Infections and Infestations

Infective cellulitis is an important cause of orbital inflammation and may develop from
contiguous inflammatory disease of the sinuses, face, and oropharynx. In addition, it may result
from foreign bodies or be secondary to pyemic deposition. Causes include a wide variety of
bacterial, viral, fungal, and parasitic pathogens that vary with regional epidemiology. In our
experience, the most significant category of orbital cellulitis arise from bacterial infections of the
sinuses. It is particularly important to consider the local infectious disease profile (epidemiology)
and to be suspicious of unusual pathogens in patients who are immunosuppressed.
Microbial
Orbital Cellulitis and Sinusitis
Clinical Features
Infective orbital cellulitis from sinusitis is extremely important to recognize as the juxtaposition
to intracranial structures may lead to rapid and serious consequences. Pathophysiologically, the
infection originates from the sinuses and can spread readily to the orbit through the thin bony
walls and foramina, or in a retrograde fashion by the interconnecting valveless venous system of
the orbit and sinuses. The process frequently develops through a sequence of edema and cellulitis
to local and contiguous pyemic destruction of tissue planes with subperiosteal, orbital, and
intracranial abscess formation and thrombophlebitis (Table 12-3). From a clinical point of view,
cellulitis is generally associated with axial displacement of the globe, whereas
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formation of an abscess, particularly in the subperiosteal space, usually causes nonaxial displacement
and may ultimately track forward causing subcutaneous induration or fistulization (Figs. 12-18,12-19,12-
20). Posterior subperiosteal tracking may lead to rapid and catastrophic visual loss and neurosensory
compromise due to apical compression (Fig. 12-21). Direct contiguity may lead to inflammatory optic
nerve damage (Fig. 12-22).
Table 12-3. Cellulitis and sinusitis
CELLULITIS ABSCESS CAVERNOUS SINUS
Clinical Features
Ocular and Group 1: Group 3: Subperiosteal Group 5: Cavernous sinus
orbital Inflammatory abscess thrombosis
edema +/- Induration and Bilaterality
Lid edema fluctuation Increased chemosis
Group 2: Orbital Increased lid swelling Increased orbital tension
cellulitis Chemosis Increased intraocular
Increasing lid +/- Nonaxial displacement pressure
swelling Increased intraocular Cranial nerve palsies (III,
Injection pressure IV, V, VI)
Chemosis Increased orbital tension Decreased sensation
Axial proptosis Decreased extraocular Decreased extraocular
Venous movement movement
congestion (choroid Local tenderness Decreased movements out
& retina) Motor and sensory signs out of proportion to cellulitis
Increasing pain of proportion to inflammation Dusky colored lids
+/- Increasing May be sudden Increased venous
intraocular pressure Group 4: Orbital abscess engorgement
Increased proptosis Papilledema
Increased inflammatory
 signs
Ophthalmoplegia
Decreased vision;
papilledema
Palpable fluctuant mass
+/- Perivasculitis
General +/- Malaise Increasing malaise and fever Headache
+/- Spiking Varying consciousness
Nausea
Vomiting
Fever
Imaging
CT Groups 1 & 2 Group 3 Group 5
Swelling of lid Homogeneous subperiosteal Increased size of superior
Sinus accumulation of pus with orbital vein (bilateral)
opacification smooth border on orbital side Increased extraocular
Mucosal enhancing capsule muscle size
thickening +/- Gas Expanded cavernous sinus
+/- Obscuration Group 4 +/- Cerebral infarct
and infiltration of Homogeneous or +/- Abscess in central
orbitat fat heterogenous mass nervous system -
+/- Contrast enhancing subdural/intracerebral
capsule
+/- Gas in orbit
Ultrasonography Clear spaces Irregular, poorly defined Increased orbital vein size
Change in fat lesion of medium or Increased extraocular
densities reflectivity muscle
Radiography Sinus opacity Sinus opacificatio Sinus opacity
+/ Air fluid level +/- Air fluid level +/- Air fluid level
Permanent visual loss can result from profound increased intraorbital tension associated with abscess
formation or from direct optic neuritis or vasculitis. The most devastating complication is spread by
means of vascular emissaries to the cavernous sinus, leading to cavernous sinus thrombosis (Fig. 12-23).
Alternatively, spread through diploic vessels to the intracranial cavity can lead to subdural empyema or
intracranial abscesses.

Figure 12-18. (A) This 10-year-old patient has the clinical features of group 1 orbital cellulitis or
inflammatory edema (periorbital cellulitis) with mild edema (especially the upper lid) due to
ethmoid sinusitis. (B) This 22-year-old woman presented with group 2 orbital cellulitis
secondary to ethmoid sinusitis following an upper respiratory illness. She had 4 mm of proptosis,
lateral displacement of the globe, pain, and tenderness associated with a slight decrease in vision.
Both responded to intravenous antibiotic treatment.
Figure 12-19. Group 3 subperiosteal abscess. (A) This 10-year-old child presented with a 2-day
history of sudden onset of right ptosis, lid injection, decreased vision (20/70), marked limitation
of ocular movements, malaise, and anorexia following 7 days of lid swelling. He had mild
papilledema and a tense orbit due to a medial subperiosteal abscess arising from ethmoid
sinusitis, demonstrated on axial CT scans. Note slight tenting of the globe and bowing of the
optic nerve (B) and medial rectus (C). He was treated with urgent drainage and systemic
antibiotics, and recovered uneventfully.
Figure 12-20. Group 4 orbital abscess is seen in axial CT scans (A, B) of an abscess arising from
the frontoethmoid complex (different window settings). The patient, a 70-year-old man, had a
history of allergic rhinitis and recurrent papillomata removed from the sinus. This episode was
preceded by a 3-week history of right supraorbital pain, inflammation, and swelling with
intermittent drainage of purulent material “from the corner of the eye.” He had developed a tense
orbit with diplopia, ptosis, decreased vision (20/50), reduced extraocular movements, and
downward displacement of the globe (5 mm). He was treated by drainage of the orbital abscess
and sinuses, and with intravenous antibiotics.
Figure 12-21. Coronal (A) and axial (B, C) CT scans of a 61-year-old woman who presented
with an explosive onset of severe proptosis, downward displacement, and vision loss due to a
right frontal sinus abscess. There had been an antecedent history of progressive frontal pain for 2
weeks. The abscess penetrated into the right superior subperiosteal space, leading to massive and
sudden proptosis. The marked posterior tenting of the globe is a result of severe orbital pressure
and forward displacement from the tense abscess. In spite of rapid drainage, visual loss was
permanent.
Figure 12-22. Axial CT scans of an 80-year-old patient with right orbital cellulitis secondary to
chronic and recurrent ethmoid sinusitis. Note lid edema, apical infiltration of the orbit (B), and
dilatation of the right superior ophthalmic vein (arrow). He had suffered profound visual
deterioration due to the apical cellulitis.
Figure 12-23. Group 5 cavernous sinus thrombosis. (A) Axial CT scan of a patient (B) who
presented clinically with catastrophic onset of bilateral proptosis, chemosis, almost total
limitation of ocular movement, ptosis, lid edema, profound decrease in vision, and varying levels
of consciousness. Note opacification of sphenoid sinus, engorgement of the cavernous sinus, and
proptosis. This was preceded by a 2-week history of retrobulbar and frontal pain. The patient
responded to systemic antibiotics, sinus drainage, and symptomatic therapy, but he had suffered a
right cerebral infarct.
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In general, progress is characterized by evidence of increasing malaise, fever, lid injection,
proptosis, chemosis, pain, orbital tension, motor dysfunction, raised intraocular pressure, and
congestion of the veins of the choroid and retina (with papilloedema and periphlebitis).
Cavernous sinus thrombosis is associated with the development of headache, nausea, vomiting,
fever, varying levels of consciousness, increased chemosis, bilaterality, nerve palsies, decreased
extraocular movements, and development of a blue-purple lid. The ophthalmological danger
signals of progression are decreased vision, reduction of extraocular movements (especially if
out of proportion to the degree of cellulitis), dilated pupil or afferent pupillary defect, engorged
fundus vessels, papilledema, perivasculitis, evidence of spread to the other orbit, a violaceous lid,
increasing proptosis, raised intraocular pressure, and decreased sensation (hypesthesia).
The ocular complications of orbital cellulitis are exposure and neurotropic keratitis, conjunctival
prolapse, secondary glaucoma, septic uveitis and retinitis, exudative retinal detachment, optic
neuropathy, and panophthalmitis.
Figure 12-24. This 9-year-old boy presented with progressive left upper lid swelling and
injection, decreased vision (20/60), limited upgaze, and chemosis. (A) Axial CT scans show a
subperiosteal abscess with bilateral maxillary and left ethmoid sinusitis. Note air in the abscess
(should raise suspicion of gas-producing organisms), which is displacing the medial rectus and
globe laterally, and the soft tissue swelling over the cheek (B). He was treated successfully with
intravenous antibiotics, external ethmoidectomy with drainage, and antral lavage.
Classification
The clinical classification of orbital cellulitis includes five groups originally described by
Chandler et al (Table 12-3).
 Group 1: Inflammatory edema (preseptal cellulitis, periorbital cellulitis) is characterized
by swelling of the eyelids with mild orbital edema, usually involving the upper eyelid
(especially medially) in the initial stages. It reflects slowing and congestion of venous
outflow. A slightly more advanced stage includes chemosis (Fig. 12-18A).
 Group 2: Orbital cellulitis. The orbit is infiltrated, leading to variable mass effect and
functional defects (edema, congestion, proptosis, motor and visual impairment). This can
be a feature of either bacterial or sterile cellulitis (Fig. 12-18B).
  Group 3: Subperiosteal abscess (Figs. 12-19 and 12-24). Purulent foci within the adjacent
subperiosteal space may lead to nonaxial displacement, local tenderness, and possible
fluctuant masses depending on size and location. The volume of the abscess and speed of
development may reflect virulence of the pathogen or the unique pathophysiology

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of the subperiosteal space. Abscess formation may be rapid, because destruction of soft
tissue planes is not a requisite to accumulation of purulent material in this potential space.
In fact, a clinical clue to the development of subperiosteal abscess may be profound
proptosis with a functional deficit in the absence of striking concomitant inflammatory
signs, such as chemosis and lid injection. Displacement of the globe is frequently
nonaxial and reflects the site of the abscess.

 Group 4: Orbital abscess (Fig. 12-20). Progression of intraorbital cellulitis or spread from
the subperiosteal space leads to intraconal or extraconal loculation. Proptosis,
inflammatory signs, ophthalmoplegia, visual deficit, and systemic toxicity are frequently
severe at this stage.
 Group 5: Cavernous sinus thrombosis is heralded by profound central nervous system deficits or
changes in local inflammatory signs with functional impairment (previously outlined).

Table 12-4. Orbital cellulitis and sinusitis

CHILD ADULT
Signs and Lid edema, diplopia, decreased Lid edema, diplopia, decreased vision,
Symptoms vision, proptosis proptosis, more frequent nonaxial displacement
General More frequent malaise, fever, Less malaise, fever, and anorexia
anorexia
Location Ethmoid or pansinusitis Frontoethmoid
History Upper respiratory infection Allergy, sinus problem, dental extraction
Bacteriology May be no growth, gram- Frequent growth, gram-positive, gram-
positive, gram-negative, H. negative, S. aureus, mixed anaerobes
influenzae, S. Aureus
Management Systemic antibiotics; drainage Surgical drainage frequently necessary, plus
rarely necessary antibiotics
Outcome Recovery with few complicationsMore frequent complications including visual
loss, central nervous system abscess, recurrent
cellulitis, osteomyelitis
Although this classification suggests an orderly progression, sudden and catastrophic events may
occur with virulent pathogens or as a result of acute pressure blowout from a sinus into
subperiosteal or orbital spaces (Fig. 12-21). In addition, spread of infection to the cavernous
sinus or orbital apex from contiguous disease (especially sphenoid sinusitis) may cause rapid,
early, profound deterioration. Another important and frequent cause of variation in this pattern is
incomplete or inappropriate treatment. Current imaging technology allows for earlier recognition
of orbital and periorbital involvement and is useful for assessing severity and guiding treatment.
Diagnosis
The clinical diagnosis of orbital cellulitis is best substantiated by imaging to identify abscess
formation and to get accurate staging and localization of lesions. CT or MR imaging will
demonstrate precise location and extent of the inflammatory process, and can be used to follow
improvement or worsening of the disease. The sinus pathology is reflected in mucosal wall
thickening, opacification, and air fluid levels. Chronic infection of the sinuses may be associated
with thickening of the walls, and a persistent inflammatory process (particularly in children) may
be due to a foreign body in the nasal passages. Depending upon the major location of the
inflammatory process, the disorder can be subclassified into subperiosteal, extraconal, and
intraconal abscess formation.
Subperiosteal infection most commonly occurs adjacent to the ethmoid sinuses but may
accumulate superiorly along with frontal sinusitis, more readily viewed on coronal scans (Figs.
12-19, 12-23, 12-24). The fluid or pus collecting in the subperiosteal space appears as a
homogeneous or heterogeneous collection, which may be surrounded by an enhancing border.
With progression, it will displace the extraconal fat and extraocular muscles. When the process is
immediately retrobulbar or in the intraconal space, the edema leads to an increase in fat density.
If the intraconal space is predominantly involved without sinus disease, a foreign body or an
immunocompromised state should be suspected. The adjacent soft tissue planes are obscured by
the inflammation when diffuse, and an abscess can be identified as a poorly defined mass with
contrast enhancement of the rim. Gas producing anaerobes may lead to abscesses with lucent
spaces within them. However, in an earlier stage before cavitation, there may be diffuse
enhancement. If the globe is involved, the scleral uveal rim may be thickened.
Epidemiology
The most important factors to consider are epidemiologic based on regional pathogens but more
particularly on differences between the disease in adults and children (Table 12-4). Because of
the delay in development of the sinuses in children, the locus of the disease is ethmoidal and the
major predisposing cause of sinusitis in childhood is intercurrent upper respiratory disease.
Children under the age of 4 may be prone to infection with Hemophilus influenzae, an
occurrence that has markedly reduced with the introduction of HI vaccines leading to fewer cases
and a wider spectrum of causative bacterial agents. Overall, sinusitis and, for that matter,
subperiosteal abscesses in childhood (under age 9) tend to be due to single aerobic organisms
such as Streptococcus pneumoniae, moraxella catarrhalis, and Hemophilus influenzae.
Subperiosteal abscess formation is less frequent in children and even when the features exist,
conservative therapy with antibiotics is suggested as long as there is no threat to vision.
In contrast, adults typically develop frontoethmoid disease with predisposing histories of
sinusitis, polyps, allergy, trauma, and recent dental extraction. The infection in adults is typically
polymicrobial and frequently contains anaerobic organisms. Nonaxial displacement is more
common in adults, reflecting abscess formation. Direct nasopharyngeal, and especially sinus
aspiration or abscess cultures, are often positive and relevant. Most patients have significant
orbital signs associated with sinusitis and orbital cellulitis, except for those with sphenoid
involvement where there may be a disproportionate visual or motor sensory loss because of
apical location. Other epidemiologic factors of note are emerging species of resistant bacteria
such as methicillin-resistant Staphyloccus aureus and penicillin-resistant pneumonococci.
Management
The management and outcome of sinusitis and orbital cellulitis differ in adults and children. The
overwhelming majority of children do not need sinus or abscess drainage.
Table 12-5. Initial empiric antimicrobial regimens for orbital cellulitis*
CLINICAL PREDOMINANT RECOMMENDED REGIMENS
TYPE ORGANISM
Preseptal Group A streptococci Child: amoxicillin/clavulanate (po) or
(periorbital) S. aureus ampicillin/sulbactam (IV), or if H. influenzae as in
H. influenzae** adults
Adult: cloxacillin (IV, high dose), or cefazolin, or
clindamycin
Orbital Streptococci Second or third generation cephalosporin (e.g.,
(sinusitis) Strep. pneumoniae cefuroxime, cefotaxime, ceftriaxone), or
H. influenzae piperacillin/taxobactam, or ticarcillin/clavulanate, or
M. Catarrhalis imipenem
Bacteroides
Fusobacterium
Anaerobic cocci
S. aureus (unusual)
Trauma or S. aureus Vancomycin + gram negative coverage***
foreign-body S. epidermidis
related Streptococci
Coliforms
Anaerobes (if soil
contamination)
* Therapy should be revised according to the results of microbiologic testing, particularly of
specimens from surgical drainage of abscesses.
** Much less common since introduction of HI vaccines.
*** Numerous options, for example, ciprofloxacin, ceftazidime, ceftazidime, ceftriaxone,
piperacillin or piperacillin/tazobactam, imipenem.

Figure 12-25. A 21-year-old patient with a severe necrotizing preseptal cellulitis 4 days after a
minor lid laceration. He responded to systemic and local antibiotics with minimal scarring and
no orbital involvement.
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In contrast, adults more frequently require such, particularly if there is orbital tension and threat
to vision. With adults, it is important to use adequate intravenous antibiotic therapy directed at
polymicrobial or cultured-specific infection for a significant period of time in order to be assured
of clearance of disease and to avoid complications of recurrence, intracranial extension, and
osteomyelitis. The advent of endoscopic sinus surgery allows for minimally invasive drainage of
subperiosteal abscesses. Imaging with CT or MR is helpful in predicting the need for, and site of,
drainage of abscesses, and can accurately monitor the progress and effect of treatment.
Antimicrobial therapy is best directed against a cultured organism and should involve the
expertise of an infectious disease consultant. As a guideline, Table 12-5 suggests empiric
antimicrobial regimens for orbital cellulitis. It should be noted that in cases without a threat to
vision or function, particularly in younger age groups, conservative intravenous antibiotic
therapy may be associated with an increase in the size of the subperiosteal abscess in the first
few days after the initiation of therapy. Drainage of an abscess should be emergent if there is a
threat to the optic nerve, retinal function, or if there is a profoundly tense orbit in patients who
have significant pain. In those with intracranial complications of frontal sinusitis, the abscess
should be drained.
The role of the ophthalmologist is to establish the diagnosis and monitor ocular function during
therapy. It is especially important to recognize increasing orbital tension and threat to ocular
function, thus observation and monitoring of visual acuity, degree of proptosis, central nervous
system function, horizontal and vertical displacement, extraocular movements, pupillary signs,
and fundus examination are extremely important. The main principles of management consist of
the prevention of ocular and nonocular complications, the use of appropriate antibiotics, surgical
drainage when necessary (i.e., evidence of significant abscess with globe tenting, deterioration of
proptosis, vision, or extraocular movements), symptomatic therapy, and careful follow-up.
Other Sources of Microbial Orbital Cellulitis
Contiguous Spread
The majority of infective cellulitides that spread from contiguous structures are preseptal, with
involvement of the deeper orbital structures being rare (Fig. 12-25). In some instances, there is
antecedent local trauma or infection, such as in dacryocystitis. Usually there is a profound degree of
swelling due to the loose subcutaneous tissues. Untreated or severe infections may rarely extend into
the deeper orbit (Fig. 12-26) but in most instances, subcutaneous disease can be treated with
intravenous antibiotics.

Figure 12-26. (A) CT findings of orbital cellulitis resulting from an infected wound of the lid in a
34-year-old man. (B) Note tenting of the globe due to severe orbital tension. In spite of drainage
and systemic antibiotics, he remained blind due to retinitis and infarction. (C) Fundus
photograph 3 months after cellulitis.
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A large variety of organisms are associated with contiguous spread, including Staphylococcus
aureus and Streptococcus pyogenes. If foul-smelling, anaerobic infections should be considered
especially if the wound has been contaminated by soil and bites. Strep. pyogenes may be
associated with erysipelas, necrotizing fasciitis, or toxic shock, requiring very aggressive
treatment.
Management includes careful bacterial isolation, systemic and local antibiotics, and surgical
drainage of abscesses. Direct culture and scraping of wounds will usually yield an organism but
in the absence of drainage, careful limited aspiration of the subcuticular tissues can aid in
isolation of bacteria.
Drug treatment depends on severity of disease, and intravenous treatment is recommended when
significant involvement is noted or an aggressive onset has occurred. If the disease is localized
and the patient systemically well, oral antibiotics may be sufficient.
Infection of the skin and subcutaneous tissues, especially of the face, are common in children
and warrant separate discussion. The immunologic and local predispositions are different in
children and produce several special syndromes, including impetigo, Hemophilus influenzae
cellulitis and conjunctivitis, and maxillary osteomyelitis. Impetigo is a superficial mixed
infection with S. aureus and group A Strep. pyogenes. Characteristically, impetigo involves the
head and neck, starting as a reddish macule and progressing to vesicular eruptions that ultimately
rupture and drain, producing yellow ocher crusts. When the lids are affected, profound injection
and edema may occur. Treatment is with local hygiene, antibiotic ointment, and systemically
administered antibiotic appropriate for penicillin-resistant organisms.
Children younger than 3 years of age are prone to a distinctive infection with H. influenzae. It is
usually associated with an upper respiratory infection, systemic malaise, and fever. Involvement
of the conjunctiva produces a mucopurulent discharge, and the lid is characteristically
violaceous. The organism may also produce sinusitis and orbital cellulitis, as noted previously.
Blood cultures are especially useful in this circumstance because bacteremia is common.
Profound and rapid progression may occur, and intravenous systemic antibiotics are mandatory.
Because H. influenzae organisms are frequently penicillin resistant, the drug of choice is
ampicillin, or chloramphenicol if the organisms are ampicillin resistant. Chloramphenicol drops
should be applied locally for the conjunctivitis. With the advent of the H. influenzae vaccination,
this disorder is less frequent in areas of use.
A rare fulminant osteomyelitis of the superior maxilla may occur in children younger than the
age of 9 months. The infection is due to S. aureus and associated with profound systemic
symptoms and rapid progression. Because of rapid spread and potential intracranial, orbital, and
systemic involvement, high-dose antimicrobial intravenous therapy is indicated.
Other Contiguous Infections
Conjunctivitis rarely spreads to involve the deeper orbital tissues and has a characteristic history.
We have encountered several instances of orbital cellulitis progressing from severe
conjunctivitis. A common preseptal cellulitis that may masquerade as bacterial is herpes zoster,
particularly noted in an older age group.
Orbital Foreign Bodies
Orbital foreign bodies are another source of infection, the most common in our experience being
eroding orbital implants. Foreign bodies tend to induce a rapidly developing cellulitis or produce
a localized abscess that may progress to fistulization (Fig. 12-27). Vegetal foreign bodies may
also induce a chronic granulomatous response with fistulization
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(Fig. 12-28). Treatment implies mandatory removal of the offending foreign body, local irrigation, and
systemic antibiotics.

Figure 12-27. Axial and coronal CT scans show an orbital abscess secondary to a foreign body.
This was seen in a 1.5-year-old girl who had a small stab wound of the upper lid 10 days earlier
after falling on a lead pencil. She presented with a 2-day history of swelling of the lid and
purulent drainage. There was no visible lid wound, but a small draining fistula was noted in the
superior fornix. The abscess was drained and it cultured mixed organisms (Streptococcus
viridans, Hemophilus influenzae, and anaerobic gram-negative rods).

Figure 12-28. (A) This 55-year-old man presented with a chronic draining sinus and low-grade
orbital cellulitis associated with trismus. He had fallen 3 months earlier into a thicket of
branches. On axial (B) and coronal (C) CT scans, the wooden foreign body that had penetrated
the left lateral orbital wall and temporalis fossa is identified as a linear density crossing the wall
at right angles. At surgery, the branch that was extracted from the temporalis fossa had extended
into the pterygopalatine fossa, accounting for the trismus.
Pyemic Cellulitis
Pyemic orbital cellulitis is rare but may occur especially in compromised hosts. In these
instances, local and systemic bacteriologic evaluation is important as unusual organisms may be
involved. Predisposition includes immunocompromise, old age, poor nutrition, and chronic
alcoholism.
Intraorbital Sources of Cellulitis
Dacryoadenitis, panophthalmitis (Fig. 12-29), and dacryocystitis are additional sources of orbital
infection. The microbial infections in these instances may spread beyond the site of origin into the orbit.

Figure 12-29. Axial CT scans demonstrate the features of severe endophthalmitis and orbital
cellulitis following a posterior chamber lens implant. Note marked infiltration of the orbital fat
and thickening of the uveoscleral envelope.
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Fungus Infections
Rhino-orbital Mucormycosis
Rhino-orbital mucormycosis is an aggressive opportunistic infection that occurs in debilitated
patients, particularly individuals with uncontrolled diabetes with ketoacidosis,
immunocompromise, and renal disease. Healthy persons are rarely affected (due to normal
containment by phagocytes) and if so, usually have localized disease. The orbit is a site in about
10% of patients with hematologic malignancies who have proven mucormycosis, a context in
which survival is uncommon.
The sinus and nasopharynx are inoculated by spores (which are ubiquitous in soil, air, skin, body
orifices, manure, and food), with growth and spread of the organism into the tissue being
associated with invasion by hyphae. This organism has a propensity to invade and occlude
vascular lumina leading to infarction, which compounds inflammatory necrosis and forms a
characteristic black eschar. Spread to adjacent tissues of the orbit and intracranial cavity may be
rapid and devastating, leading to a fatal outcome.
Early diagnosis can allow for containment and successful therapy; thus it is important to
recognize the characteristic pattern in a predisposed patient (Fig. 12-30). The earliest orbital sign
consists of apical boring pain, and progression is associated with increased cellulitis, proptosis,
abrupt visual failure, and apical neuropathies. The presence of a characteristic eschar of skin,
palate, or nasal mucosa in the early stages is rare. Imaging will show displacement of orbital
structures adjacent to the opacified sinus, with and without bony destruction. There may be
increased density of soft tissue and enlargement of the optic nerve.
Treatment requires a well-coordinated and prompt multidisciplinary approach. An early
definitive diagnosis is associated with a more favorable outcome. Tissues should be obtained for
microscopic examination, fungal culture, and histopathology. Histologically, these large
nonseptate branching hyphae can readily be seen with routine staining methods (hematoxylin &
eosin) and can be dramatically demonstrated with appropriate special stains. The underlying
metabolic disorder should be corrected and a failure to respond metabolically may be a clue to
the diagnosis. Wide local excision of the involved tissues with frozen section guidance if
possible and adequate postoperative surgical drainage are also required.
If caught sufficiently early in the localized form, treatment need not necessarily imply exenteration.
Systemic antifungals along with local irrigation of tissues with such agents is recommended. The extent
of surgical excision should be balanced against the threat to life and vision, and the expected deformity.
Some have suggested that hyperbaric oxygenation may play a role in treating this disorder.

Figure 12-30. Mucormycosis. (A) Axial CT scan and (B) biopsy specimens from 43-year-old
patient with a relapsed leukemia post-bone marrow transplant. The orbital tissues demonstrated
mucormycosis with intravascular invasion (arrow) (H&E, original magnification × 10). The
patient died of intracranial extension of the mucormycosis.
Aspergillosis
Aspergillus is a normally harmless saprophyte that is opportunistic and difficult to culture. This
organism is better known to ophthalmologists as a cause of septic endophthalmitis, corneal
ulcers, and orbital invasion. There is an increasing frequency related to drug addiction, kidney
transplants, and immunosuppression. It is the only fungus aside from Rhizopus that stains with
hematoxylin-eosin in addition to the specific fungal stains. Aspergillus, however, is septate
whereas Rhizopus is not.
There are two clinical circumstances in which aspergillosis presents. The first is a disseminated
form (often occurring in immunocompromised hosts) that causes a widespread necrotizing
angiitis due to microscopic foci of fungus in small vessels. Endophthalmitis is a common sequel
in this form. The second form of presentation in the orbit is the development of a relatively slow,
localized infiltrative mass,
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usually originating from an adjacent sinus. When anterior, it primarily leads to proptosis and
displacement of the globe, and when apical, causes a painful orbital apex syndrome (Fig. 12-2).
This can be an infection in healthy people but is more common in predisposed individuals with
recurrent sinusitis and polyps. The usual infiltrate is granulomatous but focal abscess formation
and fistulas may occur. It is frequently missed and can lead to devastating local spread and even
death.
Disseminated aspergillosis is seldom treatable. Localized disease when recognized early is
probably best treated by surgical drainage and debridement along with systemic and local
antifungals. Early diagnosis may be aided by the use of aspiration cytology.
We have recently experienced four cases of apical presentation of aspergillosis with minimal sinus
involvement. All four cases had a 4- to 6-month history of progressive apical retrobulbar pain associated
with deteriorating vision and progressive apical neuropathies. On imaging, they had minimal changes in
the sphenoid sinus lining associated with a focal, tiny gap in the sinus wall and infiltration of the apical
orbit, which sometimes extended into the cavernous sinus (Fig. 12-31). The apical infiltration was
characterized by the presence of fine, focal, low-density areas that turned out to be microabscesses. In
spite of extensive debridement, local irrigation with antifungals, and systemic therapy, two of the
patients went on to die of intracranial involvement and the remaining two have persistent disease.
Figure 12-31. This 78-year-old man presented with a 5-month history of right frontal parietal
pain and a recent (2 to 3 weeks) decrease in vision that progressed over a 1-week period from
20/25 to 20/40. This was associated with an afferent pupillary defect and color vision defect.
Axial CT scan shows erosion of the lateral wall of the sphenoid sinus with a central low density
area, which was due to a localized Aspergillus abscess arising from the sinus (arrow).
Figure 12-32. Immunocompromised patient. (A) Clinical photo of a 37-year-old woman with
cytopenia subsequent to consolidation therapy for acute myelogenous leukemia. She had
intermittent fever, left ptosis, and left intraorbital numbness with upward displacement of the
globe and premalar injection. (B) CT scan demonstrated maxillary and inferior orbital
infiltration. The sinus was explored, its contents removed, and the inferior orbital fat excised. (C)
The biopsy and culture demonstrated an infection with pseudallescheria boydii (Grocott, original
magnification × 10). (D) An irrigating system was left in the sinus and she was treated with
antifungals by irrigation and systemically. As her neutropenia recovered, local and systemic
symptoms disappeared.
P.478
Other Mycotic Infections
There are a number of other fungal organisms that may rarely affect the orbit (Fig. 12-32).
Treatment depends on specific identification of the organism and local and systemic fungal
therapy. The range of organisms seen include North American blastomycosis, African
histoplasmosis (H. duboisii), sporotrichosis, rhinosporidiosis, coccidioidomycosis, candidiasis,
and bipolaris hawaiiensis. Allergic fungal sinusitis may occur with orbital symptoms but is not
invasive and may involve a variety of fungal organisms. It should be recognized so that it may be
treated by sinus debridement and steroids (Fig. 12-33).
Tuberculosis and Syphilis
Worldwide, tuberculosis is a persistent and significantly increasing infection. Orbital
involvement occurs in two circumstances: either secondary to hematogenous spread or by direct
extension from contiguous structures, usually the sinuses. Hematogenous dissemination of the
disease can lead to two orbital manifestations. The first, and more common, is periostitis, which
characteristically affects the malar bones of people in their first and second decades. Periostitis
presents as an insidious, localized inflammatory lesion that leads to cold abscess, sequestration,
and fistula formation. The orbital tuberculoma is another manifestation of hematologic spread
and is associated with the development of an infiltrative orbital mass, which may cause
neurosensory deficit. In both instances, concomitant evidence of active tuberculosis should be
sought but may not necessarily be present. Diagnosis may be aided by fine needle aspiration
biopsy.
Another source of orbital tuberculosis is from direct spread, either from intraorbital structures or
more commonly from adjacent sinuses (Fig. 12-34). The sinus spread causes necrotizing
infiltrative lesions that may develop cutaneous fistulas. In all of the above circumstances, growth
of acid-fast bacilli from direct biopsy material is rare. Evidence of such a lesion in the presence
of a strongly positive tuberculin test may warrant treatment on a presumptive basis while
awaiting culture. Recommended therapy is systemic antituberculous drugs.
Orbital involvement with syphilis is rare but the increase in patients with immunosuppressive syndromes
has led to more infections. Syphilitic periostitis may cause diffuse or focal involvement of the orbital
bones, which appear as either acute or chronic inflammatory disease. Fistulization, bone resorption, and
secondary spread into the orbit may occur. When posterior, manifestations of a painful apex syndrome is
characteristic. Primary soft tissue gumma may occur within the orbit, extraocular muscles, and lacrimal
gland. Appropriate systemic antibiotic therapy should lead to resolution of disease.
Figure 12-33. Allergic fungal sinusitis. (A) Axial CT scan of the orbits shows a soft tissue mass
filling the ethmoid and sphenoid sinuses with extension into the anterior medial left orbit. (B)
Axial T2-weighted scan shows increased signal intensity of the ethmoid (arrow) and sphenoid
sinus mucosa and a marked decrease in signal intensity within the left ethmoid sinus and anterior
sphenoid sinus. The organism was isolated and found to be bipolaris spicifera. (Reproduced with
permission from Klapper SR, Lee AG, Patrinely JR, et al. Orbital involvement in allergic fungal
sinusitis. Ophthalmology 1997;104:2094-100.)