Bilateral Cytomegalovirus Retinitis in a Child with Rhabdomyosarcoma
Osman Bülent ZÜLFIKAR1,Halil Haldun EMIROĞLU2,Rejin KEBUDI1,Melike EMIROĞLU3,Samuray TUNCER4
11Department of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine and Institute of Oncology, İstanbul-Turkey
2Department of Pediatric Gastroenterology, Selcuk University Faculty of Medicine, Konya-Turkey
3Department of Pediatric Infectious Diseases, Selcuk University Faculty of Medicine, Konya-Turkey
4Department of Ophthalmology, İstanbul University Istanbul Faculty of Medicine, İstanbul-Turkey
DOI : 10.5505/tjo.2017.1552


Reactivation of cytomegalovirus (CMV) leading to retinitis has been commonly reported in association with human immunodeficiency virus (HIV) infection or iatrogenic suppression of the immune system, including transplant recipients. Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy in the pediatric age group, and alveolar histology is associated with unfavorable outcome. Presently described is case of RMS with alveolar histology in a 12-year-old male who developed CMV bilateral retinitis during prolonged period of neutropenic fever after 40 weeks of chemotherapy. He was diagnosed based on CMV-DNA polymerase chain reaction in blood and urine samples, and responded well to intravenous gancyclovir treatment. A high index of suspicion for reactivation of CMV leading to retinitis should be maintained and, if needed, investigated, not only in patients with HIV infection or transplant recipients, but also all patients who are iatrogenically immunosuppressed, including those who experience prolonged neutropenic fever due to lengthy courses of radiotherapy and chemotherapy.


Cytomegalovirus (CMV) can cause life-long subclinical latent infection in healthy individuals, which can be reactivated upon immunosuppression, affecting a number of different organ systems including eyes. Reactivation of CMV leading to retinitis has been reported in patients with human immunodeficiency virus (HIV) infection and in those subjects with iatrogenic suppression of the immune system including solid organ or hematopoietic stem cell transplant recipients.[1-4]

Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy in the pediatric age group. RMS with alveolar histology is more common in adolescents and is associated with a poorer prognosis than the embryonal type.[5]

Here, we describe a pediatric case of RMS with alveolar histology who had reactivation of CMV leading to retinitis without a history of solid organ or hematopoietic stem cell transplantation or HIV infection.


CMV retinitis is the most common complication of late-stage HIV infection, occurring when CD4+ T-cell counts are ≤50 cells/mL.[7,8] In our patient without HIV infection, deficiency in humoral and cell-mediated immunity as documented by low counts of CD4 lymphocytes due to immunosuppressive effects of chemotherapeutic agents might have lead to susceptibility for CMV retinitis.

In patients without HIV infection, CMV retinitis is characterized by necrotizing retinitis, often with intraretinal hemorrhage similar to that observed in patients with HIV infection.[4] Our patient had bilateral active necrotizing CMV retinitis with intraretinal hemorrhage. Ophthalmological characteristics of CMV retinitis in children are slightly different from those observed in adults. In a study involving a total of 9 immunocompromised children with CMV retinitis under 16 years of age, Baumal et al. found bilateral disease in eight children.[9] In our patient, there was bilateral disease with posterior pole involvement only in the left eye.

Management of CMV retinitis involves a number of different medical treatment modalities such as oral (ganciclovir, valganciclovir), intravenous (ganciclovir, foscarnet, cidofovir) and intravitreal (ganciclovir, foscarnet, cidofovir, fomivirsen, and ganciclovir intravitreal implant) routes.[10,11] In our patient, the rationale of the treatment of CMV retinitis was based on improving the defective immune system by discontinuing chemotherapy, and reducing viremia and organ damage with intravenous ganciclovir.

CMV reactivation can happen that not only in transplant recipients or patients with HIV infection, but also in patients with tumor who received lengthy courses of chemotherapy and radiotherapy. A high index of suspicion for reactivation of CMV leading to retinitis should be maintained and, if needed, investigated in non-transplant tumor patients with prolonged neutropenic fever. In this regard, CMV-DNA PCR analysis for early diagnosis and follow-up is of utmost importance.

We believe that early diagnosis and prompt administration of treatment for CMV retinitis was probably the single most important factor for the prevention of progression to a more serious form of the disease because of the response to ganciclovir depends on timely administration of treatment and clinical severity of the condition.

Disclosure Statement
The authors declare no conflicts of interest.

Financial Disclosure
The authors declared that this study received no financial support.


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