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MRCOG PART 2 SBAs and EMQs

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ESSAY 162 - FETAL HYDROPS

Posted by javeria F.
Various investigations to identify the cause of fetal hydrops in this lady can be divided into maternal and fetal tests. Maternal history is taken including exposure to possible teratogens, any history of infections, previous fetal hydrops and neonatal jaundice, past medical history and family history of metabolic disorders. This is important as teratogens like alcohol, infections like TORCH, medical disorders like diabetes and anemia and certain genetic metabolic disorders are important cause of hydrops. Ethnic origin of the mother is also important. Regarding examination, abdominal examination is done to check if the baby is large for dates or any hydramnios present. Maternal blood tests include full blood count and film, maternal serum alphafetoprotien, viral screen (booking serum can be tested for antibodies against syphilis, cytomegalovirus, toxoplasmosis, herpes simplex, respiratory syncytial virus and HPV B19). It also helps to compare early pregnancy samples. Hemoglobin electrophoresis, kleihauer betke test, glucose screen, anti Ro and anti La antibodies help in identifying a cause if congenital heart block is present.
Regarding fetal investigations, detailed fetal ultrasound is done to detect any fetal anomalies for example skeletal dysplasias, genitourinary, gastrointestinal and cord abnormalities, all important causes of hydrops. Fetal echocardiography and cardiac Doppler studies are done as cardiac abnormalities can cause hydrops. Further investigations like invasive prenatal diagnosis should be carried out in fetal medicine unit as expert interdisciplinary management. These may include cordocentesis for karyotyping, viral screen (TORCH and HPV B19), urea and electrolytes, full blood count, blood film and liver function tests. Alternative investigation is amniocentesis for viral culture and karyotyping.
Factors that are important in determining prognosis include cause of hydrops, gestational age at the time of diagnosis, availability of fetal medicine unit, SCBU and multidisciplinary care. Earlier gestational age at the time of diagnosis is usually not associated with favourable outcome. Certain causes like chromosomal abnormalities are also lethal associated with bad prognosis whereas infection related for example parvovirus related may resolve, hence good prognosis. Certain cardiac abnormalities can be treated and lot more depends on availability of multidisciplinary care.
Posted by Sheetal G.
In order to investigate this case maternal blood needs to be checked for various red blood cell antibodies like anti-c and anti kell antibodies which are common non D antibodies causing hydrops fetalis. Maternal blood should also be checked for Thallasemia as it is transmitted in autosomal recessive manner. It is important to check blood for IgG and IgM antibodies against CMV, Toxoplasma and pravovirus and if necessary viral culture as all these can lead to hydrops and the antibodies can be present in the absence of definite history of viral infection. Maternal blood should also be checked for SLE as it can lead to congenital heart block and hydrops secondary to cardiac failure
Detailed anomaly scan is important as presence of multiple anomalies can indicate chromosomal anomaly which is one of the commonest cause of non immune hydrops. Detailed cardiac scan and echocardiography is valuable as structural cardiac anomalies can lead to hydrops and cardiac arrythmias and congenital heart blocks are important causes of hydrops which can be present in 10 to 15% of cases of non immune hydrops. Detailed scan if also important to look for pulmonary malformations and diaphragmatic hernias as it can obstruct cardiac return and can lead to hydrops.Amniocentesis of placental biopsy should be offered to check for fetal karyotyping as various aneuploidies and trisomies can lead to hydrops. Amniotic fluid can be used to do viral culture and to check antibodies for CMV, Toxoplasma, parovirus and the level of bilirubin if alloimmunisation is suspected. Fetal blood sampling can be useful to diagnose thallasemia, viral infection and to check fetal Hematocrit, bilirubin level and red blood cell antigens. Parvovirus B19 and Kell alloimmunisation can cause fetal anaemia without significant elevation of bilirubin level.
Various factors influencing outcome of hydrops are the underlying cause , gestation at the diagnosis of hydrops and degree of severity of hydrops. Outcome also depend on how readily diagnosis is reached and treatment is available and it also depends on whether the hydrops is reversible upon treatment or not.
Early diagnosis of hydrops when it is mild and its treatment leading to successful reversion of changes is associated with better outcome. The earlier the gestation at diagnosis of hydrops poorer is the outcome. Fetal arrhythmias as a course can have better outcome if treated with maternal digoxin therapy. Alloimmunisation and Parvovirus infection can also be successfully managed with cordocentesis and fetal blood transfusions. outcome is worse for chromosomal disorders and congenital infections other than parvovirus .
Posted by uma M.
A 34 year old Rhesus positive woman is found to have a hydropic fetus during the anomaly scan at 22 weeks gestation. Evaluate the investigations that will be necessary to identify the cause of hydrops . Which factors are important in determining prognosis?


Fetal hydrops is caused by variety of maternal and fetel conditions.Various investigations are carried out to identify the cause of hydrops .
Possible causes for this condition include Fetal infections , structural anomaly, chromosomal anomaly, haemoglobinopathy, metabolic disorder, chronic feto-maternal haemorrhage, antibodies other than rhesus positive antibodies.15- 30% idiopathic.

HISTORY of Previous baby with hydrops , Ethnic origin , Family history / personal history of metabolic disorder , History / contact with infection may give a clue to diagnosis.

INVESTIGATIONS performed help identify the cause of hydrops, aid us give prognosis to the woman, and explain recurrence risk.Depending on the cause further management in this pregnancy is planned . (Either TOP/ Treatment)
MATERNAL : FBC, Hb electrophoresis for evidence of any thalasemias is a simple test,red blood cell antibodies like anti-c and anti kell antibodies which are common non D antibodies causing hydrops fetalis, Infection screen as 8-9% of hydrops are due to infections. and history of infections is mostr often unreliable.Test for toxoplasmosis, rubella, CMV, parvovirus, syphilis, coxsackie virus. Review early pregnancy results /booking sera samples and note if any seroconversion.Test for both Ig G,Ig M OR PCR test for diagnosis . Kleihauer betke test for any chronic fetomaternal haemorrhage, Lupus anti-coagulant and anti-Ro antibodies AS THESE CAUSE FETAL HEART BLOCK AND cardiac failure.
FETAL: Detailed scan for anomalies ,including cardiac scan ,with fetal echocardiogram is essential. Multiple anomalies suggest some chromosomal abnormality.Cardiac scan is esspecially important as 1/3 of cases have cardiac abn either structural or rhythm abnormalities.Ohter anomalies assosiated with hydrops include diaphragmatic hernia, cong cystic adenomatoid malformations , GI atresias etc.This should be performed in tertiary centre with expertise in fetal medicine. Not all anomalies can be made out on USG.
Amniocentesis or cordocentesis for Karyotype as 10-20% of hydrops is related to chromosomal abnormalities( like trisomies 18,13,21, aneuploides).Cordocentesis is preferrable as other investigations on fetal blood can be carried out ,whic might not be possible with amnio.But cordocentesis is assossiated with fetal loss rate of 3% vs1% for amnio, also cordocentesis is technically more demanding needs expertise, and not widely available.If any treatment is necessary is given in the same sitting.Karyotyping is also rapid (48hrs)with cordocentesis.
Hb and electrophoresis FOR THALASSEMIAS, group and Coombs? test,Haematocrit as anti Kell antibodies and Parvovirus B19 infection causes anaemia. Bilirubin levels as it may be raised in alloimmunisation , protein, infection screen by PCR or for presence of IgM antibodies.

Prognosis is dependent on underlying cause , severity of hydrops.
If Underlying cause is fetal anaemia secondary to parvovirus infection this may resolve spontaneously or may require intra-uterine transfusion .Medical therapy with digoxin for fetal arrhythmias improve outcome.Alloimmunisation can also be successfully managed with cordocentesis and fetal blood transfusions.
Drainage of effusions is not always helpful but may be necessary to enable vaginal delivery , may also imprave outcome in certain situations like structural abnormalities which may be corrected after delivery.
chromosomal disorders carry worse prognosis.
If idiopathic or secondary to infection, recurrence risk is low

Posted by Nitin P.
Fetal Hydrops is defined as the presence of serous fluid in two or more body cavities and accumulation in the subcutaneous space. It is associated with significant perinatal morbidity and mortality 50 to 70 %.
a) It is important to identify the cause as the prognosis is dependent on the cause.
However, commonest cause is idiopathic. Investigations are extensive and psychologically demanding after such a diagnosis. Ideally should be done at a tertiary feto maternal medicine unit. Some investigations may not be available in time to help with the management.
Maternal investigations are non invasive and with no risk to an already affected fetus. Whereas the mainstay of fetal investigation is invasive investigations with risk of fetal miscarriage and infection, in addition to maternal discomfort and pain.
Maternal perception to investigations should be considered before advising the investigations. Cost effectiveness, in view of the generally poor prognosis and the fact that most cases are idiopathic is also important. However, genetic syndromes, inborn errors, chromosomal anomalies and transmissible genetic conditions like Glucose 6 phosphate dehydrogenase deficiency have a risk of recurrence and hence help for future counselling and management.
The maternal investigations include haemoglobin estimation and liver function tests to determine severe anaemia and hypoalbuminaemia. Glucose tolerance test to rule out gestational diabetes.
Kleihauer test to determine feto maternal haemorrhage (FMH) as severe FMH can cause hydrops. Atypical antibodies with titre levels is also needed as they can cause immune mediated hydrops in Rhesus positive cases.
Haemoglobin electrophoresis is done to determine the risk of alpha thalassaemia in the fetus. Glucose 6 phoshpate dehydrogenase is checked in the parents.
Maternal serum is checked for infections including toxoplasma and syphillis and virology i.e. CMV, Rubella and Parvovirus B 19.
Symptoms of SLE and antiphospholipid antibodies warrants the need for Lupus Anticoagulant and Anti-Rho antibodies as these can cause dysrrhythmias in the fetus leading to hydrops.
Inborn errors of metabolism are a rare cause of fetal hydrops but need to be considered. In cases of suspected structural fetal anomalies, Maternal serum Alpha feto protein levels are checked to rule out sacro coccygeal teratoma and congenital adenomatous malformation of the lung.
Fetal investigations include a detailed scan to determine the presence of structural anomalies. Difficult in the presence of excessive liquour. Cardiac scanning with M mode with fetal cardiologist is advised to check for structural anomalies and dysrrhythmias. Repeat scanning advised as the dysrrhythmias may not be present continuously.
In nearly all cases, fetal karyotype is needed to rule out chromosomal anomalies. This can be done by amniocentesis or cord blood sampling. Cord blood offers advantage of checking for fetal anaemia, group and Coomb?s test and rapid karyotype as compared to amniocentesis. Risks of miscarriage depend upon the primary condition and are higher than the 2-3% in normal cases. This is higher than the risk of amniocentesis which is 0.5% to 1%.
Amniotic fluid or fetal blood should be checked for infections i.e. parvovirus B19, toxoplasma, rubella, CMV. If an inborn error of metabolism is suspected tests for the same may be done on fetal blood. If a specific gene sequence is known, karyotype of amniotic fluid cells suffices.
b) The prognosis depends upon the cause with severe chromosomal anomalies like Trisomy 18 having a poor prognosis. Generally conditions amenable to treatment like fetal dysrrhythmias and parvovirus induced anaemia have a better prognosis. Presentation at an early gestational age carries a poor prognosis as most often the condition is very severe.
Early referral to a tertiary fetomaternal medicine unit improves the prognosis. Preterm labour due to concomitant poly hydramnios also affects the prognosis negatively.

Posted by jyoti D.
The diagnosis in this case is nonimmune hydrops fetalis which is associated with high perinatal morbidity and mortality.
The causes include chromosomal (Turner\'s syndrome,trisomy21,18,13,triploidy).Cardiac causes like tetralogy of fallot,ebsteins anamoly,supraventricular tacycardias.Haematological causes like alfa thalassemia wich in incompatible with life and beta thalasemmia major.Infections like parve virus B19,toxoplasmosis,syphilis,coxsackie,cytomegalovirus,herpes simplex are associated with hydrops fetalis.Cystic hygroma as they are associated with chromosomal abnormalities.Lung pathology in form of adenomatous malformations.Liver problems like biliary atresia.Genitourinary anamolies,skeletal anamolies and idiopathic where cause is not known.
The diagnosis include detailed maternal history about possible exposure to teratogens or medications like indomethacin which cause premature closure of ductus arterosis,ethinicity as certain blood disorders common in mediterran and south east asian communities.History of jaundice or jaundice in previous babies.History of SLE and gluse intolerance .
Maternal investigations include full blood count,urea and electrolytes,antI RO AND ANTILA antibodies ,glucose screen,liver function test fro albumin,renal function test,HB electrophoresis.Infection screen fro TORCH syphilis,parvoviruaB19.
There should be a multidisciplinary involvement including fetal medicain expert and paediatric surgeon and neonatologist and referral to a tertiery center for further evaluation and management.Parents should be counselled after and before every test.Explain the diagnosis and option of termination should be given and depending on her wishes further evaluation should be continued.
The fetal investigations the noninvasive are detailed ultrasound for skeletal anamolies and severity of hydrops .Fetal echocardiographty to rule out any cardiac problems. Invasive investigations like cordocentesis for fetasl blood sampling and send fro full blood count ,urea and electrolytes,blood gases and PH,hb electrophoresis,infection screen ,Karyotyping.
Amnioncntesis after explaining the parents risk of procedure like misscarriage in 1% of cases and culture failure in less then 1% of cases fro viral culture and karyotyping.
In case of sudden fetal death or stillbirth counsel for postmortem examination which will help to know the cause and might be helpful in future pregnancies.
The fetal prognosis even with intrauterine therapy is associaterd with high perinatal mortality and morbidity.
The maternal risk are in the form of abruption related to procedures or hydramnious related.Psychological stress is there through out the preganancy so adequate counselling is a must about maternal and fetal prognosis.
Posted by jyoti D.
The diagnosis in this case is nonimmune hydrops fetalis which is associated with high perinatal morbidity and mortality.
The causes include chromosomal (Turner\'s syndrome,trisomy21,18,13,triploidy).Cardiac causes like tetralogy of fallot,ebsteins anamoly,supraventricular tacycardias.Haematological causes like alfa thalassemia wich in incompatible with life and beta thalasemmia major.Infections like parve virus B19,toxoplasmosis,syphilis,coxsackie,cytomegalovirus,herpes simplex are associated with hydrops fetalis.Cystic hygroma as they are associated with chromosomal abnormalities.Lung pathology in form of adenomatous malformations.Liver problems like biliary atresia.Genitourinary anamolies,skeletal anamolies and idiopathic where cause is not known.
The diagnosis include detailed maternal history about possible exposure to teratogens or medications like indomethacin which cause premature closure of ductus arterosis,ethinicity as certain blood disorders common in mediterran and south east asian communities.History of jaundice or jaundice in previous babies.History of SLE and gluse intolerance .
Maternal investigations include full blood count,urea and electrolytes,antI RO AND ANTILA antibodies ,glucose screen,liver function test fro albumin,renal function test,HB electrophoresis.Infection screen fro TORCH syphilis,parvoviruaB19.
There should be a multidisciplinary involvement including fetal medicain expert and paediatric surgeon and neonatologist and referral to a tertiery center for further evaluation and management.Parents should be counselled after and before every test.Explain the diagnosis and option of termination should be given and depending on her wishes further evaluation should be continued.
The fetal investigations the noninvasive are detailed ultrasound for skeletal anamolies and severity of hydrops .Fetal echocardiographty to rule out any cardiac problems. Invasive investigations like cordocentesis for fetasl blood sampling and send fro full blood count ,urea and electrolytes,blood gases and PH,hb electrophoresis,infection screen ,Karyotyping.
Amnioncntesis after explaining the parents risk of procedure like misscarriage in 1% of cases and culture failure in less then 1% of cases fro viral culture and karyotyping.
In case of sudden fetal death or stillbirth counsel for postmortem examination which will help to know the cause and might be helpful in future pregnancies.
The fetal prognosis even with intrauterine therapy is associaterd with high perinatal mortality and morbidity.
The maternal risk are in the form of abruption related to procedures or hydramnious related.Psychological stress is there through out the preganancy so adequate counselling is a must about maternal and fetal prognosis.
Posted by Farzana N.
The most likely diagnosis in this case is non ?immune hydrops, since the pt is Rh positive. The possibility of antibodies to (other than Rh.., such as) K system or Duffy system still remains.
Investigations required to detect the most likely diagnosis, need to be done both on mother and fetus. The cause is idiopathic in 30% of cases.
Maternal investigations include-FBC for Hb levels. Low levels are found in cases of anemia .U&E, LFTs, uric acid in cases of pre eclampsia. These would reflect the severity of the disease. Blood group and antibodies to K or Duffy system.Hb electrophoresis should be done in cases of selected ethnic groups with high prevalence of alpha-thalassemia, to detect the abnormal Hb.
MSAFP- will be raised in cases of IDDM with a high risk of Downs?s syndrome, Pre eclampsia.Infection screen should be done for evidence of infections which are commonly associated with hydropic changes in the fetus such as,TORCH,
Parvovirus B 19, syphilis, coxackie etc.
GTT should be done to see if the pt has DM.Lupus anti coagulant and Ro antibodies should be done if bradyarrhithmias are seen on cardiac scan or echo.
Fetal investigations include- a detailed anomaly scan for other structural abnormalities associated with hydrops, such as diaphragmatic hernia, severe congenital heart disease, renal anomalies, placental or fetal tumors such as sacrococcygeal teratoma.Due to high risk of associated cardiac anomalies a detailed cardiac scan should be done. Placental or fetal tumors should be excluded, such as sacro coccygeal teratoma.Fetal blood sampling is done for FBC-low Hb levels are found in cases of anemia associated with fetomaternal hemorrhage, twin-twin transfusion syndrome.
Karyotyping is done, since the following chromosomal abnormalities are associated with hydrops-trisomies, the commonest being Downs syndrome. Turner?s syndrome and triploidy.Fetal Hb electrophoresis is done, if required to detect abnormal hemoglobinopathhies associated with hemolytic.
PCR based infection screen or antibodies screening can be done in suspected cases of infection.
B) Prognosis in this case depends upon the cause and severity of hydrops. Chromosomal abnormalities with hydrops carry a poor prognosis and there is high perinatal maortality.Early onset of hydropic changes, before 24wks also have high mortality.
Cases due to parvo virus infection or alloimmunisation can be treated with transfusion.Dys rhythmias can be treated with digoxin.If the hydropic changes are secondary to infections ,the recurrence rate is low.
Posted by Mangala sundari R.
Non immune fetal hydrops are due to maternal or fetal causes., The etiology is idiopathic in 30% of cases.It can be due to maternal infections, hemoglobinopathies, Diabetes mellitus, Severe preeclampsia, hypoproteinemia,or auto immune disease.

Fetal causes are infections, cardio vascular,structural and congenital anomalies, chromosomal anomalies, homozygous alpha or beta thalassemia,.

Maternal blood investigations include ,FBC,TORCH, syphilis(VDRL), parvovirus B19 serology, MSAFP,GTT to r./o diabetes mellitus,G6PD, B thallasemia, electrophoresis ( in some ethnic patients) and lupus anticoagulant and anti R0 antibodies if fetal bradycardia.urea and electrolytes/creatinine if preeclampsia
This patient needs to be referred to fetal medicine specialist .

Ultrasound is the most important diagnostic tool for initial assessment and subsequent investigations and follow up. Fetal investigations include detailed ultrasound, cardiac scan, ECHO,amniocentesis and cordocentesis;

By ultrasound we can diagnose structural anomalies of Cardivascular system ( major Congenital heart disease, PDA), pulmonary hypoplasia, diaphragmatic hernia, renal anomalies, bradycardia, and arrythmias.and soft tissue markers of trisomies.( all anomalies cannot be diagnosed by U/S).
Amnocentesis by ultrasound guidance and submitted for karyotyping, , AFP,TORCh profile for Igg and Igm antobodies, Parvovirus B 19 antibodies and syphilis, CMV,Coxsackie and some inborn errors of metabolism,
Cordocentesis is done to evaluate the fetal anemia and hematocrit, coombs test,antibodies.karyotyping,Doppler study of middle cerebral artery is a reliable indicator of fetal anemia which is non invasive .

Prognosis depends on the cause and severity of the condition..H/o Previous hydrops fetus with an unknown etology is a poor prognostic factor.
Diabetes mellitus , correcting severe anemia and hypoproteinemia have a better prognosis.... In a fetus less than 24 weeks, the prognosis tends to be poor than in the fetus near term...Currently the only cases amenable to fetal therapy are cases due to high output failure as in twin to twin transfuson syndrome, tachyarrythmia. Fetal Cardiac , structural and renal anomalies, large polyhydramnios, severe degree of hydrops with pleural, pericardial and peritoneal effusions have poor prognosis., as also the trisomies.If the fetal hamatocrit is less than 20 ( by cordocentesis), it is a good prognostic value of resolving fetal anemia. RI of middle cerebral artery is a noninvasive method of assessing fetal anemia.
A dedicated feto maternal unit with trained specialists and neonatologists team are important factors.
Posted by Rani M.
(a): Hydrops fetalis leads to significant perinatal mortality& morbidity and maternal distress and morbidity. It is important to investigate this woman to look for a cause, to counsel parents regarding prognosis, recurrence risk and for management plan.
Investigations include maternal and fetal investigations. Maternal investigations and ultrasound should be done prior to invasive fetal investigations.

Though she is Rh. positive immune hydrops can not be ruled out.Maternal blood should be tested for red cell antibodies especially anti kell & fya & Duffy system.
Kleihaur Betke test should be performed as chronic fetomaternal haemorrhage can lead to hydrops even in the abscence of red cell antibodies.
Maternal blood is tested for F.B.C and also Hb. electrophoresis especially in minor ethnic groups ( homozygous alpha thalessemia is the most common cause of hydrops in South East Asians & worldwide ).
Glucose tolerance test is done to look for maternal diabetes. Liver function and renal function tests are done if pre eclampsia is suspected which can be a cause and also may be a complication of hydrops in mother( mirror syndrome).

Infections screening is a must. Infections are responsible in 8-10% of cases.This will include testing for syphilis, toxoplasmosis, CMV, herpes simplex, coxsackie &,human parvo virus B19.

If there is fetal bradycardia or if SLE is suspected anti Ro, anti La antibodies should be tested as can lead to congenital heart block in fetus.

If there is a family history of metabolic disease it is important to look for G6PD deficiency and pyruvate kinase disease.

A high resolution ultrasound is a non invasive preocedure and is a must. more than 50 % causes can be indentified on ultrasound.Full fetal survey is essential including looking for skeletal dysplasias, markers of aneuploidy, stigmas of conegnital fetal infections, pulmonary, renal defects. echocardiography should be done as cardiac defects are found in 15 % of cases. it should include measuring cardiac size.

Fetal karyptyping should be done. It can be done by fetal blood sampling or amniocentesis. Fetal blood sampling is preferred over amnocentesis due to advantage of able to do karyotyping which is quicker ( 48-72 hours), infection screening as well as diagnosing fetal anemia, haemoglobinopathies. Umblical venous pressure can be measured which carries prognostic significanceas well simultaneous therapeutic preocedures like intrauterine transfusions can be undertaken. But couple should be told about the fetal loss rate which is 1- 2 % in non hydropic fetuses and may be higher in hydropic diseased fetus versus fetal loss rate of 0.5 - 1 % in amniocentesiskaryotyping and infection screening is possible with amniocentesis though full culture results take time ( 2- 3 weeks) and it lacks other advantages of fetal blood sampling.
In about 30% cases no cause can be found out.

(b).Hydrops fetalis is associated with a poor progosis with upto 50- 90 % fetuses dying in utero and those surviving majority die after birth.Very few may be amenable to therapy. Fetal anemia can be treated by intra uterine transfusions, though anemia due to allo imunisation carry better prognosis than due to parvo virus infection. Fetal arhythmia without structural cardiac defects carry better prognosis than where there are structural cardiac defects.
Umblical venous pressure(U.V.P.) is useful in assessing prognosis. fetuses with raised UVP and UVP returning to normal after draining hydrothorax carry a better prognosis than in fetuses with normal UVP or in whom UVP does not come back to normal after drainage .
Congenital heart block due to anti Ro antibodies and arrythmia due to parvovirus infection usually respond to maternal transplacental digoxin
Recurrence risk is low unless there are karyotypic or metabolic disease.
Posted by A H.
This is most likely non-immune hydrops (NIH). There is a high risk of intrauterine demise and perinatal mortality. Appropriate investigations to correctly identify the cause are important as some conditions are amenable to intrauterine treatment which can improve outcome. If the condition is recurrent or carries a very poor prognosis appropriate counseling for this and future pregnancies can be done. Targeted investigations will be done based on History and preliminary investigations.



The patient should be referred to tertiary level facilities for detailed investigations and l a fetal medicine expert are available. A detailed ultrasound looking for structural defects is required. These include cystic hygroma, cardiac, CNS and skeletal survey. Sites and degree of effusions will be noted. Although detection rate for cardiac structural abnormalities is less than fifty percent, hypo plastic left heart and AV canal defects will be sought. Tachyarrhythmias can lead to cardiac failure, which precede hydrops. Bradyarrhythmias will prompt investigating for anti-ro and anti-la antibodies in maternal serum. Fetal echocardiogram will be done. If skeletal dysplasia is suspected a detailed look at long bones, hands, feet and thorax will be done. Peripheral shunting can lead to high output failure and hydrops. Sacrococcygeal teratomas and placental chorioangiomas can produce this effect and will be sought. Doppler insonation of middle cerebral arteries can detect almost all cases of fetal anemia and will be done. This condition exists with Parvo B19 infection. Twin pregnancy and possible twin-to-twin transfusion syndrome will be excluded at this time.



Fetal Blood sampling will be done for complete blood count (anemia and thrombocytopaenia seen with viral infection especially ParvoB19), liver function tests, infection screen, detection of viral DNA, enzyme level for metabolic disorders will be done if necessary. Karyotyping must be done for chromosomal and genetic abnormalities. Although karyotyping can be done via amniocentesis, which carries a lower risk of fetal wastage even when the fetus is hydropic, fetal blood sampling is the superior investigation since a more comprehensive study is possible.



Maternal blood must be tested for other red cell antigens. Kell antigen can provoke an antibody response producing a rapidly progressing hydrops out of proportion to the degree of anemia. Infection screen and a Kleihauer-Betke test to quantify fetomaternal haemorrhage can also be done, if necessary.



Prognostic factors include the cause and degree of hydrops, and risks associated with invasive procedures used for diagnosis or treatment. Chromosomal abnormalities and genetic conditions may not be treatable. Anemia secondary to infection can be treated with intravascular transfusions, which carry a fifty to sixty percent success. Pleural effusions can be shunted. Arrhythmias can be treated with anti-arrhythmics either to mother or directly to fetus. All invasive procedures carry a risk of preterm labour and miscarriage. Perhaps the single worst prognostic factor is NIH, which carries a perinatal mortality of up to ninety percent.
Posted by Srivas  P.
The diagnosis most likely in this case is non-immune Hydrops fetalis. The incidence world wide in 1:1000 and mortality ranges between 60-90 % depending primarily on the cause. Hydrops fetalis is defined as presence of fetal subcutaneous tissue edema accompanied by serous effusion in one or more body cavities. The earlier in gestation that fetal hydrops is recognized, the poorer the prognosis.

NIH is multifactorial and could be due to fetal cardiac, pulmonary, gastro intestinal, hematological malformations, apart from metabolic errors, fetal infections with Parvovirus B19, syphilis, CMV, toxoplasmosis, Herpes, Listerosis etc or neoplastic causes in the fetus. 30-40 % cases are of idiopathic origin. Chromosomal anomalies like Trisomy 13, 18, 21, Turner?s syndrome account for 13-15 % cases while fetal infections contribute to about 8 % of causes.

Maternal diseases like Connective tissue disorders, diabetes mellitus, pre-ecclampsia may be causative. Placental causes include Placental vein thrombosis, chorioangioma, true knots and twin-twin transfusion syndrome.

Diagnostic studies may be considered best by temporal grouping (i.e., fetal, maternal, placental, neonatal, postmortem. Assessments generally proceed from low-risk noninvasive tests to higher-risk invasive techniques as required for precise and complete diagnosis to properly manage the individual pregnancy. In spite of all investigations, in 40 % cases no cause may be found.

Detailed ultrasound examination on the fetus is a very useful examination and may reveal structural anomalies, cardiac arrhythmias, intracardiac tumors, diaphragmatic hernias, soft markers for trisomies, Cystic Hygromas in Turner?s syndrome etc. Congenital structural anomalies of the heart may accompany as many as 1 in 4 babies with hydrops and should always trigger a careful search for other malformations, and karyotyping should be performed in all such fetuses. Echocardiography may indicate the nature of cardiac lesion. USG may reveal evidence of same-sex twins, a monochorionic placenta, with hydramnios in one sac and oligohydramnios in the other sac, evidence of cerebral white matter damage etc. USG exam has its drawbacks, as all malformations may not be detected and it needs expertise to detect them. The USG are preferably done in tertiary centers.
Bio physical profile to assess fetal condition too may be done by USG and may reveal reduced fetal body movements accompanied by sinusoidal fetal heart rate patterns.

Maternal blood should be investigated for Complete blood count, blood group, Hb electrophoresis to rule out alpha thalassemia, Kleihauer betke?s test, G6PD and Pyruvate Kinase carrier status. Test for Lupus anticoagulant, ACA antibodies should be done to rule out SLE and anti-Rho antibodies tested especially if evidence of fetal bradycardia, suggesting congenital heart block. Maternal AFP level has been found to be elevated in hydrops associated with fetomaternal hemorrhage, umbilical cord hemangioma, polycystic kidneys, CMV, and Parvovirus; Parvovirus may be the cause of as much as one third of all incidents of hydrops fetalis.
Antibody screens for common maternal and fetal infections (toxoplasmosis, other infections, rubella, CMV infection, and herpes simplex (TORCH) and more sensitive and specific enzyme-linked immunosorbent assay (ELISA) studies. PCR technique generally is accepted as the criterion standard and should be employed whenever possible.

The most appropriate invasive fetal investigation is fetal blood sampling but risk of miscarriage are greater than with amniocentesis and may even be greater than 3% usually quoted, as risks are more with hydropic fetus. Fetus can be tested for anaemia, Hb electrophoresis, reticulocyte count and blood gas analysis to know well being of the fetus.Fetal therapy can be given at the time of FBS if required. Infection screening as on maternal blood can be done for direct evidence of fetal infection, along with fetal karyotype, the results of which can be available in 48 hrs. Direct enzyme assays or biochemical analyses of measurements of levels of specific metabolic products may be done to diagnose inborn errors of metabolism.

Amniocentesis too can be used infection screening and but takes 2-3 weeks for karyotype results but fetal anaemia cannot be diagnosed. Post mortem examination of fetus too can reveal evidence of fetal infection, structural anamolies, karyotyping etc.

Prognosis depends on gestational age of the fetus, underlying cause for the hydrops, treatability of the condition, presence of structural malformations, chromosomal abnormalities etc. Parvovirus infection has good prognosis with timely intra uterine blood transfusion and reticulocytosis is an indicator for good prognosis. Prognosis is best if the management is done in tertiary centre with trained personnel and advanced neonatal facilities.