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Chiasmal syndrome in sphenoid sinus mucocele.

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Chiasmal Syndrome
in Sphenoid Sinus Mucocele
James A. Goodwin, MD, and Joel S. Glaser, MD
Two patients with sphenoethmoid mucoceles developed visual field defects consistent with optic chiasm dysfunction. T h e presence of chiasmal field defects associated with the clinical syndrome of compressive optic neuropathy
should not rule out the diagnosis of sphenoethmoid mucocele since definitive treatment can be carried out via
transnasal drainage.
Goodwin JA, Glaser JS: Chiasmal syndrome in sphenoid sinus mucocele.
Ann Neurol4:440-444, 1978
Mucocele of t h e posterior ethmoid and sphenoid
paranasal sinuses is characterized by chronic headache and dysfunction of o n e or more of the cranial
nerves that pass through the orbital apex. Visual loss
and field defects indicating involvement of o n e or
both optic nerves have been amply described, but
reviews of the literature as late as 1970 suggest that
involvement of the optic chiasm by mucocele is rare
[ 5 ] . I n fact, it has been emphasized that the presence
of chiasmal-pattern field defects militates against the
diagnosis of mucocele and favors that of craniopharyngioma or pituitary adenoma [4, 51.
We report 2 patients with mucocele of t h e posterior ethmoid and sphenoid sinuses w h o developed
chiasmal and optic nerve dysfunction.
Case Reports
Patient 1
For three years, an 83-year-old woman had suffered severe
intermittent bifrontal headaches of steady, dull character
lasting up to several days at a time. In May, 1975, she noted
declining vision in the left eye. Her vision continued to
decrease slowly, and in January, 1976, vision was 20130 in
the right eye and limited to “hand movement” in the left;
there was central scotoma, afferent pupillary defect, and
primary optic atrophy on the left; field and fundus examinations of the right eye were normal.
On January 30, 1976, the patient suddenly lost vision
and had a particularly severe frontal headache. The acute
visual impairment and headache lasted two days then spontaneously receded within 48 hours, and the right eye vision
symptomatically returned to normal. Several days later,
perimetry revealed a superatemporal peripheral field defect in the right eye that had been absent in January. The
patient denied past or recent symptoms of paranasal sinus
From the Michael Reese Hospital and Medical Center and the
University of Chicago Prirzker School of Medicine, Chicago, IL,
and the University of Miami School of Medicine, Miami, FL..
Accepted for publication Apr 24, 1978.
disease though she admitted to a continuous discharge of
clear, watery fluid from her nose over the previous several
On February 5 , right eye vision was 20130 and left eye
vision was limited to “finger counting” at 60 cm. Near
vision was J5 in the right eye and not recordable in the left.
The patient could read 8 of 15 AO-HRR color plates with
the right eye but was unable to read any with the left. The
pupils were 4 mm and round with a relative afferent defect
on the left.
Examination of the visual field in the right eye revealed a
superotemporal defect with sharp vertical margins at the
midline and a more gently sloping margin in the inferotemporal and inferonasal quadrants. Visual field examination of
the left eye revealed a central scotoma 15 degrees in diameter and a temporal depression (Fig 1A). Ocular motility was
full with the exception of slight restriction of conjugate
upward gaze. Fundus examination of the right eye was unremarkable; the temporal aspect of the left disc was pale
and without evidence of previous or ongoing disc swelling.
Spontaneous venous pulses were present bilaterally.
Plain skull roentgenograms revealed destruction of the
floor and dorsum of the sella along with forward “ballooning” of the anterior walls of the sphenoid sinus. Tomograms of the sphenoid and ethmoid regions demonstrated
destruction of the posterior ethmoid air cells and amputation of the left anterior clinoid process from below. The
superior orbital fissures and optic foramens were normal.
Computerized axial tomography (Fig 2) reveaIed a loss of
bony contours in the sphenoid, posterior ethmoid, and
sellar regions with replacement by a homogeneous mass of
brain density that failed to enhance after intravenous injection of contrast material. Pneumoencephalography and
carotid angiography were unremarkable.
Transnasal sphenoidotomy was performed with aspiration of purulent-appearing, brownish, viscous material that
grew aspergillus on culture. Forty-eight hours after surgery
Address reprint requests to Dr Goodwin, Division of Neurology,
Michael Reese Hospital and Medical Center, 2929 S Ellis Ave,
Chicago, IL 60616.
440 0364-5 134/78/0004-0508$01.25 @ 1978 by James A. Goodwin
Fig 1. Visualfields i n Patient I. (A)Preoperativefields showing
a relative superotemporaldefect with sloping margins in the
right eye and, i n the lef, a dense centralscotoma breaking into
the temporalperiphery superiorly and inferiorly. (B) Postoperative fields at four months. The superotemporaldefect in the right
eye is no longer demonstrable,and the temporal breakthrough of
the central scotoma in the left eye has resolved.
Fig 2.Computerized axial tomography in Patient I. The normal
bony contours of the sphenoid and posterior ethmoid sinuses and
of the sella are replaced by a homogeneous mass of moderate density (black arrows) that did not enhance on intravenous injection of angiogvaphic contrast material. The mass extends into
the region of the left orbitalapex (open arrow).
the vision was unchanged from preoperative status. Two
weeks postoperatively the right superotemporal field defect was less extensive and less dense; there was still a
dense left central scotoma with “finger counting” vision,
but the peripheral temporal depression in the field of the
left eye was no longer demonstrable. T h e patient remarked, “everything seems brighter,” though acuity in the
left eye had not improved.
Four months postoperatively the visual acuity and color
vision were unchanged. O n Goldmann perimctry of the
right eye a temporal defect could be elicited only with the
I/2 target. In the left eye a centrocecal scotoma with full
peripheral isopters was demonstrable (Fig 1B). T h e fund1
were unchanged.
Fig 3 . Preoperative visualfields in Patient 2. There were central
scotomas of moderate density bilaterally and in the right eye, a
dense upper temporaldefict extending into the periphery.
Patient 2
A 16-year-old boy had complained for approximately six
months of intermittent mucoid nasal discharge and a feeling of congestion and discomfort in the frontal and maxillary regions bilaterally. Six weeks prior to examination he
noted white or yellow spots that obscured his vision when
reading without disturbing distance vision. Two weeks
prior to examination he developed discomfort in both eyes
o n movement of the globes. He had been treated for one
month with Synthroid, 0.01 mg daily, hydrochlorothiazide,
25 mg daily, and Ionamin, 15 mg daily, for obesity without
other symptoms of hypothyroidism.
O n admission there was mild bilateral lid swelling and
left ptosis. Right eye vision was 20140; left, 20160 with
difficulty. The fields were characterized by a central
scotoma in each eye and a relative peripheral defect in the
superotemporal quadrant of the right e y e (Fig 3). There
was moderate edema of the optic discs with engorgement
of retinal veins bilaterally (Fig 4 ) .
Transnasal aspiration of the mucocele yielded a large
volume of brownish, mucopurulent material that failed to
produce growth on bacterial or fungal culture. Two days
postoperatively, vision in the right eye had improved to
20/20 and in the left, to 20/30. Two months later the vision
was 20/20 in both eyes and the visual field defects had
Goodwin and Glaser: Chiasmal Syndrome in Sphenoid Sinus Mucocele 441
Fig 4 . (Patient 2) (A)Right eyefundus preoperatiuel~.There is
moderate disc swelling and venous engorgement. (B) Right
eye fundus six months postoperatively, showing resolution of
dik edema.
442 Annals of Neurology
Vol 4 No 5 November 1978
resolved. H e experienced no further headaches or facial
The earliest description of sphenoid sinus mucocele
is attributed to Berg in 1889. The clinical and radiographic features of these lesions have recently been
defined. In the 1950s, posterior mucoceles most
often were discovered during transcranial surgery directed at presumed parasellar tumors [6, 71. Specific
radiological criteria, however, now identify most
sphenoethmoid mucoceles preoperatively. Transoral
or transnasal drainage has usually proved definitive;
transcranial intervention was often not curative and
led to a higher incidence of complications 11-7, 91.
Most patients with sphenoethmoid mucocele present with ipsilateral orbital or frontal headache. A
few complain of pain in the lower face, vertex, or
occiput. In addition, many patients have signs or
symptoms referable to one or more of cranial nerves
I through VI. The most common deficits are visual
loss with features of optic neuropathy and extraocular muscle palsies along with sensory deficit in the
cutaneous territory of the first or second trigeminal
divisions or both. The headache and oculomotor palsies sometimes run a remitting, relapsing course similar to that of ophthalmoplegic migraine IS]. Mucocele
can generally be distinguished, however, by virtue of
the later age at onset, absence of family history for
migraine, and typical roentgenographic findings.
Petit-Dutaillis et al [71 believed it was possible to
differentiate an ethmoidal syndrome from a sphenoidal syndrome on clinical grounds. The ethmoidal
syndrome is characterized by relatively short duration (weeks to months prior to diagnosis), ipsilatera1 periorbital headache, visual loss with either disc
swelling or primary optic atrophy, and less frequent symptoms including anosmia, diplopia, and
exophthalmos. The sphenoidal syndrome typically
evolves over a longer period, often years, with
nonspecific headache at the vertex or occiput and
relatively late visual loss. These patients may also
complain of diplopia and atypical facial pain with
paresthesias, attributed to irritation of sensory
trigeminal branches as the mass enlarges posteriorly
along the floor of the middle fossa. Giovine [11called
attention to the occurrence of oculosympathetic
paralysis and ipsilateral frontotemporal headache in a
patient with sphenoid mucocele.
Visual loss due to posterior mucoceles is most
often slowly progressive, but it may be apoplectiform
either at its onset or punctuating the course of
otherwise insidious progression [3, 5, 81. Optic neuropathy, while generally unilateral, was bilateral in 7
of the 41 patients with visual symptoms reviewed by
Nugent et al [51.
Chiasmal type field defects are rare in patients with
visual loss due to mucoceles. Nugent and colleagues
[ 5 ] , in their extensive review, identified only 2 patients with chiasmal field defects. Both patients were
blind in one eye and had a temporal field defect in the
contralateral eye. They found none with bitemporal
The pathogenesis of involvement of optic nerve and
chiasm by sphenoethmoid mucocele has not been
defined and may vary from case to case. Most recent
patients have been treated by transnasal or oral
drainage when direct visualization of the optic nerves
and chiasm was not possible. The observations of earlier writers therefore are of importance in understanding the potential mechanisms by which the optic
pathways are damaged. In certain cases approached
transcranially, the optic nerve and chiasm were visibly compressed, elevated, and stretched by the expanding mucocele arising from beneath the intracranial end of the optic canal [ l , 7, 101. Compression of
an optic nerve was quoted as etiologically relevant in
14 cases reviewed by Lundgren and Olin 141. In 12
the optic nerves were observed during craniotomy
and in 2, at autopsy. The authors did not mention any
other pathogenetic mechanisms for optic neuropathy
due to mucocele.
Vincent et al [lo], in the course of a transcranial
approach to a mucocele, encountered dense fibrous
adhesions surrounding the optic nerves and chiasm in
1 patient. They expressed the opinion that contact
with a suppurative focus in the presence of an intact
dura had induced a sterile inflammatory cerebromeningeal reaction that was sufficient to explain
part of the symptomatology. Similar operative observations and pathogenetic speculations were recorded
by Petit-Dutaillis et al [71 and by Giovine [ll, who
invoked a form of “optic neuritis” or vascular impairment of the visual pathways superimposed on the
effects of direct compression by the expanding lesion.
Both of our patients had progressive visual loss
due to sphenoethmoid mucocele. Examination of the
fields revealed evidence of optic chiasm dysfunction
even though no mass could be demonstrated in the
chiasmal cistern. In addition, the first patient experienced transient visual loss and severe headache reminiscent of “pituitary apoplexy” [7]. It seems likely
that this patient’s acute, transitory visual loss was related to vascular or inflammatory factors that have
been invoked by earlier authors [l, 7 , 101. The second patient complained of visual loss and pain on
motion of the eyes and had disc edema suggesting
acute optic neuritis. The diagnosis of mucocele might
have been overlooked had plain skull roentgenograms not been performed.
With drainage of the mucocele, the recently ac-
Goodwin and Glaser: Chiasmal Syndrome in Sphenoid Sinus Mucocele 443
quired field defects in our first patient resolved, leaving the more longstanding visual acuity deficit. and
central scotoma unchanged. The relatively short duration of visual deficit in our second patient probably
explains his rapid recovery of visual function following drainage of the mucocele. Fixed visual loss has
been noted to carry a poor prognosis for postoperative improvement [ 5 ] . It seems, however, that recently acquired optic nerve and chiasm dysfunction
can be readily reversed by transnasal drainage of the
Sphenoethmoid mucoceles thus can have protean
clinical manifestations, including signs of a chiasmal
syndrome. Timely specific treatment of sphenoethmoid mucoceles is of special importance since
fixed visual deficits tend not to improve after drainage, whereas advancing deficits of short duration
respond well to operative intervention.
1. Giovine G Sindrome visiva ed oculo simpatica da mucocele
del sen0 sfenoidale. Chirurgia (Milano) 1:179-183, 1946
2. Hakuba A, Katsuyama J, Matsuoka Y,et al: Sphenoid sinus
444 Annals of Neurology Vol 4 No 5 November 1978
mucoceles. Report of two cases. J Neurosurg 43:368-373,
Linthicum FH, Rand CW, Reeves DL Mucocele of the
sphenoid sinus. Report of a case with autopsy findings. J
Neurosurg 3:444-45 3 , 1946
Lundgren A, Olin T: Muco-pyocele of sphenoidal sinus or
posterior ethrnoidal cells with special reference to the apex
orbital syndrome. Acta Otolaryngol (Stockh) 53:61-79, 1961
Nugent GR, Sprinkle P, Bloor BM: Sphenoid sinus
mucoceles. J Neurosurg 32:443-451, 1970
Petit-Dutaillis D, Schiff-Werrheimer M, Aubry M, et al: Syndrome du compression d‘un nerf optique par une mucocele
sphcndidale latente. Intervention par voie paralatero-nasale.
Importance des signs radiologiques pour le diagnostic.
Neurochirurgie 4:8 1-74, 1958
Petit-Dutaillis D, Thiebaut F, Fischgold H: Contribution a
1’Ctude des compressions intracraniennes des nerfs optiques
par les abcis ou Ies mucoceles excraduraies d’origine sphbnoethmoidale. Rev Neurol (Paris) 83:325-341, 1950
Pincus JH, Daroff RB: Sphenoid sinus mucocelr: a curable
cause of rhe ophthalmoplegic migraine syndrome. JAMA
187:459-460, 1964
Valvassori GE, Putterman A M Ophthalmologic and
roentgenographic findings in sphenoid rnucoceles. Trans Am
Acad Ophthalrnol Otolaryngol 77:703-713, 1973
Vincent C, Thiebault D: Les abc6s extra-duraux d’origine
sphCnoethmdidale. Rev Med kege 60:166-171, 1943
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sphenoid, syndrome, mucocele, sinus, chiasmal
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