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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
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
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
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.
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.
SURGICAL REPAIR
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.
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
SUMMARY
ACKNOWLEDGMENT
cessful management of some of the patients which prompted this review antl
tlie earlier report.
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