Cephalocele

dehiscence of intracranial tissue through an abnormal cranial opening, usually a congenital defect in the skull and dura. According to the type of the herniating structure cephaloceles may be classified into: meningoencephaloceles (herniation of meninges, brain tissue and CSF); meningocele (herniation of the meninges and CSF only); atretic cephalocele (formes frustes consisting of dura, fibrous tissue and degenerated brain tissue); and glioceles (a glial lined cyst containing CSF). A second type of classification takes into consideration location and site of the cephalocele. !ccipital cephaloceles are the most common; both supra and infratentorial structures (occipital poles and horns, cerebellum and fourth "entricle), together #ith the tentorium and ma$or "enous sinuses may herniate through the abnormal opening. Frontoethmoidal cephaloceles are anterior congenital midline anomalies, fre%uently associated #ith nasal dermoids and nasal gliomas (Fig.&). 'hey are thought to ha"e an embryologic deri"ation resulting from lack of normal regression of a pro$ection of dura that e(tends through the embryologic foramen caecum, bet#een the de"eloping cartilage and nasal bone. )arietal cephaloceles are uncommon, usually associated #ith significant brain anomalies like *andy +alker malformation, callosal agenesis, Chiari ,, malformation and holoprosencephaly and ha"e a poor prognosis. A high percentage of parietal cephaloceles are atretic and present as small midline masses near the "erte(. Atretic occipital cephaloceles are small nodular masses abo"e the e(ternal occipital protuberance that communicate #ith the intracranial ca"ity through a small cal"arial defect; the tract usually terminates in the fal( cerebri or tentorium. -asopharyngeal cephaloceles are uncommon, usually occult, and diagnosed later than encephaloceles in other locations, #hen the child manifests difficulty in nose breathing secondary to obstruction of the nasopharyn(. !ther locations for cephaloceles are frontal, temporal (along the superior surface of the petrous ridge), sphenoma(illary (through orbital fissures into the pterygopalatine fossa) and spheno orbital (through a defect in the sphenoid bone or optic canal.orbital fissure into orbit). 'he defects in embryogenesis that e(plain the formation of cephaloceles are different for the different locations and not yet completely clarified. 'he aim of the neuroradiological diagnosis is partly to confirm the presence of the lesion, #hich is fre%uently %uite ob"ious, but mainly to precisely define the structures in"ol"ed, particularly "ascular, associated anomalies of the ner"ous system, and occult lesions. /0 is the modality of choice; the midline sagittal '& #eighted image is particularly useful for midline anomalies; C' better sho#s the e(tent and characteristics of the bony defect #hile angiography may be necessary to demonstrate the position of dural "enous sinuses. 1S

Fig.&

a. )lain film of the skull, A) pro$ection; a median cleft face syndrome is obser"ed, #ith frontonasal schysis and hypertelorism b. '& #eighted /0 sagittal midline image: the three arro#s indicate the inferior margin of the nasal cephalocele c, d. '& #eighted coronal and '2 #eighted a(ial /0 images sho# that the cephalocele contains both brain tissue and CSF.

Cephalocele, Fig.& (a)

Cephalocele, Fig.& (b)

Cephalocele, Fig.& (c)

Cephalocele, Fig.& (d)

*isclaimer: 'he information contained #ithin the 1rand 0ounds Archi"e is intended for use by doctors and other health care professionals. 'hese documents #ere prepared by resident physicians for presentation and discussion at a conference held at 3aylor College of /edicine in 4ouston, 'e(as. -o guarantees are made #ith respect to accuracy or timeliness of this material. 'his material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and.or peer re"ie#ed medical literature.

Congenital Midline Nasal Masses Thomas A. Salzer, M.D. September 10, 1992
Congenital midline nasal masses are rare anomalies. Although usually detected at birth, they may not be recogni5ed until #ell into adulthood. -asal dermal sinus cysts, nasal gliomas, and encephaloceles constitute the great ma$ority of these lesions. Complications associated #ith these lesions may be se"ere, particularly if intracranial e(tension e(ists. 6ocal abscess formation, cellulitis, and cosmetic deformity are fre%uent. CSF leakage, frontal lobe abscess formation, and meningitis are not uncommon. -asal dermoids are by far the most common lesion, follo#ed by gliomas and encephaloceles. Since the process occurs early in embryogenesis, the pathogenesis of these lesions remains obscure. 'he 7prenasal theory7 cites alterations in embryogenesis as responsible for the de"elopment of these lesions. Faulty closure of the anterior neuropore can result in a defect in the fonticulus frontalis, foramen cecum, cribriform plate, sphenoid and ethmoid bones. *e"elopment of nasal dermal sinus cysts is understood to result from faulty in"olution of the dural tract. ,f brain tissue is isolated e(tracranially by fusion of the cranial sutures, a glioma results. +hen a bony defect allo#s herniation of dura mater and brain tissue e(tracranially, an encephalocele results. 'he differential diagnosis includes de"elopmental anomalies, inflammatory lesions and benign or malignant neoplasms. -asal gliomas are C-S masses #hich ha"e lost their intracranial connection. First described in &892 by 0eid, nearly &9: cases ha"e been reported. 'hey occur #ith a male to female ratio of three to t#o. -o familial predisposition or association #ith anomalies has been reported. -asal gliomas tend to gro# in proportion to the child and malignant degeneration has not occurred. '#enty percent of nasal gliomas are in communication #ith the intracranial space "ia a fibrous stalk. Clinically they occur e(tranasally in 9;< of cases, intranasally in ==<, and combined in &&< of cases. !n e(amination, e(tranasal gliomas are firm, lobular, and noncompressible, ranging in si5e from & cm to 9 cm, usually off midline o"er the root of the nose. 'he o"erlying skin may be normal, red or bluish, #ith or #ithout telangiectasias. ,ntranasal gliomas may present as a mass protruding from the nostril, or as a pale, firm, glistening polypoid mass #ithin the nasal ca"ity or nasopharyn(. Symptoms include nasal obstruction, epista(is, or CSF rhinorrhea. 'he base most often arises from the lateral nasal #all. >ncephaloceles #ere first described in the si(teenth century. 3oth se(es are affected e%ually, and malignant degeneration does not occur. '#enty fi"e percent of all cranial encephaloceles occur anteriorly, of #hich &9< are sincipital and &:< are basal. Sincipital lesions are e(tracranial and are further di"ided into nasofrontal, nasoethmoidal, and naso orbital. 3asal lesions are di"ided into spheno orbital, sphenoma(illary, and sphenopharyngeal. Clinically, encephaloceles are soft, compressible, and appear some#hat bluish. 'hey "ary in si5e and may change #ith crying or head position. 'he Forsterburg test is usually positi"e and spontaneous pulsations may be seen. >ncephaloceles may leak CSF spontaneously and a history of recurrent meningitis may be obtained. >(tranasal encephaloceles e(tend through the fonticulus frontalis, #hereas intranasal encephaloceles e(tend through the cribriform plate. !n e(amination an intranasal encephalocele al#ays arises medially. -asal dermal sinus cysts account for &:< of all dermal cysts of the head and neck and &< to =< o"erall. 'he se( ratio is e%ual. ,n rare cases an autosomal dominant mode of inheritance has been suggested. /alignant degeneration has not been described and the incidence of associated anomalies is %uite lo#. >(tradural communication occurs in &:< of cases. Clinically, a sinus tract e(ists #hich opens to the skin of the nose any#here bet#een the base of the columella and the glabella, #ith the distal one third of the nasal dorsum the most common site. -asal dermal sinus cysts are firm, noncompressible, nonpulsatile masses #hich do not transilluminate. A cheesy material may be e(pressed from the cyst and small hairs may protrude from the dermal opening. )reoperati"e e"aluation re%uires imaging studies to delineate any intracranial e(tension. 0eported findings #hich suggestintracranial e(tension include a soft tissue mass, #idened nasal septum, bifid septum, bifid perpendicular plate, bifid crista galli, interorbital #idening, and defects in the cribriform plate. )olytomography and CA' of the skull base #ith coronal plane scanning is the most accurate method of defining bony defects of the skull. 'he role of /0, has been established, citing superior soft tissue contrast, nonin"asi"eness, lack of ioni5ing radiation, and easy multidimensional reconstruction. /0, is also effecti"e in delineating intracranial e(tension of congenital midline nasal masses. 0egardless of preoperati"e e"aluations, the decision regarding the surgical resection should obser"e the

Frontoethmoidal encephaloceles are herniations of the intracranial contents through a defect in the skull at the junction of the frontal and ethmoidal bones. They are generally classified as nasofrontal, nasoethmoidal, and naso-orbital, although there may be some overlap or multiplicity.
Neuroradiology Fronto Ethmoidal Meningocele R Malik, vk pandya, s parteki Ind J Radiol Imag 2004 4!4!"#$%"&

'ey(ords! )ephelocele, Meningocele, Encephalocele

Introductions! A skull defect in association with herniated intracranial content is termed as cephalocele. If the herniation contains solely leptomeninges and CSF it is termed a meningocele. Cephaloceles in which the protruding structure consists of leptomeninges, CSF and brain are termed meningoencephalocele.

Fig

Fig 2

Fig " )ase Report! A 18 years old man presented with progressi ely enlarging swelling at nasion since birth.!Fig 1 " #n e$amination the swelling was e$pansile on alsal a, gi ing positi e Furstenbery sign and transillumination. A non contrast head C. %. was performed which re ealed a large frontoethmoidal meningocele, !Fig &,'" unilateral left side colpocephaly !Fig (, )".

Fig 4 +iscussion!

Fig *

*ncephaloceles signify the herniation of neural tissue through defect in the skull +1,. %hey may contain meninges !meningocele" or brain matter and meninges !encephalomeningocele" or they may communicate with a entricle !encephalomeningocystocele". -o familial pattern has been demonstrated with these lesions. Association with other diseases !e.g. *hlers./anlos Syndrome, frontonasal

dysplasia", howe er, may suggest a genetic component+&,',. %wenty percent of all encephaloceles occur in the cranium. #f these, 1)0 are nasal. -asal encephaloceles can be di ided into & types 1 sincipital !230" and basal !(30" Further, the sincipital form is di ided into sub types as follows1 !1" %he nasofrontal !(30", which e$its the cranium between the nasal and frontal bones4 !&" %he nasoethmoidal !(30", which e$its between the nasal bone and nasal cartilages4 and, !'" %he nasoorbital !&30", which e$its, through a defect in the ma$illary frontal process. Sincipital encephaloceles typically present as soft compressible masses o er the glabella +',(,),. %he basal form is di ided into two subtypes as follows1 !1" %he transethmoidal, which e$its through the cribriform plate into the superior meatus, e$tending medial to the middle turbinate4 !&" the sphenoethmoidal, which e$its through the cribriform plate +),, between the posterior ethmoid cells and sphenoid, to present in the nasopharyn$4 !'" the sphenoorbital, which enters the orbit ia the superior orbital fissure and may produce e$ophthalmos4 and4 !(" the transsphenoidal which herniates in the nasopharyn$ ia defect posterior to the cribriform plate. 5asal encephaloceles may remain hidden !clinically" for years. %here has been an association of cranial meningocele with obstructi e 6ydrocephalus and Colpocephaly due to obstruction at foramen of 7onro or 8uschka and 7agendie +(,. 5oth C% scan and 79I are useful in diagnosis, the former for degree of bony defect and the latter for degree of soft tissue herniation. Further ,ssociated ,nomalies ! #ccular hypertelorism, nasal widening, comple$ facial malformations and median nasal fissure, spina bifida, agenesis of the corpus callosum, ocular malformation : microcephaly. In these cases biopsy is strongly contraindicated due to risk of infection and meningitis. %he treatment in ol ing surgical e$cision with repair of bony defect, often a craniotomy is necessary to approach the encephalocele.