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Atlantoaxial rotatory subluxation/fixation and Grisel’s syndrome in children: clinical and radiological prognostic factors

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Abstract

Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children. The aim of our study is to investigate all possible clinical and radiological prognostic factors in children with AARS/F. We retrospectively reviewed all cervical spine CT scans of children with AARS/F treated in our Hospital over the last 15 years. AARS/F was classified according to Fielding and Hawkins classification and C1-C2 rotation-degree was calculated. Moreover, two orthopedic surgeons reviewed all clinical reports of these children. All patients were conservatively treated (cervical traction/neck collar). An early recovery was considered in patients with complete clinical/radiological healing at 3 months follow-up, while a late recovery was considered in patients with disease persistence or relapse at 3 months follow-up or earlier. Fifty-five patients with diagnosis of AARS/F were included in the study (mean age = 8.5 years old – 25F, 30M). In 9/55 subjects (16.4%), a late recovery was observed. The presence of a concomitant infection or inflammation in the head and neck region (Grisel’s syndrome) was significantly associated with a late recovery (p < .001). Also, the type of AARS/F (p = .019), according to the Fielding and Hawkins classification, and C1-C2 rotation-degree (p = .027) were significantly correlated with the recovery time.

Conclusion: In patients with AARS/F, the presence of a concomitant infection/inflammation in the head and neck region is the most important prognostic factor and it is associated with a late recovery. The Fielding and Hawkins classification and C1-C2 rotation-degree well correlate with patients’ recovery time.

What is Known:

• Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children and CT is the most useful imaging tool for diagnosis and classification of AARS/F.

• Conservative treatments are effective in the majority of pediatric patients with AARS/F.

What is New:

• The presence of a concomitant infection/inflammation in the head and neck region associated with AARS/F (Grisel’s syndrome) is the most important prognostic factor and it is associated with a late recovery.

• C1-C2 rotation-degrees, as well as Fielding and Hawkins classification system, well correlate with patients’ recovery time.

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Abbreviations

AARS/F:

Atlantoaxial rotatory subluxation/fixation

LL:

Ligamentous laxity

ADI:

Atlanto-dens interval

NSAIDs:

Nonsteroidal anti-inflammatory drugs

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Author information

Authors and Affiliations

  1. Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, Via GC Pupilli n 1, 40136, Bologna, Italy

    Paolo Spinnato, Sara Guerri, Massimo Barakat, Maria Carpenzano, Giulio Vara & Massimo Molinari

  2. Department of Pediatric Orthopaedics and Traumatology, IRCCS Istituto Ortopedico, Rizzoli, Bologna, Italy

    Paola Zarantonello

  3. Department of Experimental, Diagnostic and Specialty Medicine (DIMES), University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy

    Massimo Barakat & Giulio Vara

  4. IRCCS, Ospedale Pediatrico Bambino Gesù, Rome, Italy

    Alessandra Bartoloni

  5. Department of Oncologic and Degenerative Spine Surgery, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Alessandro Gasbarrini & Giuseppe Tedesco

Authors
  1. Paolo Spinnato
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  2. Paola Zarantonello
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  3. Sara Guerri
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  4. Massimo Barakat
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  5. Maria Carpenzano
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  6. Giulio Vara
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  7. Alessandra Bartoloni
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  8. Alessandro Gasbarrini
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  9. Massimo Molinari
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Contributions

PS wrote the paper, designed the research, performed image analyses (measures and classifications).

PZ evaluated all the clinical data of patients and helped in writing the manuscript.

SG and MC wrote the database and helped in clinical data analyses.

MB helped in writing the paper and made tables.

AG supervised the work and helped in research conceptualization.

MM and AB performed image analyses (measures and classifications).

GT evaluated all the clinical data of patients, helped in writing the manuscript, supervised the work.

Corresponding author

Correspondence to Paolo Spinnato.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article represents a retrospective observational research and does not contain any studies with human participants or animals performed by any of the authors.

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N/A

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Communicated by Piet Leroy

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Spinnato, P., Zarantonello, P., Guerri, S. et al. Atlantoaxial rotatory subluxation/fixation and Grisel’s syndrome in children: clinical and radiological prognostic factors. Eur J Pediatr 180, 441–447 (2021). https://doi.org/10.1007/s00431-020-03836-9

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  • DOI: https://doi.org/10.1007/s00431-020-03836-9

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