European Journal of Cardiovascular Medicine

A 6-year-old male child, 2nd twin of 3^rddegree consanguineous marriage presented to the emergency department and was admitted in Paediatric Intensive Care Unit with complaints of progressive difficulty in breathing since 3 months, cough and rhinorrhoea since 2 days and chest pain since 2 days. His birth history, immunization and developmental history were not significant for the present complaints, Anthropometry was within normal range.

On examination, he was conscious, oriented, afebrile, with heart rate, saturation within normal limits. He was tachypnoeic with respiratory distress. Pigeon chest deformity was seen with subcostal, intercostal and substernal recessions.  On auscultation, his heart sounds were muffled, there was no murmur, few crepitations were present bilaterally. Routine investigations revealed severe anaemia with thrombocytopenia.

Computed Tomography was suggestive of massive pericardial effusion. 2D ECHO was done which was also suggestive of the same (3cm thickness pericardial effusion in diastolic phase all around the heart) with normal Left Ventricular Ejection Fraction. Considering this, a pericardial drain was inserted which drained 300ml of haemorrhagic fluid and thereafter, 100 to 200ml haemorrhagic fluid daily. The pericardial fluid was sent for routine investigations which revealed numerous RBCs, no malignant cells and was negative for AFB (acid fast bacilli) and CBNAAT (Cartridge Based Nucleic Acid Amplification Test). Haematologistwas consulted in view of differential diagnosis of lymphoproliferative disease but bone marrow aspiration and biopsy were normal. Splenic biopsy was done in view of CT findings suggestive of splenic infiltrates and multiple enlarged lymph nodes which revealed reactive organizing cells and was negative for CBNAAT and malignant cells. Bronchoalveolar lavage was also normal.

Interventional radiologist, Cardiologist and Cardiovascular Thoracic Surgery opinion was taken in view of gross daily haemorrhagic output from the pericardial drain. After multidisciplinary discussion and careful consideration, a novel approach of subtotal pericardiectomy under leadership of the CVTS surgeon was planned in this case. Intraoperatively, massive haemorrhagic pericardial effusion, approximately 1000ml was drained. Petechial haemorrhagic type clusters were seen over the inner surface of parietal pericardium and on the adventitia over the main Pulmonary vein bifurcation. Cardiac contractility and size appeared normal. Subtotal pericardiectomy was done extending from innominate vein superiorly to diaphragm inferiorly and between both the phrenic nerves on either side.

Active bleeders (spurters) from the excised pericardial edges were controlled with running sutures, vascular clips and the use of electrosurgical cautery. Intercostal drains were placed and chest was closed. Patient tolerated the procedure well.

Histopathology report of the pericardial biopsy revealed benign vascular lesion suggestive of lymphangiohemangiomatosis. In immediate post operative period, patient was kept in the CVTS ICU after which, he was transferred to the paediatric ICU for further management. PET-CT was done which pointed towards a differential diagnosis of lymphangitic malformation and IgG4 disease. The patient was started on mTOR inhibitor (Sirolimus) considering the diagnosis of Lymphangiohemangiomatosis.

Postoperatively, the patient improved gradually. Intercostal drains were removed and patient was shifted out of the ICU on O2 support with nasal prongs. However, he continued to have respiratory distress and ultrasonography chest revealed consolidation and bilateral haemorrhagic pleural effusion. Repeat ultrasonographys revealed increased pleural effusion and increasing respiratory distress. The patient was put on mechanical ventilation and ultrasonography guided pleural tapping was done thrice. After starting Sirolimus, gradually, condition of the child improved, pleural effusion reduced, and he was shifted to the ward on High Flow Nasal Cannula. He was discharged on O2 with nasal prongs on Sirolimus with regular weekly follow-up.

Lymphangiohemangiomas are congenital vascular anomalies which can occur in any part of the body, except the brain [8]. They result from aberrant formation with lymphatic and blood vessel components.

Lymphangiohemangioma of the pericardium is an extremely rare disease with very limited reports in medical literature. Despite their benign nature, Lymphangiohemangiomas are known to have aggressive local spread, involving surrounding tissues and organs [4].

Benign lymphangiohemangioma can present with symptoms like dysrhythmia, chest pain, breathlessness, cough or dysphagia. Severity of symptoms depends upon the extent and location of the tumour. It can lead to pericardial effusion and massive cardiac tamponade which can be life threatening.[3]

Diagnosis depends on early suspicion in a case of recurrent haemorrhagic pericardial effusion supplemented by Transthoracic Echocardiography, Computed Tomography (CT scan), Magnetic Resonance.

Imaging (MRI) or Cardiac Catheterization. Definitive diagnosis is possible only by biopsy and histopathology.

Surgical excision is the treatment of choice. Prognosis in surgically treated symptomatic patients is excellent [5][7]. Treatment with steroids or radiotherapy have also resulted in tumour regression [6].

To our knowledge, this is the only reported case of diffuse pericardial lymphangiohematomatosis. In this particular case, subtotal pericardiectomy resulted in symptomatic relief by relieving the cardiac tamponade and resultant cardiac compression. This approach was preferred in this case over the other option of pericardial window creation taking into account the need for complete drainage of the massive pericardial tamponade, removal of adequate pericardial tissue which was acting as the source of pericardial effusion and acquiring adequate and representative tissue for histopathological diagnosis of the disease.

1. Rodriguez Ziccardi M, Tariq MA, Limaiem F, et al. Cardiac Cancer. [Updated2023Jan1]. In:StatPearls [Internet]. TreasureIsland(FL):Stat Pearls Publis hing;2023Jan. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537144/
2. Wunan Zhou, MD;Monvadi Barbara Srichai-Parsia, MD, FACC.July13,2016. CMR and pericardial masses. In American College of Cardiology
3. Jacob D, Pratap T, Kumar A, R R, K VA. Benign Pericardial Hemangioma-A RareCause of Cardiac Tamponade. Indian J Radiol Imaging. 2021 Nov 13;31(3):754-757.doi: 10.1055/s-0041 1736405. PMID:34790331; PMCID: PMC8590538.
4. V. Martínez-Bucio, R. De la Puente-Murgía, A. Gallardo-Meza, S.G. Zapata-Martínez, J.M. Zertuche-Coindreau, J.C. López Valdés, Pulmonary haemangiolymphangioma as cause of hemodynamic decompensation, Revista Médica del Hospital General de México, Volume 79, Issue4,2016
5. Gupta N. Intrapericardial hemangioma: a case report. J Clin Diagn Res.2013Jan;7 (1):169-70. doi: 10.7860/JCDR/2012/4714.2699. Epub 2013 Jan 1. PMID:23449754; PMCID:PMC3576780.
6. Wu G, Jones J, Sequeira IB, Pepelassis D. Congenital pericardial hemangioma responding to high-dose corticosteroid therapy. Can J Cardiol.2009Apr;25(4):e139-40. doi: 10.1016/s0828-282x(09)70081-9. PMID: 19340361;PMCID: PMC2706776.
7. Manickam S, Sasikumar P, Kishore BN, Joy S. Hemangiolymphangioma of buccal mucosa: A rare case report. J Oral Maxillofac Pathol. 2017 May-Aug;21(2):282 285.doi:10.4103/ jomfp.JOMFP_28_17. PMID:28932041;PMCID: PMC5596682.
8. Kothari SS, Sharma S, Bhatt K, Ray R, Bakhshi S, Chowdhury U. Recurrent hemorrhagic pericardial effusion in a child due to diffuse lymphangio hemangiomatosis: a case report. J  MedCaseRep.2010Feb22;4:62.doi: 10.1186/1752-1947-4-62.PMID:20170552;