Ocular manifestations of sphenoid mucoceles:

Skull Base Surgery

Ocular Manifestations of Sphenoid Mucoceles: CLINICAL FEATURES AND NEUROSURGICAL MANAGEMENT OF THREE CASES AND REVIEW OF THE LITERATURE Nedal Hejazi,* Alfred Witzmann* and Werner Hassler† *Department of Neurosurgery, LKH Feldkirch, Austria; †Department of Neurosurgery, Klinikum Kalkweg, Duisburg, Germany

Hejazi N, Witzmann A, Hassler W. Ocular manifestations of sphenoid mucoceles: clinical features and neurosurgical management of three cases and review of literature. Surg Neurol 2001;56:338 – 43. BACKGROUND

Sphenoid mucoceles (SMCs) are unusual lesions, with about 130 cases reported in the literature. Although benign, they may involve the orbit and cause acute restrictive ophthalmoplegia, proptosis, and reduced visual acuity. METHODS AND RESULTS

We present three cases (1 male, 2 females, aged 35, 36, and 46 years) of orbital involvement with acute decreased visual acuity by SMC. The lesions were promptly excised via a transnasal approach to decompress the optic nerve. After the decompression, the patients recovered completely.

anterior visual pathways. Ophthalmic manifestations may include restrictive ophthalmoplegia, proptosis, and reduced visual acuity because of optic nerve compression. The etiology of the SMC is still uncertain, but it is almost unanimously thought to be caused by an accumulation of mucus due to phlogistic stenosis of the sinus ostium or of one of its recesses [16,18]. The optic nerve may be involved either in a phlogosis because of contiguity or in a direct compression with development of a retro-bulbar neuropathy [1,17]. We present three personal cases of SMC with optic nerve compression and review briefly 130 other cases of SMC in the literature.

CONCLUSION

We believe that immediate surgical drainage of the SMC and prolonged antibiotic therapy are necessary and resulted in recovery of visual acuity. Prompt microsurgical transsphenoidal decompression is simple, effective, safe, and is necessary for avoiding persistent visual loss. Antibiotic therapy alone usually results in a poor outcome. We discuss the etiology, clinical manifestations, and management of this rare condition in the light of 130 other cases in the literature. © 2001 by Elsevier Science Inc. KEY WORDS

Mucocele, sphenoid sinus, optic nerve decompression.

phenoid mucoceles (SMCs) are unusual lesions, with about 130 cases reported in the world literature since Berg’s first description in 1889 [5]. Although rare, SMC should be considered in the differential diagnosis of cystic lesions of the

S

Address reprint requests to: Dr. Nedal Hejazi, Department of Neurosurgery, LKH Feldkirch, Carinagasse 9, A-6800 Feldkirch, Austria. Received November 22, 2000; accepted June 12, 2001. 0090-3019/01/$–see front matter PII S0090-3019(01)00616-4

Summary of Cases In the period from 1990 through 2000, we treated 3 patients with large SMCs accompanied by visual disturbances. CASE 1 A 36-year-old woman was referred for an opinion from a neurological department after she developed left-sided headache associated with lacrimation and nausea, which had intensified over a 4-day period. Visual acuity remained unaffected until the third day in hospital, when it was noted to decrease to 0.2 in the left eye with left-sided color-blindness. The left side of her face was slightly swollen. There was no pain on palpation of her frontal or maxillary sinuses. Palpation of the orbit rim and regional lymph nodes showed no abnormality and percussion of the sinuses was not painful; extraocular movements were full and did not evoke diplopia. Ophthalmoscopic examination of the left eye © 2001 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010

Ocular Manifestations of Sphenoid Mucoceles

Surg Neurol 339 2001;56:338 –43

(Case 1): Coronal (A) and axial (B–F) T2-weighted MRI of the orbit demonstrate a well-defined, hyperintense expansive SMC with only peripheral contrast enhancement in the area of the hyper-vascular inflammatory tissue that lines the sinusal cavity of the left sphenoidal sinus. Note the extension to the left orbit apex and compression of the left optic nerve.

1

showed disc hyperemia and edema. No abnormalities were detected in the right eye. The examination was otherwise unremarkable. The history revealed that she had been treated by her dentist with dental filling because of a tooth-cyst in the second left maxillary bicuspid tooth 4 weeks previously. Axial, sagittal, and coronal magnetic resonance imaging

(MRI) demonstrated soft-tissue swelling in the maxillary and sphenoid sinuses with extension to the orbit apex and compression of the left optic nerve (Figure 1). On the basis of these examinations, an SMC with orbital extension was diagnosed and transnasal marsupialisation and drainage of the cyst was performed. Purulent material was drained from the

340 Surg Neurol 2001;56:338 –43

Hejazi et al

(Case 1): Postoperative T1weighted unenhanced (Aⴙ B) and gadolinium-enhanced (Cⴙ D), axial MRI demonstrating the drainage of the SMC with sphenoid sinus pneumatization and decompression of the left optic nerve.

2

left sphenoid sinus and material for cultures was taken. These cultures subsequently grew Proteus enterobacteria. Postoperatively intravenous cefotiam 2,000 mg every 8 hours was given. Histopathological examination confirmed the diagnosis. Two days postoperatively the patient was pain-free, the visual acuity improving to 0.8 in the left eye with resolution of all other ocular symptoms. Postoperative MRI of the orbit demonstrated the removal of the mucocele and the decompression of the left optic nerve (Figure 2). CASE 2 A 46-year-old woman was admitted urgently because of sudden onset of headache, nausea, and visual disorders. Clinical examination reviled leftsided loss of visual acuity and left mydriasis. MRI showed a large SMC extending to the suprasellar region and peripherally contrast-enhanced. A tentative diagnosis of SMC was made. Surgery was performed by the rhino-septal route and resulted in drainage of a large mucocele, later confirmed by histology. Staphylococcus aureus was isolated and intravenous flucloxacillin 12 g daily was given. After surgery, headaches and nausea disappeared, visual acuity improved, and the visual fields returned to normal. CASE 3 A 35-year-old man was referred to our hospital for investigation of headaches and visual loss. The diagnosis of a SMC was made by MRI and drainage via a transnasal sphenoidotomy produced 21 mL of

brownish fluid (Figure 3). Biopsy of the wall confirmed the diagnosis of a mucocele. Postoperatively, intravenous ampicillin 6 g daily was given. The patient recovered completely.

Discussion Paranasal sinus mucocele is mainly found in adults aged 30 to 60. In order of frequency, the development sites are the frontal sinus (65%), the anterior ethmoid (30%), and the maxillary sinus (3–10%); posterior ethmoidal and especially sphenoidal occurrence is extremely rare (1%) [18]. As secretions accumulate in the sinus, a dilated cavity called a mucocele forms; and when infection occurs and pus is present, the lesion is called a mucopyocele. The diagnosis usually is made by surgery or at autopsy. The etiology of the SMC is still uncertain, but it is almost unanimously thought to be caused by an accumulation of mucus because of phlogistic stenosis of the sinus ostium or of one of its recesses [18]. Other hypotheses suggest glandular structures’ cystic dilatation, cyst development from embryonal epithelial residues, and even an atypical form of craniopharyngioma. In regard to histological and clinical features, the SMC shows a remarkable similarity to the Rathke’s cleft cyst. Both have been reported as lined with epithelium that varies from squamous to columnar with cilia; and each has fluid described as viscid, mucousoid, or brown with cholesterol crystals.

Ocular Manifestations of Sphenoid Mucoceles

Surg Neurol 341 2001;56:338 –43

(Case 3): (A) T1-weighted gadolinium-enhanced, sagittal MRI demonstrating the mass within the sphenoid sinus with compression of the optic nerve and the optic chiasm (*). (B) Illustration of the large cyst in the sphenoid sinus and the compressed optic nerve. The cyst lifted the hypophysis gland upwards and pressed upon the optic nerve and optic chiasm (*). T1-weighted unenhanced (C) and gadolinium-enhanced (D), sagittal MRI demonstrating the mass within the sphenoid sinus with compression of the optic nerve and the optic chiasm (*).

3

Both arise in the same area and produce a similar clinical and pathological picture [16,18]. Although benign and rare, SMCs have been reported to involve the orbit and to cause visual loss, rarely including a bitemporal hemianopsia; therefore they are frequently misdiagnosed as pituitary adenomas or chordoma [20]. The paranasal sinuses aerate between 12 and 24 months of age; consequently, SMC and secondary involvement of the orbit almost never occur before 3 years of age [2,16]. The history in such cases is typical. An upper respiratory or tooth infection develops in conjunction with the sudden onset of visual impairment and incomplete restrictive ophthalmoplegia, as seen in Case 1. Since the first report of a case of SMC by Berg in 1889 [5], nearly 130 cases of symptomatic SMC have been documented. These include 69 (53%) males and 61 (47%) females ranging in age from 8 years to 83 years, but 48% of all cases were 30 to 60 years old. The duration of symptoms before the diagnosis

ranges from 3 days to 38 years, with a mean value of 3.7 years. In 87% of the cases, the chief presenting feature was pain (often unilateral and fronto-orbital headache). Other presenting signs were ophthalmic disorders (111/130 cases, 85%), amaurosis (58%), oculomotor palsies (71/130 cases, 55%), ENT symptoms (anosmia, nasal obstruction, hypoacusis, and nasal discharge in 50 cases, 38%), endocrine disorders (4/130 cases), and panhypopituitarism (1/130). The state of the visual field was mentioned in 84 cases; it was restricted in 49 of them. The IIIrd cranial nerve is the one most commonly affected (40/130 cases), while the IVth nerve is rarely involved (6/130 cases). Exophthalmos is present in 25% and optical atrophy in 29% of the reported cases [1,2,4,6 –9,11,12,14,15,19,21–23,26]. On computed tomography and MRI, the lesion is frequently smooth, well-defined, usually hyperintense on T2weighted MRI, and shows only peripheral contrast enhancement in the area of the hyper-vascular in-

342 Surg Neurol 2001;56:338 –43

flammatory tissue that lines the sinusal cavity [25]. There is usually no intralesional enhancement and no calcification (Figure 1). Several cases of SMC showed low-intensity signal on both T1- and T2weighted MRI. This is because of dehydration of the contents [7,26]. Gram-positive organisms (Staphylococcus aureus, Staphylococcus epidermidis, and streptococci) were the most frequently isolated organisms in SMCs, followed by anaerobic bacteria species, gram-negative bacteria (Hemophilus influenza, Escherichia coli, Pseudomonas aeroginosa, Proteus), and fungi (aspergillus, chlamydia) [6,16]. Antibacterial therapy should be based on the organisms most likely to be encountered. In case of failure to isolate the bacteria, the spectrum of antimicrobial drugs should cover as many causative microorganisms as possible, considering the predisposing factors. With MRI, the diagnosis of SMC can now be suspected before surgery [7]. However, a definite diagnosis of SMC can be made only intraoperatively. The important differential diagnosis is between necrotic primary adenoma in cases with significant intrasellar extension, craniopharyngioma, and chordoma. Infection, plasmacytoma, osteoma, osteoblastoma, basal cell and squamous cell carcinoma, rhinolitis, polyps, and fibrous dysplasia should be also considered in the differential diagnosis of SMC [11,13,14,20]. On MRI, the signal in a chordoma is usually low on T1-weighted sequences and very heterogeneous and variable on T2weighted sequences. Additionally, osteolytic activity and the presence of intratumoral osseus septa are typical signs of chordoma. In contrast, bony destruction is possible, but not a common finding in SMC [22,24]. The regular backward displacement of intact sellar dura, the smooth and well-defined growing pattern of the lesion into the entire sphenoid sinus, and the lack of significant contrast enhancement on MRI should be considered in ruling out the diagnosis of clival chordoma or other neoplasms [20,25,26]. The treatment of choice for SMC is surgical drainage and may be rhinological or neurosurgical or both, according to the expansion of the disease. The transnasal-transsphenoidal route (via endonasal endoscopy or microsurgically) was the one used most often in the literature (about 75% of all cases). We used the transnasal-transsphenoidal approach in all cases and the SMC was removed without difficulty and without postoperative complications. Postoperative complications of transsphenoidal decompression in SMC are almost unknown [3,10]. Postoperative complications such as meningitis or brain abscess were seen only in patients undergo-

Hejazi et al

ing a frontal craniotomy. In all cases where the frontal route was utilized it was because the diagnosis was wrong; hence the importance of an accurate preoperative diagnosis [2,18].

Conclusion We believe that immediate surgical drainage of the SMC with decompression of the orbit and optic nerve and prolonged antibiotic therapy are necessary and usually result in resolution of ophthalmic symptoms and recovery of visual acuity. Microsurgical transsphenoidal decompression is simple, effective, and safe. Antibiotic therapy alone usually results in a poor outcome [1,2,4]. Previous cases have been confined to reports in the immunology, infectious disease, otolaryngology, and head and neck literature. However, it is essential that the neurosurgical community be aware of SMC and its potentially devastating effects such as ophthalmic disorders, blindness, and intracranial spread. REFERENCES 1. Avery G, Tang RA, Close LG. Ophthalmic manifestations of mucoceles. Ann Ophthalmol 1983;15(8): 734 –7. 2. Barat JL, Marchal JC, Bracard S, Auque J, Lepoire J. Mucoceles of the sphenoidal sinus. Report of six cases and review of the literature. J Neuroradiol 1990; 17(2):135–51. 3. Benninger MS, Marks S. The endoscopic management of sphenoid and ethmoid mucoceles with orbital and intranasal extension. Rhinology 1995;33(3):157– 61. 4. Bregeat P. Ophthalmic manifestations of sphenoidal mucoceles. Ann Ophthalmol 1977;9(3):259 – 66. 5. Berg J. Bigrag til ka¨ nnedomen om sjukdomar na inasaus bihalor saint till la¨ ran am cerebro spinal va¨ tskas. Flytiring ur crasem. Nordiskt Medicinskt Archiv 1889; 21:1–24. 6. Bynke O, Radberg C. Mucocele of the sphenoidal sinus. Neurochirurgia (Stuttg) 1985;28(1):28 –30. 7. Dawson RC, Horton JA. MR imaging of mucoceles of the sphenoid sinus. AJNR 1989;10:613– 4. 8. Doyle CS, Simeone FA. Mucocele of the sphenoid sinus with bilateral internal carotid artery occlusion. J Neurosurg 1972;36:351– 4. 9. Friedmann G, Harrison S. Mucocele of the sphenoidal sinus as a cause of recurrent oculomotor nerve palsy. J Neurol Neurosurg Psychiat 1970;33(2):172–9. 10. Garaventa G, Arcuri T, Schiavoni S, Fonzari M. Anterior clinoid mucocele: a trans-nasal endoscopic approach. Minim Invas Neurosurg 1997;40(4):144 –7. 11. Gore RM, Weinberg PE, Kim KS, Ramsey RG. Sphenoid sinus mucoceles presenting as intracranial masses on computed tomography. Surg Neurol 1980;13:375–9. 12. Hakuba A, Katsuyama J, Matsuoka Y, Shim JH, Nishimura S. Sphenoid sinus mucoceles. J Neurosurg 1975;43:368 –73. 13. Kessler L, Legaludec V, Dietemann JL, Maitrot D, Pinget M. Sphenoidal sinus mucocele after transsphe-

Ocular Manifestations of Sphenoid Mucoceles

14. 15. 16. 17.

18. 19. 20. 21. 22. 23. 24. 25. 26.

noidal surgery for acromegaly. Neurosurg Rev 1999; 22(4):222–5. Linthicum FH, Raud CW, Reeves DL. Mucoceles of the sphenoid sinus. Report of a case with autopsy findings. J Neurosurg 1946;3:444 –53. Mac Carthy W, Frenkel M, Busse BJ. Visual loss as the only symptom of sphenoid sinus mucocele. Am J Ophthalmol 1972;74:1134 – 40. Moriyama H, Nakajima T, Honda Y. Studies on mucoceles of the ethmoid and sphenoid sinuses: analysis of 47 cases. J Laryngol Otol. 1992;106(1):23–7. Moriyama H, Hesaka H, Tachibana T, Honda Y. Mucoceles of ethmoid and sphenoid sinus with visual disturbance. Arch Otolaryngol Head Neck Surg 1992; 118(2):142– 6. Natvig K, Larsen T. Mucocele of the paranasal sinuses: retrospective clinical and histological study. J Laryngol Otol 1978;92:1075– 82. Nugent GR, Sprinkle P, Bloor BM. Sphenoid sinus mucoceles. J Neurosurg 1970;32:443–51. Oruckaptan HH, Akdemir P, Ozgen T. Isolated sphenoid sinus abscess: clinical and radiological failure in preoperative diagnosis. Surg Neurol 2000;53:174 –7. Osborn AG, Johnson L, Roberts TS. Sphenoidal mucoceles with intracranial extension. J Comput Assist Tomogr 1979;3(3):335– 8. Phelps PD, Toland JA. Mucocele of the sphenoidal sinus eroding the petrous temporal bone. Br J Radiol 1969;42(503):845–7. Simms NM, Willis EB, French LA. Mucocele of the sphenoid sinus presenting as an intrasellar mass. J Neurosurg 1970;32:708 –10. Valvassori GE, Putterman AM. Ophthalmological and radiological findings in sphenoidal mucoceles. Arch Ophthalmol 1973;90(6):456 –9. Van Tassel P, Lee YY, Jing BS, De Pena CA. Mucoceles of the paranasal sinuses: MR imaging with CT correlation. AJNR 1989;10:607–12. Yamaguchi K, Ohnuma I, Takahashi S, Fuse T, Aoyagi M, Nagahata M, Hosoya T. Magnetic resonance imaging in acute optic neuropathy by sphenoidal mucocele. Int Ophthalmol 1997;21(1):9 –11.

COMMENTARY

I believe this article is a welcome and long overdue addition to the neurosurgical literature. Although periorbital sinus mucoceles occur in only 4% of all orbital disease patients, they are an important sinoorbital disorder with regard to their clinical mani-

Surg Neurol 343 2001;56:338 –43

festations, symptomatology, frequent delay in diagnosis, and potential for significant and permanent visual dysfunction. Suspicion of mucoceles as the underlying etiology of orbital disease is low on the differential diagnosis list since, as the authors noted, mucoceles rarely affect the orbit and sphenoid mucoceles in particular are extremely uncommon. When there is a delay in diagnosis or the treatment of a sphenoid sinus mucocele or mucopyocele is incomplete, it may lead to permanent visual impairment or disease recurrence. In fact, the permanent visual impairment that results from delay in diagnosis and/or inadequate primary management is responsible for the disproportionately large number of both mucocele and posterior ethmoid and sphenoid cellulitis cases (which may arise from infected mucoceles) that end up in successful plaintiff malpractice litigation. When compared to sterile mucoceles, infected mucoceles (mucopyoceles) tend to evolve much more rapidly in terms of the onset and manifestation of symptoms; furthermore, they carry a much greater risk with regard to the development of sudden and permanent visual dysfunction. Because by definition mucoceles are not infected, there is no real role for antibiotics in their management. Prolonged antibiotic therapy in addition to surgical drainage, as recommended by the authors, is only useful in the treatment of mucopyoceles. I congratulate the authors for bringing this visionthreatening disease to the neurosurgical literature. As the authors note, it is extremely important that this disorder be diagnosed quickly and treated rapidly by surgical means, with appropriate supportive antibiotic therapy if the mucocele is infected, at the instant any symptom develops that may indicate potential compromise of normal visual function. Robert A. Weiss, M.D. Chicago Eye Institute Chicago, Illinois