Sinus pericranii with facial involvement-A great mimicker.

Sinus pericranii is a rare vascular anomaly characterized by an abnormal communication between the intra- and extracranial venous systems through a calvarial defect(s). We present three cases of congenital sinus pericranii with facial involvement, emphasizing its cutaneous presentation with diagnostic pitfalls and discuss the multidisciplinary management of this vascular anomaly.

[Arterial sinus pericranii. First case report]. / Sinus pericranii arterial. Una entidad desconocida.

INTRODUCTION: Sinus pericranii (SP) is a rare vascular anomaly consisting in an abnormal intradiploic communication between intra and extra cranial venous systems. It usually presents as a congenital soft mass in the cranial midline with progressive growth, which enlarges significantly with Valsalva maneuver. A similar congenital vascular anomaly involving intra and extra cranial arterial systems has not been previously described in the medical literature. CLINICAL CASE: A 6-year-old boy with suspected midline capillary malformation of the frontal bone was referred to our Vascular Anomalies Unit for progressive increase in size. A conclusive ultrasound of high flow malformation was performed. Angio-TAC confirmed the arterial nature of the lesion and its communication with the middle meningeal artery. Embolization of the distal portion of the middle meningeal artery was performed prior to surgery. Previous to exeresis of the malformation, skin expanders were placed to cover the subsequent defect. The histopathological result was an arteriovenous malformation. Currently the patient is 10 years old and is asymptomatic, with no recurrence of the lesion. CONCLUSIONS: Since its first description, SP has been classified and described in the literature as a venous malformation. Although morphologically may resemble a SP, histologically and immunohistochemically it corresponds to an arteriovenous malformation with intracranial arterial connection. It is especially important to know how to diagnose this type of cranial malformations because their long-term evolution will depend on correct management and initial follow-up.

INTRODUCCION: El sinus pericranii (SP) es una malformación vascular rara en la que existe una conexión intradiploica entre el sistema venoso intra y extracraneal. Se presenta como una masa blanda en línea media del cráneo con crecimiento progresivo, que aumenta de tamaño con maniobras de Valsalva. Una conexión similar entre los sistemas arteriales intra y extracraneales no ha sido descrita previamente. CASO CLINICO: Niño de 6 años con sospecha de malformación capilar en línea media del hueso frontal fue derivado a nuestra Unidad de Anomalías Vasculares por aumento progresivo de tamaño. Se realizó una ecografía concluyente de malformación de alto flujo. La angio-TAC confirmó la naturaleza arterial de la lesión y su comunicación con la arteria meníngea media. Se realizó una embolización de la porción distal de la arteria meníngea media previa a la cirugía. Previo a la exeresis de la malformación se procedió a colocar expansores cutáneos para poder cubrir el defecto posterior. El resultado histopatológico fue de una malformación arteriovenosa. Actualmente el paciente tiene 10 años y se encuentra asintomático, sin recidiva de la lesión. CONCLUSION: Desde su primera descripción, el SP se ha clasificado y descrito en la literatura como una malformación venosa. Aunque morfológicamente aparentase de un SP, histológica e inmunohistoquimicamente corresponde una malformación arteriovenosa con conexión arterial intracraneal. Es de especial importancia saber diagnosticar este tipo de malformaciones craneales porque su evolución a largo plazo dependerá de un correcto manejo y seguimiento inicial.

[Sinus pericranii; a congenital swelling on the scalp]. / Sinus pericranii; een congenitale zwelling op het behaarde hoofd.

BACKGROUND: Sinus pericranii is a rare congenital disorder of the skull. It involves a venous connection between the intracranial and extracranial venous systems caused by a defect at the level of a cranial sinus. CASE DESCRIPTION: We present the case of a 20-year-old woman with a soft compressible swelling on the back of her head from birth. MRI examination revealed sinus pericranii. The treatment consisted of coagulating the venous connection and closing the cranial malformation. CONCLUSION: In a patient with soft-tissue swelling on the back of the head that has been present since birth, differential diagnostics should extend beyond epidermoid cysts alone and also include consideration of sinus pericranii. This is particularly important if the swelling is in the midline.

Clinical and imaging findings in a rare case of sinus pericranii.

BACKGROUND: Sinus pericranii is a rare, usually asymptomatic condition that is characterized by an abnormal communication between the intra- and extracranial venous drainage pathways. The etiology is unknown but both congenital and post-traumatic etiologies have been proposed. Treatment is primarily surgical but newer minimally invasive endovascular approaches have been reported and is indicated due to cosmesis, hemorrhage, and air embolism. ILLUSTRATIVE CASE: We present a case of an 11-month-old boy having sinus pericranii, who was referred for a slowly growing tumor located frontally in the midline on his scalp since 6 months of age. CT-scan with three-dimensional CT (3D-CT) reconstruction and magnetic resonance imaging along with venography was performed which confirmed the diagnosis. Simple surveillance was decided because of a limited esthetic prejudice and the absence of any functional disorder. CONCLUSION: The prognosis is nearly always good with a low risk of bleeding. A simple follow-up is often proposed because of the usual absence of complications.

Rare vascular pathology sinus pericranii; becomes symptomatic with pseudotumor cerebri.

Sinus pericranii (SP) is an uncommon vascular pathology that is characterized by a nodular structure emerging from the scalp. It is generally asymptomatic. Typically, it is aclose to soft, compressive, fluctuant swelling, intracranial sinus. The swelling increases during the Valsalva maneuver, jugular vein compression, coughing, or crying attacks. The diagnosis was confirmed by CT, MRI, and MR angiography. Here, we conclude that SP is a rare cause of headache and that the asymptomatic lesion become symptomatic as a result of association with pseudotumor cerebri.

Sinus pericranii: diagnosis and management in 21 pediatric patients.

OBJECT: Sinus pericranii (SP) is a rare venous anomaly abnormally connecting the intracranial dural sinuses with the epicranial veins. In the present study the authors aimed to clarify this clinicoradiological entity, define the role of angiography in its preoperative assessment, and suggest a diagnostic-therapeutic flow chart for management purposes. METHODS: The authors retrospectively reviewed the clinical charts and neuroimages of 21 patients with SP. All patients underwent brain MRI, MR venography, and craniocerebral CT. Diagnostic digital subtraction angiography was performed in 19 of 21 patients, and the SPs were categorized as dominant (draining the majority of the intracranial venous outflow) or accessory (draining only a minority of the intracranial venous outflow). RESULTS: SP was median or paramedian in 20 patients and lateral in 1 patient. There were 5 dominant and 14 accessory SPs. The dominant SPs were not treated. Among the patients with accessory SP, 4 were not treated, 2 underwent surgical ligature, and 8 were treated endovascularly (with either transvenous or percutaneous embolization). No complications were observed, and symptoms disappeared after treatment in all cases. CONCLUSIONS: Accepted guidelines or recommendations concerning the management, diagnosis, and treatment of SP are still lacking. The authors define here a diagnostic-therapeutic flow chart, in which angiography plays a crucial role in the classification of SP and choice of the optimal treatment. Only accessory SP is amenable to treatment, whereas dominant SP must be preserved. The endovascular approach is becoming increasingly relevant and has proven to be safe and effective.

[Pericranial sinus. Definition, diagnosis, surgical treatment].

Pericranial sinus (Sinus pericranii - SP) is a rare pathology of the extra-intracranial cerebral venous system. However, SP is not just an additional transosseous canal that connects the extra- and intracranial venous systems. This "emissary vein" connects the intracranial sinus and the variceally extended thin-walled veins localized on the outer surface of the skull where blood flows fun in opposite directions. We present a literature review and two case reports of patients with pericranial sinus who underwent surgical treatment. We discuss the problems related to etiology, clinical signs, diagnosis, and surgical treatment of the anomaly.

Malformación venosa. ¿De qué se trata? «Sinus pericranii» asociado a una malformación compleja del desarrollo venoso cerebeloso / Venous malformation, what is it? Sinus pericranii associated complex venous malformation of cerebellar development

Introducción: El sinus pericranii (SP) es una rara malformación vascular que consiste en una comunicación venosa entre los senos durales intracraneales y las venas epicraneales, que se suelen dilatar. Caso clínico: Presentamos el caso de un niño de 2 años de edad con una lesión vascular en la región parietal derecha, de coloración azulada, que de forma progresiva se ingurgitaba en decúbito lateral derecho y con el llanto. En las pruebas de imagen se detectó una lesión de localización subgaleal con adelgazamiento local de la calota, acompañada de 2 angiomas venosos cerebelosos. Se sospechó un SP asociado a una malformación venosa cerebelosa. El SP se intervino quirúrgicamente y la malformación se mantiene bajo control multidisciplinario. Conclusión: Destacamos que el SP puede asociarse a otras malformaciones del desarrollo vascular intracraneal. El tratamiento quirúrgico del sinus puede estar indicado por problemas estéticos y posibles complicaciones locales, principalmente la trombosis, el sangrado o la ulceración. El abordaje de las malformaciones intracraneales extensas resulta complejo, ya que pueden drenar zonas funcionalmente significativas del cerebro (AU)

Introduction: The sinus pericranii (SP) is a rare vascular malformation consists of a venous communication between the intracranial dural sinuses and epicranial veins, which are usually dilated. Clinical case: We report the case of a tolder with a vascular bluish lesion in his right parietal region, which progressively engorged when patient lies down to right lateral decubites or while is in tears. Findings images detected a subgaleal lesion location with a local shell thinning, together with two cerebellar venous angiomas. SP was suspected with complex cerebellar vascular malformation. The SP was surgical removed and the malformation is kept under multidisciplinar control. Conclusion: We emphasize that the SP may be associated with other intracranial vascular malformations development. The sinus surgical may be indicated for cosmetic problems and local complications particularly thrombosis, bleeding or ulceration. The approach of extensive intracranial malformations is complex, since they can drain functionally brain areas (AU)

Sinus pericranii. Serie de 5 casos / Sinus pericranii. A series of 5 cases

El sinus pericranii es una malformación vascular rara en la cual existe una comunicación anormal entre el sistema venoso extracraneal y los senos venosos durales. La historia natural, en la mayoría de casos, es la de una anomalía vascular en línea media craneal desde la nariz hasta el occipucio, que aumenta progresivamente de tamaño. Las opciones terapéuticas son diversas: desde la observación de la lesión hasta la embolización del componente intracerebral o la exéresis completa tanto del componente extra como intracraneal. En todas estas circunstancias pueden producirse complicaciones potencialmente letales, como son la trombosis y la hemorragia cerebrales. Describimos nuestra experiencia en el manejo del sinus pericranii, destacando la importancia del abordaje terapéutico multidisciplinar de esta infrecuente entidad (AU)

Sinus pericranii is a rare vascular anomaly in which an abnormal communication exists between the extracranial venous system and the dural venous sinuses. The natural history in most cases consists of a purplish nodule in the frontal region that may gradually increase in size. Different treatment options can be chosen: from observation of the lesion, to endovascular embolization of intracerebral component or a complete resection of both anomalous components (extra- and intra-cranial) by surgery. In this context, potential life-threatening complications including thrombosis and cerebral hemorrhage can occur. Here we present our experience in management of sinus pericranii, and emphasize the importance of a multidisciplinary therapeutic approach of this uncommon entity (AU)

[Sinus pericranii. A series of 5 cases]. / Sinus pericranii. Serie de 5 casos.

Sinus pericranii is a rare vascular anomaly in which an abnormal communication exists between the extracranial venous system and the dural venous sinuses. The natural history in most cases consists of a purplish nodule in the frontal region that may gradually increase in size. Different treatment options can be chosen: from observation of the lesion, to endovascular embolization of intracerebral component or a complete resection of both anomalous components (extra- and intra-cranial) by surgery. In this context, potential life-threatening complications including thrombosis and cerebral hemorrhage can occur. Here we present our experience in management of sinus pericranii, and emphasize the importance of a multidisciplinary therapeutic approach of this uncommon entity.

Surgical treatment of scaphocephaly with sinus pericranii.

A 1-year-old female was admitted with a subcutaneous, pulsatile soft mass in the midline parietal region, and abnormal head shape. Fundus examination showed papilledema, suggesting elevated intracranial pressure. Radiological findings showed sagittal suture craniosynostosis with sinus pericranii. Magnetic resonance venography showed that the drainage through the sinus pericranii was not essential for the venous outflow from the brain. The patient underwent surgical resection of the sinus pericranii and total cranial remodeling. Ligation of the stalk-like orifice attached to the superior sagittal sinus with resection of the sinus pericranii and total cranial reconstruction were performed concurrently. The postoperative course was uneventful, and her papilledema resolved. No recurrence of the sinus pericranii has occurred for 3 years. This case describes a unique one-staged operation to treat sinus pericranii with sagittal suture craniosynostosis.

Cerebrofacial venous anomalies, sinus pericranii, ocular abnormalities and developmental delay.

The clinical implications of venous cerebrovascular maldevelopment remain poorly understood. We report on the association of cerebrofacial venous anomalies (including sinus pericranii), ocular abnormalities and mild developmental delay in two children. In addition, one child had a seizure disorder. Complex cerebrofacial slow-flow vascular anomalies may herald an underlying developmental aberration affecting the cerebrofacial and orbital regions.

Sinus Pericranii: a case report and review of literature.

Normally, there is no obvious communication between the intracranial and extra cranial venous drainages in the head. In Sinus Pericranii, there is an abnormal communication, either from the extra cranial system to the intracranial venous sinuses or from the intracranial venous system to the extra cranial draining veins. Venous anomaly is a collection of non muscular venous blood vessels, adhering tightly to the outer surface of the skull and directly communicating with an intracranial venous sinus through diploic veins. The varicosities are intimately associated with the periostium, are distensible, and vary in size with changes in intracranial pressure Sinus pericranii is not a single clinico pathologic entity, rather a symptom complex with diverse clinical manifestations.In this article,the authors present a case of 8 mo old boy having Sinus Pericranii.

Sinus pericranii.

Sinus pericranii is a rare anomaly that occurs when there is communication between the intracranial and extracranial venous systems. Accurate diagnosis is complicated because several other cranial masses can mimic sinus pericranii. Clinical, laboratory, and imaging assessments are all essential for identifying the anomaly. This article examines the comparative advantages of various diagnostic imaging modalities, current imaging techniques, and typical findings associated with sinus pericranii. Treatment and management options also are discussed.

Atretic parietal cephalocele associated with sinus pericranii: embryological consideration.

We report a case of atretic parietal cephalocele with a persistent parietal falcine sinus and partial absence of the straight sinus. The direct puncture angiographic study demonstrated that there was a major venous channel through the parietal skull defect. From the embryological point of view, this association may provide us with possible pathoetiologic evidence of congenital sinus pericranii is one of the alternative venous drainage pathways to compensate venous outflow in the cases of intracranial developmental anomalies.

Sinus pericranii: an overview and literature review of a rare cranial venous anomaly (a review of the existing literature with case examples).

Sinus pericranii is a rare vascular abnormality characterised by abnormal connections between the intra- and extracranial venous systems and is usually found in children. In most instances, a sinus pericranii presents as a soft scalp swelling that appears with the patient in the recumbent position and disappears in the erect position. We review two cases of sinus pericranii presented in adulthood and treated surgically with good outcomes. We have performed a search of the English literature using the PubMed database and reviewed the published cases to date to present an overview of this pathological entity.

Scalp arteriovenous malformation associated with a superior sagittal sinus, sinus pericranii.

Scalp arteriovenous malformations (AVMs) and sinus pericranii are two rare vascular lesions of the scalp that are part of the differential diagnosis in patients with scalp masses. The necessity of treatment of sinus pericranii, due to its risk of catastrophic hemorrhage or scalp necrosis, makes its diagnosis imperative. There are several theories on the pathogenesis of sinus pericranii with some of them stressing the importance of altered hemodynamic parameters in the affected area. An 8-year-old girl presented with a five centimeter soft, reducible scalp mass that had been present since birth. Clinical and radiographic evaluation revealed a sinus pericranii in association with a scalp AVM. She underwent resection of the sinus pericranii followed by embolization and planned separate en bloc resection of the AVM. During resection of the scalp AVM at a later date, the sinus pericranii was noted to have recurred. Concurrent treatment of all vascular anomalies associated with a sinus pericranii appears to be the key to its complete eradication.

Sinus pericranii--"Don't judge a lump by its surface".

An interesting and unusual vascular malformation, sinus pericranii is presented. Originally referred as a cyst, a "community conundrum" was solved and treated successfully in the hospital setting. A discussion of sinus pericranii follows, and the teaching values of this particular case are highlighted.