Background: Klippel-Trenaunay-Weber syndrome is a rare congenital condition that may involve the central nervous system with extensive vascular malformations, and soft-tissue hypertrophy of the affected limb. Methods: A 51-year-old patient underwent previous procedures including decompressive laminectomy and endovascular embolization using Onyx and a self-expandable stent. However, recanalization of the malformation was observed, his symptoms progressed, and the decision was made to undergo selective surgical resection with intraoperative image guidance using Stealth system paired with the O-arm. Results: Adequate decompression of the affected spinal cord and cervical roots were obtained, and the patient’s symptoms improved. Conclusion: Since the typical anatomic landmarks were obliterated by the extensive vascular malformation and previous surgery, intraoperative image guidance proved to be very beneficial. This case reports on the usage of spinal navigation to efficiently dissect out the affected nerve roots and avoid vascular injury by referencing the Onyx and stent.
Nolte, L.P., Visarius, H., Arm, E., Langlotz, F., Schwarzenbach, O. and Zamorano, L. (1995) Computer-Aided Fixation of Spinal Implants. Journal of Image Guided Surgery, 1, 88-93. http://dx.doi.org/10.1002/(SICI)1522-712X(1995)1:2<88::AID-IGS3>3.0.CO;2-H
Nolte, L.P., Zamorano, L., Visarius, H., Berlemann, U., Langlotz, F., Arm, E., et al. (1995) Clinical Evaluation of a System for Precision Enhancement in Spine Surgery. Clinical Biomechanics (Bristol, Avon), 10, 293-303. http://dx.doi.org/10.1016/0268-0033(95)00004-5
Nottmeier, E.W. and Young, P.M. (2010) Image-Guided Placement of Occipitocervical Instrumentation Using a Reference Arc Attached to the Headholder. Neurosurgery, 66, 138-142. http://dx.doi.org/10.1227/01.NEU.0000346264.66833.B3
Richter, M., Cakir, B. and Schmidt, R. (2005) Cervical Pedicle Screws: Conventional versus COMPUTER-Assisted Placement of Cannulated Screws. Spine (Phila Pa 1976), 30, 2280-2287. http://dx.doi.org/10.1097/01.brs.0000182275.31425.cd
Rohany, M., Shaibani, A., Arafat, O., Walker, MT., Russell, E.J., Batjer, H.H., et al. (2007) Spinal Arteriovenous Malformations Associated with Klippel-Trenaunay-Weber Syndrome: A Literature Search and Report of Two Cases. American Journal of Neuroradiology (AJNR), 28, 584-589.