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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 2  |  Page : 237-239
Hemorrhagic Pericarditis in a child with primary varicella infection (chickenpox)


Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India

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   Abstract 

Chickenpox (Varicella) representing the primary infection by Varicella zoster virus is a common benign and self-limited infectious disease of childhood. Although the disease can be associated with complications, they are generally mild and tend to occur in adults and immunocompromised children. Severe and life-threatening complications are extremely rare, particularly those involving the cardiovascular system. We report a malnourished 5-year-old girl with chicken pox complicated by hemorrhagic pericarditis and deep vein thrombosis leading to fatal pulmonary thromboembolism. Though varicella infection runs a benign self-limiting course, it continues to cause significant morbidity and mortality when associated with complications, particularly in malnourished children. Hence, the importance of vaccination and early recognition of complications is emphasized.

Keywords: Chicken pox, deep vein thrombosis, hemorrhagic pericarditis, septic infarction

How to cite this article:
Nandeesh B N, Mahadevan A, Yasha T C, Shankar S K. Hemorrhagic Pericarditis in a child with primary varicella infection (chickenpox). Indian J Pathol Microbiol 2009;52:237-9

How to cite this URL:
Nandeesh B N, Mahadevan A, Yasha T C, Shankar S K. Hemorrhagic Pericarditis in a child with primary varicella infection (chickenpox). Indian J Pathol Microbiol [serial online] 2009 [cited 2020 Mar 28];52:237-9. Available from: http://www.ijpmonline.org/text.asp?2009/52/2/237/48930



   Introduction Top


Varicella (Chickenpox), the primary infection caused by varicella zoster virus (VZV), is a contagious, yet benign and a common self-limiting infectious disease. This infection can sometimes be associated with a variety of complications that is more common in the immunocompromised host. [1] Complications involving the cardiovascular system are life-threatening and extremely rare. We report the findings at autopsy of a child with primary varicella infection who manifested with hemorrhagic, necrotizing pericarditis, deep vein thrombosis in the leg leading to thromboembolism and septic pulmonary infarcts.


   Case Report Top


A 5-year-old child from a village near Bangalore, Karnataka, presented with swelling of left lower limb ascending from toes to knee of 5 days duration with pain and fever. She also had altered sensorium with irrelevant talking, refusal of feeds and sphincter incontinence of 3 days duration. One week prior to the present symptoms, she had fever and characteristic chickenpox rashes over the body, lasting for 5 days with subsidence of fever following scab formation. There was no history of prior exanthematous infections in the family or neighborhood or respiratory infections in the child. Developmental milestones were attained normally. No information about vaccination of the child was available.

On examination, child was afebrile, undernourished (body weight 14kg, below 80th percentile for age; Grade I malnutrition (Indian Association of Pediatricians Standard). Pulse rate - 100/ min, regular normal volume; blood pressure and respiratory rate - normal. No lymphadenopathy, icterus or anemia was detectable, but for mild hepatosplenomegaly. The left lower limb below the knee was edematous, tender with rise in temperature and avoidance of movement. Rest of the skeletal system including skull, spine and anthropometric measurements were within normal limits. Healed maculopapular rashes were observed all over the body with centripetal distribution. Lung fields had bilateral crepitations and occasional ronchi. Cardiovascular system on admission was normal. The child was conscious, irritable, responding slowly to oral commands with retained eye to eye contact. Terminal neck stiffness was present. External ocular movements were full. The neurological examination did not reveal any significant abnormality except for mute plantar response on the left side due to pain and swelling. Investigations revealed a clear cerebrospinal fluid (CSF) under normal pressure, 275 cells (lymphocytes - 70%, polymorphs - 30%), protein 100mg/dl, sugar 31mg/dl. CSF and serum were positive by enzyme linked immunosorbent assay (ELISA) for Varicella zoster IgG antibody in high titers of 3250IU/ml and 2800IU/ml, respectively (cut off being >275mIU/ml, as per manufacturers kit - EUROIMMUN, Germany) and negative for Herpes simplex virus and Japanese encephalitis antibodies. Cranial CT scan was normal. Biochemical parameters were within normal limits, except for mild elevation of serum alkaline phosphatase, serum transaminase levels. Fourteen hours after admission, the child suddenly succumbed to cardiorespiratory arrest.

Pathological findings

A complete autopsy was performed 4 hours postmortem after obtaining informed consent from parents. External examination revealed rounded annular scabbed lesions over the anterior chest wall, abdomen, back and both thighs. The lesions over the face had punched out wall with congested, ulcerated base. The left lower leg was swollen with stretched, shiny overlying skin. The heart was flabby and enveloped by thickened and edematous parietal pericardium. The epicardium (visceral pericardium) was hemorrhagic, ragged with multiple, coalescing, small, yellow abscesses over the apex and anterior wall of the left ventricle [Figure 1a]. The coronary arteries were patent but embedded within the necrotic exudate. The underlying myocardium was edematous with no obvious infarction. The main pulmonary artery was occluded by a fresh, friable thrombus that was seen extending into the left branch [Figure 2a]. Lungs were congested and exuded frothy fluid and revealed multiple wedge-shaped hemorrhagic infarct in the both the lobes of the lungs with a small abscess at the apices [Figure 2b]. Histology revealed necrotizing hemorrhagic pericarditis and myocarditis [Figure 1b]. The lungs were edematous with multiple septic infarcts, enclosing gram positive bacterial colonies in the midst of inflammatory exudates [[Figure 2b], inset]. Liver, spleen, kidneys, adrenals and pelvic organs were normal.

Brain appeared normal on gross examination, except for diffuse cerebral edema and pallor. Histology of the brain revealed mild meningeal lymphohistiocytic infiltration at the base extending along the cranial nerve roots and brain stem and mild, but diffuse microglial reaction suggesting encephalopathy. Skin lesions showed ulcerated epidermis with collection of polymorphs and rare intranuclear Varicella zoster viral inclusions. Small intestine had foci of ischemic enteritis. Correlating with exanthematous cutaneous lesions and serological evidence, the diagnosis of primary Varicella infection with secondary complications of thrombophlebitis and deep vein thrombosis in lower limb, pulmonary embolism with multiple septic infarcts in the lung and hemorrhagic, necrotizing pericarditis with myocarditis was considered. The changes in the brain were indicative of encephalopathy. Postmortem blood culture did not yield any bacterial growth.


   Discussion Top


Chickenpox or Varicella is the primary infection caused by Varicella zoster virus, a herpes virus that has probably infected humans since prehistoric times and is highly infectious. Zoster (Shingles) is another clinical entity caused by reactivation of Varicella virus following primary infection. Varicella is almost universal; an estimated 60 million cases occur worldwide each year and in India an incidence of 4.7 lakhs has been noted making it a benign, almost inevitable disease of childhood. Complications are generally mild, but it can rarely present in severe forms especially in immunocompromised children and adults. [1],[2] Secondary bacterial infection of skin lesions is the most common complication followed by soft tissue infections and pneumonia. Other complications include those affecting the central nervous system (CNS) (meningo/encephalitis, cerebellar ataxia), liver (hepatitis), vascular, heart (myocarditis) and kidney (glomerulonephritis and acute renal failure). This postmortem pathological study describes an extremely rare complication of hemorrhagic pericarditis and pulmonary thromboembolism complicating a case of chicken pox in an undernourished child. Reports of hemorrhagic pericarditis and deep vein thrombosis with pulmonary embolism are scarce in world literature. To the best of our knowledge, this is the first report in Indian literature with detailed pathological findings. The predominance of uncomplicated cases in children tends to overshadow the morbidity and mortality associated with severe cases. [3] Cardiac complications are rare, but when present may lead to severe morbidity and mortality, sometimes even life threatening. Pericarditis is an unusual complication of this disease and runs a benign course, unless myocarditis or pericardial effusion are concurrent which may have fatal consequences. [4] Myopericarditis following Varicella infection is rare and may result in congestive cardiac failure, pericardial effusion and/or cardiac tamponade. [5] Evidence of Varicella-induced carditis must be aggressively pursued in any child with signs of acute cardiac decompensation in whom chickenpox is confirmed or suspected. [6] Deep vein thrombosis is an uncommon systemic manifestation of Varicella, and vascular endothelial wall damage caused by Varicella zoster virus infection. [5] or endothelial activation or antiphospholipid antibodies are implicated in the pathophysiology of thrombosis. [7]

As the virus is difficult to document in tissues in association with VZV-associated complications, it is not surprising that the recognition of the varied spectrum of disease caused by VZV is always a challenge. In such instances, viral serology proves helpful in confirmation of diagnosis. Complications of Varicella requiring hospitalization in children are becoming more frequent than previously thought. [8] Even though a lethal outcome remains a rare occurrence, it may be of relevant concern when considering the overall incidence of chickenpox in the general population. The present case serves to emphasize the importance of including Varicella vaccine in the childhood immunization program to help decrease Varicella-related life-threatening complications.

 
   References Top

1.Ziebold C, von Kries R, Lang R, Weigl J, Schmitt HJ. Severe complications of varicella in previously healthy children in Germany: A 1-year survey. Paediatrics 2001;108:E79.  Back to cited text no. 1    
2.Preblud SR. Varicella complications and costs. Paediatrics 1986;78:728-35.  Back to cited text no. 2    
3.Almuneef M, Memish ZA, Balkhy HH, Alotaibi B, Helmy M. Chickenpox complications in Saudi Arabia: Is it time for routine varicerlla vaccination? Int J Infect Dis 2006;10:156-61.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Seddon DJ. Pericarditis with pericardial effusion complicating chickenpox. Postgrad Med J 1986;62:1133-4.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Gogos CA, Apostolidou E, Bassaris HP, Vagenakis AG. Three cases of varicella thrombophlebitis as a complication of varicella zoster virus infection. Eur J Clin Microbiol Infect Dis 1993;12:43-5.  Back to cited text no. 5  [PUBMED]  
6.Abrams D, Derrick G, Penny DJ, Shinebourne EA, Redington AN. Cardiac complications in children following varicella zoster virus. Cardiol Young 2001;11:647-52.  Back to cited text no. 6  [PUBMED]  
7.Barcat D, Constans J, Seigneur M, Guerin V, Conri C. Deep Vein thrombosis in an adult with varicella. Rev Med Interne 1998;19:509-11.  Back to cited text no. 7    
8.Koturoglu G, Kurugol Z, Cetin N, Hizarcioglu M, Vardar F, Helvacim et al . Complications of Varicella in healthy children in Izmir, Turkey. Pediatr Int 2005;47:296-9.  Back to cited text no. 8    

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Correspondence Address:
S K Shankar
Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore - 560 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.48930

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    Figures

  [Figure 1a], [Figure 1b], [Figure 2a], [Figure 2b]

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