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Diaphragmatic

Eventration
Shawn S. Groth, MDa, Rafael S. Andrade, MDb,*

KEYWORDS
 Diaphragmatic eventration  Diaphragm plication
 Diaphragmatic paralysis  Laparoscopy  Quality of life

Diaphragmatic eventration is an uncommon PATHOLOGY


condition that is usually discovered incidentally in
patients who are asymptomatic and who have an Diaphragmatic eventration can be bilateral,
elevated hemidiaphragm on chest X ray. The unilateral, total, and localized (anterior, posterolat-
etiology and pathology of diaphragmatic eventra- eral, and medial)6; localized eventrations can
tion and paralysis are distinct; however, the clinical affect any portion of the diaphragm.1 Microscopi-
presentation in adults is similar and these two cally, the eventrated portion has diffuse fibroelas-
conditions are sometimes impossible to distin- tic changes and a paucity of muscle fibers.14,15
guish from each other. The treatment for symp- Patients who have diaphragmatic paralysis have
tomatic patients with diaphragmatic eventration a normal amount of muscle fibers, albeit atrophic.
and paralysis is the same: diaphragm plication.
Minimally invasive diaphragm plication techniques PATHOPHYSIOLOGY AND CLINICAL
are now effective alternatives to open plication. PRESENTATION
This review focuses on the etiology, pathophysi-
ology, diagnosis, and treatment of diaphragmatic Most adult patients who have diaphragmatic even-
eventration in adults. tration are asymptomatic and generally present
with an elevated hemidiaphragm discovered
ETIOLOGY incidentally on a chest X ray.2,7 Some patients
who have diaphragmatic eventration do not
True diaphragmatic eventration is a congenital become symptomatic until adulthood because of
developmental defect in the muscular portion of weight gain or because of a change in lung or
the diaphragm with preserved attachments to the chest-wall compliance.7
sternum, ribs, and dorsolumbar spine.1 Diaphrag- Dyspnea on exertion and orthopnea (because of
matic eventration is rare (incidence <0.05%), is further cranial displacement of the affected hemi-
more common in males, and more often affects diaphragm when supine) are the main symptoms
the left hemidiaphragm.2–4 Embryologic theory of an elevated hemidiaphragm. Normal caudal
postulates that abnormal or delayed migration of movement of the diaphragm during inspiration
myoblasts from the upper cervical somites leads increases thoracic volume and is pivotal for appro-
to a structural deficiency of diaphragmatic priate lung inflation. Patients who have diaphrag-
muscle.5–7 matic eventration may not have the normal
In contrast to true diaphragmatic eventration, caudal movement of the diaphragm necessary
diaphragmatic paresis or paralysis is a more for appropriate inspiration16; diaphragmatic move-
common, acquired condition that generally results ment can be diminished, absent, or even
from tumor- or trauma-related phrenic nerve paradoxic. As a result, ventilation and perfusion
injury.8–13 to the basal portion of the lung ipsilateral to the
thoracic.theclinics.com

a
Department of Surgery, University of Minnesota, MMC 207, 420 Delaware Street, SE, Minneapolis, MN 55455,
USA
b
Division of General Thoracic and Foregut Surgery, Department of Surgery, University of Minnesota, MMC
207, 420 Delaware Street, SE, Minneapolis, MN 55455, USA
* Corresponding author.
E-mail address: andr0119@umn.edu (R.S. Andrade).

Thorac Surg Clin 19 (2009) 511–519


doi:10.1016/j.thorsurg.2009.08.003
1547-4127/09/$ – see front matter ª 2009 Elsevier Inc. All rights reserved.
512 Groth & Andrade

eventrated diaphragm are impaired17; ventilation/ diaphragm is exceptionally redundant, a flopping


perfusion mismatch and loss of chest-wall sound may be heard on auscultation.21
compliance are among the factors that contribute
to dyspnea. Some patients develop mild hypox-
emia and attempt to compensate by hyperventilat- Pulmonary Function Tests
ing, which can result in mild respiratory Pulmonary function tests (PFT) may provide an
alkalosis.4,18 Other patients (especially those with objective measure useful in the assessment of
left hemidiaphragm eventration) can develop patients who are dyspneic and who have an
nonspecific gastrointestinal symptoms, such as elevated hemidiaphragm. Because diaphragm
epigastric pain, bloating, heartburn, regurgitation, dysfunction reduces the compliance of the chest
belching, nausea, constipation, and inability to wall, a restrictive pattern (ie, low forced vital
gain weight.6,7 capacity [FVC] and forced expiratory volume
in 1 second [FEV1]) is often seen.4
DIAGNOSIS The diaphragm is a critical mediator of inspira-
tion; therefore, assessing inspiratory PFT parame-
The evaluation of patients who are symptomatic ters (eg, maximum forced inspiratory flow
and who have diaphragmatic eventration should [FIFmax]) is important.
include an objective assessment of dyspnea, Additionally, FVC should be assessed in the
physical examination, pulmonary function tests, upright and supine position. Supine FVC in healthy
and imaging studies. The diagnosis of symptom- individuals can decrease up to 20% from upright
atic hemidiaphragm eventration is primarily values,22 and supine lung volumes may decrease
clinical, and relies mostly on history, chest X ray, by 20% to 50% in patients who have diaphrag-
and the physician’s clinical acuity. matic eventration or paralysis.18,23,24
Although PFT are often abnormal in symptom-
Symptom Evaluation atic patients who have diaphragmatic eventration,
A careful history of the duration and progression of these changes are neither consistent nor do they
dyspnea and orthopnea is critical. Patients who correlate with the severity of dyspnea. The main
have diaphragm paralysis can often recall when value of PFT is to provide an objective evaluation
dyspnea started or worsened (eg, after cardiac of the result of surgery.
surgery); patients who have eventration may not
be able to determine a specific starting point. Imaging Studies
Clearly, any additional causes for dyspnea (eg,
morbid obesity, primary lung disease, heart failure, Chest X ray
and so forth) should be investigated and corrected On a standard full-inspiration posteroanterior and
if possible, because dyspnea secondary to lateral (PA/LAT) chest X ray, the right hemidiaph-
diaphragmatic eventration or paralysis is mainly ragm is normally 1 to 2 cm higher than the left.25
a diagnosis of exclusion. Hemidiaphragm elevation can be a sign of dia-
All patients who have dyspnea secondary to dia- phragmatic eventration or paralysis; however,
phragmatic eventration should fill out a standard- this is a nonspecific finding because a variety of
ized respiratory questionnaire to evaluate the pulmonary (ie, atelectasis and fibrosis), pleural
severity of their symptoms and to assess the (ie, pleural effusions and masses), and subdiaph-
response to treatment. ragmatic processes (ie, hepatomegaly, spleno-
megaly, gastric dilatation, and subphrenic
Physical Examination abscesses) can also cause elevation of a hemi-
diaphragm.26 Consequently, further studies may
Physical examination adds little to the diagnosis of be needed if an elevated hemidiaphragm is noted
diaphragmatic eventration. Nonetheless, two on a chest X ray in the presence of dyspnea.
characteristic findings may be present and are
worth mentioning: (1) paradoxic inward movement Fluoroscopic sniff test
of the lower costal margin during inspiration During fluoroscopy, patients are instructed to sniff,
(known as Hoover’s sign),19 and (2) abdominal and diaphragmatic excursion is assessed. Nor-
paradox (the rib cage and abdomen move out of mally, the diaphragm moves caudally. In patients
phase with each other).16,20 Other nonspecific who have hemidiaphragmatic paralysis, the dia-
respiratory signs include diminished maximal phragm may (paradoxically) move cranially.
excursion of the diaphragm on percussion, dimin- Patients who have diaphragmatic eventration,
ished breath sounds, and increased anteroposte- however, may also exhibit passive upward move-
rior diameter of the chest.6,16 If an eventrated ment of the diaphragm when sniffing.6
Diaphragmatic Eventration 513

Fluoroscopy findings should be interpreted with results,33 (3) there is a lack of consensus on the
caution. First, approxiately 6% of normal individ- most appropriate procedural technique,33,34
uals exhibit paradoxic motion on fluoroscopy27; (4) there are no population-based norms,32 (5) there
to increase the specificity of this study, at least can be significant intra- and interindividual
2 cm of paradoxic motion should be noticed.16 variability,26,32 and (6) it is not critical in aiding the
Second, an eventrated or paralyzed hemidiaph- clinician to decide whether diaphragm elevation is
ragm may move very little or not at all, without responsible for dyspnea.
paradoxic motion, making the interpretation of
the sniff test and the distinction between paralysis Phrenic nerve conduction studies
and eventration even more challenging. Phrenic nerve function can be assessed by stimu-
lating the nerve transcutaneously in the neck.16,32
Ultrasound In addition to assessing conduction time (normal,
Ultrasound (US) can be used to assess the thick- < 9.5 milliseconds),35 phrenic nerve conduction
ness and the change of thickness of the dia- studies can be coupled with (1) diaphragmatic
phragm during respiration; it has approximately electromyography using surface electrodes to
80% concordance with fluoroscopy findings.26–30 measure the action potential of the diaphragm35
However, US has not been validated in clinical or (2) Pdimax assessment during phrenic nerve
practice. stimulation.36 This test is of little clinical value
since it is cumbersome and is not essential to
CT determine whether dyspnea is secondary to hemi-
The principal utility of CT scans is to exclude the diaphragm elevation.
presence of a cervical or intrathoracic tumor as In summary, to establish that diaphragmatic
the cause of phrenic nerve paralysis or to evaluate eventration or paralysis is clinically relevant,
the possibility of a subphrenic process as the patients must have dyspnea that cannot be solely
cause of hemidiaphragm elevation. However, attributed to another process (ie, primary lung or
a CT scan is not routinely required if the clinical heart disease) and must have an elevated hemi-
suspicion of an alternate process is low. diaphragm on a PA/LAT chest X ray. On occasion
MRI it is impossible to clinically differentiate eventration
Dynamic MRI can be used to assess diaphrag- from paralysis; however, this distinction is not
essential, as long as the physician establishes
matic motion.31 As compared with fluoroscopy,
which can assess only motion of the highest points that hemidiaphragm elevation is the main cause
of the diaphragm, it has the advantage of enabling for dyspnea and that a potentially serious cause
for paralysis (ie, tumor or neuromuscular disorder)
the study of the motion of segments of the dia-
phragm in multiple planes.26 However, MRI is is not overlooked. A sniff test demonstrating para-
more expensive, more time consuming, more doxic motion adds to the evidence that dyspnea is
secondary to diaphragm paralysis (or on occasion
uncomfortable for patients than fluoroscopy, and
the additional information on segmental diaphrag- eventration), but a negative sniff test does not
matic motion is of no clinical value. Consequently, exclude hemidiaphragm elevation as the main
cause of dyspnea. PFT are of value in objectively
the authors do not recommend MRI as a routine
tool for the evaluation of patients who are symp- assessing patients’ response to diaphragmatic
tomatic with hemidiaphragm elevation. plication. The authors evaluate all patients who
have symptomatic hemidiaphragm elevation,
whether eventration or paralysis, with a standard-
Functional Studies ized respiratory questionnaire, a PA/LAT chest
Maximal transdiaphragmatic pressure X ray, and PFT; the authors obtain a sniff test
The maximal transdiaphragmatic pressure (Pdi- only if they cannot clearly ascertain that dyspnea
max) serves as a surrogate for the force generated is secondary to diaphragm dysfunction.
by the diaphragm. To measure Pdimax (the differ-
ence between intra-abdominal and intrathoracic TREATMENT: DIAPHRAGMATIC PLICATION
pressures), pressure transducers are placed inside
the stomach (to approximate intra-abdominal Surgical repair of diaphragmatic eventration was
pressure) and the esophagus (to approximate intra- first described in 1923.37 Since then, a variety of
thoracic pressure32). However, this technique is not open and minimally invasive diaphragm plication
commonly used to assess patients who have dia- techniques have been described to reduce
phragmatic dysfunction because (1) it is inconve- symptomatic dysfunctional diaphragm excursion
nient, (2) the choice of methodology significantly during respiration. Diaphragmatic pacing is an
impacts the Pdimax and reproducibility of the option generally reserved for patients who are
514 Groth & Andrade

quadriplegic and have bilateral diaphragmatic intercostal space. A variety of plication


paralysis,38–40 and has not been established as techniques have been described, including hand-
a practical approach to unilateral diaphragm sewn U stitches,42,43,46,47 mattress sutures,13,44
eventration or paralysis. This section reviews dia- running sutures with or without pledgets, and
phragm plication for unilateral hemidiaphragm stapling48 techniques with or without mesh.43,49
eventration with particular emphasis on laparo- Another technique includes resecting the redun-
scopic diaphragm plication. dant portion of diaphragm and repairing the tissue
in overlapping layers.7,15 Open transthoracic plica-
Operative Indications tion has also been described as an approach to
The only goal of diaphragm plication is to treat treating bilateral diaphragm paralysis.50
dyspnea; hence, operative intervention is indi- Multiple single-institution studies have demon-
cated exclusively for patients who are symptom- strated significant improvement in symptoms and
atic. An elevated hemidiaphragm or paradoxic respiratory function after open transthoracic plica-
motion per se does not warrant surgery in the tion.42–46,51,52 In a study of 17 subjects who had
absence of significant dyspnea. For adults who unilateral paralysis, Graham and colleagues
have phrenic nerve injury from cardiac surgery, demonstrated that open transthoracic plication
a 1- to 2-year period of observation is often recom- led to significant subjective improvement in dysp-
mended since phrenic nerve function may improve nea and orthopnea and PFT: FVC increased by
with time9,10,30,41; however, patients who are 19% in the upright position and by 42% in the
severely symptomatic may warrant a minimally supine position.45 Five to 10-year follow-up data
invasive plication even after 6 months, because was available for six subjects: durable improve-
dyspnea from diaphragm paralysis can signifi- ments in dyspnea scores and PFT were
cantly impact quality of life and rehabilitation. observed.13 In a study of 19 subjects, Higgs and
Relative contraindications to diaphragm plica- colleagues also demonstrated durable improve-
tion are morbid obesity and certain neuromus- ments in dyspnea scores and PFT after open trans-
cular disorders. Ideally, patients who are thoracic plication at 5- to 10-year follow-up. Open
morbidly obese should be evaluated for medical transthoracic plication has demonstrated that pli-
or surgical bariatric treatment before plication, cating the diaphragm for symptomatic eventration
because dyspnea may improve after significant or paralysis provides clear short- and long-term
weight loss and a plication may no longer be war- benefits. Unfortunately, open transthoracic plica-
ranted. Any type of plication is challenging in tion is invasive, which can preclude the option of
patients who are morbidly obese; the degree of plication in patients who have multiple comorbid-
plication may be compromised because of tech- ities. Consequently, alternative approaches to dia-
nical difficulties, the relief of dyspnea may be phragmatic plication have been developed to
limited, and complications are likely. Patients minimize the disadvantages of the open transtho-
who have neuromuscular disorders, such as racic approach.
amyotrophic lateral sclerosis or muscular
dystrophy, should be approached with extreme Thoracoscopic Plication
caution. The benefits of plication on dyspnea are
moderate at best, and complications are Thoracoscopic plication can be performed using
common. An individualized multidisciplinary two ports with a mini-thoracotomy,53,54 three
approach is necessary to decide on a plication ports,12,55 or four ports.56 Plication techniques
in patients who have morbid obesity or neuromus- including continuous sutures,54,56 interrupted
cular disorders. stitches,12,55 or stapling57 have been described.
Single-institution studies have demonstrated
Surgical Approaches improvement in dyspnea and PFT with thoraco-
scopic plication.12,54,55 The largest series of
The diaphragm can be approached from the thoracoscopic plication was published by
thorax or the abdomen. Either approach may be Freeman and colleagues. In this report of 25
done with open or minimally invasive techniques. subjects who had unilateral diaphragm paralysis,
thoracoscopic plication was successfully per-
Open Transthoracic Plication
formed in 22 subjects, and 3 required conversion
Open transthoracic plication is the traditional to thoracotomy. Follow-up at 6 months demon-
approach to treat patients who are symptomatic strated a significant improvement in dyspnea
and have diaphragm eventration or paralysis. scores and a significant increase in FVC (17%),
A posterolateral thoracotomy is performed FEV1 (21%), functional residual capacity (FRC)
through the 6th,42,43 7th,13,44,45 or 8th46 (20%), and total lung capacity (TLC) (16%).
Diaphragmatic Eventration 515

Thoracoscopic diaphragm plication is an


excellent minimally invasive alternative to open
transthoracic plication; mid-term follow-up data
suggest that it is as effective as the open
approach. Workspace limitation by the ribcage
and the elevated hemidiaphragm is the main
disadvantage of this approach.

Open Transabdominal Plication


Open transabdominal plication has been described
for unilateral or bilateral diaphragmatic eventration
or paralysis.58 Little outcome data are available on
the results of open transabdominal plication in
Fig. 2. The right hemidiaphragm is taut and displaced
adults. Advantages of an open transabdominal
cranially from the pneumoperitoneum; a small perfo-
approach are access to both sides of the dia- ration is made with electrocautery to induce
phragm and that it does not require selective venti- a pneumothorax.
lation. Additionally, a laparotomy is generally a less
morbid incision than a thoracotomy. Disadvan-
tages include an open approach and difficult Position
access to the most posterior portion of the Patients are in the supine position with abducted
diaphragm. arms. The abdomen and lower lateral chest wall
are prepared and draped to allow access for chest
Laparoscopic Plication tube placement, a foot board is essential for steep
Trendelenburg positioning.
Laparoscopic diaphragm plication has been
described in a single report of three patients by
Hüttl and colleagues.59 All patients improved clin- Operative technique
ically and by PFT parameters. Laparoscopic dia- (1) Ports: The authors place four 12-mm ports;
phragm plication is the preferred approach for two ports are placed along the midline for the
diaphragm eventration or paralysis at the Univer- camera and the assistant, and two working
sity of Minnesota. ports are placed in the ipsilateral upper
Anesthesia quadrant (Fig. 1). The authors insufflate the
The procedure is performed under general abdomen with CO2 at a pressure of 12 to
anesthesia with a single-lumen endotracheal 15 mmHg.
tube; selective ventilation is not necessary. (2) Exposure: Steep reverse Trendelenburg posi-
tioning helps to optimize exposure of the
posterior portion of the hemidiaphragm; for
a right-sided plication, transection of the
falciform ligament is useful for appropriate

Fig. 1. Port placement for laparoscopic right dia-


phragm plication. The two operating ports are placed
in the right upper quadrant approximately 2 cm Fig. 3. The right hemidiaphragm can be easily pulled
above the level of the umbilicus. The two assistant toward the abdominal cavity after inducing a pneu-
ports are placed along the midline above the umbi- mothorax. The diaphragm perforation is visible in
licus; the costal margins have been marked with ink. the top left corner of the image.
516 Groth & Andrade

access to the diaphragm. The thinned-out


hemidiaphragm is taut and displaced cranially
as a result of the pneumoperitoneum. The
authors make a small perforation at the dome
of the diaphragm with electrocautery (Fig. 2).
The resultant pneumothorax allows the
surgeon to easily pull the hemidiaphragm into
the abdominal cavity for suturing (Fig. 3). To
date, all patients have tolerated an ipsilateral
pneumothorax well.
(3) Stitching: The authors use pledgeted U
stitches (#2 nonabsorbable, braided suture,
31 mm curved needle) (Fig. 4). The posterior
portion is plicated first in an anteroposterior
direction followed by a plication line in latero-
medial direction. This results in a T-shaped Fig. 5. The completed T-shaped plication.
plication (Fig. 5). The initial perforation at the
dome is closed with the plication.
(4) Tube thoracostomy: the authors place an 18- to
The chest tube remains in place until output is less
20-Fr chest tube in the ipsilateral chest at the
than 200 mL/day; on occasion patients need to be
end of the procedure.
discharged with the chest tube in place. Prema-
ture removal of the chest tube can lead to
Postoperative management
symptomatic pleural effusion. The immediate
Patients should engage in intense pulmonary toilet
postoperative chest X ray should show that the
to re-expand the lower lobe of the ipsilateral lung.
plicated side is lower than the opposite side, one
may see elevation of the contralateral hemidiaph-
ragm; at 1 month both hemidiaphragms are at
approximately the same level (Fig. 6). The authors
monitor patients with the St. George’s Respiratory
Questionnaire (SGRQ),60 PA/LAT chest X ray, and
PFT at 1 month after discharge, and yearly
thereafter.

Results
To date, the authors have performed laparoscopic
plication on 18 patients with one conversion to
thoracotomy. Two patients required drainage of
a delayed ipsilateral pleural effusion. One- and
12-month follow-up revealed a significant
improvement in SGRQ scores: 50% reduction in
total score at 1 month that persisted at 12 months.
PFT also improved significantly the FIFmax
improved by a mean of 25% at 1 month and
45% at 12 months; FEV1 improved by a mean of
12% and 18% respectively. These changes are
comparable to the published results of open trans-
thoracic plication and thoracoscopic plication.

Complications of Plication
Reported complications include pneumonia,42,54
Fig. 4. (A) A row of pledgeted U stitches is first placed
in posteroanterior direction. It is important to place pleural effusions, abdominal compartment
stitches as far posteriorly as possible to achieve an syndrome,61 conversion to open (for minimally
effective plication. (B) Intracorporeal tying of a stitch. invasive approaches),16 abdominal visceral injury,
Notice the plication line emerging in the posteroante- deep venous thrombosis,12 pulmonary emboli,46
rior direction from behind the liver. and acute myocardial infarction.46
517

Fig. 6. (A) PA chest X ray of a patient with symptomatic right hemidiaphragm eventration. (B) Lateral chest X ray
of a patient with symptomatic right hemidiaphragm eventration. (C) Immediate postoperative anteroposterior
(AP) chest X ray of a patient after laparoscopic right hemidiaphragm plication; the right hemidiaphragm is lower
than the left. (D) PA chest X ray 1 month after laparoscopic right hemidiaphragm plication; both sides of the dia-
phragm are at a similar level. (E) Lateral chest X ray 1 month after laparoscopic right hemidiaphragm plication;
both sides of the diaphragm are at a similar level.
518 Groth & Andrade

Comparison of Surgical Approaches 12. Freeman RK, Wozniak TC, Fitzgerald EB. Functional
for Diaphragm Plication and physiologic results of video-assisted thoraco-
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