Hernia Diafragmatica

of the intestines were contained in that cavity except part of the
colon which was distended with meconium. Before I proceeded

further with the dissection I sent to acquaint my ingenious friend,
Dr. Hunter. We together dissected and examined this curious sub-
ject: and at the same time committed to writing the most remark-
able appearances.
When the sternum was raised, the stomach with the
greatest part of the intestines, with the spleen, and part of the
pancreas were found in the left cavity of the thorax; having been
protruded through a discontinuation, or rather an aperture of
the diaphragm, about an inch from the natural passage of the
esophagus.
From the extraordinary bulk of the parts contained in the
left side of the thorax, the mediastinum, the heart, the esopha-
gus, and the descending aorta were forced a considerable way
to the right side of the thorax; because there was not the least
mark of rupture or inflammation about the edges of the chasm:
and because it is probable that the diminished size of the left
lobes of the lungs, and the heart and mediastinum being pushed
to the right side, were gradually affected by the bulk and increase
of the viscera.
As the esophagus was pushed to the right side by the stomach
and the bowels, in the cavity of the thorax it kept the same course
and pierced the diaphragm not in the usual place, but consider-
ably further to the right side: and the aperture through which
CHAPTER 63 it passed was backwards and to the right side with respect to
that for the vena cava.
I have preserved the heart and lungs to show the dispropor-
tioned sizes of the lobes. And I have dried and prepared the
diaphragm with its connections to the vertebrae and sternum to
Congenital show the preternatural aperture through which the bowels passed
into the thorax; as also the passage of the esophagus to the right
side of the diaphragm. These preparations were at the same time
Diaphragmatic shown to the Society.
Cooper,2 in 1827, and Laennec,3 in 1834, not only
Hernia and reported clinical descriptions and gross pathology of CDH
but also suggested that laparotomy might be the proper ap-
proach for reduction and correction of the hernia. Bowditch,4
Eventration in 1847, was the first to make the bedside diagnosis of CDH
and further emphasized the clinical criteria for diagnosis.
Although Bochdalek’s understanding of the embryology
Charles J. H. Stolar and Peter W. Dillon was incorrect, this congenital defect continues to carry his
name.5 He speculated that the hernia resulted from a pos-
terolateral rupture of the membrane separating the pleuro-
peritoneal canal into two cavities. He also incorrectly
speculated that the best way to repair the defect was through
History
------------------------------------------------------------------------------------------------------------------------------------------------
the bed of the 12th rib. The record is not clear as to whether
this was actually attempted. The earliest, although unsuccess-
The earliest English language description of the gross anatomy ful, efforts to repair CDHs were by Nauman,6 in 1888, and
and pathophysiology associated with congenital diaphrag- O’Dwyer, in 1890.7 The first reports of successful repairs were
matic hernia (CDH) in a newborn was by McCauley, an asso- in an adult by Aue,8 in 1901, and a child by Heidenhain,9
ciate of Hunter, as reported in the Proceedings of the Royal in 1905.
College of Physicans, 17541: The groundwork for treating CDH in the newborn period
The child was born in the lying-in-hospital in Brownlow Street on was laid by Hedblom,10 whose review of the reported cases
the 24th of August, 1752: and was a fully grown boy, remarkably as of 1925 showed that 75% of 44 infants diagnosed in the
fat and fleshy. He was the fifth child of a healthy young woman newborn period died. He suggested that earlier intervention
who was well during her pregnancy. The child, when first born, might improve survival. Successful repair of CDH remained
started and shuddered; so that the nurse apprehended his going
rare until 1940, when Ladd and Gross11 reported 9 of 16
into fits. He breathed also with difficulty and it was some time be-
fore he could cry; which when he did, there was something pecu- patients surviving operative repair, the youngest being
liar in its note. He seemed to revive a little in about half an hour 40 hours old. It was not until 1946 that Gross12 reported
and breathed more freely: but soon relapsed and died before he survival of the first infant younger than 24 hours old after
was quite an hour and a half old. Being informed of these partic- operative repair of the defect. Until the 1980s, the standard
ulars by the mother, the matron, and the nurse, I was desirous of of care remained immediate neonatal surgery followed by
examining the body.. . .? I laid open the abdomen and found none postoperative resuscitative therapy (Fig. 63-1).
809
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810 PART VI THORAX
reported.13,27–31 The expected recurrence risk in a first-degree

relative has been estimated to be 1 in 45, or approximately
2%.30 Structural chromosomal abnormalities have been
identified in 9% to 34% of CDH infants and include triso-
mies, deletions, and translocations.32 Specific chromosomes
with deleted or translocated genes may be candidate loci
for CDH development.33,34 The combination of CDH and
an abnormal karyotype has been associated with a poor
outcome.32,35,36
The cause of CDH is unknown. As with other embryopa-
thies, there is increasing evidence that CDH may be due to
the exposure of genetically predisposed or susceptible indi-
viduals to environmental factors. Exposure to a number of
pharmacologic agents and environmental hazards has been
implicated in its development. These include insecticides
and drugs, such as phenmetrazine, thalidomide, quinine,
cadmium, lead, and nitrofen.37–40 The clinical findings of
vitamin A deficiency in CDH infants and the effects of vitamin
A administration in nitrofen-induced pulmonary hypoplasia
have strengthened the evolving hypothesis that alterations
in retinoid-regulated target genes may be responsible for
CDH development.41
Associated Anomalies
------------------------------------------------------------------------------------------------------------------------------------------------
Any newborn with a major congenital anomaly, including

infants with CDH, has an increased incidence of an additional
malformation compared with the general population. Al-
though previously thought to be low, the incidence of associ-
ated malformations in infants with a CDH ranges from 10% to
50%.42–46 Skeletal defects have been noted in as many as 32%
of CDH infants and include limb reduction and costovertebral
FIGURE 63-1 Chest radiograph of an infant with a right-sided congenital
diaphragmatic hernia demonstrating air-filled loops of intestine in the defects.44,45,47 Cardiac anomalies have been found in 24% of
right hemithorax with contralateral displacement of the mediastinum. infants.48 Cardiac hypoplasia involving the left ventricle and
The infant has been cannulated for venoarterial extracorporeal membrane often associated with hypoplasia of the aortic arch is fre-
oxygenation. quently described and can be confused with hypoplastic heart
syndromes. However, the clinical significance is limited. Most
cardiovascular malformations involve the cardiac outflow
Epidemiology and Genetics tract, such as ventricular septal defects, tetralogy of Fallot,
------------------------------------------------------------------------------------------------------------------------------------------------
transposition of the great vessels, double outlet right ventricle,
The reported incidence of CDH is estimated to be between 1 in and aortic coarctation.25,28,48–51 Anatomic anomalies of the
2000 to 5000 births. In the United States, approximately 1000 tracheobronchial tree have been found in 18% of patients with
infants per year are affected with this condition, and in CDH and include congenital tracheal stenosis, tracheal bron-
a recent study from Atlanta the birth prevalence was found chus, and trifurcated trachea.45 The incidence of associated
to be 2.4 per 10,000 births.17 The incidence in stillborns malformations in stillborn infants with CDH is even higher.
is less well documented. Approximately one third of infants In one study, 100% of stillborn infants with CDH had asso-
with CDH are stillborn, but these deaths are usually the ciated lethal anomalies.16,22 Abnormalities noted in this still-
result of associated fatal congenital anomalies.18–20 When born group were predominantly neural tube defects and
stillborns are counted with live births, females appear to be included anencephaly, myelomeningocele, hydrocephalus,
more commonly afflicted than males.13,21,22 and encephaloceles. Even in infants who survive to birth
Defects are more common on the left side, with approxi- but die shortly thereafter, neural tube defects were the most
mately 80% being left sided and 20% right sided. Bilateral common malformations noted. Cardiac defects were the sec-
CDH defects are rare and have a high incidence of associated ond most common group and included ventriculoseptal
anomalies.103 Infants with isolated CDH are more likely to be defects, vascular rings, and coarctation of the aorta.51 Other
premature, macrosomic, and male; and about one third of midline developmental anomalies have also been reported
affected infants may have associated major defects.24,25 and include esophageal atresia, omphalocele, and cleft palate.
Women who are thin or underweight for their height may have A number of syndromes have a CDH as a pathologic finding.
an increased risk of having an infant with an isolated CDH.26 These include trisomy 21, 18, and 13, and syndromes such
CDH is thought to represent a sporadic developmental as Frey, Beckwith-Wiedemann, Goldenhar, Coffin-Siris, Fryns,
anomaly, although a number of familial cases have been Meacham, and Kabuki.23,35,50,53–55
CHAPTER 63 CONGENITAL DIAPHRAGMATIC HERNIA AND EVENTRATION 811
Embryology buds form from this diverticulum by the fourth week of ges-
------------------------------------------------------------------------------------------------------------------------------------------------ tation. At 6 weeks, these lung buds have further developed
DIAPHRAGMATIC DEVELOPMENT into defined lobar structures. The pseudoglandular phase of
lung development takes place during the 7th to 16th weeks
The embryologic development of the diaphragm remains of gestation and involves airway differentiation. It is during
incompletely understood and involves multiple, complex this period that all bronchial airways develop. From the
cellular and tissue interactions. The fully developed dia- 16th to the 24th weeks of gestation, fetal lung development
phragm is derived from four distinct components: (1) the enters the canalicular phase of growth. During this period,
anterior central tendon forms from the septum transversum, airspace development occurs, as crude alveolar air sacs begin
(2) the dorsolateral portions form from the pleuroperitoneal to take shape. Type 1 pneumocytes begin to differentiate, and
membranes, (3) the dorsal crura evolve from the esophageal the precursors of type 2 pneumocytes ultimately responsible
mesentery, and (4) the muscular portion of the diaphragm for surfactant production begin to appear. Gas exchange
develops from the thoracic intercostal muscle groups. The becomes functionally possible at this stage.
precursors of diaphragmatic structure begin to form during Continued maturation of the crude alveolar airspaces takes
the fourth week of gestation with the appearance of the peri- place during the saccular phase of development that extends
toneal fold from the lateral mesenchymal tissue. At the same from 24 weeks’ gestation to term. During this time period,
time, the septum transversum forms from the inferior portion there is continued remodeling of the airspace dimensions
of the pericardial cavity. The septum transversum serves to and a maturation of surfactant synthesis capabilities.201
separate the thoracic from the abdominal cavities and eventu- Mature, adult-like alveoli begin to appear shortly after
ally forms the central tendinous area of the fully developed birth.66,67 Extensive alveolar maturation and multiplication
diaphragm. It defines the rudimentary pleuroperitoneal canals then takes place from birth until approximately 8 years of
and allows for the establishment of mesenchymal tissue within age, with a 10-fold increase in the number of functioning
these canals that ultimately results in pulmonary parenchymal alveoli.68–71 Some investigators have proposed that alveolar
development.56 formation may be completed by 2 years of age.72
Closure of the pleuroperitoneal canals with the formation Pulmonary vascular development follows the stages of air-
of a pleuroperitoneal membrane occurs during the eighth way and alveolar growth and can be divided into two ana-
week of gestation. Several theories have been proposed to tomic units based on associated airway structure. The term
explain the formation of this membrane and the subsequent acinus describes the functional unit of the lung that includes
development of a diaphragmatic structure. Progressive growth the respiratory bronchioli, alveolar ducts, and alveoli—all
of the pleuroperitoneal membrane has been one mechanism structures that evolve during or after the canalicular phase
proposed for canal closure.57–59 Other researchers have pos- of lung development. Vascular development in this region
tulated that concurrent hepatic and adrenal organogenesis is proceeds concurrently with alveolar growth and multiplica-
crucial to this process.7a,59,60,71 The involvement of a posthe- tion. The preacinar structures include the trachea, major
patic mesenchymal plate in diaphragmatic formation has been bronchi, and lobar bronchi up to the terminal bronchioles.
proposed.61 Preacinar vascular development is completed by 16 weeks’
The pleuroperitoneal folds extend from the lateral body gestational age.73–76
wall and grow medially and ventrally until they fuse with It is now recognized that pulmonary development is
the septum transversum and dorsal mesentery of the esopha- marked by a series of programmed events regulated by master
gus during gestational week 6. Complete closure of the canal genes, such as the homeobox genes, nuclear transcription fac-
takes place during week 8 of gestation. Anatomically, the right tors, hormones, and growth factors. These processes involve
side closes before the left.56 Muscularization of the diaphragm genes regulating epithelial and endothelial interactions as well
appears to develop from the innermost muscle layer of the as temporal and spatial interactions of several hormones and
thoracic cavity, although it has been proposed that the posthe- growth factors. Early developmental transcription factors,
patic mesenchymal plate is a possible source of muscular tis- such as hepatocyte nuclear factor-3b and thyroid transcription
sue.60,61 Posterolaterally, at the junction of the lumbar and factor-1, regulate pulmonary development from the foregut
costal muscle groups, the fibrous lumbocostal trigone remains mesenchyme. Additional stimuli of pulmonary development
as a small remnant of the pleuroperitoneal membrane and involve the transforming growth factor-b pathway, Sonic
relies on the fusion of the two muscle groups in the final stages Hedgehog pathway, Notch-delta pathway, Wingless-Int path-
of development for its strength. Delay or failure of muscular way, and cytokine receptor pathways. Subsequent signal
fusion leaves this area weak, perhaps predisposing to hernia- transduction control of organogenesis includes the apoptotic
tion. Bochdalek first described this area of the posterolateral pathways, nuclear receptor pathways, and interleukin path-
diaphragm in 1848, and it is for this reason that the most ways. The angiopoietins and isoforms of vascular endothelial
common site for CDH bears his name. growth factor are involved in pulmonary angiogenesis and
vascular development.77,78 Hormones such as the glucocorti-
coids, thyroid hormone, and retinoic acid have been shown to
LUNG DEVELOPMENT
regulate several of the crucial cellular interactions required for
Fetal lung development is divided into five stages: embryonic, proper pulmonary organogenesis and differentiation.
pseudoglandular, canalicular, saccular, and alveolar.63 Embry- Very little is known about the alterations in gene expres-
onic lung development begins during the third week of sion, growth factor interactions, and hormone levels asso-
gestation as a derivative of the foregut and is marked by the ciated with airway and vascular development in the
formation of a diverticulum off of the caudal end of the laryn- hypoplastic CDH lung. A number of factors have been found
gotracheal groove.64 The trachea and the two primary lung to be elevated in CDH lung specimens, including epidermal
812 PART VI THORAX
growth factor, transforming growth factor-a, vascular ipsilateral thoracic cavity. The resulting abnormal position
endothelial growth factor, insulin-like growth factor, tumor of the bowel prevents its normal counterclockwise rotation
necrosis factor-a, angiopoietin-2, and glucocorticoid receptor and fixation. No hernia sac is present if the event occurs before
gene expression.41,77,79–83 Decreased expression of Sonic complete closure of the pleuroperitoneal canal, but a nonmus-
Hedgehog, heme-oxygenase-1, and endothelial nitric oxide cularized membrane forms a hernia sac in 10% to 15% of CDH
synthase levels has been found in CDH specimens.84–86 patients.100 Although some claim the herniation can occur late
Abnormal levels of factors that contribute to the regulation in gestation or be intermittently present as a dynamic process,
of pulmonary vascular tone have also been reported. High in most cases the defect is established by gestational week
plasma concentrations of endothelin-1, a powerful vasocon- 12.100a The subsequent postnatal problems relate to the effects
strictor, have been found in association with increased expres- of the herniated viscera on the developing heart and lungs.
sion of its pulmonary artery receptors in CDH infants.87,88 The classical left-sided CDH features a 2.0- to 4.0-cm
Advances in understanding potential mechanisms respon- posterolateral defect in the diaphragm through which the
sible for the alterations in pulmonary development associated abdominal viscera have been translocated into the hemithorax
with a congenital diaphragmatic hernia have been limited by (Fig. 63-2). Herniated contents often include the left lobe of
the lack of a completely acceptable experimental animal the liver, the spleen, and almost the entire gastrointestinal
model. Currently, three model systems exist for studying tract. The stomach is frequently in the chest, which results
pulmonary maldevelopment: a surgical model of a diaphrag- in some degree of obstruction at the gastroesophageal junc-
matic defect, a nitrofen model of pulmonary and diaphrag- tion. This obstruction, in turn, causes dilation and ectasia of
matic hypoplasia, and various gene knockout mice models the esophagus. Occasionally, the kidney may be in the chest
of pulmonary hypoplasia.89 Elegant models by de Lorimier tethered by the renal vessels. In instances of a right-sided
and Harrison, in which diaphragmatic defects were surgically defect, the large right lobe of the liver can occupy much of
created in fetal sheep, showed that long-term compression of the hemithorax in addition to other abdominal viscera. The
abdominal contents into the thoracic cavity resulted in pulmo- hepatic veins may drain ectopically into the right atrium,
nary maldevelopment and lung hypoplasia.90,91 The disad- and fibrous fusion between the liver and the lung has been
vantage of this surgical model is that the defect is created reported. Both of these anatomic findings can significantly
late in the time course of fetal development and may not complicate attempted surgical repair of the diaphragmatic
address early developmental mechanisms. The nitrofen model defect.89,102
of experimental CDH coincides with the theory that many The diaphragmatic defect usually features a completely
developmental defects, including CDH, are embryopathies open space between the chest and abdomen, although some
caused by toxin exposure. Nitrofen is an herbicide with infants have a membrane of parietal pleura and peritoneum
known teratogenic effects. Its administration to pregnant mice acting as a hernia sac. This finding is to be distinguished from
results in offspring with pulmonary hypoplasia, diaphrag-
matic defects, reduced airway branching, excessive muscular-
ization of pulmonary vessels, surfactant deficiency, and
respiratory failure at birth. The pulmonary hypoplasia result-
ing from nitrofen administration has been associated with
alterations in a number of growth factors and developmental
pathways in embryonic mice.92 Finally, investigations using
specific knockout mice have contributed to the understanding
of the role of various factors and pathways in pulmonary and
diaphragmatic development.89
A number of physical factors may also affect pulmonary
growth and development.63 Adequate intrathoracic space is
a prerequisite for normal pulmonary growth. Any intratho-
racic or extrathoracic process that results in a decrease of
the intrathoracic space and pulmonary parenchymal compres-
sion can lead to the development of structurally immature
lungs.90–96 Other physical factors important in lung growth
include the maintenance of normal fetal lung liquid and
amniotic fluid dynamics.93,95,98,99,101
Pathology
------------------------------------------------------------------------------------------------------------------------------------------------
Although the cause of CDH is uncertain, its consequences on

pulmonary development and function are well documented.
During the early development of the diaphragm, the midgut
is herniated into the yolk sac. If closure of the pleuroperitoneal
FIGURE 63-2 Schematic illustration of a left congenital diaphragmatic
canal has not occurred by the time the midgut returns to the hernia showing translocation of abdominal viscera through a posterolat-
abdomen during gestational weeks 9 and 10, the abdominal eral aperture into the chest. (From Spitz L, Coran AG [eds]: Rob & Smith’s
viscera herniate through the lumbocostal trigone into the Pediatric Surgery. London, Chapman & Hall, 1996.)
an eventration of the diaphragm, which results from phrenic but appears to be postnatally derived, perhaps as a result
nerve or anterior horn cell degeneration. The muscle fibers of of treatment or secondary to the pathology of pulmonary
the diaphragm are usually present. hypertension.28,113
Bilateral CDH is an unusual occurrence and is almost always Pulmonary blood flow accounts for only 7% of cardiac
fatal because of bilateral lung growth arrest (Fig. 63-3).103 output during normal fetal development, and pulmonary
Unilateral visceral herniation affects both ipsilateral and vascular resistance remains high. The fetus preferentially
contralateral pulmonary development, although hypoplasia shunts oxygenated blood from the placenta through the fora-
is predictably more severe on the ipsilateral side. This is con- men ovale and ductus arteriosus in a right-to-left direction
firmed by an analysis of lung volumes and weights in human into the systemic circulation. At birth, a number of hemody-
autopsy specimens and animal models.90,96,104,105 Because namic changes take place that dramatically alter this circula-
the process of CDH herniation occurs at the time of bronchial tory profile. With the institution of breathing, pulmonary
subdivision, it is at this stage that lung development becomes vascular resistance falls, as does pulmonary artery pressure
compromised. Although all major bronchial buds are present allowing for an increase in pulmonary blood flow. Systemic
in a CDH lung, the number of bronchial branches in the vascular resistance and left atrial pressure rise, causing the
affected lung is greatly reduced. This finding was noted in foramen ovale to close. Increased arterial oxygen tension in-
both ipsilateral and contralateral pulmonary specimens.104 duces spontaneous closure of the ductus arteriosus. Transition
Alveolar development is severely affected, and it has been from a fetal to an adult-type circulatory pattern is accom-
reported that few normal alveoli exist in the lungs at term.106 plished. Persistent fetal circulation may develop if this process
In addition, the changes in airway structure are quite variable. is interrupted. After birth and interruption of placental
Infants requiring low ventilatory assistance during treatment circulatory support, persistently elevated pulmonary vascular
had the same airway muscle mass as controls, whereas infants resistance results in increased pulmonary artery pressures and
with prolonged ventilatory support had significantly greater decreased pulmonary vascular blood flow. The increased vas-
muscle thickness throughout the conducting airways.107 cular resistance results in right-to-left shunting of blood at
The pulmonary vascular bed is distinctly abnormal in lungs either the atrial or the ductal levels with the delivery of unsat-
from patients with CDH. A reduction in the total number of urated blood into the systemic circulation. As the blood flow
arterial branches in both the ipsilateral and the contralateral in the shunt increases, the oxygen saturation in the systemic
pulmonary parenchyma has been reported.108,109 Structur- circulation falls and the mixed venous blood returning to
ally, significant adventitial and medial wall thickening has the right side of the heart becomes progressively desaturated.
been noted in pulmonary arteries of all sizes in CDH lungs The resulting hypoxia further increases pulmonary vascular
in association with abnormal muscularization of the small resistance and compromises pulmonary blood flow while
preacinar and intraacinar arterioles.110–112 The physiologic increasing the right-to-left shunt flow. Severe and progressive
consequence of this abnormal arterial muscularization may respiratory failure ensues.
be an increased susceptibility to the development of fixed Factors that contribute to the persistence of high pulmo-
and intractable pulmonary hypertension. No significant nary vascular resistance in CDH lungs are thought to be the
changes in pulmonary venous structure have been identified structural changes in decreased total arteriolar cross-sectional
resulting from CDH development. Increased adventitial area in the involved lungs and the increased muscularization
thickness of pulmonary veins has been noted in CDH infants of the arterial structures that are present. In the postnatal
period, there is failure of the normal arterial remodeling pro-
cess, further maintaining the abnormal vascular resistance
that may be only partly reversed by treatment interven-
tions.114 Additional exacerbations of pulmonary vascular
resistance may be induced by the known stimulators of
pulmonary hypertension, including hypoxia, acidosis, hypo-
thermia, and stress.115 Alterations in the levels of various
prostaglandins, leukotrienes, catecholamines, and the renin-
angiotensin system have been implicated as mediators of this
complex process.116–118 It can only be surmised at this time
whether there is an exaggerated response to these stimuli by
the abnormal vascular structures of CDH lungs.113
DIAGNOSIS
The diagnosis of a CDH is often made on a prenatal ultrasound
(US) examination and is accurate in 40% to 90% of cases.119,119a
Although considerable variation in detection rates have been
reported, the mean gestational age at discovery is 24 weeks
and has been reported as early as 11 weeks.120 The US may
be obtained for routine obstetric care or because of suspicion
concerning the presence of polyhydramnios. Polyhydramnios
FIGURE 63-3 Operative photograph of a left congenital diaphragmatic
hernia created in a fetal lamb. The posterolateral defect can be seen has been reported present in up to 80% of pregnancies with
looking from the abdomen into the chest. (From Spitz L, Coran AG associated CDH.99a The mechanism of polyhydramnios is
[eds]: Rob & Smith’s Pediatric Surgery. London, Chapman & Hall, 1996.) thought to be due to kinking of the gastroesophageal junction
814 PART VI THORAX
by translocation of the stomach into the thorax with resultant After birth, the spectrum of respiratory symptoms in an in-
foregut obstruction. The US diagnosis of a CDH is most often fant with a CDH is determined by the degree of pulmonary
suggested by observing the stomach in the fetal thorax at the hypoplasia and reactive pulmonary hypertension. The most
same cross-sectional level as the heart (Fig. 63-4). Additional severely affected infants develop respiratory distress at birth,
US findings suggestive of a CDH include the absence of the whereas a majority demonstrate respiratory symptoms within
stomach in the abdomen and the presence of the liver or other the first 24 hours of life. Classically, these infants have a scaph-
solid viscera in the thorax. The stomach may be small because of oid abdomen and an asymmetric distended chest. The chest
interference with fetal swallowing. If the diaphragmatic defect is may become more distended as swallowed air passes into
on the right side, the liver can tamponade the hernia site and the stomach and intestines. Gastrointestinal distention further
obscure the diagnosis. The diagnosis of CDH may be missed be- compresses pulmonary parenchyma and affects ventilatory
cause intermittent herniation of abdominal viscera into the tho- characteristics. It may lead to additional mediastinal compres-
racic cavity has been reported.99a Furthermore, when the sion with impairment of the contralateral lung. Because of the
stomach is in a normal abdominal position, herniated small small size of the neonate’s chest, breath sounds may or may not
bowel loops are not easily distinguishable from lung paren- be present on the side of the defect. Mediastinal compression
chyma. The misinterpretation of the fetal US scan can be caused with shift into the contralateral thorax may cause deviation of
by other diagnoses, such as esophageal atresia and cystic lung the trachea away from the side of the hernia and also result in
anomalies. Functional information concerning fetal breathing obstruction to venous return with the hemodynamic conse-
can be obtained by duplex Doppler examination of amniotic quences of hypotension and inadequate peripheral perfusion.
flow at the fetal nares at the time of fetal US. A fetal tidal vol- The signs of respiratory distress may include cyanosis, gasp-
ume/minute ventilation can be determined that may have a bear- ing, sternal retractions, and poor respiratory effort. In babies
ing on prognosis.121 with a left CDH, heart sounds will be heard best over the right
In addition to diagnosis, prenatal US may also be of benefit chest; “dextrocardia” accompanied by respiratory distress is a
in predicting outcome by using quantitative techniques to CDH until proven otherwise.
estimate the severity of pulmonary hypoplasia of the fetal The diagnosis of a CDH can be confirmed by a plain chest
CDH lung. Three-dimensional estimation of the fetal lung vol- radiograph that demonstrates loops of intestine in the chest.
ume, calculation of the right lung area to thoracic area ratio, The location of the gastric bubble should also be noted, and
and calculation of the lung to thoracic circumference ratio its position can be confirmed by placement of an orogastric
are three different measurements that may correlate with neo- tube. Rarely, a contrast study of the upper gastrointestinal tract
natal outcome,122–125 but the lung-to-head ratio has been the is required. The chest radiograph shows angulation of the me-
most widely used prognostic indicator.126 US can be limited diastinum and a shifting of the cardiac silhouette into the con-
by the poor acoustic contrast between fetal lung and herniated tralateral thorax. Although minimal aeration of the ipsilateral
viscera, position of the fetus, and operator experience. As a re- parenchyma may be noted, chest radiographs are unreliable
sult, prenatal magnetic resonance imaging (MRI) evaluation is for estimating the degree of pulmonary hypoplasia.58,133–135
being used with increasing frequency when obstetric sonogra- Once the diagnosis of a CDH is confirmed, additional
phy has detected a complex fetal anomaly and is ideally suited radiographic and US examinations should be carried out to
for fetuses with a diaphragmatic hernia.127–129 MRI can read- search for associated anomalies. Echocardiography and both
ily determine liver position in relation to the diaphragm and renal and cranial US scans should be obtained.
detect herniated liver into either hemithorax. It may also be Although most CDHs present in the first 24 hours of life,
used to more accurately assess lung volume and perhaps cor- 10% to 20% of the infants with this defect present later.136,137
relation with outcome.130–132 These latter infants present with recurrent mild respiratory
A B
FIGURE 63-4 A, Ultrasound examination of a 28-week gestation fetus (twin B) in cross section demonstrating the fetal heart (FH) and stomach (ST) in the
same plane. B, Ultrasound examination of the same fetus but in a sagittal plane, demonstrating relationship of the stomach, liver, and heart.
illnesses, chronic pulmonary disease, pneumonia, effusion, diagnosis had a 96% survival (this drops to 86% when the
empyema, or gastric volvulus. Occasionally, neonatal strepto- babies who died in transport are included). Preselection bias
coccal pneumonia may mask a delayed-onset right CDH.138 prior to transport was not reported.
Antenatal diagnosis and antenatal referral to a full-service
children’s facility, especially those with maternal/fetal–
Differential Diagnosis medicine/lying-in services, has removed much of the postna-
------------------------------------------------------------------------------------------------------------------------------------------------
tal/pretransfer bias and selection. It was also reported that
The diagnosis of a CDH can be confused with a number of the presence of polyhydramnios was indicative of poor sur-
other congenital thoracic conditions, including eventration vival.141 A number of studies, however, have refuted this
of the diaphragm, anterior diaphragmatic hernia of Morgagni, observation and have shown that the presence of polyhy-
congenital esophageal hiatal hernia, congenital cystic disease dramnios has no predictive value on the eventual outcome
of the lung, and primary agenesis of the lung. Diaphragmatic of an infant with CDH.142,143 The anatomic position of the
eventration has many causes but is seen in the newborn with liver has not been consistently shown to be a reliable predictor
birth trauma or Werdnig-Hoffmann disease. The eventrated of mortality in CDH.120,126,131,144–146 Nevertheless, there are
diaphragm can rise as high as the third intercostal space reports suggesting the presence of liver herniation may be pre-
and have the same physiologic consequences as CDH. It dictive of the need for extracorporeal membrane oxygenation
can also be completely asymptomatic. The diagnosis is made (ECMO) as well as a requirement for prosthetic patch repair
by fluoroscopy or real-time US with the demonstration of for diaphragmatic repair.15,126,147
paradoxic movement of the diaphragm. MRI is also useful The position of the stomach has been proposed as a prog-
in determining diaphragmatic structure. Morgagni hernias nostic indicator by a number of investigators. Survival rates of
occur at the hiatus for the internal mammary arteries and infants with CDH with the stomach properly located below
are much less common than Bochdalek hernias. Most are the diaphragm at the time of diagnosis have been reported
diagnosed incidentally on plain radiographs, but some to be as high as 100% but is only 30% when the stomach
Morgagni hernias can present as a gastrointestinal crisis had herniated into the chest.148,149 Other studies have shown
because of incarceration or volvulus of the colon or small no predictive value of such positioning.150 The side of the di-
bowel and require immediate operative intervention. aphragmatic defect may be somewhat predictive of outcome.
It has been reported that patients with right-sided defects have
a worse prognosis than those with left-sided defects.14,151
Prognostic Factors A recent study reported no differences in outcomes between
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the two sides.152 However, right-sided defects may not
The search to determine clinically relevant prognostic factors become evident in the newborn period and may present with
that predict the outcome of infants with CDH has been frus- very mild respiratory symptoms at a later age.153
tratingly complex, contradictory, and for the most part unsuc- A number of different imaging parameters have been
cessful. Many studies have attempted to examine both anatomic reported in an attempt to predict the presence of pulmonary
and physiologic parameters that relate to survival, but each hypoplasia and serve as a prognostic indicator of survival. These
has been hampered by its retrospective analysis in the presence include measurements of the fetal thorax and lung, fetal breath-
of the continuing evolution of new therapies.139,139a Whereas ing movements, and various calculated blood flow measure-
consideration of multiple factors may influence one’s clinical ments within the pulmonary arteries.154 The lung–head ratio
impression regarding the survival potential of an infant with (LHR) has been the most extensively studied. The LHR is mea-
CDH, such an impression cannot be derived from one measure- sured using ultrasonography and is the ratio of the area of
ment alone. the contralateral lung (opposite the hernia defect) to the fetal
head circumference. The lung area is calculated from perpen-
dicular transverse measurements determined at the plane of
the four-chamber view of the fetal heart. This measurement
ANATOMIC FACTORS
has been suggested to predict prognosis and guide manage-
With the ability to establish the diagnosis of CDH in utero as a ment.126,155,156 However, there is no consensus as to what
result of the increased use of prenatal US, studies suggested value of LHR should serve as a determinant of prognosis or
that the antenatal diagnosis of a CDH before 24 weeks’ gesta- when during gestation that measurement should be made.157
tional age was associated with a high mortality. Others have Because LHR has been proposed to determine the timing of
shown that antenatal diagnosis, regardless of timing, of an experimental fetal intervention, most studies report LHR mea-
isolated CDH without other associated anomalies is not an surements before 32 weeks’ gestational age.158 Current data in-
indicator of outcome,140 but more recent reports confirm that dicate that the strength of association with survival is strongest
antenatal diagnosis is associated with a worse prognosis. for those fetuses with LHR greater than 1.0 compared with LHR
A CDH associated with another significant anomaly still has less than 1.0.159 A recent report by Aspelund and colleagues of
a dismal prognosis. If a CDH is not detected by prenatal US a single-institution experience of all inborn CDH infants man-
but is subsequently diagnosed after birth, survival rates may aged with a consistent treatment algorithm had significant sta-
be excellent.122 The Canadian Pediatric Surgery Network tistical power to support an LHR less than 0.85 as reliably
(CAPSNet) collected 347 CDH cases between May 2005 and predicting 100% mortality.160
May 2010 (www.capsnetwork.org/resources/annual report Because LHR changes with gestational age, use of the
2010); of the 269 patients admitted alive to a pediatric center observed-to-expected LHR (O/E-LHR) has been reported.158,161
and with a completed record, 68% had a prenatal diagnosis, A severe left CDH has been characterized by an O/E-LHR of
with a 74% survival, while the 32% without a prenatal less than 25%.
816 PART VI THORAX
Prenatal magnetic resonance imaging is being investigated offered. This represented less than 5% of all patients, for
extensively as a method for improving prognostic predictive which there was 100% mortality.
capability by more accurately determining lung volumes in Since these initial reports, investigators have derived
CDH patients.162 Measured indices include lung to head ratio a number of formulas using various blood gas components to
as well as relative and absolute fetal lung volumes. The prog- predict outcome. The most basic concept is the alveolar–
nostic accuracy appears to be slightly better than sonographic arterial oxygen gradient (AaDO2). It is calculated by the formula
determinations.146 Signal intensity analysis of fetal lung tissue AaDO2 ¼ ½fð713 FiO2 Þ PaCO2 g=0:8 PaO2
as part of MRI imaging studies may be a future application of
this modality, although a recent report found that lung Although initially used to determine entry criteria for
volumes was a better predictor.162,163 ECMO, its use has been superseded by the development of
The sophisticated analysis of cardiopulmonary structure other indices.
and function in either the prenatal or postnatal period may Using blood gas analysis and PCO2 levels in combination
also be of prognostic importance.142,164–166 A number of with ventilatory data, parameters were determined to predict
indices have been reported, including the calculation of the outcome in CDH infants managed with conventional ventila-
cardioventricular index (left ventricle/right ventricle), the tory techniques.110,180–182 To do this, a ventilatory index (VI)
cardiovascular index (Ao/PA), pulmonary artery diameters, was calculated:
the McGoon index (MGI), and pulmonary artery index VI ¼ RR ðPIP PEEPÞ
(PAI).166–170 The McGoon and pulmonary artery indices are
determined shortly after birth by the following formula: (PEEP ¼ positive end-respiratory pressure; PIP ¼ peak
MGI ¼ ðRPA diameter þ LPA diameterÞ= inspiratory pressure; RR ¼ respiratory rate)
When the PCO2 could be reduced to less than 40 mm Hg with
Descending aorta diameter
a ventilatory index less than 1000, all patients survived. A mod-
PAI ¼ ðRPA area þ LPA areaÞ=BSA ified ventilatory index (MVI) was calculated by using peak
(RPA and LPA ¼ right and left pulmonary artery, respectively; inspiratory pressure rather than mean airway pressure (MAP):
BSA ¼ body surface area) MVI ¼ ðRR PIP PaCO2 Þ=1000
MGI scores less than 1.31 have been found to be highly
predictive of mortality, while the same has been found for In infants with an MVI less than 40, the survival rate was
PAI scores less than 90. An analysis of left ventricular mass 96% using conventional therapy. All infants died if the MVI
combined with the simultaneous determination of fractional was greater than 80.183
shortening has also been used to predict outcome, with an The most commonly used calculation is the oxygenation
index of 1.2 associated with nonsurvival.171 Advanced echo- index (OI). It is calculated by the formula
cardiographic measurements with interesting clinical promise
OI ¼ ðMAP FiO2 100=PaO2 Þ
include the determination of lung tissue perfusion with the
analysis of fractional moving blood volume (FMBV) and the This formula should be calculated using the preductal PaO2,
use of pulsed Doppler measurements in the proximal branch because the postductal one varies tremendously with the
of the pulmonary artery to determine the pulsatility index (PI) amount of shunting.
and the peak end-diastolic reversed flow (PEDRF).172–174 A 1994 report showed that with conventional ventilatory
These measurements may have increasing importance in therapy, an OI less than 6 had a survival rate of 98%, whereas
the search for suitable prenatal predictors of survivability in an OI greater than 17.5 had no survivors.183 The predictive
the setting of fetal therapy.175 powers of these factors with such therapies as ECMO, high-
frequency oscillation (HFO), surfactant, and nitric oxide
(NO) have not been determined. Simple preoperative lung
function measurements are difficult to obtain but may be of
PHYSIOLOGIC PARAMETERS
some interest.184
Unfortunately, there are few physiologic parameters that can In summary, efforts to reliably predict mortality for the fetus
be measured in the neonate to assess pulmonary function or live-born infant with isolated CDH have been fraught with
other than PO2, PCO2, and pH. Thus arterial blood gas analysis uncertainty. Although a calculated LHR less than 0.85 (or an
has been the cornerstone for attempting to establish clinical observed/expected LHR less than 20% to 25%) may have high
predictive criteria. Early studies showed differences in pH predictability for mortality, better understanding of prognosis
and PCO2 between survivors and nonsurvivors in response awaits functional evaluation of the fetus. This might include
to therapeutic interventions available at that time.176–178 tidal amniotic fluid breathing/lung volumes or response to
Infants with a low PCO2 and a PO2 that was initially normal maternal hyperoxia. Postnatal use of the McGoon index, also be-
or improved with mechanical ventilation had an excellent cause of its functional nature, holds promise.184a In the absence
outcome, whereas those infants who had high PCO2 levels of substantiated, reproducible information regarding prognosis,
unresponsive to mechanical ventilation did poorly. These au- treatment continues to be guided by best clinical judgment.
thors noted the importance of measuring both preductal and
postductal blood gases to assess the degree of right-to-left
PULMONARY FUNCTION TESTS
shunting. The report by Stolar and colleagues179 advocated
preductal oximetry values in the setting of maximal con- The analysis of preoperative and postoperative pulmonary
ventional care to identify potential ECMO candidates. In the function tests has been reported to have predictive value in
absence of preductal SaO2 greater than 90%, overwhelming identifying infants who might require ECMO therapy as well
pulmonary hypoplasia was inferred, and ECMO was not as identifying survivors.184 Initial studies of respiratory
function in infants with CDH uncovered the detrimental treatments.193,196 Tracheal occlusion resulted in lung enlarge-
changes in compliance measurements resulting from surgical ment but did not reverse the pathologic process associated
repair and helped support the hypothesis of medical stabi- with pulmonary hypoplasia. The selection criteria for the se-
lization and delayed surgical intervention.185–187 Using the verity of pulmonary hypoplasia to enter the fetal treatment tri-
treatment strategies of delayed surgical repair and ECMO, als has also been questioned.93,94,193,197–199 The North
when necessary, infants did not require ECMO when their American trial used an LHR less than 1.4 as entry criterion;
initial preoperative compliance measurement was greater than the Eurofetus trials have advocated LHR less than 1.0 as entry
0.25 mL/cm H2O/kg and initial tidal volume was greater criterion. Fetoscopic tracheal occlusion trials are ongoing in
than 3.5 mL/kg. An improvement in the tidal volume of Europe, with over 200 cases reported,198 and there are plans
4 mL/kg after repair correlated with survival.188 for an international prospective randomized trial involving
Studies have indicated that preoperative measurement of North American centers that participate in NAFTNet (the
functional residual capacity may predict fatal pulmonary North American Fetal Treatment Network).
hypoplasia.184 In addition, serial measurements of total Although prenatal corticosteroids are used to enhance lung
pulmonary compliance have been found useful in predicting development in premature infants, the role of antenatal corti-
outcome in high-risk infants.189 costeroid therapy in CDH patients remains undetermined.
Although no single parameter has proven sufficient as a The rationale for such therapy to induce pulmonary matura-
prognostic factor in managing CDH infants, recent multicen- tion in a hypoplastic lung is based on animal studies and
ter studies have shown that significant independent predictors isolated case reports.200–203 Balanced against these observa-
of total mortality include prenatal diagnosis, birth weight, tions is the growing evidence from premature infant studies
low 1- and 5-minute Apgar scores, score for neonatal acute that such drugs may also have adverse perinatal and long-
physiology (SNAP-II), and right-sided defect.139,190–192 term effects.204,205 The true potential of this therapy in im-
proving CDH outcomes awaits the results of a randomized
prospective study.
Treatment
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Success in the management of CDH has improved dramati-

cally from 1929 when Greenwald and Steiner27 wrote, “For PREOPERATIVE CARE
the patient in whom the hernia makes its appearance at birth,
little or nothing can be done from a surgical standpoint.” Resuscitation
A number of innovative treatment strategies have been used, After the birth of the infant and confirmation of the diagnosis
although consistent impact on overall survival is still difficult of CDH, all efforts should be made to stabilize the cardiorespi-
to obtain. ratory system while inducing minimal iatrogenic injury from
therapeutic interventions. It is essential to consider that the
CDH is a physiologic emergency and not a surgical emergency.
PRENATAL CARE
The respiratory distress associated with a CDH in the new-
The diagnosis of a CDH is being made with increasing born results from a combination of two factors previously
frequency by prenatal US examination. This study may be discussed: uncorrectable pulmonary hypoplasia and poten-
initiated when a discrepancy between size and dates is noted. tially reversible pulmonary hypertension. The balance be-
The prenatal diagnosis of CDH should be complemented by a tween these two factors determines the response to therapy
careful search for other congenital anomalies, particularly and ultimately the outcome. Clinically, both are manifested
those affecting the cardiovascular and nervous systems. by an increase in pulmonary vascular resistance and elevated
Evaluation of fetal karyotype should be accomplished by pulmonary artery pressures, right-to-left shunting at the duc-
amniocentesis or chorionic villus or fetal blood sampling. tal and foramen levels, and progressive hypoxemia. Because
Currently, the standard of care is to support the fetus and there are no proven therapies to promote pulmonary growth
mother while bringing them to delivery as close as possible at this time, therapeutic interventions are aimed at governing
to term. The advantage of prenatal diagnosis is in being pulmonary vascular tone.
able to properly prepare and inform the parents about pos- Resuscitation begins with endotracheal intubation and
sible treatments and outcomes. The fetus and mother nasogastric tube insertion. Ventilation by mask and Ambu
should be referred to an appropriate tertiary perinatal center bag is contraindicated to avoid distention of the stomach
where the full array of respiratory care strategies, including and intestines that may be in the thoracic cavity. Arterial
NO, oscillating ventilators, and ECMO are immediately and venous access should be acquired through the umbilicus.
available. Anything less may potentially compromise the If the umbilical venous catheter can be passed across the liver
best possible outcome.143 Spontaneous vaginal delivery is into the right atrium, it can be useful for monitoring central
preferred, unless obstetric issues supervene. The mere diag- venous pressures as well as obtaining mixed venous blood
nosis of a CDH is not an indication for elective cesarean gas samples. Although the umbilical artery is excellent for
section. monitoring systemic blood pressure and obtaining postductal
At this time, fetal intervention with attempted in utero arterial blood gas specimens, additional information can be
correction of the defect is investigational and highly experi- obtained by monitoring arterial oxygen saturation in a pre-
mental.105,193–195 In North America, trials of fetal tracheal ductal position either with a right radial arterial catheter or
occlusion for CDH in an effort to promote antenatal lung a transcutaneous saturation probe. An important part of the
growth have been abandoned, because they did not show treatment algorithm is an attempted estimation as to whether
improved survival rates versus contemporary conventional the infant has enough lung capacity for meaningful gas exchange.
818 PART VI THORAX
It is important to consider this fact before exposing an infant

and family to heroic treatment strategies. Pharmacology
As in any neonatal resuscitation, meticulous attention must A broad spectrum of drugs and antihypertensive agents has
be paid to maintaining proper temperature regulation, glucose been used in attempts to modify the pulmonary vascular
homeostasis, and volume status in the neonate in an effort to resistance in infants with CDH and respiratory failure. Expe-
maintain adequate oxygen delivery. Any stressful stimulus can rience has been extrapolated from clinical trials of infants
further exacerbate already elevated pulmonary pressures and with persistent pulmonary hypertension of the newborn
lead to increased shunt flow and further systemic desatura- (PPHN) and other forms of neonatal respiratory failure.
tion. Infants should be properly sedated, and any combination In the past, agents such as tolazoline,211,212 which exerts its
of agents, including midazolam, fentanyl, or morphine, can be effects through a-receptor blockade, had been used to lower
used. Muscle paralysis is strongly discouraged because of its pulmonary vascular resistance in the face of hypoxemia and
untoward consequences on ventilatory mechanics and poten- respiratory failure.212,213 Its efficacy in CDH infants was
tial morbidity. Infants not “cooperating” with ventilator strat- marginal. Other drugs, such as nitroprusside, isoproterenol,
egies generally need attention to their discomfort, not muscle nitroglycerin, and captopril, have not been effective.214 The
paralysis. Systemic hypotension and inadequate tissue perfu- administration of various prostaglandin derivatives, including
sion may be observed and reversed with intravenous fluid prostaglandin D2 (PGD2), prostaglandin E1 (PGE1), and pros-
administration, including crystalloid, blood products, and tacyclin, and of the cyclooxygenase inhibitor indomethacin
colloid. Cardiotonic drugs, such as dopamine or dobutamine, has also been disappointing.115,215
may be required. Because of the unstable pulmonary vascular New management strategies for treating persistent pulmo-
tone and the compromised alveoli, excessive intravenous nary hypertension now undergoing clinical evaluation include
hydration should be avoided, because it may lead to pul- various calcium channel blockers, prostacyclin derivatives,
monary edema, loss of compliance, and further impairment endothelin receptor antagonists, and phosphodiesterase-5
of gas exchange. inhibitors such as sildenafil.216,217
Metabolic acid-base disturbances are usually related to
hypoperfusion and should be corrected by fluid manage- Surfactant
ment or bicarbonate administration. Metabolic acidosis can Animal models have demonstrated that experimentally
be reversed with bicarbonate administration if ventilation induced CDH lungs are surfactant deficient,218 but such re-
can be appropriately managed. Severe hypercapnia (PCO2 > sults have not been replicated in human studies. Early reports
70 mm Hg) should be managed by changing ventilator strategy. in infants with CDH demonstrated alterations in surfactant
There is no need for a chest tube in the absence of an active levels and composition.219,220 However, recent studies have
air leak, pneumothorax, or hemothorax.206,207 indicated that the surfactant pool in infants with CDH is no
different than control patients, even in infants requiring
ECMO support.221–224 There may be alterations in synthetic
Ventilation and metabolic kinetics for individual components.221 In terms
The type of mechanical ventilator needed for the infant with a of improving respiratory function and outcomes, clinical and
CDH is a matter of personal and institutional preference. Most experimental investigations with surfactant administration
infants can be successfully managed with a simple pressure- have been mixed.224–228 A multicenter review of surfactant
cycle ventilator, using a combination of high rates (100 administration in CDH patients showed no overall benefit
breaths per minute) and modest peak airway pressures (18 to its use and demonstrated a lower survival rate in preterm
to 22 cm H2O and no PEEP) or lower rates (20 to 40 breaths infants compared with full-term infants.229 At this time, there
per minute) and higher pressures (22 to 35 cm H2O, 3 to 5 cm are no clinical data to support the administration of surfactant
PEEP). The goal of such ventilatory support is to maintain in the management of CDH infants.
minute ventilation while obtaining a preductal PO2 greater
Nitric Oxide
than 60 mm Hg (SaO2 90% to 100%) with a corresponding
PCO2 of less than 60 mm Hg. pH and PCO2 levels have been NO is a potent mediator of vasodilatation and was originally
shown to be important in modifying pulmonary vascular identified as endothelial-derived relaxing factor.230 Because
tone.307b The successful clinical manipulation of these param- it is a highly diffusible gas that is rapidly inactivated by bind-
eters in therapeutic interventions in neonates with persistent ing to hemoglobin, it is particularly suited for administration
pulmonary hypertension represents an initial treatment to the pulmonary vasculature with mechanical ventilatory
strategy. It is now clear, however, that the extremes of hyper- techniques. In clinical studies, NO was effective in improving
ventilation with induced alkalosis should be avoided because oxygen saturation levels in neonates with respiratory failure
such therapy compounds the pulmonary problems with due to PPHN.231,232 In an animal model of PPHN, NO
serious iatrogenic injury.208 A respiratory strategy based on decreased pulmonary artery pressures and increased arterial
permissive hypercapnia and spontaneous respiration has oxygen saturation without discernable side effects.233 Unfor-
proven to be quite successful.206 If conventional mechanical tunately, its effects in CDH infants with respiratory failure have
ventilatory techniques cannot reverse the hypoxemia or been mixed.86,231,234–238 There are no data to show that NO
hypercarbia, high-frequency techniques using an oscillating administration improves survival or decreases the require-
ventilator may be required. This technique may be effective ment for ECMO.239 The variable physiologic response to
in removing carbon dioxide and temporarily stabilizing an NO in these infants may be related to the method of its admin-
infant in severe respiratory distress. When such techniques istration.228,240 NO administered through a nasal cannula has
have been used as initial therapy, survival results have been been used for the treatment of late pulmonary hypertension
quite good.137,209,210 following extubation.231 The exact role of NO in the treatment
of pulmonary hypertension and respiratory failure in CDH well. Abnormal drainage of the hepatic veins on either side
infants has not been defined despite its widespread use. may complicate mobilization of the liver.
Once the abdominal contents are reduced, the defect in the
diaphragm in the posterolateral position can be examined. In
SURGICAL MANAGEMENT 20% of patients, a hernia sac formed by parietal pleura and
peritoneum is present and must be excised to minimize
Timing of Surgical Repair chances of recurrence.16 Usually, there is an anterior rim of
Historically, CDH was considered a surgical emergency. diaphragm of varying size. The posterior rim of diaphragm
Infants were rushed to the operating room as soon as possible must be searched for in the retroperitoneal tissue, because it
after birth in the belief that reduction of the abdominal con- may be rolled up like a window shade by the peritoneum.
tents from the chest would relieve the compression of the The peritoneum must be opened over this fold and the dia-
lungs. Frequently, after a brief postoperative honeymoon phragmatic tissue mobilized. When tissue is adequate, a pri-
period marked by adequate gas exchange, progressive deteri- mary repair with interrupted nonabsorbable suture material
oration in the infant’s respiratory status ensued with elevated can be performed (Fig. 63-5, C). In some cases, the posterior
pulmonary vascular resistance, right-to-left shunting, hypox- rim of tissue may disappear along the lateral chest wall. If
emia, and ultimately death resulting from respiratory failure. enough diaphragmatic tissue exists anteriorly, it can be sutured
As management techniques for neonatal respiratory failure directly to the body wall with sutures placed around the ribs.
evolved, a period of medical stabilization and delayed surgical If the defect is too large to be closed in a primary fashion, a
repair, in an attempt to improve the overall condition of the number of reconstructive techniques have been described
infant with CDH, was proposed.117,180,241–249 At the same using various nearby structures, such as prerenal fascia, rib
time, there was increasing evidence of the potential detrimen- structures, and various abdominal wall muscle flaps.267–272
tal effects of early surgical repair on respiratory function.250 If there is any chance that ECMO support might be required
Since then, multiple single-institution studies have reported in the management of the infant, however, the use of complex
improved survival rates with delayed surgery as part of their reconstructive techniques requiring extensive tissue dissec-
treatment protocols, whereas others have found no changes tion is contraindicated because of the risk of bleeding. The
in overall outcome. Importantly, no study has shown a de- use of prosthetic material to complete the diaphragmatic clo-
crease in survival rates with this technique. Although delayed sure has gained widespread acceptance (Fig. 63-5, D). A
surgical repair is now widely practiced, there is no statistical floppy, tension-free diaphragmatic repair can be accom-
evidence that supports this approach over immediate repair plished, which may lessen the degree of intra-abdominal pres-
at this time.251 sure when closing the abdominal wall.273 In addition to the
The optimal timing of operative repair when using a risk of infection, the major drawback to using a prosthetic
strategy of delayed repair also remains undetermined. The patch closure is the risk of dislodgment and subsequent reher-
period of preoperative stabilization has varied from several niation.274 This complication may be lessened by using a
days to several weeks.186,253–255 Some authors have reported cone-shaped patch.275 Complications of prosthetic patch
waiting until the infant is successfully weaning off mechanical repair occur in approximately 10% to almost 50% of cases.
ventilation and requiring low ventilator settings. Others follow Patients who develop a recurrent hernia present with bowel
the severity of pulmonary hypertension with serial echocar- obstruction or respiratory distress or may be asymptom-
diographic examinations and wait until the hypertension atic.276,277 Recently, the split abdominal wall muscle flap
has abated or at least stabilized.256–258 repair was shown to be safe to use on ECMO and was associ-
ated with only one recurrence in 23 patients, with a mean
follow-up of 4.8 years.278
Operative Repair With the loss of intra-abdominal domain, abdominal wall
Most surgeons approach the defect through a subcostal inci- closure may not be possible at all or may result in unaccept-
sion, although the repair can be done through a thoracotomy able intra-abdominal pressure (i.e., abdominal compartment
incision as well. For rare cases in which reduction of the syndrome), even after extensively stretching the abdominal
herniated contents is difficult because of an abnormally wall. In these situations, simple closure of the skin can be
shaped liver or spleen, a combined approach can be used.259 accomplished with repair of the resultant ventral wall defect
Both thoracoscopic and laparoscopic techniques have been some months later. If the skin cannot be closed successfully,
used to repair these defects.260–265a Although thoracoscopic temporary closure using prosthetic material, such as a silo,
repairs have application in stable CDH patients266 and can can be used. Biologic closure should then be obtained as soon
be accomplished with primary or prosthetic material closure, as safely possible in the postoperative period. Drainage of the
a recent report by Gander and colleagues261 suggests chest cavity on the repaired side with a tube thoracostomy is
thoracoscopic repair is associated with an unacceptably high not indicated except for active bleeding or uncontrolled air
recurrence rate within 1 year after repair. leak. It has been proposed that such a tube with even a small
After division of the abdominal wall muscles and entrance degree of negative suction may add to the barotrauma and
into the abdominal cavity, the viscera are gently reduced from pulmonary hypertension imposed by mechanical ventilation
the defect and completely eviscerated for adequate visualiza- on a hypoplastic lung.254,279 Additional surgical procedures
tion. The spleen on the left side and the liver on the right are at the time of the repair, such as correction of the nonrotation
usually the last organs to be mobilized from the chest cavity as well as appendectomy, are not indicated and should be
(Fig. 63-5, A and B). Mobilization can be difficult and must avoided if ECMO is to be considered.
be done without injury to either organ. On the right side, The repair of recurrent defects can present a formidable
the kidney and adrenal gland may be found in the chest as surgical challenge. Since the most common organ involved in
820 PART VI THORAX
A
B
C D
FIGURE 63-5 A, Schematic drawing of an unreduced left congenital diaphragmatic hernia as seen from the abdomen. B, The same hernia but now
reduced, demonstrating that the spleen is usually the last organ to be reduced from the chest cavity. Sutures have been placed for a primary repair.
C, Completed primary repair of a left congenital diaphragmatic hernia. D, Repaired left congenital diaphragmatic hernia using prosthetic material.
(From Spitz L, Coran AG [eds]: Rob & Smith’s Pediatric Surgery. London, Chapman & Hall, 1996.)
recurrent herniation is either the small or large bowel, intestinal surgical repair of CDH infants in the neonatal intensive care
adhesions to the disrupted diaphragm or intrathoracic organs unit. This change in location allows for the lowest degree of
may compromise attempted closure. Repair is most commonly disruption in the neonate’s environment. Anesthesia is achie-
approached through the abdomen but can be accomplished ved by intravenous narcotic and muscle relaxant techniques.
through a thoracotomy as well. If adequate diaphragmatic tissue With intravenous anesthetics, the infant ventilator can be used
is present, then primary reapproximation should be attempted. continuously rather than a conventional anesthesia machine.
Otherwise, different techniques for prosthetic material insertion
have been tried 269,276,280 Because most recurrent CDH occur
Postoperative Management
after initial patch repair and often well away from the neonatal
period, once the baby is more stable, repair using a latissimus Postoperative management should continue the trends and
dorsi muscle flap through a thoracic approach may provide goals established before the operative procedure. Ventilator
the best means to prevent further recurrence.281 support should be tailored to keep preductal SaO2 greater than
90% and PCO2 less than 60 mm Hg.282 Echocardiograms
should be obtained routinely to assess pulmonary hyperten-
Anesthesia
sion, shunt flow, and ventricular performance. Therapeutic
To avoid the stresses of transport and sudden changes in interventions discussed previously may be used if respiratory
ventilation parameters imposed by a trip to the operating room, decompensation develops. Weaning from ventilator support
a number of centers have adopted the policy of performing should be slow and deliberate as tolerated by the infant.
Meticulous attention to fluid status must be maintained, of pulmonary development and associated pulmonary hyper-
particularly in the immediate postoperative period. As a result tension.219,256,292,294 The impact of barotrauma and oxygen
of surgical intervention, these infants are often hypovolemic toxicity from overly aggressive respiratory care strategies
and frequently require extra volume administration over time. on outcome cannot be overemphasized. As conventional
treatment strategies continue to improve, ECMO use and
EXTRACORPOREAL MEMBRANE concomitant survival rates following ECMO may decrease.
A number of surgical issues are relevant in the management
OXYGENATION
of CDH infants while on ECMO. Both venovenous and
Even with recent advances in treatment strategies, overwhelm- venoarterial techniques have been reported to be equally
ing respiratory failure requiring ECMO support occurs in 10% effective in supporting patients while on bypass.284,295 With
to 20% of CDH infants.206,283–285 Initially, infants were placed venovenous bypass, severe right-sided heart failure can be
on ECMO after developing respiratory failure following the managed temporarily with a PGE1 infusion to keep the ductus
immediate repair of the diaphragmatic defect. With the evolu- open until the pulmonary hypertension resolves or by con-
tion of delayed surgical repair, ECMO is now considered a part verting to venoarterial support. The timing of the surgical
of the preoperative stabilization process. repair of the defect in relation to ECMO support remains var-
Clinical criteria for determining ECMO use in infants with iable. As a result of the acceptance of delayed surgical repair as
CDH have been based on factors predictive of at least an 80% a treatment strategy, more than 90% of CDH infants requiring
mortality rate with mechanical ventilation.214 A number of ECMO support are placed on bypass before undergoing
parameters have been proposed, including the calculation surgical repair.242 Surgical repair of the defect while on ECMO
of the oxygenation index (OI) and the alveolar–arterial oxygen can then be accomplished but has been associated with hem-
difference AaDO2. For CDH patients, the most common rea- orrhagic complications in 60% of the patients.244,296 Survival
son for the initiation of ECMO was an OI of 40 or greater, rates after surgery on ECMO have varied from 43% to
and it is often considered for an OI as low as 25.283 Generally 80%.287,297,298 To minimize the risk of hemorrhagic compli-
accepted criteria for initiating ECMO support for neonatal cations, a number of techniques have been proposed, includ-
respiratory failure based on AaDO2 criteria include a value ing the use of heparin-bonded ECMO circuits, performance of
of 610 or greater despite 8 hours of maximal medical manage- the surgical repair just before expected decannulation, and ag-
ment. It must be realized that such criteria continue to be in- gressive management of the anticoagulant status of the infant,
stitution specific and that no calculations can replace clinical including the use of antifibrinolytic therapy. Because of the co-
judgment and frequent bedside assessment. Failure to im- agulation problems, less than 20% of infants are reportedly
prove in the setting of severe pulmonary hypertension and repaired while on ECMO.299 The majority undergo repair
progressive hypoxemia despite maximum medical interven- after the completion of ECMO. This delayed operative
tion remains a valid qualifying criterion for ECMO support. approach, sometimes not occurring until several days after
Controversy still exists as to whether ECMO support decannulation, has been extremely successful, with survival
should be offered to all infants with CDH and respiratory fail- rates of almost 80% and higher.258,284,300 However, there
ure.32,140,286,287 The issue of severe pulmonary hypoplasia are currently no available studies comparing either pathway.
incompatible with life must be kept in mind when ECMO is
being considered. This intervention is successful when used to
support an infant with a reversible process of pulmonary hyper- OUTCOME
tension. However, it is not a treatment for those infants with
irreversible hypoplasia. Differentiating these infants on clinical Survival rates for infants born with a CDH vary from approx-
parameters can be quite difficult. A newborn with a CDH who imately 60% to 90% because of the use of more physiologic
is unable to reach a preductal oxygen saturation level of at least treatment strategies, including “gentle ventilation” techniques,
90% or a markedly elevated PCO2 level unresponsive to any type high-frequency ventilation, cardiovascular pharmacologic sup-
of ventilatory intervention during the pre-ECMO course has a port, and ECMO.* With the gradual improvement in survival
high likelihood of having irreversible hypoplasia.179,289 On the rates over the past 2 decades, there is a greater appreciation
other hand, others have proposed that all infants should be for issues related to the long-term development of CDH survi-
ECMO candidates. Ultimately the decision to use ECMO is a vors and the frequency of associated morbidities, as a greater
clinical decision. If the infant’s tissue oxygen requirements are number of physiologically compromised infants are surviving
not being met, as manifest by end-organ failure, despite best beyond the neonatal period. It is now recognized that CDH
conventional care, ECMO is a reasonable consideration. survivors are at significant risk for chronic neurologic, develop-
Although widely accepted as a treatment for the respiratory mental, gastrointestinal, nutritional, pulmonary, musculoskele-
failure associated with CDH, the impact of ECMO on improv- tal, and other disorders. Late deaths have been reported in
ing overall survival continues to be debated. Over the past approximately 10% of initial survivors, mainly because of the
decade, a number of studies have demonstrated improved consequences of persistent pulmonary hypertension or iatro-
survival rates in CDH infants with ECMO as part of the treat- genic complications.19,39,306,307 The requirement for coordinated
ment strategy.283,290,291 However, other institutions have long-term follow-up and care of these patients has become
either not noted any improvements resulting from ECMO or evident from studies carried out over the past decade.303,307a,308
have been able to manage their infants without it with equiv- Pulmonary issues are the most common long-term prob-
alent success.137,248,292,293,301 Overall survival rates of infants lems in the postnatal period and include chronic lung disease
treated with ECMO vary from 34% to 87% and are clearly or bronchopulmonary dysplasia, reactive airway disease,
dependent on a number of variables, including gestational
age and birth weight, respiratory function, and the degree *References 191, 206, 211, 241, 287, 301–305.
822 PART VI THORAX
pulmonary hypertension, and pneumonia. Pulmonary devel- Pulmonary artery pressures determined by echocardiography
opmental studies have shown that alveolar multiplication normalize in approximately 50% of all patients with CDH by
continues for several years after birth. However, a normal 3 weeks of age but can remain elevated for months in up to
number is never achieved in CDH hypoplastic lungs. Over one third of surviving infants.256,307,325
time, the alveoli in both lung fields may become emphysema- A high incidence of neurodevelopmental abnormalities
tous, as the contralateral lung may herniate across the medi- have been detected in CDH survivors. Developmental delay
astinum on chest radiographs. Gradual remodeling of the has been reported in a number of surviving infants as well
pulmonary vascular bed occurs. However, vascular growth as abnormalities in motor and cognitive skills.314,326 Both
may not match alveolar growth.309,310 Pulmonary function motor and language problems are evident within the first
testing remains the most useful test, because ventilation- 3 years of life, and infants with motor problems detected at
perfusion studies have been associated with conflicting age 1 year were more likely to have abnormal postnatal neu-
reports on pulmonary growth.311 roimaging studies.327–329 Progressive sensorineural hearing
Immediately following surgical repair of the hernia and for loss has been demonstrated in up to 50% or more of CDH
the first 6 months of life, abnormalities in pulmonary function survivors with no discernable etiology. Its onset appears to
values have been reported in such measurements as functional be within the first 2 years of life, but late onset deficits in
residual capacity (FRC), compliance (C), airway resistance patients with previously normal audiology tests have been
(R), and maximum expiratory flow rate at FRC.151,312,313 reported.62,330 It is a progressive abnormality and requires fre-
Significant improvements in lung function become evident quent audiology follow-up. Aggressive use of aminoglycoside
during the first year of life, with only mild abnormalities in antibiotics and furosemide diuretics may be part of the etiol-
subsequent tests noted after 2 years of age, indicating that lung ogy. Other neurologic findings reported in CDH survivors
function does improve over time. These improvements in lung include visual disturbances, seizures, and abnormal com-
function parameters, such as compliance and airway resis- puted tomography (CT) and MRI studies.314,331 Most studies
tance, correlate with the growth of the infant, and any com- have implicated ECMO as a factor in these neurologic prob-
promise of the nutritional status of a CDH infant should lems, but infants treated without ECMO are also at risk.332–334
raise concern about lung growth and development.19 Chronic CDH survivors have a high incidence of gastrointestinal
lung disease has been reported in CDH survivors, particularly conditions, of which gastroesophageal reflux is the most
in those requiring ECMO support.314,315 Whether this finding significant (Fig. 63-6).52,314,335,336 The condition may occur
is related to the pathology of the disease or has been induced in as many as 80% of patients after CDH repair and in
iatrogenically resulting from techniques of ventilation is 60% of long-term survivors.337 Infants requiring patch clo-
unclear.250 Treatment strategies for these patients have sure of the defect, having an intrathoracic position of the
included the use of supplemental oxygen, bronchodilator stomach at the time of repair, or requiring ECMO support
therapy, corticosteroids, diuretics, and appropriate immuniza- are at higher risk for developing symptomatic reflux.52,336a–338
tions. Prolonged ventilator support and tracheostomy may be Antireflux surgery for severe disease is required in 15% to
required in a small percentage of patients.316 35% of cases. Nutritional and growth-related problems have
In long-term studies of pulmonary function in survivors, been found in a significant number of survivors.92,339,340
many adolescents and adults have been found to have nearly Aggressive nutritional management using gastrostomy feedings
normal exercise capacity and cardiorespiratory response to may be required.303
exertion.151,315,317–321 Abnormalities in such parameters as A number of skeletal disorders have been reported, inclu-
forced expiratory volume in 1 second (FEV1), forced vital ding chest wall deformities and scoliosis.341,342 Chest wall
capacity (FVC), and maximum midexpiratory flow and peak
expiratory flow rates indicate that obstructive and restrictive
ventilatory impairments are present in up to 50% of sur-
vivors.311,318,320,322 The functional consequences of these
flow abnormalities are reportedly minimal.
The risk of pneumonia in CDH survivors, particularly in
infancy and early childhood, is significant and has been
reported in up to 35% of patients by the age of 12. Viral
bronchiolitis, particularly with respiratory syncytial virus
(RSV), is a concern in the first 3 years of life.151,323
Although it is known that patients with congenital heart
disease and CDH have a poor prognosis, the long-term con-
sequences of structural cardiac abnormalities in survivors
are unknown. Although the frequency of cardiovascular
malformations ranges from 11% to 17% and includes major
structural defects, such as atrial and ventricular defects, con-
otruncal defects, and left ventricular outflow tract obstructive
defects, the risk of death in these patients can be as high as
3 times that for patients with CDH alone.48,324 Prolonged
elevation in pulmonary artery pressure, whether it results
from pulmonary hypoplasia, bronchopulmonary dysplasia, FIGURE 63-6 Barium sulfate esophagogram in an infant with a left con-
or structural cardiac disease, impacts survival, and late deaths genital diaphragmatic hernia demonstrating a dilated, ectatic esophagus.
have been associated with persistent pulmonary hypertension. The stomach was oriented vertically and emptied slowly.
deformities have been associated with patch repair of the de- pneumocytes, have also been noted.356,361–365 Improvement
fect. Most defects are minor, and treatment of these problems in physiologic lung function was seen in some studies but
has included initial attempts at bracing followed by surgical not in others.252,310,358,366 Local effects on tracheal structure
correction if progressive. have included tracheomegaly.367,368 Although this intervention
Recurrent diaphragmatic hernia and small bowel obstruc- in the prenatal management of fetuses with CDH has been
tion are the dominant surgical challenges following initial reported in several institutional studies,197–199 there are no ran-
repair. Recurrent hernias may occur in up to 50% of infants domized trials in the North American setting demonstrating a
undergoing patch repair of the defect and in 10% of primary clinical benefit in improving postnatal survival in a cohort of
repairs, and they tend to occur in the first 4 years of life.342–344 CDH fetuses with agreed upon prohibitive mortality.193,196,352
A small bowel obstruction may occur in a small percentage of The role of tracheal occlusion in the treatment of CDH remains
patients and may be related to adhesions, reherniation, or, experimental and unproven. Continued evolution in the tech-
rarely, a volvulus. Chylothorax can also occur after both niques of this procedure as well as bona fide, randomized
primary and patch closure and may require surgical interven- clinical trials will continue to generate interest and perhaps
tion if conservative management is unsuccessful.65 validation of this intervention.
There is a growing realization that infants with a right-sided Liquid ventilation techniques have been attempted in CDH
diaphragmatic defect may present with management chal- infants while on ECMO (Fig. 63-7).369,370 After perfluoro-
lenges and outcomes different from those with left-sided carbon administration, significant increases were reported
defects. Herniation of the liver into the right chest can be a in PaO2 levels and in static total pulmonary compliance
surgical challenge in diaphragmatic repair, and cases of hepa- measurements accompanied by a fall in PaCO2 levels. No
topulmonary fusion have been reported.345–347 Infants with adverse side effects were noted. Extensive studies are required
right-sided defects may have a higher requirement for patch to examine this newest form of ventilation before its efficacy
repair of the diaphragm as well as for ECMO support.348,349 can be judged.
Over the last decade, it has become clear that CDH survivors Based on the observations of fetal lung growth induced by
present many complex management challenges and require tracheal occlusion, inducement of postnatal lung growth with
lifelong medical surveillance and follow-up. Comprehensive static distention has also been investigated. During the course
multispecialty clinics that provide specialty physician and of the liquid ventilation experiments, pulmonary distention as
support services, along with evidence-based guidelines, are a result of perfluorocarbon administration was observed. Its
important in advancing the care of these patients.307a,342 use as a potential treatment to induce postnatal lung growth
in CDH patients was then reported.371–373 Preliminary studies
have shown significant radiographic enlargement of the lung
FUTURE THERAPIES
and improved gas exchange. Animal studies have supported
Despite the advancements that have been made in treating in- the concept of accelerated postnatal lung growth using
fants with CDH, it still represents a frustrating and complex perfluorocarbon distension.374 Further study of this potential
clinical problem. As the striking variance in survival rates intervention is required.
attests, no currently used therapeutic intervention or manage- Lung transplantation has also been used anecdotally in the
ment strategy has emerged for widespread successful applica- surgical treatment of CDH.375,376 Both unilateral and bilateral
tion. Even with the increasing success of current treatment
strategies, such as permissive hypercapnia, delayed operative
repair, antihypertensive pharmacology, and advanced venti-
latory techniques, a cohort of infants refractory to these inter-
ventions continue to be candidates for novel treatments.
Fetal intervention in the management of CDH remains
highly controversial. The concept of fetal surgical intervention
evolved from the experimental observations in lambs that the
reduction of compressive forces on the lung resulted in con-
tinued pulmonary growth and development.91,98,101,194,350
With the development of fetal surgical techniques, initial
studies attempted direct surgical repair of the defect with
reduction of the herniated contents.351,352 When no clinical
advantage in survival was demonstrated, this approach was
terminated. Experimental evidence for the important role of
lung fluid dynamics in fetal lung development101,353–355 has
subsequently triggered interest in the role of tracheal occlu-
sion (TO) as a fetal intervention to promote in utero lung
growth.175,356–359 The clinical technique has evolved from
the open or fetoscopic placement of extraluminal clips on
the trachea to the insertion of an endoluminal device with
either a foam plug or detachable balloon. Experimentally, lung
growth was further enhanced using intraalveolar fetal albumin
FIGURE 63-7 Chest radiograph of a left congenital diaphragmatic hernia
administration.360 Although TO has been shown in some stud- being supported with extracorporeal membrane oxygenation. The lungs
ies to induce changes in pulmonary morphology, pathologic have been opacified with perflubron for liquid ventilation. The pulmonary
changes, such as a decrease in type II (surfactant-producing) hypoplasia can be appreciated. (Courtesy R. B. Hirschl, MD.)
824 PART VI THORAX
transplants have been attempted. Currently, not enough may be similar for the most severe forms, while small
experience exists to recommend this form of treatment. localized eventrations are usually discovered incidentally
The potential role of pharmacologic augmentation of on chest radiographs. A lateral chest radiograph is essential
pulmonary growth and development is currently being investi- to the evaluation of eventration, especially on the right side.
gated. The combined administration of thyrotropin-releasing A modest posteromedial eventration that looks much more
hormone and glucocorticoid therapy has been studied in significant on an anteroposterior view is unlikely to benefit
a chemically induced rat model of CDH, with positive effects from surgical intervention. The acquired lesion is due to
on lung growth.24,377 It is also known that a number of growth paralysis of the phrenic nerve, which may occur from injury
factors are crucial to normal pulmonary development. It has during repair of congenital heart defects or through brachial
been proposed that perhaps selected administration of one or plexus injury at birth (Erb palsy).385 In these cases, the
several of these pharmacologic agents or growth factors may diaphragmatic muscle is usually present in its normal
be able to reverse the pulmonary hypoplasia of CDH.60,377 distribution, but it is attenuated and inactive.
Continued experimental work using the nitrofen model of There may be no symptoms whatsoever, even in the
CDH may uncover new candidates to promote lung growth presence of a large eventration, although the findings
and development either prenatally or after birth. may range from wheezing, frequent respiratory infections,
Finally, given the current wide-ranging survival rates at and exercise intolerance to extreme respiratory distress.
various institutions, an in-depth study and evaluation of Diagnosis is usually made on fluoroscopy of the chest or with
current management techniques and outcomes must be made. ultrasound examination. In such cases, the diaphragm
The efforts of the CDH Study Group, Extracorporeal Life moves paradoxically with respiratory motion. This para-
Support Organization (ELSO),378 and the Canadian Pediatric doxic movement may be so marked that it results in severe
Surgery Network to interpret very heterogeneous data are compromise of gas exchange. Although pneumoperitoneum
an encouraging beginning. Such studies might result in the was used frequently in the past as a diagnostic modality, CT
refinement and consolidation of current practices into a or MRI is used more often today. A right diaphragmatic even-
universally effective treatment strategy. tration may be difficult to differentiate from a CDH, because
the liver may block the defect; sometimes thoracoscopy is re-
quired for diagnosis.
Foramen of Morgagni Hernia A small eventration may be left untreated. Repair is indi-
------------------------------------------------------------------------------------------------------------------------------------------------
cated when a large functional deficit in the function of the
The diaphragmatic hernia of Morgagni is located anterome- ipsilateral lung on ventilation/perfusion studies is found in
dially on either side of the junction of the septum transversum an apparently asymptomatic patient. In such cases, the com-
and the anterior thoracic wall. The defect occurs through the pressed lung will not grow well. Repair may be performed
embryologic space of Larrey. Occasionally, bilateral Morgagni either through the abdomen or the chest, but, in most cases,
hernias communicate in the midline, constituting a large ante- a low thoracotomy is recommended, because this approach
rior diaphragmatic defect extending all the way across the mid- allows identification and preservation of phrenic nerve fibers
line from right to left. Typically, a sac is present, and herniation in case the damaged nerve recovers. A thoracic approach is
of the colon or small bowel is usually discovered to the right or especially preferred for right diaphragmatic eventration,
left of the midline. Morgagni hernias account for less than 2% of because the liver would make an abdominal approach more
diaphragmatic defects. Although this defect may be observed in difficult. Through this approach, the diaphragm is best pli-
neonates, it usually presents more commonly in older children cated with nonabsorbable interrupted 2-0 sutures. A radial
or adults. Associated anomalies may be present and include or peripheral incision may also be made in the diaphragm
malrotation, cardiac defects, and trisomy 21.379,379a An ante- and the edges overlapped and sutured. It is important to reef
rior midline deficiency in the diaphragm, with or without up and overlap the diaphragm so that it is taut—overcorrecting
the other elements of the pentalogy of Cantrell, with free peri- it somewhat, because invariably, the muscle will stretch
cardial and peritoneal communication may allow herniation of and the eventration will recur if this is not done. Although
intestine into the pericardium. The hernia is often discovered phrenic nerve injury may spontaneously recover, diaphrag-
incidentally as a mass or air–fluid level on a chest radiograph. matic plication for acquired eventration is frequently neces-
A barium enema or a CT scan may confirm the diagnosis. sary to wean infants from ventilatory support. Plication
Operative correction is easily performed through an upper can also be accomplished by either a laparoscopic or thora-
transverse abdominal incision. The diaphragm is sutured to coscopic approach. This operation does not preclude normal
the underside of the posterior rectus sheath at the costal diaphragmatic function, should phrenic nerve function
margin after reduction of the hernia and resection of the return.
sac. Laparoscopic and thoracoscopic techniques have also
been used to repair this defect, but the laparoscopic approach
is generally favored.380–384 Acknowledgments
For Carol and Judy, who wait . . . and wait. . . .
Eventration of the Diaphragm
------------------------------------------------------------------------------------------------------------------------------------------------
The complete reference list is available online at www.
expertconsult.com.
Eventration of the diaphragm may be either congenital or
acquired. The congenital form may be indistinguishable
from a diaphragmatic hernia with a sac, and symptoms

Hernia Diafragmatica

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of the intestines were contained in that cavity except part of the

colon which was distended with meconium. Before I proceeded

reported.13,27–31 The expected recurrence risk in a first-degree

Any newborn with a major congenital anomaly, including

Although the cause of CDH is uncertain, its consequences on

Success in the management of CDH has improved dramati-

It is important to consider this fact before exposing an infant

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