Accepted Manuscript Grisel's syndrome following adenoidectomy: surgical management in a case with delayed diagnosis Pietro Spennato, MD, Giancarlo Nicosia, MD, Armando Rapanà, MD, Domenico Cicala, MD, Tiziana Donnianni, MD, Silvana Scala, MD, Ferdinando Aliberti, MD, Giuseppe Cinalli, MD PII:
S1878-8750(15)00493-3
DOI:
10.1016/j.wneu.2015.04.060
Reference:
WNEU 2889
To appear in:
World Neurosurgery
Received Date: 20 December 2014 Revised Date:
23 April 2015
Accepted Date: 23 April 2015
Please cite this article as: Spennato P, Nicosia G, Rapanà A, Cicala D, Donnianni T, Scala S, Aliberti F, Cinalli G, Grisel's syndrome following adenoidectomy: surgical management in a case with delayed diagnosis, World Neurosurgery (2015), doi: 10.1016/j.wneu.2015.04.060. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Grisel's syndrome following adenoidectomy: surgical management in a case with delayed diagnosis Pietro Spennato°, MD, Giancarlo Nicosia°, MD, Armando Rapanà*, MD, Domenico Cicala', MD, Tiziana Donnianni§, MD, Silvana Scala#, MD, Ferdinando Aliberti°, MD, Giuseppe Cinalli°, MD.
Annunziata Children’s Hospital, Naples, Italy
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Corresponding author: Dr Pietro Spennato M.D. Department of Neurosurgery Santobono-Pausilipon Children’s Hospital Via Mario Fiore n. 6 80129 Naples, Italy Phone +390812205690 Fax +390812205660 Email: [email protected]
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*Neurosurgery Unit, AORN Sant'Anna e San Sebastiano Hospital, Caserta, Italy
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Neurosurgery (°), Neuroradiology ('), Radiology(§) and ENT (#) Departments, AORN Santobono-Pausilipon-
Key words: atlanto-axial fixation, atlanto-axial instability, atlanto-axial rotatory subluxation, ENT complication
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ABSTRACT
Background: Grisel’s syndrome is a non traumatic rotatory subluxation of the atlanto-axial joint, following naso-
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pharyngeal inflammation or ENT procedures. The syndrome should be suspected in cases of persistent neck pain and stiffness, especially after ENT surgical procedures. The primary treatment of early detected Grisel’s syndrome is conservative. If conservative treatment fails to achieve a stable reduction or it is followed by neurological symptoms,
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arthrodesis of the first and second cervical vertebrae is indicated. We report the case of a 9-year-old boy who developed Grisel’s syndrome after adenoidectomy and was treated with C1-C3 internal fixation and fusion Case description: A 9-year-old boy was referred to our Hospital with a 3-month history of painful torticollis, appeared 4 days after adenoidectomy. The patient underwent neuroimaging study that documented the presence of atlanto-axial rotatory subluxation. The patient underwent C1-C3 internal fixation and fusion, using lateral masses, laminar and pars interarticularis screws. On third post-operative day he was mobilized with a rigid collar. Post-operative CT scans showed the resolution of rotational deformity and a solid fusion. Conclusion: Early treatment of Grisel’s syndrome is of utmost importance to avoid neurological complications and surgical intervention. In patient with torticollis following ENT procedures, Grisel's syndrome should be always suspected. In case of failure of conservative treatment or in case of delayed diagnosis, rigid C1-C2 or C1-C2-C3
ACCEPTED MANUSCRIPT fixation is a straightforward and valid surgical technique, also in children, because it provides immediate spinal stability in all planes at the atlantoaxial complex, avoiding the need for prolonged rigid external bracing.
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Key words: atlanto-axial fixation, atlanto-axial instability, atlanto-axial rotatory subluxation, ENT complication
INTRODUCTION
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Grisel’s syndrome is a spontaneous rotatory subluxation of the atlanto-axial joint not associated with trauma or bone disease, found primarily in children [7]. It may occurs after naso-pharyngeal inflammation or ENT procedures such as
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tonsillectomy, adenoidectomy and mastoidectomy. It should be suspected in cases of persistent neck pain and stiffness, especially after ENT procedures. X-rays and computed tomography scans of the cervical spine confirm the diagnosis. Early management, consisting of closed reduction and cervical immobilization and medical treatment, is the key factor for a satisfactory outcome [8]. We report the case of a 9-year-old boy who developed Grisel’s syndrome after adenoidectomy. The radiological diagnosis and the surgical treatment are hereby discussed.
CASE REPORT
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An Italian 9-year-old boy underwent adenoidectomy and bilateral tonsillar reduction, under general anaesthesia. The procedure was uneventful and the patient was discharged home the day after surgery. On the fourth post-operative day, painful torticollis occurred with mild rotation of the patient’s head to the right. For this reason the patient was referred to the emergency room. The physical examination revealed a spasm in the right sternocleidomastoid muscle and the patient was discharged with a diagnosis of torticollis, recommending medical therapy and neck immobilization with
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cervical collar, without radiological investigations. The patient was referred to the emergency room three more times, because of persistence of painful torticollis. Finally, about 2 months after ENT surgery, the patient underwent
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neuroimaging examinations that documented the presence of an atlanto-axial rotatory subluxation. Therefore the patient was referred to neurosurgical department. At admission the patient was neurologically intact and exhibit head rotated to the right side and tilted to the left, in slight flexion. Neck mobility was restricted on each side. Pediatric and ENT consultations showed no signs of inflammation/infection of the upper airways. Haematological and biochemical tests were within the normal range. The patient was initially treated with a cervical collar, anti-inflammatory and muscle relaxant drugs for two weeks, with no improvement of the clinical and radiological scenario.
Radiological findings Radiological evaluation included X-ray of the cervical spine with antero-posterior, lateral, odontoid projections and dynamic flexion-extension study; Computed Tomography (CT) scan with 3D reconstructions and Magnetic Resonance Imaging (MRI) of cervical spine. X-ray revealed the typical head tilt of the atlanto-axial subluxation; reduced mobility during flexion and no mobility during extension. On the CT scan, the 3D reconstructions confirmed the atlanto-axial rotatory subluxation with antero-lateral dislocation of C1 and showed a clearly widened distance between the atlas and
ACCEPTED MANUSCRIPT dens, measuring 6.7 mm. The atlas was right rotated 45 degrees respect to the axis (Fig. 1). The T1-weighted magnetic resonance imaging (MRI) showed a widened atlanto-dental space with heterogeneous hyperintensity in T2-weighted images at this level, and interruption of the signal of the transverse ligament on the left (Fig. 2). Angio CT was useful to identify the relationship of the vertebral artery with the posterior elements of the upper cervical vertebrae and to exclude anomalies in its course (Fig. 3).
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Operation The patient underwent open C1-C3 internal fixation and fusion. Under general anaesthesia, with the patient in prone position and the head fixed in a three pin headframe, a reduction of the rotatory subluxation was accomplished under fluoroscopy: a gentle stretching of the patient's neck along with a gentle left rotary maneuver was given. During the procedure, an assistant fixed the shoulders of the patient to prevent any motion. Therefore, internal rigid fixation was
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achieved with C1 lateral masses screws (Fig. 4a) and C3 pars interarticularis screws (Fig. 4c); one intralaminar screw was also placed in the left lamina of C2 starting from the right in order to push the spinous process of C2 to the left to counteract the rotation of the axis (Fig. 4b). The system was assembled with lordotic shaped titanium bars (Fig. 4d-4g) ;
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the fusion was favoured with the aid of granules of tricalcium phosphate, positioned following decortication of the laminae and articular processes of the exposed vertebrae (C1-C3).
Throughout the positioning of the patient and the surgical procedure SSEP and MEP neuromonitoring was used.
Postoperative course
Post-operative period was uneventful. All pre-operative symptoms resolved. The patient was mobilized in the third post-operative day with a rigid collar. The collar was maintained for 6 weeks, while starting physiotherapy. The post-
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operative CT scan confirmed the correct position of the stabilization system (Fig. 4). At 6 months follow up the patient has returned to his ordinary life with no problems, except for a 30 degrees limitation of the rotation of the head.
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DISCUSSION:
Non-traumatic subluxation of the atlanto-axial joint is an unusual and poorly understood pathological condition which may rarely occur in children after naso-pharyngeal inflammation or otolaryngological procedures.
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There are different pathogenetic hypotheses, recently described by Osiro and coworkers in a comprehensive review [8]. A possible pathway for the spread of inflammation to the atlanto-axial ligaments is through anastomoses between lymphatic vessels and pharyngo-vertebral veins. Due to the direct connection between the periodontoid venous plexuses and pharyngo-vertebral veins, a hematogenous pathway may be provided for the transport of peripharyngeal septic effusions to the atlanto-axial ligaments. Inflammation, leading to hyperemia, may result into a laxity of both transverse and alar ligaments. In addition, because of inflammation, C1 and C2 may decalcify, causing a weakening of the insertions of the transverse ligament. Alternative hypothesis was postulated by Welinderet et al. [9] that suggested that Grisel syndrome starts as a typical torticollis from the spasm of irritated neck muscles. In the presence of preexisting lax C1–C2 ligaments, this spasm can lead to distension and eventually subluxation of the atlas and axis. A similar two-hit hypothesis was proposed by Battiata et al: the first hit should be the existence of this baseline laxity, the second hit, should be the induction of spasm caused by the inflammatory mediators carried to the cervical muscles by pharyngovertebral venous plexus [3]. Grisel’s syndrome usually manifests with torticollis, cervical pain, head tilting and restricted-painful neck movements.
ACCEPTED MANUSCRIPT Fielding and Hawkins classified the non-traumatic subluxation of the atlanto-axial joint into four types, according to the displacement of the dens and asymmetry of atlantoaxial joint [6]. Type I and II subluxations are those most commonly found and have no neurological impairment: in these cases rotatory dislocation is present without anterior displacement of the atlas (type I) or with minimally (5 mm anterior displacement of the atlas) and type IV (rotatory dislocation with posterior displacement of the atlas) subluxations, although rare, are often associated with spinal cord compression and are usually associated with
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neurological deficits or possible fatal consequences. The case reported here was consistent with Type III subluxation without neurological deficits.
The diagnosis of atlanto-axial subluxation requires a thorough neuroradiological examination including plain radiographs, CT and magnetic resonance imaging (MRI). Lateral plain radiographs of the upper cervical spine may show an increase in the atlanto-odontoid distance. The normal atlanto-odontoid distance is ≤ 3 mm in adults, and ≤ 5
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mm in children. CT is the most helpful method in establishing the diagnosis of atlanto-axial subluxation. The best visualization of this pathological state is by 3D CT. MRI is a complementary radiological tool being able to reveal the abnormality of soft tissues and neural structures, as laxity of the transverse and alar ligaments [5].
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Fielding’s Type I and II subluxations may be treated with antibiotics and cervical collar, but, in some patients, reduction can only be achieved with cervical traction, followed by a period of neck immobilization in a cervical collar or Halo Vest for 6 weeks, to prevent recurrence of the subluxation. Fielding’s Type III and IV subluxations generally need bed rest with cervical traction, followed by a period of neck immobilization.
The primary treatment of early detected Grisel’s syndrome is conservative [4, 5, 8] and includes bed rest, external fixation, antibiotic therapy, muscle relaxants, and anti-inflammatory therapy. Time to reduction of atlanto-axial rotatory subluxation, is directly related to failure of medical treatment and to an increased risk of recurrence or permanent neck
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deformity.
In the series of Fernandez Cornejo (4 patients), no patients needed surgery, but one patient developed permanent restriction of neck mobility [5]. In the study by Deichmueller et al, 8 of their 12 patients had full remission after intravenous antibiotics and oral antiinflammatory therapies alone. Four patients required reposition of the atlanto-axial joint in general anaesthesia and external fixation by halo-fixation for 6 weeks [4].
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Akbay et al recently reported their experience using a manual reduction maneuver to reduce atlanto-axial rotatory dislocation in children [2]. Most patients were affected by post-traumatic atlanto-axial rotatory dislocation. Under
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sedation, with the patient in supine position, with the left hand of the surgeon fixing the occiput-occipital protuberance and the right hand of the surgeon fixing the patient's chin, a gentle stretching of the patient's neck along with a gentle rotary maneuver was given to the opposite side of the rotational deformity. In this procedure, an assistant should fix the shoulders of the patient to prevent any motion. The patients had worn a sternal-occipital-mandibular immobilizer (SOMI) brace following discharge, for 4 weeks. Their results were very good with resolution of dislocation in 10/11 patients. However all patients presented type I or II rotatory dislocation. As suggested by the same authors, this technique has been demonstrated to be effective for patients with Fielding and Hawkins type I and type II dislocations only[2]. Our patients presented a type III dislocation, therefore following failure of conservative treatment, surgery was considered unavoidable. When surgery is needed, a rigid C1-C2 or C1-C2-C3 fixation using lateral masses and pars interarticularis screws is associated with high fusion rates and immediate spinal stability in all planes at the atlantoaxial complex, thereby circumventing the need for prolonged rigid external bracing postoperatively [1]. This technique should be preferred to
ACCEPTED MANUSCRIPT semirigid constructs using interlaminar or interspinous wires and cables that offer effective stabilization only in the sagittal plane, but does not limit the axial rotation. Hence the need for prolonged rigid external orthosis (halo-fixation for at least 6 weeks) [1-4]. Though a C1-C2 fixation is usually able to warrant a valid stability, we choose to include C3 too in the construct because due to C2 anatomy, there was not enough room to place bilateral translaminar screws, and pedicular or pars interarticularis screws were considered more risky and technically more demanding than translaminar screws technique as suggested by Wright [10]. Moreover, the translaminar screw technique is reported to provide
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adequate stability to the C1-C2 complex, when compared with pedicular or pars interarticularis screws, with lower risks [10]. Furthermore, the choice to place the single screw inside the left C2 lamina meet the concept that a right directed force vector would have been of great help to counteract the possible recurrence of rotational subluxation of the axis. On the basis of a preliminary CT scan that excluded the presence of C1-C2 bony bridges, a preoperative transkeletal traction was not utilized in this case as an open reduction was considered easily feasible at time of surgery.
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Though the negative impact in terms of adjacent disc degeneration and range of motion reduction as a consequence of a C1-C3 fusion should be always kept in mind, the complex instability coming from ligamentous damage from infective disease, mostly when the treatment is delayed, poses the need for a stable fusion as a primary goal.
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To reduce the risk of adjacent level disc degeneration and the negative aspects of rigid fixation on growth in young children, we usually plan to remove the hardware, once bony fusion is achieved. In this case, the aim of treatment is to achieve a bony fusion between C1 and C2, so removal of the hardware may increase the range of motion in the subaxial spine.
In conclusion, in patient with torticollis following ENT procedures, Grisel's syndrome should be always suspected, in order to undertake early adequate diagnostic and therapeutic measures. Early treatment of Grisel’s syndrome is of
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utmost importance to avoid neurological complications and surgical intervention.
Legend to figures:
Fig. 1: Preoperative CT scan. Fig. 1a: Volume rendering reconstruction showing the typical position of the head (rotated on the right and tilted on the left), with increases distance between C1 and C2 posterior elements and loss of alignment between right C1-C2 joints. Fig. 1b: coronal MPR. Loss of alignment between right C1-C2 joints. Fig. 1c: MIP axial
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reconstruction showing C1-C2 rotatory subluxation (about 45°), with atlanto-dental space measuring 6,7 mm (Fielding type 3 atlantoaxial subluxation).
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Fig. 2: Pre-operative MR imaging showing loss of alignment between C1-C2 joints in the sagittal plane (Fig 2a, T2 WI sequence) and interruption of the signal of the transverse ligament in the axial plane (Fig. 2b, FFE T2* sequence) Fig. 3: Pre-operative angio CT showing the normal course of the vertebral arteries and their relations with C1-C2 joints Fig. 4: Post-operative CT scan showing the positioning of the screws in the lateral masses of C1 (Fig. 4a), in the left lamina of C2 (Fig. 4b) and in the pars interarticularis of C3 (Fig. 4c); re-alignment of C1-C2 complex in the sagittal (Fig. 4d-4e), coronal (Fig. 4f-4g) and axial planes (Fig. 4h).
References:
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Ahmed R, Traynelis VC, Menezes AH: Fusions at the craniovertebral junction. Childs Nerv Syst 24: 1209– 1224, 2008 Akbay A, Bilginer B, Akalan N: Closed manual reduction maneuver of atlantoaxial rotatory dislocation in pediatric age. Childs Nerv Syst 30:1083–1089, 2014
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6. 7. 8. 9.
Battiata A, Pazos G: Grisel’s syndrome: the two-hit hypothesis —a case report and literature review. Ear Nose Throat J 83: 553–555, 2004 Deichmueller C, Welkoborsky HJ: Grisel’s syndrome—a rare complication following “small” operations and infections in the ENT region. Eur Arch Otorhinolaryngol 267: 1467–1473, 2010 Fernández Cornejo VJ, Martínez-Lage JF, Piqueras C, Gelabert A, Poza M: Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management. Childs Nerv Syst 19: 342-7, 2003 Fielding JW, Hawkins RJ: Atlanto-axial rotatory fixation. J Bone Joint Surg Am 59: 37–44, 1977 Grisel P: Enucleation de l'atlas et torticollis nasopharyngien. Presse Med 38:50–53, 1930 Osiro S, Tiwari KJ, Matusz P, Gielecki J, Tubbs RS, Loukas M: Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options. Childs Nerv Syst 28: 821–825, 2012 Welinder N, Hoffmann P, Hakansson S: Pathogenesis of non-traumatic atlanto-axial subluxation (Grisel’s syndrome). Eur Arch Otorhinolaryngol 254: 251–254, 1997
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10. Wright NM : Posterior C2 fixation using bilateral, crossing C2 laminar screws : case series and technical note. J Spinal Disord Tech 17 : 158-162, 2004
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ACCEPTED MANUSCRIPT Abbreviations
CT: Computed Tomography
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ENT: Ear Nose Thoat FFE: Fast Field Echo MEP: Motor Evocate Potentials
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MIP: Maximum Intensitive Projection MPR: Multiplanar Recontruction
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MRI: Magnetic Resonance Imaging SSEP: Somato-sensorial evocated potentials
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WI: Weighted Images
ACCEPTED MANUSCRIPT Highlights
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• •
Grisel’s syndrome is a non traumatic rotatory subluxation of the atlanto-axial joint, following naso-pharyngeal inflammation or ENT procedures Primary treatment of Grisel’s syndrome is conservative. In case of failure of conservative treatment, surgery is indicated Rigid C1-C2 or C1-C2-C3 fixation is the best surgical option, because it provides immediate spinal stability in all planes of the atlantoaxial complex
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•
S1878-8750(15)00493-3
DOI:
10.1016/j.wneu.2015.04.060
Reference:
WNEU 2889
To appear in:
World Neurosurgery
Received Date: 20 December 2014 Revised Date:
23 April 2015
Accepted Date: 23 April 2015
Please cite this article as: Spennato P, Nicosia G, Rapanà A, Cicala D, Donnianni T, Scala S, Aliberti F, Cinalli G, Grisel's syndrome following adenoidectomy: surgical management in a case with delayed diagnosis, World Neurosurgery (2015), doi: 10.1016/j.wneu.2015.04.060. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Grisel's syndrome following adenoidectomy: surgical management in a case with delayed diagnosis Pietro Spennato°, MD, Giancarlo Nicosia°, MD, Armando Rapanà*, MD, Domenico Cicala', MD, Tiziana Donnianni§, MD, Silvana Scala#, MD, Ferdinando Aliberti°, MD, Giuseppe Cinalli°, MD.
Annunziata Children’s Hospital, Naples, Italy
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Corresponding author: Dr Pietro Spennato M.D. Department of Neurosurgery Santobono-Pausilipon Children’s Hospital Via Mario Fiore n. 6 80129 Naples, Italy Phone +390812205690 Fax +390812205660 Email: [email protected]
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*Neurosurgery Unit, AORN Sant'Anna e San Sebastiano Hospital, Caserta, Italy
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Neurosurgery (°), Neuroradiology ('), Radiology(§) and ENT (#) Departments, AORN Santobono-Pausilipon-
Key words: atlanto-axial fixation, atlanto-axial instability, atlanto-axial rotatory subluxation, ENT complication
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ABSTRACT
Background: Grisel’s syndrome is a non traumatic rotatory subluxation of the atlanto-axial joint, following naso-
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pharyngeal inflammation or ENT procedures. The syndrome should be suspected in cases of persistent neck pain and stiffness, especially after ENT surgical procedures. The primary treatment of early detected Grisel’s syndrome is conservative. If conservative treatment fails to achieve a stable reduction or it is followed by neurological symptoms,
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arthrodesis of the first and second cervical vertebrae is indicated. We report the case of a 9-year-old boy who developed Grisel’s syndrome after adenoidectomy and was treated with C1-C3 internal fixation and fusion Case description: A 9-year-old boy was referred to our Hospital with a 3-month history of painful torticollis, appeared 4 days after adenoidectomy. The patient underwent neuroimaging study that documented the presence of atlanto-axial rotatory subluxation. The patient underwent C1-C3 internal fixation and fusion, using lateral masses, laminar and pars interarticularis screws. On third post-operative day he was mobilized with a rigid collar. Post-operative CT scans showed the resolution of rotational deformity and a solid fusion. Conclusion: Early treatment of Grisel’s syndrome is of utmost importance to avoid neurological complications and surgical intervention. In patient with torticollis following ENT procedures, Grisel's syndrome should be always suspected. In case of failure of conservative treatment or in case of delayed diagnosis, rigid C1-C2 or C1-C2-C3
ACCEPTED MANUSCRIPT fixation is a straightforward and valid surgical technique, also in children, because it provides immediate spinal stability in all planes at the atlantoaxial complex, avoiding the need for prolonged rigid external bracing.
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Key words: atlanto-axial fixation, atlanto-axial instability, atlanto-axial rotatory subluxation, ENT complication
INTRODUCTION
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Grisel’s syndrome is a spontaneous rotatory subluxation of the atlanto-axial joint not associated with trauma or bone disease, found primarily in children [7]. It may occurs after naso-pharyngeal inflammation or ENT procedures such as
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tonsillectomy, adenoidectomy and mastoidectomy. It should be suspected in cases of persistent neck pain and stiffness, especially after ENT procedures. X-rays and computed tomography scans of the cervical spine confirm the diagnosis. Early management, consisting of closed reduction and cervical immobilization and medical treatment, is the key factor for a satisfactory outcome [8]. We report the case of a 9-year-old boy who developed Grisel’s syndrome after adenoidectomy. The radiological diagnosis and the surgical treatment are hereby discussed.
CASE REPORT
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An Italian 9-year-old boy underwent adenoidectomy and bilateral tonsillar reduction, under general anaesthesia. The procedure was uneventful and the patient was discharged home the day after surgery. On the fourth post-operative day, painful torticollis occurred with mild rotation of the patient’s head to the right. For this reason the patient was referred to the emergency room. The physical examination revealed a spasm in the right sternocleidomastoid muscle and the patient was discharged with a diagnosis of torticollis, recommending medical therapy and neck immobilization with
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cervical collar, without radiological investigations. The patient was referred to the emergency room three more times, because of persistence of painful torticollis. Finally, about 2 months after ENT surgery, the patient underwent
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neuroimaging examinations that documented the presence of an atlanto-axial rotatory subluxation. Therefore the patient was referred to neurosurgical department. At admission the patient was neurologically intact and exhibit head rotated to the right side and tilted to the left, in slight flexion. Neck mobility was restricted on each side. Pediatric and ENT consultations showed no signs of inflammation/infection of the upper airways. Haematological and biochemical tests were within the normal range. The patient was initially treated with a cervical collar, anti-inflammatory and muscle relaxant drugs for two weeks, with no improvement of the clinical and radiological scenario.
Radiological findings Radiological evaluation included X-ray of the cervical spine with antero-posterior, lateral, odontoid projections and dynamic flexion-extension study; Computed Tomography (CT) scan with 3D reconstructions and Magnetic Resonance Imaging (MRI) of cervical spine. X-ray revealed the typical head tilt of the atlanto-axial subluxation; reduced mobility during flexion and no mobility during extension. On the CT scan, the 3D reconstructions confirmed the atlanto-axial rotatory subluxation with antero-lateral dislocation of C1 and showed a clearly widened distance between the atlas and
ACCEPTED MANUSCRIPT dens, measuring 6.7 mm. The atlas was right rotated 45 degrees respect to the axis (Fig. 1). The T1-weighted magnetic resonance imaging (MRI) showed a widened atlanto-dental space with heterogeneous hyperintensity in T2-weighted images at this level, and interruption of the signal of the transverse ligament on the left (Fig. 2). Angio CT was useful to identify the relationship of the vertebral artery with the posterior elements of the upper cervical vertebrae and to exclude anomalies in its course (Fig. 3).
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Operation The patient underwent open C1-C3 internal fixation and fusion. Under general anaesthesia, with the patient in prone position and the head fixed in a three pin headframe, a reduction of the rotatory subluxation was accomplished under fluoroscopy: a gentle stretching of the patient's neck along with a gentle left rotary maneuver was given. During the procedure, an assistant fixed the shoulders of the patient to prevent any motion. Therefore, internal rigid fixation was
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achieved with C1 lateral masses screws (Fig. 4a) and C3 pars interarticularis screws (Fig. 4c); one intralaminar screw was also placed in the left lamina of C2 starting from the right in order to push the spinous process of C2 to the left to counteract the rotation of the axis (Fig. 4b). The system was assembled with lordotic shaped titanium bars (Fig. 4d-4g) ;
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the fusion was favoured with the aid of granules of tricalcium phosphate, positioned following decortication of the laminae and articular processes of the exposed vertebrae (C1-C3).
Throughout the positioning of the patient and the surgical procedure SSEP and MEP neuromonitoring was used.
Postoperative course
Post-operative period was uneventful. All pre-operative symptoms resolved. The patient was mobilized in the third post-operative day with a rigid collar. The collar was maintained for 6 weeks, while starting physiotherapy. The post-
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operative CT scan confirmed the correct position of the stabilization system (Fig. 4). At 6 months follow up the patient has returned to his ordinary life with no problems, except for a 30 degrees limitation of the rotation of the head.
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DISCUSSION:
Non-traumatic subluxation of the atlanto-axial joint is an unusual and poorly understood pathological condition which may rarely occur in children after naso-pharyngeal inflammation or otolaryngological procedures.
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There are different pathogenetic hypotheses, recently described by Osiro and coworkers in a comprehensive review [8]. A possible pathway for the spread of inflammation to the atlanto-axial ligaments is through anastomoses between lymphatic vessels and pharyngo-vertebral veins. Due to the direct connection between the periodontoid venous plexuses and pharyngo-vertebral veins, a hematogenous pathway may be provided for the transport of peripharyngeal septic effusions to the atlanto-axial ligaments. Inflammation, leading to hyperemia, may result into a laxity of both transverse and alar ligaments. In addition, because of inflammation, C1 and C2 may decalcify, causing a weakening of the insertions of the transverse ligament. Alternative hypothesis was postulated by Welinderet et al. [9] that suggested that Grisel syndrome starts as a typical torticollis from the spasm of irritated neck muscles. In the presence of preexisting lax C1–C2 ligaments, this spasm can lead to distension and eventually subluxation of the atlas and axis. A similar two-hit hypothesis was proposed by Battiata et al: the first hit should be the existence of this baseline laxity, the second hit, should be the induction of spasm caused by the inflammatory mediators carried to the cervical muscles by pharyngovertebral venous plexus [3]. Grisel’s syndrome usually manifests with torticollis, cervical pain, head tilting and restricted-painful neck movements.
ACCEPTED MANUSCRIPT Fielding and Hawkins classified the non-traumatic subluxation of the atlanto-axial joint into four types, according to the displacement of the dens and asymmetry of atlantoaxial joint [6]. Type I and II subluxations are those most commonly found and have no neurological impairment: in these cases rotatory dislocation is present without anterior displacement of the atlas (type I) or with minimally (5 mm anterior displacement of the atlas) and type IV (rotatory dislocation with posterior displacement of the atlas) subluxations, although rare, are often associated with spinal cord compression and are usually associated with
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neurological deficits or possible fatal consequences. The case reported here was consistent with Type III subluxation without neurological deficits.
The diagnosis of atlanto-axial subluxation requires a thorough neuroradiological examination including plain radiographs, CT and magnetic resonance imaging (MRI). Lateral plain radiographs of the upper cervical spine may show an increase in the atlanto-odontoid distance. The normal atlanto-odontoid distance is ≤ 3 mm in adults, and ≤ 5
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mm in children. CT is the most helpful method in establishing the diagnosis of atlanto-axial subluxation. The best visualization of this pathological state is by 3D CT. MRI is a complementary radiological tool being able to reveal the abnormality of soft tissues and neural structures, as laxity of the transverse and alar ligaments [5].
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Fielding’s Type I and II subluxations may be treated with antibiotics and cervical collar, but, in some patients, reduction can only be achieved with cervical traction, followed by a period of neck immobilization in a cervical collar or Halo Vest for 6 weeks, to prevent recurrence of the subluxation. Fielding’s Type III and IV subluxations generally need bed rest with cervical traction, followed by a period of neck immobilization.
The primary treatment of early detected Grisel’s syndrome is conservative [4, 5, 8] and includes bed rest, external fixation, antibiotic therapy, muscle relaxants, and anti-inflammatory therapy. Time to reduction of atlanto-axial rotatory subluxation, is directly related to failure of medical treatment and to an increased risk of recurrence or permanent neck
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In the series of Fernandez Cornejo (4 patients), no patients needed surgery, but one patient developed permanent restriction of neck mobility [5]. In the study by Deichmueller et al, 8 of their 12 patients had full remission after intravenous antibiotics and oral antiinflammatory therapies alone. Four patients required reposition of the atlanto-axial joint in general anaesthesia and external fixation by halo-fixation for 6 weeks [4].
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Akbay et al recently reported their experience using a manual reduction maneuver to reduce atlanto-axial rotatory dislocation in children [2]. Most patients were affected by post-traumatic atlanto-axial rotatory dislocation. Under
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sedation, with the patient in supine position, with the left hand of the surgeon fixing the occiput-occipital protuberance and the right hand of the surgeon fixing the patient's chin, a gentle stretching of the patient's neck along with a gentle rotary maneuver was given to the opposite side of the rotational deformity. In this procedure, an assistant should fix the shoulders of the patient to prevent any motion. The patients had worn a sternal-occipital-mandibular immobilizer (SOMI) brace following discharge, for 4 weeks. Their results were very good with resolution of dislocation in 10/11 patients. However all patients presented type I or II rotatory dislocation. As suggested by the same authors, this technique has been demonstrated to be effective for patients with Fielding and Hawkins type I and type II dislocations only[2]. Our patients presented a type III dislocation, therefore following failure of conservative treatment, surgery was considered unavoidable. When surgery is needed, a rigid C1-C2 or C1-C2-C3 fixation using lateral masses and pars interarticularis screws is associated with high fusion rates and immediate spinal stability in all planes at the atlantoaxial complex, thereby circumventing the need for prolonged rigid external bracing postoperatively [1]. This technique should be preferred to
ACCEPTED MANUSCRIPT semirigid constructs using interlaminar or interspinous wires and cables that offer effective stabilization only in the sagittal plane, but does not limit the axial rotation. Hence the need for prolonged rigid external orthosis (halo-fixation for at least 6 weeks) [1-4]. Though a C1-C2 fixation is usually able to warrant a valid stability, we choose to include C3 too in the construct because due to C2 anatomy, there was not enough room to place bilateral translaminar screws, and pedicular or pars interarticularis screws were considered more risky and technically more demanding than translaminar screws technique as suggested by Wright [10]. Moreover, the translaminar screw technique is reported to provide
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adequate stability to the C1-C2 complex, when compared with pedicular or pars interarticularis screws, with lower risks [10]. Furthermore, the choice to place the single screw inside the left C2 lamina meet the concept that a right directed force vector would have been of great help to counteract the possible recurrence of rotational subluxation of the axis. On the basis of a preliminary CT scan that excluded the presence of C1-C2 bony bridges, a preoperative transkeletal traction was not utilized in this case as an open reduction was considered easily feasible at time of surgery.
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Though the negative impact in terms of adjacent disc degeneration and range of motion reduction as a consequence of a C1-C3 fusion should be always kept in mind, the complex instability coming from ligamentous damage from infective disease, mostly when the treatment is delayed, poses the need for a stable fusion as a primary goal.
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To reduce the risk of adjacent level disc degeneration and the negative aspects of rigid fixation on growth in young children, we usually plan to remove the hardware, once bony fusion is achieved. In this case, the aim of treatment is to achieve a bony fusion between C1 and C2, so removal of the hardware may increase the range of motion in the subaxial spine.
In conclusion, in patient with torticollis following ENT procedures, Grisel's syndrome should be always suspected, in order to undertake early adequate diagnostic and therapeutic measures. Early treatment of Grisel’s syndrome is of
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Legend to figures:
Fig. 1: Preoperative CT scan. Fig. 1a: Volume rendering reconstruction showing the typical position of the head (rotated on the right and tilted on the left), with increases distance between C1 and C2 posterior elements and loss of alignment between right C1-C2 joints. Fig. 1b: coronal MPR. Loss of alignment between right C1-C2 joints. Fig. 1c: MIP axial
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reconstruction showing C1-C2 rotatory subluxation (about 45°), with atlanto-dental space measuring 6,7 mm (Fielding type 3 atlantoaxial subluxation).
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Fig. 2: Pre-operative MR imaging showing loss of alignment between C1-C2 joints in the sagittal plane (Fig 2a, T2 WI sequence) and interruption of the signal of the transverse ligament in the axial plane (Fig. 2b, FFE T2* sequence) Fig. 3: Pre-operative angio CT showing the normal course of the vertebral arteries and their relations with C1-C2 joints Fig. 4: Post-operative CT scan showing the positioning of the screws in the lateral masses of C1 (Fig. 4a), in the left lamina of C2 (Fig. 4b) and in the pars interarticularis of C3 (Fig. 4c); re-alignment of C1-C2 complex in the sagittal (Fig. 4d-4e), coronal (Fig. 4f-4g) and axial planes (Fig. 4h).
References:
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Ahmed R, Traynelis VC, Menezes AH: Fusions at the craniovertebral junction. Childs Nerv Syst 24: 1209– 1224, 2008 Akbay A, Bilginer B, Akalan N: Closed manual reduction maneuver of atlantoaxial rotatory dislocation in pediatric age. Childs Nerv Syst 30:1083–1089, 2014
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6. 7. 8. 9.
Battiata A, Pazos G: Grisel’s syndrome: the two-hit hypothesis —a case report and literature review. Ear Nose Throat J 83: 553–555, 2004 Deichmueller C, Welkoborsky HJ: Grisel’s syndrome—a rare complication following “small” operations and infections in the ENT region. Eur Arch Otorhinolaryngol 267: 1467–1473, 2010 Fernández Cornejo VJ, Martínez-Lage JF, Piqueras C, Gelabert A, Poza M: Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management. Childs Nerv Syst 19: 342-7, 2003 Fielding JW, Hawkins RJ: Atlanto-axial rotatory fixation. J Bone Joint Surg Am 59: 37–44, 1977 Grisel P: Enucleation de l'atlas et torticollis nasopharyngien. Presse Med 38:50–53, 1930 Osiro S, Tiwari KJ, Matusz P, Gielecki J, Tubbs RS, Loukas M: Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options. Childs Nerv Syst 28: 821–825, 2012 Welinder N, Hoffmann P, Hakansson S: Pathogenesis of non-traumatic atlanto-axial subluxation (Grisel’s syndrome). Eur Arch Otorhinolaryngol 254: 251–254, 1997
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10. Wright NM : Posterior C2 fixation using bilateral, crossing C2 laminar screws : case series and technical note. J Spinal Disord Tech 17 : 158-162, 2004
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CT: Computed Tomography
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ENT: Ear Nose Thoat FFE: Fast Field Echo MEP: Motor Evocate Potentials
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MIP: Maximum Intensitive Projection MPR: Multiplanar Recontruction
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MRI: Magnetic Resonance Imaging SSEP: Somato-sensorial evocated potentials
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WI: Weighted Images
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Grisel’s syndrome is a non traumatic rotatory subluxation of the atlanto-axial joint, following naso-pharyngeal inflammation or ENT procedures Primary treatment of Grisel’s syndrome is conservative. In case of failure of conservative treatment, surgery is indicated Rigid C1-C2 or C1-C2-C3 fixation is the best surgical option, because it provides immediate spinal stability in all planes of the atlantoaxial complex
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