<%server.execute "isdev.asp"%> A giant occipital encephalocele in neonate with spontaneous hemorrhage into the encephalocele sac: Surgical management Satyarthee GD, Moscote-Salazar LR, Escobar-Hernandez N, Aquino-Matus J, Puac-Polanco PC, Hoz SS, Calderon-Miranda WG - J Pediatr Neurosci
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Year : 2017  |  Volume : 12  |  Issue : 3  |  Page : 268-270

A giant occipital encephalocele in neonate with spontaneous hemorrhage into the encephalocele sac: Surgical management

1 Department of Neurosurgery, Neurosciences Centre, All India Institutes of Medical Sciences, New Delhi, India
2 RED LATINO, Latin American Trauma and Intensive Neuro-Care Organization, Bogota, Colombia
3 Department of Radiology, Hospital Gea Gonzalez, Universidad Nacional Autonoma de Mexico, Ciudad de, Mexico
4 Department of Internal Medicine, Hospital Gea Gonzalez, Universidad Nacional Autonoma de Mexico, Ciudad de, Mexico
5 Department of Neuroradiology, UNC, Chapel Hill, USA
6 Department of Neurosurgery, Neurosurgery Teaching Hospital, Baghdad, Iraq

Date of Web Publication14-Nov-2017

Correspondence Address:
Luis Rafael Moscote-Salazar
Red Latino. Latin American Trauma and Intensive Neuro-Care Organization, Bogota
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_6_17

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The presence of giant occipital encephalocele represents a surgical challenge. However, preoperative magnetic resonance imaging with venography can help in delineating relation of venous sinus, content of the sac and help classify occipital encephalocele into infra-torcular and torcular depending on the relation with position of torcula. However, the presence of old hemorrhage into encephalocele sac is extremely rare and in the detailed PubMed search, the authors could find one such case, reported by Nath et al. The author reports a case of giant occipital encephalocele; during surgery, evidence of old bleed was noted. Pertinent literature and management are reviewed briefly.

Keywords: Giant encephalocele, magnetic resonance imaging, neural tube defects, occipital spontaneous hemorrhage, surgical repair

How to cite this article:
Satyarthee GD, Moscote-Salazar LR, Escobar-Hernandez N, Aquino-Matus J, Puac-Polanco PC, Hoz SS, Calderon-Miranda WG. A giant occipital encephalocele in neonate with spontaneous hemorrhage into the encephalocele sac: Surgical management. J Pediatr Neurosci 2017;12:268-70

How to cite this URL:
Satyarthee GD, Moscote-Salazar LR, Escobar-Hernandez N, Aquino-Matus J, Puac-Polanco PC, Hoz SS, Calderon-Miranda WG. A giant occipital encephalocele in neonate with spontaneous hemorrhage into the encephalocele sac: Surgical management. J Pediatr Neurosci [serial online] 2017 [cited 2021 Apr 15];12:268-70. Available from: https://www.pediatricneurosciences.com/text.asp?2017/12/3/268/218243

   Introduction Top

Encephalocele results from an anomaly of embryological mesodermal development lead to the genesis of a calvarial and dura defect, which may be associated with herniation of meninges, cerebrospinal fluid (CSF), and neural tissue. Occipital encephalocele represents the most common variety of all encephalocele. In the giant variety of encephalocele, characteristically, the head size remains smaller than the encephalocele sac.[1] Authors report a case of giant occipital encephalocele with evidence of old hemorrhage in the sac, observed intraoperatively. The incidence of encephalocele is approximately one per five thousand live births.[2]

   Case Report Top

A 2-day-old neonate was brought to neurosurgical outpatient services by parents with a complaint of the presence of a large swelling in the occipital region but no associated paucity of limb movement or ulceration or discharge. On the admission, examination revealed a healthy neonate with stable vitals, anterior fontanelle laxity, with spontaneous crying and moving all limbs as well. Local examination revealed the presence of a large globular swelling with healthy skin located in the occipital region with extension into the neck and parietal region, with a circumference of swelling was 30 cm. The consistency was smooth, which used to become prominent on crying, cystic, fluctuant. However, no evidence of bruit or murmur over the swelling was noted. The transillumination test was negative.

The hematological and biochemistry were within normal limits. Axial ultrasound shows occipital encephalocele [Figure 1]. Magnetic resonance imaging (MRI) brain angiography showed large occipital encephalocele containing predominantly cystic content and small amount of neural tissue of the occipital lobe; however, no herniation of torcula or cerebellum was seen [Figure 2] and [Figure 3].
Figure 1: Axial ultrasound showing occipital encephalocele

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Figure 2: Magnetic resonance imaging of brain showing an occipital encephalocele

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Figure 3: Magnetic resonance imaging postoperative showing correction of defects

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He was operated in a prone position with cotton wrapped over limbs to maintain normothermia. A transverse scalp incision was made and the flap was raised. The bone defect was identified and measured 3 cm × 3 cm, and the meningeal sac was identified and opened which predominantly contained hemorrhagic CSF with small gliosed occipital lobe. The gliosed neural tissue was resected, redundant dura was excised, and dural closure was done. Skin closure was done in layers. He had an uneventful postoperative course.

   Discussion Top

Occipital encephalocele is more common in female than male. Occipital encephalocele occurs between lambda and foramen magnum typically in the midline and divided into supra- and infra-torcular types.[3],[4] Occipital encephalocele is more common than anterior in western hemisphere accounting for about 85%.[4] The content of occipital encephalocele may include meninges, occipital lobes, or ventricles and rarely contain cerebellum, brainstem, or torcula.[5] Typically, bony defect may be only confined to only occipital bone or may descend down to involve defect of the posterior lip of foramen magnum and even up to the posterior arch of the  Atlas More Details and accordingly divided into supra- and infra-torcular types.[3] The size of occipital encephalocele may vary from small, large to giant. It may be associated with bony as well as a neural anomaly. Cortical dysplasia and corpus callosum agenesis are frequently present.[4]

The incidence of hydrocephalus is higher in posterior encephalocele. The herniated neural tissue, usually remains gliosed, and dysplastic and additional presence of microcephaly may cause difficulty in reposition of herniated neural tissue with increased chances of aggravation of hydrocephalus in the postoperative period.[6] Further, torcula as a content poses a great challenge as its injury may lead to cerebral deep venous system thrombosis.[5] About 60% posterior encephalocele may develop hydrocephalus requiring surgical management.[3]

Hydrocephalus may develop due to torsion of the aqueduct of sylvius or aqueductal stenosis. Hydrocephalus may also develop following surgical repair of the encephalocele due to changes in the CSF dynamics in the postoperative period.

Management of occipital encephalocele presents a challenge for the neurosurgeons.[3] However, our case had approximately 800 ml of dark hemorrhagic fluid, which may be due to bleeding from necrotized herniated occipital lobe with ulcerated cortical surface that might be contributed to the hemorrhage in the sac and further compounded by prolonged labor.

MRI head clearly delineates the contents of sac and presence of associated hydrocephalus, relation to adjoining sinus. However, magnetic resonance venography may reveal dilated venous sinuses. Visual evoked response is often used as part of diagnostic workup in cases of giant occipital encephalocele to evaluate about sac contents regarding functional visual cortex. Adequate intraoperative care may avoid development of hemianopia and cortical blindness.[7]

Surgery is advocated in cases suffering from an occipital encephalocele for the sake of cosmoses, to avert the risk of rupture, CSF leak, and meningitis. The patient is positioned in a prone position with special emphasis on care to avoid hypothermia, minimizing the blood loss, and monitoring of electrolytes is of paramount importance.[1],[3],[8]

Before incision, aspiration of CSF may aid in the dissection of sac, especially in large or giant encephalocele. A transverse incision is ideal for circular encephalocele with a small defect in the occipital bone, but the vertical incision is more safe and suitable for encephalocele extending above and below the posterior fossa, and when there is a need to separate the sac from the flap. Mahapatra and Agrawal advocated preservation of neural element and vascular elements; however, Walia et al. suggested although gliosed and ischemic herniated neural tissue can be excised; however, great caution is required to preserve venous sinus that may course through it.[9] Repair of dural defect can be done using the pericranium as a graft with a two-layered watertight closure of the dura is better. Accordingly, there is no need to cover bone defect with a cortical bone in infants and neonates.[8] In the postoperative period, the possibility of aggravation of hydrocephalus should be kept in mind and its occurring may need shunt surgery.[3],[10]

   Conclusion Top

Giant occipital encephalocele is an extremely rare occurrence condition that needs special care regarding temperature maintenance, minimizing intraoperative blood loss, maintenance of electrolytes balance, and blood sugar levels, all are of paramount importance. However, the presence of hemorrhagic fluid in the encephalocele sac is rare, and the current case represents the second case in the western literature. Authors recommend that if encephalocele detected during antenatal checkup, then the mother should undergo delivery by caesarian section without a trial of induction of labor as an uncontrolled bleeding potential into the encephalocele sac may lead to a very rapid increase in the size with raised intracranial pressure, leading to life-threatening consequences. Hence, awareness of such condition to the obstetrician, pediatrician, physician, and neurosurgeon is highly warranted and recommended.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Nath HD, Mahapatra AK, Borkar SA. A giant occipital encephalocele with spontaneous hemorrhage into the sac: A rare case report. Asian J Neurosurg 2014;9:158-60.  Back to cited text no. 1
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Mealey J Jr., Dzenitis AJ, Hockey AA. The prognosis of encephaloceles. J Neurosurg 1970;32:209-18.  Back to cited text no. 2
Verma SK, Satyarthee GD, Singh PK, Sharma BS. Torcular occipital encephalocele in infant: Report of two cases and review of literature. J Pediatr Neurosci 2013;8:207-9.  Back to cited text no. 3
[PUBMED]  [Full text]  
Bindal AK, Storrs BB, McLone DG. Occipital meningoceles in patients with the dandy-walker syndrome. Neurosurgery 1991;28:844-7.  Back to cited text no. 4
Humphreys RP. Encephalocele and dermal sinuses. In: Cheek WR, Martin AE, McLone DG, Reigel D, Walker M, editors. Pediatric Neurosurgery: Surgery of the Developing Nervous System. 3rd ed. Philadelphia: Saunders; 1994. p. 96-103.  Back to cited text no. 5
Lorber J. The prognosis of occipital encephalocele. Dev Med Child Neurol 1967;Suppl 13:75-86.  Back to cited text no. 6
Mahapatra AK, Agrawal D. Anterior encephaloceles: A series of 103 cases over 32 years. J Clin Neurosci 2006;13:536-9.  Back to cited text no. 7
Mahapatra AK. Skull base anatomy, radiology and management. In: Samii M, editor. Skull Base Anatomy, Radiology and Management. Basel: S. Karger; 1994. p. 220-3.  Back to cited text no. 8
Walia B, Bhargava P, Sandhu K. Giant occipital encephalocele. Med J Armed Forces India 2005;61:293-4.  Back to cited text no. 9
Caviness VS Jr., Evarard P. Occipital encephalocele: A pathologic and anatomic analysis. Acta Neuropathol 1975;32:245-55.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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