Nontumoral Hydrocephalus Research Articles

Pituitary enlargement in patients with cerebrospinal fluid drainage due to ventricular shunt insertion: know the condition and do not mistake for adenoma

ObjectiveChildhood hydrocephalus patients treated by ventriculo-peritoneal (v.-p.) shunting are sometimes referred years after this therapy for evaluation of suspicious pituitary enlargement. Since pituitary size has been shown to depend on cerebrospinal fluid (CSF) pressure, we assume this phenomenon to be caused by shunt overdrainage. Therefore, we studied pituitary size and morphology in shunted hydrocephalus patients with radiological signs of high CSF drainage.Patients and methodsRetrospective study of pituitary size and morphology in 15 shunted patients with non-tumoral hydrocephalus and 7 shunted hydrocephalus patients due to childhood brain tumor compared to a population mean. In five brain tumor patients also pre- and postsurgical comparisons were performed.ResultsPituitary mid-sagittal size and pituitary volume were significantly higher in both hydrocephalus groups, compared to the population mean (midsagittal size t = 5.91; p < 0.001; pituitary volume, t = 3.03; p = 0.006). In patients available for pre- and postoperative comparison, there was also a significant increase in pituitary size and volume postoperatively (mean preoperative midsagittal height 2.54 ± 1.0 mm vs. 6.6 ± 0.7 mm post-surgery; mean pre-operative pituitary volume 120.5 ± 69.2 mm3 vs. 368.9 ± 57.9 mm3 post-surgery).ConclusionOur results confirmed a significant increase in pituitary size and volume, mimicking pituitary pathology, after v.-p. shunt insertion. This phenomenon can be explained by the Monro–Kellie doctrine, stating that intracranial depletion of CSF—as caused by v.p. shunting—leads to compensatory intracranial hyperemia, especially in the venous system, with the consequence of engorged venous sinuses, most likely responsible for enlargement of the pituitary gland.

Study of Neurodevelopmental Outcome in Patients with Non-tumoral Hydrocephalus with Shunt Surgery Done in Infancy.

ABSTRACTBackground:Hydrocephalus (HC) is a common neurological disorder presenting in infancy, with a myriad of etiologies requiring early neurosurgical intervention.Objective:To study neurodevelopmental outcome in patients with HC with shunt surgery done in infancy.Materials and Methods:This was an observational retrospective cohort study of 50 pediatric patients (2 years to 16 years of age). These patients were diagnosed with HC and were operated on with ventriculo-peritoneal shunt (VP shunt) insertion in infancy (did not include patients with brain tumors) and then later following in the neurology outpatient department (OPD). Clinical records and neurodevelopmental assessment (intelligence quotient [IQ]/development quotient [DQ] and vision and hearing assessment) were reviewed.Results:Only 50% of the patients with congenital HC were diagnosed at birth, which included patients who had been diagnosed antenatally and they had lesser complications and better intellectual outcome (P = 0.12), compared with those who presented later with HC. Patient-related factors such as etiology of HC, antenatal diagnosis, and requirement of shunt revisions had poor correlation with neurodevelopmental outcome. Patients with late postoperative complications had significantly poor neurodevelopmental outcome (P ≤ 0.001). Patients with post-meningitis HC required a significantly higher number of shunt revisions than patients with other causes (P = 0.04).Conclusion:Better neurodevelopmental outcome depends on early diagnosis and early referral for the management than the cause of HC. Regular head circumference monitoring is the most feasible and sensitive screening tool for early pickup. Larger studies are needed for accurate prognostication.

Multiloculated hydrocephalus

The study aims to assess the treatment of progressive multiloculated hydrocephalus. In a retrospective study, the authors reviewed their experience with different treatment modalities. We have retrospectively evaluated 93 patients with progressive multiloculated hydrocephalus operated between 1988 and 2010. They represented around 2% (93/4,565) of all patients surgically treated for nontumoral hydrocephalus during this period of time at our institution. Ventricular septal fenestration was carried out by craniotomy in 27 patients, endoscopic septum pellucidum fenestration in 19, endoscopic ventricular septal fenestration in 18, choroid plexectomy-fulguration in 14 (8 endoscopically and 6 by craniotomy), and third ventriculostomy in 2. Hydrocephalus was resolved in 21 patients with shunting, placing two ventricular catheters as the only procedure. Out of the 72 remaining patients, 34 underwent only one treatment, 30 two treatments, and 8 three or more procedures. The majority of patients ultimately required CSF shunt placement with only one ventricular catheter. (1) Multiloculated hydrocephalus is a severe disease in which no single treatment has clearly been shown to be superior. (2) The goal of treatment is to restore communication between isolated intraventricular compartments in order to create the possibility of the implantation of a simple shunt with only one intraventricular catheter. More than improving the quality of life the patient, the objective is to reduce the number of surgical procedures. (3) Given the complexity of multiloculated hydrocephalus, each patient must be studied individually, and no procedure proposed by the literature should be ruled out, no matter how old fashioned may appear.

Hydrocephalus in toddlers: the place of shunts in sub-Sahara African countries

This study describes the epidemiological patterns of hydrocephalus in toddlers in our setting in order to determine the proportion of those who could benefit from endoscopic third ventriculostomy (ETV). This prospective and descriptive study included all toddlers operated on for hydrocephalus from 1 March 2008 to 31 March 2010 at the Yaounde Central Hospital. Forty-six toddlers were included representing 72% of all hydrocephalus cases managed at the Neurosurgery Unit during the study period. The mean age was 6.9 ± 1.6months. The delay before treatment varied from 5days to 15.8months (mean = 3.7 ± 0.5months). The commonest clinical presentation was macrocrania (78.3%). Of the toddlers, 58.7% presented with a probable blindness (loss of ocular pursuit); dilated and non-reactive pupils were found in nine patients (19.6%). The diagnosis was based on transfontanellar echography (TFE), CT scan or combined TFE and CT scan. Identified aetiologies were aqueduct stenosis (28.7%), haemorrhage (18%), Dandy-Walker's syndrome (14.3%), meningitis (10.8%), myelomeningocele (10.8%), agenesis of Monro's foramen (3.6%), brain abscess (3.2%) and posterior fossa tumour (3.6%). No specific cause was found in 7% of cases. The treatment was ventriculoperitoneal shunting in 42 cases (91.3%) and ETV in two cases (4.3%). Infections (11.1%) and shunts' obstruction (5.4%) were the main complications. Cases of hydrocephalus in toddlers are frequent in our setting. Regardless of the patient's age, the most prevailing aetiologies (infections, haemorrhage, myelomeningocele) and technological conditions (neuroendoscope) are less favourable for ETV. The use of ETV in the treatment of hydrocephalus in sub-Saharan Africa is still marginal and needs to be encouraged in selected cases. The prevention of non-tumoral hydrocephalus is of critical importance.

Etiology and cranial CT scan profile of nontumoral hydrocephalus in a tertiary black African hospital

Hydrocephalus is a common condition in the pediatric population. The cause of hydrocephalus, Evans ratio, ventricular index, and cerebral mantle thickness are some of the factors associated with poor surgical outcome. This study was conducted to evaluate the profile of these factors in the authors' patient population. The authors conducted a prospective study from the August 1, 2006, to May 30, 2010. The consecutive patients were all 6 years of age or younger. The demographic information, cause of hydrocephalus, and cranial computerized measurements were taken (including widths of the frontal/occipital horns, third ventricle, and cerebral mantle thickness) and entered into the procedural forms. One hundred thirty-seven patients presented to the unit over the stipulated period. The male/female ratio was 1.1:1. The median age at presentation was 4 months (mean 7.3 months, range 4 days to 6 years). Myelomeningocele-associated hydrocephalus, aqueductal stenosis, and postmeningitic hydrocephalus accounted for 30.7%, 22.6%, and 17.5%, respectively, of the cases. The mean Evans ratio was 0.56 (range 0.43-0.70), the mean ventricular index was 197.18 (range 135.0-245.3), and the mean cerebral mantle was 10.8 mm (10-14 mm). This study shows that the congenital form of hydrocephalus is the predominant variety in the authors' population. Myelomeningocele-associated hydrocephalus, aqueductal stenosis, Dandy-Walker malformation, and postmeningitic hydrocephalus are common causes of hydrocephalus.

Outcome of endoscopic third ventriculostomy and Chhabra shunt system in noncommunicating non-tumor childhood hydrocephalus

Background:Endoscopic third ventriculostomy (ETV) the main alternative to ventriculoperitoneal shunt (VPS) is just beginning to have a foothold in West Africa. It provides a great opportunity for a hydrocephalic child to be shunt free. The purpose of this paper is to compare outcome following ETV and VPS (using the cheap Chhabra shunt) in children with noncommunicating non-tumoral hydrocephalus in an environment where late presentation is the norm.Materials and Methods:Sixty-three pediatric patients (< 6 years) with hydrocephalus who underwent a VPS or ETV at our hospital were included in this study. The study period was of 30 months (January 2006 till June 2008). Clinically successful outcome was defined as no event occurring during or after surgery that resulted in an alternate surgical procedure, or significant post-operative complication that includes death. All complications related to the procedures were also analyzed.Results:The outcome of surgical intervention was not significantly related to the gender, age of the patient at surgery, or type of surgery. The post-operative complication rate was similar in both groups.Conclusions:The clinical outcome of ETV is comparable to that of VPS, with the added benefit of no shunt-related problems; and being a short procedure, patient anesthesia and operation time, hospital stay, and cost are significantly reduced. We consider ETV to be the procedure of choice for the treatment of noncommunicating nontumoral hydrocephalus in the pediatric population.

Disorders of growth and puberty in children with non-tumoral hydrocephalus.

Hydrocephalus may cause disorders of growth and puberty. 31 patients (25 girls) with non-tumoral hydrocephalus were seen at 8.5 +/- 3.1 (SD) years for short stature (8 patients), overweight (8 patients), central early puberty (onset before 9 years, 21 patients), premature pubarche (1 patient) and/or delayed puberty (2 patients). Among the patients with short stature, 4 had meningomyelocele and one had untreated early puberty. Only 1/11 patients evaluated had growth hormone deficiency. Among the overweight patients, 5 had early puberty. The plasma leptin concentrations were positively correlated with the body mass index (r = 0.65, p < 0.01, n = 14). Free thyroxin, cortisol, prolactin and concomitant plasma and urinary osmolalities were normal in all cases evaluated, except one who had low free thyroxin. The 7 patients with early puberty and who were given gonadotropin releasing hormone analog for over 2 years had mean predicted adult height of -2.45 +/- 1.9 SD before treatment and -2.46 +/- 1.4 SD afterwards. Ventriculocisternostomy performed on 2 girls seen for delayed puberty was followed by breast development and menarche. In conclusion, in children with hydrocephalus, short stature is frequently due to meningomyelocele and rarely to GH deficiency. Central early puberty is the most frequent endocrine disorder.

Lactate dehydrogenase enzyme as a metabolic marker for the evaluation of shunting in infants with non-tumoral hydrocephalus

Lactate dehydrogenase (LDH) enzyme activity has not been analyzed in neonatal nontumoral hydrocephalus. We studied 14 patients with nontumoral hydrocephalus (7 males and 7 females) with ages ranging between 1 and 18 months. All patients were treated by ventriculo-peritoneal shunt, and evaluated clinically and by meassurement of LDH activity in cerebrospinal fluid (CSF) and blood, both, preoperatively and 10 days after the placement of the shunt.All patients showed high levels of LDH in the CSF, and high CSF/Blood LDH ratio in the preoperative samples. Although all the patients had clinical improvement, and some of them showed radiological improvement, most retained high levels of LDH activity in the CSF (13 out of 14 patients), and high CSF/Blood ratio (9 out of 14 patients).In spite of the clinical and radiological improvement due to the control of raised intracranial pressure by shunting, the improvement of metabolic activity of the brain, as measured by LDH activity was not normalized during the investigation periodo Therefore, we feel that the follow-up of such patients using metabolic activity of the brain by monitoring LDH as a marker might be more sensitive than the morphological and clinical parameters.

Actualities in hydrocephalus classification and management possibilities

Retrospective analysis in co-operative study of hydrocephaus at institutions of members of the Research Committee on Intractable Hydrocephalus sponsored by the Ministry of Health and Welfare of Japan was performed to determine the functional prognosis. For clinico-epidemiological study we classified nontumoral hydrocephalus into eight types based on its etiology and the time of onset. Analysis of the 1,450 cases of hydrocephalus stored in the database obtained from the study was performed in order to find intractable factors In terms of factors related to patients and management. Analysis of the cases stored in the database revealed that the following types and conditions were found to be intractable factors: 1. Early fetal hydrocephalus. 2. Overt neonatal hydrocephalus. 3. Hydrocephalus associated with such severe brain malformations as hydranencephaly, holoprosencephaly and lissencephaly. 4. Hydrocephalus associated with severe brain damage. 5. Hydrocephalus associated with epilepsy. 6. Hydrocephalus shunted late after detection. 7. Hydrocephalus complicated by a shunting operation. It is impossible to determine prior to treatment whether or not a shunting operation is indicated for the patient with intractable factors, however, they may be a useful pre-operative indicator of prognosis. For the management of hydrocephalus, secondary intractable hydrocephalus may be preventable if we treat it appropriately before it becomes intractable. [Neurol Res 2000; 22: 127-130]

Late outcome of the surgical treatment of hydrocephalus.

The purpose of this report is to analyze the long-term outcome in hydrocephalic children treated by shunt placement and in particular their psycho-intellectual development. In the case of shunt placement postoperative mortality is virtually nil, and the overall mortality rate after 10 years of follow-up has been reduced to less than 5%. Morbidity, in contrast, is far from satisfactory. This study was conducted in 129 consecutive children with nontumorous hydrocephalus who underwent a first shunt insertion before the age of 2 years between 1979 and 1982 and who were followed up for at least 10 years. The final neurological examination revealed a motor deficit in 60%, visual or auditory deficits in 25%, and epilepsy in 30%. The final IQs were above 90 in 32% of the children, between 70 and 90 in 28%, between 50 and 70 in 19%, and lower than 50 in 21%. Integration into the normal school system was possible for 60% of the children, but half of them were 1-2 years behind their age group or having difficulties; 31% were attending special classes or were in institutions; and 9% were considered ineducable. The presence of behavioral disorders was a determinant factor for scholastic and social integration. Such disorders were frequent, and were characterized as severe in 30%. A relationship between final outcome and etiology, initial ventricular size, and epilepsy was observed. These results are used as the basis of a discussion on how morbidity might be improved.

Classification of hydrocephalus and outcome of treatment

Purpose: Retrospective analysis in cooperative study of hydrocephalus at institutions of members of the Research Committee on Intractable Hydrocephalus sponsored by the Ministry of Health and Welfare of Japan was performed to determine the functional prognosis for all types of hydrocephalus and thus to clarify the outcome. Methods: In preparation of this study, we have proposed the definition, clinical classification and diagnostic criteria of hydrocephalus. We have classified non-tumoral hydrocephalus into eight types based on its etiology and the time of onset. To establish the diagnosis in each type of hydrocephalus, we have set up inclusion and exclusion criteria, as well as supplementary criteria which are useful for its diagnosis. Results: Analysis of the 1450 cases of hydrocephalus of various etiologies stored in the data base obtained from the study for each type of hydrocephalus revealed that following types and conditions were associated with a neurologic deficit: (1) early fetal hydrocephalus, (2) overt neonatal hydrocephalus, (3) hydrocephalus associated with such severe brain malformations as hydranencephaly, holoprosencephaly and lissencephaly, (4) hydrocephalus associated with severe brain damage, (5) hydrocephalus associated with epilepsy, (6) hydrocephalus shunted late after detection, and (7) hydrocephalus complicated by a shunting operation. Conclusion: The postnatal functional outcome was significantly poor in fetal hydrocephalus diagnosed in the early gestation. Childhood onset hydrocephalus showed a poorer outcome than adult hydrocephalus.

Computerized use of ICP data. Preliminary elements on the shunt patient.

An incorrect choice of valve may produce a delay in psychomotor development in shunted hydrocephalic children. The intracranial pressure was measured for 24 h in 20 subjects suffering from nontumoral hydrocephalus, obtaining a pressure/volume index with compliance and perfusion pressure data. Their data were entered into a computer and used to determine the correct valve to be chosen.

Danish experience with the one-piece shunt. A long-term follow-up.

A review of the efficacy and complications of the simple one-piece ventriculoperitoneal shunt is given, based on a consecutive series of 61 children with nontumorous hydrocephalus. Control of the hydrocephalic state was satisfactory but was, however, complicated by a rather high frequency of slit ventricles and subdural effusions. No significant increase in the complication rate was observed among premature babies. The infection rate was low (3%) and no visceral perforations were observed. The one-piece shunt can be recommended, especially for smaller children with hydrocephalus.

Electrolyte levels in the CSF of children with nontumoral hydrocephalus

The levels of calcium and magnesium (by atomic absorption spectrophotometry), sodium and potassium (by flame photometry), inorganic phosphate and proteins (by spectrophotometry) were measured in the CSF of 27 children with hydrocephalus and compared with the CSF data on 10 controls, so as to study the effect of the blood-brain and blood-CSF barrier alterations observed in hydrocephalus, on the CSF electrolytes. Ca (P less than 0.001), Mg (P less than 0.01) and phosphate (P less than 0.05) were found to be increased, K (P less than 0.01) decreased, and Na levels not significantly altered. Linear regression analysis of hydrocephalic patients showed a linear correlation (r = +0.62) between phosphate and proteins (P less than 0.01) and (r = +0.66) between phosphate and Ca (P less than 0.001). Comparison of control CSF electrolyte levels with those found in the CSF of hydrocephalic patients were grouped according to clinical variables: sex (F, M), age (less than or equal to 1.5 months, greater than 1.5 months), etiological variants (congenital, acquired), treated infection (no, yes), surgical treatment (no, yes), time of onset (less than or equal to 1 month, greater than 1 month), ventricular dilation (mild, severe) and intracranial hypertension (mild or severe). These comparisons showed specific increases of Mg for males (P less than 0.05), acquired hydrocephalus (P less than 0.05) and severe intracranial hypertension (ICH) (P less than 0.001), moderate decrease in the Na levels in acquired hydrocephalus (P less than 0.05) and mild ICH (P less than 0.05), specific increases of phosphate in females (P less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Long-term intellectual prognosis of hydrocephalus with reference to 77 children.

The long-term intellectual prognosis of nontumoral hydrocephalus is described: 75% of the population have a full intellectual quotient greater than 70. But often, there is a heterogeneous pattern of the intellectual quotient with a great difference between verbal and performance results because of poor visuospatial skills. Disabilities of syntactical comprehension of language are also encountered. Sex, age, delay before shunting and frequency of shunt complications are not related to intelligence level or pattern. Meningitis and toxoplasmosis often result in a poor intelligence level, but other etiologies do not influence intelligence level or pattern. Visuospatial deficits, attention disorders and language disabilities are independent of the degree of hydrocephalus but are especially encountered when ventricular dilatation persists in spite of the operation.

Isoelectric focusing of cerebrospinal fluid proteins in children with nontumoral hydrocephalus. A preliminary report.

The cerebrospinal fluid (CSF) protein patterns of 42 children with nontumoral hydrocephalus, 33% with associated congenital malformations (meningocele, myelomeningocele, spina bifida, monoventricular prosencephalon) were determined by isoelectric focusing and polyacrylamide gel electrophoresis. 50% of the patients showed a barrier damage pattern, 19% showed a degenerative pattern, a block pattern or a normal pattern were less frequently found. 14 children presented hydrocephalus associated with other malformations, 44% of these cases showed a degenerative pattern. Combining results from quantitative polyacrylamide gel electrophoresis and qualitative isoelectric focusing, resulted in better characterization of CSF protein abnormalities, the high resolution capacity of these techniques may give a new approach to the evaluation of the blood CSF barrier in the management of hydrocephalus.

Polyacrylamide gel electrophoresis of cerebrospinal fluid proteins in children with nontumoral hydrocephalus.

The changes occurring in the electrophoretic pattern of the cerebrospinal fluid (CSF) proteins in 37 children with nontumoral hydrocephalus, and the protein pattern of 12 children without organic neurological disease, were studied with polyacrylamide gel electrophoresis. In nontumoral hydrocephalus protein was frequently increased in lumbar and ventricular fluid, in this condition, in intraventricular obstruction, the protein fractions showed a decrease in the prealbumin and an increase in the gamma-globulin fractions, and in extraventricular obstruction, the protein fractions showed a decrease in the prealbumin and an increase in the gamma-globulin and alpha 2-globulin fractions. With CSF proteins within normal ranges we found that in intraventricular obstruction, the proteins of the ventricular-CSF showed an increase in the prealbumin and/or albumin fractions, and in extraventricular obstruction, the proteins of the ventricular CSF showed an increase in the prealbumin and beta-globulin fractions, and the proteins of the lumbar CSF showed an increase in the prealbumin fraction. The diagnostic implications of these findings are discussed.

Results of treatment with ventriculoatrial and ventriculoperitoneal shunt in infantile nontumoral hydrocephalus.

The authors present the results of surgical treatment in 165 children with nontumoral hydrocephalus, treated during a period of 11 years. The minimum period of follow-up was 1 year. The results in the group of children treated with a ventriculoatrial (VA) shunt were compared to those obtained in the group with a ventriculoperitoneal (VP) shunt. 45% of patients with VA shunts had one or more revisions, against 51% of patients with VP shunts. However, the incidence of revisions after 6 months from primary insertion was lower in the VP-shunted children. Most revisions were due to shunt malfunction: obstruction of the ventricular or abdominal catheter was the commonest finding. Among complications, the most serious was constituted by shunt infection (11% of cases). Most infections occurred at less than 2 months from surgery and were caused mainly by Staphylococci. 31% of infected patients died. Other frequent complications were due to shunt tubing lost in abdomen or in the ventricles and subdural hematoma. The overall mortality rate in the whole group was 18%, while the shunt-related mortality rate was 10.9%. Complications were more frequent in the VP-shunted patients, but were less than in VA-shunted patients and accounted for a lower mortality rate. Complications and mortality rate were prevalent in the patients presenting congenital communicating hydrocephalus or myelomeningocele. It is concluded that VP shunt is preferable to VA shunt in the treatment of infantile hydrocephalus.

A Comparison of Computed Tomography and Air Ventriculography in Diagnosis of Progressive Hydrocephalus of Infancy

We have compared the efficacy of computed tomography (CT) and air ventriculography (VGM) in the diagnostic evaluation of progressive nontumoral infant hydrocephalus where both examinations have been done without significant interventing time or treatment in infants under 12 months of age. CT alone was judged to be adequate for diagnosis and treatment 21 of 30 cases reviewed, provided that cerebrospinal fluid studies were available to complete diagnosis where necessary, and provided that the question of ventriculocisternal communication was not a factor in treatment selection. The ability to visualize cerebran aqueduct and 4th ventricle on CT was not always a reliable indicator of ventriculocisternal communication. CT was most adequate as the sole radiographic study in cases of myelodysplasia with Arnold-Chiari malformation, and in premature infants with intraventricular hemorrhage.

Investigations of nontumoral hydrocephalus in children.

An analysis was made of 175 cases of pediatric nontumoral hydrocephalus. CT scan by itself permits a precise diagnosis in only one-third of the cases. However, when the clinical context suggests that hydrocephalus is secondary to certain conditions such as infection, bleeding, or trauma, CT scan may suffice, unless more advanced investigation permits more efficient treatment. When hydrocephalus is associated with brain malformations, however, more complete investigation is needed for a better understanding of the condition. Conventional contrast studies are quite safe, especially ventriculography which is easy to perform in this type of patient, who is usually investigated during the first 12 months of life.