Severe kyphoscoliosis secondary to neurofibromatosis. Case presentation

Keywords: Neurofibromatosis, scoliosis, resection, spine

Abstract

Dystrophic scoliosis in neurofibromatosis is identifiable by being an acute-angle kyphoscoliosis involving a short segment of the spine and producing severe deformity that associated with the dystrophic changes of the spine result in real surgical challenges.We report the clinical case of a 15-year male with severe dystrophic kyphoscoliosis at the thoracolumbar area, with apex at T9, scoliosis with a Cobb angle of 107 °, and segmental kyphosis of 110.7°. The patient underwent a three-stage surgery, performed through a posterior approach, involving a vertebral column resection (VCR) and titanium mesh replacement, and achieving a kyphosis correction of 56% and a scoliosis correction of 59.8%. The patient experienced no major complications nor sequelae and had a favorable course. The VCR is a powerful and demanding surgical technique that allows for the management of the complex kyphoscoliosis deformity to achieve spinal balance; however, it is not without complications, especially neurological and pulmonary complications, which may be unavoidable. Our patient’s quality of life has improved significantly. Key words: Neurofibromatosis; scoliosis; resection; spineLevel of Evidence: IV

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Author Biographies

Luis Enrique Nuñez Alvarado, Department of Orthopedics and Traumatology, Instituto Nacional de Salud del Niño - San Borja (Lima, Perú)
Department of Orthopedics and Traumatology, Instituto Nacional de Salud del Niño - San Borja (Lima, Perú)
Edgar Morales Vásquez, Department of Orthopedic and Trauma Surgery, Hospital Nacional Guillermo Almenara (Lima, Perú)
Department of Orthopedic and Trauma Surgery, Hospital Nacional Guillermo Almenara (Lima, Perú)
Raúl Macchiavello Falcon, Department of Orthopedic and Trauma Surgery, Hospital Nacional María Auxiliadora (Lima, Perú)
Department of Orthopedic and Trauma Surgery, Hospital Nacional María Auxiliadora (Lima, Perú)

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Published
2020-11-18
How to Cite
Nuñez Alvarado, L. E., Morales Vásquez, E., & Macchiavello Falcon, R. (2020). Severe kyphoscoliosis secondary to neurofibromatosis. Case presentation. Revista De La Asociación Argentina De Ortopedia Y Traumatología, 85(4), 393-402. https://doi.org/10.15417/issn.1852-7434.2020.85.4.1015
Section
Case Presentations