Introduction Orbital cellulitis is an uncommon, potentially devastating condition that, when not promptly and adequately treated, can lead to serious sequelae. The presenting clinical signs are proptosis, swelling, ophthalmoplegia, pain and redness of the peri-orbital tissues. A number of case

A Nakajima

A Salam

CM Flaitz

DK Dhariwal

GJB Simon

H Igarashi

HJ Delecluse

IA Chaudhry

IK Kim

JL Tovilla-Canales

Matthias A. W. Merkx

MP Mourits

Niek L. Gerlach

Richard Barkhuysen

S Nithyanandam

S Yeh

Stefaan J. Bergé

WC Harrill

Willem L. J. Weijs

English

PubMed

INTRODUCTION: Orbital cellulitis is an uncommon, potentially devastating condition that, when not promptly and adequately treated, can lead to serious sequelae. The presenting clinical signs are proptosis, swelling, ophthalmoplegia, pain and redness of the peri-orbital tissues. A number of cases have been reported in which these symptoms have been mistakenly interpreted as being secondary to an orbital infection whilst, in fact, other pathology was present. DISCUSSION: We add another case in which, on clinical grounds and after radiological assessment and laboratory tests, a working diagnosis of orbital cellulitis of the left eye was made. It was only after histopathological analysis of a soft tissue specimen from the maxillary sinus that a diagnosis of an AIDS-related plasmablastic lymphoma was made. The patient was referred to the department of haematology where chemotherapeutic treatment for the lymphoma and the HIV infection was started. This case report adds another differential diagnosis of orbital cellulitis to the existing literature

Barkhuysen, R.

Merkx, M.A.W.

Weijs, W.L.J.

Gerlach, N.L.

Berge, S.J.

NARCIS 

Plasmablastic lymphoma mimicking orbital cellulitis.

Crossref

Plasmablastic lymphoma mimicking orbital cellulitis

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ORIGINAL ARTICLE
Plasmablastic lymphoma mimicking orbital cellulitis
Richard Barkhuysen & Matthias A. W. Merkx &
Willem L. J. Weijs & Niek L. Gerlach & Stefaan J. Bergé
Published online: 3 July 2008
# The Author(s) 2008
Abstract
Introduction Orbital cellulitis is an uncommon, potentially
devastating condition that, when not promptly and ade-
quately treated, can lead to serious sequelae. The presenting
clinical signs are proptosis, swelling, ophthalmoplegia, pain
and redness of the peri-orbital tissues. A number of cases
have been reported in which these symptoms have been
mistakenly interpreted as being secondary to an orbital
infection whilst, in fact, other pathology was present.
Discussion We add another case in which, on clinical
grounds and after radiological assessment and laboratory
tests, a working diagnosis of orbital cellulitis of the left eye
was made. It was only after histopathological analysis of a
soft tissue specimen from the maxillary sinus that a
diagnosis of an AIDS-related plasmablastic lymphoma
was made. The patient was referred to the department of
haematology where chemotherapeutic treatment for the
lymphoma and the HIV infection was started. This case
report adds another differential diagnosis of orbital cellulitis
to the existing literature.
Keywords Orbital cellulitis . Plasmablastic lymphoma .
Orbital apex syndrome .Maxillofacial . AIDS
Introduction
Orbital cellulitis presents with proptosis, swelling, ophthal-
moplegia and redness of the surrounding tissues. Since
these symptoms can also be a manifestation of conditions
involving the orbital apex, superior orbital fissure or
cavernous sinus, clinicians should take these rather excep-
tional causes into consideration. A patient is presented in
whom initially a diagnosis of orbital cellulitis was made,
but that later proved to be a giant plasmablastic lymphoma.
Case
A 50-year-old female was referred by the department of
ophthalmology with a 3-day history of acute onset progres-
sive proptosis, visual loss and ophthalmoplegia of the left
eye (Fig. 1). Her medical history was non-contributory.
On physical examination, the patient proved to have a
high-grade fever (39.7°C). There was ophthalmoplegia,
inferior displacement and proptosis of the left globe (Hertel
[1]; left 26 mm, right 16 mm). Visual acuity was decreased.
Pupils were isocore and indirect pupillary reflexes were
normal. Direct pupillary reflex of the left eye was almost
absent. There was anaesthesia of the left maxillary and
ophthalmic branches (V1 and V2) of the trigeminal nerve.
We also found intra-orally multiple foci in the upper as well
as in the lower jaw and a painful swelling of the upper left
gingivobuccal sulcus. Leucocyte count was normal but C-
reactive protein and erythrocyte sedimentation rate were
markedly elevated.
Computed tomography (CT) showed an abscess in the
left infratemporal fossa with extension of inflammatory
tissue to the posterior cranial fossa, temporal fossa,
maxillary sinus and orbit. There was also destruction of
the skull base. Dental panoramic radiography revealed
multiple periapical radiolucencies and caries.
On the basis of these findings, a working diagnosis of an
abscess in the left infratemporal fossa with extension to the
Oral Maxillofac Surg (2008) 12:125–128
DOI 10.1007/s10006-008-0117-5
R. Barkhuysen (*) :M. A. W. Merkx :W. L. J. Weijs :
N. L. Gerlach : S. J. Bergé
Department of Oral and Maxillofacial Surgery 590,
Radboud University Nijmegen Medical Centre,
P.O. Box 9101, 6500 HB Nijmegen, The Netherlands
e-mail: R.Barkhuysen@mka.umcn.nl
left orbit causing orbital cellulitis and destruction of the
skull base was made. In the differential diagnosis, other
possibilities were also taken into consideration, such as a
complicating unilateral cavernous sinus thrombosis, osteo-
myelitis, an underlying malignancy or a rhino-orbito-
cerebral mucormycosis.
The patient was immediately transferred to the operating
ward with the intention to surgically drain a possible
abscess as well as to collect material for further histopath-
ological and microbiological investigations. All teeth were
extracted. Peroperatively, no pus was encountered. The left
maxillary sinus walls were found to be destroyed and the
sinus was filled with pathological tissue. Clindamycin
(600 mg q 8 h) was started intravenously. There was a
favourable response to this as fever resolved, redness of the
periorbita disappeared and swelling and proptosis dimin-
ished. Infectious laboratory parameters also quickly nor-
malised. Ophthalmoplegia, sensory disturbance of V1 and
V2 and visual acuity did not improve (Fig. 2 and 3).
Histopathological investigation revealed a plasmablastic
lymphoma. Because this disease is AIDS-related, an HIV
test was conducted, which proved to be positive. Postop-
eratively, magnetic resonance imaging (MRI) was made to
exclude cavernous sinus thrombosis and to better assess the
extension of the diagnosed pathology. Multiple masses
were seen (Fig. 4). There was encroachment upon the
cavernous sinus and infiltration into the orbit, but no signs
of cavernous sinus thrombosis.
The patient was sent to the haematology department where,
after dissemination investigation, 18 courses of rituximab,
cyclophosphamide, doxorubicin, vincristine and prednisolone
chemotherapy (R-CHOP) were given and highly active anti-
retroviral therapy consisting of emtricitabine, ritonavir,
tenofovir and atazanavir was started. She also received 18
sessions of intrathecal chemotherapy (methotrexate alternated
with cytarabine/DAF). Because of partial remission after eight
courses of R-CHOP, she also received radiotherapy to the
skull. A repeated CTscan showed regression of the lymphoma
and re-ossification at the skull base.
Fig. 2 Ophthalmoplegia on upward gaze
Fig. 3 Ophthalmoplegia on left gaze
Fig. 4 Axial magnetic resonance image showing huge plasmablastic
lymphoma infiltrating the left orbit
Fig. 1 Proptosis, peri-orbital swelling, redness, hypoglobus and
chemosis of the left eye as well as a temporal mass on the left side
upon presentation
126 Oral Maxillofac Surg (2008) 12:125–128
Discussion
Orbital cellulitis is an infection of the orbital soft tissues
posterior to the orbital septum that presents with rapid
progressive ophthalmoplegia, proptosis, swelling of the
eye, chemosis, decrease in visual acuity, orbital pain and
redness of the eyelid [2].
In the majority of patients, it occurs as a direct extension
of infection of the paranasal sinuses, but haematogenous
spread of bacteria or direct inoculation by trauma or surgery
is also possible [3–5]. Sometimes, a dental focus is the
underlying cause [6].
The clinical signs, laboratory abnormalities and the
presence of a potential dental focus in this patient were
suggestive of an infection, i.e. orbital cellulitis. The orbital
apex, cavernous sinus and superior orbital fissure are
contiguous structures, which may be affected by a number
of conditions (inflammatory, infectious, neoplastic, iatro-
genic/trauma, vascular) [7].
Depending on the cranial nerves involved and anatomic
areas affected, different syndromes can be described:
1. superior orbital fissure syndrome (cranial nerves III, IV,
V1, VI),
2. orbital apex syndrome (cranial nerves II, III, IV, V1,
VI),
3. cavernous sinus syndrome (cranial nerves III, IV, V1,
V2, VI).
This classification is only of theoretical importance since
the close relation between the mentioned anatomical areas
causes a rapid and significant clinical overlap in the
presentation of pathological entities.
In light of the symptoms of the described patient
(involvement of cranial nerves V1 and V2), pathology
affecting the cavernous sinus requires special attention as
cavernous sinus thrombosis can occur secondary to orbital
cellulitis when infection passes posteriorly through the
ophthalmic veins. It can begin unilaterally, but will soon
progress to become bilateral, and the patient’s state of
consciousness is often impaired. MRI scanning can and did
exclude this condition [8]. The osseous destruction seen on
the CT scan led us to suppose the presence of an aggressive
or long-standing infectious condition (e.g. mucormycosis) or
malignancy (e.g. lymphoma). This fact was not completely
appreciated at first due to the patient’s acute presentation.
Rhino-orbito-cerebral mucormycosis is an aggressive fungal
infection that occurs in immune-suppressed patients and
presents with proptosis, visual loss and ophthalmoplegia
among other symptoms [9]. On CT scanning, bony
destruction is often encountered and a definite diagnosis
can be made on histopathology [10].
Orbital cellulitis as the primary clinical appearance of a
lymphoma has been reported in the literature before [11, 12].
The diagnosis of a plasmablastic lymphoma with regard to this
is, however, unique. In this case, immunopathology revealed
expression of CD45 and a weak expression of CD138 and
CD79a. There was no expression of CD56, CD30, CD3,
CD20 and CD10. Epstein–Barr virus-encoded RNA in situ
hybridisation was positive. Plasmablastic lymphoma is a rare,
AIDS-related, non-Hodgkin lymphoma with a noticeable
predilection for the oral cavity [13]. This entity was first
described by Delecluse et al. [14]. It carries a dismal prognosis
with an average survival time of 6 months after diagnosis. At
13 months after diagnosis, our patient is still alive.
In retrospect, although there was an infectious compo-
nent, the presence of the acute orbital symptoms in this case
were most likely secondary to the lymphoma that was
widespread to the skull base and orbit.
Yeh et al. presented a diagnostic algorithm for patients
with findings consistent with an orbital apex syndrome [7].
Imaging should be performed in all cases, and MRI
scanning is considered being superior to other imaging
modalities. When inflammatory or infectious disease is
suspected, proper laboratory tests should be conducted as
well. Obtaining material for histopathological analysis may
be required to arrive at a definite diagnosis.
On the basis of this case report, plasmablastic lymphoma
may be added to the list of differential diagnoses of orbital
cellulitis.
Open Access This article is distributed under the terms of the
Creative Commons Attribution Noncommercial License which per-
mits any noncommercial use, distribution, and reproduction in any
medium, provided the original author(s) and source are credited.
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Chronic rhinocerebral mucormycosis.

Elzaridi E et al (2007) Outcome of treated orbital cellulitis in a tertiary eye care center in the middle east.

Orbital abscess from an odontogenic infection. Oral Surg Oral Med Oral Pathol Radiol Endo 103:e1–e6 Oral Maxillofac Surg

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Orbital cellulitis: a rare complication after orbital blowout fracture.

Plasmablastic lymphoma: an HIV-associated entity with primary oral manifestations.

Plasmablastic lymphomasoftheoralcavity:anewentityassociatedwiththehuman immunodeficiency virus infection. Blood 89:1413–1420 128 Oral Maxillofac Surg

Post-traumatic orbital cellulitis.

Reliability of exophthalmus measurement and the exophthalmometry value distribution in a healthy Dutch population and in Graves’ patients. An exploratory study.

Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes.

Tovilla y Pomar JL

Two cases of malignant lymphoma complicated by hemophagocytosis resembling orbital cellulitis.

Plasmablastic lymphoma mimicking orbital cellulitis

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