A 14-year-old male presented to the neurosurgical clinic with swelling just above the right eye which had been growing slowly for the last eight years. The swelling first appeared following a non-penetrating trauma eight years ago. On examination it was a non-tender, non-erythematous, firm, round swelling causing marked proptosis and diplopia on downward gaze only. The visual acuity was intact. MRI showed an intraorbital, extraconal mass isointense on T1 and hypointense on T2 imaging. A diagnosis of orbital tumor was made. A white, friable mass consistent with meningioma was resected. However histopathology report later showed it to be an Aspergilloma. The Patient was successfully treated with anti-fungal medicine and was disease-free at one year follow-up

Enam, Ather

Waheed, Syeda Sidra

Zafar, Muhammad Ahsan

eCommons@AKU

eCommons@AKUSection of Neurosurgery Department of SurgerySeptember 2009Orbital aspergillus infection mimicking a tumour: acase report.Muhammad Ahsan ZafarAga Khan UniversitySyeda Sidra WaheedAga Khan UniversityS. Ather EnamAga Khan University, ather.enam@aku.eduFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_neurosurgPart of the Neurology Commons, and the Surgery CommonsRecommended CitationZafar, M., Waheed, S., Enam, S. (2009). Orbital aspergillus infection mimicking a tumour: a case report.. Cases Journal, 2, 7860.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_neurosurg/31Case reportOpen AccessOrbital aspergillus infection mimicking a tumour: a case reportMuhammad Ahsan Zafar1, Syeda Sidra Waheed1 and S Ather Enam1,2*Addresses: 1Department of Neurosurgery, The Aga Khan University Hospital, Stadium road, 3500, Karachi, 74800, Pakistan2Department of Surgery & Biological Sciences, The Aga Khan University Hospital, Stadium road, 3500, Karachi, 74800, PakistanEmail: MAZ - ahsanzz@hotmail.com; SSW - waheed.sidra@gmail.com; SAE* - ather.enam@aku.edu*Corresponding authorReceived: 12 August 2009 Accepted: 24 August 2009 Published: 15 September 2009Cases Journal 2009, 2:7860 doi: 10.4076/1757-1626-2-7860This article is available from: http://casesjournal.com/casesjournal/article/view/7860© 2009 Zafar et al.; licensee Cases Network Ltd.This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.AbstractA 14-year-old male presented to the neurosurgical clinic with swelling just above the right eye whichhad been growing slowly for the last eight years. The swelling first appeared following a non-penetrating trauma eight years ago. On examination it was a non-tender, non-erythematous, firm,round swelling causing marked proptosis and diplopia on downward gaze only. The visual acuity wasintact. MRI showed an intraorbital, extraconal mass isointense on T1 and hypointense on T2 imaging.A diagnosis of orbital tumor was made. A white, friable mass consistent with meningioma wasresected. However histopathology report later showed it to be an Aspergilloma. The patient wassuccessfully treated with anti-fungal medicine and was disease-free at one year follow-up.IntroductionOrbital masses can be neoplastic, infectious or inflamma-tory in origin. Owing to the confined nature of the orbitalspace they present with overlapping clinical manifesta-tions. Yet based on some disease-specific symptoms, signsand radiological imaging a definite diagnosis can usuallybe reached. An indolent growth of a solitary, discrete massis usually suggestive of a tumor. Fungal infections of theorbit are almost exclusively seen in immune compromisedstates. In this report, we present a case of an orbital mass ofinfectious origin with fungal etiology that grew slowly overa period of eight years and had clinical features suggestiveof a neoplasm.Case presentationA 14-year-old South East Indian male presented to ourtertiary care hospital with a painless, non-tender swellingjust above the right eye. The patient, a school-going childfrom a rural coastal village in Pakistan, was accompaniedby his father.The swelling had developed over the last eight yearsfollowing a superficial orbital trauma by a cricket bat withno bone or eye involvement. There was marked proptosisof the right eye but no complaints of visual impairment,pain or discharge. Binocular diplopia was present ondownward gaze. He had undergone resection of the massthrough the superior palpebrae in a rural out-patient clinicpreviously. At that time, the mass was apparently partiallyresected. The tissue was not sent for histopathologicalevaluation and no medical record was available regardingthat procedure. Review of systems was unremarkable. Thepatient had no constitutional complaints, and reported nochange in appetite or weight.Page 1 of 4(page number not for citation purposes)Past medical history was remarkable only for the out-patient resection procedure three years ago. No recordswere available from that time. The patient was deliveredat home, at term, after an unremarkable pregnancy, andachieved all mile-stones at appropriate ages. No record ofchild-hood immunizations was present. There was noevidence of a disease causing immuno-suppression in thepatient, and nutritional status was within normal limits.Family history was unremarkable, and he was taking nomedications at that time. There was no history of alcohol,tobacco or illicit drug use and the patient was not sexuallyactive.The patient was at the 50th percentile for height, and 40thfor weight, and was in no obvious distress. Generalphysical exam was unremarkable with no lymphadeno-pathy noted. The neurological exam, including that of thecranial nerves II, III, IV and VI was unremarkable. A non-tender, non-erythematous, moderately hard intraorbitalmass was palpable superior-medial to the right eye-ball.The rest of the systemic exams, including head and neckexam with sinuses, were unremarkable.The MR imaging demonstrated an extraconal mass in theright orbit. The mass was isointense on T1 weightedimaging and hypointense on T2 weighted imaging.Significant mass effect was noted with the orbital contentspushed inferio-laterally. Infiltration in the surroundingtissue or extra-orbital extension was not observed. Postgadolinium T1 weighted imaging showed intense homo-genous contrast enhancement (Figure 1).Based on the history, clinical presentation and radiologicalimaging, a diagnosis of orbital tumor was made. Surgicalexcision was carried out using the anterior fossa approach.Intraoperatively, the mass was located under the periorbi-tal fascia. After opening this fascia, the frontal nerve andthen its branch the supraorbital nerve was identifiedleading into the mass but then it could not be followedany further. All the muscles including the superior obliqueand levator palpebrae superioris were pushed down bythe mass. The mass was pearly white, friable and ofcartilaginous consistency with no infiltration into thesurrounding tissue, resembling a meningioma or neurofi-broma. The mass was discrete and seemed to be arisingfrom the supraorbital nerve. The supraorbital nerve is aterminal branch of the frontal nerve which arises from theophthalmic division of the trigeminal nerve, the fifthcranial nerve. The tissue was sent for histopathologicaldiagnosis which showed chronic granulomatous inflam-mation with multi-nucleated giant cells and areas ofnecrosis. Special stains; PAS (periodic acid Schiff) andtissue culture revealed numerous septate fungal hyphaeconsistent with Aspergillus Fumigatus (Figure 2). Therewas no evidence of neoplasm on histological studies. TheFigure 1. MRI (A) T1 Coronal image; an isointense masslocated superio-medially in the right orbit, pushing the orbitalcontents. (B) T2 axial fat suppressed image; a hypointense,extraconal mass located medially in the right orbit, causingproptosis. No infiltration in the surrounding structurescan be seen (C) Post-gadolinium axial image; intensecontrast enhancement of the mass.Figure 2. Histology (A) Periodic acid schiff (PAS) staining;purplish-red fungal hyphae are seen, surrounded by moderateinfiltrate of polymorph leuckocytes. Inset-magnified view ofthe fungal hyphae. (B) H & E staining; large Giant-cellsurrounded by leuckocytic infiltrate.Page 2 of 4(page number not for citation purposes)Cases Journal 2009, 2:7860 http://casesjournal.com/casesjournal/article/view/7860patient was treated with oral itraconazole, 200 mg twicedaily for five days and then once daily for 2 months.At one year post-surgical follow-up there were nocomplaints of visual or neurological functions. Theexamination and work-up was unremarkable.DiscussionThis is a case of a 14-year-old male with an orbital mass,histologically proven to be a Aspergillus infection butclinically suggestive of a tumor.Primary orbital tumors of the pediatric age group aredermoid and epidermoid cyst, capillary hemangioma andrhabdomyosarcoma [1]; in the adult age group commonprimary tumors are lymphoid tumor, cavernous heman-gioma, meningioma, neurofibroma and schwannoma.Orbital tumors have proteanmanifestations. The signs andsymptoms are related to mass effect of the tumor withinthe compact orbit. Proptosis and exophthalmos are themost common presentations. Others are restricted ocularmovements, lagopthalmos, exposure keratitis, chemosis,visual impairment and diplopia. Most tumors are slowgrowing therefore the symptoms develop over a period oftime. In the case presented here, the swelling haddeveloped in an immuno-competent person over a periodof eight years with no acute symptoms. The onlycomplaint was slowly progressing downward gaze diplo-pia. The indolent course suggested neoplastic nature of themass.Orbital infections occur due to spread of infection fromparanasal sinuses or direct inoculation due to trauma,surgery or skin infections [2]. Orbital cellulitis is mostcommonly caused by bacterial infection. Fungal and viraletiologies occur less frequently. Mycotic orbital cellulitis isoften seen in patients with uncontrolled diabetes mellitusor other immunocompromised states such as AIDS,malignancy or steroids use. They may be invasive ornon-invasive. Fungal etiologies include Aspergillus, Mucorand Cryptococcus species [2]. Patients with orbitalinfections causing mass effect may present with symptomssimilar to tumors. In addition infections may haveinflammatory signs and signs/symptoms associated withparanasal sinusitis or intracranial extension. Fungal infec-tions of the orbit may have atypical presentation. There isone report each for orbital fungal infection presenting asoptic neuritis [3], sub-periosteal abscess [4], optic neuro-pathy [3] and orbital apex syndrome [5]. On the otherhand, orbital tumors have been reported to present asdysthyroid eye disease [2], ophtalmoplegia [2], epiphora[6] and Duane syndrome [7]. In the case presented here,the patient was immuno-competent, there was noevidence of any inflammatory process on clinicalevaluation, and there was no involvement of paranasalsinuses or intracranial extension of the disease. Lack ofsuch findings gave a false clinical impression of orbitaltumor.MR imaging is preferably used to assess the orbital diseaseas it gives a detailed picture of soft tissue structures,especially in case of fungal infections which have thepropensity to extend into surrounding structures. The MRIfindings in orbital fungal infections are very characteristic.These include a mass lesion producing hypo-to-isointensesignals on T1 weighted and extremely hypointense on T2weighted with bright homogenous enhancement on post-gadolinium T1 weighted images [8]. Optic nerve sheathmeningioma appears as a localized or fusiform enlarge-ment of the optic nerve. T1 and T2 weighted imagesusually show no significant change in intensity ofmeningioma compared to the normal optic nerve.Gadolinium images show marked or moderate contrastenhancement [3].In our case, MRI findings were of an extraconal mass,isointense on T1 weighted and hypointense on T2weighted with intense homogenous contrast enhancementon post-gadolinium T1 weighted. The literature shows thatthese findings are characteristic of an Aspergillum infec-tion [8]. The long-standing history, the slow indolentgrowth, absence of inflammatory signs and no infiltrationin the surroundings caused it to be misdiagnosed as atumor and the findings on operative resection of the massalso seemed consistent with meningioma arising from thesupraorbital nerve. Hence, fungal infections should alwaysbe kept in differentials of such solitary orbital masses withlong indolent growth.ConsentWritten informed consent was obtained from the patient’sfather for publication of this case report and accompany-ing images. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsAll the above mentioned authors, AZ, SW and AEcontributed in information collection, literature reviewand manuscript writing. Dr. S Ather Enam was also theprimary surgeon of the case presented.References1. Castillo BV Jr, Kaufman L: Pediatric tumors of the eye and orbit.Pediatr Clin North Am 2003, 50:149-172.2. Hershey BL, Roth TC: Orbital infections. Semin Ultrasound CT MR1997, 18:448-459.Page 3 of 4(page number not for citation purposes)Cases Journal 2009, 2:7860 http://casesjournal.com/casesjournal/article/view/78603. Mafee MF, Goodim J, Dorodi S: Optic nerve sheath meningio-mas. Radiol Clin North Am 1999, 37:37-58.4. Spoor TC, Hartel WC, Harding S, Kocher G: Aspergillosispresenting as a corticosteroid-responsive optic neuropathy.J Clin Neuroophthalmol 1982, 2:103-107.5. Matsuo T, Notohara K, Yamadori I: Aspergillosis causing bilateraloptic neuritis and later orbital apex syndrome. Jpn J Ophthalmol2005, 49:430-431.6. Eustis HS, Mafee MF, Walton C, Mondonca J: MRI and CT ofOrbital infections and complications in acute rhinosinusitis.Radiol Clin North Am 1998, 36:1021-1279.7. Gatzonis S, Charakidas A, Pepethanossou M: Multiple orbitaldermoid cysts located within the lateral recuts muscle,clinically mimicking Duane syndrome type II. J PediatrOphthalmol Strabismus 2002, 39:324.8. Siddiqui AA, Bashir SH, Ali Shah A, Sajjad Z, Ahmed N, Jooma R,Ather Enam S: Diagnostic MR imaging features of craniocer-ebral Aspergillosis of sino-nasal origin in immunocompetentpatients. Acta Neurochir (Wien) 2006, 148:155-166.Do you have a case to share?Submit your case report today• Rapid peer review• Fast publication• PubMed indexing• Inclusion in Cases DatabaseAny patient, any case, can teach ussomethingwww.casesnetwork.comPage 4 of 4(page number not for citation purposes)Cases Journal 2009, 2:7860 http://casesjournal.com/casesjournal/article/view/7860

Orbital aspergillus infection mimicking a tumour: A case report

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Orbital aspergillus infection mimicking a tumour: A case report

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