Surgical outcome of scoliosis in Marfan syndrome: a case series report

https://doi.org/10.19106/JMedSci005002201814

komang agung irianto(1*), Gestana R Wardana(2)

(1) Department of Orthopaedic and Traumatology, Airlangga University / Dr. Soetomo General Hospital Surabaya
(2) Department of Orthopaedic and Traumatology, Airlangga University / Dr. Soetomo General Hospital Surabaya
(*) Corresponding Author

Abstract


Scoliosis in marfan syndrome (MFS) manifests on 60% patients. Moreover, the scoliosis
noticeable in earlier age is more progressive, refracted, and rigid compared to idiopathic
adult scoliosis. The surgical correction provides notorious higher perioperative risk,
whereas conservative treatment using brace is not effective to prevent progressivity
of the scoliosis. In this a case report, we reported the surgical outcome of MFS scoliais
patients with MFS who operated using posterior fusion instrumentation by mean of the
quality of life SF-36 questioner. This was a retrospective case series involving five MFS
scoliosis patients who underwent posterior fusion instrumentation with initial Cobb angle
of 87.417.57o and initial kyphotic angle of 32.8 ± 14.52o. Clinical, radiological and
quality of life of the patients based on SF-36 questionnaire were evaluated within 6-36
months follow up. Post-operative showed the Cobb angle become 46.2 ± 16.3o and the
kyphotic angle become 21.6 ± 9.94o. No intraoperative or post-operative complications
were observed. After 6-36 months follow up, the Cobb angle became 45.2 ± 17.48o
and the kyphotic angle became 21.6 ± 9.94o. In addition, all patients had physical and
mental health scored similar to 2 years post-surgery scoliosis scoring according to SF-
36 orthopedic scoring guidelines. I conclusion, the surgical outcome of posterior fusion
instrumentation in MFS scoliosis showed good correction of Cobb angle and Kyphotic
angle. The quality of life of the patients based on physical and mental health questionnaire
is satisfactory.

Keywords


scoliosis - Marfan syndrome – quality of life - questionnaire SF-36 – Cobb angle

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DOI: https://doi.org/10.19106/JMedSci005002201814

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Journal of the Medical Sciences (Berkala Ilmu Kedokteran) by  Universitas Gadjah Mada is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
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