Management of congenital craniovertebral anomalies: a single-centre experience of 71 cases by an algorithmic approach

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ORIGINAL ARTICLE

Management of congenital craniovertebral anomalies: a single‑centre experience of 71 cases by an algorithmic approach Sudhir K. Srivastava1 · Aditya Raj1   · Sunil Bhosale1 · Shaligram Purohit1 · Nandan Marathe1 · Manojkumar Gaddikeri1 Received: 7 April 2020 / Revised: 1 June 2020 / Accepted: 24 June 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract Study design  Retrospective study. Objective  Congenital craniovertebral (CV) anomalies include a wide variety of conditions involving basilar invagination (BI), atlantoaxial dislocation (AAD), bony congenital anomalies and Chiari malformation. The management of these disorders is more surgeon dependent rather than based on clear guidelines. This study aims to provide a step-by-step algorithmic approach to the management of congenital CV anomalies to achieve good CV alignment, neural recovery and long lasting stability. Materials  The study retrospectively analyses 71 patients of congenital CV anomalies treated by our algorithmic approach. Clinical assessment was done with the help of Nurick’s grading pre- and post-operatively. Radiological outcome was assessed by plain radiographs, and computed tomographic scan was done at 12-month follow-up. Results  Mean age at presentation was 17.9 years (6–41 years). Mean Nurick score pre-operatively was 3.8 which was reduced to 1.3 at a mean follow-up of 13.6 months. Sixty-one patients had type I BI, 6 had type II BI, and 4 patients had os odontoideum. Most common symptom at presentation was weakness in limbs, neck pain and difficulty in walking. Patients of isolated AAD with os odontoideum required posterior atlantoaxial fixation and fusion only. Thirty-two patients of type I BI and 4 patients of type II BI required anterior release procedures prior to occipitocervical instrumentation. Bony fusion was achieved in 96% (68) cases with the help of autologous bone grafting. Three patients of occipitocervical fusion developed pseudoarthrosis. Conclusion  Our retrospective analysis demonstrates that the algorithm is effective in ensuring long lasting results in all types congenital CV anomalies. Keywords  Congenital craniovertebral anomalies · Algorithmic management · Basilar invagination · Atlantoaxial dislocation

* Aditya Raj [email protected] Sudhir K. Srivastava [email protected] Sunil Bhosale [email protected] Shaligram Purohit [email protected] Nandan Marathe [email protected] Manojkumar Gaddikeri [email protected] 1



Department of Orthopaedics, Seth G.S. Medical College and K.E.M. Hospital, 6th Floor MSB, Parel, Mumbai 400 012, India

Introduction Congenital CV anomalies are frequently encountered entities with varied diagnostic dilemmas and management controversies. These include anomalies in the bony and soft tissue components including the ligaments, joint capsules and neurovascular structures. There has been widely reported literature on the management of these anomalies, but there is no consistency in the management followed. The cranioverteb