Natural History, Neuroradiological Workup, and Management Options of Chronic Atlantoaxial Rotatory Fixation Caused by Drug-Induced Cervical Dystonia

Atlantoaxial rotatory fixation (AARF) resulting from drug-induced cervical dystonia (DICD) represents an extremely rare complication of antipsychotic treatment, requiring a comprehensive assessment of pharmacologic therapy and timely radiologic workup. We report a chronic case of Fielding type I, Pang type I AARF secondary to schizophrenia treatment in a 16-year-old girl, along with a review of the literature on the management challenges posed in this condition. In this scenario, torticollis may just represent the tip of the iceberg, and only an effective multidisciplinary approach increases the chances of satisfactory correction with closed reduction, hence avoiding the burden of more invasive treatment options.

Systematic review of the nonsurgical management of atlantoaxial rotatory fixation in childhood

OBJECTIVEAtlantoaxial rotatory fixation (AARF) is an acquired fixed abnormality of C1–2 joint rotation associated with torticollis in childhood. If the condition is left uncorrected, patients are at risk for developing C1–2 fusion with permanent limitation in the cervical range of movement, cosmetic deformity, and impact on quality of life. The management of AARF and the modality of nonsurgical treatment are poorly defined in both primary care and specialized care settings, and the optimal strategy is not clear. This systematic review aims to examine the available evidence to answer key questions relating to the nonsurgical management of AARF.METHODSA systematic review was performed using the following databases: PubMed, MEDLINE, Healthcare Management Information Consortium (HMIC), EMCare, Embase, Cumulative Index to Nursing and Allied Health Literature (CINAHL), British Nursing Index (BNI), and Allied and Complementary Medicine Database (AMED). Search criteria were created and checked independently among the authors. All articles with a radiological diagnosis of AARF and primary outcome data that met the study inclusion criteria were included and analyzed by the authors.RESULTSSearch results did not yield any level I evidence such as a meta-analysis or randomized controlled trial. The initial search yielded 724 articles, 228 of which were screened following application of the core exclusion criteria. A total of 37 studies met the full criteria for inclusion in this review, consisting of 4 prospective studies and 33 retrospective case reviews. No articles directly compared outcomes between modalities of nonsurgical management. Six studies compared the outcome of AARF based on duration of symptoms before initiation of treatment. Comparative analysis of studies was hindered by the wide variety of treatment modalities described and the heterogeneity of outcome data.CONCLUSIONSThe authors did not identify any level I evidence comparing different nonsurgical management approaches for AARF. There were few prospective studies, and most studies were uncontrolled, nonrandomized case series. Favorable outcomes were often reported regardless of treatment methods, with early treatment of AARF tending to yield better outcomes independent of the treatment modality. There is a lack of high-quality data, and further research is required to determine the optimal nonsurgical treatment strategy.

New Simple 3-Dimensional Computed Tomogram Classification Leading to Successful Conservative Treatment in 51 Atlanto-Axial Rotatory Fixation Children

<b><i>Objective:</i></b> to present a new and easy classification of atlanto-axial rotatory fixation (AARF) and to investigate the efficiency of conservative treatment of AARF. <b><i>Background:</i></b> Although there is a precise definition and diagnostic classification of AARF, there is still significant difficulty in measuring the atlas and axis angles because all of the atlas or axis cannot be seen in a certain 2-dimensional computed tomogram image. In addition, some recent case reports showed that long-term conservative treatment can reduce pediatric AARFs, even that are severe or chronic. <b><i>Methods:</i></b> Fifty-one children with AARF were analyzed retrospectively with new 3-dimensional computed tomogram (3DCT)-based AARF classification; the mean age was 72.7 ± 35.2 months (19–139 months). In the new AARF classification, type 1 was defined as that when the C1C2 angle is not 0° on midline and type 2 as that when the C1C2 angle is 0° on the midline. <b><i>Results:</i></b> All 7 children with AARF type 1 were treated successfully only with Halter tractions. Twenty among 44 children with type 2 did not show any difference in improvement compared with not-treated 24 children with type 2. <b><i>Conclusion:</i></b> The first new AARF classification based on 3DCT appears to be easy to use and even the most severe children with AARF may be managed only with conservative treatment such as long-term Halter traction.

Painful torticollis due to tubercular atlantoaxial rotatory fixation: A case report

Background: Tubercular atlantoaxial, rotary dislocation warranting fixation (AARF) is an extremely rare event. Case Description: AARF was suspected in a 23-year-old female with painful torticollis. When diagnostic studies documented unilateral destruction of the left lateral mass of the atlas, she underwent removal of the lateral mass, reduction of the deformity, and C1-C2 fusion/reconstruction utilizing an iliac bone graft. Laboratory tests and the pathologic surveys were all consistent with the diagnosis of underlying tuberculosis. Conclusion: We present a case of tubercular atlantoaxial, rotary dislocation (AARF) in a patient who warranted C1-C2 decompression, reduction, and fusion.

Primary treatment of atlantoaxial rotatory fixation in children: a multicenter, retrospective series of 125 cases

OBJECTIVEThe primary treatment for atlantoaxial rotatory fixation (AARF) remains controversial. The aim of this study was to investigate the primary treatment for AARF and create an algorithm for primary treatment.METHODSThe authors analyzed the data of 125 pediatric patients at four medical institutions from April 1989 to December 2018. The patients were reported to have neck pain, torticollis, and restricted neck range of motion and were diagnosed according to the Fielding classification as type I or II. As a primary treatment, 88 patients received neck collar fixation, and 28 of these patients did not show symptom relief and required Glisson traction. Thirty-seven patients were primarily treated with Glisson traction. In total, 65 patients, including neck collar treatment failure patients, underwent Glisson traction in hospitals.RESULTSThe success rate of treatment was significantly higher in the Glisson traction group (97.3%) than in the neck collar fixation group (68.2%) (p = 0.0001, Wilcoxon test). In the neck collar effective group, Fielding type I was more predominant (p = 0.0002, Wilcoxon test) and the duration from onset to the first visit was shorter (p = 0.02, Wilcoxon test) than that in the neck collar ineffective group. Using multivariate logistic regression analysis with the above items, the authors generalized from the estimated formula: logit [p(success group by neck collar fixation group)|duration from onset to the first visit (x1), Fielding type (x2)] = 0.4(intercept) − 0.15x1 + 1.06x2, where x1 is the number of days and x2 = 1 (for Fielding type I) or −1 (for Fielding type II). In cases for which the score is a positive value, the neck collar should be chosen. Conversely, in cases for which the score is a negative value, Glisson traction should be the first choice.CONCLUSIONSAccording to this formula, in patients with Fielding type I AARF, neck collar fixation should be allowed only if the duration from onset is ≤ 10 days. In patients with Fielding type II, because the score would be a negative value, Glisson traction should be performed as the primary treatment.

Atlantoaxial rotatory fixation in childhood: a staged management strategy incorporating manipulation under anaesthesia

Aims The aims were to evaluate the safety of manipulation under anaesthesia (MUA) for atlantoaxial rotatory fixation (AARF) and the relative efficacy of rigid collar vs halo-body orthosis (HBO) in avoiding relapse and the need for open surgery. Methods Cases of CT-verified AARF treated by MUA were identified from a neurosurgical operative database. Demographic details, time to presentation and aetiology of AARF were ascertained through case note review. Cases were divided according to method of immobilisation after successful reduction, either rigid collar (group 1) or HBO (group 2). The primary outcome measure was relapse requiring open surgical arthrodesis. Results Thirty-three patients (2.2–12.7 years) satisfied inclusion criteria. Time to presentation varied from 1 day to 18 months. There were 19 patients in group 1 and 14 in group 2. There were no adverse events associated with MUA. 9/19 (47%) patients in group 1 resolved without need for further treatment compared with 10/14 (71%) in group 2 (p = 0.15). Of the 10 patients who failed group 1 treatment, four resolved after HBO. A total of ten patients (30%) failed treatment and required open surgery. Conclusions MUA is a safe procedure for AARF where initial conservative measures have failed. MUA followed by immobilisation avoids the need for open surgery in over two thirds of cases. Immobilisation by cervical collar appears equally effective to HBO as an initial management, and so a step-wise approach may be reasonable. Delayed presentation may be a risk factor for relapse and need for open surgery.