Clinical manifestations of aqueductal stenosis in adults JOHN R. LITTLE, M.D., O. WAYNE HOUSES, M.D., AND COLLINS. MACCARTY, M.D. Mayo Clinic and Mayo Foundation, Rochester, Minnesota The authors review 17 cases of aqueductal stenosis in adults and describe five modes of clinical presentation. The average duration of symptoms was 6 years. Dementia was infrequent. Skull x-ray films frequently demonstrated some degree of cranial enlargement and signs of chronic increased intracranial pressure. Characteristic alterations of the sella included elongation of the anterior wall and flattening, erosion, and anterior displacement of the dorsum. Aqueductal configurations as defined by ventriculography and pneumoencephalography in all cases, and the angiographic findings in seven cases are described. Most of the patients improved substantially after shunt insertion. The clinical findings suggested that aqueductal narrowing and associated hydrocephalus had been present in most of these patients since early life. KEy WoaDs 9 aqueductal stenosis 9 t e m p o r a l lobe seizures spontaneous C S F rhinorrhea 9 hydrocephalus
QUEDUCTAL stenosis in the adult is not rare',' 9,1,,ls-ls,2o-,,,,27 but, in contrast to the infantile and childhood forms, little information is available regarding its clinical presentation and diagnosis. Here we report a review of the recent experience at the Mayo Clinic with this condition in adults.
A
A n a l y s i s of Cases
Clinical Presentation Seventeen adult patients seen at the Mayo Clinic between 1956 and 1974 were found to have nontumoral obstruction of the aqueduct of Sylvius. The 10 men and seven women ranged in age from 19 to 52 years (mean, 28 years). The symptoms and neurological find546
9
ings at the initial evaluation are listed in Table 1. The clinical presentations of these 17 patients studied can be divided into the following five groups: 1) headache indicative of increased intracranial pressure; 2) seizure disorder, usually temporal lobe seizures; 3) cerebrospinal fluid (CSF) rhinorrhea and headache; 4) endocrine dysfunction; and 5) gait disturbance and headache. There was some degree of overlap among the various groups. Headache was a frequent symptom but it was the main complaint in only four patients. Many of the patients described their headaches as being relatively mild and of short duration; however, a recent increase in frequency and severity was occasionally J. Neurosurg. / Volume 43 / November, 1975
Aqueductal stenosis in adults TABLE l
Clinical presentation, treatment, and follow-up of 17 patients with aqueductal stenosis Case No.
Age, Sex
Symptoms
Neurological Findings
Shunt Ventricle
Follow-up
1
24 F
headache (4 yr); endogenous depression (6 yr)
normal
cistern
occasional headache (14 yr)
2
23 F
chronic papilledema
cistern
3
19 M
headache (10 yr); blurred vision (5 wk) headache (1 yr); blurred vision (5 mo); unsteady gait (5 mo); paresthesias, face (4 mo)
complete recovery (2 yr) vision improved; doing well (13 yr)
4
26 M 28 M
acute papilledema; gait ataxia; It Babinski chronic papilledema
cistern
5 6
23 M
broad-based gait; hyperreflexia, legs; decreased mentation
cistern
seizures unchanged; slight improvement otherwise (7 yr)
7
31 M
normal
atrium
fewer seizures (3 yr)
8
28 M
gait ataxia; mild ataxia, arms
peritoneum no seizures (2 yr)
9
22 M
normal
peritoneum fistula repaired (6 mo)
10
39 M
normal
cistern
fistula repaired (11 yr)
11
21 F 20 M
chronic papilledema; gross obesity bulging craniectomy site
cistern
12
developed seizure disorder (I0 yr) complete recovery (10 yr)
13
19 F
headache (1 wk); blurred vision (3 days) seizure disorder (23 yr); headache (4 yr) seizure disorder (6 yr); headache (2 wk); unsteady gait (6 mo); personality changes & forgetfulness (6 rap); polyuria & polydipsia (6 mo) seizure disorder (2 yr); headache (18 yr) seizure disorder (3 wk); headache (3 wk); mild loss of memory CSF rhinorrhea (1 yr); headache (10 yr) CSF rhinorrhea (6 mo); headache (2 mo) CSF rhinorrhea (1 yr); headache (9 yr) CSF rhinorrhea (5 mo); headache (2 wk); nausea & vomiting (2 wk); moderate loss of memory (1 mo) primary amenorrhea
14
36 F
15
52 M
16
30 F
17
46 F
optic disc pallor; chronic cistern papilledema; contracted fields (large central scotoma, rt eye; ring scotoma, It eye)
cistern
pleura
occasional headache (18 yr) fewer seizures (12 yr)
small bitemporal field cistern amenorrhea persists; defects; underdeveloped normal visual fields secondary sex (6 yr) characteristics normal peritoneum amenorrhea persists (4 yr) broad-based gait; It cistern considerable improvement Babinski ; decreased (13 yr) upward gaze; stiff neck
secondary amenorrhea (2 yr); headache (2 yr) unsteady gait (2 89yr); leg weakness (2 89yr); headache (5 yr); mild loss of memory weakness, It leg (1 mo); spastic It leg; It Babinski; cistern paresthesias, It leg moderate hyperreflexia, (1 yr); headache (10 yr) arms & legs stiffness, legs (6 yr); gait ataxia; spastic legs; cistern paresthesias, legs bilateral Babinski (6 yr); headache (10 yr); urinary frequency & urgency (4 yr)
J. Neurosurg. / Volume 43 / November, 1975
complete recovery (4 yr) considerable improvement (1 89yr)
547
J. R. Little, O. W. H o u s e r a n d C. S. M a c C a r t y noted. Transient obscurations of vision occurred in three of the four patients who complained primarily of headache. Four patients presented with a seizure disorder. One patient (Case 5) who had had petit mal seizures during childhood developed temporal lobe seizures consisting of an olfactory aura, fugue state, and dbjh vu phenomena. A single generalized convulsion occurred 2 weeks before admission. Another patient (Case 7) had a 2-year history of recurrent temporal lobe seizures; he also had experienced three generalized convulsions during the I Ih years prior to admission. One patient (Case 6) had recurrent temporal lobe seizures only. The fourth patient (Case 8) had had two generalized convulsions, each followed by severe headache. Spontaneous CSF rhinorrhea and beadache, the presenting complaints of three patients, had been present for 2 months or longer with no history to explain them. A fourth patient (Case 12) developed CSF rhinorrhea about 1 month after he had suffered a depressed compound frontotemporal fracture. During the convalescent period he experienced a febrile episode that was thought to be a bacterial meningitis; however, the causative organism was never identified and the patient recovered. An attempt to repair the CSF leak was unsuccessful although a fracture line was identified. Six months after the accident, symptoms and signs of increased intracranial pressure developed. The clinical presentation of some patients suggested an endocrine disturbance. Two of the female patients had amenorrhea. One of them (Case 13) had never menstruated and had underdeveloped secondary sex characteristics. The presence of bitemporal field defects suggested a lesion in or adjacent to the chiasm. The other patient (Case 14) had not menstruated for 2 years, but prior to that time she had menstruated normally. Polydipsia and polyuria were complaints of another patient (Case 6). Marked obesity was noted only once (Case 11). The average duration of symptoms prior to diagnosis was 6 years. A history of recurrent headaches during childhood and adolescence was frequently obtained. Only three patients presented with a history suggestive of mental deterioration; most of the patients had been 548
able to perform adequately at their work prior to admission, Diagnostic Procedures
The ventricular fluid was examined in 16 patients and the spinal fluid in 12. The highest protein concentration found in the ventricular fluid was 18 mg/dl. In three instances the spinal fluid had slightly increased protein concentrations (46, 50, and 67 mg/dl). Of the 13 electroencephalograms obtained, a "projected type" dysrhythmia was identiffed in eight, four were considered to be normal, and one, from a patient with temporal lobe seizures (Case 7), showed a right temporal spike focus. Skull x-ray films of 14 patients were available for evaluation (Table 2). Measurements suggested the presence of cranial enlargement in 11 cases. Evidence of chronic increased intracranial pressure, as demonstrated by digital markings or split sutures, was present in 10 and alterations of the dorsum sellae were identified in 13. The anterior wall of the sella was longer and steeper than normal in six patients (Fig. 1). The anterior clinoid processes occasionally appeared enlarged. The skull films of the remaining three patients were reported as showing changes indicative of increased intracranial pressure. Right retrograde brachial angiography was performed in seven cases. Angiographic features indicative of marked, symmetrical enlargement of the lateral ventricles were present, and there was no evidence of a shift. The posterior cerebral arteries were consistently stretched and depressed. Flattening of the course of the superior cerebellar arteries was also a consistent finding on the lateral angiogram. Posterior displacement of the terminal basilar artery was present in one case, and a downward and lateral displacement was observed in another. Some degree of depression and flattening of the internal cerebral veins was noted in all cases. The anterior end of the basal vein of Rosenthal occasionally was depressed. The vessels in the lower posterior fossa were unaltered. Air ventriculography was performed in 16 patients. Marked dilatation of the lateral and third ventricles was present in all cases and the mean thickness of the cortical mantle was 29 mm (range, 22 to 36 mm). On concomitant J. Neurosurg. / Volume 43 / November, 1975
'O
t.a
sizet
.
.
-
+
-
(oblique) -
.
.
.
. . . .
(oblique) .
+ (post. floor) +
.
.
+ -
+G + .
E
6
.
.
.
.
.
(oblique) .
+
.
+ + +
.
8
7
.
.
.
E
8
-
. +
+ + -
+G +
* + = p r e s e n t ; - = a b s e n t . S k u l l f i l m s w e r e n o t a v a i l a b l e f o r C a s e s 2, 3, a n d 4. t E = enlarged; B = borderline; N = normal. :~ G = g e n e r a l i z e d ; F = f r o n t a l .
increased density
elongated
ant. wall
-t . . .
ant. clinoids large eroded deformed
+ + +
+ (mid. floor) +
+ + -
sella eroded
.
-FG -
B
E
+G + .
5
1
dorsum sellae short flat eroded displaced ant.
digital markings~ split sutures basilar invagination
cranium
Feature
2
.
.
.
. +
-
+
.
.
(vertical)
.
+ + + +
-FF +
E
9
.
.
.
-
-
+ . +
.
N
10
Case No.
Summary of findings on skull films*
TABLE
.
.
.
.
.
+
.
+
-
-
+ +
+G
N
11
.
.
.
.
-
.
.
-
-
-
-
B
.
12
.
.
+
.
+
+ + . +
+
+G
B
13
.
-
+
+ +
.
.
(vertical) .
+ (ant. floor) +
.
.
+G
E
14
.
.
.
-
-
-
+ + -
-
.
.
E
15
.
+
+
+
+ _ + _
(vertical) .
.
+
E
16
-
_
+
_ _ + _
+F +
N
17
~"
~:L
~..i.
O
J. R. Little, O. W. Houser and C. S. MacCarty None of the patients have developed symptoms or neurological findings to suggest the presence of a brain-stem glioma. Discussion
Ftc. 1. Case 14. Skull film, lateral view, demonstrating generalized digital markings indicative of chronic increased intracranial pressure. Anterior wall of sella is elongated and vertical (arrow), and floor is eroded. Dorsum sellae appears flattened and displaced anteriorly. pneumoencephalography in 11 patients, the fourth ventricle filled with air in 10, but it infrequently entered the lower end of the aqueduct. In only one patient was a pneumoencephalogram performed, which demonstrated uniform narrowing of the entire aqueduct. The configurations of the stenosed aqueducts are illustrated in Fig. 2. We were unable to define accurately the configuration of the obstruction in four cases. In one case computerized axial tomography (EMI scan) demonstrated marked dilatation of the lateral and third ventricles. Serological tests for syphilis were negative in the 16 patients tested. Treatment Shunting procedures were performed in all cases (Table 1). Twelve patients underwent an exploration of the posterior fossa and insertion of a ventriculocisternal shunt (Torkildsen procedure)) 5 There was no evidence of tumor m any of these patients. Two of the patients who presented with CSF rhinorrhea subsequently required direct repair of the fistula. Follow-up The results of follow-up are listed in Table 1. The mean duration of follow-up has been 10 years, and there have been no deaths. 550
Seizures are a frequent occurrence in adults with aqueductal stenosis. 6,11,18 Four patients in our series presented with a seizure disorder, and in three of them it had been a chronic problem. There also appeared to be a definite predilection for the development of temporal lobe seizures. Similarly, four of the 10 adult patients with aqueductal stenosis described by Nag and FalconeP 1 had a seizure disorder and in three of these patients it was described as the "psychomotor type." Spontaneous CSF rhinorrhea is an uncommon phenomenon and has seldom been described in association with aqueductal stenosis? 9 The development of this complication appears to be the result of erosion of the floor of the anterior fossa secondary to longstanding increased intracranial pressure. The fistulous tract may develop either spontaneously or after a trivial traumatic event. Compression of the pituitary, hypothalamus, and the optic chiasm by the greatly distended third ventricle appears to be responsible for the endocrine dysfunction and some of the visual problems observed in these patients. Also amenorrhea, impotence, gynecomastia, sexual precocity, delayed development of secondary sex characteristics, and obesity have been reported, e,~'~8'~8'2~ Treatment of the hydrocephalus occasionally results in relief of these symptoms. Nag and FalconeP 1 described a case of aqueductal stenosis in an 18-year-old woman with primary amenorrhea who began to menstruate 1 year after insertion of a ventriculocisternal shunt. One patient in our series had mild diabetes insipidus that resolved postoperatively. Another patient with small bitemporal field defects had normal visual fields 1 week postoperatively. In two of our patients, the amenorrhea failed to resolve with treatment. The triad of headache, dementia, and gait disturbance is characteristic of the clinical syndrome of chronic hydrocephalus in the adult) Headache and gait disturbance were frequently observed in our group but mental deterioration was seldom noted. This relative preservation of mental facilities may be J. Neurosurg. / Volume 43 / November, 1975
A q u e d u c t a l stenosis in a d u l t s related in part to the gradual progression of the disorder; however, we assume that decompensation would eventually occur in most of these patients if untreated. The findings on the plain skull films in most patients attested to the chronicity of this disorder. The sella frequently was affected in a characteristic way? The only patient without demonstrable sellar changes was thought to have developed aqueductal stenosis on the basis of an ependymitis only a few months prior to admission; however, a large cranial defect resulting from debridement of a compound skull fracture may have delayed the development of these changes. We agree with Du Boulay and Trickey 5 that the elongated and vertical anterior sella wall is indicative of a process that has probably been present since early life. A small posterior fossa, commonly seen in the infantile form of aqueductal stenosis, is rarely present in adults, s~ The skull films of only one of our patients demonstrated a low-placed internal occipital protuberance suggesting a small posterior fossa. The results of ventriculography, pneumoencephalography, and angiography corresponded closely to those reported by other investigators .9.~s,17,20,~7 Adult aqueductal stenosis is treated by insertion of a shunt. Ventriculocisternal shunts have consistently given good results and can be combined with exploration of the posterior fossa. Other types of surgical treatment of this condition have been reviewed by Paine and McKissock? 8 The pathogenesis of aqueductal stenosis in the adult is obscure in most cases. Inflammation, usually secondary to infection, has occasionally been implicated 8'~2-~4'~~ and appeared to be important in one of our patients (Case 12). Familial cases also have been described? ,~8 It is present sometimes in neurofibromatosis n,ls or in association with an Arnold-Chiari malformation? ~ In our series, an Arnold-Chiari (Chiari type II) malformation was found in one patient (Case 11) and tonsillar herniation alone (Chiari type I) was found in four patients (Cases 2, 4, 5 and 8). The clinical findings in our patients suggest that the aqueductal narrowing and hydrocephalus had been present in most of the patients since early life. Factors that substantiate this observation include the following: 1) long clinical histories, often J. Neurosurg. / Volume 43 / November, 1975
Configurations
No. of cases
Fie. 2. Varieties of obstructed aqueducts as demonstrated by air ventriculography and pneumoencephalography. "No aqueduct" means no filling of the upper end of the aqueduct with air; complete obstruction of the aqueduct is incompatible with life.
covering many years; 2) frequent occurrence of deterioration in early adulthood; 3) relatively normal day-to-day performance of most of the patients despite significant ventricular dilatation; and 4) characteristic changes in the sella, especially elongation of the anterior wall, and evidence of chronic increased intracranial pressure on skull films. In many of these cases, the aqueductal stenosis may have had its origin in the patient's developmental stage. 7 Although similar histologically to that found in the infantile and childhood forms, 4,~ this narrowing may be less severe; consequently, it becomes manifest at a later age. References
1. Beckett RS, Netsky MG, Zimmerman HM: Developmental stenosis of the aqueduct of Sylvius. Am J Pathoi 26:755-787, 1950 551
J. R. Little, O. W. Houser and C. S. MacCarty 2. Bickers DS, Adams RD: Hereditary stenosis of the aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 72:246-262, 1949 3. Dandy WE, Blackfan KD: Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child 8:406--482, 1914 4. Drachman DA, Richardson EP: Aqueductal narrowing, congenital and acquired: a critical review of the histologic criteria. Arch Nearoi 5:552-559, 1961 5. Du Boulay G, Trickey S: The sella in aqueduct stenosis and communicating hydrocephalus. Br J Radiol 43:319-326, 1970 6. yon Friedmann G: Die nicht durch Tumor bedingte Aqu~iduktstenose und ihr Nachweis im R6tgeniibcrsichtsbild. Radiologe 4:190194, 1964 7. Glohus JH, Bergman P: Atresia and stenosis of the aqueduct of Sylvius. J Neuropathol Exp Neurol 5:342-363, 1946 8. Hogan PA, Woolsey RM: Hydrocephalus in the adult. JAMA 198:524-528, 1966 9. Huang YP, Wolf BS, Antin SP, r al: Angiographic features of aqueductal stenosis. Am J Roentgenol Radium Ther Nucl Med 104:90-108, 1968 10. Lichtenstein BW: Atresia and stenosis of the aqueduct of Sylvius: with comments on the Arnold-Chiari complex. J Neuropathol Exp Neurol 18:3-21, 1959 11. Nag TK, Falconer MA: Non-tumoral stenosis of the aqueduct in adults. Br Med J 2:1168-1170, 1966 12. Norman RM: Malformations of the nervous system, birth injury and diseases of early life, in Blackwood W, McMcnemy WH, Meyer A, et al (eds): Greenfield's Neuropathology, ed. 2. Baltimore, Williams & Wilkins, 1963, pp 324-440 13. Paine KWE, McKissock W: Aqueduct stenosis: clinical aspects, and results of treatment by ventriculocisternostomy (Torkildsen's operation). J Neurosurg 12:127-145, 1955 14. Parker HL, Kernohan JW: Stenosis of the aqueduct of Sylvius. Arch Neurol Psychiatry 29:538-558, 1933 15. Pennybacker J: Stenosis of the aqueduct of Sylvius. Proc R Sor Med 33:507-512, 1940 16. Petit-Dutaillis D, Thi6baut F, Berdet H, et al: Apropos des st6noses de l'aqueduc de Sylvius
552
17. 18. 19.
20. 21. 22.
23.
24.
25.
26. 27.
d'origine non tumorale de l'adolescent et de l'adulte. Rev Neurol 82:417-421, 1950 Roukkula M, Halonen V: Stenosis of the aqueduct of non-tumoral origin. Aeta Radiol (Diagn) (Stockh) 12:289-297, 1972 Sajid MH, Copple PJ: Familial aqueductal stenosis and basilar impression. Neurology (Minneap) 18:260-262, 1968 Schechter MM, Rovit RL, Nelson K: Spontanecus "high pressure CSF rhinorrhoea". Cerebrospinal fluid leakage caused by longstanding increased intracranial pressure. Br J Radiol 42:619-622, 1969 Schechter MM, Zingesser LH: The radiology of aqueductal stenosis. Radiology 88:905-916, 1967 Sheldon WD, Parker HL, Kernohan JW: Occlusion of the aqueduct of Sylvius. Arch Neuroi Psychiatry 23:1183-1202, 1930 Spiller WG: Two cases of partial internal hydrocephalus from closure of the interventricular passages: with remarks on bilateral contractures caused by a unilateral cerebral lesion. Am J Meal Sei 124:44-55, 1902 Stookey B, ScarffJ: Occlusion of the aqueduct of Sylvius by neoplastic and non-necplastic processes with a rational surgical treatment for relief of the resultant obstructive hydrocephalus. Bull Neuroi Inst NY 5: 348-377, 1936 Timmons GD, Johnson KP: Aqueductal stenosis and hydrocephalus after mumps encephalitis. N Engl J Med 283:1505-1507, 1970 Torkildsen A: Ventriculocisternostomy. A Palliative Operation in Different Types of Noncommunicating Hydrocephalus. Oslo, Johan Grundt Tanum Forlag, 1947 Touche M: Hydroc6phalie interne (pr6sentation de pi6ces). Bull Mem Soc Med Hop Paris 19:141-144, 1902 Wilkinson HA, LeMay M, Drew JH: Adult aqueductal stenosis. Arch Neurol 15:643-648, 1966
Address reprint requests to: John R. Little, M.D., c/o Section of Publications, Mayo Clinic, 200 First Street S.W., Rochester, Minnesota 55901.
J. Neurosurg. / Volume 43 / November, 1975
QUEDUCTAL stenosis in the adult is not rare',' 9,1,,ls-ls,2o-,,,,27 but, in contrast to the infantile and childhood forms, little information is available regarding its clinical presentation and diagnosis. Here we report a review of the recent experience at the Mayo Clinic with this condition in adults.
A
A n a l y s i s of Cases
Clinical Presentation Seventeen adult patients seen at the Mayo Clinic between 1956 and 1974 were found to have nontumoral obstruction of the aqueduct of Sylvius. The 10 men and seven women ranged in age from 19 to 52 years (mean, 28 years). The symptoms and neurological find546
9
ings at the initial evaluation are listed in Table 1. The clinical presentations of these 17 patients studied can be divided into the following five groups: 1) headache indicative of increased intracranial pressure; 2) seizure disorder, usually temporal lobe seizures; 3) cerebrospinal fluid (CSF) rhinorrhea and headache; 4) endocrine dysfunction; and 5) gait disturbance and headache. There was some degree of overlap among the various groups. Headache was a frequent symptom but it was the main complaint in only four patients. Many of the patients described their headaches as being relatively mild and of short duration; however, a recent increase in frequency and severity was occasionally J. Neurosurg. / Volume 43 / November, 1975
Aqueductal stenosis in adults TABLE l
Clinical presentation, treatment, and follow-up of 17 patients with aqueductal stenosis Case No.
Age, Sex
Symptoms
Neurological Findings
Shunt Ventricle
Follow-up
1
24 F
headache (4 yr); endogenous depression (6 yr)
normal
cistern
occasional headache (14 yr)
2
23 F
chronic papilledema
cistern
3
19 M
headache (10 yr); blurred vision (5 wk) headache (1 yr); blurred vision (5 mo); unsteady gait (5 mo); paresthesias, face (4 mo)
complete recovery (2 yr) vision improved; doing well (13 yr)
4
26 M 28 M
acute papilledema; gait ataxia; It Babinski chronic papilledema
cistern
5 6
23 M
broad-based gait; hyperreflexia, legs; decreased mentation
cistern
seizures unchanged; slight improvement otherwise (7 yr)
7
31 M
normal
atrium
fewer seizures (3 yr)
8
28 M
gait ataxia; mild ataxia, arms
peritoneum no seizures (2 yr)
9
22 M
normal
peritoneum fistula repaired (6 mo)
10
39 M
normal
cistern
fistula repaired (11 yr)
11
21 F 20 M
chronic papilledema; gross obesity bulging craniectomy site
cistern
12
developed seizure disorder (I0 yr) complete recovery (10 yr)
13
19 F
headache (1 wk); blurred vision (3 days) seizure disorder (23 yr); headache (4 yr) seizure disorder (6 yr); headache (2 wk); unsteady gait (6 mo); personality changes & forgetfulness (6 rap); polyuria & polydipsia (6 mo) seizure disorder (2 yr); headache (18 yr) seizure disorder (3 wk); headache (3 wk); mild loss of memory CSF rhinorrhea (1 yr); headache (10 yr) CSF rhinorrhea (6 mo); headache (2 mo) CSF rhinorrhea (1 yr); headache (9 yr) CSF rhinorrhea (5 mo); headache (2 wk); nausea & vomiting (2 wk); moderate loss of memory (1 mo) primary amenorrhea
14
36 F
15
52 M
16
30 F
17
46 F
optic disc pallor; chronic cistern papilledema; contracted fields (large central scotoma, rt eye; ring scotoma, It eye)
cistern
pleura
occasional headache (18 yr) fewer seizures (12 yr)
small bitemporal field cistern amenorrhea persists; defects; underdeveloped normal visual fields secondary sex (6 yr) characteristics normal peritoneum amenorrhea persists (4 yr) broad-based gait; It cistern considerable improvement Babinski ; decreased (13 yr) upward gaze; stiff neck
secondary amenorrhea (2 yr); headache (2 yr) unsteady gait (2 89yr); leg weakness (2 89yr); headache (5 yr); mild loss of memory weakness, It leg (1 mo); spastic It leg; It Babinski; cistern paresthesias, It leg moderate hyperreflexia, (1 yr); headache (10 yr) arms & legs stiffness, legs (6 yr); gait ataxia; spastic legs; cistern paresthesias, legs bilateral Babinski (6 yr); headache (10 yr); urinary frequency & urgency (4 yr)
J. Neurosurg. / Volume 43 / November, 1975
complete recovery (4 yr) considerable improvement (1 89yr)
547
J. R. Little, O. W. H o u s e r a n d C. S. M a c C a r t y noted. Transient obscurations of vision occurred in three of the four patients who complained primarily of headache. Four patients presented with a seizure disorder. One patient (Case 5) who had had petit mal seizures during childhood developed temporal lobe seizures consisting of an olfactory aura, fugue state, and dbjh vu phenomena. A single generalized convulsion occurred 2 weeks before admission. Another patient (Case 7) had a 2-year history of recurrent temporal lobe seizures; he also had experienced three generalized convulsions during the I Ih years prior to admission. One patient (Case 6) had recurrent temporal lobe seizures only. The fourth patient (Case 8) had had two generalized convulsions, each followed by severe headache. Spontaneous CSF rhinorrhea and beadache, the presenting complaints of three patients, had been present for 2 months or longer with no history to explain them. A fourth patient (Case 12) developed CSF rhinorrhea about 1 month after he had suffered a depressed compound frontotemporal fracture. During the convalescent period he experienced a febrile episode that was thought to be a bacterial meningitis; however, the causative organism was never identified and the patient recovered. An attempt to repair the CSF leak was unsuccessful although a fracture line was identified. Six months after the accident, symptoms and signs of increased intracranial pressure developed. The clinical presentation of some patients suggested an endocrine disturbance. Two of the female patients had amenorrhea. One of them (Case 13) had never menstruated and had underdeveloped secondary sex characteristics. The presence of bitemporal field defects suggested a lesion in or adjacent to the chiasm. The other patient (Case 14) had not menstruated for 2 years, but prior to that time she had menstruated normally. Polydipsia and polyuria were complaints of another patient (Case 6). Marked obesity was noted only once (Case 11). The average duration of symptoms prior to diagnosis was 6 years. A history of recurrent headaches during childhood and adolescence was frequently obtained. Only three patients presented with a history suggestive of mental deterioration; most of the patients had been 548
able to perform adequately at their work prior to admission, Diagnostic Procedures
The ventricular fluid was examined in 16 patients and the spinal fluid in 12. The highest protein concentration found in the ventricular fluid was 18 mg/dl. In three instances the spinal fluid had slightly increased protein concentrations (46, 50, and 67 mg/dl). Of the 13 electroencephalograms obtained, a "projected type" dysrhythmia was identiffed in eight, four were considered to be normal, and one, from a patient with temporal lobe seizures (Case 7), showed a right temporal spike focus. Skull x-ray films of 14 patients were available for evaluation (Table 2). Measurements suggested the presence of cranial enlargement in 11 cases. Evidence of chronic increased intracranial pressure, as demonstrated by digital markings or split sutures, was present in 10 and alterations of the dorsum sellae were identified in 13. The anterior wall of the sella was longer and steeper than normal in six patients (Fig. 1). The anterior clinoid processes occasionally appeared enlarged. The skull films of the remaining three patients were reported as showing changes indicative of increased intracranial pressure. Right retrograde brachial angiography was performed in seven cases. Angiographic features indicative of marked, symmetrical enlargement of the lateral ventricles were present, and there was no evidence of a shift. The posterior cerebral arteries were consistently stretched and depressed. Flattening of the course of the superior cerebellar arteries was also a consistent finding on the lateral angiogram. Posterior displacement of the terminal basilar artery was present in one case, and a downward and lateral displacement was observed in another. Some degree of depression and flattening of the internal cerebral veins was noted in all cases. The anterior end of the basal vein of Rosenthal occasionally was depressed. The vessels in the lower posterior fossa were unaltered. Air ventriculography was performed in 16 patients. Marked dilatation of the lateral and third ventricles was present in all cases and the mean thickness of the cortical mantle was 29 mm (range, 22 to 36 mm). On concomitant J. Neurosurg. / Volume 43 / November, 1975
'O
t.a
sizet
.
.
-
+
-
(oblique) -
.
.
.
. . . .
(oblique) .
+ (post. floor) +
.
.
+ -
+G + .
E
6
.
.
.
.
.
(oblique) .
+
.
+ + +
.
8
7
.
.
.
E
8
-
. +
+ + -
+G +
* + = p r e s e n t ; - = a b s e n t . S k u l l f i l m s w e r e n o t a v a i l a b l e f o r C a s e s 2, 3, a n d 4. t E = enlarged; B = borderline; N = normal. :~ G = g e n e r a l i z e d ; F = f r o n t a l .
increased density
elongated
ant. wall
-t . . .
ant. clinoids large eroded deformed
+ + +
+ (mid. floor) +
+ + -
sella eroded
.
-FG -
B
E
+G + .
5
1
dorsum sellae short flat eroded displaced ant.
digital markings~ split sutures basilar invagination
cranium
Feature
2
.
.
.
. +
-
+
.
.
(vertical)
.
+ + + +
-FF +
E
9
.
.
.
-
-
+ . +
.
N
10
Case No.
Summary of findings on skull films*
TABLE
.
.
.
.
.
+
.
+
-
-
+ +
+G
N
11
.
.
.
.
-
.
.
-
-
-
-
B
.
12
.
.
+
.
+
+ + . +
+
+G
B
13
.
-
+
+ +
.
.
(vertical) .
+ (ant. floor) +
.
.
+G
E
14
.
.
.
-
-
-
+ + -
-
.
.
E
15
.
+
+
+
+ _ + _
(vertical) .
.
+
E
16
-
_
+
_ _ + _
+F +
N
17
~"
~:L
~..i.
O
J. R. Little, O. W. Houser and C. S. MacCarty None of the patients have developed symptoms or neurological findings to suggest the presence of a brain-stem glioma. Discussion
Ftc. 1. Case 14. Skull film, lateral view, demonstrating generalized digital markings indicative of chronic increased intracranial pressure. Anterior wall of sella is elongated and vertical (arrow), and floor is eroded. Dorsum sellae appears flattened and displaced anteriorly. pneumoencephalography in 11 patients, the fourth ventricle filled with air in 10, but it infrequently entered the lower end of the aqueduct. In only one patient was a pneumoencephalogram performed, which demonstrated uniform narrowing of the entire aqueduct. The configurations of the stenosed aqueducts are illustrated in Fig. 2. We were unable to define accurately the configuration of the obstruction in four cases. In one case computerized axial tomography (EMI scan) demonstrated marked dilatation of the lateral and third ventricles. Serological tests for syphilis were negative in the 16 patients tested. Treatment Shunting procedures were performed in all cases (Table 1). Twelve patients underwent an exploration of the posterior fossa and insertion of a ventriculocisternal shunt (Torkildsen procedure)) 5 There was no evidence of tumor m any of these patients. Two of the patients who presented with CSF rhinorrhea subsequently required direct repair of the fistula. Follow-up The results of follow-up are listed in Table 1. The mean duration of follow-up has been 10 years, and there have been no deaths. 550
Seizures are a frequent occurrence in adults with aqueductal stenosis. 6,11,18 Four patients in our series presented with a seizure disorder, and in three of them it had been a chronic problem. There also appeared to be a definite predilection for the development of temporal lobe seizures. Similarly, four of the 10 adult patients with aqueductal stenosis described by Nag and FalconeP 1 had a seizure disorder and in three of these patients it was described as the "psychomotor type." Spontaneous CSF rhinorrhea is an uncommon phenomenon and has seldom been described in association with aqueductal stenosis? 9 The development of this complication appears to be the result of erosion of the floor of the anterior fossa secondary to longstanding increased intracranial pressure. The fistulous tract may develop either spontaneously or after a trivial traumatic event. Compression of the pituitary, hypothalamus, and the optic chiasm by the greatly distended third ventricle appears to be responsible for the endocrine dysfunction and some of the visual problems observed in these patients. Also amenorrhea, impotence, gynecomastia, sexual precocity, delayed development of secondary sex characteristics, and obesity have been reported, e,~'~8'~8'2~ Treatment of the hydrocephalus occasionally results in relief of these symptoms. Nag and FalconeP 1 described a case of aqueductal stenosis in an 18-year-old woman with primary amenorrhea who began to menstruate 1 year after insertion of a ventriculocisternal shunt. One patient in our series had mild diabetes insipidus that resolved postoperatively. Another patient with small bitemporal field defects had normal visual fields 1 week postoperatively. In two of our patients, the amenorrhea failed to resolve with treatment. The triad of headache, dementia, and gait disturbance is characteristic of the clinical syndrome of chronic hydrocephalus in the adult) Headache and gait disturbance were frequently observed in our group but mental deterioration was seldom noted. This relative preservation of mental facilities may be J. Neurosurg. / Volume 43 / November, 1975
A q u e d u c t a l stenosis in a d u l t s related in part to the gradual progression of the disorder; however, we assume that decompensation would eventually occur in most of these patients if untreated. The findings on the plain skull films in most patients attested to the chronicity of this disorder. The sella frequently was affected in a characteristic way? The only patient without demonstrable sellar changes was thought to have developed aqueductal stenosis on the basis of an ependymitis only a few months prior to admission; however, a large cranial defect resulting from debridement of a compound skull fracture may have delayed the development of these changes. We agree with Du Boulay and Trickey 5 that the elongated and vertical anterior sella wall is indicative of a process that has probably been present since early life. A small posterior fossa, commonly seen in the infantile form of aqueductal stenosis, is rarely present in adults, s~ The skull films of only one of our patients demonstrated a low-placed internal occipital protuberance suggesting a small posterior fossa. The results of ventriculography, pneumoencephalography, and angiography corresponded closely to those reported by other investigators .9.~s,17,20,~7 Adult aqueductal stenosis is treated by insertion of a shunt. Ventriculocisternal shunts have consistently given good results and can be combined with exploration of the posterior fossa. Other types of surgical treatment of this condition have been reviewed by Paine and McKissock? 8 The pathogenesis of aqueductal stenosis in the adult is obscure in most cases. Inflammation, usually secondary to infection, has occasionally been implicated 8'~2-~4'~~ and appeared to be important in one of our patients (Case 12). Familial cases also have been described? ,~8 It is present sometimes in neurofibromatosis n,ls or in association with an Arnold-Chiari malformation? ~ In our series, an Arnold-Chiari (Chiari type II) malformation was found in one patient (Case 11) and tonsillar herniation alone (Chiari type I) was found in four patients (Cases 2, 4, 5 and 8). The clinical findings in our patients suggest that the aqueductal narrowing and hydrocephalus had been present in most of the patients since early life. Factors that substantiate this observation include the following: 1) long clinical histories, often J. Neurosurg. / Volume 43 / November, 1975
Configurations
No. of cases
Fie. 2. Varieties of obstructed aqueducts as demonstrated by air ventriculography and pneumoencephalography. "No aqueduct" means no filling of the upper end of the aqueduct with air; complete obstruction of the aqueduct is incompatible with life.
covering many years; 2) frequent occurrence of deterioration in early adulthood; 3) relatively normal day-to-day performance of most of the patients despite significant ventricular dilatation; and 4) characteristic changes in the sella, especially elongation of the anterior wall, and evidence of chronic increased intracranial pressure on skull films. In many of these cases, the aqueductal stenosis may have had its origin in the patient's developmental stage. 7 Although similar histologically to that found in the infantile and childhood forms, 4,~ this narrowing may be less severe; consequently, it becomes manifest at a later age. References
1. Beckett RS, Netsky MG, Zimmerman HM: Developmental stenosis of the aqueduct of Sylvius. Am J Pathoi 26:755-787, 1950 551
J. R. Little, O. W. Houser and C. S. MacCarty 2. Bickers DS, Adams RD: Hereditary stenosis of the aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 72:246-262, 1949 3. Dandy WE, Blackfan KD: Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child 8:406--482, 1914 4. Drachman DA, Richardson EP: Aqueductal narrowing, congenital and acquired: a critical review of the histologic criteria. Arch Nearoi 5:552-559, 1961 5. Du Boulay G, Trickey S: The sella in aqueduct stenosis and communicating hydrocephalus. Br J Radiol 43:319-326, 1970 6. yon Friedmann G: Die nicht durch Tumor bedingte Aqu~iduktstenose und ihr Nachweis im R6tgeniibcrsichtsbild. Radiologe 4:190194, 1964 7. Glohus JH, Bergman P: Atresia and stenosis of the aqueduct of Sylvius. J Neuropathol Exp Neurol 5:342-363, 1946 8. Hogan PA, Woolsey RM: Hydrocephalus in the adult. JAMA 198:524-528, 1966 9. Huang YP, Wolf BS, Antin SP, r al: Angiographic features of aqueductal stenosis. Am J Roentgenol Radium Ther Nucl Med 104:90-108, 1968 10. Lichtenstein BW: Atresia and stenosis of the aqueduct of Sylvius: with comments on the Arnold-Chiari complex. J Neuropathol Exp Neurol 18:3-21, 1959 11. Nag TK, Falconer MA: Non-tumoral stenosis of the aqueduct in adults. Br Med J 2:1168-1170, 1966 12. Norman RM: Malformations of the nervous system, birth injury and diseases of early life, in Blackwood W, McMcnemy WH, Meyer A, et al (eds): Greenfield's Neuropathology, ed. 2. Baltimore, Williams & Wilkins, 1963, pp 324-440 13. Paine KWE, McKissock W: Aqueduct stenosis: clinical aspects, and results of treatment by ventriculocisternostomy (Torkildsen's operation). J Neurosurg 12:127-145, 1955 14. Parker HL, Kernohan JW: Stenosis of the aqueduct of Sylvius. Arch Neurol Psychiatry 29:538-558, 1933 15. Pennybacker J: Stenosis of the aqueduct of Sylvius. Proc R Sor Med 33:507-512, 1940 16. Petit-Dutaillis D, Thi6baut F, Berdet H, et al: Apropos des st6noses de l'aqueduc de Sylvius
552
17. 18. 19.
20. 21. 22.
23.
24.
25.
26. 27.
d'origine non tumorale de l'adolescent et de l'adulte. Rev Neurol 82:417-421, 1950 Roukkula M, Halonen V: Stenosis of the aqueduct of non-tumoral origin. Aeta Radiol (Diagn) (Stockh) 12:289-297, 1972 Sajid MH, Copple PJ: Familial aqueductal stenosis and basilar impression. Neurology (Minneap) 18:260-262, 1968 Schechter MM, Rovit RL, Nelson K: Spontanecus "high pressure CSF rhinorrhoea". Cerebrospinal fluid leakage caused by longstanding increased intracranial pressure. Br J Radiol 42:619-622, 1969 Schechter MM, Zingesser LH: The radiology of aqueductal stenosis. Radiology 88:905-916, 1967 Sheldon WD, Parker HL, Kernohan JW: Occlusion of the aqueduct of Sylvius. Arch Neuroi Psychiatry 23:1183-1202, 1930 Spiller WG: Two cases of partial internal hydrocephalus from closure of the interventricular passages: with remarks on bilateral contractures caused by a unilateral cerebral lesion. Am J Meal Sei 124:44-55, 1902 Stookey B, ScarffJ: Occlusion of the aqueduct of Sylvius by neoplastic and non-necplastic processes with a rational surgical treatment for relief of the resultant obstructive hydrocephalus. Bull Neuroi Inst NY 5: 348-377, 1936 Timmons GD, Johnson KP: Aqueductal stenosis and hydrocephalus after mumps encephalitis. N Engl J Med 283:1505-1507, 1970 Torkildsen A: Ventriculocisternostomy. A Palliative Operation in Different Types of Noncommunicating Hydrocephalus. Oslo, Johan Grundt Tanum Forlag, 1947 Touche M: Hydroc6phalie interne (pr6sentation de pi6ces). Bull Mem Soc Med Hop Paris 19:141-144, 1902 Wilkinson HA, LeMay M, Drew JH: Adult aqueductal stenosis. Arch Neurol 15:643-648, 1966
Address reprint requests to: John R. Little, M.D., c/o Section of Publications, Mayo Clinic, 200 First Street S.W., Rochester, Minnesota 55901.
J. Neurosurg. / Volume 43 / November, 1975
