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Frontal Encephalocele – A Rare Case Report with Review of Literature

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Dinesh kumar Barolia at SMS Medical College
Dinesh kumar Barolia
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Encephalocele is a congenital anomaly that represents the protrusion of brain component through the cranial defect. Frontal encephalocele is protrusion of brain content through defect in frontal bone. Frontal encephalocele is a rare entity. We report a case of congenital frontal encephalocele which is a rare neural tube defect.
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DOI: 10.21276/sjmcr.2016.4.11.5
Available Online: http://saspjournals.com/sjmcr 822
Scholars Journal of Medical Case Reports ISSN 2347-6559 (Online)
Sch J Med Case Rep 2016; 4(11):822-824 ISSN 2347-9507 (Print)
©Scholars Academic and Scientific Publishers (SAS Publishers)
(An International Publisher for Academic and Scientific Resources)
Frontal Encephalocele – A Rare Case Report with Review of Literature
Dinesh Kumar Barolia*, Sunil Mehra, Vinita Chaturvedi, Neeraj Tuteja, Soumyodhriti Ghosh, Dr. Ramjee
Prasad
Department of Pediatric Surgery, J. K. Lon Hospital, S. M. S. Medical College, Jaipur, Rajasthan, India
*Corresponding author
Dinesh Kumar Barolia
Email: dbaroliarnt@gmail.com
Abstract: Encephalocele is a congenital anomaly that represents the protrusion of brain component through the cranial
defect. Frontal encephalocele is protrusion of brain content through defect in frontal bone. Frontal encephalocele is a rare
entity. We report a case of congenital frontal encephalocele which is a rare neural tube defect.
Keywords: Encephalocele, Frontal, Frontal Encephalocele, Frontonasal Encephalocele.
INTRODUCTION
Encephalocele is a variant of neural tube
defect. Incidence of encephalocele is not uncommon in
developing countries. The incidence ranges from 1-3
per 1000 live births worldwide [1]. Anterior
encephalocele is a rare entity among encephalocele.
Incidence of anterior encephalocele in the western
countries varies from 1/35,000 to 1/40,000 live births,
but in Asian countries its incidence increases to 1/5000
live births [2]. Occipital region is the most common site
for encephaloceles. Encephaloceles occurs commonly
in the mid sagittal plane anywhere from frontonasal
region to the occiput [3]. Occurrence of encephalocele
in occipital region (75%), followed by frontoethmoidal
(13% to 15%), parietal (10% to 12%) or sphenoidal.
Occurrence of occipital encephalocele is common in
western hemisphere where as anterior encephaloceles
are found more often in south East Asia [4, 5].
CASE REPORT
We report a three month old male child
presenting to the department of pediatric surgery in
SMS Medical College with complaint of frontal
swelling since birth. Swelling was progressively
enlarged in size. The baby was born at full term vaginal
delivery without any complications. The gestational
period of the mother was uneventful except that she
reports of not having taken folic acid tablets during this
pregnancy. The baby also has a sibling who is
asymptomatic. The swelling was soft, brilliantly
transilluminant and pulsatile (figure 2). There was no
neurological deficit. NCCT brain showed frontal bone
defect through which meninges with CSF and part of
right frontal lobe of brain protruded. MRI brain showed
cranio-bifida in right frontal region with evidence of
frontal encephalomeningocele with adjacent brain
parenchyma showing cystic gliosis, communicating
with right lateral ventricle (figure 3). Both lateral and
third ventricles are dilated. He was admitted in surgical
nursery at the age of two days. He was operated for
insertion of ventriculo-peritoneal medium pressure
shunt (VPMP) for hydrocephalous on right side. At the
age of three months, his shunt became nonfunctioning
due to blockade. The patient underwent a revision of
shunt again. After insertion of the new shunt, the size of
encephalocele was dramatically decreased (figure 1).
Patient was discharged on post operative day three. The
present status is uneventful.
Dinesh Kumar Barolia et al.; Sch J Med Case Rep, Nov 2016; 4(11):822-824
Available Online: http://saspjournals.com/sjmcr 823
Fig:-1: Showing reduction of size of encephalocele on post operative day one, two and three
Fig-2: Showing encephalocele of same baby at the age of 2 days
Fig-3: MRI shows communication between encephalocele and lateral ventricle
Dinesh Kumar Barolia et al.; Sch J Med Case Rep, Nov 2016; 4(11):822-824
Available Online: http://saspjournals.com/sjmcr 824
DISCUSSION
Frontal encephaloceles are rare entity.
Incidence of frontal encephalocele in the western
hemisphere is very less compare to Asian countries [2].
Ghosh Soumyodhriti et.al. did a retrospective study on
neural tube defect. The incidence of encephalocele in
this study was 14% but no mention was made of frontal
defect probably because of absence of cases in his study
[6]. Encephalocele is a type of neural tube defect which
occurs in fetal life where the ectodermal cells that form
the skull do not come together to close over the brain.
This leads to protrusion of brain tissue and meninges
through bony defect. Encephalocele may be present
anywhere in head but most commonly located in
midline occipital region. [7]. Etiology of encephalocele
is multifactorial. It is documented that encephalocele is
common in parents having history of neural tube defect.
There is evidence that supplementation of folic acid
significantly reduces the occurrence of neural tube
defects. Supplementation is recommended at least four
weeks prior to conception, and continued until the end
of the first trimester [8]. Amarin ZO, Obeidat AZ
reported that fortification of food with folic acid
reduces the incidence of encephalocele [9].
Confirmative diagnosis of frontal encephalocele is
made by CT head and MRI brain. CT scan helps to
know about bony abnormalities in skull. Magnetic
resonance imaging is better to know brain tissue details,
including any intracranial connection [10].
Encephalocele is often associated with craniofacial
abnormalities or other brain malformations. Symptoms
may include neurologic problems, hydrocephalus,
spastic quadriplegia, microcephaly, ataxia,
developmental delay, vision problems, mental and
growth retardation, and seizures [11]. Morina A. et al.
found that the prevalence of encephalocele is higher
among rural patients of our country with poor living
conditions, malnutrition . They advised to avoid surgery
in very young children until body weight is around 5-6
kilograms because of complication from blood loss and
hypothermia. Hydrocephalus should be treated before
corrective surgery for encephalocele, to avoid the risk
of postoperative CSF leak [12].
CONCLUSION
Frontal encephalocele is a variant of neural
tube defect. It is a rare entity. We recommend treating
the hydrocephalus initially and observing the baby for
reduction in size of the encephalocele. Definitely
surgery should be deferred for an elective date.
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... 9 Frontal encephaloceles are rare and generally known to have a higher incidence in Asian countries (1/5000 live births) than western countries (1/35,000 to 1/40,000 live births). 17 In Africans, evidence suggest that frontal encephaloceles are more common in East, then West Africa. 18 Children with severe birth defects have a 15-fold increased risk of death during the first year of life, with 9-10% of such children dying during this period. ...
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Ramachandra CR, Phelps PD. Nasal encephalocoeles associated with unilateral absence of the cochlea. The Journal of Laryngology & Otology
  • Jan 1966
  • 20-26813
  • J Austpaediat
AustPaediat J. 1966;2:20-26. 3. Ramachandra CR, Phelps PD. Nasal encephalocoeles associated with unilateral absence of the cochlea. The Journal of Laryngology & Otology. 1977 Sep 1;91(09):813-7.