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CONGENITAL
DIAPHRAGMATIC HERNIA
DR.A.NARENDRA KUMAR M.S, Mch
PROFESSOR OF PEDIATRIC SURGERY
NILOUFER HOSPITAL FOR WOMEN & CHILDREN
HYDERABAD
WHAT DO YOU NEED TO KNOW TO
COUNSEL
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CONGENITAL DIAPHRAGMATIC HERNIA
1 in 3000 live births.
Lung hypoplasia and pulmonary
hypertension.
10% risk of intrauterine fetal
demise after 30 weeks gestation
Assosciated anomalies ( 30% )
CDH are diagnosed at 20 W
Fetal MRI has a useful role
Fetal echocardiogram, detailed
anomalous scan & amniocentesis.
Not a single clinical entity
Outcomes are diverse
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SPECTRUM OF DIAPHRAGMATIC DEFECTSSmall aperture in posterior rim to complete absence of diaphragm
POSTEROLATERAL DEFECT SLIT LIKE DEFECT ABSENCE OF HEMIDIAPHRAGM
ABSENCE OF POSTERIOR RIMCDH WITH SAC
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Advances in management of CDH
Prenatal diagnosis
Prenatal steroid therapy
Advances in neonatal care
Surfactant therapy
Conventional mechanical ventilation
High frequency oscillation (HFO)
Inhaled nitric oxide therapy (iNO)
Extra corporeal membrane oxygenation therapy ( ECMO)
Minimal access surgery
Clark RH, Hardin WD, Hirschl Jr RB, Jaksic T, Lally KP, Langham MR, et al. Current surgical management of congenital
diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 1998; 33: 1004-
1009.
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Controversies
The optimal management of CDH remains unsolved.
Different patients have different degrees of pulmonary hypoplasia and
pulmonary hypertension
Hypoplastic lung not only in the ipsilateral but also in the
contralateral lung of infants born with CDH.
Role of prenatal steroids and postnatal surfactant ?
Prolonged stay in hospital requiring multidisciplinary support
Wung JT, Sahni R, Moffitt ST, Lipsitz E, Stolar CJH. Congenital diaphragmatic hernia: survival treated with very delayed
surgery, spontaneous respiration, and no chest tube. J Pediatr Surg 1995; 30: 406-409.
Integrated multi-disciplinary care across prenatal /Perinatal / postnatal /
postoperative till adolescence.
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COMPREHENSIVE COUNSELLING
INITIAL PRENATAL EVALUATION
OBSTRETIC ULTRASOUND
__________________________________
FETAL ECHOCARDIOGRAPHY
__________________________________
FETAL MAGNETIC RESONANCE IMAGING ( fetal MRI )
__________________________________
AMNIOCENTESIS
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OBSTRETIC ULTRASOUND
Exclude alternate diagnosis
Location & volume of contents of hernia
Evaluate for assosciated congenital anomalies
integrated prenatal and postnatal treatment plan.
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DIFFERENTIAL DIAGNOSIS
Pulmonary agenesis
Pulmonary hypoplasia
Pericardial teratoma
Congenital pulmonary airway malformations ( CPAM)
Bronchopulmonary sequestrations (BPS)
Mediastinal lymphatic malformations
Bronchogenic cyst
Neuroenteric cyst
Congenital Lobar Emphysema
BRONCHOGENIC CYST
Sequestration of lung
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OBSTRETIC ULTRASOUND
Fetal growth
small for gestation
Amount of amniotic fluid
Herniated stomach can result in polyhydramnios
Evaluate anatomy for assosciated anomalies
Potential congenital anomalies –cardiac / renal /CNS / GIT
Hydrops
Pleural fluid, pericardial fluid, ascites, skin edema,
placentomegaly
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OBSTRETIC ULTRASOUND
LHR ( Lung-Head Ratio)
Length x width of the lung, opposite to the side of CDH, divided by head
circumference : L X W / HC
Observed –Expected LHR
Observed to expected LHR
Modified McGoon index ( MMI )
Diameter of left pulmonary artery + right pulmonary artery divided by
aorta at the level expected diaphragm : LPA+ RPA / AORTA
Right Vs Left sided CDH
Right CDH carries worse prognosis
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PREDICTORS OF
PULMONARY HYPOPLASIA
Name points to a defect in diaphragm
But clinical relevance is abnormal lung development
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LUNG –HEAD RATIO ( LHR )
Length X width of contralateral lung divided by head
circumference : L x W / HC
LHR : > 1.4 Mild pulmonary hypoplasia
LHR : 1 -1.4 Moderate pulmonary hypoplasia
LHR : < 1 severe pulmonary hypoplasia
22 – 32 weeks of gestation
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O/E LUNG-HEAD RATIO ( LHR)
The observed-to-expected lung-to-head ratio (O/E LHR)
may be calculated and correlates with the degree
of pulmonary hypoplasia.
O/E LHR < 15% (extreme pulmonary hypoplasia):
virtually no chance of survival
O/E LHR 15-25% (severe pulmonary hypoplasia):
predicted survival ≈ 15%
O/E LHR 26-35% (moderate pulmonary hypoplasia):
predicted survival 30-75%
O/E LHR > 35% (mild pulmonary hypoplasia): very likely
to survive
18-38 weeks of gestation
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FETAL ECHOCARDIOGRAPHY
Cardiac position / structure / function
Combination of congenital heart disease and CDH
drastically increases mortality
Modified McGoon index
Obtained to evaluate risk for pulmonary hypertension
LPA + RPA / AORTA at the level of the expected diaphragm
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Foetal MRI-
HASTE sequence
Half-Fourier Acquisition Single-shot Turbo spin-
Echo (HASTE) sequences
• MRI accurately diagnoses CDH and can differentiate it from other chest
masses.
• MRI was superior to U/S in demonstrating the position of the fetal liver
above or below the diaphragm.
• MRI reliably visualized fetal lung tissue and vascular structures.
• These findings are important for counseling parents, selecting fetal surgical
candidates, and estimating prognosis.
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FETAL MAGNETIC RESONANCE
IMAGING ( fetal MRI )
Typically obtained at 24 weeks
Another done at 34 weeks
TFLV : Total fetal lung volume
O/E TFLV : observed to expected total fetal lung volume
PPLV : Percent predicted lung volume
MMI : Modified McGoon index
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• MRI allows the measurement of fetal
lung volumes which provide an estimate
of the severity of pulmonary hypoplasia.
• The total fetal lung volume (TFLV) can
be calculated from a contiguous T2-
weighted HASTE sequence and an
observed-to-expected TFLV (O/E TFLV)
derived.
• It has been found to predict well both
mortality and morbidity, including the
need for ECMO
FETAL LUNG VOLUMES
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TOTAL LUNG VOLUME ( LATE )
By 34 wk fetal lung growth should be complete
Fetal MRI to estimate total lung volume
Summation of the right & left lung volumes
TLV < 20 ml at 34 weeks indicates a poor prognosis
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PERCENT PREDICTED LUNG
VOLUME ( PPLV )
Total lung volume
_________________________
expected lung volume
ELV : ( Total thoracic volume - total mediastinal volum )
PPLV < 15% indicates poor prognosis
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PREDICTORS OF POSTNATAL
PULMONARY HYPERTENSION
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MODIFIED McGOON INDEX
MGI = RPA d + LPA d / AORTA d
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CDH Prognostic profile Early gestational age at diagnosis
Type of CDH
Volume of herniated contents
unilateral/ bilateral
Congenital heart disease
Intra thoracic liver
polyhydramnios
Karyotype
Liver / Stomach position
LHR : > 1 ( 100% survival) <1 ( 50% survival )
O/E LHR : < 25 % ( 20% survival )
PPLV ( early & late ) < 15% high risk > 25% low risk
TLV : > 25 ml favourable . < 18ml unfavourable
Modified McGoon index : < 0.8 severe pul hypertension
>1 mild pulm hypertension
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CDH-Congenital Prognostic Index (CPI)
The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital
diaphragmatic hernia
Louis D. Lea,b, Sundeep G. Keswania,b, Jacek Biesiadac, Foong-Yen Lima,b, Paul S. Kingmad, Beth E. Habermand,
Jason Frischerb, Mounira Hablia,b, Timothy M. Crombleholmea,b,e,low asterisk,'Correspondence information about
the author Timothy M. Crombleholme
January 2012Volume 47, Issue 1, Pages 57–62
four groups of prognostic parameters : Genetic / cardiac/ hernia/ lung
10 different prognostic parameters
Genetic : normal karyotype/ abnormal karyotype. Syndromic/ non-syndromic
Cardiac : no CHD/ minor CHD / severe CHD
Normal LV/RV ratio / disproportionate LV/RV ratio.
Modified Mcgoon Index
Hernia : liver position / presence –absence of sac
Lung: LHR / PPLV / TLV
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Comprehensive counselling
Obstretic ultrasound
Fetal echocardiography
Fetal MRI
Amniocentesis
MFM specialist
Pediatric surgeon
Neonatologist
Pediatric cardiologist
Pediatric radiologist
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WHEN
Scheduled delivery at full term (37-38 weeks)
WHERE
Infants with antenatally-diagnosed CDH should be delivered in
centers that could offer maximal neonatal support.
Close to level 3 NICU with acess to PS and ECMO
HOW TO DELIVER
Based on obstretic considerations
HOW TO MANAGE POSTNATALLY
Paralysed and intubated , kept initially on conventional
ventilation
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Post surgical
Risks to discuss with families
Death 5-20%
Recurrence of CDH 5-20%
Longterm O2 supplementation 10%
Ventilatory Dependence 10%
Neurological problems 5-20%
GERD 75%
Reactive Airway Disease 10-50%
Chestwall Deformities 5%
Failure to thrive 10%
G-tube dependence 10%
Intestina obstruction 5-10%
Hearing loss 1%
Courtesy : American Pediatric surgical assosciation –prenatal counselling series -2017
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FETO : fetoscopic endotracheal occlusionNorth American fetal therapy network ( NAFTNET)
O/E LHR ( left sided CDH < 25% right sided CDH < 45% )
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Thank you