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.

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.

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.

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.

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.

Sinus Pericranii with Dominant Venous Outflow in the Superior Eyelid.

Sinus pericranii (SP) located in the superior eyelid is an unusual clinical presentation. Here, we report a case of 72-year-old woman with an unruptured cerebral aneurysm presented with an SP located in the left superior eyelid. The SP was found to have a dominant venous outflow from the bilateral frontal region with an arterialized blood flow pattern on color Doppler ultrasonography (CDUS). During the aneurysmal surgery, intraoperative monitoring of the dominant venous outflow with CDUS was useful for the prevention of venous outflow obstruction. Physicians should carefully consider intracranial vascular anomalies in the differential diagnosis of vascular lesions of the superior eyelid.

Rare vascular pathology sinus pericranii; becomes symptomatic with pseudotumor cerebri.

Sinus pericranii (SP) is an uncommon vascular pathology that is characterized by a nodular structure emerging from the scalp. It is generally asymptomatic. Typically, it is aclose to soft, compressive, fluctuant swelling, intracranial sinus. The swelling increases during the Valsalva maneuver, jugular vein compression, coughing, or crying attacks. The diagnosis was confirmed by CT, MRI, and MR angiography. Here, we conclude that SP is a rare cause of headache and that the asymptomatic lesion become symptomatic as a result of association with pseudotumor cerebri.

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.

Sinus pericranii in the left frontal region involving the superior eyelid: a case report.

BACKGROUND: Sinus pericranii is a rare asymptomatic communication between the intracranial and extracranial venous drainage pathways. The venous flow in this condition circulates through abnormal dilated veins in both directions. PATIENT/METHODS: We describe an unusual location of an accessory sinus pericranii that involved the left frontal bone along with the superior orbital rim and the upper eyelid, with special focus on therapy. RESULTS: The patient did not have any complication during the first and second surgery. Final outcome was excellent. The patient did not show any evidence of disease recurrence 1 year after the surgery. CONCLUSION: We propose a multidisciplinary approach in the treatment of such lesions with a two-step surgery. Excision of the sinus pericranii is possible if the sinus pericranii is not a major venous outflow channel of the brain, which can be evaluated by angiography.

Occipital sinus pericranii superseding both jugular veins: description of two rare pediatric cases.

BACKGROUND AND IMPORTANCE: The extracranial venous drainage of the brain is provided mainly by the jugular veins with the variable participation of extrajugular pathways. CLINICAL PRESENTATION: Two cases of occipital sinus pericranii (SP) were observed. A 6-year-old girl presented with a progressively enlarging occipital lump; she was otherwise asymptomatic. An 8-year-old boy presented with mild nuchal headache. In the former case, neuroimaging examinations confirmed the diagnosis of SP; in the latter, SP was noticed on magnetic resonance imaging because it was neglected at physical examination. In both subjects, the radiological examination also showed bilateral occlusion of the jugular veins with ventricular dilation and herniation of cerebellar tonsils into the foramen magnum. CONCLUSION: Exceptionally, SP was noticed to represent the main venous drainage of the underlying brain, thereby compensating for the intracranial hypertension due to venous engorgement. Preservation of SP was advised, and any surgical or endovascular treatment was contraindicated.

Cerebrofacial venous anomalies, sinus pericranii, ocular abnormalities and developmental delay.

The clinical implications of venous cerebrovascular maldevelopment remain poorly understood. We report on the association of cerebrofacial venous anomalies (including sinus pericranii), ocular abnormalities and mild developmental delay in two children. In addition, one child had a seizure disorder. Complex cerebrofacial slow-flow vascular anomalies may herald an underlying developmental aberration affecting the cerebrofacial and orbital regions.

Atretic parietal cephalocele associated with sinus pericranii: embryological consideration.

We report a case of atretic parietal cephalocele with a persistent parietal falcine sinus and partial absence of the straight sinus. The direct puncture angiographic study demonstrated that there was a major venous channel through the parietal skull defect. From the embryological point of view, this association may provide us with possible pathoetiologic evidence of congenital sinus pericranii is one of the alternative venous drainage pathways to compensate venous outflow in the cases of intracranial developmental anomalies.

Sinus pericranii in children: report of 16 patients and preoperative evaluation of surgical risk.

OBJECT: Sinus pericranii (SP) is a rare venous varix in an extracranial location connected to the intracranial venous system. The aim of this retrospective study was to report on 16 pediatric cases of SP with consideration of the preoperative evaluation of surgical risk. METHODS: The study population consisted of 10 patients who had undergone surgery for SP and 6 patients with concomitant craniosynostosis and SP. The mean age of the patients at presentation was 3.7 years. To identify characteristics of SP with high operative risk, 8 cases in this report and 11 previously reported cases of SP with sufficient information were categorized on the basis of the number and size of SP, the number and size of transcranial channels, the venous drainage type, and the amount of blood loss. Hemorrhage amounts were classified into 3 grades based on the description of intraoperative blood loss. RESULTS: Sinus pericranii not associated with craniosynostosis were resected without any postoperative morbidity. Sinus pericranii associated with craniosynostosis were preserved. After craniofacial reconstruction, 2 cases of SP with craniosynostosis regressed, completely in one patient and partially in another. These 2 patients with SP were confirmed to have compromised intracranial sinus before craniofacial reconstruction. Among a total of 19 patients, multiplicity or size (> 6 cm) of SP (p = 0.036) and multiplicity (> 3) or size (> 3 mm) of transcranial channels (p = 0.004) was associated with more severe hemorrhage grade. Sinus pericranii with peripheral venous drainage (drainer type) was not associated with hemorrhage grade after classification into 3 grades (p = 0.192). However, all 3 cases of SP with massive Grade 3 hemorrhage were the drainer type. Hemorrhage grade was correlated with the number of risk factors for SP (r = 0.793, p < 0.001). CONCLUSIONS: Three risk factors of SP and the presence of compromised intracranial sinus are markers for highrisk SP. "Squeezed-out sinus syndrome" is suggested as a concept for SP associated with compromised intracranial sinus, mainly caused by craniosynostosis. Sinus pericranii in squeezed-out sinus syndrome probably serves as a crucial alternative to venous drainage of the brain with intracranial venous compromise. Conservative treatment for such patients with SP is recommended.