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J Thorac Cardiovasc Surg 2007;134:1596-1597
© 2007 The American Association for Thoracic Surgery
Brief Communication |
Department of Cardiothoracic Surgery, Christian Medical College & Hospital, Vellore, Tamil Nadu, India.
Received for publication July 23, 2007; accepted for publication August 15, 2007. * Address for reprints: Alpha Mathew Kavunkal, MCh, Lecturer, Department of Cardiothoracic Surgery Unit 1, Christian Medical College & Hospital, Vellore, 632 004, Tamil Nadu, India. (Email: alphakavi{at}hotmail.com).
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Cystic lymphangioma is a developmental malformation of the lymphatic system. As they develop in the body surface, 90% of all lymphangiomas are found by 2 years of age.1
However, those developing in the mediastinum, which account for less than 1%, are mostly asymptomatic and are usually found accidentally in adulthood.2 We report here a rare case of isolated mediastinal cystic lymphangioma in a 3-year-old child.
A 3-year-old girl had had intermittent low-grade fever for 2 weeks. A chest radiogram showed a large homogeneous opacity in the left lower lung field (Figure 1). A computed tomographic scan revealed a well-defined 10 x 5-cm cystic lesion in the left paracardiac region in close contact with the heart (Figure 2). Superiorly, it extended up to the level of the superior pulmonary veins and, inferiorly, to the diaphragm with pressure effect on the left lower lobe and volume loss. The patient underwent left posterolateral thoracotomy. Intraoperatively, there was a large 10 x 6-cm cyst containing serous fluid in the anterior and middle mediastinum, which was adherent to the pericardium and diaphragm. The mediastinal cyst was excised and histopathologic examination revealed cystic lymphangioma. She had an uneventful postoperative period and is doing well 1 year after surgical excision.
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Cystic lymphangiomas, also known as hygromas, lymphatic cysts, or chylous cysts, are rare congenital abnormalities of the lymphatic vessels. These cysts are mostly present at birth, and the majority of them are detected before the age of 2 years.2,3 Although most of these cysts have been described in the pediatric population, adult patients with these cysts have also been described. Cystic lymphangiomas are most frequently located in the cervical (75%) and axillary (20%) regions.4 Isolated mediastinal cystic hygromas are very uncommon, being more common in the adult age group. In adults, these cysts are often recurrences of childhood tumors that were incompletely resected. Intrathoracic cysts are mostly located in the superior mediastinum.4 Other cystic masses that must be considered in the differential diagnosis include teratoma, thymic cysts, necrotic tumors, pericardial or bronchogenic cysts, and goiters. The origin of lymphangiomas is controversial. They may be developmental, hamartomatous, or neoplastic.
These cysts in the mediastinum are mostly asymptomatic but may cause chest pain, cough, dyspnea, vocal cord paralysis, venous compression, or even stridor. Infection and hemorrhage commonly enlarge the cyst. The most common computed tomographic image is a well-circumscribed cystic mass in the anterior or superior mediastinum. Magnetic resonance imaging has the potential advantage of conclusive demonstration of cystic components, particularly in tumors with high attenuation on computed tomography because of high protein content and better demonstration of invasion of adjacent structures. Pathologically, these cysts were earlier subdivided into 3 groups: (1) capillary lymphangiomas composed of small lymphatics, (2) cavernous lymphangiomas composed of larger lymphatics, and (3) cystic lymphangiomas made up of large macroscopic lymphatic spaces with collagen and smooth muscle. This classification has largely been replaced by the all-inclusive term "lymphangioma" because the distinction among some of these lesions was arbitrary and, in fact, many lymphangiomas have both cystic and cavernous components.5
There are a few documented cases of spontaneous regression of cystic lymphangioma, but these occurred in sites other than the mediastinum. Surgical excision is the treatment of choice. Lymphangioma is a benign tumor, and a good prognosis is anticipated if the tumor is completely resected. However, it has been reported that the resected tissue contains infiltrative lesions and that recurrences are possible if the resection is incomplete.
Our patient had a large lymphangioma involving the anterior and middle mediastinum, which is unique inasmuch as isolated lymphangioma of the mediastinum in the pediatric population is exceedingly uncommon and there are only a few such reported cases. Complete resection of the cyst is the treatment of choice in these patients.
References
This article has been cited by other articles:
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  M. Takahashi, N. Okumura, K. Kameyama, and K. Notohara Emergency surgery for macrocystic lymphatic malformation in the lung of a neonate J. Thorac. Cardiovasc. Surg., September 1, 2011; 142(3): e133 - e134. [Full Text] [PDF]
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