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Headache and the

Primary Empty Sella Syndrome


J. Dale Browne, MD, Robert I. Kohut, MD

\s=b\ Otolaryngologists frequently en- the otolaryngologic community the past three years. The pain originated from
counter patients with complaints of head- association of headache with "empty the left retro-orbital area with radiation
aches. On occasion, normal physical into the frontal regions. His pain did not
sella" by reviewing its characteristics
examination findings may be accompa- prevent him from working, and he con¬
and presenting an illustrative case
nied by a roentgenographic enlargement trolled the pain with acetaminophen. Two
of the sella turcica. In the process of report.
The term empty sella was coined by years previously, he had been evaluated for
evaluation for an intrasellar neoplasm, an the same headaches by another otolaryn¬
"empty sella" is occasionally discovered. Busch1 in 1951 following 788 autopsies
gologist, who performed a septoplasty and
Patients with this condition commonly of patients with no known pituitary intranasal ethmoidectomies without relief
have headaches, although a cause-effect disease. Only 42% of the patients had or alteration of the patient's symptoms.
relationship is controversial. Endocrine a complete sella diaphragm, while in Head and neck examination findings
and visual disturbances, although rare,
approximately 5%, the diaphragm were normal. No abnormalities were iden¬
necessitate periodic evaluation of these was a thin rim of tissue, 2 mm or less. tified on neurologic examination. Roent¬
patients. In these cases, the pituitary gland was genograms revealed well-aerated paranas-
(Arch Otolaryngol Head Neck Surg al sinuses. On the lateral view, the sella
1986;112:883-885) compressed to the bottom of the sella turcica was enlarged and deepened (Fig 1).
with subarachnoid space filling the
In evaluation of this finding, prolactin,
majority of the intrasellar space. cortisol, growth hormone, follicle-stimu¬
Otolaryngologipatients
sts frequently
counter with com¬
en¬ Since then, primary and secondary
empty sella types have been identi¬
lating hormone, luteinizing hormone, thy¬
roid-stimulating hormone, and thyroxine
plaints of "sinus headaches." The fied. A secondary empty sella is seen levels were shown to be normal. Ophthal¬
presence of an enlarged sella on roent¬ following radiation or surgery on the mologic consultation found normal visual
genographic evaluation of the sinuses, pituitary gland, reducing its size with fields and funduscopic examination re¬
while suggestive of an intrasellar neo¬ subsequent intrasellar herniation of sults. A CT head scanner (General Electric
plasm, is commonly identified on com¬ the suprasellar cistern. When there is 8800) demonstrated intrasellar content
puted tomographic (CT) scan as a no history of such intervention, a pri¬ isodense with CSF in the ventricles, consis¬
tent with the diagnosis of ESS (Fig 2).
cerebrospinal fluid (CSF) density fill¬ mary empty sella exists, with those The patient was instructed to continue
ing the sella. The purpose of this patients having no coexisting pitu¬ the use of acetaminophen. He is to be
article is to bring to the attention of itary tumor described as having the reevaluated on a yearly basis with re¬
primary empty sella syndrome peated pituitary function tests and oph¬
Accepted for publication Oct 21, 1985. (ESS).2 thalmologic examinations.
From the Department of Surgery, Section on
Otolaryngology, Bowman Gray School of Medi- REPORT OF A CASE
cine, Wake Forest University, Winston-Salem, COMMENT
NC. A 60-year-old man presented to the Oto¬
Reprint requests to Department of Surgery, laryngology Clinic of Wake Forest Univer¬ The anatomy of a primary empty
Section on Otolaryngology, Bowman Gray School sella contains a deficient sella dia¬
of Medicine, Wake Forest University, 300 S Haw- sity Medical Center, Winston-Salem, NC,
thorne Rd, Winston-Salem, NC 27103 (Dr with a chief complaint of continuous, mild, phragm through which subarachnoid
Kohut). nonpulsatile left-sided headaches for the space herniates into the sella, com-

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Fig 2.—Coronal reconstruction of sella turcica demonstrating sella
contents of equal density with cerebrospinal fluid in ventricles.

Fig 1.—Lateral view showing enlarged, deepened sella turcica (ar¬


rows).

Fig 3.—Normal relationship of meninges to human pituitary gland (left). In empty sella syndrome (right) arachnoid membrane herniates through
incompetent diaphragma sellae. CSF indicates cerebrospinal fluid (from Jordan et al8).

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pressing the pituitary gland (Fig 3). is of moderate intensity, chronic, ache-related physical findings, and a
While a deficient sella diaphragm is asymmetrical, and anterior. Most history consistent with the syndrome.
thought to be a prerequisite for devel¬ authors feel these headaches are too The diagnosis is confirmed by high-
opment of an empty sella, there are a nonspecific to be of diagnostic value. resolution CT scanning, which demon¬
variety of theories concerning the Since patients with headaches are strates an enlarged sella containing
mechanism behind the actual pro¬ more likely to be evaluated with sinus material of equal density to CSF. The
lapse of arachnoid tissue. These and skull films, this association of false impression of an empty sella can
include rupture of a pituitary or intra¬ headaches and ESS might be consid¬ be created by a partial volume effect
sellar arachnoid cyst, necrosis of a ered one of selection. Moretti et al,6 inpatients with a hyperpneumatized
pituitary adenoma, benign intracrani¬ however, think that the high inci¬ sphenoid sinus or dilated perisellar
al hypertension, and fluctuation of dence of headache in patients with CSF spaces.9 In questionable cases,
CSF pressure with subsequent remod¬ ESS suggests more than a coinciden¬ intrathecal metrizamide may be nec¬
eling of the sella turcica.3"5 tal relationship. In addition, they feel essary to further demonstrate the
The typical ESS patient is middle there are several characteristics of herniated subarachnoid space.15,16
aged, female, and has normal endo¬ the ESS headache that can distin¬ Generally, ESS is a benign condi¬
crine function.2-610 When a pituitary guish it from other common headache tion with a good prognosis; neverthe¬
function abnormality is present, it is syndromes. Notably, the headaches less, it is the clinician's responsibility
most commonly hyperfunction sec¬ usually lack the family history and to rule out an intrasellar neoplasm.
ondary to a microadenoma. Acromeg- related phenomena of migraines such Equally as important, however, is the
aly, amenorrhea-galactorrhea, and as aura, nausea, and vomiting. Unlike follow-up of these patients. Although
Cushing's disease have been men¬ the common tension headache, the uncommon, the possible development
tioned in the literature.2-911 The empty sella headache is frequently of visual field disturbances and pitu¬
reported casesof demonstrated hypo- unilateral and anterior. Pathogenical- itary function abnormalities necessi¬
function have involved corticotropin ly, the headache has been postulated tates periodic examinations. Any sig¬
deficiency, panhypopituitarism, and to arise secondary to traction on pain- nificant change in the headache pat¬
impaired growth hormone response to sensitive vascular-meningeal struc¬ tern or intensity, endocrine function,
glucagon.2-910 There has been no corre¬ tures in the sellar cavity. On occasion, visual field deficits, or the develop¬
lation between the degree of empti¬ the headache has been relieved follow¬ ment of rhinorrhea should alert the
ness of the sella and pituitary func¬ ing surgical exploration of an empty clinician to reevaluate the patient for
tion.12 sella.14 possible complications of ESS. Until
Visual symptoms and CSF rhinor¬ When evaluating the patient com¬ that time, symptomatic treatment of
rhea are rare. In contrast, headaches plaining of headaches, ESS should be the headache should be the primary
are the predominant complaint in considered a possible cause when mode of therapy.912 Methods of surgi¬
60% to 80% of patients found to have there is roentgenographic evidence of cal therapy, although implied, have
ESS.6-9-1213 The characteristic headache an enlarged sella, the lack of head- not been directly addressed.914

References

1. Busch W: Die Morphologie der Sella turcica 7. Bergerson C, Kovacs K, Bilbao JM: Primary 13. Jaffer KA, Obbens EA, El Gammal TA:
und ihre Beziehungen zur Hypophyse. Virchows empty sella: A histologic and immunocystologic 'Empty' sella: Review of 76 cases. South Med J
Arch Pathol Anat 1951;320:437-458. study. Arch Intern Med 1979;139:248-249. 1979;72:294-296.
2. Brismar K, Efendic S: Pituitary function in 8. Jordan RM, Kendall JW, Kerber CW: The 14. Laws ER, Ebersold MJ, Kern EB, et al: The
the empty sella syndrome. Neuroendocrinology primary empty sella syndrome. Am J Med results of transsphenoidal surgery in specific
1981;32:70-77. 1977;62:569-580. clinical entities, in Laws ER, Randall RV, Kern
3. Kaufman B: The 'empty' sella turcica: A 9. Spaziante R, de Divitiis E, Stella L, et al: EB, et al (eds): Management ofPituitary Adeno-
manifestation of the intrasellar subarachnoid The empty sella. Surg Neurol 1981;16:418-426. mas and Related Lesions With Emphasis on
space. Radiology 1968;90:931-941. 10. Ekblom M, Ketoner L, Kuuliala I, et al: Transsphenoidal Microsurgery. New York,
4. Bjerre P, Gyldensted C, Riishede J, et al: Pituitary function in patients with enlarged sella Appleton-Century-Crofts, 1982, pp 277-305.
The empty sella and pituitary adenomas: A theo- turcica and primary empty sella syndrome. Acta 15. Sage MR, Chan E, Reilly PL: The clinical
ry on the causal relationship. Acta Neurol Scand Med Scand 1981;209:31-35. and radiological features of the empty sella
1982;66:82-92. 11. Dominque JN, Wing SE, Wilson CB: Coex- syndrome. Clin Radiol 1980;31:513-519.
5. Davis S, Tress B, King J: Primary empty isting pituitary adenomas and partially empty 16. Price MJ, Corbett J, Thompson HS: Diag-
sella syndrome and benign intracranial hyper- sellas. J Neurosurg 1978;48:23-28. nosis of the empty sella with intrathecal metri-
tension. Clin Exp Neurol 1978;15:248-257. 12. Neelon FA, Goree JA, Lebovitz HE: The zamide computed tomography. Surv Ophthalmol
6. Moretti G, Manzoni GC, Mainini P, et al: primary empty sella: Clinical and radiographic 1983;28:42-44.
Empty sella headache. Headache 1981;21:211\x=req-\ characteristics and endocrine function. Medicine
217. 1973;52:73-92.

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