Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia
Dr. Faheem ul Hassan Andrabi
M Ch

CDHCDH
 ““dextrocardia” accompanied by respiratorydextrocardia” accompanied by respiratory
distress is a CDH until proven otherwise.distress is a CDH until proven otherwise.

CDHCDH
 1679 – Riverius recorded the first CDH1679 – Riverius recorded the first CDH
 1761 – Morgagni desribed types of CDH1761 – Morgagni desribed types of CDH
 1905 – Heidenhain repair CDH1905 – Heidenhain repair CDH
 1925 – Hedbolm suggested that CDH led to1925 – Hedbolm suggested that CDH led to
pulmonary hypoplasia and early operationpulmonary hypoplasia and early operation
improve survivalimprove survival
 1946 – Gross correct CDH < 24 hours of age1946 – Gross correct CDH < 24 hours of age
 1980-1990 – delayed correction become wide1980-1990 – delayed correction become wide

CDHCDH
 1 in 2000 to 5000 births1 in 2000 to 5000 births
 The incidence in stillborns is less wellThe incidence in stillborns is less well
documenteddocumented
 80% being left sided and 20% right sided.80% being left sided and 20% right sided.
 Bilateral CDH defects are rareBilateral CDH defects are rare
 Infants with isolated CDH are more likely toInfants with isolated CDH are more likely to bebe
prematurepremature,, macrosomic,macrosomic, andand malemale

CDHCDH
 aboutabout one third of affectedone third of affected infants mayinfants may
have associatedhave associated major defectsmajor defects
 Women who are thin or underweight may haveWomen who are thin or underweight may have
an increased risk of having an infant with anan increased risk of having an infant with an
isolated CDHisolated CDH
 retinoid-regulated target genesretinoid-regulated target genes may bemay be
responsible for CDH developmentresponsible for CDH development

Associated AnomaliesAssociated Anomalies
 Associated malformations range from 10% toAssociated malformations range from 10% to
50%50%
 Skeletal defects 32%Skeletal defects 32%
 Cardiac anomalies have been found in 24 %Cardiac anomalies have been found in 24 %
 Anatomic anomalies of the tracheobronchialAnatomic anomalies of the tracheobronchial
tree have been found in 18% of patients withtree have been found in 18% of patients with

CDHCDH
 Skeletal include anomaliesSkeletal include anomalies
 limb reduction andlimb reduction and
 costovertebral defectscostovertebral defects
 Cardiac anomaliesCardiac anomalies
 ventricular septal defects,ventricular septal defects,
 tetralogy of Fallot,tetralogy of Fallot,
 transposition of the great vessels,transposition of the great vessels,
 double outlet right ventricle, anddouble outlet right ventricle, and
 aortic coarctation.aortic coarctation.

Associated AnamoliesAssociated Anamolies
 Cardiac hypoplasiaCardiac hypoplasia
 hypoplasia of the aortic arch.hypoplasia of the aortic arch.
 Anatomic anomalies of the tracheobronchialAnatomic anomalies of the tracheobronchial
tree includetree include
 congenital tracheal stenosis,congenital tracheal stenosis,
 tracheal bronchus,tracheal bronchus,
 and trifurcated tracheaand trifurcated trachea

EmbryologyEmbryology
 The fully developed diaphragm is derived fromThe fully developed diaphragm is derived from
four distinct componentsfour distinct components
 1: the1: the anterior central tendonanterior central tendon (from the(from the
septum transversum),septum transversum),
 2: the2: the dorsolateral portionsdorsolateral portions
((pleuroperitoneal membranes)pleuroperitoneal membranes)

 3: the3: the dorsal cruradorsal crura evolve from theevolve from the
esophageal mesenteryesophageal mesentery , and, and
 4 the4 the muscular portionmuscular portion of the diaphragmof the diaphragm
develops from thedevelops from the thoracic intercostalthoracic intercostal
musclemuscle groupsgroups

 The precursors of diaphragmatic structureThe precursors of diaphragmatic structure
begin to form during thebegin to form during the fourth weekfourth week ofof
gestation with the appearance of the peritonealgestation with the appearance of the peritoneal
fold from the lateral mesenchymal tissue.fold from the lateral mesenchymal tissue.
 Closure of the pleuroperitoneal canals with theClosure of the pleuroperitoneal canals with the
formation of a pleuroperitoneal membraneformation of a pleuroperitoneal membrane
occurs during theoccurs during the eighth weekeighth week of gestationof gestation

Separating the abdominal and thoracic cavities:Separating the abdominal and thoracic cavities:
development of the septum transversum and diaphragmdevelopment of the septum transversum and diaphragm
Larsen’s fig 11-10
Larsen’s fig 11-09

CDHCDH
The septum transversum stops at the gut tube, leaving two open passageways on the left and
right sides, aka the “pericardioperitoneal canals” Closing off these canals requires growth from
the dorsolateral body wall, aka the “pleuroperitoneal membranes” (shown on the right)
Defects in this process cause CDH (congenital diaphragmatic hernias)

CDHCDH
 During the early development of the diaphragm,During the early development of the diaphragm,
thethe midgut is herniatedmidgut is herniated into the yolk sac.into the yolk sac.
 Closure of theClosure of the pleuroperitoneal canalpleuroperitoneal canal isis
completed by that time the midgut returns tocompleted by that time the midgut returns to
the abdomen (weeks 9 and 10) .the abdomen (weeks 9 and 10) .
 At the time of midgut return, if PPC is still notAt the time of midgut return, if PPC is still not
closedclosed abdominal viscera herniateabdominal viscera herniate into theinto the
ipsilateral thoracic cavity.ipsilateral thoracic cavity.

CDHCDH
 Some claim the herniation can occurSome claim the herniation can occur late inlate in
gestationgestation oror be intermittently presentbe intermittently present as aas a
dynamic process,dynamic process,
 In most cases the defect is established byIn most cases the defect is established by
gestational week 12gestational week 12
 A membrane forms a hernia sac inA membrane forms a hernia sac in 10% to10% to
15%15% of CDH patients.of CDH patients.

Embryological developmentEmbryological development
 Development of lung has 5 stagesDevelopment of lung has 5 stages
 Embryonic stageEmbryonic stage
 Pseudoglandular stagePseudoglandular stage
 Canalicular stageCanalicular stage
 Terminal sac periodTerminal sac period
 Alveolar periodAlveolar period

 Embryonic phase (3-7 weeks)Embryonic phase (3-7 weeks)
 Begins with the formation of theBegins with the formation of the
respiratory diverticulumrespiratory diverticulum from thefrom the
ventral wall of the primitiveventral wall of the primitive
foregut.foregut.
 Around 33 days of gestation, theAround 33 days of gestation, the
distal end of the respiratorydistal end of the respiratory
diverticulumdiverticulum bifurcates into twobifurcates into two
budsbuds (left and right primary(left and right primary
bronchibronchi))

 Pseudoglandular phase (5-17Pseudoglandular phase (5-17
weeks):weeks):
 Segmental and sub-Segmental and sub-
segmental bronchi are formedsegmental bronchi are formed
through athrough a series of divisionsseries of divisions
 Repeated branching forms upRepeated branching forms up
toto terminal bronchiolesterminal bronchioles
 No respiratory bronchiolesNo respiratory bronchioles
and alveoli are presentand alveoli are present

EmbryologicalEmbryological
developmentdevelopment
 Canalicular phase (17-24 weeks)Canalicular phase (17-24 weeks)
 TheThe diameterdiameter of the tube increasesof the tube increases
 Respiratory bronchiolesRespiratory bronchioles andand
alveolar ducts develop.alveolar ducts develop.
 airspaces are being canalized andairspaces are being canalized and
approximated by a network ofapproximated by a network of
capillariescapillaries ..

 Saccular phase (25-36 weeks)Saccular phase (25-36 weeks)
 Primitive alveoli arePrimitive alveoli are
formedformed
 By 26 weeks the alveoliBy 26 weeks the alveoli
have becomehave become
vascularisedvascularised

 Alveolar phase (36 weeks -Alveolar phase (36 weeks -
childhood)childhood)
 It is the lung maturation periodIt is the lung maturation period
 Pneumocytes (I and II) increasePneumocytes (I and II) increase
in numberin number
 increase theincrease the surfactantsurfactant
production.production.
 Alveoli have well developedAlveoli have well developed
epithelial-endothelial contactepithelial-endothelial contact

Factors for pulmonary organogensis andFactors for pulmonary organogensis and
maturationmaturation
 Homeobox genes,Homeobox genes,
 Nuclear transcription factorsNuclear transcription factors
 Glucocorticoids ,Glucocorticoids ,
 Thyroid hormone, andThyroid hormone, and
 Retinoic acidRetinoic acid

PathologyPathology
 The classical left-sided CDH is aThe classical left-sided CDH is a 2.0- to2.0- to
4.0-cm4.0-cm posterolateral defect in theposterolateral defect in the
diaphragm.diaphragm.
 Herniated contents often include theHerniated contents often include the
 left lobe of theleft lobe of the liverliver,,
 SpleenSpleen
 almost thealmost the entire gastrointestinal tract.entire gastrointestinal tract.
 StomachStomach
 Occasionally, theOccasionally, the kidneykidney may be in the chestmay be in the chest
 thethe large right lobe of the liverlarge right lobe of the liver (right-sided(right-sided

PathologyPathology
 The stomach is frequently in the chest, which results in someThe stomach is frequently in the chest, which results in some
degree ofdegree of obstruction at the GE Jnxobstruction at the GE Jnx
 This obstruction, in turn, causesThis obstruction, in turn, causes dilation and ectasiadilation and ectasia of theof the
esophagus.esophagus.
 Occasionally, the kidney may be in the chestOccasionally, the kidney may be in the chest tethered by the renaltethered by the renal
vessels.vessels.
 In Rt. Sided CDH hepatic veins may drainIn Rt. Sided CDH hepatic veins may drain ectopically into the rightectopically into the right
atriumatrium, and, and
 fibrous fusionfibrous fusion between the liver and the lung has been reported.between the liver and the lung has been reported.

PathologyPathology
 CDH affects bothCDH affects both ipsilateral and contralateralipsilateral and contralateral
pulmonary developmentpulmonary development
 the lung development becomes compromisedthe lung development becomes compromised
at theat the time of bronchial subdivisiontime of bronchial subdivision..
 Alveolar development is severely affected, andAlveolar development is severely affected, and
only a few normal alveolionly a few normal alveoli exist in at term.exist in at term.

Pathology: vascular changesPathology: vascular changes
 AA reduction in the total number of arterialreduction in the total number of arterial
branchesbranches (in both the IPL and the CL(in both the IPL and the CL
pulmonary parenchyma)pulmonary parenchyma)
 significant adventitial and medialsignificant adventitial and medial wall thickeningwall thickening
in pulmonary arteries of all sizesin pulmonary arteries of all sizes
intractable pulmonary hypertension.

CDHCDH
 The fetus preferentially shunts oxygenatedThe fetus preferentially shunts oxygenated
blood from the placenta through theblood from the placenta through the foramenforamen
ovaleovale andand ductus arteriosusductus arteriosus in ain a right-to-leftright-to-left
directiondirection

PathologyPathology
 At birthAt birth
 Fall in pulmonary vascular resistanceFall in pulmonary vascular resistance
 Fall inFall in pulmonary artery pressurepulmonary artery pressure
 Rise inRise in Systemic vascular resistanceSystemic vascular resistance and left atrialand left atrial
pressurepressure
Closure of Foramen Ovale

PathologyPathology
 At birthAt birth
 Increased arterial oxygen tensionIncreased arterial oxygen tension
closure of the ductus arteriosus

Persistent fetal circulation
Respiratory
failure

CdhCdh
 There isThere is high pulmonary vascular resistancehigh pulmonary vascular resistance in CDHin CDH
lungs due tolungs due to
 decreased total arteriolardecreased total arteriolar cross-sectionalcross-sectional area in thearea in the
involved lungsinvolved lungs
 increasedincreased muscularization of the arterialmuscularization of the arterial structuresstructures
that are present.that are present.
 Additional exacerbations of pulmonary vascularAdditional exacerbations of pulmonary vascular
resistance may be induced byresistance may be induced by
 hypoxia, acidosis, hypothermia,and stresshypoxia, acidosis, hypothermia,and stress

DiagnosisDiagnosis
 Scaphoid abdomenScaphoid abdomen and anand an asymmetricasymmetric
distended chest.distended chest.
 The chest may becomeThe chest may become more distendedmore distended asas
patient swallows air.patient swallows air.
 breath soundsbreath sounds may or may not be present onmay or may not be present on
the side of the defect.the side of the defect.
 Mediastinal shift may causeMediastinal shift may cause deviation of thedeviation of the
tracheatrachea
 obstruction to venous return may result inobstruction to venous return may result in
 hypotension and inadequate peripheral perfusion.hypotension and inadequate peripheral perfusion.
CLINICALCLINICAL

DiagnosisDiagnosis
 The signs of respiratory distress may includeThe signs of respiratory distress may include
 cyanosis,cyanosis,
 gasping,gasping,
 sternal retractions, andsternal retractions, and
 poor respiratory effort.poor respiratory effort.
 heart sounds will be heard best over the right chest;heart sounds will be heard best over the right chest;
CLINICALCLINICAL

DiagnosisDiagnosis
 Prenatal USG examination is accurate in 40%toPrenatal USG examination is accurate in 40%to
90%of cases.90%of cases.
 the mean gestational age at discovery is 24the mean gestational age at discovery is 24
weeksweeks
 Polyhydramnios has been reported present inPolyhydramnios has been reported present in
up to 80% of pregnancies with CDH.up to 80% of pregnancies with CDH.
 Polyhydramnios is due to kinking of thePolyhydramnios is due to kinking of the
gastroesophageal junctiongastroesophageal junction
Prenatal USG

DiagnosisDiagnosis
 USG features of CDH areUSG features of CDH are
 stomach in the fetal thorax at the samestomach in the fetal thorax at the same
cross-sectional level as the heartcross-sectional level as the heart
 absence of the stomach in the abdomenabsence of the stomach in the abdomen
 presence of the liver or other solid viscerapresence of the liver or other solid viscera
in the thoraxin the thorax
Prenatal USG

DiagnosisDiagnosis
 Limitations of USG areLimitations of USG are
 poor acoustic contrast between fetal lung andpoor acoustic contrast between fetal lung and
herniated viscera,herniated viscera,
 position of the fetus, and operator experienceposition of the fetus, and operator experience
Prenatal USG
The diagnosis of CDH may be missed because of intermittent
herniation of abdominal viscera into the thoracic cavity
The diagnosis of CDH may be missed because of intermittent
herniation of abdominal viscera into the thoracic cavity
Furthermore, when the stomach is in a normal abdominal
position, herniated small bowel loops are not easily
distinguishable from lung parenchyma.

USGUSG
 Three-dimensional estimation of the fetal lungThree-dimensional estimation of the fetal lung
volume,volume,
 calculation of the right lung area to thoraciccalculation of the right lung area to thoracic
area ratio,area ratio,
 and calculation of the lung to thoracicand calculation of the lung to thoracic
circumference ratiocircumference ratio
but the lung-to-head ratio has been the most
widely used prognostic indicator

XRCXRC
 demonstratesdemonstrates loops of intestineloops of intestine in the chest.in the chest.
 position of the gastric bubble can be confirmed byposition of the gastric bubble can be confirmed by
placement of anplacement of an orogastric tubeorogastric tube..
 Rarely,Rarely, a contrast studya contrast study of the upper gastrointestinalof the upper gastrointestinal
tract is required.tract is required.
 shifting of theshifting of the cardiac silhouettecardiac silhouette into the contralateralinto the contralateral
thorax.thorax.
Chest radiographs are unreliable for estimatingChest radiographs are unreliable for estimating
the degree of pulmonary hypoplasiathe degree of pulmonary hypoplasia
Echocardiography and both renal and cranial USEchocardiography and both renal and cranial US
scans should be obtainedscans should be obtained

CDHCDH
 10% to 20% of the infants with this defect10% to 20% of the infants with this defect
present later.present later.
 These infants present withThese infants present with
 recurrent mild respiratory illnesses,recurrent mild respiratory illnesses,
 chronic pulmonary disease,chronic pulmonary disease,
 pneumonia,pneumonia,
 effusion,effusion,
 empyema, orempyema, or
 gastric volvulusgastric volvulus

Differential diagnosisDifferential diagnosis
 Eventration of the diaphragm,Eventration of the diaphragm,
 anterior diaphragmatic hernia of Morgagni,anterior diaphragmatic hernia of Morgagni,
 congenital esophageal hiatal hernia,congenital esophageal hiatal hernia,
 congenital cystic disease of the lung, andcongenital cystic disease of the lung, and
 primary agenesis of the lung.primary agenesis of the lung.

Prognostic factorsPrognostic factors
 CDH before 24 weeks’ gestational age wasCDH before 24 weeks’ gestational age was
associated with a high mortality.associated with a high mortality.
 antenatal diagnosis is associated with a worseantenatal diagnosis is associated with a worse
prognosis.prognosis.
 A CDH associated with another significantA CDH associated with another significant
anomaly still has a dismal prognosis. theanomaly still has a dismal prognosis. the
 presence of Polyhydramnios was indicative ofpresence of Polyhydramnios was indicative of
poor survival.poor survival.
 presence of liver herniation may be predictivepresence of liver herniation may be predictive
of the need for extracorporeal membraneof the need for extracorporeal membrane
oxygenation (ECMO) as well as a requirementoxygenation (ECMO) as well as a requirement
for prosthetic patch repair for diaphragmaticfor prosthetic patch repair for diaphragmatic
repair.repair.

CdhCdh
 The position of the stomach has been proposedThe position of the stomach has been proposed
as a prognostic indicator by a number ofas a prognostic indicator by a number of
investigators.investigators.
 right-sided defects have a worse prognosisright-sided defects have a worse prognosis
than those with left-sided defects.than those with left-sided defects.
 LHR less than 1.0 is a bad prognostic factor.LHR less than 1.0 is a bad prognostic factor.
 LHR less than 0.85 is a reliably predicting 100%LHR less than 0.85 is a reliably predicting 100%
mortality.mortality.

 Because LHR changes with gestational age,Because LHR changes with gestational age,
use of the observed-to-expected LHR (O/E-use of the observed-to-expected LHR (O/E-
LHR) has been reported.LHR) has been reported.
 A severe left CDH has been characterized byA severe left CDH has been characterized by
an O/E-LHR of less than 25%.an O/E-LHR of less than 25%.
 Prenatal MRI determines more accurately lungPrenatal MRI determines more accurately lung
volumes in CDH patients.volumes in CDH patients.

 The McGoon and pulmonary artery indices areThe McGoon and pulmonary artery indices are
determined shortly after birth by the followingdetermined shortly after birth by the following
formula:formula:
MGI scores less than 1.31 have been found to be highly
predictive of mortality, while the same has been found for
PAI scores less than 90

 Thus arterial blood gas analysis is theThus arterial blood gas analysis is the
cornerstone for predictive criteria.cornerstone for predictive criteria.
 infants who had high PCO2 levels unresponsiveinfants who had high PCO2 levels unresponsive
to mechanical ventilation did poorlyto mechanical ventilation did poorly
 both preductal and postductal blood gases toboth preductal and postductal blood gases to
assess the degree of right-to-left shunting.assess the degree of right-to-left shunting.

CdhCdh
VI
ventilatory index less than
1000, all patients survived
MVI
All infants died if the MVI
was greater than 80 (should
be <40)
oxygenation
index (OI)
OI less than 6 had a
survival rate of 98%,

 In the absence of substantiated, reproducibleIn the absence of substantiated, reproducible
information regarding prognosis, treatmentinformation regarding prognosis, treatment
continues to be guided by best clinicalcontinues to be guided by best clinical
judgment.judgment.

PFTPFT
 have predictive value in identifying infants whohave predictive value in identifying infants who
might require ECMO therapy as well asmight require ECMO therapy as well as
identifying survivorsidentifying survivors
 preoperative compliance PCpreoperative compliance PC
 tidal volume TVtidal volume TV
 functional residual capacity FRCfunctional residual capacity FRC
 infants did not require ECMO when theirinfants did not require ECMO when their
 PC >0.25 mL/cm H2O/kg and initialPC >0.25 mL/cm H2O/kg and initial
 TV>3.5 mL/kg.TV>3.5 mL/kg.
 An improvement in theAn improvement in the tidal volume of 4 mL/kgtidal volume of 4 mL/kg
after repair correlated with survivalafter repair correlated with survival

Independent predictorsIndependent predictors
 independent predictors includeindependent predictors include
 prenatal diagnosis,prenatal diagnosis,
 birth weight,birth weight,
 low 1- and 5-minute Apgar scores,low 1- and 5-minute Apgar scores,
 score for neonatal acute physiology (SNAP-II),score for neonatal acute physiology (SNAP-II),
 and right-sided defectand right-sided defect

Prenatal carePrenatal care
 The prenatal diagnosis of CDH should beThe prenatal diagnosis of CDH should be
complemented by acomplemented by a careful search for othercareful search for other
congenital anomaliescongenital anomalies
 Pregnancy should be broughtPregnancy should be brought as close asas close as
possible to termpossible to term
 The fetus and mother should be referred to anThe fetus and mother should be referred to an
appropriate tertiary perinatal centerappropriate tertiary perinatal center
 SpontaneousSpontaneous vaginal deliveryvaginal delivery is preferredis preferred
 CDH isCDH is not an indicationnot an indication for elective cesareanfor elective cesarean
section.section.

Antenatal interventionAntenatal intervention
 Fetoscopic Tracheal occlusionFetoscopic Tracheal occlusion resulted in lungresulted in lung
enlargement but did not reverse the pathologicenlargement but did not reverse the pathologic
process associated with pulmonary hypoplasia.process associated with pulmonary hypoplasia.
 FTOFTO did not show improved survival ratesdid not show improved survival rates
 the role ofthe role of antenatal corticosteroidantenatal corticosteroid therapy intherapy in
CDH patientsCDH patients remains undetermined.remains undetermined.

ResuscitationResuscitation
 CDH is a physiologic emergency and not aCDH is a physiologic emergency and not a
surgical emergencysurgical emergency

therapeutic interventions are aimed at governing
pulmonary vascular tone.

 Resuscitation begins withResuscitation begins with endotrachealendotracheal
intubation and NGTintubation and NGT insertion.insertion.
 Ventilation by mask and Ambu bag isVentilation by mask and Ambu bag is
contraindicatedcontraindicated
 Arterial and venous access should be acquiredArterial and venous access should be acquired
through thethrough the umbilicus.umbilicus.

 meticulous attention must be paid tometiculous attention must be paid to
 proper temperature regulation,proper temperature regulation,
 Glucose homeostasis, andGlucose homeostasis, and
 volume statusvolume status
 adequate oxygen delivery.adequate oxygen delivery.

 AnyAny stressful stimulusstressful stimulus can further exacerbatecan further exacerbate
already elevated pulmonary pressuresalready elevated pulmonary pressures
 Infants should be properly sedatedInfants should be properly sedated
 Muscle paralysis isMuscle paralysis is strongly discouragedstrongly discouraged
(Untoward consequences on ventilatory mechanics)(Untoward consequences on ventilatory mechanics)
 Systemic hypotensionSystemic hypotension andand inadequate tissueinadequate tissue
perfusionperfusion may be observed and reversed withmay be observed and reversed with
intravenous fluidintravenous fluid

 Cardiotonic drugsCardiotonic drugs, such as dopamine or, such as dopamine or
dobutamine, may be requireddobutamine, may be required
 excessiveexcessive intravenous hydrationintravenous hydration should beshould be
avoidedavoided (pulmonary edema, loss of compliance)(pulmonary edema, loss of compliance)
 Metabolic acid-base disturbances are usuallyMetabolic acid-base disturbances are usually
related to hypoperfusionrelated to hypoperfusion and should beand should be
correctedcorrected
 SevereSevere hypercapniahypercapnia (PCO2 >70 mmHg)(PCO2 >70 mmHg) should beshould be
managed by changing ventilator strategy.managed by changing ventilator strategy.

 There is no need for a chest tube in theThere is no need for a chest tube in the
absence ofabsence of an active air leakan active air leak,, pneumothoraxpneumothorax, or, or
hemothoraxhemothorax

VentilationVentilation
 pH and PCO2 levels are important in modifyingpH and PCO2 levels are important in modifying
pulmonary vascular tonepulmonary vascular tone
 hyperventilation induced alkalosishyperventilation induced alkalosis compoundscompounds
the pulmonary problems withthe pulmonary problems with serious iatrogenicserious iatrogenic
injury.injury.
 permissive hypercapniapermissive hypercapnia andand spontaneousspontaneous
respirationrespiration has proven to be quite successfulhas proven to be quite successful

Circulatory supportCirculatory support
 Goals areGoals are
 heart rate within normal limits,heart rate within normal limits,
 capillary refill time < 3 s,capillary refill time < 3 s,
 U.O > 1.0 ml/kg/h ,U.O > 1.0 ml/kg/h ,
 lactate concentration < 3 mmol/Llactate concentration < 3 mmol/L

 If hypotension is resistant to fluid therapyIf hypotension is resistant to fluid therapy
inotropic agents should be administeredinotropic agents should be administered
 According to the American guidelines, the safeAccording to the American guidelines, the safe
zone is the use ofzone is the use of dopamine up to 20 μg/kg/mindopamine up to 20 μg/kg/min
and adrenaline up toand adrenaline up to 0.1 μg/kg/min*0.1 μg/kg/min*
*Antonoff MB, Hustead VA, Groth SS, et al.: Protocolized management of infants with congenital
diaphragmatic hernia: effect on survival. J Ped Surg 2011; 46: 39–46.

 Hydrocortisone can be used for hypotensionHydrocortisone can be used for hypotension
resistant to conventional treatmentresistant to conventional treatment **
*Antonoff MB, Hustead VA, Groth SS, et al.: Protocolized management of infants with congenital
diaphragmatic hernia: effect on survival. J Ped Surg 2011; 46: 39–46.

PharmacologyPharmacology
 A broad spectrum of drugs andA broad spectrum of drugs and
antihypertensive agents has been usedantihypertensive agents has been used
 Results were disappointingResults were disappointing
 Drugs currently undergoing clinical evaluationDrugs currently undergoing clinical evaluation
includeinclude
 various calcium channel blockers, prostacyclinvarious calcium channel blockers, prostacyclin
derivatives,derivatives,
 endothelin receptor antagonists, andendothelin receptor antagonists, and
 phosphodiesterase-5 inhibitors such as sildenafilphosphodiesterase-5 inhibitors such as sildenafil
In newborns with PPHN, iNO improves oxygenation and decreases
the need for ECMO (RCT in CDH PPHN did not show beneficial effects.)

Timing of Surgical RepairTiming of Surgical Repair
 Historically, CDH was considered a surgicalHistorically, CDH was considered a surgical
emergencyemergency
 As management techniques for neonatalAs management techniques for neonatal
respiratory failure evolved, a period of medicalrespiratory failure evolved, a period of medical
stabilization andstabilization and delayed surgical Repairdelayed surgical Repair waswas
proposedproposed
 multiple single-institution studies have reportedmultiple single-institution studies have reported
improved survival ratesimproved survival rates with delayed surgerywith delayed surgery

 The optimal timing of operative remainsThe optimal timing of operative remains
undetermined.undetermined.
 Some authors wait till weaningSome authors wait till weaning off mechanicaloff mechanical
ventilationventilation and requiring low ventilator settingsand requiring low ventilator settings
 Some waitSome wait till hypertension has abatedtill hypertension has abated or ator at
least stabilizedleast stabilized

Boloker J, Bateman DA, Wung JT, et al.: Congenital diaphragmatic hernia in 120 infants treated
consecutively with permissive hypercapnoea⁄spontaneous respiration ⁄elective repair. J Pediatr Surg
2002; 37: 357–366
Wung JT, Sahni R, Moffitt ST, et al.: Congenital diaphragmatic hernia: survival treated with
very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg 1995; 30: 406–409.
West KW, Bengston K, Frederick J. Rescorla FJ, et al.: Delayed surgical rep air and ECMO
improves survival in congenital diaplragmatic hernia. Ann Surg 1992; 216: 454–460.

 Criteria for undertaking surgical repair:Criteria for undertaking surgical repair:
 mean arterial blood pressuremean arterial blood pressure normal for thenormal for the
gestational age,gestational age,
 preductal saturation within the limits ofpreductal saturation within the limits of 85 to85 to
95%95% atat FiO2 < 0.5,FiO2 < 0.5,
 lactate concentration belowlactate concentration below 3 mmol L-13 mmol L-1,,
 diuresis abovediuresis above 2 mL kg-1 h-12 mL kg-1 h-1 ..

Surgical managementSurgical management
 In 20% ofIn 20% of patients a hernia sacpatients a hernia sac is present whichis present which
must be excised to minimize chances ofmust be excised to minimize chances of
recurrencerecurrence
 (Coran)(Coran)

 There is controversy in the literature concerning theThere is controversy in the literature concerning the
management of themanagement of the hernial sac.hernial sac.
 Some authors recommend excision of theSome authors recommend excision of the
sac whereas others leave the sac sac whereas others leave the sac in situ*in situ*
Shah, A. and G. Jawaheer, Laparoscopic repair of a diaphragmatic hernia
in a child, using a trans-sternal technique.
Journal of Indian Association of Pediatric Surgeons, 2005. 10(2): p. 97.

 a primary repair witha primary repair with interrupted nonabsorbableinterrupted nonabsorbable
suture material can be performedsuture material can be performed
 If the posterior lip is not well formed, anterior lip canIf the posterior lip is not well formed, anterior lip can
be sutured directly to the body wall.be sutured directly to the body wall.

Large defectsLarge defects
 use of prosthetic material has gaineduse of prosthetic material has gained
widespread acceptance.widespread acceptance.
 A floppy, tension-free diaphragmatic repair canA floppy, tension-free diaphragmatic repair can
be accomplishedbe accomplished
 cone-shaping of the patch can be beneficialcone-shaping of the patch can be beneficial

Type of prosthetic materialType of prosthetic material
 The most common material used in prostheticThe most common material used in prosthetic
patches is polytetrafluoroethylene (PTFE: Gore-patches is polytetrafluoroethylene (PTFE: Gore-
tex)tex)
 Other prosthetic patches includeOther prosthetic patches include
 Dacron,Dacron,
 polypropylene andpolypropylene and
 fluorinated polyesterfluorinated polyester

Prosthetic materialProsthetic material
 complications of patch repairs arecomplications of patch repairs are
 Risk of infectionRisk of infection
 chest restriction,chest restriction,
 chest wall deformitychest wall deformity
 Recurrence (10-50%) patientsRecurrence (10-50%) patients
 SBOSBO
 DislodgementDislodgement

Type of prosthetic materialType of prosthetic material

Autologous graftsAutologous grafts

 abdominal compartment syndromeabdominal compartment syndrome should beshould be
should be prevented (NGT, PUC)should be prevented (NGT, PUC)
 tube thoracostomytube thoracostomy is not indicated except foris not indicated except for
active bleeding or uncontrolled air leak.active bleeding or uncontrolled air leak.
 suction may add to the barotrauma and pulmonarysuction may add to the barotrauma and pulmonary
hypertensionhypertension

Extracorporeal oxygenation of bloodExtracorporeal oxygenation of blood
 The purpose of ECMO is to allow time forThe purpose of ECMO is to allow time for
intrinsic recoveryintrinsic recovery of the lungs and to abateof the lungs and to abate
pulmonary hypertension.pulmonary hypertension.

ECMO criteriaECMO criteria
 inability to maintaininability to maintain pre-ductal SaO2 > 85% or post-pre-ductal SaO2 > 85% or post-
ductal SaO2 > 70%,ductal SaO2 > 70%,
 increased PaCO2 and development ofincreased PaCO2 and development of respiratoryrespiratory
acidosis (pH < 7.15)acidosis (pH < 7.15) despite optimisation of mechanicaldespite optimisation of mechanical
ventilation,ventilation,

 necessity to use thenecessity to use the peak inspiratory pressure >peak inspiratory pressure >
28 cm H2O28 cm H2O or mean airway pressure > 17 cmor mean airway pressure > 17 cm
H2OH2O to achieve saturation > 85%,to achieve saturation > 85%,
 hypoxia with coexisting metabolic acidosishypoxia with coexisting metabolic acidosis
(lactate concentration ≥ 5 mmol L-1, pH <(lactate concentration ≥ 5 mmol L-1, pH <
7.15),7.15),

 hypotension resistant to fluid therapy andhypotension resistant to fluid therapy and
inotropic agents withinotropic agents with decreased diuresis (< 0.5decreased diuresis (< 0.5
mL kg b.w.-1 h-1mL kg b.w.-1 h-1 for at least 12–24 h),for at least 12–24 h),
 thethe oxygenation index (OI)oxygenation index (OI) — mean airway— mean airway
pressure x FiO2 ×100/PaO2) at the level ≥ 40.pressure x FiO2 ×100/PaO2) at the level ≥ 40.

 According to the American guidelines, theAccording to the American guidelines, the
ECMO therapy should be initiated when OI is: >ECMO therapy should be initiated when OI is: >
35 for 30 min, > 30 for 2 h, or > 25 for 4 h.35 for 30 min, > 30 for 2 h, or > 25 for 4 h.

ECMO may not be useful in IrreversibleECMO may not be useful in Irreversible
HypoplasiaHypoplasia
 Irreversible HypoplasiaIrreversible Hypoplasia
 oxygen saturation level <90%oroxygen saturation level <90%or
 a markedly elevated PCO2 levela markedly elevated PCO2 level
unresponsive to any type of ventilatoryunresponsive to any type of ventilatory
interventionintervention

ECMOECMO
 is associated withis associated with hemorrhagic complicationshemorrhagic complications inin
60% of the patients60% of the patients
 <20% of infants<20% of infants are reportedly repaired whileare reportedly repaired while
on ECMO (coagulation problems)on ECMO (coagulation problems)
 Survival rates after surgery on ECMO haveSurvival rates after surgery on ECMO have
varied fromvaried from 43% to 80%43% to 80%

OutcomeOutcome
 Survival rates 60% to 90%Survival rates 60% to 90%
 Late deaths (10%)Late deaths (10%)
 PPH orPPH or
 iatrogenic complicationsiatrogenic complications
risk of death in patients of associated Cardiovascular
Malformations is 3 times more

Pulmonary issuesPulmonary issues
 chronic lung diseasechronic lung disease
 bronchopulmonary dysplasia,bronchopulmonary dysplasia,
 reactive airway diseasereactive airway disease
 pulmonary hypertension, and pneumoniapulmonary hypertension, and pneumonia
Significant improvements in lung function become evident
during the first year of life

Pulmonary issuesPulmonary issues
 obstructive and restrictive ventilatoryobstructive and restrictive ventilatory
impairments are presentimpairments are present in up to 50%in up to 50% ofof
survivorssurvivors
 The risk ofThe risk of pneumoniapneumonia is high (35%)is high (35%)
 ViralViral bronchiolitis,bronchiolitis, (particularly(particularly RSVRSV), is a), is a
concern in the first 3 years of lifeconcern in the first 3 years of life

Neurodevelopmental abnormalitiesNeurodevelopmental abnormalities
 Progressive sensorineural hearing lossProgressive sensorineural hearing loss
 Cause not knownCause not known
 AminoglycosidesAminoglycosides
 FurosemideFurosemide
 visual disturbances,visual disturbances,
 seizuresseizures

Gastrointestinal conditionsGastrointestinal conditions
 GERD is the most significant (80%)GERD is the most significant (80%)
 Antireflux surgery is required in 15% to 35% of casesAntireflux surgery is required in 15% to 35% of cases
 Nutritional and growth-related problems have been foundNutritional and growth-related problems have been found
in a significant number of survivorsin a significant number of survivors

Other issuesOther issues
 Chest wall deformities and ScoliosisChest wall deformities and Scoliosis
 Recurrent DHRecurrent DH
 SBOSBO
 ChylothoraxChylothorax

Future therapiesFuture therapies
 Fetoscopic Tracheal Occlusion:Fetoscopic Tracheal Occlusion:
 Induces lung growthInduces lung growth
 decrease in type II pneumocytesdecrease in type II pneumocytes
 tracheomegaly.tracheomegaly.
The role of FTO in the treatment of CDH remains
experimental and unproven

Liquid ventilation techniquesLiquid ventilation techniques
 Perfluorocarbon administrationPerfluorocarbon administration
 pulmonary compliancepulmonary compliance
 lung growthlung growth
Extensive studies are required

Good luck to all ofGood luck to all of
our children, and toour children, and to
their operatingtheir operating
surgeons.surgeons.
Thank You…Thank You…
drfaheemandrabi@gmail.com

Congenital Diaphragmatic Hernia

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