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Heather Spampinato, O.D.
Dayton Ohio VAMC
Residents Day 2009 Case Report
Abstract
A 62 year old HIV-positive white male presents with monocular panuveitis and
retinal necrosis. Ocular examination along with laboratory test results confirm a
diagnosis of syphilitic retinitis. Diagnosis, treatment, and management are discussed.
Case History
A sixty-two year old white male presented to the Dayton VAMC Optometry clinic
after being referred from Urgent Care for a red left eye. The patient stated that his left
eye had been red for two days and felt “grainy”. The patient denied discharge, tearing,
or pain. He also reported that his vision seemed slightly decreased as if there was a
grey film over his left eye for the past six weeks.
Past ocular history includes peripheral retinal hemorrhage of unknown etiology in
the left eye three years prior and a resolved left cranial nerve four palsy six months prior
to presentation. The patient also had a recent history of decreased vision in his left eye
for a few months of unknown etiology. Work-up at that time included a fluorescein
angiogram that was unremarkable, visual field testing with some degree of central field
loss, and MRI of the orbits that was unremarkable.
The patient’s medical history was significant for type 2 diabetes mellitus,
dyslipidemia, HIV, erectile dysfunction, hearing loss, post traumatic stress disorder, and
dermatitis. The patient takes glyburide, metformin, rosiglitazone, and insulin for
diabetes as well as efavirenz, lamivudine, and tenofovir for HIV. The patients most
recent HBA1C was 7.9%. The patient had an undetectable viral load and his CD4 count
was 397.
Pertinent Findings
Entering acuities were 20/20- in the right eye and 20/40- in the left. Pupils,
extraocular motilities, and confrontation fields were all normal. Intraocular pressures
were 15 mmHg in each eye. Slit lamp examination and dilated fundus exam were
unremarkable for the right eye. The left eye showed circumlimbal flush as well as fine
keratic precipitates on the corneal endothelium. The anterior chamber showed 1+ cells
and flare. Dilated fundus examination of the left eye revealed a well-circumscribed area
of retinal necrosis with placoid areas along the superotemporal arcade with trace
vascular sheathing. There was no hemorrhaging noted. There was trace vitreous haze,
though no inflammatory cells were noted.
Differential Diagnosis
Differentials in HIV positive patients with retinal necrosis and panuveitis include
progressive outer retinal necrosis (PORN), acute retinal necrosis (ARN), toxoplasmosis,
opportunistic infections, and syphilis.
At the initial presentation, a tentative diagnosis of PORN was made. The patient
was started on Valtrex 1000 mg BID. For the uveitis, the patient was started on
predforte every two hours and cyclogyl 1% three times a day. Toxoplasmosis titers,
HSV and VZV antibody titers, RPR and FTA, and PPD were ordered to rule out other
possible etiologies for retinitis. Given the patient’s CD4 count and undetectable viral
load, infectious causes such as cryptococcus were ruled out. Over the next seven
days, the patient experienced a worsening of symptoms along with the onset of pain.
Acuity decreased from 20/40 to 20/100 in the left eye. Ocular examination revealed no
improvement in the uveitis and marked progression of retinal necrosis toward the
inferior arcade with less discrete margins as previously noted along with a new vitritis.
At this time the patient was admitted to the hospital and started on IV acyclovir for likely
ARN. After starting IV acyclovir, the patient was continually monitored for the next five
days with continued worsening of his vitritis and slight progression of retinal necrosis.
On the fifth day of acyclovir therapy, lab results arrived showing a reactive RPR (1:32)
and reactive FTA, confirming a diagnosis of syphilitic retinitis. At this time, the patient
also underwent a lumbar puncture which showed an elevated white blood cell count,
confirming neurosyphilis. All other lab tests were negative.
Diagnosis and Discussion
Syphilis is commonly referred to as the “great masquerader” due to the fact that
the ocular presentation of this disease mimics many other conditions. In general,
syphilis can affect any ocular tissue including the conjunctiva, sclera, cornea, uveal
tract, retina, vasculature, optic nerve, pupillomotor pathways, and cranial nerves. 1 The
initial presentation of ocular syphilis is most often a uveitis in both HIV positive and HIV
negative individuals. When the initial presentation is a posterior uveitis, it is usually
characterized by a chorioretinitis with variable amounts of vitreal inflammation. 1 In the
case of this patient, syphilis presented as a panuveitis with a progressive necrotizing
retinitis.
Diagnosis of syphilis is best made using both nontreponemal and treponemal
tests. Nontreponemal tests include the Venereal Disease Research Laboratory test and
the rapid plasma reagin (RPR) test. These tests are most useful in screening for active
disease and monitoring a patient’s progress in recovery. Treponemal tests such as the
fluorescent treponemal antibody absorption (FTA-ABS) test and the T. pallidum particle
agglutination (MHA-TA) test are mostly used to confirm current or previous syphilitic
infection.2 In some studies, it has been found that syphilis may go undetected by
nontreponemal testing strategies in as many as 30% of both treated and untreated
individuals due to the high false negative rates associated with these tests. 2
Furthermore, in HIV positive individuals, nontreponemal tests may be negative due to
progressive immune dysfunction and inability to develop antibodies against T.
Pallidum.3
Often times a lumbar puncture will also be performed once a diagnosis of syphilis
has been established to rule out neurosyphilis.4 In neurosyphilis, cerebrospinal fluid will
show leukocytosis, elevated protein levels, and a reactive CSF VDRL.2 However, it is
important to note that in many HIV positive patients, CSF abnormalities are common
even in the absence of syphilis. One study estimates that as many as 63% of all HIVpositive patients have abnormal CSF results.3
Treatment and Management
The Centers for Disease Control recommends treatment of syphilis with penicillin
G 24 million units per day for 14 days in patients who are also HIV positive, regardless
of stage of disease.5 In patients who are HIV positive, a longer course of penicillin may
be used.
At diagnosis, IV acyclovir was discontinue and the patient was placed on IV
penicillin G 24 million units per day for 17 days. One week after the initiation of
penicillin therapy, the patient developed a recurrence of a left cranial nerve four palsy,
which slowly resolved over the next few weeks and was likely a sequelae to the
infection. At the completion of the penicillin course, predforte and cyclogyl were
tapered. At this time the patient’s vision had returned to 20/40 with complete resolution
of the panuveitis along with no active retinitis. Six weeks after completion of treatment
the patient’s vision had returned to 20/20. Dilated fundus exams have been repeated at
three month intervals since resolution with no further complications. Repeat lumbar
punctures and RPR testing have also remained stable.
Conclusion
Clinical Pearls
- Any instance of unexplained or atypical ocular inflammation should include a work-up
for syphilis, especially in high risk patient populations.
- Patients with HIV are at a higher risk of having ocular and systemic complications from
syphilis.
- Some studies have shown that CD4 counts do not correlate to the severity of ocular
involvement in patients with syphilis.4 Therefore, syphilis should always be included in
the list of differentials.
- Co-infection of HIV with syphilis alters the severity of the disease and increases the
likelihood of further CNS involvement.4
References
1.) Kiss, Szilard, Francisco M. Damico, and Lucy H. Young. "Ocular Manifestations and
Treatment of Syphilis." Seminars in Ophthalmology. 3.20 (2005): 161-167.
2.) Aldave, Anthony J., Julie A. King, and Emmett T. Cunningham. "Ocular Syphilis."
Current Opinions in Ophthalmology. 12 (2001): 433-441.
3.) Browning, David J. "Posterior Segment Manifestations of Active Ocular Syphilis,
Their Response to a Neurosyphilis Regimen of Penecillin Therapy, and the Influence of
Human Immunodeficiency Virus Status on Response." Ophthalmology. 107.11
(November 2000): 2015-2023.
4.) Gaudio, Paul A. "Update on Ocular Syphilis." Current Opinions in Ophthalmology. 17
(2006): 562-566.
5.) Balba, Gayle P., et al. "Ocular Syphilis in HIV-Positive Patients Receiving Highly
Active Antiretroviral Therapy." The Journal of American Medicine. 5.119 (May 2006):
448.e21-448.e25.