Abstract
Background
Spinal deformities are common in Marfan syndrome (MFS). They usually involve the thoraco-lumbar spine but rarely involves the cervical spine. Kyphosis is the common spine deformity of the cervical spine and mandates surgical correction as they are at risk of neurological deterioration since they are refractory to conservative management. Few studies of surgical correction of spine deformity included cervical deformity.
Objectives
To analyze the challenges faced during surgery, clinical and radiological outcome, and complications following surgical correction for cervical kyphosis in Marfan syndrome.
Methods
We identified that 5 patients with a diagnosis of MFS with cervical kyphosis who underwent fusion surgery between the years 2010 and 2022 were reviewed, retrospectively. We analyzed the demographic details, radiological parameters, operative variables (blood loss and nuances), perioperative complications, length of stay, clinical and radiological outcome, and complications following fusion surgery for cervical kyphosis in MFS.
Results
The mean age of patients was 16.6 ± 4.72 years (range, 12–23 years). The average kyphotic vertebra involved is 3 ± 0.7 bodies (range 2–4) with 2 patients with thoracic deformity. All patients underwent surgical deformity correction. All patients improved clinically with Nurick grade (pre vs. post: 3.4 vs. 2.2) and mJOA (pre vs. post: 8.2 vs. 12.6). There was significant deformity correction from 37.48° to 9.1°. Mean blood loss encountered was 900 ± 173.2 ml. Perioperative complications: wound complication with CSF leak (1). Late complications: ventilator dependence (1) and junctional kyphosis (1). Mean length of hospital stay was 103 ± 178.9 days. All patients were doing symptomatically better after mean follow-up of 58 ± 28.32 months. One patient is bedridden and hospitalized.
Conclusion
Cervical kyphosis is a rare spine deformity in patients with MFS, and they usually present with neurological deterioration mandating surgical correction. Multidisciplinary approach (pediatrics, genetics and cardiology) is required for systematic evaluation of these patients. They should be evaluated with necessary imaging to rule out associated spinal deformity (atlanto-axial subluxation, scoliosis, and intraspinal pathology like ductal ectasia). Our results suggest better surgical outcome in terms of low operative complications with neurologic improvement in MFS patients. These patients require regular follow-up to identify late complications (instrument failure, non-union, and pseudarthrosis).
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Datasets and imaging of patients are available.
References
Yang JS, Sponseller PD (2009) Severe cervical kyphosis complicating halo traction in a patient with Marfan syndrome. Spine 34(1):E66–E69
Marfun M (1896) Un cas de deformation congenitale des quatre membres, plus prononcee aux extremites, caracterisee par l’allongement des os avec un certain degre d’amincissement. Bull Mem Soc Med Hop paris 13:220–226
Hobbs WR, Sponseller PD, Weiss A-PC, Pyeritz RE (1997) The cervical spine in Marfan syndrome. Spine 22(9):983–989
Kurucan E, Bernstein DN, Ying M et al (2019) Trends in spinal deformity surgery in Marfan syndrome. Spine J 19(12):1934–1940
Dormans JP, Drummond DS, Sutton LN, Ecker ML, Kopacz KJ (1995) Occipitocervical arthrodesis in children. A new technique and analysis of results. JBJS 77(8):1234–1240
Dickman CA, Sonntag VK, Papadopoulos SM, Hadley MN (1991) The interspinous method of posterior atlantoaxial arthrodesis. J Neurosurg 74(2):190–198
Farey ID, Nadkarni S, Smith N (1999) Modified Gallie technique versus transarticular screw fixation in C1-C2 fusion. Clin Orthopaed Related Res (1976–2007) 359:126–135
Ahmed R, Traynelis VC, Menezes AH (2008) Fusions at the craniovertebral junction. Child’s Nerv Syst 24:1209–1224
McKay SD, Al-Omari A, Tomlinson LA, Dormans JP (2012) Review of cervical spine anomalies in genetic syndromes. Spine 37(5):E269–E277
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Contributions
Sivaraman Kumarasamy—conception and design of work, acquisition, analysis, interpretation of data, and drafted the work. Dattaraj P. Sawarkar—conception and design of work, acquisition, analysis, interpretation of data, and drafted the work. Pankaj Kumar Singh—analysis, interpretation of data, and revised the work. Rajinder Kumar—interpretation of data and revised the work. Poodipedi Sarat Chandra—interpretation of data and revised the work.
Shashank Sharad Kale—interpretation of data and revised the work.
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Kumarasamy, S., Sawarkar, D.P., Singh, P.K. et al. Cervical kyphosis correction in Marfan syndrome: our experience and literature review. Childs Nerv Syst 40, 495–502 (2024). https://doi.org/10.1007/s00381-023-06046-4
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DOI: https://doi.org/10.1007/s00381-023-06046-4