Atretic cephalocele associated with sinus pericranii: a single-center analysis.

PURPOSE: The purpose of this report is to investigate the association of atretic cephalocele (AC) with sinus pericranii (SP) in order to improve the management of AC. METHODS: Pediatric patients with AC who underwent repair surgeries were retrospectively analyzed. Anomalies including SP were evaluated using computed tomography angiography and venography (CTA/V) as well as magnetic resonance imaging. RESULTS: Fourteen patients were identified, comprising of 5 males and 9 females. The AC was located interparietally in 8 cases and occipitally in 6 cases. Seven cases (50%) were found to be associated with SP, all of which did not involve major intracranial venous circulation. Five out of 8 parietal ACs (63%) were accompanied by SP, while 2 out of 6 occipital ACs (33%) exhibited SP. All of the SP associated with AC only contributed to a small portion of the venous outflow, and the repair surgeries were successfully performed without excessive bleeding. CONCLUSION: More than half of the ACs were associated with SP, with a higher prevalence in the parietal region compared to the occipital region. For the appropriate management of AC, preoperative investigation of SP using CT venography is considered important.

Rapidly growing sinus pericranii within a short period: a case report.

INTRODUCTION: Sinus pericranii (SP) is a rare vascular malformation characterized by abnormal communication between the intracranial and extracranial venous systems [1]. Sinus pericranii is most commonly seen in the distribution areas of the superior sagittal and transverse sinuses [2]. There are no uniform criteria for the etiology, pathogenesis, diagnostic criteria, treatment options, or prognosis of sinus pericranii [3]. CASE DESCRIPTION: In this paper, we present the diagnosis and treatment of a child admitted to our neurosurgery department in 2019 with rapidly growing frontal sinus pericranii, as well as the 3-year postoperative follow-up. DISCUSSION: The authors summarize the clinical presentation characteristics, diagnosis and treatment methods, and prognosis of this disease using relevant domestic and international literature to improve clinicians' understanding of this disease. CONCLUSION: It is rare to see a significant growing of the sinus pericranii within a short period, and the mechanism of their occurrence needs to be further explored.

Percutaneous embolization of sinus pericranii.

Sinus pericranii is a rare vascular anomaly involving a venous sinus that drains into a subgaleal collection of veins through an emissary vein. Data regarding presentation, management, and outcomes are limited to case reports and small case series. There are no technical videos detailing the technique for percutaneous embolization. We present the case of a child with an enlarging, symptomatic accessory type sinus pericranii with connection to the torcula, who underwent percutaneous embolization after unsuccessful transvenous embolization. Embolization was performed with 3.4 cc Onyx-34 under live fluoroscopy and serial control superior sagittal sinus venograms . Significant reduction of flow into the sinus pericranii was achieved and the lesion had nearly completely resolved at the 3-week follow-up. Percutaneous embolization of the sinus pericranii is a reasonable alternative to transvenous embolization, but additional data are needed to determine the optimal treatment. The technical details and practical considerations discussed here may help neurointerventionalists adopt this treatment. The video also includes references 1-4 which are relevant to this topic. neurintsurg;15/8/828/V1F1V1Video 1Case presentation and technique for percutaneous embolization of sinus pericranii.

Treatment of an Elusive Symptomatic Sinus Pericranii: Case Report and Review of the Literature.

Sinus pericranii (SP) are abnormal vascular connections between extracranial scalp venous channels and intracranial dural sinuses. This vascular abnormality rarely results in significant sequelae, but in select cases, it can be symptomatic. We describe the case of a 7-year-old girl with an SP who experienced intermittent visual, motor, and sensory symptoms not previously described in the literature. Her symptoms resolved after surgical treatment of the SP. We propose a mechanism for her symptoms and the rationale for the role of neurosurgical intervention along with a review of the literature.

Sinus pericranii associated with syntelencephaly: a case report.

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.

Crouzon's Syndrome with a Dominant Sinus Pericranii Draining Transverse Sinus: Report of a Rare Association and Review of Literature.

INTRODUCTION: Crouzon's syndrome and sinus pericranii (SP) are rare entities. Only few cases having both the features are reported. SP most commonly drains in relation to superior sagittal sinus and their communication to major posterior dural sinuses is rare. CASE REPORT: We report a rare case of Crouzon's syndrome with SP at a suboccipital location with termination of left transverse sinus into the SP draining further through the extracranial suboccipital and extravertebral cervical venous plexi into external jugular veins. Distal transverse sinus and sigmoid sinus on the left side were absent. CONCLUSION: Crouzon's syndrome with SP is an extremely rare entity. SP with communication to major posterior dural venous sinuses is also rare and mostly associated with multi-suture craniosynostosis. Management depends on the volume of venous blood they are draining. Most of them are dominant type and their occlusion is not feasible. Preoperative diagnosis of a dominant SP is essential for proper surgical planning as it needs to be preserved mandatorily to prevent cerebral venous infarction.

A Simple Surgical Technique for Pediatric Sinus Pericranii: Intraoperative Manual Compression of a Major Shunting Point.

INTRODUCTION: Sinus pericranii is a vascular anomaly with extra- and intracranial venous connections. Sinus pericranii is categorized into 2 groups according to its contribution to the normal venous circulation. The accessory type sinus pericranii, which does not contribute to the normal major venous circulation, can be managed. Despite several proposed operative maneuvers, a standardized technique is yet to be established to control intraoperative bleeding. CASE PRESENTATION: A 2-week-old neonate underwent examination of a subcutaneous mass in the parieto-occipital region. The subcutaneous mass had a major venous connection to the superior sagittal sinus on ultrasonography. The subcutaneous mass was partially thrombolized on magnetic resonance imaging and was minimally enhanced on computed tomography venography. The subcutaneous mass seemed not to contribute to the normal venous circulation. Surgical removal of the subcutaneous mass was performed due to its increased size at the age of 1 year and 3 months. While subcutaneous mass was detached from the scalp, the major venous connection was manually compressed, and minor venous connections were easily detected. The intraoperative bleeding was controllable. The pathological diagnosis was sinus pericranii. The patient is now followed up in the outpatient clinic. No recurrence was seen 18 months after the surgery. DISCUSSION/CONCLUSION: Intraoperative hemostasis is essential while sinus pericranii is detached from the cranium. Hemostatic agents such as bone wax or absorbable gelatin and heat coagulation seem to be useful. However, complicative hemorrhage concerning to the preceded technique has been also reported. As seen in our case, to detect minor shunting points between the sinus pericranii and the intracranial veins, the major venous connection was manually compressed. Intraoperative manual compression of a major venous connection of sinus pericranii can be an option to manage intraoperative bleeding.

Sinus pericranii-unusual anatomic obstacle to posterior decompression on an amniotic band sequence.

Abnormal venous drainage in patients with craniofacial anomalies is relativity uncommon. Sinus pericranii is a rare vascular malformation characterized by communication between intracranial dural sinuses and extracranial venous drainage systems. The association between sinus pericranii and amniotic band syndrome has not been described. We report on a 7-month-old girl diagnosed with amniotic band sequence who underwent posterior fossa decompression due to intracranial hypertension and Chiari malformation type I. The computed tomography and magnetic resonance identified the connection between the sagittal sinus and the scalp. During the operation, the presence of sinus pericranii was a complicating factor limiting the proper exposure because of the risk of bleeding. Patients with craniofacial anomalies and sinus pericranii present an increased risk of serious surgical complications and consequences from craniofacial surgery, especially from cranioplasty and posterior decompression. Special attention must be paid to abnormal venous drainage, and vascular imaging studies are sometimes required.

Sinus Pericranii in Posterior Cranial Vault Distraction.

Anomalous venous connections between the extracranial and intracranial systems are relatively rare. These connections are also known as sinus pericranii (SP) have been reported previously in the setting of various cranial malformations including craniosynostosis. Angiography may be useful in the diagnosis of SP also allowing for differentiation of the dominance of the venous systems. Several methods have been described in the treatment of SP. In isolated cases both endovascular and open approaches have been successful in the treatment of SP. In cases wherein there is synostosis present, both single- and multiple-staged operations have been described. In this article, we describe 2 cases of posterior cranial distraction in the setting of sinus peri cranii.

Sinus pericranii: diagnóstico precoz en el lactante / Sinus pericranii: early infant diagnosis

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Sinus pericranii in the upper eyelid: diagnosis and management guidelines.

Sinus pericranii (SP) is a rare malformation involving aberrant connections between the dural venous sinuses and extracranial veins. Classically considered congenital pathology, there are reported instances of post-traumatic SP in adults. Very rarely, the upper eyelid is involved and the resulting ptosis can obstruct the visual axis. In these cases, surgical removal may be warranted. Because of the high risk of massive intraoperative hemorrhage, careful preoperative identification and diagnostic workup is essential. The authors report a patient with new onset, asymmetric eyelid ptosis referred for evaluation, and subsequently diagnosed with SP. Through a multi-disciplinary assessment, deferring surgical intervention was determined to be appropriate management. The lesion was monitored clinically, and at 6 months follow-up, there were no noted adverse outcomes.

Characteristics of Recurrent Congenital Sinus Pericranii: Case Report and Review of the Literature.

INTRODUCTION: Sinus pericranii (SP) involves transosseous vessels that connect the intra- and extracranial venous systems. Accessory-type SP can be cured by surgical or endovascular treatment. Reports of recurrence are, however, rare. CASE: A boy presented with a soft-tissue mass on the left parietal region of the head. Computed tomography and magnetic resonance imaging revealed the congenital SP with several small transosseous vessels surrounded by an area of thin bone. At the initial surgery, the vascular mass was completely excised by coagulating and cutting the -transosseous vessels. The skull defect was filled with bone wax. Two years later, SP recurred at the same site. At the second surgery, the skull defect and surrounding area were sealed with acrylic resin. The boy has been followed up for 6 years without recurrence. DISCUSSION: Potential risk factors for recurrence proposed in the existing literature include an unusually large number of emissary veins, rich circulation between intra- and extracranial venous systems, large bone defects, raised intracranial pressure, and association of other developmental venous anomalies. Our case demonstrates that thin skull bone around the original lesion can be another risk factor for recurrence. CONCLUSION: Removing all abnormal vessels and sealing the skull defect as well as the surrounding thin bone area are important to prevent recurrence of congenital SP.

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

Introducción: 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 clínico: 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 Conclusión: 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

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

A review of extraaxial developmental venous anomalies of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, venous varices or aneurysmal malformations, and enlarged emissary veins.

This paper is a narrative review of extraaxial developmental venous anomalies (eDVAs) of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, enlarged emissary veins, and venous varices or aneurysmal malformations. The article highlights the natural history, anatomy, embryology, imaging, clinical implications, and neurosurgical significance of these lesions, which the authors believe represent a continuum, with different entities characterized by distinct embryopathologic features. The indications and surgical management options are discussed for these individual intracranial pathologies with relevant illustrations, and a novel classification is proposed for persistent falcine sinus (PFS). The role of neurointervention and/or microsurgery in specific cases such as sinus pericranii and enlarged emissary veins of the skull is highlighted. A better understanding of the pathophysiology and developmental anatomy of these lesions can reduce treatment morbidity and mortality. Some patients, including those with vein of Galen malformations (VOGMs), can present with the added systemic morbidity of a high-output cardiac failure. Although VOGM is the most studied and classified of the above-mentioned eDVAs, the authors believe that grouping the former with the other venous anomalies/abnormalities listed above would enable the clinician to convey the exact morphophysiological configuration of these lesions, predict their natural history with respect to evolving venous hypertension or stroke, and extrapolate invaluable insights from VOGM treatment to the treatment of other eDVAs. In recent years, many of these symptomatic venous malformations have been treated with endovascular interventions, although these techniques are still being refined. The authors highlight the broad concept of eDVAs and hope that this work will serve as a basis for future studies investigating the role of evolving focal venous hypertension/global intracranial hypertension and possibilities of fetal surgical intervention in these cases.

Sinus pericranii: long-term outcome in a 10-year-old boy with a review of literature.

Sinus pericranii is a rare cranial venous anomaly in which there is an abnormal communication between intracranial dural sinuses and extracranial venous structures, usually via an emissary transosseous vein. We report the case of a 10-year-old boy who was successfully managed conservatively. The clinical findings, investigations and management are discussed. We focus on the long-term management of these patients, with a review of the relevant literature.

Hypoplastic Straight Sinus with Persistent Falcine Sinus in Adult with Sinus Pericranii.

Open magnetic resonance imaging of a 45-year-old man with panic disorder showed a sinus pericranii (SP). Examination of the scalp revealed a discolored scalp mass, and magnetic resonance venography displayed a hypoplastic straight sinus with persistent falcine sinus. SP is a venous anomaly consisting of a transosseous vascular channel connecting an intracranial sinus with subgaleal veins. High venous pressure in the late embryonic stage, as with a hypoplastic straight sinus, has been hypothesized to predispose affected individuals to develop other venous anomalies (i.e., falcine sinus). Because there is no major venous outflow through this accessory SP in an asymptomatic adult, no intervention was recommended for our patient.