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Anne Henriette Paulsen, Tryggve Lundar and Karl-Fredrik Lindegaard


Treatment for hydrocephalus has not advanced appreciably since the advent of CSF shunts more than 50 years ago. The outcome for pediatric patients with hydrocephalus has been the object for several studies; however, much uncertainty remains regarding the very long term outcome for these patients. Shunting became the standard treatment for hydrocephalus in Norway during the 1960s, and the first cohorts from this era have now reached middle age. Therefore, the objective of this study was to review surgical outcome, mortality, social outcome, and health-related quality of life in middle-aged patients treated for hydrocephalus during childhood.


Data were collected in all patients, age 14 years or less, who required a CSF shunt during the years 1967–1970. Descriptive statistics were assessed regarding patient characteristics, surgical features, social functioning, and work participation. The time and cause of death, if applicable, were also determined. Kaplan-Meier survival estimates were used to determine the overall survival of patients. Information regarding self-perceived health and functional status was assessed using the 36-Item Short Form Health Survey (SF-36) and the Barthel Index score.


A total of 128 patients were included in the study, with no patient lost to follow-up. Of the 128 patients in the study, 61 (47.6%) patients died during the 42–45 years of observation. The patients who died belonged to the tumor group (22 patients) and the myelomeningocele group (13 patients). The mortality rate was lowered to 39% if the patients with tumors were excluded. The overall mortality rates at 1, 2, 10, 20, and 40 years from time of initial shunt insertion were 16%, 24%, 31%, 40%, and 48% respectively. The incidence of shunt-related mortality was 8%. The majority of children graduated from a normal school (67%) or from a school specializing in education for physically handicapped children (20%). Self-perceived health was significantly poorer in 6 out of 8 domains assessed by SF-36 as compared with the background population. Functional status among the survivors varied greatly during the follow-up period, but the majority of patients were self-dependent. A total of 56% of the patients were socially independent, and 42% of the patients were employed.


Approximately half of the patients are still alive. During the 42–45 year follow-up period, the mortality rate was 48%. Two deaths were due to acute shunt failure, and at least 8% of the deaths were shunt related (probable or late onset). The morbidity in middle-aged individuals treated for pediatric hydrocephalus is considerable.

The late mortality rate was low, but not negligible. Twelve patients died during the last 2 decades, 1 of whom died because of acute shunt failure. Although the shunt revision rate was decreasing during the study period, many patients required shunt surgery during adulthood. Forty-one revisions in 21 patients were performed during the last decade. Thus, there is an obvious need for life-long follow-up in these patients.

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Shunt survival

Marion L. Walker

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A. Henriette Paulsen, Tryggve Lundar and Karl-Fredrik Lindegaard


Shunting of CSF is one of the most commonly performed operations in the pediatric neurosurgeon's repertoire. The 1st decade after initial shunt insertion has been addressed in several previous reports. The goals of the authors' study, therefore, were to determine 20-year outcomes in young adults with childhood hydrocephalus and to assess their health-related quality of life (HRQOL).


Patients younger than 15 years of age, in whom a first-time shunt insertion was performed for hydrocephalus in the calendar years 1985–1988, were included in a retrospective study on surgical morbidity, mortality rates, academic achievement, and/or work participation. Information concerning perceived health and functional status was assessed using the 36-Item Short Form Health Survey (SF-36) and Barthel Index, which were completed by patients still alive by September 1, 2009.


Overall, 138 patients participated, no patient being lost to follow-up. For the 20-year period, the overall mortality rate was 21.7%. The mortality rate was not significantly higher in the 1st decade after initial shunt insertion than in the 2nd decade (p = 0.10). Ten percent of the patients surviving still live with their primary shunt in place, whereas 81% required at least one revision, and among these individual the mean number of revisions was 4.2 (median 3, range 0–26). There was a significantly higher revision rate during the 1st decade after initial shunt insertion compared with the 2nd decade (p = 0.027). The majority of patients live lives comparable with those of their peers. At follow-up, 56% were employed in open-market jobs or were still students, 23% had sheltered employment, and 21% were unemployed. The HRQOL was slightly lower in the hydrocephalic cohort than in the normative population. A significant difference was found in 2 of 8 SF-36 domains—Physical Functioning and General Health.


During the 20-year follow-up period, 81% of the patients required at least one revision of the CSF shunt. The mortality rate was high: 24 patients died in the 1st decade and 6 died in the 2nd decade after implantation of the initial shunt. In total, 4 deaths (2.9%) were due to shunt failure. Shunt placement to treat childhood hydrocephalus has a substantial effect on social functioning in later life, although perceived health was positively found to be better than expected in young adults with hydrocephalus.

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Karl-Fredrik Lindegaard, Sverre J. Mørk, Geir E. Eide, Tore B. Halvorsen, Reidulv Hatlevoll, Torfinn Solgaard, Olav Dahl and Jeremy Ganz

✓ The postoperative survival time of 170 nonrandomized patients treated for cerebral oligodendrogliomas in Norway from 1953 to 1977 was studied. Survival times were significantly prolonged if postoperative irradiation was performed in addition to surgery (median survival time 26.5 vs. 38 months, p = 0.039). In the group without postoperative radiotherapy, the 5-year rate of survival was 27% compared with 36% in the irradiated patients. The respective survival rates after 8 years were 14% versus 17%; thus, there was little effect on long-term survival. Irradiation appears not to be of benefit after “total” removal. Patients with partly resected lesions appeared to benefit from postoperative radiotherapy; the median survival period after subtotal tumor resection was 37 months with and 26 months without radiotherapy (p = 0.0089). The findings also indicate that irradiation doses between 40 and 50 Gy were as effective as doses between 50 and 60 Gy in increasing the patients' probability of surviving 5 years after subtotal tumor resection. Since the risk of radiation necrosis is proportional to the dose applied, the lower dose is recommended. These conclusions were also valid when adjustments were made for prognostically significant histological and clinical features.

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Karl-Fredrik Lindegaard, Peter Grolimund, Rune Aaslid and Helge Nornes

✓Blood flow velocities in basal cerebral arteries were recorded noninvasively in 28 patients with cerebral arteriovenous malformations (AVM's) and were correlated with the angiographic findings. In normal arteries remote from the AVM, flow velocities ranged from 44 to 94 cm/sec (median 65 cm/sec) with pulsatility indexes from 0.65 to 1.10 (median 0.87). This is consistent with findings in normal individuals. Arteries feeding the AVM's were identified by the high flow velocities (ranging from 75 to 237 cm/sec, median 124 cm/sec). The pulsatility index ranged from 0.22 to 0.74 (median 0.48). The difference of these results from findings in normal remote arteries was highly significant (p < 0.001). Hyperventilation tests illustrated the hemodynamic difference between an AVM and normal cerebrovascular beds.

Flow velocity measurements permitted noninvasive diagnosis of AVM's in 26 of the 28 patients. Furthermore, the identification of individual feeding arteries permitted good definition of the anatomical localization of individual AVM's. Flow velocity measurements combined with computerized tomography scans are useful in the diagnosis of AVM's. With the feeding artery's configuration identified on angiography, flow velocity measurements permit a new insight into the “hemodynamic dimension” of an AVM and its possible effects on adjacent normal brain-tissue perfusion in the individual patient.

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Karl-Fredrik Lindegaard, Søren Jacob Bakke, Peter Grolimund, Rune Aaslid, Peter Huber and Helge Nornes

✓ Noninvasive transcranial Doppler recordings were correlated to the angiographic findings in 77 patients with carotid artery disease. Stenoses reducing the luminal area of the internal carotid artery by 75% or more also reduced the pulsatility transmission index (PTI) of the ipsilateral middle cerebral artery (MCA). The PTI is the pulsatility index of the artery under study expressed as a percent of the pulsatility index of another intracranial artery with presumed unimpeded inflow in the same individual. For stenoses in the 75% to 89% category, PTI reduction was significantly greater in patients with bilateral carotid stenosis, indicating an impaired potential for collateral flow in these patients. The PTI reduction probably reflects both the pressure drop across the stenosis and the cerebral autoregulatory response. Two criteria proved useful in demonstrating collateral MCA supply through the circle of Willis. On the recipient side, retrograde flow in the proximal anterior cerebral artery was demonstrated in 29 of the 31 patients when this flow pattern was disclosed angiographically. In 26 of these patients, the anterior cerebral artery on the supplying side also had clearly increased flow velocity. Increased flow velocities in the proximal posterior cerebral artery were present in 26 of the 30 vessels that were acting as a collateral channel to the ipsilateral MCA.

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Sverre J. Mørk, Karl-Fredrik Lindegaard, Tore B. Halvorsen, Egil H. Lehmann, Torfinn Solgaard, Reidulv Hatlevoll, Sverre Harvei and Jeremy Ganz

✓ The cases of 208 patients with histologically confirmed oligodendrogliomas were studied. The incidence represents 4.2% of all primary brain tumors diagnosed in the Norwegian population over a 25-year period. All of these tumors were cerebral and the majority affected the frontal lobe. The patients' median age at diagnosis was 47 years, with a range from 3 to 76 years; 6% of the oligodendrogliomas occurred in children. The median duration of symptoms before diagnosis was 20.5 months (mean 43 months). Plain skull x-ray films showed calcified deposits in 28% of the tumors. At operation, most of the tumors were poorly defined, without cyst formation, hematoma necrosis, or calcification. The median duration of disease from onset of symptoms until death was 14 months in nine untreated cases. In surgically treated oligodendroglioma patients the median survival time from onset of symptoms was 74 months. The median postoperative survival time was 35 months (mean 52 months). Tumor calcification, as seen on plain skull x-ray films, was associated with a significantly longer survival period. The surgical findings of gross necrosis, gross hypervascularity, and soft tumor consistency were all related to a shorter total duration of disease. Grossly well demarcated lesions were associated with a significantly longer postoperative survival. The length of postoperative survival correlated with the preoperative clinical status. The cumulative proportion of patients surviving 5 years was 0.342. The patient's age and sex did not have a statistically significant influence on survival time. The extent of surgical excision only seemed to play a role when the neurosurgeon considered that he had removed the whole lesion: these patients had a median postoperative survival period 14 months longer than the other oligodendroglioma patients. The ABO blood group of the oligodendroglioma patient was of prognostic value. In particular, patients with blood group A had a distinctly poorer prognosis than patients with O or B blood. The survival data from this unselected series indicate that cerebral oligodendrogliomas have a less favorable prognosis than has generally been believed.