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Radiology

Case of the Month

Case No. : 63
Month : March
Year : 2004
Contributor : Dr. Ajay Morani

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Discussion


CLINICAL PROFILE :

A 23-year-old lady was referred for routine antenatal ultrasound scan. Her blood group was AB +ve. Her blood sugar was normal and she was negative for TORCH titers.

USG features were s/o hydrops in a 17 weeks fetus. Fetal echocardiography revealed an echogenic cardiac focus and fetal Doppler velocimetry was normal. So the diagnosis of non-immune hydrops fetalis was made.


RADIOLOGICAL FINDINGS:

Ultrasound examination (Figs. 1-5) revealed a 17 weeks fetus which had scalp and subcutaneous edema with an avascular sac
behind the neck which was suggestive of a cystic hygroma. In addition, there was abdominal wall edema, a small left pleural effusion, abnormal cardiac focus and echogenic bowel. This echogenic focus, echogenic bowel and cystic hygroma in the fetus pointed towards a fetal chromosomal anomaly as the cause of hydrops.

Fig. 1
Fig. 2
Fig.1
Fig. 2
Fig. 3
Fig. 4
Fig. 3
Fig. 4
Fig. 5
Fig. 5

The couple was counseled and advised amniocentesis, but they did not give consent for it and instead opted for termination of pregnancy without further investigations The little finger of the fetus was taken for the fetal karyotype which revealed Trisomy 21 in the fetus.
Fig. 6
Fig. 7
Fig. 6
Fig. 7
Fig. 8
Fig. 8

Morphologic Features: The abortus (Figs 6-8) was a male fetus which showed generalised edema all over the body and a swelling behind the neck ( cystic hygroma) .

DISCUSSION:

Hydrops is defined as an abnormal accumulation of serous fluid in at least 2 body cavities or tissues of the fetus. It actually represents the terminal stage for many fetal insults and indicates the onset of fetal decompensation.

Classification:
1. Immune
2. Non-Immune (1 in 1500 - 1 in 4000)

Sonographic features:

a. Pleural Effusion
b. Ascites
c. Pericardial Effusion
d. Subcutaneous Edema
e. Placental Edema
f. Altered Arterial & Venous doppler velocimetry
g. Altered fetal well-being.

The pathologies which may mimick hydrops are: Pseudoascites , Pseudopericardial effusion which are usually less than
2mm rim each & depend on the angle of the ultrasound probe and usually occur with the local pathologies.

Etiopathology:
The causes may be
1. Maternal
2. Placental
3. Fetal (most common) which include
-------- CVS ( 25 %)- arrythmias, infections and heart failure
-------- Chromosomal anomalies
-------- Anemia:Thalassemia,Parvo-virus,G6PD Deficiency,Hemorrhage(Twin-Twin transfusion,Cranial,Tumoral or Placental
------- -hemorrhage)
-------- Neck & Thoracic- cystic hygroma, cystic adenomatoid malformation of lung
-------- GI & GU causes
-------- Infections ( CMV, Toxoplasma, Parvovirus)
-------- Genetic

These causes lead to hypoproteinemia (renal loss, liver affection), fetal heart failure or obstruction of the venous or lymphatic
drainage which result in abnormal fluid collection in different body parts.


Prenatal Assessment:
History
Sonography : Markers of chromosomal abnormalities,. Fluid collection in body cavities, Organs (renal, liver disorders),
Bladder, Bones(length & density to rule out bony anomalies associated with hydrops), Stigmata of congenital infections
(microcephaly, cerebral calcification etc.), Fetal Echocardiography, Placenta (chorioangioma, AVM), Doppler arterio-venous
velocimetry., BPP Maternal investigations( Hb. Blood gp, RBS , TORCH titers etc.). Fetal ( karyotype, blood sampling,
amniocentesis)
Ultrasound markers of fetal chromosomal anomalies include CNS abnormalities in the form of holoprosencephaly,
Dandy-Walker malformation etc. Cystic hygroma, increased nuchal thickening, hydrothorax, cardiac malformations,
diaphragmatic hernia, omphalocoele, duodenal atresia, obstructive uropathy, echogenic bowel, facial anomalies, club foot etc.

Medical management
Infections- Antibiotics, antiviral drugs
Arrythmias- antiarrythmics which cross the placenta)

Ultrasound guided management:
Anemia ( Intrauterine blood transfusion to fetus)
Twin Gestation (Serial amniocentesis of the hydropic fetus, laser ablation or cord embolisation for the communicating
interplacental vessels)
Ascites, Pleural Effusion. ( Diagnostic & Therapeutic aspiration to relieve the fetal respiratory distress ,shunt
placement in cases of refractory fluid collection)
Obstructive Uropathy ( vesico-amniotic shunt)
Predelivery USG
Preeclampsia, polyhydramnios ( pre-delivery aspirations, amniocentesis to avoid dystocia and post-partum hemorrhage)

Counselling of the couple to terminate the pregnancy, to get the autopsy in case of fetal demise, to have all the investigations done
not only to diagnose the cause but also to evaluate the recurrence risk to avoid the similar problem in the future pregnancy.

Neonatal Resuscitation is usually needed in the form of airway, breathing and circulation support to the fetus with pleural or ascitic
fluid aspiration to relieve the respiratory distress.




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