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COLLECTIVE REVIEW

Congenital Eventration of the Diaphragm


Thomas V. Thomas, M.D.

A nomalies of the diaphragm, particularly eventration, are not en-


countered frequently in clinical practice. A majority of the
adult patients with eventration of the diaphragm are asympto-
matic, and the diagnosis is usually entertained only during roent-
genographic surveys or during investigation for unrelated symptoms.
Although this anomaly of part or all of the hemidiaphragm is an un-
common entity, it deserves stress in the literature and consideration
in both differential diagnosis and specific therapeutic measures. Patients
with significant eventration of the diaphragm, particularly infants and
children, may be in acute pulmonary, cardiac, or gastrointestinal dis-
tress, and a clear understanding of the diagnosis and management may
be lifesaving.
Congenital eventration of the diaphragm has been described in the
medical literature under titles such as nonparalytic eventration, neuro-
genic muscular aplasia of the diaphragm, atrophy of the diaphragm,
high-rising diaphragm, and membranous diaphragm [12, 19, 28, 3 1,
461. Despite the difference in various concepts, eventration is generally
accepted as an abnormally high position of part or all of the diaphragm,
usually associated with a marked decrease in muscle fibers and a mem-
branous appearance of the abnormal area [lo, 12, 15, 291.
Eventrations of the diaphragm are divided etiologically into two
groups: congenital, or nonparalytic, and acquired, or paralytic, types
[46].Congenital eventrations are further divided on an anatomical basis
as follows: (1) complete (total); (2) partial: and (3) bilateral.
Partial eventrations are separable, based on the embryological seg-
ments contributing to the formation of the normal diaphragm, into the
following types: (1) anterior; (2) posterolateral; and (3) medial.
From the Department of Surgery, University of Kansas School of Medicine, and Veterans
Administration Hospital, Kansas City, Mo.
Address reprint requests to 4801 Linwood Blvd., Kansas City, Mo. 64128.

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COLLECTIVE REVIEW: Congenital Eventration of Diaplzragm

The clinical picture of congenital and acquired eventration may


be similar, and separation is often difficult in the absence of a history
of trauma to the phrenic nerve on the affected side. The separation of
the eventrated diaphragm from a paraesophageal diaphragmatic hernia
is valuable, particularly in asymptomatic patients.

HISTORY
Jean Louis Petit [341 in 1774 was the first to recognize this entity
during autopsy studies. It was Beclard [7] in 1829, however, who coined
the word eventration. Wood [491 in 1916 expounded further on eventra-
tion and made a plea that one should not confuse this with diaphrag-
matic herniations. In 1923 Morrison [31] performed the first successful
repair of an eventrated diaphragm and utilized one of the presently
applied techniques of plication to remove the redundancy associated
with it. Bisgard [I01 in 1947 did the first successful repair of congenital
eventration of the diaphragm. He provided the presently accepted
definition of eventration as “an abnormally high or elevated position
of one leaf of the intact diaphragm as a result of paralysis, aplasia, or
atrophy of varying degrees of the muscle fibers. T h e unbroken con-
tinuity differentiates it from diaphragmatic hernia.” Bilateral eventra-
tions of the diaphragm in infants and children have been reported by
Avnet [4] and others [21, 261. With a greater awareness of this entity,
a larger number of these lesions are suspected on plain chest roent-
genograms, and specific investigations as well as treatment are institutcd
in symptomatic patients.

DEVELOPMENT A N D INCIDENCE
Development of the diaphragm and separation of the coelomic
cavity into thoracic and abdominal cavities takes place sometime during
the eighth to tenth weeks of intrauterine life. T h e septum transversuin
arises caudal to the heart and grows dorsally to join the mesentery of
the foregut. The pleuroperitoneal membrane arises from the dorso-
lateral aspect and meets the ventral portion of the septum transversum
and medial segment of the mesentery. Defects or failure of fusion of
these elements result in herniation of the abdominal contents into the
thoracic cavity. Pleuroperitoneal folds of the diaphragm are composed
only of reflections of pleura and peritoneum until later in intrauterine
life. This layer is thought to be heavily impregnated later by an in-
growth of cervical myotomes between the two membranes. T h e right
hemidiaphragm usually closes first. Early in the development of the
septum it moves caudally from the cervical region, carrying with it the
nerve supply [29, 33, 35, 471.
With the normal progression of intrauterine changes, the midgut

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THOMAS

rotates and returns to the abdominal cavity at about the tenth week of
fetal life. A premature return of the viscera to the peritoneal cavity is
believed to be a factor in the delay or failure of the diaphragm to
develop normally, sometimes resulting in defects or stretched-out por-
tions rising high into the thoracic cavity. These may be associated with
the absence of ingrown striated muscles into the septum. Anomalies
of the abdomen or a rapid enlargement of the abdominal contents may
also be contributing factors. T h e lung may be the victim of compression,
hypoplasia, or agenesis on the ipsilateral side. T h e abdominal orgahs
may encroach on the pleural space by migration with a mediastinal
shift and rotation of the heart. T h e spleen, stomach, colon, kidney,
small bowel, liver, and adrenal gland may be found under the high-
rising portion of the diaphragm. Bony abnormalities of the sternum,
ribs, and vertebrae may also be encountered with congenital eventra-
tions [2, 3, 15, 16, 33, 47, 501.
T h e peripheral portions of eventrated diaphragm may have usually
well-developed muscular elements. T h e involved area of the diaphragm
may be sparsely muscularized or converted to a translucent aponeurotic
membrane. In an analysis of 2,500 chest roentgenograms on infants,
Beck and Motsay [6] found the frequency of eventration to be 4%, but
only 3 infants had symptoms attributable to the anomaly. Chin and
Lynn [17] in 1956 reviewed 412,000 chest roentgenograms and found
only 32 patients with the diagnosis of eventration. Only 5 of their
patients were symptomatic enough to require surgical treatment. T h e
clinical separation of congenital eventrations is unreliable in adults,
and it is often attained by a process of excluding the factors likely to
contribute to phrenic nerve paralysis.

CLINICAL PICTURE
Many patients with complete eventration of the hemidiaphragm
and most patients with partial eventration usually remain asympto-
matic; therefore, diagnosis is often an incidental feature. Changes in
the compliance of lungs and chest wall, or excessive weight gain, may
cause symptoms in a hitherto asymptomatic patient [30, 401. When the
patient is symptomatic, there may be little, if any, difference in sympto-
matology between a large diaphragmatic hernia and eventration. An
evaluation of the maternal, obstetric, and pediatric history is helpful in
excluding acquired eventrations. Acute respiratory distress and difficul-
ties in feeding are the most pronounced symptoms of eventration in
infants [15, 24, 40, 431. These infants may be dusky and occasionally
cyanotic. In children there may be evidence of repeated attacks of
pneumonitis on the affected side and in some cases bronchitis or
bronchiectasis. There may be exaggeration of cardiorespiratory and

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COLLECTIVE REVIEW: Congenital Euentralion of Dinphragnz

gastrointestinal symptoms when the patient lies flat or in a head-down


position, or after heavy meals [15, 18, 201. An acute shift of the mediasti-
num may precipitate cardiac arrhythmias and obstruction to venous
re turn.
Nonspecific gastrointestinal symptoms, such as lack of appetite,
inability to gain weight, epigastric discomfort, heartburn, belching,
constipation, and symptoms of intermittent intestinal obstruction, may
be seen frequently in children or adults. Pain may be referred to the
periumbilical area when there is pulling or stretching of the mesentery
or when there are fibrous bands across the intestines and mesentery.
Palpitation, tachycardia, and dyspnea at rest or on exertion are frequent
complaints of adults [l, 27,291. These symptoms may show characteristic
aggravation with changes in posture [15].
In patients with a moderate or severe degree of eventration, a
scaphoid-shaped abdomen and fullness in the lower chest on the affected
side may be obvious on inspection 12, 3, 81. Uninhibited divergence of
the lower intercostal spaces during inspiration was first described as a
characteristic finding of eventration by Hoover in 1920 [22]. Dullness
on percussion of the lower chest, diminished or absent breath sounds,
and the presence of peristaltic sounds high in the chest are other physi-
cal findings which could raise the question of eventration of the dia-
phragm [12, 16, 20, 251. T h e heart and trachea may be shifted to the
opposite side with cephalad or contralateral shift of apical pulsation
(pseudodextrocardia) [ 151. Increased anteroposterior diameter of the
lower chest on the ipsilateral side may be another finding. When there
is extreme redundancy of the diaphragm, a flapping sound may be heard
during respiration as an additional sign [29]. In most of the patients,
physical examination may contribute little to the diagnosis of eventra-
tion.

DZA GNOSZS
Localized eventrations are almost always asymptomatic, and the
diagnosis is usually confirmed only at thoracotomy for unrelated surgical
procedures or during autopsy (Fig. 1). Posteroanterior and lateral chest
roentgenograms may give conclusive evidence in a majority of cases, but
separation from a small diaphragmatic hernia is usually difficult [23]. In
symptomatic patients further investigations are superfluous, especially
in infants. Barium enema and upper gastrointestinal studies may show a
high-rising colon or upside-down stomach with or without volvulus
and a thin diaphragmatic outline overlying it (Fig. 2). A necklike area
on barium studies is believed to be more indicative of a diaphragmatic
hernia. Other contrast-material studies and fluoroscopy should be con-
sidered if diagnosis is uncertain, and they may be essential to exclude

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THOMAS

A B
FIG. 1. Posteroanterior ( A ) and right lateral ( B ) cheJt roentgenograrns of a n
asymptomatic patient showing partial euentration of t h e right hemidiaphragm.
Comparison with the chest roentgenograms taken 4 , 10, and 12 years earlier on
this patient showed no change in the appearance.

lesions such as pulmonary cysts, pericardial cysts, intrathoracic lipomas,


duplication cysts, and subphrenic abscesses. On fluoroscopic examina-
tion a true paradoxical motion is not present in congenital eventration
of the diaphragm. T h e initial passive upward movement of the redun-
dant portion of the diaphragm, until the slack is taken up, is sometimes
confused with paradoxical motion [lo, 15, 17, 231. T h e muscularized
portions will contract simultaneously with the opposite diaphragm. Bi-
lateral medial segmental eventrations of the diaphragm are often con-
fused with mediastinal tumors; however, on fluoroscopy these areas may
show respiratory diaphragmatic movements [4,261. Inversion of the left
hemidiaphragm with pleural effusion has been described recently as a
new diagnostic problem in diaphragmatic pathology [45].
Duplication and scalloping of the diaphragm occasionally add to
the complexity of differential diagnosis [ 151. Scalloping (hypertrophy
and contraction of muscle bundles) of the diaphragm may alternate
with localized eventrated portions in the diaphragm. Roentgenograms
of the chest, with the patient in the lateral decubitus position, are
usually helpful in delineating the location of subpulmonic (supradia-
phragmatic) accumulation of fluid, which casts the shadow of a high
diaphragm. Diagnostic pneumoperitoneography was first introduced by
Zeitlin in 1930 [50]and has been widely used since then. Carbon dioxide

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COLLECTIVE REVIEW: Congenital Evetitration of Diaphragm

F I G . 2.CheJt ioentgenogianz of a ?S-yeai-oid m a n with minimal s h o i l n e ~of ~


breath, occasional palpitation, and some left upper quadrant discomfort, showiiig
rotation of the heart to the right and a markedly elevated left hemidiaphragm.

or nitrous oxide, 7 to 10 cc. per kilogram of body weight, has been


preferred over atmospheric air or oxygen for this study [Zl]. Upright
chest roentgenograms after introduction of diagnostic pneumoperito-
neum are usually confirmatory and clearly outline the aponeurotic
portion of an eventrated diaphragm. Laminagrams may be useful in
some instances. Radioisotope photoscanning of the liver alone or in
combination with the lungs is also a valuable adjunct to diagnostic
procedures [35, 461. A decrease in perfusion in the lower lobes of the
affected side has been demonstrated by radioisotope scans and pul-
monary angiograms [2, 8, 401. Bronchospirometry of the affected lung
has shown a decrease in ventilation of as much as 50 to 70% according
to some studies [25,40,48]. This information is valuable for comparison
of postoperative results, although lungs with well-developed fibrotic
changes are likely to show little or no improvement after repair of
eventration. Patients with poor pulmonary function, however, may be
expected to have some gain following repair [40]. Bronchoscopy and
bronchography may reveal compression of the ipsilateral lower lobe
along with deviation and upward displacement of the upper lobe
bronchus [18, 25, 311. Although the normal diaphragm may account for
as much as 60% of the total ventilatory motion [48], pulmonary com-
pliance is considered to be an important factor [30]. T h e average excur-
sion of the dome is approximately 3.5 cm. [30], which may be diminished
due to poor diaphragmatic contraction in eventration. Pulmonary func-
tion studies on some patients reported in the literature show an im-

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THOMAS

provement of as much as 50'); on the affected side [12, 18, 25,40,43,441.


If history and preoperative investigations are not helpful in determin-
ing the developmental nature of eventration, it may be confirmed by
faradic stimulation of the phrenic nerve at the time of thoracotomy [29,
401. T h e technique of recording intragastric pressure during respiratory
variations (Schlippe's test) is of little value in the diagnosis of eventra-
tion of the diaphragm [39].

PREOPERA TI VE M A N A G E M E N T
Infants with cyanosis or acute respiratory distress may be saved by
prompt recognition and institution of specific therapy. Resuscitative
measures with a face mask are to be discouraged if a large eventration
or diaphragmatic hernia is suspected because of the danger that symp-
toms may be aggravated by inflating the stomach with air or oxygen
[3, 6, 29, 421. Endotracheal intubation with positive-pressure ventilation
and sufficient humidity are usually lifesaving measures. A high con-
centration of oxygen, in addition to relieving cyanosis, helps to mini-
mize the necessity of excessive diaphragmatic excursion. Humidity
prevents drying u p of bronchial secretions and reduces the insensible
loss of water. Continuous gastric decompression with the use of a
nasogastric tube is extremely helpful in removing swallowed air and
gastric secretions while minimizing impingement of the stomach on
the diaphragmatic motion. T h e patients who are not supported by
positive-pressure breathing should be maintained in a semiupright
position on the affected side. Since many of these infants are acidotic,
it may be necessary to administer sodium bicarbonate prior to opera-
tion [S].
Congenital eventrations may remain asymptomatic until later in
life when obesity or pulmonary disease becomes involved. Sometimes
congenital eventration of the diaphragm is complicated by cardiac,
pulmonary, or gastrointestinal anomalies, and it is imperative to recog-
nize the dominant lesion responsible for the symptoms. Necessary
parenteral fluids, electrolytes, and vitamins are administered while the
patient is being evaluated for surgical correction. Adult patients who
have multiple complaints and are in no acute distress should be investi-
gated carefully in order that the surgeon may ascertain the role of the
eventrated diaphragm before recommending surgical treatment [27,
29, 461. Although small localized eventrations are asymptomatic, small
diaphragmatic hernias are likely to be associated with varying degrees
of symptoms and complications. Therefore an attempt should be made
to separate the two entities. Preoperative laxatives and enemas are
helpful in obtaining an empty bowel and reducing unnecessary tension
on the suture lines in the diaphragm.

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C O I . L ~ T I V E REVIEW: Congenital Eventration of Diaphragm

SURGICAL REPAIR

My experience has been with a total of 12 symptomatic patients at


three separate institutions. Eight symptomatic patients with congenital
eventration, 6 of whom had successful repairs, have been reported
earlier [461. No operation was recommended for an infant in the remain-
ing group who had agenesis of the left lung, multiple anomalies, and
partial eventration of the right hemidiaphragm. In another case a
partial asymptomatic eventration of the left hemidiaphragm was re-
paired during a thoracotomy for a penetrating wound of the medias-
tinum. A 78-year-old man (Veterans Administration Hospital, Kansas
City, Mo.) with a history of several years of intermittent upper abdom-
inal pain, discomfort, and palpitation was not operated upon because
of prompt relief of symptoms with parenteral fluids and nasogastric
suction (Fig. 3). I collaborated in repairs done in two other patients, a 68-
year-old woman (St. Thomas Hospital, Nashville, Tenn.) and a 5-day-old
infant (Children’s Hospital, Louisville, Ky.), both of whom have made
uneventful recoveries. Recently, an asymptomatic eventration of the
entire left hemidiaphragm in a 2 l-year-old primigravida suddenly be-
came symptomatic when the woman reached full-term pregnancy, with

A B
FIG. 3. ( A ) Postcroantel-ior chest roentgenogram of patient with history sug-
gestive of recurrent large bowel obstrirction occurring mon.ths and years apart and
spontaneous remission after several hours. T h e left hemidiaphragm is thin and
located high, producing shift of nzediastinal structures. ( B ) Lateral view of the
chest of the same patient shows the entire left hemidiaphragm to be a rnern.branot.1~
.rtrzirtnre w i t h gas-filled co.’onic scgmcnts occirpying the lower thoracic region.

VOL. 10, NO. 2, AUGUST, 1970 187


THOMAS

tachycardia, orthopnea, constipation, and severe, cramping left upper


quadrant pain. Roentgenograms of the chest and abdomen were con-
sistent with volvulus of the splenic flexure and transverse colon under
the eventrated left hemidiaphragm. Induced labor and forceps delivery
corrected the volvulus of the colon and respiratory distress. She is a can-
didate for elective repair of the eventration before the next pregnancy.
T h e consensus of most authors is in favor of a transthoracic ap-
proach for repair of the eventration. Adequate mobilization of the
diaphragm to the level of the primary attachments is unlikely through
the abdominal approach. T h e overhanging costal cartilages and the
presence of an easily traumatized spleen considerably limit thorough
exploration and mobilization of the diaphragm. T h e thoracic approach
also reduces the likelihood of postoperative ileus. Surgical treatment
has been strongly advocated and successfully carried out by Bisgard [lo],
State [42], Butsch and Leahy [14], Shellito and Bartlett [41], Koop and
Johnson [24], Beck and Motsay [6] and others [18, 20, 27, 36, 40, 43, 461
on symptomatic patients. With a better understanding of the physiologi-
cal changes in eventration and improvements in surgical techniques and
anesthesia, as well as subjective and objective evidence of improvement
following repair, most authors justify surgical treatment.
T h e transthoracic approach is accomplished through a postero-
lateral thoracotomy with an incision along the fifth, sixth, or sometimes
seventh intercostal space, as has been advocated by most authors. When
the infants are in acute respiratory distress, an anterolateral thoracotomy
with the head end of the table tilted u p may be preferable. T h e dia-
phragm is carefully mobilized to the level of its primary intercostal
attachments. I like to open the membranous portion and free the at-
tachments to the colon, spleen, stomach, or other abdominal viscera,
resect the excess membranous portion, and carry out a two-layer over-
lapping type of approximation with nonabsorbable interrupted sutures
[46]. In infants with acute symptoms, this approach has not been used
because of the longer operative time and dissection required. Most
reports in the literature favor a simple plication of the diaphragm in
adults and children to remove the redundancy [lo, 20, 24, 29, 36, 421.
Opening the diaphragm provides a valuable but limited exploration
of the upper abdomen and avoids inadvertent injury to underlying
viscera.
T h e abdominal approach is recommended when bilateral eventra-
tions are encountered, since total correction can be accomplished
through one incision during the same procedure [21]. Others have
utilized two separate thoracotomies a few weeks apart [4,261. Care
should be taken to avoid injury to the major ramifications of the phrenic
nerve during repair. For better function and results, an attempt is made
to bring together the muscularized portions of the diaphragm with good

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COLLECTIVE KEviEw: Congrnital Euenti ation of Dinphi ngrn

contractility. If the medial segment of the diaphragm is inadequate, the


pericardium may be reflected and sutured to reinforce the area [46].
Different autogenous tissues, such as fascia lata, portions of the ab-
dominal or chest wall, or synthetic materials have been used by others
[37]. Gastric decompression and waterseal drainage of the pleural space
are provided in the immediate postoperative period. Enthusiastic at-
tenipts at inflating a hypoplastic or fibrotic lung to fill the dead space
are unwarranted and may cause a rupture of the alveoli or emphysema-
tous changes [8]. Pulmonary hypoplasia is a real threat to the survival
of newborn infants with corrected eventrations as well as diaphragmatic
hernia. Areechon and Reid [ Z ] believe that these small lungs have
differentiated to the alveolar phase from the glandular stage and that the
number of alveoli found distal to the terminal bronchioles is normal.
This may mean that the correction of the deformity will lead to aeration
and growth of the hypoplastic lung [Z,81.
It is possible in rare instances that with the laxity of the diaphragm
corrected and visceral contents returned, the peritoneal cavity will not
be a large enough receptacle. T h e creation of a temporary ventral
hernia may be necessary, as has been advocated with the correction of
a large diaphragmatic hernia, in infants 1241. This may be helpful in
avoiding disruption of the repair or obstruction of lymphatic and ve-
nous return. Recurrence of the eventration, although rare, may be a
factor with inadequate repair or injury to phrenic nerve. In 1954
Bingham [9] reported two cases of eventration following breech delivery.
Some of the infants with eventration noted as a complication in obstetric
procedures usually got complete relief within days or weeks [28, 381.
McNamara and associates [27] have advocated the reconstruction of the
cardioesophageal junction to prevent gastroesophageal reflux following
repair of eventration.

P A T H O L O GY
A majority of the eventrations, particularly the cornplete variety,
are located on the left side. Reed and Borden [36], in an analysis of 183
cases, found 165 eventrations to be located on the left side. Men are
affected more frequently than wcmen. Butler and Claireaux [ l S ] esti-
mated that diaphragmatic anomalies, such as hernia and eventration,
accounted for 1.4% of all Ferinatal deaths. Although several maternal
and infantile diseases have been considered to be etiological lactors
in eventration of the diaphragm, true nonparalytic or congenital even-
tration is thought to be a weakness of the diaphragm resulting from
a lack of, or inadequate, muscularization. With a history of trauma, in-
flammatory processes of the respiratory tract and upper abdomen, or
neoplastic growths, the acquired type of eventration is more likely. In
those cases a true paradoxical motion of the diaphragm is seen on

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THOMAS

fluoroscopic examination and no response can be elicited upon faradic


stimulation of the phrenic nerve [5, 9, 11, 15, 18, 19, 481.
In congenital eventration the phrenic nerve may be smaller, but it
is intact and will respond to electrical stimulation. T h e term neurogenic
muscular aplasia of t h e diaphragm is probably more representative of
the abnormalities observed than the common term euentration [12].
T h e diaphragm in these patients is usually a thin, stretched-out, aponeu-
rotic structure with a few scattered muscular fibers. A quantitative
deficiency of nerve fibers is also noted in this area [29, 401; however,
the edema, hyalinization, or degeneration of nerve fibers and muscles
seen in acquired eventrations is lacking. Eventration of the diaphragm
has often been compared to a direct defect in the inguinal region, in
contradistinction to the comparison of diaphragmatic hernia to an
indirect type of hernia with a sac forming the covering. With congenital
eventration some authors feel that three separate layers should be visible
on microscopical examination. T h e attenuated portion is often made u p
only of fibroelastic tissue. N o reports are available to indicate the long-
term changes in histological structure of the diaphragm with phrenic
paralysis, especially in regard to the possibility that such changes are
inseparable from the congenital eventration.

SUMMARY

Although abnormal elevation of the diaphragm is seen in only a


small percentage of the population, the need for accurate diagnosis and
a knowledge of the etiological factors is well known. Surgical therapy
may be required in only a small percentage of such patients, but it may
be a lifesaving measure in infants and children with respiratory insuffi-
ciency. Cardiorespiratory and gastrointestinal symptoms are usually
associated with large eventrations of the diaphragm. Surgical repair is
recommended for symptomatic patients, and success is achieved with a
low morbidity or mortality. T h e results of surgical correction are usually
gratifying and often dramatic. This review deals primarily with the
congenital variety of eventrations, the clinical picture, diagnosis, treat-
ment, results, and pathological changes of this entity. T h e observations
made in this review have been accumulated from reports in the litera-
ture as well as from clinical experience with a limited number of patients.

ACKNOWLEDGMENT

I would like LO express my appreciation to Herbert T. Ransdell, Jr., M.D.,


Professor of Surgery, Section of Thoracic Surgery, University of Louisville School
of Medicine, Louisville, Ky., for the supervision and encouragement in the suc-

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COLLECTIVE REVIEW:Congenital Eventration of Diaphragm

cessful management of some of the patients which prompted this review antl
tlie earlier report.

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192 THE ANNALS OF THORACIC SURGERY

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