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.

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.

Peculiar bony involvement of sinus pericranii in children: Extensive diploic erosion in three "karstic" variants.

OBJECT: Sinus pericranii (SP) is a rare, benign, extradural venous anomaly whose extracranial connection consists in an enlarged subgaleal drainage composed of a network of thin-walled veins that form a varix on the external table of the skull. In the present series of three patients we present three variantesof SP which have never been described, characterized by an extensive diploic erosion causing a "karstic" effect. METHODS: A systematic review was conducted in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline. A comprehensive search for relevant articles was performed on PubMed from 1992 to 2022 using the following search words: "sinus pericranii". RESULTS: The search produced 77 articles with 137 patients. In all the cases analysed, there were not reported any patients with extensive diploic erosion, namely "karstic" variants. So, the authors present clinical history, neuroimages, treatment strategies and outcome of 3 patients with a "karstic" SP with the aim to describe this clinicradiological entity. CONCLUSIONS: In this study, our group identified a peculiar variant of SP characterized by bony involvement with extensive diploic "karstic" erosion of diploe, with possible tendency to enlarge during time.

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.

Surgical Management of Sinus Pericranii With Crouzon Syndrome.

BACKGROUND: Sinus pericranii (SP) is a rare vascular malformation which connects the intracranial dural sinuses to the extracranial venous drainage system. Although the majority of SP cases are caused by trauma, some of them are congenital. Furthermore, a few SP cases have been reported in association with craniosynostosis. The authors' objective is to discuss the surgical management of SP with Crouzon's syndrome in children. METHODS: Three-Dimensional reconstruction with enhanced CT scan was used for evaluate the condition of SP with Crouzon's syndrome in all 4 cases. Two cases with small single-hole defect on skull were only treated by cranioplasty with distraction osteogenesis. In the management of the other 2 SP patients with large skull defect, titanium mesh was used for compression of dilated venous sinus to inhabit filling and promote shrinking. RESULT: Four cases of SP with Crouzon's syndrome were treated in the authors' department. With cranioplasty with distraction osteogenesis only, 2 patients with single-hole skull defect experienced SP spontaneous involution at 4 to 6 months postoperatively. With titanium mesh compression, SP with large skull defect shrank significantly without clinical recurrence, but long-term follow-up was still in need. CONCLUSION: For small single-hole skull defect in SP patient with Crouzon's syndrome, the only application of cranioplasty for decreasing intracranial pressure can promote SP spontaneous involution. 3D printed titanium mesh compression renders a safe and efficient approach for SP with large skull defect. Long-time follow up and further accumulation of cases may establish the efficacy of the authors' management.

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.

[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.

Resection of Meningiomas Involving Major Dural Venous Sinuses: Classification, Technique, and Long-Term Results.

OBJECTIVE: Management of meningiomas with major dural venous sinus involvement is challenging. We present our case series and perspective on reconstruction of the sinuses. METHODS: Fifty-five patients underwent operations between 2005 and 2016 and the retrospective data were collected and analyzed. RESULTS: The cohort was younger with a mean of 51.3 years (range, 19-72 years) predominantly involving the superior sagittal sinus (44 patients). Sinus involvement was classified into group 1 (<50% of sinus, n = 28), group 2 (50%-99%, n = 8), and group 3 (total occlusion, n = 19). Venous collateralization was present in 100% of group 2 and 3 and in 36% of group 1 occlusions. Sinus pericranii was seen in 22 patients. Gross total resection was achieved in 87.2%, and sinus reconstruction followed in 38 patients (24 by direct suture and 14 by a patch graft). Pathology showed 36 (65%) World Health Organization grade I, 18 (33%) grade II, and 1 (2%) grade III tumors. During the mean follow-up of 60 months (range, 1-132 months), sinus was patent (74%) or narrowed but patent (24%) in 98%; 2 recurrences (3.6%) were observed (at 24 and 120 months). The mean preoperative/postoperative Karnofsky Performance Status and Kaplan-Meier cumulative overall/recurrence-free survival were 84.2%/88.1% and 90.9%/80.1%, respectively. CONCLUSIONS: These meningiomas present in a younger population, are more likely to be World Health Organization grade II or III, necessitating a more aggressive tumor resection strategy. Aggressive resection coupled with sinus reconstruction results in good long-term surgical outcome and low recurrence rates.

[Occipital sinus pericranii: About an unusual situation and review of the literature]. / Sinus pericranii occipital : à propos d'une situation inhabituelle et revue de la littérature.

The sinus pericranii refers to a set of clinical presentations that share a pathological communication between the scalp veins and the underlying cranial venous sinus. The nature of this connection ranges from the simple dilatation of the emissary veins, to wide connections through a calvarial bone loss leaving almost the cranial venous sinus in direct contact with the subcutaneous tissue. The authors present the case of an occipital pericranii sinus of intraoperative discovery. Congenital, post-traumatic or spontaneous, this anomaly is most often frontal and located on or close to the midline. The usual clinical presentation is that of a soft mass that empties to the pressure while standing, while it fills in all situations that increase the intracranial pressure (Valsalva maneuver …). The diagnosis is not always easy to establish, and relies on a bundle of clinical and radiological arguments. Through a review of the literature we propose to focus on the clinical features, diagnostic means and therapeutic options of this rare entity.

Staged Raising of a Coronal Flap for Fronto-Orbital Advancement and Remodeling in Saethre-Chotzen Syndrome Complicated by Sinus Pericranii.

This article reports the surgical management of a 3-month-old girl with Saethre-Chotzen syndrome, who presented with bicoronal synostosis and a large midline sinus pericranii with abnormal cerebral venous drainage via scalp veins. Raised intracranial pressure was demonstrated on monitoring, indicating the need for calvarial expansion necessitating a coronal access incision. A 2-staged delayed raising of the coronal flap was performed to reduce the potential risk of cerebral venous infarction. Monitoring for clinical sequelae and a computerised tomography venogram followed each of these procedures, demonstrating successful redirection of the venous drainage of the brain posteriorly. Finally, a successful fronto-orbital advancement and remodeling procedure was performed with no complications.

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.

Dynamic morphological changes of thrombosed lateral sinus pericranii revealed by serial magnetic resonance images.

BACKGROUND: Sinus pericranii (SP) is a rare venous anomaly involving an abnormal connection of the intracranial dural sinuses with the extracranial veins. Magnetic resonance (MR) imaging (MRI) with MR venography can detect the typically congested intra- and extracranial venous components of SP. CLINICAL PRESENTATION: We report a rare case of lateral SP associated with the superior sagittal sinus, which might had already developed almost total thrombosis of the SP at the first MRI. As this patient had not presented with classical manifestations of SP on clinical or neuroradiological findings, the initial diagnosis of SP was difficult. Repeated MRI revealed dynamic morphological changes associated with reperfusion of the thrombosed SP via the cortical vein. CONCLUSION: MR venography combined with gadolinium enhancement was useful for diagnosis of the SP with an extremely slow flow status.

Sinus pericranii in achondroplasia: a case report and review of the literature.

In the field of dysmorphology, achondroplasia is a well-known disorder. Sinus pericranii (SP), however, is not. The latter condition is a rare vascular malformation characterized by abnormal connections between the intracranial and the extracranial venous drainage pathways. The etiology of SP remains unclear, and yet, these defects can be present at birth, develop spontaneously later, or evolve following head trauma. Here, we report on a 2-year-old male with achondroplasia, SP, and craniocervical junction stenosis. The latter two defects required surgical correction. SP is an underappreciated malformation that we propose may be induced by increased intracranial pressure. This case appears to be the first report of this condition in achondroplasia.

Multiple lateral sinus pericranii--A case report.

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.

[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.

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.