Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease

2010 ◽  
Vol 12 (3) ◽  
pp. 233-242 ◽  
Author(s):  
Gautam U. Mehta ◽  
Ashok R. Asthagiri ◽  
Kamran D. Bakhtian ◽  
Sungyoung Auh ◽  
Edward H. Oldfield ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Effective Means ◽  
Neurological Function ◽  
Von Hippel Lindau ◽  
Presenting Symptoms ◽  
Increased Risk ◽  
Functional Scores ◽  
Vhl Disease

Object Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas. Methods Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed. Results One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 ± 5.0 years (range 0.5–20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54–96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07–55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients). Conclusions Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.

scholarly journals Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease

2011 ◽  
Vol 114 (5) ◽  
pp. 1312-1318 ◽  
Author(s):  
Joshua J. Wind ◽  
Kamran D. Bakhtian ◽  
Jennifer A. Sweet ◽  
Gautam U. Mehta ◽  
Jayesh P. Thawani ◽  
...  
Keyword(s):  
Functional Status ◽  
Functional Decline ◽  
Long Term Results ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Von Hippel Lindau ◽  
Presenting Symptoms ◽  
Vhl Disease

Object Brainstem hemangioblastomas are frequently encountered in patients with von Hippel-Lindau (VHL) disease. These tumors can cause significant morbidity, and their optimal management has not been defined. To better define the outcome and management of these tumors, the authors analyzed the long-term results in patients who underwent resection of brainstem hemangioblastomas. Methods Consecutive patients with VHL disease who underwent resection of brainstem hemangioblastomas with a follow-up of 12 months or more were included in this study. Serial functional assessments, radiographic examinations, and operative records were analyzed. Results Forty-four patients (17 male and 27 female) underwent 51 operations for resection of 71 brainstem hemangioblastomas. The most common presenting symptoms were headache, swallowing difficulties, singultus, gait difficulties, and sensory abnormalities. The mean follow-up was 5.9 ± 5.0 years (range 1.0–20.8 years). Immediately after 34 operations (66.7%), the patients remained at their preoperative functional status; they improved after 8 operations (15.7%) and worsened after 9 operations (17.6%) as measured by the McCormick scale. Eight (88.9%) of the 9 patients who were worse immediately after resection returned to their preoperative status within 6 months. Two patients experienced functional decline during long-term follow-up (beginning at 2.5 and 5 years postoperatively) caused by extensive VHL disease–associated CNS disease. Conclusions Generally, resection of symptomatic brainstem hemangioblastomas is a safe and effective management strategy in patients with VHL disease. Most patients maintain their preoperative functional status, although long-term decline in functional status may occur due to VHL disease–associated progression.

scholarly journals Spinal cord hemangioblastomas: significance of intraoperative neurophysiological monitoring for resection and long-term outcome

2017 ◽  
Vol 26 (4) ◽  
pp. 483-493 ◽  
Author(s):  
Sebastian Siller ◽  
Andrea Szelényi ◽  
Lisa Herlitz ◽  
Joerg Christian Tonn ◽  
Stefan Zausinger
Keyword(s):  
Spinal Cord ◽  
Intraoperative Neurophysiological Monitoring ◽  
Neurophysiological Monitoring ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Von Hippel Lindau ◽  
Sensorimotor Deficits ◽  
Vhl Disease

OBJECTIVE Spinal cord hemangioblastomas are rare benign tumors developing either sporadically or as part of von Hippel-Lindau (VHL) disease. Generally, resection is the treatment of choice. However, the significance of intraoperative neurophysiological monitoring (IONM) for resection and postoperative outcome is still controversial. The authors analyzed the surgical and clinical courses of patients who had undergone resection of spinal cord hemangioblastoma, with special attention to preoperative imaging, the use of IONM, and short- and long-term outcomes. METHODS A series of 24 patients (male/female 1:1, lesion sporadic/associated with VHL 2.4:1) who had undergone 26 operations for the resection of 27 spinal cord hemangioblastomas was analyzed. All patients had undergone pre- and postoperative contrast-enhanced MRI. In all cases, microsurgical tumor removal had been performed under continuous IONM of both somatosensory and transcranial motor evoked potentials as well as electromyographic recording. Clinical characteristics, imaging findings, and operative records were retrospectively analyzed. Outcome parameters included short- and long-term status as regards sensorimotor deficits and a questionnaire on general performance, patient satisfaction, and Oswestry Disability Index (ODI) at the end of the follow-up period. The impact of IONM findings on postoperative deficits and outcome parameters as well as risk factors affecting functional prognosis was statistically assessed. RESULTS Preoperative symptoms (mean duration 16.2 ± 22.0 months) included sensory changes (100.0%), pain (66.7%), spinal ataxia (66.7%), motor deficit (41.7%), and bladder/bowel dysfunction (12.5%). Average age at the first operation was 36.8 ± 12.8 years. Most tumors (21 intramedullary, 6 intra- and/or extramedullary) were located dorsally (92.6%) and cervically (77.8%) and were accompanied by peritumoral edema and/or syringomyelia (81.5%). Tumor resection was achieved via laminectomy for 15 tumors, hemilaminectomy for 5, laminoplasty for 6, and interlaminar approach for 1. Gross-total resection was accomplished for 26 tumors (96.3%) with no local tumor recurrence during follow-up. Intraoperative neurophysiological monitoring was nonpathological in 11 operations (42.3%) and pathological in 15 (57.7%). Patients with nonpathological IONM had significantly fewer new sensorimotor deficits (p = 0.005). Long-term follow-up evaluation (mean 7.9 ± 4.0 years postoperatively, 7 patients lost to follow-up) revealed a stable or improved McCormick myelopathy grade in 88.2% of the patients, and 88.2% reported a stable or improved overall outcome according to Odom's criteria. Long-term general performance was excellent with 88.2% having a WHO/Eastern Cooperative Oncology Group (ECOG) Performance Status grade ≤ 1, 76.5% a Karnofsky Performance Scale score ≥ 80, and 70.6% a Barthel Index (BI) of 100. The mean ODI (11.4% ± 12.5%) indicated only minimal disability. There was a significant correlation between pathological IONM findings and a worse long-term status according to the BI and ODI (p = 0.011 and 0.024, respectively). Additionally, VHL disease was a risk factor affecting functional prognosis (p = 0.044). CONCLUSIONS Microsurgical removal of spinal cord hemangioblastomas with IONM facilitates a satisfying long-term outcome for patients. Nonpathological IONM findings are associated with a lower risk of new sensorimotor deficits and correlate with a better overall long-term outcome. von Hippel–Lindau disease is a risk factor for a worse long-term prognosis.

Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease

Neurosurgery ◽  
2001 ◽  
Vol 48 (1) ◽  
pp. 55-63 ◽  
Author(s):  
James E. Conway ◽  
Dean Chou ◽  
Richard E. Clatterbuck ◽  
Henry Brem ◽  
Donlin M. Long ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Von Hippel Lindau ◽  
Multiple Lesions ◽  
Younger Age ◽  
The Central Nervous System ◽  
Sporadic Disease ◽  
Vhl Disease

Abstract OBJECTIVE The presentation, screening, management, and clinical outcomes of patients who presented to our institution from 1973 to 1999 with central nervous system (CNS) hemangioblastomas in von Hippel-Lindau (VHL) syndrome and sporadic disease were analyzed. METHODS The surgical pathology database of our institution was searched to identify all patients with histologically verified CNS hemangioblastomas occurring from 1973 to 1999. The medical, radiological, surgical, pathological, and autopsy records from these patients were reviewed retrospectively and statistically analyzed. RESULTS Forty patients (21 males and 19 females) presented with CNS hemangioblastomas. Twenty-five patients (62%) harbored sporadic hemangioblastomas. Fifteen patients (38%) had VHL syndrome. These 40 patients presented with 61 hemangioblastomas (8 patients had multiple lesions). Ten patients (25%) harbored spinal cord hemangioblastomas (5 patients had multiple lesions). Patients with VHL disease tended to present with neurological symptoms and signs at a younger age than patients with sporadic disease (P = 0.09), to present with multiple lesions (53%), and to develop new lesions (rate, 1 lesion/2.1 yr). Hemangioblastomas of the spinal cord were more prevalent in patients with VHL syndrome (P = 0.024). Neuroradiological screening of patients with VHL syndrome allowed identification of more than 75% of new lesions before they became symptomatic. Sixty-six surgical procedures were performed (12 patients required multiple operations). Six patients with VHL syndrome required surgery for new lesions. Surgical complications occurred in six patients (15%). Symptom resolution or arrest of progression at 1 year was documented in 88% of patients. Recurrence of symptoms from partially resected lesions occurred in eight patients (20%). No deaths associated with surgery occurred. One patient with sporadic disease and one patient with VHL syndrome (5%) died as a result of late medical complications from CNS hemangioblastomas. CONCLUSION Surgical outcomes for patients with CNS hemangioblastomas are favorable. However, management of hemangioblastomas is a more difficult and prolonged endeavor for patients with VHL syndrome. In patients with VHL syndrome, neuroradiological screening allows identification of lesions before they become symptomatic. Because patients with VHL syndrome are at risk for development of new lesions, they require lifelong follow-up.

Metastases to hemangioblastomas in von Hippel–Lindau disease

2006 ◽  
Vol 105 (2) ◽  
pp. 256-263 ◽  
Author(s):  
S. Taylor Jarrell ◽  
Alexander O. Vortmeyer ◽  
W. Marston Linehan ◽  
Edward H. Oldfield ◽  
Russell R. Lonser
Keyword(s):  
Pancreatic Neuroendocrine Tumor ◽  
Von Hippel Lindau ◽  
Increased Risk ◽  
Age At Surgery ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Histological Characteristics ◽  
Tumor Metastases ◽  
Vhl Disease

Object Patients with hereditary cancer syndromes may be at increased risk for the development of tumor-to-tumor metastases. To gain insight into the biological nature of these lesions in the central nervous system (CNS), to determine their prevalence in a familial neoplasia syndrome, and to better define their management, the authors retrospectively examined a series of cases in which metastatic lesions developed within hemangioblastomas in patients with von Hippel–Lindau (VHL) disease. Methods The study included all cases of VHL disease in which patients underwent resection of a CNS hemangioblastoma that contained a metastasis or were found at autopsy to have a metastasis to a hemangioblastoma between January 2002 and December 2005 at the National Institute of Neurological Disorders and Stroke (NINDS). Clinical, histopathological, imaging, and surgical and/or autopsy findings were analyzed. Metastasis to a CNS hemangioblastoma was found in six resected tumors (8% of all hemangioblastomas resected from patients with VHL disease at the NINDS during the study period) from six patients (five women, one man; mean age at surgery 42.5 years). The primary site of metastatic disease was the kidney in five patients (renal cell carcinoma) and the pancreas in one (a pancreatic neuroendocrine tumor). Only one patient had systemic metastases at the time of resection of the hemangioblastoma containing the metastasis. Neurologically, all patients had remained at baseline or were improved at last clinical follow-up examination (mean follow-up duration 16.5 months, range 3–40 months). In all cases, postoperative imaging revealed that the hemangioblastoma resection was complete, and there was no evidence of recurrence in any of the patients at the last follow up. Two patients (including one who was also in the surgical group) were found at autopsy to have CNS metastases exclusively to spinal hemangioblastomas. Conclusions Hemangioblastomas are an early and preferred site for metastasis in VHL disease. Emerging histopathological techniques may lead to recognition of an increasing number of cases of tumor-to-hemangioblastoma metastasis. Management of cases involving tumor-to-hemangioblastoma metastases in VHL disease should be based on the histological characteristics of the primary tumor, extent of the primary disease, and completeness of the resection.

scholarly journals Syringomyelia in children with closed spinal dysraphism: long-term outcomes after surgical intervention

2020 ◽  
Vol 25 (3) ◽  
pp. 319-325 ◽  
Author(s):  
Amy K. Bruzek ◽  
Jordan Starr ◽  
Hugh J. L. Garton ◽  
Karin M. Muraszko ◽  
Cormac O. Maher ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Clinical Symptoms ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Presenting Symptoms ◽  
Age At Surgery ◽  
Intradural Lipoma ◽  
The Relationship

OBJECTIVEThe nature of the relationship between spinal cord syrinx and tethered cord is not well known. It is unclear if surgical cord untethering results in resolution or improvement of an associated syrinx. The objective of this study was to report the response of spinal cord syrinx to surgical cord untethering.METHODSThe authors retrospectively reviewed all patients with a syrinx and tethered cord who presented to a single institution over an 11-year interval. Patients with open neural tube defects were excluded. Thirty-one patients were identified, 25 of whom had both clinical and imaging follow-up after surgery. Patients were grouped according to etiology of the tethered cord. Clinical outcomes and syrinx characteristics were recorded.RESULTSOf the 25 patients with tethered cord, 68% (n = 17) were male. The average age at presentation was 2.5 years (0–10.1 years) and age at surgery was 3.7 years (range 1 day to 17 years). Etiologies of tethered cord were lipomyelomeningocele (n = 8), thickened/fatty filum (n = 7), intradural lipoma (n = 5), myelocystocele (n = 2), meningocele (n = 2), and diastematomyelia (n = 1). Twenty-three of the patients underwent primary untethering, whereas 2 patients had received untethering previously at another institution. The average syrinx length and width prior to surgery were 4.81 vertebral levels (SD 4.35) and 5.19 mm (SD 2.55 mm), respectively. Conus level ranged from L1 to S3. Patients were followed for an average of 8.4 years (1.35–15.85 years). Overall there was no significant change in syrinx length or width postoperatively; the average syrinx length increased by 0.86 vertebral levels (SD 4.36) and width decreased by 0.72 mm (SD 2.94 mm). Seven of 25 patients had improvement in at least one presenting symptom, including scoliosis, weakness, bowel/bladder dysfunction, and pain. Eight patients had stable presenting symptoms. Six patients were asymptomatic and 5 patients had new or worsening symptoms, which included scoliosis, pain, or sensory changes.CONCLUSIONSAlthough some syrinxes improved after surgery for tethered cord, radiological improvement was not consistent and did not appear to be associated with change in clinical symptoms. The decision to surgically untether a cord should be focused on the clinical symptoms and not the presence of a syrinx alone. Further studies are needed to confirm this finding.

Surgical management of brainstem hemangioblastomas in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 95-105 ◽  
Author(s):  
Robert J. Weil ◽  
Russell R. Lonser ◽  
Hetty L. Devroom ◽  
John E. Wanebo ◽  
Edward H. Oldfield
Keyword(s):  
Magnetic Resonance ◽  
Preoperative Evaluation ◽  
Magnetic Resonance Images ◽  
Long Term Outcome ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Hemangioblastomas of the brainstem constitute 5 to 10% of central nervous system (CNS) tumors in patients with von Hippel—Lindau (VHL) disease. At present, optimal management of brainstem hemangioblastomas associated with VHL disease is incompletely defined. In an attempt to clarify some of the uncertainty about the operative treatment of these lesions and its outcome, the authors reviewed all cases of VHL disease in which resection of brainstem hemangioblastomas was performed at the National Institutes of Health during a 10-year period. Methods. Twelve consecutive patients with VHL disease (six male and six female patients [mean age 31.7 ± 9 years; range 15–46 years]) who underwent 13 operations to remove 17 brainstem hemangioblastomas were included in this study (mean follow-up period, 88.4 ± 37.4 months; range 37–144 months). Serial examinations, hospital charts, magnetic resonance images, and operative records were reviewed. To evaluate clinical course, clinical grades were assigned to each patient before and after surgery. Preoperative neurological function was the best predictor of long-term outcome. In addition, patients who underwent CNS surgeries for hemangioblastomas were more likely to improve or to remain neurologically stable. Tumor or cyst size, the presence of a cyst, or the location of the tumor (intramedullary, extramedullary, or mixed; posterior medullary, obex, or lateral) did not affect outcome. No patient was neurologically worse after brainstem surgery. At long-term follow-up review (mean 88.4 months), only one patient had declined neurologically and this was due to the cumulative neurological effects caused by eight additional hemangioblastomas of the spinal cord and their surgical treatment. Conclusions. Brainstem hemangioblastomas in patients with VHL disease can be removed safely; they generally should be resected when they become symptomatic or when the tumor has reached a size such that further growth will increase the risks associated with surgery, or in the presence of an enlarging cyst. Magnetic resonance imaging is usually sufficient for preoperative evaluation and presurgical embolization is unnecessary. The goal of surgery is complete resection of the lesion before the patient experiences a disabling neurological deficit.

scholarly journals Microsurgical Treatment of Sporadic and von Hippel-Lindau Disease Associated Spinal Hemangioblastomas: A Single-Institution Experience

2017 ◽  
Vol 11 (4) ◽  
pp. 548-555 ◽  
Author(s):  
Joe M. Das ◽  
Krishnakumar Kesavapisharady ◽  
Saravanan Sadasivam ◽  
Suresh Narayanan Nair
Keyword(s):  
Postoperative Period ◽  
Functional Outcomes ◽  
Neurological Status ◽  
Neurological Deterioration ◽  
Gradual Improvement ◽  
Long Term Outcome ◽  
Von Hippel Lindau ◽  
Vhl Disease

<sec><title>Study Design</title><p>Retrospective cohort study.</p></sec><sec><title>Purpose</title><p>To examine the clinical profile and surgical complications in patients with spinal hemangioblastomas and to evaluate the long-term outcome in them.</p></sec><sec><title>Overview of Literature</title><p>Although considered to be histologically benign, hemangioblastomas may cause significant neurological deficits. The proportion of spinal hemangioblastomas associated with von Hippel–Lindau (VHL) disease has been estimated be 13%–59%. Preoperative neurological function correlates with postoperative neurological status. Studies have shown no difference in outcomes between sporadic and VHL-associated spinal hemangioblastomas.</p></sec><sec><title>Methods</title><p>This retrospective study included 14 consecutive patients treated for spinal hemangioblastomas at our institute between January 2000 and June 2013. The mean follow-up period was 5 years. Magnetic resonance imaging of the complete neuraxis was performed in all cases, and preoperative embolization was performed in two cases.</p></sec><sec><title>Results</title><p>In total, 14 patients underwent 18 surgeries, of which 15 were for spinal hemangioblastomas. Of all the patients, 86% had motor weakness and 79% presented with sensory disturbances. Preoperative McCormick functional grades were grade I in 7 (50%), grade II in 3 (21%), and grade III in 4 (29%) patients; 50% patients were diagnosed with VHL disease. All patients underwent complete resection of the tumor. Eight patients experienced deterioration in their neurological status in the immediate postoperative period; among them, five had gradual improvement. At 5-year follow-up, 11 (78.57%) patients showed good functional outcomes.</p></sec><sec><title>Conclusions</title><p>Microsurgical excision of spinal hemangioblastomas can cause postoperative morbidity, mainly in the form of neurological deterioration. Almost half of our patients had deterioration in the McCormick grade in the immediate postoperative period. However, a complete microsurgical excision can result in good long-term functional outcomes, as most of the immediate postoperative neurological deterioration in our patients was reversible. There was no difference in the long-term functional outcomes between sporadic and VHL-associated spinal hemangioblastomas.</p></sec>

Long-Term Follow-Up After Spinal Cord Injury in Canada

2006 ◽  
Author(s):  
Mark I. Tonack ◽  
Sander L. Hitzig ◽  
B. Catharine Craven ◽  
Kent A. Campbell ◽  
Kathryn A. Boschen ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Cord Injury ◽  
Long Term Follow Up

OPEN HAEMORRHOIDS SURGERY IN SPINAL CORD INJURY PATIENTS

2020 ◽  
Vol 4 (11) ◽  
Author(s):  
Khaled Hassan
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Anal Fissure ◽  
Open Surgery ◽  
Anal Fissures ◽  
Cord Injury ◽  
Long Term Follow Up ◽  
Grade Iii

This Pilot retrospective research conducted on the results of open surgery in patients with Grade III and IV haemorrhoids With SCI. No major complications had arisen at 6 weeks post-operative and all wounds had healed, but 1 patient Anal fissure recurrence. 75% of patients reported a substantial increase in anorectal anorexia during long-term follow-up. With symptoms. Five patients reported recurrences: three haemorrhoids (18 percent) and two anal fissures (25 percent).   Keywords: Haemorrhoids, Pilot retrospective research, Anorectal Anorexia.

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