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Essay 334 - fetal hydrops

Posted by Syamala H.
syamalah
ansA: potential cause of hydrops can`t be always identified as 30%are idiopathic.other possible causes could be immune mediated such as non anti D red cell isoimmunisation like anti kell and anti duffy antibodies,antiE and anti c antibodies. other possible causes could be occult infections like TORCH,parvo virusB19,leptospirosis,listeriosisandsyphlis.affection of the fetus by nonhemolytic anemia like G6PD deficiency and pyruvate kinase deficiency.disorders of redcell production like homozygous a thalssemia,heriditery spherocytosis.fetal thrombocytopenia leading to chronic fetal hemmorhage and subsequent anemia in conditions like ITP and fetal alloimmune thrombocytopenia. chromosomal anamoly like Turners syndrome,trisomy and tripliody are associated with fetal hydrops.structural anamoly like major congenital cardiac anamoly leading to CCF of fetus or cardiac arrythmia and myocarditis.pulmonary condition like diaphragmatic hernia,CAML,pulmonary hypoplasia.intraabdominal or intrathoracic tomours and placental tumour like chorioangioma.fetal renal anamolies and sacrococcygeal teratoma. fetal affection by inborn errors of metabolism. undiagnosed maternal diabetes can also be a possible cause.
mother should have full blood count to diagnose possible anemia and should have antibody screen to identify isoimmunisation other than anti D. she should have kleihaeur betke test done. also she should have baseline assesment of urea electrolyte and liver function test. infection screen using PCR should be done for TORCH,parvovirus and syphlis but it should be noted that it is useful if paired samples are available from prior to and after infection and result should be interpreted in conjuction with accurate maternal history and in consultation with virologist.. she should have glucose tolerance test to diagnose gestational diabetes.she should be tested for lupus anticoagulant and anti Ro specialy in cases of fetal badycardia.
there should be a detailed ultrasound by fetal medicine specialist to diagnose other stuctural anamolies associated,including detailed cardiac scan with echo.severity of hydrops should be assessed and degree of anemia can be predicted by measuring MCA-PSV. a value of>1.5 multiple of median has sensitivity of 100% and false positive rate of 12% in indicating moderate to severe anemia.
fetal blood sampling would be required for kayotype,full blood countand platlet,group and Rh, coombs test and PCR for infectious screen.but patientshould be counselled regarding high rateof fetal loss associated with the procedure which can be as high 20% depending on the cause and worsening isoimmune sensitisation due to fetomaternal hemmorhage.
ans b:
patient should be managed in consultation with fetal medicine specialist.she should be told that this infection doesnot carries the risk of teratogenesis. also one third of the cases will resolve spontanously but it is difficult to predict which cases will resolve . risk intrauterine fetal death in IgM positive mother is 10%.this occurs usually 4-6wks after maternal infection but can occur upto 3 mnths. there is no evidence of longterm adverse effect in babies who had recovered from hydrops.
patient will require serial ultrasoundevery 1-2weekly upto atleast 12 wks and MCA-PSV should be measured to diagnose the degree ofanemia. if the anemia or the hydrops worsen or the initial value of MA-PSV is >1.5 mom fetal blood sampling with intrauterine transfusion is indicated. counsell about risk of procedure related loss.benefit of transfusion are that oxygen carrying capacity is increased improving fetal heart function.more than 90% will resolve within 6 weeks of transfusion. high reticulocyte count >20% indicate fetal recovery and transfusion may not be required. if fetus attains viability while still being monitored for recovery mother should recieve corticosteriod to accelerate fetal lung maturity .once the fetus had recovered vaginal delivery should be anticipated.provide written information.
Posted by H H.
Hydrops fetalis is the accumilation of fluid in one or two of the extravascular spaces( eg peritoneum,pleural or pericardial cavity) .In most of the cases the cause is idiopathic, but any thing that can cause increase in hydrostatic pressure in capillaries ( chest tumours, adenomatous malformation lung, cardiac failure from anemia due to twin to twin transfusion or alpha thalasemia or parvo virus infection ),lower the osmotic pressure of blood( hypoproteinemia due to liver affection by infection or metabolic disorder ) or increase capillary permeability ( infections eg toxoplasmosis , cytomegalovirus),will shift fluid from intravascular circulation to extravascular spaces. Some of the causes can produce hydrops by a combination of all previous factors. Chromosomal abnormalities as Down, or Turner can produce hydrops. Structural malformation of the fetus can be associated with hydrops. Congenital heart block of fetus due to anti Ro antibodies(SLE) or fetal tachycardia due to Gravies disease both can cause heart failure with increase in hydrostatic cappilary pressure and hydrops.

Non invasive investigations include, an abdominal ultrasound, which will detect any structural malformations of fetus, chest tumours, sacrococcygeal tumour, detect degree of fluid accumulation and severity of condition eg bilateral hydrothorax or scalp edema mean severe condition. Will dtect fetal heart arrhythmia as congenital heart block or fetal tachycardia. Fetal echosonography can detect structural abnormalities of heart. Doppler peak velocity of middle cerebral artery of fetus will detect fetal anemia ,its severity and later the adequecy of correction of fetal anemia by intra uterine transfusion. Maternal blood for Kleuhauer test to detect feto maternal hemorrhage, red cell antibodies as Kell antibodies, Hemoglobin electrophoresis to detect alpha thalasemia, infection antibody screen for TORCH,and lupus anticoagulant anti nuclear antibody for antiphospholipid syndrome, anti RO anti bodies which can cause congenital heart block and anti double stranded DNA which is specific for associated systemic lupus erythmatosus.

Invasive investigations include cordocentesis ,to obtain fetal blood for HB estimation, hematocrit (if less than 30% will need transfusion ), HB electrophoresis , karyotyping, detection of fetal infections and PCR for single gene defects. Amniocentesis is inferior to the later as no fetal blood is obtained , karyotyping, detection of fetal infection and single gene defects can be detected.


Patient is assured that such infection is not teratogenic and that spontaneous cure can take place and that her management will be i a tertiary center specialised in fetal medicine. The cause of hydrops is fetal anemia . Correction of anemia will cure it. The fetus is monitored for fetal anemia using peak velocity in the middle cerebral artery via doppler ultrasound .Severity of hydrops is done by ultrasound in a fetal medicine unit ,scalp edema , collection of fluid in more than one cavity,bilateral hydrothorax. If the condition is resolving and fetal middle cerebral blood flow is normalising,the patient is assured . Should the condition worsen ,will need cordocentesis and intra uterine fetal transfusion. Delivery of such babies should be considered at 34 wk ,after neonatal consultation, giving antenatal corticosteroids and in presense of good neonatal services. At 34wk delivery is usually by cesarean section.
Posted by MR R.
A healthy 37 year old Rhesus positive woman attends for her anomaly scan at 22 weeks gestation. The fetus is found to be hydropic. (a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks].

The potential causes for non immune hydrops are viral infections like parvovirusB18,CMV,hepatitis and TORCH.A history of recent maternal infection can suggest a possible fetal infection.Haemoglobinopathies like A - thallesemia/spherocystosis and ethnic origin can suggest this.Maternal G6PD and pyruvate kinase defeciency can also be associatedwith hydrops.Maternal metabolic disorders & diabetes mellitus can be an underlying factor.Fetal structural malformations like diaphragmatic hernia,pulmonary hypoplasia.cystic adenomatoid malformation of the lung & polycystic kidneys can be evident from anamoly scan and can be associated with hydrops..Fetal cardiac arrythmias due to anti Ro & La antibodies can also give rise to hydrops.Placental tumours like chorioangioma can also give rise to hydrops & high output failure.Fetal tumours like sacrococcygeal tumours can potentially cause hydrops.Chronic fetomaternal haemorrhage can also cause aneemia and hydrops.
Fetal hydrops should propmt referral to a fetal medicicne specialist unit for further investigation and mangement.Initial ultrasound scan is carried out to assess the severity of the hydrops.Fetal biophysical profile will be helful to assess well being of the fetus.They cam also identify intracranial calcification associated with infections.This can also find out if there are any associated structural anamolies.If there are any abnormalities in the heart rate then Maternal anti Ro & La antibodies should be performed.If fetal cause not identified then Maternal screening for FBC and blood group for atypical antibodies and Kleihauer test is performed.Hb electrophoresis( ehnic origin ) to identify A- thalessemia.An oral glucose tolerance test with 75 g of glucose to diagnose diabetes mellitus.A recent history of maternal infection should prompt serum for IgG and IgM antibodies for parvovirus,hepatitis and CMV.Amniocentesis can identify viral excretion in the fetal urine however a negative result does not rule fetal infection.A fetal blood sampling can be diagnostic and can be tested for IgG and Ig M for specific infections.However these are invasive procedures and carry procedure related fetal loss.(amnio - 1%, FBS - 2%)

(b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks].

The patient should be managed in a feal medicine unit under a obstetrician with special interest in fetal medicine, specialist midwife and neonatologist.The condition itself can be distressing to the patient and adequate s psycological support should be provided.Written information about hydrops is given too the patient .The pan of management should be documented in the notes.Hydrops due to parvovirus can be self limiting and resolve on its own.In early gestation < 20 weeks and severe hydrops the prognosis can be poor and termination can be offered.In other cases Regular scans are performed on a 1- 2 weekly basis.This will be to assess biophysical profile and also the severity of the hydrops.Parvovirus has a prediliction to the erythropoetic cells and can cause aplatic crisis.If the hydrops is severe and FBS suggests severe anaemia and low haematocrit intrauterine transfusion is recommendedSteroids are given (betamethasone 24 hours apart) for lung maturation anticipating early delivery.If the hydrops has resolved then pregnancy is allowed to continue if not delivery is planned after 34 weeks geatation.Caesarean section might be considered if severe hydrops.Neonatal team should be available at the time of delivery and cord blood analysed for haematocrit levels and further management.Patient should be adviced about fetal movements and to contact hospital if reduced movements.Contact nos in the hospital should be provided.
Posted by A- N.
aa
a) Hydrops foetalis is usually associated with Anti D isoimmunisation, how ever the number of cases due to non anti D isoimmunisation is steadily increasing due to the decrease of Anti-D isoimmunisation that is brought about as a result of antenatal Anti-D prophylaxis.
The causes of hydrops foetalis in this instance could be either maternal, foetal, placental or idiopathic.
The idiopathic causes account for up to 30% of cases.
The maternal causes may be secondary to Diabetis mellitus, infections as Syphylis, Toxoplasmosis, Cytomegalovirus, hepatitis and parvovirus.
The other causes can be secondary to non Rh isoimmunisation.
The foetal causes for hydrops can be due to, chromosomal causes like trisomy 13,18, 21, Turners syndrome. The structural causes may be due to structural defects including cardiac defects as ebsteins anomoly, hypoplastic right or left ventrical, transposition of great vessels, thoracic abnormalities like diaphragmatic hernea, pulonary sequestration, congenital cystic adenomatoid malformation OR due to conduction defects as foetal dysarrythmias mainly tachyarrythmias.
The causes include, alpha thalasemia fetomaternal haemorrhage.
The genetic causes account for 10-15% of non immune hydrops these include mucopoly saccaridosis.
The investigation should begin with detailed history to check if there any history of rashes or contact with infections or with patients with infections or animals.
Maternal full blood count and blood group and antibodies to check for maternal anaemia and to identify any antibodies.
Kleihauer test to check for fetomaternal bleed. maternal infection screen for TORCH infections and for Parvo virus.
The presence The maternal haemogloin electrophorasis to rule out alpha thalasaemia.
Oral glucose tolerance test is done to check for diabetis millitus.
auto antibodies to identify SLE along with anti Ro and anti La anti bodies may be useful if there are bradyarrythmias of foetus.
She should be refered to fetal medicine tertiary unit for further detailed ultrasound scan to check for foetal structural abnormalities, the foetal echo cardiography to check for congenital structural and conduction defects.
middle cerebral artery doppler peak systolic velocity will help in identifing foetal anaemia if raised then may require cordocenesis for both diagosis and treatment by transfusion.
Amniocentesis may be offered which will help in identifing, chromosomal abnormalities, metabolic abnormalitis as mucopolysaccharidois,viral infections and bacterial culture.
Cordocentisis may be offered after 18 weeks, to check for foetal anaemia, thalasemia, viral screen, coombs test for non anti D antibodies and karyotyping.
The presence of IgM antibodies for parvovirus in amniotic fluid or foetal blood is diagnositc for parvovirus infection.
b) Hydrops due to parvo virus has good prognosis with intra uterine transfusion.
She should be managed with mult disiplinary team including foetal medicine specialist, neonatalogist.
About 30 % of hydrops secondary to parvovirus will resolve spontaneously with observation.
The patient should be reassured that this will not lead to congenital abnormalities and no long term effect on baby.
Doppler study of middle cerebral artery for peak systolic velocity is done. If it is more than 1.5 MoM, foetal cordocentesis is indicated. Cordocentsis is associated with 1% miscarrage rate, this is to be informed to patient.
Foetal blood obtained from cordocentisis is first checked for haemoglobin and foetal transfuson is offered depending mean haemoglobin for that gestation age. The blood may also be used to check for other causes of hydropis foetalis.
Foetal transfusion is curative in more than 90% of cases with parvo virus induced foetal hydrops.
Posted by sonu P.
S

a)The possible causes of non immune hydrops are structural malformations most commonly cardiac defects with or without fetal arrhythmia. Other structural defects are GI disorders i.e.diaphragmatic hernia, gastrochises;lung conditions like CCAM; chromosomal disoreders like turners XO or mosaics; hematological conditions like sickle cell disease and major b-thalessemia, hereditary spherocytosis, non-D alloimmunisation, Aplastic anaemia; Infections like parvovirus, CMV,rubella.
A detailed fetal anomaly scan at a tertiary fetal medicine unit should be done to detect subtle structural malformations and assess the severity of hydrops.An infection screen for toxoplasma, rubella, CMV and herpes(TORCH) is requested. A growth scan along with umbilical artery and MCA-PSV is done initially to identify fetal anaemia as they are non-invasive procedures. In case of difficulty is finding a cause with non invasive methods;invasive procedures like amniocentesis for karyotyping and infection screen should be offered. Further a cordocentesis can be considered advising women of its risks and benefit. A fetal blood sample can provide the blood group, non D antibodies,Hb conc.,serology for the infections

b) The antenatal management is best done at a tertiary fetal medicine unit in a multidisciplinary team.The patient is dealt with extreme empathy and sensitiveness as she will very anxious.I will explain to her that the mechanism of hydrops in parvovirus infection is by causing severe anaemia and congestive heart failure.The severity of anaemia can be estimated by middle cerebral artery peak systolic velocity with considerable accuracy.Mild hydrops occasionally may resolve spontaneously with close monitoring and serial USS scans. Serial scans will be offered to monitor the progression of disease. The exact progression of the condition is not clear.Sometimes there is rapid deterioration. A cordocentesis and exchange transfusion would have to be done due to presence of hydrops at such early gestation.Usually,in utero transfusion is required every 2-3 weeks with improvement in the appearances of hydrops.But there should be caution about number of times transfusion would be needed to reach an acceptable gestation with satisfactory outcome. In case of severe hydrops at early gestation, option of termination of pregnancy should be considered even in the absence of major structural and chromosomal abnormality. Patient will need constant psychological support which is best provided by support group and certain non-profit organizations like ARC. She should be given information leaflet and follow up appointment.
Posted by L S.
LS:
(a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks].
Potential causes can be divided into immune or non-immune hydrops. Immune hydrops are mainly associated with anti-D Rhesus antibodies which is unlikely in this lady but there are rarer causes of antibodies like antibodies to K (Kell system) or Fya (Duffy system) which can occur. Non-immune hydrops (NIH) are mainly idiopathic which is diagnosed after all other causes have been ruled out. The other causes of NIH are due to infections for which Parvovirus B19 is commonly seen. Other infections like syphilis, CMV, Toxoplasmosis, Herpes Simplex, Hepatitis B, Listeria monocytogenes can also be a cause of NIH. Haemolytic diseases like Glucose-6-phosphate dehydrogenase deficiency or pyruvate kinase deficiency can cause NIH. Disorders of red cell production especially homozygous alpha-thalassaemia can cause NIH. Chromosomal anomalies like Trisomies, Turner syndrome or Triploidy can also cause NIH. Fetal structural anomalies should also be assessed as a cause of NIH like cardiac anomalies (severe congenital heart disease, arrhythmia or myocarditis), pulmonary anomalies ( Congenital Cystoadenomatous Malformation of Lung, Pulmonary Hypoplasia or Diaphragmatic Hernia), or renal anomalies. Fetal sacrococcygeal teratoma or placental hemangioma can also be a cause of NIH. Maternal disease like diabetes, pre-eclampsia, severe anaemia or hypoalbuminaemia are also causes of NIH but is highly unlikely in this case as she is healthy. Investigations that can be carried out are maternal investigations by doing full blood count, urea and electrolytes, liver function test, blood group and antibodies. Her ethnic group should be asked as those ethnic from South East Asia are at higher risk of alpha thalassaemia and Hb electrophoresis can be carried out to confirm. Infection screen with TORCH with Parvovirus B19, syphilis and coxsackie and Kleihauer for fetal maternal hemorrhage and oral Glucose tolerance test is carried out. The fetus should have a detailed ultrasound scan to assess severity of hydrops, look for anomalies with a detailed cardiac scan done. The scan can also exclude fetal or placental tumours. Fetal blood or amniotic fluid sampling should be carried out after appropriate counselling with full blood count, Coombs test, karyotype, PCR infection screen or Hb electrophoresis if required

(b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks]
Maternal risk in a fit and healthy woman is usually minimal. The management of NIH depend on severity as it is potentially curable. A mild hydrops can often be managed conservatively with serial scans as spontaneous resolution may occur. Failure of the hydrops to resolve or develop more severe hydrops will require invasive intervention. This is by in-utero blood transfusion as this virus causes severe fetal anaemia. Due to this risk, her care should be in a specialist fetal medicine unit. Subsequently after transfusion the fetus will be monitored for resolution of hydrops and usually only one transfusion would suffice. Antenatal corticosteroids are administered if delivery before 34 weeks is anticipated. The decision on timing and mode of delivery is usually in association with neonatologists.
Posted by GULSHAN R.
37 year Rh positive women anomaly scan at 22 weeks gestation-fetus found to be hydropic.
(a)discuss potential causes and investigationsto confirm diagnosis.

Asmother is Rh-positive chance of Rh isoimmunization is excluded although there is chance of immunization by Kell & Duffy system.In this case nonimmune causes are very important.The causes are-
*Idiopathic in 30% of cases.
*Infections are one of the most common causes of hydrops , such as-Parvovirus B19, Toxoplasma,CMV, Rubella,Herpes simplex,Ureaplasma urealyticum,Coxsackievirus.
*Chronic fetomaternal haemorrhage,twin-to-twin transfusion & the condition causes fetal anaemia also cause fetal hydrops.
*Chromosomal causes---Trisomies/Turner\'s/Triploidy
*Metabolic disorder-glucose-6-phosphate dehydrogen deficiency
*Red cell disorder-homozygous thalassaemia,lethal hereditery sperocytosis.
*Maternal disease :uncontroll diabetes,severe anaemia,SLE

Investigations-
Maternal blood group and antibodies-can identify antibody other than d as mother is Rh positive.
Hb electrophoresis-can identify Thalassaemia and other red cell disorder.
Infection screen-
VDRL-for syphilis
TORCH antibodies-to identify toxoplasma,CMV, Rubella and Herpes.
MSAFP-screen for chromosomal abnormalities& is amarker of fetal parvovirus infections.
Maternal blood for Hb
Lupus anticoagulant & anti-Ro antibodies
Glucose tolerance test

Fetal investigations-
Fetal echo-to see congenital cardiac abnormality.
Fetal blood sampling-FBC,group,karyotype,coomb\'s test,PCR based infection screen.

(b)Disscuss the subsequent management given that hydrops due to Parvovirus.
Multidisciplinary team should be involve which include-Obstretrician,Fetal medicine specialist,neonatologist.
Management should be done in tertiary centre.
Doppler usg assesment of MCA PV.
-absence of increased MCA PV--expectant management can be done with doppler ,ultrasound assesment.
-increase MCA PV above 95th percentile--indication for intrauterine transfusion.
Delivery should be done once pulmonary maturity occur usually at34 weeks.
Premature delivery need corticosteroid.
Mode of delivery should be vaginal.Ceaserian section for obstretic indication.
Must be hospital delivery.
After delivery baby should be examined by neonatologist.If necessary admit to NICU.



Posted by Bee N.
A healthy 37 year old Rhesus positive woman attends for her anomaly scan at 22 weeks gestation. The fetus is found to be hydropic. (a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks]. (b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks].

(Bee)
A) The causes of fetal hydrops can be immune or non immune related.
Immune causes include hemolytic disease of the newborn which can also affect fetuses in utero. They occur when a fetus positive for some specific red cell antigens is exposed in utero to antibodies of the IgG group developed by the mother usually as a result of previous sensitisation. The antigens commonly implicated include the RhD, RhE, Rhc, kell and duffy blood groups.
The non immune causes in a healthy 37 year old will include chromosomal anomalies such as the trisomies (21,18,13). These can cause structural anomalies leading to heart failure in the fetus. Other structural anomalies which may not necessarily be related to chromosomal anomalies include hypoplasia of the lungs, congenital adenomatoid malformation of the lungs. Renal anomalies such as bilateral absence of the kidneys, tumours which may be intrathoracic or intraebdominal and cardiac anomalies such as hypoplastic left heart syndrome. Any cause of anemia in the fetus will also cause hydrops. Anaemia may be related to blood loss as in Twin twin transfusion syndrome or chronic feto maternal hemorrhage. Anaemia may also be aplastic as is found in Diamond blackfan Syndrome which is congenital aplasia in red cell formation. Anaemia may also be hemolytic as is found in autosomal dominant disease called spherocytosis or alpha thalessemia (homozygous). Infections affecting the fetus such as Toxoplasmosis, Rubella, CMV, parvovirus B (causing aplasia) and syphilis. Rare disorders such as placental chorioangioma and sacrococcygeal teratomer as well as inborn errors of metabolism can also be implicated as a cause of hydrops. Hydrops can be idiopathic.
For a healthy mother, maternal investigation will include Heamoglobin electrophoresis for thalassemia since mother may be heterozygous and appear healthy. Maternal blood group should be rechecked and serum taken to investigate for presence of antibodies which may cause hemolytic disease of the newborn.Maternal serology to investigate for presence of past infections such as CMV, Rubella and Toxoplasmosis and parvovirus by measuring IgG, IgM and IgG avidity related to the infections suspected. Recently to avoid fetal blood sampling for chromosome and gene analysis, fetal cells can be harvested from maternal blood.
Fetal investigations can start with detailed anomaly scan and a four chamber heart echocardiogram as soon as possible. This may detect structural anomalies or soft markers for chromosomal anomalies such as trisomies or turners. Amniocentesis can be done for karyotyping, acetlycholinesterase assay. If inborn error of metabolism is also suspected, amniocentesis may provide fetal cells fro gene analysis. More invasive tests such fetal blood sampling for FBC which will detect anaemia and for PCR in investigating of infections such as rubella, toxoplasmosis, CMV and parvovirus infections. FBC also will help group fetal blood for investigating hemolytic disease of newborn. Direct coombs test on the fetal blood will help detect presence of maternal antibodies which may cause hemolysis. Quantification of the antibody titres will help predict likelihood of causing hemolysis. Middle cerebral artery peak systolic velocity doppler is very sentisitive for the diagnosis of anaemia in the fetus though it does not tell us what the cause of anaemia is.

B) If the cause of the hydrops is parvovirus, I will explain findings in a sensitive manner to the patient and her partner. I will tell them that the cells needed to carry oxygen in the fetus is not been produced due to infection with parvovirus. I will explain that at such an early gestation, the prognosis is poor and management will of any sort cannot guarantee resolution.I will explain that termination of pregnancy is an option. I will liase with a fetal medicine specialist and neonatologist for further management if they wish. This will entail regular fetal blood transfusion which has a risk of miscarriage of at leat 3-5%. Both maternal and fetal blood will be required for grouping and crocc matching. I will arrange for weekly doppler and liqour volume check as well as growth scan (abdominal circumference every 2 weeks). Regular doppler for middle cerebral artery peak systolic velocity will dictate need for transfusion. Once fetus is up to age of viability (24weeks), I will give steriods 12mg betametasone to patient as delivery may be required at anytime.
I will ask patient to report any reduced fetal movement.
Delivery will be arranged for center with neonatal intensive care unit.Timing of delivery will be decided in conjunction with fetal medicine specialist and dictated by fetal well being. Mode of delivery will be by ceasarean section if hydrops does not settle. All plans and discussions made will be clearly documented in patients case note.
Posted by leelavathi C.
In Rhesus positive woman with fetal hydrops most likely cause would be non-immunehydrops. But Immune mediated red cell isoimmuninisation with anti-Kell antibodies, anti-Duff antibodies are also possible cause. In non-immune hydrops 30% cases are idiopathic. Other causes are fetal infections like TORCH, ParvovirusB19, syphilis and coxsackie. structural anomalies like major congenital heart disease cardiac arrhythemia, pulmonary hypoplasia, diaphragmatic hernia , intra abdominal ,and intra thoracic tumors, placental haemangioma, and sacrococcygeal tumors. Chromosomal anomalies like Trisomy 21, trisomy 13, turners syndrome are also one of causes. Chronic materno -fetal haemarrhage, haemoglobinopathies are also causes for non immune hydrops.
Investigations on mother –FBC, Hb electrophoresis, infection screening should be performed.kleihauer test should be performed to exclude feto-maternal haemarrhage. Other test like anti-R0, anti-La antibodies should be performed if fetus is bradycardic. detailed ultrasound scan including Doppler insonation of the fetal cerebral vasculature and liquor volume should be performed. Ultrasound scan also include detail fetal anatomy and placenta , umbilical cord should be examined. Fetal echo cardiography should be done to exclude cardiac anomalies. Invasive investigations like fetal blood sampling should be obtained for FBC, karyotyping, group, combes test, and infection screening by PCR.
B) Antinatal management should be continue in tertiary center under multi disciplinary team including fetal medicine specialist, neonatologist. Councel mother sencitively and explain the reasons for hydrops is viral infection, causing anemia and reassure her that it might resolve spontaneously some times. Regular ultrasound scan should be done 1-2 weekly . if anemia progressing might need intra uterine transfusing as per guided by fetal medicine specialist.delivery plans should be documented in notes
Posted by Dr Dyslexia V.
X
a) Immune causes such as anti-Kell and anti-Duffy still needs to be ruled out eventhough it is rare. This could be done by antibody identification of maternal serum. 30% of the cause of hydrops fetalis is idiopathic and the cause could not be found. Infective causes such as parvovirus B-19, rubella, hepatitis B, coxsackie B virus, syphilis, toxoplasmosis, herpes simplex could be ruled out by taking the respective IGM and IGG antibodies. If strongly suspected from history then a repeated serum level should be taken 2-3 weeks later in such cases of parvovirus B-19 or rubella. Anticardiolipin and anti Rho should be taken to rule out fetal cardiac arrhythmia due to antiphospolid syndrome. Hemoglobin for electrophoresis should be done to rule out thallesimia in which could cause Hb bart which could cause hydrops. Modified glucose tolerance test could be performed to rule out undiagnosed type 2 diabetes in pregnancy. Amniocentesis could be performed for karyotyping for chromosomal abnormalities such as Turner’s syndrome. Detail scan of the fetus should be done to look out for structural anomalies such as diaphragmatic hernia, CAML, pulmonary hypoplasia, tumour of the placenta or on the fetus. A cardiac echo cardiography must be done to assess for any cardiac anomalies such as closure of ductus arteriousus. Killhaur’s test can be done to assess any fetal maternal hemorrhage which could cause fetal anemia.

b) She could be informed that the parvovirus infection does not result to any congenital syndrome but causes fetal anemia and cardiac dysfunction due to that. If the anemia is mild there is a possibility the infection would resolve but in this case we could assure it is severe as it has resulted in hydrops. She would preferably referred to a tertiary feto maternal centre to be managed by a multidisciplinary team which include feto maternal consultant and neonatologist. She would be followed up every 2 weeks till delivery. The fetus could be transfused after assessing the HP level with packed cells cross matched against the mother to supranormal Hb level. The disease severity could be monitored via MSAFP and the anemia by middle cerebral artery Doppler as well. She should be informed regarding termination if prognosis of fetus is poor and the anemia irreversible.
Posted by Chitra.s M.
A.Potential causes for fetal hydrops include infections like toxoplasmosis,syphilis,CMV,parvo virusB19.Fetal anemia due to infections(parvo virus) ,fetomaternal haemorrhage can result in hydrops.Disorders of red cell production like alpha thalessemia major,lethal heriditary spherocytosis and non hemolytic anaemia like G6PD deficiency ,pyruvate kinase deficiency can cause hydrops. Tachyarrythmias , heart block(due to maternal auto antibodies like antiRo/La) ,myocarditis and structural cardiac defects like hypoplastic left heart,ebstein\'s anomaly can lead to cardiac failure and hydrops.Chromosomal abnormalities like trisomy(down syndrome),Turner syndrome and triploidies can be a cause for hydrops.Structural malformations in the fetus like congenital diaphragmatic hernia,congenital cystic adenomatoid malformation and prune belly syndrome could cause hydrops.Impaired fetal mobility as in achondroplasia,arthrogryposis could result in hydrops.Other causes would include inborn errors of metabolism like gaucher\'s disease, mucopolysaccharidosis and tumours like sacrococcygeal teratoma and placental chorioangioma.Maternal conditions associated with hydrops are diabetes mellitus,severe anaemia, hypoalbuminaemia and preclampsia.Presence of Anti Kell and antiDuffy antibodies could lead to immune hydrops.However hydrops is idiopathic in upto 30% of cases.
Non invasive fetal investigations would include a detailed ultrasound scan by a fetal medicine specialist for assessment of the severity of hydrops,presence of structural anomalies,fetal/placental tumours . Fetal echo is done for detecting arrythmias and cardiac malformations.Middle cerebral artery doppler for peak systolic flow is done for evidence of fetal anaemia.
Maternal investigations include full blood count for anaemia,kleihauer test for fetomaternal haemorrhage and testing red cell antibodies like kell and duffy.Haemoglobin electrophoresis for alpha thalessemia is considered if ethnicity is indicative of risk.GTT is done for detecting diabetes.Infection screen for toxoplasma,parvovirus CMV syphilis is done.AntiRo, antiLa antibody testing is done if fetal heart block is identified.Thyroid function test (hyperthroidism)are indicated if fetal tachyarrythmia is present.
Invasive fetal investigations would include fetal blood sampling for FBC-Hb%,platelet and reticulocyte count,karyotype,Hb electrophoresis(based on risk assessment)and infection screen(PCR/IgM antibodies).Amniocentesis can be considered for karyotyping and infection screen but is less informative as fetal anaemia cannot be detected.
B.The woman is explained that the cause of hydrops is due to fetal anaemia from Parvo virus infection.Spontaneous recovery and resolution of hydrops occurs in majority of cases .Further management would be in a tertiary centre in liaison with fetal medicine specialist.Serial monitoring by USS every 1-2 weeks is done for assesseing severity of hydrops and dopplers for systolic flow in MCA .Conservative management by follow up scans is done in mild-moderate hydrops to look for resolution. MCA studies indicative of fetal anemia reqiure fetal blood sampling for FBC-Hb%,platelet count and reticulocyte count.Increased reticulocyte count is indicative of recovery phase and is managed conservatively by serial scans to document resolution.Severe anaemia needs intrauterine transfusion.Multiple transfusions are rarely required.Procedure related loss rates for cordocentesis(1-2%)are expalined.Following resolution ,prospective managemant plan is made for fetal monitoring , timing and mode of delivery and documentedjn the notes.Corticosteroids for lung maturity is given if delivery is at <34 weeks gestation.
Posted by A A.
AA
(a) Diagnosis in this case is non immune fetal hydrops. There are many recognized causes for it. Idiopathic cause is in 30% of cases. Maternal and fetal infections including Parvovirus B 19,cytomegalovirus, toxoplasmosis, herpes simplex, listeriosis , syphilis and hepatitis B. Chromosomal anomalies like trisomies , triploidy and turner syndrome are present in 15%.Structural fetal anomalies including cardiovascular, pulmonary ,diaphragmatic hernia, and renal anomalies..Anemia due to chronic fetomaternal hemorrhage or secondary to fetal infection. Hemolytic diseases like glucose 6 phosphate dehydrogenase and pyruvate kinase deficiency. Red cell production disorders like alpha thalasaemia and hereditary spherocytosis. Investigations best carried out in specialized unit like fetal medicine unit. Investigations to confirm diagnosis should be undertaken on woman, fetus, placenta, neonate after delivery, and postmortem in case of fetal death. Antenatal investigations allow identification and treatment and also provide information to parents. It is important to find cause as prognosis is dependent on cause. Antenatal investigations are maternal blood for FBC, hemoglobin, non RH antibodies like Kell and Duffy. Hemoglobin Electrophoresis depending on ethnic group. Kleihaur Bethe test for chronic fetomaternal haemorrhage. Glucose tolerance test for gestational diabetes. Infection screen for TORCH, Parvovirus B 19 and syphilis serology. Fetal noninvasive test include ultrasound and Doppler studies. Ultrasound to assess severity of hydrops, presence of ascites, or hydrothorax. Detailed anomaly scans for structural anomalies and to exclude fetal, placental and umbilical cord tumours. Some chromosomal anomaly markers can be found on USG but it has limited value. Cardiac scan including echocardiogram is essential to find cardiac structural and rhythm abnormalities. Assess fetal heart rate it may show bradycardia in heart block or tachycardia in tachyarrhythmias. Doppler study of Middle cerebral artery for peak systolic velocity to find fetal anaemia. Other ultrasound markers like reduced fetal movement, amniotic fluid volume, placental thickness, and liver length have not been shown to be useful. CTG sinusoidal pattern is rare even in severe anemia. Invasive fetal investigations are amniocentesis and fetal blood sampling. (FBS) Invasive fetal investigations carry risk of fetal loss so risk of investigation balanced against potential benefits. Amniocentesis allows Karyotype and assessment of fetal infection but does not allow assessment of fetal haemoglobin.FBS will allow assessment of fetal hemoglobin and reticulocyte count. Karyotype and fetal infections by polymerase chain reaction. Assessment of single gene defect, in born errors of metabolism, and red cell disorders. Direct comb test for fetomaternal hemorrhage. It also allow intrauterine transfusion if needed. After delivery neonatal examinations and in case of fetal death post mortem investigations will be of value. It also confirms antenatal diagnosis and complete investigations.

(b) Patient should be referred to tertiary fetal medicine centre. Treatment should be in conjunction with neonatologist and physician. Woman with acute infection should be treated symptomatically. Parvovirus is not an indication for Termination of pregnancy. Ultrasound and clinical criteria are insufficient to predict prognosis. Mild hydrops will be monitored by Middle cerebral artery Doppler velocity and serial scan. Moderate to severe hydrops indicate FBS to confirm fetal anemia and intrauterine transfusion to correct it Conservative management and reassessment may be appropriate if fetal reticulocyte count are high as fetal hydrops and fetal anemia resolve spontaneously. Fetal medicine specialist must carefully weigh risk of intervention against condition and prognosis of fetus. Administer betamethasone for fetal lung maturity if delivery before 34 weeks anticipated .Decide timing and mode of delivery in association with neonatologist or neonatal surgeon if required. Recurrence risk in future pregnancy unknown.
Posted by Bobey B.
The potential causes include cardiovascular malformations, arrhythmia and neoplastic cause about 20 % . The other important causes are chromosomal anomalies ( trisomy 21, 18, 13 and turner\'s syndrome), haematological disorders ( such as homozygous a- thalassaemia, lethal hereditary shpherocytosis), non-immune haemolytic disease (glucose-6-phosphate dehydrogenase deficiency , pyruvate kinase deficiency) , pulmonary malformations such as (congenital cystic adenomatoid malformation of lung , pulmonary hypoplasia and diaphragmatic hernia) , genitourinary anomalies , intrauterine infections , cystic hygroma and placental chorioangioma . About 30 % of cases are idiopathic in origin. Maternal disease can cause NIHF like diabetes mellitus, preeclampsia, severe anaemia and hypoalbuminaemia.
Most useful investigations will be detailed anomaly scan. Detailed fetal ultrasound to look for structural anomalies such as skeletal dysplasia, genitourinary, gastrointestinal and placental and umbilical cord haemangiomas is carried out by an expert in fetal medicine. Assessment of fetal heart rate at ultrasound scan may show bradycardia (suggestive of heart block) or tachycardia (tachy-arrythmia). Fetal echocardiography and cardiac Doppler studies to rule out cardiac abnormalities must be done. Maternal blood tests should include blood tests for full blood count (to detect anaemia ), glucose screen ( GTT), haemoglobin electrophoresis (depending on ethnic group) , Kleihauer-Betke test for chronic feto-maternal haemorrhage , syphilis serology and glucose 6-phosphatase dehydrogenase activity. Investigations for infection screen ( parvovirus , rubella cytomegalovirus , toxoplasma , echovirus , coxsackievirus) should be performed.
If congenital heart block is diagnosed or brady-arrythmia detected then it is useful to test anti-Ro and anti-La antibodies. Referral to fetal medicine centre should be done for expert interdisciplinary approach and further investigations. Fetal blood sampling is invasive but most appropriate is carried out for blood count , fetal karyotype , blood gases , cultures ,assessment of fetal infection by PCR , liver function ( including serum albumin ) and metabolic tests ( urea and electrolytes ) and haemoglobin electrophoresis if indicated . Amniocentesis for assessment of fetal infection and karyotyping is another alternative.
b) Referral to a tertiary centre should be done for a decision on care, fetal surveillance and treatment. Counselling in sympathetic manner and discussion of implications and investigations required should be explained.
The fetal effects include rather than non-immune hydrops fetalis, premature delivery and stillbirth should be informed. Fetal serial scans every two weeks should be performed for features of severity of hydrops fetalis like extent of ascites , pleural effusions , scalp oedema and increased middle cerebral artery Doppler velocity indices. Increased MCA-PSV is indicative of anaemia and intrauterine transfusion is required after proper counseling.
Counselling should be done to inform the patient and partner about the risk of procedure regarding fetal loss and the benefit of recovery with follow-up.
Management in dedicated fetal medicine unit and presence of appropriate facilities will result in better prognosis.
Fetal hydrops due to parvovirus infection can spontaneously resolve in mild-moderately cases. Fetal blood sampling will allow assessment of fetal hemoglobin and reticulocyte count. Fetal reticulocytosis is a good prognostic sign and an evidence of recovery of anaemia . Conservative management and ultrasound follow-up should be done for monitoring of hydrops. If severe anaemia is detected, intrauterine transfusion can be considered as an option for treatment. If viability achieved , corticosteroids should be given at 34 weeks . Timing and mode of delivery should be decided in association of neonatologists. Management in dedicated fetal medicine unit and presence of appropriate facilities will result in better prognosis.
Posted by NIRMALA M.
Nirmala
a. The causes of non immune hydrops (NIH) may be idiopathic or due to many specific causes. Most common cause of NIH is cardiovascular defect in the fetus like hypoplastic right heart, hypoplastic left heart, AV canal defects, TGA, ebsteins anomaly. Hydrops mostly due to cardiac failure secondary to raised central venous pressure. Other CVS causes like SVT, atrial flutter which leads on to reduced cardiac cardiac output to mardium which in turn leads to depressed myocardial function and cardiac failure.
Chromosomal abnormalities like turners syndrome,
trisomies 21,13,18, triploidies can cause NIH.
Thoracic malformations like cystic adenomatous malformations of the lung, diaphragmatic hernia due to the pressure on the venous return lead to hydrops.
Fetal infections are more important cause for NIH especially parvovirus B19 and others like CMV, herpes, toxoplasmosis, syphilis due to fetal anaemia and myocarditis.
Sacrococcygeal teratoma causes hydrops because of high output cardiac failure.
Placental causes are chorioangioma and angioma of the cord.
In multiple pregnancy, TTTS is an important cause and the recipient twin is affected. Other causes are fetomaternal haemorrhage, metabolic, genetic conditions.

Investigations should include detailed ultrasound scanning including fetal echo showing four chambered view with outflow tracts in a fetomaternal unit by a Consultant.
Detailed anatomical examination should be carried by USS to look for any skeletal malformations, head, spine, femur, thoracic circumference, humerus.
Placental angiomas should be ruled out. Site of fluid collection should be noted whether ascites, pleural or pericardial effusion.
Soft tissue markers for chromosomal anomalies like cystic hygroma, echogenic bowel, choroid plexus cyst should be looked for.
Detailed cardiac USS and echo should be done to rule out any cardiac causes.
Liquor volume should be noted.
Dopplers especially peak systolic velocity of MCA, > 1.5 of the mean suggests fetal anaemia. Fetal blood sampling from umbilical cord without compromising the fetus to look for FBC (to measure HB, MCV), karyotyping, Virology, LFT to include proteins along with others, enzymes like pyruvate kinase, G6PD levels, acidosis.
Maternal bloods to look for fetomaternall hemorrhage by doing Kleihauer test, virology screen(TORCH), hemoglobin electrophorhesis and enzyme defects.

b. 50% hydrops fetalis are fatal if not treated. One third of hydrops subside without any treatment. However its difficult to find which hydrops have good prognosis without treatment. Therefore, those fetus with HB less than for mean for the gestational age should be treated with intrauterine transfusion. IUT is done by a feto maternal consultant preferably at the site of cord insertion to placenta or directly into the ventricle or directly into the straight portion of the hepatic vein. There is about 1% fetal loss due to IUT. About 94% improves with transfusion and hydrops clears in about 6 weeks after IUT. Reassurance given if hydrops settles as this would not lead on to any congenital abnormalities. No further follow up needed once hydrops settles.
If hydrops does not settle, Repeated transfusions might be needed depending on the fetal anaemia and follow up needed.
Posted by zara A.
a]Causes of hydrops are fetal structural abnormalities cardiovascular[ hypoplastic left ventricle;ebstein anamoly;supravetricular tachycardia,complete heart block,],thoracic anamolies[diaphragmatic hernia,,congenital cystic adenometiod malformation ],causes hydrops by imApairing venous return.Sacrococygeal teratoma and chorioangioma causes hydrops by high cardiac output failure.RENAL malformations can cause hydrops.Chromosomal causes include trisomy,tripliody,turners syndrome.FETAL infections cause hydrops include parvovirus B19 ,cytomegalovirus,syphilis ,rubella.herpes.HEMATOLOGICAL causes of hydrops are. fetomaternal haemorrhage ,alphathalasemia, genetic causes myotonic dystrophy,METABOLIC causes include gauchers disease.THE investigations should be undertaken on mother and foetus,to identify cause and management in sub sequent pregnancy.PATIENTshould referred to fetal medicine unit.FOETAL investigations are invasise and noninvasive.NONINVASIVE offered first .DEtailed fetal anamoly scan to look for cardiovascular anamolies ,renal anamolies and thoracic anamolies, and assess extent of hydrops ascites and hydrothorax.ASSESSMENT of fetal heart rhytham ,show brady cardia [heart block ]tachyarrythmia.CAREful evaluation of fetal skeltel abnormalities carried out,and look for soft markers of chromosomal disordes .PEAK SYSTOLIC flow in middle cerebral artery done, to predict fetal anaemia.Placenta and cord examined for haemengioma and vascular abnormalities.FETAL echocardiography and cardiac doppler studies done for heart anamolies.The invasive fetal investigations are fetal blood sampling for FETAL HB,and reticulocyte count hematocrit,karyotype ,PCR for fetal infections [parvovirus,herpes .cytomgalovirus.rubella,syphilis].liver function tests and enzyme studies.DNA extraction for single gene defects haematological disorders G6PD .Amniocentesis can be done for karyotype and infection screen not provide information on HB .Maternal blood taken kliehaur betkhe test to detect, feto maternal haemmorage,FBC and hb electophoeresis [for alpha thaLasemia]. Virology screening[TORCH paired booking serum],autoimmune screening anti R0 AND ANTILA if fetal heart block.b]THE patient should be sympathetically as she will be anxious. referred to fetal medicine unit involving consultant obstetrician and neonatologist . SHE SHOULD EXPLAIN that cause of hydrops is parvovirus,which causes anemia due to red cell destruction, reassure prognosis is good . it is not an indication for termination .The further management depends on severity of hydrops ,reticulocyte count ,and degree of anaemia .,.IF mild and reticuloyte count more than70% then serial moniteringof fetuswith middle cerebral artery doppler and usG done 2 weekly , most cases resolve spotaneously.IF hydrops progresses or severe and fetal anaemia [HB LESS FOR GESTATIONAL AGE OR MC A PSV >1.5 MOM] then intrauterine trans fusion carried out ,explain risks toparents [premature labour ,cord haematoma ,fetal loss].THe benefits are that it prevents hypoxia to fetal tissues,allow time to recover bone marrow .Routine antenatal care carried out ,vaginal delivery possible if no fetal maternal complication.
Posted by Penelope T.
a) Immune hydrops can be caused by red blood cell antibodies. The commonest is Rhesus (although this would not occur in this lady), and others include c, Kell and Duffy. Maternal group and antibody screen will elicit this.
Non-immune hydrops (NIH) has multiple causes. Chromosomal anomaly is one cause, particularly aneuploidy eg. Turners syndrome. The woman should be offered amniocentesis and fetal karyotype. Structural anomalies particularly cardiac and thoracic can also lead to NIH. These include left heart hypoplasia and tetralogy of fallot. Additionally fetal cardiac arrhythmias can lead to heart failure and hydrops. These include tachyarrhythmias (eg. SVT), and bradyarrhythmias (eg. congenital heart block, whihc can be associated with maternal SLE or anti-Ro and La antibodies). Thoracic lesions include CCAM (congenital cystic adenomatous malformation of the lung) and Chylo/Hydrothorax. Tertiary detailed anomaly ultrasound and fetal Echocardiography should be performed. Additionally, maternal ANA, double stranded DNA, anti-Ro antibody and anti-La antibody should be done.
Congenital infections particularly Parvovirus B19 can also lead to fetal hydrops. At the time of amniocentesis (as above), amniotic fluid should be sent for PCR for Parvovirus, Toxoplasmosis, Rubella, CMV, Herpes simplex, and Syphilis. Maternal serum IgG and IgM should be taken for each of these also.
Alpha thalassaemia major can result in hydrops, so both parents should have a haemoglobin electrophoresis done.
Rare genetic syndromes can also cause hydrops. These are usually associated with structural anomalies which can be examined for at the detailed ultrasound.
b) The parents should be offered support and discussion of causes/investigation and management. They should be counselled by a neonatologist and materno-fetal medicine specialist. Hydrops may resolve spontaneously so repeat ultrasound should be done in one week if it is not severe. If persistent or severe then fetal MCA doppler will give an idea of level of anaemia. Cordocentesis to check fetal haemoglobin level and give an intra-uterine transfusion should be discussed and arranged with consent. There are risks of cord accident, ruptured membranes, and fetal death. The majority of hydropics fetuses however will improve with transfusion and only a small proportion will need a second transfusion. Expectant management of the pregnancy is an alternative option however the parents should be aware of the risk of in-utero fetal demise in this setting.
Posted by R v P.
RVP

A healthy 37 year old Rhesus positive woman attends for her anomaly scan at 22 weeks gestation. The fetus is found to be hydropic. (a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks].

ABO incompatibility could be a cause of hydrops. Non Rh sensitisation that may cause Anti Kell/ Duffy and anti c antibodies could also cause hydrops.
Infective causes such as Toxoplasma/CMV and Herpes could cause hydrops (TORCH syndrome)
Other infections such as Parvo B19/Listeria and Syphilis could also be the cause.
Anuploeidy including Downs, Edwards and Patau Syndromes and Turners sydrome may also cause hydrops.
Structural heart disease including Aortic stenosis, Hypoplastic left ventrical (left heart), Pulmonary atresia/transposition or hypoplastic right ventricle (right heart are known to cause hydrops.
Defects of the conductive pathway of fetal heart including complete heart block and supraventricular tachycardia may also be the cause.
Inborn errors of metabolism such as G6PD deficiency, pyruate kinase deficiency, lysosomal storage disease and glycogen storage disease are also known to cause hydrops.
Thoracic and abdominal tumors and placental chorioangiomas may also be the cause.
Idiopathic hydrops also accounts for a significant proportion of the condition.


Investigations are determined by the suspected underlying cause.
Paired maternal IgG/ IgM (EIA or ELISA) and PCR could be used in suspected Parvo/CMV and Toxoplasmosis to confirm maternal infection. Vesicular fluid for culture or paired serum samples could be used in HSV. Syphilis serology should also be undertaken if infection is suspected.
parental karyotyping will help to suspect autosomal resessive inborn errors of metabolism in the fetus.
Fetal infection could be confirmed with Amniocentesis and PCR. Patient needs to be counselled and consented regarding the risks of the procedure including risk of miscarrige (1%) and culture failure(0.5%)
Chromosomal anomalies mentioned above could also be investigated with amniocentesis followed by FISH and culture.
Cardiac anomalies in the fetus could be diagnosed by an expert with four chamber and outflow track view.
Fetal CTG may reveal tachycardia and heart block.
Cell free fetal DNA is not widely available but could be used to determine fetal genotype to identify single gene disorders mentioned above.


(b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks].

Multideciplinary care involving obstetrician, fetal expert, paediatrician and virologist is essential.
Treatment should be individualised according to the severity of hydrops and maternal wishes.
Written information should follow clear verbal explanation about management options and risks.
If the condition is severe pation may opt for termination of pregnancy.
If she wishes to continue, she needs to be managed at a regional centre where advanced fetal therapy is available.
Fetal survaillance requires serial growth liquor and doppler measurements. The frequency needs to be determined by the severity of the condition. MCA - PSV is a non invasive method to assess the severity of fetal anaemia. >1.5 of multiples of median for the gestation is used as the cut off.
Bilirubin spectrophotometry in the amniotic fluid is an invasive way to monitor the degree of fetal anaemia. Serial amniocentesis is required for this with increased risk of infection.
Cordocentesis allows direct fetal Hb estimation as well as fetal transfusion. Packed cells should be crosmatched against maternal serum. However the procedure increases the risk of infection and preterm labour.
Timing and the method of delivery needs to be individualised according to the fetal condition and maternal wishes. Hydrops on its own is not an indication for C section


Posted by Ir A.
A healthy 37 year old Rhesus positive woman attends for her anomaly scan at 22 weeks gestation. The fetus is found to be hydropic. (a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks]. (b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks].

Ir
Fetal hydrops may have an immune or non immune etiology. Immune causes include anti D, anti C and anti Kell antibodies. Thus maternal blood should be checked for Rhesus antigen status, full blood count and indirect Coomb\'s test for atypical antibodies. Alpha thalassaemia may cause fetal hydrops, hence Hb electrophoresis should be done. The father\'s Rh antigen status and Hb electrophoresis should also be determined if possible. Non immune hydrops may be caused by chromosomal anomalies like trisomy 21, 18 or turner\'s syndrome. Amniocentesis and karyotype should be done to rule out these causes. A detailed fetal anomaly scan should be done to rule out congenital anomalies like congenital diaphragmatic hernia (CDH), congenital adenomatoid malformation of the lung (CCAML) or other anomalies. A fetal echocardiogram should be done to rule out cardiac malformations. The ultrasound scan should specifically look for any placental anomalies like chorioangioma. Doppler ultrasound and velocimetry of the middle cerebral artery should be done to look for fetal anemia. An infection screen should be done to rule out infectious causes eg parvovirus B19, rubella, herpes, CMV, toxoplasma and syphilis. Kleihauer-Betke test should be done for feto-maternal haemorrhage. Fetal blood sampling should be done and can be used for a variety of investigations like fetal blood count, karyotype and virology. I the patient has family history suggestive of inherited disorders like galactosemia, gaucher\'s disease specific tests may be undertaken. If the hydropic fetus shows bradycardia on ultrasound, investigations for maternal SLE like anti Ro and anti La antibodies may be done. Fetal hydrops may also be idiopathic or unexplained. This patient should be managed by a maternal fetal specialist.

b) The patient should be managed in a tertiary center by a maternal fetal specialist. Serial growth scans are required to monitor fetal growth. Doppler ultrasound and measurement of peak systolic flow in the middle cerebral artery can predict fetal anemia with 100% sensitivity. Fetal blood sampling should be offered to determine fetal blood count, reticulocyte count, karyotype and liver function tests. Presence of reticulocytosis is a good prognostic marker and may result in spontaneous resolution of infection. Before undertaking FBS, the mother should be counselled about the risk of intrauterine death in hydropic fetuses. If fetal anemia is severe, intrauterine transfusion (IUT) is indicated. Steroids should be given at 28 weeks for fetal lung maturity. IUT may be undertaken every 2 weeks with aim to deliver at 34 weeks. There is no role of antiviral agents in management of parvovirus infection. The mother should be provided with written information.
Posted by A H.
AH
a) This fetus has non-immune hydrops (NIH).The potential causes in his 27 year old healthy woman include chromosomal abnormalities Trisomy 21, 18 and 13, and Turners syndrome. For this woman the risk of trisomy 21 is low.
Fetal structural abnormalities, especially cardiac, pulmonaryand renal are associated with NIH.
Any condition which causes severe or prolonged fetal anaemia can lead to cardiac failure and hydrops. Fetal anaemia in this woman can be secondary to significant fetomaternal haemorrhage, or infections like Parvovius B19 which can cause transient aplastic anaemia. Other infections implicated are syphilis,CMV and toxoplasmosis. Fetal tumours, for example,sacrococcygeal tumors can also NIH.NIH is idiopathic in about 30% of cases.
Maternal investigations include a complete blood count and haemoglobin electrophoresis. If the patient is a carrier for one of the haemoglobinopathies, the bay can be affected. Maternal blood will also be taken for a TORCH screen, as well as testing for Parvovirus B19, and syphilis.
An abdominopelvic ultrasound scan will be done by a fetal medicine expert to identify any structural abnormalities or fetal tumor. The detection rate for cardiac abnormalities is only about 40- 50%.
Invasive testing available are amniocentesis and fetal blood sampling (FBS). The fetal loss rate at invasive testing is higher than for non hydropic fetuses.
Amniocentesis has a lower fetal loss rate than FBS. Karyotyping as well as an infection screen can be done. Results for karyotying can take up to 3 weeks.
However FBS is the preferred method of investigation as results of aryotyping can be available in a few days. Also testing for infections can be done. A full blood count can also be done on the sample to determine the haemoglobin concentration.

b) The patient will be referred to a tertiary centre for continued care. She will be counselled by the fetal medicine expert about the prognosis based on the results of investigations and her options.
FBS for Haemoglobin and reticulocyte count will done. Reticulocytosis is a good sign as recovery from anaemia and resolution of hydrops is likely.
Intra-uterine blood transfusion will be done if anaemia is severe.
There is an increased risk of fetal growth restriction so serial ultrasound for fetal biometry, liquor volume and if necessary umbilical artery doppler waveform will be performed.
If preterm delivery is anticipated maternal corticosteroids will be given to enhance fetal lung maturity, and reduce the risk of RDS and intracranial haemorrhage.
The neonatal team will be infomed about the patient.

Posted by Bgk H.
bgk

a. This fetus most probably suffer from non immune fetal hydroph, however anti Kell and anti Duff antibody need to be checked to conclude that. Non immune fetal hydroph may have no identified cause or idiopathic in about 30% but other cause need to be ruled out.

One of the causes is fetal structural anomaly. This can be due to cardiac abnormalities causing arrhythmias or heart block. Other structural abnormalities like polycystic kidney, anencephaly, fetal intra-abdominal tumor like adrenal or ovarian origin need to be rule out.

Fetal chromosomal abnormalities such as down syndrome, Edward syndrome or Patau syndrome is a recognized cause of fetal hydroph. Fetal haemolytic anaemia is also cause of fetal hydroph. This includes Alpha thalassaemia, severe G6PD or Sickle cell anaemia.
Fetal anaemia is one of the causes and this might be due to fetomaternal hemorrhage, or Parvovirus infection.

Fetal infection need to be ruled out. The possible infection can be due to Literiosis, Parvovirus or Congenital Rubella.

Investigation need to be taken form maternal and fetal. I will take maternal blood Hb electrophoresis to rule out any underlying thalassemia or haemoglobinopathies. It can be sent for for specific infection as seroconeversion of paired sera may signify an infection. I will also send her blood for antiphospholipid screening, including Anti Ro or Anti La that may lead to congenital heart block. I will perform a detail scan to look for fetal anomaly including fetal echocardiogram, and other structure. I will also perform a doppler for middle cerebral artery to non-invasively detect fetal anaemia.
Fetal blood sampling can be taken to objectively quantify fetal anaemia if present and also can be sent for culture to identify any organism.

b. I will explain to patient regarding the diagnosis in sensitive manner. Further referral to fetomaternal unit is indicated. Repeat scan need to be done to assess the severity of the hydroph. Fetal anaemia may need fetal blood transfusion. However presence of fetal erythroblasts may indicate fetal response to the fetal anaemia and the prognosis is good. If there is any evidence of hydrothorax or pericardial effusion in the scan symptomatic drainage for the fetus.

In very severe hydroph, termination of pregnancy can be offered. But continuation of pregnancy may be an option. Opportunity to meet neonatologist to discuss about the prognosis and future plan should be arranged.

Antenatal corticosteroids can be administered at 32 weeks gestational age and aim to deliver by 34 weeks if there is no further deterioration of the fetal well well being.

Repeat fetal blood transfusion is indicated if there is any evidence of anaemia on serial surveillance weekly scan. Risk of intrauterine death should be clearly informed and risk related to fetal blood transfusion such as infection, pain and fetal trauma need to be informed. Patient information sheet and support group neeeed to be given.
Posted by Mohamed D.
Mohamed
A) Causes are immune and non-immune. Immune hydrops is related to maternofetal blood group incompatebility as anti-C and other rare abnormal antibodies. Non immune cases are mostly due to fetal infections and mostly parvovirus B19 infection. Less commonly; varicella, rubella, CMV and toxoplasma. Other cases include massive feto-maternal haemorrhage. Also, fetal conditions as structural fetal anomalies, fetal arrythmias, congenital heart block, trisomies 13, 18 and 21, Turner syndrome, diaphragmatic hernia, CCAM, and pleural effusion. Rare conditions as placental chorioangiomas.
Ultrasound in a tertiary centre to role out other structural anomalies as it can part of a syndrome. Fetal echocardiography to diagnose cardiac lesions and arrythmias. Doppler ultrasound to measure PSV of the MSA to check for fetal anaemia. Amniocentesis for chromosomal analysis, and virology screen. Cordocenteses and fetal blood sample when anaemia is suspected and can be used for transfusion if needed. Maternal bloods for kleihauer test to diagnose feto-maternal haemorrhage, antibody screen for anti-C and other abnormal antibodies, and virology screening for TORCH, EBV and parvovirus.

B) PArvovirus is not teratogenic and is associated with low fetal loss rate. No evidence of long term effects on the baby with mild-moderate infection in most of the cases. Termination of pregnancy is an option before 24 weeks if she opt for. Referral to tertiary centre should be arranged for follow up. Ultrasound for estimation of the severity of hydrops should be done. Doppler on the MSA and estimation of PSV for assessment of fetal anaemia, and use of cordocenteses when severe anaemia is suspected and intrauterine blood transfusion is needed. Delivery and extrauterine transfusion should be considered after 34 weeks after steroid injection to promote lung maturity. Monitor maternal blod pressurre and urinary development of protinurea as she is at risk of developing preeclampsia (Mirror syndrome).
Posted by millionaire2004 A.
Ag
A healthy 37 year old Rhesus positive woman attends for her anomaly scan at 22 weeks gestation. The fetus is found to be hydropic. (a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks].
Recognise that this is associated with maternal anxiety and distress and approach her empathetically. In 30% of cases, no cause could be found. The cause could be red cell alloimmunisation (antibody against Kell and Duffy system). Antibodies against this blood systems cross the placenta and cause haemolysis of fetal red blood cell. Intrauterine infections (TORCHES) with toxoplasmosis,cytomegalovirus,hespes simplex,syphilis, and infection with hepatitis B, parvovirus B19, Coxsaxievirus type B can cause non-immune hydrops fetalis. Non-immune haemolytic causes are glucose-6-pyruvate dehydrogenase (G6PD) deficiency and pyruvate kinase deficiency. Problems in fetal red blood cell production such as alfa thalasemia and lethal congenital spherocytosis can cause hydrops. Severe fetal anaemia (due to parvovirus b19 infection or rbc production or destruction) can cause fetal hydrops. Fetal chromosomal anomalies such as triploidy, trisomies and Turner;s syndrome can cause hydrops. Fetal structural anomalies such as congenital cardiac anomalies, congenital cystic adematoid malformation of lung, renal anomalies, intrathoracic tumours, abdominal tumours (ovarian cyst), sacrococygeal teratoma can cause hydrops. Fetal cardiac arrhythmias can cause hydrops. Chorioangioma can be a cause of hydrops. Maternal disease associated with hydrops are type1 DM and presence of anti-Ro antibody.
Investigation could be performed on mother,fetus,placenta, neonate and via post mortem to obtain diagnosis. Obtain history regarding any previously affected fetus due to red cell alloimmunisation. Ask regarding exposure (timing) to known viruses such as parvovirus b19 or recent febrile illness. Ask about past history of infection with TORCHES organisms. Other affected child with G6PD deficiency could indicate possibility of this fetus being G6PD deficient if the fetus is male. Review antenatal screening results for thalasemia and irregular antibodies.. If not done and mother is from high risk ethnic group for thalasemia, send for Hb electrophoresis. If mother is a carrier for a-thalasemia, partner status need to be determined. If both of them are carriers (with 2 gene deletion) for a-thalasemia, the fetus has 1 in 4 chance of having Hb Barts. Review down syndrome screening results if any. Sent maternal blood for fbc (detect anaemia), u&e, serum urate and liver function test (deranged in hepatitis ). Elevated maternal serum alfa fetoprotein is a marker of fetal infection with parvovirus b19. Send blood for screening for TORCHES ,parvovirus b19 looking for IgM and IgG. Presence of IgM or seroconversion demonstrated in paired sera indicate new infection. Do glucose tolerance test to exclude maternal diabetes. Send for lupus anticoagulant and anti-Ro antibody in the presence of fetal brady-arrythymia. Detailed ultrasound scan of the fetus to look for associated anomalies such as overlapping fingers,rokkerbottom foot,cleft lip/palate,structural heart defects, cystic hygroma which could suggest chromosal anomaly. Also look for severity of ascites/hydrothorax. Look for presence of fetal brady-arrhthymia or tachy-arrhythmia by ultrasound. Do fetal blood sampling and sent for fbc (for anaemia), Coomb’s test (suggest hemolysis), karyotyping (for chromosomal anomaly), Hb electrophoresis (for alpha-thal) , PCR based infection screen and antibody screen for infection and for single gene defects such as G6PD deficiency,hereditary spherocytosis.. Amniocentesis can assess fetal karyotype and infection but unable to assess fetal Hb. Invasive testing is associated with fetal loss
(b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks].
Manage her in a tertiary centre by a multidisciplinary team consist of obstetrician, physician, neonatologist/paediatric surgeon, and midwife. Explain to the mother that it is associated with fetal loss rate of 9%. It does not cause any congenital syndromes. Assess her attitude towards the fetus, whether she wants to continue pregnancy or wants termination of pregnancy. However, tell her that this infection is not an indication for termination of pregnancy. If severe fetal anaemia, offer intrauterine fetal blood transfusion. This procedure is associated with risk of fetal loss. In the presence of marked fetal reticulocytosis (>20%), conservative management can be adopted as recovery of fetal anaemia is likely. Do 2 weekly ultrasound scan to look for resolution of fetal hydrops and to assess fetal growth. Umbilical artery Doppler can be done 2 weekly to assess fetal wellbeing. If appropriate, prolong pregnancy till acceptable gestation (ie 34 weeks)/ till evident of fetal lung maturity, delivery can be planned with consultation with neonatologist . Administer maternal antenatal corticosteroid to enhance fetal lung maturity and inform neonatologist if preterm delivery anticipated.
Posted by Ida I.
I.

A) Patient is having non immune hydrops. Non immune hydrops can be classified as idiopathic when there is no identifiable cause. Antibodies to the Duffy and Kell systems can cause fetal hydrops. A major fetal cause of fetal hydrops is fetal anaemia, and this is largely attributed to alpha Thalassemia major. Congenital heart diseases, such as atrial septal defect, ventricular septal defect and Ebstein\'s anomaly has also been identified as a causative factor for fetal hydrops. Presence of tachyrrhythmias or bradyrhythmias in a structurally normal heart can cause this condition as well. Severe fetal lung formation, such as congenital cystic adematoid malformation of the lung can also cause fetal hydrops. Chromosomal abnormalities, such as Trisomies and triploidy is also known to cause fetal hydrops. Infections, such as parvovirus B19, cytomegalovirus, HSV and toxoplasmosis is also a recognised cause of fetal hydrops. GEnetic diseases, such as Gaucher\'s dieasea or mucopolysaccharidosis are recognised causes. Other known causes are fetal renal and liver anomalies, such as renal vein thrombosis and biliary atresia.
Maternal diseases, such as hyperthyroidism, diabetes and anaemia are known causes. Placental causes of fetal hydrops include chorioangioma or umbilical vein thrombosis. Structural anomalies, such as sacrococcygeal tumours or achondroplasia, are also known to cause fetal hydrops.

Maternal investigations should include full blood count to exclude anaemia. Hemoglobin electrophoresis done to ascertain thalassemia status. Blood group and antibody screening done to confirm her blood group and rhesus and to detect antibodies. G6PD carrier status should be ascertained as this condition is associated with hemolytic anaemia. Kleihauer test to detect fetal cells in maternal circulation. Serology for toxoplasma, parvovirus, CMV and HSV to detect viremia. VDRL test to exclude syphilis. MGTT needs to be done to exclude diabetes. Thyroid function test, including antibodies done to exclude autoimmune hyperthyroidism. Anti Ro and Anti La antibodies done to exclude SLE.
Fetal investigations should include ultrasound to check site and severity of hydrops, biophysical profile, liquor volume and Doppler studies. Fetal echocardiogram essential to exclude any congenital cardiac diseases. Invasive fetal investigations include amniocentesis for karyotyping and to detect infections, such as parvovirus and CMV.. Fetal blood sampling can also be done for fetal full blood count to exclude anaemia, Coombs test and umbilical artery pressure.

B) She should be managed by a fetomaternal specialist. She should be counselled that risk of fetal loss is less than 1% if the infection is after 20 weeks gestation, and that one third of fetal hydrops can resolve spontaneously. However, the management depends on severity and the fetal outcome is difficult to predict. Serology for parvovirus IgM and IgG should be taken to detect seroconversion. She should have serial ultrasound scans every 1-2 weeks to monitor progress. She should be offered amniocentesis and fetal blood sampling, and the risks with each procedure explained ( premature labour and fetal death). She should also be informed that there is a therapeutic benefit with fetal blood sampling, as fetal transfusion can be done in the same sitting, and fetal reticulocyte counts can be taken to predict the outcome. Antenatal steroids given in anticipation of premature delivery. Aim for vaginal delivery and caesarean sections is reserved for obstetrics indication only.
Posted by Kiran  J.
A healthy 37 year old Rhesus positive woman attends for her anomaly scan at 22 weeks gestation. The fetus is found to be hydropic. (a) Discuss the potential causes and your investigations to confirm the diagnosis [14 marks]. (b) Discuss the subsequent antenatal management given that hydrops is due to parvovirus infection [6 marks].

(Iam sorry Ive been on night shifts all week hence I could not post my answer before hand, can you please mark)


a:The potential causescan be divided as maternal or fetal. causes include infections like parvovirus B19 infection,CMV,Syphillis,toxoplasmosis,adenovirus,Hep B,Listerioses.Investigations for infectionc include maternal history of exposure,ultrasound markers for hydrops and ascites and Raised Middle cerebral artery Peak systolic Velocity >1.5 mom indicationg severe fetal aneamia.More invasive investiagtions include fetal blood sampling,amnicentesis for IgM and IgG or viral genome PCR.Matrnal blood can be taken in that case for serology and PCR and paired with booking bloods to see evidence of seroconversion.
Structural conditions are cardivascular abnormalities of fetus i e atrial septal defect,ventricular septal defect,hypoplastic left heart,ebstien anomaly,premature closure of ductus,cardimyopathy.This can be investigated by a detailed structural heart survey on USS and a fetal echocardiogram.Assessment of featl heart on USS can also show tachrythmias or bradycardia( or heart blocks) which can esclate investigations like maternal autoimmune screen and anti Rho antibodies due to its association of Maternal SLE with congenital heart blocks.
Other causes pertaining to fetal thorax are Cystic Adenamatoid malformation of the lung, pulmonary sequesteration,diaphragmatic hernia,bronchogenic cyst,intrathoraci mass.Again a detailed structural review on USS coupled with finding of polyhydramnios due to compression of esophagus due to mass can impair swallowing .longstanding compression of fetal lung due to hernaited abdominal contents can cause pulmonary hypoplasia.Ultrasound can also help in identifying other structural causes of hydrops which include placental chorioangioma,saccrococcygeal teratoma,umbilical cord heamangioma,and AV malformations.
Aneuploidy can be a potential cause of hydrops and ultrasound markers for down/Turner syndrome or triploidy may be present and confirmation with a fetal Karyotype using FISH to confirm anueploidy.
Other fetal causes include alpha thallasemia major for which maternal blood for Haemoglobin electrophoresis can be taken from mother if approprtiate ethinic background.
Fetal causes also includegenetic syndromes including anthrogyroposis,myotonic dystrophy and in born errors of metabolism like Gauchers disease,Galactosilidoses,red cell disorders such as G6 Pdehydrogenase deficiency and heriditary sperocytosis.investigation include fetal blood sampling for single gene defects.
Maternal invistigations include Kliehaur betke test for for chronic fetomaternal haemorhage,maternal red cell antibodies for example AntiKell,Duffy,c,C,and E for non Anti D isoimmunization.This can be coupled with fetal Hb and reticulocyte count to assess for fetal aneamia.

B:
Subsequent management in the face of Hydrops indicates that the fetus has moderate to severe fetal aneamia.If in a district set up make urgent refferal to fetomaternal tertiary level care unit and involve multidisciplinary care including Virologist,haeamtologist,paidatrician and fetomaternal specielist.
Cordocentsis is performed and councell regarding procedurre related loss of 1%.Cordocentesis should be done in a unit equipped with facilities for urgent transfusion if fetal aneamia confirmed. Fetal blood transfusion should be done at the site of cord insertion,alternative sites include straight portion of hepatic vien or intraventricular.The family should be councelled of risk of perinatal death is <10%.During transfusion there is risk of fetal arrythmias which can be treated by administration specific cardiotrophic drugs to the fetus.
She should be followed up every 1-2 weeks after blood transfusion with USS and Middle cerebral artery Peak systolic volume dopplers and There is a 94% chance of resolution of severe fetal aneamia 6 weeks after blood transfusion.