Poliostotic fibrous dysplasia with affectation of cervical rachis.
The fibrous dysplasia is a benign although progressive dysfunction, in which a gene mutation originates the production of fibrous disorganized bony matrix. The bony tissue is replaced by bony tissue in expansion (amorph conjuntival tissue) that produces bony deforrnities in sorne patients, pain, pat...
Guardado en:
| Autores principales: | , , , , , |
|---|---|
| Formato: | Artículo revista |
| Lenguaje: | Español |
| Publicado: |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2019
|
| Materias: | |
| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/26070 |
| Aporte de: |
| id |
I10-R10-article-26070 |
|---|---|
| record_format |
ojs |
| institution |
Universidad Nacional de Córdoba |
| institution_str |
I-10 |
| repository_str |
R-10 |
| container_title_str |
Revistas de la UNC |
| language |
Español |
| format |
Artículo revista |
| topic |
pibrous dysplasia polyostotic cervical rachis diagnosis displasia fibrosa poliostótica raquis cervical diagnóstico |
| spellingShingle |
pibrous dysplasia polyostotic cervical rachis diagnosis displasia fibrosa poliostótica raquis cervical diagnóstico Lumbreras, Ruth Aznar, José María Castro, Ángel Modrego, Francisco Jaiver Ballester, Juan José Espallargas, Teresa Poliostotic fibrous dysplasia with affectation of cervical rachis. |
| topic_facet |
pibrous dysplasia polyostotic cervical rachis diagnosis displasia fibrosa poliostótica raquis cervical diagnóstico |
| author |
Lumbreras, Ruth Aznar, José María Castro, Ángel Modrego, Francisco Jaiver Ballester, Juan José Espallargas, Teresa |
| author_facet |
Lumbreras, Ruth Aznar, José María Castro, Ángel Modrego, Francisco Jaiver Ballester, Juan José Espallargas, Teresa |
| author_sort |
Lumbreras, Ruth |
| title |
Poliostotic fibrous dysplasia with affectation of cervical rachis. |
| title_short |
Poliostotic fibrous dysplasia with affectation of cervical rachis. |
| title_full |
Poliostotic fibrous dysplasia with affectation of cervical rachis. |
| title_fullStr |
Poliostotic fibrous dysplasia with affectation of cervical rachis. |
| title_full_unstemmed |
Poliostotic fibrous dysplasia with affectation of cervical rachis. |
| title_sort |
poliostotic fibrous dysplasia with affectation of cervical rachis. |
| description |
The fibrous dysplasia is a benign although progressive dysfunction, in which a gene mutation originates the production of fibrous disorganized bony matrix. The bony tissue is replaced by bony tissue in expansion (amorph conjuntival tissue) that produces bony deforrnities in sorne patients, pain, pathological fractures or deambulation disorders. The diagnosis is important since ocasionally the first symptom is the fracture. We show up the case of a 21 year -oid patient with pain clinic in high cervical region. The complementary tests (radiology, bone scintigrraphy and MRI) and anatomo-pathology confirmed the diagnosis of polyostotic fibrous dysplasia with cranial (occipital esfenoides and right frontal and temporal bone), iiiac, femoral, tibial and cervical (apophysis of C2) affectation. Our attitude was of canying out a narrow observation by means of periodical strict controls, advising to avoid hard activities or contact sports. To the five years the patient is free of symptomatology. Radiologicaily the injuries have been stabilized. The fibrous dysplasia can affect to a single bone (monostotic) or to several (polyostotic). In occasions it is associated to endocrine dysfunctions and skin pigmentations in McCune- Albright's syndrome (7). We confront a pathology that specifies an anatomo-pathologic diagnosis to be confirmeci, an extension diagnosis to detect asymptomatic focuses and whose treatment is symptomatic in most of the cases only using surgery in frank deformities or when the fracture risk is considerable, although the recurrence is frequent. The malignization is exceptional but possible that's why continuous observation is needed. The radiation therapy is radically contraindicated (6). |
| publisher |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2019 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/26070 |
| work_keys_str_mv |
AT lumbrerasruth poliostoticfibrousdysplasiawithaffectationofcervicalrachis AT aznarjosemaria poliostoticfibrousdysplasiawithaffectationofcervicalrachis AT castroangel poliostoticfibrousdysplasiawithaffectationofcervicalrachis AT modregofranciscojaiver poliostoticfibrousdysplasiawithaffectationofcervicalrachis AT ballesterjuanjose poliostoticfibrousdysplasiawithaffectationofcervicalrachis AT espallargasteresa poliostoticfibrousdysplasiawithaffectationofcervicalrachis AT lumbrerasruth displasiafibrosapoliostoticaconafectacionderaquiscervical AT aznarjosemaria displasiafibrosapoliostoticaconafectacionderaquiscervical AT castroangel displasiafibrosapoliostoticaconafectacionderaquiscervical AT modregofranciscojaiver displasiafibrosapoliostoticaconafectacionderaquiscervical AT ballesterjuanjose displasiafibrosapoliostoticaconafectacionderaquiscervical AT espallargasteresa displasiafibrosapoliostoticaconafectacionderaquiscervical |
| first_indexed |
2022-08-20T01:27:01Z |
| last_indexed |
2022-08-20T01:27:01Z |
| _version_ |
1770719038018682880 |
| spelling |
I10-R10-article-260702019-11-06T21:07:07Z Poliostotic fibrous dysplasia with affectation of cervical rachis. Displasia fibrosa poliostótica con afectación de raquis cervical. Lumbreras, Ruth Aznar, José María Castro, Ángel Modrego, Francisco Jaiver Ballester, Juan José Espallargas, Teresa pibrous dysplasia polyostotic cervical rachis diagnosis displasia fibrosa poliostótica raquis cervical diagnóstico The fibrous dysplasia is a benign although progressive dysfunction, in which a gene mutation originates the production of fibrous disorganized bony matrix. The bony tissue is replaced by bony tissue in expansion (amorph conjuntival tissue) that produces bony deforrnities in sorne patients, pain, pathological fractures or deambulation disorders. The diagnosis is important since ocasionally the first symptom is the fracture. We show up the case of a 21 year -oid patient with pain clinic in high cervical region. The complementary tests (radiology, bone scintigrraphy and MRI) and anatomo-pathology confirmed the diagnosis of polyostotic fibrous dysplasia with cranial (occipital esfenoides and right frontal and temporal bone), iiiac, femoral, tibial and cervical (apophysis of C2) affectation. Our attitude was of canying out a narrow observation by means of periodical strict controls, advising to avoid hard activities or contact sports. To the five years the patient is free of symptomatology. Radiologicaily the injuries have been stabilized. The fibrous dysplasia can affect to a single bone (monostotic) or to several (polyostotic). In occasions it is associated to endocrine dysfunctions and skin pigmentations in McCune- Albright's syndrome (7). We confront a pathology that specifies an anatomo-pathologic diagnosis to be confirmeci, an extension diagnosis to detect asymptomatic focuses and whose treatment is symptomatic in most of the cases only using surgery in frank deformities or when the fracture risk is considerable, although the recurrence is frequent. The malignization is exceptional but possible that's why continuous observation is needed. The radiation therapy is radically contraindicated (6). La displasia fibrosa es un trastorno benigno aunque progresivo, en el que una mutación genética origina la producción de matriz ósea fibrosa desorganizada. El tejido óseo se reemplaza por tejido óseo en expansión (tejido conjuntivo amorfo) que en algunos pacientes produce deformidades óseas, dolor, fracturas patológicas o problemas con la deambulación. Su diagnóstico es importante ya que ocasionalmente el primer síntoma es la fractura. (1-3) Presentarnos el caso de un paciente de 21 años con clínica de dolor en región cervical alta. Las pruebas complementarias (radiología gammagrafia y RNM) y la anatomía patológica confirmaron el diagnóstico de displasia fibrosa poliostótica con afectación craneal (occipital, esfenoides y fronto-temporal deréchas),iliaca, femoral, tibial y cervical (apófisis espinosa de C2). Nuestra actitud fue de realizar una observación estrecha mediante controles periódicos estrictos, aconsejando evitar actividades o deportes de contacto. A los cinco años el paciente está libre de sintomatología. Radiológicamente las lesiones se han estabilizado. La displasia fibrosa puede afectar a un solo hueso (monostótica) o a varios (poliostótica). En ocasiones se asocia a trastornos endocrinos y igmentaciones cutáneas en el denominado síndrome de McCune-Albright. (7) Nos encontramos ante una patología que precisa un diagnóstico anatomopatológico para confirmarla, de extensión para detectar focos asintomáticos y cuyo tratamiento es sintomático en la mayoría de los casos recurriendo a la cirugía sólo en ocasión de deformidades francas o cuando el riesgo de fractura es considerable, ya que las recidivas con frecuentes. La malignización es excepcional pero posible por lo que necesita observación continuada. La radioterapia está radicalmente contraindicada (6). Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2019-10-29 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/26070 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 64 No. 2 (2007); 48 - 52 Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 64 Núm. 2 (2007); 48 - 52 Revista da Faculdade de Ciências Médicas de Córdoba; v. 64 n. 2 (2007); 48 - 52 1853-0605 0014-6722 spa https://revistas.unc.edu.ar/index.php/med/article/view/26070/27919 Derechos de autor 2019 Universidad Nacional de Córdoba |