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.

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.

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 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.

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.

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.

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 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.

[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.

[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.

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.

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.

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.

Extremely large sinus pericranii with involvement of the torcular and associated with Crouzon's syndrome.

INTRODUCTION: Sinus pericranii is a rare vascular malformation that connects the intracranial dural sinuses to the extracranial venous drainage system and is caused by either trauma or congenital defects. Although the majority of these vascular structures are due to trauma, some are congenital. CASE REPORT: Herein, we report a 5-month-old patient with a very large and fluctuating subcutaneous mass over the occiput and the diagnosis of Crouzon's syndrome. The child presented with a large midline mass that on imaging, connected to the underlying torcular and was diagnosed as a sinus pericranii. At long-term follow-up and without operative intervention, the sinus pericranii resolved. This uncommon relationship is reviewed. CONCLUSION: Premature closure of posterior fossa sutures as part of Crouzon's syndrome can present with large sinus pericranii. Such subcutaneous swellings might resolve spontaneously.

Three-dimensional printing of a sinus pericranii model: technical note.

BACKGROUND: Sinus pericranii (SP) is a rare venous malformation consisting of a single or multiple abnormal emissary veins communicating between intracranial sinuses and dilated epicranial veins. There is no consensus concerning diagnosis, management, and treatment of SP. TECHNICAL NOTE: We report the case of a 4-month-old infant with a SP for whom we used a three-dimensional printed model in order to define the angioarchitecture, improve management, and help parents' understanding of this uncommon condition.

[Sinus pericranii in adults: clinical features and therapeutic management (13 cases report)].

Objective: To study the character of sinus pericranii in the adults and enhance the levels of the diagnosis and treatment of sinus pericranii. Methods: The clinical data of 13 adult patients with sinuses pericranii undergoing surgery in Beijing Hospital from 1992 to 2016 were analyzed retrospectively, including clinical manifestations, imaging data, surgical results. Results: Each of the patients presented with a nonpulsatile and soft tissue mass in their heads.Their ages ranged from 18 to 48 years (mean, 26 years). The diagnosis of the sinus pericranii was made by the clinical manifestations combined with CT and/or MRI examinations.The sinus pericranii did not play the main role in the drainage of the cerebral vein blood.All the patients were cured.The follow up time was from 6 months to 20 years with no sinus pericranii recurrence in all the patients. Conclusions: The analysis of the drainage pattern of sinus pericranii was necessary before surgery in order to get a safe and good operation.The curative effect of the surgery on the sinus pericranii in adult was reliable.

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.