Fig. 3: Color Doppler demonstrates normal flow in the heart. Note the hydrops (arrows).
Shortly after, the patient delivered a premature 832g female infant. The karyotype was normal. The autopsy revealed multiple anomalies such as hydrops, esophageal atresia with tracheoesophageal fistula, single umbilical artery, low set ears, and blood under the tentorium cerebelli. The pericardial sac contained 50 ml of blood with clots. The fetal heart was otherwise normal. No cause could be found for this pericardial hemorrhagic effusion with non-immune hydrops.
The incidence of non-immune hydrops is about 2.5-6.6:10,0000 deliveries, but hemorrhagic pericardial effusion has never been reported in a fetus and its prevalence is unknown1.
In our case, the pericardial effusion presented as a large hyperechoic mass, poorly differentiated from the myocardium. The mass circumscribed the fetal heart and was pulsatile. The lungs were compressed.
The etiology of hemopericardium in infants includes chest trauma either accidental or iatrogenic (i.e.: birth trauma, closed chest massage), generalized diathesis, pericardial teratomas, and rupture of cardiac aneurysms or diverticula2. Although a birth trauma cannot be excluded, the presence of blood in the posterior fossa might suggest a bleeding diathesis in this case. No pericardial or cardiac lesion could be found at autopsy.
Hemopericardium, as any pericardial effusion, may induce cardiac tamponade with cardiac decompensation. This might explain the fetal hydrops in our case. Hemopericardium has been associated in children with pericardial teratoma, with cardiac aneurysm rupture, and with generalized bleeding diathesis2. Furthermore, in our case, there were a single umbilical artery, esophageal atresia with tracheoesophageal fistula, and low set ears.
The differential diagnosis includes other hyperechogenic cardiac and pericardiac masses, such as: fibroma, rhabdomyoma, myxoma, teratoma, and hemangioma3. In these tumors, the echogenic mass (single or multiple) is more localized and not around the fetal heart, as in our case.
Pulmonary malformations (adenomatoid malformation, and sequestrum) must also be included in the differential diagnosis; an intrapericardial extralobar sequestration, appearing as a solid mass, has been described in the pericardium after birth4.
Diaphragmatic hernia was also possible, but the continuity of the diaphragm was confirmed by sonograms. In the other known causes of fetal pericardial effusions (Rh disease, cardiac failure, hypoalbuminemia, immune diseases, renal dysplasia, pericarditis, other causes of hydrops, and normal transitory pericardial effusion of the second half of gestation), the fluid accumulation appeared always hypoechoic5,6.
It depends upon associated anomalies and upon cardiac function. The prognosis of nonimmune fetal hydrops is very poor with a perinatal mortality rate between 70 to 90 percent1.
Sonography must search for signs of heart failure. When the diagnosis of a pericardial hemorrhagic effusion with hydrops is made before viability, the option of pregnancy termination should be offered.
1. Romero R, Pilu G, Jeanty P et al.: Nonimmune hydrops fetalis. In Prenatal diagnosis of congenital anomalies, East Norwalk, CT: Appleton & Lange, 1988; pp414-426
2. Swischuk LE: Pericardial abnormalities. In Imaging of the newborn, infant, and young child. Baltimore, Williams & Wilkins, 3rd edition, 1989; 334-338
3. Malisch TW, Jeanty P: Cardiac fibroma. The Fetus. 1991,p 2746-1
4. Levi A, Findler M, Dolfin T, et al.: Intrapericardial extralobar pulmonary sequestration in a neonate. Chest. 1990; 98:1014-1015
5. Shenker L, Reed KL, Anderson CF, et al.: Fetal pericardial effusion. Am J Obstet Gynecol. 1989; 160:1505-1507
6. Jeanty P, Romero R, Hobbins JC: Fetal pericardial fluid: a normal finding of the second half of gestation. Am J Obstet Gynecol. 1984; 5:529-532