Drowning in the Effusion: Cardiac Tamponade as Presenting Feature of Lymphoma in a Young Boy

Anil Kumar Singhi; AP Bharat; Roy Varghese; Kothandam Sivakumar

doi:10.4103/jiae.jiae_14_21

INTERESTING CASE REPORT

Year : 2022 | Volume : 6 | Issue : 1 | Page : 63-65

Drowning in the Effusion: Cardiac Tamponade as Presenting Feature of Lymphoma in a Young Boy

Anil Kumar Singhi¹, AP Bharat², Roy Varghese³, Kothandam Sivakumar⁴
¹ Department of Pediatric Cardiology, Medica Super Specialty Hospital, Kolkata, West Bengal, India
² Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India
³ Department of Cardiovascular Surgery, The Madras Medical Mission, Chennai, Tamil Nadu, India
⁴ Department of Pediatric Cardiology, The Madras Medical Mission, Chennai, Tamil Nadu, India

Date of Submission	14-May-2021
Date of Acceptance	13-Jun-2021
Date of Web Publication	30-Sep-2021

Correspondence Address:
Dr. Anil Kumar Singhi
Department of Pediatric Cardiology, Medica Super Specialty Hospital, Mukundapur, Kolkata - 700 099, West Bengal
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_14_21

Abstract

Cardiac tamponade in children has multiple etiological factors such as infection, post-inflammatory, malignancy, or blunt trauma. Detailed evaluation of the accompanying clinical features along with laboratory investigations and imaging help in appropriate diagnosis and management. We describe a case where cardiac tamponade manifested as the first presentation of childhood lymphoma but was mistaken as post-traumatic hemopericardium and tamponade.

Keywords: Blunt trauma, cardiac tamponade, lymphoma, pediatric

How to cite this article:
Singhi AK, Bharat A P, Varghese R, Sivakumar K. Drowning in the Effusion: Cardiac Tamponade as Presenting Feature of Lymphoma in a Young Boy. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:63-5

How to cite this URL:
Singhi AK, Bharat A P, Varghese R, Sivakumar K. Drowning in the Effusion: Cardiac Tamponade as Presenting Feature of Lymphoma in a Young Boy. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 Jun 10];6:63-5. Available from: https://jiaecho.org/text.asp?2022/6/1/63/327244

Introduction

Pericardial effusion and tamponade in the pediatric population can have various causes. Usually, the symptom of the etiological factor accompanies, which is helpful in the diagnosis. Rarely, cardiac tamponade can be the initial presentation of some disease in an unsuspecting asymptomatic child. We present a rare case of apparent blunt trauma-linked cardiac tamponade in a young boy, eventually found to have hematological malignancy.

Case Report

A 9-year-old, reportedly asymptomatic child weighing 24 kg had a history of fall from bicycle resulting in chest trauma. He was taken to the emergency department of the nearby medical college with progressive chest pain and fast breathing. Parents had not noted any significant symptoms of fever or weight loss before the accident. There was no past history of any major illness requiring hospital admission. He was evaluated and found to have large pericardial effusion. There was suspicion of perforation of the lateral right ventricle (RV) wall near the apex. This was presumed to be due to the sustained trauma to the chest. He was referred to our tertiary care pediatric cardiac center for further management. On arrival within a few hours of the initial presentation, the child was anxious, afebrile, irritable, tachypneic with oxygen saturation of 98%. He had tachycardia with heart rate of 150/min, palpable upper, and lower limb pulses. The periphery was warm. The systolic blood pressure was 90 mmHg (5^th percentile). Mean jugular venous pulse was elevated. Cardiac evaluation revealed muffled heart sounds. There was no additional heart sound or any significant murmur. The liver was palpable 2 cm below the right costal margin. The chest was clear with vesicular breath sounds. The referral chest X-ray (CXR) showed a cardiothoracic ratio of 0.65, wide mediastinal shadow with normal pulmonary blood flow. The CXR was not suggestive of configuration such as “flask” or the “water bottle.” The electrocardiogram suggested sinus tachycardia and right axis deviation. Emergency echocardiographic evaluation done with Philips Epic 7C echocardiography machine (Philips, Best, Netherlands) revealed large circumferential pericardial effusion (20 mm). There was evidence of right atrial and RV wall collapse. The right atrial collapse was seen at the onset of R wave in the systole and the RV collapse seen in the diastole [Video 1]. The tricuspid valve inflow pulse Doppler variation with respiration was more than 50% [Figure 1]a, [Figure 1]b, [Figure 1]c and [Video 1]. There was no evidence of rent across the RV wall in any of the echocardiographic views. The parasternal and subcostal short axis views showed to and fro color flow Doppler in the pericardial space [Figure 2]a, [Figure 2]b and [Video 2]. Dense echogenic mass was seen around RV and great arteries which was considered as possible trauma-induced clot [Figure 2]c and [Video 2]. No evidence of dissection of aorta or any evidence of congenital heart disease was seen. The cardiothoracic surgeon was consulted. The possibility of a computed tomographic scan in the late hours of presentation was discussed but was not done in view of the sick status of the child. The child was moved from the echocardiogrphy room to cardiac operation theatre for emergency sternotomy pending basic laboratory evaluation. The surgical team planned to perform the operation on cardiopulmonary bypass in view of the possibility of a cardiac rupture. The groin vessels on the right side were used for the cannulation. After the sternotomy, there was surprise for the team. The pericardium was found grossly thickened and there was a hard mass occupying the middle mediastinum overlying the heart. The macroscopic appearance of the mass was suggestive of a tumor. There was also pericardial fluid, which was straw (amber) colored. It was drained and sent for examination. The tumor could be incised to reach the anterior surface of the heart to which the tumor was adherent. The anterior surface of the heart was freed to relieve the compression [Figure 3]a. The heart itself was encased within the tumor mass and there was no definite plane around it. The posterior and inferior surfaces of the heart were not dissected out as it was not considered necessary in view of a provisional diagnosis of lymphoma, which responds well to radiotherapy and chemotherapy. Tissue biopsy was taken for histopathological evaluation. No evidence of any rent in the RV or any other cardiac injury was found. There was profuse bleeding from the raw surface of the incised tumor. Amber-colored fluid in the setting of a lymphoma was considered to be an exudate due to blockage of the lymphatic channels. The cause of the tamponade most likely would have been the presence of the tumor mass itself, causing compression of the cardiac chambers as seen by the raised venous pressure. The child had a stormy postoperative period marked by continued significant bleeding requiring re-exploration and factor VII replacement. His laboratory results subsequently showed anemia, leukocytosis (Total count 115,500 cells/cu mm) with predominant 74% blast cells of immature cell lymphoid lineage, and metamyelocyte 2%. The pericardial fluid was straw yellow, with predominant polymorphs but no organism was found in culture for up to 7 days. Histopathology evaluation of the tissue showed evidence of malignant small round cell tumor with features suggestive of diffuse high-grade non-Hodgkin's lymphoma, probably lymphoblastic lymphoma [Figure 3]b and [Figure 3]c. The patient was transferred to pediatric oncology center of another hospital on the 7^th day after operation in a hemodynamically stable state. His echocardiogram before transfer showed no evidence of pericardial effusion with good biventricular systolic function. He underwent further evaluation and management for the lymphoid malignancy at the other hospital. The follow-up also happened in the oncology unit of that hospital. Reportedly, he had complete remission and was doing well in the short-term telephonic follow-up.

Figure 1: Echocardiogram in (a) apical four-chamber and (b) modified short-axis view showing large circumferential pericardial effusion (˜20 mm, white arrow). Collapse of right atrial wall (a-yellow arrow) and right ventricle (red arrow-b) are also seen. (c) Pulsed-wave Doppler showing more than 50% respiratory variation in tricuspid valve inflow velocities

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Figure 2: Echocardiogram in (a) subcostal modified view and (b) short-axis view showing circumferential pericardial effusion. Evidence of colour flow in the pericardial space is seen (white arrow). (c) Parasternal short- axis view showing dense echogenic mass around the right ventricle and great arteries which was considered to be possible trauma-induced clot (yellow arrow)

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Figure 3: (a) Post sternotomy a large middle mediastinal tumour was seen (yellow arrow). Histopathology examination in (b) hematoxylin–eosin stain and (c) reticulin stain showing large mediastinal tumor, malignant small round cell tumor with features suggestive of diffuse high-grade non-Hodgkin's lymphoma, probably lymphoblastic lymphoma

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Video 1: Echocardiogram in (a) apical four-chamber and (b) modified short-axis views showing large circumferential pericardial effusion. Collapse of right atrial wall (a) and right ventricle (b) is also seen.

[Additional file 1]

Video 2: Echocardiogram in (a) subcostal modified view and (b) short-axis view showing circumferential pericardial effusion. Evidence of color flow in the pericardial space is also seen. (c) Parasternal short-axis view showing dense echogenic mass around the right ventricle and great arteries which was considered to be possible trauma-induced clot.

[Additional file 2]

Discussion

Pericardial effusion and cardiac tamponade in pediatric age groups can have varied etiology such as infection, post-inflammatory, malignancy, radiation-induced, or cardiac trauma-related. Kühn et al. in their study of 116 pediatric patients with pericardial effusion found neoplastic cause in 39%, idiopathic causes in 37%, collagen vascular disease in 9%, renal disease in 8%, bacterial in 3%, and others in 5%.^[1] Infective etiologies are dominant in the developing world. The patients usually have symptoms of the underlying pathology which helps in diagnosis of the causative factor.^[2],[3] A history of blunt trauma naturally points toward a possible cardiac injury as cause for pericardial effusion and tamponade. Blunt trauma necessitates urgent intervention.^[4] The lymphoma and related malignancies are known to cause pericardial effusion and also tamponade in a subset of patients. In case of malignancies, pericardial effusion can be seen during follow-up also, either as a direct effect of the malignancy or due to treatment-related complication. Some patients with malignancy rarely can have cardiac tamponade as initial presentation but they usually also have some constitutional and other symptoms. There are limited case reports about children with lymphoma and leukemia presenting with pericardial effusion and tamponade as the first symptom.^[5],[6],[7] A detailed clinical and laboratory evaluation helps to diagnose the case and guide the treatment. The natural conclusion in our index case of a reportedly healthy asymptomatic child with blunt trauma pointed towards cardiac injury as a possible causative factor for the cardiac tamponade. The treatment flow was also directed in the same line, resulting in urgent thoracotomy, pending laboratory and other advanced imaging. At the time of the operation, it was felt that the tumor was a primary mediastinal intrapericardial tumor arising from the pericardium. The etiology of the tamponade was due to the large intrapericardial tumor compressing the heart. The tumor must have been growing over the preceding few months till it reached a critical size which was then evident as tamponade. The hemodynamic diagnosis was correct in our case but the etiological diagnosis wrongly indicated trauma, which was only a coincidence , leading to the actual diagnosis. In retrospect, the color flow in the pericardial space was an artefact and the dense echogenic tissue considered initially as a possible clot actually was the lymphoma mass. Truly, it is said, that “what the mind does not know, eyes cannot see.” The stormy postoperative course and morbidity and cost could have been avoided had the etiological diagnosis been suspected during a detailed initial evaluation.

Pericardial tamponade is an emergency situation requiring urgent attention. The treatment is usually directed to hemodynamic relief by pericardiocentesis or surgical intervention. Careful clinical evaluation and investigations, including the analysis of the pericardial fluid, help in diagnosing and treating the underlying etiology. Clinical echocardiographer has a great responsibility to holistically analyze the case history, clinical findings, and echocardiographic data together. The correct diagnosis and appropriate management are greatly aided by an accurate clinical input. In spite of all the precautions taken in a given situation, a different disease can present in disguise, with significant therapeutic implication as in our index case. An alert clinical cardiologist can avert such diagnostic errors.

Conclusion

Pericardial effusion with cardiac tamponade is an urgent clinical condition which requires quick, yet proper evaluation for appropriate diagnosis and treatment. In children, malignancies like lymphoma or leukemia can rarely present with pericardial effusion as the first manifestation. In an emergent condition, such as hemodynamically significant pericardial effusion with tamponade effect, sometimes it is very difficult to reach the correct etiological diagnosis when the history is misleading. Alert, out of box thinking and examination may save the fortunate clinician and the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

K Sivakumar is an editorial board member of the Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging. The article was subject to the journal's standard procedures, with peer review handled independently of this editor and their research groups.

There are no other conflicts of interest.

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