Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 117-124 ◽  
Author(s):  
Ryszard M. Pluta ◽  
Brian Iuliano ◽  
Hetty L. Devroom ◽  
Tung Nguyen ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Anterior Approach ◽  
Posterior Approach ◽  
Long Term Results ◽  
Surgical Approaches ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Ventral Spinal Cord ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant neoplastic syndrome with manifestations in multiple organs, which is evoked by the deletion or mutation of a tumor suppressor gene on chromosome 3p25. Spinal hemangioblastomas (40% of VHL disease—associated lesions of the central nervous system) arise predominantly in the posterior aspect of the spinal cord and are often associated with an intraspinal cyst. Rarely, the tumor develops in the anterior aspect of the spinal cord. Ventral spinal hemangioblastomas are a surgical challenge because of difficult access and because vessels feeding the tumor originate from the anterior spinal artery. The goal of this study was to clarify whether an anterior or posterior surgical approach is better for management of hemangioblastomas of the ventral spinal cord. Methods. The authors performed a retrospective analysis of clinical outcomes and findings on magnetic resonance (MR) imaging studies in eight patients (two women and six men with a mean age of 34 ± 15 years) who underwent resection of ventral spinal hemangioblastomas (nine tumors: five cervical and four thoracic). Two surgical approaches were used to resect these tumors. A posterior approach was selected to treat five patients (laminectomy and posterior myelotomy in four patients and the posterolateral approach in one patient); an anterior approach (corpectomy and arthrodesis) was selected to treat the remaining three patients. Immediately after surgery, the ability to ambulate remained unchanged in patients in whom an anterior approach had been performed, but deteriorated significantly in patients in whom a posterior approach had been used, because of motor weakness (four of five patients) and/or proprioceptive sensory loss (three of five patients). This difference in ambulation, despite significant improvements over time among patients in the posterior access group, remained significant 6 months after surgery. In all cases, MR images revealed complete resection of the tumor and in five patients significant or complete resolution of the intramedullary cyst was demonstrated (present in six of eight patients). Conclusions. The outcomes of these eight patients with hemangioblastomas of the ventral spinal cord indicate that both immediate and long-term results are better when an anterior approach is selected for resection.

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

2003 ◽  
Vol 98 (1) ◽  
pp. 106-116 ◽  
Author(s):  
Russell R. Lonser ◽  
Robert J. Weil ◽  
John E. Wanebo ◽  
Hetty L. Devroom ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Surgical Management ◽  
Postoperative Outcome ◽  
Tumor Location ◽  
Neurological Dysfunction ◽  
Autosomal Dominant Disorder ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients. Methods. Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20–58 years) at the National Institutes of Health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. Patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered. Conclusions. Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.

The natural history of hemangioblastomas of the central nervous system in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 82-94 ◽  
Author(s):  
John E. Wanebo ◽  
Russell R. Lonser ◽  
Gladys M. Glenn ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Mass Effect ◽  
Content Type ◽  
Von Hippel Lindau ◽  
History Of ◽  
The Central Nervous System ◽  
Brainstem Tumors ◽  
Vhl Disease ◽  
Fine Print

Object. The goals of this study were to define the natural history and growth pattern of hemangioblastomas of the central nervous system (CNS) that are associated with von Hippel—Lindau (VHL) disease and to correlate features of hemangioblastomas that are associated with the development of symptoms and the need for treatment. Methods. The authors reviewed serial magnetic resonance images and clinical histories of 160 consecutive patients with VHL disease who harbored CNS hemangioblastomas and serially measured the volumes of tumors and associated cysts. Six hundred fifty-five hemangioblastomas were identified in the cerebellum (250 tumors), brainstem (64 tumors, all of which were located in the posterior medulla oblongata), spinal cord (331 tumors, 96% of which were located in the posterior half of spinal cord), and the supratentorial brain (10 tumors). The symptoms were related to a mass effect. A serial increase in hemangioblastoma size was observed in cerebellar, brainstem, and spinal cord tumors as patients progressed from being asymptomatic to symptomatic and requiring surgery (p < 0.0001). Twenty-one (72%) of 29 symptom-producing cerebellar tumors had an associated cyst, whereas only 28 (13%) of 221 nonsymptomatic cerebellar tumors had tumor-associated cysts (p < 0.0001). Nine (75%) of 12 symptomatic brainstem tumors had associated cysts, compared with only four (8%) of 52 nonsymptomatic brainstem lesions (p < 0.0001). By the time the symptoms appeared and surgery was required, the cyst was larger than the causative tumor; cerebellar and brainstem cysts measured 34 and 19 times the size of their associated tumors at surgery, respectively. Ninety-five percent of symptom-producing spinal hemangioblastomas were associated with syringomyelia. The clinical circumstance was dynamic. Among the 88 patients who had undergone serial imaging for 6 months or longer (median 32 months), 164 (44%) of 373 hemangioblastomas and 37 (67%) of 55 tumor-associated cysts enlarged. No tumors or cysts spontaneously diminished in size. Symptomatic cerebellar and brainstem tumors grew at rates six and nine times greater, respectively, than asymptomatic tumors in the same regions. Cysts enlarged seven (cerebellum) and 15 (brainstem) times faster than the hemangioblastomas causing them. Hemangioblastomas frequently demonstrated a pattern of growth in which they would enlarge for a period of time (growth phase) and then stabilize in a period of arrested growth (quiescent phase). Of 69 patients with documented tumor growth, 18 (26%) harbored tumors with at least two growth phases. Of 160 patients with hemangioblastomas, 34 patients (median follow up 51 months) were found to have 115 new hemangioblastomas and 15 patients new tumor-associated cysts. Conclusions. In this study the authors define the natural history of CNS hemangioblastomas associated with VHL disease. Not only were cysts commonly associated with cerebellar, brainstem, and spinal hemangioblastomas, the pace of enlargement was much faster for cysts than for hemangioblastomas. By the time symptoms appeared, the majority of mass effect—producing symptoms derived from the cyst, rather than from the tumor causing the cyst. These tumors often have multiple periods of tumor growth separated by periods of arrested growth, and many untreated tumors may remain the same size for several years. These characteristics must be considered when determining the optimal timing of screening for individual patients and for evaluating the timing and results of treatment.

Posterior approach for cervical intramedullary arteriovenous malformation with diffuse-type nidus

1999 ◽  
Vol 91 (1) ◽  
pp. 105-111 ◽  
Author(s):  
Kenji Ohata ◽  
Toshihiro Takami ◽  
Alaa El-Naggar ◽  
Michiharu Morino ◽  
Akimasa Nishio ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Poor Prognosis ◽  
Posterior Approach ◽  
The Other ◽  
Anterior Spinal Artery ◽  
Neural Element ◽  
Feeding Artery ◽  
Diffuse Type ◽  
Content Type ◽  
Fine Print

✓ The treatment of spinal intramedullary arteriovenous malformations (AVMs) with a diffuse-type nidus that contains a neural element poses different challenges compared with a glomus-type nidus. The surgical elimination of such lesions involves the risk of spinal cord ischemia that results from coagulation of the feeding artery that, at the same time, supplies cord parenchyma. However, based on evaluation of the risks involved in performing embolization, together with the frequent occurrence of reperfusion, which necessitates frequent reembolization, the authors consider surgery to be a one-stage solution to a disease that otherwise has a very poor prognosis. Magnetic resonance (MR) imaging revealed diffuse-type intramedullary AVMs in the cervical spinal cords of three patients who subsequently underwent surgery via the posterior approach. The AVM was supplied by the anterior spinal artery in one case and by both the anterior and posterior spinal arteries in the other two cases. In all three cases, a posterior median myelotomy was performed up to the vicinity of the anterior median fissure that divided the spinal cord together with the nidus, and the feeding artery was coagulated and severed at its origin from the anterior spinal artery. In the two cases in which the posterior spinal artery fed the AVM, the feeding artery was coagulated on the dorsal surface of the spinal cord. Neurological outcome improved in one patient and deteriorated slightly to mildly in the other two patients. Postoperative angiography demonstrated complete disappearance of the AVM in all cases. Because of the extremely poor prognosis of patients with spinal intramedullary AVMs, this surgical technique for the treatment of diffuse-type AVMs provides acceptable operative outcome. Surgical intervention should be considered when managing a patient with a diffuse-type intramedullary AVM in the cervical spinal cord.

Spinal Cord Herniation

2005 ◽  
Vol 3 (6) ◽  
pp. 508-509
Author(s):  
Laurence Marshman
Keyword(s):  
Spinal Cord ◽  
Dura Mater ◽  
Posterior Approach ◽  
Direct Manipulation ◽  
Dural Repair ◽  
Content Type ◽  
Novel Approach ◽  
Spinal Cord Herniation ◽  
Thoracic Cord ◽  
Fine Print

Anterior spinal cord herniation is a well-documented condition in which the thoracic cord becomes tethered within a defect in the anterior dura mater. Typical procedures have involved a posterior approach with direct manipulation of the thoracic cord to expose and blindly release its point of tethering. The authors report three cases in which a novel approach for the treatment of anterior thoracic cord herniation was performed, cord manipulation and traction are minimized, and direct dural repair of the defect is performed.

Lipoma with dumb-bell extradural extension through the intervertebral foramen into the spinal canal

2005 ◽  
Vol 2 (1) ◽  
pp. 69-71 ◽  
Author(s):  
Jin Soo Park ◽  
Isao Shirachi ◽  
Kimiaki Sato ◽  
Noriyuki Ando ◽  
Kensei Nagata
Keyword(s):  
Vertebral Artery ◽  
Spinal Canal ◽  
Anterior Approach ◽  
Posterior Approach ◽  
Intervertebral Foramen ◽  
Nerve Roots ◽  
Content Type ◽  
First Report ◽  
Fine Print

✓ The authors present the case of a 60-year-old woman with a neck lipoma that developed dumb-bell extradural extension, causing radiculopathy. To the best of the authors' knowledge, this is the first report of a lipoma originating in the neck with dumb-bell extradural extension through the intervertebral foramen and into the spinal canal. The lipoma was first excised from the foramen via a posterior approach to allow decompression of the nerve roots. The remaining lipomatous tissue was then resected via an anterior approach to avoid the region around the vertebral artery.

Lateral interscalenic multilevel oblique corpectomies to repair ventral root avulsions after brachial plexus injury in humans: anatomical study and first clinical experience

2001 ◽  
Vol 95 (2) ◽  
pp. 202-207 ◽  
Author(s):  
Henri-Dominique Fournier ◽  
Philippe Mercier ◽  
Philippe Menei
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Anterior Approach ◽  
Cervical Spinal Cord ◽  
Anatomical Study ◽  
Ventral Root ◽  
Anterolateral Approach ◽  
Content Type ◽  
Anterior Aspect ◽  
Fine Print

Object. Because central nervous system white matter exerts a powerful inhibitory effect on axonal growth, implantation of nerve grafts or rootlets into the cervical spinal cord following ventral root avulsion injury should, ideally, be performed directly through the ventral root exit zone (VRExZ), which is located near the anteromedial aspect of the anterior horn; the grafts/rootlets should not be implanted into the white matter of the lateral cord. This is not possible when using a conservative posterior approach. Therefore, the authors have studied the anatomy encountered when using the anterolateral approach and evaluated the technique in the particular case of avulsed ventral nerve roots. They also present a case illustration of the procedure, which is used currently in their department. Methods. Anterior access to the rootlets is obtained using a lateral interscalenic approach; the vertebral artery is exposed and mobilized, and oblique drilling of the vertebral bodies (VBs) is performed. Because the articular processes and half of the VBs are preserved, fusion is not required. The approach allows the surgeon to expose the anterior aspect of the cervical dura and the entire length of the emerging spinal nerves. The anterior aspect of the dura is opened at the desired levels for VRExZ exposure, and the position is ideal for implantation of the graft/rootlets. The interscalenic dissection is mandatory so that the lesions of the supraclavicular plexus can be evaluated and repaired. If necessary, the anterior approach allows for exploration of the infraclavicular plexus during the same procedure. Conclusions. The use of a true anterior approach to the ventral rootlets appears to be a valuable and appropriate approach that avoids extensive laminectomy/facetectomy while reimplantation is performed through the anterolateral sulcus itself. In this approach, however, reimplantation of dorsal roots into the spinal cord remains impossible.

Gamma knife surgery for trigeminal neuralgia: outcomes and prognostic factors

2005 ◽  
Vol 102 (3) ◽  
pp. 434-441 ◽  
Author(s):  
Jason Sheehan ◽  
Hung-Chuan Pan ◽  
Matei Stroila ◽  
Ladislau Steiner
Keyword(s):  
Trigeminal Neuralgia ◽  
Gamma Knife ◽  
Univariate Analysis ◽  
Gamma Knife Surgery ◽  
Long Term Results ◽  
Surgical Approaches ◽  
Content Type ◽  
Facial Numbness ◽  
Fine Print

Object. Microvascular decompression (MVD) and percutaneous ablation surgery have historically been the treatments of choice for medically refractory trigeminal neuralgia (TN). Gamma knife surgery (GKS) has been used as an alternative, minimally invasive treatment in TN. In the present study, the authors evaluated the long-term results of GKS in the treatment of TN. Methods. From 1996 to 2003, 151 cases of TN were treated with GKS. In this group, radiosurgery was performed once in 136 patients, twice in 14 patients, and three times in one patient. The types of TN were as follows: 122 patients with typical TN, three with atypical TN, four with multiple sclerosis—associated TN, and seven with TN and a history of a cavernous sinus tumor. In each case, the chosen radiosurgical target was located 2 to 4 mm anterior to the entry of the trigeminal nerve into the pons. The maximal radiation doses ranged from 50 to 90 Gy. The median age of the patients was 68 years (range 22–90 years), and the median time from diagnosis to GKS was 72 months (range 1–276 months). The median follow up was 19 months (range 2–96 months). Clinical outcomes and postradiosurgical magnetic resonance (MR) imaging studies were analyzed. Univariate and multivariate analyses were performed to evaluate factors that correlated with a favorable, pain-free outcome. The mean time to relief of pain was 24 days (range 1–180 days). Forty-seven, 45, and 34% of patients were pain free without medication at the 1-, 2-, and 3-year follow ups, respectively. Ninety, 77, and 70% of patients experienced some improvement in pain at the 1-, 2-, and 3-year follow ups, respectively. Thirty-three (27%) of 122 patients with initial improvement subsequently experienced pain recurrence a median of 12 months (range 2–34 months) post-GKS. Among those whose symptoms recurred, 14 patients underwent additional GKS, six MVD, four glycerol injection, and one patient a percutaneous radiofrequency rhizotomy. Twelve patients (9%) suffered the onset of new facial numbness post-GKS. Changes on MR images post-GKS were noted in nine patients (7%). On univariate analysis, right-sided neuralgia (p = 0.0002) and a previous neurectomy (p = 0.04) correlated with a pain-free outcome; on multivariate analysis, both right-sided neuralgia (p = 0.032) and patient age (p = 0.05) were statistically significant. New onset of facial numbness following GKS correlated with undergoing more than one GKS (p = 0.002). Conclusions. At the last follow up, GKS effected pain relief in 44% of patients. Some degree of pain improvement at 3 years post-GKS was noted in 70% of patients with TN. Although less effective than MVD, GKS remains a reasonable treatment option for those unwilling or unable to undergo more invasive surgical approaches and offers a low risk of side effects.

Combined posteroanterior approach to a tumor of the cervical spinal foramen

1972 ◽  
Vol 37 (1) ◽  
pp. 113-116 ◽  
Author(s):  
Mutaz B. Habal ◽  
J. Gordon McComb ◽  
John Shillito ◽  
Howard M. Eisenberg ◽  
Joseph E. Murray
Keyword(s):  
Anterior Approach ◽  
Posterior Approach ◽  
Content Type ◽  
Fine Print

✓ A case is presented to illustrate the technique of combined posteroanterior approach to tumors of the cervical spinal foramen. The anterior approach offers a direct, feasible complementary route when this type of tumor cannot be completely excised through the conventional posterior approach.

Endolymphatic sac tumors in von Hippel—Lindau disease

2004 ◽  
Vol 100 (3) ◽  
pp. 480-487 ◽  
Author(s):  
Daniel Choo ◽  
Lawrence Shotland ◽  
Maryann Mastroianni ◽  
Gladys Glenn ◽  
Carter van Waes ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Family Members ◽  
Magnetic Resonance Images ◽  
Endolymphatic Sac ◽  
Specific Gene ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is a hereditary multiple-neoplasia syndrome mapping to chromosome 3p25–26. Endolymphatic sac (ELS) tumors have been identified as a neoplastic manifestation of VHL disease. The purpose of this study was to evaluate comprehensively the natural history of inner ear disease in a large population of patients with confirmed or suspected VHL disease and to correlate the clinical features with the VHL genotype. Methods. The authors collated and analyzed clinical and genotypic data obtained in patients enrolled in an Institutional Review Board—approved protocol in which families and individuals affected by VHL disease were studied. These data included results from multidisciplinary history workups and physical examinations, imaging studies, and a battery of audiological tests. One hundred seventy-five patients were enrolled in the study, 129 with confirmed VHL disease and 46 of their family members in whom test results for VHL disease were negative and who served as controls. Twenty-one patients had ELS tumors that were evident on magnetic resonance images; three of them had bilateral ELS lesions. Hearing loss, often sudden in onset and severe to profound in nature, vestibulopathy, aural fullness, and tinnitus represented the primary symptoms of ELS tumor. Distinct patterns of auditory and vestibular dysfunction occurred at different stages of the disease. Phenotypic data showed that 17 of 21 patients with ELS tumors did not have pheochromocytomas, whereas all had VHL disease affecting the kidney, all but two had VHL disease affecting the central nervous system, and all but one had disease affecting the pancreas. Genotyping revealed 10 rearrangements (partial deletions), eight single bp substitutions, and one 3-bp insertion. Although there was no difference in the incidence of hearing loss between populations, symptoms of imbalance and aural fullness were more common in patients with VHL disease but without imaging evidence of ELS tumor than they were in family members who did not have VHL disease (p < 0.01). Conclusions. Endolymphatic sac tumors are frequently associated with VHL disease. Symptoms of disequilibrium or aural fullness in patients with VHL disease may be an early indication of endolymphatic dysfunction. Patients with VHL disease provide a unique opportunity to examine the effects of specific gene mutations and a discrete neoplastic process on the human inner ear. The study of ELS tumors in this group also provides a pathological model of ELS function and supplies evidence for a role of the ELS in clinical Ménière-like disease(s).

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.

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