Key-words:
Anesthetic implications - giant encephalocele - prognosis in encephalocele
Introduction
Giant encephalocoele are rare entity and pose unique challenges to Neurosugeons, Anesthetists,
and Neuroradiologists. There is only one case series and few case reports about this
in literature.[[1]] We report to you a rare case of giant encephalocele with delayed motor milestones
which gradually improved following surgery.
Case Report
We report to you a 6-month-old child who presented with the swelling in the occipital
region from birth which gradually increased in size [[Figure 1]]. It was a home delivery with no antenatal check-up in rural eastern India. It was
a of 26 cm × 34 cm × 30 cm soft translucent, containing fluid. No e/o cerebrospinal
fluid (CSF) leak. On examination, she had delayed motor milestones in the form of
inability to hold her head. Breathing, feeding, lower spine were unremarkable. Rest
of the neurological examination was unremarkable. She was weighing 4.5 kg before surgery.
Magnetic resonance imaging (MRI) revealed a large encephalocoele with small amount
of neural tissue herniating into the sac with gross hydrocephalus and cervicothoracic
syrinx [[Figure 2]]. Anesthetic management of children with giant encephalocoels present challenges
with regard to patient positioning, airway management, temperature monitoring, and
estimating blood and fluid loss.[[2]] In our case, the encephalocoele was so big that it was not possible to intubate
the child with the head supine as the giant encephalocoele 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]]. On the operating table, encephalocoele was first drained with replacement of fluids
to prevent volume loss. We did ventriculoperitoneal shunt followed by opening of the
encephalocoele, excision of the redundant neural tissue, and primary closure of the
dura. Skin was reconstructed. One year follow-up, she is able to hold her head, sit
without support and mental milestones are grossly normal for her age.
Figure 1: Large occipital encephalocoele
Figure 2: Magnetic resonance imaging showing large encephalocoele with small amount of cerebellar
tissue herniating with gross hydrocephalus and cervicothoracic syrinx
Figure 3: Assistant supporting the patient's head with the giant encephalocoele as the head
is positioned beyond the edge of the table for intubation
Discussion
Giant encephalocoeles are rare phenomenon 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 encepholocoeles.[[1]],[[2]],[[3]],[[4]] Authors feel that they should be called real giant when the encephalocoele size
reaches head size. Most of the children are neonates with chief complaints being enlarging
swelling with difficulty to feed.[[1]],[[4]],[[5]] They may have microcephaly, craniosynostosis, cleft lip or associated brain anomalies
such as microgyria, polygyria, chiari malformation, and hydrocephalus.[[1]],[[2]],[[3]],[[4]],[[5]],[[6]] 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 remaining
viable tissue should be placed back weighing the risk of coming.[[6]],[[7]],[[8]],[[9]] Dura should be repaired with pericranium or artificial dura. Large bone defects
to be covered with methylmethacrylate. Associated microcephaly craniosynostosis, hydrocephalus,
chiari malformation, syrinx may require treatment.[[1]],[[6]],[[7]],[[8]] Postoperatively, one has to look for hypothermia, raised intracranial pressure,
apnea, cardiac arrest, CSF leak, and infection.[[6]],[[7]],[[8]],[[9]] In our case, maybe child had not attained head holding due to the weight of the
sac. She is now head holding with mild ataxic gait. Rest of the examination is unremarkable.
Patient 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.[[7]]
Conclusion
Giant encephaloceles are rare but challenging entities requiring multidisciplinary
approach. Patients with less amount of brain tissue in the sac, no severe neurological
deficits carry a good prognosis
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form, the legal guardian has given his consent for images and other clinical
information to be reported in the journal. The guardian understands that names and
initials will not be published and due efforts will be made to conceal identity, but
anonymity cannot be guaranteed.
