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

EncephaloceleEncephaloceleEncephaloceles are rare (1~4/10000) Encephaloceles are rare (1~4/10000)

neural tube defects where brain tissue neural tube defects where brain tissue protrudes through abnormal openings in protrudes through abnormal openings in the skull; 75% are occipital.the skull; 75% are occipital.

Often accompanied by deformities of the Often 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, developmental spastic quadriplegia, developmental delay, vision problems, mental and delay, vision problems, mental and growth retardation and seizures.growth retardation and seizures.

EncephaloceleEncephalocele

With flexed neck & head from With flexed neck & head from encephalocele.encephalocele. At 4 months

Extreme encehaloceleExtreme encehalocele

Left parieto-occipetal encephalocele, excised without development of any neurological deficit other than mild limbic spasticity

Prof. Samii Prof. Samii reviewed the reviewed the

case…case…

……you are dealing with you are dealing with an extreme encephalocelean extreme encephalocele

Methods:Methods:

Cranial Ultrasound of the Cranial Ultrasound of the sac revealed cystic structuressac revealed cystic structures

Through study of Through study of accompanied accompanied anomalies,anomalies,Neuorological and Neuorological and radiological radiological examinations were examinations were undertakenundertaken

Methods:Methods:CT scan Brain CT scan Brain helped assessing helped assessing the lesion;the lesion;

Size & content of Size & content of the sac are the the sac are the most important most important prognostic factors.prognostic factors.

Methods:Methods:

Brain MRI was carried out to assess the presence of other Brain MRI was carried out to assess the presence of other anomalies and vascularity.anomalies and vascularity.

Treatment of Treatment of EncephaloceleEncephalocele

Immediate surgical excision & Immediate surgical excision & Closure is the rule with-Closure is the rule with-Excision of nonviable neural Excision of nonviable neural tissue tissue in the sac & watertight dural in the sac & watertight dural closure.closure.Bony defect does not warrant Bony defect does not warrant repair.repair.

60% develop post-op 60% develop post-op hydrocephalus; 3hydrocephalus; 3rdrd ventriculostomy or shunt ventriculostomy or shunt placement is essential. placement is essential.

Steps of surgical resection Steps of surgical resection & repair& repair

Incise dome of the sac along side wall Incise 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 stalk Identify dural defect & neural stalk Isolating stalk from the dural tunnel wallIsolating stalk from the dural tunnel wall Transect the stalk avoiding vessels on Transect 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

Excised EncephaloceleExcised Encephalocele

Excision after drainage of the altered colored CSF was done and contents Excision after drainage of the altered colored CSF was done and contents were examined. Samples were sent for histopathologic evaluation were examined. Samples were sent for histopathologic evaluation

Outcome & Outcome & ConclusionConclusion

High mortality; 29% n recent series High 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 developmentIn 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 with Our patient is alive 15 months with Visual and other issues in growth.Visual and other issues in growth.

Big Boy!Big Boy!

The parents named him: The parents named him: ARAB!ARAB!

The goal of treatment The goal of treatment for NTDs is to allow for NTDs is to allow the individual to the individual to achieve the highest achieve the highest level of function and level of function and independence. independence.

Arab at 9 months