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Abstract
Pulmonary lymphangiectasia is a rare congenital malformation of lymphatic vessels. We report the case of a 5-year-old boy with recurrent pericardial effusion which was diagnosed to be due to pericardial and pulmonary lymphangiectasia.
Keywords: Congenital, lymphangiectasia, pericardial effusion
How to cite this article: Introduction 
Pericardial effusion in children is mostly caused by pericarditis of viral and bacterial origin, metabolic disorders or malignancies. Recurrent pericardial effusion poses various diagnostic and management challenges. We demonstrate an instance where recurrent pericardial effusion was related to pulmonary and pericardial lymphangiectasia.
Case Report A 5-year-old boy presented with a history of cough and exertional dyspnea for 10 days, not associated with a fever, chest pain, or coryza. He had a history of recurrent episodes of lower respiratory tract infections since childhood. There was no history of contact with tuberculosis or similar history among family members.
He was evaluated elsewhere and found to have pericardial effusion with strands on echocardiography, for which multiple sessions of pericardiocentesis were done. Pericardial fluid analysis revealed lymphocytic predominance (2950 cells per cumm) with elevated adenosine deaminase (ADA) (32 IU/L) and protein (6.8 g/dl) with normal glucose levels (114 mg/dl) and lactate dehydrogenase (LDH) (252 IU/L), for which tubercular etiology was suspected, and he was started on antitubercular therapy.
On examination, he had no features of cardiac failure, with slight muffling of heart sounds. Systemic examination was unremarkable without any dysmorphism. Chest X-ray showed cardiomegaly, with prominent bronchovascular markings [Figure 1]. Echocardiogram showed large circumferential pericardial effusion (28 mm posterior to left ventricle [LV], 4.5 mm at apex, 11 mm lateral to LV, and 14 mm lateral to the right ventricle), with good biventricular function and no evidence of tamponade. Pericardial fluid analysis revealed lymphocytic predominance (9700 cells) with elevated ADA (18.6 IU/L) and protein (4.3 g/dl) with normal glucose levels (117 mg/dl) and LDH (142 IU/L) [serum protien 6.7 g/dl]. Cytology for malignant cells was negative. Gram stain and Ziehl‒Neelsen stain were negative, and the culture of the fluid was sterile. Mantoux was negative, and hemogram showed normal leukocytic count (10,400/cumm N56 L41 E3), erythrocyte sedimentation rate (10 mm); C-reactive protein was negative. Differentials considered were the human immunodeficiency virus infection, indolent infectious pericarditis, systemic lupus erythematosus, hypothyroidism, and occult malignancy. Serology for human immunodeficiency virus and antinuclear antibody was also negative. Thyroid function test was normal.
Figure 1: Chest X-ray showing cardiomegaly with prominent bronchovascular markingsOn computed tomography, pericardial thickness was normal (3 mm) without any evidence of calcification and prominent bronchovascular markings [Figure 2], with no features of malignancy. Magnetic resonance imaging (MRI) of the thorax revealed normal pericardial thickness (3 mm) with moderate pericardial effusion, and T2 hyperintensities along with thickening along peribronchovascular regions, interlobular septa, and lymphatics in the thoracic inlet and supraclavicular regions, with pericardial effusion, overall suggestive of pericardial pulmonary lymphangiectasia [Figure 3]a and [Figure 3]b. Syndromic association was evaluated, but he did not have any evidence of any other abnormality or lymphangiectasia anywhere else. Magnetic resonance (MR) lymphangiography was attempted but unsuccessful. The child was put on medium-chain triglyceride diet with coconut oil but was discontinued by the family in a week. The matter was discussed at the Joint Cardiac Conference of the institute. He was planned for pericardiectomy in view of recurrent pericardial effusion.
Figure 2: Axial section of contrast-enhanced computed tomography of the chest showing pericardial effusion with prominent bronchovascular markings
Figure 3: (a and b) T2-weighted image axial section showing pericardial effusion and hyperintensities along with thickening along peribronchovascular regions, interlobular septa, and lymphatics (blue arrow), suggestive of lymphangiectasiaIntraoperatively, the pericardium was found to be thickened with several pockets of effusion and lymphangiectasia. Intraoperative fluid was found to be straw colored with lymphocytic predominance, elevated triglycerides (200 mg/dl), and slightly elevated cholesterol (86 mg/dl). Post pericardiectomy, he had an uneventful recovery with no residual pericardial effusion on echocardiogram. He was followed by for 6 months with no recurrence of effusion.
Discussion Lymphangiectasia is a rare disorder involving dilatation of lymphatic channels, either due to maldevelopment or secondary to obstruction of lymphatic flow. The most common site of involvement is the intestines, which manifests as malabsorption syndrome, followed by the lungs.[1]
Pulmonary involvement is characterized by the presence of dilated subpleural, interlobar, perivascular, and peribronchial lymphatics on imaging. Clinical manifestations may range from hydrops fetalis, neonatal respiratory distress to recurrent lower respiratory tract symptoms in children.[2] Computed tomography of the chest usually shows thickening of the peribronchovascular and septal interstitium, whereas MRI shows T2 hyperintensities along the pulmonary interstitium. Lymphoscintigraphy and MR lymphangiogram may be further beneficial in delineating the disease.[3] Although there is no definitive cure for this disorder, creating of a pericardial window may be of palliative benefit in preventing reaccumulation of lymph in the pericardium.
Isolated pericardial lymphangiectasia is extremely rare; usually, it is associated with the involvement of the intestinal tract, pericardium, and limbs as seen in Hennekam lymphangiectasia‒lymphedema syndrome, which is an autosomal recessive condition.[4] To our knowledge, there is no reported case of isolated involvement of only lungs and pericardium.
Keeping a differential diagnosis of pericardial lymphangiectasia is of utmost importance in a case as reported above. Earlier identification prevents unnecessary investigations and therapy, as was seen in the index case.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
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Conflicts of interest
There are no conflicts of interest.
References 
Correspondence Address:
Dr. Arun Gopalakrishnan
Associate Professor, Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/apc.apc_212_21
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