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A trapped fourth ventricle is a clinic-radiological entity characterised by progressive neurological symptoms due to an enlargement of the fourth ventricle secondary to obstruction to its outflow. This condition is most commonly observed in ex-preterm patients shunted for a post-haemorrhagic or post-infective hydrocephalus. Until the introduction of endoscopic aqueductoplasty and stent placement, through a supratentorial or an infratentorial approach, treatment of trapped fourth ventricle entailed high rates of complications, repeated procedures and consequent morbidity. We describe the first case of successful treatment of trapped fourth ventricle by fenestration of superior medullary velum through an infratentorial approach in a 20-month-old child with a functional supratentorial ventriculoperitoneal shunt and an aqueductal anatomy not favourable for stenting. To the best of our knowledge, this is the first reported case of utilisation of this technique in a patient with a trapped fourth ventricle, and we wish to highlight this new alternative approach in cases where conventional aqueductoplasty and stenting may not be feasible., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Agenesis of the internal carotid artery (aICA) is a rare congenital vascular condition that can affect one or both sides of the patient. Most patients remain asymptomatic, but ischemic/hemorrhagic stroke, intracranial aneurysm, and other neurologic findings can occur. CT scan can demonstrate the absence of the bony carotid canal and helps to differentiate a complete aICA from aplasia or hypoplasia. The association of aICA and aqueductal stenosis (AS) has never been reported in the literature. We report the case of a 9-year-old with agenesis of the right ICA associated with AS and hydrocephalus, which was treated successfully with an endoscopic third ventriculostomy (ETV). We review the literature looking for the association of the clinical findings and the evolution of the patient., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: An extensive literature has postulated multiple etiologies for aqueductal stenosis. No publications were found, discussing that evolutionary modifications might explain aqueductal anomalies. This study's objectives were to review the evolutionary modifications of vertebrates' tectum structures that might explain human aqueduct anomalies. Undertaking vertebrate comparative study is currently not feasible in view of limitations in obtaining vertebrate material. Thus, vertebrate material collected, injected, dissected, and radiographed in the early 1970s was analyzed, focusing on the aqueduct and components of the midbrain tectum., Methods: Photographs of brain dissections and radiographs of the cerebral ventricles and arteries of adult shark, frog, iguana, rabbit, cat, dog, and primate specimens, containing a barium-gelatin radiopaque compound, were analyzed focusing on the aqueduct, the optic ventricles, the quadrigeminal plate, and collicular ventricles. The anatomic information provided by the dissections and radiographs is not reproducible by any other radiopaque contrast currently available., Results: Dissected and radiographed cerebral ventricular and arterial systems of the vertebrates demonstrated midbrain tectum changes, including relative size modifications of the mammalian components of the tectum, simultaneously with the enlargement of the occipital lobe. There is a transformation of pre-mammalian optic ventricles to what appear to be collicular ventricles in mammals, as the aqueduct and collicular ventricle form a continuous cavity., Conclusions: The mammalian tectum undergoes an evolutionary cephalization process consisting of relative size changes of the midbrain tectum structures. This is associated with enlargement of the occipital lobe, as part of overall neocortical expansion. Potentially, aqueductal anomalies could be explained by evolutionary modifications., (© 2021. The Author(s).)

Background: Endoscopic aqueductoplasty with aqueductal stenting is an effective surgical procedure for the treatment of isolated fourth ventricle (IFV). Due to the rarity of the underlying pathology, it can be considered a rare procedure that can be performed with different surgical techniques and approaches., Objectives: To assess long-term functioning of permanent aqueductal stents implanted in children affected by hydrocephalus and IFV and to describe some variations of the same procedure., Methods: We reviewed retrospectively all the patients presenting at our institution in the years 1999-2019 for symptoms of isolated fourth ventricle who underwent a surgical procedure of endoscopic aqueductoplasty and/or aqueductal stent. Surgical reports, radiological images, and surgical videos were retrospectively analyzed., Results: Thirty-three patients with symptomatic isolated fourth ventricle (IFV) underwent fifty (50) neuroendoscopic procedures in the period observed. The median age of the patients at the time of first surgery was 7 months, with 22 premature babies. In twenty-nine patients (87.8%), a precoronal approach was performed, while four patients received a suboccipital burr hole. Ten patients were never reoperated since. Twenty-three patients underwent further surgeries: a new aqueductoplasty with aqueductal stent was performed in 13 cases. The remaining 10 patients required a combination of other procedures for management of hydrocephalus. Long-term follow-up showed a permanent stent functioning rate of 87% at 2 years and 73% at 4 years, remaining stable afterwards at very long term (20 years)., Conclusion: Endoscopic aqueductoplasty and stenting is a reliable procedure in the long-term management of isolated fourth ventricle.

Developmental venous anomalies (DVAs), previously also known as venous angiomas, are variations of normal trans-medullary veins draining from white and gray matter. DVAs are usually asymptomatic and mostly discovered incidentally on brain imaging. However, some studies have reported symptomatic cases associated with DVAs. In this report, we report an extremely rare case of a 14-month-old boy with obstructive hydrocephalus following aqueductal stenosis caused by developmental venous anomalies. At the age of 14 months, his head circumference exceeded + 2SD significantly. Brain magnetic resonance imaging (MRI) showed triventriculomegaly and dilated collector vein coursing through the Sylvian aqueduct, causing aqueductal stenosis. Endoscopic third ventriculostomy (ETV) was successfully performed. During the procedure, a dilated collector vein was confirmed obstructing the Sylvian aqueduct. Postoperative cine MRI showed good flow signal through the opening and improvement of hydrocephalus was noted. Obstructive hydrocephalus following aqueductal stenosis caused by DVAs is very rare; nonetheless, it can be considered as a causal differential diagnosis for hydrocephalus. Whether ETV should be chosen, as the technique for diversion of cerebrospinal fluid (CSF) flow, remains controversial. This case report showed that ETV was effective and safe.

Introduction: Post-traumatic hydrocephalus following head injury is a well-known entity. Most cases occur in patients with severe head injuries, often following decompressive craniectomy. On the contrary, acute post-traumatic hydrocephalus, caused by aqueductal obstruction by a blood clot, following mild head injury is uncommon., Clinical Material: Six patients aged between 6 and 15 months presented hydrocephalus secondary to a blood clot in the aqueduct. Because of intracranial hypertension at presentation, 4 patients were urgently treated with external ventricular drains (EVDs). Post-operative course was uneventful. In 2 cases, EVDs were removed without further treatments. In 2 cases, hydrocephalus recurred. These patients were successfully treated with endoscopic third ventriculostomy. The remaining two patients developed symptoms a few days after the trauma. One, that presented hydrocephalus at imaging, was managed with a ventriculo-peritoneal shunt; the other, that presented subdural hygroma, was managed with subduro-peritoneal shunt that was removed later. All patients had complete recovery., Discussion and Conclusion: Hydrocephalus secondary to clot in the aqueduct may rarely be the result of mild head injury in young children. Usually, prompt surgical management warrants a very good outcome. Most children may be treated without a permanent shunt, by using external drains and endoscopic third ventriculostomy.

Introduction: Recognizing patients with ventriculomegaly who are at risk of developing acute hydrocephalus presents a challenge for the clinician. The association between disturbed cerebrospinal fluid flow (CSF) and impaired brain compliance may play a role in the pathogenesis of hydrocephalus. Phase contrast MRI is a noninvasive technique which can be used to assess CSF parameters. The aim of the work is to evaluate the effectiveness of phase contrast MRI in recognizing patients at risk of acute hydrocephalus, based on measuring the pulsatile CSF flow parameters in the Sylvian aqueduct and prepontine cistern in children with ventriculomegaly., Aim: The aim of the work is to characterize the parameters of cerebrospinal fluid (CSF) flow in the Sylvian aqueduct and prepontine cistern in children with ventriculomegaly with regard to patient age and symptoms. We hypothesize that the relationship between CSF flow parameters in these two regions will vary according to analyzed factors and it will allow to recognize children at risk of hydrocephalus., Materials and Methods: A group of 26 children with ventriculomegaly (five girls and 21 boys) underwent phase contrast MRI examinations (Philips 3T Achieva with Q-flow integral application). Amplitudes of average and peak velocities of the CSF flow through the Sylvian aqueduct and prepontine cistern were used to calculate ratios of oscillation and peak velocities, respectively. The relationship between the oscillation coefficient, the peak velocity coefficient, and stroke volume was then assessed in accordance with age and clinical symptoms., Results: The peak velocity coefficient was significantly higher in patients with hyper-oscillating flow through the Sylvian aqueduct (3.04 ± 3.37 vs. 0.54 ± 0.28; p = 0.0094). Moreover, these patients tended to develop symptoms more often (p = 0.0612). No significant age-related changes were observed in CSF flow parameters., Conclusion: Phase contrast MRI is a useful tool for noninvasive assessment of CSF flow parameters. The application of coefficients instead of direct values seems to better represent hemodynamic conditions in the ventricular system. However, further studies are required to evaluate their clinical significance and normal limits.

Introduction: The cyst-peritoneal shunt is a recognised surgical alternative in the management of sylvian fissure arachnoid cysts. Shunt overdrainage is well described in literature on ventriculo-peritoneal shunts, but not often appreciated as a complication of cysto-peritoneal shunts., Case Report: A 5-year-old boy presented with a symptomatic left sylvian fissure arachnoid cyst. This was initially treated by craniotomy and membrane fenestration in the carotid cistern. Recurrence led to insertion of a valveless cyst-peritoneal shunt 5 months later. Initial progress was followed by persistent headaches 18 months after shunt insertion. CT scan revealed a significant reduction in the cyst size, enlargement of the ipsilateral lateral ventricle, collapse of the contra-lateral ventricle and midline shift towards the side of the shunt. These findings were interpreted as over-drainage of the cyst-peritoneal shunt., Result: A Codman Medos adjustable valve was inserted, with the intention of gradually increasing the pressure until the midline shift was restored and the contra-lateral ventricle was reconstituted. This was achieved with the valve set at 90 mm H(2)O, verified by CT scan. Radiological improvement was associated with dramatic symptomatic improvement., Conclusion: Over-drainage of cyst-peritoneal shunts is often not appreciated, especially when the main manifestation is headaches. As it is difficult to predict the required valve pressure setting, it may be advisable to consider the use of an adjustable valve.

Ventriculoperitoneal (VP) shunt placement in patients with aqueductal stenosis has recently been reported as a cause of pneumocephalus. We report on a patient with pneumocephalus associated with aqueductal stenosis treated by VP shunting. A 29-year-old woman who had undergone a shunt operation for aqueductal stenosis 7 years previously sustained a whiplash injury in a minor traffic accident. Computed tomography (CT) revealed massive subdural pneumocephalus, and three-dimensional reconstructions of CT images clearly demonstrated defects in the skull base overlying the ethmoid sinuses. Both endoscopic III ventriculostomy and placement of external ventricular drainage were came free of symptoms and rhinorrhea ceased. Three-dimensionally reconstructed CT images were useful in detecting the extent of the patient's skull base defect. III Ventriculostomy was not effective in this case. Direct closure of the skull base by craniotomy was not necessary, and a programmable valve system was effective in preventing recurrence of either pneumocephalus or rhinorrhea.

We report the case histories of three sisters with congenital hydrocephalus associated with stenosis of the cerebral aqueduct. The parents were a young consanguineous couple. In two cases hydrocephalus was detected before birth by ultrasonography. We consider these three cases to be of the rare autosomal form of hereditary hydrocephalus.