Endoscopic third ventriculostomy for hydrocephalus in children younger than 1?year of age

Objective  

The purpose of this study is to assess the role of endoscopic third ventriculostomy (ETV) in the treatment of hydrocephalus in children under 1 year of age. The authors analyzed data of ETV in their institution.     

Endoscopic third ventriculostomy for obstructive hydrocephalus in children younger than 6 months of age

Purpose  

The outcome of endoscopic third ventriculostomy (ETV) is worse in children younger than 2 years old and especially in infants, and controversies still exist whether ETV might be superior to shunt placement in this age group. We retrospectively analyzed the data of 23 patients younger than 6 months of age treated with ETV and assessed its feasibility as a first choice of treatment for hydrocephalus.     

Endoscopic third ventriculostomy: the best option in the treatment of persistent hydrocephalus after posterior cranial fossa tumour removal?

Background

Ten to 40% of children operated on for a posterior fossa tumour require a further surgical procedure for the management of a persisting active ventricular dilation. The management of this kind of hydrocephalus is still controversial.

Objective

To prospectively evaluate the effectiveness of post-operative endoscopic third ventriculostomy (ETV) in the management of persistent active hydrocephalus in a series of children operated on for a posterior cranial fossa tumour.

Methods

The management protocol consisted of: (1) placement of a peri-operative antibiotic impregnated external ventricular catheter (Bactiseal®) and tumour removal, (2) post-operative intracranial pressure (ICP) monitoring through the external ventricular drainage, (3) ETV in case of persistent ventricular dilation and persistently abnormal high ICP values and (4) ventriculoperitoneal shunt implantation in case of ETV failure.

Results

Thirty on a total of 104 children (28.8%) operated on between January 2001 and February 2007 at our institution needed a further surgical treatment for the persistence of the hydrocephalus after the removal of their posterior cranial fossa tumour. They were sub-divided in two groups according to the early (group 1—21 patients) or later (group 2—nine patients) definition of the persistence of an active ventricular dilation based on clinical, radiological and ICP monitoring data. ETV was successful in 90.0% of the patients in the present series (27/30 patients), without statistically significant differences among the two groups considered.

Conclusions

Post-operative ETV should be considered the best option to treat persistent hydrocephalus after the removal of posterior fossa tumours.

     

Treatment factors associated with outcomes in children less than 3 years of age with CNS tumours

Introduction  This study aimed to document cognitive outcomes in children treated for brain tumours with surgery and/or chemotherapy before the age of 3 years and to investigate the relationship between treatment factors and cognitive outcome. Materials and methods  Participants were 31 children aged 7–14 years who had been diagnosed and treated for brain tumours under the age of 3 years. Neuropsychological assessment included tests of cognitive functioning (WASI), memory (CMS) and executive functioning (BADS-C). Results and discussion  IQ and memory functioning scores were within the normal range but executive function was significantly below the expected level. Lower socio-economic status, younger age at treatment, having undergone more than one surgical intervention, motor problems and speech and language difficulties were found to be related to cognitive functioning. Although this group of children have good outcomes in terms of IQ and memory they have significant difficulties with executive functioning. Conclusion  Further research in this area is needed to allow for development of appropriate support packages for those who are most at risk.     

Split calvarial bone grafting in patients less than 1?year of age: technical note and use in craniofacial surgery for craniosynostosis

Purpose  

The material of choice for filling cranial defects is autologous split calvarial bone. Up to now, the thin calvarial bone and lack of diploic space in very young children has led surgeons to believe that harvesting of split calvarial grafts can generally not be done under the age of 2 years. We describe a simple technique for successful harvesting of split cranial vault bone in infants less than 1 year of age.     

Neurosurgical vascular malformations in children under 1 year of age

Purpose  

This study aims to analyze the clinical and radiological findings, timing and type of treatment, and outcome in children under 1 year of age that presented with neurosurgical vascular malformations.     

Does treatment with endoscopic third ventriculostomy result in less concern among parents of children with hydrocephalus?

Purpose  

A possible benefit of endoscopic third ventriculostomy (ETV) is that families might harbor less concern and anxiety compared to shunt. This has not yet been demonstrated, however. Our goal was to compare parental concern in a large sample of children with hydrocephalus treated with ETV or shunt, using our previously developed measure of parental concern, the Hydrocephalus Concerns Questionnaire for Parents (HCQ-P).     

Endoscopic third ventriculocisternostomy in hydrocephalic children under 2 years of age: appropriate or not? A single-center retrospective cohort study

Purpose

Treating hydrocephalus can be difficult in children under the age of 2 years because a high amount of uncertainty exists as to which treatment to perform. In this retrospective cohort study, we analyzed children under the age of 2 years with hydrocephalus undergoing an endoscopic third ventriculocisternostomy (ETV) with respect to ETV outcome.

Methods

In 59 consecutive patients under the age of 2 years, an ETV was performed between 1999 and 2010 at the Erasmus MC, Sophia Children’s Hospital. Demographics, etiology of hydrocephalus, and radiological data were extracted retrospectively from the patients’ medical records and operative reports and related to outcome. ETV Success Score (ETVSS) was used to retrospectively calculate the probability of success related to the actual outcome.

Results

In this series, 42.4 % of patients had a successful ETV. The only statistically significant finding concerned age. The failed ETV patients appeared to be younger (0.52?±?0.60 vs. 0.86?±?0.56 year, p?=?0.005), and when using a cutoff age of 6 months only, five out of 32 infants had a successful ETV (p?=?0.002). Of the children with an arachnoid cyst, 57.1 % were treated successfully with an ETV. Of the five patients with a high probability of ETV success, four (80 %) were indeed successfully treated with ETV (p?=?0.049).

Conclusions

Our data confirm the overall ineffectiveness of an ETV in children under the age of 6 months. Nevertheless, using the ETVSS is recommended to aid in the decision-making process even in patients under the age of 6 months.     

Does breastfeeding in the neonatal period influence the cognitive function of very-low-birth-weight infants at 5 years of age?

Aim: Breast milk is rich in docosahexaenoic acid (DHA), which is selectively concentrated in neuronal membranes and is thought to be necessary for optimal neurodevelopment. This study evaluated the relationship between breastfeeding, especially the resultant DHA level in the red blood cell (RBC) membranes of infants, and the cognitive function of very-low-birth-weight infants at 5 years of age. Methods: Eighteen patients were classified into groups that were breastfed or formula-fed or both. We measured the DHA concentration in the RBC membranes of 18 preterm infants at 4 weeks of age. To evaluate cognitive function at the age of 5 years, we asked the children to perform five tests: the Kaufman Assessment Battery for Children, Day–Night Test, Kansas Reflection Impulsivity Scale for Preschoolers (KRISP), Motor Planning Test, and Strengths and Difficulties Questionnaire. Results: The DHA level at 4 weeks after birth was significantly higher in the breastfed infants than in the formula-fed infants. The scores for the Day–Night Test, KRISP, and Motor Planning Test were significantly higher in the breastfed group. There were significant correlations between the scores for the Day–Night Test and for the KRISP and the level of DHA at 4 weeks of age. Conclusion: Breastfeeding in the neonatal periods increases the DHA level in preterm infants and may have an important influence on brain development not only during early infancy but also during the preschool years, especially in terms of cognitive function.     

Natural history of neurofibromatosis type 2 with onset before the age of 1 year

Neurofibromatosis type 2 (NF2) with onset before the first year of life has been anecdotally reported in the literature. We (a) prospectively (years 1997–2012) followed up three unrelated NF2 children, all harbouring NF2 gene mutations whose onset of disease was before age 1 year, and (b) systematically reviewed published reports on NF2 in the youngest age group (i.e. onset <1 year). The present three children had (1) small (<1 cm), bilateral vestibular schwannomas (VSs) detected (as an incidental finding) at magnetic resonance imaging (MRI) by the age of 4 to 5 months that were asymptomatic for 10 to 14 years, with sudden and rapid (<12 months) progression in two cases at the age of 11 and 15 years, respectively; (2) development of large numbers of skin NF2 plaques mainly in atypical locations (i.e. face, hands, legs and knees), which reverted to normal skin appearance at the time of VSs progression; (3) lens opacities (n?=?1) and NF2 retinal changes (n?=?2) detected as early as age of 3-4 months; (4) diffuse (asymptomatic) high signal lesions at brain MRI in the periventricular regions (alike cortical dysplasia); and (5) unaffected first-degree relatives who did not harbour NF2 gene abnormalities. This represents the youngest NF2 group with the longest prospective follow-up so far reported. NF2 may present as a congenital form with bilateral VSs presenting as early as the first months of life and with natural history different to that which occurs in classical NF2.     

Ventricular volume and neurocognitive outcome after endoscopic third ventriculostomy: is shunting a better option? A review

Background

Shunts are generally associated with a smaller post-treatment ventricular size in comparison to endoscopic third ventriculostomy (ETV).

Methods

To determine whether such a difference in ventricular size has neurocognitive implications, we reviewed the current literature pertaining to the (1) neurocognitive sequelae of hydrocephalus, (2) neurocognitive outcome after ETV, (3) extent of reversal of neurocognitive changes associated with hydrocephalus after shunting, and (4) data on correlation between post-treatment ventricular volume and neurocognitive outcome after ETV.

Results

Collectively, the results of the available studies should call into question the correlation between the residual postoperative ventricular volume and neurocognitive outcome.

Conclusion

The available literature is so far in support of ETV as a valid and effective treatment modality in hydrocephalic patients. No sufficient evidence is available to justify resorting to shunting on the premise that it is associated with a better neurocognitive outcome.

     

Pfropfschizophrenia in the age of deinstitutionalization: whose problem?

BACKGROUND: The Kraepelinian concept of pfropfschizophrenia refers to the intertwined coexistence of mental retardation and schizophrenia. Patients with this syndrome are relatively treatment resistant and are often harmed by diverging policies and cost cuts within the framework of mental health care services. Thus, a better understanding of this syndrome has important practical implications. The multiple problems that these patients encounter in various educational and health care agencies and what questions need to be asked to better elucidate these patients' needs are assessed. DATA: Three case reports of adult long-term inpatients fulfilling the criteria for pfropfschizophrenia are presented. Their current mental status and illness histories are discussed in relation to hypothesized pathophysiological processes and current needs. LIMITATIONS: Small cohort, naturalistic study. CONCLUSIONS: Pfropfschizophrenia is a phenotypically heterogeneous syndrome. The chronology of the appearance of cognitive deficits and psychotic symptoms during the course of this disorder should be carefully assessed because it may reflect diverse pathophysiological processes, necessitating differentiated, specific treatment interventions.     

Study of a group of extremely preterm infants (25-27 weeks): how do they function at 1 year of age?

The functional outcome of 49 extremely preterm infants (gestational age: 25-27 weeks) was studied at the corrected age of 12 months. Apart from pediatric follow-up, a full neurologic assessment and the Bayley Motor and Mental Scales of Infant Development was done. Emphasis was placed on postural control, spontaneous motility, hand function, and elicited infantile reactions. Special attention was given to symmetric development. The infants were then categorized as having optimal or nonoptimal or asymmetric outcome. Overall, an optimal outcome was found in 19 infants (39%) and nonoptimal outcome in 30 infants (61%), 7 of whom failed on all domains of function. Postural control had a significant influence on the different domains of development such as motility (P < or = .001) and persistent infantile reactions (P < or = .001) and slightly less on hand function (P = .08) and asymmetry (P = .06). The outcome on spontaneous motility was significantly related to the results on infantile reactions (P < or = 005) and hand function (P = .05). Also, the score on the motor scale of the Bayley Developmental test was clearly related to outcome on spontaneous motility (P < or = .001) and reactions (P< or = .02). Abnormal brain ultrasonograms were related to the asymmetry of the infantile reactions (P < or = .05). Poor coordination of gross motor function will have consequences for appropriate visuomotor and sensorimotor integration, thereby hampering motor learning and later cognitive function, as is often described in preterm infants. It is suggested that the poor postural control found in many infants born preterm is the result of both myogenic and neurogenic deviations caused by the preterm birth and its nursing consequences.     

Does age at the onset of narcolepsy influence the course and severity of the disease?

ObjectiveThe aim of the study is to compare the course and severity of narcolepsy in relation to different ages at the disease onset.MethodsClinical interviews with the completion of the Stanford questionnaire, Epworth Sleepiness Scale (ESS) and Multiple Sleep Latency Test (MSLT) were evaluated in 105 patients (44 males, 61 females, mean age 45.4 ± 19.2, BMI 29.2 ± 5.8) suffering from narcolepsy.ResultsThe severity of the disease was judged by clinical complaints, ESS value and MSLT results. No relations with the age at onset and clinical tetrad were found, however, smoking may be associated with an increased risk of hypnagogic hallucinations. There was no correlation between the number of sleep and cataplectic attacks and the age at onset, nor did subjective ESS show any significant dependence. However, earlier onset of the disease correlated with shorter MSLT mean latency. A correlation was found between the BMI and narcolepsy sleepiness rating in the elderly and between degree of education attained and subjective complaints.ConclusionsThe clinical severity of narcolepsy does not depend on the age at onset.