Childs Nerv Syst (2011) 27:15711573
DOI 10.1007/s00381-011-1556-0
SPECIAL ANNUAL ISSUE
Fetal ventriculomegaly: postnatal management
Kyu-Chang Wang & Ji Yeoun Lee & Seung-Ki Kim &
Ji Hoon Phi & Byung-Kyu Cho
Received: 30 July 2011 / Accepted: 9 August 2011
# Springer-Verlag 2011
Abstract
Introduction It is the current status of fetal ventriculomegaly
that although the technology for diagnosis is advanced, it does
not have significant impact on the management outcome.
Fetal ventriculomegaly is mainly treated after birth.
Methods We reviewed the literature and suggested policies of
postnatal evaluation and surgical management of fetal hydro-
cephalus. Our experience of 44 cases of fetal ventriculomegaly
diagnosed by fetal ultrasonography, in which major poor
prognostic factors were absent and for which prenatal pediatric
neurosurgical consultation was sought, was also presented.
Results Our experience showed etiologic heterogeneity of fetal
ventriculomegaly although our cases seemed to be surgical
candidates more likely than whole group of fetal ventriculo-
megaly. There were limitations in prenatal evaluation of fetal
hydrocephalus. The first step for postnatal management is
etiologic classification. It should be clarified after birth whether
there is remarkable disturbance of cerebrospinal fluid dynamics
or not. The rate of postnatal progression of ventricular dilatation
is also important for the decision of treatment plan. For surgical
treatment in very young children, special considerations should
be paid on technical feasibility, rate of postoperative infection or
malfunction, prevention of rapidly developing nervous system
from the possible damage, and great plasticity of young brain.
Conclusion Indication, methods, and timing of surgical
treatment must be individually tailored according to the
etiology, degree and rate of progression of ventriculomegaly,
and patients age when surgical treatment is considered.
K.-C. Wang (*) : J. Y. Lee : S.-K. Kim : J. H. Phi : B.-K. Cho
Division of Pediatric Neurosurgery,
Seoul National University Childrens Hospital,
101 Daehak-ro, Jongno-gu,
Seoul 110-769, South Korea
e-mail: kcwang@snu.ac.kr
Keywords Ventriculomegaly . Fetus . Cerebrospinal fluid
dynamics . Progression . Postnatal management
Introduction
In spite of advancements in imaging and prenatal molecular
diagnosis of fetus, prenatal management of fetal ventricu-
lomegaly is still limited [1]. The heterogeneity of this entity
hinders clear etiologic classification. Trials of intrauterine
ventricular shunting procedure revealed no definite evi-
dence of improved outcome [1, 11]. In cases with
progressing fetal hydrocephalus, early delivery and postna-
tal management may be beneficial. However, the incidence
of rapidly progressing true hydrocephalus [ventriculome-
galy with evident disturbance of cerebrospinal fluid (CSF)
dynamics] is low [9, 13, 16] and the lung maturation issue
in prematurity is more important than hydrocephalus even
when it is true. It is the current status of fetal
ventriculomegaly that although the technology for diagno-
sis is advanced, it does not have significant impact on the
management outcome. Therefore, the postnatal manage-
ment of fetal ventriculomegaly is basically not different
from cases in which a diagnosis is made after birth.
The first step for postnatal management is etiologic
classification of fetal ventriculomegaly. It should be
clarified after birth whether there is remarkable disturbance
of CSF dynamics or not. The rate of postnatal progression
of ventricular dilatation or head enlargement is also
important for treatment decision.
For surgical treatment of very young children, special
considerations should be paid on technical feasibility, rate
of postoperative infection or malfunction, prevention of
rapidly developing nervous system from the possible
damage, and great plasticity of young brain.
1572
Postnatal evaluation
Further etiologic specification of ventriculomegaly
In spite of advancements in imaging and molecular
diagnosis, the etiology of fetal ventriculomegaly is not
clear even after birth in many cases.
Ventriculomegaly is the result of three different categories
of brain lesions: (1) disturbance of CSF dynamics, (2) brain
volume loss (destruction of brain tissue after its formation),
and (3) another group of abnormal dilatation of ventricles.
In cases of disturbed CSF dynamics (true hydroceph-
alus, hydrocephalus in a strict sense), primary pathology is
not in the brain itself. Brain is injured by the secondary
effects. The degree of neurological dysfunction is less
severe than in cases with brain volume loss. The cranium
tends to be large, sutures separated, and fontanel bulged.
Contrary to the first category, cases with brain volume
loss have the primary pathology in the brain itself.
Intrauterine infection, infarction, and hemorrhage are
leading causes of primary brain damage. The cranium
tends to be small with overriding parietal bones on the
frontal or occipital bones, and sunken or closed fontanel.
Developmental delay or seizures are more common than in
the patients of disturbed CSF dynamics.
In the third category, ventriculomegaly is not caused by
disturbed CSF dynamics or brain volume loss, but by
different shape of the brain. Ventricular dilatations in cases
with agenesis of corpus callosum or some of neuronal
migration disorders are included in this category. There are
cases of combination of different categories.
The postnatal management plan differs according to the
etiologic classification of ventriculomegaly. Only the
patients of the first category are potential candidates for
CSF diversion treatment.
Postnatal evaluation of ventriculomegaly is more accurate
than prenatal investigations. Some of investigations are not
feasible for a fetus. The shape of skull, sutures, fontanel, and
the images of brain should be reevaluated after birth.
Moreover, detailed information on the functional status of a
baby such as vision, hearing, sucking and swallowing, general
motor and sensory, or presence of seizure is available only
after birth. Postnatal evaluation may elucidate the etiology of
ventriculomegaly more clearly than before birth.
Rate of progression
The progression of hydrocephalus may be accelerated after
birth compared to the prenatal course. Absence of uterine
containment may allow liberal enlargement of skull and
ventricles. Rapid enlargement of ventricles can cause
marked stretching and ischemia of the brain parenchyma
leading to irreversible damage. On the other hand, stable
Childs Nerv Syst (2011) 27:15711573
mild to moderate hydrocephalus can be managed in rather
conservative ways [5, 13, 16, 17]. Frequently, it does not
need surgical management.
Because surgical treatment in very young children has
limitation in its technical feasibility and high rate of infection
or failure, if patient condition allows, delayed surgery up to
6 months of age or more is a reasonable option [2, 4, 15].
Therefore, to make a decision on the indication and timing
of surgery, information on the degree and progression rate of
ventriculomegaly is essential. Periodic measurement of head
circumference, examination of fontanel and sutures, and
neuroradiological imaging follow-up of ventricular size are
mandatory, especially during the first year of life.
Surgical management
Indication for surgical treatment
For patients with definite abnormalities of CSF dynamics
and significant ventricular dilatation or increased intracra-
nial pressure (ICP), surgical treatment should be provided.
Those without definite abnormalities of CSF dynamics or
children with stable mild to moderate ventriculomegaly are
managed by close observation.
Generally, large head, bulging of fontanel, suture
separation, large ventricle (especially width 20 mm),
progressive ventriculomegaly, and relatively intact neuro-
logical function are clues for high probability of postnatal
surgical treatment [6, 7, 13]. On the other hand, small head,
sunken fontanel, overlapped bones at the suture area, mild
to moderate ventriculomegaly without progression, and
marked neurological dysfunction suggest low possibility of
postnatal surgical management.
We had reviewed a series of fetal ventriculomegaly cases
in which major poor prognostic factors (chromosomal
abnormalities, associated major anomalies, and intrauterine
infection) were absent and for which prenatal pediatric
neurosurgical consultation was sought [9]. Among 44
patients who seemed to be surgical candidates more likely
than whole group of fetal ventriculomegaly, only 11
underwent surgical treatment after birth. We also found
associated anomalies and functional deficits only after birth
in 22 cases. Among cases of fetal ventriculomegaly
without major prenatal poor risk factors, still cases with
brain parenchymal loss are included. Decision on the
surgical treatment should be made carefully by the detailed
investigation on the etiology of ventriculomegaly.
Considerations for surgical treatment during young infancy
For the management of progressive true hydrocephalus in
young infants, surgical CSF diversion is the main stream.
Childs Nerv Syst (2011) 27:15711573
Medical treatment such as hyperventilation and administra-
tion of mannitol or acetazolamide are helpful as temporary
measures. Long-term management needs CSF diversion or
fenestration by surgery. As was stated, surgical management
of fetal hydrocephalus is basically not different from the
cases in which hydrocephalus was diagnosed after birth.
Various kinds of ventricular drainage, reservoir placement,
shunting, and fenestration procedures are used.
Nonetheless, special attention should be paid on for those
young infants with fetal hydrocephalus. Some of them need
surgical management when they are very young. For
premature babies, ventricular shunting to the other body
cavity is not feasible. In these patients, tentative measures
including external ventricular drainage, reservoir placement,
and ventriculo-subgaleal shunting are options [8, 10].
Hydrocephalus caused by obstruction at intra- and
extraventricular spaces with preserved CSF absorption
capacity is a good indication of endoscopic third ventricu-
lostomy (ETV). However, the effects of ETV in infants
younger than 6 months or 1 year are not as satisfactory as
cases of older children even though it is controversial [2, 3,
12, 14]. Immaturity of CSF absorption mechanism and
enlargement of head and ventricles before ICP increases
over the threshold pressure of CSF absorption in young
infants seem to be the reasons of increased failure rates of
ETV in young infants.
As was stated previously, it is well known that the
infection and malfunction rates of CSF shunts are higher in
young infants especially before the age of 6 months.
Delayed shunting will be helpful to avoid shunt infection
or malfunction if the patients condition permits.
Summary
Indication, method, and timing of surgical treatment must
be individually tailored according to the etiology, degree,
rate of progression of ventriculomegaly, and patients age
when surgical treatment is considered.
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