Surgical Dissection of a Perimedullary Fistula of Conus Medullaris With Long-Term Follow-Up: 2-Dimensional Operative Video

2018 ◽  
Vol 17 (2) ◽  
pp. E59-E59 ◽  
Author(s):  
Thomas J Sorenson ◽  
Giuseppe Lanzino

Abstract Perimedullary fistulae of the spinal cord are rare vascular lesions that can present with different clinical patterns: hemorrhage, progressive myelopathy due to arterial steal and/or venous congestion, or symptoms due to compression of neural structures by engorged vessels. Treatment consists of surgical excision, endovascular embolization, or a combination of the two. If complete obliteration of the nidus exposes the patient to undue risk of permanent neurological deficits, incomplete obliteration with reduction of the vascular supply is a reasonable compromise to improve clinical symptomatology. Partial devascularization may also alter the natural history by decreasing the risk of further growth and bleeding. In this video we illustrate the case of a patient with a perimedullary fistula of the conus treated with surgical disconnection of the main fistulous component. Partial devascularization resulted in resolution of MRI signal changes and symptoms with documented good clinical and radiological outcome and progressive regression of the residual nidus over six years of follow-up.

2018 ◽  
Vol 16 (2) ◽  
pp. E44-E44
Author(s):  
M Neil Woodall ◽  
Robert F Spetzler

Abstract Arteriovenous malformations (AVMs) involving the conus medullaris have a unique angioarchitecture due to their involvement of the arterial basket of the conus medullaris, which represents an arterial anastomotic network between the anterior spinal artery (ASA) and posterior spinal arteries (PSAs) at the level of the conus medullaris.1 These lesions consist of a combination of a true AVM nidus, which is usually extramedullary, and direct shunts between the ASA, PSAs, and the venous system. Patients may present with radiculopathy, myelopathy, or subarachnoid hemorrhage.2 A 40-yr-old woman status post T11-L1 laminoplasty for resection of a ruptured conus AVM 6 yr prior presented with routine follow-up angiography suggestive of an arteriovenous fistula. She was counseled regarding treatment options including endovascular embolization and microsurgical ligation or resection, and she elected to proceed with surgical treatment. At the time of surgery, a recurrent AVM was noted. A 2-dimensional intraoperative video illustrates the microsurgical treatment of her recurrent conus AVM. The patient recovered well postoperatively. Spinal angiography demonstrated complete obliteration of the lesion. The patient experienced transient urinary retention that was self-limited but otherwise was without any new neurological deficit. Due to the retrospective nature of this report, informed consent was not required. Video used with permission from Barrow Neurological Institute, all rights reserved.


2014 ◽  
Vol 37 (3) ◽  
pp. E14 ◽  
Author(s):  
Leonardo Rangel-Castilla ◽  
Jonathan J. Russin ◽  
Hasan A. Zaidi ◽  
Eduardo Martinez-del-Campo ◽  
Min S. Park ◽  
...  

Object Spinal arteriovenous fistulas (AVFs) and arteriovenous malformations (AVMs) are rare, complex spinal vascular lesions that are challenging to manage. Recently, understanding of these lesions has increased thanks to neuroimaging technology. Published reports of surgical results and clinical outcome are limited to small series. The authors present a large contemporary series of patients with spinal AVFs and AVMs who were treated at Barrow Neurological Institute in Phoenix, Arizona. Methods Retrospective detailed review of a prospective vascular database was performed for all patients with spinal AVFs and AVMs treated between 2000 and 2013. Patient demographic data, AVF and AVM characteristics, surgical results, clinical outcomes, complications, and long-term follow-up were reviewed. Results Between 2000 and 2013, 110 patients (57 male and 53 female) underwent obliteration of spinal AVFs and AVMs. The mean age at presentation was 42.3 years (range 18 months–81 years). There were 44 patients with AVFs and 66 with AVMs. The AVM group included 27 intramedullary, 21 conus medullaris, 12 metameric, and 6 extradural. The most common location was thoracic spine (61%), followed by cervical (22.7%), lumbar (14.5%), and sacral (1.8%). The most common presenting signs and symptoms included paresis/paralysis (75.5%), paresthesias (60%), pain (51.8%), bowel/bladder dysfunction (41.8%), and myelopathy (36.4%). Evidence of rupture was seen in 26.4% of patients. Perioperative embolization was performed in 42% of patients. Resection was performed in 95 patients (86.4%). Embolization alone was the only treatment in 14 patients (12.7%). One patient was treated with radiosurgery alone. Angiographically verified AVF and AVM obliteration was achieved in 92 patients (83.6%). At a mean follow-up duration of 30.5 months (range 1–205 months), 43 patients (97.7%) with AVFs and 57 (86.4%) with AVMs remained functionally independent (McCormick Scale scores ≤ 2). Perioperative complications were seen in 8 patients (7%). No deaths occurred. Temporary neurological deficits were observed in 27 patients (24.5%). These temporary deficits recovered 6–8 weeks after treatment. Recurrence was identified in 6 patients (13.6%) with AVFs and 10 (15.2%) with AVMs. Conclusions Spinal AVFs and AVMs are complex lesions that should be considered for surgical obliteration. Over the last several decades the authors have changed surgical strategies and management to achieve better clinical outcomes. Transient neurological deficit postoperatively is a risk associated with intervention; however, clinical outcomes appear to exceed the natural history based on patients’ ability to recover during the follow-up period. Due to the recurrence rate associated with these lesions, long-term follow-up is required.


2018 ◽  
Vol 16 (3) ◽  
pp. E94-E94 ◽  
Author(s):  
Daniel M Heiferman ◽  
Hasan R Syed ◽  
Daphne Li ◽  
Brian D Rothstein ◽  
Ali Shaibani ◽  
...  

Abstract Cirsoid aneurysms, also known as scalp arteriovenous malformations (AVM), are rare congenital extracranial vascular anomalies that often present as an enlarging pulsatile scalp mass. A 14-yr-old male presented with a pulsatile scalp lesion that was first noticed 3 yr prior and had progressively enlarged. No history of trauma was reported. MRI demonstrated a 4 cm wide and 2 cm tall nidus and catheter angiography was performed to further define the vascular supply and drainage. The patient underwent transvenous endovascular embolization followed by surgical excision via a bicoronal incision, as shown in this operative video. Care was taken to identify, cauterize, and transect feeding vessels from the superficial temporal, supratrochlear, and supraorbital arteries circumferentially to completely devascularize and resect the galeal nidus from overlying scalp tissue and underlying pericranium. Previously unreported in the literature, transosseous emissary veins partially draining the lesion were noted on angiography and were waxed thoroughly during surgery. Six-month follow-up examination demonstrated a well-healed incision without evidence of AVM recurrence. The unique venous drainage of this cirsoid aneurysm highlights the value of diagnostic angiography to fully characterize these rare and complex vascular lesions prior to pursuing definitive treatment.  IRB approval was obtained from the Ann & Robert H. Lurie Children's Hospital of Chicago Institutional Review Board (IRB #2018-1799). The IRB waives the requirement of obtaining informed consent for this study in accordance with 45 CFR 46.116(d).


2014 ◽  
Vol 13 (5) ◽  
pp. 536-540 ◽  
Author(s):  
Jetan H. Badhiwala ◽  
Eric M. Thompson ◽  
Armando J. Lorenzo ◽  
Abhaya V. Kulkarni

Congenital spinal lipomas of the conus (SLCs) are among the most common closed neural tube defects. The treatment of SLC is an area of controversy because the true natural history of this condition is unknown. Here, the authors present two cases of SLC presenting in infancy with compromised lower urinary tract function, which was objectively confirmed by abnormal urodynamic studies. In both cases, there was spontaneous improvement in urodynamic parameters, with stable normal urinary function at the long-term follow-up. Although cases of spontaneous radiological regression of SLC have very infrequently been reported, they have not been associated with the reversal of already present neurological deficits. This report reinforces the need for further delineation of the true natural history of SLC and highlights the dynamic nature of associated neurological compromise over time.


2016 ◽  
Vol 25 (2) ◽  
pp. 234-238 ◽  
Author(s):  
Arya N. Bagherpour ◽  
Gustavo J. Rodriguez ◽  
Chetan Moorthy ◽  
Todd T. Trier ◽  
Alberto Maud

Parkes Weber syndrome (PWS) is a congenital overgrowth disorder characterized by unilateral limb and axial hypertrophy, capillary malformations of the skin, and high-flow arteriovenous fistulas (AVFs). Spinal AVFs in the setting of PWS are challenging vascular lesions that often contain multiple arteriovenous (AV) shunts. The present case report highlights an adolescent girl with PWS who presented with a ruptured complex high-flow conus medullaris AVF. She was successfully treated with endovascular embolization and microsurgery. At the 2-year follow-up, the patient remained free of neurological symptoms and had no recurrence of the vascular malformation.


Author(s):  
V. Hellstern ◽  
P. Bhogal ◽  
M. Aguilar Pérez ◽  
M. Alfter ◽  
A. Kemmling ◽  
...  

Abstract Background Adenosine induced cardiac standstill has been used intraoperatively for both aneurysm and arteriovenous malformation (AVM) surgery and embolization. We sought to report the results of adenosine induced cardiac standstill as an adjunct to endovascular embolization of brain AVMs. Material and Methods We retrospectively identified patients in our prospectively maintained database to identify all patients since January 2007 in whom adenosine was used to induce cardiac standstill during the embolization of a brain AVM. We recorded demographic data, clinical presentation, Spetzler Martin grade, rupture status, therapeutic intervention and number of embolization sessions, angiographic and clinical results, clinical and radiological outcomes and follow-up information. Results We identified 47 patients (22 female, 47%) with average age 42 ± 17 years (range 6–77 years) who had undergone AVM embolization procedures using adjunctive circulatory standstill with adenosine. In total there were 4 Spetzler Martin grade 1 (9%), 9 grade 2 (18%), 15 grade 3 (32%), 8 grade 4 (18%), and 11 grade 5 (23%) lesions. Of the AVMs six were ruptured or had previously ruptured. The average number of embolization procedures per patient was 5.7 ± 7.6 (range 1–37) with an average of 2.6 ± 2.2 (range 1–14) embolization procedures using adenosine. Overall morbidity was 17% (n = 8/47) and mortality 2.1% (n = 1/47), with permanent morbidity seen in 10.6% (n = 5/47) postembolization. Angiographic follow-up was available for 32 patients with no residual shunt seen in 26 (81%) and residual shunts seen in 6 patients (19%). The angiographic follow-up is still pending in 14 patients. At last follow-up 93.5% of patients were mRS ≤2 (n = 43/46). Conclusion Adenosine induced cardiac standstill represents a viable treatment strategy in high flow AVMs or AV shunts that carries a low risk of mortality and permanent neurological deficits.


2001 ◽  
Vol 7 (1_suppl) ◽  
pp. 149-154 ◽  
Author(s):  
Y. Kaku ◽  
K. Hayashi ◽  
M. Sawada ◽  
N. Sakai

We evaluated long-term angiographical follow-up of cerebral aneurysms treated with detachable platinum coils with special reference to the long-term morphological outcomes of incompletely obliterated aneurysms. Serial long-term follow-up cerebral angiograms (>1 year) were obtained in 47 cases out of 134 cases treated with GDCs or IDCs from 1994 to 1999. In 47 patients, intial angiographical results demonstrated ten complete aneurysmal occlusion, seven aneurysms with a small neck remnant, twenty aneurysms with body filling and ten aneurysms with both of neck remnant and body filling. In the ten completely obliterated aneurysms, follow-up angiograms revealed no aneurysmal recanalization. In seven aneurysms with neck remnant, three remained unchanged, four showed enlargement of contrast filling in the part of the neck of the aneurysm. In twenty aneurysms with body filling, five had developed into complete obliteration, three remained unchanged, 11 had recanalization, and one aneurysm displayed regrowth. In ten aneurysms with both of neck remnant and body filling, two remained unchanged, five had recanalization, and three aneurysms displayed regrowth. Long-term angiographical follow-up results are less satisfactory in cases involving incompletely obliterated lesions. A higher incidence of recanalizations were promoted in cases with neck remnant and/or body filling. Strict follow-up angiography is mandatory when a complete obliteration is not achieved.


FACE ◽  
2021 ◽  
pp. 273250162110536
Author(s):  
Joshua Harrison ◽  
Samantha Marley ◽  
Shawhin Shahriari ◽  
Christian Bowers ◽  
Anil Shetty

We report a rare case of an extramedullary plasmacytoma (EMP) in the frontal sinus with an indolent clinical presentation. Although a history of trauma was absent, the initial diagnosis was a mucocele, based on the radiological findings. Upon surgical excision, the patient was found to have an EMP. EMP, a form of solitary plasmacytoma, has a significantly high rate of conversion to multiple myeloma. This mandates long-term follow-up, even after successful radiotherapy and/or resection. While radiation therapy is generally considered a first line treatment for EMP, surgical intervention may provide optimal treatment in complicated cases. This case presentation highlights the prognosis of patients diagnosed with EMP.


2006 ◽  
Vol 104 (3) ◽  
pp. 376-381 ◽  
Author(s):  
Aaron A. Cohen-Gadol ◽  
Jeffrey T. Jacob ◽  
Diane A. Edwards ◽  
William E. Krauss

Object The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients’ mean age at presentation was 50 years (range 13–82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.


2008 ◽  
Vol 1 (6) ◽  
pp. 488-492 ◽  
Author(s):  
Thomas Daenekindt ◽  
Frank Weyns ◽  
Kuan Hua Kho ◽  
Dieter Peuskens ◽  
Koen Engelborghs ◽  
...  

✓ The authors describe the case of a patient with an intracranial capillary hemangioma, and they review the recent literature on intracranial capillary hemangiomas with special attention to their differential diagnosis and management. The only sign in this 7-week-old boy was head enlargement. There were no neurological deficits, and imaging revealed a large intracranial lesion in the right temporal fossa. The results of biopsy confirmed the diagnosis, and, after endovascular embolization, the entire lesion was resected. The incidence of intracranial capillary hemangioma is very low but may be underestimated. In the present case, the size of the tumor prompted surgical treatment. The natural behavior of extracranial capillary hemangiomas, however, suggests that a conservative approach with follow-up and steroid therapy may also be considered.


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