Lumbar laminotomy for the intrathecal administration of nusinersen for spinal muscular atrophy: technical note and outcomes

被引:17
作者
Ko, Diana [1 ]
Blatt, Daniel [1 ]
Karam, Chafic [2 ]
Gupta, Kunal [3 ]
Raslan, Ahmed M. [3 ]
机构
[1] Oregon Hlth & Sci Univ, Sch Med, Portland, OR 97201 USA
[2] Oregon Hlth & Sci Univ, Dept Neurol, Portland, OR 97201 USA
[3] Oregon Hlth & Sci Univ, Dept Neurol Surg, Portland, OR 97201 USA
关键词
spinal muscular atrophy; nusinersen; lumbar puncture; lumbar laminotomy; surgical technique; SHAM CONTROL; MANAGEMENT; SCOLIOSIS;
D O I
10.3171/2019.2.SPINE181366
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Nusinersen (Spinraza) is a US Food and Drug Administration-approved intrathecal medication for the treatment of spinal muscular atrophy (SMA). Adult patients with SMA often undergo thoracolumbar fusion to treat neurogenic scoliosis, preventing thecal access. The authors report a laminotomy technique and the ease of intrathecal access in three SMA patients with prior thoracolumbar fusions. Patients were positioned in the lateral decubitus position or prone. Lumbar laminotomy was performed below the conus, between the lateral longitudinal rods, to preserve mechanical stability. Fluoroscopy provided real-time identification of instruments. Hardware was contoured with a carbide drill bit to develop the surgical window. Fiducial screws were placed along the perimeter for demarcation. Sublaminar wire removal caused dural defects that were repaired with a layer of dural substitute onlay and sealant. All patients successfully received nusinersen thecal injections via lumbar puncture by an interventional radiologist. Fluoroscopy time ranged from 6 to 36 seconds. No postoperative pseudomeningoceles, cerebrospinal fluid leaks, or wound complications occurred. For patients with SMA and posterior fusion from prior scoliosis treatment, lumbar laminotomy is an effective method for creating thecal access for the administration of nusinersen.
引用
收藏
页码:217 / 221
页数:5
相关论文
共 24 条
[1]  
[Anonymous], 1993, GeneReviews
[2]   SPINAL MUSCULAR ATROPHY: DIAGNOSIS AND MANAGEMENT IN A NEW THERAPEUTIC ERA [J].
Arnold, W. David ;
Kassar, Darine ;
Kissel, John T. .
MUSCLE & NERVE, 2015, 51 (02) :157-167
[3]   Copy Number Variations in the Survival Motor Neuron Genes: Implications for Spinal Muscular Atrophy and Other Neurodegenerative Diseases [J].
Butchbach, Matthew E. R. .
FRONTIERS IN MOLECULAR BIOSCIENCES, 2016, 3
[4]   Spinal Muscular Atrophies [J].
Darras, Basil T. .
PEDIATRIC CLINICS OF NORTH AMERICA, 2015, 62 (03) :743-+
[5]   Nusinersen versus Sham Control in Infantile-Onset Spinal Muscular Atrophy [J].
Finkel, R. S. ;
Mercuri, E. ;
Darras, B. T. ;
Connolly, A. M. ;
Kuntz, N. L. ;
Kirschner, J. ;
Chiriboga, C. A. ;
Saito, K. ;
Servais, L. ;
Tizzano, E. ;
Topaloglu, H. ;
Tulinius, M. ;
Montes, J. ;
Glanzman, A. M. ;
Bishop, K. ;
Zhong, Z. J. ;
Gheuens, S. ;
Bennett, C. F. ;
Schneider, E. ;
Farwell, W. ;
De Vivo, D. C. .
NEW ENGLAND JOURNAL OF MEDICINE, 2017, 377 (18) :1723-1732
[6]   Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study [J].
Finkel, Richard S. ;
Chiriboga, Claudia A. ;
Vajsar, Jiri ;
Day, John W. ;
Montes, Jacqueline ;
De Vivo, Darryl C. ;
Yamashita, Mason ;
Rigo, Frank ;
Hung, Gene ;
Schneider, Eugene ;
Norris, Daniel A. ;
Xia, Shuting ;
Bennett, C. Frank ;
Bishop, Kathie M. .
LANCET, 2016, 388 (10063) :3017-3026
[7]   Outcomes of Primary Posterior Spinal Fusion for Scoliosis in Spinal Muscular Atrophy: Clinical, Radiographic, and Pulmonary Outcomes and Complications [J].
Holt, Joshua B. ;
Dolan, Lori A. ;
Weinstein, Stuart L. .
JOURNAL OF PEDIATRIC ORTHOPAEDICS, 2017, 37 (08) :E505-E511
[8]   Anesthesia and spinal muscle atrophy [J].
Islander, Gunilla .
PEDIATRIC ANESTHESIA, 2013, 23 (09) :804-816
[9]   Spinal Muscular Atrophy [J].
Kolb, Stephen J. ;
Kissel, John T. .
NEUROLOGIC CLINICS, 2015, 33 (04) :831-+
[10]   Nusinersen versus Sham Control in Later-Onset Spinal Muscular Atrophy [J].
Mercuri, E. ;
Darras, B. T. ;
Chiriboga, C. A. ;
Day, J. W. ;
Campbell, C. ;
Connolly, A. M. ;
Iannaccone, S. T. ;
Kirschner, J. ;
Kuntz, N. L. ;
Saito, K. ;
Shieh, P. B. ;
Tulinius, M. ;
Mazzone, E. S. ;
Montes, J. ;
Bishop, K. M. ;
Yang, Q. ;
Foster, R. ;
Gheuens, S. ;
Bennett, C. F. ;
Farwell, W. ;
Schneider, E. ;
De Vivo, D. C. ;
Finkel, R. S. ;
Bradley, Walter G. ;
Kaufmann, Petra ;
Dickson, Patricia I. ;
Reingold, Stephen C. ;
Davis, Charles S. ;
Arredondo, Kristen ;
Castro, Diana ;
Cowie, Margaret ;
Farrow-Gillespie, Alan ;
Hebert, Andrew ;
Kauk, Melissa ;
Miller, Nancy ;
Nelson, Leslie ;
Spain, Thomas, Jr. ;
Cappell, Joshua ;
Constantinescu, Andrei ;
Cruz, Rosangel ;
Dastgir, Jahannaz ;
De Vivo, Darryl ;
Dunaway, Sally ;
Engelstad, Kristin ;
Khandji, Alexander G. ;
Kramer, Samantha ;
Marra, Jonathan ;
Popolizio, Molly ;
Salazar, Rachel ;
Weimer, Louis H. .
NEW ENGLAND JOURNAL OF MEDICINE, 2018, 378 (07) :625-635