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Ladislau Steiner, 1920–2013

Historical vignette

Dan Leksell and Christer E. H. Lindquist

The authors commemorate the life and career of Dr. Ladislau Steiner, one of the world's most highly regarded neurosurgeons, from Stockholm and Charlottesville, Virginia, who has died at age 92. They review the events of Dr. Steiner's early life, including his early training in his native Romania, his escape with his family from East Berlin, and his postgraduate training in neurosurgery at the Karolinska Institute in Stockholm. Dr. Steiner's work in the development of microsurgery and his collaboration with Lars Leksell in the development of Gamma Knife radiosurgery are described. After his retirement from Karolinska, Dr. Steiner had a second career as head of the Lars Leksell Gamma Knife Center at the University of Virginia in Charlottesville. The authors recall their own long association with Dr. Steiner and celebrate his contributions to the field of neurosurgery.

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Christer Lindquist, Wan-Yio Guo, Bengt Karlsson and Ladislau Steiner

✓ Radiosurgical treatment with the gamma knife for venous angiomas was used as an alternative to microsurgical removal in order to avoid abrupt cessation of venous drainage, which may be shared by the venous angioma and important parts of the brain. Thirteen cases of venous angioma were treated between 1977 and 1991. In two cases cavernous angiomas were also present and in one case a distant arteriovenous malformation (AVM) was also found. In two cases the angioma shared the venous drainage with an adjoining AVM; this is the first description of such pathology. For venous angiomas irradiation was prescribed to cover at least the convergence of the medullary veins. For AVM's close to a venous angioma the treatment was exclusively prescribed to the AVM nidus. After treatment, complete obliteration of the venous angioma was observed in one case, partial obliteration was observed in three cases, and five venous angiomas were unaffected by the treatment. Undue effects of radiation occurred in four cases: one focal edema and three radionecroses. Extirpation of the radionecrotic tissue 6 months after radiosurgery was necessary in one case. In the other three cases, the venous angioma was observed to be completely or partially obliterated, or unaffected by the treatment (one case each). In two cases of combined AVM and venous angioma, complete obliteration of the treatment AVM nidus was obtained. It is concluded that radiosurgery for venous angioma, although conceptually attractive, still does not fulfill the rigid criteria of minimal risk which must be set for the treatment of a lesion with a benign natural history.

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Tiit Mathiesen, Per Grane, Lars Lindgren and Christer Lindquist

✓ A continuous follow-up review of colloid cysts including aspects of natural history and evaluation of treatment options is necessary to optimize individual treatment. Thirty-seven consecutive patients with colloid cyst of the third ventricle seen at Karolinska Hospital between 1984 and 1995 were reviewed. Five patients were admitted in a comatose state, and two died despite emergency ventriculostomy. Three had recurrent cysts following previous aspiration procedure. During the study period, patients underwent a total of 10 ventriculostomies, 10 aspirations, 26 microsurgical operations, and two shunt operations.

Twenty-four of 26 microsurgical operations were transcallosal and two were transcortical. Twenty-four operations (22 transcallosal and two transfrontal approaches) without permanent morbidity were performed by four surgeons. Transient memory deficit from forniceal traction was noted in 26%. The remaining two transcallosal operations, which led to permanent morbidity or mortality, were performed by two different surgeons. Aspiration of cysts performed by four different surgeons carried a 40% risk of transient memory deficit (10% permanent) and an 80% recurrence rate. One patient was found to be cured on radiological studies obtained at the 5-year follow-up review.

Seven cysts were followed by means of radiological studies with no treatment for 6 to 37 months. Five of these cysts grew, indicating that younger patients with colloid cysts will probably need surgical treatment.

The main causes of unfavorable results were: 1) failure to investigate symptoms that proved fatal; 2) subtotal resection; and 3) surgical complications. Transcallosal microsurgery produced excellent results when performed by experienced surgeons. A colloid cyst of the foramen of Monro is a disease that should be detected before permanent neurological damage has occurred. Permanent morbidity or mortality should not be accepted in modern series of third ventricle colloid cysts.

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Elfar Úlfarsson, Christer Lindquist, Maud Roberts, Tiit Rähn, Melker Lindquist, Marja Thorén and Bodo Lippitz

Object. The purpose of this study was to assess the long-term treatment efficacy and morbidity of patients who undergo gamma knife radiosurgery (GKS) for craniopharyngioma.

Methods. Twenty-one consecutive Swedish patients were evaluated retrospectively: 11 children (≤ 15 years) and 10 adults. The time from diagnosis to the most recent follow-up imaging study was 6.3 to 34.3 years (mean 18.2 years, median 16.8 years). Tumor volumes and morbidity from GKS or other treatments were assessed at the time of the most recent imaging study or at the time of a subsequent new treatment. The observation period ranged from 0.5 to 29 years (mean 7.5 years, median 3.5 years). The prescription dose ranged from less than 3 Gy to 25 Gy. The mean tumor volume was 7.8 cm3 (range 0.4–33 cm3).

There were 22 tumors in 21 patients treated with GKS. Five of these tumors were reduced in size, three were unchanged, and 14 increased. Tumor progression correlated with a low dose to the tumor margin. Eleven (85%) of 13 tumors that received a dose of less than 6 Gy to the margin increased in size, whereas only three (33%) of nine tumors that received 6 Gy increased. This difference was statistically significant (p = 0.01). In five of six patients tumors that became smaller after GKS there were no recurrences within a mean follow-up period of 12 years. Nine (82%) of 11 tumors in children ultimately increased after GKS, compared with five (50%) of 10 in adults. In eight patients there was a deterioration of visual function. In all except one this could be related to a volume increase but radiation-induced damage could not be excluded as a factor in any of them. Four patients developed pituitary deficiencies.

Conclusions. Gamma knife radiosurgery is effective in controlling growth of craniopharyngiomas with a minimum dose of 6 Gy. The findings also suggest that other stereotactic techniques, such as cyst aspiration and intracystic treatment, are only of value in reducing tumor volume in preparation for safe GKS.

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Tiit Mathiesen, Per Grane, Christer Lindquist and Hans von Holst

✓ Sixteen patients treated between 1969 and 1989 for a colloid cyst of the foramen of Monro by stereotactically guided aspiration (not stereotactic extirpation) were evaluated to assess the long-term outcome of the procedure. Thirteen of these patients required reoperation due to an acute comatose state, failure to achieve permanent reduction of the cyst, or symptomatic hydrocephalus. Of these 13, six were treated twice and two were treated three times by stereotactic aspiration. Five patients underwent microsurgical extirpation and three had a shunt placed following a failed aspiration. Failure of the first procedure was detected within the first 2 months after treatment in eight patients and after more than 6 years in seven. Following stereotactic aspiration, three patients experienced a temporary memory deficit and confusion and one patient suffered a central pain syndrome. Eleven of the 26 procedures were followed by a recurrence 6 to 15 years after treatment; seven recurrent cysts were detected after more than 8 years. Of the patients with recurrences, three did not undergo repeat surgery but showed an increase in cyst size at the latest follow-up examination. It is suggested that radical removal by open or stereotactically guided microsurgery is the method of choice since stereotactic aspiration fails to offer a radical or permanent treatment for colloid cysts of the third ventricle.

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Ladislau Steiner, Christer Lindquist, Wayne Cail, Bengt Karlsson and Melita Steiner

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Bengt Karlsson, Lars Kihlström, Christer Lindquist, Kaj Ericson and Ladislau Steiner

Object. The authors examined 22 patients with cavernous malformations (CMs) who had undergone gamma knife radiosurgery (GKRS) to assess the value of this procedure in treating these lesions.

Methods. At the Karolinska Hospital, GKRS was used to treat 23 patients with CMs during the period of 1985 through 1996. One of the patients was lost to follow up and the treatment results of the 22 remaining patients were analyzed. In the first half of the series, the CMs were treated with high doses of radiation (> 15-Gy dose to the periphery); in the second half of the series, lower doses were used.

Nine of the 22 patients suffered a post-GKRS hemorrhage and six developed a radiation-induced complication (two of these patients experienced both). Some time after GKRS was performed, surgical removal of the CM had to be undertaken in four patients because of hemorrhage and in two patients because of radiation-induced complications. Four of the nine patients who experienced no post-GKRS hemorrhage or radiation-induced complication were treated before 1990; recent magnetic resonance imaging revealed a decrease in the size of the CM in three of these individuals and no size change in the other.

The annual post-GKRS hemorrhage rate was 8% in this group. There was a trend in the hemorrhage rate to decrease 4 years postsurgery. There was also a trend for higher radiation doses administered to the periphery of the lesion to result in a lower risk of posttreatment hemorrhage. However, it could not be concluded whether GKRS affects the natural course of a CM. The incidence of radiation-induced complications was approximately seven times higher than that expected if the same number of patients had been treated by GKRS with the same radiation dose distributions for arteriovenous malformations instead of CMs.

Conclusions. The high incidence of radiation-induced complications does not seem to justify the limited protection the treatment may afford in only exceptional cases. A prospective randomized study is needed before the role of radiosurgery in the management of these lesions can be defined. Until such a study has proved differently, a caveat must be raised for the treatment of CM with GKRS.

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Mika Niemelä, Young Jin Lim, Michael Söderman, Juha Jääskeläinen and Christer Lindquist

✓ One suprasellar, one mesencephalic, and nine cerebellar hemangioblastomas were treated with the gamma knife in 10 patients (median age 48 years) in Stockholm between 1978 and 1993. Four patients had von Hippel—Lindau disease, a dominant inherited trait predisposing to multiple hemangioblastomas. Six hemangioblastomas were treated with radiotherapy at a median margin dose of 25 Gy (20–35 Gy) before 1990 and the next five with a median of 10 Gy (5–19 Gy). Computerized tomography or magnetic resonance images were available for 10 of the 11 hemangioblastomas at a median follow-up time of 26 months (4–68 months) after radiosurgery. The solid part of six hemangioblastomas shrank in a median of 30 months, whereas four hemangioblastomas were unchanged at a median of 14 months. Five hemangioblastomas had an adjoining cyst and three of these cysts had to be evacuated after radiosurgery. One solitary hemangioblastoma later developed a de novo cyst that also needed evacuation. One patient with two cerebellar hemangioblastomas (margin dose 25 Gy each) developed edema at 6 months and required a shunt and prolonged corticosteroid treatment. The combined follow-up data of the 23 hemangioblastomas in 15 patients from previous literature and the present series indicate that, first, a solitary small- or medium-sized hemangioblastoma usually shrinks or stops growing after radiosurgery. The recommended margin dose is 10 to 15 Gy. Second, the adjoining cyst often does not respond to radiosurgery but requires later, sometimes repeated evacuation.

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Isaac Feuerberg, Christer Lindquist, Melker Lindqvist and Ladislau Steiner

✓ In a series of 715 patients operated on by microsurgical techniques for intracranial saccular aneurysms between 1970 and 1980, part of the aneurysmal sac was not obliterated in 28 aneurysms in 27 patients (3.8% of 715 cases). Clinical follow-up evaluation for 8 years (range 4 to 13 years) and angiographic follow-up studies for 6 years (range 2 to 10 years) in these 27 cases revealed that one aneurysm rest increased in size and bled twice, five were spontaneously obliterated, two decreased in size, 13 remained unchanged, and in seven cases no late follow-up angiography was performed. The incidence of rebleeding from an aneurysm rest was 3.7% of the 27 in whom the sac was not obliterated and 0.14% of all 715 patients who were operated on.

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Daria Riva, Chiara Pantaleoni, Monica Devoti, Christer Lindquist, Ladislau Steiner and Cesare Giorgi

✓ Eight patients, ranging in age from 9 to 18 years, were treated for arteriovenous malformations using gamma knife radiosurgery and were evaluated an average of 6 years after treatment to record potential effects of radiosurgery on cognitive and neuropsychological performance. Tests for general intelligence, nonverbal intelligence, memory and its components, and attention performance were administered to patients and compared with test results of age-matched siblings or first cousins. No statistically significant difference was found between the performance of patients and controls in any of the tests administered. Additionally, a specially designed questionnaire completed by the patients, their parents, and their teachers revealed that the patients' emotional and relational behavior was stable and unchanged after treatment. No correlation was found between the neurocognitive test performance and the lesion volumes irradiated, but the lesion site was found to contribute to the type of deficit recorded after treatment. The less invasive nature of the radiosurgical approach, combined with the brevity or absence of hospitalization, presumably contributed to the patients' successful physical, mental, and emotional recovery.