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.

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.

Calvarial tuberculosis in a paediatric patient: a diagnosis not to forget.

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.

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-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.

Intracranial venous malformations: Incidence and characterization in a large pediatric cohort.

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.

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, skull defects, and structural brain anomalies in TRAF7-related disorder.

BACKGROUND: Several somatic mutations in TRAF7 have been reported in cancers, whereas a few germline heterozygous mutations have been recently linked to a neurodevelopmental disorder, characterized by craniofacial dysmorphisms, congenital heart defects, and digital anomalies. CASES: We report two subjects harboring de novo heterozygous missense variants in TRAF7, namely the recurrent 1964G>A(p.Arg655Gln) and the novel missense c.1204C>G(p.Leu402Val) variants. In addition to the typical hallmarks of the TRAF7-related disorder, both subjects presented with a recognizable "pear-shaped" skull due to multiple craniosynostosis, sinus pericranii, skull base/cranio-cervical junction anomalies, dysgyria, and inferior cerebellar vermis hypoplasia. CONCLUSIONS: Hence, we expand the genotypic and phenotypic spectrum of this neurodevelopmental disorder, discussing possible implications for clinical management of subjects with germline TRAF7 mutations.

Sinus Pericranii Complicated by Hydrocephalus: Case Report and Literature Review.

BACKGROUND: Sinus pericranii (SP) is a rare vascular condition that results when abnormal communication between the intracranial and extracranial venous systems is present. Here we report a rare case of hydrocephalus revealed through a SP scalp mass with a review of literature. CASE DESCRIPTION: A 13-year-old girl presented with a history of bulging left eye, bilateral gradual deterioration of vision, nausea, and progressive headaches. The family reported the sudden appearance of a scalp mass prior to these symptoms. Brain imaging showed supra- and infratentorial communicating hydrocephalus and subcutaneous vascular collaterals from the midportion of the superior sagittal sinus. After ventriculoperitoneal shunt insertion, interval reduction of the ventricular size and disappearance of extracranial veins without associated intra- or extracranial vascular anomalies was observed. CONCLUSIONS: SP is divided into two main types: (1) Spontaneous type, in which lesions are formed due to acquired causes without a syndromic association or other vascular anomalies or stenosis. The most commonly reported cause is trauma in which SP may develop at the trauma site rather than the midline. (2) Congenital type, in which case the most commonly reported association is craniosynostosis. It can also be seen with vein of Galen hypoplasia, vein of Galen malformations, dural sinus malformations, solitary developmental venous anomalies, and intraosseous arteriovenous malformations. We are reporting the first case of idiopathic SP with hydrocephalus without a congenital association or acquired cause, including trauma. The sudden change in SP size can indicate a change in intracranial pressure and the development of hydrocephalus.