Original Paper
Pediatr Neurosurg
DOI: 10.1159/000494562
Association of Hydrocephalus with Neural
Tube Defect: Our Experience with the Surgical
Treatment in One or in Two Operative Stages
(on Separate Days)
D. Sylvain Zabsonre a Henri Lankoande a Chantal F.C. Zoungrana/Ouattara b
Abdoulaye Thiombiano a Soumaila Ouedraogo a Abdoulaye Sanou a
Pacome Yameogo a Sonia J.E. Kaboret-Douamba c Abel Kabre a
a Department of Neurosurgery, University Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso; b Department
of Pediatrics, University Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso; c Pediatrics University Hospital
Charles de Gaulle, Ouagadougou, Burkina Faso
Keywords
Spina bifida · Cephalocele · Hydrocephalus · Neural tube
defect
Abstract
Background: Neural tube defects (spina bifidas or cephalo-
celes) are congenital malformations that can be associated
with hydrocephalus. Even if the surgical management of
each of these pathologies separately is well established, this
is not the case for a combination of these conditions. Aim: To
report our results of simultaneous or separate surgery for the
association of hydrocephalus with neural tube defect in the
same patient. Methods: This was a retrospective study of the
association of hydrocephalus with neural tube defect (spina
bifida or cephalocele) managed over a period of 7 years at the
Department of Neurosurgery, Yalgado Ouedraogo University
Hospital, Ouagadougou, Burkina Faso. Results: Thirty-eight
cases were included. The mean age was 8.1 months, and the
sex ratio was 0.81. There were 27 cases of spina bifida and 11
cases of cephalocele associated with hydrocephalus. A cere-
bral CT scan was performed in all patients. In 30 cases, the
operative management of these pathologies was performed
© 2018 S. Karger AG, Basel
E-Mail karger@karger.com
www.karger.com/pne
Received: March 1, 2018
Accepted after revision: October 13, 2018
Published online: December 5, 2018
 
at the same operative time. Eight cases were operated in 2
separate operative stages with a mean time of 30 days be-
tween the 2 operations. The course was favorable in 22 pa-
tients operated by the simultaneous approach and in 3 pa-
tients operated by the separate approach (p = 0.07).
Conclusion: Surgical management of the association of hy-
drocephalus with neural tube defect in 1 or 2 operative stag-
es gave similar clinical results. However, the treatment in 1
surgical stage would considerably reduce the charges.
© 2018 S. Karger AG, Basel
Introduction
Congenital malformations are always feared by par-
ents and practitioners. They pose more difficulties when
they are combined with other conditions and particularly
affect the central nervous system. Spina bifidas and ceph-
aloceles are part of neural tube defects. These are con-
genital malformations defined as a neural tube closure
failure. Spina bifidas and cephaloceles may be associated
with hydrocephalus in the context of a polymalformative
syndrome or not.
130.241.16.16 - 12/6/2018 1:59:30 PM
Dr. D. Sylvain Zabsonre
Centre Hospitalier Universitaire Yalgado Ouedraogo
Ouagadougou 03 (Burkina Faso)
Göteborgs Universitet
E-Mail szabsonre @ gmail.com
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 The treatment of these conditions (hydrocephalus,
cephalocele, spina bifida) is surgical and is well estab-
lished when performed separately [1, 2]. This is not the
case for the association of neural tube defects with hy-
drocephalus in the same patient. Thus, the choice can be
made for the surgical treatment of these 2 conditions in
1 operative stage (simultaneous surgery) or in 2 opera-
tive stages separated by a few weeks (separate surgery).
Each of these choices has its advantages and disadvan-
tages. The aim of our study is to report on our results of
simultaneous or separate surgery for the association of
hydrocephalus with neural tube defect in the same pa-
tient.
Methods
It was a retrospective, descriptive, and analytical study carried
out at the Department of Neurosurgery of the Yalgado Ouedraogo
University Hospital, Ouagadougou, Burkina Faso, from January 1,
2009 to December 31, 2015. Medical records of patients admitted
for the association of hydrocephalus with neural tube defect were
analyzed. Hydrocephalus and neural tube defect were diagnosed at
the same time. Thus, cases of hydrocephalus that occurred after the
cure of a neural tube defect were not considered for this study.
Neural tube defects included spina bifidas and cephalocele. Cases
of the association of a hydrocephalus with a neural tube defect di-
agnosed at the same time but not operated (20 cases) were exclud-
ed from our study which aimed to study only the results of the
surgery. Only the records of the operated patients were included
in the study. Of these, non-exploitable records were excluded (5
cases).
Ethical considerations were taken into account when collecting
data. Thus, the anonymity and confidentiality of the data have
been rigorously respected. The Fisher test allowed us to compare
the qualitative variables with a 95% confidence interval. Any prob-
ability value (p) <0.05 was considered statistically significant.
Results
Epidemiology
Thirty-eight cases were included. There were 21 girls
and 17 boys with a sex ratio of 0.81. Patient ages ranged
from 1 day to 8 years with an average of 8.1 months.
Twenty-seven patients had spina bifida associated with
hydrocephalus and 11 had cephalocele associated with
hydrocephalus. The delay between the first symptoms
and a neurosurgical consultation ranged from 1 day to 9
years with an average of 22.5 months. Patients came from
rural areas with poor socioeconomic conditions in 86.2%
of cases. There were 8 cases of second degree consanguin-
ity and 2 cases of spina bifida in the family. Maternal age
2 Pediatr Neurosurg
DOI: 10.1159/000494562
ranged between 16 and 41 years with an average age of
26.4 years. Half of the mothers were under 25 years of age.
All mothers took iron-folic acid supplements from the
second month of pregnancy. Thirty mothers had at least
3 prenatal consultations while the other 8 had less than 3.
There were 10 maternal hyperthermia cases in the first
trimester of pregnancy.
Clinical Aspects
Among the 27 patients who had spina bifida associated
with hydrocephalus, there were 4 cases of alteration of the
general condition, including 2 cases of infections of the
spina bifida and 2 cases of dehydration by leakage of ce-
rebrospinal fluid (rupture of the spina bifida). The tume-
faction of the spina bifida was lumbosacral (11 cases),
lumbar (9 cases), lumbodorsal (4 cases), and sacral (3 cas-
es). It was covered with healthy or scarred skin in 23 cas-
es. A total of 20 patients had motor deficit, including 10
cases of complete paraplegia with sphincter disorders.
Thirteen patients had another associated malformation
or deformation including 10 cases of deformation of feet,
1 case of interventricular communication, 1 case of ano-
rectal malformation, and 1 case of renal malformation.
Figure 1a, b shows the clinical aspects of the association
of hydrocephalus with spina bifida.
Among the 11 patients with cephalocele associated
with hydrocephalus, 1 case experienced deterioration of
the general condition in the context of severe malnutri-
tion. The tumefaction of the cephalocele was covered
with a healthy or scarred skin in all cases. It was situated
in the occipital (8 cases), parietal (1 cases), frontal (1
case), or frontonasal (1 case) region. With respect to hy-
drocephalus, 23 patients had macrocrania, 19 Parinaud’s
syndrome, 2 internal strabismus, and 14 had other signs
of intracranial hypertension (e.g., headache, vomiting).
Medical Imaging
All patients underwent a cerebral CT scan, and all cas-
es with spina bifida also underwent a CT scan of the spinal
segment affected by swelling. Hydrocephalus was triven-
tricular in 31 cases, tetraventricular in 5 cases, and biven-
tricular in 2 cases. Among the spina bifida cases, there
were 19 cases of myelomeningocele and 8 cases of menin-
gocele. The cephaloceles included 7 meningoceles and 4
meningoencephaloceles. Six antenatal ultrasounds were
performed. Five cases of hydrocephalus were diagnosed.
The clinical and scannographic aspects of the association
of hydrocephalus with encephalocele (encephalocysto-
cele) are shown in Figure 2a–c.
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Zabsonre et al.
Göteborgs Universitet
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 Color version available online
b

Fig. 1. a, b The clinical images of the association of hydrocephalus

a
with spina bifida are shown.

Color version available online

a b c

Fig. 2. a–c The clinical and scannographic images of the association of hydrocephalus with encephalocele (en-
cephalocystocele) are shown.

Treatment and Postoperative Course
The waiting time for surgery varied from 7 days to 22
months with an average of 4 months. Thirty cases of hy-
drocephalus associated with neural tube defects (23 spina
bifidas, 7 cephaloceles) were operated in 1 operative time
(simultaneous surgery). A ventriculoperitoneal shunt
with a medium pressure valve was applied in all these cas-
es. It preceded the treatment of the neural tube defect in
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Surgical Management of Hydrocephalus Pediatr Neurosurg 3

and Neural Tube Defect DOI: 10.1159/000494562
Göteborgs Universitet
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 Table 1. Surgical treatment and postoperative course

Group 1a (27 cases) Group 2b (11 cases) Total

c (38
approach 1 approach 2d approach 1c approach 2d cases)
(23 cases) (4 cases) (7 cases) (4 cases)

Ventriculoperitoneal shunt 23 0 7 4 34
Ventriculocisternostomy 0 4 0 0 4
Hospital stay ≥19 days 18 3 4 2 27
Favorable course 18 1 4 2 25
Complications 5 3 3 2 13
a Group 1: association with hydrocephalus/spina bifida. b Group 2: association with hydrocephalus/cephalocele.
c
 Approach 1: surgery performed in 1 operative stage (simultaneous surgery). d Approach 2: surgery performed
in 2 operative stages (separate surgery).

24 cases (23 spina bifidas, 1 cephalocele). In the other 6 Discussion

cephalocele cases, the ventriculoperitoneal shunt was ap-
plied after the cephalocele cure.
Eight cases of hydrocephalus associated with neural
tube defects (4 spina bifidas, 4 cephaloceles) were oper-
ated separately on different days (separate surgery). The
time between the first and the second operation ranged
between 3 and 120 days with an average of 30 days. Hy-
drocephalus treatment consisted of a ventriculoperito-
neal shunt in the 4 cases of cephaloceles and a ventricu-
locisternostomy in the 4 cases of infected or ruptured
spina bifida. Ventriculoperitoneal shunts were per-
formed after the cephalocele cures, while ventriculocis-
ternostomy was performed in the first position in the
spina bifidas.
Postoperative hospital stay ranged from 8 to 51 days
with an average of 19 days. Twenty-two patients out of
the 30 operated simultaneously and 5 out of the 8 pa-
tients operated separately had a hospital stay of ≤19 days
(p = 0.42). The postoperative course was favorable in 25
patients. A complication was observed in 8 patients op-
erated simultaneously (7 hyperthermia, 2 anemia, and 1
surgical wound suppuration) and in 5 patients operated
on separate days (4 hyperthermia, 1 anemia, and 1 surgi-
cal wound suppuration) (p = 0.07). Of the 4 ventriculo-
cisternostomy cases, 3 had a postoperative complica-
tion.
Table 1 summarizes the surgical treatment and the
postoperative course of hydrocephalus and spina bifida
(group 1) or hydrocephalus and cephalocele (group 2)
treated simultaneously (approach 1) or separately (ap-
proach 2).
The frequency of 9 cases/year of the association of hy-
drocephalus with neural tube defect observed in our study
is as high as that observed by other authors in Africa: De
Paul et al. [1] in Cameroon (9 cases/year) and Warf et al.
[2] in Uganda (22 cases/year). In Saudi Arabia, Alshail et
al. [3] found a higher frequency of 59.83 cases/year. The
frequency noted in our study is certainly underestimated
because it does not consider the cases not admitted to our
department. The high frequency of the association of hy-
drocephalus with neural tube defect in Africa may be due
to the existence of favorable factors (pregnancy diseases,
nutritional deficiencies, consanguinity) which have also
been observed by other authors [4–8]. The inadequacy of
antenatal diagnosis and the fact that the therapeutic inter-
ruption of pregnancy is not permitted by law in most of
these countries could also explain the high frequency of
congenital malformation in these African countries.
Clinical signs (congenital cephalic or spinal tumefac-
tion, macrocrania or other signs of intracranial hyperten-
sion) and CT scan (bone defect, precision on the contents
of the tumefaction, increase in ventricular size) make a
postnatal diagnosis of these conditions easy. Similarly,
the treatment of each condition separately poses only few
problems [9–12]. However, in case of a combination of
these conditions, the problem of a simultaneous or sepa-
rate surgical treatment is posed. Simultaneous manage-
ment, which is by far the most beneficial for all, is feared
because of a possible increase in postoperative complica-
tions known for each of these conditions with the most
frequent being infection [3, 9, 11]. Furthermore, the
young age and the small blood mass of the patients is not
in favor of a long surgical intervention. Obstruction of the
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4 Pediatr Neurosurg Zabsonre et al.

DOI: 10.1159/000494562
Göteborgs Universitet
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ventriculoperitoneal shunt by a clot resulting from the
treatment of a neural tube defect is also feared.
We performed simultaneous surgery of these 2 pathol-
ogies (hydrocephalus and neural tube defect) in almost all
patients of our series because we work in a difficult socio-
economic context. Patients came from rural areas with
poor socioeconomic conditions in 86.2% of cases. The
main aim of this simultaneous approach was to reduce
the charges (financial, social, etc.) related to the manage-
ment of these malformations on separate days. This si-
multaneous approach was consistently indicated in ab-
sence of signs of infection in the patient. The classic com-
plications of this type of surgery were noted in our study
but without a significant difference (p = 0.07) depending
on whether the malformations were performed simulta-
neously or delayed.
Some patients were operated on a separate day because
they had a particular problem, such as difficulties in buy-
ing a shunt or bad skin condition (e.g., rupture, superin-
fection of the spina bifida). Infected or ruptured spina
bifidas were ventriculocisternostomized first to treat ma-
jor hydrocephalus while not risking to infect the shunt
until resolution of tumefaction allows its cure.
All patients operated simultaneously had normal or
scarred skin in the neural tube defect without any preop-
erative infection sign. They all benefited from the ven-
triculoperitoneal shunt because the endoscope was not
available for indications of ventriculocisternostomy. In
24 patients (23 spina bifidas and 1 cephalocele), the ven-
triculoperitoneal shunt was applied before the cure of the
neural tube defect in order to prevent the implantation of
the ventricular catheter being laborious or impossible due
to the loss of cerebrospinal fluid associated with the cure
of a neural tube defect. The simultaneous treatment of
hydrocephalus associated with neural tube defect did not
entail a particular risk for the patients. The simultaneous
surgery did not require any special preoperative prepara-
tion of the patient and did not prolong the hospital stay
postoperatively (p = 0.42). However, the prognosis of
these conditions depends on the initial neurological dam-
age [13].
Conclusion
Hydrocephalus associated with neural tube defect af-
fects more girls than boys. Spina bifidas were more com-
monly associated with hydrocephalus than cephalocele.
Almost all patients received treatment of hydrocephalus
and neural tube defect simultaneously at the same opera-
tive time. Their postoperative course was satisfactory and
there was no statistically significant difference compared
to patients operated on a separate day. Thus, simultane-
ous surgical treatment in 1 operating stage of this combi-
nation of conditions would considerably reduce the
charges (financial and social) for affected patients.
Disclosure Statement
The authors have no financial interest or conflicts of interest to
disclose.

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and Neural Tube Defect DOI: 10.1159/000494562
Göteborgs Universitet
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