Prenatal diagnosis of intrapericardial teratoma

PRENATAL DIAGNOSIS, VOL.

10,199-202 (1990)

SHORT COMMUNICATION

PRENATAL DIAGNOSIS OF INTRAPERICARDIAL TERATOMA M. ALEGRE, M. TORRENTS, E. CARRERAS, C. MORTERA, V. CUSi AND J.

M. CARRERA

Instituto Dexeus, Bonanova, 67.0801 7 Barcelona, Spain

SUMMARY '4 case of intrapericardial teratoma diagnosed by ultrasound before birth is described.

Planning for delivery and immediate neonatal intensive management allowed successful cardiac surgery, full resection of the tumour and full recovery. K E Y WORDS

Intrapericardial teratoma

Prenatal diagnosis Surgical treatment

INTRODUCTION Primary cardiac tuniours in infancy and in the fetus are rare. Most ventricular tumours are rhabdomyomas, fibromas, or teratomas which frequently result in obstruction to the outflow of blood from the heart. We described an intrapericardial tumour accompanied by a large pericardial effusion detected during fetal life by ultrasound and successfully removed by cardiac surgery.

CASE REPORT

4 29-year-old pregnant woman was examined by routine ultrasonagraphy at 10 weeks of gestation in another hospital and suspected of having twins. However, since the development of only a single fetus was seen on successive examinations, the patient was told that only one fetus had survived. Further obstetrical ultrasound examination at 35 weeks of gestation disclosed an abnormal image in the thorax of the fetus which was considered to be a possible hydrothorax. The patient was then referred to our hospital to confirm the diagnosis and for advice on further obstetrical management. The history included an abortion during the first trimester in 1981 and a normal-term delivery in 1983. On ultrasound examination a female fetus in cephalic presentation was seen at the 36th week. The biparietal diameter was 9.3 cm and the cephalic and abdominal areas were 83 and 100 cm2, respectively. The femur length was 7.3 cm. The placenta was located on the anterior wall of the uterus, grade I of Grannum, and a moderate hydramnios was present. Different ultrasonic sections of the fetal thorax disclosed the presence of a large pericardial effusion that practically filled the left hemithorax. The heart was morphologically normal: both atria and ventricles as well as the valves and septa were easily seen, with normal ventriculo4ddressee for correspondence: E. Carreras, Instituto Dexeus, Dept. Obstetricia y Ginecologia, P" Bonanova, 67.08017 Barcelona, Spain.

0 197-385 1/90/030 199-04$05.00 0 1990 by John Wiley & Sons, Ltd.

Received27 February 1989 Revised 1 Seplember 1989 Accepted5 November 1989

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Figure 1. Intrapericardial mass close to the left atrium. The heart is morphologically normal. Note the large pericardial effusion

arterial connections. The heart rate was normal (140 per min) and no rhythm disturbances were detected. A solid heterogeneous mass of approximately 2 cm in diameter was seen within the pericardial space, attached to the left part of the heart but clearly outside the cardiac cavities, close to the left atrium and the aorta (Figure 1). No other malformations were detected. Ascites was not visualized and the lung parenchyma maintained the correct size and echogenicity for 36 weeks of amenorrhea. The final echographic diagnosis was an intrapericardial tumour, probably a teratoma. Fetal echocardiography was performed in order to obtain further information about the presence and location of the tumour, and to evaluate the large pericardial effusion and possible signs of cardiac tamponade. Cardiac structures were confirmed to be normal. Outstanding features were a dominant right ventricle with relatively reduced left cardiac cavities and a large pericardial effusion. The right atrial wall showed abrupt movements that suggested a degree of cardiac tamponade. Intracardiac Doppler blood flows were normal as was that in the thoracic aorta. Umbilical cord and inferior vena cava Doppler flows demonstrated variations related to the constant diaphragmatic fetal movements, which imposed a pulling pump on the venous return, so that it contributed to the ventricular filling overcoming the signs of cardiac tamponade. Fetal Doppler haemokinetics at the arcuate and umbilical arteries were thought to be preserved and within normal limits. At this stage, the case was reported to the Committee for the Study of Congenital Defects. First an amniocentesis was recommended to determine fetal pulmonary maturity, and later an elective caesarean section delivery (since it was a pre-hydropic fetus which would probably not endure a vaginal delivery). Elective Caesarean section W,BS performed at 38 weeks of gestation with the neonatologist, paediatric cardiologist, and paediatric cardiac surgeon standing by.

INTRAPERICARDIAL TERATOMA

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Figure 2. Removal of the turnour

Inimediate postnatal intensive assistance was thought to be required in view of the signs of partial cardiac compression of the tumour over the left side cavities, apart

from the impaired ventricular filling produced by the pericardial effusion. A 3430 g girl was delivered in a relatively good general condition, although S rnin later she presented acute respiratory distress which required endotracheal intubation. The prenatal diagnosis was confirmed by echocardiography and after 2 h of intensive treatment, medial thoracotomy was performed, and 125 cm3 of pericardial fluid was aspirated. The tumour was situated on the left and posterior to the aorta. After difficult dissection, the tumour was entirely and successfully removed (Figure 2). The pathological findings were as follows: round, capsulated tumour measuring 3 cm in diameter. The external surface was smooth and showed slight concavity corresponding to the impression of the aorta. The cut surface was multicystic, the cysts measuring 0.1-1.4 cm in diameter. Microscopically, the tumour was a mixture of mature somatic elements (glandular epithelium, pancreas, thyroid tissue, cartilage, vessels, and glial tissue) including less than 5 per cent of immature glia. The diagnosis was mature teratoma.

DISCUSSION This appears to be the first case of prenatal diagnosis ofan intrapericardial teratoma with successful treatment and survival of the newborn infant (de Geeter et al., 1983; Rasmussen el al., 1987; Cyr et af.,1988). On admission of the patient to our department, the diagnosis was thought to be a pleural effusion. When the ultrasonographic examination was carefully performed by experienced staff, the diagnosis did not jhou any difficulties. However, a more specific study was thought to be necessary to e\ aluate the haemodynamic conditions and the potential repercussions of this

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malformation on the fetus. Echocardiography was carried out by a specialist and allowed an accurate evaluation of the cardiac performance conditioned by partial compression. This greatly helped future obstetrical management (De Geeter et d., 1983). When the case was considered by the Committee for the Study of Congenital Defects, several options were ]presented. First, there was the possibility of draining the pericardial effusion to obtain cardiac decompression. This option was rejected, as it would not have been possible to maintain the drainage until the 39th week. The second option was t o continue with ultrasound monitoring and to follow the usual obstetrical management. The third one was immediate fetal delivery to enable surgery as soon as possible. Left cardiac output during fetal life was considered to be greatly maintained by the right ventricle; therefore, partial compression of the left cardiac chambers could be tolerated. However, after birth the degree of pulmonary venous obstruction and the restricted left ventricular filling pattern produced by the tumour and pericardial effusion were thought to be responsible for the impaired maintenance of systemic cardiac output. The Committee considered the fact that although the fetus was well nourished (growth parameters were above the 90th percentile), it was pre-hydropic as well, and this would have made a vaginal delivery difficult and also would have required specific preparation at birth. Therefore an elective caesarean section was decided. The choice proved to be correct, as it allowed the new-born to be delivered in optimal condition to endure the difficult operation to remove the tumour. The recovery was completely normal, and she is well, at present. REFERENCES Cyr, D., Guntherod, W., Nyberg, D., Smith, J., Nudelman, S., Marit, E.K. (1988). Prenatal diagnosis of an intrapericardial teratoma. A cause of nonimmune hydrops, J . Ultrasound Med., 7,87-90. De Geeter, B., Kretz, J.G., Nisand, I., ef al. (1983). Intrapericardial teratoma in a newborn infant. Use of fetal echocardiography, Ann. Thorac.Surg., 35,664. Rasmussen, S.L., Hwang, W.S., Harder, J., Nicholson, S., Davies, D., Nimrod, C.A. (1987). Intrapericardial teratoma. Ultrasonic and pathological features, J . Ultrasound Med., 6, 159-162.