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Ann Thorac Surg 1998;66:559-560
© 1998 The Society of Thoracic Surgeons

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Case Reports

Fetal cardiac tamponade due to an intrapericardial teratoma

^a Department of Cardiac Surgery, University Hospitals Gasthuisberg, Leuven, Belgium
^b Department of Thoracic Surgery, University Hospitals Gasthuisberg, Leuven, Belgium
^c Department of Neonatology, University Hospitals Gasthuisberg, Leuven, Belgium
^d Department of Gynecology and Obstetrics, University Hospitals Gasthuisberg, Leuven, Belgium

Accepted for publication February 27, 1998.

Address reprint requests to Prof Daenen, Department of Cardiac Surgery, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
e-mail: (willem.daenen{at}uz.kuleuven.ac.be)

	Abstract

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A case of an intrapericardial tumor diagnosed in utero at 26 weeks of gestation is presented. The prenatal echocardiographic follow-up of an incipient hydrops fetalis determined the management and the emergency surgical treatment. Histologically, the tumor appeared to be a benign teratoma, grade I. In the postoperative period an unexpected mediastinal tumor was found and removed later. This tumor also appeared to be a benign teratoma, grade 0. Both teratomas were independent and therefore primary.

	Introduction

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Extrapericardial mediastinal teratomas are not uncommon, but intrapericardial teratomas are rare [1–3]. They may cause nonimmunologic hydrops fetalis and cardiac tamponade [1, 2, 4]. However, as demonstrated in the following case, structured planning guided by ultrasound follow-up can lead to a satisfying outcome.

A 26-year-old primigravida, without a previous medical history, presented with polyhydramnios at 26 weeks of gestation. The ultrasound examination showed an intrapericardial mass (3.8 x 2.9 x 3.1 cm), pericardial effusion, and ascites. Hydrops fetalis developed with obstruction of venous return and cardiac tamponade. To improve the fetal survival chances, regular follow-up was planned. Initially the hydrops fetalis remained stable. Lung maturity was stimulated with thyrotropin-releasing hormone and betamethasone (Celestone, Schering Corp, Kenilworth, NJ). At 30 weeks of gestation the intrapericardial mass appeared cystic on ultrasound, suggesting a teratoma [4]. The pericardial effusion had increased.

At 32 weeks of gestation there was a considerable increase in hydrops fetalis. Cardiac tamponade developed and fetal cardiac output decreased dramatically. Because of sudden fetal distress a cesarean section was performed.

The 1,930-g male newborn had immediate cardiorespiratory distress. Peripheral circulation was poor and cyanosis was obvious. No murmurs were heard on auscultation. The Apgar score was 5 after 1 minute. Endotracheal intubation was performed immediately. The chest radiograph showed an extremely widened cardiac shadow, and on echocardiography a large intrapericardial cystic tumor was confirmed. A pericardiocentesis yielded 15 mL of serous fluid.

Subsequently, a severe respiratory distress syndrome developed, which was treated with high-frequency oscillation and surfactant therapy. Despite stabilization of the respiratory distress syndrome, the hemodynamic status deteriorated rapidly and we decided to operate 14 hours after the infant’s birth.

After sternotomy and pericardiotomy, a large intrapericardial cystic tumor was found, compressing the right atrium, right ventricle, and aorta (Fig 1). The tumor was well demarcated and easily removed by dividing a single nutrient artery from the ascending aorta. The hemodynamic status improved immediately. The postoperative chest radiograph was apparently normal, except for a widened upper mediastinum. Histologic examination of the resected specimen yielded a mature benign cystic teratoma, grade I.

View larger version (105K): [in this window] [in a new window]   Fig 1. Operative picture after sternotomy and opening of the pericardium showing a large cystic tumor compressing the right heart and aorta.

At 10 weeks the patient underwent a left thoracotomy with opening of the mediastinal pleura. A multilobulated cystic mass was found with a nutrient artery arising from the left internal mammary artery. The tumor was excised and histologic examination showed a mature teratoma, grade 0, without signs of malignancy. Because there was no connection between the intrapericardial and mediastinal tumor, it was obvious that we were dealing with two independent primary teratomas.

	Comment

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Cardiac neoplasms are known to originate in any part of the heart, including the pericardium, but are extremely rare [3]. A teratoma usually appears as a cystic mass outside the cardiac cavities and is sometimes calcified [2]. These tumors are voluminous and sometimes larger than the heart [5]. In contrast to our patient, they mostly occur in female patients [3]. The rapid growth and differentiation is somewhere between 20 and 40 weeks of gestation [1, 2, 6]. As in our case, the majority are located anteriorly to the right side, where they may compress the heart, great vessels, and in some cases the trachea [5, 6]. In general, the tumor is attached to the heart or great vessels by a fibrous pedicle [6] and, like in our patient, resection is easy. Multiple intrathoracic localizations are rare; however, our patient demonstrates that a second localization should always be ruled out.

Clinical signs usually occur in the newborn period or in early infancy [7]. However, if detected in utero, nonimmunologic hydrops fetalis, characterized by fetal anasarca and pleural or pericardial effusion, is the main clinical feature [2, 4, 6, 7]. In the newborn with pericardial teratoma, ascites, cardiomegaly, and cardiopulmonary distress with dyspnea, hypoxia and acidosis are common [1, 3]. The clinical findings result from an obstructed systemic venous return by the tumor and the pericardial effusion. Consequently, the cardiac output is reduced. Therefore, pericardiocentesis may be lifesaving [4].

In the neonate, diagnosis is based on clinical, radiographic, and echocardiographic findings [3]. Fetal echocardiography may already have shown the cystic intrapericardial mass, pericardial effusion, and ascites. Chest radiography may show a widened mediastinum. Heart catheterization should be avoided, especially in the symptomatic patient, because of the high risk involved [5]. The combination of a large cardiothymic shadow and a pericardial effusion is characteristic of an intrapericardial teratoma and should cause suspicion of this diagnosis [5]. Echocardiography again will confirm the diagnosis.

Although these tumors are histologically benign, surgical excision is mandatory because of the hemodynamic repercussion. Preoperative hemodynamic and respiratory stabilization should be achieved as coexisting pulmonary disease, especially in the premature infant, might considerably increase the operative risk. The intrinsic prognosis is generally good because this tumor is well delimited, usually without associated cardiac malformations [2]. With early intrauterine fetal hydrops, the prognosis is certainly worse, and optimal management presents a challenge.

In conclusion, this article presents a preterm infant with an intrapericardial teratoma associated with an unexpected and very rare second mediastinal teratoma. Our study is furthermore of clinical interest because prenatal diagnosis improved obstetric management, avoiding hasty decisions and optimizing accurate neonatal care. In this case, the decision for surgical excision was taken after hemodynamic and respiratory stabilization. The life-threatening cardiac tamponade was released in time and the final recovery was complete.

	References

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1. Todros T., Gaglioti P., Presbitero P. Management of a fetus with intrapericardial teratoma diagnosed in utero. J Ultrasound Med 1991;10:287-290.[Medline]
2. Perez-Aytes A., Sanchis N., Barbal A., et al. Nonimmunological hydrops fetalis and intrapericardial teratoma: case report and review. Prenatal Diagn 1995;15:859-863.[Medline]
3. De Geeter B., Kretz J., Nisand I., Eisenmann B., Kieny M., Kieny R. Intrapericardial teratoma in a newborn infant: use of fetal echocardiography. Ann Thorac Surg 1983;35:664-666.[Abstract/Free Full Text]
4. Rheuban K., McDaniel N., Feldman P., Mayes D., Rodgers B. Intrapericardial teratoma causing nonimmune hydrops fetalis and pericardial tamponade: a case report. Pediatr Cardiol 1991;12:54-56.[Medline]
5. Sumner T., Crowe J., Klein A., McKone R., Weaver R. Intrapericardial teratoma in infancy. Pediatr Radiol 1980;10:51-53.[Medline]
6. Guirgis N., Azancot A., Blot P. Unusual evolution of a pericardial mass diagnosed by fetal echocardiography. Pediatr Cardiol 1991;12:57-59.[Medline]
7. Zerella J., Halpe D. Intrapericardial teratoma—neonatal cardiorespiratory distress amenable to surgery. J Pediatr Surg 1980;15:961-963.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Filip Casselman Willem J. Daenen Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Tollens, T. Articles by Daenen, W. J. Search for Related Content PubMed PubMed Citation Articles by Tollens, T. Articles by Daenen, W. J.