J Neurosurg 92:933–940, 2000

Pathophysiology of long-standing overt ventriculomegaly

in adults

SHIZUO OI, M.D., MASAMI SHIMODA, M.D., MASAYOSHI SHIBATA, M.D.,

YUMIE HONDA, M.D., KOUJI TOGO, M.D., MASAKI SHINODA, M.D.,
RYUICHI TSUGANE, M.D., AND OSAMU SATO, M.D.
Department of Neurosurgery, Tokai University, School of Medicine, Isehara, Kanagawa, Japan

Object. Long-standing overt ventriculomegaly in adults (LOVA) is a unique form of hydrocephalus that devel-
ops during childhood and manifests symptoms during adulthood. The aim of the present study was to analyze the
specific pathophysiological characteristics of LOVA.
Methods. The specific diagnostic criteria for LOVA include severe ventriculomegaly in adults that is associated
with macrocephalus measuring more than two standard deviations in head circumference and/or neuroradiologi-
cal evidence of a significantly expanded or destroyed sella turcica. Twenty patients who fulfilled these criteria, 14
males and six females, were retrospectively studied. These patients’ ages at diagnosis ranged from 15 to 61 years
(mean 39.4 years). All had symptoms and/or signs indicating that hydrocephalus first occurred at birth or during in-
fancy in the absence of any known underlying disease. The authors performed a pathophysiological study that in-
cluded specific variations of magnetic resonance (MR) imaging, such as fluid-attenuated inversion recovery and
cardiac-gated cine-mode imaging; intracranial pressure (ICP) monitoring; three-dimensional computerized tomog-
raphy (CT) scanning; and other techniques.
Hydrocephalus was caused by aqueductal stenosis in all patients. Severe ventriculomegaly involving the lateral and
third ventricles was associated with a marked expansion or destruction of the sella turcica in 17 cases. Cardiac-gated
cine-MR imaging did not reveal any significant movements of cerebrospinal fluid in the aqueduct. Three-dimension-
al CT ventriculography confirmed that the expanded third ventricle protruded into the sella and, sometimes, extended
a diverticulum. Fourteen patients revealed symptoms and signs that indicated increased ICP with prominent pressure
waves. Dementia or mental retardation was seen in 11 patients, gait disturbance in 12, and urinary incontinence in
eight; all three of these symptoms were observed in seven patients. Thirteen patients experienced visual disturbance.
Nine patients underwent ventriculoperitoneal shunt implantation as the initial treatment, leading to postoperative sub-
dural hematoma in all seven cases in which a differential pressure valve was used. Nine patients, three of whom were
initially treated by shunt placement, underwent a neuroendoscopic procedure, mainly for third ventriculostomy. Post-
operatively, ICP returned to normal, and marked to-and-fro pulsatile movements at the site of ventriculostomy were
recognized on cine-MR imaging in patients treated endoscopically. However, the ventriculomegaly was little im-
proved. Consequently, all patients eventually demonstrated improvement in response to either a shunt equipped with
a pressure-programmable valve or an endoscopic procedure; however, depression appeared in six patients, who re-
quired psychiatric consultation or medication.
Conclusions. Such remarkably decreased intracranial compliance but relatively high ICP dynamics are the patho-
physiological characteristics of LOVA. The therapeutic regimen should be determined based on the individual’s
specific pathophysiological makeup.

KEY WORDS • hydrocephalus • macrocephaly • empty sella turcica •

aqueductal stenosis • subdural hematoma • neuroendoscopic surgery

EFORE recent diagnostic procedures were intro- sess 46 children and adolescents in whom a clinical di-

B duced for the diagnosis of hydrocephalus, one of

the most accepted indicators for progressive hy-
drocephalus in infants was head circumference. However,
formulating a diagnosis of arrested hydrocephalus on the
basis of this indicator alone would be questionable. Whit-
tle and colleagues41 used continuous ICP monitoring to as-
Abbreviations used in this paper: CSF = cerebrospinal fluid;
CT = computerized tomography; DPV = differential pressure valve;
ICP = intracranial pressure; IQ = intelligence quotient; LOVA =
long-standing overt ventriculomegaly in adults; MR = magnetic
resonance; PPV = pressure-programmable valve; ROI = region of
interest; VP = ventriculoperitoneal; 3D = three dimensional.
agnosis of arrested hydrocephalus had been made. They
found that 80% of patients who had previously been treat-
ed had a nonfunctioning shunt and that 63% of patients
who had received no previous treatment had episodic or
persistent increased ICP. When serial psychometric testing
demonstrated a fall in cognitive functioning, ICP monitor-
ing revealed abnormal ICPs in 88% of patients. The re-
sults of this study may indicate that CSF dynamics change
over time, shifting hydrocephalus between active and in-
active states; however, the factors affecting such changes
remain uncertain.
In 1996, we reported on the specific clinical features
of infantile hydrocephalus that continues into adulthood,

J. Neurosurg. / Volume 92 / June, 2000 933


TABLE 1
Concept, diagnostic criteria, and treatment of LOVA*
Category Definition
concept chronological entity of progressive hydrocephaly w/ long-
standing ventriculomegaly in adult, most likely starting
from infancy
diagnostic 1) overt ventriculomegaly involving the lateral &
criteria third ventricles w/ obliterated cortical sulci on CT/MR im-
aging
2) clinical symptoms include macrocephaly w/ or w/o sub-
normal IQ, headaches, dementia, gait disturbance, urinary
incontinence, vegetative state, akinetic mutism, apathetic
consciousness, & parkinsonism
3) neuroimages may demonstrate expanded or destroyed
sella turcica as evidence of long-standing ventriculomegaly
therapeutic treatable w/ shunt, but extremely delicate pressure control
specificity such as that provided by a PPV is required; neuroendo-
scopic third ventriculostomy is mostly effective
* Definitive LOVA applies to patients having the diagnostic criteria of 1
and also 2 and/or 3.
proposing a long-term form of hydrocephalus, “long-
standing overt ventriculomegaly in adult” (LOVA).25 Al-
though its mechanism still remains unclear, patients with
LOVA often suffer from a progressive course of hydro-
cephalus that continues into adulthood. We also reported
that the hydrocephalic state in LOVA is extremely diffi-
cult to treat with a shunt because of lost intracranial com-
pliance. Currently, there are various methods of patho-
physiological evaluation of hydrocephalus and advanced
techniques to treat even complicated cases. The purpose
of the present study was to analyze the unique pathophys-
iological characteristics of LOVA and to discuss therapeu-
tic outcomes in our series based on these specific patho-
physiological characteristics.
Clinical Material and Methods
Diagnostic Criteria and Patient Population
Patients with LOVA in whom significant progressive
symptoms of hydrocephalus had developed were first di-
agnosed as being hydrocephalic during adulthood.25 In all
patients, ventriculomegaly was prominent, involving the
lateral and third ventricles as demonstrated on CT and/or
MR images. None of the patients had any known under-
lying disease or symptoms or signs, indicating that the
hydrocephalus had first occurred at birth or during infan-
cy in accordance with neuroimaging findings of long-
standing hydrocephalus. In this study, to prove this histo-
ry objectively, the specific diagnostic criteria for LOVA
included macrocephaly greater than two standard devia-
tions in head circumference, 57 cm in female and 58 cm
in male patients, and/or neuroradiological evidence of a
significantly expanded or destroyed sella turcica (Table
1). The study population consisted of 14 male and six fe-
male patients whose disease was diagnosed between 1985
and 1998. The patients’ ages ranged from 15 to 61 years
(mean 39.4 years). All 20 patients fulfilled these diagnos-
tic criteria. The youngest patient was 15 years old at diag-
nosis and 16 to 17 years of age when treated. We includ-
ed this patient as an adult.
934
S. Oi, et al.
Pathophysiological Analyses
All patients underwent CT and MR imaging (1.5-tesla
MR system). The MR imaging session included T1- and
T2-weighted sequences. Fluid-attenuated inversion recov-
ery and cardiac-gated cine-MR techniques were also used
in most cases. During the cine-MR imaging, the pulse se-
quence was set at a repetition time of 22 msec, an echo
time of 14 msec, and a flip angle of 10˚. To analyze re-
gional CSF movement, the axial view of the aqueduc-
tal region and the sagittal and coronal views of the floor
of the third ventricle and basal cistern were obtained in
multiple images, cine frames, during an R–R interval that
commenced immediately after the R-wave was measured
on electrocardiography. Quantitative analysis focused on
ROIs at individual target points on these images. Intra-
cranial pressure monitoring was performed using either
lumbar drainage or external ventricular drainage via a burr
hole made preoperatively. After an external ventricular
drainage system had been placed and overnight continu-
ous ICP monitoring had been performed, 5 ml of water-
soluble iodine contrast (Iohexol, Omnipaque 180) was
injected into the ventricle and 3D CT scans were obtained
for evaluation of communication in the CSF pathway. In-
tracranial pressure monitoring, 3D CT scanning, and car-
diac-gated cine-MR imaging were repeated postoperative-
ly, especially after neuroendoscopic surgery.
Treatment of LOVA
Between 1985 and 1991, patients were mainly treated
using a DPV-regulated shunt system (Pudenz type). Since
1992 a PPV (Sophy Valve)-regulated shunt system and/or
neuroendoscopic ventriculostomy were used in most cas-
es. The shunting operation was performed using the stan-
dard technique and a VP shunt was usually placed on
the right side. The PPV was placed at the subclavicular re-
gion subcutaneously. Neuroendoscopic third ventriculos-
tomy or related procedures were performed by the senior
author (S.O.) in the standard fashion20 by using a rigid-rod
endoscope for morphological observation and a steerable,
flexible fiber-rod endoscope for the surgical procedure.
We induced coagulation in several spots using a monopo-
lar coagulator and used microforceps to remove the cen-
tral area of the membranous floor by performing an inten-
sive to-and-fro movement.
Sources of Supplies and Equipment
Magnetic resonance imaging was performed using the
1.5-tesla Gyroscan MR system (ACN-NI) produced by
Phillips (Best, The Netherlands). The Omnipaque 180 was
obtained from Daiichiseiyaku (Tokyo, Japan). The DPV
(Pudenz type) was designed by Heyer–Schulte NeuroCare
(Pleasant Prairie, WI) and the PPV (Sophy Valve) by
Sophisa (Besançon, France). The rigid-rod endoscope was
furnished by Karl Storz GmbH & Co. (Tuttlingen, Ger-
many), and the flexible fiber-rod endoscope by Codman
(Boston, MA).
Results
Signs and Symptoms
Among the 20 patients who fulfilled the specific diag-
J. Neurosurg. / Volume 92 / June, 2000
Long-standing overt ventriculomegaly in adults
TABLE 2
Findings in 20 patients with LOVA
Finding
skull x-ray films (20 patients)
expanded or destroyed sella turcica
digital marking
CT or MR images (20 patients)
overt ventriculomegaly involving lateral & third ventricles
aqueductal stenosis
expanded or destroyed & empty sella turcica
ICP monitoring (10 patients w/ continuous monitoring)
increased ICP with prominent pressure waves
nostic criteria of LOVA, head circumference ranged from
54 to 63 cm (mean 59 cm). Sixteen patients had macro-
cephaly, three patients appeared normocephalic, and the
status of the other one was unknown. Information con-
cerning patients’ prenatal history and development was
scarce, but six were known to have exhibited macroceph-
aly at birth or during infancy, and five had some delay in
psychomotor development. Intelligence quotients ranged
from 66 to 122 (mean 91.5) and 10 patients were thought
to have subnormal intelligence. Dementia or mental retar-
dation was seen in 11 patients, gait disturbance in 12, and
urinary incontinence in eight; all three symptoms were ob-
served in seven patients. Symptoms and signs suggestive
of increased ICP were seen in 14 patients with headaches,
in one with a decreased level of consciousness, and in
three with papilledema of the optic fundi. Visual symp-
toms included decreased visual acuity in four patients; up-
ward gaze palsy, third cranial nerve palsy, and proptosis in
three patients each; and visual field defect in one patient.
Parkinsonism was observed with tremor in four patients,
and akinesia or masked face in three. Three patients had
a history of epilepsy and three others were dysarthric
or mute.
Neuroimaging Studies
All patients had severe ventriculomegaly involving the
lateral and third ventricles with decreased thickness of
the cortical mantle and obliterated sulci. Skull x-ray films
revealed an enlarged or completely destroyed (phantom)
sella turcica with or without digital markings in 17 pa-
tients. Axial CT and MR images demonstrated marked
symmetrical dilation of the lateral ventricles with signifi-
cant dilation of the third ventricle. Midline sagittal MR
images revealed vertically expanded third ventricles in-
volving the pituitary fossa, pushing the herniated floor
into the sella turcica, which was usually remarkably en-
larged or appeared as a phantom (Table 2). The corpus cal-
losum was stretched upward in an unrolling configuration
(Fig. 1).
Cardiac-gated cine-MR imaging revealed no significant
CSF movements within the aqueduct. The aqueductal ste-
nosis was confirmed both morphologically and function-
ally on MR images after we began to use quantitative CSF
flow measurement in recent cases (Fig. 2). There were re-
markably turbulent CSF movements in the third ventricle,
which expanded the floor onto the sellar region and creat-
ed a diverticulum in some cases (Fig. 3 upper left). The
J. Neurosurg. / Volume 92 / June, 2000
No. of
Cases
17
3
20
20
17
10
FIG. 1. Case 1 in Fig. 5. Preoperative axial (upper left and right)
and sagittal (lower left) T1-weighted MR images (TR 500 msec, TE
15 msec) and a coronal T2-weighted MR image (lower right) (TR
2491 msec, TE 120 msec). Note severe ventriculomegaly involv-
ing the lateral and third ventricles caused by aqueductal stenosis
with a totally destroyed (phantom) sella. There is little CSF move-
ment in the dilated ventricle, appearing with void artifact, on the
T2-weighted image.
communication of these structures, as well as obstruction
of the aqueduct, were confirmed by preoperative 3D CT
ventriculography (Fig. 3 upper right). The ICP dynamics
were studied by continuous monitoring in 10 patients.
High pressure was found in all patients, with prominent
pressure waves in most cases; the ICP sometimes was re-
corded at levels greater than 30 mm Hg (Fig. 4).
Surgical Procedures and Complications
Surgical treatment was selected in 18 patients for the
initial therapeutic procedure; nine patients underwent VP
shunt placement and the other nine underwent neuroendo-
scopic ventriculostomy. Among those patients in whom
shunting was chosen, a DPV-regulated shunt was initially
used in seven (a medium-pressure valve was used in two
patients, a low-pressure valve in two, and the valve pres-
sure was unknown in three), and a PPV-regulated shunt in
two. All patients treated initially with a DPV experienced
subdural hematoma and underwent reoperation. In the two
patients in whom PPVs were placed, CSF flow was ini-
tially controlled by using the high-pressure range of the
shunt pressure setting. One of these patients improved to
the condition of shunt-dependent arrested hydrocephalus,
and after one shunt revision so did the other patient.
Among the seven patients who experienced subdural he-
matoma after receiving a shunt with a DPV, the shunt sys-
tem was revised by changing the valve to a higher pres-
sure–range DPV in one patient and to a PPV in four
patients. Three patients underwent a neuroendoscopic
third ventriculostomy following shunt complications. One
935

FIG. 2. Case 1 in Fig. 5. Preoperative cardiac-gated cine-MR
images (TR 20 msec, TE 13 msec) and corresponding graphs.
Upper: Axial view with ROI No. 869 set at the sylvian aqueduct.
Note that no to-and-fro CSF movements can be detected. Lower:
Preoperative sagittal view with ROI No. 9262 set at the basal cis-
tern. Note the rapid and intense to-and-fro movements.
patient in whom shunt malfunction resulted in signifi-
cant ventriculomegaly underwent neuroendoscopic opera-
tion and the ventriculostomy was effective without fur-
ther treatment. The other two patients experienced severe
forms of slit ventricle syndrome affecting their levels of
consciousness. Third ventriculostomies were performed
according to the protocol for neuroendoscopic treatment
of slit ventricle syndrome reported by Baskin, et al.3 How-
ever, in both patients shunt dependency was not improved
and the delicate shunt-regulated CSF flow control afford-
ed by a PPV was needed.
In the nine patients who initially underwent neuroen-
doscopic ventriculostomy, a third ventriculostomy was
performed in eight and aqueductal plasty in one. Six of
nine patients improved without need of a subsequent pro-
cedure within a few weeks to a few months after the pro-
cedure, their conditions becoming arrested or cured hy-
drocephalus, demonstrated clinically and radiologically.
Two patients who experienced significant hemorrhages
(one intraoperative subarachnoid and one postoperative
intracerebral hematoma), both without significant symp-
toms, required placement of a VP shunt because of pro-
gression of their preoperative symptoms. One of these pa-
tients had initially improved, but experienced recurrence
of preoperative symptoms and required implantation of a
VP shunt with a PPV (Table 3).
Long-Term Follow-Up Review
Final outcomes in the study group at follow-up periods
ranging from 6 months to 13 years showed that all treated
patients had radiological evidence of arrested hydroceph-
alus and had improved clinically by various degrees. The
936
S. Oi, et al.
final procedures undertaken were VP shunt placement in
10 patients (in seven cases a PPV was used and in three a
DPV was used) and neuroendoscopic ventriculostomy in
eight patients (in seven cases a third ventriculostomy and
in one case an aqueductal plasty). Types of morbidity in-
cluded: motor weakness due to postoperative subdural he-
matoma in three patients; parkinsonism in two patients
(one patient experienced shunt malfunction and the other
experienced symptoms after neuroendoscopic third ven-
triculostomy for postshunt slit ventriculostomy); initial
improvement with subsequent progressive visual field de-
fect after a neuroendoscopic procedure in one patient; epi-
lepsy in one patient; and depression requiring either psy-
chiatric consultation or medication in six patients (four
after implantation of a VP shunt and two after a neuroen-
doscopic procedure) (Table 4 and Fig. 5). There were no
deaths in this study population.
Discussion
Chronological Changes in Hydrocephalus
Cerebrospinal fluid dynamics change over time19 un-
der disturbed conditions. A large head is not necessari-
ly indicative of hydrocephalic progression, even if it is
accompanied by prominent ventriculomegaly, if CSF for-
mation and absorption are well balanced.13,17 Arrested
hydrocephalus13,41 is the opposite of progressive hydro-
cephalus. However, in our experience, arrested and pro-
gressive hydrocephalus do not remain constant, but some-
times change to other forms of hydrocephalus. Based on
this concept, an individual’s hydrocephalic state may shift
into a different or even the opposite subtype during certain
periods. Arrested hydrocephalus does not always remain
asymptomatic, but can change to a form of progressive
hydrocephalus with active symptoms. The LOVA is a
chronological concept of hydrocephalus. As described
here, LOVA may be summarized as a complex entity with
the following compatible subtypes: 1) onset may be con-
genital in origin but becomes manifest during adulthood;
2) the underlying lesion is aqueductal stenosis; 3) symp-
toms include macrocephaly, increased ICP symptoms, de-
mentia, subnormal IQ, and others; 4) pathophysiological
characteristics include noncommunicating CSF circula-
tion and an ICP dynamics that mainly consists of high
ICP; 5) the chronology is long term and progressive; and
6) the hydrocephalus becomes arrested after shunt place-
ment or ventriculostomy. During the study period we
treated 31 cases of idiopathic hydrocephalus in adults. Of
these, there were 20 cases (64.5%) of LOVA, 13 cases
(41.9%) of hydrocephalic dementia,19 and three cases
(9.7%) of true normal-pressure hydrocephalus (unpub-
lished data).19 Therefore, LOVA was the most common
type of idiopathic adult hydrocephalus in our experience,
although many cases overlapped with respect to subtypes.
The reason for such a high incidence of LOVA in our se-
ries may be the large number of cases of severe LOVA re-
ferred to our institution.
Pathophysiology of Hydrocephalus Secondary to
Aqueductal Stenosis
Aqueductal stenosis causing hydrocephalus in infancy
J. Neurosurg. / Volume 92 / June, 2000
Long-standing overt ventriculomegaly in adults

FIG. 3. Case 2 in Fig. 5. Preoperative and postoperative cine-MR images and 3D CT ventriculograms. Upper Left:
Preoperative T2-weighted MR image. Note the significant turbulent CSF movements in the third ventricle producing a
diverticulum posteriorly and eroding the sella turcica. Upper Right: Preoperative 3D CT ventriculogram. Note the evi-
dence of a noncommunicating sylvian aqueduct and contrast material filling the diverticulum. Lower Left: Postoper-
ative T2-weighted MR image. Note the change in CSF movements. The site of ventriculostomy contains marked CSF
to-and-fro movement. Lower Right: Immediate postoperative 3D CT ventriculogram. Note the contrast material dis-
tributed in the basal cistern and the reduced size of the diverticulum.

is usually congenital in origin. Underlying conditions or
pathogeneses include a hereditary form known as x-linked
recessive4,8,14,16,33,38,42 or neurofibromatosis mutation,11,12,32
periaqueductal tumor,12,32,34,35 abnormal vessel,40 arachnoid
cyst,26 secondary membranous occlusion,20 secondary
functional occlusion found either in progression of com-
municating hydrocephalus27 or with a shunt in place, re-
sulting in various forms of isolated compartment;21,28 and
idiopathy.9 Cases in which there is clear evidence of a con-
genital origin, that is, a neurofibromatosis or x-linked mu-
tation, usually display symptoms or signs of increased
ICP, such as headaches, papilledema, sixth nerve palsy, in-
creased head circumference, and others.11 However, be-
cause of the high compensatory capacity of the brain and
skull during neonatal and infantile periods, progression of
the hydrocephalic state can be compensated for, and the
increased head circumference may diminish at a certain
age. The patients presented here were all adults. During

FIG. 4. Case 2 in Fig. 5. Preoperative and postoperative ICP traces. Left: Note remarkable pressure waves with a
high baseline pressure recorded preoperatively. Right: Postoperatively, baseline pressure returned to the upper limit of
the normal range with significant pulse pressure preserved.

J. Neurosurg. / Volume 92 / June, 2000 937

 S. Oi, et al.

TABLE 3 TABLE 4
Initial therapeutic modalities and outcomes in 20 patients Final treatment modalities and outcomes in 20 patients
with LOVA* with LOVA
Initial Outcome (No. of Patients) Final Treatment (No. of Patients)

Progres- Slit Neuroen-

Arrest- sive Hematoma Ventricle doscopic
No. of ed HC Syn- VP Shunt Implantation Third No
Initial Treatment Patients HC (MF) SDH SAH ICH drome† Ventric- Treat-
DPV PPV ulostomy ment
VP shunt implantation 9 Final Outcome (3 patients) (7 patients) (8 patients) (2 patients)
w/DPV 7 0 2 7 0 0 2
w/PPV 2 1 1 0 0 0 0 hydrocephalus
neuroendoscopy 9 radiological evidence 3 7 8 2
third ventriculostomy 8 5 2 0 1 1 0 of arrest
aqueductal plasty 1 1 0 0 0 0 0 clinical improvement 3 7 8 0
morbidity
* HC = hydrocephalus; ICH = intracerebral hematoma; MF = malfunc- motor weakness 3 0 1 0
tion; SAH = subarachnoid hemorrhage; SDH = subdural hematoma includ- parkinsonism 1 0 1 0
ing subdural effusion. progressive visual loss 0 2 1 0
† In these two patients, slit ventricle syndrome developed more than sev-
epilepsy 1 0 0 0
eral years after the subdural hematoma had subsided.
death 0 0 0 0
residual symptoms
macrocephaly 2 2 6 1
subnormal IQ 2 1 2 1
their early childhoods in the pre–CT scanning era (1940s– visual symptoms 0 3 1 0
1970s), there was no definitive diagnostic procedure depression 1 2 3 0
available except for assessing head-growth curves and
identifying other symptoms or signs. However, as indicat-
ed in the study by Whittle and colleagues,41 patients in lus can be reversed may be based on long-standing slow-
whom the clinical diagnosis is arrested hydrocephalus of- ly progressive high ICP dynamics reaching a threshold.
ten experience decreases in IQs and abnormally high ICP
dynamics later, that is, progressive hydrocephalus. These Pathophysiological Aspects in Treatment
authors suggested a very low incidence of true arrest of In the present series of patients with LOVA, we demon-
hydrocephalus. However, the alterations in CSF dynamics strated the therapeutic problems of regulating CSF flow
that lead to reversal of arrest remain obscure. Johnston, et using a shunt system. Because of long-standing severe
al.,13 described three major possible mechanisms of arrest- ventriculomegaly, the involved brain parenchyma has lost
ed hydrocephalus: 1) reestablishment of the normal CSF its compliance. Also, the craniocerebral disproportion of
pathway; 2) utilization of an alternative pathway; and 3) structures due to macrocephaly presents difficulties. Post-
changes in CSF production. If the progressive course of a operative subdural hematoma occurred in all seven pa-
once-arrested hydrocephalus occurs in cases of LOVA, it tients initially treated by implantation of a DPV-regulated
may be caused by the development of a blockage in the shunt system. As indicated in the present study, LOVA
once-reestablished CSF pathway, a decreased capacity of usually appears as a high- or very high–pressure hydro-
CSF flow in the once-developed alternative pathway, or cephalus when it becomes symptomatic. Şenveli, et al.,32
an increase in CSF production. None of the patients pre- reported six cases of aqueductal stenosis in association
sented here had evidence of any disease or insult to the with von Recklinghausen neurofibromatosis and aqueduc-
brain that may have caused a secondary morphological tal stenosis. The ages of the patients in that study at the
change in the CSF pathway. McComb17 described factors time of diagnosis ranged from 14 to 24 years, and all of
affecting CSF formation and absorption rates. How to de- the patients had extremely high ICPs (270–630 mm H2O).
tect these factors and analyze the alteration in CSF dy- The shunt system initially chosen should be equipped with
namics leading to a reversal of arrested hydrocephalus in a highly resistant valve. The use of a flow-regulated shunt
LOVA remains a subject of future study. system7 or antisiphon device5 may be best. Use of a PPV
Clinical features of aqueductal stenosis in adults in- (Sophy Valve with eight settings)-regulated shunt system
clude a wide variety of symptoms. It has been suggested may be ideal to manage specific problems presented in
that long-standing ventriculomegaly may involve skull- LOVA. Nevertheless, although it may prevent develop-
base structures, resulting in visual (ophthalmoplegia,11,18 ment of a subdural hematoma, other specific postshunting
visual field defect,15 or decreased visual acuity11), hor- complications occasionally occur. Slitlike ventricles usu-
monal (amenorrhea39), and auditory (hearing loss2 or otor- ally develop in young hydrocephalic children after im-
rhea36) symptoms. Hypothalamic or pineal syndromes plantation of a shunt22–24 and should be a rare complication
(hypothalamic hypopituitarism,6 hypothalamic dysfunc- in long-standing hydrocephalus or hydrocephalus that pre-
tion,10 or precocious puberty37) and psychiatric disorders sents during adulthood, along with decreasing brain com-
(schizophrenic psychosis29 or anorexia nervosa1) are ex- pliance.22 However, among 11 cases of LOVA treated by
amples reported in the literature as well as results ob- shunt placement, we experienced slit ventricle syndrome
served in our patients. These symptoms and signs may in two patients. Both patients were referred from other
occur as the initial symptoms of LOVA in adulthood. hospitals because of severe symptoms, such as episodic
Therefore, the mechanism by which arrested hydrocepha- decreased level of consciousness. We applied the ther-

938 J. Neurosurg. / Volume 92 / June, 2000

Long-standing overt ventriculomegaly in adults
FIG. 5. Summary of the therapeutic procedures performed in 18 cases of LOVA. Numerals indicate case numbers.
Two patients received no treatment.
apeutic regimen reported by Baskin, et al.,3 for neuro-
endoscopic third ventriculostomy. However, the patients
needed delicate shunt-controlled CSF flow in spite of the
ventriculostomy achieved. This regimen remains contro-
versial. It is our impression that preoperative expansion of
the slit ventricle achieved by clamping the overfunction-
ing shunt carries risks and postoperative CSF circulation
after third ventriculostomy may often be of limited use
in patients with long-standing noncommunicating hydro-
cephalus.
Recently, our rule in treating patients with LOVA has
been to select neuroendoscopic surgery as the initial treat-
ment. Nine patients were initially treated with neuroen-
doscopic surgery: eight with third ventriculostomy and
one with aqueductal plasty. Six of these patients had satis-
factory results, reaching the goal of clinically and radio-
logically documented arrested hydrocephalus. This can
be technically improved, because the CSF pathway may
be affected by clot due to hemorrhage. Especially in se-
vere cases of LOVA, the floor of the third ventricle may
be widely expanded and replaced by an arachnoid-like
membrane, which is somewhat elastic and tough, floating
with a to-and-fro movement in the widely eroded skull
base. Avoidance of major vessel injury30,31 is the most seri-
ous subject in neuroendoscopic surgery in LOVA as well.
The goal of the neuroendoscopic technique is to keep
steady and handle the rapidly moving target.20
Conclusions
Long-standing noncommunicating hydrocephalus re-
J. Neurosurg. / Volume 92 / June, 2000
veals unique pathophysiological and chronological chang-
es in the brain and skull. In our experience of 20 cases of
LOVA, in all patients there was evidence of either early
childhood onset or long-standing progression of hydro-
cephalus into adulthood. It remains enigmatic why the hy-
drocephalic state becomes active and progressive in adult-
hood. The pathophysiological characteristics of LOVA
made therapeutic outcomes complicated. Neuroendoscop-
ic surgery is a promising procedure to avoid the problems
of shunt dependency or overdrainage syndrome; however,
the success rate of arrest after ventriculostomy may de-
pend on the preserved function of the CSF dynamics in the
subarachnoid space in the prolonged condition of long-
standing noncommunicating hydrocephalus.
References
1. Artmann H, Grau H, Adelmann M, et al: Reversible and non-
reversible enlargement of cerebrospinal fluid spaces in anorex-
ia nervosa. Neuroradiology 27:304–312, 1985
2. Barlas O, Gökay H, Turantan I, et al: Adult aqueductal stenosis
presenting with fluctuating hearing loss and vertigo. Report of
two cases. J Neurosurg 59:703–705, 1983
3. Baskin JJ, Manwaring KH, Rekate HL: Ventricular shunt re-
moval: the ultimate treatment of the slit ventricle syndrome. J
Neurosurg 88:478–484, 1998
4. Bickers DS, Adams RD: Hereditary stenosis of the aqueduct
of sylvius as a cause of congenital hydrocephalus. Brain 72:
246–262, 1949
5. da Silva MC, Drake JM: Effect of subcutaneous implantation of
anti-siphon devices on CSF shunt function. Pediatr Neurosurg
16:197–202, 1990/1991
6. Dempsey RJ, Chandler WF, Sauder SE: Surgically reversible
939

hydrothalamic hypopituitarism due to aqueductal stenosis. Case
report. J Neurosurg 58:932–934, 1983
7. Duplessis E, Deco P, Barat JL, et al: [The surgical treatment of
chronic hydrocephalus in adults with a flow-regulated shunt:
review of 46 patients.] Neurochirurgie 37:40–43, 1991 (Fr)
8. Halliday J, Chow CW, Wallace D, et al: X linked hydrocepha-
lus: a survey of a 20 year period in Victoria, Australia. J Med
Genet 23:23–31, 1986
9. Hanigan WC, Morgan A, Shaaban A, et al: Surgical treatment
and long-term neurodevelopmental outcome for infants with
idiopathic aqueductal stenosis. Childs Nerv Syst 7:386–390,
1991
10. Harper CG, Thomas W, ApSimon HT: Hydrocephalus and
hypothalamic dysfunction in a young woman. Med J Aust 2:
394–398, 1983
11. Horwich A, Riccardi VM, Francke U: Aqueductal stenosis lead-
ing to hydrocephalus—an unusual manifestation of neurofibro-
matosis. Am J Med Genet 14:577–581, 1983
12. Ito T, Norman D, Newton TH: MR imaging of aqueductal ste-
nosis due to tectal region tumors. CT Kenkyu 11:519–524,
1989
13. Johnston IH, Howman-Giles R, Whittle IR: The arrest of treat-
ed hydrocephalus in children. A radionuclide study. J Neu-
rosurg 61:752–756, 1984
14. Kelley RI, Mennuti MT, Hickey WF, et al: X-linked recessive
aqueductal stenosis without macrocephaly. Clin Genet 33:
390–394, 1988
15. Kojima N, Kuwamura K, Tamaki N, et al: Reversible congru-
ous homonymous hemianopia as a symptom of shunt malfunc-
tion. Surg Neurol 22:253–256, 1984
16. Kuzniecky RI, Walters GV, Walters L, et al: X-linked hydro-
cephalus. Can J Neurol Sci 13:344–346, 1986
17. McComb JG: Recent research into the nature of cerebrospinal
fluid formation and absorption. J Neurosurg 59:369–383,
1983
18. Nishizaki T, Tamaki N, Nishida Y, et al: Bilateral internuclear
ophthalmoplegia due to hydrocephalus: a case report. Neuro-
surgery 17:822–825, 1985
19. Oi S: Hydrocephalus chronology in adults: confused state of the
terminology. How should “normal-pressure hydrocephalus” be
defined? Crit Rev Neurosurg 8:346–356, 1998
20. Oi S, Hidaka M, Honda Y, et al: Neuroendoscopic surgery for
specific forms of hydrocephalus. Childs Nerv Syst 15:56–68,
1999
21. Oi S, Kudo H, Yamada H, et al: Hydromyelic hydrocephalus.
Correlation of hydromyelia with various stages of hydroceph-
alus in postshunt isolated compartments. J Neurosurg 74:
371–379, 1991
22. Oi S, Matsumoto S: Hydrocephalus in premature infants. Char-
acteristics and therapeutic problems. Childs Nerv Syst 5:
76–82, 1989
23. Oi S, Matsumoto S: Infantile hydrocephalus and the slit ventri-
cle syndrome in early infancy. Childs Nerv Syst 3:145–150,
1987
24. Oi S, Matsumoto S: Slit ventricles as a cause of isolated ventri-
cles after shunting. Childs Nerv Syst 1:189–193, 1985
25. Oi S, Sato O, Matsumoto S: Neurological and medico-social
problems of spina bifida patients in adolescence and adulthood.
Childs Nerv Syst 12:181–187, 1996
940
S. Oi, et al.
26. Pascual-Castroviejo I, Roche MC, Martínez Bermejo A, et al:
Primary intracranial arachnoidal cysts: a study of 67 childhood
cases. Childs Nerv Syst 7:257–263, 1991
27. Raimondi AJ, Clark SJ, McLone DG: Pathogenesis of aque-
ductal occlusion in congenital murine hydrocephalus. J Neuro-
surg 45:66–77, 1976
28. Raimondi AJ, Samuelson G, Yarzagaray L, et al: Atresia of the
foramina Luschka and Magendie: the Dandy-Walker cyst. J
Neurosurg 31:202–216, 1969
29. Roberts JKA, Trimble MR, Robertson M: Schizophrenic psy-
chosis associated with aqueduct atenosis in adults. J Neuro-
surg Psychiatry 46:892–898, 1983
30. Schmidt RH: Use of a microvascular Doppler probe to avoid
basilar artery injury during endoscopic third ventriculostomy.
Technical note. J Neurosurg 90:156–159, 1999
31. Schroeder HWS, Warzok RW, Assaf JA, et al: Fetal subarach-
noid hemorrhage after endoscopic third ventriculostomy. Case
report. J Neurosurg 90:153–155, 1999
32. Şenveli E, Altinörs N, Kars Z, et al: Association of von Reck-
linghausen’s neurofibromatosis and aqueduct stenosis. Neuro-
surgery 24:99–101, 1989
33. Shannon MW, Nadlar HL: X-linked hydrocephalus. J Med Ge-
net 5:326–328, 1968
34. Sola J, Arcas I, Martínez-Lage JF, et al: Astrocytoma of the ce-
rebral aqueduct. Childs Nerv Syst 3:294–296, 1987
35. Steinbok P, Boyd MC: Periaqueductal tumor as a cause of late-
onset aqueductal stenosis. Childs Nerv Syst 3:170–174, 1987
36. Takase M, Sanada T, Watanabe O, et al: [Spontaneous cere-
brospinal fluid otorrhea in association with an aqueductal steno-
sis. Case report.] Shinkei Geka 27:550–553, 1987 (Jpn)
37. Tomono Y, Maki Y, Ito M, et al: Precocious puberty due to
postmeningitic hydrocephalus. Brain Dev 5:414–417, 1983
38. Toriello HV, Higgins JV: X-linked midline defects. Am J Med
Genet 21:143–146, 1985
39. Turpin G, Heshmati HM, Darbois R, et al: [Stenosis of aqueduct
of sylvius: a rare cause of amenorrhea—successful treatment
by atrioventricular shunt in three cases.] Sem Hop Paris 59:
2369–2372, 1983 (Fr)
40. Watanabe A, Ishii R, Kamada M, et al: Obstructive hydroceph-
alus caused by an abnormal vein in the aqueduct. Case report.
J Neurosurg 75:960–962, 1991
41. Whittle IR, Johnston IH, Besser M: Intracranial pressure chang-
es in arrested hydrocephalus. J Neurosurg 62:77–82, 1985
42. Willems PJ, Brouwer OD, Dijkstra I, et al: X-linked hydroceph-
alus. Am J Med Genet 27:921–928, 1987
Manuscript received June 23, 1999.
Accepted in final form February 14, 2000.
This study was supported by Grant-in-Aid for Scientific Research
No. 08671618 from the Ministry of Education, Science, Sports and
Culture of Japan, 1996–1998; and a grant-in-aid for the 1998 Re-
search Committee of “Intractable Hydrocephalus” to Dr. Oi from
the Ministry of Health and Welfare of Japan.
Address reprint requests to: Shizuo Oi, M.D., Department of
Neurosurgery, Tokai University, School of Medicine, Bohseidai,
Isehara-city, Kanagawa 259–1193, Japan.
J. Neurosurg. / Volume 92 / June, 2000