1. Case report on Excision of
an Extreme Encephalocele
Mahmood Hassan, MD, PhD.Mahmood Hassan, MD, PhD.
Consultant NeurosurgeonConsultant Neurosurgeon
Royal Commission Hospital,Royal Commission Hospital,
Jubail, KSAJubail, KSA

2. EncephaloceleEncephalocele
 Encephaloceles are rare (1~4/10000)Encephaloceles are rare (1~4/10000)
neural tube defects where brain tissueneural tube defects where brain tissue
protrudes through abnormal openings inprotrudes through abnormal openings in
the skull; 75% are occipital.the skull; 75% are occipital.
 Often accompanied by deformities of theOften accompanied by deformities of the
skull or face and/or brain malformations.skull or face and/or brain malformations.
 Symptoms may include hydrocephalus,Symptoms may include hydrocephalus,
spastic quadriplegia, developmentalspastic quadriplegia, developmental
delay, vision problems, mental anddelay, vision problems, mental and
growth retardation and seizures.growth retardation and seizures.

3. EncephaloceleEncephalocele
With flexed neck & head fromWith flexed neck & head from
encephalocele.encephalocele. At 4 months

4. Extreme encehaloceleExtreme encehalocele
Left parieto-occipetal encephalocele, excised without development of
any neurological deficit other than mild limbic spasticity

5. Prof. Samii reviewed the case…Prof. Samii reviewed the case…
……you are dealing withyou are dealing with
an extreme encephalocelean extreme encephalocele

6. Methods:Methods:
Cranial Ultrasound of theCranial Ultrasound of the
sac revealed cystic structuressac revealed cystic structures
Through study ofThrough study of
accompaniedaccompanied
anomalies,anomalies,
Neuorological andNeuorological and
radiologicalradiological
examinations wereexaminations were
undertakenundertaken

7. Methods:Methods:
CT scan BrainCT scan Brain
helped assessing thehelped assessing the
lesion;lesion;
Size & content of theSize & content of the
sac are the mostsac are the most
important prognosticimportant prognostic
factors.factors.

8. Methods:Methods:
Brain MRI was carried out to assess the presence of otherBrain MRI was carried out to assess the presence of other
anomalies and vascularity.anomalies and vascularity.

9. Treatment ofTreatment of
EncephaloceleEncephalocele
Immediate surgical excision &Immediate surgical excision &
Closure is the rule with-Closure is the rule with-
Excision of nonviable neural tissueExcision of nonviable neural tissue
in the sac & watertight dural closure.in the sac & watertight dural closure.
Bony defect does not warrant repair.Bony defect does not warrant repair.
60% develop post-op hydrocephalus;60% develop post-op hydrocephalus;
33rdrd
ventriculostomy or shuntventriculostomy or shunt
placement is essential.placement is essential.

10. Steps of surgical resection &Steps of surgical resection &
repairrepair
 Incise dome of the sac along side wallIncise dome of the sac along side wall
leaving enough tissue to close skinleaving enough tissue to close skin
 Dissect cerebral tissue from fused dura &Dissect cerebral tissue from fused dura &
skinskin
 Identify dural defect & neural stalkIdentify dural defect & neural stalk
 Isolating stalk from the dural tunnel wallIsolating stalk from the dural tunnel wall
 Transect the stalk avoiding vessels onTransect the stalk avoiding vessels on
passagepassage
 Repair of dural defectRepair of dural defect
 Repair bony defect with split calverial graftRepair bony defect with split calverial graft

11. Excised EncephaloceleExcised Encephalocele
Excision after drainage of the altered colored CSF was done and contentsExcision after drainage of the altered colored CSF was done and contents
were examined. Samples were sent for histopathologic evaluationwere examined. Samples were sent for histopathologic evaluation

12. Outcome &Outcome &
ConclusionConclusion
High mortality; 29% n recent seriesHigh mortality; 29% n recent series
(Kiymaz et al Pedatr Neurosurg 2010)(Kiymaz et al Pedatr Neurosurg 2010)
Higher severity of mental retardation.Higher severity of mental retardation.
French BN reported normal developmentFrench BN reported normal development
In 17% & physical delay, mental retard-In 17% & physical delay, mental retard-
ation in 83%.ation in 83%. Youmans JR: Neurological Surgery 1990.Youmans JR: Neurological Surgery 1990.
Our patient is alive 15 months withOur patient is alive 15 months with
Visual and other issues in growth.Visual and other issues in growth.

13. Big Boy!Big Boy!
The parents named him:The parents named him:
ARAB!ARAB!
The goal of treatmentThe goal of treatment
for NTDs is to allow thefor NTDs is to allow the
individual to achieveindividual to achieve
the highest level ofthe highest level of
function andfunction and
independence.independence.
Arab at 9 months