Author Information
Vora P*, Jassawalla MJ**, Bhalerao S***, Bansal V****.
(* Third year resident, ** Medical Director, *** Honorary, ****
Associate Professor,
Department of Obstetrics and Gynecology, Nowrosjee Wadia Maternity
Hospital, Mumbai, India.)
Abstract
A hydropic fetus without maternal Rh isoimmunization
is indeed a rare phenomenon. Prognosis of a future pregnancy also
poses a challenge to the obstetrician. We present a case of non
immune hydrops fetalis (NIHF) in a Rh
negative non iso-immunized mother and the challenges faced in
reaching the etiology of hydrops.
Introduction
NIHF is defined as excessive extra vascular collection
of fluid in the interstitial compartment secondary to
disruption of normal cardiovascular interstitial fluid homeostatic
mechanisms.
Case Report
Twenty nine year old primigravida,
conceived with assisted reproduction, was referred with an ultrasound
finding of hydropic fetus at 29.4 weeks of gestation. Her hemoglobin
(Hb) was 9.4 gm% and Hb
electrophoresis showed no evidence of hemoglobinopathy. Her blood
group (BG) was O negative, husbands BG was
O positive, Indirect Coombs test was
negative and Rh titres (direct
and indirect) were nil.
Glucose tolerance test was normal. Her
TORCH titres and double stranded DNA were negative. Antinuclear
Antibody was weakly positive but hematologist suggested no
active management. Color Doppler (CD) at 30
weeks gestation revealed single viable fetus, in
breech presentation, with an amniotic
fluid index (AFI) of 12 cm, an estimated
weight (EBW) of 2.5 kg, a composite
gestational age of 33.5 weeks (falsely increased due to the hydrops),
with scalp edema, pericardial effusion, cardiomegaly
and ascites. Middle cerebral
artery – peak
systolic velocity(MCA-PSV)
was in zone A
suggesting fetal anemia (Figure 1 & 2).
Fetal 2D echo was done and was normal.
Steroids were given for anticipated preterm birth. Under
tocolytic and antibiotic cover, cordocentesis was performed and blood
was sent for direct Coombs
test (DCT), TORCH PCR and Parvovirus
B 19 which were negative. BG was O positive and karyotype was normal.
However the Hb of the fetus was 7.3 gm% and haematocrit of 22.8.
Hence intrauterine transfusion (IUT) was
performed and 90 ml of fresh O negative,
double packed, CMV negative, irradiated blood was transfused into the
umbilical vein at the junction of the umblical
cord to the placenta.
HEMOGLOBIN
|
HEMATOCRIT
|
MCA-PSV
|
|
DONOR BLOOD
|
24.2
|
75.7
|
|
CORD BLOOD
|
7.3
|
22.8
|
50 CM/SEC
|
Figure 1. USG showing hepatomegaly, spleenomegaly and ascites
Figure 2. USG showing scalp edema
Figure 3 . Pre-transfusion MCA-PSV.
Figure 4. Post transfusion MCA-PSV.
CD two days post procedure showed MCA-PSV reducing and in zone B
(37.3 cm/sec) with an impression of resolving hydrops. Hence a repeat
CD was planned after a week. However, on
the sixth day post procedure, patient complained of abdominal
discomfort. On examination mild anemia with a pulse rate of 92/min
and blood pressure of 120/80 mm Hg were noted. On per abdomen
examination uterus was 32 weeks with breech presentation with fetal
heart rate dropping to 60 beats per minute. CD revealed single viable
breech with AFI of 21 cm and EBW of 2.7 kg with no retroplacental
collection and MCA-PSV in zone A. An
emergency lower segment cesarean section was done at 31.5 weeks of
gestation. She delivered a male child, 2.6 kg with APGAR score of
5/10, 6/10. The neonate did not cry immediately after birth and was
intubated. Heart rate was 180 beats per minute and respiratory rate
was 40 beats per minute with shallow breathing and jerky respiration.
Neonate was put on ventilator, intravenous fluids and antibiotics.
Cord blood showed Hb of 8 gm%, O positive BG and bilirubin of 5mg/dl.
An exchange transfusion was performed. Neonatal death occurred within
six hours of life due to respiratory distress syndrome. Lactation
suppression and injection Anti - D 300 microgram was given to the
mother and she had an uneventful recovery.
Discussion
The relative incidence of hydrops fetalis has changed dramatically in
the last 2 decades due to prevention of
immune related hydrops fetalis secondary to Rhesus isoimmunization
by Rh anti D prophylaxis. With the use of Anti - D prophylaxis
immunological causes account for
less than 20% of the cases.[1] NIHF is more
common than hydrops fetalis and the incidence of NIHF in Southeast
Asia varies from 1 in 500 to 1 in 1500.[1]
Maternal causes of NIHF are idiopathic, alpha thalassemia, TORCH
infections, thyrotoxicosis, diabetes
mellitus, preeclampsia, anemia, hypoprotenemia. Fetal causes are
cardiovascular diseases leading to low or high output cardiac
failure, chromosomal abnormalities, thoracic masses, intrauterine
infections, twin pregnancies, renal malformations, placental
abnormalities, metabolic conditions.[1]
Lysosomal storage disorders like mucopolysaccharidosis 7,
Niemann–Pick disease, galactosialidosis, mucolipidosis and type 2
gaucher disease must be considered when dealing with recurrent
hydrops. Mechanism by which these present as NIHF is still
unknown.[2,3] Other rare cause of recurrent
fatal fetal hydrops is nucleotide substitution in the erythrocyte
Beta spectrin gene.[4] Intrauterine
investigations done include CD with 2D- ECHO and
MCA-PSV. In addition cord blood Hb, DCT, TORCH, parvovirus and
karyotype can be offered. In utero treatment includes tertiary care
management, intraperitoneal and intrauterine transfusions, fetal
thoracocentesis or pericardiocentesis, transplacental drug therapy
for fetal dysarrhythmias and treatment of
polyhydramnios. The management at birth consists
of vigorous and zealous resuscitation, appropriate treatment
with fluids, diuresis, dialysis,
treatment of cardiac failure, partial or total exchange transfusions.
It is also important to screen for metabolic disorders when other
common etiologies have been excluded.[1,5]
Though rare, obstetricians must keep in mind the possibility of a
neonate with NIHF in a Rhesus non immunized mother.
References
- Begum N, Kazal RK, Anwary SA, Nahar KN, Shamsunnahar PA, Akhter N. A neonate with Non –immune Hydrops Fetalis in Rh Non-immunized Mother. Bangladesh J Child Health 2010; 34(2): 70-72.
- Van de Kamp JM, Lefeber DJ, Ruijter GJ, Steggerda SJ, den Hollander NS, Willems SM, et al. Congenital disorder of glycosylation type 1a presenting with hydrops fetalis. J Med Genet 2007; 44(2): 277-280.
- Cheng Y, Verp MS, Knutel T, Hibbard JU. Mucopolysaccharidosis type 7 as a cause of recurrent non-immune hydrops fetalis. J Perinat Med 2003; 31: 535-537.
- Gallagher PG, Weed SA, Tse WT, Benoit L, Morrow JS, Marchesi SL et al. Recurrent fatal hydrops fetalis associated with a nucleotide substitution in erythrocyte beta-spectrin gene. J Clin Invest 1995; 95(3): 1174-1182.
- Fatusic Z. Nonimmune Hydrops Fetalis. Donald School Journal of Ultrasound in Obstetrics and Gynecology 2007; 1(1): 105-110.
Vora P, .Jassawalla MJ, Bhalerao S, Bansal V. Non Immune Hydrops – An Enigma. JPGO 2016. Volume 3 No. 2. Available from: http://www.jpgo.org/2016/02/non-immune-hydrops-enigma.html