«“√ “√®Case Reports—°…ÿ∏√√¡»“ µ√ : Visual å Field ªï∑’Ë 3 ©∫Defect—∫∑’Ë 1 in¡°√“§¡-¡ Primary‘∂ ÿπ“¬πEmpty 2551 Sella Syndrome 5

Case Reports : Visual Field Defect in Primary Empty Sella Syndrome

Jenjit Choovuthayakorn, MD.* Rungkiat Changwiwit, MD.* Watana Navacharoen, MD.** Sopa Wattananikorn, MD.* *Department of Ophthalmology, Faculty of Medicine, Chiang Mai University **Department of Internal Medicine, Faculty of Medicine, Chiang Mai University

ºŸâªÉ«¬ empty sella syndrome πÕ°®“°¡’§«“¡º‘¥ª°µ‘‡°’ˬ«°—∫√–∫∫ŒÕ√å‚¡π·≈â« ¬—ßÕ“® àߺ≈°√–∑∫ µàÕ≈“𠓬µ“¢ÕߺŸâªÉ«¬¥â«¬ ¥—ßπ—ÈππÕ°®“°®—°…ÿ·æ∑¬å®– “¡“√∂„Àâ°“√√—°…“ªí≠À“‡°’ˬ«°—∫≈“𠓬µ“¢Õß ºŸâªÉ«¬·≈â« ¬—ßÕ“®™à«¬π”‰ª Ÿà°“√«‘π‘®©—¬ºŸâªÉ«¬∑’Ë¡’ªí≠À“ empty sella syndrome ‰¥âµ—Èß·µà„π√–¬–µâπ‰¥âÕ’°¥â«¬

Introduction Typically primary empty sella syndrome Empty sella occurs as a result of a deficient occurs in obese, multiparous women, ranging in diaphragma sellae, with subarachnoid space age from 27 to 72 years, with a mean age of protruding into the cavity of sella tursica. Pituitary 49 years old. Empty sella Aas been associated gland is flattened and distorted against the sella with hypertension, pseudotumor cerebri, hypopi- floor and walls. The sella is usually enlarged, but tuitarism, spontaneous cerebrospinal fluid (CSF) this feature is not always present. The term rhinorrhoea, visual field defects, diminished visual çprimary empty sellaé is used when anomaly occurs acuity and headache4. in patients who never had surgery radiation on Endocrine abnormalities are not commonly pituitary or Para pituitary tumor1. The diagnosis found in empty sella patient. However if such of empty sella is radiographically made when the abnormalities are found, they are most commonly sella tursica is seen to be enlarged or deformed found in the form of Hyper-prolactinaemia. Growth that is partially or completely filled with cerebrospinal hormone, thyrotropin and gonadotropin deficien- fluid2. cies are also noted. To establish the diagnosis Most patients with primary empty sella are of hypopituitarism (partial or complete) and to asymptomatic and incidentally detected. The determine the hypophyseal cause of dysfunction, presence of empty sella was found in 5 percent measurement of the following hormones are of normal subjects on autopsy studies3. required in order to obtain serum thyrotropin, T4, 6 Jenjit Choovuthayakorn et al. corticotrophin, cortisol, LH, testosterone, basal The endocrine studies showed hypothy- stimulated growth hormone and prolactin level5. roidism and adrenal insufficiency, the patient was The cause of visual field changes is due then replaced with hormone therapy. Finally, the to either traction on the chiasm or involvement Patient was operated through trans-sphenoidal of chiasmal blood vessels, which result in ischemic approach which has been traditionally known as damage to the optic nerve2. çchiasmapexyé. On during the 4 months follow In this report we describe two patients with up, there was still no improvement on his visual primary empty sella syndrome, who had visual fields. field defected.

Case reports Case 1 A 33-year-old man has chronic progres- sive visual loss in both eyes one year prior came OS OD to the hospital. On eye examination, his visual acuity measured to 20/20 in the right eye and 20/20 in the left eye. Anterior segment was examined and found to be within normal limit in both eyes and no RAPD. The IOP measured 12 and 10 OS OD mmHg in the right and the left eye respectively. There were normal cupping with sharp disc margin, Figure 1 This 33-year-old man presented with however they both looked pale. Visual field showed chronic progressive visual loss. Visual a tubular field defect in both eyes. (Fig 1) The acuity was 20/20 bilaterally. Fundus Visual fields were thought to be highly suggestive shows bilateral optic disc atrophy. A; of chronic papilledema that had progressed into Visual fields performed by Goldmann atrophic stage. The patient was thought to have perimetry shows central island of developed pseudotumor cerebri and this was vision on both eyes. B; 4 months after confirmed by high initial pressure in lumbar chiasmapexy, there was still no puncture. improvement on his visual fields. MRI of brain revealed an empty sella. Most Case 2 of the sella was filled with CSF and traction of A 56-year-old multiparous woman was the optic nerves and chiasm. Based on these find- found to have multinodular goiter and hyperten- ings, a diagnosis of primary empty sella syndrome sion after feeling ill about ten years prior to this was made. Case Reports : Visual Field Defect in Primary Empty Sella Syndrome 7 visit. She was a regular attendant of the out patient department with good control of both underlying conditions. Her illness began 2 months ago when she first developed painless visual loss in both eyes. OS OD The visual acuity was found to be hand motion in the right eye and 10/200 in the left eye. The Figure 2 This 56-year-old woman presented intraocular pressure (IOP) measured to be 15 and with chronic progressive visual loss. 16 mmHg in the right and left eye respectively. Visual acuity was hand motion in the Fundoscopic examination showed a full blown right eye, 10/200 in the left eye. Fun- bilateral papilledema with sphincter hemorrhage. dus shows bilateral papilledema with Visual field showed an inferonasal and central field sphincter hemorrhage. Visual fields defects in the right eye and two inferior para- done by Goldmann perimetry shows central scotomas in the left eye. (Fig 2) MRI of inferonasal and central field defects in the brain revealed flattening of pituitary gland at the right eye and two inferior para- floor of sella turcica with normal midline of pi- central scotoma in the left eye. tuitary stalk. There was no herniation of the optic chiasm or nerve into the sella tursica Neurological Discussion examination was completely normal as well as the Primary empty sella syndrome is postu- endocrinological examination. Lumbar puncture lated as a congenital defect in diaphragm, coupled with normal and constant pulsation of cerebrospi- revealed high initial pressure of 23 mmH2O. She was diagnosed as pseudotumor cerebri with primary nal fluid, pushes the subarachnoid space further empty sella syndrome and acetazolamide was and further into the sella, which results in the prescribed. The papilledema was seen to have compression of pituitary gland and the enlarge- improved in during the three months follow up, ment of sella5. This enlargement of sella turcica and the repeated lumbar puncture showed a normal can be caused by pituitary tumor, suprasellar tumor, initial pressure. optic neuroma, tuberculum sella meningioma, intratrasellar internal carotid syndrome, or increased intracranial pressure4. 8 Jenjit Choovuthayakorn et al.

Empty sella is a term that was first in- incidence of the signs of primary empty sella was troduced by Busch in l951 to describe herniation observed with aging, the increase being more of the suprasellar arachnoid space, and possibly conspicuous in the 5th decade of life in females the optic nerve, into the sella turcica because of (37.5%) and in the 6th decade in males (40%). a diaphragmatic defect. Busch performed an On the whole, signs of primary empty sella were autopsy study of 788 subjects without known detected in 140/500 cases (38%), in 9.6% of the pituitary disease. In 38.4% he found a complete subjects under 40 and in 39.9% of those above covering of pituitary gland by the diaphragm. He 407. observed an empty sella in 5.5%. In 1926 Schaeffer Reported mechanisms of visual loss may have been the first to observe various associated with primary empty sella include anatomical forms of the diaphragma sellae; these prolapsed of chiasm into the sella, compression ranged from densely thick with a complete roof of chiasm from above by the anterior cerebral to that composed of a peripheral veil. Busch, in arteries, and occasionally, the involvement of optic his study of 788 sellae in patients without a history nerves and chiasm by arachnoidal adhesion6. The of pituitary disorders, classified the diaphragma incidence is approximately 20% in primary empty sellae into the following categories, which were sella syndrome. In secondary empty sella syn- later resorted by Kaufman in l9723. drome, the incidence is much higher because of Primary empty sella syndrome typically the underlying sellar pathology. occur in middle-aged, obese, multiparous woman, Pituitary function is stated to be normal many of whom are also have hypertension and in most patients. Only 31% of these were referred pseudotumor cerebri6. The associated disorders due to endocrinological problems. There is a of an empty sella are mucopolysaccharidosis, general agreement in the literature that anterior virilization and renal tubular acidosis. Headache pituitary dysfunction necessitating hormonal is the most common symptom and with no replacement therapy is rare in primary empty sella characteristic pattern or localization. In 1991, Foresti syndrome. Pituitary hyper-secretion strongly points reported 500 consecutive patients, aged from 11 to the presence of a pituitary adenoma that is to 82 years, who underwent MR imaging of the partly necrotized. The involvement of the posterior brain for a variety of conditions not related to pituitary gland rarely occurs. To establish the pathologic processes of the sellar or juxtasellar diagnoses of hypopituitarism, the measurement of regions, this report showed primary totally empty the following hormones are required; serum thy- sella in 28/248 males (11.3%) and in 34/252 rotropin, T4, corticotrophin, cortisol, LH, testoster- females (13.5%). Primary partially empty sella was one, basal and stimulated growth hormone and found in 40/248 males (16.1%) and in 38/252 prolactin levels. Children with empty sella and GH females (15.1%). A progressive increase in the deficiencies may be prone to develop rhGH- Case Reports : Visual Field Defect in Primary Empty Sella Syndrome 9 induced pseudotumor cerebri. The resumption of of herniation of the SVS in either the primary or rhGH therapy after 3 months at a lower dose lead secondary sellae9. to an excellent catch-up growth without the Patients with primary empty sella are recurrence of pseudotumor cerebri8. asymptomatic and do not require any manage- Visual field defects were bitemporal hemi- ment except if visual deficits are developed or anopia, binasal hemianopia, quadrantanopia, cerebrospinal fluid leakage occurs. Surgical treat- generalized field constriction, central scotoma, ment would then be considered to be necessary. homonymous hemiachromatopsia1. Pollock and Most of the empty sella cases that need surgical Bromberg2 suggested that stable visual field treatment are operated on through a transs- defects, particularly nasal defects or generalized phenoidal approach. This procedure has been constriction in primary empty sella syndrome might traditionally known as çchiasmapexyé10. be secondary to previous, under diagnosed From previous discussion, it can be said pseudotumor cerebri3. Harri Rouhiainen et al1 that it is necessary to properly perform both suggested that primary empty sella syndrome was neurological, endocrinological assessment with found in patients with glaucomatous optic disc and ophthalmologic examination on patients who have visual field changes, so follow up visual field is visual field defects and thought to be associated essential. Only when no progression is observed with pseudotumor cerebri with primary empty sella. primary empty sella syndrome be blamed for defect Particular attention should be paid to the IOP and unnecessary to start the anti-glaucomatous measurement, optic disc and visual field patterns therapy6. Although primary empty sella syndrome on follow up visits. may cause visual field changes through mechani- cal traction or ischemic effects on chiasm, visual References field defects are not always secondary to the 1. Rouhiainen H, Terasvirta M. Co-existence of syndrome. Therefore if there are some degrees empty sella syndrome and low tension of glaucomatous field and disc changes are glaucoma. Acta Ophthalmol 1989;67:367-70. detected, further investigation for glaucoma should 2. Bettie AM, Trope GE. Glaucomatous optic be performed2. neuropathy and field loss in primary empty Visual disturbances may be absent or sella syndrome. Can J Ophthalmol 1991; minimal in primary empty sellae and secondary 26:377-82. empty sellae with herniation of the supra sella 3. Busch w Die morphologie der sella und ihre visual system. Progression of the symptoms--visual beziehungen zur hypophyse. Virchows Arch field defects, optic atrophy, and loss of vision is 1951;320:437. not inevitable. There was no correlation between 4. Niphon P, Apichati V, Adulya V. The primary the severity of visual symptoms and the degree empty sella syndrome: A report of 3 cases 10 Jenjit Choovuthayakorn et al.

with computed axial tomography and a com- 8. Francois I, Casteels J, Silberstein P. Empty plete pituitary function study. Siriraj Hosp Gaz sella growth hormone deficiency and pseudo- 1983;35:65-71. tumor cerebri: effect of initiation withdrawal and 5. Maire TB, Muhammed H, Thomas JC. resumption of growth hormone therapy. Eur Primary empty sella syndrome with visual field J Pediatr 1997;156:67-70. defects. The American Journal of Medicine 9. B Kaufman, RL Tomsak, BA Kaufman, BU 1976;61:124-8. Arafah, EM Bellon, WR Selman, and MT Modic. 6. Pollock SC, Bromberg BS. Visual loss in a Herniation of the suprasellar visual system and patient with primary empty sella. Arch third ventricle into empty sellae: morphologic Ophthalmol 1987;105:1487-8. and clinical considerations. American Journal 7. Foresti M, Guidali A, Susanna P. Primary empty of Roentgenology 1989;152:597-608. sella. Incidence in 500 asymptomatic subjects 10. Gerardo G, Ramiro DV, Elisa Nl. Primary empty examined with magnetic resonance 1991; sella syndrome: the role of visual system 81:803-7. herniation. Surgical Neurology 2002;58:47-8.