NEURO-OPHTHALMOLOGIC MANIFESTATIONS* BY Melvin G. Alper, MD

29 Pages·2005·6.63 MB·English

MUCOCELES OF THE SPHENOID SINUS:
NEURO-OPHTHALMOLOGIC
MANIFESTATIONS*
BY Melvin G. Alper, MD
INTRODUCTION
MUCOCELES OCCUR LESS OFTEN IN THE SPHENOID SINUS THAN IN THE OTHER
paranasal sinuses. Since their first description by Berg' in 1889,
preoperative or ante mortem diagnosis was rarely established until
recent years. Delayed diagnosis in the past often led to severe visual
loss while misdiagnosis resulted in exploratory craniotomy for pitui-
tary tumor with disastrous consequences.
Nugent and associates2 recentLy reviewed the world literature find-
ing 81 cases, reporting 63 of these and adding 2 of their own. Iso-
lated case histories have appeared since this review so that approxi-
mately 100 cases of sphenoid sinus mucoceles have been reported to
date.3-10
Since many ofthese patients are first seen because ofeye complaints,
it is important for the ophthalmologist to be aware of the ocular mani-
festations. With an increased index of clinical suspicion and the use
of presently available sophisticated neuroradiologic techniques, early
diagnosis and proper management should become more common in
the future.
This paper will report seven additional cases of sphenoid sinus
mucocele with ocular findings varying from isolated ocular motor nerve
palsies to total blindness. The diagnostic neuroradiologic features
will be presented, and the proper management will be described.
PATHOLOGY
Although some doubt exists concerning the etiology, mucoceles in
general have been divided into primary and secondary types (Table
*From the Department of Ophthalmology, Washington Hospital Center, Washington,
D. C.
TR. AM. OPHTH. Soc., vol. LXXIV, 1976
54
TABLEI:CLASSIFrCATIONOFMUCOCELES
I. PrimaryTypes
A. Retentioncystsarisefrommucousglandsofsinusepithelium.
II. SecondaryTypes
A. Due toobstruction ofsinus ostia.
B. Arisefrom cysticdegeneration ofsinuspolyps.
I). Primary types arise from retention cysts of the mucous glands
of the sinus epithelium. Secondary types arise either from obstruc-
tion of the sinus osteum or from cystic degeneration of sinus polyps.13
This process precludes normal drainage of the sinus and leads to a
dilated cavity filled with a thick dark or sometimes clear fluid. They
may be unilateral or bilateral. Ciliated columnar cells of the sphenoid
sinus are replaced by cuboidal cells or layers of pigment cells. The
mucocele wall is composed offibrous connective tissue with round cell
infiltration.14
Because of their relationship to many vital structures, sphenoid
sinus mucoceles can cause a wide variety of symptoms and signs de-
FIGURE 1
Coronal diagram demonstrating relationship ofthe sphenoid sinus to surrounding vital
structures. Arrows indicate pathways of extension of sphenoid sinus mucoceles en-
countered in this series ofsevenpatients.
Mucoceles 55
FIGURE2
Lateraldiagramillustratingroutesofextensionofmucoceles ofthesphenoidsinus. Muco-
celes may extend upward eroding the bone ofthe sella floor to press against the pitui-
tary gland and chiasm simulating an intrasellar adenoma. More often, extension is
anterior against the ethmoid cells, which offer little resistance, and thence to the or-
bital apices. Displacement ofthe cavernous portion ofthe carotid artery commonly oc-
cursfromlateralandupwardextension. InvolvementofthecranialnervesIII, IV,VIandV
(first and second division) comes from lateral extension. Erosion ofthe clivus posteriorly
simulates chordomas. Inferiorextension intothe nasopharynxwitherosion ofthepalatine
boneisanotherroute ofextension.
pending upon which adjacent structure is involved by the expanding
cyst. Because the posterior ethmoidal cells offer the least resistant
barrier to erosion, pressure from the expanding sphenoidal sinus is pri-
marily transmitted to this area and thus to the apices ofthe orbit, caus-
ing headache and visual symptoms (Fig. 1 and 2).
The sphenoid sinus itself lies in the body of the sphenoid bone
usually incompletely divided byamidline septum. Anteriorly, the sphe-
noid sinus abuts the posterior ethmoid air sinus and cribiform plate
and forms part of the posterior upper nasopharyngeal wall where it
drains by two ostia into the nasopharynx. Posteriorly, it is located in
front of and under the sella turcica. Inferiorly, it is bounded by the
nasopharynx and the pterygoid fossa. Laterally, the sphenoid sinus
is bounded posteriorly by the cavernous sinuses and internal carotid
arteries, while anteriorly it is separated from the optic canal by a bony
56 Alper
wall 0.5 mm thick. The volume ofthe sphenoid sinus can vary from 0.05
to 14.0 cc. The structures that surround the sphenoid sinus include
the first six cranial nerves, the internal carotid arteries, the cavernous
sinuses, the tuberculum sellae, the sella turcica, the anterior and pos-
terior clinoids, the planum sphenoidale, the pituitary gland, the op-
tic canals, the medial walls of the superior orbital fissures and the or-
bit, and the clivus posteriorly.
CASEREPORTS
CASE 1
A 23-year-old white women, was seen on January 2, 1969, with a chief com-
plaint of severe headache behind her left eye associated with double vision
and drooping of the left upper lid for 24 hours duration. Three weeks be-
fore the onset of the present illness she had had flu and had been confined
to bed for one week. For two weeks prior to the onset of the present illness
she had had a full sensation in her head with stuffy ears and a dull ache be-
hind her left eye. Twenty-four hours before the onset of her present illness
the pain behind her left eye became intense. She awakened with double vision
and drooping ofher left upper lid. The pain behind her left eye was charac-
terized as being more intense in the supine position and somewhat alleviated
when assuming the erect position.
A review of systems revealed menarche at age 11 years. The menses were
regular, occurring everythirtydaysforfour tofivedays durationuntil 18 months
before the present illness. At that time they became irregular and were char-
acterized by heavy flooding every two to three months with amenorrhea in
the interim. Seven years prior to the onset of the present illness she de-
veloped a great gain in weight, reaching a body weight of278 lbs. Her energy
was said to be normal but there was a loss oflibido.
The vision of the right eye was 20/20 and the left eye was 20/20 with best
correction. There was an external third nerve paralysis of the left eye (Fig.
3); pupillary reflexes were normal in both eyes. The anterior segments ofeach
eye were within normal limits. The optic nerve and retinawere normal in each
eye. Visual fields by Goldmann perimeter as well as by tangent screen re-
vealed a bitemporal hemianopia to small test targets but there were full fields
to large test targets. Applanation tension in each eye was 14 mm mercury.
The neurological evaluation was within normal limits except for the above
described third nerve palsy. She appeared to be an obese, well developed,
well nourished white female in no acute distress. Her blood pressure was 160/
100. Her heart and lungs were normal. The remainder ofthe physical exami-
nation was non-revealing.
There was a normal blood count and urinalysis. Her blood sugar was 77 mg/
100 ml; urea nitrogen, 6 mg/100 ml; sodium, 137.5 mEq; potassium, 3.7 mEq;
chloride, 95 mEq; cortisol, 6.5 mEq per 100 ml (normal 7 to 27). Glucose
Mucoceles 57
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58 Alper
FIGURE4
Postoperative photograph ofpatient in Figure 3 demonstrating recovery ofthe left third
nerve following sphenoidectomy andsinusotomy.
tolerance test showed the following: fasting, 82 mg/100 ml; one-halfhour, 138
mg/100 ml; one hour, 138 mg/100 ml; two hours, 120 mg/100 ml; three hours,
108 mg/100 ml. Tests for urine sugar and acetone were negative in all ofthese
collections. The urine 17 ketosteroids measured 12.3 mg per twenty-four
hours; the 17 hydroxysteroids, 6.4 mg per twenty-four hours (17 ketosteroids
normalforfemales: 7to 13; 17hydroxysteroids normalforfemales: 2.7). Twenty-
four hour urine gonadotropin (FSH) was less than eight rat units per twenty-
four hour urine specimen. (Normal reproductive range for females is 8 to 30
rat units per twenty-four hour urine specimen.) Serum protein electrophore-
sis was within normal limits. Cerebrospinal fluid evaluation: protein 18.3 mg/
100 ml; 19 lymphocytes; sugar 50 mg/100 ml; chlorides 120 mg/100 ml; the
Kolmer and colloidal gold were normal. Serum protein electrophoresis of
the spinal fluid protein was within normal limits. Iodine-131 thyroid uptake
after twenty-four hours, 15.7% (normal euthyroid range 16 to 30%). T 3 was
36.1% (normal range 27 to 38%). Urine creatinine measured 1,618 mg per
twenty-four hours.
On the second hospital day the patient spiked a fever to 103 F. Blood cul-
tures showed a twenty-four hour gram negative Hemophilus influenzae ba-
cillus which was sensitive to ampicillin, chloramphenicol, colistine, erythro-
mycin, canomycin, neomycin, penicillin, streptomycin and tetracycline. The
urine cultured Escherichia coli and Staphylococcus epidermis.
Ampicillin and streptomycin were given. Her fever returned to normal and
the blood culture was reported as negative after 48 hours. The sella appeared
to be enlarged on normal skull films, but with tomography the sella turcica
floor was well demonstrated and there was a mass measuring 1 X 4 cm in the
FIGURE 5
A: Lateral roentgenogram of skull (Case 1), showing enlargement and distention of the
sphenoid sinus. White and black arrow demonstrates partial destruction ofsella turcica
floor. Two small arrows show involvement of the clivus. A nasopharyngogram has
been performed. Single black arrow demonstrates protrusion of soft tissue mass into
the roof and posterior wall of the nasopharynx. B: Modified waters view of paranasal
sinus (Case 1). Black arrow indicates lateral displacement of left lamina papyracea.
60 Alper
FIGURE 6
Brachial arteriogram (Case 1) demonstrates lateral displacement of the cavernous por-
tion of the internal carotid artery in the AP view (A), and upward displacement with
some arterial narrowing in the lateral view (B). Normally, the cavernous portion of
the internal carotid artery bows inward in contradistinction to the outward bowing de-
formity notedinA.
sphenoid sinus. The posterior clinoid was destroyed in its lower part. There
was partial destruction of the clivus and some of the bony floor of the sella.
Protrusion of a soft tissue mass 1 cm in diameter into the roof and posterior
wall of the nasopharynx was also noted with destruction of the left posterior
clinoic, leftdorsum sellae andleftanterior clinoid. Nasopharyngograms demon-
strated the nasopharyngeal lesion to good advantage. Brachial arteriography
with filling of both internal carotids revealed lateral and upward displace-
ment of the portion of the internal carotid artery lying within the carvernous
sinus (Fig. 6). Pneumoencephalography demonstrated no abnormalities
of the ventricular system. A chest roentgenogram was within normal limits.
Scintiscan utilizing technetium 99 failed to demonstrate any definite abnor-
mality in the brain.
The preoperative impression was that of a mucocele of the sphenoid sinus
although a pituitary tumor such as chromophobe adenoma or achordoma ofthe
clivus could not be excluded. On the twenty-second hospital day a sub-
mucous resection ofthe nasal septum and transnasal removal ofa mass from the
sphenoid sinus was performed under general anesthesia. The sinus was filled
with a thick cheesy white material which was mostly suctioned out and cul-
tured. The entire sac lining the sphenoid sinus was carefully removed and
the sinus cleansed of its debris. Two small ureteral catheters were placed
in the sinus and sutured to the nasal vestibule for drainage. Culture of the
material from the sphepoid sinus revealedHaemophilus influenzae. The micro-
scopic diagnosis ofthe tissue removed from the sphenoid sinus was interpreted
as apyomucocele.
Mucoceles 61
FIGURE 7
Preoperative photographs (Case 2) demonstrating right third nerve paralysis and loss
oflight reflex in both eyes (A, B, C, D). A right third nerveparalysis andblindness was
noted on admission to hospital with left upper quadrantanopia. Following a sponta-
neous convulsion while undergoing a brain scan, the patient became blind in the left
eye. Atrophy ensued in both the right (E) and left (F) optic nerves, although the third
nerveparalysis recovered.
Twenty-four hours following evacuation of the material from the sphenoid
sinus the patient regained normal function ofthe third nerve. In the year fol-
lowing the operation thepatientlost55poundswithoutchange in herdiet. Her
menses became regular and the cortisol levels returned to normal. Follicular
stimulating hormone, which had been less than eight rat units for twenty-
four hour urine specimen preoperatively returned to more normal limits
postoperatively measuring 16 units per twenty-four hour unit specimen. Her
visual fields returned to normal in forty-eight hours.
62 Alper
FIGURE8
Cerebral flow study (Case 2) performed with Tc-99m-pertechnetate demonstrating de-
creased activity in the right internal carotid artery (one arrowhead) when compared to
theleft (twoarrowheads).
The patient has remained well and her endocrine abnormalities have dis-
appeared.
CASE 2
A 24-year-old black man was admitted to the hospital on July 17, 1972 with
a history ofseverely progressive headache for eight days which was generalized
in character with some preponderance for localization in the right supraorbi-
tal area. Four days before admission he became nauseated without vomiting.
The headache was unremitting and was not relieved by analgesics. Three
days before admission he awoke with increased pain and ptosis ofthe right up-
per lid. There was an associated blurring of the vision in the right eye. He
was seen in the emergency room where the problem was felt to be migraine
and he was referred to an evening "walk in" clinic. At the "walk in" clinic he
was given fiorinal and an appointment was made for thorough evaluation one
week later. Three days later he was seen in the emergency room because of
unrelieved headache which had increased in severity and had now become as-
sociated with a complete ptosis ofthe right upper lid (Fig. 7). He was referred
immediately to the eye clinic where it was determined that the right eye was
blind. The vision in the left eye was 20/200 and there was a complete third