Childs Nerv Syst (2006) 22: 1182–1186 DOI 10.1007/s00381-006-0053-3 Dimitris Kombogiorgas Ihsan Hussain Spyros Sgouros Received: 27 May 2005 Published online: 11 April 2006 # Springer-Verlag 2006 D. Kombogiorgas . I. Hussain . S. Sgouros (*) Department of Neurosurgery, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK e-mail: S.Sgouros@bham.ac.uk Tel.: +44-121-3338075 Fax: +44-121-3338701 CASE REPORT Atlanto-axial rotatory fixation caused by spontaneous intracerebral haemorrhage in a child Abstract Introduction: Atlanto-axial rotatory fixation is rare in children and usually associated with upper respiratory tract infection or trauma. We present a patient who developed the condition acutely as a consequence of rapidly evolving hemiplegia secondary to intracerebral haemorrhage. Case report: A previously well 12-year-old boy suddenly developed left hemiparesis within 30 min. Soon after, he developed painful torticollis. On examination, a dense left hemiplegia was associated with left facial and hypoglossal weakness. On computerised tomography (CT) scan there was an intracerebral haemorrhage in the deep aspect of the right frontal lobe in the corona radiata, with extension in the ventricular system. The plain radiographs of the cervical spine, and CT scan later, confirmed the presence of C1–C2 rotatory subluxation. The haematoma was aspirated stereotactically through a burr hole, and at the same session, the subluxation was Introduction Torticollis is a clinical sign encountered in a wide variety of childhood diseases. It can be caused by congenital, traumatic, inflammatory, neurological, neoplastic, ocular or vestibular disorders, as well as psychogenic and iatrogenic reasons [2, 3, 11, 13, 19, 29]. Non-traumatic torticollis was firstly reported by Bell in 1830 [24]. Since then, several names have been used to describe this condition, such as Griesel’s syndrome [10], reduced by manipulation and traction of the cervical spine. The patient was treated with a hard collar. The torticollis did not recur. The hemiparesis resolved within 6 months. A subsequent magnetic resonance imaging and magnetic resonance angiography scan indicated possible arterio-venous malformation (AVM) at the site of the haemorrhage, which was confirmed with digital subtraction angiography. The AVM was surgically excised 1 year from the original haemorrhage. Discussion: In the absence of any other predisposing factor, it is postulated that the acute atlanto-axial rotatory subluxation was secondary to the acute loss of muscular tone due to the acute hemiparesis. This explains that the subluxation has not recurred a year after, while the hemiparesis has completely resolved. Keywords Atlanto-axial subluxation . Atlanto-axial rotatory fixation . Intracerebral haemorrhage . Torticollis . Stroke atlanto-axial rotatory subluxation (AARS) [12], rotatory fixation of atlanto-axial joint [28] and atlanto-axial rotatory fixation (AARF) [8]. However, acutely acquired torticollis is not necessarily a sign of a pathologic condition of the atlanto-axial joint [11]. The pathophysiological and biomechanical mechanisms that result in this condition are still unclear [8, 9, 21, 24]. We report a case of atlanto-axial rotatory subluxation due to neck muscle paralysis secondary to intracerebral haemorrhage. This is the first case in the literature to our 1183 knowledge. There is only one other similar reported case in our knowledge due to paralysis of the stabilizing muscles on one side of the neck, but this was secondary to poliomyelitis [4]. Case report A 12-year-old boy was admitted in a local hospital because of sudden spontaneous onset of difficulty in speech, followed by development of left-sided facial and limb weakness within the ensuing half hour. He neither lost consciousness nor had seizures. He complained of headache and neck pain and vomited once a few hours after the onset of the stroke. On admission to the hospital the patient was fully orientated. He had severe facial and hypoglossal nerve palsy on the left hand side and left sided flaccid hemiplegia with preservation of sensation and increased plantar reflex. A computerised tomography (CT) scan showed a right parietal intracerebral haematoma (ICH) in the region of the corona radiata, with extension to the ipsilateral lateral ventricle (Fig. 1). The patient was transferred to our unit for further treatment. Torticollis of the neck to the right was diagnosed on admission to our unit, in addition to the already identified clinical signs. Cervical spine radiographs showed malalignment at the C1/C2 level (Fig. 2). CT scan of the spine showed rotatory subluxation of C1 on C2 (Fig. 3). A CT angiogram showed unclear appearances. The patient had stereotactic aspiration of the haematoma under general anaesthetic followed by manipulation and close reduction of C1/C2 rotatory subluxation 6 days after acute onset. The hard neck collar brace was kept in place for 6 weeks, which prevented recurrence of the subluxation. Follow-up CT of the spine was performed 6 weeks after reduction of C1/C2 subluxation. It showed normal alignment of the upper cervical spine, on neutral neck Fig. 1 CT scan of a 12-year-old boy. There is an intracerebral haematoma deep in the right frontal lobe, in the region of the white matter of the corona radiata Fig. 2 Plain radiograph, “open mouth” antero-posterior view, showing abnormal position of the dens axis. There is unequal distance between the lateral borders of the dens axis and the two lateral masses of C1, the distance on the right of the patient is smaller than the left. This is indicative of C1–C2 subluxation position and very minor displacement of the left lateral mass of C1 on C2 during turning the head to the right, considered within the normal limits (Fig. 4). An magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) raised the possibility of an arterio-venous malformation (AVM) at the site of the previous clot. At 12 months from the acute haemorrhage, the patient had minimal left-hand dyspraxia with normal gait. Clinical examination of his neck was normal. On digital subtraction angiography (DSA) the presence of an AVM was con- Fig. 3 Three-dimensional reconstruction of a CT scan of the cervical spine (3DCT), taken in the restricted position of torticollis, showing a dorsal view the spinous processes of all cervical vertebrae. There is obvious mal-alignment at the C1–C2 region, the spinous processes of C1 is obviously out of alignment with C2, deviating to the left, and there is compensatory deviation of the spinous process below C2 to the left. This abnormal deviation of the spinous processes below C2 is characteristic of atlanto-axial rotatory subluxation 1184 Fig. 4 Three-dimensional reconstruction of a CT scan of the cervical spine (3DCT), dorsal view showing the spinous processes of the upper three cervical vertebrae. Following successful reduction of the rotatory deformity, there is normal alignment of the spinous processes firmed, feeding by branches of the right middle cerebral artery (Fig. 5). The AVM was surgically excised successfully. The patient did not develop any additional neurological deficit. Discussion The term atlanto-axial rotatory fixation was first used by Fielding and Hawkins in 1977 [8] to describe the rotatory deformities of the atlanto-axial complex in the form of Fig. 5 Digital subtraction Angiography (DSA) lateral view, following right internal carotid artery injection. There is an obvious arterio-venous malformation (AVM) in the posterior frontal region deep in the hemisphere, fed by arterial branches of the middle cerebral artery subluxation or dislocation. Atlanto-axial rotatory subluxation is an uncommon cause of childhood torticollis and may occur as a result of pharyngeal infection (Griesel’s syndrome), surgical procedure of head and neck, traumatic injury, congenital atlanto-axial abnormalities such as Down syndrome, juvenile rheumatoid arthritis and poliomyelitis [2–4, 16, 19, 23, 29]. The principal motion of the atlanto-axial joint is rotation, but a degree of flexion extension, vertical approximation and lateral translation also take place at this joint. Approximately 50% of all rotary cervical motion occur at atlanto-axial joint [7]. Atlanto-axial rotation as little as 36° may be associated with an excessive contact loss (approximately 80%) of the corresponding facets of atlas and axis [26]. When the atlas is fully rotated on the axis the cervical portion of the spine loses from 2 to 3 mm in height because the opposed articulating surfaces of their lateral masses are both convex. A rotation of 65° of atlas on axis is required for dislocation to occur when the transverse ligament is intact. This degree of rotation results in narrowing of the diameter of an average-sized cervical canal to 7 mm [4]. The transverse ligament is the primary stabilizer of the atlanto-axial joint and prevents excessive anterior shift of the atlas on axis. The alar ligaments contribute to the prevention of the anterior shifts of the atlas as secondary stabilizers but also prevent excessive rotation of occipito-atlantal joint [8]. Both alar ligaments must be intact to limit axial rotation [5]. In contrast to adults, the paediatric cervical spine has some characteristic differences. Those include ligamentous laxity, horizontally orientated facets joints, wedge-shaped cervical vertebrae and poorly developed cervical musculature. Also, the uncinate processes are poorly developed in children and may allow excessive lateral bending and rotation [14, 15, 24, 27]. All these factors may predispose to overrotation and subluxation of atlanto-axial joint and contribute to a higher risk of rotational deformities in children. For the diagnosis of atlanto-axial subluxation the history helps to identify predisposing factors and symptoms duration. There are three clinical signs that may help distinguish muscular torticollis from AARF: (1) the palpable deviation of the spinous process of atlas is in the same direction as the head rotation in AARS. (2) The sternocleidomastoid muscle ipsilateral to the direction of head rotation may be in spasm as an attempt to reduce the deformity. (3) There is inability to rotate the head past midline in the direction opposite that of the injury [24, 25]. Fielding and Hawkins classified AARF in four types in 1977 [8]: 1. Type I, Rotatory fixation without anterior displacement of the atlas (displacement of 3 mm or less); 2. Type II, Rotatory fixation with anterior displacement of the atlas (displacement of 3 to 5 mm); 3. Type III, Rotatory fixation with anterior displacement of the atlas more than 5 mm; 1185 4. Type IV, Rotatory fixation with posterior displacement of the atlas. Type I AARF is the only type that can occur without rupture of the ligaments. The other types are usually associated with rupture of the ligaments. An exception to this is when the atlanto-axial joint in children is lax due to inflammation such as after upper respiratory tract infection. Synovial and vascular engorgement of atlanto-axial joint may cause mechanical and chemical damage to the transverse and facet capsular ligaments leading to subluxation [1, 19, 29]. The stability of the upper cervical spine relies largely on the ligamentous integrity existing between the vertebrae and the occiput. The assessment of integrity of these ligaments can be made with MR and CT scanning [6, 17]. The static CT scanning is adequate for demonstrating pathology in patients with AARF types II–IV, but it cannot differentiate between AARF type I and normal subjects [11]. Hicazi et al. compared the range of motion of the atlanto-axial joint in paediatric patients with acute torticollis secondary to minor or no trauma with those of normal using measurements from CT scans. They could not demonstrate the presence of AARS or AARF type I in their patients, suggesting that it may not be necessary to obtain CT scans (static or dynamic) in this group of patients at the time of presentation [11]. MRI accurately depicts the anatomical integrity of the transverse ligament [6]. It is important for optimal management of paediatric patients with atlanto-axial subluxation to establish early diagnosis [20, 24]. Duration of AARS less than 1 week requires application of soft collar, analgesia and bed rest. If the duration of AARS is more than 1 week but less than 1 month, application of Halter cervical traction is required, and skeletal cervical traction is required for duration of subluxation more than 1 month. Operative treatment is indicated for more long-standing and complex cases [22]. In such complex cases, posterior upper cervical fusion is highly successful [18]. 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