Arne Torkildsen was a pioneering Norwegian neurosurgeon who introduced the ventriculocisternal shunt, the first clinically successful shunt for CSF diversion in hydrocephalus. The procedure, usually referred to as ventriculocisternostomy (VCS), Torkildsen’s operation, orTorkildsen’s shunt, became internationally recognized as an efficient operation for the treatment of noncommunicating hydrocephalus. The operation gained widespread use in the 1940s and 1950s before the introduction of extracranial shunts. In this paper, the authors look more closely at Torkildsen’s development of the VCS and examine how this surgical approach differed from other procedures for treating hydrocephalus before World War II. Long-term results of the VCS are presented.
Per Kristian Eide and Tryggve Lundar
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.
Tryggve Lundar, Søren Jacob Bakke, and Helge Nornes
✓ A 10-month-old child with achondroplasia with progressive head enlargement, ventriculomegaly, and wide subarachnoid spaces over the hemispheres was referred for evaluation. A steady-state lumbar infusion test revealed increased cerebrospinal fluid (CSF) outflow resistance (14 mm Hg/ml/min), and intra-arterial digital subtraction angiography (DSA) demonstrated bilateral venous outflow obstruction due to stenosis of the jugular foramen. Surgical decompression by opening the right jugular foramen relieved the clinical signs of intracranial hypertension. During the following year, the patient's head enlargement was moderate with relative normalization of size. Repeat DSA demonstrated improved venous runoff on the right side, and a steady-state lumbar infusion test demonstrated reduced CSF outflow resistance (10 mm Hg/ml/min). Venous decompression is causal therapy and may prove to be preferable to shunting in children with hydrocephalus and bilateral stenosis of the jugular foramen.
Marion L. Walker
Tryggve Lundar, Bernt Johan Due-Tønnessen, Radek Frič, Petter Brandal, and Paulina Due-Tønnessen
Ependymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.
The authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.
The authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.
Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.
Pediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.
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.
Bernt Johan Due-Tønnessen, Tryggve Lundar, Arild Egge, and David Scheie
The objective of this study was to delineate the long-term results of surgical treatment of pediatric low-grade cerebellar astrocytoma.
One hundred consecutive children and adolescents (0–19 years old) who underwent primary tumor resection for a low-grade cerebellar astrocytoma during the years 1980–2011 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.
Of the 100 patients, 61 children were in the 1st decade, and 39 were 10–19 years old. The male/female ratio was 1.13:1 (53 males, 47 females). No patients were lost to follow-up. There were no deaths in this series and all 100 patients are currently alive. In 29 patients, the follow-up duration was less than 10 years, in 37 it was between 10 and 19 years, and in 34 it was between 20 and 31 years. The Barthel Index was 100 (normal) in 97 patients, 90 in 2 patients, and 40 in the last patient. A total of 113 tumor resections were performed. Two patients underwent further tumor resection due to MRI-confirmed residual tumor demonstrated on the immediate postoperative MR image (obtained the day after the initial procedure). Furthermore, 9 children underwent repeat tumor resection after MRI-confirmed progressive tumor recurrence up to 10 years after the initial operation. Two of these patients also underwent a third resection, without subsequent radiation therapy, and have experienced 8 and 12 years of tumor-free follow-up thereafter, respectively. A total of 15% of the patients required treatment for persistent hydrocephalus.
Low-grade cerebellar astrocytoma is a surgical disease, in need of long-term follow-up, but with excellent long-term results. Nine percent of the children in this study underwent repeated surgery due to progressive tumor recurrence, and 15% were treated for persistent hydrocephalus.
Tryggve Lundar, Bernt Johan Due-Tønnessen, Radek Frič, Bård Krossnes, Petter Brandal, Einar Stensvold, and Paulina Due-Tønnessen
The authors conducted a study to delineate the long-term results of the surgical treatment of pediatric pleomorphic xanthoastrocytomas (PXAs).
All consecutive children and adolescents (0–20 years) who underwent primary tumor resection for a PXA during the years 1972–2015 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.
Of the 12 patients, 8 patients were in the 1st decade of life and 4 in the 2nd. The male/female ratio was 6:6. No patient was lost to follow-up. One patient presented with severe progressive tumor disease and died within 3 months after repeated resection. Another child died 3 days following a second surgical procedure involving gross-total resection (GTR) 8 years after the initial operation. The other 10 patients were alive at the latest follow-up when they reached the median age of 34 years (range 11–60 years). The median follow-up duration was 22 years (range 2–41 years). Barthel Index score was 100 in all 10 survivors. A total 18 tumor resections were performed. Five patients underwent a second tumor resection after MRI/CT confirmed recurrent tumor disease, from 6 months up to 17 years after the initial operation. Only one of our patients received adjuvant therapy: a 19-year-old male who underwent resection (GTR) for a right-sided temporal tumor in 1976. This particular tumor was originally classified as astrocytoma WHO grade IV, and postoperative radiotherapy (54 Gy) was given. The histology was reclassified to that of a PXA. Seven of 8 children whose primary tumor resection was performed more than 20 years ago are alive as of this writing—i.e., 88% observed 20-year survival. These are long-term survivors with good clinical function and all are in full- or part-time work.
Pediatric patients with PXA can be treated with resection alone with rewarding results. Recurrences are not uncommon, but repeated surgery is well tolerated and should be considered in low-grade cases before adjuvant therapy is implemented. Follow-up including repeated MRI is important during the first postoperative years, since individual patients may have a more aggressive tumor course.
Tryggve Lundar, Bernt Johan Due-Tønnessen, Arild Egge, David Scheie, Einar Stensvold, and Petter Brandal
The object of this study was to delineate long-term results of the surgical treatment of pediatric CNS tumors classified as oligodendroglioma (OD) or oligoastrocytoma (OA) WHO Grade II or III.
A cohort of 45 consecutive patients 19 years or younger who had undergone primary resection of CNS tumors originally described as oligodendroglial during the years 1970–2009 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Gross motor function and activities of daily living were scored using the Barthel Index (BI).
Patient records for 35 consecutive children and adolescents who had undergone resection for an OA (17 patients) or OD (18 patients) were included in this study. Of the 35 patients, 12 were in the 1st decade of life at the first surgery, whereas 23 were in the 2nd decade. The male/female ratio was 1.19 (19/16). No patient was lost to follow-up. The tumor was localized to the supratentorial compartment in 33 patients, the posterior fossa in 1 patient, and the cervical medulla in 1 patient. Twenty-four tumors were considered to be WHO Grade II, and 11 were classified as WHO Grade III. Among these latter lesions were 2 tumors initially classified as WHO Grade II and later reclassified as WHO Grade III following repeat surgery.
Fifty-four tumor resections were performed. Two patients underwent repeat tumor resection within 5 days of the initial procedure, after MRI confirmed residual tumor. Another 10 patients underwent a second resection because of clinical deterioration and progressive disease at time points ranging from 1 month to 10 years after the initial operation. Six patients underwent a third resection, and 1 patient underwent a fourth excision following tumor dissemination to the spinal canal. Sixteen (46%) of the 35 children received adjuvant therapy: 7, fractionated radiotherapy; 4, chemotherapy; and 5, both fractionated radiotherapy and chemotherapy. One patient with primary supratentorial disease experienced clinically malignant development with widespread intraspinal dissemination 9 years after initial treatment. Only 2 patients needed treatment for persistent hydrocephalus.
In this series there was no surgical mortality, which was defined as death within 30 days of resection. However, 12 patients in the study, with follow-up times from 1 month to 33 years, died. Twenty-three patients, with follow-up times from 4 to 31 years, remained alive. Among these survivors, the BI was 100 (normal) in 22 patients and 80 in 1 patient. Nineteen patients had full- or part-time work or were in normal school programs.
Pediatric oligodendroglial tumors are mainly localized to the supratentorial compartment and more often occur in the 2nd decade of life rather than the 1st. Two-thirds of the patients remained alive after follow-ups from 4 to 31 years. Twelve children succumbed to their disease, 9 of them within 3 years of resection despite combined treatment with radio- and chemotherapy. Three of them remained alive from 9 to 33 years after primary resection. Among the 23 survivors, a stable, very long-term result was attainable in at least 20. Five-, 10-, 20-, and 30-year overall survival in patients with Grade II tumors was 92%, 92%, 92%, and 88%, respectively.
Tryggve Lundar, Bernt Johan Due-Tønnessen, Arild Egge, David Scheie, Petter Brandal, Einar Stensvold, and Paulina Due-Tønnessen
The authors delineate the long-term results of surgical treatment for pediatric low-grade midbrain glioma.
A series of 15 consecutive patients (age range 0–15 years) who underwent primary tumor resection for a low-grade midbrain glioma during the years 1989–2010 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.
Of the 15 patients, 10 were in their 1st decade (age 0–9 years) and 5 were in their 2nd decade of life (age 10–15 years) at the time of surgery. The male/female ratio was 0.50 (5:10). No patients were lost to follow-up. One patient died in the postoperative period (32 days posttreatment). Another 2 patients died during follow-up. One patient succumbed to acute bleeding in the resection cavity 8 months after surgery, and the other died of shunt failure 21 years after initial treatment. Twelve patients are alive at the time of this writing, with follow-up periods from 3 to 24 years (median 8 years).
Among the 12 survivors, the Barthel Index scores were normal (100) in 11 patients and 80 in 1 patient. A total of 25 tumor resections were performed. In 1 patient, further resection was performed 5 days after initial resection due to MRI-confirmed residual tumor. Another 5 patients underwent repeat tumor resection after MRI-confirmed progressive tumor disease and clinical deterioration ranging from 3 months to 4 years after the initial operation. Three of these 5 patients also underwent a third resection, and 1 of the 3 underwent a fourth operation. Six children received adjuvant therapy: local radiotherapy in 2 patients, chemotherapy in 3 patients, and both in 1 patient. Twelve (80%) of the 15 patients needed treatment for persistent hydrocephalus.
Selected cases of low-grade midbrain gliomas may clearly benefit from resection with favorable results, even for prolonged periods. Three patients in the present series died, one of whom had a prolonged survival period of 21 years. Among the 12 survivors, stable long-term results appeared obtainable in at least 9. One patient died of acute hemorrhage 8 months after initial resection; otherwise, rapid tumor progression and death were not observed. Forty percent of the patients received adjuvant treatment, with local radiotherapy, chemotherapy, or both.