS,
presented in the neonatal period had ede- J. D. Infantile lobar emphysema; etiological
concept. A.M.A. Arch. Path., 1956, 6z, 289-294.
matous lobes, both roentgenographically
3. FISCHER, H. W., LUCIDO, J. L., and LYNXWILER,
and pathologically. Lucent, air-filled lobes C. D. Lobar emphysema. 7.A.M.A., 1958, z#{243}#{243},
typical of lobar emphysema were found in 340-345.

the patients whose onset of symptoms was 4. HAMILTON, L. C., and GILLESPIE, R. W. Con-
late. Further experience is necessary to de- genital hypertrophic emphysema. AM. J.
ROENTGENOL., RAD. THERAPY & NUCLEAR
termine if this is of real significance.
MED., 1958, 8o, 42I428.
As a result of experience with these 2
. LEAPE, L. L., and LONGINO, L. A. Infantile lobar
cases, we suggest that the diagnosis of in- emphysema. Pediatrics, I 964,34, 246-255.
fantile lobar emphysema should be con- 6. VAN Epps, E. F., and DAVIES, D. H. Lobar
sidered in infants with respiratory distress emphysema. AM. J. ROENTGENOL., RAD.
THERAPY & NUCLEAR MED., 1955, 73, 375-386.
if roentgenographic signs of an overex-
7. ZATZKIN, H. R., COLE, P. M., and BRONSTHER,
panded lobe are present, even in the ab- B. Congenital hypertrophic lobar emphysema.
sence of hypenlucency of the lobe. Surgery, 1962,52, 505-5 12.