127
Convexity meningioma associated with empty Sella presenting as primary amenorrhea K. von Wild. H. P. Hobik ', A. Ku/ali
Summary A cas e of a 30-yea r-old female seen for a convexity meningioma is pres ented . History and investi-
gations revealed an empty sella turcica as sociat ed with primary amenorrhea . The rem oval of thc convex ity meningioma resulted in the disappearance of the amenorrhea aod in the rise of plasma gonadotropins. The importance of the consideration of intracranial causes in patients wh o have a delayed puh erty or the ab sen ce of a men strual history is briefly empha sized in the light ofliterature. Konvexitätsmeningiom bei leerer Sella und primärer Amenorrhoea Es wird über eine 30jährige Patientin mit Konvexitätsm eningiom berichtet. Die Anamn es e und
Unters uchung erg ab eine leere Sella turcica mit gleichzeitiger primärer Amenorrhoea. Nach Entfern ung des Konvexitätsmeningiom s verschwand die Amenorrhoea und der Plasm a-Gonadotropinspiegel stieg an. Anh and der bisherigen Literatur wird in gedrängter Form die Wichtigkeit der Berüc ksichtigung intrakrani eller Urs ache n bei Pati enten betont. bei den en die Pubertät versp ätet bzw . verzögert ist oder keine Menstruation eingetreten ist.
Key-Words
Th e term "prim a ry ernpty" is used (3, 7, 8, 15,1 9, 21,26) whe n ther e is no history of prior surge ry (10, 18, 25) or irra diation (10, 11, 17, 18, 22) of the sella r region . The primary empty sella is considered to be a benign condition (21) pr obably ca use d by multiple pr egnancies (6, 21), the block of th e CSF dr ain age due to a rac hnoid adhesions over the convexity (8), at rophies or infa rcts of the pituitary gland (21, 22), infarct of a pituita ry adenoma (9, 21), post partum necrosis of th e pitui tary gland (10, 20), primary cystic lesions ofthe pituitary gland (10 , 21), suba rac hno id adhesions in the sellar region (15,10), deficient diaphragma sella without increased intracr ani al pr essure (3, 17 , 19, 21, 22 ,27) or with increased intracrani al pressure seconda ry to
hypertension (17), or cor pulm onale a nd congestive heart failure (8, 17), or pseud otumor cereb ri (6, 8, 13, 17 , 29), hydro cephalus (1, 2, 5, 6, 7, 8) or due to meta sellartumors (at a dist an ce from the sella) (7, 12,2 7). A number ofdi verse ph enomena h ave been reported in pati ent s with em pty sellas in pr esence (1, 5, 6, 7, 19) or a bse nce (6, 16, 17 . 21, 24, 29) of endocrine abnormalities . Endocrine dysfunction is undoubtedly pr odu ced by continued pr essure on the anterior pituitary glan d (10). We report a 30-year -old female patient with a large conve xity meningioma in the right temp oro-parietooccipital regi on in association with an empty sella and primary amenorrhea in who m the tumor rem oval result ed in a rise in plasm a gona dotropins and in the disappearance of the amenorrhoea. To our knowledge this is the p t patient with conve xity meningioma, empt y se lla and ame no rrhea occurring s imultaneo us ly.
Convexity meningioma - Raised intracranial pr ess ur e - Empty sella - Primary am enorrhea Magn etic resonance tomography
Introduction The term "empty sella" wa s coine d by Busch in 1951 (9) a nd applied to th e app earan ce ofthe sella turcica wh en th e diaphragma sellae is incompl ete or forms only a small peripheral rim to allow the sub arachnoid space to extend into th e sella and the pituitary gland is not gro ssly visible as one views the se lla turcica from ab ove at necropsy
or at surgery.
Neurochirurgia 35 ( 992) 127-1 30 © GeorgThieme Verlag Stuttgart - New York
Case Report A Sü-year-old woma n was admitted to our department in February 1987 with complaints of having increasing headaches , occasional giddiness an d weakness of the left hand of two month 's duration. The hist ory revea led that the patient had primary amenorrhea and her first menses occurred by means of induction of ovulanon a few times which have stopped on cessation of hormone therapy w hen she was 20 years old. An electro encep ha logram showed focal slowing over the right tem poroparietal regten. Skull radiography showe d an enlarged ballooned se lla turcica with eros ion ofth e dorsum sella (Fig. 1). A CTscan revealed a large convexity mening ioma in the right tempcro -parieto -occipital region causing a shirt of the midline structures to the left and enlargemen t of the se lla turcica showing an internal lucen cy (Fig. 2 a, b, cl, On MRT·investigation the flatten ed pituitary gland covers the floor of an enlarged balloon sha ped se lla. The stalk and infundibulum are especially we il visualized in the lateral view . Most of the sella volume is filled with
Downloaded by: Georgetown University Medical Center. Copyrighted material.
Department of Neurosurgery 1 Institute of Pathology Clemenshospital Münster, Germany
Neur ochirurgi a 35 (1992)
K. [,Oll
wu« 11. P. lIohik. A. Kul ali a
b
Fig. 1 Lateralview of theskull showlüg theenlargedand balloonedsellawith erosion of the dorsum sella
structures of equal density to fluid (CSF).lIypoth a lamus and brain stern are normal (Fig. Sa . h]. Results of physical examinati on was normal except for hypert en sion, obesity and the ophtha lmologie findings of slight pa pillederna. Endocr inologic exa minatio ns showed LH to be 3,7 mUlml a nd FSIf to be 2,7 mUlml preopc ratively. The patient was oper ated on. The fibroblast ic meni ngioma was removed . The postoperati ve course was uneventfull except for a slight weak ness of the left arm which disappeared completely before discharge. Two weeks after the removal of the meningioma her first sponta neous norm al menses occurr ed and menscs have been continuing with further normal menstrua l bleedin gs so far. Endocr ine examinations postope rat ively showed a rise in plasma gonadotropins with LH value of 10.8 mUlml and FSII value of 6.2 mU/mI. She has had no more high blood pressur e values.
c
Discussion The primar y empty sella syndrome is cornmonly encountered in midd le-aged , obese , hypertensive women (16, 21). The syndro me may result from several mechanism s. but a unifying concept is ass ume d that mild. intermittent or chronic elevation of intracrani al pressure remodelling the anatomy of the sella through a congenilal incomplelen ess of the sellar diaphragm (8, 16, 21) is the cause ofthe syndrome . Intracranial metasellar tumors have been observed with th e prim ar y ernpty sella by others (7, 12, 27). The menin gioma is a ben ign enca psulated tumorwhich ma y grow for man y years before producing serious symptom s corresponding to the increase in the intracranial pressure and may conse que ntly reach a large size up to 10 cm across (in Dur case 7 crn) (28). Continued pressure by CSF on th e anl erior piluil ary gland should be responsible for piluitary hypofunclion in our palient with the emply sella syndrome. From review oft he literature as well as our own patient th e occas ional instances of pituitary endocrine dysfunction appear to be unrelated to sellar enlargement or to the exte nsion of th e subarac hnoid space into the sellar cavity.
Fig. 2 a Preoperative Cl seen showinga largeconvexitymeningioma inthe righttemporo-paneto-occipital regioncausingashift of themidline structuresto the left. b and c: En largement of theSella turcicashowing aninternallucency
Downloaded by: Georgetown University Medical Center. Copyrighted material.
128
Convexity mening ioma aesociated unth emp ty S ella presen ting os primary amenorrhea
N eu roch iru rg ia 35 (1992)
•
(arrowl
S
9
10
II
12
13 14
15 16
1; 18
19
Referen ces 20
1
2
3
4 5
Avmall. N., G. Erdogan, Y. Kanpolat: Pituita ry pseudotumor. Surg. Neural. 9 (197 8) 107 - 110 Bergland, R. M.. B. S. Ray, R. At. Tarack: Anat omical va rtatl on s in the pituitary gla nd and adjacent structures in 225 huma n autopsy cases. J . Neurosurg. 28 (1968) 9 3- 99 Bemasconi. V.. A. M. Giaoonelli. I. Papa: Primary empty sella . J . Neurosurg.36 (1972)157-161 Bjerre, P., J. Ltndotm : Adult hydr ocephalus and empty sella. Acta Neu ra l. Sca nd. 70 {l9841201 -203 Bous iq ue, I. Y.. Ph. Caron. Pli. Adam, AI. Tremo u let: Associat ion d'une sten ose de I'a queduc de sylvius d'une sella turcique vide et d' une am er orrhee primair e. Rev. Otoneuroophthalm ol. 56 (1984)
21
22
23 24
25
31-34 6
Brisman , H., J. E. O. lIughes , D. A. !lolub: Endocrine function in nineteen patients wit h em pty sella synd rorne. J . Clin. End ocrinol. Metab . 34 (1972) 5 70 -573
26
Brismur, K.. X. Bajraktari, R. Goulaua. S. Efendic. The em pty sella synd rome-tntrasella r ctsternal herni ati on in norma l pat ients and in pa tients with comrnunicaüng hyd rocephalu s a nd lntracra nla l tumor s. Neuroradiologv 17 (1978) 35-43 lt rismar. K.. G. Bergstrand: CSF circulation in subjects with the em pty sella synd rome . Neuroradiology 21 (1981) 167- 175 Bus ch, W.: Die Morpho logie der Sella turcica und ihre Beziehu ngen zur Hypop hyse. Arch. Path . Anat. 320 (t 951) 43 7- 458 Caplcn. R. H , G. D. Dobben : Endocrine studies in pati ents with the em pty sella syndrome . Arch . intern . Med. 123 (t 969 ) 611-619 Colby, M. Y.. T. P. Keams. Radiatio n therapy of pituitary ade nomas with ass ociated visua l impa irme nt. Proc. StafT. Meet. Mayo CH. 37
(1962) 15-24 du Boulay. G. H , T. EI Gammal: The elass ification, elinical value and mechanism of sella tur cica cha nges in raised int ra cra nial pr essur e (intracranial tumour). Brit. J . Rad io L. 39 (1966 ) 421-442 Foley . K. M.. J . B. Posn er: Pseudotumor cereb ri and em pty sella syndrome Iabstractl. Neurology 24 (19 74) 358 Foley. K. M.. 1. B. Posner: Does pseudotum or cerebrl ca use the em pty sella synd rome? Neurology 25 (1975 ) 565 -569 Friedman n. G.. F. Ma rguth: Intraselläre Liquorzysten. Zentra lbl. Neurochir. 21 (1961) 33 Jordan . R. M., J. W Kendall. C. W Kerber: The pr ima ry em pty sella syndrome . Am. J. Med. 62 (19 77) 569 -580 Kaufmann, B.: The empty sella turcica: A manifestation of the in trasellar subaraehnoid space . Rad iology 90 (1968l 9 31-941 Lee, W M.. 1. E. Adams: The empty sella syndrome . J. Neurosurg. 28119681 351- 356 Ma ria. G.. C. Anile. B. Cioni et al.: Relationship s between intracranial pressure and diu rnal pro lactin secreüon in primary empty sella . Neuroendocrinolog. 38 (t 984) 10 2-1 07 Meadar. C. K.. 1. Worrell.· The sella tu reica in post pa rtum pituita ry nec rosis (Sheehan 'ssyndromel . 65 (1966) 259- 264 Nee ton. F., J . Goree, 11. Lebovitz: The pr imary em pty sella : clinical and radi ographie cha ra cteristics and endoc rine function . Medicine 5211973173- 92 Obrado r. S.: Empty sella and some related syndromes. J. Neurosurg.36(1972) 162 -168 Orlondo . E G.: The em pty sella syndrome. Am. T. Roentgenol. 104 (1968 ) 168-170 van Petrykowski. W, H Reimeein. C. Ostertag, R. Fürmaier: Asym ptom ati c primary empty sella: com puter tomography a nd n uelear magnetic resonance imagi ng. Horm one Res. 22 (1985) 58 -62 Robeneon. E. G.: Pneumoen cephal ograph y. Springfeld 111: Cha rles C, Thomas Pu blish er (t 95 71 pp 230 - 232 Spagn a, V. A., M. C.Maeder. 1. C. Johnson. E. L. Mazz af er ri: Normal pr essu re hyd roce phalus . parkinson ism a nd primary em pty sella coinc idence or cause effect? Neurology 28 (19 781119 1- 1193
Downloaded by: Georgetown University Medical Center. Copyrighted material.
b
Fig. 3 MRI tomography through the seue a:Theflattened pituitarygland covers thefloorot theenlarged ballooned shaped sella(Iower arrowl. Mostof thesellavolumeisfilled withstructureof equal density to CSF(upper arrow]. b:ThestalkandinfundibulumareweHvisualized
Only one similar obse rva tion to Durs was report ed by Bousigue et al. in 1984 (5), in a case ofa 17 years old girl seen for primary a me norrhea in whom investigations re vealed hydroc ephalu s due to stenosis of the aqueduct of Sylvius and a n empty sella turcica. After ventriculo-peritoneal shunting the ame norrhea, disapp eared and the pu bertal developme nt occurred . According to these two ca ses including Dur own case an d literatu re (1 , 4 , 6) it sbould be suggested tha t, in the event of a reta rd ed puberty or in the a bse nce of a me ns trua l history, the patients should be invest igat ed for intra cr an ial causes an d all patients with abn orm ally enlarged sella sus pected of ha rb oring a tum or of the pituitary on the basis of plain ra diogra phy sh ould have MRI investigati on pr ior to the ra peutic inter venti on , not only to delineate possible sup rasellar extension of tumors, but to determine whethe r or not the sellar enlargemen t is dU B to an empty sella seco ndary to ra ised int racranial pre ssur e for multiple rea son s, since MRI helps to avoid radiation exposure , is mu ch bett er accepte d by the patient than intrathecal cist ern ography and delineates the sellar contents very well, even compa red to a n optima l CT ciste rnography (24).
129
Neuro chir urgia 35 (1992) 27
28 29
Tönnie. W. W. Schief er. F. Rausch: Sellaverä nderu ngen bei geste igerte m Schädelinn endruck. Deutsche Ztschr. f. Nervenh. 171 (1954) 351-3 69 Tretp. C. S.: A colour Atlas ofNe uropathology. Wolfe Medical Publlcations ltd. Lond on, 1978 Weis berg. L. A.. E. M. Housepian, D. P. S our : Empty sella syndrome as complication of beni gn lntracranl al hypert ension. J . Neurosurg. 43(1975)177-180
K. VOn Wild. H. P. Hobik; A. Kulali
Prof Dr. Klaus von Wild Chefarzt der Neurochiru rgischen Abteilung Clemenshospital Duesber gweg 124 0- 4400 Münst er. Germa ny
Downloaded by: Georgetown University Medical Center. Copyrighted material.
130
Convexity meningioma associated with empty Sella presenting as primary amenorrhea K. von Wild. H. P. Hobik ', A. Ku/ali
Summary A cas e of a 30-yea r-old female seen for a convexity meningioma is pres ented . History and investi-
gations revealed an empty sella turcica as sociat ed with primary amenorrhea . The rem oval of thc convex ity meningioma resulted in the disappearance of the amenorrhea aod in the rise of plasma gonadotropins. The importance of the consideration of intracranial causes in patients wh o have a delayed puh erty or the ab sen ce of a men strual history is briefly empha sized in the light ofliterature. Konvexitätsmeningiom bei leerer Sella und primärer Amenorrhoea Es wird über eine 30jährige Patientin mit Konvexitätsm eningiom berichtet. Die Anamn es e und
Unters uchung erg ab eine leere Sella turcica mit gleichzeitiger primärer Amenorrhoea. Nach Entfern ung des Konvexitätsmeningiom s verschwand die Amenorrhoea und der Plasm a-Gonadotropinspiegel stieg an. Anh and der bisherigen Literatur wird in gedrängter Form die Wichtigkeit der Berüc ksichtigung intrakrani eller Urs ache n bei Pati enten betont. bei den en die Pubertät versp ätet bzw . verzögert ist oder keine Menstruation eingetreten ist.
Key-Words
Th e term "prim a ry ernpty" is used (3, 7, 8, 15,1 9, 21,26) whe n ther e is no history of prior surge ry (10, 18, 25) or irra diation (10, 11, 17, 18, 22) of the sella r region . The primary empty sella is considered to be a benign condition (21) pr obably ca use d by multiple pr egnancies (6, 21), the block of th e CSF dr ain age due to a rac hnoid adhesions over the convexity (8), at rophies or infa rcts of the pituitary gland (21, 22), infarct of a pituita ry adenoma (9, 21), post partum necrosis of th e pitui tary gland (10, 20), primary cystic lesions ofthe pituitary gland (10 , 21), suba rac hno id adhesions in the sellar region (15,10), deficient diaphragma sella without increased intracr ani al pr essure (3, 17 , 19, 21, 22 ,27) or with increased intracrani al pressure seconda ry to
hypertension (17), or cor pulm onale a nd congestive heart failure (8, 17), or pseud otumor cereb ri (6, 8, 13, 17 , 29), hydro cephalus (1, 2, 5, 6, 7, 8) or due to meta sellartumors (at a dist an ce from the sella) (7, 12,2 7). A number ofdi verse ph enomena h ave been reported in pati ent s with em pty sellas in pr esence (1, 5, 6, 7, 19) or a bse nce (6, 16, 17 . 21, 24, 29) of endocrine abnormalities . Endocrine dysfunction is undoubtedly pr odu ced by continued pr essure on the anterior pituitary glan d (10). We report a 30-year -old female patient with a large conve xity meningioma in the right temp oro-parietooccipital regi on in association with an empty sella and primary amenorrhea in who m the tumor rem oval result ed in a rise in plasm a gona dotropins and in the disappearance of the amenorrhoea. To our knowledge this is the p t patient with conve xity meningioma, empt y se lla and ame no rrhea occurring s imultaneo us ly.
Convexity meningioma - Raised intracranial pr ess ur e - Empty sella - Primary am enorrhea Magn etic resonance tomography
Introduction The term "empty sella" wa s coine d by Busch in 1951 (9) a nd applied to th e app earan ce ofthe sella turcica wh en th e diaphragma sellae is incompl ete or forms only a small peripheral rim to allow the sub arachnoid space to extend into th e sella and the pituitary gland is not gro ssly visible as one views the se lla turcica from ab ove at necropsy
or at surgery.
Neurochirurgia 35 ( 992) 127-1 30 © GeorgThieme Verlag Stuttgart - New York
Case Report A Sü-year-old woma n was admitted to our department in February 1987 with complaints of having increasing headaches , occasional giddiness an d weakness of the left hand of two month 's duration. The hist ory revea led that the patient had primary amenorrhea and her first menses occurred by means of induction of ovulanon a few times which have stopped on cessation of hormone therapy w hen she was 20 years old. An electro encep ha logram showed focal slowing over the right tem poroparietal regten. Skull radiography showe d an enlarged ballooned se lla turcica with eros ion ofth e dorsum sella (Fig. 1). A CTscan revealed a large convexity mening ioma in the right tempcro -parieto -occipital region causing a shirt of the midline structures to the left and enlargemen t of the se lla turcica showing an internal lucen cy (Fig. 2 a, b, cl, On MRT·investigation the flatten ed pituitary gland covers the floor of an enlarged balloon sha ped se lla. The stalk and infundibulum are especially we il visualized in the lateral view . Most of the sella volume is filled with
Downloaded by: Georgetown University Medical Center. Copyrighted material.
Department of Neurosurgery 1 Institute of Pathology Clemenshospital Münster, Germany
Neur ochirurgi a 35 (1992)
K. [,Oll
wu« 11. P. lIohik. A. Kul ali a
b
Fig. 1 Lateralview of theskull showlüg theenlargedand balloonedsellawith erosion of the dorsum sella
structures of equal density to fluid (CSF).lIypoth a lamus and brain stern are normal (Fig. Sa . h]. Results of physical examinati on was normal except for hypert en sion, obesity and the ophtha lmologie findings of slight pa pillederna. Endocr inologic exa minatio ns showed LH to be 3,7 mUlml a nd FSIf to be 2,7 mUlml preopc ratively. The patient was oper ated on. The fibroblast ic meni ngioma was removed . The postoperati ve course was uneventfull except for a slight weak ness of the left arm which disappeared completely before discharge. Two weeks after the removal of the meningioma her first sponta neous norm al menses occurr ed and menscs have been continuing with further normal menstrua l bleedin gs so far. Endocr ine examinations postope rat ively showed a rise in plasma gonadotropins with LH value of 10.8 mUlml and FSII value of 6.2 mU/mI. She has had no more high blood pressur e values.
c
Discussion The primar y empty sella syndrome is cornmonly encountered in midd le-aged , obese , hypertensive women (16, 21). The syndro me may result from several mechanism s. but a unifying concept is ass ume d that mild. intermittent or chronic elevation of intracrani al pressure remodelling the anatomy of the sella through a congenilal incomplelen ess of the sellar diaphragm (8, 16, 21) is the cause ofthe syndrome . Intracranial metasellar tumors have been observed with th e prim ar y ernpty sella by others (7, 12, 27). The menin gioma is a ben ign enca psulated tumorwhich ma y grow for man y years before producing serious symptom s corresponding to the increase in the intracranial pressure and may conse que ntly reach a large size up to 10 cm across (in Dur case 7 crn) (28). Continued pressure by CSF on th e anl erior piluil ary gland should be responsible for piluitary hypofunclion in our palient with the emply sella syndrome. From review oft he literature as well as our own patient th e occas ional instances of pituitary endocrine dysfunction appear to be unrelated to sellar enlargement or to the exte nsion of th e subarac hnoid space into the sellar cavity.
Fig. 2 a Preoperative Cl seen showinga largeconvexitymeningioma inthe righttemporo-paneto-occipital regioncausingashift of themidline structuresto the left. b and c: En largement of theSella turcicashowing aninternallucency
Downloaded by: Georgetown University Medical Center. Copyrighted material.
128
Convexity mening ioma aesociated unth emp ty S ella presen ting os primary amenorrhea
N eu roch iru rg ia 35 (1992)
•
(arrowl
S
9
10
II
12
13 14
15 16
1; 18
19
Referen ces 20
1
2
3
4 5
Avmall. N., G. Erdogan, Y. Kanpolat: Pituita ry pseudotumor. Surg. Neural. 9 (197 8) 107 - 110 Bergland, R. M.. B. S. Ray, R. At. Tarack: Anat omical va rtatl on s in the pituitary gla nd and adjacent structures in 225 huma n autopsy cases. J . Neurosurg. 28 (1968) 9 3- 99 Bemasconi. V.. A. M. Giaoonelli. I. Papa: Primary empty sella . J . Neurosurg.36 (1972)157-161 Bjerre, P., J. Ltndotm : Adult hydr ocephalus and empty sella. Acta Neu ra l. Sca nd. 70 {l9841201 -203 Bous iq ue, I. Y.. Ph. Caron. Pli. Adam, AI. Tremo u let: Associat ion d'une sten ose de I'a queduc de sylvius d'une sella turcique vide et d' une am er orrhee primair e. Rev. Otoneuroophthalm ol. 56 (1984)
21
22
23 24
25
31-34 6
Brisman , H., J. E. O. lIughes , D. A. !lolub: Endocrine function in nineteen patients wit h em pty sella synd rorne. J . Clin. End ocrinol. Metab . 34 (1972) 5 70 -573
26
Brismur, K.. X. Bajraktari, R. Goulaua. S. Efendic. The em pty sella synd rome-tntrasella r ctsternal herni ati on in norma l pat ients and in pa tients with comrnunicaüng hyd rocephalu s a nd lntracra nla l tumor s. Neuroradiologv 17 (1978) 35-43 lt rismar. K.. G. Bergstrand: CSF circulation in subjects with the em pty sella synd rome . Neuroradiology 21 (1981) 167- 175 Bus ch, W.: Die Morpho logie der Sella turcica und ihre Beziehu ngen zur Hypop hyse. Arch. Path . Anat. 320 (t 951) 43 7- 458 Caplcn. R. H , G. D. Dobben : Endocrine studies in pati ents with the em pty sella syndrome . Arch . intern . Med. 123 (t 969 ) 611-619 Colby, M. Y.. T. P. Keams. Radiatio n therapy of pituitary ade nomas with ass ociated visua l impa irme nt. Proc. StafT. Meet. Mayo CH. 37
(1962) 15-24 du Boulay. G. H , T. EI Gammal: The elass ification, elinical value and mechanism of sella tur cica cha nges in raised int ra cra nial pr essur e (intracranial tumour). Brit. J . Rad io L. 39 (1966 ) 421-442 Foley . K. M.. J . B. Posn er: Pseudotumor cereb ri and em pty sella syndrome Iabstractl. Neurology 24 (19 74) 358 Foley. K. M.. 1. B. Posner: Does pseudotum or cerebrl ca use the em pty sella synd rome? Neurology 25 (1975 ) 565 -569 Friedman n. G.. F. Ma rguth: Intraselläre Liquorzysten. Zentra lbl. Neurochir. 21 (1961) 33 Jordan . R. M., J. W Kendall. C. W Kerber: The pr ima ry em pty sella syndrome . Am. J. Med. 62 (19 77) 569 -580 Kaufmann, B.: The empty sella turcica: A manifestation of the in trasellar subaraehnoid space . Rad iology 90 (1968l 9 31-941 Lee, W M.. 1. E. Adams: The empty sella syndrome . J. Neurosurg. 28119681 351- 356 Ma ria. G.. C. Anile. B. Cioni et al.: Relationship s between intracranial pressure and diu rnal pro lactin secreüon in primary empty sella . Neuroendocrinolog. 38 (t 984) 10 2-1 07 Meadar. C. K.. 1. Worrell.· The sella tu reica in post pa rtum pituita ry nec rosis (Sheehan 'ssyndromel . 65 (1966) 259- 264 Nee ton. F., J . Goree, 11. Lebovitz: The pr imary em pty sella : clinical and radi ographie cha ra cteristics and endoc rine function . Medicine 5211973173- 92 Obrado r. S.: Empty sella and some related syndromes. J. Neurosurg.36(1972) 162 -168 Orlondo . E G.: The em pty sella syndrome. Am. T. Roentgenol. 104 (1968 ) 168-170 van Petrykowski. W, H Reimeein. C. Ostertag, R. Fürmaier: Asym ptom ati c primary empty sella: com puter tomography a nd n uelear magnetic resonance imagi ng. Horm one Res. 22 (1985) 58 -62 Robeneon. E. G.: Pneumoen cephal ograph y. Springfeld 111: Cha rles C, Thomas Pu blish er (t 95 71 pp 230 - 232 Spagn a, V. A., M. C.Maeder. 1. C. Johnson. E. L. Mazz af er ri: Normal pr essu re hyd roce phalus . parkinson ism a nd primary em pty sella coinc idence or cause effect? Neurology 28 (19 781119 1- 1193
Downloaded by: Georgetown University Medical Center. Copyrighted material.
b
Fig. 3 MRI tomography through the seue a:Theflattened pituitarygland covers thefloorot theenlarged ballooned shaped sella(Iower arrowl. Mostof thesellavolumeisfilled withstructureof equal density to CSF(upper arrow]. b:ThestalkandinfundibulumareweHvisualized
Only one similar obse rva tion to Durs was report ed by Bousigue et al. in 1984 (5), in a case ofa 17 years old girl seen for primary a me norrhea in whom investigations re vealed hydroc ephalu s due to stenosis of the aqueduct of Sylvius and a n empty sella turcica. After ventriculo-peritoneal shunting the ame norrhea, disapp eared and the pu bertal developme nt occurred . According to these two ca ses including Dur own case an d literatu re (1 , 4 , 6) it sbould be suggested tha t, in the event of a reta rd ed puberty or in the a bse nce of a me ns trua l history, the patients should be invest igat ed for intra cr an ial causes an d all patients with abn orm ally enlarged sella sus pected of ha rb oring a tum or of the pituitary on the basis of plain ra diogra phy sh ould have MRI investigati on pr ior to the ra peutic inter venti on , not only to delineate possible sup rasellar extension of tumors, but to determine whethe r or not the sellar enlargemen t is dU B to an empty sella seco ndary to ra ised int racranial pre ssur e for multiple rea son s, since MRI helps to avoid radiation exposure , is mu ch bett er accepte d by the patient than intrathecal cist ern ography and delineates the sellar contents very well, even compa red to a n optima l CT ciste rnography (24).
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Neuro chir urgia 35 (1992) 27
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Tönnie. W. W. Schief er. F. Rausch: Sellaverä nderu ngen bei geste igerte m Schädelinn endruck. Deutsche Ztschr. f. Nervenh. 171 (1954) 351-3 69 Tretp. C. S.: A colour Atlas ofNe uropathology. Wolfe Medical Publlcations ltd. Lond on, 1978 Weis berg. L. A.. E. M. Housepian, D. P. S our : Empty sella syndrome as complication of beni gn lntracranl al hypert ension. J . Neurosurg. 43(1975)177-180
K. VOn Wild. H. P. Hobik; A. Kulali
Prof Dr. Klaus von Wild Chefarzt der Neurochiru rgischen Abteilung Clemenshospital Duesber gweg 124 0- 4400 Münst er. Germa ny
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