Spinal cord hemangioblastoma treatment

Spinal cord hemangioblastoma treatment

Although radiosurgery has been used to treat multiple hemangioblastoma, particularly in the cerebellum, complete microsurgical removal is the treatment of choice for spinal cord hemangioblastoma 1).

Partial resection or biopsy may cause postoperative bleeding and should therefore not be performed. Bleeding during dissection, due to the vascularity of HBs, increases the risk of adverse events.

minimally invasive approach for the resection of selected spinal hemangioblastomas is safe and allows complete tumor resection with good clinical results in experienced hands 2).


They are almost always associated with a syrinx or significant edema.

Cases associated with edema and syrinx are more space-occupying than those only with solid part of the tumor. Consequently, the mass effect producing neurological symptoms derives from the cyst rather than the tumor itself. On the removal of hemangioblastomas in association with a syrinx, the syrinx is spontaneously opened and always stops growing and usually regresses in size. Thus, the additional opening of the syrinx or surgical removal of the syrinx is not necessary 3).

Preceding Embolization

Although some investigators recommend preoperative embolization, 4) 5) in the series of Harati et al. it was usually not necessary to achieve complete resection 6). This is in concordance to several other series so that preoperative embolization is generally not recommended 7) 8) 9) 10) 11). To prevent intraoperative bleeding in selected cases, temporary artery occlusion was performed. This technique is described in detail by Clark et al.12).

Fluorescent dye

As vascular tumors, intramedullary hemangioblastomas are associated with significant intraoperative blood loss, making them particularly challenging clinical entities. The use of intraoperative indocyanine green or other fluorescent dyes has previously been described to avoid breaching the tumor capsule, but improved surgical outcomes may result from identifying and ligating the feeder arteries and arterialized draining veins.

Molina et al. presented a written and media illustration of a technique for intraoperative indocyanine green use in the en bloc resection of intramedullary hemangioblastoma 13).

Radiosurgery

Cyberknife radiosurgery has proven to be safe in the treatment of spinal HBs 14). However, as radiographic regression was achieved in only 22%, microsurgical resection remains the gold standard for spinal HBs that are clearly symptomatic or have developed radiographic progression in size, spinal cord edema, or syrinx 15) 16) 17).

References

1)

Samii M, Klekamp J. Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery. 1994 Nov;35(5):865-73; discussion 873. PubMed PMID: 7838335.
2)

Krüger MT, Steiert C, Gläsker S, Klingler JH. Minimally invasive resection of spinal hemangioblastoma: feasibility and clinical results in a series of 18 patients. J Neurosurg Spine. 2019 Aug 9:1-10. doi: 10.3171/2019.5.SPINE1975. [Epub ahead of print] PubMed PMID: 31398701.
3)

Na JH, Kim HS, Eoh W, Kim JH, Kim JS, Kim ES. Spinal cord hemangioblastoma : diagnosis and clinical outcome after surgical treatment. J Korean Neurosurg Soc. 2007 Dec;42(6):436-40. doi: 10.3340/jkns.2007.42.6.436. Epub 2007 Dec 20. PubMed PMID: 19096585; PubMed Central PMCID: PMC2588179.
4)

Montano N, Doglietto F, Pedicelli A, Albanese A, Lauretti L, Pallini R. Embolization of hemangioblastomas. J Neurosurg. 2008. 108: 1063-4
5)

Yang Y, Wang D, Jiang H, Sha C, Yuan Q, Liu J. [Treatment of spinal cord hemangioblastoma by microoperations combined with embolization]. Zhonghua Yi Xue Za Zhi. 2008. 88: 1309-12
6)

Harati A, Satopää J, Mahler L, Billon-Grand R, Elsharkawy A, Niemelä M, Hernesniemi J. Early microsurgical treatment for spinal hemangioblastomas improves outcome in patients with von Hippel-Lindau disease. Surg Neurol Int. 2012;3:6. doi: 10.4103/2152-7806.92170. Epub 2012 Jan 21. PubMed PMID: 22347675; PubMed Central PMCID: PMC3279991.
7)

Cornelius JF, Saint-Maurice JP, Bresson D, George B, Houdart E. Hemorrhage after particle embolization of hemangioblastomas: Comparison of outcomes in spinal and cerebellar lesions. J Neurosurg. 2007. 106: 994-8
8)

Mandigo CE, Ogden AT, Angevine PD, McCormick PC. Operative management of spinal hemangioblastoma. Neurosurgery. 2009. 65: 1166-77
9)

Mehta GU, Asthagiri AR, Bakhtian KD, Auh S, Oldfield EH, Lonser RR. Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine. 2010. 12: 233-42
10)

Oppenlander ME, Spetzler RF. Advances in spinal hemangioblastoma surgery. World Neurosurg. 2010. 74: 116-7
11)

Pietilä TA, Stendel R, Schilling A, Krznaric I, Brock M. Surgical treatment of spinal hemangioblastomas. Acta Neurochir (Wien). 2000. 142: 879-86
12)

Clark AJ, Lu DC, Richardson RM, Tihan T, Parsa AT, Chou D. Surgical technique of temporary arterial occlusion in the operative management of spinal hemangioblastomas. World Neurosurg. 2010. 74: 200-5
13)

Molina CA, Pennington Z, Ahmed AK, Westbroek E, Goodwin ML, Tamargo R, Sciubba DM. Use of Intraoperative Indocyanine Green Angiography for Feeder Vessel Ligation and En Bloc Resection of Intramedullary Hemangioblastoma. Oper Neurosurg (Hagerstown). 2019 Apr 1. pii: opz053. doi: 10.1093/ons/opz053. [Epub ahead of print] PubMed PMID: 31220325.
14)

Moss JM, Choi CY, Adler JR, Soltys SG, Gibbs IC, Chang SD. Stereotactic radiosurgical treatment of cranial and spinal hemangioblastomas. Neurosurgery. 2009. 65: 79-85
15)

Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: Implications for treatment. J Neurosurg. 2006. 105: 248-55
16)

Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery. 2001. 48: 55-62
17)

Samii M, Klekamp J. Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery. 1994. 35: 865-73

Moyamoya Disease Diagnosis

Moyamoya Disease Diagnosis

Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement).

Other characteristic findings include:

1. stenosis/occlusion starting at the termination of ICA and at origins of ACA and MCA

2. abnormal vascular network in the region of BG (intraparenchymal anastomosis).

3. transdural anastomosis(rete mirabile), AKA “vault moyamoya.”Contributing arteries: anterior falcial, middle meningeal, ethmoidal, occipital, tentorial, STA

4. moyamoya collaterals may also form from the internal maxillary artery via ethmoid sinus to the forebrain in the frontobasal region.

CT

Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. Low-density areas (LDAs) may be seen, usually confined to cortical and subcortical areas (unlike atherosclerotic disease or acute infantile hemiplegia which tend to have LDAs in basal ganglia as well). LDAs tend to be multiple and bilateral, especially in the PCA distribution (poor collaterals), and are more common in children.

MRI

MRA usually discloses the stenosis or occlusion of the ICA. Moyamoya vessels appear as flow voids on MRI (especially in basal ganglia) and a fine network of vessels on MRA and are demonstrated better in children than adults. Parenchymal ischemic changes are commonly shown, usually in watershed areas.

MRI images show:

1) diminished blood flow in the internal carotid artery (ICA) and the middle cerebral artery (MCA) and anterior cerebral artery (ACA) and 2) prominent collateral blood flow at the base of the brain. To confirm the diagnosis of moyamoya disease, an angiogram is typically required.

Diagnostic criteria of definitive moyamoya disease include all of the following items based on the conventional angiographic findings.

(1) Stenosis or occlusion of the terminal portion of the intracranial ICA or proximal portions of the anterior cerebral artery (ACA) and/or the middle cerebral artery (MCA).

(2) Development of abnormal vascular networks near the occlusive or stenotic lesions in the arterial phase.

(3) Bilateral lesion 3).

Results demonstrate distinct alterations in the temporal correlations of low-frequency BOLD signals, predominantly in resting-state networks in moyamoya disease. Additionally, Resting state functional magnetic resonance imaging (rs-fMRI) measures were associated with ischemic motor-related symptoms and cognitive performance in the patients. Thus, rs-fMRI may offer a useful non-invasive method of acquiring additional information beyond cerebral perfusion as part of clinical investigations in patients with moyamoya disease 4).


Territorial arterial spin labeling (t-ASL) could reveal comprehensive Moyamoya disease (MMD) cerebral blood perfusion and the vivid perfusion territory shifts fed by the unilateral ICA and ECA and bilateral vertebral arterys (VAs) in a noninvasive, straightforward, nonradioactive, and nonenhanced manner. 3D Time of flight magnetic resonance angiography (3D-TOF-MRA) could subdivide t-ASL perfusion territory shifts according to their shunt arteries. A perfusion territory shift attributable to the secondary collaterals is a potential independent risk factor for preoperative hemorrhage in MMD patients. A perfusion territory shift fed by the primary collaterals may not have a strong effect on preoperative hemorrhage in MMD patients. These findings make the combined modalities of t-ASL and 3D-TOF-MRA a feasible tool for MMD disease assessment, management, and surgical strategy planning 5).

Angiography

In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. The angiography-related complication rate is higher than with atherosclerotic occlusive disease. Avoid dehydration prior to and hypotension during the procedure. Six angiographic stages of MMD are described by Suzuki and Takaku 6) that tend to progress up until adolescence and stabilize by age 20.

1 stenosis of suprasellar ICA, usually bilateral

2 development of moyamoya vessels at the base of the brain; ACA MCA & PCA dilated

3 increasing ICA stenosis & prominence of moya-moya vessels (most cases diagnosed at this stage); maximal basal moyamoya

4 entire circle of Willis and PCAs occluded, extracranial collaterals start to appear, moyamoya vessels begin to diminish

5 further progression of stage 4

6 complete absence of moyamoya vessels and major cerebral arteries.

EEG

Non-specific in the adult. Juvenile cases: high-voltage slow waves may be seen at rest, predominantly in the occipital and frontal lobes. Hyperventilation produces a normal buildup of monophasic slow waves (delta-bursts) that return to normal 20–60 seconds after hyperventilation. In >50%of cases, after or sometimes continuous with buildup is a second phase of slow waves (this characteristic finding is called “rebuild up”) which are more irregular and slower than the earlier waves, and usually, normalize in ≤10 minutes 7).

Cerebral blood flow (CBF) studies

CBF is decreased in children with MMD, but relatively normal in adults. There is a shift of CBF from the frontal to the occipital lobes 8) probably reflecting the increasing dependency of CBF on the posterior circulation. Children with MMD have impaired autoregulation of CBF to blood pressure and CO2 (with more impairment of vasodilatation in response to hypercapnia or hypotension than vasoconstriction in response to hypocapnia or hypertension) 9). Xenon (Xe-133) CT can identify areas of low perfusion. Repeating the study after an acetazolamide challenge (which causes vasodilatation) evaluates the reserve capacity of CBF and can identify areas of “steal” which are at high risk of future infarction.

References

1)

Smith ER, Scott RM. Surgical management of moyamoya syndrome. Skull Base. 2005; 15:15–26
2)

Nishimoto A. Moyamoya Disease. Neurol Med Chir. 1979; 19:221–228
3)

Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis; Health Labour Sciences Research Grant for Research on Measures for Infractable Diseases. Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis). Neurol Med Chir (Tokyo). 2012;52(5):245-66. PubMed PMID: 22870528.
4)

Kazumata K, Tha KK, Uchino H, Ito M, Nakayama N, Abumiya T. Mapping altered brain connectivity and its clinical associations in adult moyamoya disease: A resting-state functional MRI study. PLoS One. 2017 Aug 4;12(8):e0182759. doi: 10.1371/journal.pone.0182759. eCollection 2017. PubMed PMID: 28783763.
5)

Gao XY, Li Q, Li JR, Zhou Q, Qu JX, Yao ZW. A perfusion territory shift attributable solely to the secondary collaterals in moyamoya patients: a potential risk factor for preoperative hemorrhagic stroke revealed by t-ASL and 3D-TOF-MRA. J Neurosurg. 2019 Aug 9:1-9. doi: 10.3171/2019.5.JNS19803. [Epub ahead of print] PubMed PMID: 31398708.
6)

Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969 Mar;20(3):288-99. PubMed PMID: 5775283.
7)

Kodama N, Aoki Y, Hiraga H, et al. Electroencephalographic Findings in Children with Moyamoya Disease. Arch Neurol. 1979; 36:16–19
8)

Ogawa A, Yoshimoto T, Suzuki J, Sakurai J. Cerebral Blood Flow in Moyamoya Disease. Part 1. Correlation with Age and Regional Distribution. Acta Neurochir. 1990; 105:30–34
9)

Ogawa A, Nakamura N, Yoshimoto T, Suzuki J. Cerebral Blood Flow in Moyamoya Disease. Part 2. Autoregulation and CO2 Response. Acta Neurochir. 1990; 105:107–111

Medical student

Medical student

For students beginning their medical education, the neuroscience curriculum is frequently seen as the most difficult, and many express an aversion to the topic. A major reason for this aversion amongst learners is the perceived complexity of neuroanatomy 1).

Osler created the first residency program for specialty training of physicians, and he was the first to bring medical students out of the lecture hall for bedside clinical training. Historically, medical student education in neurological surgery has generally limited student involvement to assisting in research projects with minimal formal clinical exposure before starting sub-internships and application for the neurosurgery match. Consequently, students have generally had little opportunity to acquire exposure to clinical neurosurgery and attain minimal proficiency 2).

Neurosurgery seeks to attract the best and brightest medical students; however, there is often a lack of early exposure to the field, among other possible barriers.

United States

Lubelski et al. sought to identify successful practices that can be implemented to improve medical student recruitment to neurosurgery.

United States neurosurgery residency program directors were surveyed to determine the number of medical student rotators and medical students matching into a neurosurgery residency from their programs between 2010 and 2016. Program directors were asked about the ways their respective institutions integrated medical students into departmental clinical and research activities.

Complete responses were received from 30/110 institutions. Fifty-two percent of the institutions had neurosurgery didactic lectures for 1st- and 2nd-year medical students (MS1/2), and 87% had didactics for MS3/4. Seventy-seven percent of departments had a neurosurgery interest group, which was the most common method used to integrate medical students into the department. Other forms of outreach included formal mentorshipprograms (53%), lecture series (57%), and neurosurgery anatomy labs (40%). Seventy-three percent of programs provided research opportunities to medical students, and 57% indicated that the schools had a formal research requirement. On average, 3 medical students did a rotation in each neurosurgery department and 1 matched into neurosurgery each year. However, there was substantial variability among programs. Over the 2010-2016 period, the responding institutions matched as many as 4% of the graduating class into neurosurgery per year, whereas others matched 0%-1%. Departments that matched a greater (≥ 1% per year) number of medical students into neurosurgery were significantly more likely to have a neurosurgery interest group and formal research requirements. A greater percentage of high-matching programs had neurosurgery mentorship programs, lecture series, and cadaver training opportunities compared to the other institutions.

In recent decades, the number of applicants to neurosurgery has decreased. A major deterrent may be the delayed exposure of medical students to neurosurgery. Institutions with early preclinical exposure, active neurosurgery interest groups, research opportunities, and strong mentorship recruit and match more students into neurosurgery. Implementing such initiatives on a national level may increase the number of highly qualified medical students pursuing neurosurgery 3).


A medical student training camp was created to improve the preparation of medical students for the involvement in neurological surgery activities and sub-internships.

A 1-day course was held at Weill Cornell Medicine, which consisted of a series of morning lectures, an interactive resident lunch panel, and afternoon hands-on laboratory sessions. Students completed self-assessment questionnaires regarding their confidence in several areas of clinical neurosurgery before the start of the course and again at its end.

A significant increase in self-assessed confidence was observed in all skill areas surveyed. Overall, rising fourth year students who were starting sub-internships in the subsequent weeks reported a substantial increase in their preparedness for the elective rotations in neurosurgery.

The preparation of medical students for clinical neurosurgery can be improved. Single-day courses such as the described training camp are an effective method for improving knowledge and skill gaps in medical students entering neurosurgical careers. Initiatives should be developed, in addition to this annual program, to increase the clinical and research skills throughout medical student education 4).

Canada

Medical students in Canada must make career choices by their final year of medical school. Selection of students for a career in neurosurgery has traditionally been based on marks, reference letters and personal interviews. Studies have shown that marks alone are not accurate predictors of success in medical practice; personal skills and attributes which can best be assessed by reference letters and interviews may be more important. A study was an attempt to assess the importance of, and ability to teach, personal skills and attitudes necessary for successful completion of a neurosurgical training program.

questionnaire was sent to 185 active members of the Canadian Neurosurgical Society, asking them to give a numerical rating of the importance of 22 personal skills and attributes, and their ability to teach those skills and attributes. They were asked to list any additional skills or attributes considered important, and rate their ability to teach them.

Sixty-six (36%) questionnaires were returned. Honesty, motivation, willingness to learn, ability to problem solve, and ability to handle stress were the five most important characteristics identified. Neurosurgeons thought they could teach problem solving, willingness to consult informed sources, critical thinking, manual dexterity, and communication skills, but honesty, motivation, willingness to learn and ability to handle stress were difficult or impossible to teach.

Honestymotivationwillingness to learnproblem solving and Stress management are important for success in a neurosurgical career. This information should be transmitted to medical students at “Career Day” venues. Structuring letters of reference and interviews to assess personal skills and attributes will be important, as those that can’t be taught should be present before the start of training 5).

References

1)

Larkin MB, Graves E, Rees R, Mears D. A Multimedia Dissection Module for Scalp, Meninges, and Dural Partitions. MedEdPORTAL. 2018 Mar 22;14:10695. doi: 10.15766/mep_2374-8265.10695. PubMed PMID: 30800895; PubMed Central PMCID: PMC6342347.
2) , 4)

Radwanski RE, Winston G, Younus I, ElJalby M, Yuan M, Oh Y, Gucer SB, Hoffman CE, Stieg PE, Greenfield JP, Pannullo SC. Neurosurgery Training Camp for Sub-Internship Preparation: Lessons From the Inaugural Course. World Neurosurg. 2019 Apr 1. pii: S1878-8750(19)30926-X. doi: 10.1016/j.wneu.2019.03.246. [Epub ahead of print] PubMed PMID: 30947014.
3)

Lubelski D, Xiao R, Mukherjee D, Ashley WW, Witham T, Brem H, Huang J, Wolfe SQ. Improving medical student recruitment to neurosurgery. J Neurosurg. 2019 Aug 9:1-7. doi: 10.3171/2019.5.JNS1987. [Epub ahead of print] PubMed PMID: 31398709.
5)

Myles ST, McAleer S. Selection of neurosurgical trainees. Can J Neurol Sci. 2003 Feb;30(1):26-30. PubMed PMID: 12619780.
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