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Empty Sella Syndrome Associated with Partial Hypopituitarism (Visualized on MRI Scan)

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Empty Sella Syndrome Associated with Partial Hypopituitarism (Visualized on MRI Scan) • • Empty sella syndrome associated with partial hypopituitarism (visualized on MRI scan) PETER C. SERPICO, DO JEFFREY S. FREEMAN, DO BURTON MARKS , DO The empty sella syndrome has moencephalography, cerebrospinal fluid evalu­ become a rather frequent finding in mag­ ation, metrizamide infusions, and angiography netic resonance imaging (MRI) scanning. are costly and not without risk. The usual presentation of empty sella syn­ We present a case of empty sella syndrome drome involves an incidental finding on associated with hypopituitarism that illus­ a computed tomography scan or an MRI trates these unusual circumstances. The newer scan. It is unusual to find empty sella syn­ type of MRI scanner aided in the establish­ drome associated with hypopituitarism. ment of this diagnosis, obviating the need for The authors describe such an unusual find­ further invasive imaging studies. ing in a 54-year-old nulliparous woman. (Keywords: Empty sella syndrome, hy­ Report of case popituitarism, magnetic resonance imag­ A 54-year-old nulliparous woman came to our medi­ ing, pituitary function) cal center complaining of bilateral lower extrem­ ity edema that had been progressing during a 6- month period. She also complained of generalized The empty sella syndrome occurs when the weakness, fatigue, cold intolerance, and weight subarachnoid space extends through the dia­ gain, which had begun 3 months previously. The phragma sellae into the infrasellar space in patient's past medical history included sinusitis, association with one or more clinically recog­ anemia, chronic bronchitis, and osteoarthritis. Her nized features that will be discussed later. Al­ surgical history included two previous dilation and though reports in the literature indicate a gen­ curettage procedures for persistent menorrhagia. erally benign course for this syndrome, it ap­ She reported that there had been no other surger­ pears that there is a certain subset of patients ies nor any history of radiation therapy. who may harbor a pituitary tumor.l The patient weighed 60 kg and had a blood pres­ Frequent evaluations of pituitary function sure reading of 130/60 mm Hg. Her mental status can help to distinguish primary empty sella appeared dulled. The scalp hair was coarse. There 1 was no evidence of macroglossia. The pupils were from pituitary tumors in some patients. With equally reactive to light and accommodation. The the advent of high-resolution computed to­ funduscopic examination revealed no evidence of mography (CT) and magnetic resonance im­ papilledema, hemorrhage, or exudates. The neck aging (MRI) , the affirmation of primary empty was supple and the thyroid gland was of normal sella syndrome may not require more invasive size. There were bilateral carotid bruits. procedures to establish the diagnosis. Pneu- Examination of the heart revealed a regular rhythm at 84 beats per minute with a IINI sys­ From the Osteopathic Medical Center of Philadelphia, where, Dr Freeman is professor and chairman, Division tolic ejection murmur at the second right intercos­ of Endocrinology and Metabolism, and where, at the time tal space. The lungs were clear to auscultation and this article was written, Dr Serpico was a resident in percussion bilaterally. The abdomen was obese, internal medicine and Dr Marks was professor of radio 1- soft, and nontender. Bowel sounds were present. ogy. Drs Serpico and Marks are currently in clinical prac­ The integument was sallow, dry, and of normal tem­ tice. perature. There was evidence of bilateral pedal Reprint requests to Jeffrey S. Freeman, DO , 4190 City Ave, Rowland Hall, Suite 501 , Philadelphia, PA 19131- edema. The reflexes were graded + 2/4 and slightly 1696. hung-up. 1172 • JAOA • Vol 92 • No 9 • September 1992 Case report • Serpico et al Figure Left frame: A pituitary MRI scan in the sagittal projection with the 0.5 Tesla GE Max using a Tl-weighted pulse sequence. Pituitary gland is displaced inferiorly and anteriorly. Tl-weighted pulse sequences reveal characteristic low signal intensity of the cerebrospinal fluid (arrowhead), and high signal intensity of the pituitary gland (open arrow). Right frame: Coronal view: Pituitary is displaced inferiorly (open arrow); cerebrospinal fluid (arrowhead). Laboratory studies revealed the following abnor­ phragm sella permitting herniation ofthe suba­ mal values (normal values are in parentheses): thy­ rachnoid space into the infrasella region. Some roxine radioimmunoassay, 2.5 j..Lg/dL (4.4-12.5 j..Lg/ series report the incidence of this to be as high dL); triiodothyronine resin uptake, 32% (25%-35%); as 25% of autopsy patients.2 free thyroxine index, 0.39 (1.1-4.3); thyroid stimu­ Empty sella syndrome may be classified as lating hormone less than 0.75 j..LUlmL (1-10 j..LUI a primary congenital disorder or as secondary mL); random cortisol, 1.0 j..Lg/dL (5-15 j..L/dL). Follicle­ stimulating hormone and luteinizing hormone val­ to surgery, irradiation, postpartum infarction ues were less than 1.0 mIU/mL. The prolactin value (Sheehan's syndrome), or tumors. The empty was 29.7 ng/mL (6-24 ng/mL). sella denotes the radiologic appearance, which Hormonal replacement therapy with L-thyrox­ results from the aforementioned downward her­ ine and hydrocortisone was started. The patient niation of subarachnoid CSF. Forsham states underwent radiographic studies. A sella turcica x­ that it is the most common cause of enlarged ray study showed normal results. A pituitary MRI sella turcica.3 scan revealed evidence of a partial empty sella (Fig­ When concomitant features-such as head­ ure). No associated tumor or aneurysm was noted. ache, visual disturbances, papilledema, CSF The patient was continued on hormonal therapy. rhinorrhea, or endocrine dysfunction-occur, She had gradual improvement of hypopituitarism the condition is termed the empty sella syn,­ symptoms. drome.4 The syndrome is seen most commonly in obese, middle-aged, multiparous women. 1 Discussion Other significant associations include hyper­ When cerebrospinal fluid (CSF) is noted to tension, pseudotumor cerebri, hydrocephalus, have partially or completely filled the sella tur­ and congestive heart failure. Less frequent as­ cica, the diagnosis of "empty sella" can be es­ sociations include diabetes insipidus, hyper­ tablished. It is thought that this condition oc­ prolactinemia, and renal tubular acidosis.5,6 curs as a result ofthe incompetence ofthe dia- Magnetic resonance imaging has several Case report • Serpico et al JAOA, • Vol 92 • No 9 • September 1992 • 1173 advantages over CT scanning in the evaluation empty sella. 12 Both high-resolution CT scan­ of empty sella syndrome.7,8 These include the ning and MRI scanning are useful in distin­ absence of ionizing radiation, the absence of guishing empty sella from a large, radiolucent, the need to use contrast material, the mul­ nonenhancing pituitary tumor.13 In so doing, tidirectional capabilities of MRI, and the ex­ they may obviate the need for more invasive cellent anatomic detail that can be obtained studies, thereby reducing overall costs and without bone artifact.9,lO Computed tomogra­ alleviating patient risk. phy with its noninvasive quality has sup­ planted pneumoencephalography, and now 1. Jordan RM, Kendall JW, Kerber CW: The primary empty MRI will probably supplant CT scanning. sella syndrome. Am J Med 1977;62:569-580. On spin density (TR 2000, TE 30), the pitui­ 2. Bergland RM, Ray BS, Torack RM: Anatomical variations tary gland appears to be of moderate signal in the pituitary gland and adjacent structures in 225 human intensity, whereas the CSF is oflow signal in­ autopsy cases. J Neurosurg 1968;28:93-99. 3. Greenspan FS, Forsham PH: Basic and Clinical Endocri­ tensity. On T2-weighted pulse sequences (TR nology, ed 2. Los Altos, Calif, Lange Medical Publications, 1986, 2000, TE 90), CSF is of high signal intensity p 66. and the pituitary gland is of relatively low sig­ 4. Stanhope R, Adlard P: Empty sella syndrome. Dev Med Child nal intensity.9,lO Tl-weighted pulse sequences Neurol 1987;29:397-399. 5. Bar RS, Mazzaferri EL, Malarkey WB: Primary empty sella, show a characteristic low signal intensity of galactorrhea, hyperprolactinemia and renal tubular acidosis. the CSF and the higher signal density of the Am J Med 1975;59:863-866. pituitary. The posterior lobe of the pituitary 6. Matisonn R, Pimstone B: Diabetes insipidus associated with gland has a characteristic higher signal inten­ an empty sella. Postgrad Med J 1973 ;49:274. 7. National Academy of Sciences NCRP report No. 86. Biologi­ sity caused by the cholesterol content of the cal Effects and Exposure Criteria for Radiofrequency Electro­ cells. 11 magnetic Fields. Recommendations of the National Council on Radiation Protection and Measurement, Bethesda, Md, April In the case of the patient reported here, the 2, 1986. pituitary gland was displaced inferiorly and 8. Saunders RD, Smith H: Safety aspects of NMR clinical im­ anteriorly. The excellent anatomic detail aging. Br Med Bull 1984;40(2):148-152. should rule out all equivocation in the diag­ 9. Bydder GM: Nuclear magnetic resonance imaging of the nosis. brain. Br Med Bull 1984;40(2):170-174. 10. Bydder GM, Steiner RE, Young IR, et al: Clinical NMR im­ Summary aging of the brain: 140 cases. AJR 1982;139:215-236. 11. Mark L, Pech P, Daniels D, et al: The pituitary fossa: A The history and findings regarding the patient correlative anatomic and MRI study. Radiology 1984;153:453. described here are consistent with empty sella 12. Brismar K, Efendic S: Pituitary function in the empty sella syndrome with associated panhypopituitarism. syndrome. N euroendocrinology 1981;32:70-77. 13. Naheedy MH, Haag JR, Azar-Kia B, et al: MRI and CT of Increasingly, endocrine dysfunction and pitui­ sellar and pansellar disorders. Radiol Clin North Am tary tumors are being associated with the 1987;25:819-847. 1174 • JAOA • Vol 92 . No 9 . September 1992 Case report • Serpico et al .
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