Ann Thorac Surg 2006;81:e11-e12
© 2006 The Society of Thoracic Surgeons
Case report
A Rare Intrapericardial Enterogenous Cyst Presenting in Infancy
Sathiakar Paul Collison, DNB*,
Muneesh Tomar, MD,
Savitri Shrivastava, DM,
Krishna Subramony Iyer, MCh
Escorts Heart Institute and Research Centre, New Delhi, India
Accepted for publication December 2, 2005.
* Address correspondence to Dr Collison, Escorts Heart Institute and Research Centre, Okhla Rd, New Delhi, 110025 India (Email: spcollison{at}rediffmail.com).
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Abstract
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We report an infant with an intrapericardial enterogenous cyst, and discuss the differential diagnosis of intrapericardial cysts presenting in infancy.
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Introduction
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Symptomatic intrapericardial cysts are uncommon in infancy. An intrapericardial cyst of enterogenous origin is extremely rare. The differential diagnosis and the embryology of such a cyst is not well known. We will present a case and discuss these issues.
A 5-month-old boy presented to us with a history of respiratory distress and nonproductive cough of 10 days duration. He had not had any fever. There was no history of respiratory infection in the past. Examination revealed a tachypneic baby with mild intercostal recessions. Auscultation of the heart and lungs was unremarkable. The liver was palpable 2.5 cm below the costal margin. Pulse oximetry revealed normal saturation. Chest roentgenogram showed an enlarged cardiac shadow with normal pulmonary vascular markings.
Transthoracic echocardiography demonstrated a structurally normal heart that was pushed leftward and inferiorly by an intrapericardial cyst measuring 5 cm x 4.3 cm with homogenous echogenicity (Fig 1A). The right atrium and the right ventricle were compressed (Fig 1A) and superior vena caval flow was obstructed (Fig 1B). In view of the symptomatic status, urgent surgery was organized without resorting to any further investigations.
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Fig 1. Transthoracic echocardiographic images of the cyst. (A) The cyst is causing compression to the right atrium (arrow) and ventricle. (B) Superior vena caval compression. (LV = left ventricle; RV = right ventricle; SVC = superior vena cava.)
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At operation through a median sternotomy, the cyst was found to be completely intrapericardial, lying in the right para-aortic space, compressing the right atrium and the right ventricle and pushing the apex of the heart leftward and inferiorly. The superior vena cava was stretched along the medial aspect of the cyst. The cyst had flimsy adhesions (some vascular in nature) to the pericardium, superior vena cava, and the right atrium. The cyst could be resected en masse after it was decompressed by the aspiration of some of the fluid content, which was grayish in color and opalescent. The postoperative period was uneventful.
Histopathologic examination of the cyst revealed a lining of ciliated columnar epithelium with some areas of squamous epithelium (Fig 2A). The cyst wall was composed of smooth muscle layers in which islands of pancreatic and duodenal glands were seen (Fig 2B). These features are consistent with a diagnosis of an enterogenous cyst.
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Fig 2. Histopathologic slides of the cyst. (A) A single layer of columnar epithelium with smooth muscle fibers in its wall (low power microscopy). (B) A high-power detail of the pancreatic type of gland found interspersed in the epithelium.
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Comment
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Primary cysts and tumors of the heart, either intracardiac or extracardiac in location, are rare at all ages with the prevalence in autopsy studies ranging from 0.0017% to 0.28% [1]. Enterogenous cysts in an intrapericardial location are much less common than intrapericardial bronchogenic cysts [2].
Both enterogenous and bronchogenic cysts originate from the primitive foregut. At approximately 21 days after fertilization, the foregut or the primitive tracheobronchial out-pouching of the foregut lies in close proximity to the developing cardiac tube. Hence, abnormal budding of the primitive tracheobronchial tree at this stage can lead to seeding of endodermal tissue in and around the developing cardiac tube [3]. Tissue from the ventral foregut would form bronchogenic cysts, whereas tissue from the dorsal foregut would form enterogenous cysts. Nobuhara and colleagues [4] have advocated naming both types of cysts as "foregut cysts" to emphasize their common embryological origin, anatomic proximity, and histologic similarities.
Both types of cysts are lined by ciliated columnar epithelium and have smooth muscle in the wall. However, bronchogenic cysts would show bronchial glands and cartilage in the cyst wall; conversely, presence of gastrointestinal tissue in the form of gastric, duodenal, or pancreatic tissue is mandatory for histologic classification as an enterogenous cyst.
Pancreatic tissue in the mediastinum is uncommon [5], with few reported cases [6, 7]. In most cases the pancreatic tissue is part of enteric duplication cysts, intralobar pulmonary sequestrations, or teratomas. The presence of this tissue in these unusual locations is either related to heteroplasia, pancreatic cell migration [7], or pancreatic tissue heterotopia over a digestive structure [5].
The differential diagnoses of intrapericardial cysts in infancy include rhabdomyoma (58%), teratoma (18%), fibroma (12%), bronchogenic cyst (2.1%), and rarely hemangioma, lipoma, and thymic cyst (10%) [7]. Teratomas are usually lobulated cysts with both solid and cystic regions. Furthermore, teratomas consist of tissue derived from all three germinal layers, whereas a bronchogenic cyst consists of tissue derived from endoderm and mesoderm only. Rhabdomyomas can be derived from ventricular muscle toward the epicardial surface and grow into the pericardial cavity and undergo cystic degeneration. Fibromas and lipomas present as homogenous masses on the surface of the heart but are small and solid. Preoperative differentiation can be challenging, although recent reports emphasize the role of computed tomography and magnetic resonance imaging [8].
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Acknowledgments
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We thank Sudheer Jain, MD, New Delhi, for reviewing the histopathology of the cyst.
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References
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- McAllister Jr HA, Fenoglio JJ. Fascicle 15, 2nd series. tumors of the cardiovascular system. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1978. pp. 1-3.
- Burke A, Virmani R. Fascicle 16, 3rd series. tumor of the heart and great vessels. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1996. pp. 111-112.
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- Nobuhara KK, Gorski YC, La Quaglia MP, et al. Bronchogenic cysts and esophageal duplications. common origins and treatment. J Pediatr Surg 1997;32:1408-1413.[Medline]
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- Lugo-Olivieri CH, Schwartzman GJ, Beall BP, Lima JAC, Fishman EK. Intrapericardial bronchogenic cyst. assessment with magnetic resonance imaging and transesophageal echocardiography. Clin Imaging 1999;23(2):81-85.[Medline]
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Abstract
Introduction
