Posterior fossa tumours in childhood: Associated speech and language disorders post-surgery

Six children aged between 6 and 16 years who had undergone surgery for the removal of a posterior fossa tumour were assessed at least one year postoperatively to determine the incidence and severity of any associated speech or language deficits. Five males and one female were included in the sample. The subjects were administered a battery of speech/language assessments including: a language screening test, an articulation test, a dysarthria assessment and a perceptual speech analysis. The results indicated that dysarthria and/or language impairment occurs in some cases subsequent to surgical removal of posterior fossa tumours. The occurrence of muteness immediately post-surgery would appear to indicate a poor prognosis for speech abilities. A possible link between the occurrence of long term language disabilities in these children and post-surgical radiotherapy is documented

Benign external hydrocephalus: a review, with emphasis on management

Benign external hydrocephalus in infants, characterized by macrocephaly and typical neuroimaging findings, is considered as a self-limiting condition and is therefore rarely treated. This review concerns all aspects of this condition: etiology, neuroimaging, symptoms and clinical findings, treatment, and outcome, with emphasis on management. The review is based on a systematic search in the Pubmed and Web of Science databases. The search covered various forms of hydrocephalus, extracerebral fluid, and macrocephaly. Studies reporting small children with idiopathic external hydrocephalus were included, mostly focusing on the studies reporting a long-term outcome. A total of 147 studies are included, the majority however with a limited methodological quality. Several theories regarding pathophysiology and various symptoms, signs, and clinical findings underscore the heterogeneity of the condition. Neuroimaging is important in the differentiation between external hydrocephalus and similar conditions. A transient delay of psychomotor development is commonly seen during childhood. A long-term outcome is scarcely reported, and the results are varying. Although most children with external hydrocephalus seem to do well both initially and in the long term, a substantial number of patients show temporary or permanent psychomotor delay. To verify that this truly is a benign condition, we suggest that future research on external hydrocephalus should focus on the long-term effects of surgical treatment as opposed to conservative management

Venous hemodynamics in neurological disorders: an analytical review with hydrodynamic analysis.

Venous abnormalities contribute to the pathophysiology of several neurological conditions. This paper reviews the literature regarding venous abnormalities in multiple sclerosis (MS), leukoaraiosis, and normal-pressure hydrocephalus (NPH). The review is supplemented with hydrodynamic analysis to assess the effects on cerebrospinal fluid (CSF) dynamics and cerebral blood flow (CBF) of venous hypertension in general, and chronic cerebrospinal venous insufficiency (CCSVI) in particular.CCSVI-like venous anomalies seem unlikely to account for reduced CBF in patients with MS, thus other mechanisms must be at work, which increase the hydraulic resistance of the cerebral vascular bed in MS. Similarly, hydrodynamic changes appear to be responsible for reduced CBF in leukoaraiosis. The hydrodynamic properties of the periventricular veins make these vessels particularly vulnerable to ischemia and plaque formation.Venous hypertension in the dural sinuses can alter intracranial compliance. Consequently, venous hypertension may change the CSF dynamics, affecting the intracranial windkessel mechanism. MS and NPH appear to share some similar characteristics, with both conditions exhibiting increased CSF pulsatility in the aqueduct of Sylvius.CCSVI appears to be a real phenomenon associated with MS, which causes venous hypertension in the dural sinuses. However, the role of CCSVI in the pathophysiology of MS remains unclear

Pathogenesis of hydrocephalus in achondroplastic dwarfs: a review and presentation of a case followed for 22 years

© 2019, Springer-Verlag GmbH Germany, part of Springer Nature. Object: The purpose of this work is to review the pathogenesis and pathophysiology of hydrocephalus in patients with achondroplasia as a guide to its management throughout life. Methods: A review of the literature related to neurosurgical issues in achondroplasia with specific focus on cerebrospinal fluid physics, clinical management, and outcome of affected individuals. Issues involved in this review are highlighted by a case report of a patient shunted for achondroplasia first shunted in infancy and followed for 22 years. Each of the management issues is explored with respect to this patient. Findings: Head circumferences in achondroplasia are abnormally large in this condition usually caused by excess cerebrospinal fluid in the cortical subarachnoid space. Increase in ventricular size (hydrocephalus) is not rare but should not be treated unless rapidly progressive or symptomatic. The underlying cause of the abnormalities of cerebrospinal fluid dynamics relates to abnormal venous drainage at the skull base. Patients shunted in infancy for hydrocephalus usually remain dependent on the shunt for life, and crises of high intracranial pressure may occur with no distention of the ventricles. Conclusions: In infants with achondroplasia, large heads and enlarged ventricles without symptoms should be watched initially for progression. If hydrocephalus progresses or if symptoms of intracranial hypertension occur, endoscopic third ventriculostomy can be tried. If shunt is necessary, it should have a high opening pressure and a device to retard siphoning. In the case of recurrent ventricular catheter blockage, it may be necessary to create a communication between the ventricles and the cortical subarachnoid space

Hydrocephalus in infants: the unique biomechanics and why they matter

© 2020, Springer-Verlag GmbH Germany, part of Springer Nature. Object: Hydrocephalus diagnosed prenatally or in infancy differs substantially from hydrocephalus that develops later in life. The purpose of this review is to explore hydrocephalus that begins before skull closure and full development of the brain. Understanding the unique biomechanics of hydrocephalus beginning very early in life is essential to explain two poorly understood and controversial issues. The first is why is endoscopic third ventriculostomy (ETV) less likely to be successful in premature babies and in infants? The second relates to shunt failure in a subset of older patients treated in infancy leading to life-threatening intracranial pressure without increase in ventricular volume. Methods: The review will utilize engineering concepts related to ventricular volume regulation to explain the unique nature of hydrocephalus developing in the fetus and infant. Based on these concepts, their application to the treatment of complex issues of hydrocephalus management, and a review of the literature, it is possible to assess treatment strategies specific to the infant or former infant with hydrocephalus-related issues throughout life. Results: Based on engineering, all hydrocephalus, except in choroid plexus tumors or hyperplasia, relates to restriction of the flow of cerebrospinal fluid (CSF). Hydrocephalus develops when there is a pressure difference from the ventricles and a space exterior to the brain. When the intracranial volume is fixed due to a mature skull, that difference is between the ventricle and the cortical subarachnoid space. Due to the distensibility of the skull, hydrocephalus in infants may develop due to failure of the terminal absorption of CSF. The discussion of specific surgical treatments based on biomechanical concepts discussed here has not been specifically validated by prospective trials. The rare nature of the issues discussed and the need to follow the patients for decades make this quite difficult. A prospective registry would be helpful in the validation of surgical recommendations. Conclusion: The time of first intervention for treatment of hydrocephalus is an important part of the history. Treatment strategies should be based on the assessment of the roll of trans-mantle pressure differences in deciding treatment strategies. Following skull closure distension of the ventricles at the time of shunt failure requires a pressure differential between the ventricles and the cortical subarachnoid space