1979, British Journal of Radiology, 52, 998-1001 Case reports
Mucocoele of the sphenoid sinus complicating fibrous dyspiasia By S. Birch, B.Sc, M.B., B.S. and P. L. Cook, F.R.C.R. Wessex Neurological Centre, Southampton General Hospital {Received October 1978 and in revised form June 1979)
Mucocoeles of the sphenoid sinus are rare (Nefson, 1957). A case is presented where such a lesion occurred in an area affected by fibrous dyspiasia, a previously unreported combination.
outstanding clinical feature was headache, characteristically frontal or periorbital and unilateral. This was not so in the present patient, but her presentation with progressive bilateral second and third nerve palsies and external ophthalmoplegia is otherCASE HISTORY wise typical. A 25-year-old woman was referred to the Wessex Neurological Centre having rapidly become blind in the left eye This is confirmed by other authors (Nefson, 1957; ten days earlier. The vision in her right eye then deteriorated Hayes, 1964; Hakuba et al., 1975) and they emprogressively such that on admission she was completely blind. There was no history of earlier visual disturbance, phasize the difficulty of distinguishing from ophthalchronic sinus disease or craniofacial trauma. Examination moplegic migraine if there be spontaneous remission, confirmed that she was totally blind and the pupils were fixed and dilated. There was slight left proptosis but no or more commonly from an intracranial lesion, orbital or facial asymmetry. usually a pituitary tumour. Skull radiographs demonstrated density and obliteration As with the more common mucocoeles of the of the left ethmoidal air cells especially the posterior group. There was bulging and partial bony destruction of the orbital other paranasal sinuses, (Jackson, 1977), there is plate of the left ethmoid extending to the nasal septum with frequently a history of allergic rhinitis, nasal polyps obliteration of the left nasal airway. The basisphenoid was or chronic sinusitis. There was no such history in the also destroyed across the full width of the floor of the pituitary fossa with involvement of the left optic canal and present case. Mucocoeles are usually considered to the medial half of the right optic canal. There was marked be simple retention cysts of the sinuses resulting developmental asymmetry of the lesser wings of the sphenoid. Computerized tomographic (CT) scanning con- when the drainage is blocked. They may, however, firmed the plain radiographic findings. In particular, the left occur when an ostium is open and blockage of an ethmoidal and orbital deformities, together with the ostium does not always result in a mucocoele. destruction of the optic canals, were well shown in the Fibrous dyspiasia, causing sclerosis and expansion transverse axial plane. The left eyeball was distorted by local pressure and the left optic nerve was deviated. of the skull base, is seen fairly frequently (Leeds and The sclerosis and more particularly the sphenoid and orbital deformities were taken to indicate longstanding Seaman, 1962). Samy et al. (1967) and Grabias and abnormality. Fibrous dyspiasia was regarded as a possibility. Campbell (1977) have suggested that an expanding The destructive element was obviously more recent and bone lesion of this type could occlude the ostium of associated with the rapid clinical change. It was thought possible that malignant change had taken place in the under- the sphenoid sinus. We cannot, however, find any lying lesion (Jaffe, 1958; Aegerter and Kirkpatrick, 1963; reference to the associated development of a mucoGreenfield, 1969). coele and Nugent et al. (1970) do not mention this In an attempt to relieve the pressure on the optic nerves, radiotherapy was immediately instituted. Subsequent biopsy possibility in their review. Other foramina, e.g. the of the posterior ethmoids showed fibrous dyspiasia. A bi- optic canals, not uncommonly show narrowing in frontal craniotomy was performed and both optic nerves association with the bone thickening of fibrous were found to be encircled by a mucocoele of the sphenoid sinus. Haemorrhage had occurred into the mucocoele and dyspiasia (Leeds and Seaman, 1962). In the present this was felt to be the reason for the sudden deterioration of case, the absence of any other cause of occlusion of her vision. The optic nerves were decompressed, but her the sphenoid sinus suggests that this might have visual function did not recover. occurred. This would be similar to the development of a mucocoele of the frontal sinus when the frontoDISCUSSION Mucocoeles of the sphenoid sinus are uncommon, nasal duct is occluded by an osteoma (Jackson, 1977). The radiological features of mucocoeles of the the series of Zizmor and Noyek (1968) suggesting that they constitute 1 % of all mucocoeles. Nugent sphenoid sinus were described by Norman and et al. (1970), reviewing the world literature, found a Yanagisawa (1964) and Hakuba et al. (1975). The total of eighty-five case reports. In nineteen of these sinus is opaque with destruction of its interseptum the clinical information was sparse and these and the floor of the pituitary fossa. This detogether with three reports that were not translated struction may extend into the posterior clivus and were eliminated from their analysis which was dorsum sellae or forwards into the anterior clinoid confined to the remaining sixty-three patients. The processes. The optic foramen and superior orbital 998
DECEMBER 1979
Case reports
FIG. 1. (A) A view of the skull. (B) Full axial view of the skull. Showing increased bone density and thickening obliterating the left ethmoid air cells and left nasal airway with deviation of the nasal septum. There is marked asymmetry of the sphenoid wings (arrows). (c) Biopsy of the tumour showed numerous irregular bony trabeculae set in a vascular fibrous stroma. H & E X 125. (D) High power detail of top right hand corner of A. showing the lack of appositional osteoblastic activity along the margins of the bone. H & E x 320.
999
VOL.
52, No. 624 Case reports
B
2. Lateral views of the skull (A) showing opacity of sphenoid sinus with destruction of the floor of the pituitary fossa extending to the dorsum sellae. (B) showing post operative reconstitution of the pituitary fossa and reaeration of the sphenoid sinus. FIG.
FIG. 3. Views of the optic canals showing complete loss of definition of the left canal and loss of the medial margins of the right canal. 1000
DECEMBER 1979
Case reports 4. CT Scan views showing bony obliteration of the left ethmoid air cells and upper nasal airway with encroachment on the left orbit and displacement of orbital contents. The thickening extends posteriorly as far as the optic canal but the apex of the orbit is unaffected. The dorsum sellae is thinned and there is asymmetry of the sphenoids. FIG.
fissure may be widened and eventual extension into the ethmoid air cells may displace the lamina papyracea into the orbit. In the present patient (Figs. 1-3), the sphenoid sinus was opaque and the floor of the pituitary fossa destroyed with loss of definition of both optic canals. In addition bone sclerosis and thickening due to fibrous dysplasia caused asymmetry of the sphenoid wing and obliterated the left ethmoids and left nasal airway, obscuring some of the features described above. C T scanning (Fig. 4) provided confirmation of the plain film findings and gave additional information about the extent of the lesion. T h e destruction of the sphenoid and the optic canals was demonstrated and the displacement of the orbital contents was shown to be due to bone thickening and not to direct invasion of either orbital apex. Nevertheless the exact nature of the destruction could not be diagnosed. REFERENCES AEGERTER, E. and KIRKPATRICK, J., 1973. Orthopaedic
Disease (W. B. Saunders, Philadelphia). GRABIAS, S. and CAMPBELL, C , 1977. Fibrous dysplasia.
Orthopedic Clinics of North America, 8, 771-783. GREENFIELD, G. B., 1969. Radiology of Bone Disease (J. B. Lippincott, Philadelphia). HAKUBA, A., KATSUYAMA, J., MATSUOKA, Y., SHINN, J. H.
and NISHIMUSA, S., 1975. Sphenoid sinus mucoceles. Journal of Neurosurgery, 43, 368-373. HAYES, J., 1964. Mucocele of the sphenoid sinus. Archives of Otolaryngology, 79, 653. JACKSON, A., 1977. Bone thinning in frontal mucocele. British Journal of Radiology, 50, 181-184. JAFFE, M. L., 1958. Tumors and Tumorous Conditions of the Bones and Joints (Lea and Febiger, Philadelphia). LEEDS, N. and SEAMAN, W. B., 1962. Fibrous Dysplasia of
the skull and its differential diagnosis. Radiology, 78, 570-582. NEFSON, A. H., 1957. Mucocele of the sphenoid sinus. Archives of Otolaryngology, 66, 1 57—164. NORMAN, P. S. and YANAGISAWA, E., 1964. Mucocele of
sphenoid sinus. Archives of Otolaryngology, 79, 646-656. NUGENT, G. R., SPRINKLE, P. and BLOOR, B. M., 1970.
Sphenoid sinus mucoceles. Journal of Neurosurgery, 32, 443-451. SAMY, L., GINGIS, I. andWASEF, S., 1967. Fibrous dysplasia
in relation to the paranasal sinuses and the ear. Journal of Laryngology and Otology, 81, 1357-1371. ZIZMOR, J. and NOYEK, A., 1968. Cysts and benign tumours
of the paranasal sinuses. Seminars in Roentgenology, 3, 172-201.
1001
Mucocoele of the sphenoid sinus complicating fibrous dyspiasia By S. Birch, B.Sc, M.B., B.S. and P. L. Cook, F.R.C.R. Wessex Neurological Centre, Southampton General Hospital {Received October 1978 and in revised form June 1979)
Mucocoeles of the sphenoid sinus are rare (Nefson, 1957). A case is presented where such a lesion occurred in an area affected by fibrous dyspiasia, a previously unreported combination.
outstanding clinical feature was headache, characteristically frontal or periorbital and unilateral. This was not so in the present patient, but her presentation with progressive bilateral second and third nerve palsies and external ophthalmoplegia is otherCASE HISTORY wise typical. A 25-year-old woman was referred to the Wessex Neurological Centre having rapidly become blind in the left eye This is confirmed by other authors (Nefson, 1957; ten days earlier. The vision in her right eye then deteriorated Hayes, 1964; Hakuba et al., 1975) and they emprogressively such that on admission she was completely blind. There was no history of earlier visual disturbance, phasize the difficulty of distinguishing from ophthalchronic sinus disease or craniofacial trauma. Examination moplegic migraine if there be spontaneous remission, confirmed that she was totally blind and the pupils were fixed and dilated. There was slight left proptosis but no or more commonly from an intracranial lesion, orbital or facial asymmetry. usually a pituitary tumour. Skull radiographs demonstrated density and obliteration As with the more common mucocoeles of the of the left ethmoidal air cells especially the posterior group. There was bulging and partial bony destruction of the orbital other paranasal sinuses, (Jackson, 1977), there is plate of the left ethmoid extending to the nasal septum with frequently a history of allergic rhinitis, nasal polyps obliteration of the left nasal airway. The basisphenoid was or chronic sinusitis. There was no such history in the also destroyed across the full width of the floor of the pituitary fossa with involvement of the left optic canal and present case. Mucocoeles are usually considered to the medial half of the right optic canal. There was marked be simple retention cysts of the sinuses resulting developmental asymmetry of the lesser wings of the sphenoid. Computerized tomographic (CT) scanning con- when the drainage is blocked. They may, however, firmed the plain radiographic findings. In particular, the left occur when an ostium is open and blockage of an ethmoidal and orbital deformities, together with the ostium does not always result in a mucocoele. destruction of the optic canals, were well shown in the Fibrous dyspiasia, causing sclerosis and expansion transverse axial plane. The left eyeball was distorted by local pressure and the left optic nerve was deviated. of the skull base, is seen fairly frequently (Leeds and The sclerosis and more particularly the sphenoid and orbital deformities were taken to indicate longstanding Seaman, 1962). Samy et al. (1967) and Grabias and abnormality. Fibrous dyspiasia was regarded as a possibility. Campbell (1977) have suggested that an expanding The destructive element was obviously more recent and bone lesion of this type could occlude the ostium of associated with the rapid clinical change. It was thought possible that malignant change had taken place in the under- the sphenoid sinus. We cannot, however, find any lying lesion (Jaffe, 1958; Aegerter and Kirkpatrick, 1963; reference to the associated development of a mucoGreenfield, 1969). coele and Nugent et al. (1970) do not mention this In an attempt to relieve the pressure on the optic nerves, radiotherapy was immediately instituted. Subsequent biopsy possibility in their review. Other foramina, e.g. the of the posterior ethmoids showed fibrous dyspiasia. A bi- optic canals, not uncommonly show narrowing in frontal craniotomy was performed and both optic nerves association with the bone thickening of fibrous were found to be encircled by a mucocoele of the sphenoid sinus. Haemorrhage had occurred into the mucocoele and dyspiasia (Leeds and Seaman, 1962). In the present this was felt to be the reason for the sudden deterioration of case, the absence of any other cause of occlusion of her vision. The optic nerves were decompressed, but her the sphenoid sinus suggests that this might have visual function did not recover. occurred. This would be similar to the development of a mucocoele of the frontal sinus when the frontoDISCUSSION Mucocoeles of the sphenoid sinus are uncommon, nasal duct is occluded by an osteoma (Jackson, 1977). The radiological features of mucocoeles of the the series of Zizmor and Noyek (1968) suggesting that they constitute 1 % of all mucocoeles. Nugent sphenoid sinus were described by Norman and et al. (1970), reviewing the world literature, found a Yanagisawa (1964) and Hakuba et al. (1975). The total of eighty-five case reports. In nineteen of these sinus is opaque with destruction of its interseptum the clinical information was sparse and these and the floor of the pituitary fossa. This detogether with three reports that were not translated struction may extend into the posterior clivus and were eliminated from their analysis which was dorsum sellae or forwards into the anterior clinoid confined to the remaining sixty-three patients. The processes. The optic foramen and superior orbital 998
DECEMBER 1979
Case reports
FIG. 1. (A) A view of the skull. (B) Full axial view of the skull. Showing increased bone density and thickening obliterating the left ethmoid air cells and left nasal airway with deviation of the nasal septum. There is marked asymmetry of the sphenoid wings (arrows). (c) Biopsy of the tumour showed numerous irregular bony trabeculae set in a vascular fibrous stroma. H & E X 125. (D) High power detail of top right hand corner of A. showing the lack of appositional osteoblastic activity along the margins of the bone. H & E x 320.
999
VOL.
52, No. 624 Case reports
B
2. Lateral views of the skull (A) showing opacity of sphenoid sinus with destruction of the floor of the pituitary fossa extending to the dorsum sellae. (B) showing post operative reconstitution of the pituitary fossa and reaeration of the sphenoid sinus. FIG.
FIG. 3. Views of the optic canals showing complete loss of definition of the left canal and loss of the medial margins of the right canal. 1000
DECEMBER 1979
Case reports 4. CT Scan views showing bony obliteration of the left ethmoid air cells and upper nasal airway with encroachment on the left orbit and displacement of orbital contents. The thickening extends posteriorly as far as the optic canal but the apex of the orbit is unaffected. The dorsum sellae is thinned and there is asymmetry of the sphenoids. FIG.
fissure may be widened and eventual extension into the ethmoid air cells may displace the lamina papyracea into the orbit. In the present patient (Figs. 1-3), the sphenoid sinus was opaque and the floor of the pituitary fossa destroyed with loss of definition of both optic canals. In addition bone sclerosis and thickening due to fibrous dysplasia caused asymmetry of the sphenoid wing and obliterated the left ethmoids and left nasal airway, obscuring some of the features described above. C T scanning (Fig. 4) provided confirmation of the plain film findings and gave additional information about the extent of the lesion. T h e destruction of the sphenoid and the optic canals was demonstrated and the displacement of the orbital contents was shown to be due to bone thickening and not to direct invasion of either orbital apex. Nevertheless the exact nature of the destruction could not be diagnosed. REFERENCES AEGERTER, E. and KIRKPATRICK, J., 1973. Orthopaedic
Disease (W. B. Saunders, Philadelphia). GRABIAS, S. and CAMPBELL, C , 1977. Fibrous dysplasia.
Orthopedic Clinics of North America, 8, 771-783. GREENFIELD, G. B., 1969. Radiology of Bone Disease (J. B. Lippincott, Philadelphia). HAKUBA, A., KATSUYAMA, J., MATSUOKA, Y., SHINN, J. H.
and NISHIMUSA, S., 1975. Sphenoid sinus mucoceles. Journal of Neurosurgery, 43, 368-373. HAYES, J., 1964. Mucocele of the sphenoid sinus. Archives of Otolaryngology, 79, 653. JACKSON, A., 1977. Bone thinning in frontal mucocele. British Journal of Radiology, 50, 181-184. JAFFE, M. L., 1958. Tumors and Tumorous Conditions of the Bones and Joints (Lea and Febiger, Philadelphia). LEEDS, N. and SEAMAN, W. B., 1962. Fibrous Dysplasia of
the skull and its differential diagnosis. Radiology, 78, 570-582. NEFSON, A. H., 1957. Mucocele of the sphenoid sinus. Archives of Otolaryngology, 66, 1 57—164. NORMAN, P. S. and YANAGISAWA, E., 1964. Mucocele of
sphenoid sinus. Archives of Otolaryngology, 79, 646-656. NUGENT, G. R., SPRINKLE, P. and BLOOR, B. M., 1970.
Sphenoid sinus mucoceles. Journal of Neurosurgery, 32, 443-451. SAMY, L., GINGIS, I. andWASEF, S., 1967. Fibrous dysplasia
in relation to the paranasal sinuses and the ear. Journal of Laryngology and Otology, 81, 1357-1371. ZIZMOR, J. and NOYEK, A., 1968. Cysts and benign tumours
of the paranasal sinuses. Seminars in Roentgenology, 3, 172-201.
1001
