Neuro-Ophthalmology
ISSN: 0165-8107 (Print) 1744-506X (Online) Journal homepage: http://www.tandfonline.com/loi/ioph20
Foster Kennedy syndrome caused by an extensive pituitary adenoma Michael Küchleand & Hendrikus Seyer To cite this article: Michael Küchleand & Hendrikus Seyer (1992) Foster Kennedy syndrome caused by an extensive pituitary adenoma, Neuro-Ophthalmology, 12:2, 77-83, DOI: 10.3109/01658109209058121 To link to this article: http://dx.doi.org/10.3109/01658109209058121
Published online: 08 Jul 2009.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ioph20 Download by: [New York University]
Date: 31 March 2016, At: 09:23
0 Aeolus Press Neuro-ophthalmology 0165-8 107/92/US$3.50
(Accepted 4 June 1991)
Foster Kennedy syndrome caused by an extensive pituitary adenoma MICHAEL KUCHLE’* and HENDRIKUS SEYER2
Departments of Ophthalmology and 2Neurosurgery, University Erlangen-Nurnberg, Erlangen, FRG Downloaded by [New York University] at 09:23 31 March 2016
~~
~~
~
ABSTRACT. A 5 I-year-old woman presented with signs of Foster Kennedy syndrome including right papilledema. left simple optic atrophy, and left anosmia. CT scan and magnetic resonance imaging disclosed a giant pituitary tumor growing superiorly and anteriorly to the left and compressing the chiasm and the left optic nerve. Endocrinological work-up and histology confirmed the diagnosis of a non-secreting pituitary adenoma. The possible mechanisms causing ipsilateral optic atrophy and contralateral papilledema in this case are discussed. The authors’ case seems unique since it represents, to their knowledge, the first report of a pituitary adenoma presenting with the full picture of Foster Kennedy syndrome. Pituitary adenoma should not be excluded from the differential diagnosis of lesions presenting as Foster Kennedy syndrome. Key words: Foster Kennedy syndrome; pituitary adenoma; papilledema ; optic atrophy
INTRODUCTION
processes. To our knowledge, the occurrence of FKS in a patient with pituitary adenoma has never been reported. In this paper we describe a case of nonfunctional giant pituitary adenoma which presented clinically as FKS.
Foster Kennedy syndrome (FKS) is a rare entity of clinical signs. It consists of ‘primary optic atrophy on the side of the lesion together with concomitant papilledema in the opposite eye’ (Kennedy 1911’) and usually occurs with frontobasal tumors, most frequently meningiomas of the CASE REPORT sphenoid wing and the olfactory g r o o ~ e ~ . ~ . A 51-year-old white female was referred to our in‘True’ FKS also includes anosmia or hyposmia, stitution after consulting her ophthalmologist bethus consisting of a triad which actually is only cause of deteriorating vision. For several years, she found in a few extraordinary cases. FKS has had experienced insidiously progressing visual loss been observed in a variety of intracranial lesions of her left eye but had not seen an ophthalmologist until now. During the last three months prior to including neoplasms and non-neoplastic changes admission, she had also noticed some visual deterisuch as infectious, inflammatory and vascular
*
Correspondence 10: Michael Kiichle, M.D.,University Eye Hospital, Schwabachanlage 6, W-8520 Erlangen,
BRD
Neum-ophthalmology - 1992, Vol. 12, No. 2, pp. 77-83 0 Aeolus Press Buren (The Netherlanh) 1992
oration of her right eye. Her past medical history was unremarkable except for a hysterectomy four years earlier. Apart from her visual loss, she did not complain of any symptoms. On ophthalmological examination, visual acuity
77
M . Kuchle and H. Seyer
Downloaded by [New York University] at 09:23 31 March 2016
~~
was 20/20 unaided in her right eye and light perception in her left eye. Intraocular pressure was 17 mm Hg in both eyes using applanation tonometry. The left eye showed exotropia and a marked afferent pupillary defect. Ocular motility was undisturbed in both eyes. On slitlamp examination, the anterior segment of both eyes showed no abnormalities. Fundus examination of the right eye disclosed a markedly swollen disc (prominence 5 dpt.) with dilated capillaries on the disc surface, obliteration of the physiologic central cupping, blurred disc margins obscuring the surface vessels, and some small splinter hemorrhages in the parapapillary nerve fiber layer (Fig. la). On the left side, the disc showed advanced simple optic atrophy without prominence or blurring of the disc margins (Fig. 1b). The retinal arterioles appeared narrowed and the retinal nerve fiber layer of the left eye was not discernible. Kinetic perimetry of the right eye showed an incomplete temporal hemianopia and an enlarged blind spot (Fig. 2). Perimetry of the left eye could not be performed (V/5 not discerned). As FKS was diagnosed, an intracranial lesion was suspected and the patient was referred to the
neurosurgical department for further diagnostic evaluation. On neurologic examination, the patient was alert and well oriented. She gave the impression of being slightly euphoric. There was a left anosmia and a subtle, mainly leftsided tetraspasticity. Sensitivity and coordination were normal. Paresis of the extremities did not exist. Roentgenograms of the skull revealed an enormously enlarged sella turcica (Fig. 3) and destruction of its posterior wall. CT scan disclosed a large mass in the left frontal lobe, which was evidently growing up from the sella turcica (Fig. 4). The tumor elevated the wall of the left lateral ventricle and led to a midline deviation of about 1 cm to the right. I t was contrast-enhancing. Surrounding brain edema was evident. Magnetic resonance imaging showed a large space-occupying lesion which arose from the sella turcica in front of the brainstem, lifted the left frontal lobe up and bulged the left lateral ventricle (Fig. 5 ) . The tumor compressed the optic chiasm and the left optic nerve between the chiasm and the optic canal. The left anterior cerebral artery was
Fig. 1. A. Right optic nerve head showing marked papilledema with prominent disk, blurred disk margins, engorged retinal veins and dilated capillaries. B. Right optic nerve head showing advanced simple optic atrophy. Note attenuated retinal arterioles.
78
Downloaded by [New York University] at 09:23 31 March 2016
Foster Kennedy syndrome due to pituitary adenoma
Fig. 2. Visual field of right eye showing incomplete temporal hemianopia and enlargement of the blind spot.
Fig. 3. Roentgenogram showing
tor
enormously enlarged sella turcica and destruction of its posterior wall.
79
Downloaded by [New York University] at 09:23 31 March 2016
M. Kuchle and H . Seyer
Fig. 4. CT scan showing a large pituitary tumor growing superiorly and anteriorly. Note elevation of the wall of the left lateral ventricle and midline deviation to the right.
displaced backwards. Hydrocephalus had not yet developed. Thus, a giant pituitary adenoma was suspected radiologically. Endocrinological investigation (stimulation of the anterior pituitary lobe with ACTH, TRH and LRH ; insulin-induced hypoglycemia; stimulation with CRH and GRH) showed mild hyperprolactinemia but surprisingly no hypocortisolism. Growth hormone was normal. Hypothalamic regulation seemed to be intact. Hence, a non-functional adenoma was probable. The tumor was nearly inoperable. However, the patient was under the threat of getting blind in both eyes and her life being shortened by the tumor growth. She was treated therefore with dexamethasone for one week and operated via the left subfrontal approach. As much tumor tissue as possible was removed and the chiasm decompressed. Histological examination of excised tumor tissue disclosed a non-functional pituitary adenoma. Unfortunately, the patient experienced malignant brain edema postoperatively and developed
80
Fig. Magnetic resonance showing a large pituitary tumor and compression of the chiasm and left optic nerve.
maximal intracranial pressure. She died on the fourth day after operation.
DISCUSSION FKS representing the combination of optic atrophy with contralateral papilledema is an infrequently encountered combination of clinical signs. FKS was first described in 1905 by SchultzZehden4 in a patient with a cholesteatoma of the anterior cranial fossa. Its diagnostic value, however, was established in 191 l by F. Kennedy'. FKS is a rare finding. Francois and Neetens5, in 1955, found a total of 169 cases in the literature. Bynke2 saw 17 cases of FKS among 1400
Downloaded by [New York University] at 09:23 31 March 2016
Foster Kennedy syndrome due to pituitary adenoma patients with expanding lesions of the brain. In Trobe22 recently reported a boy with FKS and a series of 3033 intracranial tumors, Tonnis6 juvenile nasopharyngeal angiofibroma without found 28 cases of FKS. Skrzypczak7 reported 19 increased intracranial pressure. The authors becases of FKS among 2000 patients with histologi- lieved that FKS was caused by bilateral, asymcally proven intracranial tumors. Huber8 saw metric optic nerve compression, and in a review of 36 previously reported cases of FKS they susfive cases of FKS among 8100 brain tumors. In 'true' FKS, there is anosmia or hyposmia in pected that in 12 of these cases FKS was also addition to papilledema and optic atrophy as was caused by bilateral optic nerve compression. In our case, the leftsided optic atrophy and originally described by Kennedy9, but olfactory dysfunction is only seen in a small portion of anosmia were obviously caused by direct tumor compression to the optic nerve and olfactory patients with FKS2* FKS is usually associated with frontobasal tu- tract (Fig. 5 ) . Contralateral papilledema may mors, most frequently sphenoidal ridge me- have been the result of increased intracranial n i n g i o m a ~ ~ . ~17-*I*, olfactory groove and also pressure caused by tumor volume. However, disuprasellar m e n i n g i o m a ~ ~ *19,~ , but other tu- rect compression of the right optic nerve near the chiasm may also have played'a role in the develmors such as g l i o b l a ~ t o r n a ~ .astrocyto~~~~, ma7,20, olig~dendroglioma~~, meningioma of the opment of papilledema similar to the mechafalx cerebri2', ch~lesteatoma~, craniopharyngio- nisms proposed by Watnick and Trobe22. Addima2.7, nasopharyngeal angiofibroma22, tionally, compression of the chiasm had led to myxo~hondroma~,ganglio~ytoma~, lymph- the rightsided superior temporal visual field dea n g i o ~ a r c o m aand ~ ~ metastases7 have been de- fect (Fig. 2). In contrast to tumors of the anterior cranial scribed in conjunction with FKS. About onethird of cases of FKS are caused by non-tumorous fossa, pituitary adenoma typically presents with lesions such as abscess of the frontal lobe1*2*13,bitemporal visual field loss and optic atrophy. Papilledema is characteristically absent in cases opticochiasmal ara~hnoiditis'~. 24.25, osteomyelitisI3, sclerotic carotid arteries5*l2,aneurysm of of pituitary adenoma3' and is only very infrethe internal carotid arteries9. 26, cerebral syphilis' quently seen in cases in which pituitary adenoma causes h y d r o ~ e p h a l u s ~On ~ . reviewing some of and Paget's disease5. FKS should be distinguished from the so- the literature on pituitary adenomas, we found called pseudo-FKS which is seen in patients with that only in two of 1197 reported cases of pituiunilateral ischemic optic neuropathy and contra- tary adenoma (1.7%) papilledema was obserlateral, usually postischemic, optic a t r o ~ h y ~ ~vedl0,15,31-38. ,~~. Our case seems to be unique since it is, to our The mechanisms by which FKS develops are discussed controversially. Its pathogenesis is usual- knowledge, the first report of a pituitary ly believed to consist of ipsilateral direct optic adenoma presenting with the complete triad of nerve compression causing optic atrophy, and FKS that is explainable by tumor extension simisubsequent increase of intracranial pressure lar to olfactory groove or other frontobasal meleading to contralateral papilledema1*29.30. Oth- ningiomas. Despite the enormous size of the er authors believed that bilateral papilledema pituitary adenoma, the only symptoms that our may lead to early optic atrophy on one side, thus patient had experienced were longstanding, resulting in a picture of FKS7g20. Watnick and slowly progressive visual loss that had finally
81
M. Kiichle and H. Seyer made her consult an ophthalmologist. Although presenting only very rarely with symptoms of FKS, pituitary adenoma should be included in the differential diagnosis of lesions causing FKS.
ACKNOWLEDGEMENT We wish to thank Prof. W.J. Huk for providing skull roentgenograms, C T scans and magnetic resonance imaging.
REFERENCES
Downloaded by [New York University] at 09:23 31 March 2016
1. Kennedy F : Retrobulbar neuritis a s an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Am J
Med Sci 142:355-368, 1911 2. Bynke H : The syndrome of Foster Kennedy. Acta Ophthalmol 36: 129-140, 1958 3. Massey EW, Schoenberg B: Foster Kennedy syndrome. Arch Neurol41:658-659, 1984 4. Schultz-Zehden P : Ein Beitrag zur Kenntnis der Genese einseitiger Stauungspapille. Klin Mbl Augenheilk 43 : 153-156, 1905 5 . FranCois J , Neetens A : Le syndrome de Foster Kennedy et son etiologie. Ann Oculist 188:219-253, 1955 6. Tonnis W : Diagnostik der intrakraniellen Geschwulste. In: Olivecrona H, Tonnis W : Handbuch der Neurochirurgie, Vol 4. pp 1-490. Berlin: Springer 1962 7. Skrzypczak J : Zur Bedeutung des Foster Kennedy-Syndroms. Klin Mbl Augenheilk 150: 504-509, 1967 8. Huber A : Eye Signs and Symptoms in Brain Tumors, 3rd edn, pp 171-173. St. Louis: Mosby 1976 9. Kennedy F: A further note on the diagnostic value of retrobulbar neuritis in expanding lesions of the frontal lobes. JAMA 67: 1361-1363, 1916 10. Elkington S G : Pituitary adenoma. Preoperative symptomatology in a series of 260 patients. Br J Ophthalmol 52: 322328, 1968 1 1 . Frenkel REP, Spoor T C : Visual loss and intoxication. Surv Ophthalmol 30:391-396, 1986 12. Glees M : Dem Foster Kennedyschen Syndrom ahnliche Veranderungen der Sehnerven durch Arteriosklerose. Klin Mbl Augenheilk 100: 865-873, 1938 13. Jefferson G : The Doyne lecture: On compression and invasion of the optic nerves and chiasma by neighbouring gliomas. Trans Ophthalmol SOCUK 65 :262-304, 1945 14. Meves H : Zur Differentialdiagnose des Foster Kennedyschen Syndroms. Z Augenheilk 78 :242-258, 1932 15. Rovit RL, Duane T D : Eye signs in patients with Cushing’s syndrome and pituitary tumors. Arch Ophthalmol 79: 512522, 1968 16. Schmelzer H : Einseitige Stauungspapille und Storung des Geruchsinnes. Ein Beitrag zum sog. Foster-Kennedyschen Syndrom. Klin Mbl Augenheilk 91 :479-485, 1933 17. Frezzotti R : Sul valore localizzatore della sindrome di Foster Kennedy. Osservazione di un raro caso di sindrome ‘inversa’. Boll Oculist 34:498-509, 1955 18. Garcia-Miranda A : Umgekehrtes Foster-Kennedy-Syndrom. Ophthalmologica 112: 72-77, 1946 19. Jarus GD, Feldon SE: Clinical and computed tomographic findings in the Foster Kennedy syndrome. Am J Ophthalmol 93: 317-322, 1982 20. Lobeck E: Uber die Bedeutung des Augenbefundes fur die Diagnostik und Therapie von Hirntumoren. v Graefes Arch Ophthalmol 140: 599-628, 1939 21. Custodis E : Meningeom der Falx und lokalisatorisch irrefuhrendes Foster-Kennedy’sches Syndrom. Klin Mbl Augenheilk 101 :823-830, 1938 22. Watnick RL, Trobe J D : Bilateral optic nerve compression as a mechanism for the Foster Kennedy syndrome. Ophthalmology 96: 1793-1798, 1989 23, Rifat A : L’atrophie du nerf optique provoquke par la compression d’une tumeur du lobe frontal. Turk Oftalmol Gaz 1 :280-286. 1930 24. Utermann D : Beitrag zur Behandlung der Arachnoiditis opticochiasmatica. Klin Mbl Augenheilk 139 :640-652, 1961 25. Yaskin HE, Schlezinger NS : Foster Kennedy syndrome associated with non-neoplastic intracranial conditions. Arch Ophthalmol28:704-710, 1942 26. Tassman IS: Foster Kennedy syndrome with fusiform aneurysm of internal carotid arteries. Arch Ophthalmol32: 125127, 1944
82
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Foster Kennedy syndrome due to pituitary adenoma 27. Sanders MD, Sennhenn RH: Differential diagnosis of unilateral optic disc oedema. Trans Ophthalmol SOCUK 100: 123131, 1980 28. Wieser D : Zur Kenntnis des Pseudo-Foster-Kennedy-Syndroms. Ophthalmologica 145 :362-368, 1963 29. Duke-Elder S, Scott G I : Neuro-Ophthalmology. I n : Duke-Elder S : System of Ophthalmology, Vol XII, pp 294-295. London: Henry Kimpton 1971 30. Miller NR: Walsh & Hoyt’s Clinical Neuroophthalmology, 4th edn, Vol I , pp 187-189. Baltimore: Williams & Wilkins 1982 31. Chamlin M, Davidoff LM, Feiring E H : Ophthalmologic changes produced by pituitary tumors. Am J Ophthalmol 40:353-368. 1955 32. Shenkin HA, Crowley JN : Hydrocephalus complicating pituitary adenoma. J Neurol Neurosurg Psychiat 36: 1063-1068. 1973 33. Anderson D, Faber P , Marcovitz S, Hardy J. Lorenzetti D: Pituitary tumors and the ophthalmologist. Ophthalmology 90: 1265-1270, 1983 34. Bynke H : Pituitary adenomas with ocular manifestations. Incidence of cases and clinical findings. Neuro-Ophthalmology 6: 303-3 1 I , 1986 35. Herold S, von Kummer R, von der Groben Ch : Augensymptome bei Hypophysenadenomen, Kraniopharyngeomen und Meningeomen der vorderen und mittleren Schadelgrube. Klin Mbl Augenheilk 185 :495-504, 1984 36. Klauber A, Rasmussen P , Lindholm J : Pituitary adenoma and visual function. The prognostic value of clinical, ophthalmological and neuroradiological findings in 5 1 patients subjected to operation. Acta Ophthalmol 56: 252-263, 1978 37. Wilson P , Falconer MA: Patterns of visual failure with pituitary turnours. Clinical and radiological correlations. Br J Ophthalmol 52:94-110, 1968 38. Wray SH : Neuro-ophthalmologic manifestations of pituitary and parasellar lesions. Clin Neurosurg 24: 86-1 17, 1976
83
ISSN: 0165-8107 (Print) 1744-506X (Online) Journal homepage: http://www.tandfonline.com/loi/ioph20
Foster Kennedy syndrome caused by an extensive pituitary adenoma Michael Küchleand & Hendrikus Seyer To cite this article: Michael Küchleand & Hendrikus Seyer (1992) Foster Kennedy syndrome caused by an extensive pituitary adenoma, Neuro-Ophthalmology, 12:2, 77-83, DOI: 10.3109/01658109209058121 To link to this article: http://dx.doi.org/10.3109/01658109209058121
Published online: 08 Jul 2009.
Submit your article to this journal
Article views: 8
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ioph20 Download by: [New York University]
Date: 31 March 2016, At: 09:23
0 Aeolus Press Neuro-ophthalmology 0165-8 107/92/US$3.50
(Accepted 4 June 1991)
Foster Kennedy syndrome caused by an extensive pituitary adenoma MICHAEL KUCHLE’* and HENDRIKUS SEYER2
Departments of Ophthalmology and 2Neurosurgery, University Erlangen-Nurnberg, Erlangen, FRG Downloaded by [New York University] at 09:23 31 March 2016
~~
~~
~
ABSTRACT. A 5 I-year-old woman presented with signs of Foster Kennedy syndrome including right papilledema. left simple optic atrophy, and left anosmia. CT scan and magnetic resonance imaging disclosed a giant pituitary tumor growing superiorly and anteriorly to the left and compressing the chiasm and the left optic nerve. Endocrinological work-up and histology confirmed the diagnosis of a non-secreting pituitary adenoma. The possible mechanisms causing ipsilateral optic atrophy and contralateral papilledema in this case are discussed. The authors’ case seems unique since it represents, to their knowledge, the first report of a pituitary adenoma presenting with the full picture of Foster Kennedy syndrome. Pituitary adenoma should not be excluded from the differential diagnosis of lesions presenting as Foster Kennedy syndrome. Key words: Foster Kennedy syndrome; pituitary adenoma; papilledema ; optic atrophy
INTRODUCTION
processes. To our knowledge, the occurrence of FKS in a patient with pituitary adenoma has never been reported. In this paper we describe a case of nonfunctional giant pituitary adenoma which presented clinically as FKS.
Foster Kennedy syndrome (FKS) is a rare entity of clinical signs. It consists of ‘primary optic atrophy on the side of the lesion together with concomitant papilledema in the opposite eye’ (Kennedy 1911’) and usually occurs with frontobasal tumors, most frequently meningiomas of the CASE REPORT sphenoid wing and the olfactory g r o o ~ e ~ . ~ . A 51-year-old white female was referred to our in‘True’ FKS also includes anosmia or hyposmia, stitution after consulting her ophthalmologist bethus consisting of a triad which actually is only cause of deteriorating vision. For several years, she found in a few extraordinary cases. FKS has had experienced insidiously progressing visual loss been observed in a variety of intracranial lesions of her left eye but had not seen an ophthalmologist until now. During the last three months prior to including neoplasms and non-neoplastic changes admission, she had also noticed some visual deterisuch as infectious, inflammatory and vascular
*
Correspondence 10: Michael Kiichle, M.D.,University Eye Hospital, Schwabachanlage 6, W-8520 Erlangen,
BRD
Neum-ophthalmology - 1992, Vol. 12, No. 2, pp. 77-83 0 Aeolus Press Buren (The Netherlanh) 1992
oration of her right eye. Her past medical history was unremarkable except for a hysterectomy four years earlier. Apart from her visual loss, she did not complain of any symptoms. On ophthalmological examination, visual acuity
77
M . Kuchle and H. Seyer
Downloaded by [New York University] at 09:23 31 March 2016
~~
was 20/20 unaided in her right eye and light perception in her left eye. Intraocular pressure was 17 mm Hg in both eyes using applanation tonometry. The left eye showed exotropia and a marked afferent pupillary defect. Ocular motility was undisturbed in both eyes. On slitlamp examination, the anterior segment of both eyes showed no abnormalities. Fundus examination of the right eye disclosed a markedly swollen disc (prominence 5 dpt.) with dilated capillaries on the disc surface, obliteration of the physiologic central cupping, blurred disc margins obscuring the surface vessels, and some small splinter hemorrhages in the parapapillary nerve fiber layer (Fig. la). On the left side, the disc showed advanced simple optic atrophy without prominence or blurring of the disc margins (Fig. 1b). The retinal arterioles appeared narrowed and the retinal nerve fiber layer of the left eye was not discernible. Kinetic perimetry of the right eye showed an incomplete temporal hemianopia and an enlarged blind spot (Fig. 2). Perimetry of the left eye could not be performed (V/5 not discerned). As FKS was diagnosed, an intracranial lesion was suspected and the patient was referred to the
neurosurgical department for further diagnostic evaluation. On neurologic examination, the patient was alert and well oriented. She gave the impression of being slightly euphoric. There was a left anosmia and a subtle, mainly leftsided tetraspasticity. Sensitivity and coordination were normal. Paresis of the extremities did not exist. Roentgenograms of the skull revealed an enormously enlarged sella turcica (Fig. 3) and destruction of its posterior wall. CT scan disclosed a large mass in the left frontal lobe, which was evidently growing up from the sella turcica (Fig. 4). The tumor elevated the wall of the left lateral ventricle and led to a midline deviation of about 1 cm to the right. I t was contrast-enhancing. Surrounding brain edema was evident. Magnetic resonance imaging showed a large space-occupying lesion which arose from the sella turcica in front of the brainstem, lifted the left frontal lobe up and bulged the left lateral ventricle (Fig. 5 ) . The tumor compressed the optic chiasm and the left optic nerve between the chiasm and the optic canal. The left anterior cerebral artery was
Fig. 1. A. Right optic nerve head showing marked papilledema with prominent disk, blurred disk margins, engorged retinal veins and dilated capillaries. B. Right optic nerve head showing advanced simple optic atrophy. Note attenuated retinal arterioles.
78
Downloaded by [New York University] at 09:23 31 March 2016
Foster Kennedy syndrome due to pituitary adenoma
Fig. 2. Visual field of right eye showing incomplete temporal hemianopia and enlargement of the blind spot.
Fig. 3. Roentgenogram showing
tor
enormously enlarged sella turcica and destruction of its posterior wall.
79
Downloaded by [New York University] at 09:23 31 March 2016
M. Kuchle and H . Seyer
Fig. 4. CT scan showing a large pituitary tumor growing superiorly and anteriorly. Note elevation of the wall of the left lateral ventricle and midline deviation to the right.
displaced backwards. Hydrocephalus had not yet developed. Thus, a giant pituitary adenoma was suspected radiologically. Endocrinological investigation (stimulation of the anterior pituitary lobe with ACTH, TRH and LRH ; insulin-induced hypoglycemia; stimulation with CRH and GRH) showed mild hyperprolactinemia but surprisingly no hypocortisolism. Growth hormone was normal. Hypothalamic regulation seemed to be intact. Hence, a non-functional adenoma was probable. The tumor was nearly inoperable. However, the patient was under the threat of getting blind in both eyes and her life being shortened by the tumor growth. She was treated therefore with dexamethasone for one week and operated via the left subfrontal approach. As much tumor tissue as possible was removed and the chiasm decompressed. Histological examination of excised tumor tissue disclosed a non-functional pituitary adenoma. Unfortunately, the patient experienced malignant brain edema postoperatively and developed
80
Fig. Magnetic resonance showing a large pituitary tumor and compression of the chiasm and left optic nerve.
maximal intracranial pressure. She died on the fourth day after operation.
DISCUSSION FKS representing the combination of optic atrophy with contralateral papilledema is an infrequently encountered combination of clinical signs. FKS was first described in 1905 by SchultzZehden4 in a patient with a cholesteatoma of the anterior cranial fossa. Its diagnostic value, however, was established in 191 l by F. Kennedy'. FKS is a rare finding. Francois and Neetens5, in 1955, found a total of 169 cases in the literature. Bynke2 saw 17 cases of FKS among 1400
Downloaded by [New York University] at 09:23 31 March 2016
Foster Kennedy syndrome due to pituitary adenoma patients with expanding lesions of the brain. In Trobe22 recently reported a boy with FKS and a series of 3033 intracranial tumors, Tonnis6 juvenile nasopharyngeal angiofibroma without found 28 cases of FKS. Skrzypczak7 reported 19 increased intracranial pressure. The authors becases of FKS among 2000 patients with histologi- lieved that FKS was caused by bilateral, asymcally proven intracranial tumors. Huber8 saw metric optic nerve compression, and in a review of 36 previously reported cases of FKS they susfive cases of FKS among 8100 brain tumors. In 'true' FKS, there is anosmia or hyposmia in pected that in 12 of these cases FKS was also addition to papilledema and optic atrophy as was caused by bilateral optic nerve compression. In our case, the leftsided optic atrophy and originally described by Kennedy9, but olfactory dysfunction is only seen in a small portion of anosmia were obviously caused by direct tumor compression to the optic nerve and olfactory patients with FKS2* FKS is usually associated with frontobasal tu- tract (Fig. 5 ) . Contralateral papilledema may mors, most frequently sphenoidal ridge me- have been the result of increased intracranial n i n g i o m a ~ ~ . ~17-*I*, olfactory groove and also pressure caused by tumor volume. However, disuprasellar m e n i n g i o m a ~ ~ *19,~ , but other tu- rect compression of the right optic nerve near the chiasm may also have played'a role in the develmors such as g l i o b l a ~ t o r n a ~ .astrocyto~~~~, ma7,20, olig~dendroglioma~~, meningioma of the opment of papilledema similar to the mechafalx cerebri2', ch~lesteatoma~, craniopharyngio- nisms proposed by Watnick and Trobe22. Addima2.7, nasopharyngeal angiofibroma22, tionally, compression of the chiasm had led to myxo~hondroma~,ganglio~ytoma~, lymph- the rightsided superior temporal visual field dea n g i o ~ a r c o m aand ~ ~ metastases7 have been de- fect (Fig. 2). In contrast to tumors of the anterior cranial scribed in conjunction with FKS. About onethird of cases of FKS are caused by non-tumorous fossa, pituitary adenoma typically presents with lesions such as abscess of the frontal lobe1*2*13,bitemporal visual field loss and optic atrophy. Papilledema is characteristically absent in cases opticochiasmal ara~hnoiditis'~. 24.25, osteomyelitisI3, sclerotic carotid arteries5*l2,aneurysm of of pituitary adenoma3' and is only very infrethe internal carotid arteries9. 26, cerebral syphilis' quently seen in cases in which pituitary adenoma causes h y d r o ~ e p h a l u s ~On ~ . reviewing some of and Paget's disease5. FKS should be distinguished from the so- the literature on pituitary adenomas, we found called pseudo-FKS which is seen in patients with that only in two of 1197 reported cases of pituiunilateral ischemic optic neuropathy and contra- tary adenoma (1.7%) papilledema was obserlateral, usually postischemic, optic a t r o ~ h y ~ ~vedl0,15,31-38. ,~~. Our case seems to be unique since it is, to our The mechanisms by which FKS develops are discussed controversially. Its pathogenesis is usual- knowledge, the first report of a pituitary ly believed to consist of ipsilateral direct optic adenoma presenting with the complete triad of nerve compression causing optic atrophy, and FKS that is explainable by tumor extension simisubsequent increase of intracranial pressure lar to olfactory groove or other frontobasal meleading to contralateral papilledema1*29.30. Oth- ningiomas. Despite the enormous size of the er authors believed that bilateral papilledema pituitary adenoma, the only symptoms that our may lead to early optic atrophy on one side, thus patient had experienced were longstanding, resulting in a picture of FKS7g20. Watnick and slowly progressive visual loss that had finally
81
M. Kiichle and H. Seyer made her consult an ophthalmologist. Although presenting only very rarely with symptoms of FKS, pituitary adenoma should be included in the differential diagnosis of lesions causing FKS.
ACKNOWLEDGEMENT We wish to thank Prof. W.J. Huk for providing skull roentgenograms, C T scans and magnetic resonance imaging.
REFERENCES
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1. Kennedy F : Retrobulbar neuritis a s an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Am J
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