Diaphragm

Surgical Anatomy of the Diaphragm

The diaphragm is composed of striated muscle originating in the chest and body walls at the thoracic
outlet and inserting into the central tendon. Appropriately named apertures allow passage of the
inferior vena cava, aorta, and esophagus between the thorax and the abdomen. The generous arterial
blood supply is through superior and inferior phrenic arteries, usually directly from the aorta, as well
as the musculophrenic and pericardiophrenic arteries. Venous drainage generally follows the arterial
supply, with drainage primarily into the inferior vena cava or azygous vein on the right and into either
the adrenal or renal and hemiazygous veins on the left. Innervation is principally by the phrenic
nerve. In addition, there are minor contributions from the lower thoracic nerves bilaterally. The phrenic
nerves descend from their cervical origins along the ventral surface of the scalenus anterior muscle
between the subclavian vein and the subclavian artery. The right phrenic nerve passes lateral and
immediately adjacent to the brachiocephalic vein and superior vena cava, descending across the
pericardium anterior to the pulmonary hilum. The left phrenic nerve passes directly posterior to the
thoracic duct and subclavian vein, descending across the pericardium in a course similar to that on the
right. The phrenic nerves enter the muscular diaphragm in anteromedial locations and promptly give
off sternal, posterior, and anterolateral branches with a roughly radial orientation. The phrenic nerve is
vulnerable to surgical trauma from a wide variety of cervical, thoracic, and diaphragmatic procedures.
Unlike adults, infants may depend on diaphragmatic function for adequate ventilation. Therefore, the
anatomic relations of the phrenic nerve are of particular importance in the pediatric age group. Phrenic
nerve injuries and a variety of neuromuscular disorders, including primary myopathies and anterior
horn cell degenerative diseases, may produce diaphragmatic paralysis and require operative plication
of the diaphragm in infants and young children. The diaphragm alone is responsible for the ease of
inhalation and exhalation. However, when exhalation becomes difficult, then intercostal muscles,
pectoral muscles, shoulder and neck muscles participate as well.
Embryogenesis
The mammalian diaphragm is a composite organ formed from four embryonic sources: transverse
septum, mediastinum, pleuroperitoneal membranes, and muscles of the body wall.
Anatomy of Congenital and Acquired Diaphragmatic Hernias
During the first 2 months of fetal life, there is no pressure on the developing diaphragm from above or
below. Above, the lungs are not inflated; below, the growth of the gut is taking place extra-
abdominally, into the umbilical cord. The first mechanical pressure on the diaphragm comes during the
tenth week when the intestines return from the umbilical cord to the abdomen. By that time, all of the
diaphragmatic components are normally in place and have sufficient strength to contain the abdominal
viscera, which might not be the case if the normal developmental timetable is disturbed.
A number of areas of the diaphragm can give way under pressure from the abdominal viscera. Most
diaphragmatic hernias start in these small areas of weakness and enlarge with age.

Hiatal Hernia
Ascent of the stomach into the thorax through the esophageal hiatus of the diaphragm is a common and
poorly understood lesion. It has been found in stillborn infants, but its congenital origin is not well
established.
The two requirements for a sliding hiatal hernia appear to be an enlarged hiatus and a weakened
phrenoesophageal ligament.
Because both conditions are exacerbated by the hernia, the opening is further dilated and the ligament
further stretched. When actual herniation occurs, there is an empty hernial sac of peritoneum on the
left side of the stomach. On the right, the small bare area of the stomach has no peritoneal covering.
Congenital short esophagus can simulate hiatal hernia. It is present in children, although it might be
asymptomatic. The phrenoesophageal ligament is normal, there is no hernial sac, and the left gastric
artery is not displaced upward. The condition is often familial, and it is more common in males. It is

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sometimes associated with pyloric stenosis, malrotation, and Marfan syndrome. We believe that
although it is rare, short esophagus is a true congenital malformation.
Nyhus believes that short esophagus is not congenital and the shortening is caused by secondary
factors in an esophagus of normal length. He stated that infants with chalasia develop peptic
esophagitis and then shortening of the esophagus.
Congenital short esophagus has long been the subject of debate. Three conditions must be
considered.
1.Grossly normal esophagus. The lower portion of the esophagus is lined with gastric mucosa
(Barrett’s esophagus). This condition can also be described as heterotopic gastric mucosa. Far from
being a benign anomaly, as Gray and colleagues believe, it can be a precursor of adenocarcinoma. This
metaplasia is often associated with gastroesophageal reflux.
2.Irreducible partially supradiaphragmatic true stomach. The stomach has herniated into the
thorax through an enlarged diaphragmatic esophageal hiatus and become fixed. This condition is true
fixed hiatal hernia.
3.Partially supradiaphragmatic true stomach existing from birth and not reducible. This
condition is true congenital short esophagus and is very rare.
Barrett believed that congenital short esophagus could be recognized by the absence of a hernial sac.
Branches of the left gastric artery do not pass upward through the hiatus. According to Gray and
Skandalakis, only a small percentage of hiatal hernias belong to this group. Gozzetti and colleagues
stated that acquired short esophagus is the result of gastroesophageal reflux of gastric and
biliopancreatic fluids.
If the gastroesophageal junction remains in its normal position, the fundus of the stomach can herniate
through an enlarged hiatus anterior to the esophagus producing a paraesophageal hiatal hernia.
There is a peritoneal sac anterior to the esophagus containing stomach and, in extreme instances,
transverse colon and omentum. Obstruction of the distal esophagus or the stomach is the usual result.

Posterolateral (Bochdalek) Defects

The defect begins at the vertebrocostal trigone, above and lateral to the left lateral arcuate ligament. At
the time of intestinal return to the abdomen, this trigone is membranous, with few muscle fibers; even
at maturity it is variable in size and degree of muscular development. Spreading of the muscle fibers
permits a defect, the foramen of Bochdalek, to form and spread upward and forward on the dome of
the diaphragm to include the site of the embryonic pleuroperitoneal canal.
The defect can be as small as 1 cm in diameter or it might involve almost the entire hemidiaphragm. It
is much more common on the left. During an operation, usually no hernial sac is found. The small
intestine, stomach, colon, or spleen can be present in the thorax at birth. The lung on the affected side
is usually hypoplastic.
Bilateral defects have been reported
Incidence
The estimated incidence of CDH is 1 : 4000 to 5000 live births, with the incidence rising as high as 1 :
2000 if all stillbirths are included. Associated anomalies, especially of the central nervous system, are
very common in stillborn infants with CDH.
Anatomy
Usually a 1- to 2-cm defect is present in the posterolateral part of the diaphragm, but it can vary
significantly in size and the entire hemidiaphragm may be absent. The size of the diaphragmatic defect
does not correlate with the mass of herniated organs. The defect occurs on the left side in 80 to 85
percent of patients. Very occasionally, it is bilateral. In fewer than 10 percent of cases, a hernia sac is
seen. Intestinal malrotation and malfixation are always present. In left-sided CDH herniated viscera
consist of stomach, small intestine, colon, spleen, and the left lobe of the liver. Rarely, the left kidney,
adrenal gland, and pancreas are displaced into the chest. In right-sided defects, the liver usually
herniates along with the small bowel and occasionally the colon.
There is significant hypoplasia of both lungs, with the weight of the ipsilateral lung being as low as 31
percent and that of the contralateral lung being 60 percent that of control subjects. Along with

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reduction in the size of bronchi, bronchial branching is reduced and the surface area of alveolae is
decreased. In addition, the total size of the pulmonary vascular bed is decreased. There are fewer
vessels per unit of lung. Pulmonary vascular resistance is elevated due to increase in the thickness of
arteriolar smooth muscle with muscular growth extending down to the capillary level.
Herniated viscera compound the problem by forming a space-occupying lesion in the hemithorax that
displaces the mediastinum to the contralateral side, thus kinking the venae cavae and compressing the
contralateral lung.
Pathophysiology
Infants with CDH have to deal with two distinct pathophysiologic entities: pulmonary hypoplasia,
which results in inadequate gas exchange, and pulmonary hypertension, which disrupts pulmonary
blood flow. Of these two elements pulmonary hypertension seems to be the more dangerous problem.
The highly reactive pulmonary vessels are sensitive to acidosis, hypoxia, and hypercarbia. Resultant
pulmonary hypertension produces persistence of fetal circulation with shunting of blood through the
patent ductus arteriosus and the foramen ovale. The shunt further aggravates acidosis, hypoxia, and
hypercarbia, thus perpetuating a vicious cycle. This pulmonary hypertension may also be due to lack of
production or early depletion of pulmonary vasodilators such as prostaglandins and nitric oxide.
Barotrauma caused by ventilation and a transpulmonary pressure gradient (airway pressure minus
intrapleural pressure) seem to play a role in the deterioration in these patients. Surfactant deficiency
also is noted in infants with CDH.
Clinical Findings
Congenital diaphragmatic hernia is often diagnosed antenatally when ultrasonography is carried out for
evaluation of polyhydramnios. Sometimes it is an incidental discovery on a routine antenatal
ultrasound. Ultrasonography will demonstrate a mediastinal shift and loops of intestine, stomach, or
liver in the thoracic cavity. Herniation of the stomach is indicative of a serious lesion. In most patients
not diagnosed antenatally, respiratory distress, tachypnea, and cyanosis develop at birth. On
examination, breath sounds are absent on the ipsilateral side and the heart is displaced toward the
contralateral hemithorax. In addition, the abdomen is scaphoid. A few patients do not develop
symptoms until after 24 hours of life and occasionally not until a few months or years. Patients in
whom symptoms are delayed more than 24 hours have well-developed lungs, and they do very well
after surgery. It is essential to identify associated anomalies, particularly chromosomal abnormalities
(trisomy 13), central nervous system malformations (myelomeningocele), and cardiovascular
anomalies (ventricular septal defect).
Diagnosis
Plain anteroposterior radiographs of the chest and abdomen are generally sufficient to make the
diagnosis of CDH. Typically, the ipsilateral hemithorax has a “soap-bubble” appearance with
contralateral displacement of the mediastinum. In addition, there is a paucity of intestinal gas in the
abdomen. On occasion the nasogastric tube may be seen entering the chest. If liver constitutes the
major part of herniated viscera, one may find a solid shadow in the hemithorax, and a normal amount
of intestinal gas may be present in the abdomen. In such cases cystadenomatoid malformation of the
lung and intralobar pulmonary sequestration have to be differentiated from CDH, and an upper GI
examination may be required.

Parasternal (Morgagni) Defects

Anatomy
Between attachments of the diaphragm to the xiphoid process and to the seventh costal cartilage, there
is a small gap in the musculature on either side of the xiphoid process (foramina of Morgagni).
Herniation at these sites represent only approximately 3 percent of surgically treated hernias of the
diaphragm. The gaps are filled with fat, and the superior epigastric arteries and veins pass through
them.
Herniation through these muscular gaps is almost always the result of postnatal trauma. The hernia is
more often on the right, and the herniated organs are usually the omentum, colon, and, eventually,
stomach. A sac can be present, or it might have ruptured and disappeared. There might be a

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predisposition to herniation in persons with large foramina or in individuals with more fat between
muscle fibers, but this pattern has not been demonstrated.
The foramina of Morgagni are small triangular areas on either side of the inferior end of the sternum at
the anterior aspect of the diaphragm. They are bound by muscle fibers from the xiphoid process
medially and from the costal cartilages laterally. These small gaps, also known as the spaces of Larrey
or parasternal spaces, permit the passage of the superior epigastric vessels. Morgagni hernias usually
occur on the right side but they can be bilateral. A hernia sac is always formed and the superior
epigastric artery is located lateral to it. Transverse colon and omentum are the most common contents
of these hernias.
Clinical Findings
Since patients are often asymptomatic, clinical findings may be discovered incidentally on a chest
radiograph. If the hernia is difficult to differentiate from an anterior mediastinal mass, an upper GI
series is helpful.
Treatment
Due to the potential of Morgagni hernia for incarceration, surgical repair is indicated. An upper
abdominal transverse incision gives adequate exposure. After reduction of the viscera, the empty sac is
grasped through the defect, everted, and excised at its neck. The defect is repaired by placing
horizontal mattress sutures of nonabsorbable material of appropriate size between the lip of diaphragm
at the posterior edge of the defect and the undersurface of the sternum and posterior rectus sheath. The
sutures are tied after they have all been placed.
Omental herniation through the foramen of Morgagni has been reported. Laparoscopic procedure has
been used to repair this defect.
It is not within the scope of this chapter to present the physiology of the diaphragm, but the following
information is pertinent. Roentgenographic findings support the hypothesis that the cardiac mass is
responsible for the caudad displacement of the related hemidiaphragm; this finding is contrary to the
classic teaching that it is the liver that lifts the corresponding hemidiaphragm.
The function of the diaphragm, which is composed of skeletal voluntary muscle, is as automatic as the
function of the heart, with the difference that the cardiac myocytes function as a syncytium. It has been
suggested that the diaphragm is second in importance only to the heart in maintaining life.
The Openings of the Diaphragm
The Hiatus of the Inferior Vena Cava.
The esophageal hiatus is of great surgical importance and is considered in more detail later.
In a study of 50 human diaphragms there were five variations in the formation of the esophageal
hiatus. In 62 percent, the hiatus was formed by both crura, with the right constituting most of the
border and the left sharing only in the formation of the posterior border. In 10 percent, the hiatus was
formed by the medial parts of both crura equally. In 10 percent, the hiatus was formed by the right crus
only. In 2 percent, the left crus exclusively formed the hiatus. In 16 percent, both crura were located
posteriorly and the hiatus was bounded from the median arcuate ligament by a V-shaped band.
Hiatal hernia can be defined as the protrusion of a portion of the stomach into the thoracic
mediastinum through the esophageal hiatus of the diaphragm. A hernial sac is present. Akerlund was
one of the first to classify hernia in this area, and he might have been first to use the term “hiatal
hernia.” He recognized three types of hernia that occur: sliding, paraesophageal, and congenital
short esophagus. Today we recognize an additional type: combined sliding and paraesophageal.
Sliding Hiatal Hernia. The esophagus moves freely through the hiatus with the gastroesophageal
junction being in the thorax or in the normal position at different times. It is usually found in the
normal position at autopsy. Sliding hernias (Fig. 52-15B) constitute 90 percent of all hiatal hernias.
Although these hernias do slide back and forth through the hiatus, they are called sliding hernias
because the stomach composes part of the wall of the hernial sac. Thus, they are analogous with sliding
inguinal hernias.
A sliding hernia can become secondarily fixed in the thorax by adhesion. In such instances, the
esophagus appears to be too short to reach the diaphragm because of contraction of the longitudinal
muscle coat. This type is uncommon.

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 Paraesophageal Hiatal Hernia.
In this type of hernia, the gastroesophageal junction remains in its normal location. The gastric fundus
and greater curvature bulge through the hiatus anterior to the esophagus. Volvulus of the herniated
stomach is a major complication.
Paraesophageal hernias usually are of the combined type.
Surgical Management of Paraesophageal Herniation
Paraesophageal herniation is an uncommon disorder of the gastroesophageal hiatus characterized by
the potential for life-threatening complications resulting from mechanical obstruction and vascular
compromise of the stomach. This scenario of paraesophageal hernias contrasts with the more common
sliding esophageal hiatal hernia, which is principally defined by its frequent association with
symptomatic gastroesophageal reflux.
The sliding esophageal hiatal hernia is anatomically characterized by a laxity in the phrenoesophageal
ligament, which usually anchors the gastroesophageal junction to its normal intra-abdominal location
and maintains the relationship of the distal esophagus to the gastric cardia and fundus. A variable
cephalad migration of the gastroesophageal junction through the hiatus into the posterior mediastinum
is characteristic. These abnormalities in the phrenoesophageal ligamentous attachments of the
gastroesophageal junction also increase the risk for impaired function of the lower esophageal
sphincter. This in part explains the common association of pathologic gastroesophageal reflux with
sliding (type I) hiatal hernias.
In distinction, primary paraesophageal (type II) hiatal hernias are associated with preservation of
the normal posterior phrenoesophageal ligamentous anchorage of the gastroesophageal junction within
the abdomen. These true hernias are defined by a large peritoneal lined opening in the esophageal
hiatus anterior to a normally positioned gastroesophageal junction (Fig. 56-2).
The combination of sliding and paraesophageal hernia components (type III hiatal hernia) is also
commonly encountered. Patients with this combined hernia process will often have symptoms related
to pathologic gastroesophageal reflux and to mechanical obstruction of the stomach within the
paraesophageal component of the hernia. The gastroesophageal junction is displaced upward as in a
sliding hernia, and the fundus and greater curvature are herniated as in a paraesophageal hernia
In the vast majority of circumstances, the body of the gastric fundus is the lead point of the
paraesophageal herniation; however, other intra-abdominal viscera may also herniate (type IV
hiatal hernia) into the mediastinum anterior to the stationary gastroesophageal junction. Air
within the obstructed stomach or other herniated hollow viscera results in the characteristic
roentgenographic findings of an air fluid level within a retrocardiac mass.
Clinical Presentation
As with other true hernias of the abdominal cavity, paraesophageal hernias are primarily distinguished
by symptoms related to intermittent mechanical obstruction of the herniated gastric fundus. Typically,
patients with paraesophageal hernia are older than patients identified with sliding hiatal hernias. On
careful questioning paraesophageal hernia patients will usually admit to having a long history of upper
aerodigestive complaints that can be attributed to their hiatal hernias. This has led some investigators
to believe that paraesophageal hernias are simply a more advanced stage of the commoner sliding
hiatal hernia. The physiologic case against this is that only a minority of patients with primary
paraesophageal hernias admit to having classic gastroesophageal reflux symptoms and anatomically
the gastroesophageal junction usually remains in its normal intra-abdominal position. Indeed, the
primary complaints of patients with paraesophageal hernia are intermittent substernal chest pain,
cough, and aspiration events related to distention and obstruction of the esophagus and intrathoracic
stomach. The patient may also present with occult or frank gastrointestinal bleeding from the
intrathoracic stomach related to “acid stasis” ulceration or chronic mucosal venous engorgement. The
gastroesophageal reflux symptoms of regurgitation and heartburn are usually minor complaints unless
the patient also has impairment in the integrity of the lower esophageal sphincter mechanism. This
latter circumstance is usually associated with the anatomic, physiologic, and roentgenographic findings

5
found with the minority of patients who have “truly” mixed (type III) sliding and paraesophageal
herniation.
The most devastating complications of paraesophageal hernias are gastric incarceration and vascular
strangulation, which can be associated with a mortality approaching 50 percent. Surgical treatment is
generally indicated to avoid these catastrophes related to gastric obstruction or volvulus.
We believe in individualizing the operative approach utilized for paraesophageal hernia repair to the
patient’s pathophysiologic condition rather than attempting to apply a single repair for all patients with
this heterogeneous clinical problem. Accordingly, we advocate selective application of primary repair
alone or the alternative of repair with fundoplication in the management of paraesophageal herniation.
This chapter describes our surgical approach to the broad clinical spectrum of paraesophageal
herniation.
Diagnostic Workup
The primary diagnostic approach to paraesophageal herniation is the barium esophagogram and upper
GI series. Endoscopic assessment is also an important preoperative evaluation. Because insufflation
during the endoscopic procedure can precipitate acute gastric incarceration requiring urgent surgery,
we usually defer this intervention until the time of the proposed elective surgical procedure. If
significant esophagitis is identified at the endoscopic examination, fundoplication should be included
in the management of the good-risk paraesophageal hernia patient with a clinical history consistent
with symptomatic gastroesophageal reflux.
Because of technical difficulty in passing the manometric catheters into the stomach for the assessment
of lower esophageal sphincter function, esophageal manometry is not routinely performed in the
elective evaluation of patients with primary paraesophageal hernias who do not have reflux symptoms.
The diagnostic importance of esophageal manometric studies in this clinical setting of primary
paraesophageal herniation is therefore limited to determining the integrity of esophageal body
function. A relatively normal lower esophageal sphincter pressure is usually noted among patients with
paraesophageal hernia, which contrasts with the low mean sphincter pressure noted among patients
with sliding hiatal hernias and pathologic reflux esophagitis. Even among paraesophageal hernia
patients with visible esophagitis, the most consistent manometric abnormality identified is a reduction
in the overall length of the lower esophageal sphincter. At the present time, our primary use of
esophageal manometric testing is to determine the adequacy of esophageal body peristalsis before
adding fundoplication to the elective repair of mixed sliding and paraesophageal hernias.
Prolonged intraesophageal pH testing is also of limited benefit in the evaluation of patients with
primary paraesophageal hernia, as the primary cause of increased esophageal acid exposure is impaired
emptying of gastric acid from the herniated intrathoracic gastric fundus. Reduction of the herniated
stomach to its normal intra-abdominal position during the course of the repair of the paraesophageal
hernia usually results in normal gastric emptying and resolution of the abnormal esophageal acid
exposure. This consideration has dissuaded us from relying on prolonged esophageal pH studies in the
preoperative assessment of the integrity of the lower esophageal sphincter for most paraesophageal
hernia patients.
Preoperative Decision Making Regarding the Operative Approach
For the most part, the operative approach to paraesophageal herniation is determined by the clinical
urgency of the situation, and the age and functional status of the patient. The preoperative
determination of associated pathologic gastroesophageal reflux will also affect the operative strategy.
We will usually include a fundoplication with the paraesophageal hernia repair if significant
esophagitis is identified in a patient with a consistent clinical history for gastroesophageal reflux. If
esophageal mucosal injury is absent and the gastroesophageal junction is in its normal intra-abdominal
location, we rely upon anatomic repair of the paraesophageal hernia defect and reduction of the
stomach to its normal intra-abdominal position.
Under most circumstances, an abdominal approach is used to accomplish the paraesophageal hernia
repair; however, thoracotomy is chosen for the management of “primary” paraesophageal hernias
when significant esophageal shortening is identified by preoperative barium contrast studies and

6
endoscopy examination (Fig. 56-6). We also favor the transthoracic approach to manage the patient
with symptomatic paraesophageal herniation that occurs after a previous antireflux surgical procedure.
Transabdominal Repair of Paraesophageal Herniation
Anatomic Repair
After induction of general anesthesia, esophagoscopy is routinely performed to identify the presence of
significant esophagitis and the degree of anatomic shortening of the esophagus. We usually defer this
endoscopic evaluation until the time of operation because of the potential risk of inducing gastric
incarceration within the paraesophageal hernia when insufflation maneuvers are utilized during the
examination. We attempt to examine the stomach and duodenum at this endoscopic procedure;
however, it is often impossible to negotiate the endoscope through the gastroesophageal junction when
the entire stomach is herniated into the chest. The finding of significant esophagitis in the patient with
significant reflux symptoms will usually lead us to include fundoplication with the paraesophageal
hernia repair.
Following the endoscopic examination, the patient is surgically prepared in the supine position. The
abdomen is opened through an upper midline abdominal incision extending from the xiphoid process
to the umbilicus. Attention is directed to the esophageal hiatus and the left upper quadrant after a
general exploration of the abdominal cavity is performed. The fundus of the stomach is usually found
to have migrated within the enlarged esophageal hiatal opening. When the paraesophageal herniation is
large, it is also common to have the omentum and even the transverse colon within the hernia (see Fig.
56-3 and Fig. 56-4).
The technical aspects of anatomic repair of paraesophageal hernias are straightforward. The primary
management principles are reduction of the herniated abdominal viscera, hernia sac excision, and
crural repair.
Laparoscopic Approach
Familiarity with the technical nuances of endosurgical instrumentation and the general conduct of
laparoscopic surgical approaches is a vital prerequisite before laparoscopic repair of paraesophageal
hernias is attempted. Likewise, the surgeon should be experienced with the “open surgical” approaches
to repair of paraesophageal hernias, as the technical standards of “open surgical” management must be
maintained to avoid suboptimal results. The surgeon must also be prepared to convert to an open
surgical approach when the operative conditions preclude a safe or effective surgical repair of the
paraesophageal hernia.
Laparoscopic techniques can be readily applied in the management of paraesophageal herniation.
Concomitant Antireflux Procedures
To reiterate, we selectively utilize fundoplication as a specific antireflux procedure in the management
of patients with paraesophageal hernia. The decision to include fundoplication with the repair is based
upon the endoscopic findings of esophagitis, a redo operative status, or the intraoperative evidence of a
significant sliding hiatal hernia and esophageal shortening.
In general, patients with primary paraesophageal hernia who are elderly or physiologically impaired,
and those without evidence of clinical esophagitis, are not considered for fundoplication. In these
circumstances, the “anatomic repair of Collis” is the primary antireflux mechanism relied upon. When
equivocal clinical findings of significant gastroesophageal reflux are identified, we utilize the “Hill”
arcuate ligament repair to anchor the gastroesophageal junction within the abdomen and restore an
effective antireflux barrier.
When a fundoplication procedure is necessary, dissection about the esophageal hiatus is completed
with division of the posterior phrenoesophageal ligamentous attachments along the entire
circumference of the distal esophagus and the lesser curvature of the stomach. The fundoplication
procedure chosen is dictated by determination of the severity of the reflux process and the integrity of
the esophageal peristaltic pump. This is determined by preoperative esophageal barium studies and
standard esophageal manometric testing of esophageal body function.
When the adequacy of esophageal peristalsis is in question, we rely upon a partial fundoplication to
avoid potential postoperative dysphagia related to too competent a wrap following total fundoplication.
The “D’or” anterior partial fundoplication is usually chosen for patients approached through the

7
abdomen. The “Toupet” posterior partial fundoplication procedure is avoided because of the inherent
difficulties in providing adequate posterior diaphragmatic crural support for the mobilized fundus
resulting from the anatomic distortion in the esophageal hiatus. The “Belsey” partial fundoplication is
chosen when thoracotomy is used to approach the paraesophageal hernia patient with impaired
esophageal peristalsis. When esophageal peristalsis is normal, a standard “floppy” Nissen
fundoplication (2–3 cm in length) about a No. 50 to 60 French intraesophageal bougie is utilized. The
extensive pexy of the gastric fundus to the diaphragm utilized with anatomic repair alone is not
necessary when a total fundoplication is created. We do include a few pexing stitches at the completion
of the repair between the fundus and the crural margins to secure the redundant aspect of the fundus.
Pexing gastrostomy is only utilized with fundoplication in the management of the patient with
pulmonary functional impairment whose postoperative respiratory hygiene will be potentially
compromised by the discomfort associated with an indwelling nasogastric tube.
Management of Incarcerated Paraesophageal Herniation
Gastric incarceration most commonly occurs in patients with large paraesophageal hernias associated
with chronic intrathoracic displacement of the entire stomach. Before the incarcerating event, the
individual’s chief complaint is chronic substernal pain related to intermittent distention/obstruction of
the intrathoracic stomach. Incarceration of the stomach within the paraesophageal hernia defect results
when the distended fundus of an intrathoracic stomach slips back into the abdomen. This results in the
development of two closed loop obstructions of the stomach: one within the intrathoracic antrum and
the other within the gastric fundus that has slipped back into the abdomen through the hiatal opening.
The distal esophagus and the proximal duodenum are also obstructed by the incarcerated mass bound
at the diaphragmatic hiatus (Fig. 56-15). In spite of the acute upper abdominal distress, the patient is
unable to vomit, although retching attempts are commonly observed. Chest roentgenography will
usually demonstrate a characteristic distended, retrocardiac air-filled mass representing the
intrathoracic gastric antrum. A similarly distended gastric fundus will be noted beneath the diaphragm
on chest film or on flat plate roentgenographic examination of the abdomen. The primary therapeutic
maneuver for this emergency situation is passage of a nasogastric tube to decompress the distended
gastric fundus. Immediate relief of the patient’s symptoms can be seen if the nasogastric tube can be
successfully passed into the fundus of the stomach. The patient can then be prepared for semielective
paraesophageal hernia repair since relief of the gastric volvulus has been accomplished.
An emergency situation exists when nasogastric decompression of the stomach cannot be
accomplished. Mesenteric vascular compromise of the obstructed antrum and fundus is imminent. We
approach these patients through an upper midline laparotomy; however, the left chest is always
surgically prepared for thoracotomy should this approach be necessary to facilitate reduction of the
stomach or resection of a devitalized gastric segment. On entry into the abdomen, the ischemic and
distended fundus is immediately visualized in the upper abdomen. The surgeon should attempt to
gently reduce the gastric antrum into the abdomen through the hiatal opening. Decompressive
gastrotomy to reduce the intragastric tension may allow for successful reduction. If the reduction of the
antrum is not possible after decompression of the fundus, we incise the anterior rim of the diaphragm
at the hiatal margin to gain a larger opening for hernia reduction. If the antrum remains incarcerated,
thoracotomy will be necessary to accomplish decompression of this distended gastric segment before it
can be reduced into the abdomen.
After reduction of the stomach has been accomplished, the general principles of paraesophageal hernia
management outlined for anatomic repair are used. In this clinical circumstance, we are less likely to
include a fundoplication to the paraesophageal hernia repair. Creation of a fundoplication with the
edematous gastric fundus about similarly distorted distal esophageal tissue is prone to result in
suboptimal operative results. This, taken together with the fact that fewer than one-third of patients
with paraesophageal hernia have a history of classic gastroesophageal reflux symptoms, leads us to
avoid fundoplication in this urgent clinical setting unless definitive evidence of pathologic
gastroesophageal reflux exists.
When gastric necrosis has occurred, resection of the devitalized segment must be performed. We
usually rely upon stapled transection of the esophagus at the gastroesophageal junction and resection

8
of the segment of devitalized stomach. Distal gastrostomy and jejunostomy are performed. A colonic
interposition is utilized to restore gastrointestinal continuity at a later date once the patient has
recovered from this primary surgical intervention.
Thoracic Management of Paraesophageal Hernias
As was previously mentioned, our primary indication for the thoracotomy approach to paraesophageal
hernia repair is the situation in which significant “shortening” of the esophagus is noted
roentgenographically and endoscopically. Thoracotomy is also preferred when approaching
symptomatic paraesophageal hernias that develop after a previous antireflux surgical procedure. In this
latter circumstance, thoracotomy can provide the best exposure of the hernia process. The thoracotomy
approach also allows for improved mobilization of the esophagus so that tension-free positioning of
the gastroesophageal junction is accomplished within the abdomen at the completion of the hernia
repair.
Elective repair of these “true” hernias of the esophageal hiatus is usually recommended, regardless of
the severity of symptoms, to avoid the life-threatening complications of obstruction, perforation,
bleeding, and gastric strangulation. This elective surgical management approach to paraesophageal
herniation is supported by us for all but the seriously unfit patient.
Although we recognize that symptomatic reflux may be seen in 10 to 15 percent of patients with
primary paraesophageal hernias (type II) following anatomic repair, we do not favor universal
application of fundoplication in their management. Our approach is affected by our belief that the risk
of significant postfundoplication problems outweighs the possible benefits of obtaining total control of
reflux symptoms in this characteristically elderly group of patients. If mild to moderate postoperative
reflux symptoms develop in these patients, present-day antisecretory and prokinetic medical therapies
will almost always control the patient’s symptoms. It is also important to realize that the routine
addition of fundoplication to the paraesophageal hernia repair does not reliably protect against
postoperative reflux symptoms. Actually the occurrence of significant postoperative reflux symptoms
has been found to be similar whether anatomic repair alone or repair with routine fundoplication is
applied for patients with primary paraesophageal herniation (type II hiatal hernias). However, the
use of fundoplication does appear to be valid when managing patients with pathologic
gastroesophageal reflux associated with a “mixed” (type III) sliding and paraesophageal hernia.
One should tailor repair of paraesophageal hernias to each patient’s pathophysiologic condition rather
than applying one universal surgical treatment policy. Accordingly, a good clinical result can be
expected in over 90 percent of patients with paraesophageal hernia when appropriate surgical
management principles are followed. The surgeon addressing such complex problems of the
esophageal hiatus should utilize a flexible repair approach adapted to the individual needs of the
patient rather than narrowly following preconceived doctrines. Likewise, it is important that the
esophageal surgeon approaching paraesophageal hernia repair be well versed in the various options
available for the management of these challenging cases.

Congenital Short Esophagus.

Absent Diaphragm
On occasion, upon reduction of CDH, one finds a near-complete or total absence of the
hemidiaphragm. This cannot be predicted preoperatively since neither the severity of symptoms nor
the amount of herniated viscera noted on a radiograph correlates with the size of the diaphragmatic
defect. In such patients primary closure of the diaphragm is not feasible. If the low transverse
abdominal incision described is made, one may be able to turn the cut edge of the transversus
abdominis muscle inward to suture it to the posterior rim of the diaphragm or to the ribs posteriorly. If
a high transverse incision or a subcostal incision is used, the length of muscle is insufficient to permit
such a repair. If the muscle flap is of inadequate size, a prosthetic patch is sutured into the defect. We
prefer to use Goretex (polytetrafluoroethylene). We have not noted the higher incidence of disruption
and recurrence of hernia reported in the literature. We have seen seepage of abdominal fluids and
blood into the left hemithorax through the suture line, and in these patients the chest tube is used to

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evacuate fluid to maintain the mediastinum in the midline. These babies invariably require the creation
of a ventral hernia.

Eventration of the Diaphragm

Congenital eventration describes the abnormal elevation of one leaf of the diaphragm. The entire
leaf bulges upward in contrast to the localized defect of a foramen of Bochdalek hernia. The
left side is affected more often than the right, and males are affected more often than females.
The phrenic nerve appears normal, but the eventrated leaf can consist of a fascial layer with
few or no muscle fibers between the pleura and peritoneum. The failure is of muscularization
rather than fusion of embryonic components. Intestinal malrotation is often associated. The
lung is usually partially collapsed but not hypoplastic; the mediastinum is shifted to the
contralateral side, which further reduces ventilation.
By contrast, acquired eventration is the result of phrenic nerve injury with normal musculature. The
acquired lesion can be temporary; the congenital lesion is permanent unless repaired.
Eventration can be unilateral or bilateral and can rupture in later life. Eventration and acute gastric
volvulus have been seen in pediatric patients.
Peritoneopericardial Hernia
Peritoneopericardial hernia is a rare hernia that is embryologically inexplicable. It has been reported in
newborn infants and in adults. A hernial sac, or a trace of one, has been found in a few instances.
Because the defect is in the central tendon and the overlying pericardium, it originates in that part of
the diaphragm formed by the transverse septum.
Liver herniation into the pericardium through the central tendon has been reported.
Clinical Presentation
Most adult patients with diaphragmatic eventrations are asymptomatic, and require no specific
surgical therapy. When present, symptoms arise as a result of inadequate ventilation or displacement of
the abdominal viscera within the eventration. Respiratory distress, cyanosis, tachypnea, tachycardia,
and atelectasis with or without pneumonia predominate. Significant respiratory symptoms are most
common in infants and children secondary to the greater mobility of the mediastinum and their
reliance upon diaphragmatic rather than intercostal muscles for respiration. On inspiration, the
involved hemidiaphragm rises, causing mediastinal shift to the opposite side. This impairs the
ventilation of the involved hemithorax, compresses the contralateral lung, and places torsion on the
great vessels and heart. The degree of paradoxical diaphragmatic motion is slightly greater but not
restricted to patients with acquired eventrations.
In infants, the respiratory symptoms increase during feedings or with abdominal distention. In
addition, patients with asymptomatic eventrations can develop significant respiratory distress when
mild respiratory infections occur. Symptoms associated with displacement of the abdominal viscera
include abdominal pain, dysphagia, belching, heartburn, and epigastric pain in older patients. Infants
with congenital eventration can also have a variety of other anomalies, including malrotation of the
intestine, megacolon, hypospadias, situs inversus, congenital heart disease, Ehlers-Danlos syndrome,
cleft palate, tracheomalacia, and bony abnormalities. The presence of these associated anomalies is a
strong argument for a congenital etiology in these infants.
Infants and children with diaphragmatic eventration are also predisposed to acute gastric volvulus.
Although this condition is rare, it requires prompt recognition and operative treatment. Organoaxial
volvulus is more common than mesenteroaxial volvulus. However, both have been described.
Eventration may predispose the patient to gastric volvulus by elongation of the gastrophrenic and
gastrosplenic ligaments, allowing free rotational mobility of the stomach. This diagnostic possibility,
heralded by unexplained vomiting, requires urgent investigation in infants with a known eventration.
Diagnosis
Dullness of the involved hemithorax, poor diaphragmatic excursion, and findings that suggest
pneumonitis are the hallmarks on physical examination. Although the diagnosis can be suggested by
physical examination, in most cases, eventration is first recognized during the radiographic
investigation of respiratory distress. Chest radiographs demonstrate the characteristic elevation of the

10
involved hemidiaphragm. Demonstration of paralysis or paradoxical motion of the diaphragm requires
chest fluoroscopy or ultrasonography. Radionuclide ventilation-perfusion scans often show a 50 to 75
percent decrease in ventilation on the involved side; however, this quantification of the ventilation
defect is not always closely correlated with the severity of symptoms. Radiographic differentiation
between CDH with a sac and eventration is difficult, if not impossible, in extensive cases.
The characteristic physical findings and radiographs depicting eventration are not present in patients
undergoing mechanical ventilation at the time of examination. Positive-pressure ventilation reverses
the diaphragmatic displacement, and pulmonary ventilation appears normal. Chest radiographs with
the patient breathing spontaneously are necessary to identify the characteristic radiographic findings,
and should be undertaken in all patients who fail ventilator discontinuation. In addition, eventration
must be considered in infants with unexplained respiratory distress who require urgent intubation
before baseline chest radiography can be obtained.
Indications for Operation
The indications for operation include 1) progressive respiratory distress, atelectasis, and/or
pneumonia; 2) eventration associated with birth injury in infants who do not improve during
observation; and 3) patients with known phrenic nerve injury during thoracic or mediastinal operation
Operative Correction
The goals of surgical therapy should be 1) to restore the diaphragm to a normal location within the
involved hemithorax, 2) to restore normal capacity to the hemithorax to allow lung growth in infants
and children, 3) to restore normal visceral location in the abdomen, and 4) to stabilize the mediastinum
by eliminating paradoxical motion of the diaphragm. These goals have been achieved by two different
surgical procedures, one using plication and the other resection of the redundant hemidiaphragm.
Either procedure should effectively eliminate the passive redundant diaphragmatic tissue, and establish
in its place a rigid diaphragmatic structure. Although the potential for recovery of phrenic nerve
function may exist in cases of neural paralysis, the recovery of normal neuromuscular function cannot
be predicted or anticipated.
The operative approach uses a posterolateral thoracotomy in most cases. All right-sided eventrations
are best approached through the chest. Left-sided lesions can be approached via a thoracotomy or
laparotomy. Our preference is to proceed with thoracotomy due to the better exposure of the phrenic
nerve branches. Bilateral eventrations, although rare, are best approached through a laparotomy
incision. Patients who present with gastric volvulus and eventration should undergo laparotomy for
gastric fixation and diaphragmatic plication.

Traumatic Rupture of the Diaphragm

Sennertus was the first to report the postmortem finding of a strangulated stomach associated with a
diaphragmatic hernia in 1541. Three hundred years later, in 1853, Bowditch recognized the injury
before death and is given credit for describing some of the classic clinical findings, such as mediastinal
shift, dullness to percussion, and bowel sounds auscultated in the chest.
Diaphragmatic rupture resulting from blunt or penetrating trauma is a relatively common occurrence in
the injured patient. While the true incidence is unknown, autopsy series of patients with blunt trauma
who died before arrival at a hospital suggest that approximately 5 percent have a ruptured diaphragm.
The injury is found more frequently in patients who sustain penetrating trauma, and in some subsets,
such as anterior wounds below the nipple, the occurrence may be as high as 30 percent.
Mechanism of Injury
Although diaphragmatic injuries occur with both blunt and penetrating trauma, the latter is far more
prevalent. The relative risk of injury associated with knife wounds is dependent upon variables that are
frequently unknown. These include the size and length of the offending weapon, the position of the
diaphragm in the respiratory cycle, and the effect of concurrent diseases. The large surface area of the
diaphragm leaves it susceptible to injury with gunshot and shotgun wounds. These may be more
difficult to assess because of the unpredictable trajectory often taken by missiles. Missiles may enter
the chest, perforate the diaphragm, injure intra-abdominal organs, and somehow end up back in the
chest cavity. Awareness of these erratic pathways will alert the astute physician to the possibility of

11
diaphragmatic injury in patients who on first assessment seem to have their injuries limited to the
chest. Seemingly innocuous external wounds may be deceptive and despite their minuscule size may
traverse the diaphragm.
Penetrating injuries often leave a small rent in the diaphragm. The combination of intra-abdominal
pressure, which may be as high as 150 mm Hg; negative intrathoracic pressure; and the constant
motion of the diaphragm may prevent spontaneous healing of the injury. On the contrary it is not
unusual for these small injuries to increase in size with time to the point where they allow herniation
and subsequent strangulation of intra-abdominal viscera.
Blunt trauma may cause a burst type of injury to the diaphragm when associated with a sudden
increase in intra-abdominal pressure. This is commonly seen in patients wearing lap-type seat belts
who are suddenly compressed with rapid deceleration-type collisions. Victims of lateral impact
collisions may be more likely to sustain a ruptured diaphragm than those subjected to frontal
collisions. Deformation of the chest wall, which creates a shearing force on the diaphragm; crush
injuries; and other types of trauma that cause an increase in pressure in the abdominal cavity are all
forces that are capable of producing a disruption. The injury usually extends in a posterolateral fashion
from the central tendon and is more common on the left. The same mechanism occurs on the right;
however, it is thought that some of the force may be dissipated by the liver, which in turn may afford
some protection to the right hemidiaphragm. The defect in the diaphragm tends to be larger when
associated with blunt trauma. The diaphragm may be avulsed from its lateral attachments to the chest
wall and less frequently may involve its pericardial surface. Fragments of rib fractures can also cause
penetration of the diaphragm.
It is often reported that diaphragmatic injuries occur more frequently on the left side. In patients who
sustain stab wounds, this is attributed to the fact that most assailants are right handed. The
preponderance of left-sided wounds associated with blunt trauma is explained on the basis of the left
posterior leaf being the weaker portion of the diaphragm. Recent reports seem to describe a more equal
distribution of these injuries, which is certainly the case with penetrating injuries and many blunt
injuries as well.
Diagnosis
The high incidence of associated intra-abdominal injuries results in early diagnosis and surgical
exploration in many patients. In those without indications for surgical intervention, the recognition of
this injury becomes much more difficult. Although history and physical examination are the hallmark
of clinical diagnosis, their accuracy is disappointing in detecting diaphragmatic injury. With visceral
herniation one may detect decreased breath sounds, the presence of bowel sounds on auscultation, or
dullness and on occasion tympany to percussion. More commonly these injuries produce no abnormal
physical signs
Routine chest roentgenograms are limited in their ability to identify a diaphragmatic defect unless
abdominal visceral herniation has occurred (Fig. 55-3).
Nonspecific findings suggestive of this injury are a hemothorax, basal subsegmental atelectasis,
elevation of the injured hemidiaphragm, or irregularity in the diaphragmatic contour. The seemingly
elevated hemidiaphragm may represent the arcuate contour of a partially herniated gastric fundus or a
portion of the liver. On the contrary, elevation of the hemidiaphragm caused by phrenic nerve damage
or eventration may permit the abdominal viscera to occupy a position high in the thorax, thus
mimicking herniation. This confusion may be resolved by instilling contrast material in either the
stomach or colon. A contrast study may demonstrate constriction of the viscera as it passes through the
defect in the diaphragm. With the routine use of nasogastric tubes in trauma patients, one may
occasionally see the tube in the chest on the routine chest film.
Serial x-rays may be of some value. Because of the pressure differential between the chest and
abdominal cavity, the visceral organ may be sucked into the chest and become apparent at a later time.
It is also possible that the defect will expand with time, allowing herniation to occur, hence delaying
the diagnosis.
Pneumoperitoneum may be seen on a preoperative chest x-ray. If a hollow viscus injury is not found at
celiotomy, one should be suspicious of a diaphragmatic injury. Pneumoperitoneum has been used in

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the past as a diagnostic test; however, it is unreliable and has essentially been discarded. The omentum
or herniated viscus often plugs the defect rendering the study useless.
Diagnostic peritoneal lavage has been a poor predictor of diaphragmatic injury. The defect in the
diaphragm rarely produces significant bleeding and unless there is hemorrhage from associated injuries
the lavage is likely to be negative. Most series report a 20 to 25 percent incidence of false-negative
lavages. On occasion useful information is gained, by lavage, if the patient has a chest tube inserted
before the study. If a large clear effluent is noted consistent with the infusion of fluid into the
peritoneal cavity, one can surmise that a defect is present in the diaphragm.
In spite of the frequent use of computed tomography, its ability to demonstrate diaphragmatic injuries
has been disappointing. Gelman and associates reported the study as being diagnostic in only one of
seven patients with proven injury in whom it was performed. These authors noted that an MRI
identified the injury in the two patients in whom it was utilized. There has not been enough experience
with this study to determine its efficacy at this time.
With the current interest in laparoscopy varying reports are beginning to appear in the literature. Many
are favorable; however, there is no question that some of these injuries are missed. The accuracy is
dependent upon the laparoscopic technique that is used and the location of the injury. Possible
insufflation of the chest cavity with its potential pulmonary complications was an early concern, but at
present has not been a major problem. Ivatury and his coauthors reported finding eight clinically
unsuspected diaphragmatic injuries in a series of 40 patients who sustained penetrating trauma to the
lower chest.
The newest and perhaps most accurate study in the evaluation of diaphragmatic injuries is video-
assisted thoracoscopy. Videothoracoscopy is an accurate, safe, and minimally invasive method for the
assessment of diaphragmatic injuries.
Treatment
Patients with diaphragmatic hernia are resuscitated in the usual fashion. A nasogastric tube is routinely
inserted as part of the initial assessment; however, resistance may be encountered if there is distortion
of the esophagogastric junction. Placement of the tube in the distal esophagus will often evacuate air
from the stomach; however, forceful attempts to pass the tube should be avoided. If there has been
visceral herniation with resultant respiratory compromise, it may be necessary to intubate the patient.
Caution must be exercised if there is an associated pneumothorax or hemothorax as chest tube
insertion may cause further injury to the herniated abdominal viscus.
Acute injuries are best approached through an abdominal incision. This affords the advantage of being
able to assess and treat any associated abdominal injury. Most chest injuries can be managed
nonoperatively, thus eliminating the need for two operations. Thoracotomy is reserved for chronic
hernias, where adhesions between the herniated organs and the lung are difficult to take down from an
abdominal approach and associated injuries are not a concern.
The patient is placed on the operating table in the supine position. Pneumatic compression stockings
are used for prophylaxis of thromboembolism if permitted by the patient’s condition. A wide surgical
prep extending from the proximal thighs to the neck is performed. The chest should always be included
in the surgical field in case urgent anterolateral thoracotomy or tube thoracostomy is required. The
patient is draped before induction of anesthesia, which will allow rapid access to the abdomen should
rapid deterioration occur.
The abdomen is entered through a midline incision from the xiphoid process to just below the
umbilicus. If present, intraperitoneal blood and clot are rapidly evacuated, and packs are placed to
control ongoing hemorrhage. The abdomen is explored in a systematic and thorough fashion. Blood
loss is controlled and gross soilage is limited by closing any hollow organ perforation. The diaphragm
is carefully evaluated by both visual inspection and manual palpation.
Herniated viscera are reduced from the thoracic cavity by gentle traction. When resistance is met,
careful passage of a small nasogastric tube alongside the herniated organs into the chest will often
release a vacuum, facilitating reduction. Extending the phrenotomy a short distance allows reduction
when other maneuvers are unsuccessful. Lateral extension for central ruptures and anterior

13
phrenotomy for medial and parahiatal defects avoid the phrenic nerve branches, minimizing
postoperative diaphragmatic dysfunction.
The pleural space is thoroughly irrigated with warm saline to remove retained blood and clot. Several
liters are required in the presence of a perforated viscus. The diaphragmatic defect is closed using
interrupted figure-of-eight or horizontal mattress sutures of 2-0 polypropylene. The tail of the
previously placed suture is used as a handle to provide exposure, permitting careful suture placement
during repair of the posterior portion of the defect. Teflon pledgets are occasionally used when the
diaphragm is attenuated or the closure is tenuous. Some authors recommend a two-layer closure for
defects greater than 2 cm. The inner layer is an interlocking horizontal mattress that everts the edges of
the diaphragm. This is reinforced with a running 3-0 polypropylene or similar type of nonabsorbable
suture.
Chronic Herniation
A small defect in the diaphragm may not produce symptoms and therefore may not be recognized at
the time of injury. On occasion visceral herniation may occur early, but the patient will remain
asymptomatic for varying periods of time. Symptoms may be vague and range from nonspecific
pleuritic or chest pain to frank sepsis resulting from free perforation into the thoracic cavity. The
diagnostic workup is similar to that for patients who present with acute injury.
Patients who are operated on more than a week or two after the acute injury are best approached
through the thoracic route. The lack of a peritoneal hernia sac allows the bowel to adhere to the lung,
thus making reduction through a celiotomy difficult. Solid organs may also become adherent to the
lung, thus requiring tedious and meticulous dissection. Once reduction is accomplished the diaphragm
is repaired using the same techniques as described for patients with an acute injury. Delayed
recognition, especially when associated with incarceration or strangulation, is associated with
significant mortality.

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