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Three-dimensional (3D) constructive interference in steady state (CISS) is a gradient-echo MRI sequence that is used to investigate a wide range of pathologies when routine MRI sequences do not provide the desired anatomic information. The increased sensitivity of the 3D CISS sequence is an outcome of the accentuation of the T2 values between cerebrospinal fluid (CSF) and pathological structures. Apart from its well-recognized applications in the evaluation of the cranial nerves, CSF rhinorrhea and aqueduct stenosis, we have found the CISS sequence to be useful for the cisternal spaces, cavernous sinuses and the ventricular system, where it is useful for detecting subtle CSF-intensity lesions that may be missed on routine spin-echo sequences. This information helps in the management of these conditions. After a brief overview of the physics behind this sequence, we illustrate its clinical applications with representative cases and discuss its potential role in imaging protocols.

Summary ‘Benign’ aqueduct stenosis is a common cause of hydrocephalus in the paediatric population and is frequently treated by endoscopic third ventriculostomy. Occasionally, aqueduct stenosis can be a prelude to the development of other pathology, as is seen in these two cases of pineal tumours developing in patients whose hydrocephalus was successfully treated with endoscopic third ventriculostomy. The case histories are presented, along with the recommendation for increased radiological screening of patients with this usually ‘benign’ presentation.

Tumours of the pineal region are uncommon. We report on a 62-year-old male presenting with Parinaud's syndrome and aqueduct stenosis caused by a cystic tumour in the pineal region. During surgery, adjacent to the cystic tumour, a second smaller tumour was identified, which was clearly separate from the first tumour and from the pineal gland. Histological examination disclosed the cystic tumour as an epidermoid cyst, whereas the second tumour demonstrated histological and immunohistochemical features of a pineocytoma. The unique finding of two different types of tumours in the pineal region is evaluated with regard to the histogenesis of epidermoid cysts and pineocytomas.

This study was designed with an aim to detect the congenital anomalies appear to be linked to and in conjunction with hydrocephalus fetuses in Sudan, when ultrasound is used to exam fetuses in the second and third trimesters of pregnancy. This prospective cohort study was performed from December 2011 to December 2013, in a group consists of 5000 single gestation pregnant Sudanese women. In all cases, maternal ages were 35 years up to 48 years; mean age of 42.5 years. Pelvic; obstetric ultrasound scanning protocol used should meet the standards established by the American Institute of Ultrasound in Medicine (AIUM) for scanning in the second and third trimesters of pregnancy. Statistical Package for the Social Sciences (SPSS) was used to analyze the results. Diagnosed hydrocephalus cases (0.4%) were found to be associated with other fetal anomalies as aqueduct stenosis (45%), spina bifida (30%), Arnold-Chiari malformation (20%) and Dandy-Walker malformation (5%). The incidence of congenital anomalies and hydrocephalus in Sudanese fetuses showed considerable variation among different regions of Sudan. Hydrocephalus is associated with certain congenital anomalies. In agreement with previous studies, hydrocephalus is predominantly in male rather than female fetuses. The prevalence of fetal anomalies and hydrocephalus are within previously reported ranges.

Objects: The most important function of the programmable valve (PV) is to limit the shunt-dependent flow of the cerebrospinal fluid by upgrading valve pressure. This activates the regular circulation of cerebrospinal fluid, which may make successful removal of the shunt possible once sufficient cerebral development has been achieved. The purpose of this paper is to indicate the possibility of shunt removal using the programmable Medos and Sophy valves (one programmable Sophy valve was specially designed for this situation). Methods: Prior to regular use of the PV, removal of existing shunt systems was attempted in 57 children, since some systems malfunctioned and others had abdominal tubes that were meanwhile too short as the children had grown as they became older. Shunt removal was successfully achieved in only 18 patients (32%). However, in patients in whom PV valves were used, shunt removal was successful in 68 out of 114 patients (57%). This shows that the success rate of shunt removal becomes significantly higher when PV valves are used. The 68 cases in which PV valves were used and shunt removal was successful were divided into three groups: A, B, and C. In group A (36 cases, 53%), the Medos valve was used for the initial PV shunt implantation and the pressure was gradually increased up to 200 mmH2O. The shunt systems were then withdrawn. Group B (29 cases, 43%) includes patients who experienced both the minor symptoms and ventricular enlargement attributable to increased valve pressure. The pressure was gradually upgraded by pumping several times and was maintained at close to 200 mmH2O. After 6–24 months' observation shunt removal was performed, and in 21 out of 29 cases the outcome was good. However, the remaining 8 patients (12%) still had symptoms and required shunt reinsertion. The specially designed Sophy valves were then used, which allowed the pressure to be set at above 200 mmH2O. The pressure was increased by degrees up to 400 mmH2O and kept at the same level for 6–24 months. The shunt systems were then removed successfully. Although a high pressure setting was required over a sustained period, a total of 29 patients (43%) were able to have their shunts removed. In group C (3 cases, 4%), which included patients with aqueduct stenosis, the pressure was raised and thus allowed ventricle enlargement. Third ventriculostomy was performed under neuroendoscopy with the shunt pressure maintained at a high level. Shunt systems were removed successfully. Conclusions: This study showed that it is possible to remove the shunt systems in 50% or more of pediatric hydrocephalus cases in which PV valves are used. This is achieved through careful control of the valve pressure. Close observation is essential during the period when the PV pressure is maintained at a high level, as well as 6–12 months after shunt removal.

Chronic hydrocephalus, most commonly the result of aqueduct stenosis, is associated with both primary and secondary amenorrhea. Only six cases of secondary amenorrhea have been reported to date. We describe a women with cystic fibrosis who presented with secondary amenorrhea as a consequence of Chiari-I malformation and resultant hydrocephalus. The biochemical picture was characterized by hypogonadotropic hypogonadism. Resolution of the amenorrhea was observed to occur following 3rd ventriculostomy.

Thunderclap headache is well known to be a presenting feature of a variety of causal events. Indeed, a primary form is considered in the International Classification of Headache Disorders-II, but such diagnosis must be made only after exclusion of a possible secondary cause. We report a case of late-onset idiopathic aqueductal stenosis presenting with thunderclap headache, in the absence of abnormal neurological findings or indirect signs of raised intracranial pressure. The patient recovered completely after endoscopic third ventriculostomy. This case indicates primary aqueduct stenosis as a possible, never previously reported, cause of thunderclap headache.

We report on two abortuses with hydrocephalus due to congenital stenosis of the aqueduct of Silvius. The occurrence of this disorder in two siblings (a male and a female) with normal parents supports the autosomal recessive pattern of inheritance. Such a mechanism of inheritance should be taken into account when counselling families with congenital hydrocephaly due to aqueduct stenosis.

The purpose of this study is to evaluate the changing epidemiology of paediatric hydrocephalus over the past three decades in a single institution. All children treated for newly diagnosed hydrocephalus during the 1985–1990 (group A) and the 2000–2005 periods (group B) were enrolled and classified according to the associated cause of hydrocephalus. A significant 8.8% decrease of the incidence of hydrocephalus was noticed between the two time periods, resulting from the reduction of hydrocephalus associated to myelomeningocele, aqueduct stenosis (p = 0.04), CNS infection (p = 0.03), cranio-cerebral malformation and head injuries; post-haemorrhagic hydrocephalus remained stable, while the tumour-associated one increased (p

The authors examined the visual function in 19 cases of non-tumoral aqueduct stenosis. Visual symptoms, acuity and fields were assessed, and optic disc abnormalities noted. Age at presentation ranged from three months to 59 years, and follow-up from ten months to 20 years. Although only two of 19 patients presented with visual symptoms, 13 patients were found to have ophthalmic involvement on careful examination. A high proportion of post-operative patients reported ophthalmic symptoms when ventricular shunt blockage occurred. A classification of the degree of visual involvement has been devised. It was concluded that every patient with aqueduct stenosis should undergo regular ophthalmic examination, which provides a readily quantifiable assessment of progress in most cases.

In the 14-year period from 1974-1987 during which CT scanning was available in Edinburgh, 36 patients with benign, non-tumour related aqueduct stenosis were identified from a group of 342 patients with hydrocephalus. In terms of age at presentation, the patients separate into two groups, 9 cases diagnosed during the first year of life and 27 patients aged from 7 to 72 years. The presentation in the early cases was fairly acute, the diagnosis was secure and the operative treatment by ventricular peritoneal shunt was relatively free of complications. In the older patients, the first presentation was more chronic, and further investigation required a change of diagnosis in 5 of the 27 patients. More than half of the patients had recurrent symptoms which were much more acute and operative treatment was attended by a significant number of complications including death from intraventricular haemorrhage in one case.

Aims of present investigations. Adult patients with clinical and radiological findings consistent with normal pressure hydrocephalus (NPH) were studied. Two of the aims were to document intracranial pressure (ICP) levels and quantity of B-waves during sleep and wakefulness, and to compare the results with preoperative clinical symptoms and outcome after surgery. The third aim was to document if a pressure gradient over the mantle of the brain (transmantle pressure gradient) exists. The fourth aim was to evaluate the effectiveness of endoscopic third ventriculostomy (ETV) as a treatment of primary aqueduct stenosis. The fifth aim was to determine if adult patients with an early-onset hydrocephalus that resurfaces later in life and diagnosed with ¡°asymptomatic¡±, ¡°arrested¡±, or ¡°compensated¡± hydrocephalus really were ¡°asymptomatic¡± and evaluating if they would benefit from surgery. Material and methods. A total of 95 patients with communicating or noncommunicating hydrocephalus were investigated. Demographic and clinical data, brain and cerebrospinal fluid imaging were systematically documented and analysed before and after surgery. Surgical treatment consisted of ETV or shunt surgery, and in cases of failed ETV a shunt implantation was recommended. Follow up was three months after last surgery in all surgically treated patients. Fifty-six underwent a long term ICP registration. Computerised analysis of ICP levels and B-waves were performed (in 55) after the final postoperative results and compared to clinical and demographic data. Ten patients had a measurement of the transmantle pressure. Eighteen patients who had been treated with ETV for aqueduct stenosis were identified and examined. Those who had not improved as expected were further investigated and recommended a shunt implantation. Twenty-three patients with clinical and radiological characteristics consistent with one of the diagnoses ¡°asymptomatic¡±, ¡°compensated¡±, ¡°arrested¡± or symptomatic congenital hydrocephalus were investigated and advised to have surgery. Results and conclusions. Contrary to the general conviction, the preoperative magnitudes of the cardinal NPH symptoms and signs were strongly interrelated. There was no correlation of the number of B-waves or magnitude of ICP with demographic data, clinical findings or outcome after surgery. The mean ICP was higher in noncommunicating hydrocephalus; however, most patients in both groups had a normal mean ICP (¡Ü 15 mm Hg). The lack of correlation between the clinical picture of NPH and ICP undermines the use of pressure in classifying hydrocephalus. There is no transmantle pressure gradient in patients with communicating or noncommunicating hydrocephalus. The long term efficacy of ETV for adult patients with noncommunicating hydrocephalus was sufficient in only 50% of the cases. One-third demonstrated temporary improvement after ETV and then deteriorated to an even worse clinical condition, despite patent ventriculostomies. All patients who did not permanently improve after ETV benefited from shunt surgery. Patients with longstanding nonprogressive hydrocephalus, even for decades, can benefit from surgery.The traditional model of CSF circulation and hydrocephalus development cannot adequately explain clinical findings and investigational results in hydrocephalic patients. A new model is proposed that assumes a strong relationship between the CSF dynamics and the fluid equilibrium of the brain.

In Sweden, the annual incidence of surgery for hydrocephalus was 3.4 per 100,000 adults between the years 1996 and 1998. The most common indication for surgery was normal pressure hydrocephalus (NPH; 47%), followed by high-pressure hydrocephalus (27%). Seventy-three percent of the patients had communicating hydrocephalus, of which 63% had NPH. Twenty percent of the patients had non-communicating hydrocephalus, of which half resulted from aqueduct stenosis. The annual incidence of operations varied between regional clinics from 2.3 to 6.3 per 100,000 inhabitants.

A dog with hydrocephalus as a result of aqueduct stenosis and cerebellar herniation underwent ventriculoperitoneal shunting. Magnetic resonance images and computed tomography scans taken after the surgery revealed subdural accumulations of haemorrhagic fluid and cereberocortical collapse caused by overshunting and leakage of cerebrospinal fluid from the site of insertion of the shunt. However, the degree of cerebellar herniation was reduced after the shunt was inserted, and the dog did not develop any neurological signs and made good progress.

The beneficial effects of stereotactic third ventriculostomy versus ventriculoperitoneal shunt were evaluated in 62 paediatric patients and analysed in relation to age, sex, clinical history, presence of meningomyelocele, magnetic resonance imaging measurements of hydrocephalus and third ventricle floor size. The third ventriculostomy were done on 50 patients using the Richard-Wolf® Caemaert Endoscope and the Leksell Stereotactic Frame Model G. These patients were operated using the 4-French Fogarthy catheter to open the base of the third ventricle. During the same period of study 12 paediatric patients with aqueduct stenosis who were managed by ventriculoperitoneal shunt were included. Both surgical procedures were compared. Statistically univariate analysis revealed that those patient with an age group of more than six months undergoing ventriculostomy had good outcome. Multivariate analysis revealed that past history of haemorrhage and/or meningitis were predictors of poor outcome. Sex, size of lumbar meningocele at birth, abnormal ventricular anatomy or narrow third ventricular floor size were non predictors of bad outcome in these patients. There was no difference in outcome in both the shunt or ventriculostomy group.

In contrast to shunt operation the indication for an endoscopic ventriculostomy in patients diagnosed for normal pressure hydrocephalus is not scientifically established. From September 1997 to March 1999 we operated on 36 patients diagnosed for normal pressure hydrocephalus. Diagnosis was established by means of the intrathecal lumbar or ventricular infusion test, the cerebrospinal fluid tap test and MRI-CSF flow studies pre- and post-operatively. In 30 patients (83%) we implanted a ventriculo-peritoneal shunt, and in 6 patients (17%) we performed the endoscopic assisted third ventriculostomy. With our created NPH recovery rate and use of the clinical grading for normal pressure hydrocephalus created by Kiefer and Steudel we compared the operative results of both patient groups. In patients with a pathologically increased resistance to CSF outflow in the lumbar infusion test a shunt implantation is indicated. Patients whose outflow resistance is increased in the ventricular infusion test but with a physiological lumbar infusion test are suspected for a functional aqueduct stenosis and should be treated by means of an endoscopic assisted ventriculostomy.

Genetic counselling in families with congenital hydrocephalus internus (CHI) in combination with aqueduct stenosis (AS) is often difficult due to an uncertain aetiology. We present a series of 35 patients with CHI and AS focusing on the aetiology and presumed recurrence risk for siblings. In 13 patients (37.1%) a genetic aetiology was identified with an increased recurrence risk for siblings. The relative frequency of patients with X-linked hydrocephalus in our sample was in accordance with the literature (2/35), but was more frequent in other diseases with Mendelian inheritance. Conclusion In addition to the well-known X-linked and autosomal recessive forms of aqueduct stenosis with hydrocephalus, this malformation can occur in other diseases with Mendelian inheritance. This finding is of considerable importance for genetic counselling and prognosis.

A kindred is reported on with suspected autosomal dominant congenital hydrocephalus and aqueduct stenosis. In contrast to patients with X-linked congenital hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS) our patients were not mentally retarded and they did not show any pyramidal tract dysfunction or clasped thumbs; the pyramids were not affected either, as was confirmed by autopsy, CT or MRI. Molecular genetic studies in our patients have not revealed abnormalities of eight exons of the L1 neural adhesion molecule gene that is related to HSAS.

In order to evaluate the usefulness of transendoscopic sonography, we studied two new sonographic probes of 6-F diameter in 15 fresh specimens. We saw precise imaging of well-known anatomical structures and an additional dimension in endoscopy, as the sonograph adds to the endoscopic view a transversal scan like, as if it were a mini CT at the tip of the probe. Thus, we also experienced the navigation characteristics of this imaging technique, both in real time and on-line. Some three-dimensional reconstructions of the foramen of Monro region are examined. First clinical use has been carried out, and adaptation of the equipment to neurosurgery is necessary.

In two adults, both schizophrenia and hydrocephalus were associated with aqueduct stenosis. The prevalence is argued to be above chance, and may contribute to the neurodevelopmental hypothesis of the causality of schizophrenia.

Various ways in which blockage of cerebrospinal fluid pathways can lead to increasing intracranial pressure and enlargement of the ventricles are discussed. The necessity of understanding the valvular morphology involved is stressed as fundamental to the search for optimal treatment methods.

We have operated on 47 patients, achieving significant improvement or total shunt independence in 80% with only one major complication. We have not restricted the procedure only to those with late onset aqueduct stenosis, but rather have included 29 patients with both early and late onset aqueduct stenosis, 8 patients with associated myelomeningocoele, 7 patients with neoplastic secondary aqueduct stenosis and 3 patients with previous central nervous system (CNS) infection or hemorrhage. We believe that a procedure which carries an appreciable failure rate is acceptable if the only alternative to improve patient selection is an invasive and time-consuming investigation such as a cerebrospinal fluid (CSF) infusion study. Such a study may or may not identify those patients in whom CSF absorptive capacity is inadequate. Furthermore, this study illustrates two important principles that may improve the overall outcome of third ventriculostomy. Firstly, comprehensive preoperative structural studies will help to eliminate some of those patients anatomically unsuitable for this procedure. Secondly, an adequate period should be allowed to pass before declaring the operation unsuccessful, as head growth did not show an improved trend for several weeks in two patients. We believe that in these patients, the CSF absorptive capacity improved over that time.