Fungsional Outcome Cong Hydrocephalus

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Original Article
Evaluation of functional outcomes in
congenital hydrocephalus
N. K. Venkataramana, C. R. Mukundan1
Department of Neurosurgery, Advanced Neuro Science Institute, BGS Global Hospital, 1Department of Neuropsychology,
NIMHANS, Bangalore, India
Address for Correspondence: Dr. N. K. Venkataramana, Department of Neurosurgery, Advanced Neuroscience Institute, BGS Global Hospital,
BGS Health and Education City, No. 67, Uttarahalli Road, Kengeri, Bangalore 560 060, India. E-mail: advanced.neuroscience@gmail.com
ABSTRACT
Aim: The long term outcomes of congenital hydrocephalus are still not clearly known despite it being a common
clinical condition. Several clinical, radiological factors were correlated to predict the functional outcomes. This
study aimed to correlate the clinical, radiological parameters with the regional functional outcomes of the brain.
Materials and Methods: Children with congenital hydrocephalus were divided into Group A with hydrocephalus
alone and Group B hydrocephalus with spina bifida. Ventriculoperitoneal shunt surgery was performed by the
same surgeon. CT scans and neuropsychological assessments were performed before and serially after the
shunt. The clinical and the radiological findings were correlated with the developmental levels during the followup. Results: There were 25 children in Group A and 15 children in Group B; 72% in Group A and 93% in Group B
were less than 6 months of age at the time of treatment. Forty percent in Group A and 92% in Group B had the
signs of hydrocephalus at admission. Cerebrospinal fluid (CSF) diversion results in the reduction in ventricular
dilatation and corresponding increase in the cortical mantle thickness. The ventricular size and the cortical mantle
thickness were measured serially and correlated with the development in the neuropsychological function. In this
study, 80% in Group B reached near normal development in comparison to 33% in Group A. We have noticed a
significant correlation in the increase in the regional cortical mantle thickness with corresponding improvement
in the functional development. This clearly ratifies the improvement in the frontal and parietal areas having
their distinctive effect on the functional development of the child. Conclusion: Early CSF diversion and timely
intervention seems to benefit functional recovery. It is interesting to note that reconstitution of cortical mantle
in different areas of the brain showing corresponding improvement in their respective areas. Large ventricles
(head circumference more than 50 cm) recurrent subdural collections and repeated shunt obstructions have a
bad influence on the long-term outcome. Unlike the previous belief the children with myelomeningocele can
have equal benefit in terms of neuropsychological development after the shunt surgery.
Key words: Congenital hydrocephalus, functional outcome, myelomeningocele, neuropsychological development
Introduction
Congenital hydrocephalus is one of the common conditions
in paediatric neurosurgical practice characterized by abnormal
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DOI:
10.4103/1817-1745.84399
4 / Journal of Pediatric Neurosciences / Volume 6 / Jan-Jun / 2011
dilatation of cerebral ventricular system. The incidence of

congenital hydrocephalus varies from 0.92.9/1000 births.
Increased ventricular volume due to accumulation of
cerebrospinal fluid (CSF) results in increased intraventricular
pressure. This causes corresponding compression on the
surrounding cerebral parenchyma, leading to structural
changes in white matter, cortex, and seepage of CSF into the
periventricular spaces through ependymal tears.
Congenital hydrocephalus can occur in association with a
number of developmental anomalies of brain and spinal cord.
Hydrocephalus with an elevated intracranial pressure can lead
to deterioration in motor, intellectual, cognitive, and other
neuropsychological functions.
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Venkataramana and Mukundan: Evaluation of functional outcomes in congenital hydrocephalus
Therapy for hydrocephalus includes diversion of CSF or

endoscopic ventriculostomy. Although shunts have greater
propensity to reduce ventricular volumes on imaging, its
efficacy in reversing the pathophysiological changes and its
effect on functional and intellectual outcomes are variable.
Similarly the precise role of anatomical reconstitution
of cortical mantle following therapy in restoring normal
functions needs to be studied.
Materials and Methods

Many studies have been published concerning the intelligence
of hydrocephalic children.[1-15] They include both treated
and untreated groups. Most studies have been purely
descriptive and only a few have compared the treated and
untreated groups. The determinants of intelligence level
in hydrocephalic children are not fully established and the
pattern of intelligence is also imperfectly understood.
It is very difficult to evaluate the literature regarding
intellectual function in children with treated group of
congenital hydrocephalus. The diagnostic criteria used were
different in different studies. It is also difficult to compare
different studies due to lack of adequate information, use
of different methods of intelligence quotient assessment,
different methods of data presentation, lack of proper
definition of Normal, or the basis of sample selection.
This study includes children with congenital hydrocephalus
who were surgically treated by ventriculoperitoneal shunt.
Children with possible infections and multiloculated ventricles
were excluded. Age, sex, economic status, intrapartum events,
and associated congenital anomalies were not considered in
the selection of sample.
Forty such children were divided into Group Acongenital
hydrocephalus (N = 25) alone and Group Bhydrocephalus
with myelomeingocoele (N = 15).
All children underwent elective ventriculoperitoneal shunts
(Indian) using medium pressure valve. In Group B, shunt
was performed first in 14 children, which was followed-up
by the repair of myelomeningocoele. Clinical, radiological,
and neuropsychological assessment was done before surgery,
at discharge, and during follow-up at 36 month intervals.
Preoperative assessments were taken as baseline.
Computed tomography (CT) scans repeated at intervals, ranging
from 3 to 6 months to ensure that the shunt was functioning.
Additional scans were performed in case of shunt malfunction
and they were revised appropriately. Totally 9 children required
revision of shunts5 in Group A and 4 in Group B.
Linear measurements were used to assess the degree of
ventricular dilatation and cortical mantle thickness. A scale in
which 1 cm was divided into 20 equal parts was used to achieve
an accuracy of 0.5 mm. The measurements include bifrontal
diameter, absolute III ventricular diameter, the minimum

width of the body of the lateral ventricle (cella media), and the
maximum transverse inner diameter of the skull. From these
measurements, the following indices (68) were calculated.
1. The ratio between cella media and maximum transverse
inner diameter, of skull,
2.
Maximum inner transverse
Cella media
diameter of skull
100
index =
Maximum width of cella media
3.
Anterior horn
index =
Maximum transverse inner

diameter of skull
Maximum distance between
tips of frontal horn
100
Cortical measurements were taken in frontal and parietal

areas on both right and left sides. Frontal cortex was measured
from tip of the corresponding frontal horn to the inner table
in the sagittal plane and parietal cortex as the maximum
diameter from ventricle to inner table in the coronal plane.
Temporal and occipital cortices were not measured due to the
variations of the size and shape of the temporal and occipital
horns. The measured readings were multiplied by 3.5, the
magnification factor for the design of the scanner used.
Postoperatively, corresponding cuts were used in the
assessment of reduction in ventricular size and to measure the
cortical thickness. In 10 patients ventricles were collapsed and
postoperative measurements could not be done. These were
labeled as Slit ventricles. Apart from this, postoperative
subdural collections, and other complications were taken
into cognizance in the final analysis.
Psychological assessment
Psychological development was assessed either by Gesell
developmental schedules [16] or Bayley scales of infant
development. [17,18] The Gesell developmental schedules
were developed based on a series of longitudinal studies
of the normal course of behavioral development in infants
and preschool children. The schedules cover major areas of
behavioral development, namely, motor, adaptive, language,
personal, and social development. It involves a standardized
procedure of observing and evaluating the various elements of
behavior in the child. It includes tests, as well as observations
of the child. These schedules are applicable for children from
the age of 4 months to 6 years. Although the tests greatly
depend on observational findings, a high examiner reliability
of over 0.95 was reported.[7]
Bayley scales of infant development
This is a developmental schedule exceptionally suited for
infants. The scale covers mental development up to 2 years
and 6 months. As in the case of the Gesell developmental
schedules, the Bayley scales are the outcome of continuous
and longitudinal studies by Bayley and other co-workers.
2011 / Jan-Jun / Volume 6 / Journal of Pediatric Neurosciences / 5
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It has also incorporated the findings of Berkeley growth

study. The Bayley scales provide three complimentary tools
for assessing the developmental status of children between
the ages of 2 months and 2 years. The mental scale
assesses functions, such as perceptions memory, learning,
problem solving vocalization, and visual communication.
It may be kept in mind that only the elementary aspects of
these functions could be observed and assessed during the
very early ages. Another component of the schedule is the
motor scale, which measures the motor abilities, such as
sitting, standing, walking, and so on. The scales provide the
normal age range for the occurrence of each event during
the developmental process. The third component of the
test helps to measure essentially the emotional, personality
development, and social behavior of the child. The Bayley
scales are considered to be the most reliable and outstanding
tool for assessing developmental processes in the infants.
The normative data of these scales were established based
on observation of 1262 children. During standardization
procedure, the test has yielded reliability co-efficient of
0.810.93 in separate age groups.
The choice of the test depends on the age of the patient
investigated initially. The child was observed for the period
ranging from 1 to 2 h and later the parents were interviewed
in detail. The developmental age of the child was derived from
either of the two tests used, on eight of the tests the child was
tested and observed to determine if the child would pass an
individual item related to a specific age level. The maximum
age level that could be reached on the developmental
schedules in each child was considered his mental age.
The tests were administered both preoperatively and
postoperatively. However, considerable caution was exercised
in the preoperative assessment, in avoiding errors caused
by the clinical condition of the child due to hydrocephalus.
Since the patients were infants or children in the very early
ages, emphasis was laid on ascertaining their developmental
levels rather than intelligent quotients. Although the
mental age could be easily connected into an intellectual
quotient (IQ) measure, it was considered more meaningful
to use the criterion of mental age rather than that of IQ
as the nature of the disease entity can produce a cluster of
different functions. Although deriving an IQ will average
out these discrepancies, the actual/developmental pattern
of the child would be lost.
An attempt was made to find the association between
the developmental age of the child and (different clinical
findings such as) clinical and radiological parameters, both
preoperatively and postoperatively. From these comparisons,
an attempt was made to determine the developmental rate in
the child after the shunt. Prognostic factors, the predictability
of the future development and intellectual outcome based on
the initial assessment were attempted for each child.
Results
Forty children who underwent ventriculoperitoneal shunts for
congenital hydrocephalus were studied. They were divided
into two groups. Group A with hydrocephalus alone and
Group B with an associated myelomeningocele.
The results of both the groups with respect to each investigation
were analyzed. The postoperative neurological improvement
was correlated with radiological and psychological findings.
Age and sex distribution is shown in Table 1a and 1b.
Progressive increase in head size was the most common
symptom in both the groups. Four children (16%) of Group
A had gross delay in milestones at the time of admission.
In Group A, 6 patients (24%) had symptoms of increased
intracranial tension and 3 (12%) had preoperative seizures.
In two children, big head was noticed from birth [Table 2].
The onset of clinical symptoms of hydrocephalus was quite
early in both the groups. Progressive increase in head
circumference was noticed from birth in 16% of cases in Group
A and 40% of cases in Group B. They were presented to our
service with established disease in 92% of Group A and 93.2%
of Group B. The mean ages of onset of symptoms were 2.85
months for Group A and 0.27 months for Group B [Table 3].
Three children in Group A were drowsy at the time of
admission and another 3 patients had impaired visual
development. Most common cranial nerve deficit was
sixth nerve palsy and lower motor neuron type of facial
paresis, was noticed in 2 children, one in each group.
Nystagmus and cerebellar signs were seen only in 2 children of
Group A [Table 4].
In Group B the location of myelomeningocele sac was
lumbar in 6 (39.6), thoraco lumbar in 5 (33%), lumbosacral
in 2, and thoracic and sacral in one each [Table 5]. They
Table 1a: Distribution of sample

No. of cases
Male
Female
Group A
Group B
25
20
05
15
06
09
Table 1b: Age distribution

Age group in months
16
712
1318
1924
2530
3136
36
Mean age
SD
Group A
Group B
12
06
01
01
02
03
15.04
16.94
14
01
4.8
3.52
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had corresponding neurological deficits below the level of

the myelomeningocele. Thirteen children had complete
weakness of lower limbs and paraparesis in 8. Twelve had
urinary sphincter disturbance. Head circumference of these
children varies from 39 to 62 cm in Group A and 4153 cm
in Group B with a mean value of 49.8 and 45.97, respectively
[Table 6].
The cell media index and anterior horn index and absolute 3rd
ventricular diameter were calculated in all these patients both
preoperatively and postoperatively. There was a significant
improvement of these indices toward normalcy in all the
children (P < 0.01) after the shunt [Tables 7 and 8].
In 10 patients (6 in Group A and 4 in Group B) the
ventricles became SLIT and hence postoperative ventricular
measurements could not be measured accurately.
The cortical mantle thickness varied with the degree of

ventricular dilatation. In 8 patients the cortex was severely
thinned out so that no measurement of cortical mantle is
possible. The mean cortical mantle thickness in the frontal
and parietal region is mentioned in Table 9.
Mean duration of 5.25 months in Group A and 5.99
months in Group B. The increase in thickness of cortical
mantle observed in the last scan was statistically significant,
P < 0.01 [Table 10]. In 19 out of 40 cases the cortical mantle
thickness was greater than 3 cm. There seems to be regional
variation in cortical mantle reconstitution after surgery.
Among those with almost invisible cortical mantle,
preoperatively only one had shown improvement in
cortical mantle and the rest did not show improvement in
thickness.
Table 2: Clinical features

Presenting symptoms
Table 3: Age of onset of symptoms

No. of cases
Progressive increase in head size

Retardation of mile stones
Symptoms of increased intracranial pressure
Seizures
24
04
06
03
13
01
0
Table 4: Clinical features

Clinical features
Age in months
Since birth
13
46
79
1012
Mean
SD
24
03
03
02
01
07
01
14
01
01
03
06
04
02
01
0.27
0.44
05
08
08
04
02
01
06
05
01
05
Table 5: Group B
Site of myelomeningocele
Table 7: Ventricular measurement after shunt

Cella media index
Ant. horn index
Absolute
III ventricular dia.
Preop
Postop
Preop
Postop
Preop
Postop
218.73
90.80
332.85
214.44
175.13
53.57
233.88
80.39
4.96
1.62
5.62
2.89
187.67
38.32
265.23
130.85
222.44
34.56
265.8
60.22
4.37
2.77
3.18
2.76
No. of cases
Thoracic
Thoracolumbar
Lumbar
Lumbosacral
Sacral
1
5
6
2
1
Table 6: Head circumference

Head circumference (cm)
Range
Mean
SD
Group A
Group B
3962
49.8
4.57
4153
45.97
4.19
Table 8: Postoperative ventricular indices on followup

Group
Group A
Mean
S.D.
Group B
Mean
S.D.
04
14
05
02
2.85
4.053
No. of patients
Large head
Visual defects
Altered consciousness
Abducens palsy
Facial palsy
Setting sun sign
Nystagmus
Motor deficits
Paraplegia
Paraparesis
Sensory deficits
Lower limbs
Sacral
Incontinence
Dribbling
Stress
Overflow
Cerebellar signs
Talipes equino varus
Group
No. of patients
A
Mean
SD
T value
B
Mean
SD
T value
Cella media index
Ant. horn index
136.15
97.05
3.385
**
104.75
97.67
5.254
**
141.36
114.90
4.60
**
86.22
96.01
3.359
**
**P <0.01
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Table 9: Measurements of cortical mantle

Group
Frontal (mm)
Right
Group A
Mean
SD
Group B
Mean
SD
Parietal (mm)
Left
Right
Left
Preop
Postop
Preop
Postop
Preop
Postop
Preop
Postop
6.71
9.27
19.73
11.84
6.51
8.30
18.51
10.21
15.29
10.86
28.99
12.04
14.71
11.13
28.18
12.72
18.78
7.16
25.25
8.51
17.97
7.10
25.88
9.03
11.4
4.30
20.29
10.25
11.75
4.72
23.75
10.29
Psychological assessment
Among the 40 children, Gesell developmental schedules could
be successfully administered in 36 children. The following
relationships were determined between demographic, clinical,
neuroradiological, and psychological development in each
group.
Association between pre- and postoperative interval and
change in the functional levels of motor, language, social,
and adaptive areas.
Correlation between the differences between pre- and
postoperative cella media index, anterior horn index, and
absolute 3rd ventricular diameter and the ratio of change
in motor, language, social, and adaptive levels divided by
respective chronological age [Table 10].
Correlation between changes in the thickness of the cortical
mantle in the right, left, frontal, and parietal areas the ratio of
changes in motor, language, social and adaptive levels divided
by the respective chronological ages.
Preoperative assessment
Preoperatively only 4 children in Group B and 2 children in
Group A had normal age-related psychological development.
The rest were below the normal levels in their psychological
development for their chronological age. The difference
between mean chronological age and the mean mental age
becomes very obvious in children presenting beyond 9 months
of age to the hospital. It may be suggested, if the duration of
hydrocephalus is longer preoperatively, the lower the mean
mental age attained by the child.
The mean mental age preoperatively was 5.81 months in
Group A and 3.36 months in Group B. [Table 11]
Postoperatively there was a consistent improvement in
psychological development. This improvement was assessed
in terms of development in motor, language, adaptive, and
social function in both groups, respectively.
In general near normal psychological development was seen
in 8 cases (33.3%) of Group A and in a surprisingly large
number of 12 (80%) in Group B [Table 12].
Table 10: Improvement in the cortical mantle

(postshunt)
Group
A
Mean
SD
T value
B
Mean
SD
T value
**P <0.01
Frontal (mm)
Parietal (mm)
Right
Left
Right
Left
12.22
10.53
5.683**
13.52
9.72
6.812**
13.28
12.38
4.90**
13.93
15.21
4.484**
7.13
7.12
3.75**
7.91
8.89
3.327**
18.96
9.80
3.421**
12.23
9.65
4.738**
Table 11: Preoperative psychological assessment

Group A
N=22
Group B
N=13
Mean age
(months)
SD
Mean mental age
(months)
SD
15.27
Mean age
5.53
17.85
5.806
SD
Mean mental age
3.93
3.362
8.036
SD
2.036
Minimal improvement subgroup analysis of 9 (6 in Group A

and only 3 in Group B (22.5%) did not reveal any definite
accountable factor. However, the common findings were
the higher age group, symptoms of raised ICT, gross delay in
milestones at presentation. Their mean head circumference
was 48.72 cm with very thin cortical mantle [Table 13].
Correlative analysis between the psychological development
and clinical and radiological parameters revealed that the
percentage of improvement in each psychological function in
relation to the chronological age was highly related to the age
at which therapeutic intervention was made. The percentage
of improvement of mental age was significantly greater when
intervention was made at age level of less than 6 months. The
improvement was also found to be rapid, when the surgical
intervention was done at an earlier age.
Postoperative improvement in cella media index was seen with
consistent improvement in motor development in both the
groups. In Group B it could be correlated to improvement in
language and adaptive functions [Table 14].
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Table 12: Postoperative psychological development

Degree of improvement
Near normal
Moderate improvement
Very minimal improvement
B No. of cases
N=22
N=15
Index
8
10
6
33.3
41.6
25
12
3
80
20
Cella media index
Table 13: Analysis of minimal improvement subgroup

N=9
Clinical feature
Mean age (months)
Mean head circumference (cm)
Symptoms from birth
Symptoms of raised ICT
Delay in milestones
Repeated shunt revisions
Postop subdural hematoma
Postop ventricular loculation
Extremely thin cortex
Table 14: Correlative values of ventricular indices

with psychological development
A No. of cases
Group A (N=6) Group B (N=3)

2425
6.66
49.33
47.5
4
2
3
3
1
1
1
2
-2
-4
--
Psychological development
Ant. horn index

Absolute III
Ventricular
diameter
Postoperative improvement in absolute 3 rd ventricular

diameter as suggested by Tromp et al, [11] has significant
correlative value with improvement in language, adaptive
and social development in Group B. Unlike in Group B, 3rd
ventricular diameter correlated only with improvement in
social functions in Group A.
Developmental pattern in group A
Improvement in social and adaptive function development
was highly correlated with increase in right frontal cortical
mantle. Similarly, language development was associated with
increase in left frontal cortical mantle thickness. Both right
and left frontal cortical mantle thickness were correlated with
improvement in motor development.
Increase in right parietal cortical mantle has a high correlation
value with social development, whereas left parietal cortex
correlates with motor and language development, but in a
lesser degree compared to frontal cortex.
Developmental pattern in group B
Increase in right frontal and right parietal cortical mantle
thickness has no correlation with any of the developmental
functions except a minimal correlation of increase in right
frontal cortex and improvement in adaptive function. On the
other hand, improvement in motor and adaptive development
were correlated with increase in left frontal and left parietal
cortical mantle thickness.
No correlation has been found between improvement in language
function and increase in cortical mantle in Group B [Table 15].
Motor
Languages
Adaptive
Social
A
B
A
B
A
B
0.047
0.0497
0.139
0.212
0.096
0.036
0.216
0.335
0.182
0.195
0.143
0.722
0.04
0.496
0.211
0.352
0.201
0.796
0.097
0.20
0.512
0.20
0.4522
0.590
Table 15: Correlative values of cortical mantle

thickness with psychological developments
Psychological development and cortical mantle thickness
Frontal
Right
Left
On the contrary, improvement in anterior horn index has

significant correlation with improvement in adaptive function
only in Group A.
Group
Parietal
Right
Left
Group
Motor
Language
Adaptive
Social
A
B
A
B
0.483
0.025
0.454
0.469
0.277
0.179
0.403
0.180
0.451
0.320
0.019
0.394
0.468
0.221
0.295
0.128
A
B
A
B
0.257
0.215
0.355
0.413
0.180
0.192
0.352
0.247
0.261
0.249
0.317
0.396
0.422
0.095
0.337
0.048
Discussion
CSF diversion in hydrocephalic children reduces the
ventricular dilatation. Though improvement in neurological
and intellectual functioning is often seen, there is a wide
variability in their outcome. A number of reports are
available in the literature correlating the ultimate outcome of
hydrocephalic children with various clinical and radiological
parameters.
Thomson et al,[19] reported an inverse correlation of Ventricle
Brain Ratio (VBR) with Bayley Mental scale showing a
correlation between brain mass and IQ in older children with
uncomplicated hydrocephalus in their series.
Prigatano et al,[9] reported average academic performance
during follow-up in children with uncomplicated hydrocephalus
despite normal-sized ventricles and functioning shunt.
The results of these reports are not comparable in view of the
variability of parameters adopted.
In this study, we have attempted to correlate the clinical
radiological findings with the outcome in surgically treated
congenital hydrocephalus. Eighteen (72%) children in
congenital hydrocephalus group and 93% of children in
myelomeningocele group were of less than 6 months of age.
More number of children in the second group were seen at
a younger age initially. The early detection of hydrocephalus
in this group could be due to the follow-up of these children
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for myelomeningocele. In contrast, the children in Group A

were brought when the symptoms were obvious. In our study
a large head was noticed at birth, along with a progressive
increase in head size in 16% of cases in Group A and 40%
in Group B; 92% of them had symptoms of hydrocephalus
within 6 months after birth.
Asymmetrical dilatation of lateral ventricles was detected on
radiology in 13 children. Similar observations were made by
Dennis et al.[2] The asymmetry in ventricles in our study was
noticed between the two lateral ventricles and between the
various parts of the same lateral ventricle.
Predominant occipital horn dilatation was noticed in 60%
with myelomeningocele. Dennis et al,[10] have hypothesized
that the nuclear masses in the frontal region offer resistance
to the dilating frontal horns and absence of such resistance
allows occipital horns to dilate more. In two of our cases,
there was only frontal horn dilatation in spite of the presence
of normal occipital horns. The exact mechanisms by which
this could happen are not clear.
The developmental anomalies of various areas of cerebral
hemispheres could result in reduced quantum of cerebral
parenchyma in a particular region of cerebral hemisphere.
When the intraventricular pressure builds up these areas of
decreased resistance, as compared to that offered by normal
cerebral parenchyma, dilate more. This could explain the
regional and hemispheric asymmetries in dilated ventricles.
The exact significance of this asymmetric dilatation in the
ultimate neuropsychological outcome is yet to be established.
CSF diversion leads to a reduction in ventricular dilatation
and a corresponding increase in cortical mantle thickness.
Rubin et al[14-16] reported that the reconstitution of cortical
mantle results because of a diminution of white matter edema
and due to reactive astrocytosis. He postulated that the
clinical improvement results from functional improvement
of the remaining neuronal elements rather than replacement
of lost elements and the neurological recovery is reversible
only up to a stage. In this study, the reconstitution of cortical
mantle following shunt surgery occurred in both subgroups
to a statistically significant extent (P value less than 0.01).
Many studies have been published concerning the intelligence
of the hydrocephalic children. These studies have compared
the results of treated and untreated hydrocephalus. Most of
the reports have been poorly descriptive and not comparable
because of:
1. the lack of information regarding the shunt and the
child shunted;
2. the use of different instruments to measure intelligence
quotient;
3. differing methods of data presentation; and
4. inclusion of varying causes.[10]
Preoperative neuropsychological assessment using Gesell
development schedules revealed only 6 children among our
patients having age appropriate psychological development.

The gap between the mean chronological age and the mean
mental age widens in patient population after the age of 9
months. Probably shunt surgery performed early in course of
the disease before 9 months may minimize the damage due
to hydrocephalus. Raimondi et al[10] have also highlighted
the importance of early surgery in hydrocephalus. The
recovery in these infants could be due to reversible stage of
disease, recovery of partially damaged neurons, and neuronal
plasticity.
In this series, all the patients had significant ventricular
dilatation. However, ventricular sizes were significantly larger
in Group A children as compared to infants in Group B.
This difference in degree of ventricular dilatation could be
explained by earlier detection of hydrocephalus in Group B.
Nulsen and Rekate[21] have reported failure in the restitution
of cortical mantle to 2 cm in thickness (or more), when infants
were operated after 6 months of age.
The wide variation in cognitive outcome for treated
hydrocephalus children has stimulated numerous studies.
Only a few of these reports had included infants in their
study group - presumably on the assumption that cognitive
and neurologicalpsychological assessment techniques for
infants do not have predictive validity. However, it has now
been shown that there is adequate correlation between infant
mental quotient and the IQ, recorded later in childhood.[19]
This finding suggests that cognitive assessment of infants
with hydrocephalus may be useful in monitoring the effects
of treatment and predicting the outcome.
Preoperatively only 4 children in Group B, and 2 children in
Group A had normal age-related psychological development.
Postoperatively a near normal psychological development
was seen in 33.3% cases in Group A and in 80% cases in
Group B. Analysis of cases who did not reach normalcy in
neuropsychological development did not reveal any clinical/
radiological factors responsible. However, the mean age of
patients who did not improve or minimally improved was
significantly higher as compared to the recovery group.
Correlative analysis between the psychological development
and clinical and radiological parameters revealed that the
percentage of improvement in each psychological function was
related to age at which therapeutic intervention was made.
The percentage of improvement in mental age was significantly
greater when intervention was made before 6 months of age.
In the present study, 80% of children with myelomeningocele
and hydrocephalus had reached near normal psychological
development as compared to only 33% of children in the
hydrocephalus group. This finding is encouraging since
most believed that the psychological development in
myelomeningocele children should be restrictive in view of
physical disability and associated anomalies of brain. The
present study proves that given the benefit of early detection
and therapy, majority of these children can reach near normal
mental development. Raimondi et al[10] also reported near
thisjournal
normal cognitive functioning in infants with myelodysplasia

and hydrocephalus. Although our results are in agreement
with this observation, a follow-up study of these children,
when they reach the school age, is necessary to detect specific
neuropsychological deficits. Such deficits have been found in
older myelodysplasia patients.
Although majority of our patients were in their infancy,
some amount of functional lateralization was observed on
neuropsychological assessment. On correlating the regional
cortical mantle thickness with neuropsychological outcome,
it was detected that improvement in social and adaptive
function development was better correlated with increase in
right frontal cortical mantle compared to the left, whereas
language development was more effective with increase in
left frontal cortical mantle compared to the right.
Conclusions
The aim of management of hydrocephalus is to achieve total
clinical recovery and to obtain normal/near normal intellectual
development. However, the outcome seems to depend
on multiple factors. Among the 40 children prospectively
studied in this series, 15 had associated myelomeningocele,
only 6 children out of 40 had normal neuropsychological
development preoperatively. Postoperative assessment
revealed improvement in neuropsychological function in all
cases, although normal age-related development was observed
only in 33% of cases in hydrocephalic children. However, it
was observed that the extent of improvement in relation to
mental age was significantly higher when surgical intervention
was done prior to 6 months of age.
Contrary to common belief, the children with
myelomeningocele and hydrocephalus demonstrated
excellent potential for improvement after CSF diversion.
About 80% of children in this subgroup reached normal
psychological development. This appears to be related to
earlier institution of therapy in this subgroup since these
infants were in close observation since birth, which would
mean that earlier therapeutic intervention has a significant
effect on functional brain development.
The study also revealed that the postoperative increase in
cortical mantle was related to improvement in clinical and
neuropsychological outcome in all, though to a variable
extent. Recovery of neuropsychological function appeared
to follow a particular pattern and supports the normal
hemispheric functional lateralization established through
several functional neuroimaging studies. It was observed that
the social and adaptive functional development has higher
levels of correlation with increase in right frontal cortical
mantle and the language development was correlated to
increase in left frontal and parietal cortical mantle.
The motor development was related to both the frontal
cortices. It will be interesting to correlate the ultimate
functional outcome to the asymmetries in the ventricular

dilatation, both localized and generalized on long-term
follow-up. Although the result of this study suggests that
there is overall improvement in cognitive functioning, in the
majority of children who were provided with early surgical
intervention, follow-up for prolonged periods is necessary to
delineate the ultimate outcome. Assessment of these children
in school going age is necessary to detect minor and other
specific neuropsychological deficits, which may prevent the
total functional recovery in these children and for planning
the therapeutic strategies to overcome these deficits.
Improvement in outcome of hydrocephalic children to
normalcy or near normalcy appears to the possible with early
detection and prompt institution of therapy along with serial
and periodic follow-up program for diagnosing and treating
complications and dysfunctions, which may be detected from
time to time.
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Cite this article as: Venkataramana NK, Mukundan CR. Evaluation of
functional outcomes in congenital hydrocephalus. J Pediatr Neurosci
2011;6:4-12.
Source of Support: Nil. Conflict of Interest: None declared.
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4 / Journal of Pediatric Neurosciences / Volume 6 / Jan-Jun / 2011

dilatation of cerebral ventricular system. The incidence of

Therapy for hydrocephalus includes diversion of CSF or

Materials and Methods

diameter, absolute III ventricular diameter, the minimum

Maximum transverse inner

Cortical measurements were taken in frontal and parietal

It has also incorporated the findings of Berkeley growth

Table 1a: Distribution of sample

Table 1b: Age distribution

had corresponding neurological deficits below the level of

The cortical mantle thickness varied with the degree of

Table 2: Clinical features

Table 3: Age of onset of symptoms

Progressive increase in head size

Table 4: Clinical features

Table 7: Ventricular measurement after shunt

Ant. horn index

Table 6: Head circumference

Table 8: Postoperative ventricular indices on followup

Cella media index

Ant. horn index

Table 9: Measurements of cortical mantle

Table 10: Improvement in the cortical mantle

Table 11: Preoperative psychological assessment

Minimal improvement subgroup analysis of 9 (6 in Group A

Table 12: Postoperative psychological development

Cella media index

Table 13: Analysis of minimal improvement subgroup

Table 14: Correlative values of ventricular indices

Group A (N=6) Group B (N=3)

Ant. horn index

Postoperative improvement in absolute 3 rd ventricular

Table 15: Correlative values of cortical mantle

On the contrary, improvement in anterior horn index has

for myelomeningocele. In contrast, the children in Group A

patients having age appropriate psychological development.

normal cognitive functioning in infants with myelodysplasia

functional outcome to the asymmetries in the ventricular

Brain TE, Laurence KM. The effect of Hydrocephalus on intelligence,

following hydrocephalus. In: Wood JH, editor. Neurobiology of

21. Nulsen FE, Rekate HL. Results of Treatment for Hydrocephalus as

Source of Support: Nil. Conflict of Interest: None declared.

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