Paediatric head trauma: influence of age and sex

A consecutive unselected series of 1812 children (up to 15 years old) admitted for head injuries over a period of 8.5 years was studied. The cases were divided up according to five categories of pathology: benign injury, extradural haematoma, subdural haematoma, open brain laceration and brain contusion in a broad sense. All cases of benign injury were from the Geneva area (57 000 children) and 52% of the cases of severe injury were referred from other places. To these 1812 cases were added those of 23 children who died before admission recorded by the police. In the Geneva area the mortality was 6.8/100 000 per year. Patients were divided into three age groups: I (0–3 years), II (3–9 years), and III (9–15 years); group I was further subdivided into subgroups I a (0–1 year) and I b (1–3 years). The incidence of each type of accident was calculated for each age group, separately for girls and boys. Each type of pathology was correlated, sex by sex and for different ages, with the type of accident. Overall, two boys were injured for each girl. Road accidents were responsible for 15% of head injuries in group I girls, 17% in group I boys, 43% in group II girls, 45% in group II boys, 50% in group III boys and 61% in group III girls. They were responsible for 94% of all deaths and 85% of deaths of hospitalized patients. Falling was the most frequent cause of injury. Benign injuries were more frequent in group I. Only 1 of 25 patients with extradural haematomas died, and there were only 8 patients with subdural haematomas, 4 in subgroup I a (babies aged less than 1 year).     

Do children with severe head injury benefit from intensive care?

Intensive management of severe closed head injury is pursued in most neurosurgical units, as it no doubt reduces mortality. However, the fate of those salvaged from death is less clearly defined. The impact of aggressive management on the psychological recovery is even less clear. This retrospective study of children admitted to a regional paediatric neurosurgical service attempts to address these issues. Eighty-four children between the ages of 3 to 16 years with severe head injuries were studied and categorized according to conservative or intensive treatment groups. The availability of intensive management led to a greater number of such children referred. The mortality in those children sustaining more severe injury, i.e. Glasgow Coma Score (GCS) 3 or 4, was halved (44%) with intensive management from 80% in the conservative group. For those with less severe injury (GCS 5–8), it was unchanged at about 23%. The reduced mortality is at the expense of increased morbidity. Psychological testing showed that the cognitive recovery was comparable.     

Extradural haemorrhage in infancy and childhood

One hundred and two paediatric cases of extradural haemorrhage (EDH) were treated in Adelaide, South Australia, during the period 1954–1988; 10 were infants (0–2 years) and 92 were children (2–14 years). There were 9 deaths (mortality 8.8%). Long-term disabilities severe enough to interfere with school and/or employment were seen in 8 (7.8%) survivors. This relatively low number of adverse outcomes is partly an expression of a low incidence (5.9%) of associated intradural haematomas and few high-velocity impacts due to vehicular accidents. There is reason to believe that the results of treatment have improved in the decade 1977–1988. We attribute this in part to early diagnosis by computed tomography (CT), but a contributory factor may be earlier referrals from country centres to a paediatric trauma centre and rapid transfer, by air or road, by medical retrieval teams.The substance of this paper was presented to the 12th Congress of the European Society for Paediatric Neurosurgery, Warsaw 1990     

Pediatric head injuries caused by traffic accidents

In an unselected series of 488 patients with head injuries referred to a general surgical department, there were 126 children aged 0–19 years whose head injuries were a result of traffic accidents. In age group 0–4 years, only 23% of the head injuries were due to traffic accidents. In age groups 5–9, 10–14, and 15–19 years, however, traffic accidents were the main cause of the injuries, being responsible for 47%, 65% and 82% of the cases respectively. Both age groups 5–9 and 10–14 years had an unusually high proportion of bicycle injuries, while motorcycle and automobile accidents were the leading causes of injury in age group 15–19 years. Eight children (6%) died as a result of head trauma. Furthermore, among the survivors there were 8 children with severe head injuries (post-traumatic amnesia lasting 24 h), the rest being minor head injuries. All the survivors but one returned to school and achieved reasonable performances. Repeated follow-up studies at 3 months, 1 and 5 years, including interviews with the parents, disclosed that several of the children had headache, dizziness and other complaints. These subjective complaints subsided with time, but with different patterns, in the younger and older age groups. It is concluded that the postconcussional syndrome is not uncommon in children, but it may be better tolerated and resolves more completely with time than in adults. Eight children (7%) had one seizure or more during the 5-year follow-up period.     

Increasing frequency of multiple sclerosis in Padova,Italy: a 30 year epidemiological survey

OBJECTIVE: To determine the incidence and prevalence rates of multiple sclerosis (MS) and their temporal profiles over the last 30 years in the province of Padova (northeast Italy). BACKGROUND: In the early 1970s an epidemiological survey in the province of Padova showed a MS prevalence and incidence of 16/100 000 and 0.9/100 000 population, respectively; these figures are much lower than current estimates in other regions of Italy and Central Europe. METHODS: The population of the study area was approximately 820 000 (422 028 women, 398 290 men) in the 1991 census. All possible sources of case collection were used, but only clinically definite/probable and laboratory-supported definite/probable MS were considered in the analysis of incidence and prevalence trends from 1971 to 1999. RESULTS: On 31 December 1999, the crude prevalence rate was 80.5/100 000 (95% CI 70.3-90.7); prevalence was higher in women (111.1/100 000; 95% CI 99.0-123.1) than in men (49.7/100 000; 95% CI 41.3-58.1). This difference was significant (F/M = 2.43; z = 10.1, P < 0,00001); a rate adjusted for the European population was 81.4/100 000. On 31 December 1980 and on 31 December 1990 the estimated prevalence rates were 18/100 000 and 45.7/100 000, respectively. Thus, a fivefold increase in prevalence was observed from the 1970s. The mean annual incidence was 2.2/100 000 in the period 1980-89, 3.9 in the period 1990-94 and 4.2 in the period 1995 99. Thus, incidence increased more than fourfold from the 1970s through 1994 and remained quite stable in the last several years. Mean age at onset was 31.3 +/- 9.88 years. Mean diagnostic latency decreased significantly from 49.2 +/- 44.5 months in 1985 to 23.0 +/- 30.3 months in 1990, 12.9 +/- 15.61 in 1995 and 5.3 +/- 4.7 in 1999. CONCLUSIONS: The actual prevalence (80.5/100 000) and incidence (4.2/100 000) of MS in the province of Padova agree with the most recent epidemiological estimates/trends observed in other Italian and European areas, except for Sardinia and Scotland. The increase in both incidence and prevalence rates observed in much of this region over the last 30 years parallels the introduction of more sensitive diagnostic techniques and a highly significant decrease in diagnostic latency. These findings probably do not support a real increase in the frequency of MS in northeast Italy because recent estimates of incidence have increased only slightly (3.9 to 4.2, which is < 10% in five years) and increase in the prevalence rate was almost completely due to the accumulation of new incidence cases.     

Epidemiological study of Guillain-Barré syndrome in south east England

OBJECTIVES—To determine the incidence, treatment,and outcome of Guillain-Barré syndrome in south east England.
METHODS—Patients presenting with confirmedGuillain-Barré syndrome between 1 July 1993 and 30 June 1994 wererecruited via a voluntary reporting scheme coordinated by the BritishNeurological Surveillance Unit, hospital activity data collected fromacute admitting hospitals within the South East and South West ThamesRegional Health Authorities, death certificates, and a contemporaryresearch study of Guillain-Barré syndrome and Campylobacterjejuni infection. All patients were followed up for one year todetermine outcome.
RESULTS—Seventy nine patients were recruited, 35 (44%) male, 44 (56%) female, including three children (two boys, onegirl). The crude (95% confidence interval (95% CI)) annual incidencewas 1.2 (0.9-1.4) cases/100 000 population and 1.5 (1.3-1.8)/100 000 when adjusted for undetected cases. Twenty (25%) patients requiredventilation for an average (SD) of 42 (64) days. Thirty six (46%)patients received intravenous human immunoglobulin, five (6%) received plasma exchange, 11 (14%) both treatments, three (4%) steroids, and25 (32%) no immunomodulatory treatment. One year later, six patients(8%) had died, all of whom were older than 60, three (4%) remainedbedbound or ventilator dependent, seven (9%) were unable to walkunaided, 14 (17%) were unable to run, and 49 (62%) had made acomplete or almost complete recovery. Increasing age was significantlyassociated with a poorer outcome at one year.
CONCLUSIONS—Despite the frequent use of modernimmunomodulatory treatments Guillain-Barré syndrome still carriesconsiderable morbidity and mortality.

     

International trends in mortality from stroke, 1968 to 1994

BACKGROUND AND PURPOSE: The World Health Organization data bank is an invaluable source of information for international comparison of mortality trends. We present rates and trends in mortality from stroke up to 1994, with a particular emphasis on the last 10-year period. Data are presented for men and women in 51 industrialized and developing countries from different parts of the world. METHODS: We included all deaths from cerebrovascular disease for the population aged 35 to 84 years from all the countries in which death certificates were estimated to be available for at least 80% for the period from 1968 to 1994. Age-standardized mortality rates from stroke were calculated for each country for the last available 5 years. Time trends were calculated by using ordinary linear regression and are presented for the entire study period and for 3 separate time periods: 1968 to 1974, 1975 to 1984, and 1985 to 1994. The last 10-year period was further subdivided into 2 parts of 5 years each. We analyzed data separately for men and women and for groups aged 35 to 74 years and 75 to 84 years. RESULTS: The highest rates at the end of the study period for the population aged 35 to 74 years were observed in eastern Europe and previous Soviet Union countries (309 to 156/100 000 per year among men and 222 to 101/100 000 per year among women), Mauritius (268/100 000 per year among men and 138/100 000 per year among women), and Trinidad and Tobago (185/100 000 per year among men and 134/100 000 per year among women). Relatively low to average rates (<100/100 000 per year among men and <70/100 000 per year among women) were reported for Western Europe, with an exception of Portugal (162/100 000 per year among men and 95/100 000 per year among women). The countries with lowest stroke mortality rates at the end of the study period, such as the United States, Canada, Switzerland, France, and Australia, experienced steep declining trends. However, the slope of the decline was substantially reduced during the last 5 years in these countries. Mortality from stroke increased most in the eastern European countries, especially during the last 5 years. Among other high-risk populations, no change in stroke mortality trends was observed in Mauritius, whereas somewhat declining trends were seen in Trinidad and Tobago. CONCLUSIONS: We observed large differences in mortality rates from stroke around the world together with a wide variation in mortality trends. A widening gap was observed between 2 groups of nations, those with low and declining stroke mortality rates and those with high and increasing mortality, in particular, between western and eastern Europe. Eastern European countries should initiate actions aiming at the reduction of stroke risk, perhaps by looking at the examples of Japan and Finland and the other countries that have been the most successful in reducing previously very high mortality from stroke.     

Epidemiology of head injury in rural Taiwan -a four year survey

An epidemiological study of head injury in Hualien County, Taiwan, was undertaken from January 1, 1988 to December31, 1991. The age-adjusted incidence rate of head injury was 382/100 000, 499/100 000 for males and 244/100 000 for females. The highest incidence rate was observed in the elderly group 70 years or older. It is notable that 79% of head injuries were associated with traffic accidents, 75% of which directly involved motocycle use. The age-adjusted mortality rate was 89/100 000 per year, 125/100 000 for males and 47/100 000 for females. As to the severity of head injury, the elderly group presented the highest percentage of moderate to severe degrees of injury as well as mortality. The victims of motorcycle accidents and pedestrians had the highest percentage of relatively severe head injury. Hospitalised patients showed an outcome worse than that ever reported in the literature.     

The epidemiology of epilepsy in Europe – a systematic review

Population-based epidemiological studies on epilepsy are available mainly from the UK and the Nordic, Baltic and western Mediterranean countries. No studies were identified from large areas of Europe, especially from the former eastern Europe (except the Baltic countries) and the eastern Mediterranean countries. Based on the prevalence of epilepsy in different studies and accounting for incomplete case identification the estimated number of children and adolescents in Europe with active epilepsy is 0.9 million (prevalence 4.5–5.0 per 1000), 1.9 million in ages 20–64 years (prevalence six per 1000) and 0.6 million in ages 65 years and older (prevalence seven per 1000). Approximately 20–30% of the epilepsy population have more than one seizure per month. Based on the age-specific incidence rates in European studies, the estimated number of new cases per year amongst European children and adolescents is 130 000 (incidence rate 70 per 100 000), 96 000 in adults 20–64 years (incidence rate 30 per 100 000) and 85 000 in the elderly 65 years and older (incidence 100 per 100 000). The proportion of both new and established cases with epilepsy in the young, adults and elderly in individual countries may differ substantially from total European distribution because of differences in age structure.     

Intracranial aneurysms in children aged under 15 years: review of 59 consecutive children with 75 aneurysms

Objective The objective was to review the clinical aspects and therapeutic strategies in a series of aneurysmal vasculopathies seen in children 15 years or under.Methods From our dedicated neurovascular databank of patients, we reviewed 59 consecutive children who had 75 separate lesions.Results The children were divided into four age groups: below 2 years (22%), 2–5 years (24%), 6–10 years (24%) and 11–15 years (30%). Thirty-three children had dissecting aneurysms, 2 had chronic post-traumatic aneurysms, 8 had infectious aneurysms and 16 had saccular lesions. Twenty-seven percent of the lesions were in the posterior circulation, and 21% developed on the middle cerebral artery. Most dissecting lesions were encountered in the vertebrobasilar system, while saccular lesions were present mostly in the anterior circulation. Half of all cases presented with haemorrhage. Haemorrhage in patients below 2 years of age was due to dissecting aneurysms, while saccular aneurysms were responsible for haemorrhage in patients above 5 years of age. Five children had familial disease and 9 presented with multiple aneurysms. Forty-eight children were referred to us for treatment. Thirty-two underwent surgical (21.9%), endovascular (62.8%) or combined (9.3%) treatment. Eleven patients were treated conservatively and in 5 patients the aneurysms had spontaneously thrombosed at admission. Overall, complete or partial spontaneous thrombosis was seen in 10 patients (16.9%). Dissecting aneurysms were frequent in children of all ages with either associated thrombosis or arterial tear with repeated acute haemorrhage and poor outcome. Two types of dissection seem identifiable despite the small number of cases collected: acute segmental arterial tear without thrombosis, acute subarachnoid haemorrhage (SAH) and recurrence before 5 years; and subacute focal dissection with partial thrombosis (or mural haematoma), rare SAH and no early recurrence. The former would require aggressive management whereas the latter often do not require interventional approaches. The mortality in our series of aneurysms is low in the treated group (10.42%). The overall tolerance to haemorrhage seems better than in adults, as already stressed in the literature.Conclusion The multiple etiologies encountered confirm the heterogenous nature of aneurysms. The variety of treatments used suggests the need to categorise aneurysms into subgroups in sufficient numbers to fully appreciate the behavior of the lesions and make the appropriate therapeutic decisions.     

Pre- and neonatal hydrocephalus in the Middle East: experience in Qatar

In a retrospective study, all cases of hydrocephalus diagnosed antenatally or within the first 10 days of life during the period 1986–1989 were reviewed. Thirtyfour cases were diagnosed antenatally and 31 after delivery. Among the last, 13 were hemorrhagic. In 17 cases a meningomyelocele was present. In 12 cases other malformations outside the nervous system were observed. This represents an incidence of 157/100 000 live births for hydrocephalus and 41/100 000 for meningomyelocele. All cases were compared against a control group that had normal antenatal ultrasound findings at the same time (35 cases), and against a second control group consisting of 53 women with five or more normal pregnancies at the time, and all normal babies. The differences are avaluated and their significance and implications for the future discussed.     

Ruptured intracranial arterial aneurysms of children and adolescents

Sixteen children and adolescents under 20 years of age were treated at the Neurosurgical Department of Helsinki University Central Hospital between 1977 and 1986. These children represent 1.2% of the total 1,346 patients with aneurysm. Fourteen patients with Hunt and Hess grades I–III were operated on without surgical mortality or morbidity. Eight aneurysms were at the carotid bifurcation and six at the anterior communicating artery. One patient was grade IV and one grade V. Both died before operation. The hospital management mortality was 2 out of 16 or 12.5%. Seven patients of that age group within our area of responsibility died of a ruptured aneurysm during the years before patients were referred for neurosurgical treatment. Thus, the total mortality was 9 of 23 patients or 39%. Assuming transfer without delay to the neurosurgical clinic, operation within the first few days of bleeding, and no surgical mortality, the theoretical minimum mortality would be 22%.     

Suicide attempts in a Danish region

Summary The aim of this investigation, which is part of a Nordic research project, is to describe all patients who were admitted to a psychiatric department following attempted suicide. The region covered by the investigation is geographically limited and reasonably representative of Denmark. This study is retrospective and includes consecutive registration of 230 attempts carried out by 208 persons in the period 1 October 1980–20 April 1981. The number of admissions following attempted suicide in the period 1976–1980/81 increased by 47% to 201 per 100 000 inhabitants. Distribution by sex was equal. The highest rates found, both for men and for women, were those for the age groups 30–40 years; 80% had taken an overdose of medicine and only 20% were deeply unconscious. Only 10% were psychotic. More than half had previously been admitted to psychiatric departments for another reason and about the same number had previously attempted suicide. After 20–26 months 5.8% had committed suicide. In an international context the following factors are noteworthy: increasing rates, equal distribution by sex, the peak rates for women between the ages of 30 and 40, many previous attempts and many subsequent suicides.     

Management of brain abscess in children: review of 130 cases over a period of 21 years

The data on 130 children with brain abscesses treated over 21 years (1970–1990) were analyzed retrospectively. The whole group included four infants. Chronic ear infection and cyanotic congenital heart disease were the most common predisposing factors. In infants, meningitis and/or ventriculitis were dominant in the etiopathogenesis. Cases were evaluated according to the treatment received and also according to time periods. More than half of the patients (n=74) in this series were treated by primary or secondary excision. Computed tomography (CT) facilitated the diagnosis and helped the planning of treatment. Aspiration gained increasing credit after the advent of CT. Microorganisms could be identified in 54% of the cultured specimens. Staphylococci, streptococci and Proteus were the dominating microorganisms. Penicillin and chloramphenicol have long been the mainstay of antimicrobial therapy but have recently been replaced by third-generation cephalosporins and sulbactam-ampicillin combinations. Overall mortality was 15.5% but showed a decline from 30% in the pre-CT era to 6% in the last 5 years and to zero in the last three. Neither the location nor associated heart disease contributed to the mortality, but mortality among infants was as high as 50%.     

Effect of topiramate, in combination with lidocaine, and phenobarbital, in acute encephalitis with refractory repetitive partial seizures

Objective: Acute encephalitis with refractory repetitive partial seizure (AERRPS) is a peculiar type of post-encephalitic/encephalopathic epilepsy. Here we report an analysis of AERRPS in a series of children and propose an effective treatment option for seizure control in these children. Methods: We retrospectively reviewed cases of AERRPS treated in a pediatric intensive care unit, between February 2002 and June 2006. Clinical characteristics were systemically assessed. Burst suppression coma was induced by high-dose suppressive therapy; 24-h electroencephalogram (EEG) monitoring was performed on each patient. The goal of treatment was to achieve complete clinical seizure control or burst-suppression pattern on EEG, aiming for an interburst interval of >5 s. Brain imaging was done for each patient. Results: There were nine patients (seven boys), aged 5–15 years. Clinical symptoms included fever (100%), upper respiratory symptoms (66.7%) and altered consciousness (66.7%). All patients received multiple high-dose suppressive drugs and were intubated with/without inotropic agents. Seizures in three patients were stopped after high-dose lidocaine infusion (6–8 mg/kg/h) in the acute stage and three patients were stopped after high dose phenobarbital (serum level 60–80 ug/mL) combined with high-dose oral topiramate (15–20 mg/kg/day). Follow-up for this study was 16–61 months. Two subjects died while seven developed epilepsy and/or neurologic deficits; none returned to baseline. All survivors were discharged and continued multiple antiepileptic medications. Conclusions: Our data indicates that children with AERRPS have high mortality and morbidity rates. High-dose topiramate combined with high-dose lidocaine infusion or high-dose phenobarbital in the acute stage might be an effective treatment option for children with AERRPS.     

Severe intracranial hemorrhage and hydrocephalus in low-birthweight infants treated with CSF shunts

The high-risk low-birth-weight newborn not uncommonly develops intracranial hemorrhage and intraventricular hemorrhage (ICH/IVH) from the immature state of the germinal matrix. Posthemorrhagic hydrocephalus may develop. Infants with small hemorrhages (grades I, II of Papile), with or without hydrocephalus have been shown to develop normally in 80%–90% of cases. There is limited information in the literature about the management and outcome of infants with more severe hemorrhages (grades III, IV of Papile), due to the dismal outlook as to their outcome in most centers. The current status and concerns as to the management of these infants is reviewed, and the aspects of neurosurgical and neonatal follow-up and outcome are described. A significant number of these infants have severe handicaps, which are primarily motor. However, a group of infants is noted who have normal intellectual performance despite varying degrees of motor handicaps: 18% have normal intellectual and motor development. In the current series predictors of poorest outcome are the presence of grade IV hemorrhage and/or seizures. The vast majority of the grades III and IV hemorrhages develop hydrocephalus that is a complex management issue for the neurosurgeon.     

17p duplicated Charcot–Marie–Tooth 1A

The most frequent type of Charcot–Marie–Tooth (CMT) neuropathy is that associated with the 17p11.2–p12 chromosome duplication, whose characteristics have been well described in European and North American populations. In this study, we analyzed a Brazilian population exhibiting the mutation, found in 57 patients from 42 families (79%) of a cohort of 53 families with demyelinating CMT. Almost 20% of the duplicated cases were sporadic. In 77% of the duplicated families the mutation event occurred in the hot spot area of the CMT1A–Rep region. Forty–five percent of patients were females, 84% were Caucasians and 13% of African descent. Distal limb weakness was the most frequent abnormality, appearing in 84% of patients, although uncommon manifestations such as severe proximal weakness, floppy baby syndrome, diaphragmatic weakness and severe scoliosis were also observed. One patient was wheelchair–bound, and three suffered severe hand weakness. Sensory abnormalities were detected in 84% of the cases, but 80% were unaware of this impairment. Twelve patients complained of positive sensory manifestations such as pain and paresthesias. Progression was reported by 40%. Motor conduction velocities in the upper limbs were always less than 35 m/s, and less than 30.4 m/s in the peroneal nerve. The findings of this study expand the clinical spectrum of the disease.     

Epidemiology of status epilepticus in a rural area of northern Italy: a 2-year population-based study

We performed a 2-year population-based study on status epilepticus (SE) in adults in the rural area of Lugo di Romagna, northern Italy, to verify whether an area of low-level urbanization has a lower risk of occurrence of SE (as recently suggested), different clinical features and short-term prognosis than areas of high-level urbanization. We found crude and age- and sex-adjusted annual incidence rates of SE of 16.5/100 000 and 11.6/100 000, respectively. In patients under 60 years crude incidence was 2.9/100 000 and in the elderly (>/=60 years) 38.6/100 000. Acute symptomatic SE accounted for 30% and a cerebrovascular pathology was the most frequently associated etiologic condition (60%). A history of seizures was reported in 41% of patients. The first therapeutic intervention was mainly benzodiazepines (lorazepam 46%; diazepam 33%). The 30-day case fatality was 7%. We observed that the adult population of an area with a low level of urbanization has the same risk for SE, clinical features and short-term prognosis as European urban areas. The only contrasting result is the 30-day case fatality of 7% against the 39% found in the other Italian study (Bologna), despite the similarity of the SE features in these two areas of the same region. We infer that the short-term prognosis of SE could also be considerably influenced by differences in health service organization (and hence management) possibly due to different levels of urbanization.     

Determination of interferon-γ in the peripheral blood of multiple sclerosis patients in remission, using a chemiluminescence technique

The plasma from eight patients with multiple sclerosis (MS) whose disease was in remission, was investigated by a chemiluminescence technique for its ability to stimulate the oxidate metabolism of peripheral blood monocytes (PBM). The active fraction identified had a molecular weight between 13 700 and 43 000 Da. Its activity was reduced by incubation at pH 2, pH 4 or pH 6, or by treatment at 56°C for 1–3 h. The activity was also decreased, 58–100%, by prior incubation with antibodies to human interferon-γ (IFN-γ). We suggest that these results indicate that the increased chemiluminescence activity (CL-A) of PBM in MS patients in remission is due mainly to the presence of circulating IFN-γ.     

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

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.