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Original Article

Pediatric encephaloceles: A series of 20cases

over a period of 3years
Shashank Ravindra Ramdurg, M. Sukanya, Jayabrata Maitra
Department of Neurosurgery, MR Medical College, Kalaburagi, Karnataka, India

Address for correspondence: Dr.Shashank Ravindra Ramdurg, Department of Neurosurgery, Basaveshwara Teaching and General Hospital,
MR Medical College, Kalaburagi, Karnataka, India. Email:shashank.ramdurg@gmail.com

ABSTRACT
Background: Encephalocele is the protrusion of the cranial contents beyond the normal confines of the skull
through a defect in the calvarium and is far less common than spinal dysraphism. The exact worldwide
frequency is not known. Aims and Objectives: To determine the epidemiological features, patterns of
encephalocele, and its postsurgical results. Materials and Methods: The study was carried from year
July 2012 to June 2015. Patients with encephalocele were evaluated for epidemiological characteristics, clinical
features, imaging characteristics, and surgical results. Results: 20 encephaloceles patients were treated
during the study period. Out of these 12(60%) were male and 8(40%) female. Age range was 1day to 6years.
The most common type of encephalocele was occipital 12(60%), occipitocervical 4(20%), parietal 2(10%),
frontonasal 1(5%), and frontonasoethmoidal 1(5%). One patient had a double encephalocele(one atretic
and other was occipital) with dermal sinus tract and limited dermal myeloschisis. Other associations: Chiari 3
malformation(2), meningomyeloceles(4), and syrinx(4). Three patients presented with rupture two of whom
succumbed to meningitis and shock. Seventeen patients treated surgically did well with no immediate surgical
mortality(except a case of Chiari 3 malformation who succumbed 6months postsurgery to unrelated causes).
Shunt was performed in 4cases. Conclusion: The most common type of encephalocele is occipital in our set
up. Early surgical management of encephalocele is not only for cosmetic reasons but also to prevent tethering,
rupture, and future neurological deficits.

Key words: Encephalocele, occipital, sincipital, split pons, surgical management

Introduction factors as potential causes.[14] So far, only aflatoxin has

Encephalomeningocele is a congenital malformation
characterized by protrusion of meninges and/or brain tissue
due to a skull defect. It is one form of neural tube defects as
the other two, anencephaly and spina bifida.[1]
Despite the higher incidence of this congenital defect in
this area, little is known about its etiology and pathogenesis.
Some evidence from previous studies suggest environmental
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been proposed to be a teratogenic agent for this anomaly.[2]
Indirect evidences from its closely related anomaly, spina
bifida, [5] may suggest the role of folate deficiency in
encephalomeningocele. However, again, there were no
studies on the relationship between maternal folate level
and incidence of encephalomeningocele, and some evidences
have suggested different underlying mechanisms between
these two forms of neural tube defects.[68]
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DOI: Cite this article as: Ramdurg SR, Sukanya M, Maitra J. Pediatric
10.4103/1817-1745.174462 encephaloceles: A series of 20 cases over a period of 3 years. J Pediatr
Neurosci 2015;10:317-20.

2015 Journal of Pediatric Neurosciences | Published by Wolters Kluwer - Medknow / 317

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Ramdurg, etal.: Pediatric encephaloceles

Here, we report 20cases of encephaloceles in 19patients and Table1: Age Distribution

review their epidemiological, clinical, imaging characteristics,
Age(months) Number of patients
as well as analyze the surgical results.
03 11
46 3
Aims and objectives 79 3
1012 2
The aims and objectives of the study are to observe and >12 1
analyze the epidemiological, clinical, imaging characteristics,
as well as analyze surgical results of all cases of encephaloceles
that were treated at our institute from July 2012 to June 2015. Table2: Associated anomalies
Associated Anomalies No of cases
Meningomyelocoeles 4
Materials and Methods Chiari 3 malformation 2
Syrinx 4
Dermal sinus tract 1
The study is a prospective observational study conducted from
Limited dermal myeloschisis 1
2012July to 2015June. Atotal of 20cases of encephalocele Split pons 1
in 19patients were analyzed during the study period. Patients
with encephalocele were evaluated for epidemiological
characteristics, clinical features, imaging characteristics, Clinical course and surgical treatment
and surgical results. Data were recorded from case records, Three patients presented with rupture, one of whom
operation notes, and death records. succumbed to shock even before surgery. Of the other
2patients, in whom repair of sac was performed, the
patient with meningocele improved while the patient with
Results encephalocele succumbed to meningitis and shock.

A total of 20 encephaloceles cases in 19patients were One patient with frontonasoethmoidal encephalocele was
treated during the study period. One patient had a double managed with combined open and endoscopic approach. Rest
encephalocele one of which was atretic[Figure1]. patients underwent open exploration and repair.

Epidemiological features
Out of the 20 encephaloceles, 12(60%) were male and 8(40%)
female patients. The youngest was a 1day old infant while the
oldest child presented at 6years of age(median=1month,
mode=1month)[Table1]. Adequate antenatal checkup
and folic acid supplementation were received by mothers.
However, none of them received preconceptional folic acid.
One patient was given a normal report on preoperative scan.
The mode of delivery in most of the cases was by cesarean
section. Only one patient was delivered per vagina.
The other 17 encephaloceles treated surgically did well with no
immediate surgical mortality [Figure3]. One child with Chiari
3 malformation succumbed 6months postsurgery to unrelated
causes. Shunt was performed in 4cases due to the development
of hydrocephalus. Wound site infection and cerebrospinal
fluid(CSF) leak was seen in 4cases which were conservatively
managed with 1 patient requiring reexploration and repair.
The case with frontonasal encephalocele developed ocular
CSF leak which was conservatively managed.
The followup period ranged from 6months to 3years.

Pattern and clinical features

Most common type of encephalocele was occipital. Occipital
encephaloceles accounted for 12 (60%), occipitocervical 4 (20%),
parietal 2 (10%), frontonasal 1 (5%), and frontonasoethmoidal
1(5%). One patient had a double encephalocele (one atretic
and other was occipital) with dermal sinus tract and limited
dermal myeloschisis and a split pons. Other associations: Chiari 3
malformation (2), meningomyeloceles (4), and syrinx (4). Three
patients presented with ruptured encephalocele [Tabl 2].
Imaging
All patients underwent magnetic resonance imaging scan
of the head with screening of whole spine(except patients
with ruptured encephaloceles). On imaging, 2patients had
only meningoceles, 2 were atretic, while the rest had varying
content of brain tissue[Figure2].
Discussion
Encephaloceles represents a congenital defect of the cranium
in which a portion of central nervous system herniates through
the defect. It is a common congenital problem in the practice
of neurosurgery worldwide.[9]
Occipital encephaloceles can vary from a small swelling
to extremely large one. In our series, the most common
site was occipital. The contents of the sac vary from small
dysplastic diverticulum to a large amount of degenerative
brain tissue. Large sacs were always filled with CSF with or
without septations. The bony defect can vary in size. In one
of the interesting case of ruptured meningocele, the defect
had almost closed and hence the patient had a good outcome
despite presenting with rupture.

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Ramdurg, etal.: Pediatric encephaloceles

Elective surgery provides time for the patients to gain

weight and strength and allows the surgeon to select the
best technique. Most large encephaloceles required urgent
surgical treatment to avoid damage to sac. In all the occipital,
parietal and nasal encephaloceles there was dysplastic brain
tissue which was removed safely.

Postoperative hydrocephalus should be managed through

ventriculoperitoneal (VP) shunts as one or two stage
procedures.[12] Four of our cases required postoperative VP
shunt in addition to the repair of the sac. In one case of
frontonasoethmoidal encephalocele, combined open and
endoscopic procedures were utilized.

The immediate outcome was good in all except in 2patients.

Both patients had ruptured encephaloceles. One of the
Figure 1: Clinical photographs and magnetic resonance imaging images of
double encephalocele patients succumbed before surgery. The other patient died
due to intractable meningitis and sepsis.

CSF leak and wound infection observed in four cases

improved on conservative treatment. An interesting result
of CSF leak through eyes was noted in one of the cases with
frontonasal encephalocele.

Conclusion
The most common type of encephalocele is occipital in our set
up. Early surgical management of encephalocele is not only for
cosmetic reasons but also to prevent tethering, rupture, and
future neurological deficits. Complications like hydrocephalus
may need to be managed with shunt surgery. Endoscopic
procedures play an important role in frontonasoethmoidal
encephaloceles.
Figure 2: Magnetic resonance imaging images

Declaration of patient consent

The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest
There are no conflicts of interest.
Figure 3: Pre- and post-operative images of representative cases

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Ramdurg, etal.: Pediatric encephaloceles

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