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Show I" umal "/ Ollll, al N~ II"'-" l'hthallll"/( lgy 12( 41: 2511- 256. 1992. Bipolaris- Induced Orbital Cellulitis Mercedes Jacobson, M. D., Steven L. Galetta, M, D., Scott W. Atlas, M. D., Mark T. Curtis, M. D., Ph. D., and Allan W. Wulc, M. D. © 1992 Raven Press, Ltd .. New York We report a patient who rapidly developed unilateral proptosis and complete ophthalmoplegia following blunt trauma to the orbit. Computed tomography ( CT) scan revealed a mass involving the sinuses and orbit with erosion of the cribriform plate. Biopsy of this suspected neoplasm revealed features consistent with allergic Aspergillus sinusitis, but cultures later grew a Bipolaris species. Our review of the literature suggests that when allergic fungal sinusitis involves the orbit, Bipolaris is a more commonly isolated organism than Aspergillus. Additionally, extraocular mOtility impairment and proptosis occur more frequently than visual loss. Treatment is controversial, but surgical drainage followed by corticosteroids is the most often recommended therapy. Key Words: Bipolaris- Allergic sinusitis- Ophthalmoparesis- Orbital cellulitis. From the Departments of Neurology ( M. J., S. L. G.), Ophthalmology ( S. L. G., A. E. W.), Radiology ( S. W. A.), and Pathology ( M. T. C.), UmveTSIty of Pennsylvama School of Medicine, Philadelphia, Pennsylvania. Address correspondence and reprint requests to Dr. Steven L. Galetta, Department of Neurology, Hospital of the UniverSIty of Pennsylvama, 3400 Spruce Street, Philadelphia PA 19104, USA. ' 250 Allergic fungal sinusitis is a disorder characterized by recurrent pansinusitis, elevated serum IgE, cutaneous hypersensitivity, and histologic features, suggesting an allergic reaction ( 1,2). Pathologic features are similar to those of allergic bronchopulmonary aspergillosis; however, cultures for the Aspergillus organism are often negative ( 1,2). Only recently has the family of dermatiaceous fungi been recognized as the etiology for most cases of allergic fungal sinusitis ( 3). This family includes the fungal saprophytes, Bipolaris, Curvularia lunata, and Alternaria ( 4). In the series of Manning et al. ( 3), Bipolaris was the most commonly isolated organism. Allergic fungal sinusitis is usually seen in young, immunocompetent patients with a history of asthma, atopy, or nasal polyposis ( 1). Many patients have been participants in contact sports or avid swimmers ( 5- 7). We report a patient with Bipolaris- associated " allergic" orbital cellulitis, whose symptoms evolved precipitously following blunt trauma. CASE HISTORY A 26- year- old male with a 3- month history of bifrontal headache and rhinorrhea was struck in the face with a basketball. He presented to a community hospital emergency room with a swollen left eyelid, and an ice pack was prescribed. Over the next 48 hours he developed progressive proptosis, chemosis, and complete ophthalmoparesis of the left eye. He was referred to the Scheie Eye Institute on August 29, 1990, where a presumptive diagnosis of orbital cellulitis was made. However, computed tomography ( CT) of the brain and orbits revealed a soft tissue mass, involving the superior aspect of the orbit, with erosion of the cribriform plate and extension to the surface of the left infer~ or frontal lobe ~ Fig. ~): Opa~ ification of multiple smuses was also Identified, WIth destruction of the BlPOLARIS- INDUCED ORBITAL CELLULITIS 251 A FIG. 1. Axial computed tompgraphy scans after intravenous contrast. ( A) Note left nasal cavity hyperdense soft tissue mass with opacification of left posterior maxillary sinus and sphenoid sinuses. ( B) More superiorly, intraorbital retrobulbar soft tissue mass deforms posterior aspect of proptotic globe. Enhancing subfrontal soft tissue ( arrows) is seen along cribriform plate, indicating intracranial spread. B lateral wall of the left frontal sinus and the medial wall of the left maxillary sinus. Intravenous ceftriaxone and metronidazole therapy were begun, and the patient was transferred that day to the Hospital of the University of Pennsylvania. The patient was glucose 6- phosphate dehydrogenase ( G6PD)- deficient and had a history of childhood asthma. He was a frequent intranasal cocaine abuser and consumed six beers daily. He denied intravenous drug abuse and homosexual contacts and was otherwise in good health. On physical examination the patient was afebrile. The neck was supple. Visual acuity was 20/ 20 in both eyes, with normal visual fields. The right eye examination was normal. There was marked proptosis ( 8 mm) of the left eye with 3+ chemosis and complete ophthalmoplegia of the left eye ( Fig. 2). The pupils were equal and briskly reactive to light without afferent pupillary defect. Funduscopic examination was normal. The remainder of the examination was normal. Laboratory studies revealed the hemoglobin concentration was 14.0 g/ dl; the white blood cell count was 8.2 Klmm3 , with a differential of 81 neutrophils, 15 lymphocytes, and 3 monocytes. Absolute eosinophil count was not obtained. Platelet count was 330,000. Serum IgE was elevated: 793 IV/ ml ( normal less than 180 IU/ ml). Electrolytes were normal: serum glucose was 94 mgldl. HIV antibody titer was negative. A chest roentgenogram was normal. The patient's ophthalmoplegia and chemosis failed to respond to antibiotics and on August 30, 1990, a biopsy of the nasal mass and intranasal subtotal ethmoidectomy was performed. The frozen sections showed inflamed sinus mucosa with dense mucus and eosinophils. The preliminary diagnosis was allergic Aspergillus sinusitis. Gram stain was negative. A postoperative magnetic resonance imaging ( MRI) scan revealed a persistent left superior orbital mass ( Fig. 3). In addition to the erosion through the cribriform plate, there was extension of the inflammatory process from the ethmoid sinuses through the skull base to adjacent cerebral tissue. Abnormal enhancement of the meninges along the left inferior frontal lobe was seen. The following day, a Caldwell- Luc procedure, left external ethmoidectomy, left frontal sinus trephination, sphenoid sinusotomy, nasoantral window, and drainage of the orbital abscess were performed. Severe chronic sinusitis was discovered, which, on pathologic examination, showed necrotic debris, dense mucus, and eosinophilic infiltration of the submucosa ( Fig. 4). Bony erosion was evident. Grocott ( methamine- silver) stain of the sinus tissue identified hyphated fungal elements, with branching in the dense mucinous material and necrotic debris ( Fig. 5). On August 31, 1990, the patient was begun on amphotericin Bfor presumptive allergic Aspergillus sinusitis with intracranial extension, receiving a total of 1580 mg. Both ceftriaxone and metronidazole were administered for a 10- day course. Several days following the second surgery, the patient's I Cli" Neuro- ophthalmol. Vol. 12, No. 4, 1992 252 M. JACOBSON ET AL. B FIG. 2. ( A). External photograph showing chemosis and proptosis of left eye. ( B) 5 weeks after therapy. showing resolution of proptosis and ophthalmoplegia. proptosis improved and motility returned to normal ( Fig. 2). At 26 days following his initial biopsy, all fungal cultures revealed a species of Bipolaris. Bacterial cultures were negative. Over the next year, the patient had recurrent bouts of pansinusitis, requiring one surgical drainage procedure and several corticosteroid regimens. There were no further neuro- ophthalmologic sequelae. DISCUSSION Aspergillus is a common cause of fungal sinusitis and the nature of the illness that it produces is greatly dependent on host immunity. Invasive aspergillosis is a fulminating infection commonly ob- TClI/ 1 NClIro- ophlhalmol. Vol. 12, No. 4, 1992 served in patients with lymphoreticular malignancies and is rarely seen in the normal host ( 8). In contrast, noninvasive aspergillosis occurs when a colony of organisms form a fungal ball or mycetoma ( 9). Often the mycetoma is solitary and surgical resection may be the only intervention required. In 1983, Katzenstein ( 1) recognized another form of fungal sinusitis with a distinct histopathologic appearance, characterized by mucinous material containing eosinophils, CharcotLeyden crystals, and fungal hyphae. The fungal elements tended to be sparse without invasion of the mucosa or mycetoma formation. The pathologic features appeared similar to that of pulmonary allergic aspergillosis. BIPOLARIS- INDUCED ORBITAL CELLULITIS FIG. 3. Coronal T1- weighted magnetic resonance imaging ( MRI), pre- and postintravenous contrast. ( A) Precontrast MRI demonstrates high- intensity nasal cavity soft tissue, primarily representing postoperative hemorrhage ( 1). Intraorbital extension of the mass lesion is seen along the superior and medial extraconal space ( 2). Lesion is also seen in olfactory groove on left, obscuring olfactory tract ( arrow), impinging upon and elevating left gyrus rectus and olfactory gyrus. Note effacement of left olfactory sulcus ( compare to normal right side). ( B) Postcontrast MRI more clearly delineates enhancing intracranial tissue superior to left cribriform plate ( solid arrow), which compresses left olfactory gyrus and gyrus rectus. Also note extra- axial enhancement along left frontal region ( open arrow), representing meningeal involvement. B 253 Manning et a1. ( 3) have recently identified several organisms in the family of dermatiaceous fungi associated with presumed Aspergillus fungal sinusitis. In this series of 22 patients, Bipo/ aris was the most commonly isolated species and Aspergillus was recovered in only one culture ( 3). This report emphasized that the Aspergillus species may not be the most prevalent organism in allergic fungal sinusitis. Our patient had an outcome typical for the orbital disease associated with allergic fungal sinusitis; 13 similar cases have been described ( Table 1), all but 2 occurring in adolescents or young adults. This infectious/ inflammatory process was most often recognized when the patient had been previously treated for sinusitis or pOlyps. For several patients ( 10, II), the presence of a soft tissue mass, expanding sinuses, and eroding bone led to a differential diagnosis that included neoplasm. Proptosis, ptosis, and diplopia were the most com-mon presenting symptoms. When compared to the Hedges and Leung ( 12) review of 47 cases of parasellar or orbital aspergillosis ( this series included all types of Aspergillus infection), extraocular muscle palsy was more common in allergic fungal sinusitis, while optic nerve involvement and central nervous system invasion were rare. This might reflect the propensity of invasive nonallergic Aspergillus to involve blood vessels with early compromise of the ophthalmic artery and its branches ( 13). In allergic fungal sinusitis, the orbit may be affected by contiguous extension of the inflammatory process from the paraorbital sinuses ( 9). In our review of allergic fungal sinusitis, the ethmoid was the most frequent route of entry into the orbit ( Table 1). Several features of our patient's presentation remain unexplained. Blunt trauma to the periorbital area appeared to initiate an explosive onset of symptoms. Although no other organisms were , Clill Neuro- ophthalmol. Vol. 12, No. 4, 1992 254 M. JACOBSON ET AL. FIG. 4. Dense laminated " allergic mucin," containing eosinophils ( black arrow) from nasal mass. ( Hematoxylin and eosin; xaoo.) cultured, bacterial superinfection remains a possibility for this patient's fulminant presentation. The relationship of his cocaine abuse to his disease process is also unclear. Intranasal cocaine abuse is associated with osteolytic sinusitis, friable mucosa, and necrosis ( 14,15), none of which were identified in this patient. Other reported pathologic manifestations of chronic cocaine inhalation include optic neuropathy associated with osteolytic sinusitis or orbitopathy associated with a nonspecific chronic sinusitis ( 16). Goldberg et a1. ( 17) identified three cases of chronic sinusitis with nonspecific mucosal inflammation. Fungal cultures were negative in all three patients, although Staphylococcus aureus was isolated in one. It remains speculative as to whether this patient's cocaine use may have accel- FIG. 5. Branching fungal hyphae and necrotic debris. ( Grocott fungal stain; xaoo.) I Clill Neuro- ophtllalmol, · Vol. 12, No. 4, 1992 BIPOLARIS- INDUCED ORBITAL CELLULITIS TABLE 1. Neuro- ophthalmologic complications of allergic fungal sinusitis 255 Age ( yr)/ gender Species Signs & sx 46/ M Curvu/ aria Diplopia, H/ A, fever 14/ M Alterneria Diplopia, proptosis, epiphoria 21/ M Curvularia Diplopia, ptosis, R exo 111M Hyphal fungi Epiphora, medial canthal mass 13/ F Hyphal fungi Proptosis, blepharptosis 69/ F Aspergillus Proptosis, diplopia, RAPD, Va 6/ 24 26/ F Aspergillus Proptosis, " visual disturbance" 16/ M Bipolaris Va 20/ 400, central scotoma 171M Bipolaris Proptosis 321F Bipolaris Visual blurring w/ valsalva & rhinorrhea 221M Bipolaris Proptosis Source Recurrence? Residual Rx ( ref no.) No No Surgery, allergy Bartynski et at. immunotherapy ( 10) No No Surgery, Bartynski et at. intranasal ( 10) steroids Yes No Surgery & 10- day Brummond et at. ampho B ( 6) x3 No Surgery, allergy DeJuan et at. ( 16) immunotherapy No No Surgery DeJuan et at. ( 16) No Va 6/ 9, Surgery, steroids Dunlop & Billson VER abn'l ( 20) No n'opth. No Surgery, steroids Goldstein et at. ( 11) Yes Count fingers Surgery, ampho Jay et at. ( 7) B Yes No Surgery Jay et at. ( 7) Yes No Surgery, ampho Washburn et at. B, ( 9) ketoconazole Yes No Surgery, ampho Washburn et al. B, terbinafine ( 9) 16/ F 15/ M Curvularia Drechslera Proptosis, ptosis Proptosis, diplopia, periorbital edema Radiology Erosion medial wall L orbit w/ intraorbital mass Large vascular mass in ethmoid sinus, bowing into medial orbit Lamina papyracae bows into orbit & R medial rectus Thinned medial orbit, distended ethmoid sinus, erosion lacrimal fossa Expanded frontal sinuses w/ erosion media & sup orbit roof Orbital soft tissue density sparing optic nerve Facial pain, ethmoid dis. w/ bowing medial orbit Mult. sinus involv. inc'l ant. clinoid Ethmoid/ sphenoidal mass expanding to medial orbit Ethmoid/ sphenoid mass, thought to be compo optic nerve against sphenoid w/ valsalva Pansinusitis w/ erosion lamina papyracea, lat. displacement of globe erosion of clivus Pansinusitis w/ nasal No mass extending into L maxillary sinus & orbit Pansinusitis, x4 eventually developing L retro- orbital mass, involving optic nerve w/ mass in R medial orbit No No Surgery & ampho Berry et at. ( 5) B Surgery, steroids, Young et al. ( 21) ampho B VER, visual evoked response: ampho B, amphotericin B. erated the course of disease or augmented the inflammatory process. Based on the treatment of allergic bronchopulmonary aspergillosis, high- dose steroids for 14 days followed by alternate day steroids for 6 months, and then a slow taper over another 6 months have been recommended ( 10). Most patients are treated with a combination of surgery and steroids. Surgery provides a mechanism to aerate and irrigate the sinuses. DeJuan and colleagues ( 18) advocate immunotherapy, although others ( 11) note that this is unproved. Many have followed the radioallergosorbent test ( RAST) or other immunologic indices to assess the response to treatment ( 6,10,11). Before this syndrome was well recognized, many patients were treated with systemic antifungal agents ( 2,8,9,19). Even with intracranial extension, without actual central nervous system involvement ( dura is intact) systemic antifungal therapy, as used initially in our patient, may be unnecessary ( 3). REFERENCES 1. Katzenstein, AL. Allergic Aspergillus sinusitis: a newly recognized entity. ] Allergy Clin ImmunoI1983; 72: 89- 93. 2. Waxman JE, Spector JG, Sale SR, Katzenstein AL. Allergic Aspergillus sinusitis: concepts in diagnosis and treatment of a new clinical entity. Laryngoscope 1987; 97: 261~. 3. Manning SC, Schaefer SD, Close lG, Vuitch F. Culture positive allergic fungal sinusitis. Arch Otolaryngol Head Neck Surg 1991; 117: 174-- 8. I Gin Neuro- ophthalmol, Vol. 12, No. 4, 1992 256 M. JACOBSON ET AL. 4. McGinnis MR, Rinaldi MG, Winn RE. Emerging agents of phaeohyphomycosis: pathogenic species of Bipo/ aris and Exserohilll1n. I CIin Microbial 1986; 24: 25~ 9. 5. Berry A). Kerkering TM, Giordano AM, Chiancone I · Phaeohyphomycotic sinusitis. Pediatr Infect Dis 1984; 3: 15~ 2. 6. 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