chapter

115

EVALUATION AND MANAGEMENT

OF ELEVATED DIAPHRAGM
Clemens Aigner
Walter Klepetko

Key Points
Elevated diaphragm is a rare indication for surgery in adult

patients.
Clinical key symptoms are dyspnea and orthopnea.
Complete diagnostic workup is crucial in treatment planning.
Surgery yields good results in carefully selected patients.

When discussing the evaluation and management of diaphragmatic elevation, it is necessary to distinguish between
congenital conditions of diaphragmatic eventration or diaphragmatic hernias and acquired elevation of the diaphragm.
Congenital diaphragmatic pathologies are discussed in separate chapters; this chapter focuses on acquired conditions in
which patients present with an elevated diaphragm (Table
115-1).
In most adult patients, elevation of the diaphragm is primarily detected on chest radiography.1 Patients are often
asymptomatic or present with only mild symptoms. Further
confirmation and evaluation of the underlying mechanism
may be gained by computed tomography (CT),2 ultrasonography,3 or, in rare cases, magnetic resonance imaging (MRI);
MRI is, however, useful in detecting paradoxical motion
(Iwasawa et al, 2002).4-6 The underlying pathologies for unilateral or bilateral diaphragmatic elevation encompass a wide
spectrum. The cause may be supradiaphragmatic, diaphragmatic, or subdiaphragmatic. Frequently, the exact cause of
an elevated diaphragm is difficult to determine even after
complete surgical exploration.7 It remains a point of discussion in the literature whether an underlying occult malignant
process is a likely cause.8
The main symptoms of an elevated diaphragm are respiratory problems.9 The underlying mechanism is a restrictive
breathing pattern. The severity obviously depends on the
degree of diaphragmatic elevation.10 In addition to restriction, various degrees of compressive atelectasis with decreased
ventilation and perfusion in the affected lung base have been
described.11 Another important factor influencing respiratory
function is the paradoxical movement of a paralyzed diaphragm. There is paradoxical deflation of the lung during
inspiration, caused by elevation of the diaphragm, and during
expiration there is paradoxical inflation of the lung, resulting
in rebreathing of dead air space. The normal diaphragm
creates a negative intrapleural pressure, which is absent in
a diseased elevated diaphragm, resulting in paradoxical
movement.12

ETIOLOGY
Supradiaphragmatic Causes
The most frequently encountered reason for unilateral hemidiaphragm elevation is lung volume loss. Additional signs
accompanying unilateral lung volume loss may be ipsilateral
shifting of the mediastinum and narrowing of the intercostal
spaces. Bilateral lung volume loss may also be responsible for
bilateral diaphragmatic elevation. Underlying causes include
atelectasis (Fig. 115-1), pulmonary fibrosis (Fig. 115-2),
partial lung resection, lobar collapse, and encasement by
tumor formation, as seen in mesothelioma patients (Fig. 1153).13-15 Also, the diaphragm is frequently elevated after pneumonectomy. Obviously, all diseases leading to loss of
pulmonary volume can eventually lead to diaphragmatic elevation. Additionally, osseous alterations such as rib fracture
and scoliosis may provoke elevation of the diaphragm. Such
cases are mainly related to mechanical factors.16 With an
intact phrenic nerve, they are usually associated with normal
diaphragmatic function or only temporary paralysis, with full
recovery expected.

Diaphragmatic Causes
Diaphragmatic elevation is frequently caused by diaphragmatic eventration, in which a portion or the entire hemidiaphragm is elevated with a marked decrease in muscular
fibers,17 yet retains an unbroken continuity with normal
attachments to the costal margins. In such cases, the abnormally thin diaphragm is stretched and displaced by the
abdominal organs. Aside from congenital eventration, which
is caused by incomplete muscularization of the pericardioperitoneal membrane,18 the incidence of eventration,
especially of the right hemidiaphragm, increases with age,
suggesting an acquired process. Eventration frequently does
not affect the entire hemidiaphragm but is incomplete. The
most commonly involved part is the anteromedial right hemidiaphragm.19 In some cases, a congenital eventration that
remained unrecognized during childhood becomes symptomatic with decreasing pulmonary function. Also, blunt trauma
can lead to diaphragmatic elevation with an abnormally thin
and elevated diaphragm, with the macroscopic aspect of
eventration.20,21
A major cause leading to diaphragmatic paralysis, a condition in which the diaphragm still has its normal muscular
basis even if it is atrophic, is phrenic nerve paralysis.
Involvement of the phrenic nerve can be classified in posttraumatic, iatrogenic, neoplastic, related to infectious or neuromuscular disease, or, if no underlying pathology is found,
idiopathic.22

1396

Ch115-F06861.indd 1396

1/21/2008 3:43:59 PM

Chapter 115 Evaluation and Management of Elevated Diaphragm

1397

TABLE 115-1 Frequent Causes of Acquired Elevated Diaphragm

Supradiaphragmatic
Pulmonary resection
Pulmonary fibrosis
Atelectasis
Pleural tumor
Pneumonia
Pulmonary abscess
Pulmonary infarction
Rib fracture
Diaphragmatic
Eventration
Blunt trauma
Phrenic nerve palsy
Traumatic
Iatrogenic (surgery, chest tubes, central venous catheter)
Infectious (poliomyelitis, diphtheria, tuberculosis, herpes zoster,
influenza, syphilis, echinococcus, subphrenic abscess,
pericarditis)
Neoplastic (N2 disease, mediastinal tumors)
Dystrophia myotonica
Lead poisoning
Idiopathic
Infradiaphragmatic
Obesity
Pregnancy
Bowel dilation
Hepatosplenomegaly
Abdominal tumors
Ascites

FIGURE 115-1 Right lower lobe atelectasis with consecutive

elevation of the right hemidiaphragm. (COURTESY OF DR. BHM,
DEPARTMENT OF RADIOLOGY, ST. ELISABETH HOSPITAL, LINZ, AUSTRIA.)

Systemic Disease
Neuromuscular disorders (quadriplegia, multiple sclerosis,
amyotrophic lateral sclerosis, Guillain-Barr syndrome, EatonLambert syndrome, myasthenia gravis, muscular dystrophy,
steroid myopathy, alcohol myopathy, rhabdomyolysis)
Connective tissue disease leading to pulmonary fibrosis
(rheumatoid arthritis, scleroderma, ankylosing spondylitis) or
diaphragmatic weakness (systemic lupus erythematosus,
polymyositis)
Endocrine and metabolic diseases (hypothyroidism,
hyperthyroidism, Cushings syndrome, low potassium or
phosphate or magnesium, metabolic alkalosis)

Injuries to the phrenic nerve are a potential complication

of any type of thoracic or cardiac surgery, as well as neck
surgery. A higher risk seems to be associated with correction
of congenital cardiovascular anomalies (Joho-Arreola et al,
2005).23-26 The use of ice slush to achieve topical hypothermia in adult cardiac surgery also leads to a higher incidence
of temporary postoperative phrenic nerve paralysis (Efthimiou et al, 1991).27,28 The use of ice slush is also a potential
source of phrenic nerve injury in lung transplantation. Diabetes mellitus and grafting of the internal thoracic artery have
been described as risk factors for postoperative phrenic nerve
paralysis.29 Phrenic nerve palsy has also been described
after insertion of chest tubes30-32 and central venous
catheters.33-35
Numerous neuromuscular and infectious diseases, including tuberculosis, diphtheria, poliomyelitis, herpes zoster,
syphilis, influenza, dystrophia myotonica, pericarditis, sub-

Ch115-F06861.indd 1397

FIGURE 115-2 Bilateral diaphragmatic elevation in a patient with

pulmonary fibrosis. (COURTESY OF DR. BHM, DEPARTMENT OF
RADIOLOGY, ST. ELISABETH HOSPITAL, LINZ, AUSTRIA.)

phrenic abscess, echinococcal liver infection, Lyme disease,

and lead poisoning, have been reported to be associated with
phrenic nerve palsy and elevated diaphragm.36-41
Phrenic nerve injury can also be caused by neoplastic
involvement. Mainly malignant mediastinal masses, such as
enlarged N2 lymph nodes in bronchogenic carcinomas, or

1/21/2008 3:43:59 PM

1398

Section 6 Diaphragm

EVALUATION AND DIAGNOSTIC EXAMINATIONS

FIGURE 115-3 Unilateral diaphragmatic elevation caused by

malignant pleural mesothelioma.

primary mediastinal tumors such as thymomas, lymphomas,

or germ cell tumors are responsible.42
In diaphragmatic hernia, the regular continuity of the diaphragm is broken, which can mimic diaphragmatic elevation
in radiologic examinations.
The most common causes of bilateral diaphragmatic elevation are severe obesity and pregnancy. Nonetheless, neuromuscular, connective tissue, and metabolic disorders are
important differential diagnoses and must be ruled out.4 In
connective tissue diseases such as polymyositis and systemic
lupus erythematosus, diaphragmatic weakness causes the
elevation, whereas scleroderma, rheumatoid arthritis, and
ankylosing spondylitis cause elevation as a result of pulmonary fibrosis.43

Infradiaphragmatic Causes
Abdominal disease and pregnancy may also lead to diaphragmatic elevation. Large tumors, fluid collections, subphrenic
cysts or abscesses, organomegaly, and gastrointestinal dilation
are potential causes.

Usually, the diagnosis is established by posteroanterior and

lateral chest radiographs. The diaphragm is unilaterally on
the affected side or clearly elevated bilaterally. Additionally,
an abnormal position of the stomach may be detected. Diaphragmatic elevation on a chest radiograph does not predict
diaphragmatic paralysis, although paralysis is unlikely if no
elevated diaphragm is found (Chetta et al, 2005).48 If the
phrenic nerve is affected, paradoxical motion of the diaphragm is detectable on fluoroscopy. Additionally, diaphragmatic motion can be depicted on MRI to assess paradoxical
movement.5 Phrenic nerve function can be tested by electromyography. Ultrasonography (Gerscovich et al, 2001)49 and
CT scanning2,13,15 are further diagnostic modalities used to
detect underlying pathologies. However, they are often not
able to distinguish between elevated diaphragm with intact
continuity and true herniation. A CT scan and, if necessary,
other diagnostic workup should be performed to rule out
malignancy. MRI primarily has a role in the assessment of
congenital or acquired hernias and in the evaluation of paradoxical diaphragmatic movement.4-6,16
A technique that was initially described 1930 is the diagnostic pneumoperitoneum, which can be used to outline the
continuity of the diaphragm by induction of air, nitrogen
(N2), or carbon dioxide (CO2) into the abdomen, followed
by an upright chest radiograph to differentiate between elevation and hernia.
Some pathologies can mimic an elevated diaphragm on
radiologic examination. Subpulmonal effusion or pleural
tumor50 can potentially arouse the impression of an elevated
diaphragm.
The degree of impact on respiratory function should by
documented by spirometry and, eventually, by exercise
studies.

CONSERVATIVE MANAGEMENT
Unless severe dyspnea, orthopnea, or gastrointestinal problems are clearly related to an elevated diaphragm, eventration
should be treated conservatively in most cases. Optimal management of the underlying disease should be sought to avoid
progression. As mentioned, the degree of impact on respiratory function should be documented by spirometry, and
control spirometries can detect a beginning decline in
pulmonary function. Additionally, exercise studies can be
performed (Celli, 2002).51

CLINICAL PRESENTATION

SURGERY

The leading symptoms in adult patients with acquired diaphragmatic elevation are respiratory problems.44 Because the
diaphragm is the main respiratory muscle, most patients complain about dyspnea or orthopnea and, less commonly, about
cough and retrosternal or epigastric pain. In case of underlying lung disease or reduced pulmonary reserve, this may
lead as far as complete respiratory failure (Simansky et al,
2002).45,46 Other possible symptoms include a variety of
digestive tract symptoms such as nausea, vomiting, gas bloat,
belching, or abnormally increased bowel noises.47

Surgery for elevated diaphragm is indicated in relatively few

cases. A careful evaluation of symptoms potentially related
to the elevated diaphragm should be performed.
The surgical technique of plication of the diaphragm was
described in the early 20th century by Wood,58 and later by
Morrison.59 The goal of the operation is to immobilize the
diaphragm in a lower, relatively flat position, to reduce compression of the lung and mediastinum and eventually reduce
paradoxical movement. Functional recovery is potentially
possible if there is an adequate muscular reserve.

Ch115-F06861.indd 1398

1/21/2008 3:43:59 PM

Chapter 115 Evaluation and Management of Elevated Diaphragm

1399

TABLE 115-2 Results of Operative Plication for Acquired Elevated Diaphragm

Improvement (%)
Author (Year)

No. Patients

Mouroux53 (2005)

12

(2003)

Higgs61 (2001)

60

Hines

62

Ribet

(1992)
63

Graham

(1990)

Wright64 (1985)
7

Donzeau-Gouge (1982)
65

Pastor

(1982)

NcNamara1 (1968)

Mortality (%)

Clinical

Radiographic

Functional

110

100

100

100

100

Not stated

15

93.3

Not stated

100

11

90.9

Not stated

Not stated

17

100

100

100

100

100

100

11.1

Not stated

Not stated

15

86.6

100

Not stated

13

92.3

Not stated

Not stated

Initially, plication was performed through a posterolateral

approach, whereas now it can also be performed through a
less invasive anterolateral approach (Lai et al, 1999)52 or by
a video-assisted technique.53,54 An abdominal approach, which
may also be laparoscopic, is recommended if there is infradiaphragmatic involvement or a gastric volvulus requiring
repositioning.55
The repair may be performed without incising the diaphragm by a simple plication with various suture materials
or even endostaplers,56 or by excising part of the elevated
diaphragm.
Surgical reanastomosis of the phrenic nerve after transection has been reported in patients as late as 4 months after
the lesion. Sural or intercostal nerve can be used as autologous grafts to bridge a gap between intact portions of the
phrenic nerve. A success rate of 75% can be expected;
however, full recovery usually takes months because of slow
nerve growth.57
Various clinical studies have reported on series of patients
undergoing diaphragmatic plication. Most of the studies
required patients to have dyspnea interfering with everyday
life, orthopnea, and respiratory function tests demonstrating
impairment. A complete thoracic and abdominal diagnostic
workup was recommended to rule out other correctable
causes. An overview is given in Table 115-2. All studies concluded that plication is a safe and effective procedure.
However, surgical correction of bilateral eventration seems
to be associated with a higher operative mortality rate due to
associated malformations and hypoplasia of the lung. More
recent papers describing endoscopic techniques stress the
reduction of operative trauma and shorter recovery period as
advantages in choosing this approach.

SUMMARY
Indication for surgery in acquired elevated diaphragm in
adults should be determined very cautiously, after thorough
evaluation of the patient and after exclusion of other potential causes for respiratory problems. In many cases, conservative measures such as weight loss, respiratory hygiene, optimal

Ch115-F06861.indd 1399

87.5

treatment of the underlying disease, and physiotherapy are

sufficient to improve symptoms. For patients presenting with
only gastrointestinal symptoms, the indication should be
made even more restrictively. Yet, growing evidence exists
that carefully selected patients show substantial benefit in
lung function and respiratory symptoms after plication of the
diaphragm. Less invasive techniques, such as video-assisted
thoracic surgery (VATS) or minithoracotomy, help in minimizing the operative trauma.

COMMENTS AND CONTROVERSIES

The elevated diaphragm is a common finding in the thoracic surgery
clinic. The variable position of the normal diaphragm and the effects
of age and body habitus on its position must be remembered to
prevent misdiagnosis. A thorough history, including prior surgery,
trauma, malignancy, and so on, is essential in the determining the
cause of an elevated diaphragm. The authors useful classification
of etiologies into supradiaphragmatic, diaphragmatic, and subdiaphragmatic brings to attention and reminds the clinician that the
problem may not exist in the diaphragm or phrenic nerve. The presence of a mass or fluid collection may change the contour of the
diaphragm and be misinterpreted as an elevated diaphragm. Simple
investigations such as posteroanterior and lateral chest radiographs,
decubitus radiography, and fluoroscopy are very helpful and should
not be overlooked in the evaluation. Multidetector CT and multiplanar MRI are essential in problematic cases of elevated diaphragm.
T. W. R.

KEY REFERENCES
Celli BR: Respiratory management of diaphragm paralysis. Semin Respir
Crit Care Med 23:275-282, 2002.
Chetta A, Rehman AK, Moxham J, et al: Chest radiography cannot
predict diaphragm function. Respir Med 99:39-44, 2005.
Efthimiou J, Butler J, Woodham C, et al: Diaphragm paralysis following
cardiac surgery: Role of phrenic nerve cold injury. Ann Thorac Surg
52:1005-1008, 1991.
Gerscovich EO, Cronan M, McGahan JP, et al: Ultrasonographic
evaluation of diaphragmatic motion. J Ultrasound Med 20:597-604,
2001.

1/21/2008 3:44:00 PM

1400

Section 6 Diaphragm

Iwasawa T, Kagei S, Gotoh T, et al: Magnetic resonance analysis of

abnormal diaphragmatic motion in patients with emphysema. Eur
Respir J 19:225-231, 2002.
Joho-Arreola AL, Bauersfeld U, Stauffer UG, et al: Incidence and
treatment of diaphragmatic paralysis after cardiac surgery in children.
Eur J Cardiothorac Surg 27:53-57, 2005.

Ch115-F06861.indd 1400

Lai DT, Paterson HS: Mini-thoracotomy for diaphragmatic plication

with thoracoscopic assistance. Ann Thorac Surg 68:2364-2365,
1999.
Simansky DA, Paley M, Refaely Y, Yellin A: Diaphragm plication following phrenic nerve injury: A comparison of paediatric and adult
patients. Thorax 57:613-616, 2002.

1/21/2008 3:44:00 PM