Turkish Neiirosurgery 6: 21 - 24, 1996 tnci: SiliilS Pericraiiii

SInus PerIcranii AssocIated With Epilepsy

SERVET INCi, MEHMET TURGUT, SERAP SAYGi, ÖZDEMIR GÜRÇAY

Hacettepe University, School of Medicine, Department of Neurosurgery (SI, MT, ÖG) and Neurology (SS). Ankara, Türkiye.

Ahsiract: Sinus pericranii is a rare vascular lesion of the craniaIvauli. Abnormal diploic veins provide communication belweenthe lesion and the intracranial venous sinus. Allhough sinuspericranii is well dociimented in the literature, we report a patientwith sinus pericranii and epileplic seizures. To our knowledge, a

INTRODUCTION

Sinus pericranii is a subperiostal vascularanomaly which is connected with the Intracranialvenous sinus through abnormal diploic or emissaryveins. it was first described by Stromayer in 1850,as"a cyst containing circulating blood" (13). The lesionis usuaIly located midline lying along the superiorsagittal sinus. Patients are usuaIly asymptomatic: Toour knowledge, this is the first case of sinus pericraniiassociated with epilepsy.

CASE REPORT

First Admission (March 1981) : A 34 year-oldman was admitted to the Neurosurgery Service ofHacettepe University Hospital for evaIuation of theaetiology of epilepsy. He had had generalized tonicdonic seizures foIlowing nausea, blurred vision anddizziness, since 1978. There was no remarkablehistory for epilepsy. On admission, physicalexamination revealed only a subcutaneous mass 1cmin diameter In the right frontal area which wasconsidered to be a lipoma or saebaceous cyst.Neurological examination was entirely normaL.Repeated electroencephalograms demonstratednonspecific slow wave activity on the vertex andfronto-temporal areas. Percutaneous carotid

case of sinus pericranii associated with epilepsy has not beenprevioiisly reported. The palient was seizure free after the removalof the lesion. The relationship between sinus pericranii andepilepsy is also briefly disciissed. .Key wards: Epilepsy, Sinus pericraniL

angiography and cranial computed tomography wereperformed. No pathology was found in these studies.The patient was discharged with diphenylhidantoin300 mg per day. At follow-up, rare focal motorseizures either on the right or the left side of the bodyhad continued with or without secondarygeneralization.

Second Admission (November 1990) - Thepatient was readmitted for a progressive sweIling onhis forehead. At physical examination; a painless, soft3x3cm diameter tumour covered with normal skin

was seen in the right frontal area. The massdisappeared with compression or in the sittingposition. Other physical and neurological findingswere normaL. Plain skuIl x-rays showed a smaIl bonedefect in the right frontal area. Soft tissue density CT(Window width: 75) was almost normal (Fig.1) butin the bone window CT (Window width: 800), thesubperiostal mass and bone defect were dearly seen(Fig.2). Selectiye carotid angiography (with digitalsubtraction) was performed and revealedopacification of the mass and drainage to the superiorsagittal sinus in the Iate venous phase (Fig.3). Thislesi on was diagnosed as a sinus pericranii in thepreoperative period. At operation, a semicircularscalp incision was made around the tumour. it wasseen beneath the periosteum. The lesion was like avarix and was filled with venous blood. Craniectomy

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TiLrkisli Neiirosii rgery 6: 21 - 24, 1996

was performed and the tumour was totallyextirpated. The few vessel communications betweenthe tumour and the superior sagittal sinus werecoagulated and cut. At the same operation,cranioplasty was performed with an autogen bonegraft. The postoperative period was uneventful.Histopathological studies of the lesion showedirregular sinuses lined with alayer of endotheliumin fibrotic tissue (Fig. 4). At five- year follow up, thepatient was seizure free without antiepilepticmedication.

Fig. 1: Soft tissiie deiisity CT (Wiiidow widtli: 75) wnsnlmost iiormnl.

Fig. 2 : "Bone wiiidow" CT (Wiiidow width: 800) C1enrlyshoived the siibperiostnl mnss niid boiie defect iii theright froiitnl nren (snme slice ns Fig.1).

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Iiici: SililiS Pericraiiii

Fig. 3 : IV OSA Appenraiice: Vnsciilnr lesiaii (nrrowhend) niiddraiiinge veiiis to siiperior sngittnl SililiS seeii iiitlie Inte veliolis plinse.

Fig. 4 : Histopnthologicnl stiidy shoived irregiilnr siiiiises Iiiied

by n Inyer of eiidotlieliimi iii fibrotic tissiie (H&Ex130).

DISCUSSION

Sinus pericranii, first described by Stromeyerin 1850 (B), is a rare vascular tumor that is causedby abnormal communications between intracranialand extracranial venous system. it is found beneathor in the periosteum of the cranial vault. Abnormaldiploic veins provide communication between thelesion and intracranial venous sinus (S, 6, LO, 11).Formerly, this lesion was called " , "Fistulaosteovasculare" or "Varix sprius circumscriptusvenae diploicae frontalis" in the li tera ture (7).According to Ohta, sinus pericranii is a collection of

Tiirkis/i Neiirosiirgery 6: 21 - 24, 1996

non-muscular venous blood vessels or a venous

hemangioma (ll). There is no feeding of the sinuspericranii from the arterial system, therefore, italways contains venous blood and is not pulsatile(6, 10).

Etiologically, three types of the sinus pericraniiwas described (l, lO, ll, 12). One is head trauma.Fractures of the external table, tear of an emissaryvein or venous extradural haematoma due to sinus

tearing have been proposed as the pathogenesis of atraumatic origin. This traumatic haemorrhageextends below the periosteum and is then coveredwith the epithelium. Stromeyer stated that, subgalealhaematoma arose first from head trauma the ncommunicated with the intracranial venous sinus via

abnormal diploic veins (13). According to Nimuraet.a!, venous sinuses or feeding vessels are injuredby linear fracture of the skull and then a vascularmass develops by bleeding (9).

Congenital origin of sinus pericranii has alsobeen proposed. In addition sinus periicranii,coexisting with congenital anomalies support thecongenital theory (2, 15). However head trauma inthe course of labour may be responsible for theproduction of sinus pericranii in some so-calledcongenital cases (ll). Histologically, Cohn (3)believed that the walls of the traumatic type had aconnective tissue lining and congenital tumours havean endothelial lining.

Finally, spontaneous origin has been proposed.According to Mastin; osteitis and venous diseases arecausative factors (7). In contrast, Arnies et al, statedthat, spontaneous sinus pericranii is probably avariety of the congenital type which is revealedbelatedly during the patient's lifetime (1).

Sinus pericranii is generally in the frontal regionmost1y in the midline. Lateral situated cases are rare(6, 10). The lesion is made more pronounced by theValsalva manoeuvre or placing the head in adependent position; it disappears with compression(6, LO, 11, 12). These dinical features are importantin the diagnosis of the sinus pericranii.

CT is helpful for the diagnosis. According toSadler et aL, on noncontrast CT sinus pericranii isslightly hyperdense compared to the brain (l2). Inour case, the lesion was isodense and the mass was

not dearly enhanced following intravenous contrastmedium adminstration. This may be due a narrowingor thrombosis of some abnormal diploic veins.According to our experience, density of the CT is

Iiici: Siiiiis Perieraiiii

more important than contrast mediumadminstration. Especial1y "Bone window" CT(Window width: 700-900) dearly shows the cranialdefect and the subperiostal tumour.

Cerebral angiography demonstrates abnormalcommunicating veins and the accumulation of thecontrast medium within the lesion. Multiangle viewsmay be necessary to demonstrate the mass, especiallythe Iate venous phase as in our case. Same authorscontend that direct injection into the lesion isnecessary when there is non-visualisation by cerebralangioraphy (l, 6, ll, 14). We found that, "bonewindow" CT and cerebral angiography with digitalsubtraction gaye enough information for a truediagnosis.

Meningocele, encephalocele, sebaceous cyst,eosinophylic granuloma and especial1y venouscavernoma of the scalp should be differentiated fromsinus pericranii. Cavernoma is located in th\:;subcutaneous space, and does not communicatedirectly with the intracranial venous sinuses.

Although a few spontaneous ocdusions havebeen reported (4) , sinus pericranii must be treatedsurgically because, there is a danger of severebleeding and air emboli due to trauma . Afterextirpation of the tumour, cranioplasty should beperformed with bone graft or acrylic, if necessary.

Patients are usually asymptomatic but maypresent with headache (6, ll, 14). Vague symptomsof nausea, vomiting and vertigo have alsobeeneported (ll). We were unable to find a case ofsinus pericranii with epileptic seizures in theliterature. In 1975, Nakayama and Matsukado (8)reported a patient with predominant theta-rangedirregular pattern in electroencephalogram, but therewas no epileptic discharge or clinical seizure. In ourcase, we can not explain the relationship betweensinus pericranii and epilepsy. We postuIate that bothfrontallobes may be affected by transient impairmentof blood flow in the superior sagittal sinus andchanges of metabolism due to the indirect effect ofsinus pericranii. In our patient, stopping the seizureswith removal of the tumour is good evidence for thecausative relationship between sinus pericranii andepileptic seizures.

Correspondence: Servet INCIEmek Mah. 4. Cad. 70/8

Ankara-TürkiyeFax: 90-312-311 1131

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