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Moyamoya disease presenting with acute subdural hemorrhage Sir, Moyamoya disease is characterized by progressive occlusion of distal internal carotid artery and its branches resulting in ischemic infarcts and collateral formation as a compensatory mechanism[1] Angiographic appearance of this collateral network is like “puff of smoke” which in Japanese language called “moyamoya.”[1] Moyamoya disease itself is very rare and is more common in Japanese population.[1,2] This report presents a rare presentation of moyamoya disease.

Figure 1: Computed tomography scan showing acute subdural hemorrhage

A 46-year-old male presented with sudden onset loss of consciousness followed by headache and vomiting. Computed tomography scan showed right acute subdural hematoma (SDH) with mass effect [Figure 1]. Neurologic examination revealed altered mental status and left hemiparesis. Considering nontraumatic nature of hemorrhage, magnetic resonance angiography was done and it showed right internal carotid artery occlusion. As there was neurologic deterioration, right decompresive craniotomy and evacuation of SDH was done [Figure 2]. Patient had good recovery. Postoperative digital subtraction angiography had confirmed moyamoya disease [Figure 3]. He was discharged in a stable condition with future plan of revascularization. Moyamoya disease in pediatric patients, presents with ischemic events and intracranial hemorrhage accounts only for 10%, whereas in adults intracranial hemorrhage accounts for more than 60% of cases.[3] Spontaneous acute SDH in moyamoya disease is rare and only 6 cases have been reported.[4-8] This is yet another case. SDH as a complication of moya and moya is due to the rupture of transdural anastomotic vessels.

Figure 2: Postoperative scan showing evacuated subdural hematoma

Amit Kumar Ghosh Department of Neurosurgery, Medica North Bengal Clinic, Siliguri, West Bengal, India E-mail: [email protected]

References 1. 2. 3.

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Guzman R, Lee M, Achrol A, Bell-Stephens T, Kelly M, Do HM, et al. Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article. J Neurosurg 2009;111:927-35. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, et al. Epidemiological features of moyamoya disease in Japan: Findings from a nationwide survey. Clin Neurol Neurosurg 1997;99 Suppl 2:S1-5. Jha VC, Behari S, Singh B, Jaiswal AK. Adult hemorrhagic moyamoya disease: The paradoxical role of combined revascularization. Indian J

Figure 3: Digital subtraction angiography showing moyamoya disease

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Neurosurg 2012;1:108-16. Kawakami K, Takahashi S, Sonobe M, Koshu K, Hirota S, Kusunose M. Subacute subdural hematoma associated with moyamoya phenomenon: A case report. No Shinkei Geka 1988;16:205-9. Shen WC, Lee WY. Moyamoya disease causes acute subdural hematomas Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2

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and sudden death: A case report. Zhonghua Yi Xue Za Zhi (Taipei) 1998;61:619-23. Nakakita K, Tanaka S, Fukuda A, Fujii C, Kohama A, Miyasato H. Nontraumatic acute subdural hematoma caused by the rupture of transdural anastomotic vessels in moyamoya disease. No Shinkei Geka 1994;22:561-5. Takeuchi S, Ichikawa A, Koike T, Tanaka R, Arai H. Acute subdural hematoma in young patient with moyamoya disease: Case report. Neurol Med Chir (Tokyo) 1992;32:80-3. Oppenheim JS, Gennuso R, Sacher M, Hollis P. Acute atraumatic subdural hematoma associated with moyamoya disease in an African-American. Neurosurgery 1991;28:616-8. Access this article online Quick Response Code:

Website: www.neurologyindia.com

PMID: *** DOI: 10.4103/0028-3886.132406

Received: 23-02-2014 Review completed: 24-02-2014 Accepted: 19-04-2014

Intra-operative K-wire breakage during odontoid screw fixation Sir, Traumatic spine injuries involve the cervical spine in about 60% of patients and a quarter of these involve the C2 or axis level.[1,2] Odontoid screw fixation is considered the most elegant technique for type II odontoid fractures as it preserves the full range of movements of the cervical spine. We present a case of traumatic type II odontoid fracture with an uncommon complication of K-wire breakage during the odontoid screw fixation, which was managed without incurring any additional morbidity. A 50-year-old lady presented to the emergency department with a history of motor vehicle accident 10 days before and neck pain since then. The neurological examination was within normal limits. Computed tomography (CT) of the cervical spine showed a type II odontoid fracture. Awake intubation was done with nonreinforced tube using fiberoptic bronchoscope to prevent any motion at the neck. The patient was then positioned on Allen’s spine table (Allen Medical Systems, A Hill-Rom Company, MA, USA) with the neck placed in extension under traction. The O-arm® Intra-operative Imaging System (Medtronic, Inc., Minneapolis, MN, USA) was used for the procedure. Head was positioned (under O-arm imaging) so as to achieve proper alignment of the odontoid and a Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2

satisfactory screw trajectory. A vertical 5 cm incision was given on the right side of the neck along the anterior border of the sternocleidomastoid muscle at C5-6 disc space with dissection carried up to C2 body. Using a pneumatic drill under O-arm guidance, the K-wire was drilled into the body of the odontoid, through the fracture line and into the distal fragment being careful not to breach the distal cortex of the fragment (the distal cortex is not breached to prevent K-wire migration during reaming). A reamer was used over the guide-wire to make threads for the screw. While reaming a significant length of the K-wire broke off which was traversing the length from the C2 body into the fracture fragment [Figure 1]. Fluoroscopy showed no part of the K-wire breaching the cortex of the distal fragment. A second K-wire was then drilled into the fracture fragment adjacent to the previous one being careful not to displace the first K-wire. The drill hole was tapped and a 36 mm partially threaded titanium cannulated lag screw was inserted gradually over the guide-wire under O-arm guidance. The screw was satisfactorily placed and no migration of the broken K-wire was seen. Postoperatively, there were no neurological deficits. Follow-up CT scans 3 months later showed fusion of the fragment and no migration of the broken K-wire [Figure 2]. Another CT scan at 12 months showed good fusion with no migration of broken K-wire and no neurological deficit.

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Figure 1: Intra-operative O-arm images. (a) Lateral view showing type II odontoid fracture; (b) lateral view showing the broken K-wire seen traversing the fracture line into the distal fragment; (c) anteroposterior view, a new K-wire drilled adjacent to the broken wire; (d) lateral view, odontoid screw being placed

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Moyamoya disease presenting with acute subdural hemorrhage.

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