[Hydrocephalus].
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Biomedical subjects
Publications and source records attributed to W Serlo.
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The social status of 52 disabled young adults (29 men, 23 women) was reviewed at the age of 19 to 25 years, and compared with that of 209 age-matched controls. 40 had successfully completed elementary school and 10 had completed high-school. 28 had gone on to basic further education and 11 had progressed to more advanced levels. 35 per cent of the study group and 11 per cent of the controls had had no further education. Half of the study group were receiving disability benefit and 12 had no occupation outside the home. There was little difference in present employment status between groups, but the study group had experienced twice as much unemployment as controls. Leaving the parents' home and sexual experience were delayed among the study group. The results indicate the need for more practical support for these adolescents and their parents.
Head posture and dentofacial status of sixteen females and six males 12 to 34 years of age, who had been treated for scoliosis with a Boston-brace in their teens was investigated clinically and radiologically. When compared to an age and sex-matched sample the main findings were an increased craniocervical angle, particularly in frontal view; rotated orbital, maxillary and mandibular planes, a midline shift of the lower dental arch and a flattened atlas dorsal arch combined with an elongation of the dens axis. There was also a high prevalence of lateral malocclusions and developmentally missing premolars among the scoliotic patients.
Shunt function and complications in different etiologies of childhood hydrocephalus were studied in a series of 306 patients involving 1102 shunt operations. Shunts in patients with hydrocephalus caused by neoplasms proved to be most prone to shunt complications. The patency time for shunts in these patients was significantly shorter than for shunts in other patient categories [Standard number of deviations (SND) 5.9; P less than 0.001, Meyer-Kaplan life table analysis]. When the two main groups of infantile hydrocephalus-congenital obstructive hydrocephalus and hydrocephalus caused by perinatal intracerebral hemorrhage-were compared, the latter group proved to be significantly more prone to shunt infections (P less than 0.01), with an infection rate of 17.8% compared with 8.9% for the former group. The importance of this fact is stressed by the observation that these patients appear to constitute an increasing percentage of hydrocephalic patients. According to the present study, patients with congenital intracranial cysts and hydrocephalus are less prone to shunt complications, i.e., the infection rate is 6.8%, which is significantly less than that of patients with other types of hydrocephalus (P less than 0.01; chi-square test).
Epilepsy and epileptogenic activity in EEGs were studied in 168 shunt treated hydrocephalic (HC) children, the mean age at first operation 1.62 years (SD 1.87). 80 patients (47.6%) suffered from epileptic seizures during the follow-up period (mean 8.9 years). The epileptic seizures appeared before the initial shunting, and after the first shunt implantation in 43 (25.6%). There was no correlation between epilepsy and the aetiology of HC, number of shunt revisions, or shunt infections. Sixteen patients suffered from seizures during the neonatal period. Generalized spike and wave activity (SWA) was seen in EEG in eight out of these, and only one was seizure-free at the end of the follow-up period. All eight patients with epileptic seizures during the neonatal period without generalized SWA in EEG were seizure-free, however. SWA was seen in the first EEG prior to shunting in 75/168 patients (44.6%). All those patients who did not receive prophylactic medication, developed epileptic seizures, whereas 68.1% of those who received prophylactic anticonvulsive medication remained free of seizures. Partial epilepsy after shunting manifested itself in 15 patients, but this did not correlate with the side of the shunt or with the side of the SWA in the EEG. Slit ventricles (SLV) developed in 75 patients during the follow-up period, while the ventricles remained normal or dilated in 66 cases (27 patients had no CT follow-up). Epilepsy manifested itself in 8 out of these 141 patients (2 SLV, 6 non-SLV) during the first postoperative year, and in 29 patients in the SLV group and one in the non-SLV group at some time after the first postoperative year.
The incidence of slit ventricles of shunt treated hydrocephalic children was evaluated in a follow up study of 141 patients. Slit like ventricles on computer tomography was seen in 75 patients (53%). 52 patients (37%) suffered from clinical symptoms corresponding with overdrainage of cerebrospinal fluid. Those 52 patients with the "Slit Ventricle Syndrome" (SLVS) were treated by changing the valve to one with a higher opening pressure and/or adding an antisiphon device (ASD) to the shunt. 22 patients, initially treated by changing the opening pressure of the valve, needed the ASD later. Altogether 74 episodes of the SLVS were treated. The ASD proved reliable for the management of the SLVS. Normalization of ventricular size occurred in 54% of patients. whereas only in 15% treated without the ASD. Reduction of paroxysmal activity on EEG was seen in 70% of patients treated with the ASD, whereas only in 23% of patients treated without the ASD. Clinical relief of symptoms occurred in every patient, and ventricular catheter obstructions could be avoided, even if the ventricular size remained slit-like on CT.
Craniofacial morphology was studied in 45 shunt-treated hydrocephalic children and 7 untreated hydrocephalic patients. A sample of 74 normal children from northern Finland were used as controls. Following shunt treatment the sella turcica became shallow and J-shaped. The cranial base angles changed markedly during shunt treatment. The cranial base angles were more obtuse in untreated patients than in control subjects, whereas the opposite was the case in shunt treated patients. The Nasion-Sella-Basion angle was 143.4 degrees in untreated hydrocephalic patients, 132.6 degrees in normal subjects and 127.9 in shunt-treated hydrocephalics. The changes in cranial base angles appeared to be progressive during a two-year follow-up period.
47 hydrocephalic children (mean age 10.4 years) were examined on average 7.9 years after initial shunting. The etiology of hydrocephalus was classified into 5 groups as follows: perinatal intraventricular hemorrhage 19, congenital obstructive hydrocephalus 15, intracranial cysts 5, severe intracranial anomalies 4 and central nervous system infections 4 children. Audiological examination included pure-tone audiometry, tympanometry, registration of stapedius reflex thresholds and adaptation. A sensorineural high-frequency hearing loss was found in 18 (38%) of 47 examined shunt-treated hydrocephalic children, and 11 of the losses could be classified as the retrocochlear type. The differences of the mean hearing thresholds between the etiological groups of childhood hydrocephalus were minimal.
Muscular torticollis is a medically well-known condition that is usually diagnosed in early childhood and in which early surgical intervention is recommended to prevent the development of facial asymmetries. The purpose of this study is to examine head posture and possible dentofacial asymmetries in patients who have undergone surgical treatment for muscular torticollis in early childhood. Natural head position roentgenograms were taken in frontal projection, a clinical examination of oral status was performed, and dental casts were made. Marked craniofacial and dental asymmetries were observed, combined with a deviant head posture, in spite of surgical treatment for muscular torticollis earlier in childhood.
Overdrainage of the cerebrospinal fluid (CSF) and collapse of the ventricles, slit ventricles (SLV), can cause clinical symptoms and result in the slit ventricle syndrome (SLVS). The EEG changes and the frequency and type of epilepsy in patients with SLV was analysed from a material of 113 shunt-treated hydrocephalic children. During the follow-up time (mean 8.9 years), 63 patients (56%) had developed SLV. The age at initial shunting was significantly lower (1.2 years) in patients who developed SLV than for those who did not (2.7 years). After initial shunting generalized spike and sharp wave activity (SWA) developed more frequently in patients who developed SLV (81%) than in those who did not (54%). Severe generalized SWA developed almost entirely in patients in the SLV group. This severe generalized SWA disappeared from the EEG in patients after treatment of the SLVS. Epileptic seizures appeared after initial shunting in 44% of patients in the SLV group but in only 6% of the non-SLV group. Treatment of the SLVS decreased the frequency of epilepsy to a level corresponding with the non-SLV group. Repeated EEG evaluation of shunt-treated hydrocephalic children is a valuable aid in follow-up. If EEG abnormality appears after initial shunting, especially SWA, shunt malfunction and overdrainage of the CSF should be suspected.
Assessment of hydrocephalus shunt dysfunction, especially when partial, causes severe differential diagnostic problems. Ordinary computer assisted tomography gives only indirect information about shunt dynamics and the estimation of intraventricular pressure is vague. In a series of 50 valvographies, this examination proved to be especially valuable in the diagnosis of partial obstruction of the distal catheter. Valvography is also superior to other forms of examination in the localization of x-ray negative catheter types. In the slit ventricle syndrome valvography will reveal the position and function of ventricular catheters, which by other means would be impossible preoperatively.
An antenatal diagnosis of fetal hydrocephalus was made in 38 cases. Using certain criteria for the assessment of fetal prognosis, 23 cases were considered to be severely affected. Postnatal evaluation of these 23 cases established the extensive severity of fetal abnormality in all cases. The prognosis was estimated to be more favorable in 10 cases, of which 8 were delivered by elective cesarean section and 2 by spontaneous vaginal delivery. In 9 cases a ventriculoatrial shunting procedure was performed early in the neonatal period, while 1 case was treated conservatively. Follow-up of these 10 cases (at 7 months to 5 years of age) revealed normal or subnormal development in 6 cases and severe retardation in 4. Fetal hydrocephalus proved to have several etiological causes and was associated with other anomalies in 84% of cases. Severe forms of fetal hydrocephalus can, by means of modern ultrasound techniques, be detected before the 20th gestational week. Some cases of fetal hydrocephalus progress slowly during the fetal period. These can be followed until term by repeated ultrasound examinations and good or moderate prognosis can be expected with the use of early postnatal therapy. Only a minority of hydrocephalic fetuses seem to be potential objects for antenatal shunting.
Ventriculoperitoneal or ventriculoatrial shunting is the main method used in the management of hydrocephalic children. It is, however, associated with frequent complications. In order to reduce the risk of complications several types of shunts have been developed. In vitro studies have been performed to compare different types of shunts (1), but clinical studies concerning the efficiency of different shunt constructions are scanty (2). In the present study the Hakim-Cordis valve and the Pudenz-Heyer valve were compared in a series of 148 patients who had undergone 573 shunt operations for hydrocephalus. No significant differences were observed in the efficiency of these two shunts, though there was an increased rate of catheter rupture for the Pudenz-Heyer valve and an increased tendency for development of slit ventricles for the Hakim-Cordis valve. The patency rate for the Pudenz-Heyer valve proved to be statistically significantly higher (p less than 0.001) than that for the Hakim-Cordis valve, while there was a slight difference in the tolerance for high CSF protein levels favoring the Hakim-Cordis valve.
A total of 32 patients with overdrainage of CSF, fulfilling the radiological and clinical criteria for collapsed ventricles ("slit ventricles"), were classified into acute, subacute and chronic forms. The basis of classification into these categories was neurologic symptomatology. The majority (29 patients) originally had a ventriculoatrial shunt and 3 had ventriculoperitoneal shunts. Operative correction was performed in 23 patients (insertion of a high pressure valve in 18 and an antisiphon device in 5). Of these, 5 had acute, 10 subacute, and 6 chronic symptoms. Two patients without symptoms were operated on also. During the follow-up period, which varied from 2 to 11 months, no patient has shown recurrence of the original symptoms of the slit ventricle syndrome; two patients developed subacute signs and an antisiphon device was inserted in addition to the high-resistance valve. On the basis of this series, it is concluded that the slit ventricle syndrome can also develop in patients with an atrioventricular shunt and can be treated by preventing further overdrainage of CSF. Though the results are acceptable by present methods, the need for a servo-regulated shunt persists. The surgical correction should preferably be performed before the acute phase. A flowchart is presented for management of a child with suspected slit ventricles.
In three cases an antenatal diagnosis of isolated Dandy-Walker cyst was made at 22-28 weeks of gestation by ultrasound examination. Biparietal growth and the relative severity of the anomaly remained constant, so that all cases could be safely delivered at term. Postnatally, the diagnosis was confirmed during the first days of life by ultrasound and computed tomography (CT). Early neonatal shunting procedures were performed. Dual shunts were inserted in all three cases, and mental and physical development were observed to be normal during a follow-up of from 1.5 to 4.5 years. Early antenatal diagnosis of Dandy-Walker cyst may improve the prognosis and reduce the mortality, which has been reported to be 30%-48%. Dual shunting of both the cyst in the posterior fossa and the lateral ventricles has proved to be a safe and reliable method during the neonatal period.
Widely diverging opinions on the optimal therapy for intracranial cerebrospinal fluid cysts (CSF), mainly arachnoid cysts and the Dandy-Walker cysts, exist. Excision of the cyst walls in the treatment of the Dandy-Walker cyst has been replaced by shunting procedures, but the recommended method for primary treatment of arachnoid cysts in childhood is still cyst wall excision. Membrane excision is, however, often complicated by recurrence, subsequently requiring shunting-procedures. In a series of 19 cases primary shunting of intracranial CSF cysts proved to be a reliable method. In those cases where hydrocephalus (ventricular dilatation) is present at the time of the primary operation the ventricles should be shunted as well as the cyst. The catheter from the ventricle and that from the cyst should be connected to the same valve, otherwise an increased risk of intracranial herniation exists. The prognosis for infants and children suffering from intracranial CSF cysts is in general good; in 17 out of 19 cases mental development was normal and in 15 out of 19 motor development was normal. The risk of permanent motor damage seems to be particularly high when an arachnoid cyst is located on the quadrigeminal plate.
The data on all 881 primary or revision shunt operations performed on 158 paediatric patients treated in Gothenburg, Sweden from 1967 to 1984 and 101 patients treated in Oulu, Finland from 1968 to 1983 were pooled for the purpose of comparative evaluation of the function of ventriculoatrial (VA) and ventriculoperitoneal (VP) shunts. Ventriculoperitoneal shunting was the method of choice in Gothenburg and ventriculoatrial shunting in Oulu. The results of the 723 operations (305 VA and 418 VP shunts) were evaluated as the other 158 operations were for ventriculostomas, shunt removals and other procedures. 80 children had exclusively VA shunts and 133 children had exclusively VP shunts. Irrespective of the method of analysis the VP shunts were more frequently infected. The estimated relative risk for obstruction of the shunt (Meyer-Kaplan method) was shown to be significantly higher in VA shunts, but only at a low level of statistical significance (p less than 0.1). All other shunt complications were distributed uniformly in both groups. There was, however, a trend towards a higher mortality among children with exclusively VA shunts. Therefore it was concluded that despite the higher risk for infection in VP shunts, these still should be considered a safer choice, as the complications of VA shunts present greater risks.
The personality, psychosomatic symptoms and family characteristics of 55 shunted hydrocephalic children older than four years were studied. Hydrocephalic childrens' self-concept, measured by the Children's Appereception Test, was found to be very significantly poorer than that of the control children. They also frequently showed behaviour disorders of the MBD-type, e.g. concentration difficulties, aggressiveness, fastidious eating and nervousness. The hydrocephalic childrens' families showed very significantly more cohesion and less rigidity, and significantly less authoritarianism than average Finnish families. When examining the parents' attitudes to their sick child, one third of the children were seen to be in a healthy role, with parental expectations realistically related to the child's abilities. One third of the children were seen as "babies", with unnecessarily over-protective attitudes on the part of their parents, and one third as "scapegoats", with accusatory attitudes from their mother and father. Those in the role of "scapegoats" had the poorest perceptual skills, the highest frequencies of behaviour disorders and the poorest self-concept.