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CASE REPORT
Year : 2020  |  Volume : 23  |  Issue : 4  |  Page : 242-244

A giant occipital encephalocele in an infant


Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Submission09-Oct-2019
Date of Decision25-Feb-2020
Date of Acceptance25-Feb-2020
Date of Web Publication23-Feb-2021

Correspondence Address:
Dr. Muhammad Sule Baba
Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Nigeria
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DOI: 10.4103/smj.smj_55_19

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  Abstract 


Encephaloceles are neural tube defects that are characterized by protrusion of brain and meninges through a defect in the cranium. Encephaloceles are very rare with an incidence of about 1 in 5000 live births and a female preponderance with an unknown etiology. This is a case of a 3-month-old female infant with a huge occipital swelling, delayed developmental milestones, and microcephaly without neck control and social smile and poor suckling. The patient was referred by the neurosurgical unit of the hospital to the department of radiology for a contrast-enhanced computed tomography (CECT) of the brain on account of a huge occipital mass and microcephaly. The CECT showed a huge cystic mass with brain density substance and cerebrospinal fluid density fluid. Conservative management which includes the parent's reassurance, supportive care, symptom management, and close monitoring plays a role in their nonsurgical management.

Keywords: Encephalocele, giant, infant, occipital


How to cite this article:
Baba MS, Gele IH, Ahmed SS, Ma'aji SM. A giant occipital encephalocele in an infant. Sahel Med J 2020;23:242-4

How to cite this URL:
Baba MS, Gele IH, Ahmed SS, Ma'aji SM. A giant occipital encephalocele in an infant. Sahel Med J [serial online] 2020 [cited 2021 Mar 4];23:242-4. Available from: https://www.smjonline.org/text.asp?2020/23/4/242/310025




  Introduction Top


Encephaloceles are neural tube defects that are characterized by protrusion of brain and meninges through a defect in the cranium.[1] It is also defined as the protrusion of brain substance connected with the rest of the brain by a pedicle, which occurs due to herniation through a dehiscence or defect in the surrounding bone.[2]

Encephaloceles are very rare with an incidence of about 1 in 5000 live births.[3],[4] This has a female preponderance with an unknown etiology.[3],[5]

This is a case of an infant with a huge occipital swelling, delayed developmental milestones, and microcephaly.


  Case Report Top


A 3-month-old female infant was referred by the neurosurgical unit of the hospital to the department of radiology for a contrast-enhanced computed tomography (CECT) of the brain on account of a huge occipital mass and microcephaly (the head circumference, 19.5 cm).

The patient had not achieved neck control and social smile with poor suckling at the age of 3 months. There was no history of seizures or syncopal attack since birth.

On physical examination, this was the case of a female infant with no neck control, not pale, not dehydrated, and with an intact mental state. She had no obvious limb nor body abnormality and no feature to suggest paralysis of any limb. The patient had no abnormal eye movements or reflexes. A huge mass located posteriorly in the occipital region of the head measuring about 75mm x 40mm x 62mm in craniocaudal, anteroposterior and mediolateral dimensions respectively was also noted (See [Figure 1]).
Figure 1: A plain radiograph showing a huge soft tissue density mass arising from the skull vault posteriorly

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Following CECT, a huge sac-like structure was demonstrated in the occipital region of the skull vault where a bony defect was also noted. Brain density substance and cerebrospinal fluid (CSF) were the contents of this mass. This is the encephalocele [see [Figure 2] and [Figure 3]]. Poor gyri formation with dysmorphic ventricles were also demonstrated [see [Figure 4]]. No coexisting hydrocephalus was however demonstrated. No any other herniation was noted.
Figure 2: Bone window following computed tomography scan reconstructed sagittal plane showing a defect in the occipital skull bone with a huge sac-like extrusion containing cerebrospinal fluid density fluid, meninges, and brain substance

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Figure 3: Reconstructed coronal plane of a bone window computed tomography showing a huge sac-like structure posteriorly containing cerebrospinal fluid density fluid, meninges, and brain parenchyma

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Figure 4: Computed tomography brain window axial plane at the level of the lateral ventricles showing dysmorphic ventricles, absent sulci, and gyri

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The patient was still on admission in the pediatric neurosurgery ward and being managed conservatively by reassuring the parent, supportive care, symptom management, and close monitoring of the patient with preparation for cranioplasty and corrective surgery of the encephalocele at a later period at the time of this case report. The patient was lost to follow-up much later and all efforts to get in touch failed.


  Discussion Top


Occipital encephaloceles represent about 85% of lesions in the Western Hemisphere.[3],[6] This index case is also in the occipital region conforming to the above facts. Encephaloceles are more preponderant in the female gender; this case happens to be a female.

The word giant encephalocele is used when the mass appears larger than the head; the occipital encephalocele in this patient is bigger than the patient's head, which is microcephalic. Most encephaloceles have no known etiology; the same is also applicable in this patient where a cause is not known.

There is often association with delayed/poor developmental milestone; this patient had not achieved neck control and social smile with poor suckling at the age of 3 months.

Patients with encephaloceles do have associated central nervous system anomalies; this patient showed dysmorphic ventricles and poor gyrilation. Ventriculomegaly is not a common association in encephalocele; this patient had no ventriculomegaly following CECT.

Patients with encephaloceles and coexistent hydrocephalus do have a far worse neurological outcome;[7] the absence of hydrocephalus in this patient may be the reason for a better neurological outcome in this index patient.

Encephaloceles are sac-like structures with brain tissue and CSF that herniated through bone defects;[1],[2],[3],[4],[5],[6],[7] these were the findings noted following CECT in this patient.


  Conclusion Top


Occipital encephaloceles are the most common form of encephalocele predominantly seen in females, depending on the size and presence or absence of coexisting hydrocephalus, conservative management like parents reassurance, patients supportive care, symptom management and close monitoring plays vital role in their nonsurgical management.

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.



 
  References Top

1.
Mahajan C, Rath GP, Dash HH, Bithal PK. Perioperative management of children with encephalocele: An institutional experience. J Neurosurg Anesthesiol 2011;23:352-6.  Back to cited text no. 1
    
2.
Williams DC. Encephalocele of the middle ear. J Laryngol Otol 1986;100:471-3.  Back to cited text no. 2
    
3.
Yιldιrιm ZB, Avci E, Torun F, Cengiz M, Cigdem A, Karabağ H, et al. Airway management for occipital encephalocele in neonatal patients: A review of 17 cases. J Neurosci Rural Pract 2011;2:159-61.  Back to cited text no. 3
    
4.
Walia B, Bhargava P, Sandhu K. Giant occipital encephalocele. Med J Armed Forces India 2005;61:293-4.  Back to cited text no. 4
    
5.
Padmanabhan R. Etiology, pathogenesis and prevention of neural tube defects. Congenit Anom (Kyoto) 2006;46:55-67.  Back to cited text no. 5
    
6.
Shimizu T, Kitamura S, Kinouchi K, Fukumitsu K. A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity. Paediatr Anaesth 1999;9:73-6.  Back to cited text no. 6
    
7.
Da Silva SL, Jeelani Y, Dang H, Krieger MD, McComb JG. Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele. J Neurosurg Pediatr 2015;15:392-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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   Abstract
  Introduction
  Case Report
  Discussion
  Conclusion
   References
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