Dr Bibek Mohan RakshitMD; MRCOG; DNB; MNAMSAssociate Professor (Gynaecology & Obstetrics)Burdwan Medical college and Hospital

Nonimmune Hydrops Fetalis (NIHF) is now responsible for 90% of all hydrops.

It has an estimated incidence of 1/1500 to 1/3800 births.

The pathogenesis of NIHF is not clear, but it is associated with numerous potential mechanisms and underlying disorders.

NIHF has an obvious poor prognosis for the fetus, but can also have maternal consequences as well with a 10% incidence of Mirror syndrome.

Newzealand maternal fetal medicine network

NIHF is established by the ultrasound findings of at least two of the following

Ascites Pleural effusion: ANY fluid abnormal Pericardial effusion: > than 2mm Skin edema: > than 5mm on chest and

scalp Polyhydramnios: Max pocket > 8cm, or AFI >

24cm Placentomegaly: Thickness >4cm

Immune hydrops Isolated fluid collection

Ascites Pleural effusion Pericardial effusion

Skin Oedema Polyhydramnios or

placentomegaly.

Aneuploidy Cardiovascular abnormalities

Structural Functional Arrhythmias Tachyarrythmia Bradyarrhythmia •

Vascular

Shunt or Thrombosis

Thoracic abnormalities CCAM Diaphragmatic hernia Masses Pulmonary sequestration Chylothorax Airway obstruction

Non cardiac/thoracic anomalies Lymphatic Gastrointestinal Genitourinary

Neurological/Decreased movement

Anaemia Decreased production

Parvovirus Infiltration/Storage diseases Myeloproliferative/ congenital leukaemia

Increased loss Intrinsic cell abnormality (alpha-thalassemia, G6PD, membrane…)

Hemangioma Haemorrhage/abruption

Infectious disease toxo, syphilis, varicella, adenovirus,

coxsackie Metabolic storage disease Placental

TTTS/TRAP MATERNAL DISEASE

Trauma Cord anomalies Chorio-angioma

Idiopathic Decreasing significance with

knowledge

35% incidental finding Size > dates Decreased fetal movement Mirror Syndrome in mother

Personal and family history to look for inheritable disorders associated with Alpha thalassemia Metabolic disorders Genetic syndromes

Infectious exposures Parvovirus

Consanguinity Previous baby with hydrops

Detailed ultrasound Anatomy MCA PSV Echocardiogram

Mother Blood group and RBC antibody screen

Baseline BP and urinalysis

FBC and film screen for thalassemia

Parvo, toxo, rubella, syphillis,

HSV(if recent primary infection)

Kleihauer-Betke LFT, urea,urate,electrolytes

Infant Amniocentesis Karyotype PCR for TORCH pathogens Storage for further testing

Cordocentesis RBC for anaemia

Metabolic/genetic tests UTERINE VENOUS PRESSURE

The overall perinatal mortality rate is 50 – 98%.

There has been no significant change over past 15 years.

Mortality rates will vary according to Gestation (earlier has worse

prognosis) Pleural effusions (worse prognosis) Underlying etiology

Termination Selective therapeutic intervention

if possible Ongoing pregnancy with hydrops

Monitor for PET/Mirror syndrome Consider neonatal palliation

If for active intervention Monitor with CTG’s or BPP’s 2. Delivery at tertiary care center 3. Consider risks of:

birth trauma PPH non reassuring fetal heart dystocia caesarean

Low recurrence if no inheritable disorder identified

Recommendations .. 1. All patients with fetal hydrops should be

referred promptly to a tertiary care centre for evaluation. Some conditions amenable to prenatal treatment represent a therapeutic emergency after 18 weeks. (II-2A)

2. Fetal chromosome analysis and genetic microarray molecular testing should be offered where available in all cases of non-immune fetal hydrops. (II-2A)

3. Imaging studies should include comprehensive obstetrical ultrasound (including arterial and venous fetal Doppler) and fetal echocardiography. (II-2A)

SOGC GUIDELINES.....2013

4.Investigation for maternal–fetal infections, and alpha-thalassemia in women at risk because of their ethnicity, should be performed in all cases of unexplained fetal hydrops. (II-2A)

5. To evaluate the risk of fetal anemia, Doppler measurement of the middle cerebral artery peak systolic velocity should be performed in all hydropic fetuses after 16 weeks of gestation. In case of suspected fetal anemia, fetal blood sampling and intrauterine transfusion should be offered rapidly. (II-2A)

6. All cases of unexplained fetal hydrops should be referred to a medical genetics service where available. Detailed postnatal evaluation by a medical geneticist should be performed on all cases of newborns with unexplained non-immune hydrops. (II-2A)

SOGC GUIDELINES.....2013

7. Autopsy should be recommended in all cases of fetal or neonatal death or pregnancy termination. (II-2A) Amniotic fluid and/or fetal cells should be stored for future genetic testing. (II-2B)

8. SOGC GUIDELINES.....2013