Internal Hydrocephalus Research Articles

Case report: Human neonatus with spina bifida, clubfoot, situs inversus totalis and cerebral deformities: sequence or accident?

An unreported coincidence of malformations has been studied by anatomical dissection in a mature male fetus. Auxiliary, this fetus was examined by computed tomography and the skeletal malformations were three-dimensionally reconstructed. The organs of the thorax as well as of the abdomen showed a visceral inversion. In the lumbar region, a hyperkyphosis was detected in addition to a split-notochord. A hydrocephalus internus with enlarged lateral ventricles and an agenesis of the corpus callosum were apparent. The lower extremities were dystrophic with both showing a talipes equinovarus-deformation. Following the case report, we discuss if this is coincidental or manifestations in consequence of one defect.

Laparoscopy-Assisted Abdominal Shunt Revisions in Children with Hydrocephalus

Abdominal shunt revisions are frequently necessary for ventriculoperitoneal shunt systems in children with hydrocephalus internus. There are many well-known problems related to abdominal shunt revisions such as difficulties in reaching the abdominal cavity, intra-abdominal adhesions, cerebrospinal fluid pseudocyst, or problems in retrieving dislocated catheters. From 6/1996 to 12/1997 we operated on twenty (20) children aged from 2-15 years (body weight 4.8-68 kg) with abdominal shunt insufficiency using the laparoscopy assisted technique. In only one case was it impossible to reach the abdominal cavity laparoscopically and conventional open surgery was necessary. We had no complications such as injured vessels or bowel and saw a complete recovery in all patients. In comparison to conventional surgery, we found handling simple, minor trauma to the patients, good intra-abdominal view, short operation times and good cosmetic results to be the advantages of laparoscopy-assisted abdominal shunt revision. In addition hospitalisation time can be reduced.

Congenital hydrocephalus internus and aqueduct stenosis: aetiology and implications for genetic counselling.

Genetic counselling in families with congenital hydrocephalus internus (CHI) in combination with aqueduct stenosis (AS) is often difficult due to an uncertain aetiology. We present a series of 35 patients with CHI and AS focusing on the aetiology and presumed recurrence risk for siblings. In 13 patients (37.1%) a genetic aetiology was identified with an increased recurrence risk for siblings. The relative frequency of patients with X-linked hydrocephalus in our sample was in accordance with the literature (2/35), but was more frequent in other diseases with Mendelian inheritance. In addition to the well-known X-linked and autosomal recessive forms of aqueduct stenosis with hydrocephalus, this malformation can occur in other diseases with Mendelian inheritance. This finding is of considerable importance for genetic counselling and prognosis.

Maternal and fetal toxicity of N-methylmorpholine by oral administration in rats

N-methylmorpholine, which is used as a catalyst in polyurethane foams producing, in solvents, stabilizing agents, and corrosion inhibitors, was administered to female rats by gavage at 100, 200, 600, and 900 mg/kg during organogenesis. It did not exhibit selective toxicity toward the developing conceptus. This compound administered to pregnant females was fetotoxic and teratogenic in the presence of maternal toxicity. N-methylmorpholine induced anophthalmia, internal hydrocephalus, and hydronephrosis but only at one dose which was also maternotoxic. Teratogenesis Carcinog. Mutagen. 19:369-376, 1999.

Minimally invasive lesionectomies through a stereotactically guided working sleeve

The authors report on their experience of lesionectomies close to or in the thalamus, basal ganglia, third ventricle and in the temporal lobe. The resection itself is performed stereotactically, MRI or CT guided, either microscopically or endoscopically through a sleeve designed by one of the authors and named PAN working sleeve. Over the last four years this new minimally invasive technique has been successfully applied in 39 cases. Eighteen patients with 11 astrocytoma (6AA, 5AII), 5 cavernoma and 2 metastases (melanoma, adenocarcinoma) of the basal ganglion-thalamus area and the trigonum were resected by means of a frontal or an occipital burr-hole, whereby in some cases there were subtotal resections. With four of these patients an existing hemiparesis increased by one degree (according to the proposal of the British Medical Research Council l-V). Seventeen patients with lesions in the foramen Monroi and in the third ventricle also underwent operation by means of frontal access, and in each case there was a total resection. Two of the patients required a shunt due to a persistent hydrocephalus internus. In one of these cases there was intraventricular bleeding which necessitated an intra-operative craniotomy. Four patients with intractable epilepsy were operated through a burr-hole in the anterior area of the os zygomaticum. Three patients were submitted to a selective resection of mesial structures and one to an anterior temporal lobe resection. To date the four patients have had no further seizures and no deficits have been observed. [Neurol Res 1999; 21: 51–59]

Phosphatidylcholine-specific phospholipase C from Listeria monocytogenes is an important virulence factor in murine cerebral listeriosis.

Meningoencephalitis is a serious and often fatal complication of Listeria monocytogenes infection. The aim of the present study was to analyze the role of internalin A (InlA) and B, which are involved in the invasion of L. monocytogenes into cultivated host tissue cells, and that of phosphatidylcholine-specific phospholipase C (PlcB), which mainly promotes the direct cell-to-cell spread of L. monocytogenes, in murine cerebral listeriosis by use of an InlA/B (DeltainlAB2)- and a PlcB (DeltaplcB2)-deficient isogenic deletion mutant strain and the wild-type (WT) L. monocytogenes EGD. Listeria strains were directly applied to the brain, a technique which has been employed previously to study the pathogenesis of cerebral listeriosis (D. Schlüter, S. B. Oprisiu, S. Chahoud, D. Weiner, O. D. Wiestler, H. Hof, and M. Deckert-Schlüter, Eur. J. Immunol. 25:2384-2391, 1995). We demonstrated that PlcB, but not InlA or InlB, is an important virulence factor in cerebral listeriosis. Nonimmunized mice infected intracerebrally with the DeltaplcB2 strain survived significantly longer and had a reduced intracerebral bacterial load compared to mice infected with the DeltainlAB2 strain or WT bacteria. In addition, immunization with the WT prior to intracerebral infection significantly increased the survival rate of mice challenged intracerebrally with the DeltaplcB2 strain compared to that of mice infected with the WT or DeltainlAB2 strain. Histopathology revealed that the major difference between the various experimental groups was a significantly delayed intracerebral spread of the DeltaplcB2 mutant strain, indicating that cell-to-cell spread is an important pathogenic feature of cerebral listeriosis. Interestingly, irrespective of the Listeria mutant used, the apoptosis of hippocampal and cerebellar neurons and an internal hydrocephalus developed in surviving mice, indicating that these complications are not dependent on the virulence factors InlA/B and PlcB. In conclusion, this study points to PlcB as a virulence factor important for the intracerebral pathogenesis of murine L. monocytogenes meningoencephalitis.

Prognose von Kindern mit pränatal erkannten Fehlbildungen des Zentralnervensystems

Background: Even though developmental anomalies of the central nervous system (CNS) count for a major portion of prenatally diagnosed malformations, there are only a few studies about prognostic criteria and the longtime follow-up of children with prenatally detected hydrocephalus internus or myelomeningocele. Purpose: Keeping a close check on the neurological development of surviving children with malformations of the CNS. To establish prognostic criteria for the outcome of these children in prenatal sonography. Patients and Methods: In a prospective study from 1984 to 1990 we examined 3853 fetuses at the First Women's Hospital of the University of Munich. The neurological development of the surviving children with prenatally detected hydrocephalus internus and those with myelomenigocele was examined and their situation in daily life was documented. The prenatal findings were related to the outcome for the purpose of defining prognostic criteria. Results: 258 of examined 3853 fetuses had at least one malformation of the CNS (prevalence 6.7%). Of 30 fetuses with spina bifida aperta 11 survived. Only one of them had a normal motor development, seven had normal cognitive ability. Two of them had cerebral convulsions. 37 of 78 fetuses with prenatally diagnosed ventriculomegaly survived. 24% had normal motor development, 38% normal cognitive ability. 49% had convulsions. In both groups nearly half of the children are in regular institutions during the day. Positive prognostic criteria in children with ventriculomegaly are constand course, monosymptomatic disease, absence of chromosomal aberrations or infections, in children with myelomenigocele a lesion under the level of L3. Pathological amounts of amniotic fluid, intrauterine growth retardation and additional malformations are negative prognostic criteria. We did not find a prognostic correlation of the diameter of cerebral tissue, as measured by prenatal ultrasound to the outcome.

Three brothers with mental and physical retardation, hydrocephalus, microcephaly, internal malformations, speech disorder, and facial anomalies: Mutchinick syndrome

We describe three brothers from a non-consanguineous family with microcephaly, mental and physical retardation, speech disorder, facial anomalies, and internal hydrocephalus in two of the three affected brothers. The youngest brother died at the age of 5 months. He had situs abdominalis inversus, ASD II, and had been operated for internal hydrocephalus and atresia of the biliary duct. A search in the Oxford Medical Database Dysmorphology Program suggested phenotypic similarities with two sisters described in 1972 by Osvaldo Mutchinick in Argentina. Although differences in their phenotypes exist, it is possible that the two sets of sibs represent the same, rare syndrome. This interpretation is supported by the origin of both families from the same geographic region.

Risk factors for latex hypersensitivity in childhood.

As hypersensitivity to natural rubber latex (NRL) has become an increasingly recognized problem in children, identification of all groups at risk seems important. In this study hypersensitivity to NRL was evaluated in 337 children with potential risk factors. We identified by questionnaire children's underlying diseases, history of surgical procedures, evidence of atopy and patient's history of NRL-specific reactions. Hypersensitivity to NRL and other allergens relevant in the Düsseldorf area was evaluated by skin prick test and specific IgE. In 9.2% of all children hypersensitivity to NRL was observed. Significant risk factors for hypersensitivity to NRL were, among the underlying diseases, spina bifida (odds ratio 29.2), hydrocephalus internus (10.1), gastrointestinal malformation (5.2) and atopy (2.2). Surgical procedures with significant risk were the implantation of a ventriculo-peritoneal shunt (15.7) and surgery of the gastrointestinal tract (3.1). Frequency of surgical procedures correlated (p < 0.001) with risk of hypersensitivity. Frequent surgery and atopy were found to have an additional effect on the risk of hypersensitivity. Information about previous NRL-specific reactions obtained by questionnaire was of little predictive value when performing multivariate analysis. For children at high risk for hypersensitivity to NRL preoperative evaluation and, in case of hypersensitivity, preventive measures seem to be advised.

Jeune syndrome with tongue lobulation and preaxial polydactyly, and Jeune syndrome with situs inversus and asplenia: Compound heterozygosity Jeune-Mohr and Jeune-Ivemark?

We report on a male infant with internal hydrocephalus, absence of corpus callosum, papillomas and lobulation of the tongue, notches of the alveolar ridges, short ribs, dysplastic pelvis, hypospadias, short limbs with bowed long tubular bones and postaxial polydactyly of hands, and preaxial polydactyly in one foot. Radiologically this case shares manifestations with Jeune syndrome; the tongue lobulation and the preaxial polydactyly are similar to findings in Mohr syndrome, or short-rib polydactyly syndrome (SRPS), type Majewski. In addition, a female newborn presented with manifestations of Jeune and Ivemark syndromes. One explanation for this overlap may be compound heterozygosity for these syndromes.

Effect of oral Sulfenamide TS administration on prenatal development in rats.

Sulfenamide TS (N-cyclohexyl-2-benzothiazolesulfenamide), accelerator of rubber vulcanization, was administered to female rats by gavage at doses of 50, 150, and 450 mg/kg (1%, 3%, and 8% of LD50) during organogenesis. The maternal toxicity of Sulfenamide TS was found at the highest dose of 450 mg/kg. Oral administration of this compound to female rats during organogenesis induced fetotoxic effects at doses of 50 mg/kg and higher. The dose-dependent increase in the frequency of fetuses/ litters with internal hydrocephalus was observed.

Epileptic seizures in subcortical vascular encephalopathy

Cerebrovascular disease is one of the most common causes of epilepsy in the elderly. Most of the studies published relate to cortical infarction, subarachnoid, and intracranial hemorrhage, whereas the incidence of epilepsy from subcortical ischemia, i.e. deep lacunar infarctions and diffuse white matter lesions, is obscure. Therefore, we prospectively examined 18 patients with the precisely defined diagnosis of subcortical vascular encephalopathy (SVE), who were admitted to our hospital due to epileptic seizures (group A), and compared them to a similarly selected group matched for age, sex, risk factors, and neurological deficits with an equivalent severity of SVE but without seizures (group B). Subcortical lacunar infarctions were significantly more frequent in group A than group B ( 15 18 versus 4 18 , p < 0.001), whereas neither the extension, degree, distribution of periventricular white matter changes, nor the presence of internal hydrocephalus, focal or diffuse cortical atrophy showed any statistical significance. However, a temporal constant theta or delta EEG focus was present in 10 18 patients in group A but only in 1 18 patients from group B (p ⩽ 0.005). 10 18 patients developed epilepsy with further seizures during follow-up. The association of SVE, multiple subcortical lacunas, and temporal EEG abnormalities are suggestive for an increased risk for epileptic seizures, which is particularly important for the treatment of patients with SVE if uncertain paroxysmal episodes occur, e.g. transient ischemic attacks, seizures, or cardiac syncope.

Lung Injury Leading to Respiratory Distress Syndrome in Young Dalmatian Dogs

A progressive pulmonary disease resulting in severe respiratory failure and death in an average of 3 weeks was diagnosed in 11 young Dalmatian dogs. The dogs were from 4 litters, all genetically related by a common ancestor. The initial clinical signs were tachypnea and noisy respiration. Respiratory distress developed shortly before death and was characterized by strenuous and rapid respirations, along with cyanosis and vomiting. On blood gas analysis, there were severe arterial hypoxemia, hypercapnia, and marked alveolar-arterial oxygen difference. Radiographically, a diffuse pattern of alveolar, interstitial, and peribronchial densities was observed in the lungs. Most dogs developed pneumomediastinum and gastroesophageal intussusception in the terminal phase of the disease. There was no response to treatment with antibiotics, corticosteroids, diuretics, or oxygen. At necropsy, the lungs were wet, heavy, and relatively airless. Absence of 1 kidney in 2 dogs and severe internal hydrocephalus in 2 dogs were additional necropsy findings. Pulmonary histopathology included metaplasia and atypia of the alveolar and bronchiolar epithelium, a nonpurulent inflammatory reaction characterized mainly by mononuclear cells and macrophages, eosinophilic hyaline membrane formation, and focal pulmonary fibrosis. The histological manifestations were typical of acute lung injury. Clinically, the findings were consistent with adult respiratory distress syndrome (ARDS), except for the relatively long course. No known risk factors for ARDS, such as trauma, toxin exposure, infection, or endotoxemia could be identified. The relationship of the other abnormalities (ie, renal aplasia, hydrocephalus) to the pulmonary disease also remains obscure. An inherited defect is suspected, because segregation analysis of the 4 litters suggests autosomal recessive inheritance.

Brain lesions in calves following transplacental infection with bovine-virus diarrhoea virus.

In 33 calves and subadult cattle of the Holstein-Friesian breed ranging from 1 to 210 days of age, the spectrum of brain lesions induced by intra-uterine infection with bovine-virus diarrhoea virus (BVDV) was retrospectively analysed. Of these, 27 animals originated from herds with a long history of BVD. Six calves were derived from dams vaccinated between the 90th and 118th day of gestation with a BVD live vaccine. The most frequent lesion was cerebellar hypoplasia, being present in 25 out of 33 (76%) of the animals. In most of these cases, cerebellar hypoplasia was associated with hydranencephaly, internal hydrocephalus, microencephaly or porencephaly. In cases with hydranencephaly, the fluid-filled cavities were devoid of ependymal lining. The lumina of the lateral ventricles of these cases were surrounded by glial fibrillary acidic protein (GFAP)-positive cells and a dense layer of immunoreactive cell processes. In the white matter adjacent to the dilated ventricular lumina, a reactive astrocytosis was present. Porencephalic cysts were surrounded by astrocytes with increased expression of GFAP and vimentin-positive cells and cell processes. In hydranencephalic brains, staining for neuron-specific enolase (NSE) revealed a marked reduction of NSE-positive nerve cells in cortical areas. In all six experimental cases and in several field cases with hydranencephaly or internal hydrocephalus, small groups of heterotopic NSE-positive neurons were present in the white matter of the cerebral hemispheres. In markedly hypoplastic cerebella, reduction of the cortical cell layers and degenerative changes in, and heterotopia of, Purkinje cells were found. In these cases, NSE- and neurofilament-positive cell processes were either markedly diminished or only remnants of immunoreactive cell processes were present. In five animals without significant gross cerebellar abnormalities, degenerative changes of Purkinje cells were found. Immunohistochemical staining using antibodies against glial and neuron-specific proteins on these brains, which represent postnatal end-stage lesions of BVDV-induced disturbances of the normal brain development, did not provide any insight into the possible pathogenetic mechanisms of these alterations. Application of immunohistochemistry, however, revealed changes, such as reactive astrocytosis and loss of nerve cell processes, which were not obvious on haematoxylin and eosin (H&E) stained sections.

Hydrozephalus eines Zwillings nach massivem Rückstrom in der Nabelarterie im 2. Trimenon

The case of a twin pregnancy with severe diastolic reverse flow in the umbilical artery of one of the twins at 23 weeks' gestation is reported. After delivery at 29 weeks' gestation, a congenital internal hydrocephalus was diagnosed in this twin. The most probable aetiology is an intrauterine periventricular haemorrhage. The severe diastolic reverse flow in the umbilical artery seems to represent a highly pathological flow pattern of prefinal degree, possibly leading to cerebral defects. The pathophysiology of the Doppler findings and the pathogenesis of the internal hydrocephalus are discussed.

A unifying theory for the definition and classification of hydrocephalus

If the cerebrospinal fluid (CSF) is considered to be all the fluid (liquid), other than blood or the derivatives of its breakdown, that is normally contained within the brain, its cavities, and its spaces, this could be regarded as "brain fluid" in its most elemental form. "Pathological increases in intracranial CSF volume, independent of hydrostatic or barometric pressure", then, could be considered a definition of hydrocephalus. The observation of significant episodic variation in intracranial pressure (ICP) suggests the necessity of substituting the concept of "time-related pressure variations" for the older one of "level of pressure" in patients with defective ICP control mechanisms. It has been assumed that the subarachnoid channels are the first CSF compartment to dilate in response to the hydrocephalic process, reducing the CSF pressure and thereby establishing an equilibrium. When the equilibrium is disturbed, with progressive dilation of the subarachnoid channels, the increase in CSF pressure is transmitted to the ventricular system, resulting in its dilation (extraprenchymal hydrocephalus). Progressive ventricular dilation causes cerebral edema (intraparenchymal hydrocephalus) and obliterates the subarachnoid spaces as the hemispheres are compressed against the dura, resulting in apparent "internal hydrocephalus" in the absence of "external hydrocephalus". Thus, subarachnoid space or ventricular dilation occur as a result of intermittent increases in extraparenchymal CSF volume: the primary pressure force emanating from the subarachnoid and subdural spaces and from the intraventricular compartment. Hydrocephalus, therefore, may be present in a child who does not yet have dilated ventricles but in whom both CSF volume and pressure are increased. Thus, it becomes obvious that the term internal hydrocephalus is of little significance, since increases in intraparenchymal fluid--cerebral edema--cause the same volumetric changes as increases in intraventricular fluid volume. I suggest that hydrocephalus is a pathologic increase in intracranial CSF ("brain fluid") volume, whether intra- or extraparenchymal, independent of hydrostatic or barometric pressure. It may be classified as (1) intraparenchymal (cerebral edema) and (2) extraparenchymal, with the extraparenchymal types subclassified into subarachnoid, cisternal, and intraventricular forms.

犬水頭症2例の脳波

Two dogs with internal hydrocephalus were examined by electroencephalography (EEG), which was characterized by diffuse and high voltage slow activities mixed with sharp waves, sometimes being not synchronious among tracings. One case showed poor correlation between the intrahemispheric different sites. In one case with clinical visual disorders, no response was seen in visual evoked potential. Autopsy revealed markedly dilated ventricles and thin cerebral cortices in the two cases.

鹿児島県に多発した子牛の中枢神経系先天異常の疫学と病理学的所見

From December 1990 to March 1992 in Kagoshima, 142 out of 275 calves with congenital defects had central nervous system disorders; 94 cases of hydranencephaly with visual disturbance, 21 cases of cerebellar hypoplasia, 23 cases of internal hydrocephalus, and 4 cases of only nervous signs. Most cases of hydranencephaly were born in February and March 1992 from dams at the first or second parity, and showed nonpurulent encephalitis, suggesting Akabane virus infection. Those with cerebellar hypoplasia mostly had severe ataxia and sacrificed early in life. Calves with internal hydrocephalus were nursed for rather long time in spite of retarded growth. No apparent lesions were seen in those only with nervous signs.

Echocardiographic proof of pulmonary hypertension with irreversible increased resistance in the pulmonary circulation as a complication after placement of a ventriculo-atrial shunt for internal hydrocephalus : E. Trowitsch, M. Ostrejz, D. Evers, et al. Eur J Pediatr Surg 2:361–364, (December), 1992

Echocardiographic proof of pulmonary hypertension with irreversible increased resistance in the pulmonary circulation as a complication after placement of a ventriculo-atrial shunt for internal hydrocephalus : E. Trowitsch, M. Ostrejz, D. Evers, et al. Eur J Pediatr Surg 2:361–364, (December), 1992

Surgical experience with defibrillator implantation using nonthoracotomy leads

Between October 10, 1989, and June 17, 1991, 109 patients with life-threatening ventricular arrhythmias received an implantable cardioverter-defibrillator at our institution. In 50 patients, nonthoracotomy lead systems consisting of a subcutaneous chest wall patch electrode near the cardiac apex and one (Cardiac Pacemakers, Inc: Endotak C) or two (Medtronic: Transvene) transvenous electrodes were tested. The mean ejection fraction was 0.44 ± 0.15 with a range from 0.19 to 0.81. Twenty-four patients had a history of open heart operation. In 43 patients the nonthoracotomy lead system was implanted, whereas in 7 patients epicardial patch lead systems were required due to elevated defibrillation thresholds during nonthoracotomy lead testing. There was one perioperative death unrelated to the operative procedure (hydrocephalus internus). Hemorrhage from the subcutaneous patch pocket or the device pocket that required reintervention occurred in 4 patients. During a mean follow-up of 13 ± 5 months (range, 4 to 26 months) no dislocation of an endocardial lead, insulation defect, or lead fracture has been observed. In 1 patient, the lead system had to be removed due to infection of the subcutaneous patch pocket. The cardiovascular survival rate was 98% at 12 and 18 months, and freedom from sudden cardiac death was 100% at 6 and 12 months. In conclusion, defibrillator implantation using nonthoracotomy lead systems is feasible with a success rate of 86%, and during short-term follow-up no problems related to the lead system such as dislocation, fracture, or insulation defect have occurred.