Final Case Presentation File[1]

5/21/2018 Final Case Presentation File[1]

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PRESENTATION

By: Dr. Saba Kalhoro


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HYDROPS

FETALIS


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Table of contents Introduction

Why Hydrops Fetalis is a concern? Types

Epidemiology

Causes of immune hydrops fetalis

Causes of non-immune hydrops fetalis

Pathogenesis

Symptoms

Complications Management

Diagnosis

Treatment


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Introduction:

Hydrops fetalis refers to the presence of two or

more of the following abnormal fetal fluid

collections:

Ascites

Pleural effusion

Pericardial effusion

Skin edema(more than 5mm)


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Why Hydrops Fetalis is a concern?

It has a very poor outcome (>80% mortality),

regardless of the aetiology.

Dewhursts Textbook Of Obstetrics & Gynaecology Eighth Edition


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Types:

In 1943, Potter defined two forms ofhydrops fetalis based upon etiology:

Immune-mediated (10 percent )

Non-Immune ( 90 percent )

Potter EL. Universal edema of the fetus unassociated with erythroblastosis.Am J Obstet Gynecol 1943; 46:130.


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Epidemiology:

The reported incidence of NHF ranges from 1

in 1500 to 3800 births. It accounts for more

than 90% of all cases of hydrops.


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Epidemiology...contd

Review from a large national data set from theUnited States:

598 live-born infants with hydrops fetalis

The most common causes of hydrops fetaliswere:

Congenital heart problems (14 percent)

Heart rate abnormalities (10 percent)

Twin to twin transfusions (9 percent)


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Epidemiology...contd

Chromosomal abnormalities (8 percent)

Congenital viral infections (7 percent)

Congenital anemia (5 percent)

Congenital chylothorax (3 percent)

Abrams ME, Meredith KS, Kinnard P, Clark RH. Hydrops fetalis: a retrospective review of cases

reported to a large national database and identification of risk factors associated with death.

Pediatrics 2007; 120:84.


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In Asia, the most common causes of hydrops

fetalis are homozygous alpha thalassemia and

cardiac disease.

Evidence Based Text Book of Obstetrics & Gynaecology - 2ndEdition


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Causes of immune hydrops

fetalis:

Anti-D antibodies

Anti-C antibodies

Anti-Kell antibodies


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RhD iso-immunisation

IgG antibodies to RhD Antigen will persist in the circulation

and the numbers of antibodies can be multiplied hugely if

there is repeated contact with these foreign RhD red cells

IgG Antibodies are small molecules and cross freely into the

placenta

If the fetal cells are RhD positive they are targeted by the

IgG molecules and are destroyed by the fetal Reticullo-

endothelial system (hemolysis) which may leads to anemia


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Causes of non-immune hydrops

fetalis:

Cardiac

Hypoplastic left/right heart

Fetal arrhythmias Premature closure of foramen ovale

Cardiomyopathy

Sacrococcygeal teratoma


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Causes:

Chromosomal abnormalities

Trisomy 13, 18, 21

Turner syndrome


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Causes:

Thoracic anomalies

Diaphragmatic hernia

Pulmonary sequestration

Intrathoracic mass

Bronchogenic cyst


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Causes:

Infections

Viral (Parvovirus B19, Herpes, CMV)

Toxoplasmosis Syphilis


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Causes:

Hematologic

Alpha Thalassemia

Red-cell enzyme deficiencies


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Other causes:

Skeletal conditions

Osteogenesis imperfecta

Chondrodysplasia

Genetic metabolic disease

Gaucher Disease

Mucopolysaccharidosis


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PATHOGENESIS:

Compensatory response activated due to fetalanemia in extramedullary hematopoiesis.

Spleen and liver enlarge in size

Immature red cell called erythroblast enter intocirculation help in O2 carrying to cells.

If this response failed then there is compensatoryplacental hyperplasia


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PATHOGENESIS:contu

In extreme case compensatory response

exceed and baby goes into high cardiac out

put and result in cardiac failure

Result in accumulation of fluid in body cavity

including scalp edema, ascites, pleural andpericardial effusion ( hyfrops fetalis )


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Features of hydrops fetalis :

Depend on the severity of the condition.

Mild forms may cause: Liver swelling

Change in skin color (pallor)

More severe forms may cause: Breathing problems

Bruising on the skin

Heart failure

Severe anemia Severe jaundice

Total body swelling


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Maternal Complications:

Polyhydraminos

Placental abruption

Uterine atony

Premature-labour

Hydropic placenta ( more than 6cm)

Retained placenta

Pre-eclampsia


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Mirror Syndrome:

Mother develops pre-eclampsia along with

severe oedema that is similar to that of thefetus

Caused by vascular changes in the swollenhydropic placenta


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Fetal complications:

Babies born with hydrops are very swollen and

have a large, round abdomen due to the fluid

collection in the abdominal cavity.

Severe respiratory distress Hypoglycemia

Apnea

Anemia Neurological injury and fetal death


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MANAGEMENT OF NONIMMUNE HYDROPIC FETALIS


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Diagnosis & Investigations for non

immune

Ultrasound - Several etiologies are confirmed or

excluded based upon ultrasound findings

twin-to-twin transfusion

cardiac arrhythmias

structural anomalies

Ultrasound doppler

Fetal echo Amniocentesis

Maternal thyroid antibodies


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Diagnosis & Investigations for non

immune

Viral serology.

Assessment of maternal blood type


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Treatment of non immune :

Fetal anemia

Fetal arrythmia

Intrinsic thoracicmalformations

Fetal blood

sampling followedby in uterotransfusion.

Medications such as

digoxin, propanolol. Thoracentesis or

thoracoamnioticshunt for pleural

effusions.

Causes Treatment


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Treatment of non immune

Twin to twin

transfusion

syphilis

Fetoscopic laser

ablation of

communicating

vessels

penicillin


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Management:

Antepartum

If treatment has been successful or hydrops is

resolving spontaneously, the fetus may be

followed with repeat sonograms every 1 to 2weeks and antenatal testing.

Consultation with the neonatologist

Signs of "mirror" syndrome.


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Management:

Delivery

In tertiary care center with neonatologists and

other appropriate specialists.

Delivery by caesarean section has no marked

effect on outcome.

Cord blood should be obtained at delivery


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Counselling and fetal outcome:

Long term prognosis and severity of

the disease.

Prognosis is much poorer if diagnosed at less

than 24 weeks , pleural effusion is present, or

structural abnormalities are present, option of

termination of pregnancy may be aconsideration.

C lli d f t l


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Counselling and fetal

outcomecontd

In survivors of hydrops fetalis, poor

neurodevelopment outcome appears to be themost significant morbidity.

Nakayama H, Kukita J, Hikino S, et al. Long-term outcome of 51 liveborn neonates with non-immune

hydrops fetalis. Acta Paediatr 1999; 88:24.


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Immune Hydrops Fetalis

Two major problem :

1) Fetal anemia

2) Hyperbilirubinemai

I ti ti f hD


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Investigation of rhD

immunized

Anti D Anitibodies level :shows risk offetal hemolysis

1) Indirect coombs test : >1:16 titer

2)Immunoassay measure : maternal serum

level shows ; 10iu/ml------ moderate

>30iu/ml------ sever

I ti ti f RhD


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Investigations for RhD

immunized

Fetal blood group1) Paternal genotype : RhD +is 100% in

homozygous

2)Invasive procedure :

-fetal blood sampling :umbilical cord at placentalinsertion site is most common site for FBS

-Free fetal DNA :The maternal blood can now betested to determine the fetal Rh status

NICE GUIDE LINE 2008

nvas ve an on- nvas ve


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nvas ve an on- nvas veMethods

of assessing for fetal anaemia Doppler ultrasound : Middle Cerebral Artery

Amniocentesis for bilirubin level (indirect

measurement of fetal haemolysis)

Cordocentesis ( direct Hb measurement and

transfusion if needed)

How does blood flow in the Middle


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How does blood flow in the Middle

Cerebral Artery alter in an anaemic

fetus

Fetuses suffering from hypoxia will preferentially divertblood to the brain

Anaemia will result in a low blood viscosity and a higher

cardiac output

All blood vessels in the fetus will shows high blood

velocities but the Middle Cerebral Artery shows this to

exaggerated effect(peak systolic blood flow )


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MANAGEMENT OF IMMUNEHYDROPIC FETALIS

D d Ti i f A ti D


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Dose and Timing of Anti-D

Immunoglobulin

Routine Antenatal Anti-D Prophylaxis

Anti-D should be administered at 28 and 34 weeks ofpregnancy ( 500iU im)

OR Single dose regime

1500 iU im at 28-30 weeks

A KLEIHAUER BETKE test. Fetomaternaltransfusion (calculate 100iu of antiD antibodies cannuterlize 1ml of fetal blood ) should be performedafter delivery

After delivery within 72 hr anti D 500IU imNICE GUIDE LINE JUNE 2008


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Management of immune

MODE OF DELIVERY :

In moderate to severely affected babies < 37 week

gestational age caesarean section .. more prone todevelop hypoxia during labour and due to largesize of bay and placenta .

If mild anemia labour may be induced after 37week by vaginal delivery

Precaution after delivery of


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Precaution after delivery of

baby

Once baby delivered transfusion of fetal blood

from placenta into maternal circulation should

be reduced by

early cord clamping Avoiding milking of umbilical cord toward fetus

Maintaining the fetal level at , or above the

maternal level Avoid manual removal of placenta


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Recurrence:

The risk of recurrence depends upon the

underlying etiology. The recurrence rate is

greatest in families with infants who have a

chromosomal abnormality.

Etiology and outcome


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Etiology and outcome

of Hydrops fetalisa ten year

experience

Objective

To determine etiology and outcome of hydrops fetalis in a largeIrish tertiary referral centre.

Study Design

All antenatally diagnosed cases of hydrops fetalis from January2000 to January 2010 were studied prospectively

Conclusions

Cardiac cause (most common)

Majority die (Antenatally/Neonatally)

66% with diagnosed Etiology

53% survived who undergone intervention

American Journal of Obstetrics and Gynecology - Volume 204, Issue 1 Suppl 1 (January 2011)


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References

Evidence Based Text Book of Obstetrics & Gynaecology

Dewhursts Text Book of Obstetrics & Gynaecology

Ballantyne JW. The diseases and deformities of the foetus: An attempt

towards a new system of ante-natal pathology. Edinburgh, Oliver & Boyd,

1892.

Potter EL. Universal edema of the fetus unassociated with erythroblastosis.

Am J Obstet Gynecol 1943; 46:130.

Non-immunological hydrops fetalis. J Obstet Gynaecol Br Commonw 1970;

77:226.

Clark RH. Hydrops fetalis: a retrospective review of cases reported to a

large national database and identification of risk factors associated withdeath. Pediatrics 2007; 120:84.

American Journal of Obstetrics and Gynecology - Volume 204, Issue 1

Suppl 1 (January, 2011)


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THANK YOU

Documents

Final Case Presentation File[1]