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Year : 2019  |  Volume : 8  |  Issue : 3  |  Page : 176-177

Double encephalocele arising from single bone defect: A rare case

Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Web Publication6-Aug-2019

Correspondence Address:
Dr. Aditya Pratap Singh
Near the Mali Hostel, Main Bali Road, Falna, Pali District - 306 116, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_10_19

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Encephalocele is the protrusion of the cranial contents beyond the normal confines of the skull through a defect in the calvarium. The occurrence of more than one encephalocele in a patient is very rare, and very few cases of double encephalocele have been reported. We report here a case of double encephalocele over the occipital area in a newborn. At the time of surgery, we found that both were arising from a single bone defect with separate sac. Both were repaired, and the patient was discharged uneventfully.

Keywords: Double, encephalocele, newborn

How to cite this article:
Garg D, Singh AP, Tanger R, Gupta AK. Double encephalocele arising from single bone defect: A rare case. J Clin Neonatol 2019;8:176-7

How to cite this URL:
Garg D, Singh AP, Tanger R, Gupta AK. Double encephalocele arising from single bone defect: A rare case. J Clin Neonatol [serial online] 2019 [cited 2020 Aug 15];8:176-7. Available from: http://www.jcnonweb.com/text.asp?2019/8/3/176/264030

  Introduction Top

Encephalocele is a congenital malformation characterized by protrusion of the brain tissue due to a skull defect.[1] Based on the location of the skull defect, they are classified into sincipital, basal, occipital, or parietal varieties. It is a neural tube defect. Other neural tube defects are anencephaly and spina bifida. Neural tube defects are caused by the abnormal closure of the embryonic neural tube between 22 and 28 days after conception. The resulting structural defects, which may occur anywhere along the neuraxis.[2],[3]

  Case Report Top

A newborn male child came in an emergency with a complaint of scalp swelling since birth. On examination, we found two swellings in the occipital region [Figure 1]. The sizes of both swellings were 4 cm × 4 cm and 2 cm × 2 cm. There were no neurological deficits preoperatively. There was no family history. Routine blood investigations were within normal limits. The patient was admitted and prepared for surgery. The size of the defect was around 2 cm × 1.5 cm. we tried to reduce the content but could not possible. Decapping and repair were done with resection of the mass. During the surgery, we found that both swellings were arising from a single bone defect with separate sac. Both sacs were repaired separately. Postoperatively, the patient did well and discharged uneventfully. There have been no neurological deficits or hydrocephalus in follow-up for the last 1 year.
Figure 1: Photograph showing double occipital encephalocele

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  Discussion Top

The main cause of neural tube defects are abnormalities that occur during neurulation, which should be complete by 4 weeks postconception.[4] The occipital region is the most common location (75%), and encephaloceles in this region are frequently associated with other malformations such as agenesis of the corpus callosum, Chiari II malformation, and Dandy–Walker syndrome.[5]

Double encephalocele is very rare and only few cases are reported.[6],[7] In all reported cases, patients had separate bone defect. Our case is unique because both sacs were arising from a single bone defect.

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.


We would like to acknowledge Dr. Neelam Dogra, MD Anaesthesia, Senior Professor, SMS Medical College Jaipur.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Suwanwela C, Sukabote C, Suwanwela N. Frontoethmoidal encephalomeningocele. Surgery 1971;69:617-25.  Back to cited text no. 1
Grillo E, da Silva RJ. Neural tube defects and congenital hydrocephalus. Why is prevalence important?. J Pediatr (Rio J) 2003;79:105-6.  Back to cited text no. 2
Tamura T, Picciano MF. Folate and human reproduction. Am J Clin Nutr 2006;83:993-1016.  Back to cited text no. 3
American Academy of Pediatrics, Committee on Genetics. Folic acid for the prevention of neural tube defects. Pediatrics 1999;104:325-7.  Back to cited text no. 4
Kosnik EJ, Meagher JN, Quenemoen LR. Parietal intradiploic encephalocele. Case report. J Neurosurg 1976;44:617-9.  Back to cited text no. 5
Canaz H, Ayçiçek E, Akçetin MA, Akdemir O, Alataş I, Özdemir B. Supra- and infra-torcular double occipital encephalocele. Neurocirugia (Astur) 2015;26:43-7.  Back to cited text no. 6
Sharma S, Ojha BK, Chandra A, Singh SK, Srivastava C. Parietal and occipital encephalocele in same child: A rarest variety of double encephalocele. Eur J Paediatr Neurol 2016;20:493-6.  Back to cited text no. 7


  [Figure 1]


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