Visual pathway tumors and hydrocephalus

The authors evaluated the impact of hydrocephalus on the clinical picture of children with visua pathway tumor (VPT) with or without neurofibromatosis (NF).Charts of children with VPT treated in the authors' center since 1985 were retrospectively reviewed, and those with hydrocephalus were selected and summarized. Thirty-five children with VPT were found, of whom 20 had NF.Hydrocephalus was found in 4 children with NF (20% ) and in 5 without NF (33.3% ). In 6 ofthechildren, ventricular dilatation with signs of acute increased intracranial pressure already existed at the time of diagnosis and the hydrocephalus was shunted at this time. In the other 3 children, all with NF,the hydrocephalus resulted from slowly developing aqueductal stenosis, leading in 2 to severe visual acuity deterioration. The results suggest that in children with VPT and NF, hydrocephalus, and especially hydrocephalus resulting from aqueductal stenosis, is more frequent than in the general population of NF patients, and less frequent than in VPT patients without NF. The possibility of the indolent development of hydrocephalus should be borne in mind while following children with NF. The optic nerve, when already involved with a glioma, is more vulnerable to increased pressure. Thus, in children with VPT and NF, any ventricular dilatation should lead to a consideration of early shunting.     

Experimental Hydrocephalus in Suckling Hamster Induced by Myxovirus Infection

Abstract This study was undertaken to elucidate the pathogenesis of the hydrocephalus and aqueductal stenosis induced by intracerebral mumps virus inoculation in suckling hamsters.
Mild ventricular dilatation became apparent after 5 days of inoculation. Focal denuding of the ependymal layer and subsequent aqueductal stenosis were observed by 14 days after inoculation. The virus antigen was detected not only in the ependymal cells and choroid plexus, but also in some neurons in the cerebral cortex, hippocampus, midbrain and cerebellum. In the cerebral aqueduct, the orderly arrangement of the cilialy clusters was destroyed on the 5th day after inoculation. After 10 days, proliferation of GFAP positive cells was noticed around the cerebral aqueduct and subsequently caused aqueductal stenosis. In the advanced state of hydrocephalus, the cerebellum was displaced downward and showed an elongated, atrophic and sleevelike structure similar to the Arnold-Chiari malformation. It was suggested that the extensive damage of the ependymal cilia may account for early ventricular dilatation, and subsequent aqueductal stenosis with glial proliferation is the main cause of the advanced hydrocephalus. It has not yet been determined whether the mumps virus can pass through the human placenta or not. If it can, however, our results strongly suggest that mumps virus infection in the human fetus will cause congenital hydrocephalus.     

Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children

Background

Rhombencephalosynapsis is a rare genetic aberration characterized by variable vermian hypoplasia/aplasia in conjunction with united cerebellar hemispheres. Genetic defects in the isthmic organizer at the mesencephalic–metencephalic junction are presumably responsible for the associated aqueductal stenosis.

Objective

We performed a retrospective review of 20 children with rhombencephalosynapsis to evaluate for and emphasize the association of aqueductal stenosis and hydrocephalus.

Materials and methods

We retrospectively reviewed the MR and CT images of 20 children (0–11 years old) with rhombencephalosynapsis encountered at two academic children’s hospitals. Rhombencephalosynapsis spectrum severity was graded based on pre-existing literature. We analyzed examinations for ventriculomegaly and degree of aqueductal stenosis. The collicular distances were measured from the collicular apices. Imaging studies were also analyzed for malformations of cortical and cerebellar development.

Results

Thirteen of the 20 children (65%) with rhombencephalosynapsis presented with clinical or imaging evidence of hydrocephalus and aqueductal stenosis, principally involving the caudal cerebral aqueduct. All children with aqueductal stenosis had collicular fusion. All six children with complete rhombencephalosynapsis had aqueductal stenosis. The cerebral aqueduct varied from normal to stenotic in children with incomplete rhombencephalosynapsis. Corpus callosum dysgenesis was present in four children.

Conclusion

Aqueductal stenosis in the setting of rhombencephalosynapsis is an under-recognized cause of noncommunicating hydrocephalus. Our findings support the hypothesis that a defect involving the common gene(s) responsible for the differentiation and development of both the roof plate and midline cerebellar primordium at the mesencephalon/first rhombomere junction may be responsible for the association of aqueductal stenosis and rhombencephalosynapsis.     

MRI findings in children with neurofibromatosis type 1: a prospective study

To determine the frequency and nature of MRI lesions in children with neurofibromatosis type I (NF1), 50 patients aged 8 to 16 years were evaluated prospectively with cranial MRI. Forty-one children were asymptomatic with respect to central nervous system pathology, and 50 % were macrocephalic. Sixteen patients (32 %) had normal MRI examinations. Thirty-two patients (64 %) had high intensity lesions on T2-weighted images and 16 patients (32 %) had hyperintense lesions on T1-weighted images. Seven patients (14 %) had ventricular dilatation (associated with increased intracranial pressure in 2) and 11 patients (22 %) had optic pathway lesions (optic glioma in 7). MRI was superior to CT in differentiating optic sheath thickening and optic nerve tortuosity from optic glioma in four patients. An intracranial tumour (ependymoma) and sphenoid wing dysplasia were evident in individual patients. Findings previously unreported in NF1 included an aqueductal web resulting in hydrocephalus, intraocular neurofibroma resulting in retinal detachment, and asymptomatic enlargement of the septum pellucidum. T1 and T2 signal abnormalities in isolation were not associated with neurological deficits or the occurrence of macrocephaly, and all lesions that required intervention were suspected clinically. Macrocephaly in the absence of increased intracranial pressure or accelerated head growth is not an indication for neuroimaging in children with NF1. However, the majority of children (68 %) had disease-specific abnormalities and thus MRI may provide a useful adjunct to clinical evaluation in the diagnosis of equivocal cases.     

Aqueductal stenosis presenting as isolated tremor: case report and review of the literature

Essential tremor is rare in children, particularly in the absence of a significant family history. We report the case of a child with compensated hydrocephalus secondary to aqueductal stenosis whose sole presenting symptom was tremor. An otherwise healthy 6-year-old male developed a fine hand tremor, which over the course of 4 years both increased in intensity and spread to involve the lower limbs and head. After an MRI had confirmed hydrocephalus due to aqueductal stenosis, the patient underwent an endoscopic third ventriculostomy. His tremor improved markedly, but did not completely resolve. Occult hydrocephalus should be considered in the differential diagnosis of new-onset tremor. Progression of the tremor should halt with treatment of the hydrocephalus, and clinical improvement may be seen.     

Shunt revision in hydrocephalus

Objective : A retrospective analysis of 50 hydrocephalic children having a minimum follow-up of 6 months was carried out to see their etiology, clinical features, complications, incidence of shunt revisions, outcome and the variation from their Western counterparts.Methods : Clinical features, image findings and treatment of all the cases were recorded from their discharge summaries. Record of shunt revision complications and outcome was maintained by the principal author. The data of all the cases were analyzed.Results : The age of children varied from 1 month to 12 yr (mean 2.2 yr). The most common etiology of hydrocephalus was aqueductal stenosis in 18 (36%) children. Post infective hydrocephalus, either of post-tubercular meningitis (TBM) or following bacterial meningitis, remained the cause in 15 children (30%). Congenital TORCH infection was responsible for 3 cases of hydrocephalus making infective etiology as the cause in 18 (36%) cases. Intra 4^th ventricular neurocysticercus cyst caused blockade of CSF pathway in 2 children. 15 out of 50 children required shunt revision, either due to infection (8,16%) or shunt obstruction (7, 14%). Multiple shunt revisions were required in 2 children only. These revisions were required due to infection, obstruction or malfunction of the shunt.Conclusions : Infective etiology is responsible for hydrocephalus in significant number of children (36%). The possibility of TORCH infection, as a cause of hydrocephalus should be considered even amongst the children of screened mothers during antenatal check-up. Pure intra 4^th ventricular neurocysticercus cysts (without intraparenchymal cyst), though rare, can manifest with outlet obstruction. Incidence of shunt revision using Chhabra’s medium pressure shunt is very high in children at an average follow up of 1.6 yr. Post infective hydrocephalus is a major cause of delayed milestones, contributing to mental retardation.     

Bobble-head doll syndrome

A new case of Bobble-head doll syndrome with aqueductal stenosis is presented in a 14 year-old boy. Ventriculocisternostomy performed 8 years after the onset of the abnormal movement resulted in moderate reduction of the head bobbling. Twenty-two cases were found in a review of the literature. In all cases there was a chronic slowly progressive hydrocephalus with usually a cyst of the third ventricle; aqueductal stenosis was less frequent. When recorded, psychomotor development was impaired. Treatment is neurosurgical. Pathogenesis remains unknown.     

Endoscopic third ventriculostomy through the interfornicial space

Endoscopic third ventriculostomy (ETV) is the most common cranial endoscopic surgery being done worldwide for a variety of conditions resulting in hydrocephalus. We report ETV in a case of congenital hydrocephalus with deficient third ventricular roof and wide interfornicial space. A 6-month-old male child presented with gradual progressive head enlargement and signs of raised intracranial pressure. Computed tomography of the head showed aqueductal stenosis with enlargement of third and lateral ventricles. The patient was operated under general anesthesia with endotracheal intubation. A right coronal burr hole and free-hand entry were made into the right lateral ventricle. The anterior part of the septum and the third ventricular roof were deficient with a narrow Monro's foramen and the third ventricular floor was visible through the space between both fornices. ETV was done in standard fashion. Postoperatively the patient showed uneventful recovery clinicoradiologically. We review the literature and discuss the findings and technique of ETV in this rare entity.     

Neuropathology and immunohistochemistry of the brain-stem in neonates with congenital hydrocephalus: Comparative studies between aqueductal stenosis and Arnold-Chiari malformation

Neuropathological and immunohistochemical studies were done on the brain-stem of neonates who had congenital hydrocephalus with aqueductal stenosis or Arnold-Chiari malformation (ACM). The infants with aqueductal stenosis showed heterogeneity in their clinicopathological findings while the infants with ACM were relatively similar in neuropathological findings. There were prominent astrogliosis, decreased immunoreactivity with antisera to tyrosine hydroxylase and myelin basic protein in the periaqueductal area, and an increased reactivity with antiserum to substance P in the tegmentum of most patients with aqueductal stenosis and other malformations. In ACM, there was little gliosis in the tegmentum and periaqueductal area and minimal immunoreactivity of tyrosine hydroxylase, myelin basic protein and substance P. In both groups of cases, the cells in the periaqueductal region differ in neurotransmitter/neuromodulator immunoreactivity and degree of myelination reflecting a difference possibly in their maldevelopment.     

Gliomas of the tectum and periaqueductal region of the mesencephalon.

Gliomas that arise in the tectal and periaqueductal region of the mesencephalon usually present with hydrocephalus secondary to occlusion of the aqueduct of Sylvius. A review of 486 brain tumors in children treated during a 5-year period revealed 6 children with gliomas of the tectal plate. The 6 children were shunted for hydrocephalus, presumed secondary to aqueductal stenosis, prior to establishing the diagnosis of tectal plate glioma. No abnormalities were noted on the initial, uncontrasted computed tomography (CT) scans. The tumors are isodense without contrast enhancement which makes the CT diagnosis difficult. Magnetic resonance imaging (MRI) is diagnostic and demonstrates the characteristic enlargement of the tectum with increased density on T2 images. T1 density and gadolinium enhancement are variable. Pathological confirmation was obtained by open biopsy in 2 patients, a stereotaxic biopsy was performed on 2 children; 2 children were not biopsied. The tumor histology obtained was that of pilocytic astrocytoma. Two patients were treated with radiation therapy at the time of diagnosis. One child was followed closely and subsequently irradiated after tumor progression. All patients in this series are alive and functioning adequately 2-10 years after the onset of symptoms.     

Idiopathic hydrocephalus in adolescence]

BACKGROUND: Decompensation of chronic idiopathic hydrocephalus can occur at every age, but seems to be rather frequent in the middle of the second decade. From this observation, the question arises, whether or not in these cases a special manifestation of hydrocephalus occurs and, should the situation arise, whether this finding might influence the discussion about pathogenesis. METHODS: We give some casuistic material about six hydrocephalic patients who became symptomatic between their 12th and 16th year of life. RESULTS: All of these patients showed a tri-ventricular appearance of hydrocephalus as well as radiological signs of chronicly elevated intracranial pressure, while clinical signs and symptoms differed considerably. These findings are linked to the discussion of the pathogenesis of so-called aqueductal stenosis. CONCLUSIONS: Whenever, after normal development in childhood, symptoms like increasing headache, poor concentration, dizziness or disturbances of gait appear during puberty, X-ray diagnosis of the skull is recommended. If it shows the signs of chronic elevated intracranial pressure, further investigations are necessary.     

Non-tumoral neonatal hydrocephalus. Results of surgical treatment in the first month of life]

Two hundred eight patients with non-tumoral congenital hydrocephalus underwent CSF shunting below the age of one month. Ultrasonography was the most frequently utilized tool; hydrocephalus was recognized during pregnancy in 52% of the cases. Hydrocephalus was associated with myelomeningocele in 97 infants; in 38 subjects ventricular dilation was secondary to aqueductal stenosis. Post-hemorrhagic and post-infective hydrocephalus accounted for only 20 and 13 cases respectively. At the follow-up observation, a normal psychomotor development was recorded in 67.3% of the cases. Seventeen patients died. CSF infection was the most common cause of death (41%); in 6 patients the exitus was determined by the natural evolution of congenital associated malformations. Shunt revision did not influence the morbidity and the mortality significantly. On the other hand, CSF infections appeared to influence the prognosis negatively. In our experience, the prognosis of hydrocephalus operated on in the first month of life does not differ from that of hydrocephalus operated on late in life.     

The prognosis of children with hydrocephalus and congenital heart disease

The association of congenital hydrocephalus and heart disease in children is infrequent, but may present considerable dilemmas in management. This report describes the treatment and prognosis of 11 children with both clinical problems. There were 5 males and 6 females. Hydrocephalus occurred following aqueductal stenosis in 5 children and the Dandy-Walker malformation in 3. Three children were diagnosed with idiopathic hydrocephalus. Ten children underwent cerebrospinal fluid diversion procedures for control of hydrocephalus. Five children received pharmacological therapy for cardiac disease; 4 children required surgical correction. Two children died from medical conditions; 2 families declined treatment. Follow-up from 2 to 7 years in the remaining 7 children demonstrated moderate or severe neurodevelopmental disability in 5. One child at 2 years of age showed borderline developmental disability while 1 child is developing normally at 10 years of age. Overall the occurrence of symptomatic hydrocephalus and heart disease in the perinatal period resulted in mortality or neurodevelopmental disability in 9/11 children.     

Intraventricular neurocysticercus cyst(s) in Indian children

Eleven cases of intraventricular neurocysticercosis were managed over a 10-year period. All patients presented with features of hydrocephalus. In 9 cases the cysts were blocking the cerebrospinal fluid (CSF) pathway resulting in hydrocephalus. The 4 cases of lateral ventricular cyst had communicating hydrocephalus, while 2 of these had asymmetrical ventriculomegaly. Two of 4 cases had more than one cyst. One of 4 children with lateral ventricular cyst had narrowing of the foramen of Monro causing asymmetrical hydrocephalus. Following cyst removal, CSF diversion was not required in 6 cases, while 5 required CSF diversion despite removal of their cysts in 4 of 5. One patient had a cyst at the aqueductal inlet and he was managed by shunt and albendazole therapy. Thus, we managed 6 of our cases with excision of the cysts obstructing the CSF pathway, and in 4 cases excision of cysts with ventriculoperitoneal shunt was adequate.     

X-linked hydrocephalus. Further observations

The neuropathologic findings in a case of hydrocephalus of the X-linked recessive aqueductal stenosis type have been studied and compared with those previously reported from the same pedigree as well as with other cases from the literature. Additional pathological findings not previously recorded included: absence of the septum pellucidum and corpus callosum with malformation of the corpora quadrigemina and partial midline fusion of the fornices and thalami and fusion of thalamus with basal ganglia. These new findings indicate a broader phenotypic spectrum of this form of congenital X-linked recessive aqueductal stenosis than was previously known. Additional data from a diagnostic/genetic study of severely mentally retarded individuals are cited to show that 4 out of 5 familial cases of hydrocephalus without spina bifida are compatible with X-linked inheritance.Supported by USPHS Grants GM15422, GM20130 and 5KO4 HD18982. Paper No. 1785 from the University of Wisconsin Genetics Laboratory.     

Hydatid cyst of the ambient cistern radiologically mimicking an arachnoid cyst

Hydatid cysts of the posterior fossa are extremely rare. Intracranial hydatid cysts are more common in children and occur more frequently in the supratentorial space. A 7-year-old boy was admitted to the emergency department because of intense headache, nausea, vomiting, and progressive drowsiness that developed within the period of a week. On radiological examination a round, 2.5 × 2.5-cm cystic lesion appeared in the ambient cistern and caused hydrocephalus as a result of extrinsic aqueductal stenosis. The cyst was successfully removed using the puncture, aspiration, irrigation, and resection technique via an infratentorial-supracerebellar approach with the patient in the sitting position. The authors here described an unusual case of a hydatid cyst in the left ambient cistern with hydrocephalus due to extrinsic aqueductal stenosis, which seems to be the first such case in the literature. Hydatid cyst may be considered in the differential diagnosis of arachnoid cysts of the quadrigeminal cistern to determine which surgical procedure to perform and to avoid unexpected complications.     

Treatment of progressive posthemorrhagic hydrocephalus with temporary external ventricular drainage. Preliminary results

Peri-intraventricular hemorrhage (PIVH) is a major problem of preterm neonates: over 40% of infants with birth weight less than 1500 g have been found to experience this disorder. The posthemorrhagic hydrocephalus (PHH) is generally believed to occur secondary to obliterative posterior fossa arachnoiditis. Its management in critically ill premature infants with multiple medical problems has thus become quite significant. This paper reports the results of the placement of a temporary external ventricular drainage in three patients in whom progressive ventricular dilatation began within two weeks after severe neonatal PIVH. In all patients the hydrocephalus and increased intracranial pressure were controlled within three weeks of treatment. One infant died from other problems associated with his prematurity. Early demonstration of progressive ventricular dilatation by ultrasound and prompt external drainage has been found to be a safe initial method to treat PHH in preterm infants.     

Longitudinal comparison of diffusion tensor imaging parameters and neuropsychological measures following endoscopic third ventriculostomy for hydrocephalus

The authors report the case of a 25-month-old boy who underwent endoscopic third ventriculostomy (ETV) for hydrocephalus resulting from aqueductal stenosis. The patient's recovery was monitored longitudinally and prospectively using MR diffusion tensor imaging (DTI) and formal neuropsychological testing. Despite minimal change in ventricle size, improvement in the DTI characteristics and neurodevelopmental trajectory was observed following ETV. These data support the use of DTI as a biomarker to assess therapeutic response in children undergoing surgical treatment for hydrocephalus. In the patient featured in this report, DTI appeared to provide more information regarding postoperative neurodevelopmental outcome than ventricle size alone.     

Conservative treatment of non-resorptive hydrocephalus in premature infants

Following perinatal asphyxia and intracranial hemorrhage frequently progressive ventricular dilatation develops in preterm infants. Most common is communicating hydrocephalus due to obliterative arachnoiditis. Ventricular dilatation is reported to affect normal brain development and early therapy is recommended. Cerebrospinal fluid shunting is still accompanied by multiple complications, esp. in preterm infants with a birth-weight below 1,500 g. Seven preterm infants, born between the 27th and 34th gestational week with a birthweight of 910-1,940 g were medically treated for their progressive communicating hydrocephalus. The therapy consisted of intermittant lumbar punctures, medication of acetazolamide and furosemide as well as electrolyte and base replacement. Therapy was started at the 14th-31st postnatal day and lasted from 46 to 149 days. In all children the ventricular dilatation diminished. A steady state of cerebrospinal fluid production and absorption was regained in four children. Due to reoccurrence of ventricular dilatation shunting was performed in three others at the age of more than 3 months and with a weight of 3,620-5,170 g. Thus, medical therapy of hydrocephalus provides time for development of preterm infants, delay of shunting procedures and normalisation of cerebrospinal fluid dynamics.     

Progressive posthämorrhagische Ventrikelerweiterung des Frühgeborenen Inzidenz, Prognose und Therapie

Background. 35% of preterm infants with intraventricular hemorrhage develop ventricular dilatation. The posthemorrhagic ventricular dilatation can persist, be transient or be progressive. Finally 1–2% of all very low birthweight (VLBW <1500 g) infants require shunt placement for the treatment of the posthemorrhagic hydrocephalus. Outcome. The neurodevelopmental outcome is extremely poor in children surviving progressive posthemorrhagic ventricular dilatation. Therapy. There are no uniform guidelines for the treatment of preterm infants with progressive posthemorrhagic ventricular dilatation. Randomised multicenter trials demonstrated, that inhibition of cerebrospinal fluid production with acetazolamide and furosemide or early cerebrospinal fluid tapping did not reduce the need for shunt placement and may worsen the outcome or can be associated with adverse side-effects. Preterm infants with posthemorrhagic ventricular dilatation should be treated by standard therapy with uniform guidelines for cerebrospinal fluid tapping and shunt placement.