Flow ventricular catheters for shunted hydrocephalus: initial clinical results.

BACKGROUND: The non-homogenous flow of the cerebrospinal fluid within the ventricular catheter is one of the causative factors in shunt obstructions during the treatment of hydrocephalus. Previously, we studied the flow in ventricular catheters under the steady and pulsatile boundary conditions by means of computational fluid dynamics (CFD) in three-dimensional paradigms. Subsequently, several catheter designs with homogeneous flow patterns were developed out of which one prototype was chosen after a validation study. OBJECTIVE: To test the effectiveness of the flow ventricular catheter in a prospective, multicenter, comparative study. METHODS: Eligible centers were three pediatric hospitals: two with sole adult practice and one a mixed pediatric-adult. Standard silicone material was used to develop a parametric catheter model with homogenous flow characteristics. The flow catheters were inserted in pediatric (n = 30) and adult (n = 10) patients with all types of hydrocephalus. Simultaneously, regular ventricular catheters were inserted in another 43 control patients in the participating centers. Catheter positioning was standardized according to the Schaumann and Thomale classification. RESULTS: All ventricular catheters had a cephalad grade I or II positioning, and caudally, its extension had a peritoneal location. Programmable valves were utilized in 70% and antisiphon devices in 20% of the cases. Regular differential pressure valves were utilized in the remaining. No case of flow catheter obstruction was identified during a mean follow-up period of 2 years at the time of this writing. There were four catheter obstructions in the control cohort, all pediatric cases, during the first year. Shunt infections occurred in two cases in the control group, while there was one recurrent case of adult ventriculitis in the flow catheter group. CONCLUSIONS: This prototype model represents the next generation of ventricular catheters with a homogeneous flow pattern. The flow catheter can be inserted safely in hydrocephalic patients, and this preliminary prospective comparative study showed a possible obstruction-free functionality.

Pediatric head injuries from earthquakes.

By means of some illustrations, the authors briefly report the effects of some accidental head injuries caused by diverse mechanisms occurring in children. Many of these accidents seem to be preventable, but others are completely unavoidable and escape prevention as the one that is depicted in the cover of this issue.

Pediatric crushing head injury: biomechanics and clinical features of an uncommon type of craniocerebral trauma.

BACKGROUND: Head injuries constitute one of the leading causes of pediatric morbidity and mortality. Most injuries result from accidents involving an acceleration/deceleration mechanism. However, a special type of head injury occurs when the children sustain a traumatism whose main component is a static load in relation to a crushing mechanism with the head relatively immobile. PATIENTS AND METHODS: We report a series of children who sustained a craniocerebral injury of variable severity produced by head crushing. We also analyze epidemiological and clinical data, and biomechanics in these injuries. RESULTS: Mean age of the group (13 boys/6 girls) was 4.1 years. All patients showed external lesions (scalp wounds or hemorrhage from the nose, ears, or throat). Eleven children were initially unconscious. Six children presented cranial nerve deficits in addition to impaired hearing. Skull base fractures were seen in most cases with extension to the vault in 11 instances. Fourteen patients had an associated intracranial lesion, including two with diffuse axonal injury. Surgery was performed in three instances. Only seven patients were left with sequelae. DISCUSSION AND CONCLUSIONS: The observed skull, brain, and cranial nerve lesions corresponded to a mechanism of bilateral compression of the children's heads mainly occasioned by a static load, although an associated component of dynamic forces was also involved. The skull and its covering and the cranial nerves were the most severely affected structures while the brain seemed to be relatively well preserved. Most crush injuries appear to be preventable by the appropriate supervision of the children.

Hydrocephalus and arachnoid cysts.

AIM: The management of arachnoid cysts (AC) remains controversial. An additional problem derives from the management of hydrocephalus associated with an AC. In this work, we discuss existing procedures proposed in the current literature for their treatment. METHODS: We reviewed selected reports on intracranial ACs placing special interest in those about the association of hydrocephalus and ACs. We also briefly surveyed data of our patients with this association. RESULTS AND DISCUSSION: Hydrocephalus is often found in midline and posterior fossa ACs. Interhemispheric lesions may also evolve with ventriculomegaly, while middle fossa lesions rarely produce hydrocephalus. Patients' age, cyst location and size, and macrocephaly have all been related to the development of hydrocephalus. Some authors remark on the role played by hydrocephalus and hypothesize that some ACs would result from disturbed cerebrospinal fluid (CSF) dynamics. They also propose that ACs might represent a localized form of hydrocephalus. We also comment on hydrocephalus in relation to the diverse locations of ACs. Neuroendoscopic techniques have transformed previous ways of management as cystoperitoneal shunting and open fenestration. CONCLUSIONS: ACs may be pathogenetically related with hydrocephalus, and conversely, ACs may cause hydrocephalus. In some patients, aberrant CSF dynamics seems to play a major role in the development of both cyst and hydrocephalus. Hydrocephalus and ACs may be treated exclusively with neuroendoscopic procedures, although some patients will still require CSF shunting. The ideal option seems to consist of choosing the method that offers the highest success with a single procedure for treating the hydrocephalus and the AC simultaneously.

Craniosynostosis in Kabuki syndrome.

Niikawa-Kuroki, or Kabuki syndrome (KS), is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, short stature, and mental retardation. Neurological manifestations and CNS anomalies have been described in some patients with this condition. However, craniosynostosis has been documented in only 4 patients with KS who did not undergo operations. The authors report a case of KS with unicoronal synostosis that constitutes the first documented instance of a patient with this syndrome submitted to surgery. Previous reported instances of craniosynostosis occurring in KS are briefly reviewed. Although rarely documented, craniosynostosis might represent a relatively frequent feature of this syndrome. Kabuki syndrome should be considered at the time of evaluating children with craniosynostosis. The diagnosis of KS can be suspected from the patients' characteristic facial features. Kabuki syndrome appears to be an underdiagnosed condition in the craniosynostosis population. Given that most patients with this syndrome suffer from only mild to moderate mental retardation, surgical correction can be considered in instances of KS with craniosynostosis.

Bilateral ethmoidal dural arteriovenous fistula: unexpected surgical diagnosis.

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies.

Posterior fossa arachnoid cysts and cerebellar tonsillar descent: short review.

The objective of this study was to analyze the association of cerebellar tonsillar descent and syringomyelia in patients with posterior fossa arachnoid cysts. We reviewed the medical records of ten patients (mean, age 33; range, 24-49 years) diagnosed with posterior fossa arachnoid cyst and tonsillar descent. Symptoms evolved over a mean of 12 months (range, 6 months to 3 years). Syringomyelia was present in six cases. Six patients underwent a suboccipital craniectomy, three cases underwent an additional C1 laminectomy, and a further case had a limited craniectomy and tonsillar reduction. Three patients were also treated for hydrocephalus: one with a ventriculoperitoneal shunt and two with endoscopic third ventriculostomy. Two patients had conservative treatment. The posterior fossa arachnoid cysts were located at the vermis-cisterna magna (n = 4), the cerebellar hemispheres (n = 2), the cerebellopontine angle (n = 3), and the quadrigeminal cistern (n = 1). A patient with achondroplasia showed features of platybasia. Associated malformations included craniofacial dysmorphism in a patient diagnosed of trichorhinophalangeal syndrome and a case with a primary temporal arachnoid cyst. After a mean follow-up of 2 years (range, 3 months to 5 years), four patients showed resolution of their neurological symptoms, and two exhibited persisting ocular findings. Headaches and nuchalgia improved in four cases and persisted in four. Syringomyelia was resolved in four patients and improved in two. Patients harboring a posterior fossa arachnoid cyst may evolve with acquired Chiari malformation and syringomyelia. Initial management should be directed to decompressing the foramen magnum and should include the resection of the arachnoid cyst's walls. A wait-and-see attitude can be implemented in selected cases. In our experience, hydrocephalus should be properly addressed before treating the arachnoid cyst.

Massive hemorrhage in hemangioblastomas Literature review.

Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal-radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.

Cervical extramedullary mass lesion due to chronic CSF overshunting: case report and literature review.

INTRODUCTION: Several emerging clinical and neuroimaging conditions due to CSF intracranial hypotension have been documented. Recently, a few instances of a severe form of cervical myelopathy due to CSF overshunting have also been reported. Patients with this type of cervical myelopathy usually evolve with marked clinical manifestations of spinal cord involvement. MATERIALS AND METHODS: In this article, we report a 20-year-old girl, previously given a ventriculoperitoneal shunt at age 6 years, who presented with neck pain after a whiplash injury. RESULTS AND DISCUSSION: Magnetic resonance imaging revealed a cervical extramedullary mass that mimicked a cervical extradural hematoma. Further neuroimaging studies demonstrated that it corresponded to an engorged cervical epidural venous plexus that we attributed to chronic CSF overdrainage. After expectant management, the patient experienced a total recovery of her symptoms. To our knowledge, this is the first report of this condition occurring in an asymptomatic patient. Our aim in reporting this patient is twofold: (1) to warn the clinician about these neuroimaging findings that may mimic a cervical extradural hematoma and (2) to illustrate that engorged cervical epidural plexus may occur in patients in the absence of clinical manifestations of myelopathy. Recognition of this entity is important to distinguish it from a mass lesion in which a surgical intervention might be indicated.