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Ali Nader-Sepahi, Adrian T. H. Casey, Richard Hayward, H. Alan Crockard and Dominic Thompson

neurological morbidity and particularly high failure rates for fusion in this group of patients. Ours is the largest series to investigate symptomatic atlantoaxial subluxation and its management exclusively in a population of pediatric patients with Down syndrome. We conducted a retrospective review of symptomatic atlantoaxial instability in patients with Down syndrome treated at the Great Ormond Street hospital to accomplish the following: 1) audit our own surgical experience in children with Down syndrome; 2) evaluate the underlying anatomical changes specific in this

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Lars de Jong, Michiel Verfaillie, Steven Pans, Philippe Lauweryns, Jan Goffin and Bart Depreitere

A tlantoaxial instability can occur as a result of traumatic disruption of the transverse ligaments with or without fracture and is a potentially life-threatening situation. Nontraumatic causes of atlantoaxial instability, such as rheumatoid arthritis causing progressive destruction of the C1–2 joints, os odontoideum resulting in insufficiency of the odontoid peg, or metabolic diseases with ligamentous insufficiency, can lead to progressive or repetitive narrowing of the anteroposterior diameter of the spinal canal with compression of the spinal cord and

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Curtis A. Dickman, Alexander Mamourian, Volker K. H. Sonntag and Burton P. Drayer

D isruption of the transverse ligament of the atlas has been implicated as the most important pathological abnormality responsible for atlantoaxial instability. 15, 16, 29, 31, 35–37 It has also been considered responsible in certain cases for nonunion of atlas and axis fractures. 1, 2, 5, 19–21, 26, 28, 31, 34, 35 Conventional radiographic studies and computerized tomography (CT) are insufficient to demonstrate the anatomy of the transverse ligament, and the probability for ligamentous injury has been indirectly assessed from correlations of autopsy data

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Kai Shen, Zhongliang Deng, Junsong Yang, Chao Liu and Ranxi Zhang

T he C1–2 joint, which consists of the atlas (C-1) and the axis (C-2), has the largest range of motion (ROM) of all the joints in the cervical spine and is responsible for approximately 50% of the rotation of this part of the spine. 20 , 24 Atlantoaxial instability (AAI) caused by cervical degeneration or trauma is a common condition in spine surgery and may lead to neck pain, spinal cord injury, or even death. 18 Anterior and/or posterior C1–2 fusion is a common treatment for AAI. However, surgical decompression can aggravate C1–2 joint instability, and C1

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Atlantoaxial instability in Dyggve-Melchior-Clausen syndrome

Case report and review of the literature

Frank Kandziora, Luitgard Neumann, Klaus John Schnake, Cyrus Khodadadyan-Klostermann, Stefan Rehart, Norbert P. Haas and Thomas Mittlmeier

radiological signs. Spinal cord compression due to atlantoaxial instability is a serious and preventable complication in both disorders. We report on a 17-year-old man with DMC syndrome and severe atlantoaxial instability treated with anterior transarticular screw fixation. Case Report History At birth the patient weighed 3500 g; his mother's pregnancy was uneventful. The parents are healthy first cousins from Georgia with four children. Two daughters are healthy with normal development. A 19-year-old brother, who still lives in Georgia, seems to be similarly

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biomechanical strength of the anterior and posterior stabilization techniques used for atlantoaxial instability. From their study they conclude that the posterior C-1 lateral mass combined with C-2 pedicle screw/rod fixation technique has the highest biomechanical strength. We have to inform the authors (and the readers of Journal of Neurosurgery: Spine ) that we originally described this technique and have been using it since 1987. The anatomical and technical details of the procedure and our experience with the technique are discussed at length in our various publications

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Justin Slavin, Marcello DiStasio, Paul F. Dellaripa and Michael Groff

mild deformation of the spinal cord but no overt compression ( A ). The axial T2-weighted view shows mild deformation of the spinal cord ( B ). Fig. 3. Sagittal T1-weighted views without ( A ) and with ( B ) gadolinium showing contrast enhancement in the odontoid process. Rheumatoid arthritis is the most common cause of the described atlantoaxial instability with large dorsal C2 pannus. However, her medical history of sarcoidosis and suspicion for active gout in the setting of erosive contrast-enhancing bony lesions aroused further suspicion of these other rare

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Sung-Min Kim, T. Jesse Lim, Josemaria Paterno, Tae-Jin Hwang, Kun-Woo Lee, Raju S. V. Balabhadra and Daniel H. Kim

M any disorders can cause atlantoaxial instability, such as fractures, malignancy, rheumatoid arthritis, congenital anomalies, or infectious diseases. Atlantoaxial instability disrupts bones and ligaments and causes local pain, limitation of ROM, C-2 rhizopathy, or myelopathy. The goals of surgical management in cases of atlantoaxial instability are to provide neural decompression, restoration of occipitocervical spinal alignment, and stabilization of unstable segments to allow for successful osseous fusion. Since Gallie 8 introduced the posterior wiring

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Scott Y. Rahimi, E. Andrew Stevens, David John Yeh, Ann Marie Flannery, Haroon Fiaz Choudhri and Mark R. Lee

The atlantoaxial region has been extensively described as a spinal segment especially prone to injury in children. In this clinical review, the authors evaluate and summarize the management of 23 pediatric cases of atlantoaxial instability treated between March 1990 and October 2002. Four broad categories of atlantoaxial problems were observed—atlantoaxial rotatory subluxation in six patients, anterior–posterior atlantoaxial instability caused by ligamentous injury or congenital ligamentous laxity (10 patients), atlantoaxial fracture with or without dislocation (five patients), and atlantooccipital dislocation (two patients). Most cases (60.9%) were treated without surgical intervention, resulting in excellent outcomes; however, 21.7% of cases were treated with a cervical halo (mean patient age 72.6 months) alone for 3 months. Various techniques of surgical stabilization including transarticular screws with sublaminar wiring, trans-oral decompression with posterior plating, and laminectomy with Steinmann pin occipital–cervical fusion were used with good results. Both patients with atlantooccipital dislocation underwent immediate Locksley occipital–cervical fusion, with marked neurological improvement. Individualized case management must be based on clinical presentation, with internal fixation being the last resort.

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John Vassilouthis

TO THE EDITOR: I follow with great interest the scientific debate 1 regarding the role of atlantoaxial instability in the pathogenesis of Arnold-Chiari malformation Type I (CM-I), described by Prof. Atul Goel 2 (Goel A: Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of patients treated by atlantoaxial fixation. J Neurosurg Spine 22: 116–127, February 2015). In his landmark description of the condition Prof. Goel uses the term “listhesis.” 3 The Greek word ολίσθησις, olisthesis (slippage), comes from the verb ολισθαίνω