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Table 2 Summary of the current and previously reported cases of cytomegalovirus (CMV) retinitis in the setting of immunodeficiency associated with thymoma (Good syndrome)

From: Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients—case report and comprehensive review of the literature

 

Author (year)

Age (years)

Gender

Unilateral (U) or bilateral (BL)

Timing of CMV retinitis relative to thymoma diagnosis (months)

Associated opportunistic infectionsa

Zone (involved)b

Retinitis treatmentc

CMV testing

Vision when retinitis was first diagnosed

Follow-up (months)

Vision at the last visit

Previously published cases

Ho et al. (2010) [10]

68

M

U

75 months after thymoma

Recurrent pneumonia; disseminated CMV; CMV colitis

Zone I

IV ganciclovir and then PO valganciclovir

Lung biopsy

20/200

1

20/100

Mateo-Montoya et al. (2010) [11]

57

M

U

NR. “Long time after thymoma”

Recurrent pneumonia; Camplyobacter sepsis

Zone I

IVT ganciclovir, IVT foscarnet, and IV ganciclovir then PO valganciclovir

Aqueous PCR; vitreous PCR

20/100

6

20/40

Park et al. (2009) [12]

56

M

BL

3 months after thymoma

NR

Zone I and zone II

IVT ganciclovir and IV ganciclovir, then PO valganciclovir

Aqueous PCR; serum IgG

20/800 OD, 20/125 OS

6

CF at 30 cm OD, NLP OS

Sen et al. (2005)1 [13]

48

M

U

60 months after thymoma

Pneumocystis jiroveci pneumonia; history of retinitis and optic neuropathy

Zone I

IVT ganciclovir and IVT ganciclovir implant (Vitrasert)

Aqueous PCR

20/200

7

20/200

Wan et al. (2012) [14]

51

F

U

60 months after thymoma

Recurrent sinopulomary infections; CMV enterocolitis

Zone II

PO valganciclovir then IVT ganciclovir weekly

Vitreous PCR

20/40

6

20/50 then subsequently to CF due to the development of autoimmune retinopathy

Yong et al. (2008) [15]

50

F

U

6 months after thymoma

Herpes zoster (T10 dermatome)

NR

IV ganciclovir, then PO valganciclovir

Vitreous PCR

NR

2

NLP

Assi et al. (2002) [16] case 1

45

F

U

24 months after thymoma

Recurrent pneumonia; zoster dermatitis

Zone II and zone III

IV valaciclovir, then IVT ganciclovir implant

Vitreous PCR

20/40

6 weeks

NR

Assi et al. (2002) [16] case 2

65

F

U

24 months after thymoma

Recurrent pneumonia

Zone I

IVT foscarnet then PO ganciclovir

Vitreous PCR

HM

NR

HM

Current case

Downes, et al. (2016)

65

F

U

1 month before thymoma

Oropharyngeal candidiasis; Candida esophagitis; pneumonia

Zone II and zone III

IVT ganciclovir, then PO valganciclovir

Aqueous PCR

20/100

7

20/80

Summary

Total n = 9

Mean: 56 years

Male: 4/9 (44.4 %)

8/9 (88.9 %) unilateral

Retinitis diagnosed after thymoma: 8/9 (88.9 %)

Respiratory infections: 7/9 (77.8 %)

Zone I: 5/8 reported (62.5 %)

Intravitreal therapy alone: 1/9 (11.1 %)

Positive aqueous PCR: 4/9 (44.4 %)

Acuity better than 20/40: 0/9 eyes (0.0 %)

Mean = 4.56 months

Acuity better than 20/40: 0/9 eyes (0.0 %)

Median: 56 years

Female: 5/9 (55.5 %)

Mean = 31.4 months after thymoma

Non-ocular CMV: 2/9 (22.2 %)

Zone II: 4/8 reported (50 %)

Systemic therapy alone: 2/9 (22.2 %)

Positive vitreous PCR: 5/9 (55.5 %)

Acuity between 20/40 and 20/200: 7/9 eyes (77.8 %)

Median = 6 months

Acuity between 20/40 and 20/200: 5/9 eyes (55.5 %)

Range: 48–68 years

Male to female ratio 0.8:1

Median = 24 months after thymoma

Other opportunistic infections: 3/9 (33.3 %)

Zone III: 2/8 reported (25 %)

Combo intravitreal and systemic therapy: 6/9 (66.6 %)

Confirmed by other means: 1/9 (11.1 %)

Acuity worse than 20/200: 2/9 eyes (22.2 %)

Range = 1.5–7 months

Acuity worse than 20/200: 4/9 eyes (44.4 %)

Range = 75 months after to 1 month before

  1. Abbreviations: IgG immunoglobulin G, M male, F female, U unilateral, BL bilateral, CMV cytomegalovirus, NR not reported, CF count fingers, NLP no light perception, LP light perception, HM hand motion, IV intravenous, PO per oral, IVT intravitreal, PCR polymerase chain reaction
  2. aAll patients were tested for HIV and found to be negative
  3. bZone definitions are as follows: zone I defined as macula or optic nerve involvement; zone II defined as mid-periphery; and zone 3 defined as outer periphery. Zone definitions referenced in this paper: Cunningham ET Jr, Hubbard LD, Danis RP, Holland GN. Proportionate topographic areas of retinal zones 1, 2, and 3 for use in describing infectious retinitis. Arch Ophthalmol. 2011;129(11):1507–8 [115]
  4. cDosing with each modality varied widely across studies