Uncomplicated Hydrocephalus Research Articles

The Ventriculo-Gallbladder Shunt in the Treatment of Refractory Hydrocephalus: A Review of the Current Literature

The ventriculo-gallbladder (VGB) shunt has been reported on several occasions for the alleviation of ventriculo-peritoneal (VP) -shunt-refractory hydrocephalus. There is little data regarding VGB shunts and a need for delineating appropriate surgical therapy when cerebrospinal fluid drainage to the peritoneum becomes infeasible. We report our experience with VGB shunt placement in three patients with chronic hydrocephalus. All three had a history of prior VP-shunt placements and revisions due to distal obstruction or infection, or contraindications to alternative forms of ventricular drainage. In one patient, the VGB shunt functioned well for 9 years but was revised due to contamination during an unrelated operation. Neither of the other two patients have experienced VGB shunt-related complications. VP shunts are presently regarded as the standard of care for uncomplicated hydrocephalus. When VP shunts fail, the most common alternatives have been ventriculo-atrial and ventriculo-pleural shunts. In five case series involving 59 patients with VGB shunts, the long-term success rate was 62.7 per cent. Infection (10.2%) and obstruction (10.2%) were the most common complications. Based on durability and a low incidence of complications, it is the current consensus that VGB shunts are a viable alternative with good outcomes in the case of failed VP shunts.

Medical and social factors associated with cognitive outcome in individuals with myelomeningocele.

The interrelationship between biological and social risk factors and cognitive outcome in individuals with myelomeningocele was examined. The Kaufman Brief Intelligence Test (K-BIT) was administered to 65 children and young adults, age range 4 to 29 during a recent clinic visit. Unshunted individuals had scores in the average range and individuals with uncomplicated hydrocephalus in the low-average range. Although the level of lesion was found to be most strongly associated with total K-BIT score, examination of subscores indicated that socioeconomic status was the factor most strongly associated with Vocabulary score. The importance of both social and biological factors in predicting cognitive outcome in this population is useful in planning intervention strategies.

Information processing and reading competencies in hydrocephalic children

Cross-sectional studies of heterogeneous groups of hydrocephalic children report reading lags of 18 months compared to controls matched for physical disability. This longitudinal study investigated the relationship between the global skill of reading and basic sensory information processing capacities in three homogeneous groups of children: 21 children with uncomplicated hydrocephalus; 24 children who made excessive letter reversals (LR), and 30 normal school children. All children had normal range IQ, and were 4–10 years of age. Three information processing tasks were administered once per year for two years: a tactile matching-to-sample, a visual tachistoscopic matching-to-sample task and an auditory dichotic listening task. Woodcock Reading Mastery Tests were given to measure reading proficiency. Hydrocephalics demonstrated a number of consistent information processing deficits when compared to letter reversal and normal school children. Hydrocephalics showed tactile information processing deficits, quantitatively and qualitatively, both years. Hydrocephalic children showed consistent qualitative differences in visual information processing both years. Hydrocephalics did now show consistent differences (i.e., both years) in auditory information processing. Global reading proficiency appeared to be normal both in children with uncomplicated hydrocephalus and letter reversal children with normal range IQ. Some subcomponent reading skills were impaired in hydrocephalics. Several significant correlations were found between information processing errors and reading scores in both hydrocephalics and letter reversal children. However, these correlations were considered unreliable due to either (1) a failure to reach significance the second year or (2) a sign change from positive to negative by the second year of testing. The conclusion was drawn that there were no consistent associations between specific tactile, visual and auditory information processing deficits and reading proficiency in any of the three groups tested. These results are discussed in light of the importance of longitudinal research with homogenous groups as opposed to cross-sectional sampling in children with proficiency skill problems.

Ocular motility, visual acuity and dysfunction of neuropsychological impairment in children with shunted uncomplicated hydrocephalus.

Children with shunted, uncomplicated, communicating hydrocephalus were tested to determine (1) the persistence of neuropsychological impairment and (2) the relationship between neuropsychological functioning, ocular motility, and acuity abnormalities. Eighteen hydrocephalic and 18 individually age- and sex-matched controls were given a neuropsychological battery, repeated after an interval of 1 year. Hydrocephalic children were also tested at the beginning of the second year for strabismus, amblyopia and visual acuity. Their medical records were reviewed for history of ocular motility and/or acuity abnormalities. Hydrocephalic children with normal range IQ were found to have lower verbal IQ, memory, and fine motor skills compared to controls. A history of ocular motility and acuity abnormalities was associated with impaired visuospatial and verbal problem-solving skills.

The family history of "simple" congenital hydrocephalus. An epidemiological study based on 270 probands.

A longitudinal study was carried out on the family history of 270 babies with uncomplicated congenital hydrocephalus. They had 453 siblings of whom 5 (11.1 per 1,000) had congenital hydrocephalus and 9 (19.9 per 1,000) had neural tube defects. These data, together with those obtained from the literature, indicate that the aetiology of the two conditions is probably the same in many cases, although there are unusual exceptions in special families, even apart from families with the sex-linked recessively inherited hydrocephalus. These findings suggest that the parents of children with uncomplicated hydrocephalus should have adequate antenatal tests for the detection of neural tube defects as well as for hydrocephalus and may need similar prophylactic treatment as do the parents of children with spina bifida and anencephaly.

Genetic aspects of "uncomplicated" hydrocephalus and its relationship to neural tube defect.

Congenital hydrocephalus is a relatively uncommon abnormality in population malformation surveys accounting for between four and ten out of every 10,000 births. In 30%, it is caused by aqueduct stenosis. The majority of cases have a multifactorial aetiology, the genetic component, which is polygenic, rendering the developing foetus susceptible to largely unidentified environmental factors in early development. Some cases are caused by single gene defects. Family studies suggest that the risk to siblings of a child with uncomplicated congenital hydrocephalus, where the anatomical site is not specified, is about 1 in 50 (1 in 40 for males, 1 in 80 for females). With aqueduct stenosis, the risk to brothers of affected boys is 1 in 22, to sisters only 1 in 50. The risks to siblings of sisters is less. Probably less than 2% of uncomplicated hydrocephalus has an X-linked basis and such an aetiology should be suspected if the special clinical features are present, there is more than one male sibling affected, or there are affected male relatives on the mother's side. Dandy-Walker's syndrome may be recessively inherited and there are some other, but very rare, monogenic causes for hydrocephalus. There is not obvious additional risk for neural tube defect. Non-directive genetic counselling should be given either at the genetic clinic or at a specially arranged clinic appointment and should include not only the risk to the family members, but also the options for avoiding recurrences, and any prenatal diagnostic tests available, such as high precision ultrasonography and a serum alphafetoprotein estimation at about sixteen weeks gestation.(ABSTRACT TRUNCATED AT 250 WORDS)

HYDROCEPHALIC INFANTS

29 hydrocephalic infants (less than 30 months) who had shunts were studied with the 'Bayley Mental Scale' and with computed tomography. It was found that patients with a medical history indicating brain injury in addition to hydrocephalus had lower scores on the 'Bayley Mental Scale' than a comparable group whose hydrocephalus had been uncomplicated. Dilatation of the lateral ventricles was assessed by computing the ventricle-brain ratio (VBR) from the CT scan. The VBR was inversely correlated with the 'Bayley Mental Scale' score. Two thirds of the sample were reevaluated after 1 year; there was a significant decrease in VBR, but mental quotient was unchanged. These findings are in agreement with studies on older children in that the prognosis for cognitive functioning in hydrocephalic children is affected by brain injury. A correlation between brain mass and intelligence has been reported in older children with uncomplicated hydrocephalus, but not in groups with mixed etiology.

Neuropsychological functioning in children with shunted uncomplicated hydrocephalus.

A neuropsychological battery was given to a CT-defined group of children shunted for uncomplicated hydrocephalus with estimated normal range IQs. When no other brain anomalies were present, verbal IQ estimates were inferior to age and sex matched controls, but still in the normal range. In the presence of average academic performance and normal range IQ, impairment of neuropsychological functioning in verbal and nonverbal memory, fine motor speed, and visuospatial problem solving was observed. Successful neurosurgical management of hydrocephalus is not necessarily equated with achieving normal neuropsychological functioning.

Hydrocephalic Infants: Developmental Assessment and Computed Tomography

29 hydrocephalic infants (less than 30 months) who had shunts were studied with the 'Bayley Mental Scale' and with computed tomography. It was found that patients with a medical history indicating brain injury in addition to hydrocephalus had lower scores on the 'Bayley Mental Scale' than a comparable group whose hydrocephalus had been uncomplicated. Dilatation of the lateral ventricles was assessed by computing the ventricle-brain ratio (VBR) from the CT scan. The VBR was inversely correlated with the 'Bayley Mental Scale' score. Two thirds of the sample were reevaluated after 1 year; there was a significant decrease in VBR, but mental quotient was unchanged. These findings are in agreement with studies on older children in that the prognosis for cognitive functioning in hydrocephalic children is affected by brain injury. A correlation between brain mass and intelligence has been reported in older children with uncomplicated hydrocephalus, but not in groups with mixed etiology.

1112 VENTRICULITIS IN GROUP B STREPTOCOCCAL (GBS) MENINGITIS

Ventriculitis is a well known feature of gram negative meningitis but has not been documented as a serious complication of gram positive central nervous system infections. This report describes five patients with GBS meningitis and ventriculitis. In each case ventriculitis was shown either by ventricular cerebrospinal fluid (CSF) pleocytosis, positive GBS counter immuno-electrophoresis, or positive culture for GBS. The lumbar CSF was sterile with no pleocytosis in two of the five patients despite concomitant abnormal ventricular fluid. Computerized axial tomography was of great value diagnostically and in management of increasing head size in these patients. Four of the five infants presented after one month of age and all had marked hy-drocephalus. Three of the five infants were initially thought to have uncomplicated hydrocephalus and only after diagnostic ventricular puncture was ventriculitis and/or meningitis suspected. All five patients had significant intellectual and motor neurological sequelae subsequent to their infection. Ventriculitis appears to be a common and significant complication of GBS meningitis. Ventriculitis caused by GBS may resemble uncomplicated hydrocephalus in its presentation, emphasizing the need to examine all CSF carefully before shunting procedures are pursued.

Opsoclonus in hydrocephalus.

A clinical and pathological study was made of a three-month-old infant with opsoclonus and hydrocephalus. Morphological descriptions of the brain in opsoclonus are available for only eight other cases in the literature; six of these showed parenchymatous changes in the cerebellum, and one showed changes in the hypothalamus, midbrain, and pons. Our patient showed a communicating hydrocephalus and a large cisterna magna, but parenchymatous changes in the cerebellum were absent. There has been no previous report of opsoclonus with otherwise uncomplicated hydrocephalus. The possible role of pressure on the cerebellum by a large cisterna magna or dilated fourth ventricle in the pathogenesis of opsoclonus is discussed.

THE FAMILY HISTORY OF SPINA BIFIDA CYSTICA

1. The family histories of 722 infants who were born with spina bifida cystica were studied. 2. The index cases were referred for surgical treatment and were not selected in any way from the genetic point of view. 3. Intensive inquiries were made to obtain a complete family pedigree, including a prospective follow-up of siblings born after the index case. 4. Of 1,256 siblings 85 or 6.8% had gross malformation of the central nervous system: spina bifida cystica in 54, anencephaly in 22, and uncomplicated hydrocephalus in 9. 5. Of 306 children born after the index case 25 (8%) or 1 in 12 were affected. 6. There was a progressive increase in multiple cases in the family with increasing family size. In sibships of five or more, multiple cases occurred in 24.1%. 7. In 118 families cases of gross malformation of the central nervous system were known to have occurred among members of the family other than siblings. Cases occurred in three generations. 8. It is possible that spina bifida cystica might be a recessively inherited condition.