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Case Report

Posterior Reversible Encephalopathy Syndrome After Transsphenoidal Resection of


Pituitary Macroadenoma
Marcus Wong1, Sibi Rajendran1, Shruti Harish Bindiganavile3, Nita Bhat3, Andrew G. Lee2,4-10, David S. Baskin1,2

Key words - BACKGROUND: Posterior reversible encephalopathy syndrome is manifested


- CSF diversion by a reversible neurologic deficit such as vision loss, encephalopathy, and a
- Endoscopic endonasal
- Intracranial hypotension
posterior location, typically the occipital lobes. It is commonly thought to be
- PRES related to acute, severe hypertension.
- Transsphenoidal
- CASE DESCRIPTION: A 51-year-old woman presented with visual loss for
Abbreviations and Acronyms several months, and a suprasellar mass was diagnosed. She underwent trans-
CSF: Cerebrospinal fluid sphenoidal surgery, which was complicated by cerebrospinal fluid leak, and she
MRI: Magnetic resonance imaging
PRES: Posterior reversible encephalopathy syndrome developed posterior reversible encephalopathy syndrome while undergoing
SBP: Systolic blood pressure postoperative cerebrospinal fluid drainage via lumbar catheter. Her visual acuity
TSS: Transsphenoidal surgery progressed to blindness, but blindness was reversed by discontinuation of
From the 1Neurological Institute, Department of
lumbar drainage, tight blood pressure control, and high-dose steroid drip.
Neurosurgery, Houston Methodist Hospital, Houston, Texas; - CONCLUSIONS:
2
Kenneth R. Peak Brain and Pituitary Tumor Treatment
To our knowledge, this is only the second case of posterior
Center, Houston, Texas; 3Blanton Eye Institute, Department reversible encephalopathy syndrome following transsphenoidal surgery to be
of Ophthalmology, Houston Methodist Hospital, Houston, reported in the neurosurgical or ophthalmic English language literature.
Texas; 4Department of Ophthalmology, Weill Cornell
Medicine, New York, New York; 5Department of
Ophthalmology, Baylor College of Medicine, Houston, Texas;
6
Department of Ophthalmology, University of Texas Medical may cause irreversible (due to cytotoxic CASE DESCRIPTION
Branch, Galveston, Texas; 7Department of Ophthalmology,
edema) damage rather than being fully
University of Texas MD Anderson Cancer Center, Houston, A 51-year-old woman presented with visual
Texas; 8Department of Ophthalmology, Texas A&M College reversible), and may not include
loss for several months, and a suprasellar
of Medicine, Bryan, Texas; 9Department of Ophthalmology, encephalopathy. Though many drugs and
mass was diagnosed. Her past medical,
University of Iowa Hospitals and Clinics, Iowa City, Iowa; and predisposing factors have been implicated,
10
Department of Ophthalmology, University at Buffalo, surgical, social, and family history was
the most common risk factor for PRES
Buffalo, New York, USA negative. She was taking no medications
development is acute, severe
To whom correspondence should be addressed: and had no allergies. Her blood pressure
hypertension.2 Other associated conditions
Marcus Wong, M.D. before surgery was normal. Preoperative
include preeclampsia/eclampsia, sepsis,
[E-mail: mswong@houstonmethodist.org] MRI showed a 2.7 cm anteroposterior  2.5
autoimmune disease, chemotherapy, and
Citation: World Neurosurg. (2020) 142:171-175. cm transverse  2.9 cm craniocaudal mass
https://doi.org/10.1016/j.wneu.2020.06.136 solid-organ or bone marrow/stem cell
in the suprasellar region with displacement
Journal homepage: www.journals.elsevier.com/world- transplantation.3 The diagnosis is made
and compression of the optic chiasm
neurosurgery clinically and is supported by magnetic
(Figure 1). Preoperative eye examination
Available online: www.sciencedirect.com resonance imaging (MRI) showing typical
showed normal visual acuity (20/20 in
1878-8750/$ - see front matter Published by Elsevier Inc. white matter changes and diffusion-
both eyes) but a dense bitemporal
weighted imaging showing vasogenic
hemianopsia (Humphrey visual field 24-2)
(rather than cytotoxic) edema in the poste-
INTRODUCTION (Figure 2) with associated optic atrophy in
rior parietal and occipital lobes.4 Several
both eyes on examination and on optical
Posterior reversible encephalopathy syn- mechanisms have been proposed, but
coherence tomography.
drome (PRES) is a well-described final ultimately the pathophysiologic basis
appears to be linked to failure of proper A periprocedural lumbar drain was
common pathway for a number of patho-
cerebral vascular autoregulation.5 PRES inserted before surgery. The patient was
genic mechanisms affecting the blood-brain
following neurosurgical procedures is positioned in a park-bench position with
barrier. PRES is typically characterized by
distinctly rare and has been reported only tumor and sellar localization performed
posterior location (e.g., occipital lobes), a
once previously after transsphenoidal with intraoperative navigation and fluo-
reversible neurologic deficit (due to vaso-
surgery (TSS). To our knowledge, this is roscopy. A radical subtotal excision was
genic edema), and encephalopathy (e.g.,
only the second such case to be reported in achieved with good decompression of the
headache, altered consciousness, and sei-
optic apparatus. A small intraoperative
zures).1 Atypical PRES, however, may be the neurosurgical or ophthalmic English
language literature. CSF leak was noted at the base of the
anterior rather than posterior in location,

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CASE REPORT
MARCUS WONG ET AL. PRES AFTER TRANSSPHENOIDAL SURGERY

tumor capsule, which was sealed intra-


operatively, and a fat graft was placed.
Preoperative and intraoperative blood
pressure measurements were normal.
The immediate postoperative course
was uneventful. Notably there was dra-
matic improvement in her bitemporal
hemianopsia (Humphrey visual field 24-2)
(Figure 3). She was started on a
hydrocortisone taper and prophylactic
antibiotics for her lumbar drain, which
drained approximately 200 mL a day. On
postoperative day 4, her blood pressure
began to increase from her baseline
systolic blood pressure (SBP) of 120 mm
Hg to 160 mm Hg sustained over 24
hours, with a maximum of 180 mm Hg.
She had a concurrent headache that
progressed throughout the evening, and
by morning she began having quickly
declining vision to no light perception
bilaterally. Emergent computed
tomography and MRI revealed bilateral
occipital hypodensity with an associated
abnormal T2 fluid-attenuated inversion
recovery signal compatible with ongoing
edema (Figure 4). A new thin subdural
collection consistent with intracranial
hypotension was also noted. Notably no
sinus thrombosis, pituitary apoplexy, or
Figure 1. Preoperative imaging. (AeC) Contrast-enhanced T1-weighted magnetic resonance imaging
coronal (A), sagittal (B), and axial (C) views. (D) Sagittal computed tomography angiography shows the compression of the optic chiasm was seen.
pituitary adenoma with expansion of the sella turcica. She was transferred to the intensive
care unit for 5.4 mg/kg/hour methyl-
prednisolone drip and strict blood pres-
sure control to keep her SBP between 120
and 145 mm Hg using an antihyperten-
sive drip. The lumbar drain was clamped
and removed. Over the day, her vision
improved significantly, and she was able
to discern rough shapes. Blood pressure
control and steroid drip were maintained
for 3 days, and she was noted on formal
ophthalmologic examination to have
improved vision from counting fingers
(20/100 right eye, 20/200 left eye, to right
eye 20/70, left eye 20/60). The pupil ex-
amination was normal in both eyes, and
the remainder of the eye examination,
including the fundus examinations, was
normal in both eyes. By postoperative day
10 (intensive care unit day 6), she was
noted to have improved bitemporal
hemianopsia and vision good enough to
read and use her cellular phone. Sur-
veillance MRI revealed resolution of pre-
vious fluid-attenuated inversion recovery
Figure 2. Preoperative visual fields with classic dense bitemporal hemianopsia on Humphrey visual
signal and subdural collections. She was
fields.
discharged on postoperative day 14 on

172 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2020.06.136


CASE REPORT
MARCUS WONG ET AL. PRES AFTER TRANSSPHENOIDAL SURGERY

low-dose hydrocortisone and oral anti-


hypertensives. She was seen in both the
neurosurgery and the ophthalmology
clinic, and her postoperative bitemporal
hemianopsia had improved compared
with preoperative baseline, with some
residual field loss present (Figure 3). Her
visual acuity returned to preoperative 20/
50 in both eyes.

DISCUSSION
We present a case of PRES following
endoscopic endonasal transsphenoidal
resection of a pituitary tumor where the
patient developed worsening visual
disturbance and headache. Given that
Figure 3. Postoperative visual fields with bitemporal hemianopsia denser superiorly on Humphrey both visual changes and headache can be
visual fields. related to pituitary tumors and post-
operative complications of TSS (e.g.,
hemorrhage into the resection cavity,
apoplexy, edema), the immediate
concern was for those entities. Emergent
imaging should always be performed in
post-operative patients with new deficit,
and in our case immediate CT and MRI
were obtained. Our patient had classic
bitemporal hemianopsia before surgery,
but developed complete blindness
following onset of PRES after surgery.
Similarly, in the other case of PRES
following TSS in the literature, vision
decline in the form of new right hom-
onymous hemianopsia was observed.6 In
that case, there was no intraoperative
CSF leak and no lumbar drain used.
Neuro-ophthalmology consultation and
formal visual field testing was obtained
in our case, and we suggest the same for
all patients with PRES-associated visual
disturbances.
The current most accepted pathophysio-
logic basis of PRES involves a hyper-
perfusion injury model related to
hypertension.7 A sudden elevation in blood
pressure causes critical failure of cerebral
autoregulation. Arteriolar dilation and
endothelial dysfunction cause disruption of
the blood-brain barrier and thus the resul-
tant vasogenic edema that can be appreci-
ated on imaging. Though this theory seems
sufficient to explain PRES related to acute
hypertension, it fails to address the cases
Figure 4. (A) Emergent computed tomography coinciding with progressive vision loss reveals bilateral (estimated at 30%) of PRES not related to
occipital lobe hypodensity. (B) Magnetic resonance imaging T2 fluid-attenuated inversion recovery hypertension.1 The other competing
signal concerning for PRES in the same location as hypodensity. (C) Follow-up magnetic resonance hypothesis implicates hypotension as a
imaging reveals resolving T2 fluid-attenuated inversion recovery signal 6 days after initiation of blood
pressure control and steroids and removal of lumbar drainage.
cause, where decreased blood pressure
leads to reflexive vasoconstriction, cerebral

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CASE REPORT
MARCUS WONG ET AL. PRES AFTER TRANSSPHENOIDAL SURGERY

ischemia, and ultimately vasogenic edema. developed PRES following surgery drugs where appropriate, and serial im-
Another theory describes an immunologic characterized by altered consciousness aging and neurologic examinations to
mechanism, where T-cell/endothelial cell and status epilepticus. evaluate for resolution. Corticosteroids
activation leads to trafficking of leukocytes, A final point remains in treatment of remain controversial, but most reports
cerebral vasoconstriction, hypoperfusion, PRES. While there remains much specu- seem to demonstrate a positive effect,
ischemia, and subsequent vasogenic lation on the true mechanisms behind especially with regard to vasogenic
edema.8 PRES, nearly all studies support strict edema. We report a favorable outcome
Regardless of the underlying mecha- blood pressure control in the treatment of in our patient, which we owe to timely
nism, it remains unclear how recent PRES once it has developed. In our case, diagnosis and medical management.
neurosurgical intervention plays a role in the patient was maintained on strict blood
development of PRES. Case reports of pressure parameters (SBP between 120 REFERENCES
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174 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2020.06.136


CASE REPORT
MARCUS WONG ET AL. PRES AFTER TRANSSPHENOIDAL SURGERY

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Conflict of interest statement: This work was supported in Journal homepage: www.journals.elsevier.com/world-
13. Meyer M, Niemöller U, Stein T, et al. Positive part by Donna and Kenneth Peak, the Kenneth R. Peak neurosurgery
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Available online: www.sciencedirect.com
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173-177. Wolff Memorial Foundation, the Kelly Kicking Cancer 1878-8750/$ - see front matter Published by Elsevier Inc.

WORLD NEUROSURGERY 142: 171-175, OCTOBER 2020 www.journals.elsevier.com/world-neurosurgery 175

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