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Case

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Turkish Neurosurgery 2012, Vol: 22, No: 2, 242-245242

Received: 01.05.2010 /accepted: 15.07.2010

DOI: 10.5137/1019-5149.JTN.2902-10.1

ABSTRACT

One type o congenital intrasphenoidal meningoencephalocele is remnant o lateral craniopharyngeal (Sternbergs) canal. We present a caseo a 23-year girl with 10-month history o right side CSF rhinorrhea. CT scan, MRI revealed congenital meningoencephalocele and CSF leakrom middle ossa to right side o sphenoid sinus, and there were bony deects at the foor o the anterior aspect o the right middle ossa.Transcranial repair was perormed with right side pterional craniotomy. Careul preoperative evaluation and localization o the sphenoiddeect are essential or selection o the best possible surgical approach and skull base reconstruction or repair o sphenoid sinus CSF leaksand meningoencephaloceles. In this case, an endoscopic technique was not successul so, transcranial repair was perormed with right side

pterional craniotomy.

KeywOrds: Meningoencephalocele, Rhinorrhea, Transcranial repair

Z

Senoid kanat iine yerlemi meningoensephalosel, lateral kranioarengial kanaln (Stenbergin kanal) artndan meydana gelen bir konjenitalanomalidir. Sa burun deliinden 10 aydan beri BOS gelen 23 yanda kadn olgu olarak sunulmutur. Bilgisayarl tomograi ve manyetikrezonans grntlemede; sa orta ossa tabannn anterior yzndeki kemik deekti ile seneoid sinsn sa tara arasnda iliki bulunmutur.Sa piterional kraniotomi yaplarak; kranial yoldan kemik deekti onarld. Senoid sinsden BOS kaa olan ve meningoenseeloseli olanhastalarda ameliyat ncesi dnemde detayl grntleme almalar ile deekt ortaya konmal ve en iyi cerrahi yaklam yolu bu grntlemealmalarna gre belirlenmelidir. Olgumuzda endoskopik giriimin baarsz olmas nedeni ile sa piterional kraniotomi yaplarak deektonarm yaplmtr.

AnAhtAr sZCKler: Meningoensealosel, Rinore, Kranial yoldan onarm

Correpodece ddre:Mohmmd saMaDIan / E-mil: [email protected]

Mhmm saMadIaN1, Hbibllh MogHaddasI2, Mhen VazIrNEzaMI2, kim HaddadIaN1,omiv rEzaEE1, Mehn arMaNFar1, Ftemeh kHorMaEE3

1Loqman-Hakim Hospital, Shahid Beheshti University M.C., Department of Neurosurgery, Tehran, Islamic Republic of Iran2Loqman-Hakim Hospital, Shahid Beheshti University M.C., Department of ENT, Head and Neck Surgery, Tehran, Islamic Republic of Iran3Loqman-Hakim Hospital, Shahid Beheshti University M.C., Department of Emergency, Tehran, Islamic Republic of Iran

Trcri approch for spoteou CsFRhiorrhe due to sterberg C Itrpheoid

Meigoecephocee: Ce Report d Review ofthe literture

Stenberg Kanalna Yerleen ve Spontan Rinoreye Neden OlanMeningoensefalosele Transkranial Yaklam: Olgu Sunumu veLiteratr Taramas

InTRoduCTIon

A meningoencephalocele is an uncommon malormation

described by a protrusion o cerebral tissue and meninges

through a congenital deect in the cranial bones. Serious

complications rom this condition usually happen In the rst

ew years (10,11,21) but occasionally symptoms do not occur

until adulthood (15,24).

We report here an adult case o Sternbergs canal intrasphe-

noidal meningoencephalocele with a 10-month history o

right side CSF rhinorrhea.

Clinical details

A 23-year girl with a 10-month history o right side

CSF rhinorrhea was reerred to the neurosurgery clinic.

Neuroimaging studies included coronal and axial paranasal

multislice CT scan with 3D reconstruction o skull base,

MRI and CT cisternography and showed sphenoid sinuses

overpneumatization with extension tothe parasellar region.

There was a well-dened bony deect containing CSF at the

right parasellar region just lateral to the rotundum oramen

measuring 3 mm that communicated with the anterior

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Turkish Neurosurgery 2012, Vol: 22, No: 2, 242-245 243

Samadian M. et al:Intrasphenoidal Meningoencephalocele

aspect o right middle cranial ossa and extended tothe

right sphenoid sinus. The bony deect was a remnant o the

craniopharyngeal canal (Sternbergs canal) (Figures 1, 2, 3).

She was a candidate or endoscopic repair o the meninoen-

cephalocele. Endoscopic transethmoidal - pterygoidal - sphe-

noidal repair was perormed. We tried to repair the deectwith the endoscopic approach. Because o very lateral exten-

sion o the deect we did not have good view o the deect,

and with standard endoscopic instrument the acute angleo

the working area was not accessible. Then transcranial repair

was perormed with right side pterional craniotomy. With

right side standard craniotomy with an intradural approach,

dissection was perormed in the subtemporal region in the

middle ossa. The bony deect was seen including part o the

brain and meninges that protruded into the sphenoid sinus.

The sot tissue mass was amputated and the bony deect

repaired with a small cranial bone grat and duraplasty

was perormed with ascia o temporalis muscle. Duraand craniotomy closure was perormed with the standard

method. The postoperative state was uneventul and she

was discharged rom the hospital ater 3 days without any

neurological decit or CSF rhinorrhea. Post op MRI showed

that the deect was well repaired (Figure 4). In ollow-up or

one year she is in good condition without CSF leak.

Figr 1: Coronal T2W MRI shows communication o the middleossa with the sphenoid sinus via a bony deect. Brain, meninges

and CSF protruded rom the middle ossa to the RT sphenoid

sinus.

Figr 2: Coronal CT cisternography o patient; Overpneumatiza-tion o sphenoid sinus and CSF leak rom middle ossa to RT

sphenoid sinus through small bony deect (arrow) were seen.

Figr 3: 3D reconstructed multislice CT scan o skull base showsintracranial view o skull base and bony deect anterolateral to

oramen rotundum (arrow).

Figr 4: Coronal T2W MRI shows good repair o the deect.

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Turkish Neurosurgery 2012, Vol: 22, No: 2, 242-245244


dISCuSSIon

The incidence o cephaloceles (meningoceles, encephaloceles)

is assumed to range between 1 and 3 in 10,000 children (7,19).

Occipital, sincipital (nasorontal, nasoethmoidal, nasoorbital),

and basal (sphenoorbital, sphenomaxillary, transethmoidal,

sphenoethmoidal, sphenonasopharyngeal, basioccipital)cephaloceles can be distinguished in relation to the site o

origin (16). A rare nding is a basal cephalocele that is limited

to the sphenoid sinus and has been proposed to present a

urther subtype o basal cephaloceles (3).

It may be dicult to determine the accurate etiology o

cephaloceles especially i no trauma is evident in the patients

history. Generally, non-traumatic cephaloceles include

congenital and acquired orms (4).

Clinically, transsphenoidal encephaloceles are the only con-

genital type ound in the sphenoid sinus. Abiko et al. (1).

urther distinguished two types o transsphenoidal menin-

goencephaloceles: the true transsphenoidal and the intra-sphenoidal. The rst one describes meningoencephaloceles

extending into the sphenoid sinus but restricted by its foor.

The latter describes encephaloceles traversing the foor o the

sphenoid sinus and protruding into the nasal cavity or naso-

pharynx. Abnormalities o the ace will most commonly coex-

ist with the true transsphenoidal-type encephaloceles, which

are transmitted through the sphenoid bone, optic system,

and brain, corresponding to the median clet ace syndrome

(14). In the early inantile period, high surgical risks may be

encountered with transsphenoidal encephaloceles as the

pituitary-hypothalamic structures are usually incorporated in

the herniated encephaloceles o this age group (12,18). Most

patients present during childhood, Cases diagnosed in later

adult lie are rare (15,24).

Generally, non-traumatic cephaloceles include congenital

and acquired orms. One type o congenital intrasphenoidal

meningoencephalocele is a remnant o the craniopharyngeal

(Sternbergs) canal. The complex ontogeny o the sphenoid

bone and the embryological conditions can cause the

development o congenital sphenoidal cephaloceles (17,

20). During embryological development, the anterior

sphenoid bone (presphenoid), the lesser wings, the posterior

sphenoid bone (basisphenoid), the greater wings and the

lateral pterygoid processes are built up rst as independent

cartilaginous precursors. At the third month o etal lie

ossication o the cartilaginous precursors starts rom 18 to

19 ossication centers (22).

Union o the various ossied components results in ormation

o the complex sphenoid bone (16). Bony usion o the greater

wings with the presphenoid/basisphenoid starts in its anterior

portion. Posteriorly usion can be incomplete, creating a

lateral craniopharyngeal canal (Sternbergs canal). Sternbergs

observations showed incomplete usion o the greater wings

and presphenoid in lower mammals (e.g. Paradigitatae).The

development o the sphenoid sinus starts with usion o the

sphenoid turbinates (ossicula Bertini) and sphenoid bone. At

the age o 3 to 4 years this connection is expected (23).

The shape o the later sphenoid sinus depends on the

various extent o pneumatization due to bone absorption. A

congenital bony deect resulting rom incomplete usion o

the dierent sphenoid bone components can communicate

with the sphenoid sinus ater the necessary pneumatization

has taken place. However, as extensive pneumatization othe sphenoid sinus usually results in only a thin bony border

to the intracranial space, the prevalence o the sphenoid

sinus border disorders in the development o acquired bony

dehiscences has to be considered (13).It is important to take

into account that usion planes oer special resistance to

pneumatization to dierentiate between a congenital and an

acquired non-traumatic bony deect in the sphenoid sinus.

Consequently, sphenoidal cranial base deects at usion planes

are more likely to be o congenital origin than the acquired

type. The lateral craniopharyngeal canal (Sternbergs canal)

may act as the site o origin o congenital meningoceles or

cerebrospinal fuid stulas when there is sucient sphenoid

sinus pneumatization (26).

Traumatic or spontaneous CSF rhinorrhea make up 4% o

the cases. These are subdivided into normal pressure and

high pressure leaks. High-pressure leaks include tumors and

hydrocephalus. They are due to long-standing increases in

intracranial pressure and account or 45% o spontaneous

leaks. Increased pressure in the subarachnoid space orces CSF

through a weak or potential pathway. Normal pressure leaks

account or 55% o cases. They are thought to be due to slow

erosion o the skull base secondary to normal fuctuations

in intracranial pressure leading to point erosion and CSF

rhinorrhea. 90% originate rom a congenital or potential

pathway such as a persistent craniopharyngeal canal. Direct

erosion o the skull base by tumor or inections make up theremaining 10%. In either case, the initial leak is requently

precipitated by coughing, sneezing or straining (22).

CT cisternography, 3D reconstructed multislice CT scan and

MRI provide excellent three-dimensional denition o the

lesion useul or both diagnosis and surgical planning (2,5),

and to classiy other related intracranial anomalies.

The treatment o intranasal basal meningoencephaloceles

is solely surgical. Although the rst successul operation

or intranasal meningoencephalocele was perormed via a

transmaxillary route (6), an intracranial approach have been

widely adopted more recently (10,11,15,21,22,24) as well.

I the deect in the skull base is small, an endoscopic transnasalapproach can be considered to reduce surgical complications

(25). The endoscopic approach also provides the most

fexibility or visualization and exact localization o the deect

site. However, in some cases like our case, the endoscopic

approach may be unsuccessul. Failure with the endoscopic

approach may be related to inaccurate localization o the

deect, insucient grat size and its displacement, incomplete

apposition o the grat to the skull base deect, and non-

compliance o the patient with post-operative instructions

(9). Beore deciding on the operative approach, the site

and size o the meningoencephalocele and the associated

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Turkish Neurosurgery 2012, Vol: 22, No: 2, 242-245 245


12. Jabre A, Tabbaddor R, Samaraweera R: Transsphenoidal

meningoencephalocele in adults. Surg Neurol 54: 183188,

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13. Kauman B, Nulsen FE, Weiss MH, Brodkey JS, White RJ, Sykora

GF: Acquired spontaneous, nontraumatic normalpressure

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Radiology 122:379387, 1977

14. Kitakara Y, Tagaki H, Ichikava F, Yamada M, Ootsuka E: Basal

encephalocele: A report o two cases and consideration o

its pathogenetic classication. No shinkei Geka 16:983-988,

1988

15. Kumar KK, Ganapathy K, Sumathi V, Rangachari V, Sundara-

rajan I, Govindaraj R: Adult intranasal meningoencephalocele

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16. Nager G T: Cephaloceles. Laryngoscope 97:7784, 1987

17. Peltonen E, Sedlmaier B, Brock M, Kombos T: Persistent cere-

brospinal fuid rhinorrhea by intrasphenoidal encephalocele

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century. Childs Nerv Syst 15: 635645, 1999

19. Rowland CA, Correa A, Cragan JD, Alverson CJ: Are

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257:430-432, 2000

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malormations should be properly determined by detailed

neuroradiological examination

ConCluSIon

Careul preoperative assessment and localization o the

sphenoid deect and a thorough understanding o underlying

etiologies are necessary in the selection o the best possible

surgical approach and skull base rebuilding or repair o

sphenoid sinus CSF leaks and meningoencephaloceles. In

this case, endoscopic technique was not successul and

transcranial repair was thereore perormed with right side

pterional craniotomy.

ACknowledGeMenT

The authors would like to thank Farzan Institute or Research

and Technology or technical assistance.

ReFeRenCeS

1. Abiko S, Aoki H, Fudaba H: Intrasphenoidal encephalocele.

Neurosurgery 22: 933936, 1988

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Congenital transsphenoidal meningocele: Case report and

review o the literature. Am J Otolaryngol 27:422-424, 2006

3. Daniilidis J, Vlachtsis K, Ferekidis E, Dimitriadis A: Intrasphe-

noidal encephalocele and spontaneous CSF rhinorrhea. Rhi-

nology 37: 186189, 1999

4. David D J: Cephaloceles classication pathology and

management: A review. J Cranioac Surg 4:192202, 1993

5. Diebler C, Dulac O: Cephaloceles clinical and neuroradiologi-

cal appearance associated cerebral malormations. Neurora-

diology 25: 199216, 1983

6. Fenger C: Basal hernias o the brain. Am J Med Sci 62: 117,1895

7. Giunta G, Pappagallo G, Piazza I, Girardi A: A rare case o

recurrent meningitis: Intranasal encephalocele. Minerva Med

81:577-581, 1990

8. Gra von SF: Keilbein Ossphenoidale. In von Bardeleben K,

Disse J (eds) Skelettlehre. Handbuch der Anatomie. Jena:

Fischer Verlag 1896: 125148

9. Greenberg J: Cerebrospinal Fluid Rhinorrhea. Available rom:

http://www.bcm.edu/oto/grand/120398.html. Access 1 May

2010

10. Hayashi T, Hashimoto T, Anegawa S, Utsunomiya H:

Transethmoidal encephalomeningocele in neonate: Report

o two cases and review o literatures. No To Shinkei 42:175-182, 1990

11. Izquierdo M, Gil-Carcedo LM: Recurrent meningitis and

transethmoidal intranasal meningoencephalocele. Dev Med

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