This document discusses nonimmune hydrops fetalis (NIHF), which accounts for 90% of cases of hydrops fetalis. NIHF has numerous potential underlying causes and carries significant risks for both the fetus and mother. The document defines NIHF, discusses its differential diagnosis and evaluation, potential etiologies, presentation, prognosis, management options, and recommendations from clinical practice guidelines.

1. NONIMMUNE HYDROPS FETALIS
Dr Bibek Mohan Rakshit
MD; MRCOG; DNB; MNAMS
Associate Professor (Gynaecology & Obstetrics)
Burdwan Medical college and Hospital

2. BACKGROUND
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

3. DEFINITION.......
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

5. DIFFERENTIAL DIAGNOSIS
Immune hydrops
 Isolated fluid collection

Ascites
 Pleural effusion
 Pericardial effusion

Skin Oedema
 Polyhydramnios or placentomegaly.


6. ETIOLOGY.....

Aneuploidy

Cardiovascular abnormalities







Structural
Functional
Arrhythmias
Tachyarrythmia
Bradyarrhythmia
Vascular

Shunt or Thrombosis
Thoracic abnormalities






CCAM
Diaphragmatic hernia
Masses
Pulmonary sequestration
Chylothorax
Airway obstruction
•

7. Non cardiac/thoracic anomalies



Lymphatic
Gastrointestinal
Genitourinary
Neurological/Decreased movement
Anaemia

Decreased production




Increased loss




Parvovirus
Infiltration/Storage diseases
Myeloproliferative/ congenital leukaemia
Intrinsic cell abnormality
(alpha-thalassemia, G6PD, membrane…)
Hemangioma
Haemorrhage/abruption

8. 
Infectious disease

toxo, syphilis, varicella, adenovirus,
coxsackie
Metabolic storage disease
 Placental

TTTS/TRAP
 Trauma
 Cord anomalies
 Chorio-angioma


MATERNAL DISEASE
Idiopathic

Decreasing significance with knowledge

9. PRESENTATION




35% incidental finding
Size > dates
Decreased fetal movement
Mirror Syndrome in mother

10. IMPORTANT HISTORY

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


11. EVALUATION.....
Detailed ultrasound
 Anatomy
 MCA PSV
 Echocardiogram

12. LABORATORY INVESTIGATIONS
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


13. Infant




Amniocentesis
Karyotype
PCR for TORCH pathogens
Storage for further testing
Cordocentesis
RBC for anaemia
Metabolic/genetic tests
UTERINE VENOUS PRESSURE

14. PROGNOSIS
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

15. MANAGEMENT OPTIONS
Termination

Selective therapeutic intervention if
possible
Ongoing pregnancy with
hydrops

Monitor for PET/Mirror syndrome

Consider neonatal palliation


16. INTERVENTION
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

17. SOGC CLINICAL PRACTICE GUIDELINES
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

18. 4.Investigation for maternal–fetal infections, and alphathalassemia 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. (II2A)
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)

19. 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