Journal of Cerebrovascular and Endovascular Neurosurgery

pISSN 2234-8565, eISSN 2287-3139, https://doi.org/10.7461/jcen.2019.21.3.163 Case report

Symptomatic Sinus Pericranii with Adult Onset Headache :

A Case Report with Pathologic Perspective
Young Soo Chung1, Jung-jae Kim2, Se Hoon Kim3, Joonho Chung1, Jae Whan Lee1, Keun Young Park1
1
Department of Neurosurgery and 3Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea ;
2
Department of Neurosurgery, Ewha Womans University Seoul Hospital, Ewha Womans University School of Medicine,
Seoul, Korea

J Cerebrovasc Endovasc Neurosurg.

Sinus pericranii (SP) is a rare vascular anomaly of the scalp that consists 2019 September;21(3):163-168
of an abnormal pericranial venous channel connected to adjacent dural Received : 9 July 2019
Revised : 16 September 2019
venous sinuses. Most SP are asymptomatic and are found in the pediatric
Accepted : 20 September 2019
age group. We aim to report a case of symptomatic SP in adult and de-
scribe the clinical, radiological, and pathohistological findings to help un- Correspondence to Keun Young Park
Department of Neurosurgery, Severance
derstand and differentiate this lesion from other scalp lesions. A
Hospital, Yonsei University College of Medicine,
40-year-old man with a scalp mass was admitted to our hospital com- 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752,
plaining of headache. The lesion enlarged when the patient was in a re- Republic of Korea
cumbent position or during Valsalva maneuver. The radiologic imaging Tel : 82-2-2228-2150
suggested its diagnosis as an accessory type of SP with bone erosion. Fax : 82-2-393-9979
Surgical resection and cranioplasty were successfully performed, and the E-mail : [email protected]
ORCID : http://orcid.org/0000-0002-3254-4577
related headache also gradually subsided. At the 3-year follow-up, there
was no recurrence on magnetic resonance imaging.
This is an Open Access article distributed under the
terms of the Creative Commons Attribution Non-
Commercial License (http://creativecommons.org/li-
censes/by-nc/3.0) which permits unrestricted non-
Keywords Sinus pericranii; Vascular malformation; Headache commercial use, distribution, and reproduction in any
medium, provided the original work is properly cited.

INTRODUCTION DESCRIPTION OF CASE

Sinus pericranii (SP) is a rare vascular anomaly, A 40-year-old man with a mass on the vertex visited
which forms as a scalp varix consisting of an ex- our clinic. In his medical history, the mass had ex-
tracranial–intracranial venous communication with or isted since his childhood without any change in size.
without a skull defect.9) Generally, SP is a benign le- However, the mass had grown gradually in the recent
sion and presents as an asymptomatic mass in several months, and a newly developed headache
11)
childhood. Sometimes, its rarity and similarity with occurred. The headache was characterized with a per-
other scalp lesions can lead to misdiagnosis of SP as sistent waxing and waning, and relieved using analgesics.
2) 3)
a scalp hemangioma, atretic cephalocele, or other Due to this headache, he was distressed while
scalp vascular anomalies. However, its unique charac- working. Apart from headache, there was no other
teristics are helpful to differentiate a SP from other symptom related with the lesion. The mass was soft,
vascular anomalies. Herein, we describe the clinical, round-shaped, and non-tender lesion. Characteristically,
radiological, and pathohistological findings of a case its size increased when in a recumbent position and
of SP to help understand this rare disease entity. during Valsalva maneuver, and returned to its origi-
nal size in a standing position.

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SINUS PERICRANII AND HEADACHE

Radiologic findings dissected with coagulation of small scalp vessels in a

The initial computed tomography (CT) imaging layer by layer fashion. The reddish, hyperemic, and
showed a slight high-density scalp mass with a re- soft lesion was located on the calvarium. Coagulation
lated bone erosion of the calvarial outer table. The in- and cutting of the small vessels around the lesion
ner table was very thin; however, it was not com- were performed using a bipolar coagulator, then the
pletely destroyed. The lesion was located in the mid- lesion was carefully dissected from a nearby soft tis-
line, adjacent to the superior sagittal sinus (Fig. 1a, b). sue and bone. Finally, the lesion was completely removed.
On magnetic resonance (MR) imaging, a scalp mass During the surgery, a lot of small holes were identi-
with heterogenous signals and flow-void in both T1 fied within the calvarium. These holes formed tiny
and T2 weighted imaging was identified. Entangled channels connected with the SSS; some hemorrhagic
morphology and enhancement after gadolinium con- leakage occurred from them. After achieving hemo-
trast implied a vascular anomaly (Fig. 2a, b, c). No stasis using a bone wax and a glue, cranioplasty was
definite brain parenchymal lesion was seen. On the done using a mesh plate system. These surgical proce-
venous phase of digital subtraction angiography dures were performed without any complications.
(DSA), small vascular channel was noted on right car-
Postoperative clinical course
otid angiogram, and this anomaly was connected to
Immediate postoperative CT imaging showed a
the superior sagittal sinus (SSS). While a majority of
complete removal of SP and a well-formed cranial
the cerebral venous outflow occurred through the SSS,
vault (Fig. 1c). At discharge, the patient had com-
only a part of the extracranial venous outflow was
pletely recovered. At the 3-year follow-up, there was
draining through the SP (Fig. 3), implying “accessory
no evidence of disease recurrence on MR imaging
type” of SP.4) There were no other vascular anomalies
(Fig. 2d) and the patient was doing well without any
such as arteriovenous malformation or cavernous
headache and neurologic signs.
malformation. These clinical and radiologic findings
strongly suggested a tentative SP. Pathologic findings
A thin endothelial lining with CD-31(vascular mark-
Operative findings
As a definite treatment, surgical excision was performed. er) positive and D2-40 (lymphatic marker) negative

Under general anesthesia, the scalp was meticulously histology was identified; small, multiple void chan-

A B C

Fig. 1. Computed tomography (CT) scan. (A) Sagittal view, isodense round-shaped mass (arrow) located above the skull. (B) Sagittal
view of bone window setting, the related calvarial erosion (arrow) was noted. (C) Complete removal of sinus pericranii and
well-formed cranial vault with mesh plate was noted in postoperative CT scan.

164 J Cerebrovasc Endovasc Neurosurg

 YOUNG SOO CHUNG ET AL

A B

C D

Fig. 2. Preoperative magnetic resonance (MR) imaging showed heterogenous signals and flow voids within the mass in both T1 (A)
and T2 (B) weighted images. Gadolinium-enhanced T1-weighted MR imaging (C) showed a contrast-enhancing mass between galea
aponeurotica and skull. Three year’s follow-up gadolinium-enhanced T1-weighted MR imaging (D) showed a complete obliteration of
mass without any recurrence.

nels were well described. A thick fibrous stroma in- which is seen in encephalocele. No thrombus was noted.
tervening endothelium indicated its presence for sev-
eral years, which indicated a possibility of congenital DISCUSSION
type SP (Fig. 4). Unlike cavernous hemangioma, inter-
woven capillary with hemorrhage was not found. Sinus pericranii (SP) is a kind of scalp vascular le-
Furthermore, there was no neural or meningeal tissue, sion characterized by an extracranial–intracranial ve-

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SINUS PERICRANII AND HEADACHE

A B C

Fig. 3. Digital subtraction angiography (DSA) findings of sinus pericranii (SP). (A) Internal carotid angiography lateral view; in late ve-
nous phase, a majority of the cerebral venous outflow occurred through the superior sagittal sinus (SSS, arrow). Small contrast filling
of extracranial–intracranial venous channel was also identified (arrowhead). (B) External carotid angiography AP view; in late venous
phase, the SP (arrowhead) was connected with SSS (arrow). (C) External carotid angiography lateral view; in late venous phase, only
a part of the extracranial venous outflow was draining through the SP (arrowhead). These findings indicated an accessory type SP.

A B C

Fig. 4. Pathologic findings of sinus pericranii (SP). (A) H&E staining (x 100); it showed a single-layer flattened endothelium (arrow)
with thick venous stroma (asterisk), which suggested a congenital type of SP. (B) CD-31 staining (x 100); thin brownish endothelial
layers showed the vascular nature. (C) D2-40 staining (x 40); it showed the absence of lymphatic wall.

nous communication, which can be formed either by imaging shows a hyperemic vascular channeling in
focal venous hypertension and abnormal development the scalp layer with the related bone destruction. DSA
of diploic veins (congenital type) or by trauma can be helpful for definitive diagnosis and deciding
1)10)
(acquired type). SP is very rare and unfamiliar to treatment option, through analyzing venous flow dy-
clinicians, thus, it can be easily misdiagnosed as other namics and discovering the vascular nature.7) Some
scalp diseases. However, SP has its own unique clin- authors recommend direct percutaneous venography
6)14)
ical and radiological features, which can be helpful as a confirmatory tool,1) however, it is associated with
for diagnosis. Unlike other scalp pathology including a high risk of bleeding and infarction.
vascular anomaly, SP is usually located along the Approximately 80% of SP present as an asympto-
midline11) and its size can fluctuate depending on matic palpable mass.1) Nevertheless, headache is a
body positioning or Valsalva maneuver.8) Usually, SP common feature of symptomatic SP.8)13) The SP-related
can be associated with bone erosion, thus, CT or MR headache usually has a waxing and waning pattern

166 J Cerebrovasc Endovasc Neurosurg

 YOUNG SOO CHUNG ET AL

for several months, however, it can also present as a CONCLUSION

sudden striking headache in some cases. Although the
pathophysiology of SP-related headache is uncertain, Sinus pericranii (SP) is known as a rare vascular
intracranial hypertension can be one of the causes of anomaly and it can be easily misdiagnosed. However,
5)
the headache. Therefore, clinicians should keep in its unique clinical and radiological characteristics can
mind that SP is one of the possible causes of head- be helpful as regards differential diagnoses and treat-
ache and intracranial hypertension. ment decision.
Because most SP has benign features, asymptomatic
Disclosure
SP can be observed or conservatively treated. On the
The authors have no conflict of interest concerning
other hand, active treatment should be considered in
this case report.
a symptomatic or cosmetically problematic SP. However,
all symptomatic SP cannot be actively treated. Using
the venous drainage pattern, SP can be classified into
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