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

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

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

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.

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.

COMPREHENSIVE COUNSELLING

INITIAL PRENATAL EVALUATION

OBSTRETIC ULTRASOUND

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FETAL ECHOCARDIOGRAPHY

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FETAL MAGNETIC RESONANCE IMAGING ( fetal MRI )

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AMNIOCENTESIS

OBSTRETIC ULTRASOUND

Exclude alternate diagnosis

Location & volume of contents of hernia

Evaluate for assosciated congenital anomalies

integrated prenatal and postnatal treatment plan.

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

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

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

PREDICTORS OF

PULMONARY HYPOPLASIA

Name points to a defect in diaphragm

But clinical relevance is abnormal lung development

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

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

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

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.

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

• 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

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

PERCENT PREDICTED LUNG

VOLUME ( PPLV )

Total lung volume

_________________________

expected lung volume

ELV : ( Total thoracic volume - total mediastinal volum )

PPLV < 15% indicates poor prognosis

PREDICTORS OF POSTNATAL

PULMONARY HYPERTENSION

MODIFIED McGOON INDEX

MGI = RPA d + LPA d / AORTA d

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

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

Comprehensive counselling

Obstretic ultrasound

Fetal echocardiography

Fetal MRI

Amniocentesis

MFM specialist

Pediatric surgeon

Neonatologist

Pediatric cardiologist

Pediatric radiologist

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

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

FETO : fetoscopic endotracheal occlusionNorth American fetal therapy network ( NAFTNET)

O/E LHR ( left sided CDH < 25% right sided CDH < 45% )

Thank you