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1.
World J Surg Oncol ; 17(1): 186, 2019 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-31706319

RESUMO

BACKGROUND: Monostotic fibrous dysplasia (MFD) involving the spine is rare, and the treatment options are controversial. Surgery is needed when patients suffer from persistent pain, spinal cord compression/injury, and vertebral collapse/instability. Treatment methods include biopsy/observation, corpectomy with instrumented fusion, posterior fusion, vertebroplasty (VP), curettage and bone graft, and complete removal of the vertebra with a combined anterior and posterior fusion procedure. CASE PRESENTATION: The patient was a 56-year-old woman with a 2-year history of neck pain. No obvious abnormalities were detected on neurological or physical examination, and laboratory findings were all within normal limits. An imaging examination suggested a C7 vertebral bone tumor. The patient refused to continue conservative observation treatment and requested surgery. Open VP of the C7 vertebral body was carried out, and her postoperative neck pain was completely relieved. The postoperative pathological results supported the diagnosis of fibrous dysplasia, and the patient was ultimately diagnosed with MFD. At the 12-month follow-up visit, the patient reported no clinical symptoms, and no signs of tumor recurrence were detected. CONCLUSION: VP can relieve pain while stabilizing the spine. Thus, the surgical treatment of MFD vertebral lesions by VP is a valuable option.


Assuntos
Vértebras Cervicais/patologia , Displasia Fibrosa Monostótica/cirurgia , Cervicalgia/cirurgia , Vertebroplastia , Biópsia , Cimentos para Ossos , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Feminino , Displasia Fibrosa Monostótica/complicações , Displasia Fibrosa Monostótica/patologia , Humanos , Pessoa de Meia-Idade , Cervicalgia/etiologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
2.
Autops. Case Rep ; 9(2): e2018092, Abr.-Jun. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-999552

RESUMO

Fibrous dysplasia (FD) is a relatively rare osseous disease of unknown etiology, wherein the normal bone is replaced by collagen-rich tissue, comprising of fibroblasts and variably abundant immature woven bone. Clinically, it may involve a single bone or multiple bones. It commonly arises in the jaw bone, skull, rib, and proximal femur. Those arising in the skull and the jaw are together termed "craniofacial fibrous dysplasia." The differential diagnosis at this location includes meningioma and metastatic carcinoma. In this report, we highlight two diagnostically challenging cases presenting with orbital swelling and headache as the main complaints. Our first case was misinterpreted as meningioma on intraoperative squash smear, and paraffin sections revealed characteristic features of FD. The second case highlights the morphological feature of non-specific cystic degeneration occurring in FD. Radiographs in such cases show cystic swelling, which is indicative of a secondary aneurysmal bone cyst.


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Cistos Ósseos Aneurismáticos/patologia , Displasia Fibrosa Monostótica/patologia , Neoplasias Meníngeas , Meningioma/patologia , Diagnóstico Diferencial
3.
Pediatr Radiol ; 49(2): 187-195, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30443667

RESUMO

BACKGROUND: Focal fibrocartilaginous dysplasia is a rare growth disturbance of bone resulting in deformity. In the ulna, focal fibrocartilaginous dysplasia is particularly rare, and the characteristic fibroligamentous tether can result in progressive deformity with progressive dislocation of the radial head. The fibroligamentous tether is similar in appearance and function to the Vickers ligament seen in Madelung deformity. The imaging features of ulnar focal fibrocartilaginous dysplasia include a unilateral angular deformity in the ulna with a radiolucent cortical defect and surrounding sclerosis, with secondary radial bowing or radial head dislocation. Focal fibrocartilaginous dysplasia of the ulna has been described using the term ulnar focal cortical indentation. OBJECTIVE: To review the clinical and radiologic features of four cases of ulnar focal cortical indentation occurring in pediatric patients. MATERIALS AND METHODS: We retrospectively reviewed picture archiving and communication system and electronic medical record imaging findings with surgical correlation of ulnar focal cortical indentation at two large specialty pediatric hospitals. RESULTS: Ulnar focal cortical indentation lesions typically arise in the ulnar metaphysis and result in angular forearm deformities with progressive radial deformity including radial head dislocation. Early surgical intervention prevents progression of the deformity and retains range of motion. CONCLUSION: Ulnar focal cortical indentation, although rare, is likely a progressive form of focal fibrocartilaginous dysplasia resulting in significant deformity and disability. Early recognition of the characteristic imaging features is important for early surgical intervention to preserve range of motion and prevent radial head dislocation.


Assuntos
Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Ulna/diagnóstico por imagem , Ulna/patologia , Pré-Escolar , Progressão da Doença , Feminino , Displasia Fibrosa Monostótica/cirurgia , Humanos , Lactente , Fixadores Internos , Masculino , Osteotomia , Estudos Retrospectivos , Ulna/cirurgia
4.
World J Surg Oncol ; 15(1): 1, 2017 Jan 05.
Artigo em Inglês | MEDLINE | ID: mdl-28057011

RESUMO

BACKGROUND: Reports showing high recurrence rates for intralesional curettage and bone grafting have made the current treatment principle for fibrous dysplasia controversial. This study aimed to report the postoperative clinical outcomes from three minimally invasive surgical strategies we use for monostotic fibrous dysplasia (MFD). PATIENTS AND METHODS: Twelve patients with MFD presenting with no pathologic fracture or deformity and treated with one of three surgical strategies-plain open biopsy, plain alpha-tricalcium phosphate (ATP) reconstruction, and prophylactic bridge plating-were included. There were nine men and three women, with median age of 38 years. Mean follow-up was 88 weeks. Five cases involved the proximal femur, two each involved the femoral and tibial diaphyses, and one each involved the distal humerus, radial diaphysis, and proximal tibia. All cases were reviewed for functional and radiological outcomes. RESULTS: Median time to full activity was 1 day (range 1 to 3) for the plain open biopsy group, while the prophylactic bridge-plating and plain ATP reconstruction groups had longer median recovery times (59 days, range 3 to 143, and 52 days, range 11 to 192, respectively). Musculoskeletal Tumor Society scores at last follow-up were excellent for all the cases (mean 29.6, range 25 to 30). Radiological analysis using Gaski et al.'s criteria showed plain open biopsy resulted in partial resolution of proximal femoral lesions, while ATP reconstruction and prophylactic plating resulted in no change and progression in this lesion site, respectively. For femoral diaphyseal lesions, prophylactic plating resulted in partial resolution, while ATP reconstruction resulted in no change. In the tibial diaphysis, prophylactic plating resulted in partial resolution, while plain open biopsy resulted in no change. For the lesions involving the distal humerus and the proximal tibia, plain open biopsy resulted in partial resolution, while for the radial diaphyseal lesion, ATP reconstruction resulted in no change. Radiological progression was limited in 11 (92%) cases, and none had postoperative complications. CONCLUSION: Plain open biopsies for asymptomatic lesions; prophylactic bridge plating for symptomatic, large diaphyseal lytic lesions; and plain ATP reconstructions for both small and large nondiaphyseal symptomatic lytic lesions may be acceptable alternatives to curettage-incorporating procedures for MFD.


Assuntos
Displasia Fibrosa Monostótica/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Adolescente , Adulto , Idoso , Transplante Ósseo , Criança , Feminino , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Radiografia , Estudos Retrospectivos , Adulto Jovem
5.
Foot Ankle Spec ; 10(1): 72-74, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27325625

RESUMO

Fibrous dysplasia in the calcaneus is extremely rare; the most common locations of monostotic fibrous dysplasia of extremity bones being the proximal femoral shaft, tibia, humerus, and radius. An extensive literature search has revealed only 3 case reports that have been published concerning this topic, the most recent published in 2003. This is a case report of fibrous dysplasia in the calcaneus of a young male, including a discussion of the classical presentation of fibrous dysplasia and how this case differed. LEVELS OF EVIDENCE: Therapeutic, Level IV: Case study.


Assuntos
Calcâneo/diagnóstico por imagem , Displasia Fibrosa Monostótica/diagnóstico por imagem , Adulto , Edema/etiologia , Displasia Fibrosa Monostótica/patologia , Humanos , Masculino , Dor/etiologia
7.
Acta Otorrinolaringol Esp ; 67(5): 288-92, 2016.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-26589487

RESUMO

Frontal swelling can be due to multiple etiologies, including: mucocele, Pott's puffy tumor, fibro osseous lesions, benign and malignant neoplasms of the nose and paranasal sinuses, intracranial lesions, and metastasis. The objective of this study was to describe the clinical protocol used for the diagnosis of patients presented with frontal swelling and the proposal for staging of inflammatory lesions. We performed an observational retrospective analysis. We found 7 cases of patients with frontal swelling: 4 cases secondary to inflammatory pathology (3 Potts puffy tumors and one frontal mucocele), and 3 cases secondary to neoplasms (one benign and 2 malignant neoplasms). It's very important to consider the wide differential diagnosis that can present as frontal swelling, from inflammatory pathologies secondary to possible advanced infections of the paranasal sinuses to invasive malignant neoplasms. We propose a system of staging of frontal inflammatory lesions.


Assuntos
Edema/etiologia , Testa , Osso Frontal/patologia , Mucocele/patologia , Neoplasias dos Seios Paranasais/patologia , Tumor de Pott/patologia , Algoritmos , Carcinoma Papilar/complicações , Carcinoma Papilar/diagnóstico por imagem , Carcinoma Papilar/patologia , Carcinoma Papilar/secundário , Celulite (Flegmão)/diagnóstico por imagem , Celulite (Flegmão)/etiologia , Estudos Transversais , Diagnóstico Diferencial , Edema/diagnóstico por imagem , Displasia Fibrosa Monostótica/complicações , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Testa/diagnóstico por imagem , Osso Frontal/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Mucocele/complicações , Mucocele/diagnóstico por imagem , Neoplasias Nasais/complicações , Neoplasias Nasais/diagnóstico por imagem , Neoplasias Nasais/patologia , Papiloma Invertido/complicações , Papiloma Invertido/diagnóstico por imagem , Papiloma Invertido/patologia , Neoplasias dos Seios Paranasais/complicações , Neoplasias dos Seios Paranasais/diagnóstico por imagem , Tumor de Pott/complicações , Tumor de Pott/diagnóstico por imagem , Estudos Retrospectivos , Sinusite/complicações , Sinusite/diagnóstico por imagem , Neoplasias Cranianas/complicações , Neoplasias Cranianas/diagnóstico por imagem , Neoplasias Cranianas/patologia , Neoplasias da Glândula Tireoide/patologia , Tomografia Computadorizada por Raios X
9.
Medicine (Baltimore) ; 94(3): e369, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25621678

RESUMO

Malignant transformation in fibrous dysplasia (FD) is uncommon. The purpose of this study was to investigate clinical and imaging features, and outcomes of malignant transformation in monostotic FD.Data for 10 pathologically confirmed malignant transformations in monostotic FD from January 2005 to December 2013 were retrospectively reviewed. Patient data were recorded, and radiographs (n = 10), computed tomography (CT) (n = 5), magnetic resonance (MR) (n = 4), and bone scintigrams (n = 10) were evaluated for lesion location, margin, cortical destruction, marrow involvement, periosteal reaction, and soft tissue mass by 2 musculoskeletal radiologists with agreement by consensus. Clinical features, management, and prognosis were also analyzed for each of the 10 cases.There were 8 male and 2 female patients (mean age 46.5 ±â€Š15.9 years). The affected sites were the femur (n = 4), humerus (n = 2), tibia (n = 3), and ilium (n = 1). Five cases had received previous surgery and 5 cases had no history of surgery. No patients had been given prior irradiation treatment. For the 5 cases with surgery, radiographs and CT showed purely osteolytic lesions with poor margination in the curettage area (n = 5), cortical destruction (n = 5), obvious soft tissue mass (n = 1), and mineralization (n = 2). For the 5 cases without surgery, radiographs and CT identified poorly marginated, osteolytic lesions within or near the area with "ground-glass" opacity (n = 4), cortical erosion (n = 4), and mineralization (n = 2). Magnetic resonance imaging (MRI) also identified lesions with heterogeneous signal intensity and pronounced enhancement. Bone scintigraphy revealed eccentric increased uptake of radionuclide in monostotic lesion (n = 10). Pathology reports revealed osteosarcoma (n = 7), fibrosarcoma (n = 2), and malignant fibrous histiocytoma (MFH) (n = 1). At the end of the study, 1 patient died from tumors, 1 patient was alive with lung metastasis, 1 patient experienced recurrence, and 7 patients were alive without recurrence.Patients with FD and a history of surgery should be followed up, for the osteolytic lesions in the operative areas strongly indicate the malignant transformation. The radiographic feature of FD-related malignancies is poorly marginated, mineralized, and osteolytic lesions with cortical destruction. Further investigations are needed to explore the pathogenesis of malignancies in FD and to establish optimal therapeutic strategies.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Transformação Celular Neoplásica/patologia , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Imagem por Ressonância Magnética , Tomografia Computadorizada por Raios X , Adolescente , Adulto , Idoso , Neoplasias Ósseas/terapia , Terapia Combinada , Feminino , Fêmur/diagnóstico por imagem , Fêmur/patologia , Fibrossarcoma/diagnóstico por imagem , Fibrossarcoma/patologia , Fibrossarcoma/terapia , Displasia Fibrosa Monostótica/diagnóstico , Histiocitoma Fibroso Benigno/diagnóstico por imagem , Histiocitoma Fibroso Benigno/patologia , Histiocitoma Fibroso Benigno/terapia , Humanos , Úmero/diagnóstico por imagem , Úmero/patologia , Ílio/diagnóstico por imagem , Ílio/patologia , Masculino , Pessoa de Meia-Idade , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , Osteossarcoma/terapia , Prognóstico , Estudos Retrospectivos , Tíbia/diagnóstico por imagem , Tíbia/patologia , Adulto Jovem
10.
J Med Case Rep ; 8: 452, 2014 Dec 20.
Artigo em Inglês | MEDLINE | ID: mdl-25526966

RESUMO

INTRODUCTION: Fibrous dysplasia is one of many well-known disorders in which there is a defect in the remodeling process of immature bone to mature into lamellar bone, and it often exists in metaphyseal and diaphyseal parts of the long bone. In this report, we describe a rare case where fibrous dysplasia was found only in the proximal part of the epiphysis of the tibia without other bony lesions. CASE PRESENTATION: A 14-year-old Asian girl was referred to our hospital after slipping down with pain on the left knee. A radiograph showed an abnormal finding of a central radiolucent lesion with a marginal sclerotic border near the proximal tibial spine. A magnetic resonance image showed the lesion at low signal intensity on a T1-weighted image and at high signal intensity on a T2-weighted image. The biopsy results led us to conclude that the lesion was a fibrous dysplasia. CONCLUSION: If an abnormal lesion on the epiphysis, especially in long bones, is detected on a radiograph, several differential diagnoses can be made. Although fibrous dysplasia is usually not encountered as an epiphyseal lesion, it is important to incorporate all the clinical, radiographic and pathologic features to diagnose monostotic fibrous dysplasia when the lesion is located at the epiphyseal location.


Assuntos
Epífises/patologia , Displasia Fibrosa Monostótica/diagnóstico , Imagem por Ressonância Magnética , Tíbia/patologia , Tomografia Computadorizada por Raios X , Adolescente , Diagnóstico Diferencial , Epífises/diagnóstico por imagem , Feminino , Displasia Fibrosa Monostótica/patologia , Displasia Fibrosa Monostótica/cirurgia , Humanos , Fixadores Internos , Tíbia/diagnóstico por imagem , Resultado do Tratamento
11.
World J Surg Oncol ; 12: 349, 2014 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-25407316

RESUMO

The surgical treatment of fibrous dysplasia remains a challenge for the pediatric orthopedist because of its high recurrence rate. Although a few successful treatments have been reported by using en bloc resection and bone graft in adults, this has not been reproduced in children. In this report, the authors present two children (2.5 and 6 years old) with monostotic expansile fibrous dysplasia involving the ulna and fibula, respectively, who underwent en bloc resection and autograft to replace the involved bones. Good bone union and functional recovery were obtained postoperatively. However, during a follow-up period of 8 and 5 years, respectively, the lesions recurred completely, and the deformities remained progressing over time. En bloc resection and bone graft cannot prevent recurrence in skeletally immature patients with monostotic expansile fibrous dysplasia, and cannot alter for the natural history of the disease. A combination of other management should be considered in children with fibrous dysplasia.


Assuntos
Transplante Ósseo , Displasia Fibrosa Monostótica/cirurgia , Fíbula/cirurgia , Complicações Pós-Operatórias , Ulna/cirurgia , Adulto , Criança , Pré-Escolar , Displasia Fibrosa Monostótica/patologia , Fíbula/patologia , Humanos , Masculino , Prognóstico , Transplante Autólogo , Ulna/patologia
12.
Bone Joint J ; 96-B(5): 673-6, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24788504

RESUMO

Monostotic fibrous dysplasia of the proximal femur has a variable clinical course, despite its reported limited tendency to progress. We investigated the natural history and predisposing factors for progression of dysplasia in a group of 76 patients with a mean follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented with an asymptomatic incidental lesion while 45 (59%) presented with pain or a pathological fracture. A group of 23 patients (30%) underwent early operative treatment for pain (19: 25%) or pathological fracture (4: 5%). Of the 53 patients who were initially treated non-operatively, 45 (85%) remained asymptomatic but eight (15%) needed surgery because of pain or fracture. The progression-free survival of the observation group was 81% (sd 6.4%) at five-years follow-up. An initial presentation of pain (p < 0.001), a limp (p < 0.001), radiological evidence of microfracture (p = 0.001) and younger age (< 17 years) (p = 0.016) were significant predisposing factors for disease progression. The risk of experiencing pain or pathological fracture is considerable in monostotic fibrous dysplasia of the proximal femur. Patients presenting with pain, a limp or radiological evidence of microfracture have a high chance of needing surgical treatment.


Assuntos
Fêmur , Displasia Fibrosa Monostótica/diagnóstico , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Pré-Escolar , Progressão da Doença , Feminino , Fraturas do Fêmur/diagnóstico por imagem , Fraturas do Fêmur/etiologia , Fêmur/patologia , Fêmur/cirurgia , Displasia Fibrosa Monostótica/complicações , Displasia Fibrosa Monostótica/patologia , Displasia Fibrosa Monostótica/cirurgia , Seguimentos , Fraturas Espontâneas/diagnóstico por imagem , Fraturas Espontâneas/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Prognóstico , Radiografia , Fatores de Risco , Adulto Jovem
13.
Minerva Stomatol ; 63(1-2): 43-9, 2014.
Artigo em Inglês, Italiano | MEDLINE | ID: mdl-24487949

RESUMO

The concept of "fibro-osseous lesions" of bone has evolved over the last several years and now includes two mayor entities: the fibrous dysplasia (FD) and the cement-ossifying fibroma (COF). Fibrous dysplasia is considered to be a developmental, tumor-like (hamartomatous), fibro-osseous disease of unknown etiology. There is a maxillary predominance when craniofacial FD occurs in the jaws and the maxillary sinus is commonly involved. Differentiation of OF from FD is important because of differences in treatment and behaviour. This article report a case of 60-year-old female with a history of painless unilateral palatal swelling.


Assuntos
Displasia Fibrosa Monostótica/cirurgia , Doenças Maxilares/cirurgia , Palato Duro/cirurgia , Antibioticoprofilaxia , Biópsia , Cromograninas , Diagnóstico Diferencial , Feminino , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/genética , Displasia Fibrosa Monostótica/patologia , Subunidades alfa Gs de Proteínas de Ligação ao GTP/genética , Humanos , Doenças Maxilares/diagnóstico por imagem , Doenças Maxilares/genética , Doenças Maxilares/patologia , Pessoa de Meia-Idade , Osteoclastos/patologia , Osteossarcoma/diagnóstico , Palato Duro/diagnóstico por imagem , Palato Duro/patologia , Mutação Puntual , Tomografia Computadorizada Espiral
14.
Pan Afr Med J ; 19: 124, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25745531

RESUMO

Fibrous dysplasia (FD) is a progressive systemic bone tumour of young and it can be seen on cranial bones. FD is divided into three types according to radiological features. The second most common subtype is polyostotic subtype. With this article, we aimed to review and present clinical features, radiological examination, differential diagnosis and treatment management of a case of solitary monostotic fibrous dysplasia of occipital bone. 15 years old female patient admitted to our hospital for a bump and in the back of his head that she noticed 1 month ago. Her physical and neurological examination was normal. On cranial CT examination we detected a bony defect. Her gadolinium enhanced cranial MRI revealed bony defect along with massive gadolinium enhancement in adjacent tissue. On histopathologic examination; PANCK, CD68, CD1a were found negative and CD45, S-100, Vimentine were found positive. Ki-67 was 4,8%. In conclusion, fibrous dysplasia is a progressive bone disease of the young patients. Despite its resemblance to a benign lesion by not being symptomatic it can progress and cause severe bony defects and skin lesions. Total surgical resection is necessary and sufficient for total treatment.


Assuntos
Displasia Fibrosa Monostótica/patologia , Imagem por Ressonância Magnética , Osso Occipital/patologia , Adolescente , Diagnóstico Diferencial , Progressão da Doença , Feminino , Displasia Fibrosa Monostótica/diagnóstico , Displasia Fibrosa Monostótica/cirurgia , Humanos , Tomografia Computadorizada por Raios X
15.
J Craniofac Surg ; 24(3): 758-62, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23714874

RESUMO

BACKGROUND: Fibrous dysplasia (FD) is a tumor-like growth that consists of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. The most commonly affected bones are facial bones, causing a number of facial cosmetic and functional problems. METHODS: From December 2008 to July 2012, 10 patients with craniomaxillofacial fibrous dysplasia were treated by conservative resection and local recontouring. The patients were followed up yearly, with an average of 3 years; the longest follow-up period was 5 years. RESULTS: All the 10 patients received appropriate treatment and histopathological examinations were performed to confirm the diagnosis of FD. Four patients with zygoma involved had received partial zygoma osteoectomy and 2 patients received mandibular partial osteoectomy. Average time of follow-up was 3 years, with a range from 1 to 5 years, and all patients obtained satisfactory aesthetic and functional results. CONCLUSION: In most patients, a conservative surgery will achieve good functional and aesthetic results. For patients with mild symptoms, the aesthetic effect should be given priority while for the heavier patients the restoration of function and aesthetic effects should all be taken into account.


Assuntos
Ossos Faciais/cirurgia , Displasia Fibrosa Poliostótica/cirurgia , Crânio/cirurgia , Adolescente , Adulto , Curetagem/métodos , Estética , Assimetria Facial/cirurgia , Ossos Faciais/patologia , Feminino , Displasia Fibrosa Monostótica/patologia , Displasia Fibrosa Monostótica/cirurgia , Displasia Fibrosa Poliostótica/patologia , Seguimentos , Humanos , Masculino , Doenças Mandibulares/cirurgia , Doenças Maxilares/cirurgia , Osteotomia/métodos , Adulto Jovem , Zigoma/cirurgia
16.
Int J Clin Exp Pathol ; 6(3): 531-5, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23411641

RESUMO

Fibrous dysplasia (FD) is a benign skeletal disorder in which abnormally overgrowing bony lesion replaces normal bone. FD can affect one bone (monostotic form) or multiple bones (polyostotic form). The craniofacial bones are involved in about 10% of subjects with monostotic FD. However, its occurrence in the sinonasal tract is very rare. We report a case of monostotic FD developed only in the inferior turbinate in a 29-year-old woman. To the best of our knowledge, it is the second report of monostotic FD involving the inferior turbinate in the medical literature. We, therefore, report this rare case with a review of literature.


Assuntos
Displasia Fibrosa Monostótica/patologia , Obstrução Nasal/patologia , Conchas Nasais/patologia , Adulto , Feminino , Displasia Fibrosa Monostótica/complicações , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/cirurgia , Humanos , Obstrução Nasal/diagnóstico por imagem , Obstrução Nasal/etiologia , Obstrução Nasal/cirurgia , Tomografia Computadorizada de Emissão , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Conchas Nasais/diagnóstico por imagem , Conchas Nasais/cirurgia
17.
Genet Couns ; 24(4): 373-9, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24551979

RESUMO

Focal fibrocartilaginous dysplasia (FFCD) is an uncommon, benign bone lesion that causes angular deformities of the long bones in young children. Most deformities were seen around the knee. Diagnostic criteria are based on clinical and radiological signs: unilateral angular deformity in a long bone of a young child, associated with on X-ray a typical lucent bony defect with surrounding sclerosis at the concavity of the deformity with a cortical defect. The etiology is not understood. There were 16 cases described previously involving the upper extremity: 9 cases with FFCD of the ulna, 3 of the humerus, 2 of the radius and 2 of the phalanx. We report a case of cubitus varus deformity in a young girl of 2 years and 2 months where the deformity worsened very quickly. We believe that debridement of the fibrous tether at very young age will prevent further deformity and can correct spontaneously by remaining growth.


Assuntos
Fibrocartilagem/patologia , Displasia Fibrosa Monostótica/patologia , Pré-Escolar , Progressão da Doença , Feminino , Humanos
18.
J Hand Surg Am ; 37(11): 2300-3, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23101526

RESUMO

Focal fibrocartilaginous dysplasia is an uncommon, benign bone lesion that causes deformity in young children. It is most commonly encountered in the proximal tibia, with few cases documented in the upper extremity. We report 3 cases affecting the ulna. In each case, the fibrous tissue appeared to anchor itself in the ulnar diaphysis, behaving as a tether that retards growth. All 3 patients had excision of the fibrous tissue. There was concern for radial head subluxation before surgical intervention, but all patients maintained a congruent radiocapitellar articulation. The visible deformity improved in all 3 patients, although the limbs remained shorter than the contralateral side. In our limited series, we believe that early excision of the fibrous tissue tether can prevent radiocapitellar joint dislocation in patients with focal fibrocartilaginous dysplasia of the ulna.


Assuntos
Displasia Fibrosa Monostótica , Ulna/patologia , Adulto , Pré-Escolar , Feminino , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Displasia Fibrosa Monostótica/cirurgia , Antebraço/diagnóstico por imagem , Humanos , Masculino , Radiografia , Ulna/diagnóstico por imagem
19.
J Craniofac Surg ; 23(5): e452-4, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22976704

RESUMO

Fibrous dysplasia is a benign fibro-osseous disease that affects 1 or more bones. Deformities leading to aesthetic and functional disorders are observed in almost all cases. Plastic surgery is often recommended when the jaws are involved. Monostotic fibrous dysplasia of the mandible is an unusual manifestation of the disease that is usually benign, occurs in young individuals, and is managed by conservative curettage or debridement, such as surgical sculpting. The authors report a case of a 15-year-old patient with a large monostotic fibrous dysplasia located in the right mandible, which was treated by contouring bone. The lesion did not recur on follow-up for 4 years after the surgical procedure.


Assuntos
Displasia Fibrosa Monostótica/cirurgia , Doenças Mandibulares/cirurgia , Adolescente , Biópsia , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Humanos , Masculino , Doenças Mandibulares/diagnóstico por imagem , Doenças Mandibulares/patologia , Tomografia Computadorizada por Raios X
20.
Orphanet J Rare Dis ; 7 Suppl 1: S1, 2012 May 24.
Artigo em Inglês | MEDLINE | ID: mdl-22640754

RESUMO

The surgical management of Polyostotic Fibrous Dysplasia (FD) of bone is technically demanding. The most effective methods to manage the associated bone deformity remain unclear. The marked variation in the degree and pattern of bone involvement has made it difficult to acquire data to guide the surgeon's approach to these patients. In light of the paucity of data, but need for guidance, recognized experts in the management of these patients came together at the National Institutes of Health in Bethesda, Maryland as part of an International meeting to address issues related to fibrous dysplasia of bone to discuss and refine their recommendations regarding the surgical indications and preferred methods for the management of these challenging patients. The specific challenges, recommended approaches, and "lessons learned" are presented in hopes that surgeons faced with typical deformities can be guided in the surgical reconstruction of both children and adults with FD.


Assuntos
Osso e Ossos/cirurgia , Displasia Fibrosa Monostótica/cirurgia , Displasia Fibrosa Poliostótica/cirurgia , Administração dos Cuidados ao Paciente/métodos , Adulto , Transplante Ósseo/métodos , Osso e Ossos/diagnóstico por imagem , Osso e Ossos/patologia , Criança , Congressos como Assunto , Displasia Fibrosa Monostótica/diagnóstico por imagem , Displasia Fibrosa Monostótica/patologia , Displasia Fibrosa Poliostótica/diagnóstico por imagem , Displasia Fibrosa Poliostótica/patologia , Fraturas Ósseas/patologia , Humanos , Fixadores Internos , Radiografia , Escoliose/diagnóstico por imagem , Escoliose/patologia , Escoliose/cirurgia
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