Irreversible Unilateral Visual Lossdue to Acute SinusitisAntoine E. Tarazi, MD, Alan H. Shikani, MD

\s=b\Extension of sphenoethmoiditis intothe orbital apex may result in visual lossand ophthalmoplegia, but minimal signs oforbital pathology such as proptosis, che-mosis, or lid edema. This entity is termedorbital apex syndrome. The case of a 74\x=req-\year-old woman with orbital apex syndromeand irreversible unilateral visual loss sec-

ondary to bacterial sphenoethmoiditis ispresented. This case, and our review of theliterature, suggest that patients with symp-tomatic acute sphenoethmoiditis are at a

relatively higher risk of permanent visualloss than those with sinusitis not involvingthe posterior ethmoid and/or sphenoidsinuses.(Arch Otolaryngol Head Neck Surg.

1991;117:1400-1401)

Orbital involvement by sinusitis is a

well-recognized entity that is gen¬erally due to direct extension of theinfection. Visual dysfunction may coin¬cide with or follow signs of orbitalinvolvement such as proptosis, chemo-sis, and lid edema. Orbital apex syn¬drome (OAS) is a rare form of compli¬cation that classically presents withvisual loss and ophthalmoplegia, butwith minimal or no signs of orbitalinflammation. The pathology starts inthe sphenoethmoid region and extendsinto the orbital apex with subsequentblindness. We present a case of acutesphenoethmoiditis that was complicat¬ed by monocular and irreversible visu¬al loss in an elderly diabetic patient.Extensive investigation ruled out fun¬gal or neoplastic disease.

Accepted for publication April 23, 1991.From the Department of Otolaryngology Head

and Neck Surgery, The Johns Hopkins MedicalInstitutions, Baltimore, Md.Reprints not available.

REPORT OF A CASEA 74-year-old woman presented to the

emergency department because of acute lossof vision on the right side associated withright-sided periorbital pain of 24 hours' dura¬tion. Her history was significant for con¬

trolled insulin-dependent diabetes mellitusfor the past 6 years and right-sided headacheof several months' duration. She denied nasaldischarge, fever, postnasal drip, or nasalcongestion. Physical examination revealed a

temperature of 36.5°C and stable vital signs.Ophthalmologic evaluation revealed minimalproptosis of the right eyelid with moderateophthalmoplegia and no light perception. Aright-sided afferent pupillary defect was not¬ed; the anterior chamber, conjunctiva, andcornea were all normal. Funduscopic exami¬nation revealed mild disk pallor without atro¬phy, and a background of mild diabetic reti-nopathy that could not explain the visualloss. Examination of the nose with the Storztelescope revealed a mass in the middlemeatus that was clinically suspected to be a

tumor. No black or necrotic ulcers could beseen in the nose or palate. The rest of hergeneral and neurologic examination was

normal.Computed tomography andmagnetic reso¬

nance imaging of the orbits revealed a mass

involving the right sphenoid, ethmoid, andmaxillary air cells with some degree of bonyerosion in the posterior portion of the lami¬nae papyracea and extension into the orbitalapex (Fig 1). This mass had a high signalintensity on T2 imaging and was suspected tobe an inflammatory process (Fig 2). Labora¬tory work-up included a normal white bloodcell count, with a blood glucose level of12.5 mmol/L. The patient was given 1 g ofcefazolin intravenously every 8 hours. A bi¬opsy procedure of the intranasal mass re¬

vealed acute and chronic inflammatorychanges. No tumor or fungus could beidentified.

Because the ophthalmologist believed that

the visual loss was irreversible, a limiteddecompression with intranasal endoscopiesphenoethmoidectomy was performed.Management also included aggressive con¬

trol of the blood glucose and intravenousadministration of cefazolin, which was con¬

tinued for 2 weeks. The patient's vision didnot recover postoperatively nor at the lastfollow-up, which was 2 months later. Theintranasal endoscopie examination, on theother hand, showed resolution of the parana-sal pathologic condition.

COMMENT

Orbital apex syndrome, or isolatedvisual loss with minimal inflammatoryorbital signs caused by adjacent poste¬rior sphenoethmoidal sinusitis, is ex¬

tremely rare. It was originally de¬scribed by Rochon-Du Vigneaud in18961 and believed to be caused bysyphilis. Trantas2 in 1893 described a

case of total ophthalmoplegia and "ocu¬lar complications" secondary to para-nasal sinusitis. In 1945, Kjoer3 re¬

ported on unilateral blindness,ophthalmoplegia but no proptosis,caused by sphenoethmoidal sinusitis.Since then, only a handful of cases

fulfilled the criteria of OAS. In thelatest study in 1987, three cases ofOAS were described by Slavin andGlaser,4 who renamed the entity poste¬rior orbital cellulitis. This syndrome ismuch less common than the orbitalcomplications of anterior ethmoid si¬nusitis. Chandler et al5 summarizedthe clinical spectrum of the latter;these range from inflammatory orbitaledema to orbital cellulitis, orbital andsubperiosteal abscesses, optic neuritis,and cavernous sinus thrombosis. De¬pending on the degree of involvement,

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Fig 1 .—Computed axial tomogram of the orbits demonstrating right-sidedethmoidal and sphenoidal sinusitis with extension into the orbital apex.There is some bony erosion in the posterior portion of the laminaepapyraceae.

Fig 2.—Magnetic resonance imaging of the head showing right-sidedmaxillary and ethmoidal sinusitis. The high signal intensity on T2 imaging issuggestive of an inflammatory process.

the patient may present with propto-sis, ophthalmoplegia, eyelid edema,chemosis, and possibly visual loss.The incidence of permanent visual

loss with documented orbital or sub-periosteal abscesses is 15%.M0 Thecause has been suggested to includevascular compromise, compression, orinfiltration of the optic nerve. Septicvasculitis and increased intraorbitalpressure have also been postulated as

contributing factors.4Why is the contiguous spread from

posterior sphenoethmoiditis much lessfrequent than from anterior ethmoidi-tis? Perhaps the answer lies in thepeculiar anatomy of the interface be¬tween the periorbita and the paranasalsinuses. The periosteum of the orbit isloosely attached to the bone anteriorlyand may be elevated by a purulentcollection resulting in a subperiostealabscess. In contrast, the periorbita isthicker and more firmly attached pos¬teriorly, providing a barrier to thespread of infection.4 In addition, thesphenoid bone is thicker and hencemore difficult to penetrate than thevery thin anterior lamina papyracea.Finally, the ethmoid foramina mayserve as a conduit for a direct spread ofinfection from the sinuses into the or¬bit anteriorly.

The cause of visual loss in OAS is thesame as in orbital cellulitis; however,the importance of compressive and in¬flammatory factors is magnified be¬cause the optic nerve is confined in theorbital apex and within a bony canal.The marked bowing of the posteriorethmoid air cells seen in some patientsmay further increase the compressionof the nerve.4 In the only autopsy ofOAS reported in the literature, Kjoer3describes marked necrosis of the proxi¬mal segment of the optic nervewith severe thromboangiitis of itsvasculature.The treatment of OAS as recom¬

mended by Slavin and Glaser4 isprompt use of intravenous antibioticswith early decompression and drainageof the sinuses. None of the reportedcases had decompression of the opticnerve in the canal itself. In all thecases, the prognosis was very poorwith no or very little improvement invision. Whether a total decompression,including the optic canal in the posteri¬or ethmoid and sphenoid areas is help¬ful, is not clear. Corticosteroids havebeen tried but found to be ineffective.1Otolaryngologists should be aware

that patients with acute sphenoethmoi¬ditis extending into the orbit are at ahigher risk of permanent visual loss.

Although the prognosis is poor, ag¬gressive surgical and medical interven¬tion are warranted, since it is possiblethat some improvement of vision mayoccur and that the disease may spreadto the fellow eye if left untreated.Complete work-up to rule out othertreatable disease entities, such as neo¬

plasms or granulomatous disorders, isa must.

References1. Kronschnabel EF. Orbital apex syndrome

due to sinus infection. Laryngoscope. 1974;84:353\x=req-\371.2. Trantas A. Ophtalmoplegie totale et autres

complications occulaires dans les polysinusites.Arch Ophtalmologie. 1893;13:357.

3. Kjoer I. A case of orbital apex syndrome incollatral pan-sinusitis. Acta Ophthalmol.1945;23:357-366.4. Slavin ML, Glaser JS. Acute severe irrevers-

ible visual loss with sphenoethmoiditis: 'posterior'orbital cellulitis. Arch Ophthalmol.. 1987;105:345\x=req-\348.

5. Chandler JR, Langenbrunner DJ, StevensER. The pathogenesis of orbital complications inacute sinusitis. Laryngoscope. 1970;80:1414-1428.6. Jarret WH, Gutman FA. Ocular complica-

tions of infection in the paranasal sinuses. ArchOphthalmol. 1969;81:683-688.

7. Quick CA, Payne E. Complicated acute sinus-itis. Laryngoscope. 1972;82:1248-1263.

8. Hornblass A, Herschorn BJ, Stern K, et al.Orbital abscess. Surv Ophthalmol. 1984;29:169\x=req-\178.9. Welsh LW, Welsh JJ. Orbital complications of

sinus disease. Laryngoscope. 1974;84:848-856.10. Morgan PR, Morrison WV. Complications of

frontal and ethmoidal sinusitis. Laryngoscope.1980;90:661-666.

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