Cystic lymphangioma of pericardium presenting as isolated chylopericardium – A case report

Vinayakumar, Desabandhu; Arunkumar, G.; Sajeev, C.G.; Rajesh, Gopalan Nair; Muneer, Kader; Vellani, Haridasan; Babu, K Govind; Krishnan, M.N.

doi:10.1016/j.ihj.2013.12.020

Cited by 7 publications

(4 citation statements)

References 4 publications

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“…A European multicenter study of pediatric cardiac tumors reported no cases of lymphangioma [2], and a recent study reported only one case in their 26-year experience (2.1%) [1]. Cardiac lymphangiomas are often detected incidentally as asymptomatic masses, as was the case with our patient [3], and they represent a proliferation of sequestered endothelial-lined lymphatic channels that do not communicate with the normal lymphatic system [4]. Most lymphangiomas develop in areas of primitive lymph sacs, but they can also be present in the mediastinum.…”

Section: Commentsupporting

confidence: 55%

“…Although a lymphangioma is a benign lesion, it can lead to complications because of compression of adjacent structures. It can also cause recurrent episodes of cough, respiratory distress [5], cardiac tamponade [4], arrhythmias, or chylopericardium [3]. The tumor may arise from the atria, as in our case, or rarely from the ventricular myocardium [6].…”

Section: Commentmentioning

confidence: 89%

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Cardiac Lymphangioma Encasing Right Coronary Artery in an Infant

Bansal

Haidar-El-Atrache

Walters

et al. 2017

The Annals of Thoracic Surgery

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Cardiac lymphangioma is a rare primary benign tumor of the heart. We report a 3-year-old with cystic lymphangioma encasing the right coronary artery. Cardiac magnetic resonance imaging confirmed a intra-pericardial heterogeneous mass measuring 2.6 × 2.4 × 3.9 cm and situated right anterolateral to the ascending aorta and extending into the right atrioventricular groove. Furthermore, the right coronary artery traversed through the center of the mass. Surgical resection, on cardiopulmonary bypass, consisted of excision by skeletonizing the right coronary artery along the length of the mass. The pathology report was consistent with a lymphatic malformation. The postoperative course was uneventful without recurrence at follow-up.

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Section: Commentsupporting

confidence: 55%

Section: Commentmentioning

confidence: 89%

Cardiac Lymphangioma Encasing Right Coronary Artery in an Infant

Bansal

Haidar-El-Atrache

Walters

et al. 2017

The Annals of Thoracic Surgery

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“…Since then, till November 2013, a total of 128 cases have been described in the literature [ 4 ]. Idiopathic chylopericardium refers to an abnormal lymphatic system and mediastinal lymphangiectasia causing chylopericardial effusions [ 5 ]. These abnormal lymphatic channels which may be a part of lymphangiomas or other lymphatic tumors, permit retrograde flow of lymph through them to the rich pericardial plexus causing primary chylous pericardial effusions [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Chylous Pericardial Effusion Due to Lymphatic Malformation of Mediastinum

Mishra,

Das,

Goswami

et al. 2023

Cureus

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Chylopericardium can be due to a variety of secondary causes like trauma, radiation, tumors, following cardiac surgery, etc., or may be idiopathic due to abnormal lymphatic system and mediastinal lymphangiectasia, which is a rare entity. Here, we present a case of a 34-year-old previously healthy male presenting with idiopathic chylopericardium. 2D echocardiography revealed massive pericardial effusion without features of cardiac tamponade. Following pericardiocentesis, a CT scan of the thorax and MR lymphangiogram were done to arrive at a diagnosis of idiopathic chylopericardium. In addition to medical management, surgical treatment included partial pericardiectomy and sclerotherapy of the mediastinal lymphatic sac. The patient had an uneventful post-operative period.

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“…Late presentation of pericardial cystic lymphangiomas at 16 months of age with sudden-onset respiratory distress is the most unusual feature of this case report. Although late presentations of pericardial cystic lymphangiomas have been reported in literature, the common presentations include chest pain, cough, palpitations, gradual-onset dyspnea, and chylopericardium [8][9][10]. A recent review of 35 cases of cardiac or pericardiac cystic lymphangioma revealed that eight patients were asymptomatic and were diagnosed incidentally.…”

Section: Discussionmentioning

confidence: 99%

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report

et al. 2022

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Background Lymphangiomas are rare benign malformations of the lymphatics that occur due to blockage of the lymphatic system during fetal development. They commonly occur in the neck and axilla, while involvement of the pericardium is rare. We report herein the case of a 16-month-old Sri Lankan child with a large pericardial cystic lymphangioma presenting with sudden-onset shortness of breath. Case presentation A 16-month-old Sri Lankan boy presented with sudden-onset dyspnea for 1-day duration following a febrile illness that lasted 2 days. On examination, he was afebrile and had subcostal, intercostal, and suprasternal recessions, with a respiratory rate of 50 breaths per minute. He had a loud expiratory grunt. The chest expansion was reduced on the right side, which was dull to percussion. Auscultation revealed a marked reduction of air entry over the right lower and mid zones. Chest X-ray showed a well-demarcated opacity involving the lower and mid zones of the right hemithorax associated with a tracheal shift to the opposite side. Ultrasound scan of the chest revealed fluid-filled right hemithorax suggesting a septate pleural effusion. A contrast-enhanced computed tomography scan of the thorax showed a large multiloculated extrapulmonary cystic lesion involving the right hemithorax with a mediastinal shift towards the left side associated with displacement of the right-side mediastinal structures. He underwent mini-thoracotomy and surgical excision of the cyst. A large cyst originating from the pericardium was observed and excised during surgery. Histological examination revealed a lesion composed of cysts devoid of a lining epithelium but separated by connective tissue, mature adipose tissue, and lymphoid aggregates. The child showed complete recovery postoperatively with full expansion of the ipsilateral lung. Conclusion We report the case of a patient with cystic lymphangioma who was perfectly well and asymptomatic until 16 months of age. This case report presents the very rare occurrence of a large cystic lymphangioma originating from the pericardium. It highlights the importance of considering rare possibilities and performing prompt imaging in situations of diagnostic uncertainty to arrive at an accurate diagnosis that can be lifesaving.

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Cystic lymphangioma of pericardium presenting as isolated chylopericardium – A case report

Cited by 7 publications

References 4 publications

Cardiac Lymphangioma Encasing Right Coronary Artery in an Infant

Cardiac Lymphangioma Encasing Right Coronary Artery in an Infant

Idiopathic Chylous Pericardial Effusion Due to Lymphatic Malformation of Mediastinum

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report

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