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Key Clinical Message: A 17‐year‐old girl with a congenital, painless right forehead swelling obstructing her visual field was diagnosed with sinus pericranii. Radiological imaging confirmed extracalvarial arteriovenous malformation with serpentine vessels and bony erosion. Endovascular surgery was chosen for management, highlighting the necessity of considering SP in differential diagnosis for scalp swellings. Despite its rarity, awareness of it is essential to prevent complications from injury, misdiagnosis, or invasive procedures. [ABSTRACT FROM AUTHOR]

Sinus pericranii is a rare venous anomaly characterized by abnormal communication between intracranial and subperiosteal veins, and reports of trauma-induced sinus pericranii are even rarer. Herein, we report a case of delayed-onset sinus pericranii resulting from a traumatic injury to the left side of the midline of the forehead sustained in early childhood. The anomaly was successfully resected via a coronal incision within the hairline, followed by meticulous bone wax sealing. In this paper, we aim to provide details on the diagnosis and surgical techniques of trauma-induced sinus pericranii, contributing valuable insights for the management of such rare condition.

Key Clinical Message A 17‐year‐old girl with a congenital, painless right forehead swelling obstructing her visual field was diagnosed with sinus pericranii. Radiological imaging confirmed extracalvarial arteriovenous malformation with serpentine vessels and bony erosion. Endovascular surgery was chosen for management, highlighting the necessity of considering SP in differential diagnosis for scalp swellings. Despite its rarity, awareness of it is essential to prevent complications from injury, misdiagnosis, or invasive procedures.

Abstract Background Sinus pericranii is a rare cranial venous malformation resulting in a subcutaneous mass due to abnormal communication between intracranial and subperiosteal/interperiosteal veins. To date, to the best of our knowledge, there are no reports of sinus pericranii associated with syntelencephaly, a subtype of lobar holoprosencephaly. We herein report a case of sinus pericranii associated with syntelencephaly. This report can provide us better understanding of the etiology of sinus pericranii, the potential risks, and the treatment options for these patients. Case presentation A 2-year-4-month old female patient who received the diagnosis of syntelencephaly as a neonate presented with a subcutaneous mass in the parietal region. The mass was soft, nonpulsatile, 3 × 2 cm in size, and showed enlargement in the lying position. Color cranial Doppler ultrasound, head magnetic resonance imaging (MRI), and cerebral angiography revealed a dilated vessel passing through the parietal bone and forming a communication between the superior sagittal sinus and scalp veins. Based on these findings, sinus pericranii was diagnosed. The head MRI also showed coronal craniosynostosis, a tight posterior fossa. At age 2 years and 7 months, the patient underwent a transection of the sinus pericranii and the mass resolved without any complications or recurrences for more than 2.5 years to date. Conclusion Sinus pericranii is a rare cranial and venous malformation sometimes accompanied by brain malformations or craniosynostosis that may become more apparent as the brain and skull develop. Since this condition can be complicated by intracranial hemorrhage and sinus thrombosis, early detection is necessary to determine the treatment options. Physicians should be alert to the possibility of this condition if they observe a soft cranial mass that appears to decrease in size in the sitting position and bulge in the lying position.

Abstract Background Sinus pericranii (SP) is a rare venous anomaly abnormally connecting the intracranial dural sinuses with the epicranial venous channels. Various authors have attempted to classify this entity to decide management for this potentially life-threatening condition. With approximately 200 cases reported in world literature till date, no definitive guidelines for management have emerged. Case presentation A female child aged 7 years presented to us with complaints of occipital swelling since birth. This swelling was gradually increasing in size as reported by the parents. On examination, the swelling was located in the right parieto-occipital region, size 10 cm diameter in lying position, soft, non-tender, fluid-like diffuse swelling with underlying areas of palpable bony defects, partially reducible in sitting up position, and cough impulse was present. On evaluation, a case of dominant SP was established. Conclusion Traditionally, SP has been reported mostly in paediatric population and managed surgically or endovascularly for selected cases, while the remaining cases have been observed. Profuse haemorrhage has been a major deterrent to manage these lesions surgically. Long-term complications like trauma, haemorrhage, infection and thrombosis have been reported in conservatively managed cases. The neurosurgical community has thus been left searching for the optimal management of SP. The authors report this case attempting to classify this case according to the available classification systems and hence decide the best possible management.

Background: Sinus pericranii is a rare cranial venous malformation resulting in a subcutaneous mass due to abnormal communication between intracranial and subperiosteal/interperiosteal veins. To date, to the best of our knowledge, there are no reports of sinus pericranii associated with syntelencephaly, a subtype of lobar holoprosencephaly. We herein report a case of sinus pericranii associated with syntelencephaly. This report can provide us better understanding of the etiology of sinus pericranii, the potential risks, and the treatment options for these patients.Case Presentation: A 2-year-4-month old female patient who received the diagnosis of syntelencephaly as a neonate presented with a subcutaneous mass in the parietal region. The mass was soft, nonpulsatile, 3 × 2 cm in size, and showed enlargement in the lying position. Color cranial Doppler ultrasound, head magnetic resonance imaging (MRI), and cerebral angiography revealed a dilated vessel passing through the parietal bone and forming a communication between the superior sagittal sinus and scalp veins. Based on these findings, sinus pericranii was diagnosed. The head MRI also showed coronal craniosynostosis, a tight posterior fossa. At age 2 years and 7 months, the patient underwent a transection of the sinus pericranii and the mass resolved without any complications or recurrences for more than 2.5 years to date.Conclusion: Sinus pericranii is a rare cranial and venous malformation sometimes accompanied by brain malformations or craniosynostosis that may become more apparent as the brain and skull develop. Since this condition can be complicated by intracranial hemorrhage and sinus thrombosis, early detection is necessary to determine the treatment options. Physicians should be alert to the possibility of this condition if they observe a soft cranial mass that appears to decrease in size in the sitting position and bulge in the lying position. [ABSTRACT FROM AUTHOR]

Background: Sinus pericranii (SP) is a rare venous anomaly abnormally connecting the intracranial dural sinuses with the epicranial venous channels. Various authors have attempted to classify this entity to decide management for this potentially life-threatening condition. With approximately 200 cases reported in world literature till date, no definitive guidelines for management have emerged. Case presentation: A female child aged 7 years presented to us with complaints of occipital swelling since birth. This swelling was gradually increasing in size as reported by the parents. On examination, the swelling was located in the right parieto-occipital region, size 10 cm diameter in lying position, soft, non-tender, fluid-like diffuse swelling with underlying areas of palpable bony defects, partially reducible in sitting up position, and cough impulse was present. On evaluation, a case of dominant SP was established. Conclusion: Traditionally, SP has been reported mostly in paediatric population and managed surgically or endovascularly for selected cases, while the remaining cases have been observed. Profuse haemorrhage has been a major deterrent to manage these lesions surgically. Long-term complications like trauma, haemorrhage, infection and thrombosis have been reported in conservatively managed cases. The neurosurgical community has thus been left searching for the optimal management of SP. The authors report this case attempting to classify this case according to the available classification systems and hence decide the best possible management. [ABSTRACT FROM AUTHOR]

Purpose: to better understand the pathological process of sinus pericranii and the safety of the surgical intervention. Methods: patients’ archive review of patients who underwent surgical management for sinus pericranii with a confirmed diagnosis and with follow up period greater than 6 months. Results: 6 infants were included; all underwent disconnection surgically with good outcome in all cases. Conclusion: disconnection surgery for sinus pericranii is a safe procedure with a good prognosis.

Purpose: to better understand the pathological process of sinus pericranii and the safety of the surgical intervention. Methods: patients' archive review of patients who underwent surgical management for sinus pericranii with a confirmed diagnosis and with follow up period greater than 6 months. Results: 6 infants were included; all underwent disconnection surgically with good outcome in all cases. Conclusion: disconnection surgery for sinus pericranii is a safe procedure with a good prognosis. [ABSTRACT FROM AUTHOR]

Background: Significant advances have been reported recently in the genetic and mechanistic characterization of extracranial venous malformations. However, intracranial purely venous malformations (icVM) analogous to those outside the CNS have not been systematically described. Purpose: We sought to ascertain whether such an entity as icVM could in fact be identified, distinct from previously described CNS venous anomalies and analogous to extracranial venous malformations. Methods: Our prospectively collected pediatric cerebrovascular database was reviewed to identify patients with icVM; 1458 consecutive angiograms and/or angiographic interventions performed on 706 children at our institution from October, 2006 through May, 2019 were evaluated, in addition to outside imaging studies on 192 additional patients sent to our Vascular Anomalies Center for cerebrovascular review during the same time period. Thus, the cohort consisted of 898 children. Results: Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. There was no intracranial hemorrhage or venous hypertension seen in the cohort. Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. Conclusion: Venous malformations, both extracranial and icVM, share many characteristics that are distinct from developmental venous anomalies. icVM were not associated with venous hypertension. The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. [ABSTRACT FROM AUTHOR]

Sinus pericranii is a rare vascular anomaly, and most cases occur in children and develop at the midline. In previous reports of sinus pericranii, T2 hyperintensity lesion has not been regarded as a common sequela. We report an extremely rare case of orbital sinus pericranii with associated T2 hyperintensity lesion. A 50-year-old man was admitted to our hospital with a history of right upper eyelid swelling that had been present for several years. Computed tomography, magnetic resonance imaging, and digital subtraction angiography demonstrated a connection between the lesion and normal cerebral venous system. Thus, we diagnosed the lesion as a sinus pericranii despite its atypical features. We elected to observe the patient, and the lesion had remained the same size without any adverse events, such as hemorrhage, occurring throughout the 5-year follow-up. An atypical sinus pericranii should be considered in patients with a soft compressible swelling on the head, even if the lesion is located off the midline.

Sinus pericranii is a rare vascular anomaly characterized by abnormal venous communication between the inner and outer regions of the cranial cavity. Here, we report a case of sinus pericranii and venous malformations in the right periorbital region of a 2-year-old girl. Radiologic findings showed venous malformations in the right parietal region communicating with the superior sagittal sinus in the intracranial region. There were notable improvements following surgical resection for the abnormal venous lesions and several sclerotherapies. Presence of a bluish and pulsating mass on the scalp, which showed bruit on auscultation, may indicate sinus pericranii, which should be included in the differential diagnosis. [ABSTRACT FROM AUTHOR]

Sinus pericranii (SP) is a rare vascular anomaly of the scalp that consists of an abnormal pericranial venous channel connected to adjacent dural venous sinuses. Most SP are asymptomatic and are found in the pediatric age group. We aim to report a case of symptomatic SP in adult and describe the clinical, radiological, and pathohistological findings to help understand and differentiate this lesion from other scalp lesions. A 40-year-old man with a scalp mass was admitted to our hospital complaining of headache. The lesion enlarged when the patient was in a recumbent position or during Valsalva maneuver. The radiologic imaging suggested its diagnosis as an accessory type of SP with bone erosion. Surgical resection and cranioplasty were successfully performed, and the related headache also gradually subsided. At the 3-year follow-up, there was no recurrence on magnetic resonance imaging. [ABSTRACT FROM AUTHOR]

Introduction. Sinus pericranii is a rare vascular anomaly. It is characterized by abnormal communication between the extracranial and intracranial venous system, usually involving the superior sagittal sinus and occasionally the transverse sinus. Off the midline lesions are extremely rare. Multiplicity, associated venous lakes, venous angioma and lateral location are unusual and unique presentation of sinus pericranii. Case report. A case of multiple congenital off-midline sinus pericranii in the left frontotemporal and parietal region is presented. Magnetic resonance imaging showed an extracranial vascular anomaly connected with the intracranial venous system through abnormal diploic or emissary veins. The lesions were removed completely by surgery. Conclusion. Sinus pericranii is a rare vascular malformation with unique clinical and radiological features. Sinus pericranii may cause fatal complications, and it must be treated by surgical or endovascular procedures.

Background: Sinus pericranii (SP) is a rare vascular malformation consisting of an abnormal communication between the extra- and the intracranial venous system. It occurs due to the adhesion of vessels without a muscular layer or a hemangioma on the outer surface of the skull through diploic vessels, communicating with an intracranial venous sinus. Case Description: A 10-month-old female presented with a pulsatile mass on the posterior parietal region. Investigation with brain vascular examinations showed a venous malformation communicating with the superior sagittal sinus under the scalp, without arterial feeder or nidus. An endovascular embolization with coils and a percutaneous embolization with Onyx ® were performed. The final venography showed complete exclusion of the lesion, which was gradually being absorbed. Conclusion: This is the first case of an SP successfully treated in a single session by embolization with coils and onyx. [ABSTRACT FROM AUTHOR]

Aims: Scalp arterio-venous malformation (AVM) and scalp venous malformation (SVM) are rare conditions that usually need surgical treatment. Here, we have reported our experience of the surgical management of such lesions with a short review of the literature. Materials and Methods: In this prospective study, 11 patients with scalp AVM and SVM, who underwent surgical excision of lesion in our hospital from 2006 to 2012, were included. All suspected high-flow AVM were investigated with the selective internal and external carotid digital subtraction angiogram (DSA) ± computed tomography (CT) scan of brain with CT angiogram or magnetic resonance imaging (MRI) of brain with MR angiogram, and all suspected low-flow vascular malformation (VM) was investigated with MRI of brain + MR angiogram. Eight were high-flow and three were low-flow VM. Results: All lesions were successfully excised. Scalp cosmetic aspects were acceptable in all cases. There was no major post-operative complication or recurrence till last follow-up. Conclusions: With preoperative appropriate surgical planning, scalp AVM and SVM can be excised without major complication.

Les lacunes de la voûte du crâne sont observées sur environ 40% des radiographies standard du crâne. Elles sont découvertes le plus souvent de façon fortuite lors d'un bilan radiologique pour sinusite ou traumatisme crânien ou de façon systématique, au cours du bilan d'extension d'hémopathies ou de cancers ostéophiles. Elles sont exceptionnellement révélatrices d'un sinus pericranii. Il s'agit d'une anomalie vasculaire rare réalisant un drainage veineux entre les systèmes extra- et intracrâniens. Le diagnostic repose sur le scanner et/ou l'IRM, qui visualisent une importante prise de contraste de la lésion. Le traitement chirurgical est assez lourd et rarement indiqué. Nous rapportons l'observation d'une patiente âgée de 53 ans, aux antécédents de carcinome basocellulaire du nez, adressée pour bilan étiologique d'une lacune du crane. Le bilan biologique (numération formule sanguine, vitesse de sédimentation, protéine C-réactive, calcémie, électrophorèse des protéines) était normal. La tomodensitométrie du massif facial objectivait une image lacunaire de 9 mm à l'emporte pièce de l'os temporal gauche. A l'imagerie par résonance magnétique, la lacune est en regard du sinus transverse gauche avec lequel elle se continue et se rehausse de la même façon que le sinus après injection de gadolinium. Le diagnostic de sinus pericranii a été retenu. Devant l'absence de plainte fonctionnelle une simple surveillance a été décidée.

Background: Vascular anomalies accompanied with the diploic veins are rare. Among them, sinus pericranii, which is characterized by abnormal connections between intra- and extracranial venous systems, is relatively common. Besides sinus pericranii, a few cases of subepicranial varix with connections to diploic veins have been reported, but these varices had no connections to intracranial venous sinuses. Herein, we present a rare case of an expanding venous sac in the diploe which communicated with the intracranial sinus but not with the extracranial venous systems. Case presentation: An adult woman presented to us with a minor transient headache. Although no abnormal appearances were found on her scalp, imaging studies showed a club-shaped venous sac in the left parietal diploe that communicated with the superior sagittal sinus and diploic veins on the medial and lateral sides, respectively. It was revealed that the lesion had expanded as compared with a previous computed tomography image. Surgery was performed to intercept venous supply from the diploic veins, and the lesion was filled with thrombi. In a follow-up of 15 months, there was no recurrence of abnormal venous flow. Histological examination showed the endothelial lining in the membranous wall of the sac, which is typically observed in sinus pericranii. However, no communication with the pericranial veins of the scalp was identified on the imaging studies and intraoperative observation. Accordingly, it was diagnosed as another entity 'intradiploic varix'. Conclusions: The abnormal connection between the intracranial and the diploic venous systems via the large venous sac was surgically treated. It was pathologically similar to sinus pericranii, but anatomically considered to be another form of venous anomaly. In cases of expanding lesions, surgical treatment is recommended. [ABSTRACT FROM AUTHOR]

Background: Sinus pericranii (SP) is a rare vascular anomaly, with an uncertain etiology. Often discovered as superficial lesions, they can be primary or secondary in nature. Herein, we report a rare case of SP in the setting of a large posterior fossa pilocytic astrocytoma associated with a significant venous network., Observations: A 12-year-old male presented with acute clinical deterioration in extremis with a 2-month history of lethargy and headaches. Outside plain computed tomography imaging revealed a large posterior fossa cystic lesion, probably a tumor, with severe hydrocephalus. There was also a midline small skull defect at the opisthocranion, without visible vascular anomalies. An external ventricular drain was placed with rapid recovery. Contrast imaging revealed a large midline SP emanating from occipital bone with a large intraosseous, and subcutaneous venous plexus in the midline draining inferiorly into venous plexus around the craniocervical junction. A posterior fossa craniotomy without contrast imaging could have resulted in catastrophic hemorrhage. A small modified off-center craniotomy provided access to the tumor with a gross total excision., Lessons: SP is a rare but significant phenomenon. Its presence does not necessarily preclude resection of underlying tumors, provided that a careful preoperative assessment of the venous anomaly is undertaken.

Aims: Scalp arterio-venous malformation (AVM) and scalp venous malformation (SVM) are rare conditions that usually need surgical treatment. Here, we have reported our experience of the surgical management of such lesions with a short review of the literature. Materials and Methods: In this prospective study, 11 patients with scalp AVM and SVM, who underwent surgical excision of lesion in our hospital from 2006 to 2012, were included. All suspected high-flow AVM were investigated with the selective internal and external carotid digital subtraction angiogram (DSA) ± computed tomography (CT) scan of brain with CT angiogram or magnetic resonance imaging (MRI) of brain with MR angiogram, and all suspected low-flow vascular malformation (VM) was investigated with MRI of brain + MR angiogram. Eight were high-flow and three were low-flow VM. Results: All lesions were successfully excised. Scalp cosmetic aspects were acceptable in all cases. There was no major post-operative complication or recurrence till last follow-up. Conclusions: With preoperative appropriate surgical planning, scalp AVM and SVM can be excised without major complication. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

nomalous connections between an extracranial venous sac and intracranial dural sinuses through dilated diploic and emissary veins of the skull result in sinus pericranii (SP). In this study, two patients with the rare presentation of multiple, congenital SP with associated dural venous lakes and venous anomalies are described. In one patient, multiple SPs were located in the frontal, parasagittal region with an associated subcortical venous angioma; and, in the other, peritorcular and juxta-transverse-sigmoid sinus junction SP coexisted. The venous anomalies drained into venous lakes in close proximity to major sinuses. They also communicated with extracranial tributaries via interosseous veins leading to the development of venous hypertension that presumably caused pressure erosion of the skull. This may have been responsible for the pathogenesis of multiple subgaleal venous sacs of SP and may also lead to profuse hemorrhage, cortical venous thrombosis, or air embolism. Multiplicity, associated venous lakes, venous angioma, and a lateral location are unique presentations of SP. Sac excision, transcranial venous anastomotic channel blockage, and reinforcement/replacement of the underlying bone are the recommended modalities of treatment. [ABSTRACT FROM AUTHOR]

Sinus pericranii is a rare venous anomaly, representing a transosseous connection between the intracranial venous system and the epicranial venous system. We present an unusual case of bilateral frontal sinus pericranii in a 12-year-old boy, with associated lacrimation. Instead of the usual short bridging vein between the intra- and extracranial venous circulation, in our case the veins connecting the superior sagittal sinus and the left superior ophthalmic/orbital vein coursed intratabularly over a distance of several centimeters. To our knowledge, such a course has not previously been reported in literature. [ABSTRACT FROM AUTHOR]

Sinus Pericranii is a rare venous anomaly in which the communication between the intraand extracranial venous system is constituted by a network of thin-walled veins that form a varix on the external table of the skull. This varix is continuous with the pericranial veins of the scalp. We describe a 31-year-old woman with frontal sinus pericranii treated using an original endovascular technique by transvenous approach combined with direct punction. [ABSTRACT FROM AUTHOR]

The improved knowledge of the clinical and haemodynamical aspects of extracranial arteriovenous malformations in the 18th century provided a foundation for the understanding and treatment of cerebrovascular pathology. It was not until the late 19th century that detailed clinicopathological reports of intracranial arteriovenous malformations were published. A seminal report by the Italian surgeon Francesco Rizzoli described Giulia, a 9-year-old girl who presented with seizures and an occipital pulsanting swelling in 1873. Rizzoli used Giulia’s signs and symptoms to predict the complex angioarchitecture of her “arteriovenous aneurysm passing through the wall of skull”. Postmortem examination confirmed the supposed diagnosis, and showed a direct communication between the hypertrophic branches of the ocipital artery and the transverse sinus. The clinical course of that case is briefly reviewed in this article and the diagnosis of this unusual arteriovenous shunt is discussed in light of current neurosurgical knowledge. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We report the case of a 10-year-old boy that presented with a palpable, painless, frontal lesion. Laboratory assessments were unremarkable and the patient was asymptomatic. Initial investigation, with a skull radiograph and unenhanced CT scan, showed a lytic midline frontal lesion involving the inner and outer tables of the skull and a large subgaleal hypodense component. MRI further depicted communication with the epidural space and contact with the superior sagittal sinus (SSS). Subsequent evaluation by Doppler ultrasound and MR angiography excluded a sinus pericranii and showed normal patency of the SSS. Surgical biopsy revealed chronic granulomatous inflammation; PCR was positive for Mycobacterium sp. One year after surgical resection and antitubercular therapy, there are no signs of recurrence. Primary calvarial involvement by tuberculosis is rare, even in developing countries. Familiarity with the expected clinical and imaging features is required to avoid diagnostic delay., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

We present a rare case of right parietal sinus pericranii in a 2-year-old female child who presented with a compressible swelling on the right side of the scalp since 3 months of age, with a large head. Magnetic resonance imaging along with venography and conventional angiogram was performed, which confirmed the diagnosis. [ABSTRACT FROM AUTHOR]