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

Recurrent hemifacial spasm after microvascular decompression

Recurrent hemifacial spasm after microvascular decompression

Microvascular decompression (MVD) is a highly effective treatment for hemifacial spasm (HFS), but even if the root exit zone (REZ) from the brainstem is adequately decompressed, residual spasms after surgery or early reappearance of spasms are not uncommon 1) 2) 3) 4) 5)

Return of symptoms after a period of complete resolution of hemifacial spasm occurs in up to 10% of patients, 86% of recurrences happen within 2 yrs of surgery, and the risk of developing recurrence after 2 yrs of post-op relief is only ≈ 1% 6).


Among more than 2500 patients who underwent microvascular decompression for hemifacial spasm, 23 patients received a second MVD in the Kyung Hee University Hospital from January 2002 to December 2017. Three-dimensional time of flight magnetic resonance angiography and reconstructed imaging were used to identify the culprit vessel and its conflict upon root exit zone (REZ) of the facial nerve. They reviewed patients’ medical records and operation videos to identify the missing points of the first surgery.

8 patients had incomplete decompression, such as single-vessel decompression of multiple offending vessels. Teflon was not detected at the REZ, but was found in other locations in 12 patients. Three patients had severe adhesion with previous Teflon around the REZ. Nineteen patients had excellent surgical outcomes at immediate postoperative evaluation; 20 patients showed spasm disappearance at 1 year after surgery and 3 patients showed persistent symptoms. Neuro-vascular contacts around REZ of facial nerve were revealed on MRI of incomplete decompression and Teflon malposition patient groups. There were no clear neuro-vascular contacts in the patients with severe Teflon adhesion.

The decision on secondary MVD for persistent or recurrent spasm is troubling. However, if the neurovascular contact was observed in the MRI of the patient and there were offending vessels, the surgical outcome might be favorable 7).

References

1)

Fukushima T: Microvascular decompression for hemifacial spasm: results in 2890 cases, in Carter LP, Spetzler RF, editors. (eds): Neurovascular Surgery. New York, McGraw-Hill, 1995, pp 1133–1145
2)

Huang CI, Chen IH, Lee LS: Microvascular decompression for hemifacial spasm: analyses of operative findings and results in 310 patients. Neurosurgery 30: 53– 56; discussion 56–57, 1992.
3)

Ishikawa M, Nakanishi T, Takamiya Y, Namiki J: Delayed resolution of residual hemifacial spasm after microvascular decompression operations. Neurosurgery 49: 847– 854; discussion 854–856, 2001.
4)

Li CS: Varied patterns of postoperative course of disappearance of hemifacial spasm after microvascular decompression. Acta Neurochir (Wien) 147: 617– 620; discussion 620, 2005.
5)

Shin JC, Chung UH, Kim YC, Park CI: Prospective study of microvascular decompression in hemifacial spasm. Neurosurgery 40: 730– 734; discussion 734–735, 1997.
6)

Payner TD, Tew JM. Recurrence of Hemifacial Spasm After Microvascular Decompression. Neurosurgery. 1996; 38:686–691
7)

Park CK, Lee SH, Park BJ. Surgical Outcomes of Revision Microvascular Decompression for Persistent or Recurrent Hemifacial Spasm after Surgery: Analysis of radiological and intraoperative findings. World Neurosurg. 2019 Aug 2. pii: S1878-8750(19)32107-2. doi: 10.1016/j.wneu.2019.07.191. [Epub ahead of print] PubMed PMID: 31382068.

Insular Cavernous Malformation

Insular Cavernous Malformation

Surgical management of cavernous malformation (CM) of the insula consists of total resection of the lesion and the surrounding gliosis to avoid or reduce seizures. When located in the dominant hemisphere, an awake craniotomy with intraoperative mapping reduces the risk of functional damage. The insula is covered by the operculum and has a relationship with the middle cerebral artery and its branches that run along its lateral cortical surface. Therefore high expertise is required to manage the exposure of the insula and its complex anatomy.

Videos

https://www.neurosurgicalatlas.com/cases/insular-cavernous-malformation


A video of Burkhardt et al. demonstrated the microsurgical resection of a de novo CM adjacent to a previously treated high-grade AVM and clipping of a middle cerebral artery (MCA) aneurysm. A 70-yr-old male with history of radiosurgery for AVM presented with aphasia and confusion. Preoperative angiography showed complete occlusion of the AVM. MRI showed multiple cystic lesions suspicious for radiation-induced necrosis and CM. IRB approval and patient consent was obtained. A pterional craniotomy was performed with transsylvian exposure of the insula. The radiated feeding arteries were followed to the occluded AVM nidus. A CM was noted deep to this candelabra of the MCA vessels, which were mobilized to access and resect the CM. A small incision was made in this insular cortex underneath the malformation circumferentially freeing it of adhesions. The sclerotic AVM nidus was circumferentially dissected and removed en bloc. Thorough exploration of the resection cavity revealed no residual CM or AVM nidus. Attention was then turned to the M2-MCA bifurcation aneurysm, which was occluded with a straight clip. Postoperative imaging confirmed complete CM resection. The patient recovered from his aphasia. This case demonstrates the management of a radiation-induced de novo CM following treatment of a high-grade AVM. Radiographic follow-up for radiosurgically treated AVM is needed to rule out long-term complications. Bleeding from a de novo CM mimics bleeding from residual AVM nidus, requiring careful angiographic evaluation 1).


A video of Norat et al. illustrated the use of a trans-Sylvian, trans-sulcal approach to resect a deep insular/basal ganglia cavernous malformation in a young patient. The use of the neuronavigation is essential for success in these types of operation as this tool limits the surgeon’s footprint in eloquent brain. Unlike superficial lesions where the removal of hemosiderin stained brain is possible and often safe, resection of deep-seated lesions requires the surgeon to distinguish between hemosiderin-stained brain and residual cavernous malformation. This task is not simple, and residual cavernous malformation is the most common reason for re-bleed in patients who have undergone surgery. Resection of symptomatic cavernous malformations in deep locations can be performed safely, but outcomes are heavily influenced by proper patient selection and surgeon experience. In patients with multiple cerebral cavernous malformations, such as the one in this case, genetic testing should be performed 2).


A video of Vigo et al. demonstrated the surgical management of a large left insular CM. A 29-year-old female with multiple CM and 7 years of partial seizures and recent onset of short memory loss. Neuroimaging showed a large left insular and planum polare CM with important mass effect and hemorrhage signs. The patient consented to surgery, and an awake pretemporal craniotomy was carried out with continuous motor evoked potential monitoring. No language function was localized in the superior temporal gyrus; therefore corticectomy of the middle portion was performed to expand the operative corridor. The vessel manipulation during wide opening of the sylvian fissure increased the risk of postoperative vasospasm and blood drain into the surgical field. The CM was exposed and completely removed without functional damage. The patient recovered from surgery without complications, and no seizures occurred at 2 months’ follow-up. Postoperative imaging showed complete removal of the CM 3).

Case reports

A 25-yr-old female presented with an acute-onset right homonymous hemianopsia. Neuroimaging revealed a large left insular CM, adjacent to the posterior limb of IC. After obtaining IRB approval and patient consent, a left pterional craniotomy with a wide distal Sylvian fissure split was completed. Using neuronavigation, an insular entry point was chosen for corticectomy. The CM was opened with subsequent hematoma evacuation and intracapsular resection technique. Inspection of the cavity revealed remnants anteromedially near the IC, which were removed meticulously, mobilizing the CM away from the IC. Postoperative MRI demonstrated gross total resection of the CM. The patient was discharged home on postoperative day 5 with persistent homonymous hemianopia.This case describes the use of a transsylvian-transinsular approach to access deep lesions with the shortest surgical distance and minimal cortical transgression. A wide Sylvian fissure split exposes the M2 MCA and accesses a safe insular zone, keeping the most eloquent structures deep to the lesion in the surgical corridor. This approach can safely expose vascular pathologies in the insular region without the risk of injury to overlying eloquent frontal and temporal lobes, even in the dominant hemisphere 4).

References

1)

Burkhardt JK, Gandhi S, Tabani H, Benet A, Lawton MT. Left Transsylvian-Transinsular Approach for Radiation-Induced Cavernous Malformation: 3-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Aug 1;17(2):E62-E63. doi: 10.1093/ons/opy357. PubMed PMID: 30418603.
2)

Norat P, Yagmurlu K, Park MS, Kalani MYS. Keyhole, Trans-Sylvian, Trans-Sulcal Resection of an Insular Cerebral Cavernous Malformation: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Jul 1;17(1):E18. doi: 10.1093/ons/opy326. PubMed PMID: 30496497.
3)

Vigo V, Zanabria Ortiz R, Paganelli SL, da Costa MDS, Campos Filho JM, Chaddad-Neto F. Awake Craniotomy for Removal of Left Insular Cavernous Malformation. World Neurosurg. 2019 Feb;122:209. doi: 10.1016/j.wneu.2018.10.220. Epub 2018 Nov 9. PubMed PMID: 30415050.
4)

Mascitelli J, Gandhi S, Wright E, Lawton MT. Transsylvian-Transinsular Approach for an Insular Cavernous Malformation Resection: 3-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Feb 1;16(2):50. doi: 10.1093/ons/opy155. PubMed PMID: 29905877.
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