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  • Author or Editor: Juha Hernesniemi x
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Juri Kivelev, Mika Niemelä and Juha Hernesniemi

Object

Spinal cavernomas are rare, but can cause significant neurological deficits due to mass effect and extralesional hemorrhage. The authors present their results of microsurgical treatment of 14 consecutive patients with spinal cavernoma, and review the literature.

Methods

Of the 376 patients with cavernomas of the CNS treated at Helsinki University Central Hospital (a catchment area close to 2 million inhabitants) between January 1980 and June 2009, 14 (4%) had a spinal cavernoma. The authors reexamined and analyzed the patient files and images retrospectively. Median patient age at presentation was 45 years (range 20–57 years). The female/male ratio was equal. Median duration of symptoms before admission to the department was 12 months (range 0.1–168 months). Patients suffered from sensorimotor paresis, radicular pain, or neurogenic micturition disorders in different combinations or separately. Hemorrhage had occurred in 7 patients (50%) before surgery. In 9 patients (64%) the cavernoma was intramedullary, in 4 (29%) extradural, and in 1 intradural extramedullary. On MR imaging, 6 patients (43%) had a cavernoma in the cervical region, 7 (50%) in the thoracic region, and 1 (7%) in the lumbar region.

Results

Postoperatively, patients were followed up for a median of 3 years (range 1–10 years). At follow-up, 13 patients (93%) experienced significant improvement in motor ability after surgery, and all patients were able to walk with or without aid. Ten of the 11 patients with pain syndrome (91%) showed significant pain relief without recurrence. Micturition disorder was noted in 6 patients (43%) at follow-up, but in 5 the condition had existed before surgery. No patient improved in bladder function after surgery, and 1 patient developed micturition dysfunction postoperatively.

Conclusions

Microsurgical removal of spinal cavernomas alleviates sensorimotor deficits and pain caused by mass effect and hemorrhage. However, bladder dysfunction remains unchanged after surgery.

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Roberto C. Heros

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Juri Kivelev, Mika Niemelä, Riku Kivisaari and Juha Hernesniemi

Object

Intraventricular cavernomas (IVCs) occur in only 2–10% of patients with cerebral cavernomas. Reports concerning IVC are scarce and are limited mostly to sporadic case reports. In this paper, the authors present a series of 12 patients with IVCs that were treated at a single neurosurgical department. In addition, the authors reviewed the literature.

Methods

All clinical data were analyzed retrospectively. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale. The authors also conducted a PubMed search and found 77 cases of IVC.

Results

The patients' median age was 47 years, and the male/female ratio was 2:1. A cavernoma occurred in the lateral ventricle in 6 patients, in another 5 it was in the fourth ventricle, and 1 had a lesion in the third ventricle. Almost all patients presented with acute headache on admission and in more than half, the symptoms were related to cavernoma bleeding. In total, 8 rebleedings occurred in 5 patients during a median of 0.4 years. Three patients with a cavernoma of the fourth ventricle presented with a cranial nerve deficit. In 8 cases, a cavernoma was surgically treated an average of 1.3 years after the diagnosis. Only 1 patient underwent surgery in the acute phase after a major intraventricular/intracerebral hemorrhage. The median follow-up time was 2 years. No patient was lost to follow-up, and no patient died. In total, on follow-up 9 patients improved and 3 had a persistent neurological deficit, of which 2 existed before surgery.

Conclusions

In the present series, the IVCs had a high tendency for rehemorrhage. Surgery is advocated when hemorrhages are frequent, and the mass effect causes progressive neurological deficits. Microsurgical removal of the IVC is safe, but in the fourth ventricle it can carry increased risk for cranial nerve deficits.

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Juri Kivelev, Elina Koskela, Kirsi Setälä, Mika Niemelä and Juha Hernesniemi

Object

Cavernomas in the occipital lobe are relatively rare. Because of the proximity to the visual cortex and incoming subcortical tracts, microsurgical removal of occipital cavernomas may be associated with a risk of visual field defects. The goal of the study was to analyze long-term outcome after operative treatment of occipital cavernomas with special emphasis on visual outcome.

Methods

Of the 390 consecutive patients with cavernomas who were treated at Helsinki University Central Hospital between 1980 and 2011, 19 (5%) had occipital cavernomas. Sixteen patients (4%) were surgically treated and are included in this study. The median age was 39 years (range 3–59 years). Seven patients (56%) suffered from hemorrhage preoperatively, 5 (31%) presented with visual field deficits, 11 (69%) suffered from seizures, and 4 (25%) had multiple cavernomas. Surgery was indicated for progressive neurological deterioration. The median follow-up after surgery was 5.25 years (range 0.5–14 years).

Results

All patients underwent thorough neuroophthalmological assessment to determine visual outcome after surgery. Visual fields were classified as normal, mild homonymous visual field loss (not disturbing the patient, driving allowed), moderate homonymous visual field loss (disturbing the patient, driving prohibited), and severe visual field loss (total homonymous hemianopia or total homonymous quadrantanopia). At the last follow-up, 4 patients (25%) had normal visual fields, 6 (38%) had a mild visual field deficit, 1 (6%) complained of moderate visual field impairment, and 5 (31%) had severe homonymous visual field loss. Cavernomas seated deeper than 2 cm from the pial surface carried a 4.4-fold risk of postoperative visual field deficit relative to superficial ones (p = 0.034). Six (55%) of the 11 patients presenting with seizures were seizure-free postoperatively. Eleven (69%) of 16 patients had no disability during the long-term follow-up.

Conclusions

Surgical removal of occipital cavernomas may carry a significant risk of postoperative visual field deficit, and the risk is even higher for deeper lesions. Seizure outcome after removal of these cavernomas appeared to be worse than that after removal in other supratentorial locations. This should be taken into account during preoperative planning.

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Päivi Koroknay-Pál, Hanna Lehto, Mika Niemelä, Riku Kivisaari and Juha Hernesniemi

Object

Population-based data on pediatric patients with aneurysms are limited. The aim of this study is to clarify the characteristics and long-term outcomes of pediatric patients with aneurysms.

Methods

All pediatric patients (≤ 18 years old) with aneurysms among the 8996 aneurysm patients treated at the Department of Neurosurgery in Helsinki from 1937 to 2009 were followed from admission to the end of 2010.

Results

There were 114 pediatric patients with 130 total aneurysms during the study period. The mean patient age was 14.5 years (range 3 months to 18 years). The male:female ratio was 3:2. Eighty-nine patients (78%) presented with subarachnoid hemorrhage. The majority of the aneurysms (116 [89%]) were in the anterior circulation, and the most common location was the internal carotid artery bifurcation (36 [28%]). The average aneurysm diameter was 11 mm (range 2–55 mm) with 16 giant aneurysms (12%). Eighty aneurysms (62%) were treated microsurgically, and 37 (28%) were treated conservatively due to poor medical and neurological status of the patient or due to technical reasons during the early years of the patient series. No connective tissue disorders common to pediatric aneurysm patients were diagnosed in this series, with the exception of 1 patient with tuberous sclerosis complex. The mean follow-up duration was 24.8 years (range 0–55.8 years). At the end of follow-up, 71 patients (62%) had a good outcome, 3 (3%) were dependent, and 40 (35%) had died. Twenty-seven deaths (68%) were assessed to be aneurysm-related. Factors correlating with a favorable long-term outcome were good neurological condition of the patient on admission, aneurysm location in the anterior circulation, complete aneurysm closure, and absence of vasospasm. Six patients developed symptomatic de novo aneurysms after a median of 25 years (range 11–37 years). Fourteen patients (12%) had a family history of aneurysms. There was no increased incidence for cardiovascular diseases in long-term follow-up.

Conclusions

Most aneurysms were ruptured and of medium size. Internal carotid artery bifurcation was the most frequent location of the aneurysms. There was a male predominance of pediatric patients with aneurysms. Most patients experienced good recovery, with 91% of the long-term survivors living at home independently without assistance and meaningfully employed. Altogether, almost a third of these patients finished high school and one-fifth had a college or university degree. Pediatric patients had a tendency to develop de novo aneurysms.

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Anna Piippo, Aki Laakso, Karri Seppä, Jaakko Rinne, Juha E. Jääskeläinen, Juha Hernesniemi and Mika Niemelä

Object

The aim of this study was to assess the early and long-term excess mortality in patients with intracranial dural arteriovenous fistula (DAVF) compared with a matched general Finnish population in an unselected, population-based series.

Methods

The authors identified 227 patients with DAVFs admitted to 2 of the 5 Departments of Neurosurgery in Finland—Helsinki and Kuopio University Hospitals—between 1944 and 2006. All patients were followed until death or the end of 2009. Long-term excess mortality was estimated using the relative survival ratio compared with the general Finnish population matched by age, sex, and calendar year.

Results

The median follow-up period was 10 years (range 0–44 years). Two-thirds (67%) of the DAVFs were located in the region of transverse and sigmoid sinuses. Cortical venous drainage (CVD) was present in 28% of the DAVFs (18% transverse and sigmoid sinus, 42% others). Of the 61 deaths counted, 11 (18%) were during the first 12 months and were mainly caused by treatment complications (5 of 11, 45%). The 1-year survivors presenting with hemorrhage experienced excess mortality until 7 years from admission. However, DAVFs with CVD were associated with significant, continuous excess mortality. There were more cerebrovascular and cardiovascular deaths in this group of patients than expected in the general Finnish population. Location other than transverse and sigmoid sinuses was also associated with excess mortality.

Conclusions

In the patients with DAVF there was excess mortality during the first 12 months, mainly due to treatment complications. Thereafter, their overall long-term survival became similar to that of the matched general population. However, DAVFs with CVD and those located in regions other than transverse and sigmoid sinuses were associated with marked long-term excess mortality after the first 12 months.

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Anna Piippo, Mika Niemelä, Jouke van Popta, Marko Kangasniemi, Jaakko Rinne, Juha E. Jääskeläinen and Juha Hernesniemi

Object

Management of dural arteriovenous fistulas (DAVFs) has changed during the last decades due to increased knowledge of their pathophysiology and natural history as well as advances in treatment modalities. The authors describe the characteristics and long-term outcome of a large consecutive series of patients with DAVFs.

Methods

Altogether 251 patients with 261 DAVFs were treated in 2 of the 5 neurosurgery departments at Helsinki and Kuopio University Hospitals between 1944 and 2006. Clinical data and radiological examinations were reviewed to assess patients' overall long-term clinical outcome.

Results

The detection rate of DAVFs increased markedly in the 1970s and again in the 1990s when digital subtraction angiography was introduced. The incidence of DAVFs in a defined southern Finnish population was 0.51 per 100,000 individuals per year, which represents 32% of all the brain arteriovenous malformations. In the early part of the series, DAVFs were treated by proximal ligation of the feeding arteries. Later, most of the patients underwent preoperative embolization and subsequent craniotomy, and since 2000 stereotactic radiosurgery has been increasingly used in the treatment of DAVFs. Fifty-nine percent of the 261 fistulas were totally occluded. Treatment-related major complications were seen in 21 patients.

Conclusions

The advances in diagnostic methods (digital subtraction angiography, CT, and MRI) increased the detection rate of DAVFs, and as treatment modalities developed, the results of treatment and outcome of patients markedly improved with the introduction of endovascular techniques and stereotactic radiosurgery. Microsurgery is of limited use in DAVFs resistant to other treatment modalities.

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Leena Kivipelto, Mika Niemelä, Torstein Meling, Martin Lehecka, Hanna Lehto and Juha Hernesniemi

Object

The object of this study was to describe the authors' institutional experience in the treatment of complex middle cerebral artery (MCA) aneurysms necessitating bypass and vessel sacrifice.

Methods

Cases in which patients with MCA aneurysms were treated with a combination of bypass and parent artery sacrifice were reviewed retrospectively.

Results

The authors identified 24 patients (mean age 46 years) who were treated with bypass and parent artery sacrifice. The aneurysms were located in the M1 segment in 7 patients, MCA bifurcation in 8, and more distally in 9. The mean aneurysm diameter was 30 mm (range 7–60 mm, median 26 mm). There were 8 saccular and 16 fusiform aneurysms.

Twenty-one extracranial-intracranial and 4 intracranial-intracranial bypasses were performed. Partial or total trapping (only) of the parent artery was performed in 17 cases, trapping with resection of aneurysm in 3, and aneurysm clipping with sacrifice of an M2 branch in 4.

The mean follow-up period was 27 months. The aneurysm obliteration rate was 100%. No recanalization of the aneurysms was detected during follow-up.

There was 1 perioperative death (4% mortality rate) and 6 cerebrovascular accidents, causing permanent morbidity in 5 patients. The median modified Rankin Scale score of patients with an M1 aneurysm increased from 0 preoperatively to 2 at latest follow-up, while the score was unchanged in other patients. Most of the permanent deficits were associated with M1 aneurysms. Twenty-one patients (88%) had good outcome as defined by a Glasgow Outcome Scale score of 4 or 5.

Conclusions

Bypass in combination with parent vessel occlusion is a useful technique with acceptable frequencies of morbidity and mortality for complex MCA aneurysms when conventional surgical or endovascular techniques are not feasible. The location of the aneurysm should be considered when planning the type of bypass and the site of vessel occlusion. Flow alteration by partial trapping may be preferable to total trapping for the M1 aneurysms.