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MUCOCELES OF THE SPHENOID SINUS ROBERT G. WEAVER, MD BY INVITATION
GEORGE A. GATES, 'MD SAN ANTONIO, TEXAS Mucoceles of the sphenoid sinuses are uncommon; 60 cases have been reported In the world literature to date. Signs and symptoms are caused by local expansion of the mucocele and Include headache (the most common symptom), ocular field deficits, ellternal ophthalmoplegia (particularly the sixth cranial nerve), and proptosis. Radiologic correlation In the form of plain films and polytomography is the most reliable guide in the diagnosis of sphenoid sinus disease. Therapy of these lesions Is surgical, and drainage can be achieved via the external ethmoid or sublablal transseptal route.
Pithology
INTRODUCTION
The neuropathologic conditions associated with sphenoid mucocele are neurapraxic in nature and probably result from pressure occlusion of the vasa nervorum. The neuropathy generally recovers if the mucocele is removed. It is conceivable that with una bating vascular embarrassment, axonal degeneration could occur.
Mucoceles are chronic, expansile, cystlike lesions of the paranasal sinuses,s containing sterile mucoid secretions from the mucosa of the involved sinus. As the mass expands, pressure results in resorption and eventual erosion of the bony walls. The immediate perisphenoid area contains 13 important structures that are subject to injury by the expanding mucocele. They include the second through Three cases are presented: two had classical sixth cranial nerves in their adjacent bony findings and the third was conspicuous by the ' canals (optic foramen and the superior presence of aseptic meningitis in the absence orbital fissure), the dura mater, pituitary of local findings. gland, cavernous sinus, carotid artery, sphenopalatine ganglion and artery, and the pterygoid canal and its nerve.'
History INTEREST in sphenoid sinus disorders began in 1872 when Rouge' noted pus in the sphenoid sinus during a cadaver dissection. Berg,2 a Swedish surgeon, was the first to describe an isolated sphenoid mucocele, and Schuller.! initially described increased radiologic density within the sphenoid sinus. Meisels," gave a detailed description of the bony changes seen secondary to expansion of a sphenoid mucocele.
Submitted for publication Sept 12, 1978. From the Division ofOtorhinolaryngology, University of Texas Health Science Center, San Antonio. Presented at the 1978 Annual meeting of the Ameriean Academy ofOtolaryngology, las Vegas, Septl()' 13.
SIGNS AND SYMPTOMS
The clinical signs and symptoms of sphenoid mucocele occur from expansion of ,the lesion. into adjacent areas. Because' of their direct proximity, the second through sixth cranial nerves are at risk. Bilateral cranial nerve involvement may be seen, but unilateral symptoms are more commonplace. Headache is a frequent clinical symptom. f f In a review 0 S5 patients 0 posterior ethmoid and sphenoid mucoceles, Lund-
Oto/aryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
MUCOCELES OF SPHENOID SINUS gren and Olin" found the most universal symptom to be headache. This was generally seen in a retro-ocular and temporoparietal distribution on the side of the lesion. Other common symptoms resulted from internal and, more commonly, external ophthalmoplegias. Proptosis was frequently observed. Ocular field deficits were discovered in 45% (25) of these patients, with total blindness seen in seven patients. Only nine patients gave histories of chronic nasal obstruction or sinusitis. Endocrine symptoms and intracranial extension are exceedingly rare.
RADIOLOGIC FINDINGS
Sphenoid sinus mucoceles generally produce a definite syndrome, with associated radiologic evidence sufficient for diagnosls.t Plain radiographs of the paranasal sinuses are the mainstay of radiologic diagnosis of sphenoid sinus disease. The submental-vertex view is especially informative. Hypocycloidal polytomography enhances the ability to evaluate the size and the expansion of these lesions into contiguous areas. In general, correlation with polytomographic and surgical findings has been excellent. Computed axial tomography appears to offer little advantage over standard polytomographic techniques. Radiologic evaluation should not be considered complete until the following structures can be reliably identified: superior orbital fissures, optic foramina, lateral walls of the ethmoid sinuses, floor of the sella, walls of the sphenoid sinus, walls of the orbit, and the lesser wing of the sphenoid bone. Distinctive radiographic features of sphenoid sinus mucocele include a soft tissue mass within the sinus, bulging, thin contours, destroyed septa, and rarefaction with deformation of adjacent bony structures.' The most common path of expansion follows the line of least resistance, which is forward
169
into the orbit and posterior ethmoid cells, thus producing the ocular symptoms and signs observed previously.
CASE REPORTS CASE 1.-A 75-year-old woman who had had diplopia on left lateral gaze and a left temporal headache for five days was seen by an ophthalmologist. An isolated left sixth nerve palsy was noted. Visual fields and fundus examinations were normal, and no proptosis was observed. With the exception of adult-onset diabetes, the patient's health was good. A brain scan, a CT scan, and arteriograms failed to reveal a lesion, but polytomograms revealed an expanding lesion of the left sphenoid sinus with disruption of the lateral wall of the sphenoid and irregularity in the floor of the sella (Fig 1). A diagnosis of probable sphenoid mucocele was made. An external ethmoid approach to the sphenoid was performed, and posterior ethmoidal cells contained minimally inflamed mucosa. Upon entering the sphenoid, a mucopyocele was encountered. This was excised, and wide drainage into the nose was established. Postoperatively, the sixth nerve palsy resolved immediately. Final pathologic diagnosis revealed an aspergillus fungus ball as the probable cause of the mucopyocele. The patient was discharged after a short postoperative stay and has done well, with postoperative follow-up carried out to 18 months without evidence of recurrence.
CASE 2.-A 60-year-old woman was referred because of painful proptosis of the right eye, right temporoparietal headache, and chronic right nasal obstruction of six months duration. Except for adult-onset diabetes, the patient's health was good. Examination revealed right proptosis with intense pain to palpation. Funduscopic examination revealed minimal diabetic changes. Visual fields and extraocular movements were normal. Nasal examination showed no polyposis or suppuration; plain sinus films were normal. Polytomograms of the orbit, ethmoid, and sphenoid sinuses showed an opacity In the anterior portion of the right sphenoid sinus without evidence of local expansion (Fig 2). A diagnosis of mucocele was made.
An exploration via an external ethmoid approach was done, and the ethmoid labyrinth was found to be normal. However, upon entering the sphenoid sinus, thickened mucosa and viscid yellowish-brown fluid was noted, but no
Oto/aryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
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Fig 1.-Anterior-posterior polytomogram suggesting disruption of the lateral wall of the sphenoid with generalized left-sided opacity of sphenoid sinus.
Fig 2.-Anlerior-posterior polytomogram revealing right-sided sphenoid sinus opacity without evidence of local bony erosion.
pus was encountered. Wide drainage into the nose was established. The patient had immediate postoperative resolution of her symptoms and was discharged after a short postoperative stay. Six- and 12-month follow-up visits revealed no evidence of recurrence. CASE 3.-A 21-year-old woman had had headaches, meningismus, photophobia, and intermittent chilling for five days. The patient was admitted to the hospital with the diagnosis of meningitis. Neurologic examination, including cranial nerve testing and funduscopic examination, revealed no abnormalities; proptosis was not noted. The patient was treated with oxacillin and chloramphenicol after a
cerebrospinal fluid (CSF) gram stain revealed no organisms. Repeat CSF studies failed to produce an identifiable organism, although, the white blood cell counts in the CSF were continually above 200/cu mm. The headaches and photophobia continued while the nuchal rigidity resolved. On the fifth day of hospitalization, a sphenoid sinus opacity was noted on a routine sinus series. Sphenoid polytomograms confirmed the sphenoid opacity but adjacent expansion was not noted (Fig 3). A provisional diagnosis of mucocele was made. Using a sublabial transseptal approach, a sphenoid mucocele was encountered and removed. A septal mucosal flap based posterosuperiorly was elevated and laid back into the
Otolaryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
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171
Fig 3.-Polytomographic section revealing generalized sphenoid opacity without local bony destruction. Note distinctiveness of superior orb ital fissures, foram ina rotundi, and pterygoid canals.
sphenoid to aid in reepithelialization and to insure drainage into the nose. The patient had immediate resolution of her headache and photophobia; packing was removed after 72 hours, and the patient discharged. Follow-up to eight months has shown no recurrence.
DISCUSSION Mucoceles of the sphenoid sinus must be differentiated primarily from neoplasms arising in the pituitary or closely adjacent areas. These include craniopharyngioma, meningioma, glioma, intracranial cordoma, cholesteatoma, and nasopharyngeal neoplasms." In general, the expansile characteristics of these lesions can be accurately separated radiographically from sphenoid mucoceles. The chromophobe adenoma is the most likely lesion to radiologically simulate a mucocele. Treatment of these lesions is surgical and requires wide drainage into the nose. An external ethmoid approach through a subbrow incision should be used if orbital or posterior ethmoid involvement is suspected. This allows excellent exposure to the ethmoid complex and sphenoid sinus. The incision is M-shaped to facilitate closure and avoid postoperative scar contracture.
In the case of isolated sphenoid sinus disease, the sublabial transseptal approach provides straightforward access to the sphenoid with good visibility. Bleeding is generally negligible, and undesirable injury to neighboring structures is avo ided. As the incision is sublabial, no cosmetic defect ,is expected . In each case, wide drainage into the nose is established. Mucosal flaps are packed into place and left undisturbed for three to four days, at which time the pack ing is removed and the patient is ready for dismissal. The use of polyethylene catheters to maintain drainage has been popularized in the past. This procedure was found to be unnecessary in the three cases presented.
COMMENT The cause of mucoceles remains obscure. One theory suggests that the mucocele develops from goblet cell hypertrophy. Another more generally conceded theory is that of outlet obstruction from several causes, le, trauma, tumor, or inflammation. Schenck et al" have experimentally produced mucoceles by obstructing the nasofrontal duct in dogs, thus lending some objective credibility to
Otolaryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
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the "obstruction" theory. Without a proven history of trauma, sphenoid mucoceles are generally believed to arise from obstruction secondary to chronic inflammation in the area of the sinus itself. The diagnosis of sphenoid sinus disease poses problems not encountered in chronic infections of other paranasal sinuses because of its relative inaccessability to examination and rarity of involvement by disease. Therefore, the first problem is localizing the lesion to the sphenoid sinus on the basis of symptoms and physical findings. The second problem is determining the cause of the lesion (either inflammatory or neoplastic), and, third, whether the disease process is primary or secondary to sphenoid bone invasion. Van Alyea12 commented on the role played by the sphenoid sinus in vague, but harassing, headache and warned that one must develop the habit of suspecting and investigating the sphenoid in cases of deep-seated headache with an undetermined cause. Proetz6 also emphasized, when discussing problems peculiar to this sinus, the particular anatomy, physiology, and proximity of the sphenoid to its 13 important and vulnerable neighboring structures. Although this entity is rare, its presence must be suspected in patients who have headache in combination with localized ocular findings and in the absence of nasal obstructive pathology. If it is considered, sphenoid mucocele can generally be proved or disproved with appropriate radiographic studies. Patients with this disease will frequently visit an ophthalmologist, initially, because of the ocular symptomatology; this was the case in two of the three patients. The third patient was of interest because of the absence of usual signs and symptoms and the presence of aseptic meningitis. Basilar meningitis has been reported associated with sphenoid inflammatory
processes, apparently from extension to the perichiasmatic clsterns.n Whether this was the case in this patient is only speculative. It does illustrate, however, the need for evaluation of the paranasal sinuses in every case of suspected or proved meningitis.
SUMMARY Sphenoid mucocele is a rare entity with 60 cases noted in the world literature. Outstanding signs and symptoms include headache, proptosis, ophthalmoplegias, and ocular field deficits. Radiographic correlation provides the cornerstone of diagnosis of sphenoid sinus disease, with surgical treatment via the external ethmoid or sublabial transseptal route the basic treatment for this lesion.
REFERENCES
1. Rouge, cited by Grunwald L: Die Lehre yon den Naseneiterungen. Munich, JF Lehman, 1896, p 242. 2. Berg J: Bidrag till kannedomen om sjuk domarna i nasans bihalor samt till laran om cerebrospinal vatskas flytning ur nasal. II. Hydrops sinus sphenoidalis m. Trepanation av kilbenshalan, halsa. Nordiskt Medicinskt Arkiy 3:5-24, 1889. 3. Schuller A: Sphenoidale mucocele oder zystischer hypophysentumor? Mschr Ohrenheilk 66:166·172, 1932. 4. Meisels El: Mukozele der keilbenhohle. Fortschr Ronlgenslr 34:905-908, 1926.
5. Canalis RF, Zajtchuk IT, lenkins AH: Ethmoidal mucoceles. Arch Oto/aryngo/104:286291, 1978. 6. Proetz AW: Sphenoid sinus. Br Med J 2:243-245, 1948. 7. Lundgren A, Olin T: Muco-pyocele of sphenoidal sinus or posterior ethmoidal cells with special reference to apex orbitae syndrome. Acra Olo/aryngol (Slockh) 53:61-79, 1961. 8. Simon HM, Tingwald FR: Syndrome associated with mucocele of sphenoid sinus: Report of 2 cases and their radiographic findings. Radiology 64:538-545, 1955.
Oto/aryngol Head Neck Sutg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
MUCOCELES OF SPHENOID SINUS 9. Phelps PO, Toland jA: Mucocele of the sphenoidal sinus eroding the petrous temporal bone. Br J Radial 42:845-847, 1969. 10. Maxwell jH, Hill B]: The diagnosis of chronic inflammatory lesions of the sphenoid sinus. Ann 0101 Rhinol Laryngol 68:411-452, 1959. 11. Schenck NL, Rauchback E, Ogura jH: Frontal sinus disease: II. Development of the
173
frontal sinus model: Occlusion of the nasofrontal duct. Laryngoscope 84:1233-1247, 1974. 12. Van Alyea OE: Sphenoid sinus: Anatomic study, with consideration of the clinical significance of the structural characteristics of the sphenoid sinus. Arch Olo/aryngol 34:225253, 1941. 13. Farooki NQ, Brodovsky OM, Fewer 0: Mucocele of the sphenoid sinus presenting as spontaneous pneumocephalus. J Olo/aryngol 5:350-354, 1976.
Otolaryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
MUCOCELES OF THE SPHENOID SINUS ROBERT G. WEAVER, MD BY INVITATION
GEORGE A. GATES, 'MD SAN ANTONIO, TEXAS Mucoceles of the sphenoid sinuses are uncommon; 60 cases have been reported In the world literature to date. Signs and symptoms are caused by local expansion of the mucocele and Include headache (the most common symptom), ocular field deficits, ellternal ophthalmoplegia (particularly the sixth cranial nerve), and proptosis. Radiologic correlation In the form of plain films and polytomography is the most reliable guide in the diagnosis of sphenoid sinus disease. Therapy of these lesions Is surgical, and drainage can be achieved via the external ethmoid or sublablal transseptal route.
Pithology
INTRODUCTION
The neuropathologic conditions associated with sphenoid mucocele are neurapraxic in nature and probably result from pressure occlusion of the vasa nervorum. The neuropathy generally recovers if the mucocele is removed. It is conceivable that with una bating vascular embarrassment, axonal degeneration could occur.
Mucoceles are chronic, expansile, cystlike lesions of the paranasal sinuses,s containing sterile mucoid secretions from the mucosa of the involved sinus. As the mass expands, pressure results in resorption and eventual erosion of the bony walls. The immediate perisphenoid area contains 13 important structures that are subject to injury by the expanding mucocele. They include the second through Three cases are presented: two had classical sixth cranial nerves in their adjacent bony findings and the third was conspicuous by the ' canals (optic foramen and the superior presence of aseptic meningitis in the absence orbital fissure), the dura mater, pituitary of local findings. gland, cavernous sinus, carotid artery, sphenopalatine ganglion and artery, and the pterygoid canal and its nerve.'
History INTEREST in sphenoid sinus disorders began in 1872 when Rouge' noted pus in the sphenoid sinus during a cadaver dissection. Berg,2 a Swedish surgeon, was the first to describe an isolated sphenoid mucocele, and Schuller.! initially described increased radiologic density within the sphenoid sinus. Meisels," gave a detailed description of the bony changes seen secondary to expansion of a sphenoid mucocele.
Submitted for publication Sept 12, 1978. From the Division ofOtorhinolaryngology, University of Texas Health Science Center, San Antonio. Presented at the 1978 Annual meeting of the Ameriean Academy ofOtolaryngology, las Vegas, Septl()' 13.
SIGNS AND SYMPTOMS
The clinical signs and symptoms of sphenoid mucocele occur from expansion of ,the lesion. into adjacent areas. Because' of their direct proximity, the second through sixth cranial nerves are at risk. Bilateral cranial nerve involvement may be seen, but unilateral symptoms are more commonplace. Headache is a frequent clinical symptom. f f In a review 0 S5 patients 0 posterior ethmoid and sphenoid mucoceles, Lund-
Oto/aryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
MUCOCELES OF SPHENOID SINUS gren and Olin" found the most universal symptom to be headache. This was generally seen in a retro-ocular and temporoparietal distribution on the side of the lesion. Other common symptoms resulted from internal and, more commonly, external ophthalmoplegias. Proptosis was frequently observed. Ocular field deficits were discovered in 45% (25) of these patients, with total blindness seen in seven patients. Only nine patients gave histories of chronic nasal obstruction or sinusitis. Endocrine symptoms and intracranial extension are exceedingly rare.
RADIOLOGIC FINDINGS
Sphenoid sinus mucoceles generally produce a definite syndrome, with associated radiologic evidence sufficient for diagnosls.t Plain radiographs of the paranasal sinuses are the mainstay of radiologic diagnosis of sphenoid sinus disease. The submental-vertex view is especially informative. Hypocycloidal polytomography enhances the ability to evaluate the size and the expansion of these lesions into contiguous areas. In general, correlation with polytomographic and surgical findings has been excellent. Computed axial tomography appears to offer little advantage over standard polytomographic techniques. Radiologic evaluation should not be considered complete until the following structures can be reliably identified: superior orbital fissures, optic foramina, lateral walls of the ethmoid sinuses, floor of the sella, walls of the sphenoid sinus, walls of the orbit, and the lesser wing of the sphenoid bone. Distinctive radiographic features of sphenoid sinus mucocele include a soft tissue mass within the sinus, bulging, thin contours, destroyed septa, and rarefaction with deformation of adjacent bony structures.' The most common path of expansion follows the line of least resistance, which is forward
169
into the orbit and posterior ethmoid cells, thus producing the ocular symptoms and signs observed previously.
CASE REPORTS CASE 1.-A 75-year-old woman who had had diplopia on left lateral gaze and a left temporal headache for five days was seen by an ophthalmologist. An isolated left sixth nerve palsy was noted. Visual fields and fundus examinations were normal, and no proptosis was observed. With the exception of adult-onset diabetes, the patient's health was good. A brain scan, a CT scan, and arteriograms failed to reveal a lesion, but polytomograms revealed an expanding lesion of the left sphenoid sinus with disruption of the lateral wall of the sphenoid and irregularity in the floor of the sella (Fig 1). A diagnosis of probable sphenoid mucocele was made. An external ethmoid approach to the sphenoid was performed, and posterior ethmoidal cells contained minimally inflamed mucosa. Upon entering the sphenoid, a mucopyocele was encountered. This was excised, and wide drainage into the nose was established. Postoperatively, the sixth nerve palsy resolved immediately. Final pathologic diagnosis revealed an aspergillus fungus ball as the probable cause of the mucopyocele. The patient was discharged after a short postoperative stay and has done well, with postoperative follow-up carried out to 18 months without evidence of recurrence.
CASE 2.-A 60-year-old woman was referred because of painful proptosis of the right eye, right temporoparietal headache, and chronic right nasal obstruction of six months duration. Except for adult-onset diabetes, the patient's health was good. Examination revealed right proptosis with intense pain to palpation. Funduscopic examination revealed minimal diabetic changes. Visual fields and extraocular movements were normal. Nasal examination showed no polyposis or suppuration; plain sinus films were normal. Polytomograms of the orbit, ethmoid, and sphenoid sinuses showed an opacity In the anterior portion of the right sphenoid sinus without evidence of local expansion (Fig 2). A diagnosis of mucocele was made.
An exploration via an external ethmoid approach was done, and the ethmoid labyrinth was found to be normal. However, upon entering the sphenoid sinus, thickened mucosa and viscid yellowish-brown fluid was noted, but no
Oto/aryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
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Fig 1.-Anterior-posterior polytomogram suggesting disruption of the lateral wall of the sphenoid with generalized left-sided opacity of sphenoid sinus.
Fig 2.-Anlerior-posterior polytomogram revealing right-sided sphenoid sinus opacity without evidence of local bony erosion.
pus was encountered. Wide drainage into the nose was established. The patient had immediate postoperative resolution of her symptoms and was discharged after a short postoperative stay. Six- and 12-month follow-up visits revealed no evidence of recurrence. CASE 3.-A 21-year-old woman had had headaches, meningismus, photophobia, and intermittent chilling for five days. The patient was admitted to the hospital with the diagnosis of meningitis. Neurologic examination, including cranial nerve testing and funduscopic examination, revealed no abnormalities; proptosis was not noted. The patient was treated with oxacillin and chloramphenicol after a
cerebrospinal fluid (CSF) gram stain revealed no organisms. Repeat CSF studies failed to produce an identifiable organism, although, the white blood cell counts in the CSF were continually above 200/cu mm. The headaches and photophobia continued while the nuchal rigidity resolved. On the fifth day of hospitalization, a sphenoid sinus opacity was noted on a routine sinus series. Sphenoid polytomograms confirmed the sphenoid opacity but adjacent expansion was not noted (Fig 3). A provisional diagnosis of mucocele was made. Using a sublabial transseptal approach, a sphenoid mucocele was encountered and removed. A septal mucosal flap based posterosuperiorly was elevated and laid back into the
Otolaryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
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171
Fig 3.-Polytomographic section revealing generalized sphenoid opacity without local bony destruction. Note distinctiveness of superior orb ital fissures, foram ina rotundi, and pterygoid canals.
sphenoid to aid in reepithelialization and to insure drainage into the nose. The patient had immediate resolution of her headache and photophobia; packing was removed after 72 hours, and the patient discharged. Follow-up to eight months has shown no recurrence.
DISCUSSION Mucoceles of the sphenoid sinus must be differentiated primarily from neoplasms arising in the pituitary or closely adjacent areas. These include craniopharyngioma, meningioma, glioma, intracranial cordoma, cholesteatoma, and nasopharyngeal neoplasms." In general, the expansile characteristics of these lesions can be accurately separated radiographically from sphenoid mucoceles. The chromophobe adenoma is the most likely lesion to radiologically simulate a mucocele. Treatment of these lesions is surgical and requires wide drainage into the nose. An external ethmoid approach through a subbrow incision should be used if orbital or posterior ethmoid involvement is suspected. This allows excellent exposure to the ethmoid complex and sphenoid sinus. The incision is M-shaped to facilitate closure and avoid postoperative scar contracture.
In the case of isolated sphenoid sinus disease, the sublabial transseptal approach provides straightforward access to the sphenoid with good visibility. Bleeding is generally negligible, and undesirable injury to neighboring structures is avo ided. As the incision is sublabial, no cosmetic defect ,is expected . In each case, wide drainage into the nose is established. Mucosal flaps are packed into place and left undisturbed for three to four days, at which time the pack ing is removed and the patient is ready for dismissal. The use of polyethylene catheters to maintain drainage has been popularized in the past. This procedure was found to be unnecessary in the three cases presented.
COMMENT The cause of mucoceles remains obscure. One theory suggests that the mucocele develops from goblet cell hypertrophy. Another more generally conceded theory is that of outlet obstruction from several causes, le, trauma, tumor, or inflammation. Schenck et al" have experimentally produced mucoceles by obstructing the nasofrontal duct in dogs, thus lending some objective credibility to
Otolaryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
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the "obstruction" theory. Without a proven history of trauma, sphenoid mucoceles are generally believed to arise from obstruction secondary to chronic inflammation in the area of the sinus itself. The diagnosis of sphenoid sinus disease poses problems not encountered in chronic infections of other paranasal sinuses because of its relative inaccessability to examination and rarity of involvement by disease. Therefore, the first problem is localizing the lesion to the sphenoid sinus on the basis of symptoms and physical findings. The second problem is determining the cause of the lesion (either inflammatory or neoplastic), and, third, whether the disease process is primary or secondary to sphenoid bone invasion. Van Alyea12 commented on the role played by the sphenoid sinus in vague, but harassing, headache and warned that one must develop the habit of suspecting and investigating the sphenoid in cases of deep-seated headache with an undetermined cause. Proetz6 also emphasized, when discussing problems peculiar to this sinus, the particular anatomy, physiology, and proximity of the sphenoid to its 13 important and vulnerable neighboring structures. Although this entity is rare, its presence must be suspected in patients who have headache in combination with localized ocular findings and in the absence of nasal obstructive pathology. If it is considered, sphenoid mucocele can generally be proved or disproved with appropriate radiographic studies. Patients with this disease will frequently visit an ophthalmologist, initially, because of the ocular symptomatology; this was the case in two of the three patients. The third patient was of interest because of the absence of usual signs and symptoms and the presence of aseptic meningitis. Basilar meningitis has been reported associated with sphenoid inflammatory
processes, apparently from extension to the perichiasmatic clsterns.n Whether this was the case in this patient is only speculative. It does illustrate, however, the need for evaluation of the paranasal sinuses in every case of suspected or proved meningitis.
SUMMARY Sphenoid mucocele is a rare entity with 60 cases noted in the world literature. Outstanding signs and symptoms include headache, proptosis, ophthalmoplegias, and ocular field deficits. Radiographic correlation provides the cornerstone of diagnosis of sphenoid sinus disease, with surgical treatment via the external ethmoid or sublabial transseptal route the basic treatment for this lesion.
REFERENCES
1. Rouge, cited by Grunwald L: Die Lehre yon den Naseneiterungen. Munich, JF Lehman, 1896, p 242. 2. Berg J: Bidrag till kannedomen om sjuk domarna i nasans bihalor samt till laran om cerebrospinal vatskas flytning ur nasal. II. Hydrops sinus sphenoidalis m. Trepanation av kilbenshalan, halsa. Nordiskt Medicinskt Arkiy 3:5-24, 1889. 3. Schuller A: Sphenoidale mucocele oder zystischer hypophysentumor? Mschr Ohrenheilk 66:166·172, 1932. 4. Meisels El: Mukozele der keilbenhohle. Fortschr Ronlgenslr 34:905-908, 1926.
5. Canalis RF, Zajtchuk IT, lenkins AH: Ethmoidal mucoceles. Arch Oto/aryngo/104:286291, 1978. 6. Proetz AW: Sphenoid sinus. Br Med J 2:243-245, 1948. 7. Lundgren A, Olin T: Muco-pyocele of sphenoidal sinus or posterior ethmoidal cells with special reference to apex orbitae syndrome. Acra Olo/aryngol (Slockh) 53:61-79, 1961. 8. Simon HM, Tingwald FR: Syndrome associated with mucocele of sphenoid sinus: Report of 2 cases and their radiographic findings. Radiology 64:538-545, 1955.
Oto/aryngol Head Neck Sutg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
MUCOCELES OF SPHENOID SINUS 9. Phelps PO, Toland jA: Mucocele of the sphenoidal sinus eroding the petrous temporal bone. Br J Radial 42:845-847, 1969. 10. Maxwell jH, Hill B]: The diagnosis of chronic inflammatory lesions of the sphenoid sinus. Ann 0101 Rhinol Laryngol 68:411-452, 1959. 11. Schenck NL, Rauchback E, Ogura jH: Frontal sinus disease: II. Development of the
173
frontal sinus model: Occlusion of the nasofrontal duct. Laryngoscope 84:1233-1247, 1974. 12. Van Alyea OE: Sphenoid sinus: Anatomic study, with consideration of the clinical significance of the structural characteristics of the sphenoid sinus. Arch Olo/aryngol 34:225253, 1941. 13. Farooki NQ, Brodovsky OM, Fewer 0: Mucocele of the sphenoid sinus presenting as spontaneous pneumocephalus. J Olo/aryngol 5:350-354, 1976.
Otolaryngol Head Neck Surg 87:168-173 (Mar-Apr) 1979 Downloaded from oto.sagepub.com at University of Otago Library on June 5, 2016
