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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. [ABSTRACT FROM AUTHOR]

Abstract Patients and methods. In this retrospective study, 36 children referred to paediatric neurology and neurosurgery during April 1995-June 1998 with a diagnosis of subdural haematoma (SDH) were studied. Nine were accidental secondary to witnessed trauma and 4 were iatrogenic. Non-accidental head injury (NAHI) was suspected in the remaining 23 children. Results. After a full clinical, radiological and social assessment, NAHI was diagnosed in 14, lateral sinus thrombosis in 1, 2 were accepted as accidental and 6 remained unexplained. In the NAHI group ( n=14), 12 were between 4 and 16 weeks of age, 12 (85%) had retinal haemorrhages and skeletal surveys showed evidence of additional injury in 8. Computerised tomography (CT) brain scans showed bilateral SDH in 11, and 6 had inter-hemispheric bleeding along with loss of grey-white differentiation. Eleven had magnetic resonance imaging (MRI), which yielded additional information in 7. Seven required intensive care, and 2 died. Twelve had surgical aspiration. In the group with no satisfactory explanation for SDH ( n=6); 5 had neonatal problems, all except 1 were older than 5 months of age and not as ill with bilateral, old SDH. All but 1 had skeletal surveys, which were normal, and eye examination showed no retinal haemorrhages. A social services enquiry was non-contributory. Conclusions. SDH is frequently traumatic whether accidental or non-accidental. SDH due to NAHI tends to present before 4 months of age with an inconsistent history; the patients are more seriously ill and have other findings, such as fractures and retinal haemorrhages. A small subgroup of patients was identified who had isolated, old SDH and in whom full investigation remained inconclusive. A consistent, comprehensive approach needs to be maintained in all cases with the essential backup of detailed neuro-imaging including MRI. [ABSTRACT FROM AUTHOR]

Purpose: Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors resected through craniotomy with posterior interhemispheric occipital transtentorial (OT) approach are reviewed. We present the surgical techniques, pitfalls, and their results. Material and methods: Eighty patients ranging in age from 3 months to 21 years old, and treated over 3 decades were reviewed. Hydrocephalus caused the main presenting symptoms and was noted in 74 patients. It was treated prior to the craniotomy for tumor resection with endoscopic third ventriculostomy (ETV) in 33, external ventricular drainage in 26, and precraniotomy shunt in 15. Nine patients had ETV together with endoscopic biopsy. All patients had a parieto-occipital craniotomy in a prone position. Through a tentorial section, a gross total resection of the tumor was attempted except for germinomas. Results: The tumor pathology showed 32 germ cell tumors (GCT), 22 benign astrocytomas, 13 pineal parenchymal tumors, 5 ATRTs, 3 papillary tumors, and 5 others. Of GCTs, 18 were teratomas. The extent of resection consisted of 55 gross total resections, 13 subtotal resections, 10 partial, and 2 biopsies with one postoperative death. Hemiparesis in 2, cerebellar ataxia in another 2, and hemiballismus in 1 were transient and improved over time. One had permanent hemisensory loss and another patient had bilateral oculomotor palsy. Postoperative homonymous hemianopia occurred in 2 patients but subsided over a short period of time. Parinaud's sign was noted in 24 patients, of which 16 were transient. Conclusion: The posterior interhemispheric OT approach provides a safe route and comfortable access to the pineal region in children. A great majority of postoperative neurological complications are the results of direct manipulations of the midbrain at tumor resection. Identification and preservation of the tumor-brain interface are of paramount importance. GCTs other than teratomas are treated with neoadjuvant chemotherapy and may eliminate the need for craniotomy. Exophytic midbrain JPAs are amenable to resection. [ABSTRACT FROM AUTHOR]

Purpose: Cerebral sinus vein thrombosis (CSVT) associated with acute mastoiditis is a rare complication of acute otitis media. Elevated intracranial pressure (ICP) frequently occurs secondary to CSVT. The study aims to review the 5 years of experience of four medical centres to treat sigmoid sinus thrombosis and elevated intracranial pressure in children. Methods: Patients with CSVT that developed secondary mastoiditis from 2016 through 2021 were evaluated in four centres from Turkey. Patients diagnosed with a preceding or synchronous mastoiditis and intracranial sinus thrombosis were included in the study. Magnetic resonance imaging (MRI), magnetic resonance venography (MRV), ICP measurements, ophthalmological examinations, thrombophilia studies and treatments for increased ICP have also been recorded. Results: The study group comprises 18 children. Twelve patients were diagnosed with right-sided, six patients with left-sided sinus vein thrombosis. All of the patients had ipsilateral mastoiditis. The most common presenting symptoms were fever, ear pain, headache, visual disorders and vomiting. The most encountered neurologic findings were papilledema, strabismus and sixth cranial nerve palsy. ICP was over 20 cm H2O in eleven patients. Anticoagulant treatment, antibiotics, pressure-lowering lumbar puncture and lumboperitoneal shunt were among the treatment modalities. Conclusion: Elevated ICP can damage the brain and optic nerve irreversibly, without treatment. For treating elevation of ICP associated with cerebral sinus thrombosis, pressure-lowering lumbar puncture (LP), acetazolamide therapy, optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF)-shunting procedures are suggested in case of deteriorated vision. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the anatomical variations of dural venous sinuses in children with external hydrocephalus, proposing a radiological grading of progressive anatomic restriction to venous outflow based on brain phase-contrast magnetic resonance venography (PC-MRV); to evaluate the correlation between positional plagiocephaly and dural sinuses patency; and to compare these findings with a control group to ascertain the role of anatomical restriction to venous outflow in the pathophysiology of external hydrocephalus. Methods: Brain MRI and PC MRV were performed in 97 children (76 males, 21 females) diagnosed with external hydrocephalus at an average age of 8.22 months. Reduction of patency of the dural sinuses was graded as 1 (stenosis), 2 (complete stop) and 3 (complete agenesis) for each transverse/sigmoid sinus and sagittal sinus. Anatomical restriction was graded for each patient from 0 (symmetric anatomy of patent dural sinuses) through 6 (bilateral agenesis of both transverse sinuses). Ventricular and subarachnoid spaces were measured above the intercommissural plane using segmentation software. Positional plagiocephaly (PP) and/or asymmetric tentorial insertion (ATI) was correlated with the presence and grading of venous sinus obstruction. These results were compared with a retrospective control group of 75 patients (35 males, 40 females). Results: Both the rate (84.53% vs 25.33%) and the grading (mean 2.59 vs mean 0.45) of anomalies of dural sinuses were significantly higher in case group than in control group. In the case group, sinus anomalies were asymmetric in 59 cases (right-left ratio 1/1) and symmetric in 22. A significant association was detected between the grading of venous drainage alterations and diagnosis of disease and between the severity of vascular anomalies and the widening of subarachnoid space (SAS). Postural plagiocephaly (39.1% vs 21.3%) and asymmetric tentorial insertion (35.4% vs 17.3%) were significantly more frequent in the case group than in the control group. When sinus anomalies occurred in plagiocephalic children, the obstruction grading was significantly higher on the flattened side (p ≤ 0.001). Conclusion: Decreased patency of the dural sinuses and consequent increased venous outflow resistance may play a role in the pathophysiology of external hydrocephalus in the first 3 years of life. In plagiocephalic children, calvarial flattening may impact on the homolateral dural sinus patency, with a possible effect on the anatomy of dural sinuses and venous drainage in the first months of life. [ABSTRACT FROM AUTHOR]

Purpose: Lambdoid synostosis (LS) is a rare condition, which is either isolated; associated with sagittal synostosis, the "Mercedes-Benz" syndrome (MBS); or with synostosis of the coronal sutures (oxycephalic form). In addition, LS is part of the phenotype of a growing number of genetic diseases. The nosology, pathophysiology, and management are controversial. We decided to review our experience with LS. Methods: We reviewed retrospectively pediatric cases of LS proved on CT-scanner, isolated or associated with other conditions, followed in our craniofacial center during the last 15 years, regarding clinical presentation, anatomical lesions, syndromic associations, surgical management, and outcome. Results: We reviewed 48 cases: 6 isolated LS, 22 MBS, and 20 oxycephalic. A syndromic context was present in 72% (up to 80% of oxycephalic cases), and faciostenosis was present in 23%, mostly oxycephalic cases (40%). Transverse sinus agenesis was found in 61% of documented patients. A total of 31% of children had a dystocic birth, up to 45% of MBS. Decompressive craniectomy or cranioplasty was needed in a majority of patients, often young infants, while posterior fossa decompression was mostly performed in older children. Conclusion: LS is rarely isolated and non syndromic; most cases are found in a wide spectrum of diseases, and LS is often associated with sagittal or coronal synostosis. Genetic evaluation is mandatory for LS; conversely, geneticists may require neurosurgical advice for LS in an increasing number of very rare diseases. The surgical management of LS should be tailored according to clinical presentation, age, and syndromic context. [ABSTRACT FROM AUTHOR]

Purpose: Our purpose is to present an atypical case of a 4-month-old patient with a giant dural arteriovenous fistula (DAVF). Methods: Presentation of a case report and review of the literature. Results: The DAVF arterial supply was through the middle meningeal artery bilaterally and the anterior and middle cerebral arteries on the right hemisphere. The venous drainage was through the posterior two-thirds of the superior sagittal sinus. The endovascular team performed an embolization to reduce the flow of the lesion, and finally, the surgical team completed the excision of the residual venous sac, without causing any significant neurological deficit. We used a double surgical approach done with two surgical teams in order to optimize the hemostasis control and reduce morbidity and mortality. Conclusion: Midline DAVF usually has devastating consequences in children. Endovascular treatment is the first choice since it has lower mortality. Nevertheless, it requires multiple interventions, and the cure of the disease may not be achieved. We believe that joint endovascular and surgical treatment, supported by a reliable multidisciplinary medical team, is a good option for this type of lesions. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Cerebral venous sinus thromboses (CVST) occur in children with a variety of etiologies. However, no standard treatment paradigm is established. We sought to identify what treatments have been applied, their outcomes, and the role of anticoagulation in pediatric patients with CVST. Methods: A systematic review was conducted exploring all treatments of pediatric CVSTs using PubMed, Embase, Scopus, and Cochrane Library and Cochrane Central Register of Controlled Trials. Studies meeting inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes. Results: Of 2946 resultant articles, 51 full-text articles were included. Management of infectious CVST included broad-spectrum antibiotics, surgery, and anticoagulation. Neoplastic and traumatic CVST treatment included anticoagulation. Treatment of CVSTs associated with metabolic abnormalities centered on correction of metabolic derangements, or supplementation where appropriate, and anticoagulation. Autoimmune, congenital, and thrombotic pathway CVSTs were treated with anticoagulation and treatment of the underlying disorder. Unfractionated heparin and low molecular weight heparin were most commonly used and seen to be effective and safe. Uncommonly, endovascular interventions including venous thrombectomy and intravenous injection of thrombolytic therapy were used with varying success. Conclusions: While conservative, medical, thombolytic, endovascular, and surgical treatment all have a role in in the treatment of pediatric CVSTs, anticoagulation is commonly applied and found to be safe and effective in pediatrics. Risks and benefits of anticoagulation must be considered on an individual basis as no randomized trials have established a standard of care. Based on our findings, we propose an approach to CVST treatment and look to future study aimed at more clearly delineating treatment dose, duration, and timing of re-evaluation in these patients. [ABSTRACT FROM AUTHOR]

Purpose: This study reviews paediatric patients with raised intracranial pressure as a result of venous sinus thrombosis secondary to otogenic mastoiditis, requiring admission to the paediatric neuroscience centre at the University Hospital Wales, Cardiff. The consensus regarding the management of otogenic hydrocephalus in the published literature is inconsistent, with a trend towards conservative over surgical management. We reviewed our management of this condition over a 9-year period especially with regard to ventriculo-peritoneal (VP) shunting. Methods: Analysis of a prospectively collected database of paediatric surgical patients was analysed and patients diagnosed with otogenic hydrocephalus from November 2010 to August 2018 were identified. Our data was compared with the published literature on this condition. Results: Eleven children, 7 males and 4 females, were diagnosed with otogenic hydrocephalus over the 9-year period. Five (45.5%) required VP shunt insertion to manage their intracranial pressure and protect their vision. The remaining six patients (54.5%) were managed medically. Conclusions: When children with mastoiditis and venous sinus thrombosis progress to having symptoms or signs of raised intracranial pressure, they should ideally be managed within a neuroscience centre. Of those children, almost half will need permanent cerebrospinal fluid diversion to protect their sight. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: Despite being the second most frequent tumor in children, pediatric central nervous system (CNS) tumors are rare, and the published European epidemiological data is limited. Our goal is to present the first surgical series of pediatric CNS tumors in Portugal and to review other similar worldwide series. Methods: Retrospective review of all patients younger than 19 years old, operated to a CNS tumor in the Neurosurgery Department at Hospital de Santa Maria (Lisbon, Portugal) between January 2004 and December 2014. Demographic data, tumor location, clinical data, histopathology, and surgical treatment were analyzed and compared to surgical series of pediatric CNS tumors published in PubMed indexed journals over the last 20 years. Results: We performed 253 surgeries in 215 patients, with a male:female ratio of 1.2:1 and a mean age of 9.2 years old. Primary brain tumors accounted for 95 % of all tumors and had more often a supratentorial location. Tumors of neuroepithelial tissue, particularly astrocytic tumors, embryonal tumors, neuronal and mixed neuronal-glial tumors, and oligodendrogliomas accounted for 81 % of cases. A gross-total resection was achieved in most cases. There was no mortality, and the overall morbidity was low. Conclusions: The demography, topography, and clinical presentation of the tumors and the surgical results of this series are comparable to other European ones. We found a higher incidence of neuronal and mixed neuronal-glial tumors and oligodendrogliomas and a slight lower incidence of ependymomas. Our results should encourage further national multi-institutional studies to better characterize these tumors in the pediatric population. [ABSTRACT FROM AUTHOR]

Purpose: We present a technical development of the endovascular treatment technique for vein of Galen aneurysmal malformation, using Onyx™ (ethylene vinyl alcohol copolymer) delivered under flow control through double-lumen balloon microcatheters. Materials and methods: An 11-month-old patient that initially presented with increasing head circumference was diagnosed with mural type vein of Galen aneurysmal malformation. Complete cure was obtained in a single endovascular treatment session. Onyx was delivered simultaneously through two double-lumen balloon microcatheters (Scepter™, Microvention, Terumo Group). In both arterial feeders, balloon inflation occluded the high-flow arterial-venous shunting and thus facilitated the controlled propagation of the embolic product in the afferent artery and the shunt point while preventing distal migration into the venous system. Results: Two years post-procedure, MR imaging showed persistent occlusion of arterial-venous shunts with complete regression of the venous dilatation. On clinical examination, the patient had no neurological deficits, and no cognitive impairment was detected at neuropsychological testing. Conclusion: The use of double-lumen balloon microcatheters for flow control during delivery of Onyx represents a viable alternative for the endovascular treatment of vein of Galen aneurysmal malformations. [ABSTRACT FROM AUTHOR]

The article presents a case study of an 11-month-old boy with growing mass on his scalp. Topics mentioned include the computed tomography (CT) scan performed to him which revealed a sinus pericranii, the treatment for the disease, and the intramembranous ossification experienced by the boy. Also mentioned was the reduction of the frontal lesion in size after his treatment.

Introduction: Cerebral venous sinus thrombosis (CVST) following a blunt head trauma is a rare condition, described in the literature along with the lack of consensus regarding diagnosis and management. Case summary: We present a case of a pediatric patient with a blunt head injury and epidural hematoma, who developed dural sinus and internal jugular vein thrombosis with fatal outcome. Discussion: Most of reports show good outcome and recovery, but CVST might be related to poor recovery and even lead to death. The diagnosis and management of this condition are discussed based on a literature review. Conclusion: It is important to keep a high degree of suspicion of CVST since early diagnosis may prevent potentially treatable catastrophic outcomes. [ABSTRACT FROM AUTHOR]

Introduction: The intracranial bridging veins are pathways crucial for venous drainage of the brain. They are not only involved in pathological conditions but also serve as important landmarks within neurological surgery. Methods: The medical literature on bridging veins was reviewed in regard to their historical aspects, embryology, histology, anatomy, and surgery. Conclusion: Knowledge on the intracranial bridging veins and their dynamics has evolved over time and is of great significance to the neurosurgeon. [ABSTRACT FROM AUTHOR]

In this article the author discusses aspects of a preliminary Magnetic resonance imaging (MRI) study on fosterior fossa decompression and the cerebellum in Chiari type II malformation. He is critical on the findings that Chiari II malformation is the effect of decompression of the embryonic vesicles and fetal ventricular system due to open neural tube. Also investigated is the 35-year foolw up on the 1500 children and young adults with spina bifida.

Background: Non-syndromic coronal synostoses oxycephaly and brachycephaly (NSCSOB) are rare. Their natural history, surgical management, and outcome are debated. Material and methods: We reviewed the available literature on NSCSOB. In addition, we reviewed retrospectively our experience with cases of NSCSOB, managed and operated by our joint craniofacial team since 1984. Newborns underwent perifrontal craniectomy, while infants and older children underwent fronto-orbital advancement with frontoparietal remodeling. Cases with delayed onset of faciosynostosis were excluded. Results: Some cases of NSCSOB present at birth or even prenatally with gross dysmorphism and severe brachycephaly; others present later in life with harmonious restriction of intracranial volume and are at risk for development and vision. Some NSCSOB evolve from initially unisutural synostoses. We treated 61 cases, operated aged 1.4 to 106.7 months, 30 with brachycephaly and 31 with oxycephaly. Twelve of these (19.7 %), had been treated initially for sagittal or unicoronal synostosis, and evolved into NSCSOB. Intracranial hypertension was present initially in 28 (45.9 %), with ophthalmological consequences in 13 (21.3 %) and mental retardation in 6 (9.6 %). No patient had hydrocephalus; only two had asymptomatic Chiari malformation. The mean postoperative follow-up was 113.6 months. At last control, 21 patients had developmental delay and 5 had visual impairment. Conclusions: NSCSOB are a complex entity. They have in common a high risk of severe intracranial hypertension and rare hydrodynamic complications. Genetic screening is necessary to assert their non-syndromic nature. Prolonged follow-up of all craniosynostoses is necessary because unisutural synostoses can evolve into NSCSOB, and NSCSOB can evolve into craniofaciosynostosis. [ABSTRACT FROM AUTHOR]

Introduction: Pseudotumor cerebri is a condition characterized by raised intracranial pressure, normal CSF contents, and normal brain with normal or small ventricles on imaging studies. It affects predominantly obese women of childbearing age; however, its incidence seems to be increasing among adolescent and children. While among older children the clinical picture is similar to that of adults, younger children present demographic and clinical peculiarities. Different diagnostic criteria for adults and pre-pubertal children have been proposed. Etiology and pathogenesis are still unclear, particular concerning the role of obstruction to venous outflow. Methods: An extensive literature review concerning all the aspects of pseudotumor cerebri has been performed, both among adults and pre-pubertal children. Conclusion: Pseudotumor cerebri is an avoidable cause of visual loss, both in adults and children. Few diagnostic measures are usually sufficient to determine the correct diagnosis. Since pseudotumor cerebri is a diagnosis of exclusion, the differential diagnosis work out is of special importance. Modern neuroimaging techniques, especially magnetic resonance imaging and magnetic resonance venography may clarify the role of obstruction to venous outflow in each case. Various therapeutic options are available: medical, surgical, and endovascular procedures may be used to prevent irreversible visual loss. Treatment is usually effective, and most patients will experience complete resolution of symptoms without persistent deficits. [ABSTRACT FROM AUTHOR]

ims: The aims of this study were to analyze the clinical features, radiologic findings, bacteriologic spectrum, and management protocols and outcomes in posterior fossa subdural empyemas in children. Materials and methods: This study is a retrospective analysis of all children (age, <18 years) treated over a ten-year period (1994-2004) at NIMHANS, India. Clinical, bacteriologic, radiologic, and follow-up data were analyzed. Results: Twenty-seven children with posterior fossa empyemas were treated during this period, making this the largest series to date dealing with this rare entity. Posterior fossa empyemas are seen more commonly in the summer months and in males. Of the patients, 74.1% were in altered sensorium. The clinical features included the triad of fever, headache, and vomiting, which is a nonspecific picture. Cerebellar signs were elicited only in 40%. The most common source was untreated middle ear infection. Pus usually accumulates over the cerebellar convexity and is associated with hydrocephalus in 74% of patients. Cultures of the empyema pus were positive in 74% of cases, and 18.5% had polymicrobial infections. Only 21% of the patients needed a permanent CSF diversion procedure. Craniectomy is the treatment of choice in these cases. Conclusions: The clinical features are nonspecific. Early surgery can salvage most patients and obviate the need for permanent CSF diversion procedures. Surgery (evacuation of empyema and mastoidectomy), antibiotics, and management of hydrocephalus are the mainstays of treatment. [ABSTRACT FROM AUTHOR]

Venous hypertension is emerging as a significant contributor to intracranial pressure in children with syndromic craniosysnostosis. This is associated with jugular foramen stenosis or atresia and with the development of collateral emissary veins. We demonstrate how computed tomography venography can document the prevalence of these emissary veins and how their visualisation plays an important role in operative planning. Patients with known syndromic craniosynostosis underwent computed tomography venography as part of their routine pre-operative evaluation. The venous drainage pattern was examined in these patients, with a special note of the presence of abnormal venous pathways. Eleven patients were recruited into the study from ages 3 months to 22 years. All had a diagnosis of syndromic craniosynostosis with six Crouzon’s, four Pfeiffer’s and one patient with Crouzon’s and acanthosis nigricans. Nine of 11 patients had demonstrable evidences of transosseous venous drainage through an identifiable abnormal emissary vein. Four of 11 had a transosseous route as the main mechanism of drainage for the cerebral venous system. Patients with syndromic craniosynostosis often demonstrate abnormal venous anatomy, which can have serious consequences on craniofacial surgery, especially when a posterior decompression is being considered. Based on these findings, the authors assert that those children with some syndromic craniosynostosis being considered for surgery should undergo venographic studies as part of their pre-operative evaluation. [ABSTRACT FROM AUTHOR]

Case reports: We report two girls with a typical presentation of sinus pericranii. Both patients received total excision of the subgaleal masses with complete obliteration of the communications to the intracranial portion. However, different gross appearances, neuroimaging findings and intraoperative findings were found. It took more time and attention to control the bleeding due to the large communication between the extracranial and intracranial components in the second case. Discussion: Sinus pericranii is composed of abnormal extracranial vascular tissues that communicate directly with intracranial dural sinuses or veins. A review of the literature showed that 13 out of 31 patients with sinus pericranii had a high-flow pattern. Detailed physical examination and imaging studies to detect dilated scalp veins and a large bony defect should remind surgeons of the presence of large venous communications. Meticulous treatment of the large communication during surgery can avoid massive bleeding. [ABSTRACT FROM AUTHOR]

Introduction Vein of Galen aneurysmal malformations (VGAM) are assumed to be related to the persistence of the embryonic median prosencephalic vein of Markowski, which does not involute in cases of VGAM and becomes the venous collector of the shunt, characteristic of the malformation. The arterial feeders of VGAMs are all primitive meningeal arteries present during the embryonic period of the constitution of the malformation. It has also been assumed that the internal cerebral veins were absent in patients presenting with VGAM. There is no clear evidence indicating, however, that the deep venous structures cannot develop normally. Case reports We report on two cases of VGAM in which superselective retrograde transvenous catheterization and MRI demonstrated that normal internal cerebral veins were draining into the aneurysmal vein of Galen. Conclusions It is conceivable that, as in our patients, this drainage pattern is only angiographically detectable via selective retrograde transvenous drainage. The possibility of such normal deep galenic venous drainage must be considered in VGAM management, as it may imply occurrence of adverse effects when the malformation is occluded on the venous side. [ABSTRACT FROM AUTHOR]

Objective In neonatal lambs, the quantitative evidence suggests that a significant volume of cranial CSF drainage is associated with transport along olfactory nerves with absorption primarily into extracranial lymphatics in the paranasal region. Arachnoid granulations appear to be poorly developed at this level of development and their function is unknown. In this report, we tested whether a CSF protein tracer ( 131I-human serum albumin) could transport directly into the superior sagittal sinus of newborn lambs. Methods and results The concentration of the tracer administered into the CSF compartment was measured in the confluence of the intracranial venous sinuses (torcula) and in the peripheral blood (inferior vena cava). Enrichment of the CSF tracer in the cranial venous system was most evident when the CSF-venous sinus pressure gradients approached 20?30 cm H 2O. Conclusion The data suggests that neonatal CSF can be absorbed directly into the cranial venous system. However, contrary to the classical view, this route may represent an auxiliary system that is recruited to compliment lymphatic transport when intracranial pressures are very high. [ABSTRACT FROM AUTHOR]

Abstract Objects. We hoped to itemize the clinical and neuroradiological features of six neonates with mitochondrial disorders. Methods. We examined a case series of six neonates. The diagnosis of mitochondrial cytopathy was made on the basis of spectrophotometric measurements of respiratory chain enzyme activities in skeletal muscle biopsy specimens. Magnetic resonance (MR) imaging was performed in all cases. Conclusions. The antenatal onset in five cases and the lack of any symptom-free interval are suggestive of fetal expression of the disease. No specific symptoms were found: arthrogryposis congenita multiplex in one, progressive hepatocellular dysfunction in three, encephalomyelopathy and cardiomyopathy in four. Complex I deficiency was found in three patients, while one patients had a defect of complex IV and the last a combined defect of complexes I and IV. Neuroradiological findings were either cerebral atrophy or white matter abnormalities of the brain stem in all cases but one and gave additional information, because clinical symptoms are not quite specific. The combination of clinical and MRI findings in neonatal cases can rule out hypoxic ischemic encephalopathy, which suggests an additional screening method to look for mitochondrial disorder. [ABSTRACT FROM AUTHOR]

Background and purpose: Mild ventricular dilatation or an asymmetric aspect of the atrium with prominent subrachnoid spaces is a challenging clinical condition in utero that requires prenatal MRI to rule out a destructive lesion or brain malformation. We report five cases that demonstrated benign external hydrocephalus postnatally, together with the prenatal MRI to define prenatal criteria of so-called benign external hydrocephalus. Methods and results: The prenatal MR images of five cases showing typical features of external hydrocephalus postnatally were reviewed. All cases showed in utero mild ventricular di- latation at the level of the atrium with enlargement of the subarachnoid spaces homo-, contra- or bilaterally, and predominantly in the parieto- occipital areas. The head circumference was normal in all cases with no family history of macrocephaly. MRI did not demonstrate either abnormal signal within brain parenchyma or loss of the normal layering of the developing brain. Conclusion: Posterior mild ventricular dilatation and prominent subarachnoid spaces in a poste-rior distribution can be considered an early stage of benign external hydrocephalus that is nicely illustrated by MRI. [ABSTRACT FROM AUTHOR]

This article reviews the range of hydrodynamic disorders affecting the CSF circulation. Initially consideration is given to questions of definition and classification. A scheme for the practical, clinical analysis for the diagnosis of such disorders is then presented. The physiology and pathophysiology of the CSF circulation is reviewed, with particular emphasis on issues that remain unresolved. This provides a background to consideration of the adverse consequences of abnormal CSF hydrodynamics, again focusing on areas where further information is required. Methods of clinical investigation of CSF hydrodynamics are reviewed, followed by general considerations of treatment. Finally, each of the main, clinically important, forms of disordered CSF hydrodynamics is briefly considered, with particular emphasis, again, on areas where current knowledge is deficient. The conditions considered include hydrocephalus of various forms (childhood, adult, arrested, multi-compartment), infantile macrocephaly, arachnoid and glio-ependymal cysts, syringo- and hydromyelia, pseudotumour cerebri, impaired cranial venous outflow, altered CSF composition, shunt obstruction without ventricular enlargement and low-CSF-pressure states. [ABSTRACT FROM AUTHOR]

This report presents a case of arteriovenous malformation (AVM) in the region of the superior vermis and quadrigeminal plate, which was partially embolized using a endovascular technique and subsequently surgically excised. An occipital transtentorial approach was employed to excise the AVM, and the patient did well without neurological complications postoperatively. Comparing various surgical approaches, we believe the occipital transtentorial approach is the best for the AVM of this region. [ABSTRACT FROM AUTHOR]

Intracranial dural cyst is a very rare congenital malformation. The presence of such a cyst was confirmed by means of computed tomography and magnetic resonance findings in a child with an enlargement of the head and developmental retardation and confirmed histologically in surgically obtained specimens of cyst wall. [ABSTRACT FROM AUTHOR]

Infantile myofibromatosis (IM) is a proliferative disorder of infancy and early childhood characterized by the nodular or diffuse growth of lesions that are comprised of a mixture of mesenchymal elements. Intracranial involvement is reportedly rare, only eight such patients having been reported to our knowledge. We report on a 4-year-old boy with intracranial IM with a mass in his left temporal bone. A previous report on intracranial IM proposed that the underlying dura mater should be resected because of the possibility of early recurrence. At surgery in this case, the tumor was noted to be located in the bone itself and did not arise from the underlying dura. Therefore, the underlying dura mater and venous sinus were preserved. The follow-up MRI showed no sign of recurrences. It may not to be necessary to resect the dura mater in patients with intracranial IM. [ABSTRACT FROM AUTHOR]

In the past, the diagnosis of 'growing skull fracture' or 'diastatic fracture' has included a subset of injuries better referred to as 'cranial burst fracture.' Cranial burst fracture, typically associated with severe injury in infants less than 1 year of age, is a closed, widely diastatic skull fracture accompanied by acute cerebral extrusion outside the calvarium. We treated 11 such infants at the LeBonheur Children's Medical Center and 2 at the Children's National Medical Center from January 1986 through December 1994. Infants ranged in age from 1 to 17 months, with an average age of 5.7 months. All presented with marked scalp swelling and a Glasgow Coma Scale score of 10 or less. Twelve had a history consistent with severe injury (motor vehicle accident, 7, abuse 5). The cause of injury in one patient remains unproven. Surgery (reduction of herniated cerebral tissue, repair of large dural laceration, and cranioplasty) was usually performed within 10 days of injury, a time period long enough to assure hemodynamic stability and resolution of acute cerebral swelling, yet sufficiently brief to avoid the chronic changes (scarring, parasitization of scalp vessels by damaged cortex) associated with a 'growing skull fracture.' Prompt repair of cranial burst fracture may prevent ongoing brain injury such as has been neuropathologically demonstrated in patients with 'growing skull fracture.' Magnetic resonance imaging establishes the diagnosis of cranial burst fracture in equivocal cases, rendering unnecessary a 'waiting period' to see if scalp swelling resolves. Our experience, together with information in the neuropathological and neurosurgicla literature, suggests that cranial burst fracture is associated with severe trauma, requires expeditious treatment, and has been underdiagnosed in the past, leading to 'growing skull fracture,' a condition requiring more extensive surgery. [ABSTRACT FROM AUTHOR]

We present 26 consecutive cases of nongalenic pial arteriovenous malformations (PAVMs) diagnosed in the neonatal period or in infancy. No diagnosis was made antenatally. Presenting symptoms in neonates were systemic cardiac manifestations (54%), seizures (31%), and hemorrhages (15%). In infants, hemorrhagic strokes and hydrodynamic disorders (external or internal hydrocephaly, macrocephaly, atrophy) both occurred in 38% of cases. Systemic cardiac manifestations and seizures were rare at that age (respectively 16% and 8%). Sixty-two percent of neonates and 31% of infants already had neurocognitive disorders (assessed by pediatric neurocognitive testing: Brunet-Leizine and Denver tests) when referred. The venous drainage and its anomalies (ectasias, stenoses, thromboses) were the main causes of symptoms. Atrophy and leukomalacic lesions occurred rapidly; they express local hydrovenous disorders and are specific to this population group. Untreated neonates and infants have a poor prognosis. Endovascular treatment, although partial and challenging in all instances, represents the treatment of choice in our series. Of the eight neonates treated, one improved to normal (12.5%), while four remained stable (50%): two neurologically normal, two with mild neurological deficit. Three (37%) died despite embolization (heart failure, multiorgan failure, postoperative death). Transient neurological complications occurred in two cases (25%): hemiparesis in one patient with a rolandic and in one with a thalamic AVM. Of the eight infants successfully embolized, one was significantly improved (12.5%) and is now neurologically normal, while five remained stable (62.5%): four neurologically normal, one with mild neurological deficit. One died between two sessions of embolization from intracerebral hemorrhage (12.5%). Hemianopsy occurred in one case (12.5%) after embolization of an occipital AVM. In one additional case in a normal child we failed to embolize the last small pial AVM of four after the three others had spontaneously thrombosed. With a minimal follow-up of 18 months and a maximum of 7 years, the review of our series shows 53% of the initial group of neonates and infants growing neurologically normal after therapeutic management in our institution; 23.5% died despite treatment, and the remaining 23.5% present minor neurological deficit. When targeted at the points of angioarchitectural weakness, embolization contributes to stabilizing a lesion. It should be undertaken rapidly to avoid loss of brain substance secondary to hemorrhage, atrophy, or leukomalacia, and to allow neurocognitive recovery and normal brain maturation. In our experience, theses lesions are the most aggressive ones for the maturating brain, and the most difficult to approach technically. They represent a new therapeutic field and have their own specific anatomy and physiology. [ABSTRACT FROM AUTHOR]

We report eight cases of vein of Galen aneurysmal malformation (VGAM) assoicated with a 'Chiari type I malformation.' In four cases magnetic resonance imaging (MRI) or computed tomography performed in the neonatal period did not demonstrate the posterior fossa anomaly, which appeared on later scans. In the other cases the MRI was performed in infancy and the anomaly was already present. We compared the venous phases of the posterior fossa angiograms and the MRI in these patients. In all eight cases, the angiograms showed a reflux in the cerebellar veins, via the petrous vein, associated with a uni-or bilateral stenosis or thrombosis of the distal posterior dural sinuses. Furthermore, in two cases the posterior fossa returned to normal on MRI following endovascular treatment, while in three cases the herniation of the cerebellar tonsils decreased after the embolization. Tonsillar prolapse becomes irreversible when the venous outlet is incapable of taking the flow even when the VGAM has been treated adequately. In eight additional cases of VGAM for which MRI and angiogram studies were available and in which stenosis or thrombosis of posterior dural sinuses was present without tonsillar prolapse, no reflux into the cerebellar veins was shown. We suggest that the posterior fossa hydrovenous congestion is a result of inadequate venous drainage and that the tonsillar descent is reversible if adequate venous drainage is reconstituted following therapeutic embolization of the fistula. Tonsillar prolapse is not a consequence of mass or raised intraventricular pressure. Our observation suggests that in some other conditions, the 'Chiari I malformations' may be secondary to early hydrovenous dysfunction of the posterior fossa. [ABSTRACT FROM AUTHOR]

Two cases of retroflexed holoprosencephaly are presented. Reports of nine cases of retroflexion were available in the literature. Case analysis indicates that retroflexion results from subdural fluid collection. The incidence of retroflexion in holoprosencephaly was apparently higher than that of ordinary subdural hygromas. Possible explanations for this high incidence are given. The clinical significance and management of retroflexion are touched upon. [ABSTRACT FROM AUTHOR]

The results of a prospective study on excision of the outer and inner membrane for symptomatic primary middle fossa arachnoid cysts in children are presented. During the period 1982-1989, among 48 cases of cyst in various locations, 18 symptomatic patients were treated by excision of both the outer and inner membranes with an opening to the basal cistern. The mean patient age was 3.2 years. All patients were evaluated by examinations, including magnetic resonance imaging (MRI), X-ray computed tomography (CT), quantitative CT cisternography (CTC), digital substraction angiography (DSA), N-isopropyl-p[123]iodoamphetamine single-photon emission CT (IMP-SPECT) and IQ. All showed abnormal cerebrospinal fluid flow dynamics. Significant complications included massive subdural effusion in two patients and transient pulmonary edema in one. There were no recurrences during the follow-up period (mean 4.7 years). Two morphological types were noted: type I, the classical anteromedial type where the cysts are attached directly to the adjacent parasellar cisterns, and type II, the anterolateral type, where the diagonally concave anterior temporal lobe covers the adjacent cisterns, making wide opening difficult. Type I accounted for 78% of all cases, and the reduction of the cyst volume with clinical improvement was remarkable within 6 months after surgery. Angiographically, 22% of cases showed tapering and retrograde filling of the superficial middle cerebral vein. This pattern is not included in Hacker's normal variations [18] and suggest mild but chronic compression of the developing brain. Even in patients with hemispheric cyst, the reconstituted brain showed sufficient cerebral perfusion on SPECT, suggesting that the nature of this disease entity is reversible developmental arrest. These results confirm that excision is a safe, effective shunt-independent procedure for middle-fossa arachnoid cysts, especially for those of type I. [ABSTRACT FROM AUTHOR]

Data concerning venous anatomy, interstitial fluid pressure and cerebral blood flow indicate that obstruction of cerebral venous outflow (as a whole or involving the deep venous system alone) is the essential cause of hydrocephalus. Choroidal and ventricular veins both belong to the deep system. Choroidal venous pressure determines cerebrospinal fluid pressure; pressure in the ventricular veins determines interstitial fluid pressure in the paraventricular white matter. A decrease in deep cerebral blood flow causes paraventricular atrophy. CSFP is higher than interstitial fluid pressure, normally and in venous obstruction. Thus, CSFP prevents venocongestive edema (but not inflammatory edema) of the brain. Collateral venous pathways are described. Venous obstruction causes hydrocephalus only when it leads to insufficient blood flow. Cerebrospinal fluid shunting causes increased CBF as essential therapeutic effect in hydrocephalus, but also causes venocongestive brain edema, which explains the decrease in ventricle size and the side effects of shunting. [ABSTRACT FROM AUTHOR]

Acute intracranial hematomas in 17 term neonates are reported: 3 were subdural in the posterior fossa; 14 supratentorial, either subdural (8), intracerebral (1 frontal, 4 occipital) or intraventricular (1). The hematomas were aspirated in 3 cases and surgically evacuated in 10. In the remaining 4, the effusion was left in situ. Three patients died: 2 due to dramatic preoperative deterioration and 1 to hemostatic difficulties during surgery. In the 14 survivors, neurological sequellae are major in 3, nil or minor in 11. Comparison of these cases to the 110 others already published allows the following conclusions: (1) the cause of such hematomas is usually multifactorial, combining obstetrical trauma, anoxia and/or coagulation disorders; (2) early diagnosis of the hematoma requires early echography and/or tomodensitometry; (3) when the hematoma causes a midline shift, the authors advocate its early evacuation by aspiration, or craniotomy if it is clotted; (4) the prognosis is poor when there is associated severe perinatal anoxia; if there is no anoxia, the prognosis seems good, but as the follow-up is usually short, definite conclusions are difficult; (5) in our series hydrocephalus requiring treatment occurred in 2 of the 14 survivors. [ABSTRACT FROM AUTHOR]

A 12-month-old infant presented with cerebral seizures and neurological deficits. MRI scan of the brain and angiography showed massive cerebral venous sinus thrombosis complicated by a dural arteriovenous fistula. Subsequent clotting analysis revealed a protein S deficiency. Screening for inherited coagulation inhibitor deficiency is recommended in children with unexplained or atypical thrombotic events. [ABSTRACT FROM AUTHOR]

An 8-year-old boy suffered severe craniocerebral trauma with left-sided fronto-orbitobasal fracture. The CT scan showed minor subdural air inclusions. The child recovered well and had no clinical signs of aftereffects. Eight years after the accident, symptoms of intracranial pressure developed progressively with nuchal rigidity and elevated temperature. The CT showed an extensive left fronto-orbitobasal abscess. The intraoperative finding was a brain prolapse both into the frontal sinus and into the ethmoidal cavity with a large dura-bone defect at the site of the former fracture line, which was closed with refobacin-bone-meal fibrin sealant plasty and glued periostal patch. The postoperative course was unremarkable. Evidently, the accident had caused a brain prolapse into the bone defect, which prevented liquorrhea. Due to the lack of bone and dura barrier, a late brain abscess developed in the course of sinusitis. In such cases, primary surgical revision seems to be indicated. [ABSTRACT FROM AUTHOR]