Sinus Pericranii

Background

This rare condition is chracterized a congenital or acquired connection between an extracranial blood filled nodule and an intracranial dural sinus. The importance lies in recognizing this rare disorder so that proper management may occur. Potentially lethal complications include an air embolism, hemorrhage, and infection.

OUTLINE

Epidemiology

Disease Associations

Pathogenesis

Laboratory/Radiologic/Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/Immunohistochemistry/Electron Microscopy

Differential Diagnosis

Prognosis and Treatment

Commonly Used Terms

Internet Links

EPIDEMIOLOGY CHARACTERIZATION

INCIDENCE Rare

PATHOGENESIS CHARACTERIZATION

Sinus pericranii: radiological and etiopathological considerations. Case report.

Bollar A, Allut AG, Prieto A, Gelabert M, Becerra E.

Neurosurgical Service, Hospital Xeral de Galicia, Santiago de Compostela, Spain.
J Neurosurg 1992 Sep;77(3):469-72 Abstract quote
Sinus pericranii is a rare vascular anomaly involving an abnormal communication between the extracranial and intracranial circulations. A case of frontal sinus pericranii is presented which appeared to be a posttraumatic sinus because it developed 2 years after a cranial injury.

However, the presence of vascular endothelium in the pathological examination and its association with a vascular anomaly (persistent trigeminal artery) suggested a congenital origin. The lesion, pericranial blood sinuses, and bone were totally removed. The computerized tomography, angiography, and magnetic resonance imaging findings are presented. The literature is reviewed and the pathogenesis of sinus pericranii is discussed.

LABORATORY/RADIOLOGIC/
OTHER TESTS
CHARACTERIZATION

RADIOLOGIC

Sinus pericranii: neuroradiologic findings and clinical management.

Higuchi M, Fujimoto Y, Ikeda H, Kato A.

Department of Neurosurgery, Itami Municipal Hospital, Hyogo, Japan.
Pediatr Neurosurg 1997 Dec;27(6):325-8 Abstract quote
We report an 11-year-old boy with a sinus pericranii. Magnetic resonance images demonstrated a small hole in the cranium underneath the subcutaneous mass. Magnetic resonance angiography (MRA) with sequential two-dimensional time-of-flight (2D-TOF) method demonstrated that the venous flow via the sinus pericranii was directed from the intra- to the extracranial venous system (the drainer type). This was confirmed by a combination of conventional cerebral angiography and percutaneous sinography.

We advocate that both the correct diagnosis and classification of sinus pericranii can be established with a combination of current noninvasive neuroradiologic studies, especially for the pediatric age group. MRA with the 2D-TOF method is useful in the assessment of this venous anomaly.

Usefulness of MR venography in diagnosing sinus pericranii: case report.

Azusawa H, Ozaki Y, Shindoh N, Sumi Y.

Department of Radiology, Juntendo University Urayasu Hospital, Chiba, Japan.
Radiat Med 2000 Jul-Aug;18(4):249-52 Abstract quote
A case of sinus pericranii communicating with the superior sagittal sinus is reported. Computed tomography (CT), magnetic resonance (MR) imaging, and angiography showed characteristic findings of sinus pericranii.

However, MR venography was useful to visualize the entire lesion.

Subepicranial varix mimicking sinus pericranii: usefulness of three-dimensional computed tomography angiography and bone window computed tomography--case report.

Asano K, Sobata E, Kubo O.

Department of Neurosurgery, Hirosaki University, School of Medicine.
Neurol Med Chir (Tokyo) 2000 Sep;40(9):467-71 Abstract quote
A 16-year-old female presented with a rare case of subepicranial varix in the left temporal area manifesting as a soft mass in the left temporal area when she laid down in the left lateral position. Bulging of the mass was observed when intracranial venous pressure was raised by the Valsalva maneuver, the left lateral position, or the prone position. Bone window computed tomography (CT) revealed a tiny hole, 1 mm in diameter, in the outer bone table.

Three-dimensional CT (3D-CT) angiography clearly visualized a mass with a diameter of approximately 10 mm connected to the diploic vein. The mass was totally resected by operation. Venous bleeding was observed from the tiny hole. Histological examination revealed a venous lesion mimicking sinus pericranii and containing endothelial cells. No communication with the intracranial venous sinuses was identified, so the diagnosis was subepicranial varix.

Radiological examination by direct injection of contrast medium is usually performed to identify subepicranial varix, but 3D-CT angiography is a non-invasive preoperative examination that can visualize this small venous lesion. Adjustment of the CT acquisition conditions may allow 3D-CT angiography to identify sinus pericranii in the future.

Sinus pericranii: advantages of MR imaging.

Bigot JL, Iacona C, Lepreux A, Dhellemmes P, Motte J, Gomes H.

Service d'Imagerie Pediatrique, American Memorial Hospital, Reims, France.
Pediatr Radiol 2000 Oct;30(10):710-2 Abstract quote
Sinus pericranii is a rare vascular anomaly involving an abnormal communication between the extracranial and intracranial circulations. A 3-year-old girl presented with a 2 x 2-cm, midline soft-tissue mass at the vertex. Plain skull films and CT using bone windows showed erosion of the parietal bones.

MRI confirmed the clinical diagnosis by identifying communication of the vascular mass with the intracranial dural venous sinus. The advantages of MRI are discussed.

GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION

GENERAL

VARIANTS

CAVERNOUS HEMANGIOMA

Scalp cavernous angioma presenting as sinus pericranii: diagnostic value of cerebral angiography and magnetic resonance imaging.

Nakayama Y, Tanaka A, Ueno Y, Yoshinaga S, Takano K.

Department of Neurosurgery, Fukuoka University, Chikushi Hospital, Chikushino, Japan.
Childs Nerv Syst 2000 Sep;16(9):598-602 Abstract quote
OBJECTS: Sinus pericranii is only a symptom complex, and it can have a variety of etiologies. Therefore, it is important to differentiate these etiologies preoperatively by means of radiological examinations. A 5-year-old boy was admitted with a soft and fluctuant tumor in the right parietal region near the midline. The tumor appeared when the child was in a recumbent position, distending noticeably with the Valsalva maneuver and disappearing completely when the patient was in the sitting position.

METHODS: Magnetic resonance imaging showed the lesion with honeycomb-like heterogeneous iso- and low-intensity signals on the T1-weighted image and with heterogeneous high- and isointensity signal on the T2-weighted image. Dynamic study with an injection of gadolinium diethylene-triaminopentaacetic acid demonstrated and nodular peripheral enhancement at early phase and subsequent progressive enhancement towards the center of tumor. The internal carotid angiogram was normal. The external carotid angiogram, however, showed a tumor stain fed by the superficial temporal arteries. The stain was retained until the late phase and drained into the scalp veins and into the superior sagittal sinus. Following direct injection of contrast medium into the tumor there was prolonged retention of the medium in the tumor and leakage into scalp veins and the superior sagittal sinus. The mass under the periosteum was totally removed and proved to be a cavernous angioma.

CONCLUSIONS: Scalp cavernous angioma is one of the etiologies of sinus pericranii and may be diagnosed preoperatively by cerebral angiography or magnetic resonance imaging. Serial dynamic magnetic resonance imaging will be particularly helpful for this diagnosis.

HISTOLOGICAL TYPES CHARACTERIZATION

GENERAL

Sinus pericranii: Dermatologic considerations and literature review.

Sheu M, Fauteux G, Chang H, Taylor W, Stopa E, Robinson-Bostom L.

Departments of Dermatology and Pathology, Brown University School of Medicine, Rhode Island Hospital.
J Am Acad Dermatol 2002 Jun;46(6):934-41 Abstract quote
Sinus pericranii is a rare disorder characterized by a congenital or acquired epicranial blood-filled nodule of the scalp that is in communication with an intracranial dural sinus through dilated diploic veins of the skull.

We describe two patients with sinus pericranii: a 3-year-old boy with a congenital lesion and a 3-year-old girl whose lesion appeared after head trauma. We discuss the clinical presentation, dermatologic manifestations, differential diagnosis, and management as described in the available published literature. Patients with sinus pericranii may be brought to the attention of dermatologists and dermatopathologists because of skin changes in the scalp or forehead. The diagnosis is difficult to make clinically, because the skin manifestations are highly variable and may resemble other disorders of the scalp and cranium.

The potentially lethal complications including hemorrhage, infection, and air embolism warrant a high index of suspicion for sinus pericranii.

VARIANTS

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES

VENOUS MALFORMATION

PROGNOSIS AND TREATMENT CHARACTERIZATION

PROGNOSTIC FACTORS

RECURRENCE

Recurrent sinus pericranii in a 14-year-old boy.

Brown JA, Woldenberg L, Velasco ME.

Department of Neurological Surgery, Medical College of Ohio, Toledo 43699-0008.
Childs Nerv Syst 1987;3(3):185-7 Abstract quote
A case of symptomatic and recurrent sinus pericranii in a 14-year-old boy is presented. A blood-filled cyst was diagnosed after head trauma. The sinus pericranii was surgically removed.

One year later the patient presented with blurred vision, headaches, and recurrence at a different site in the skull.

TREATMENT

SURGICAL

Surgical management of cutis aplasia with high-flow sinus pericranii.

Madsen JR, Robertson RL, Bartlett R.

Department of Neurosurgery, Children's Hospital, Harvard Medical School, Boston, Mass 02115, USA.
Pediatr Neurosurg 1998 Feb;28(2):79-83 Abstract quote
Cutis aplasia (cutis aplasia congenita cystica) is a rare congenital disorder with failure of completion of the extracerebral coverings, involving a defect in the scalp and often a cranial defect. A case of cutis aplasia is reported with an associated high-flow venous fistula or sinus pericranii, resulting in altered venous drainage of sagittal sinus blood through dilated scalp veins.

Radiological workup to ensure patency of the superior sagittal sinus was critical to the surgical management in this case.

Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Commonly Used Terms

Brain and Spinal Cord

Skin

Soft Tissue

Last Updated 7/1/2002

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Differential Diagnosis
Prognosis and Treatment
Commonly Used Terms
Internet Links

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
VARIANTS
CAVERNOUS HEMANGIOMA
Scalp cavernous angioma presenting as sinus pericranii: diagnostic value of cerebral angiography and magnetic resonance imaging. Nakayama Y, Tanaka A, Ueno Y, Yoshinaga S, Takano K. Department of Neurosurgery, Fukuoka University, Chikushi Hospital, Chikushino, Japan.	Childs Nerv Syst 2000 Sep;16(9):598-602 Abstract quote OBJECTS: Sinus pericranii is only a symptom complex, and it can have a variety of etiologies. Therefore, it is important to differentiate these etiologies preoperatively by means of radiological examinations. A 5-year-old boy was admitted with a soft and fluctuant tumor in the right parietal region near the midline. The tumor appeared when the child was in a recumbent position, distending noticeably with the Valsalva maneuver and disappearing completely when the patient was in the sitting position. METHODS: Magnetic resonance imaging showed the lesion with honeycomb-like heterogeneous iso- and low-intensity signals on the T1-weighted image and with heterogeneous high- and isointensity signal on the T2-weighted image. Dynamic study with an injection of gadolinium diethylene-triaminopentaacetic acid demonstrated and nodular peripheral enhancement at early phase and subsequent progressive enhancement towards the center of tumor. The internal carotid angiogram was normal. The external carotid angiogram, however, showed a tumor stain fed by the superficial temporal arteries. The stain was retained until the late phase and drained into the scalp veins and into the superior sagittal sinus. Following direct injection of contrast medium into the tumor there was prolonged retention of the medium in the tumor and leakage into scalp veins and the superior sagittal sinus. The mass under the periosteum was totally removed and proved to be a cavernous angioma. CONCLUSIONS: Scalp cavernous angioma is one of the etiologies of sinus pericranii and may be diagnosed preoperatively by cerebral angiography or magnetic resonance imaging. Serial dynamic magnetic resonance imaging will be particularly helpful for this diagnosis.

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
Sinus pericranii: Dermatologic considerations and literature review. Sheu M, Fauteux G, Chang H, Taylor W, Stopa E, Robinson-Bostom L. Departments of Dermatology and Pathology, Brown University School of Medicine, Rhode Island Hospital.	J Am Acad Dermatol 2002 Jun;46(6):934-41 Abstract quote Sinus pericranii is a rare disorder characterized by a congenital or acquired epicranial blood-filled nodule of the scalp that is in communication with an intracranial dural sinus through dilated diploic veins of the skull. We describe two patients with sinus pericranii: a 3-year-old boy with a congenital lesion and a 3-year-old girl whose lesion appeared after head trauma. We discuss the clinical presentation, dermatologic manifestations, differential diagnosis, and management as described in the available published literature. Patients with sinus pericranii may be brought to the attention of dermatologists and dermatopathologists because of skin changes in the scalp or forehead. The diagnosis is difficult to make clinically, because the skin manifestations are highly variable and may resemble other disorders of the scalp and cranium. The potentially lethal complications including hemorrhage, infection, and air embolism warrant a high index of suspicion for sinus pericranii.
VARIANTS

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
VENOUS MALFORMATION

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSTIC FACTORS
RECURRENCE
Recurrent sinus pericranii in a 14-year-old boy. Brown JA, Woldenberg L, Velasco ME. Department of Neurological Surgery, Medical College of Ohio, Toledo 43699-0008.	Childs Nerv Syst 1987;3(3):185-7 Abstract quote A case of symptomatic and recurrent sinus pericranii in a 14-year-old boy is presented. A blood-filled cyst was diagnosed after head trauma. The sinus pericranii was surgically removed. One year later the patient presented with blurred vision, headaches, and recurrence at a different site in the skull.
TREATMENT
SURGICAL
Surgical management of cutis aplasia with high-flow sinus pericranii. Madsen JR, Robertson RL, Bartlett R. Department of Neurosurgery, Children's Hospital, Harvard Medical School, Boston, Mass 02115, USA.	Pediatr Neurosurg 1998 Feb;28(2):79-83 Abstract quote Cutis aplasia (cutis aplasia congenita cystica) is a rare congenital disorder with failure of completion of the extracerebral coverings, involving a defect in the scalp and often a cranial defect. A case of cutis aplasia is reported with an associated high-flow venous fistula or sinus pericranii, resulting in altered venous drainage of sagittal sinus blood through dilated scalp veins. Radiological workup to ensure patency of the superior sagittal sinus was critical to the surgical management in this case.