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© 2006 WebMD, Inc. All rights reserved.                                                       ACS Surgery: Principles and Practice
4 THORAX                                                                             15 DIAPHRAGMATIC PROCEDURES — 1


15            DIAPHRAGMATIC PROCEDURES
Ayesha S. Bryant, M.S.P.H., and Robert James Cerfolio, M.D., F.A.C.S., F.C.C.P.




Although the diaphragm is sometimes thought of as little more          drainage occurs via the inferior vena cava and the azygos vein on
than a partition between the thoracic organs and the abdominal         the right and via the suprarenal and renal veins and the hemiazy-
organs, it is in fact a dynamic anatomic structure that plays a piv-   gos vein on the left.
otal role in the physiology of respiratory mechanics. For example,
                                                                       INNERVATION
paralysis of just one hemidiaphragm can lead to the loss of 50% of
a patient’s vital capacity.1 Like any other anatomic structure, the       The diaphragm receives its muscular neurologic impulse from
diaphragm may be affected by either benign or malignant condi-         the phrenic nerve, which arises primarily from the fourth cervical
tions. Overall, benign diseases of the diaphragm (e.g., paralysis)     ramus but also has contributions from the third and fifth rami.
are far more common than malignant ones. With either type of           The phrenic nerve originates around the level of the scalenus ante-
condition, however, the development of a safe surgical treatment       rior and runs inferiorly through the neck and thorax before reach-
strategy depends on a solid knowledge of diaphragmatic anatomy         ing its terminal point, the diaphragm. Because the phrenic nerve
and physiology. Accordingly, we begin with a brief review of the       follows such a long course before reaching its final destination, a
embryology and anatomy of the diaphragm.We then describe the           number of processes can disrupt the transmission of neurologic
main procedures performed to treat the more common congeni-            impulses through the nerve at various points and thereby cause
tal diseases (e.g., congenital diaphragmatic hernia [CDH]) and         diaphragmatic paralysis [see 4:6 Paralyzed Diaphragm].
acquired pathologic conditions (e.g., paralysis and tumor) that
affect this structure.
                                                                       Procedures for Congenital Diaphragmatic Hernia

Anatomic Considerations                                                REPAIR OF BOCHDALEK HERNIA

                                                                          Bochdalek hernia, named after the Czech anatomist Vincent
DEVELOPMENTAL ANATOMY
                                                                       Alexander Bochdalek, is the most common form of CDH and is
   The diaphragm is a modified half-dome of musculofibrous tis-          also the most common surgical emergency in neonates.1 The usual
sue that lies between the chest and the abdomen and serves to sep-     presenting symptoms are severe respiratory distress and a scaphoid
arate these two compartments. It is formed from four embryolog-
ic components: (1) the septum transversum, (2) two pleuroperi-
                                                                                                Sternal Portion      Esophagus
toneal folds, (3) cervical myotomes, and (4) the dorsal mesentery.
                                                                                  Inferior
Development of the diaphragm begins during week 3 of gestation                    Vena Cava
and is complete by week 8. Failure of the pleuroperitoneal folds to
                                                                        Costal                                                     Rib
develop, with subsequent muscle migration, results in congenital        Portion
defects.
CLASSICAL ANATOMY

   The diaphragmatic musculature originates from the lower six
ribs on each side, from the posterior xiphoid process, and from the
external and internal arcuate ligaments. A number of different
structures traverse the diaphragm, including three distinct aper-
tures (foramina) that allow the passage of the vena cava, the esoph-
agus, and the aorta [see Figure 1].The aortic aperture is the lowest
and most posterior of the diaphragmatic foramina, lying at the
level of the 12th thoracic vertebra. Besides the aorta, the thoracic
duct and, sometimes, the azygos and hemiazygos veins also pass
through this aperture.The esophageal aperture is the middle fora-
men; it is surrounded by diaphragmatic muscle and lies at the level
of the 10th thoracic vertebra.The vena caval aperture is the high-
est of the three foramina, lying level with the disk space between
T8 and T9.                                                              Lumbar
                                                                        Portion                                                  Tendon
VASCULAR SUPPLY

   The diaphragm is supplied by the right and left phrenic arter-
ies, the intercostal arteries, and the musculophrenic branches of
the internal thoracic arteries. Some blood is supplied by small
                                                                                                 Spine               Aorta
branches of the pericardiophrenic arteries that run with the phrenic
nerve, mainly where the nerves penetrate the diaphragm. Venous         Figure 1    Shown is an inferior view of the diaphragm.
© 2006 WebMD, Inc. All rights reserved.                                                       ACS Surgery: Principles and Practice
4 THORAX                                                                             15 DIAPHRAGMATIC PROCEDURES — 2

                                                                           Preoperative Evaluation
abdomen. The primary pathologic condition is the presence of
posterolateral defects of the diaphragm, which result either in            On chest radiography, a Morgagni hernia appears as a mass at
maldevelopment of the pleuroperitoneal folds or in improper or           the right cardiophrenic angle [see Figure 2]. Computed tomogra-
absent migration of the diaphragmatic musculature.                       phy of the chest and abdomen, liver scintigraphy, and multiplanar
   Bochdalek hernias occur in approximately one of every 2,500           magnetic resonance imaging are occasionally helpful in the diag-
live births and are twice as common in male neonates as in female        nostic process.
neonates. Mortality ranges from 45% to 50%. The bulk of the
                                                                           Operative Technique
morbidity and mortality of CDH is attributable to the resultant
hypoplasia of the lung on the affected side and to various associ-          Morgagni hernias can be repaired via a subcostal, a paramedi-
ated abnormalities (e.g., malrotation of the gut, neural tube defects,   an, or a midline incision. We prefer to use an upper midline
and cardiovascular anomalies).                                           abdominal incision. Once the peritoneal cavity is entered, the her-
                                                                         nia sac is identified just posterior to the xiphoid and the posterior
  Preoperative Evaluation                                                sternal border, then opened. The herniated abdominal viscera are
   Prenatal ultrasound examination accurately diagnoses CDH in           restored to their normal abdominal anatomic positions, and the
40% to 90% of cases.2 In most instances, the examination is per-         sac is ligated.The entire hernia sac is defined, resected, and closed.
formed to rule out polyhydramnios. It is noteworthy that polyhy-         The diaphragmatic defect may be repaired in several different
dramnios is present in as many as 80% of pregnant women whose            ways, depending on its size and position. Because there is weak tis-
fetuses have CDH.3 In neonates with CDH, besides the upper               sue in the area of the defect, we generally use a prosthetic patch for
gastrointestinal tract, parts of the colon, the spleen, the kidneys,     the repair. Either polypropylene mesh (e.g., Marlex; C. R. Bard,
and the pancreas may herniate, and the abnormal position of              Inc., Murray Hill, New Jersey) or polytetrafluoroethylene (PTFE)
these organs can be identified by means of ultrasonography. Mal-          mesh (e.g., Gore-Tex; W. L. Gore and Associates, Newark, Del-
rotation and malfixation of the small bowel should be ruled out.          aware) may be used for this purpose. We prefer PTFE because it
Once the diagnosis is confirmed, additional radiographic, echo-           may cause fewer adhesions to the underlying abdominal structure,
cardiographic, and ultrasonographic studies should be performed          which may be an important consideration if further abdominal
to rule out associated anomalies.                                        surgery subsequently proves necessary. The prosthetic patch is
                                                                         sewn to the midline abdominal fascia, with wide bites taken to pre-
  Operative Technique                                                    vent an abdominal incisional hernia.The rest of the patch is sewn
                                                                         to the thickened investing fascia that made up the edges of the her-
   As a rule, neonates with Bochdalek hernias are taken to the oper-
                                                                         nia sac. As noted, the frequently marginal quality of this tissue is
ating room immediately after birth. Some studies, however, have
                                                                         the reason why a patch repair is almost always required.
shown that delayed surgical repair yields improved survival rates.4
                                                                            Repair of a Morgagni hernia via a thoracic incision follows the
   For left-side hernias, a transabdominal subcostal approach is
                                                                         same basic principles.The hernia sac is entered, the visceral con-
generally preferred, whereas for right-side hernias, a transthoracic
                                                                         tents are mobilized and reduced into the abdomen, the sac is
approach may be more useful.The herniated organs are returned
                                                                         resected, and the diaphragm is repaired. Again, the closure should
to the peritoneal cavity. The lung is inspected, but no attempt to
                                                                         be completed without tension. If the defect cannot be closed with
expand the hypoplastic lung should be made. If any extralobar
                                                                         horizontal mattress sutures, a prosthetic patch should be used.
pulmonary sequestration is present (as is occasionally the case), it
should be excised. Most of the defects may be closed primarily             Complications
with interrupted nonabsorbable sutures; particularly large defects          The potential complications of surgical treatment of Morgagni
may be closed with a prosthetic patch. The left pleural space is         hernia depend to an extent on the type of procedure undertaken
drained with a chest tube, which should be placed on water seal.         to repair the defect. Laparoscopy may result in failure to reduce
                                                                         the contents of the hernia sac, which necessitates conversion to an
   Alternative technique Some surgeons have attempted sur-               open procedure. Laparotomy has been associated with postoper-
gical correction of severe Bochdalek hernias in the prenatal period.     ative pleural effusion,9 wound infection,10 deep vein thrombosis,11
The safety and feasibility of this therapeutic approach continue to      and pulmonary embolism.12 Thoracotomy has been associated
be debated. Prenatal correction of these hernias poses a risk to both    with pneumonia, sepsis, and bowel obstruction in the postopera-
the mother and the fetus, with possibly fatal results for both.5         tive period.13
REPAIR OF MORGAGNI HERNIA                                                  Outcome Evaluation
   Morgagni hernias, named after the Italian anatomist and pathol-          Most patients do not have any significant postoperative limita-
ogist Giovanni Battista Morgagni, are related to maldevelopment          tions after repair of a Morgagni hernia, nor are such hernias like-
of the embryologic septum transversum and to failed fusion of            ly to recur. In one study, 16 patients who underwent transthoracic
the sternal and costal fibrotendinous elements of the diaphragm.6         repair of a Morgagni hernia were followed for 5.7 years; no recur-
These hernias are generally asymptomatic7 and are usually detect-        rences or symptoms related to the operation were reported.14
ed as incidental findings on radiographs. Accordingly, the average
age at diagnosis is typically greater for Morgagni hernia than for
Bochdalek hernia: in one report, the mean age at which the for-          Procedures for Diaphragmatic Paralysis
mer was diagnosed was 45 years.8 Morgagni hernias are most                  The diaphragm is the most important of the respiratory mus-
commonly seen on the right side.The hernia sac usually contains          cles: diaphragmatic contraction decreases intrapleural pressure dur-
omentum, but it may also contain part of the transverse colon            ing inspiration, expands the rib cage, and thereby facilitates the move-
or, less commonly, parts of the stomach, the liver, or the small         ment of gases into the lungs. Accordingly, paralysis of the dia-
bowel; almost any upper abdominal structure may herniate in this         phragm can have a major adverse effect on respiratory function
setting.                                                                 [see 4:6 Paralyzed Diaphragm]. Diaphragmatic paralysis may involve
© 2006 WebMD, Inc. All rights reserved.                                                  ACS Surgery: Principles and Practice
4 THORAX                                                                         15 DIAPHRAGMATIC PROCEDURES — 3

                         a




                  b




                  Figure 2 Repair of Morgagni hernia. The differential diagnosis of a cardiophrenic-angle mass
                  includes pericardial fat, a lipoma, a pericardial cyst, a Morgagni hernia, and a thymoma. Shown
                  are (a) chest x-rays and (b) chest CT scans from a 33-year-old man with an incidental finding of
                  a Morgagni hernia.



either the whole diaphragm (bilateral paralysis) or only one leaflet      In most cases, the diagnosis of hemidiaphragmatic paralysis is
or hemidiaphragm (unilateral paralysis). The possible causes of       suspected on the basis of incidental findings on a chest x-ray.
diaphragmatic paralysis are numerous [see Table 1]; the most com-     Typically, the roentgenogram reveals an elevated hemidiaphragm,
mon causes are phrenic nerve trauma related to a surgical proce-      diminished lung volume, and basilar atelectasis. Fluoroscopy may
dure (e.g., stretching, crushing, or transection) and invasion by a   also be performed. The diagnosis is confirmed by performing a
malignant neoplasm.                                                   fluoroscopic sniff test, in which paradoxical elevation of the para-
                                                                      lyzed diaphragm is observed with sniffing.15 The sniff test is the
DIAPHRAGMATIC PLICATION FOR UNILATERAL PARALYSIS
                                                                      gold standard for the diagnosis of this condition. In certain pa-
                                                                      tients, a chest CT scan may be indicated for evaluating the poten-
  Preoperative Evaluation                                             tial cause of the paralysis. If an obvious cause is not apparent from
   With unilateral diaphragmatic paralysis, the paralyzed             the history or a previous evaluation, CT scanning of the chest
hemidiaphragm paradoxically moves upward on inspiration and           should be performed to ensure there is no pathologic process that
downward on expiration, passively following changes in                is compressing or invading the phrenic nerve. Similarly, MRI of
intrapleural and intra-abdominal pressure. Patients with a par-       the neck or the spine may be indicated in certain patients to look
alyzed hemidiaphragm who are otherwise healthy usually have           for conditions that might be causing the diaphragmatic paralysis.
no symptoms at rest but experience dyspnea during exertion               Two other tests that are also (albeit less commonly) used for the
and show a decrease in exercise performance. Physical exami-          diagnosis of unilateral diaphragmatic paralysis are electromyogra-
nation may reveal dullness to percussion and an absence of            phy and transdiaphragmatic pressure assessment. In the first, the
breath sounds over the lower chest on the involved side.              phrenic nerve is electrically stimulated in the neck in an effort to
© 2006 WebMD, Inc. All rights reserved.                                                       ACS Surgery: Principles and Practice
4 THORAX                                                                             15 DIAPHRAGMATIC PROCEDURES — 4

                                                                            Operative Technique
                 Table 1 Common Causes of
                   Diaphragmatic Paralysis                                   Diaphragmatic plication may be performed with either sutures
                                                                          or staples; we prefer sutures for this procedure. The chest is
                         Spinal cord transection                          entered through a thoracotomy in the seventh or eighth intercostal
                         Multiple sclerosis                               space. Horizontal mattress sutures buttressed with Teflon pledgets
                         Amyotrophic lateral sclerosis                    are then placed in a lateral-to-medial direction [see Figure 3]. We
                         Cervical spondylosis                             typically use monofilament nonabsorbable sutures that pass easily
                         Poliomyelitis                                    through the muscle and can be tightened without dragging through
                         Guillain-Barré syndrome
                                                                          tissue. To distribute the tension, multiple sutures must be placed;
                         Phrenic nerve dysfunction
 Neurologic conditions
                                                                          this is especially important on the right side, where the diaphragm
                           Compression by tumor
                           Cardiac surgery cold injury
                                                                          must be pulled down against the upward force exerted by the pres-
                           Blunt trauma                                   ence of the right hemiliver.When the sutures are tied, the hemidi-
                           Idiopathic phrenic neuropathy                  aphragm should be almost back to its normal anatomic location.
                           Diabetes mellitus                              Care must be taken to ensure that the repair is not under undue
                           Postviral phrenic neuropathy (herpes zoster)   tension: excessive tension is likely to result in early dehiscence.
                           Radiation therapy                              Occasionally, a prosthetic patch may be used to buttress the repair
                           Cervical chiropractic manipulation             further, but in the majority of cases, this measure should be unnec-
                         Limb-girdle dystrophy                            essary. If the choice is made to use staples rather than sutures, care
                         Hyperthyroidism/hypothyroidism                   must be taken to ensure that the underlying abdominal contents
                         Malnutrition                                     are not caught in the staple line.
                         Acid maltase deficiency
                         Connective tissue disease                        DIAPHRAGMATIC PACING FOR BILATERAL PARALYSIS
 Myopathic conditions       Systemic lupus erythematosus
                            Dermatomyositis                                 Preoperative Evaluation
                            Mixed connective tissue disease
                         Amyloidosis
                                                                             In patients with bilateral diaphragmatic paralysis, the respirato-
                         Idiopathic myopathy                              ry accessory muscles assume all the work of breathing by con-
                         Muscular dystrophy                               tracting more intensely. Both hemidiaphragms move upward on
                         Multiple sclerosis                               inspiration, concomitant with inward (rather than the normal out-
                                                                          ward) movement of the abdominal wall.17 Patients typically pre-
                                                                          sent with severe respiratory failure or with dyspnea (sometimes
                                                                          misinterpreted as a sign of heart failure) that worsens in the supine
distinguish between neuropathic and myopathic causes of paraly-
                                                                          position, and they generally exhibit tachypnea and rapid, shallow
sis. In the second, transdiaphragmatic pressures are measured by
placing a thin-walled balloon transnasally at the lower end of the
esophagus in such a way as to reflect changes in pleural pressure;
a second balloon manometer is then placed in the stomach in such
a way as to reflect changes in intra-abdominal pressure. The dif-
ference between the two pressures is the transdiaphragmatic pres-
sure. Measurement of transdiaphragmatic pressure can help dif-
ferentiate diaphragmatic paralysis from other causes of respiratory
failure.
   Yet another test involves measurement of maximal inspiratory
pressures. Patients with diaphragmatic dysfunction and paralysis
show a decrease in their maximal inspiratory pressures. These
patients cannot generate high negative inspiratory pressures, and
thus, their maximal inspiratory pressures will be less negative than
–60 cm H2O.
  Operative Planning
   Surgical treatment of hemidiaphragmatic paralysis is reserved
for symptomatic patients who, after a follow-up period of at least
6 months, have persistent shortness of breath with exertion that is
sufficiently pronounced to interfere with lifestyle. For a patient to
be considered for operation, the sniff test should show significant
paradoxical motion.
   The basic principle of the surgical procedure is to “reef” (i.e.,
reduce the surface area of) the redundant floppy diaphragm by
plicating it. This measure lowers the resting position of the
hemidiaphragm and thus affords the lung the opportunity to
expand fully. The effective result is an increase in the functional
vital capacity of the ipsilateral lung. A 2002 study found that pli-      Figure 3 Diaphragmatic plication. Shown is suture plication of
cation of the diaphragm led to long-term improvements in pul-             the right hemidiaphragm. Placement of sutures buttressed with
monary function test results, as well as reduced dyspnea.16               pledgets extends anteriorly to the level of the vena cava.
© 2006 WebMD, Inc. All rights reserved.                                                     ACS Surgery: Principles and Practice
4 THORAX                                                                           15 DIAPHRAGMATIC PROCEDURES — 5


                        External      Internal                          anterior horn cells in the nerve roots, diaphragmatic pacing is fea-
                                                    Receiver and        sible only when the lesion is above the C2-C3 level. Accordingly,
                                                    Electrode
            Antenna                                                     it is sometimes employed in patients with high spinal cord injuries.
                                                              Phrenic       To date, only one pacing device has been approved by the Food
                                                              Nerve     and Drug Administration: the Mark IV Breathing Pacemaker
                                                                        System (Avery Biomedical Devices, Commack, New York). This
                                                                        device possesses an internal component and an external compo-
                                                                        nent [see Figure 4]. The internal component is surgically placed
                                                                        adjacent to the skeletonized phrenic nerve and is connected to a
                                                                        small, wafer-shaped receiving unit placed under the skin. A bat-
                                                                        tery-powered external transmitting box connected to an antenna
                                                                        is taped over the surface of the skin, just above the subcutaneous
                                                                        receiver. This transmitting box permits adjustment of pulse dura-
                                                                        tion, pulse train duration, respiratory rate, pulse frequency, and
                                                                        current amplitude. In most patients, the only parameters that the
                                                                        clinician adjusts are current amplitude and respiratory rate.
                                                                            Implantation of a diaphragmatic pacer requires significant ex-
                                                                        perience on the part of the surgeon—not so much because of any
                                                                        particular technical demands imposed by the implantation itself
                                                                        but because of the procedures for diaphragm training that must be
                                                         Diaphragm      carried out in the postoperative period.
  Mark IV

                                                                           Choice of surgical approach Surgical implantation of a
                                                                        diaphragmatic pacer can be done via either a cervical approach or
                                                                        a thoracic approach. The primary advantage of the cervical ap-
                                                                        proach is that it avoids the morbidity associated with bilateral tho-
                                                                        racotomies, which may not be well tolerated in patients who have
                                                                        marginal pulmonary function or a history of severe pulmonary con-
                                                                        tusions. However, there are other ways of avoiding this morbidity,
                                                                        such as using video-assisted thoracoscopic surgery (VATS) or per-
                                                                        forming small muscle-sparing, rib-sparing, nerve-sparing thoracot-
Figure 4 Diaphragmatic pacing. Shown are internal and external          omies.19 One disadvantage of the cervical approach is that in a small
pacer connections.                                                      percentage of patients, the current amplitude necessary to stimu-
                                                                        late the phrenic nerve results in transmission of the current through
                                                                        the soft tissues.The transmitted current stimulates the functioning
breathing when in the recumbent position. Increased expenditure         portions of the brachial plexus, causing rhythmic jerking motions
of effort in the struggle to breathe may fatigue the accessory mus-     of the upper extremities. Another disadvantage is that there are a
cles and lead to ventilatory failure. Patients also report anxiety,     number of accessory nerve branches arising distal to the neck.
insomnia, morning headache, excessive daytime somnolence,                  One advantage of the thoracic approach is that inadvertent stim-
confusion, fatigue, poor sleep habits, and signs of cor pulmonale.18    ulation of portions of the brachial plexus (as sometimes occurs
   During physical examination, auscultation of the chest reveals       with the cervical approach) may be avoided. Another advantage is
limitation of diaphragmatic excursion and bilateral lower-chest         that there is some neuroanatomic evidence that a small branch of
dullness with absent breath sounds. The finding that establishes         the phrenic nerve joins the main nerve trunk only after it enters
the diagnosis is a paradoxical inward movement of the abdomen           the chest cavity; thus, the thoracic approach may stimulate a larg-
with inspiration. As with unilateral diaphragmatic paralysis, how-      er portion of the phrenic nerve than the cervical approach would.
ever, it is more common for the diagnosis to be suspected on the        The disadvantage of the thoracic approach is the preconceived
basis of a chest roentgenogram that shows bilateral diaphragmat-        notion that entry into the chest is associated with a higher mor-
ic elevation, then confirmed by means of the sniff test.                 bidity than entry into the neck.
  Operative Planning                                                      Operative Technique
   Treatment depends on the cause and severity of the diaphrag-            Cervical placement In the neck, the phrenic nerve runs
matic paralysis. Most patients are treated with ventilatory support,    between the scalenus anterior and the scalenus medius. A trans-
but some are treated with bilateral plication or with pacing. Plica-    verse skin incision is made in the midportion of the neck, just lat-
tion for bilateral paralysis is performed in the same way as plica-     eral to the sternocleidomastoid muscle, and the borders of the two
tion for unilateral paralysis [see Diaphragmatic Plication for          scalene muscles are dissected.The scalene muscles are then divid-
Unilateral Paralysis, above], except that both hemidiaphragms are       ed, and the phrenic nerve is identified lying in a layer of fascia just
lowered. Diaphragmatic pacing is not useful in the treatment of         anterior to the anterior surface of the scalenus medius.
unilateral diaphragmatic paralysis, because of the difficulty of syn-    Identification of this nerve is often facilitated by the use of a hand-
chronizing the contractions of the normal hemidiaphragm with            held nerve stimulator. Intraoperative fluoroscopy allows observa-
those of the paralyzed hemidiaphragm. However, it is sometimes          tion of diaphragmatic contraction in response to phrenic nerve
an appropriate choice for the treatment of bilateral paralysis, espe-   stimulation, which confirms that pacing is successful.
cially in patients who have a central condition that is causing their      Once the phrenic nerve is identified, it is carefully dissected free
apnea. Because the phrenic nerve stimulates the C3, C4, and C5          of its investing fascia, and the Y-shaped electrode is placed under
© 2006 WebMD, Inc. All rights reserved.                                                         ACS Surgery: Principles and Practice
4 THORAX                                                                                 15 DIAPHRAGMATIC PROCEDURES — 6


                  a                                                                  b




                                         Right Lower-Lobe
                                         Tumor Invading                                                               Gore-Tex Patch
                                         Diaphragm




                       Figure 5 Resection of diaphragmatic tumor. The patient has a right lower-lobe bronchogenic
                       malignancy with erosion into the right hemidiaphragm. (a) The tumor is resected en bloc with the
                       diaphragmatic fibers; the electrocautery is used to achieve clear surgical margins and hemostasis.
                       (b) The defect in the right hemidiaphragm is closed with a mesh patch.



it and secured with sutures. Care must be taken to ensure that the          there are several specific complications associated with diaphragmat-
nerve is not injured during this step.The connecting wire from the          ic pacing.The most common of these are dislodgment of the pacer
electrode is then tunneled subcutaneously to a subcutaneous pock-           electrode, transmission of pacer impulses to the brachial plexus with
et that is created just below the ipsilateral clavicle.The connections      resultant rhythmic jerking of the upper extremity (seen with cervical
are made and sealed, and the small incisions are closed.                    placement of the electrode), and hardware malfunction.

   Thoracic placement In the thoracic approach, the chest is                  Outcome Evaluation
entered through a high thoracotomy (usually over the fourth inter-             Retrospective analysis of the collective experience at a single cen-
space), and the proximal phrenic nerve is identified. On the right           ter between 1981 and 1987 suggested that long-term pacing did not
side, the nerve lies along the mediastinum, situated just anterior to       lead to progressive diaphragmatic dysfunction.23 Six of the 12 patients
the vena cava and coursing along the pericardial surface. On the            in this cohort continued to undergo diaphragmatic pacing on a full-
left side, it lies on the pericardium for most of its length.The prox-      time basis for a median period of more than 14 years. Pacing was
imal nerve is freed of its fibrous investments, and the electrode is         well tolerated in this group; the reasons for discontinuance included
placed under it and secured with sutures. The electrode is con-             intercurrent medical illness and lack of social support. Concerns
nected to the receiver, which is placed in a subcutaneous pocket.           have been raised that prolonged diaphragmatic pacing might dam-
   As noted (see above), there are alternative approaches to pacer im-      age the phrenic nerve. In the series cited, however, the ability to pace
plantation that avoid the cervical approach but also do not involve stan-   the phrenic nerve was not lost in any of the patients, and the mean
dard thoracotomies. For example, there is limited (but growing) ex-         threshold currents for pacing did not change significantly over time.
perience with thoracoscopic placement of phrenic nerve pacing leads.20
In addition, laparoscopic implantation of intramuscular pacing elec-
trodes onto the inferior aspect of the diaphragm has been reported.21       Resection of Diaphragmatic Tumors
   Taking into account all the advantages and disadvantages of each            Primary tumors of the diaphragm are extremely rare. Benign
approach to pacer implantation, we generally prefer the thoracic            tumors (e.g., lipomas and cystic masses) are more common than
approach, either via VATS or via a thoracotomy.20,22 The evolution          malignant tumors, which mostly are sarcomas of fibrous or muscu-
of less invasive surgical techniques may allow the thoracic approach        lar origin.Thoracic and abdominal tumors (e.g., bronchogenic car-
to be employed in patients who are unable to tolerate thoracotomy.          cinomas, pleural malignancies, and chest wall malignancies [see 4:3
                                                                            Chest Wall Mass]) may involve the diaphragm secondarily through
  Complications
                                                                            direct extension. Malignant pleural mesothelioma represents a dif-
  Besides the usual complications associated with any thoracic pro-         ferent scenario and is not discussed here. Schwannomas, chondro-
cedure (i.e., infection, bleeding, atrial fibrillation, and pneumonia),      mas, pheochromocytomas, and endometriomas have all been re-
© 2006 WebMD, Inc. All rights reserved.                                                                         ACS Surgery: Principles and Practice
4 THORAX                                                                                              15 DIAPHRAGMATIC PROCEDURES — 7


ported. Bilateral occurrence, calcification, sharp margins, and flat-                    the edges of the defect; this prevents the edges from retracting,
tened contours are indicative of a malignant process, such as pleur-                   helps keep the defect as small as possible, and keeps abdominal
al metastases, mesothelioma, or a primary diaphragmatic tumor.                         contents from interfering with the resection. In addition, we place
   The most common indication for diaphragmatic resection is                           clips on the edges for guidance purposes, in case adjuvant radio-
mesothelioma. This remains true even though mesothelioma is                            therapy is delivered after the operation. If adequate margins are
relatively uncommon in comparison with bronchogenic malig-                             obtained, which is usually relatively easy in a diaphragmatic resec-
nancy and even though few patients with mesothelioma are actu-                         tion, postoperative radiotherapy should be unnecessary. If, how-
ally candidates for resection. Again, resection of a mesothelioma is                   ever, the tumor abuts vital structures (e.g., the suprahepatic vena
not addressed here. The ensuing operative description focuses on                       cava), postoperative radiotherapy may have a useful role to play.
diaphragmatic resection to treat either a lung cancer invading the                         Once the entire tumor has been resected and clear margins
diaphragm or a primary diaphragmatic tumor [see Figure 5].                             have been confirmed by frozen-section examination, the diaphragm
                                                                                       is reconstructed. Primary repair is rarely indicated, because in most
OPERATIVE TECHNIQUE
                                                                                       cases, the defect is too large and the tension on the repair would
   Once the decision is made to resect a tumor involving the                           be too great. Moreover, the tissue in the anterior aspect of the
diaphragm, the key considerations are (1) the surgical approach to                     diaphragm is thin and is likely to tear under tension. Accordingly,
be taken and (2) the placement of the incision in the diaphragm.                       repair with a prosthetic patch is the usual choice. Infection of such
We prefer a skin incision that is lower and slightly more anterior                     a patch is exceedingly rare, and with the exception of the cost,
than a normal posterolateral thoracotomy; such an incision allows                      there is little downside to the use of prosthetic material in this set-
easy entry over the top of the seventh rib. After entry into the chest,                ting. As in the repair of a CDH, we prefer PTFE mesh [see Figure
the lung, the pericardium, and the pleural surface are carefully vis-                  5b] to polypropylene mesh because it is less likely to adhere to
ualized and palpated to search for any signs of metastatic disease.                    underlying abdominal structures.
   Next, the incision in the diaphragm is planned. Ideally, the inci-                      The mesh patch is sewn to the edges of the defect (preferably
sion should be made anterior or lateral to the tumor so that a hand                    with nonabsorbable suture material, such as 0 polypropylene),
can be placed easily into the peritoneal cavity [see Figure 5a]. Intra-                starting at the most anterior and inferior portion of the opening
abdominal palpation confirms that the tumor has not extended                            and continuing toward the surgeon [see Figure 5].The inferior half
into underlying structures. This information is almost always                          of the repair is done with a continuous suture.The repair is com-
gleaned from the preoperative CT scan, but if any uncertainty                          pleted with two or three sutures, which are tied circumferentially.
remains after the scan, diagnostic laparoscopy may be performed                        To prevent paradoxical motion, the diaphragm must not be too
before the thoracotomy to look for possible tumor extension.                           redundant or floppy. It should remain in the normal anatomic
   The tumor is then resected with 2 to 4 cm margins. The large                        position so that the remaining lung can expand completely. In
arteries that course through the diaphragmatic fibers are ligated.                      general, however, it is best to keep the repair taut so as to optimize
It is our practice also to place a few silk sutures (stay stitches) in                 pulmonary mechanics after the procedure.




References

 1. Kirks DR, Caron KH: Gastrointestinal tract.           9. Jani PG: Morgagni hernia: case report. East Afr        18. Piehler JM, Pairolero PC, Gracey DR, et al:
    Practical Pediatric Imaging, 2nd ed. Kirs DR, Ed.        Med J 78:559, 2001                                         Unexplained diaphragmatic paralysis: a harbinger
    Little, Brown & Co, Boston, 1991                     10. Ngaage DL, Young RA, Cowen ME: An unusual                  of malignant disease? J Thorac Cardiovasc Surg
 2. Lewin D, Bowerman R: Hirschel R: Prenatal                combination of diaphragmatic hernias in a patient          84:861, 1982
    ultrasonogram frequently fails to diagnose con-          presenting with the clinical features of restrictive   19. Cerfolio RJ, Bryant AS, Patel B, et al: Intercostal
    genital diaphragmatic hernia. J Pediatr Surg (in         pulmonary disease: report of a case. Surgery Today         muscle flap decreases the pain of thoracotomy: a
    press)                                                   31:1079, 2001                                              prospective randomized trial. J Thorac Cardiovasc
 3. Adzick NS, Harrison MR, Glick PL, et al:             11. Missen AJB: Foramen of Morgagni hernia. Proc R             Surg 130:987, 2005
    Diaphragmatic hernia in the fetus: prenatal diag-        Soc Med 66:654, 1973                                   20. Morgan JA, Morales DL, John R, et al: Endo-
    nosis and outcome in 94 cases. J Pediatr Surg        12. Dawson RE, Jansing CW: Case report: foramen of             scopic, robotically assisted implantation of phrenic
    20:357, 1985                                             Morgagni hernias. J Kentucky Med Assoc 75:325,             pacemakers. J Thorac Cardiovasc Surg 126:582,
 4. Breaux CW Jr, Rouse TM, Cain WS, et al:                  1997                                                       2003
    Improvement in survival of patients with congeni-    13. Lev-Chelouche D, Ravid A, Michowitz M, et al:          21. DiMarco AF, Onders RP, Kowalski KE, et al:
    tal diaphragmatic hernia utilizing a strategy of         Morgagni hernia: unique presentations in elderly           Phrenic nerve pacing in a tetraplegic patient via
    delayed repair after medical and/or extracorporeal       patients. J Clin Gastroenterol 28:81, 1999                 intramuscular diaphragm electrodes. Am J Respir
    membrane oxygenation stabilization. J Pediatr                                                                       Crit Care Med 166:1604, 2002
                                                         14. Kiliç D, Nadir A, Döner E, et al: Transthoracic
    Surg 26:333, 1991
                                                             approach in surgical management of Morgagni            22. Cerfolio RJ, Price TN, Bryant AS, et al: Intracostal
 5. Wenstrom KD,Weiner CP, Hanson JW: A five year             hernia. Eur J Cardiothorac Surg 20:1016, 2001              sutures decrease the pain of thoracotomy. Ann
    statewide experience with congenital diaphrag-                                                                      Thorac Surg 76:407, 2003
                                                         15. Miller JM, Moxham J, Green M: The maximal
    matic hernia. Am J Obstet Gynecol 165:838, 1991
                                                             sniff in the assessment of diaphragm function in       23. Elefteriades JA, Quin JA, Hogan JF, et al: Long-
 6. Panicek DM, Benson CB, Gottlieb RH, et al: The           man. Clin Sci (Colch) 69:91, 1985                          term follow-up of pacing of the conditioned dia-
    diaphragm: anatomic, pathologic, and radiologic                                                                     phragm in quadriplegia. Pacing Clin Electro-
                                                         16. Higgs SM, Hussain A, Jackson M, et al: Long term
    considerations. Radiographics 8:385, 1988                                                                           physiol 25:897, 2002
                                                             results of diaphragmatic plication for unilateral
 7. Fraser RS, Pare JAP, Fraser RG, et al: Synopsis of       diaphragm paralysis. Eur J Cardiothorac Surg
    Diseases of the Chest, 2nd ed. WB Saunders Co,           21:294, 2002
    Philadelphia, 1984                                   17. Higgenbottam T, Allen D, Loh L, et al: Abdominal
 8. Minneci PC, Deans KJ, Kim P, et al: Foramen of           wall movement in normals and patients with hemi-
                                                                                                                                    Acknowledgment
    Morgagni hernia: changes in diagnosis and treat-         diaphragmatic and bilateral diaphragmatic palsy.
    ment. Ann Thorac Surg 77:1956, 2004                      Thorax 32:589, 1977                                    Figures 1 and 3 through 5     Tom Moore.

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ACS Surgery Guide to Diaphragmatic Procedures

  • 1. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 1 15 DIAPHRAGMATIC PROCEDURES Ayesha S. Bryant, M.S.P.H., and Robert James Cerfolio, M.D., F.A.C.S., F.C.C.P. Although the diaphragm is sometimes thought of as little more drainage occurs via the inferior vena cava and the azygos vein on than a partition between the thoracic organs and the abdominal the right and via the suprarenal and renal veins and the hemiazy- organs, it is in fact a dynamic anatomic structure that plays a piv- gos vein on the left. otal role in the physiology of respiratory mechanics. For example, INNERVATION paralysis of just one hemidiaphragm can lead to the loss of 50% of a patient’s vital capacity.1 Like any other anatomic structure, the The diaphragm receives its muscular neurologic impulse from diaphragm may be affected by either benign or malignant condi- the phrenic nerve, which arises primarily from the fourth cervical tions. Overall, benign diseases of the diaphragm (e.g., paralysis) ramus but also has contributions from the third and fifth rami. are far more common than malignant ones. With either type of The phrenic nerve originates around the level of the scalenus ante- condition, however, the development of a safe surgical treatment rior and runs inferiorly through the neck and thorax before reach- strategy depends on a solid knowledge of diaphragmatic anatomy ing its terminal point, the diaphragm. Because the phrenic nerve and physiology. Accordingly, we begin with a brief review of the follows such a long course before reaching its final destination, a embryology and anatomy of the diaphragm.We then describe the number of processes can disrupt the transmission of neurologic main procedures performed to treat the more common congeni- impulses through the nerve at various points and thereby cause tal diseases (e.g., congenital diaphragmatic hernia [CDH]) and diaphragmatic paralysis [see 4:6 Paralyzed Diaphragm]. acquired pathologic conditions (e.g., paralysis and tumor) that affect this structure. Procedures for Congenital Diaphragmatic Hernia Anatomic Considerations REPAIR OF BOCHDALEK HERNIA Bochdalek hernia, named after the Czech anatomist Vincent DEVELOPMENTAL ANATOMY Alexander Bochdalek, is the most common form of CDH and is The diaphragm is a modified half-dome of musculofibrous tis- also the most common surgical emergency in neonates.1 The usual sue that lies between the chest and the abdomen and serves to sep- presenting symptoms are severe respiratory distress and a scaphoid arate these two compartments. It is formed from four embryolog- ic components: (1) the septum transversum, (2) two pleuroperi- Sternal Portion Esophagus toneal folds, (3) cervical myotomes, and (4) the dorsal mesentery. Inferior Development of the diaphragm begins during week 3 of gestation Vena Cava and is complete by week 8. Failure of the pleuroperitoneal folds to Costal Rib develop, with subsequent muscle migration, results in congenital Portion defects. CLASSICAL ANATOMY The diaphragmatic musculature originates from the lower six ribs on each side, from the posterior xiphoid process, and from the external and internal arcuate ligaments. A number of different structures traverse the diaphragm, including three distinct aper- tures (foramina) that allow the passage of the vena cava, the esoph- agus, and the aorta [see Figure 1].The aortic aperture is the lowest and most posterior of the diaphragmatic foramina, lying at the level of the 12th thoracic vertebra. Besides the aorta, the thoracic duct and, sometimes, the azygos and hemiazygos veins also pass through this aperture.The esophageal aperture is the middle fora- men; it is surrounded by diaphragmatic muscle and lies at the level of the 10th thoracic vertebra.The vena caval aperture is the high- est of the three foramina, lying level with the disk space between T8 and T9. Lumbar Portion Tendon VASCULAR SUPPLY The diaphragm is supplied by the right and left phrenic arter- ies, the intercostal arteries, and the musculophrenic branches of the internal thoracic arteries. Some blood is supplied by small Spine Aorta branches of the pericardiophrenic arteries that run with the phrenic nerve, mainly where the nerves penetrate the diaphragm. Venous Figure 1 Shown is an inferior view of the diaphragm.
  • 2. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 2 Preoperative Evaluation abdomen. The primary pathologic condition is the presence of posterolateral defects of the diaphragm, which result either in On chest radiography, a Morgagni hernia appears as a mass at maldevelopment of the pleuroperitoneal folds or in improper or the right cardiophrenic angle [see Figure 2]. Computed tomogra- absent migration of the diaphragmatic musculature. phy of the chest and abdomen, liver scintigraphy, and multiplanar Bochdalek hernias occur in approximately one of every 2,500 magnetic resonance imaging are occasionally helpful in the diag- live births and are twice as common in male neonates as in female nostic process. neonates. Mortality ranges from 45% to 50%. The bulk of the Operative Technique morbidity and mortality of CDH is attributable to the resultant hypoplasia of the lung on the affected side and to various associ- Morgagni hernias can be repaired via a subcostal, a paramedi- ated abnormalities (e.g., malrotation of the gut, neural tube defects, an, or a midline incision. We prefer to use an upper midline and cardiovascular anomalies). abdominal incision. Once the peritoneal cavity is entered, the her- nia sac is identified just posterior to the xiphoid and the posterior Preoperative Evaluation sternal border, then opened. The herniated abdominal viscera are Prenatal ultrasound examination accurately diagnoses CDH in restored to their normal abdominal anatomic positions, and the 40% to 90% of cases.2 In most instances, the examination is per- sac is ligated.The entire hernia sac is defined, resected, and closed. formed to rule out polyhydramnios. It is noteworthy that polyhy- The diaphragmatic defect may be repaired in several different dramnios is present in as many as 80% of pregnant women whose ways, depending on its size and position. Because there is weak tis- fetuses have CDH.3 In neonates with CDH, besides the upper sue in the area of the defect, we generally use a prosthetic patch for gastrointestinal tract, parts of the colon, the spleen, the kidneys, the repair. Either polypropylene mesh (e.g., Marlex; C. R. Bard, and the pancreas may herniate, and the abnormal position of Inc., Murray Hill, New Jersey) or polytetrafluoroethylene (PTFE) these organs can be identified by means of ultrasonography. Mal- mesh (e.g., Gore-Tex; W. L. Gore and Associates, Newark, Del- rotation and malfixation of the small bowel should be ruled out. aware) may be used for this purpose. We prefer PTFE because it Once the diagnosis is confirmed, additional radiographic, echo- may cause fewer adhesions to the underlying abdominal structure, cardiographic, and ultrasonographic studies should be performed which may be an important consideration if further abdominal to rule out associated anomalies. surgery subsequently proves necessary. The prosthetic patch is sewn to the midline abdominal fascia, with wide bites taken to pre- Operative Technique vent an abdominal incisional hernia.The rest of the patch is sewn to the thickened investing fascia that made up the edges of the her- As a rule, neonates with Bochdalek hernias are taken to the oper- nia sac. As noted, the frequently marginal quality of this tissue is ating room immediately after birth. Some studies, however, have the reason why a patch repair is almost always required. shown that delayed surgical repair yields improved survival rates.4 Repair of a Morgagni hernia via a thoracic incision follows the For left-side hernias, a transabdominal subcostal approach is same basic principles.The hernia sac is entered, the visceral con- generally preferred, whereas for right-side hernias, a transthoracic tents are mobilized and reduced into the abdomen, the sac is approach may be more useful.The herniated organs are returned resected, and the diaphragm is repaired. Again, the closure should to the peritoneal cavity. The lung is inspected, but no attempt to be completed without tension. If the defect cannot be closed with expand the hypoplastic lung should be made. If any extralobar horizontal mattress sutures, a prosthetic patch should be used. pulmonary sequestration is present (as is occasionally the case), it should be excised. Most of the defects may be closed primarily Complications with interrupted nonabsorbable sutures; particularly large defects The potential complications of surgical treatment of Morgagni may be closed with a prosthetic patch. The left pleural space is hernia depend to an extent on the type of procedure undertaken drained with a chest tube, which should be placed on water seal. to repair the defect. Laparoscopy may result in failure to reduce the contents of the hernia sac, which necessitates conversion to an Alternative technique Some surgeons have attempted sur- open procedure. Laparotomy has been associated with postoper- gical correction of severe Bochdalek hernias in the prenatal period. ative pleural effusion,9 wound infection,10 deep vein thrombosis,11 The safety and feasibility of this therapeutic approach continue to and pulmonary embolism.12 Thoracotomy has been associated be debated. Prenatal correction of these hernias poses a risk to both with pneumonia, sepsis, and bowel obstruction in the postopera- the mother and the fetus, with possibly fatal results for both.5 tive period.13 REPAIR OF MORGAGNI HERNIA Outcome Evaluation Morgagni hernias, named after the Italian anatomist and pathol- Most patients do not have any significant postoperative limita- ogist Giovanni Battista Morgagni, are related to maldevelopment tions after repair of a Morgagni hernia, nor are such hernias like- of the embryologic septum transversum and to failed fusion of ly to recur. In one study, 16 patients who underwent transthoracic the sternal and costal fibrotendinous elements of the diaphragm.6 repair of a Morgagni hernia were followed for 5.7 years; no recur- These hernias are generally asymptomatic7 and are usually detect- rences or symptoms related to the operation were reported.14 ed as incidental findings on radiographs. Accordingly, the average age at diagnosis is typically greater for Morgagni hernia than for Bochdalek hernia: in one report, the mean age at which the for- Procedures for Diaphragmatic Paralysis mer was diagnosed was 45 years.8 Morgagni hernias are most The diaphragm is the most important of the respiratory mus- commonly seen on the right side.The hernia sac usually contains cles: diaphragmatic contraction decreases intrapleural pressure dur- omentum, but it may also contain part of the transverse colon ing inspiration, expands the rib cage, and thereby facilitates the move- or, less commonly, parts of the stomach, the liver, or the small ment of gases into the lungs. Accordingly, paralysis of the dia- bowel; almost any upper abdominal structure may herniate in this phragm can have a major adverse effect on respiratory function setting. [see 4:6 Paralyzed Diaphragm]. Diaphragmatic paralysis may involve
  • 3. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 3 a b Figure 2 Repair of Morgagni hernia. The differential diagnosis of a cardiophrenic-angle mass includes pericardial fat, a lipoma, a pericardial cyst, a Morgagni hernia, and a thymoma. Shown are (a) chest x-rays and (b) chest CT scans from a 33-year-old man with an incidental finding of a Morgagni hernia. either the whole diaphragm (bilateral paralysis) or only one leaflet In most cases, the diagnosis of hemidiaphragmatic paralysis is or hemidiaphragm (unilateral paralysis). The possible causes of suspected on the basis of incidental findings on a chest x-ray. diaphragmatic paralysis are numerous [see Table 1]; the most com- Typically, the roentgenogram reveals an elevated hemidiaphragm, mon causes are phrenic nerve trauma related to a surgical proce- diminished lung volume, and basilar atelectasis. Fluoroscopy may dure (e.g., stretching, crushing, or transection) and invasion by a also be performed. The diagnosis is confirmed by performing a malignant neoplasm. fluoroscopic sniff test, in which paradoxical elevation of the para- lyzed diaphragm is observed with sniffing.15 The sniff test is the DIAPHRAGMATIC PLICATION FOR UNILATERAL PARALYSIS gold standard for the diagnosis of this condition. In certain pa- tients, a chest CT scan may be indicated for evaluating the poten- Preoperative Evaluation tial cause of the paralysis. If an obvious cause is not apparent from With unilateral diaphragmatic paralysis, the paralyzed the history or a previous evaluation, CT scanning of the chest hemidiaphragm paradoxically moves upward on inspiration and should be performed to ensure there is no pathologic process that downward on expiration, passively following changes in is compressing or invading the phrenic nerve. Similarly, MRI of intrapleural and intra-abdominal pressure. Patients with a par- the neck or the spine may be indicated in certain patients to look alyzed hemidiaphragm who are otherwise healthy usually have for conditions that might be causing the diaphragmatic paralysis. no symptoms at rest but experience dyspnea during exertion Two other tests that are also (albeit less commonly) used for the and show a decrease in exercise performance. Physical exami- diagnosis of unilateral diaphragmatic paralysis are electromyogra- nation may reveal dullness to percussion and an absence of phy and transdiaphragmatic pressure assessment. In the first, the breath sounds over the lower chest on the involved side. phrenic nerve is electrically stimulated in the neck in an effort to
  • 4. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 4 Operative Technique Table 1 Common Causes of Diaphragmatic Paralysis Diaphragmatic plication may be performed with either sutures or staples; we prefer sutures for this procedure. The chest is Spinal cord transection entered through a thoracotomy in the seventh or eighth intercostal Multiple sclerosis space. Horizontal mattress sutures buttressed with Teflon pledgets Amyotrophic lateral sclerosis are then placed in a lateral-to-medial direction [see Figure 3]. We Cervical spondylosis typically use monofilament nonabsorbable sutures that pass easily Poliomyelitis through the muscle and can be tightened without dragging through Guillain-Barré syndrome tissue. To distribute the tension, multiple sutures must be placed; Phrenic nerve dysfunction Neurologic conditions this is especially important on the right side, where the diaphragm Compression by tumor Cardiac surgery cold injury must be pulled down against the upward force exerted by the pres- Blunt trauma ence of the right hemiliver.When the sutures are tied, the hemidi- Idiopathic phrenic neuropathy aphragm should be almost back to its normal anatomic location. Diabetes mellitus Care must be taken to ensure that the repair is not under undue Postviral phrenic neuropathy (herpes zoster) tension: excessive tension is likely to result in early dehiscence. Radiation therapy Occasionally, a prosthetic patch may be used to buttress the repair Cervical chiropractic manipulation further, but in the majority of cases, this measure should be unnec- Limb-girdle dystrophy essary. If the choice is made to use staples rather than sutures, care Hyperthyroidism/hypothyroidism must be taken to ensure that the underlying abdominal contents Malnutrition are not caught in the staple line. Acid maltase deficiency Connective tissue disease DIAPHRAGMATIC PACING FOR BILATERAL PARALYSIS Myopathic conditions Systemic lupus erythematosus Dermatomyositis Preoperative Evaluation Mixed connective tissue disease Amyloidosis In patients with bilateral diaphragmatic paralysis, the respirato- Idiopathic myopathy ry accessory muscles assume all the work of breathing by con- Muscular dystrophy tracting more intensely. Both hemidiaphragms move upward on Multiple sclerosis inspiration, concomitant with inward (rather than the normal out- ward) movement of the abdominal wall.17 Patients typically pre- sent with severe respiratory failure or with dyspnea (sometimes misinterpreted as a sign of heart failure) that worsens in the supine distinguish between neuropathic and myopathic causes of paraly- position, and they generally exhibit tachypnea and rapid, shallow sis. In the second, transdiaphragmatic pressures are measured by placing a thin-walled balloon transnasally at the lower end of the esophagus in such a way as to reflect changes in pleural pressure; a second balloon manometer is then placed in the stomach in such a way as to reflect changes in intra-abdominal pressure. The dif- ference between the two pressures is the transdiaphragmatic pres- sure. Measurement of transdiaphragmatic pressure can help dif- ferentiate diaphragmatic paralysis from other causes of respiratory failure. Yet another test involves measurement of maximal inspiratory pressures. Patients with diaphragmatic dysfunction and paralysis show a decrease in their maximal inspiratory pressures. These patients cannot generate high negative inspiratory pressures, and thus, their maximal inspiratory pressures will be less negative than –60 cm H2O. Operative Planning Surgical treatment of hemidiaphragmatic paralysis is reserved for symptomatic patients who, after a follow-up period of at least 6 months, have persistent shortness of breath with exertion that is sufficiently pronounced to interfere with lifestyle. For a patient to be considered for operation, the sniff test should show significant paradoxical motion. The basic principle of the surgical procedure is to “reef” (i.e., reduce the surface area of) the redundant floppy diaphragm by plicating it. This measure lowers the resting position of the hemidiaphragm and thus affords the lung the opportunity to expand fully. The effective result is an increase in the functional vital capacity of the ipsilateral lung. A 2002 study found that pli- Figure 3 Diaphragmatic plication. Shown is suture plication of cation of the diaphragm led to long-term improvements in pul- the right hemidiaphragm. Placement of sutures buttressed with monary function test results, as well as reduced dyspnea.16 pledgets extends anteriorly to the level of the vena cava.
  • 5. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 5 External Internal anterior horn cells in the nerve roots, diaphragmatic pacing is fea- Receiver and sible only when the lesion is above the C2-C3 level. Accordingly, Electrode Antenna it is sometimes employed in patients with high spinal cord injuries. Phrenic To date, only one pacing device has been approved by the Food Nerve and Drug Administration: the Mark IV Breathing Pacemaker System (Avery Biomedical Devices, Commack, New York). This device possesses an internal component and an external compo- nent [see Figure 4]. The internal component is surgically placed adjacent to the skeletonized phrenic nerve and is connected to a small, wafer-shaped receiving unit placed under the skin. A bat- tery-powered external transmitting box connected to an antenna is taped over the surface of the skin, just above the subcutaneous receiver. This transmitting box permits adjustment of pulse dura- tion, pulse train duration, respiratory rate, pulse frequency, and current amplitude. In most patients, the only parameters that the clinician adjusts are current amplitude and respiratory rate. Implantation of a diaphragmatic pacer requires significant ex- perience on the part of the surgeon—not so much because of any particular technical demands imposed by the implantation itself but because of the procedures for diaphragm training that must be Diaphragm carried out in the postoperative period. Mark IV Choice of surgical approach Surgical implantation of a diaphragmatic pacer can be done via either a cervical approach or a thoracic approach. The primary advantage of the cervical ap- proach is that it avoids the morbidity associated with bilateral tho- racotomies, which may not be well tolerated in patients who have marginal pulmonary function or a history of severe pulmonary con- tusions. However, there are other ways of avoiding this morbidity, such as using video-assisted thoracoscopic surgery (VATS) or per- forming small muscle-sparing, rib-sparing, nerve-sparing thoracot- Figure 4 Diaphragmatic pacing. Shown are internal and external omies.19 One disadvantage of the cervical approach is that in a small pacer connections. percentage of patients, the current amplitude necessary to stimu- late the phrenic nerve results in transmission of the current through the soft tissues.The transmitted current stimulates the functioning breathing when in the recumbent position. Increased expenditure portions of the brachial plexus, causing rhythmic jerking motions of effort in the struggle to breathe may fatigue the accessory mus- of the upper extremities. Another disadvantage is that there are a cles and lead to ventilatory failure. Patients also report anxiety, number of accessory nerve branches arising distal to the neck. insomnia, morning headache, excessive daytime somnolence, One advantage of the thoracic approach is that inadvertent stim- confusion, fatigue, poor sleep habits, and signs of cor pulmonale.18 ulation of portions of the brachial plexus (as sometimes occurs During physical examination, auscultation of the chest reveals with the cervical approach) may be avoided. Another advantage is limitation of diaphragmatic excursion and bilateral lower-chest that there is some neuroanatomic evidence that a small branch of dullness with absent breath sounds. The finding that establishes the phrenic nerve joins the main nerve trunk only after it enters the diagnosis is a paradoxical inward movement of the abdomen the chest cavity; thus, the thoracic approach may stimulate a larg- with inspiration. As with unilateral diaphragmatic paralysis, how- er portion of the phrenic nerve than the cervical approach would. ever, it is more common for the diagnosis to be suspected on the The disadvantage of the thoracic approach is the preconceived basis of a chest roentgenogram that shows bilateral diaphragmat- notion that entry into the chest is associated with a higher mor- ic elevation, then confirmed by means of the sniff test. bidity than entry into the neck. Operative Planning Operative Technique Treatment depends on the cause and severity of the diaphrag- Cervical placement In the neck, the phrenic nerve runs matic paralysis. Most patients are treated with ventilatory support, between the scalenus anterior and the scalenus medius. A trans- but some are treated with bilateral plication or with pacing. Plica- verse skin incision is made in the midportion of the neck, just lat- tion for bilateral paralysis is performed in the same way as plica- eral to the sternocleidomastoid muscle, and the borders of the two tion for unilateral paralysis [see Diaphragmatic Plication for scalene muscles are dissected.The scalene muscles are then divid- Unilateral Paralysis, above], except that both hemidiaphragms are ed, and the phrenic nerve is identified lying in a layer of fascia just lowered. Diaphragmatic pacing is not useful in the treatment of anterior to the anterior surface of the scalenus medius. unilateral diaphragmatic paralysis, because of the difficulty of syn- Identification of this nerve is often facilitated by the use of a hand- chronizing the contractions of the normal hemidiaphragm with held nerve stimulator. Intraoperative fluoroscopy allows observa- those of the paralyzed hemidiaphragm. However, it is sometimes tion of diaphragmatic contraction in response to phrenic nerve an appropriate choice for the treatment of bilateral paralysis, espe- stimulation, which confirms that pacing is successful. cially in patients who have a central condition that is causing their Once the phrenic nerve is identified, it is carefully dissected free apnea. Because the phrenic nerve stimulates the C3, C4, and C5 of its investing fascia, and the Y-shaped electrode is placed under
  • 6. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 6 a b Right Lower-Lobe Tumor Invading Gore-Tex Patch Diaphragm Figure 5 Resection of diaphragmatic tumor. The patient has a right lower-lobe bronchogenic malignancy with erosion into the right hemidiaphragm. (a) The tumor is resected en bloc with the diaphragmatic fibers; the electrocautery is used to achieve clear surgical margins and hemostasis. (b) The defect in the right hemidiaphragm is closed with a mesh patch. it and secured with sutures. Care must be taken to ensure that the there are several specific complications associated with diaphragmat- nerve is not injured during this step.The connecting wire from the ic pacing.The most common of these are dislodgment of the pacer electrode is then tunneled subcutaneously to a subcutaneous pock- electrode, transmission of pacer impulses to the brachial plexus with et that is created just below the ipsilateral clavicle.The connections resultant rhythmic jerking of the upper extremity (seen with cervical are made and sealed, and the small incisions are closed. placement of the electrode), and hardware malfunction. Thoracic placement In the thoracic approach, the chest is Outcome Evaluation entered through a high thoracotomy (usually over the fourth inter- Retrospective analysis of the collective experience at a single cen- space), and the proximal phrenic nerve is identified. On the right ter between 1981 and 1987 suggested that long-term pacing did not side, the nerve lies along the mediastinum, situated just anterior to lead to progressive diaphragmatic dysfunction.23 Six of the 12 patients the vena cava and coursing along the pericardial surface. On the in this cohort continued to undergo diaphragmatic pacing on a full- left side, it lies on the pericardium for most of its length.The prox- time basis for a median period of more than 14 years. Pacing was imal nerve is freed of its fibrous investments, and the electrode is well tolerated in this group; the reasons for discontinuance included placed under it and secured with sutures. The electrode is con- intercurrent medical illness and lack of social support. Concerns nected to the receiver, which is placed in a subcutaneous pocket. have been raised that prolonged diaphragmatic pacing might dam- As noted (see above), there are alternative approaches to pacer im- age the phrenic nerve. In the series cited, however, the ability to pace plantation that avoid the cervical approach but also do not involve stan- the phrenic nerve was not lost in any of the patients, and the mean dard thoracotomies. For example, there is limited (but growing) ex- threshold currents for pacing did not change significantly over time. perience with thoracoscopic placement of phrenic nerve pacing leads.20 In addition, laparoscopic implantation of intramuscular pacing elec- trodes onto the inferior aspect of the diaphragm has been reported.21 Resection of Diaphragmatic Tumors Taking into account all the advantages and disadvantages of each Primary tumors of the diaphragm are extremely rare. Benign approach to pacer implantation, we generally prefer the thoracic tumors (e.g., lipomas and cystic masses) are more common than approach, either via VATS or via a thoracotomy.20,22 The evolution malignant tumors, which mostly are sarcomas of fibrous or muscu- of less invasive surgical techniques may allow the thoracic approach lar origin.Thoracic and abdominal tumors (e.g., bronchogenic car- to be employed in patients who are unable to tolerate thoracotomy. cinomas, pleural malignancies, and chest wall malignancies [see 4:3 Chest Wall Mass]) may involve the diaphragm secondarily through Complications direct extension. Malignant pleural mesothelioma represents a dif- Besides the usual complications associated with any thoracic pro- ferent scenario and is not discussed here. Schwannomas, chondro- cedure (i.e., infection, bleeding, atrial fibrillation, and pneumonia), mas, pheochromocytomas, and endometriomas have all been re-
  • 7. © 2006 WebMD, Inc. All rights reserved. ACS Surgery: Principles and Practice 4 THORAX 15 DIAPHRAGMATIC PROCEDURES — 7 ported. Bilateral occurrence, calcification, sharp margins, and flat- the edges of the defect; this prevents the edges from retracting, tened contours are indicative of a malignant process, such as pleur- helps keep the defect as small as possible, and keeps abdominal al metastases, mesothelioma, or a primary diaphragmatic tumor. contents from interfering with the resection. In addition, we place The most common indication for diaphragmatic resection is clips on the edges for guidance purposes, in case adjuvant radio- mesothelioma. This remains true even though mesothelioma is therapy is delivered after the operation. If adequate margins are relatively uncommon in comparison with bronchogenic malig- obtained, which is usually relatively easy in a diaphragmatic resec- nancy and even though few patients with mesothelioma are actu- tion, postoperative radiotherapy should be unnecessary. If, how- ally candidates for resection. Again, resection of a mesothelioma is ever, the tumor abuts vital structures (e.g., the suprahepatic vena not addressed here. The ensuing operative description focuses on cava), postoperative radiotherapy may have a useful role to play. diaphragmatic resection to treat either a lung cancer invading the Once the entire tumor has been resected and clear margins diaphragm or a primary diaphragmatic tumor [see Figure 5]. have been confirmed by frozen-section examination, the diaphragm is reconstructed. Primary repair is rarely indicated, because in most OPERATIVE TECHNIQUE cases, the defect is too large and the tension on the repair would Once the decision is made to resect a tumor involving the be too great. Moreover, the tissue in the anterior aspect of the diaphragm, the key considerations are (1) the surgical approach to diaphragm is thin and is likely to tear under tension. Accordingly, be taken and (2) the placement of the incision in the diaphragm. repair with a prosthetic patch is the usual choice. Infection of such We prefer a skin incision that is lower and slightly more anterior a patch is exceedingly rare, and with the exception of the cost, than a normal posterolateral thoracotomy; such an incision allows there is little downside to the use of prosthetic material in this set- easy entry over the top of the seventh rib. After entry into the chest, ting. As in the repair of a CDH, we prefer PTFE mesh [see Figure the lung, the pericardium, and the pleural surface are carefully vis- 5b] to polypropylene mesh because it is less likely to adhere to ualized and palpated to search for any signs of metastatic disease. underlying abdominal structures. Next, the incision in the diaphragm is planned. Ideally, the inci- The mesh patch is sewn to the edges of the defect (preferably sion should be made anterior or lateral to the tumor so that a hand with nonabsorbable suture material, such as 0 polypropylene), can be placed easily into the peritoneal cavity [see Figure 5a]. Intra- starting at the most anterior and inferior portion of the opening abdominal palpation confirms that the tumor has not extended and continuing toward the surgeon [see Figure 5].The inferior half into underlying structures. This information is almost always of the repair is done with a continuous suture.The repair is com- gleaned from the preoperative CT scan, but if any uncertainty pleted with two or three sutures, which are tied circumferentially. remains after the scan, diagnostic laparoscopy may be performed To prevent paradoxical motion, the diaphragm must not be too before the thoracotomy to look for possible tumor extension. redundant or floppy. It should remain in the normal anatomic The tumor is then resected with 2 to 4 cm margins. The large position so that the remaining lung can expand completely. In arteries that course through the diaphragmatic fibers are ligated. general, however, it is best to keep the repair taut so as to optimize It is our practice also to place a few silk sutures (stay stitches) in pulmonary mechanics after the procedure. References 1. Kirks DR, Caron KH: Gastrointestinal tract. 9. Jani PG: Morgagni hernia: case report. East Afr 18. Piehler JM, Pairolero PC, Gracey DR, et al: Practical Pediatric Imaging, 2nd ed. Kirs DR, Ed. Med J 78:559, 2001 Unexplained diaphragmatic paralysis: a harbinger Little, Brown & Co, Boston, 1991 10. Ngaage DL, Young RA, Cowen ME: An unusual of malignant disease? J Thorac Cardiovasc Surg 2. Lewin D, Bowerman R: Hirschel R: Prenatal combination of diaphragmatic hernias in a patient 84:861, 1982 ultrasonogram frequently fails to diagnose con- presenting with the clinical features of restrictive 19. Cerfolio RJ, Bryant AS, Patel B, et al: Intercostal genital diaphragmatic hernia. J Pediatr Surg (in pulmonary disease: report of a case. Surgery Today muscle flap decreases the pain of thoracotomy: a press) 31:1079, 2001 prospective randomized trial. J Thorac Cardiovasc 3. Adzick NS, Harrison MR, Glick PL, et al: 11. Missen AJB: Foramen of Morgagni hernia. Proc R Surg 130:987, 2005 Diaphragmatic hernia in the fetus: prenatal diag- Soc Med 66:654, 1973 20. Morgan JA, Morales DL, John R, et al: Endo- nosis and outcome in 94 cases. J Pediatr Surg 12. Dawson RE, Jansing CW: Case report: foramen of scopic, robotically assisted implantation of phrenic 20:357, 1985 Morgagni hernias. J Kentucky Med Assoc 75:325, pacemakers. J Thorac Cardiovasc Surg 126:582, 4. Breaux CW Jr, Rouse TM, Cain WS, et al: 1997 2003 Improvement in survival of patients with congeni- 13. Lev-Chelouche D, Ravid A, Michowitz M, et al: 21. DiMarco AF, Onders RP, Kowalski KE, et al: tal diaphragmatic hernia utilizing a strategy of Morgagni hernia: unique presentations in elderly Phrenic nerve pacing in a tetraplegic patient via delayed repair after medical and/or extracorporeal patients. J Clin Gastroenterol 28:81, 1999 intramuscular diaphragm electrodes. Am J Respir membrane oxygenation stabilization. J Pediatr Crit Care Med 166:1604, 2002 14. Kiliç D, Nadir A, Döner E, et al: Transthoracic Surg 26:333, 1991 approach in surgical management of Morgagni 22. Cerfolio RJ, Price TN, Bryant AS, et al: Intracostal 5. Wenstrom KD,Weiner CP, Hanson JW: A five year hernia. Eur J Cardiothorac Surg 20:1016, 2001 sutures decrease the pain of thoracotomy. Ann statewide experience with congenital diaphrag- Thorac Surg 76:407, 2003 15. Miller JM, Moxham J, Green M: The maximal matic hernia. Am J Obstet Gynecol 165:838, 1991 sniff in the assessment of diaphragm function in 23. Elefteriades JA, Quin JA, Hogan JF, et al: Long- 6. Panicek DM, Benson CB, Gottlieb RH, et al: The man. Clin Sci (Colch) 69:91, 1985 term follow-up of pacing of the conditioned dia- diaphragm: anatomic, pathologic, and radiologic phragm in quadriplegia. Pacing Clin Electro- 16. Higgs SM, Hussain A, Jackson M, et al: Long term considerations. Radiographics 8:385, 1988 physiol 25:897, 2002 results of diaphragmatic plication for unilateral 7. Fraser RS, Pare JAP, Fraser RG, et al: Synopsis of diaphragm paralysis. Eur J Cardiothorac Surg Diseases of the Chest, 2nd ed. WB Saunders Co, 21:294, 2002 Philadelphia, 1984 17. Higgenbottam T, Allen D, Loh L, et al: Abdominal 8. Minneci PC, Deans KJ, Kim P, et al: Foramen of wall movement in normals and patients with hemi- Acknowledgment Morgagni hernia: changes in diagnosis and treat- diaphragmatic and bilateral diaphragmatic palsy. ment. Ann Thorac Surg 77:1956, 2004 Thorax 32:589, 1977 Figures 1 and 3 through 5 Tom Moore.