BRIEF REPORT • OFID • 1
Open Forum Infectious Diseases
BRIEF REPORT
Rapid Reversal of Complete
Binocular Blindness With High-Dose
Corticosteroids and Lumbar Drain in a
Solid Organ Transplant Recipient With
Cryptococcal Meningitis and Immune
Reconstitution Syndrome: First Case
Study and Literature Review
Zoe Weiss,
1
Nihaal Mehta,
2
Su Nandar Aung,
3
Michael Migliori,
4
and
Dimitrios Farmakiotis
5
1
Department of Internal Medicine, Warren Alpert Medical School of Brown University,
Providence, Rhode Island;
2
Warren Alpert Medical School of Brown University, Providence,
Rhode Island;
3
Division of Infectious Disease, Warren Alpert Medical School of Brown
University, Providence, Rhode Island;
4
Division of Ophthalmology, Rhode Island Hospital,
Warren Alpert Medical School of Brown University, Providence, Rhode Island;
5
Division of
Infectious Disease, Rhode Island Hospital, Warren Alpert Medical School of Brown University,
Providence, Rhode Island
Blindness is a rare, devastating, usually permanent complica-
tion of cryptococcal meningitis (CM). We present the frst case
of complete vision loss in a solid organ transplant recipient with
CM treated with placement of a lumbar drain who had a dra-
matic visual recovery that started afer 3 doses of high-dose
steroids.
Keywords. cryptococcal meningitis; immune reconstitu-
tion infammatory syndrome; CM-IRIS; vision loss.
CASE PRESENTATION
A 62-year-old woman with history of renal transplantation
18 years earlier presented with headache, photophobia, and
right facial droop. Her immunosuppressive regimen included
mycophenolate, tacrolimus, and prednisone in the setting of
chronic rejection. Brain MRI, MRA, and MRV were negative
for stroke. Laboratory results on admission are summarized
in Table 1. She underwent a lumbar puncture (LP) on day 3
with opening pressure (OP) of 28 cm H
2
O; her cerebrospinal
fluid (CSF) had 208 nucleated cells (88% lymphocytes), glucose
51 mg/dL, and protein 222 mg/dL; Gram stain revealed yeast
forms, and culture was positive for Cryptococcus neoformans.
She was started on liposomal amphotericin-B and renally
adjusted flucytosine; mycophenolate was discontinued, and
tacrolimus dose decreased, aiming at a trough level of 4 ng/mL.
Both blood and CSF cultures were negative by day 6 (day 3 of
antifungal treatment).
She continued to have elevated OP, receiving sequential LP
(Figure 1). All post-LP OP were <20 cm H
2
O. Funduscopic
examination did not show papilledema. On hospital day 12
(day 9 of treatment), she developed right-sided vision loss
with no light perception, right aferent pupillary defect (APD),
decreased lef-sided visual acuity with diminished color percep-
tion, and bilateral conjunctival chemosis. Computed tomogra-
phy angiography of the brain and contrast magnetic resonance
imaging of the orbits were unremarkable. On day 15, despite
repeated LP, she had bilateral complete loss of vision with no
light perception and grade I papilledema. An epidural drain was
placed to allow for continuous CSF drainage without improve-
ment. Prednisone 80 mg twice daily was started on day 16 for
presumed early immune reconstitution infammatory syn-
drome (IRIS). She recovered light perception afer the frst 3
doses. Te lumbar drain was removed the following day. Her
vision returned to baseline in both eyes by day 25 (Figure 1).
Prednisone was tapered to 10 mg over 4 weeks. She had no
further eye complaints. She completed 4 weeks of induction
for cryptococcal meningitis, followed by 6 weeks of eradica-
tion treatment, and now receives renally adjusted maintenance
fuconazole.
METHODS
We performed a literature search in the National Center for
Biotechnology Information’s PubMed database using the term
“cryptococcal meningitis,” in conjunction with “immune recon-
stitution syndrome,” “immune reconstitution inflammatory
syndrome,” “IRIS,” “CM-IRS,” “CM-IRIS”, “ocular,” “optic,”
“ophthalmic,” “ophthalmologic,” “blindness,” “visual loss,” and
“visual.” After publications were identified, their references
were reviewed. For the purposes of this analysis, we included
cases of visual impairment independent of suspected etiology
(eg, increased intracranial pressure, inflammatory optic neu-
ropathy, direct infection of the optic nerve).
RESULTS AND DISCUSSION
CM is a highly morbid opportunistic fungal infection result-
ing in elevated intracranial pressure (ICP) and neurologic
symptoms. Though classically described in the setting of HIV
infection, CM is nowadays recognized at least as frequently in
patients with other forms of immunosuppression, including
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© The Author(s) 2018. Published by Oxford University Press on behalf of Infectious Diseases
Society of America. This is an Open Access article distributed under the terms of the Creative
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DOI: 10.1093/ofd/ofy007
Received 12 November 2017; editorial decision 22 December 2017; accepted 5 January 2018.
Correspondence: Z. Weiss, MD, 593 Eddy St, Providence, RI 02906 (zoe.weiss@lifespan.org,
zoe_weiss@brown.edu).