Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report
Introduction: Neurofibromatosis (NF) is a group of multisystem diseases with an autosomal dominant inheritance pattern. It affects the neural crest cells, being able to compromise all tissues. NF type 1 is the most prevalent, also known as the peripheral type. This includes multiple musculoskeletal...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-12-01
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| Series: | Interdisciplinary Neurosurgery |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2214751923000890 |
| _version_ | 1797796124498591744 |
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| author | Silvia Suárez-Monsalve Julian Alfonso Sierra-Peña Alejandro Durán-Ojeda Jaime Eduardo Becerra-Ospina Juan Carlos Pérez-Rodríguez |
| author_facet | Silvia Suárez-Monsalve Julian Alfonso Sierra-Peña Alejandro Durán-Ojeda Jaime Eduardo Becerra-Ospina Juan Carlos Pérez-Rodríguez |
| author_sort | Silvia Suárez-Monsalve |
| collection | DOAJ |
| description | Introduction: Neurofibromatosis (NF) is a group of multisystem diseases with an autosomal dominant inheritance pattern. It affects the neural crest cells, being able to compromise all tissues. NF type 1 is the most prevalent, also known as the peripheral type. This includes multiple musculoskeletal manifestations, such as severe and difficult-to-manage scoliosis. Including two types: dystrophic and non-dystrophic. Case report: 40-year-old man with a history of NF1, grave spine deformity, extreme back pain, and severe functional limitation. The initial diagnostic images of the spine showed a thoracolumbar kyphoscoliosis over 80 degrees which collapsed the thoracolumbar junction and generated spinal cord compression. He was managed with a Vertebral Column Resection (VCR) at the thoracolumbar junction level, thoracoplasty, and posterior thoracic to lumbosacral arthrodesis. The patient required hospitalization and long rehabilitation therapy. Currently, he has good clinical and diagnostic imaging results after one year of following. Discussion: This patient's case corresponds to dystrophic kyphoscoliosis. This group of patients requires more aggressive surgical management, considering the high risk of progression and deterioration even after surgery. |
| first_indexed | 2024-03-13T03:28:22Z |
| format | Article |
| id | doaj.art-69ce7e2c0b0644388fe4d3344add929a |
| institution | Directory Open Access Journal |
| issn | 2214-7519 |
| language | English |
| last_indexed | 2024-03-13T03:28:22Z |
| publishDate | 2023-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Interdisciplinary Neurosurgery |
| spelling | doaj.art-69ce7e2c0b0644388fe4d3344add929a2023-06-25T04:42:59ZengElsevierInterdisciplinary Neurosurgery2214-75192023-12-0134101806Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE reportSilvia Suárez-Monsalve0Julian Alfonso Sierra-Peña1Alejandro Durán-Ojeda2Jaime Eduardo Becerra-Ospina3Juan Carlos Pérez-Rodríguez4Neurosurgery Research Group, Pontificia Universidad Javeriana Bogotá, Colombia; Neurosurgery Research Group, NeuroAxis S.A.S., Bogotá, Colombia; Clinical Epidemiology Masters Degree, Pontificia Universidad Javeriana Bogotá, Colombia; Corresponding author at: Department of Clinical Epidemiology and Biostatics, Pontificia Universidad Javeriana Bogotá. Cra. 7 No. 40-62 Hospital Universitario San Ignacio Piso 2 - Bogotá, Colombia.Neurosurgery Research Group, Pontificia Universidad Javeriana Bogotá, ColombiaNeurosurgery Research Group, NeuroAxis S.A.S., Bogotá, Colombia; Clinical Epidemiology Specialization, Universidad de los Andes, Bogotá, ColombiaNeurosurgery Research Group, NeuroAxis S.A.S., Bogotá, Colombia; Neurosurgery specialist, Pontificia Universidad Javeriana, Bogotá, ColombiaNeurosurgery Research Group, NeuroAxis S.A.S., Bogotá, Colombia; Neurosurgery specialist, Pontificia Universidad Javeriana, Bogotá, Colombia; Spinal deformity surgery, Instituto Nacional de Traumatologia e Ortopedia Jamil Haddad, Rio de Janeiro, BrazilIntroduction: Neurofibromatosis (NF) is a group of multisystem diseases with an autosomal dominant inheritance pattern. It affects the neural crest cells, being able to compromise all tissues. NF type 1 is the most prevalent, also known as the peripheral type. This includes multiple musculoskeletal manifestations, such as severe and difficult-to-manage scoliosis. Including two types: dystrophic and non-dystrophic. Case report: 40-year-old man with a history of NF1, grave spine deformity, extreme back pain, and severe functional limitation. The initial diagnostic images of the spine showed a thoracolumbar kyphoscoliosis over 80 degrees which collapsed the thoracolumbar junction and generated spinal cord compression. He was managed with a Vertebral Column Resection (VCR) at the thoracolumbar junction level, thoracoplasty, and posterior thoracic to lumbosacral arthrodesis. The patient required hospitalization and long rehabilitation therapy. Currently, he has good clinical and diagnostic imaging results after one year of following. Discussion: This patient's case corresponds to dystrophic kyphoscoliosis. This group of patients requires more aggressive surgical management, considering the high risk of progression and deterioration even after surgery.http://www.sciencedirect.com/science/article/pii/S2214751923000890KyphosisScoliosisNeurofibromatosis 1Case reports |
| spellingShingle | Silvia Suárez-Monsalve Julian Alfonso Sierra-Peña Alejandro Durán-Ojeda Jaime Eduardo Becerra-Ospina Juan Carlos Pérez-Rodríguez Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report Interdisciplinary Neurosurgery Kyphosis Scoliosis Neurofibromatosis 1 Case reports |
| title | Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report |
| title_full | Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report |
| title_fullStr | Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report |
| title_full_unstemmed | Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report |
| title_short | Management of a very severe Dystrophic Kyphoscoliosis on a patient with Type 1 Neurofibromatosis - CASE report |
| title_sort | management of a very severe dystrophic kyphoscoliosis on a patient with type 1 neurofibromatosis case report |
| topic | Kyphosis Scoliosis Neurofibromatosis 1 Case reports |
| url | http://www.sciencedirect.com/science/article/pii/S2214751923000890 |
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