Non Immune Hydrops – An Enigma

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

1. 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.
2. 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.
3. 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.
4. 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.
5. Fatusic Z. Nonimmune Hydrops Fetalis. Donald School Journal of Ultrasound in Obstetrics and Gynecology 2007; 1(1): 105-110.

Citation

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