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Acta Neurochir (2011) 153:109–114 DOI 10.1007/s00701-010-0811-6

CLINICAL ARTICLE

Concurrent Chiari decompression and spinal cord untethering in children: feasibility in a small case series
Vicko Gluncic & Michael Turner & Delilah Burrowes & David Frim

Received: 4 June 2010 / Accepted: 16 September 2010 / Published online: 1 October 2010 # Springer-Verlag 2010

Abstract Background andpurpose We describe the rationale and safety of concurrent decompression of Chiari type 1 malformation (CM1) and untethering of the spinal cord. Spinal cord traction is considered one of the pathogenic mechanisms involved in the development of CM, and 14% of patients with CM1 have tethered cord syndrome (Milhorat et al., Surg Neurol 7:20–35, 2009; Roth, Neuroradiology 21:133–138, 1981;Royo-Salvador, Rev Neurol 24:937–959, 1996; RoyoSalvador et al., Acta Neurochir 147:515–523, 2005). Therefore, intraspinal anomalies that require intervention are commonly treated before surgical decompression of Chiari malformation (Cheng et al., Neurologist 8:357–362, 2002; Menezes, Pediatr Neurosurg 23:260–269, 1995; Milhorat et al., Surg Neurol 7:20–35, 2009; Royo-Salvador et al., Acta Neurochir147:515–523, 2005; Schijman and Steinbok, Childs Nerv Syst 20:341–348, 2004; Yamada et al., Neurol Res 26:719–721, 2004). However, in the interval between the spinal cord untethering and the decompression surgery, patients may continue to suffer from the untreated symptoms of CM. In a series of four patients with concurrent severe and progressive symptoms referable to both conditions, we performed bothsurgeries simultaneously. Methods Charts of four patients who underwent concurrent Chiari decompression and spinal cord untethering were reviewed.
V. Gluncic (*) : M. Turner : D. Frim Section of Neurosurgery, University of Chicago, 5841 S. Maryland Avenue, MC 3026, Chicago, IL 60637, USA e-mail: vicko.gluncic@gmail.com D. Burrowes Section of Neuroradiology, University of Chicago, 5841 S. MarylandAvenue, MC 2026, Chicago, IL 60637, USA

Results All patients tolerated the procedures well without complication. They reported significant or complete early postsurgical resolution of headaches and ambulating difficulties. On average, patients started to walk on postoperative day 3 (3±1 days) and were discharged on hospital day 6 (6± 1 days). No patient experienced a persistent subcutaneous ortranscutaneous cerebrospinal fluid leak. Subsequent postoperative courses were uneventful. Conclusion Concurrent Chiari decompression and untethering of the spinal cord is a feasible option and in some patients may be preferred in lieu of staged procedures. Keywords Tethered spinal cord . Chiari type 1 malformation . Pediatric

Introduction The prevalence of Chiari malformation type 1 (CM1) isthought to be 0.6–0.9% [17, 20, 31, 36]. Although the exact incidence of tethered cord (TC) is unknown since it often goes undetected, the general prevalence of primary TC syndrome (TCS) is approximately 0.1% [2]. Both can be associated with scoliosis [6, 12, 25, 26]. The introduction of magnetic resonance imaging (MRI) has played a major role in the diagnosis of coexisting anomalies and revealed thatthe association of distinct craniospinal anomalies is more frequent than previously thought [6, 10, 12, 26]. The incidence of coexisting craniospinal anomalies was reported as 15% by Kumar et al. [14]. Orakdogen reported coexisting CM in 30% of the cases with cervical or thoracic spinal dysraphism [23]. CM has been also linked to lipomyelomeningoceles. Thirteen percent of lipomyelomeningocelepatients had CM, while 2.3% of CM patients had lipomyelomeningoceles. In addition, association of CM2

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Acta Neurochir (2011) 153:109–114

and myelomeningocele has been established, while recent study by Milhorat et al. reported TCS in up to 14% of patients with CM1 [2, 3, 8, 13, 14, 20, 33, 36]. Primary CM1 symptoms include head pain, dysphagia, apnea, and drop attacks [19, 20, 39]....