Encephaloceles,and Cranialand Cranial

Sinus

, Meningoceles,Cranial DermalCranial Dermal

Tracts

nosology• cephalocele: herniation of any of the intracranial contents through a defect in

the skul

• Encephalocele: contains either normal brain or • Encephalocele: contains either normal brain or

• meningocele:contains only cerebrospinal arachnoid tissue

• encephalocystocele:contains ventricle

nosologyherniation of any of the intracranial contents through a defect in

contains either normal brain or gliotic tissuecontains either normal brain or gliotic tissue

only cerebrospinal fuid (CSF) surrounded by a capsule of

Encephaloceleclassification

• encephalocele can be congenital or inflammation,infection or previous surgical procedure

• Other classification based on the location of the defect anterior and posterior• Other classification based on the location of the defect anterior and

posterior

• Anterior divided two group: sincipitalherniation is anterior to the cribriform plate, and “basal,” when the herniation is through the sphenoid sinus

Encephaloceleclassification

can be congenital or aquaired due to truma,tumoror previous surgical procedure

Other classification based on the location of the defect anterior and Other classification based on the location of the defect anterior and

sincipital (or frontoethmoidal) when the herniation is anterior to the cribriform plate, and “basal,” when the herniation is through the sphenoid sinus

Embryology

• The exact error in development leading to the occurrence of occipital and sincipital encephaloceles is not entirely known.

• As encephaloceles are covered in skin, a defect in • As encephaloceles are covered in skin, a defect in cannot completely explain the etiology, as a problem with would necessitate the absence of skin coverage.

• McClone show that the membranous bones of the skull and the three meningeal layers originate from a mesoderm that is locate between the closed neural tube and superfcial

Embryology

The exact error in development leading to the occurrence of occipital is not entirely known.

are covered in skin, a defect in neurulation alone are covered in skin, a defect in neurulation alone cannot completely explain the etiology, as a problem with neurulationwould necessitate the absence of skin coverage.

show that the membranous bones of the skull and the three meningeal layers originate from a mesoderm that is locate between

superfcial squamous ectoderm

• In normal embryogenesis, a diverticula of anteriorly through a small fontanelleand frontal bones (fonticulus nasofrontalisand frontal bones (fonticulus nasofrontalis

In normal embryogenesis, a diverticula of dura normally projects fontanelle between the developing nasal

nasofrontalis)nasofrontalis)

• ossifcation of the sphenoid bone, which occurs during the first year of life.A developmental insult here could lead to membranous sphenoid ala allowing for protrusion of temporal lobe

• Incomplete development of the medial sphenoid bone can lead to the persistent development of a lateral • Incomplete development of the medial sphenoid bone can lead to

the persistent development of a lateral known as Sternberg’s canal, which can act as gateway for encephaloceles to protrude into the lateral sphenoid sinus

of the sphenoid bone, which occurs during the first year developmental insult here could lead to nonossifed

allowing for protrusion of temporal lobe

Incomplete development of the medial sphenoid bone can lead to the persistent development of a lateral craniopharyngeal canal,Incomplete development of the medial sphenoid bone can lead to the persistent development of a lateral craniopharyngeal canal,known as Sternberg’s canal, which can act as gateway for

to protrude into the lateral sphenoid sinus

Epidemiology

• The incidence of encephalocelesdeclining because of using folic acid and declining because of using folic acid and

• Over 80 % of encephaloceles in the Western Hemisphere are occipital with most cases of frontoethmoidaloccurring in Southeast Asia and parts of Russia

• Using a lot of tea and pesticide can be a reason of

Epidemiology

and other neural tube defects is declining because of using folic acid and imporove diagnose methoddeclining because of using folic acid and imporove diagnose method

in the Western Hemisphere are occipital frontoethmoidal and basal encephaloceles

occurring in Southeast Asia and parts of Russia

Using a lot of tea and pesticide can be a reason of encephalocel

• Genetics

• High rate in female and association with some

• DIAGNOSIS• DIAGNOSIS

• maternal serum AFP

• prenatal ultrasound

• Amino synthesis

Genetics

High rate in female and association with some syndromic disorder

DIAGNOSISDIAGNOSIS

• in patient with nasal obstruction, obligate oral breathing, snoring, potential CSF rhinorrhea and meningitis, and purulent nasal discharge we should rule out encephalocell

• Frontonasal and frontobasal encephaloceles• Frontonasal and frontobasal encephalocelesanatomic portions of the hypothalamus, optic apparatus and anterior cerebral arteries

• MANAGEMENT

• Mode of Delivery• Mode of Delivery

in patient with nasal obstruction, obligate oral breathing, snoring, potential CSF rhinorrhea and meningitis, and purulent nasal discharge

encephaloceles can also contain encephaloceles can also contain anatomic portions of the hypothalamus, optic apparatus and anterior

MANAGEMENT

Encephaloceles and Hydrocephalus

• Hydrocephalus has been reported in both posterior and anterior encephalocelesencephaloceles

• In most cases, it is usually recommended to treat the hydrocephalus first

and Hydrocephalus

Hydrocephalus has been reported in both posterior and anterior

In most cases, it is usually recommended to treat the

Anesthetic Considerations

• ask anesthesiology team to perform a preoperative assessment of these patients,including an endoscopic evaluation to work out these patients,including an endoscopic evaluation to work out the airway anatomy

• Goals of Surgery

• The principles of surgical treatment of of the herniation with preservation of as much viable brain as possible, water-tight dural closure with adequate skin coverage, possible, water-tight dural closure with adequate skin coverage, repair of any cosmetic deformity, and cranial or craniofacial reconstruction

Anesthetic Considerations

ask anesthesiology team to perform a preoperative assessment of an endoscopic evaluation to work out an endoscopic evaluation to work out

Goals of Surgery

The principles of surgical treatment of encephaloceles are reduction with preservation of as much viable brain as

closure with adequate skin coverage, closure with adequate skin coverage, repair of any cosmetic deformity, and cranial or craniofacial

Surgical TechniquePosterior Encephaloceles

• The encephalocele is grasped and raised with tissue forceps, and an elliptical incision is made around the sack close to the neck an elliptical incision is made around the sack close to the neck of the herniation

TechniqueEncephaloceles

is grasped and raised with tissue forceps, and an elliptical incision is made around the sack close to the neck an elliptical incision is made around the sack close to the neck

AnteriorCephaloceles

• anterior encephaloceles often involves approach including OMFS,ENT and plastic surgeryapproach including OMFS,ENT and plastic surgery

• transcranial approach typically involves a bifrontal craniotomy, and an extradural dissection of the anterior cranial fossa to separate the sac from the surrounding tissue and base. Next, an intradural transection of the performed then OMFS/ENT team to come from belowperformed then OMFS/ENT team to come from below

Cephaloceles

involves a multidisciplinary OMFS,ENT and plastic surgeryOMFS,ENT and plastic surgery

approach typically involves a bicoronal scalp flap, a craniotomy, and an extradural dissection of the anterior

fossa to separate the sac from the surrounding tissue and skull transection of the encephalocele stalk is

performed then OMFS/ENT team to come from belowperformed then OMFS/ENT team to come from below

Prognosis

• The location and eloquence of the brain tissue comprising the encephalocele are also important prognostic factorsencephalocele are also important prognostic factors

• Ant encephalocele have better prognosis than post

• The coexistence of hydrocephalus extracranial anomalies with any type of portend a poorer prognosis

Prognosis

of the brain tissue comprising the prognostic factors.prognostic factors.

have better prognosis than post

hydrocephalus and associated intracranial and any type of encephalocele will also

CRANIAL MENINGOCELES• Cranial meningoceles, by defnition

meninges and CSF through the skull and are bereft of any tissue

• A common location for these lesions is directly over the anterior • A common location for these lesions is directly over the anterior fontanelle

• These lesions commonly abut the sagittal sinus, this area has to be done carefully

• These lesions can be mass effect, and the skull base and epileptogenic focus in the temporal lobethe skull base and epileptogenic focus in the temporal lobe

CRANIAL MENINGOCELESdefnition, are congenital herniations of

and CSF through the skull and are bereft of any cerebral

these lesions is directly over the anterior these lesions is directly over the anterior

These lesions commonly abut the sagittal sinus, and dissection in

mass effect, and source recurrent meningitis at focus in the temporal lobefocus in the temporal lobe

CRANIAL DERMAL SINUS TRACTS

• In the presence of an unexplained meningitis, especially due to Staph aureus hemolyticus, the entire skin surface along the midline aureus hemolyticus, the entire skin surface along the midline shouldbe examined carefully for dimples, palpable thickening, or sinuopenings. This search should include shaving the head.

CRANIAL DERMAL SINUS TRACTS

In the presence of an unexplained meningitis, especially due to Staph , the entire skin surface along the midline , the entire skin surface along the midline

examined carefully for dimples, palpable thickening, or . This search should include shaving the head.

Embryology

• Cranial dermal sinus tracts are uncommon, potentially deleterious, congenital, epithelialized tunnels that originate at the skin surface congenital, epithelialized tunnels that originate at the skin surface and can occur anywhere along the

• The most common sites are at the occipital protuberance or

• result from failure of dysjunctionectoderm

Embryology

Cranial dermal sinus tracts are uncommon, potentially deleterious, congenital, epithelialized tunnels that originate at the skin surface congenital, epithelialized tunnels that originate at the skin surface and can occur anywhere along the

sites are at the occipital protuberance or nasion

dysjunction neuroectoderm and cutaneous

Epidemiology

• Cranial dermal sinuses are uncommon more than 100 cases published as single reports or small more than 100 cases published as single reports or small

• Cranial DSTs have also been reported in the head, including lateral, parietal,anteriortemporal (pterional),and retroauricular

• Genetics

• Usally sporadic but female predominance and be with • Usally sporadic but female predominance and be with anomaly show genetic basis

Epidemiology

Cranial dermal sinuses are uncommon dysraphic lesions with slightly cases published as single reports or small seriescases published as single reports or small series

Cranial DSTs have also been reported in non-midline locations in parietal,anterior temporal, lateral

retroauricular

Genetics

sporadic but female predominance and be with Klippel-Feilsporadic but female predominance and be with Klippel-Feil

Diagnosis• Cranial dermal sinus tracts commonly appear

as a small, asymptomatic cutaneous dimple or pit

• they can also appear as fulminant • they can also appear as fulminant meningitis or acute hydrocephalus or and diffculty feeding in nasal DST

• In an Iranian series of 18 patients with cranial DSTs, infection was the most common reason for referral (50%), followed by an asymptomatic skin dimple followed by an asymptomatic skin dimple (33%), with only one patient (5.6%) exhibiting signs and symptoms of raised ICP due to mass or abscess

DiagnosisCranial dermal sinus tracts commonly appear

cutaneous dimple

they can also appear as fulminant they can also appear as fulminant hydrocephalus or stridor

patients with most

%), skin dimple skin dimple

%) signs and symptoms of raised

Association with Dermoid and and Intracranial Abscesses

• Dermoid and epidermoid cysts can be found at any depth up to 50% of cranial dermal sinuses.

• These lesions can be sources of mass effect or abscess or spontaneously and cause of Aseptic meningitisspontaneously and cause of Aseptic meningitis

and Epidermoid Cysts and Intracranial Abscessescysts can be found at any depth along the tract in

abscess or can rupture of Aseptic meningitisof Aseptic meningitis

Management

• For asymptomatic cranial DSTs, most authors agree that surgical removal of the tract and any associated surgical removal of the tract and any associated epidermoid cyst is warranted “as soon as the child can tolerate the operation

• Goals of Surgery

• Cranial DSTs should be removed entirely to eliminate the risk of meningitis and abscess formationmeningitis and abscess formation

• In the presence of CSF leak, infection, or raised intracranial pressure, surgery should be performed emergently

Management

For asymptomatic cranial DSTs, most authors agree that prophylactic surgical removal of the tract and any associated dermoid or surgical removal of the tract and any associated dermoid or

cyst is warranted “as soon as the child can tolerate

Goals of Surgery

Cranial DSTs should be removed entirely to eliminate the risk of meningitis and abscess formationmeningitis and abscess formation

In the presence of CSF leak, infection, or raised intracranial pressure, surgery should be performed emergently

Prognosis

• When completely resected, cranial DSTs rarely recur, and the prognosis is usually favorableprognosis is usually favorable

• recurrence following partial removal has been documented as late as 20 years from the original operation

Prognosis

When completely resected, cranial DSTs rarely recur, and the

removal has been documented as late operation