silla turca vacia 1

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Dyn ami c Roentgenographic Cha nge s in the Empty Sella Syndrome 1 Charles B. Grossman, M.D. Two cases of empty sella syndrome are presented. Progressive sellar enlargement was documented together with evidence of increased intracranial pressure. One patient ex hibited de novo development of an empty sella turcica. INDEX TERMS: Cisterns, subarachnoid. Sella Turcica. Skull, pres sur e in RadIology 116:341-344 , August 1975 Neuroradio logy B USCH (3) USED the term " empty sella" to describe the anatomical appearance of pituitary remodeling a ss oc iat ed with extension of the subarachnoid space into the sella turcica. Robertson (15) described air ex tending into the sella during pneumoencephalography . Many recent reports concern ng enlargement of the sella turcica associated with an intrasellar cistern have been published (7, 9, 10, 17). The triad of sellar remod eling in association with pneumoencephalographic evi dence of an i nt ra se lla r c is te rn and p it ui ta ry re mo de li ng represents the radiological correlates of the anatomical term " empty sella " as defined by Busch (3, 9). Enlarge ment of the sella is not necessary fo r the radiological di agnosis (10). Hodgson et al . (8) used the terms " idio pathic" to describe spontaneous occurrence and "sec ondary" to describe the p os ts urg ic al or p os tir ra di ati on empty sella. Very fe w longit udinal studies of sellar re modeling have been r ep or te d. F ur th er enlargement of an already enlarged sella turcica ha s been documented in one case of pseudotumor cerebri (16) and suggested in 2 cases of idiopathic empty sella syndrome (8) . The frequent association of the e mp ty sella syndrome and cerebrospinal fluid rhinorrhea has been reported (2, 14). Tw o cases in which dynamic radiological changes were seen in association with the empty sella syndrome ar e described below. C AS E R EPO RT S CASE I: A 3 5- ye ar -o ld woman was h os pi ta li ze d for e val ua ti on of mild systemic hypertension and headaches. Five years earlier she had had a transient episode of occipital and vertex headaches. Skull films were n or ma l. M il d s ys te mi c h yp er te ns io n h ad be en no te d t wo ye ar s b ef o re a dm is si on . T he p at i en t h ad h ad e pi so di c ipital and vertex headaches for one year as well as occasional ring ing in the r ight ear and was being treated with small doses of Valium . She was not using oral contraceptives and had given birth to a normal child ten years earlier. Her only other pregnancy terminated in a first-tri mester spontaneous abortion eight years before admi ss ion. She had n o m aj o r i ll ne sse s, s ymptomatic crises, or menstrual abnormalities. On clinical examination , the patient was mildly obese and her blood pressure was 130/88. The remainder of the physical examina tion was normal, including visual fields and thorough examination of the nervous system , ears , nose, and throat . Cerebrospinal flu id pres su re a nd a na ly si s, ro ut ine l a bo ra t or y t est s, ser um chemistries and e le ct ro ly te s, and e xt en si ve e nd oc ri ne t es ts w er e n or ma l, as w er e the c he st films. e xc re to ry u ro gr am , e le ct ro en ce ph al og ra m , and brain scan. Comparison of the skull films with those taken five years earlier showed that the mean volume of the sella turcica , which had previously been normal according to the criteria of Oi Chiro et a l. ( 5) (Fig. 1) , was now almost four times as large (Fig . 2). The diameter of Fig. 1 . A and B . CASE I. Ant eroposter ior (A) and lat eral (B) skull projections taken in 1968. The sella turcica appears normal and its vol ume is 637 mm 3 . The arrow i nd ic at es t he f l oo r of t he se ll a t ur ci ca . Fig . 2. A and B. CASE I. Anteroposterior (A) and lateral (B) skull projections takenin 1973 . The volume of the sella turcica has expand ed to 2,430 mm " , The lamina dura and dorsum sellae are intact. The arrow indicates the floor of the sella turcica . 1 From the Departments of Radiolog y of Episcopal Hospital an d Temple Universi ty Heal th Sciences Center, Phil adel phia , Pa . Revis ed version accepted for publication in April 1975. sjh 341

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Dynamic Roentgenographic Changes in the Empty

Sella Syndrome1

Charles B. Grossman, M.D.

Two cases of empty sella syndrome are presented. Progressive sellar enlargement wasdocumented together with evidence of increased intracranial pressure. One patient ex

hibited de novo development of an empty sella turcica.

INDEX TERMS: Cisterns, subarachnoid. Sella Turcica. Skull, pressure in

RadIology 116:341-344, August 1975

Neuroradio logy

BUSCH (3) USED the term " empty sella" to descr ibe

the anatomical appearance of pituitary remodeling

associated with extension of the subarachnoid space

into the sella turcica. Robertson (15) described air ex

tending into the sella during pneumoencephalography.

Many recent reports concerning enlargement of the

sella turcica associated with an intrasellar cistern have

been published (7, 9, 10, 17). The triad of sellar remod

eling in association with pneumoencephalographic evi

dence of an intrasellar cistern and pitui tary remodeling

represents the radiological correlates of the anatomical

term " empty sella " as defined by Busch (3, 9). Enlarge

ment of the sella is not necessary for the radiological di

agnosis (10). Hodgson et al. (8) used the terms " idio

pathic" to describe spontaneous occurrence and "sec

ondary" to descr ibe the postsurgical or postir radiation

empty sella. Very few longitudinal studies of sel lar re

modeling have been reported. Further enlargement of

an already enlarged sella turcica has been documented

in one case of pseudotumor cerebri (16) and suggested

in 2 cases of idiopathic empty sella syndrome (8) . The

frequent associat ion of the empty sella syndrome and

cerebrospinal fluid rhinorrhea has been reported (2, 14).

Two cases in which dynamic radiological changes were

seen in association with the empty sella syndrome are

described below.

CASE REPORTS

CASE I: A 35-year-old woman was hospi ta lized for evaluation of

mild systemic hypertension and headaches. Five years earlier she

had had a transient episode of occipital and vertex headaches. Skull

films were normal. Mild systemic hypertension had been noted two

years before admission. The patient had had episodic occ ipital and

vertex headaches for one year as well as occasional ringing in the

r ight ear and was being treated with small doses of Valium. She was

not using oral contraceptives and had given birth to a normal child

ten years ear lier . Her only other pregnancy terminated in a f irst-tri mester spontaneous abortion eight years before admission. She had

no major i llnesses, symptomatic crises, or menstrual abnormalities.

On clinical examination, the patient was mildly obese and her

blood pressure was 130/88. The remainder of the physical examina

tion was normal, including visual fields and thorough examination of

the nervous system, ears , nose, and throat. Cerebrospinal fluid pres

sure and analysis, routine laboratory tests, serum chemistries and

electro ly tes, and extensive endocrine tests were normal, as were

the chest films. excretory urogram, electroencephalogram, and

brain scan. Comparison of the skull films with those taken five years

ear lier showed that the mean volume of the sella turcica, which had

previously been normal according to the criteria of Oi Chiro et al. (5)

(Fig. 1), was now almost four t imes as large (Fig. 2). The diameter of

Fig. 1. A and B. CASE I. Anteroposterior (A) and lateral (B) skull projections taken in 1968. The sella turcica appears normal and its volume is 637 mm 3

. The arrow indicates the floor of the sella turcica.Fig. 2. A and B. CASE I. Anteroposterior (A) and lateral (B) skull projections takenin 1973 . The volume of the sella turcica has expand

ed to 2 ,430 mm", The lamina dura and dorsum sellae are intact. The arrow indicates the floor of the sella turcica.

1 From the Departments of Radiology of Episcopal Hospital and Temple University Health Sciences Center, Philadelphia , Pa. Revised versionaccepted for publication in April 1975. sjh

341

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342 CHARLES B. GROSSMAN August1975

Fig. 3. A and B. CASE I. Half-axial skull project ions taken in 1968 (A) and 1973 (B). The diameter of the occipital emissary venous canals (arrows) has doubled.Fig. 4. A and B. CASE I. Lateral skull project ions taken in 1968 (A) and 1973 (B) show a marked increase in the d iameter of the f rontal

diploic venous canals (arrows).

Fig. 5. A and B. CASE I. Standard basal skull projection (A) and tomogram (B), both taken in 1973. The lef t foraminaovale and spinosum are enlarged, with smooth, clearly defined margins (large arrows). Note the remnant of the septum separat

ing the foramina (small arrow). The normal right foramina ovale and spinosum are seen on both basal projections.

the occipital emissary venous foramina had doubled (Fig. 3) and the

diameter of the frontal diploic venous canals was markedly in

c reased (Fig. 4). Erosion of the left foramina ovale and spinosum

with clearly defined margins was noted on the basal project ion (Fig.

5), not obtained in 1968.

Selective catheter arteriography of the left internal and exter

nal carotid, right common carotid, and left vertebral arteries failed to

demonstrate evidence of an aneurysm, mass, abnormal arteries, or

abnormal blush. Two left middle meningeal arteries originating from

the left maxil lary artery appeared to traverse the anter ior and poste

r ior limits of the eroded foramina at the lef t base. Nei ther artery was

enlarged, stretched, or displaced. A prominent left pterygoid venous

plexus was seen on the left carotid a r t e ~ i o g r a m s (Fig. 6). The dural

venous sinuses were patent. The ophthalmic, occipital emissary,

and frontal diplo ic veins were opacified, and the d ip lo ic veins were

enlarged as well (Fig. 6). Pneumoencephalography demonstrated an

air-f il led intrasellar cistern, a remodeled pituitary gland, a posteriorly

positioned opt ic chiasm, and normal ventr icular anatomy (Fig. 7).

There was no evidence of a temporal or subtemporal mass. The pa

tient was discharged without addit ional medications or therapy and

has remained asymptomatic . Repeat examination 15 months later

revealed no abnormalities. Skull films were unchanged, and a com

puted axial tomogram of the brain was normal.

CASEII: A 35-year-old multiparous obese woman was evaluated

for systemic hypertension. A hysterectomy had been per formed

three years earlier for i rregular menstrual bleeding. Due to question

able papil ledema, a lumbar puncture was performed and a skull se

ries was obtained. The cerebrospinal f luid opening pressure was 195

mm Hg. Endocrinologic tests were normal. The skull ser ies demon

strated an enlarged sella turcica with a demineral ized lamina dura

(Fig. 8). Pneumoencephalography documented an empty sella turci

ca (Fig. 9). Bilateral carotid angiograms were normal.

Four years later the pat ient was evaluated for cerebrospinal fluid

rhinorrhea which developed following the ab rupt remission of a

three-week episode of severe headaches. A skull series demon

strated a twofold increase in the vo lume of the sella as well as ero

sion of the lamina dura (Fig. 10). Pneumoencephalography recon

f irmed the empty sella turcica (Fig. 11) and 111In_DTPA cisternogra

phy documented CSF rhinorrhea (Fig. 12). The cerebrospinal f luid

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Vol. 116 DYNAMIC ROENTGENOGRAPHIC CHANGES IN THE EMPTY SELLA SYNDROME 343 Neuroradiology

Fig. 6. A and B. CASE I. Left internal carotid arteriogram, late venous phase : anteroposterior (A) and lateral tB) pro

jec tions. 1 = opacified ophthalmic veins; 2 = frontal diploic veins; 3 = occipital emissary vein; 4 = prominent left ptery

goid venous plexus.

Fig. 7. A and B. CASE I. Brow-up anteroposterior (A) and lat

eral (B) tomograms taken during pneumoencephalography. Air in theintrasellar c is tern out lines the sur face of the remodeled p itui tary

gland (large arrows). The poster ior position of the optic chiasm

(small arrow) suggests that the diaphragma sellae is widened.

pressure and endocrinologic findings were normal, At surgery, the

large intrasellar cistern was confirmed . The sella was packed with

muscle. and the patient had an uneventful recovery.

DISCUSSION

Cerebrospinal fluid pulsation through an incomplete

diaphragma sellae is generally accepted as the mecha-

nism by which an empty sella develops (10), and elevat

ed intracrania l pressure has also been considered as an

etiologic factor (6, 7, 10, 11). Characteristically, the id

iopathic type of empty sella syndrome occurs in middle

aged obese women complaining of headaches or sinus

itis who have normal endocrine function (8) or mild

hypopituitarism (1). Cerebrospinal fluid rhinorrhea fre-

quently occurs in the empty sella syndrome (2, 14),

enhancing speculation as to the etiologic role of raised

intracranial pressure in this condition (7, 10).

The first patient had clinical, laboratory , and radiologi

cal f indings typical of the empty sella syndrome. In addi-

Fig. 8. A and B. CASE II. First admission, lateral (A) and basal

(B) skull projections. The lam ina dura is demineralized (large arrow)

and the sella turcica is greatly expanded, now measuring 3,230 mm3

in volume. The small arrows i nd icate the anter ior limit of the sella

turcica.

tlon, there was evidence of increased intracranial pres-

sure. The posterior position of the optic chiasm and the

f ree access of cflr to the intrasellar c istern suggested a

deficient diaphragma sellae (17), while the enlargement

of the occipital emissary vein was evidence of increased intracrania l pressure (4). EI Gammal and Allen

noted that 2 of their 24 patients with an empty sella had

prominent pterygoid veins and reported the same finding

in other unrelated cases (7). The direction of venous

flow through the ophthalmic, frontal diploic, pterygoid,

and occipital emissary veins formed an unusual pattern.

It is known that the rete of the foramen ovale connects

the cavernous sinus with the pterygoid venous plexus

(12); thus it would seem likely that eros ion of the lef t fo

ramina ovale and spinosum is secondary to an increase

in the caliber of the travers ing veins, as is enlargement

of the occipital emissary and frontal diploic veins.The second pat ient also had cl inical and roentgeno-

graphic findings typical of the empty sella syndrome.

The marginally elevated intracranial pressure during the

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344 CHARLES B. GROSSMAN August 1975

Fig. 9. CASE II. First admission: pneumoencephalo

gram, brow-up lateral project ion with the head hanging dem

onstrates an air-fluid level (arrows) in the intra sellar cistern.

The optic chiasm is in a posterior posit ion (single arrow).

Fig. 11. A and B. CASE II. Second admission: brow-up frontal

(A) and lateral tomograms {B) taken during pneumoencephalogra

phy. Air f il ls the intrasellar cistern (arrows). The optic chiasm is opa

cif ied (B, black arrow).

first admission and the lamina dura erosion that oc

curred during the interval between admissions suggest

that the intracranial pressure probably increased at this

time, and the twofold increase in sellar volume and the

development of cerebrospinal fluid rhinorrhea may have

been secondary to this rise in intracranial pressure.

ACKNOWLEDGMENTS: I wish to thank Dr. Herbert Goldberg of the

Philadelphia General Hospital for the roentgenograms representing

the f irst admission of the pat ient in CASE IIand Dr. D. Gordon Potts of

The New York Hospital for his help in reviewing this manuscript.

Department of RadiologyEpiscopal HospitalPhiladelphia, Pa. 19125

REFERENCES

1. Brisman R, Hughes JEO, Holub DA: Endocrine function innineteen patients with empty sella syndrome. J Clin Endocrinol MetaboI34:570-573, Mar 1972

2. Brisman R, Hughes JEO, Mount LA: Cerebrospinal flu id rhinorrhea and the empty sella . J Neurosurg 31:538-543, Nov 1969

3. Busch W: Die Morphologie der Sella turcica und ihre Beziehungen zur Hypophyse. Arch Pathol Anat 320:437-458, 1951

4. Chynn KY: Occ ip ital emissary vein enlargement . A sign of

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Fig. 10. A and B. CASE II. Second admission: lateral (A) and

basal (B) skull project ions. The lamina dura is eroded (large arrow)

and the sel la r volume has increased to 6,413 mm", Note the pro

gressive anterior extension of the sella turcica (small arrows).

Fig. 12. CASEII. Second admission: four-hourright lateral 111In_DTPA cisternogram. There is ac

t iv ity in the int rase llar c is tern (large arrows) and

nasal mucosa (small arrows).

6. EI Gammal T, Allen MB Jr: Fur ther consideration of sellarchanges associated with increased intracranial pressure. Br J Radiol45:561-569 , Aug 1972

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and configuration of the sella turcica as the result of prolonged cere

brospinal fluid shunting. Radiology 97:537-542, Dec 1970

12. KrayenbiJhl HA, Yasargil MG: Cerebral Angiography. Philadelphia, Lippincott, 2d Ed, 1968, P 120

13. Lee WM, Adams JE: The empty sella syndrome. J Neurosurg 28:351-356, Apr 1968

14. Ommaya AK. Di Ch iro G, Baldwin M, et al: Non- traumati ccerebrospinal fluid rhinorrhoea. J Neurol Neurosurg Psychiatry 31:

214-225, Jun 1968

15. Rober tson EG: Pneumoencephalography. Spr ingf ield, III.,Thomas, 1957, p 314

16. Sones PJ, Heinz ER: The sella turcica in multiparity; withcomments on the effects of pseudotumor cerebrt. Br J Radiol 45:503 -506,JuI197217. Zatz LM, Janon EA, Newton TH: The enlarged sella and the

intrasellar cistern. Radiology 93:1085-1091, Nov 1969