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Diaphragmatic Hernia

Incidence
1.Congenital diaphragmatic hernia (CDH) refers to a
congenital defect in the posterolateral diaphragm at the
foramen of Bochdalek.
It is a relatively common case of neonatal respiratory
distress !ith an overall incidence "et!een 1#$%%% and
1#&%%% live "irths. CDH acconts for a"ot '%( of
congenital diaphragmatic defects.
)ighty to ninety percent of congenital diaphragmatic
hernias occr on the left side. * hernia sac is only present
$%( of the time.
$.+etrosternal hernias (,orgagni) are mch less
common and only accont for $-.( of congenital
diaphragmatic defects. /.Diaphragmatic eventration
is even rarer "t is a postoperative complication in 1-$(
of children ndergoing srgery to repair congenital heart
defects.
Etiology
0he speci1c etiology of CDH is nkno!n "t it is "elieved
to reslt from a defective formation of the
pleroperitoneal mem"rane.
In the early !eeks of development2 the pleral and
peritoneal cavities commnicate via the paired
pleroperitoneal canals. Dring the 3th !eek2 the pleral
cavity "ecomes separated
from the peritoneal cavity "y the developing
pleroperitoneal mem"rane.
If the pleroperitoneal mem"rane fails to develop2
closre of the pleroperitoneal canal is incomplete and a
posterolateral diaphragmatic defect reslts.
* ne!er hypothesis has arisen from the nitrofen rat
model of CDH.
)lectron microscopy of these nitrofen e4posed rat
em"ryos sggests that CDH reslts from a defective
development of the
posthepatic mesenchymal plate !hich also contri"tes
to closre of the pleroperitoneal canal.
*lthogh familial cases are reported2 most cases of CDH
are sporadic.
CDH is associated !ith trisomies 132 $12 and $/ "t a
speci1c genetic etiology has yet to "e identi1ed.
,orgagni hernias reslt from failre of the sternal and
crral portions of the diaphragm to fse at the site !here
the sperior epigastric artery traverses the diaphragm.
,orgagni hernias are associated !ith congenital heart
disease and trisomy
* variant of the retrosternal hernia is associated !ith the
pentalogy of Cantrell !hich incldes# omphalocoele2
inferior sternal cleft2 severe cardiac defects (inclding
ectopia cordis)2 diaphragmatic hernia and pericardial
defects.
0he diaphragmatic defect reslts !hen the septm
transversm fails to develop in the em"ryo.
)ventration of the diaphragm may "e either a congenital
or ac5ired lesion.
6eonatal eventration may "e de to defective central
development or enervation of the diaphragm. It may also
reslt from a traction in7ry to the nerve roots of the
phrenic nerve dring tramatic delivery. )ventration most
often reslts from iatrogenic phrenic nerve in7ry
complicating cardiac or mediastinal srgery.
Defnitions
The diaphragm develops largely from three
structures:
1 the pleuroperitoneal membrane;
2 the septum transversum;
the marginal ingro!ths from the muscles of the
body !all"
#ongenital diaphragmatic hernia results from
failure of formation or fusion of the components of
the diaphragm$ such that abdominal contents can
move through a defect into the chest"
%ometimes$ failure of muscularisation may produce
a thin$ !ea& diaphragm$ referred to as an
eventration of the diaphragm"
The 'ochdale& type is the most common variety of
congenital diaphragmatic hernia and results from
a defect in the posterolateral part of the
diaphragm" During intra(uterine development$ the
small bo!el$ stomach$ spleen and left lobe of the
liver pass through the defect in the diaphragm into
the chest$ limiting the space available for the
developing lung" This causes lung hypoplasia$
!hich in many infants is severe enough to produce
severe respiratory distress !ithin minutes of birth$
and may not be compatible !ith life"
The )orgagni *retrosternal+ type of diaphragmatic
hernia is rare$ and results from a defect in the
anterior midline$ ,ust behind the sternum -.ig/" It
usually contains part of the colon or small bo!el$
and less commonly$ part of the liver" 0ccasionally$
a hernia may occur through the ape1 of the cupola
or at the periphery ad,acent to the costal margin"
0esophageal hiatal hernias also occur and usually
produce symptoms of gastro(oesophageal re2 u1"
#linical features
3ntenatal diagnosis
)ost congenital diaphragmatic hernias are
diagnosed !ell before birth$ on antenatal
ultrasonography" .actors that may indicate a !orse
prognosis on antenatal scanning *summarised in
Table + may in2uence counselling of the parents(to(
be" 3ntenatal ultrasonographic diagnosis of
diaphragmatic hernias also allo!s the mother(to(
be to be transferred to a tertiary paediatric
surgical centre before birth" %uccessful in utero
correction of diaphragmatic hernia and fetoscopic
tracheal occlusion has been achieved in research
setting$ but the techni4ues are comple1 and the
indications are still being ref ned" To date$ they
have not resulted in improved survival or reduced
morbidity compared !ith modern postnatal
techni4ues"
Table " 5ossible antenatal mar&ers of severity
Early gestational age at diagnosis on
ultrasonography
6ung(to(head ratio *6H7+ at 2892:(!ee&s;
gestation <1"=
%mall fetal lung volume on D ultrasonography
and )7I
>?=@ liver in chest on right side
6iver in chest on left side
%tomach and spleen in chest on left side
Ao hernial Bsac; *diC cult to conf rm on
scanning+
5ostnatal diagnosis
The ma,ority of infants born !ith a 'ochdale&
*posterolateral+ diaphragmatic hernia become
symptomatic at or shortly after birth" Dhere
pulmonary hypoplasia is severe$ the infant
becomes cyanosed !ith severe respiratory distress
!ithin minutes of birth" In other patients there is
tachypnoea$ increased respiratory eEort$ hyperin2
ated chest and scaphoid abdomen$ and heart
sounds are on the right side" This is because F?@
of posterolateral hernias involve the left
hemidiaphragm" The remainder are rightsided
*12@+ or bilateral *@+" 3ssociated anomalies
occur in up to 8=@$ but most are minor and do not
aEect survival$ for e1ample$ undescended testes"
The most common serious abnormalities are heart
defects" Gnli&e posterolateral hernias$ most
anterior *retrosternal+ hernias are symptomless
unless strangulation occurs"
Hery rarely the hernia may protrude into the
pericardial cavity rather than into the inferior
mediastinum and cause cardiac tamponade$
presenting as cardiorespiratory distress in the
neonatal period"
I(ray of congenital diaphragmatic hernia
*'ochdale& type+" )ultiple bo!el loops f ll the left
pleural cavity$ and the heart is displaced to the
right"
Investigation
Diagnosis of a posterolateral hernia is confrmed
by a chest 1(ray -.ig" ?"2/" In left(sided defects$
loops of bo!el can be seen in the left chest" The
heart is deviated to the right" 6ittle room is left for
the lungs$ particularly the left lung !hich is
mar&edly compressed" %ometimes$ the appearance
may be diCcult to distinguish from basal lung
cysts$ in !hich case a repeat chest 1(ray is
performed after a nasogastric tube has been
inserted$ the tip of !hich can be seen in the chest"
3lternatively$ a barium study !ill sho! bo!el
!ithin the thoracic cavity !hen there is a
diaphragmatic hernia"
Treatment
5osterolateral *'ochdale&+ hernia
Dhere an antenatal ultrasound e1amination has
identify ed a diaphragmatic hernia$ the best
outcomes are achieved if the infant is transferred
to a tertiary paediatric surgical centre prior to
birth" This is because these infants may develop
severe pulmonary distress very 4uic&ly after birth$
ma&ing subse4uent transfer diCcult and
potentially dangerous" Initial treatment involves
intensive cardiorespiratory support and insertion
of a nasogastric tube to prevent bo!el dilatation
!ithin the chest" #are must be ta&en to avoid
hyperin2 ation and barotrauma of the small
hypoplastic lungs" High(fre4uency oscillatory
ventilation in combination !ith nitric o1ide has
improved survival rates"
Hentilation !ith a face mas& *Bbagging;+ should be
avoided as this may force air into the stomach$
increasing its volume at the e1pense of the already
compromised lungs" Higorous endotracheal
ventilation should also be avoided because of the
ris& of causing barotrauma and a tension
pneumothora1$ !hich can lead to the rapid demise
of the infant" E1ogenous surfactant provides no
specifc beneft in ne!borns !ith diaphragmatic
hernia" The &ey to success is careful gentle
ventilation that minimiJes in,ury to the hypoplastic
lungs" %udden deterioration of the infant;s
condition during initial resuscitation or during
transport suggests the development of a tension
pneumothora1$ and this may necessitate prompt
drainage by needle aspiration or insertion of an
intercostal drain"
.ortunately$ strict avoidance of hyperventilation
and limited in2 ation pressures have made this
complication rare" %urgery to return the bo!el to
the abdominal cavity and to repair the defect in
the diaphragm is performed !hen the infant;s
condition is stable" This may be any!here bet!een
12 h and K or more days after birth" In left(sided
defects$ a left transverse or subcostal abdominal
incision is used" The management of the infant
!ith severe hypoplastic lungs is diC cult and may
involve highfre4uency oscillation or e1tracorporeal
membrane o1ygenation" The ma,or cause of death
remains pulmonary hypoplasia and pulmonary
hypertension" 5ulmonary hypertension is due to
the small pulmonary vascular bed and to the
changing resistance of the pulmonary arterioles: it
resolves in most patients !ith time$ provided
ventilation does not produce additional lung in,ury"
%urvival rates of about F=@ are no! being
reported"
Diagram of the diaphragm and its attachment to
the sternum$ sho!ing the site of an anterior
diaphragmatic hernia"
3nterior diaphragmatic hernia
3nterior diaphragmatic *)orgagni+ hernias are
often diagnosed on an incidental 1(ray of the chest
in a symptomless patient$ but repair is still
advisable because of the ris& of strangulation of
the bo!el that protrudes through the defect" This
is usually performed as a laparoscopic procedure
-.ig" ?"/" The results are e1cellent"
Ley 5oints
Diaphragmatic hernia is diagnosed antenatally
or by chest 1(ray in a baby !ith a barrel
chest$scaphoid abdomen and respiratory
distress"
Hentilatory support$ especially during
transport$ should be the minimum re4uired to
prevent deterioration$ as hyperin2 ation !ith
2M barotraumas is a signif cant complication"
%udden deterioration is usually caused by
tension pneumothora1"
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#ase 1
Dithin minutes of birth$ a full(term infant boy
develops increasing respiratory distress and
becomes cyanosed" He fails to improve !ith upper
air!ay suctioning" The pregnancy !as uneventful"
He loo&s barrel(chested and his abdomen is
scaphoid"
O 1"1 Dhat is the diagnosisP
O 1"2 Dhat investigation !ill conf rm the
diagnosisP
O 1" Dhat factors determine the outcome in these
situationsP
#ase 2
3 ne!born infant !ith a recently diagnosed left(
sided congenital diaphragmatic hernia is about to
be transferred to a paediatric surgical institution
by air" He is currently being ventilated through an
endotracheal tube and ,ust maintaining ade4uate
blood gas levels"
O 2"1 %hould his ventilation be increased during
transportP
O 2"2 %hould any other manoeuvre be performed to
reduce the li&elihood of problems during
transportP
O 2" If he suddenly deteriorates$ !hat
complication may have happenedP
than&s