Journal of Neuro-Ophthalmology, September 1992, Volume 12, Issue 3

Chronic idiopathic inflammation of the retropharyngeal space presenting with sequential abducens palsies.

We describe a patient who presented with sequential, bilateral abducens palsies associated with a mass of the nasopharynx. Biopsy of the mass showed chronic non-specific inflammation and fibrosis. The diagnosis of idiopathic inflammatory pseudotumor was arrived at by exclusion of other known causes of inflammation of the retropharyngeal space. Magnetic resonance imaging suggested that injury to the sixth cranial nerves probably occurred as they traversed the dura and subarachnoid space overlying the clivus.

(" uri/ at " r Clill/( al N<' IIro-" l'htllllllll" t" gy 120): 154- 157, 1992. Chronic Idiopathic Inflammation of the Retropharyngeal Space Presenting with Sequential Abducens Palsies Maher M. Fanous, M. D., Curtis E. Margo, M. D., and Latif M. Hamed, M. D. ' 0 1992 Ra yen Press, Ltd., New York We describe a patient who presented with sequential, bilateral abducens palsies associated with a mass of the nasopharynx. Biopsy of the mass showed chronic non­specific inflammation and fibrosis. The diagnosis of id­iopathic inflammatory pseudotumor was arrived at by exclusion of other known causes of inflammation of the retropharyngeal space. Magnetic resonance imaging suggested that injury to the sixth cranial nerves probably occurred as they traversed the dura and subarachnoid space overlying the clivus. Key Words: Idiopathic inflammatory pseudotumor­Sequential abducens palsies. From the Department of Ophthalmology ( M. M. F., C. E. M., L. M. H.) and Pathology ( C. E. M.), University of Florida College of Medicine, Gainesville, Florida, U. S. A. Address correspondence and reprint requests to Dr. Curtis E. Margo, Department of Ophthalmology, University of Florida, Box )- 284, ) HMHC, Gainesville, FL 32610, U. S. A. 154 The loose connective tissue that exists between the middle and deep layers of the deep cervical fascia resides within the retropharyngeal space. Superiorly the fascia is bound by the sphenoid bone and clivus, and is contiguous on each side to the carotid sheath. Caudally, the retropharyngeal space is continuous with the mediastium ( 1). Pri­mary disease processes within this space are rare ( 1,2). We describe a patient with an idiopathic in­flammatory pseudotumor of the retronasopharyn­geal space, who presented with sequential sixth nerve palsies. REPORT OF A CASE A 39- year- old man presented to the eye clinic with a I- month history of horizontal diplopia and intermittent frontal headaches. He had a history of multiple substance abuse, including intravenous and intranasal cocaine but was otherwise healthy. Clinical evaluation showed an isolated partial right sixth nerve palsy with otherwise normal ocular and physical examinations. A computed tomo­gram showed thickening of the mucosal lining of the sphenoid sinus. Magnetic resonance ( MR) re­vealed abnormal signals arising from the mucosa and periosteum of the sphenoid sinus and from the dura overlying the clivus. Laboratory studies including serologic tests for syphilis ( rapid plasma reagin and hemagglutination treponemal test for syphilis ( HATIS » , antinuclear antibodies, anticy­toplasmic neutrophilic antibodies, and angioten­sin- converting enzyme were all normal. Sickle cell preparation and Lyme titer were negative. IgM an­tibody to Epstein- Barr viral capsid antigen was negative. Chest x- ray was normal, but a purified p: otein derivative of tub~ rculin skin test was pos­Itive ( unknown converSIOn time). Serum human CHRONIC IDIOPATHIC INFLAMMATION OF RETROPHARYNGEAL SPACE 155 immunodeficiency virus ( HIV) antibody assay was negative. The patient refused lumbar puncture and was treated empirically with INH ( isoniazid) and pyridoxine. Two months later, the ocular dysmotility pro­gressed. Examination revealed isolated bilateral sixth nerve palsies ( Fig. 1). General and neurologic examinations were normal. Laboratory tests were again normal, including negative serologic tests for syphilis and HIV. Ocular forced ductions and a Tensilon test were negative. Lumbar puncture re­vealed a normal opening pressure and normal chemical profile of the cerebral spinal fluid; the cerebrospinal fluid contained 24 WBC/ dl with 88% lymphocytes, 5% monocytes, 7% neutrophils, and no erythrocytes. The cryptococcal antigen titer was negative; bacterial and fungal cultures showed no growth. A repeat MR showed a mass lesion of the retro­pharyngeal space at the level of the nasopharynx ( Fig. 2). There was persistent mucoperiosteal thickening of the sphenoid sinus and further meningeal enhancement overlying the clivus. Nasopharyngeal biopsies revealed nonspecific chronic inflammation with focal lymphoid hyper­plasia and fibrosis ( Fig. 3). There was no evidence of vasculitis, necrosis, or granuloma formation. Immunoperoxidase stains for immunoglobulins showed a polyclonal population of lymphocytes. Bacterial and fungal cultures of the biopsy tissue were negative. The bilateral abducens palsy remained stable over a 17- month follow- up. MR scans 6 and 13 months after the onset of symptoms revealed the same findings as detailed above. Additional biop­sies from the nasopharynx were obtained on two separate occasions during the 17- month interval of follow- up; both showed chronic nonspecific in­flammation, although the proportion of fibrocon­nective tissue increased in each successive biopsy. Bacterial and fungal cultures were negative on each occasion. COMMENT Chronic nonspecific inflammation of the retro­pharyngeal space showing a tumorlike configura­tion on neuroimaging is exceptionally rare ( 1,2). So- called inflammatory pseudotumor of the head and neck is most common in the orbit, where it usually presents with painful proptosis ( 3). The diagnosis of inflammatory pseudotumor is one of exclusion. Included in the differential diagnosis are infectious cellulitis, Wegener's granulomatosis, midline destructive disease, rhinoscleroma, and lymphoma ( 4). These disorders were effectively eliminated from consideration by biopsy on three occasions and by laboratory studies. The nosologic classification of cryptogenic in­flammatory tumifaction is imprecise. We prefer the term idiopathic inflammatory pseudotumor to de­scribe this condition. The clinical spectrum of in­flammatory pseudotumor is wide and varies ac­cording to anatomic location. Patients can present with acute, subacute, or chronic symptoms. Histo­logically, the inflammatory infiltrate is polymor­phic, and, depending on the stage of the disease, is associated with varying amounts of edema and fibrosis. The natural history of inflammatory pseu­dotumor is best characterized in the orbit, where the clinical course is variable, but self- limited ( 5). Acute episodes are typically sensitive to systemic corticosteroids. Inflammatory pseudotumor in the retropharyngeal space is uncommon and its clini­cal characteristics less well defined than pseudo­tumor of the orbit. Corticosteroids were not used initially in our pa­tient for several reasons. First, several infectious diseases, including acquired immunodeficiency syndrome ( AIDS) and tuberculosis, were in the differential diagnosis ( 6,7). After the apparent spontaneous arrest of this inflammatory process, we believed that potential therapeutic value of cor­ticosteroids was outweighed by their potential risk. The amount of collagenous connective tissue FIG. 1. The patient is esotropic in primary position and has bilateral limitation of abduction, more severe on the left side. I Clin Neuro- ophthalmol. Vol. 12, No. 3, 1992 156 M. M. FANOUS ET AI. FIG. 2. Magnetic resonance with gadolinium, sagittal view, showing enhancement of the dural- venous plexus in the area of the sphenoid sinus and clivus, and a mass lesion in the retropharyngeal space ( arrows). in repeat biopsy specimens increased, so that sen­sitivity to corticosteroids would theoretically be less. There also was concern that corticosteroids could potentially aggravate the osteolytic sinusitis in a cocaine user. Nonspecific inflammation of the retropharyn­geal space has been described in patients with AIDS ( 6). The possibility of AIDS cannot be abso­lutely excluded in our patient. His history of drug use places him at risk, although two negative se-rologic tests for HIV and the absence of other signs of immune dysfunction make the diagnosis of AIDS unlikely. Injury to the sixth cranial nerves in our patient might have occurred as they traversed the dura or subarachnoid space overlying the clivus where they are vulnerable to injury from chronic inflam­mation. The mild, chronic pleocytosis found in the cerebral spinal fluid is consistent with a para­meningeal focus of inflammation. , CIII' NCliro- OI, hthalrnol, Vol. 12, No. 3, 1992 FIG. 3. Biopsy from the nasopharynx showing chronic inflammation. A germinal center ( lower right corner) is surrounded by mature lympho­cytes. ( Hematoxylin- eosin, x 175.) CHRONIC IDIOPATHIC INFLAMMATION OF RETROPHARYNGEAL SPACE 157 Intranasal cocaine use has been associated with chronic osteolytic sinusitis, chronic inflammation of the orbit, and optic neuritis ( 8- 10). Cranial neu' ropathies are probably due to contiguous inflam­mation. The mechanism by which intranasal cocaine causes these complications is unclear, al­though the concurrent use of nasal sprays contain­ing steroids and vasoconstrictors may contribute to osteolysis ( 8). The history of drug abuse in our patient may be coincidental with his inflammatory processes, although the possibility that the two are causally related cannot be completely discounted. REFERENCES 1. Davis WL, Harnsberger HR. Smoker WRK, Watanabe AS. Retropharvngeal space: evaluation of normal anatomy and diseases with CT and MR imaging. Radiology 1990; 174: 59­64 2. Silver AI, Mawad ME, Hilal SK, Sane P, Ganti SR. Com-puted tomography of the nasopharynx and related spaces. Part II. Pathology Radiology 1983; 147: 733- 8. 3. Rootman I, Nugent R. The classification and management of acute orbital pseudotumor. Ophthalmology 1982; 89: 104CWl. 4. Pickens JP, Modica L. Current concepts of the lethal mid­line granuloma syndrome. Otolaryll:.: ol Head Neck Surg 1989; 100: 623- 30. 5. Kennerdell JS, Dresner ST. The nonspecific orbital inflam­matory syndromes. Surv Ophthalmol 1984; 29: 93- 103. 6. Stern JC, Pi- Tang L, Lucente FE. Benign nasopharyngeal masses and human immunodeficiency virus infection. Arch Otolaryllgol Head Neck SlIrg 1990; 116: 206-- 8. 7. Waldman SR, Levine HL, Sebek BA, et al. Nasal tubercu­losis: a forgotten entity. Laryngoscope 1981; 91: 11- 16. 8. Goldberg RA, Weismann JS, McFarland JE, Krauss HR, Helper RS, Shorr N. Orbital inflammation and optic neu­ropathies associated with chronic sinusitis of intranasal co­caine abuse: possible role of contiguous inflammation. Arch OphthalllloI1990; 107: 831- 5. 9. Schwitzer YG. Osteolytic sinusitis and pneumomediasti­num: deceptive otolaryngologic complications of cocaine abuse. Laryngoscope 1985; 96: 206- 10. 10. Newman NM, DiLoretto DA, Ho JT, Klein IC, Birnbaum NS. Bilateral optic neuropathy and osteolytic sinusitis: complications of cocaine abuse. lAMA 1988; 259: 72- 4. I Oi" Nellro- ophtha/ mo/, Vol. 12, No. 3, 1992