Showing papers in "Childs Nervous System in 1995"

Cerebral arteriovenous malformations in children. Management of 179 consecutive cases and review of the literature

Abstract: Over the past 10 years (1982-1992), we have been actively involved in the management of 179 cerebral arteriovenous malformations (CAVMs) in children and infants. Seventy-seven were true vein of Galen malformations (VGAMs) and 102 were pial AVMs (PAVMs), i.e., developed in the subpial space. Hemorrhage occurred as the first symptom in 50% of the children with pial AVMs, but was present in none of the VGAM cases. Only 31 children were found to be unsuitable for endovascular treatment, and in 124 cases embolization was indicated as the primary treatment (104 embolization performed). Only 21 children underwent a direct surgical approach (none in the VGAM group). In the embolized group in whom treatment has been completed (n = 56), 8 children died, 39 have an anatomical cure, and 34 are clinically normal. In the group under treatment (n = 48), 16 are not normal. The problems are timing and the aims (total or partial treatment) of the therapeutic procedures. In the nonembolized group (n = 31), 8/13 of the pial lesions were operated on (no mortality, 2 patients with moderate neurological deficits). In the VGAM group 13/18 died and 4 had spontaneous thrombosis (only 1 is neurologically normal). In the nonembolized group 13 lesions have been completely excluded, but only 5 patients are neurologically normal. This fact again stresses the need for prognostic evaluation before treatment and a clear definition of the treatment aims. Analysis of a large number of published series on the management of children with AVMs (1017 cases) reveals inconsistencies that hamper proper evaluation and comparison. In our experience, endovascular treatment always seems to be the best primary treatment in both VGAMs and PAVMs. However, management of children with these lesions requires a large multidisciplinary team, which is the only way of offering the most suitable and effective treatment, the sole guarantee of a good result.

Overdrainage and shunt technology. A critical comparison of programmable, hydrostatic and variable-resistance valves and flow-reducing devices.

Abstract: When vertical body position is simulated, conventional differential pressure valves show an absolutely unphysiological flow, which is 2–170 times the normal liquor production rate. Although this is compensated in part by the resistance of the silicon tubes, which may produce up to 94% of the resistance of the complete shunt system, a negative intracranial pressure (ICP) of up to 30–44 cmH2O is an unavoidable consequence, which can be followed by subdural hematomas, slit ventricles, and other well-known complications. Modern shunt technology offers programmable, hydrostatic, and “flow-controlled” valves and anti-siphon devices; we have tested 13 different designs from 7 manufacturers (56 specimens), using the “Heidelberg Valve Test Inventory” with 16 subtests. “Programmable” valves reduce, but cannot exclude, unphysiological flow rates: even in the highest position and in combination with a standard catheter typical programmable Medos-Hakim valves allow a flow of 93–232 ml/h, Sophy SU-8-valves 86–168 ml/h with 30 cmH2O. The effect of hydrostatic valves (Hakim-Lumbar, Chhabra) can be inactivated by movements of daily life. The weight of the metal balls in most valves was too low for adequate flow reduction. Antisiphon devices are highly dependent on external, i.e. subcutaneous, pressure which has unpredictable influences on shunt function, and clinically is sometimes followed by shunt insufficiency. Two new Orbis-Sigma valves showed relatively physiological flow rates even when the vertical position (30 cmH2O) was simulated. One showed an insufficient flow (5.7 ml/h), and one was primarily obstructed. These have by far the smallest outlet of all valves. Additionally, the ruby pin tends to stick. Therefore, a high susceptibility to obliterations and blockade is unavoidable. Encouraging results obtained in pediatric patients contrast with disappointing experiences in some German and Swedish hospitals, which suggests that our laboratory findings are confirmed by clinical results. The concept of strict flow limitation seems to be inadaequate for adult patients, who need a relatively high flow during (nocturnal) ICP crises. The problem of shunt overdrainage remains unsolved.

Intellectual outcome in children with malignant tumors of the posterior fossa: influence of the field of irradiation and quality of surgery

Abstract: The purpose of this study was to determine the respective parts played by cerebral hemisphere irradiation, posterior fossa irradiation, and surgery in the poor late functional results often observed in children treated for medulloblastoma. To do this we compared the intellectual outcome in a series of 59 children operated on for medulloblastoma, who had received whole-brain irradiation, to that observed in a series of 37 children operated on for ependymoma of the posterior fossa, who had received radiotherapy only on the posterior fossa. Only patients who had survived for more than 2 years without recurrence were included. At the assessment 1 year after treatment, intellectual outcome was somewhat better in the ependymoma group, but the difference was not statistically significant. At the long-term follow-ups at 5 and 10 years the results remained stable in the children treated for ependymoma, around 60% having an IQ above 90, whereas the intellectual level of the children treated for medulloblastoma was seen to have deteriorated progressively: 20% had an IQ above 90 5 years after treatment and only 10% at the 10-year followup. This progressive degradation is most likely due to the irradiation of the cerebral hemispheres, as this prophylactic irradiation constituted the only difference between the two groups. Moreover, irradiation to the posterior fossa did not seem to affect intellectual functions, since in the group of children with ependymomas the proportion of IQs above 90 was high and remained stable over the years. Surgery was certainly responsible for some poor results. The percentage of IQs above 90 observed 1–2 years after treatment was between 70 and 80 when no postoperative complications occurred, and only between 20–40% in the presence of postoperative complications. Postoperative aggravation was in most cases related to a brain-stem lesion. These results encourage the reduction, when possible, of irradiation to the cerebral hemispheres and underline the importance of the quality of surgery.

Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management.

Abstract: In the management of craniosynostosis subdural intracranial pressure (ICP) monitoring has proved a useful and safe means of identifying those children with raised ICP who are at risk from its long-term sequelae and who would benefit from early surgical intervention. Overnight subdural ICP recordings have been obtained in 136 unoperated cases of craniosynostosis. Fifteen patients were studied both before and after cranial vault remodelling procedures. ICP was raised (>15 mmHg) in 35%, borderline (10–15 mmHg) in 27% and normal (<10 mmHg) in 27% of cases. Raised ICP was present in 28/53 of the syndromic craniofacial dysostosis cases and in 20/83 non-syndromic craniosynostosis cases investigated (P<0.001). Raised mean ICP and periodic plateaux of sustained ICP during sleep were particularly associated with the syndromic cases. Of the 15 patients studied following cranial vault surgery, 9 showed a reduction in ICP, 3 were unchanged and 3 had higher ICP postoperatively. The results of ICP monitoring can contribute significantly to formulating a rational and staged surgical management plan incorporating the need to normalise ICP and correct the frequently severe functional and cosmetic consequences of these disorders.

Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils.

Abstract: This is an analysis of 19 consecutive cases of symptomatic patients with Chiari I deformities, undertaken to evaluate the long-term effect of posterior fossa decompression and duraplasty, assessed by postoperative imaging. Sixteen of the patients had syringomyelia and three had foramen magnum syndromes without a syrinx. Eighteen patients underwent posterior fossa craniectomy, subpial resection of the cerebellar tonsils, and duraplasty. Four patients were 16 years of age or younger. One of the children with syringomyelia had a posterior fossa decompression without resection of the tonsils. In the 15 patients with syringomyelia whose surgery included resection of the tonsils, the syrinx was reduced or resolved in 14. The patient whose syrinx did not change was a child with a lumbosacral lipoma. Three patients had syndromes of the foramen magnum without a syrinx, and of these only a patient with prior chemical and bacterial meningitis caused by a lumboureteral shunt failed to improve dramatically. When our patients are combined with 40 in the literature treated by decompression and duraplasty, 51 of 55 patients had reduction or resolution of the syrinx. Although it does not clearly affect the result, resection of the tonsils can be done safely.

Clinical and angiographic follow-up of childhood-onset moyamoya disease

Abstract: To clarify the differences between childhood-onset moyamoya disease and that with onset in adulthood, we studied the clinical course and angiographic findings of adult patients (over 20 years of age) with moyamoya disease of childhood onset (up to 15 years of age). The clinical course in 25 patients could be assessed. The follow-up period was 5-27 years. Neurological deficits were noted in 11/23 and mental disorders in 9/21. In all except one, the illness had started before the age of 7 years. Neither neurological nor mental condition changed during or after adolescence (15-20 years of age). Two patients died of intracranial hemorrhage. The disease progressed in angiographic stage until adolescence, but had stabilized or almost stabilized by the age of 20 years. This study indicates that moyamoya disease with onset in childhood carries high morbidity and mortality. The disease advances in angiographic stage between childhood and adolescence, but stabilizes or almost stabilizes between adolescence and adulthood.

Transient mutism following a posterior fossa approach to cerebellar tumors in children: a critical review of the literature.

Abstract: Transient mutism has been known as a rare complication following a posterior fossa approach to cerebellar tumors and its cause has not been clearly elucidated. The cerebellar mutism is not accompanied by cranial nerve deficits and disorders of consciousness. Since 1985 only 23 cases of mutism following removal of a cerebellar tumor in children have been reported in the literature. Two additional cases have been operated upon in our department. Extensive injury to the vermian and paravermian cerebellar area, involving the hemispheric cortex, cerebellar peduncles, fibers from the dentato-thalamocortical pathway, and dentate and interpositum nuclei may be the most important anatomical substrate of mutism. The mechanism of such transient mutism seems to be a complex of two or more factors (vascular disturbances due to manipulation or retraction of the cerebellar region around the IV ventricle and emotional factors). On the basis of these 25 cases the major features of the cerebellar mutism are discussed.

Regional cerebral hemodynamics in childhood moyamoya disease.

Abstract: Regional cerebral blood flow (rCBF) and its reactivity to acetazolamide were analyzed in a series of 15 cases of childhood moyamoya disease, using the xenon-133 inhalation method and single photon emission CT (SPECT). Most of the patients had normal mean hemispheric cerebral blood flow (mCBF), comparable to that of age-matched healthy children. However, they had abnormal rCBF distribution and disturbed reactivity to acetazolamide in the territory of the internal carotid artery, especially in the frontal lobe. Surgical revascularization for the anterior circulation, which consisted of STA-MCA anastomosis and indirect synangiosis, dramatically improved these cerebral hemodynamics in the anterior circulation, including the frontal lobe, and reactivity to acetazolamide in the whole brain. These results suggest that surgical revascularization for the anterior circulation should be carried out in order to improve cerebral hemodynamics as widely as possible, especially in the frontal lobe, and may improve cerebral perfusion reserve in the posterior circulation.

Cerebrospinal fluid shunt infections in infants

Abstract: Infection remains a major cause of morbidity and mortality following CSF shunt procedures. In this study 191 shunt procedures carried out from January 1981 to December 1992 in a series of 81 infants (less than 6 months old) were retrospectively analyzed for possible risk factors. The overall surgical infection rate was 7.8%, with 15 infections occurring in 14 patients (17.2%). No significant difference in the rate of infections was found in relation to sex, birth weight, gestational age, and type of shunt procedure (primary insertion/revision). The occurrence of other infections during the period of shunt surgery did not influence the infection risk either. Intraventricular hemorrhage and central nervous system infections as causes of the hydrocephalus were found to correlate with septic risk. Young age (less than 6 months) seems to represent the main risk factor, and this is related both to the immunologic deficiency and to the particular features of residential bacterial flora in this age group.

Long-term follow-up in 233 patients with congenital hydrocephalus

Abstract: Data on 233 children with congenital hydrocephalus who were operated on in our department from 1964 to 1984 were analyzed. The study was started in January 1990 and was based on questionnaire followed by a psychological examination. Thirty-two patients (13.7%) died during the follow-up period. The average number of operations during the observation period was 2.7; in 166 patients (=71.2%) the shunt had to be revised at least once. The reason for revision was a mechanical problem in 79.4% and infection in 15.5%. The psychological evaluation in 115 patients showed a normal performance in 62.8%, while 29.8% had mild retardation, evident in speech impairment and/or impaired speech development, in memory and concentration, in poor intellectual performance and decreased performance in school. Severe retardation was seen in 7.4% of cases. Our results showed that the best outcome can be achieved with early shunt implantation. We did not, however, find any correlation between shunt revision rate and psychological results.

Symptomatic intracranial hemorrhage in full-term infants

Abstract: A retrospective analysis was undertaken in a consecutive series of 33 full-term infants (birth weight >2500 g and a minimum of 37 weeks gestational age) with symptomatic intracranial hemorhage (ICH) admitted to a regional neonatal intensive care unit from January 1986 to December 1992. Eleven infants were born in our institution; 17 were male. The estimated local incidence of symptomatic ICH for the inborn population was 4.9/10 000 live births, with a regional incidence of 2.7/10 000 live births. Twenty-four (72.3%) infants presented with seizures, apnea, or respiratory distress. Five (15.1%) children developed ICH associated with extracorporeal membrane oxygenation, ventriculoperitoneal shunting, and cardiac surgery. There were two deaths (6.1%) associated with a grade IV periventricular hemorrhage (PVH) and cardiac surgery. Nine infants (27.3%) showed PVH, while an additional nine children developed multifocal cortical hemorrhages. Eight infants (24.%) showed extraxial ICH, four children (12.1%) sustained lobar hemorrhages, and three children (9.1%) showed ICH associated with prenatal CNS abnormalities. Excluding five children with iatrogenic ICH, coagulopathies occurred in 9 of 28 infants (32.1%) and constituted a major determinant of the development of ICH. Neurosurgical intervention was limited to one infant with massive ICH and one child with hydrocephalus as a late sequela of ICH. Developmental follow-up was complete in 32 children with a mean and median duration of 3.4 years. Full-term infants with ICH associated with risk factors for hypoxic-ischemic injury showed a significantly greater risk of developmental delay compared to infants with uncomplicated ICH.

Cerebellar astrocytoma of childhood : long-term follow-up

Abstract: Cerebellar astrocytoma of childhood has always been regarded as a benign tumour. Certain issues still attract debate, in particular the role of radiotherapy as an adjuvant to surgery, management of the cyst wall in cystic lesions and treatment of recurrent tumours. To provide some answers to these questions, the experience in Birmingham (United Kingdom) between the years 1959 and 1991 was reviewed. Ninety-seven patients aged 0-14 years had been treated and the histological material was reassessed independently. Detailed statistical and clinical analysis revealed that the main factor of negative prognostic value was the presence of brain stem involvement. In cystic tumours the surgical excision of the cyst wall did not offer significant advantage on survival. Similarly, postoperative radiotherapy did not improve survival, and there is a suggestion that it may actually predispose to malignant transformation. The implications on management are discussed.

Nongalenic cerebral arteriovenous malformations in neonates and infants. Review of 26 consecutive cases (1982-1992).

Abstract: We present 26 consecutive cases of nongalenic pial arteriovenous malformations (PAVMs) diagnosed in the neonatal period or in infancy. No diagnosis was made antenatally. Presenting symptoms in neonates were systemic cardiac manifestations (54%), seizures (31%), and hemorrhages (15%). In infants, hemorrhagic strokes and hydrodynamic disorders (external or internal hydrocephaly, macrocephaly, atrophy) both occurred in 38% of cases. Systemic cardiac manifestations and seizures were rare at that age (respectively 16% and 8%). Sixty-two percent of neonates and 31% of infants already had neurocognitive disorders (assessed by pediatric neurocognitive testing: Brunet-Leizine and Denver tests) when referred. The venous drainage and its anomalies (ectasias, stenoses, thromboses) were the main causes of symptoms. Atrophy and leukomalacic lesions occurred rapidly; they express local hydrovenous disorders and are specific to this population group. Untreated neonates and infants have a poor prognosis. Endovascular treatment, although partial and challenging in all instances, represents the treatment of choice in our series. Of the eight neonates treated, one improved to normal (12.5%), while four remained stable (50%): two neurologically normal, two with mild neurological deficit. Three (37%) died despite embolization (heart failure, multiorgan failure, postoperative death). Transient neurological complications occurred in two cases (25%): hemiparesis in one patient with a rolandic and in one with a thalamic AVM. Of the eight infants successfully embolized, one was significantly improved (12.5%) and is now neurologically normal, while five remained stable (62.5%): four neurologically normal, one with mild neurological deficit. One died between two sessions of embolization from intracerebral hemorrhage (12.5%). Hemianopsy occurred in one case (12.5%) after embolization of an occipital AVM. In one additional case in a normal child we failed to embolize the last small pial AVM of four after the three others had spontaneously thrombosed. With a minimal follow-up of 18 months and a maximum of 7 years, the review of our series shows 53% of the initial group of neonates and infants growing neurologically normal after therapeutic management in our institution; 23.5% died despite treatment, and the remaining 23.5% present minor neurological deficit. When targeted at the points of angioarchitectural weakness, embolization contributes to stabilizing a lesion. It should be undertaken rapidly to avoid loss of brain substance secondary to hemorrhage, atrophy, or leukomalacia, and to allow neurocognitive recovery and normal brain maturation. In our experience, these lesions are the most aggressive ones for the maturing brain, and the most difficult to approach technically. They represent a new therapeutic field and have their own specific anatomy and physiology.

Value of transcranial Doppler indices in predicting raised ICP in infantile hydrocephalus. A study with review of the literature.

Abstract: Cerebral hemodynamic changes in infants with progressive hydrocephalus have been studied with the transcranial Doppler (TCD) technique. Several authors have referred to the correlation between the hemodynamic changes and increased intracranial pressure (ICP). Despite conflicting conclusions on the value of pulsatility index (PI) and resistance index (RI) measurements for monitoring infantile hydrocephalus, these pulsatility indices are the most commonly used for this purpose. Although clinical signs of raised ICP are highly variable and unreliable in infants, assumptions have been made in most of the studies about the presence of elevated ICP on the basis of the patient's clinical state. Few studies have reported on actual ICP values, however, and a direct relationship between ICP and TCD changes has never been adequately demonstrated. In the present study, this relationship was investigated in long-term simultaneous TCD/ICP measurements, in an attempt to develop a noninvasive method of monitoring the effect of ICP on intracranial hemodynamics. Two groups of data sets were established. Group I consisted of pre- and postoperative (shunt implantation) TCD/ICP measurements. Group II were long-term simultaneous TCD/ICP recordings showing significant ICP variations. In most of the postoperative measurements there was a decrease in the average PI and RI values. The correlation between PI or RI and ICP in the long-term simultaneous recordings, however, was generally poor. The risk of obtaining false positive or false negative PI or RI values in short-term measurements was also demonstrated. It can be concluded from our results, besides the wide range of reference values for the Doppler indices and extracranial influences upon them, that the present Doppler indices are inadequate for monitoring the complex intracranial dynamic responses in patients with raised ICP.

Supratentorial interhemispheric cysts associated with callosal agenesis: surgical treatment and outcome in 16 children

Abstract: Cerebrospinal-fluid-filled interhemispheric cysts associated with callosal agenesis are relatively rare lesions, and have been subject to a varied and confusing terminology. From a pragmatic surgical point of view, we believe that the dorsal III ventricular cyst [35] and the giant interhemispheric cyst [23], although of different embryological origin, can be classified as one group. The most important condition that must be distinguished from interhemispheric cysts is the alobar form of holoprosencephaly. We describe the clinical symptomatology in 16 children who were surgically treated with a cysto-peritoneal shunt. The outcome, both neurologically and developmentally, was good in the large majority of cases, and compared favorably to similar cases in the older literature. It therefore seems reasonable, at the present state of knowledge and until further studies clarify the origin and natural history of these cysts, to treat them as early as possible in order to prevent gross developmental deficits.

Radiotherapy in the treatment of low-grade astrocytomas. II. The physical and cognitive sequelae.

Abstract: The purpose of the present study was to define the late effects, both physical and psychological, of treating low-grade astrocytomas with radiotherapy. Fifty patients, half of whom received radiotherapy, underwent an assessment of neurological and neuropsychological function. There was no difference in neurological function between the two groups. The radiotherapy recipients, including those with cerebellar tumours, performed significantly worse on measures of intelligence and information processing. In addition, there was a greater incidence of special education needs in the irradiated group. We conclude that children with low-grade astrocytomas who receive radiotherapy have no greater neurological deficit but that the use of radiotherapy carries a penalty in terms of long-term cognitive function and confirmed the findings of many previous reports that supratentorial irradiation is detrimental. More surprisingly, it has been demonstrated that local field irradiation to the posterior fossa can also produce significant cognitive impairment.

Radiotherapy in the treatment of low-grade astrocytomas. I. A survival analysis.

Abstract: Between 1954 and 1986 inclusive, 160 children in the North West Region of England were registered with histologically proven lowgrade astrocytomas (grade 1 or 2). Ten died before receiving any treatment, and a further seven died within 28 days of surgery, leaving 143 children whose survival in relation to treatment modality is the subject of this paper. Low-grade astrocytomas are responsive to radiation therapy. This treatment has no clear benefit to offer children with superficial tumours that can be resected completely or nearly so, but significantly improves survival rates when tumours are deep-seated and not amenable to excision.

Cerebral hydatid cyst in children. Experience of 27 cases.

Abstract: The authors present 27 cases of cerebral hydatid cyst (CHCy) treated between 1980 and 1992. These cases of CHCy represent 2.8% of all cases of expansive nontraumatic lesions in children. Most of the patients were between 6 and 10 years of age. There was a substantial prevalence of male patients — 18 cases (66.6%). Most of the children with CHCy were from rural areas. The cysts were all located in the cerebral hemispheres (none in the posterior fossa). Usually two or three lobes were affected and the cysts were most often retrorolandic. Only 8 patients (29.6%) also had pulmonary or hepatic infestation. All patients were operated on immediately the diagnosis was established. Operative mortality was very low (1 patient died immediately after surgery). There have been 11 cases of recurrence; all these patients have been reoperated on. Neurological sequelae were mainly partial and general seizures. Epilepsy developed postoperatively in five patients. Paresis, which usually disappears in time, was the most common motor disturbance. Two other important postoperative complications were subdural effusion and ventricular dilatation (six cases). There have been five cases of multiple recurrences (four of these patients have since died). Treatment with albendazole was used in three cases without significant results.

Surgical treatment of intraventricular tumors associated with tuberous sclerosis.

Abstract: Six children with intraventricular tumors associated with tuberous sclerosis (TS) were treated at the Children's Health Center, Department of Pediatric Neurosurgery, in the period 1987–1992. The age of the patients ranged from 7 to 15 years. TS was diagnosed according to Gomez diagnostic criteria. Computer tomography (CT) and magnetic resonance imaging showed intraventricular tumors associated with ventricular enlargement and multiple subependymal nodules commonly observed in cases of TS. All tumors were removed totally through frontal transcortical approach, with uneventful postoperative recovery. One patient, with two parallel tumors in the two frontal horns, underwent one-stage surgery with successful total removal. Histopathological examination in all cases showed subependymal giant cell astrocytoma (SGCA). The growth pattern of SGCA associated with TS, documented by sequential CT scans over several years, is described. The diagnosis and surgical treatment of the tumor are discussed, and periodic CT scanning, at least every 2 years, is recommended for patients with TS.

Treatment of hydrocephalus in patients with meningomyelocele or encephalocele: a recent series.

Abstract: Meningomyelocele/encephalocele with associated ventriculomegaly can be treated as a single-stage procedure (i.e., both lesions treated simultaneous) or as two-stage procedures (i.e., each lesion treated at a separate time). A delay in closure of the meningomyelocele/encephalocele is associated with a higher incidence of ventriculitis/ventricular shunt infection-particularly when closure is performed more than 36 h after birth. In these situations, closure followed by surveillance cultures, appropriate antibiotics, ventricular drainage, and then delayed ventricular shunting seems more reasonable.

Cerebral hemodynamics and “re-build-up” phenomenon on electroencephalogram in children with moyamoya disease

Abstract: “Re-build-up” phenomenon, induced by hyperventilation, is a characteristic finding on electroencephalogram (EEG) in children with moyamoya disease, and suggests close correlation with cerebral ischemic attack. Its mechanism, however, remains obscure. In this study, we examined the relationship between the cerebral hemodynamics and this phenomenon before and after vascular reconstructive surgery. Regional cerebral blood flow and its reactivity to acetazolamide were analyzed using the xenon-133 inhalation method and single photon emission computed tomography in six children with moyamoya disease. These results were compared with the EEG. Regional vasoreactivity to acetazolamide was significantly lower in “re-build-up”-positive regions than in “re-build-up”-negative regions. Postoperative studies revealed a significant improvement of reactivity in the regions where the “re-build-up” phenomenon disappeared after surgery. These results suggest that the “re-build-up” phenomenon represents a focal reduction of the cerebral perfusion reserve in moyamoya disease.

Selective posterior rhizotomy for children with cerebral palsy: a 7-year experience.

Abstract: Although selective posterior rhizotomy (SPR) was pioneered as early as 1913, only over the past decade has the procedure gained popular use for the treatment of spasticity in cerebral palsy. The medical knowledge base regarding this procedure is expanding, and surgical techniques continue to be revised. We present our 7 years of experience in treating spastic cerebral palsy using SPR. The aspects of preoperative evaluation used by the multidisciplinary team to determine candidacy are outlined. The surgical procedure is detailed with a particular emphasis on the role of intraoperative nerve root stimulation to aid in selection for rootlet sectioning. Historical nerve stimulation protocols are reviewed and compared to our findings over the years. The functional goals are discussed in the context of the postoperative evaluation and therapies. Specific outcome in relation to joint range of motion, self care tasks, and ambulation is reported. The paper outlines a concise overview of our experiences and will assist the clinician in defining a protocol and expectations for SPR.

Ventriculosubgaleal shunt: a treatment option for progressive posthemorrhagic hydrocephalus

Abstract: Among premature infants born at less than 1500 g, the incidence of intraventricular hemorrhage is greater than 45%. Of these, 40% will develop progressive posthemorrhagic hydrocephalus (PPHH). Optimum treatment remains controversial. Ventriculosubgaleal (VSG) shunts were first proposed as a means of temporarily diverting cerebrospinal fluid (CSF) in a more physiological manner for those infants less than 1500 g in weight who would not tolerate a ventriculoperitoneal (VP) shunt. The VSG shunt could then be converted into a VP shunt when the infant had gained the desired weight. Despite favorable reports, the procedure has not gained universal acceptance and is unknown to many neurosurgeons. The present authors report a series of 15 patients who had VSG shunts inserted with excellent temporary CSF diversion and no complications. Furthermore, 3 out of the 15 patients required no further treatment. We suggest that VSG shunting is a safe and effective means of treating the premature infant with PPHH.

Intracranial pressure and cerebral arterial flow velocity indices in childhood hydrocephalus: current review

Abstract: Because of its noninvasive and repeatable nature, Doppler ultrasound has been increasingly used to assess changes in cerebral haemodynamics in infants and children with hydrocephalus. There is general agreement that a direct correlation exists between the intracranial pressure (from experimental, fontanometric and direct measurement evidence) and the resistance index. In addition, this increasing index has been predominantly due to a reduction in the end-diastolic velocity. Stable ventriculomegaly is associated with normal pulsatility. The cerebral blood flow velocity parameters change significantly following CSF drainage by tapping or shunting. The measurement of intracranial pressure and cerebral blood flow velocity are currently the best ways of assessing the need for CSF diversion and monitoring subsequent shunt function.

Enlarging skull fractures in children

Abstract: Large skull fractures are conventionally followed radiographically until healing occurs. Fractures which enlarge or remain unhealed are commonly termed “leptomeningeal cysts” or “growing skull fractures”. This study of ten children with this injury and a review of the literature shows that a true leptomeningeal cyst is seldom present and that skull fractures do not “grow”. Moreover, careful history-taking and physical examination will correctly identify all enlarging or unhealed skull fractures of childhood without the need for plain radiographs or computed tomography of the skull.

Congenital dermal sinus associated with an abscessed intramedullary epidermoid cyst in a child: case report and review of the literature

Abstract: A 16-month-old male infant presented with paraparesis and a high thoracic skin dimple After myelography the child underwent complete surgical removal of the dermal sinus and of an abscessed tumor located within the cervicothoracic cord Histological study showed that the lesion was an intramedullary epidermoid cyst Only four descriptions have previously been published of dermal sinuses associated with intramedullary epidermoids, three of them complicated by intramedullary abscesses This appears to be the first report in the current literature of the association of a dermal sinus and an infected intramedullary epidermoid occurring out of the confines of the lumbosacral region

Imaging of childhood torticollis due to atlanto-axial rotatory fixation.

Abstract: Atlanto-axial rotatory fixation is a rare cause of childhood torticollis. It may occur spontaneously or may be associated with trauma, upper respiratory tract infection or congenital abnormality of the cervical spine. Presentation is usually with persistent torticollis and "cock robin" deformity of the neck. In this paper the radiological experience in three patients is presented. Investigations included plain radiographs, plain anteroposterior tomography, CT and MRI. Displacement of the lateral mass of the atlas and the eccentric position of the odontoid peg can be seen in the plain films. CT scan can exclude fractures and confirm atlanto-axial rotation. The superimposition of CT images is demonstrated as a way of diagnosing subluxation. MRI offers better soft tissue differentiation and allows assessment of the integrity of the transverse ligament. This has an important bearing on the prognosis and may influence surgical treatment. It is important to recognise the plain film features of this uncommon condition and confirm the diagnosis with CT or MRI. The treatment options are discussed with particular reference to long term outcome.

Chronic ("normal pressure") hydrocephalus in childhood and adolescence. A review of 16 cases and reappraisal of the syndrome.

Abstract: “Normal pressure” hydrocephalus (NPH) is generally considered to be a disorder of the adult and geriatric population. Only a few reports have described the possible occurrence of this condition in children. A series of 16 patients aged less than 20 years forms the basis of the present report. Among these 16 patients, 11 had a clearly identified etiologic factor and 7 had had a shunt previously implanted. The majority of patients exhibited at least two elements of the adult's triad of psychomotor retardation (14 cases) and/or psychotic-like symptoms (4 cases), gait anomalies (8 cases), and sphincter disturbances (3 cases). Six patients had their intracranial pressure (ICP) monitored. ICP values were estimated to be within the normal limits for age. All the 16 patients underwent shunting or shunt revision. Surgical results were as follows (mean follow-up 20±17.2 months): a good response to shunting was obtained in 12 cases (“cured”: 5, improved: 7), while the other 4 patients failed to improve. It seems likely that associated parenchymal disorders have played a major role in therapeutic failures. In children showing ventricular dilation on computed tomographic (CT) analysis and a clinical picture of subtle psychomotor deterioration, it may be difficult to distinguish an active disorder of the CSF dynamics from “arrested hydrocephalus.” Since intracranial manometry cannot be undertaken as a routine procedure, less invasive methods such as cerebrospinal fluid (CSF) tap test, psychometric or urodynamic tests deserve special attention as reliable predictors of outcome after shunting. Because most patients undergo shunting without prior assessment of their CSF pressure, the term “chronic hydrocephalus” is proposed as an alternative designation to “NPH,” since there is little argument for maintaining an instrumentally based definition of the syndrome.

Acquired torticollis as the only presenting symptom in children with posterior fossa tumors.

Abstract: Acquired torticollis is a symptom of an underlying disorder. It requires a thorough, meticulous search for the cause, because some of the problems associated with torticollis, such as posterior fossa tumor, can be life-threatening.

Horner's syndrome due to jugular venous ectasia.

Abstract: Horner's syndrome may be caused by a lesion anywhere along the three-neuron pathway from the hypothalamus to the eye. This syndrome has been reported secondary to numerous causes to date. In this paper, we describe an extremely rare case of Horner's syndrome due to jugular venous extasia demonstrated by computed tomography and intravenous digital subtraction angiography.