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Ahead of print publication

Giant encephaloceles: Are always challenging task?

1 Department of Neurosurgery and Spine Surgery, Hyderabad, India
2 Department of Pediatrics, Hyderabad, India
3 Department of Radiology, Hyderabad, India
4 Department of Anaesthesia, SLG Hospital Bachupally, Hyderabad, India
5 Department of Anaesthesia, MNJ RCC Hospital, Hyderabad, India

Date of Submission12-Apr-2021
Date of Decision24-Feb-2022
Date of Acceptance13-Oct-2021
Date of Web Publication30-Jan-2023

Correspondence Address:
Raghavendra Harpanahalli,
Department of Neurosurgery and Spine Surgery, SLG Hospital Bachupally, Hyderabad, India.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_84_21



Giant encephaloceles are rare entities with only one case series and few case reports reported in the medical literature. Encephaloceles which reach a size larger than the head size are called giant encephaloceles. We report a case of a 6-month-old child who had giant encephalocele with delayed motor milestones in the form of inability to hold neck. Because of their enormous size, they pose a surgical challenge. Anesthetic implications include difficulty in securing airway without undue pressure on the sac. The baby underwent ventriculoperitoneal shunt followed by excision of the encephalocele sac with repair. We report this case to highlight the difficulties in the management of giant occipital encephaloceles.

Keywords: Anesthetic implications, giant encephalocele, prognosis in encephalocele

How to cite this URL:
Harpanahalli R, Kumar MA, Prayaga J, Srinivas V, Arun TM, Joginipally SR, Reddy AS, Varsha KS. Giant encephaloceles: Are always challenging task?. J Pediatr Neurosci [Epub ahead of print] [cited 2023 Dec 2]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=368818

   Introduction Top

Occipital encephaloceles can vary from a small pedunculated swelling in a narrow neck to an extremely large swelling. In one study, up to 16% of the occipital encephaloceles were more than 20 cm in diameter.[1] In giant occipital encephaloceles, the size of the swelling is larger than the size of the head from which they arise.[2],[3],[4],[5],[6] There is only one case series and few case reports about this in the literature.[1] We report this case to highlight the difficulties in the management of giant occipital encephaloceles.

   Case Study Top

A 6-month-old female baby presented with progressively increasing swelling over the occipital region since birth. The baby was born at 37 weeks of gestation by lower segment cesarean section with a birth weight of 3 kg with normal cry with cystic swelling since birth. There was no abnormality on physical examination except for a large soft (34*40*38cms) cystic multilobulated swelling arising from occipital pedunculated mass in the occipital region. It was larger than the size of the head [Figure 1]. The skin over the swelling was stretched with multiple healed ulcers. The anterior fontanelle was bulging. She had delayed motor milestones in the form of inability to hold her head. Breathing, feeding, and lower spine were unremarkable. The baby was not able to track objects and light, and pupils were reactive. Routine hematological and biochemical investigations were reported as normal. Magnetic resonance imaging (MRI) revealed a large encephalocele with small amount of neural tissue herniating into the sac with severe dilated supraventricular system with partial absence of intraventricular septum and syringomyelia in the cervical region [Figure 2]. Surgery was planned to treat hydocephalus and excise the sac. Anesthetic management of children with giant encephaloceles presents challenges with regard to patient positioning, airway management, temperature monitoring, and estimating blood and fluid loss. In our case, the encephalocele was so big that it was not possible to intubate the child with the head supine as the giant encephalocele limited head extension severely and also there was the risk of rupturing the sac with sudden uncontrolled third space volume loss. We were able to intubate the child by placing the child’s head beyond the edge of the operating table and supported by an assistant [Figure 3]. Initially, ventriculoperitoneal shunt was done, and subsequently, the baby was positioned in the lateral position. A circumferential incision was placed over the sac, and the neck was dissected out. Sac was then opened. The herniated brain tissue looking redundant was excised. The sac was reduced in size and repaired. Normal dural margins were identified by nibbling the bone margins; dural defect was repaired from excised sac graft along with dural regeneration matrix with tissue sealants [Figure 4]. The skin was closed with interrupted sutures. The baby remained well during follow-up.
Figure 1: A large soft cystic occipital giant encephalocele

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Figure 2: MRI brain; large encephalocele

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Figure 3: Difficult intubation

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Figure 4: Dural Regeneration Matrix

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   Discussion Top

Giant encephaloceles are rare phenomena with only few case reports being reported.[1] Only one case series has been reported until the date of 14 cases.[1] They are known by different names such as giant massive or large encepholoceles.[1],[7],[8],[9] The authors feel that they should be called real giant when the encephalocele size reaches the head size. Most of the children are malformation and hydrocephalus.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] MRI with magnetic resonance venography is the investigation of choice, with computed tomography to look for bony defects.[1] One has to carefully examine the contents of the sac for any torcula or veins herniation. Most of the contents are atretic and can be excised; however, torcula and the remaining viable tissue should be placed back weighing the risk of coming.[10],[11],[12],[13] Dura should be repaired with pericranium or artificial dura like Dural Regeneration Matrix, which is an easy-to-handle, soft, pliable, non-friable, porous collagen matrix, when used is known to reduce 8.3% CSF leak rate.[14]

Large bone defects to be covered with methyl methacrylate. Associated microcephaly craniosynostosis, hydrocephalus, chiari malformation, and syrinx may require treatment.[1],[8],[11],[12] Postoperatively, one has to look for hypothermia, raised intracranial pressure, apnea, cardiac arrest, CSF leak, and infection.[10],[11],[12],[13] In our case, maybe baby had not attained head holding due to the weight of the sac. She is now able to turn her head. Rest of the examination is unremarkable. Patients with a large amount of cerebrum, cerebellum, and brain stem herniating into sac have a poor prognosis. Irrespective of the sac size, patients with less amount of brain tissue in the sac and good preoperative neurological condition carry a good prognosis.[11]

   Conclusion Top

Giant encephaloceles are rare but challenging entities requiring multidisciplinary approach. Patients with less amount of brain tissue in the sac and no severe neurological deficits carry a good prognosis; neonates chief complaints being enlarging swelling with difficulty to feed.[1],[5],[14]

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Mahapatra AK. Giant encephalocele: A study of 14 patients. Pediatr Neurosurg 2011;47:406-11.  Back to cited text no. 1
Agrawal A, Lakhkar BB, Lakhkar B, Grover A. Giant occipital encephalocele associated with microcephaly and micrognathia. Pediatr Neurosurg 2008;44:515-6.  Back to cited text no. 2
Lettau M, Halatsch ME, Hähnel S. [Occipital giant encephalocele]. Rofo 2007;179:971-2.  Back to cited text no. 3
Ozlü O, Sorar M, Sezer E, Bayraktar N. Anesthetic management in two infants with giant occipital encephalocele. Paediatr Anaesth 2008;18:792-3.  Back to cited text no. 4
Agrawal D, Mahapatra AK. Giant occipital encephalocele with microcephaly and micrognathia. Pediatr Neurosurg 2004;40:205-6.  Back to cited text no. 5
Bayramiçli M, Sönmez A, Yavaş T. Microvascular reconstruction of a giant encephalocele defect in a 10-week-old infant. Br J Plast Surg 2004;57:89-92.  Back to cited text no. 6
Bozinov O, Tirakotai W, Sure U, Bertalanffy H. Surgical closure and reconstruction of a large occipital encephalocele without parenchymal excision. Childs Nerv Syst 2005;21:144-7.  Back to cited text no. 7
Cevik B, Orskiran A, Yilmaz M, Ekti Y. Anesthetic management of a newborn with giant occipital meningoencephalocele: Case report. Int J Case Rep Images 2012;3:10-2.  Back to cited text no. 8
Shokunbi T, Adeloye A, Olumide A. Occipital encephalocoeles in 57 Nigerian children: A retrospective analysis. Childs Nerv Syst 1990;6:99-102.  Back to cited text no. 9
Chapman PH, Swearingen B, Caviness VS. Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management. J Neurosurg 1989;71:375-81.  Back to cited text no. 10
Agarwal A, Chandak AV, Kakani A, Reddy S. A giant occipital encephalocele. APSP J Case Rep 2010;1:16.  Back to cited text no. 11
Mahapatra AK, Gupta PK, Dev EJ. Posterior fontanelle giant encephalocele. Pediatr Neurosurg 2002;36:40-3.  Back to cited text no. 12
Mohanty A, Biswas A, Reddy M, Kolluri S. Expansile cranioplasty for massive occipital encephalocele. Childs Nerv Syst 2006;22:1170-6.  Back to cited text no. 13
Varun RK, Bjorn L, Joshua L, Burak S, Soichi O, Joung HL, Evaluation of non-watertight dural reconstruction with collagen matrix onlay graft in posterior fossa surgery. J Korean Neurosurg Soc 2016;59:52-57.  Back to cited text no. 14


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


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