Non-immune Hydrops Fetalis

August 17, 2011

Generalized skin thickening of more than 5mm and / or two or more of the following • Pericardial Effusion • Pleural Effusion • Ascites • Placental Enlargement

Non-immune Hydrops Fetalis
Asim Iqbal
Dept. Obstetrics & Gynecology
Nishtar Hospital, Multan, Pakistan.


Presence of excess extra-cellular fluid in two or more sites without any identifiable circulating antibody to red cell antigens


Generalized skin thickening of more than 5mm and / or two or more of the following
• Pericardial Effusion
• Pleural Effusion
• Ascites
• Placental Enlargement


Normal four Chamber Cardiac View


Pericardial Effusion


Body wall edema in a hydropic fetus


Fetal Ascites


Fetal Ascites


Fetal Ascites


Hydrocele can be an early manifestation in hydrops


Placenta of Hydropic Pregnancy
Placenta of Normal Pregnancy


Soft Tissue shadow and pleural effusion in hydropic neonate

Incidence
5 - 8 per 10,000
This data represents the figure of live born hydropic neonates admitted in neonatology units. Actual incidence is much higher, since majority die either in-utero or the pregnancies are terminated electively.

Pathogenesis
• It is an end result of an array of disorders of the fetus, umbilical cord and placenta that leads to deranged fluid homeostasis.
• A wide range of fetal organs are involved - No common mechanism is responsible for the signs of hydrops.

Pathogenesis
Three main hypothesis:
• Anemia
• Cardiac Failure
• Reduction in Osmotic Pressure (Hypoproteinaemia)

Pathogenesis
Fetal Anemia:
• High output cardiac failure
• Increase in umbilical venous pressure
• Portal hypertension in severely effected fetuses due to increase in hepatic erythropoetic tissue
• Hypoxia and acidosis predispose to epithelial damage in capillaries that ...

Pathogenesis
Fetal Anemia:
• Alpha (α) Thalasaemia
• Secondary to Feto-maternal Hemorrhage
• Twin-twin transfusion
• Other Hemoglobinopathies

Pathogenesis
Cardiac Failure:
• Commonest Mechanism
• Increase in Cardiac Size
• Increased Fetal Venous Pressure

Pathogenesis
Cardiac Failure:
Disorders of cardiac function / Structure include
• Cardiomyopathies
• Tachyarythmias
• Bradycardias (congenital heart block)
• Obstructive left heart disease
• Ebstien's anomaly
• Atrial isomerism

Pathogenesis
Reduced Osmotic pressure:
Hypoproteiaemia with Subsequent Reduction in Osmotic Pressure
• Anemia Along With Destruction of Hepatic Architecture
• Congenital Nephrosis
Hypoproteiaemia may be the result of loss of proteins from circulation, rather than the cause.

Pathogenesis
Other:
Obstruction to venous return
   • Congenital cystic adenomatoid malformation of lung
Impaired lymphatic drainage
   • Cystic hygroma
   • Karyotypic abnormalities (45XO)

Pathogenesis
Fetal Infections:
• TORCH
• Parvo Virus B19

Associated Maternal Complications:
• Polyhydramnios
• Pre-mature labour
• Oligohydramnios
• Problems with third stage
In an attempt to compensate for the fetal hypoxia, placenta increases in size and sometimes also penetrate deeper into the myometrium. Thus causes the morbid adherence of placenta and can cause the problems for third stage of labor necessitating the manual removal of Placenta.

Investigations:
• Detailed Ultrasound Scan
• Fetal Echocardiography
• Doppler Blood Flow Studies
• Liquor Volume Assessment (AFI)
• Placental Thickness & Echogenicity

Fetal Blood Sampling:
• Full Blood Counts
• Karyotype
• Blood Gas Analysis
• Virology screening
• Bio-chemical Evaluation
• Umbilical Venous Pressure

Fetal Blood Sampling:
Blood Can be Obtained From Various Sites
• Placental Insertion of Umbilical Cord
• Fetal Insertion of Umbilical Cord
• Intra-hepatic Vein
• Heart
Procedure related fetal loss is 25% in hydropic fetus

Management:
•  Multi-disciplinary
•  Option of Termination of pregnancy
•  Postmortem examination, Photographs & X-rays
•  Therapy should only be commenced in the light of a firm diagnosis
•  Ideal to wait until fetal karyotype is available

Management:
Prenatal Therapy
• Transplacental drug therapy
• Direct fetal drug therapy
• Invasive procedures

Transplacental drug therapy
Drugs are given to the mother and are passed to the fetus through the placenta
• The main conditions which respond to this approach are fetal dysrrhythmias (SVT)
• Once the type of dysrrhythmia is identified, antiarrhythmic agent is given to the mother, with careful monitoring of her ECG & blood levels.
Drugs: Digoxin, Verapamil, Amiadarone, Flecanide.
Careful Maternal & Fetal Monitoring is Essential

Direct fetal drug therapy
Maternal administration of drugs may be ineffective due to:
• Maternal Metabolism
• Maternal Side Effects
• Variable Passage Across Placenta
Routes for direct fetal drug therapy:
• Intraperitoneal
• Intramuscular
• Intravascular

Invasive Procedures
Blood / Albumen Transfusion to Fetus
• Intraperitoneal
• Intravenous
• Umbilical Vein
• Hepatic Vein

Invasive Procedures
Drainage Procedures:
• Large Pleural Effusions
• Ascities

All invasive procedures carry an inherent increased risk of fetal demise or pre-mature labor.

Non-immune Hydrops Fetalis
• Rarely straightforward
• Temptation to deliver the sick fetus before term should be avoided
• Common maternal complications should also be considered like associated polyhydramnios.
• Amniocentesis before delivery makes the delivery easy and less traumatic for both mother & Fetus, and facilitates resuscitation

Obstetric Management
• Most of the times such fetuses are delivered with elective cesarean section.
• No evidence that mode of delivery has a marked effect on outcome.
• Pediatric team should be aware about the nature of fetal problem before delivery as resuscitation is often difficult and adequate senior assistance must be available.
• High incidence of third stage complications should not be overlooked.

Prognosis?
Generally very poor with very high peri-natal mortality


A hydropic neonate under extensive intensive care

Treatment - Conclusion
•  Successful therapy can be Treatment - Conclusion administered in some cases, yet majority are associated with a poor prognosis.
• Appropriate prenatal investigations must be performed to make a correct diagnosis to identify an effected fetus in whom a good outcome may be anticipated.

Acknowledgements:

Basky Director, Fetal Medicine Unit
St.Geroge's Hospital Medical School
Cranmer Terrace
London SW17 0QT
http://www.fetalmedicine.ac.uk/fmu.shtml

Philippe Jeanty, MD, PhD
www.thefetus.net

I am extremely grateful to them for granting me the permission to include some of the ultrasound images from their web sites.