313 results on '"Monostotic fibrous dysplasia"'
Search Results
2. Slow Growing Hard Lump over the Cheek: Fibrous Dysplasia
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Kallarakkal, Thomas George, Ngeow, Wei Cheong, Tilakaratne, Wanninayake M, editor, and Kallarakkal, Thomas George, editor
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- 2023
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3. Research on Monostotic Fibrous Dysplasia Published by a Researcher at SRM Kattankulathur Dental College and Hospital (Monostotic fibrous dysplasia of anterior & posterior mandible: A rare case report & review of literature).
- Abstract
A recent research article published by a researcher at SRM Kattankulathur Dental College and Hospital in Tamil Nadu, India, discusses monostotic fibrous dysplasia, a condition characterized by the replacement of bone tissue with fibro-osseous tissue. The article reports on a case of a 14-year-old with monostotic fibrous dysplasia in the lower jaw, supported by clinical, radiographic, and pathological findings. The patient underwent surgical shaving and re-contouring of the jaw, with no evidence of progression or malignancy during follow-up assessments. The article provides valuable insights into this rare condition and its management. [Extracted from the article]
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- 2024
4. University of Ciencias Medicas Researchers Further Understanding of Monostotic Fibrous Dysplasia (Mandibular monostotic fibrous dysplasia in a 23-year-old young woman).
- Abstract
A new report from the University of Ciencias Medicas discusses the rare anomaly of skeletal development known as monostotic fibrous dysplasia. This condition is caused by a genetic mutation that leads to the replacement of normal bone with irregular bone trabeculae due to increased fibrous tissue proliferation. The report presents a case study of a 23-year-old patient with mandibular monostotic fibrous dysplasia, describing their symptoms, diagnosis, and treatment. The researchers highlight the rarity and high postoperative morbidity of this condition, emphasizing the importance of individualized treatment based on disease progression. [Extracted from the article]
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- 2024
5. Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case report and its surgical management.
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Stocsits, Anna, Lener, Sara, Girod, Pierre Pascal, Abramovič, Anto, Thomé, Claudius, and Hartmann, Sebastian
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LUMBAR vertebrae , *DYSPLASIA , *HUMAN abnormalities , *SYMPTOMS - Abstract
Monostotic fibrous dysplasia (MFD) of the lumbar spine represents an exceedingly rare lesion. A 26-year-old patient presented with a progressive osteolytic lesion of the vertebral body L2 and the diagnosis of MFD. A minimally invasive left-sided eXtreme Lateral Interbody Fusion (XLIF) approach with resection of the vertebral body L2 with placement of a mesh cage was performed. No complications were observed perioperatively and the symptoms rapidly improved. Minimally invasive piecemeal resection with a combined dorsolateral approach showed a favorable clinical and radiological outcome and seems to be a safe and reliable technique for MFD. [ABSTRACT FROM AUTHOR]
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- 2020
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6. Fibrous Dysplasia
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Ruggieri, Pietro, Picci, Piero, editor, Manfrini, Marco, editor, Fabbri, Nicola, editor, Gambarotti, Marco, editor, and Vanel, Daniel, editor
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- 2014
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7. ¿Displasia fibrosa o fibroma osificante? Caracterización histológica de dos casos inusuales.
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TOVÍO MARTÍNEZ, EILIEN G., DEL VALLE, SAMUEL E. URBANO, VERGARA HERNÁNDEZ, CLARA I., and DÍAZ CABALLERO, ANTONIO JOSÉ
- Subjects
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BONES , *ORAL surgery , *DYSPLASIA , *ORAL medicine , *FIBROUS dysplasia of bone , *PATHOLOGISTS - Abstract
Background: Fibrous dysplasia and ossifying fibroma are benign pathologies that generally have no painful symptoms, are rare, and affect bone tissue. They produce aesthetic and functional alterations, which is why patients consult. Purpose: To describe the cellular disorders characteristic of fibrous dysplasia and ossifying fibroma, as well as their relationship with clinical forms and other histopathological variables that constitute a challenge for the oral pathologist and the clinician. Description of the cases: Two cases of adult patients, male and female, who attended the Stomatology and Oral Surgery Unit of the University of Cartagena in Colombia, are described. The patients shoed facial, asymptomatic, and unilateral asymmetries that compromised patients' aesthetics. Clinical, radiographic, and histological characteristics are described. Conclusion: Fibrous dysplasia and ossifying fibroma are similar benign bone disorders that require differential diagnosis in order to establish suitable and specific treatments. [ABSTRACT FROM AUTHOR]
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- 2019
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8. Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex.
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Jeyaraj, Priya
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DYSPLASIA ,SURGICAL excision ,SKULL base ,FACIAL pain ,FIBROUS dysplasia of bone ,CRANIAL nerves ,MASTICATION - Abstract
Background: Owing to the overlapping clinical, radiographic and histopathological features among the diverse group of Fibro-osseous lesions, a precise and definitive diagnosis of Fibrous Dysplasia (FD) can be quite challenging. Moreover, FD itself may manifest with widely varying clinical presentations, radiographic appearances and histological pictures, depending upon the maturity of the lesion, and the relative quantum of its 'fibrous' and 'osseous components'. Prompt and accurate diagnosis of Fibrous Dysplasia (FD) of the Craniomaxillofacial region is particularly important, as the condition is capable of causing considerable facial asymmetry or deformity leading even to marked disfigurement, which can have a profound psychosocial impact on the patient. Involvement of Maxillofacial bones by aggressive forms of FD, can produce serious functional debility as well, by compromising airway, breathing, vision, hearing, occlusion, mastication and mouth opening. Calvarial bone involvement can produce cranial asymmetry, and cranial base involvement can lead to persistent headaches, facial pain, numbness, and other neurological deficits owing to compression of cranial nerves. Aims and Objectives: To evaluate the importance of early and precise diagnosis, with prompt surgical management of these lesions, for a successful overall esthetic and functional outcome. Materials and Methods: A Case series of 15 patients, showcasing the principal variants of FD affecting the Craniomaxillofacial complex, namely, the Monostotic and Craniofacial forms have been described. Diversity in their Clinical, Radiographic and Histopathological presentations; their management modalities elucidating the various surgical approaches employed to access and excise these bone pathologies, have been provided along with a review of existing literature. Results: Various surgical approaches may be employed to access the lesions, depending upon their location, extent and involvement. Treatment protocols range from complete surgical excision to surgical shaving and recontouring, and must be decided upon on a case to case basis, with the aim to achieve the best possible esthetic and functional outcome with the least postoperative morbidity. Conclusion: Correlation of HPE with history, clinical features, biological behaviour, radiographic and CT appearance, laboratory findings, and intra-operative findings is imperative, so that they can be distinguished from other bony lesions and an appropriate, ideal and effective treatment modality can be instituted in time, so as to achieve the most favourable esthetic and functional outcome. [ABSTRACT FROM AUTHOR]
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- 2019
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9. Differential diagnosis of fibrous dysplasia
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László Simonffy, Szabolcs Gyulai-Gaál, Csaba Dobó Nagy, and Bence Tamás Szabó
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orthopantomography ,cone beam computed tomography ,fibrous dysplasia of bone ,monostotic fibrous dysplasia ,differential diagnosis ,Dentistry ,RK1-715 - Abstract
Following taking the medical and dental history and performing a stomato-oncological screening of a 69-year-old female patient referred to our department, a panoramic X-ray acquisition was taken. On the panoramic image multiple sclerotic and cyst-like lesions were visible in the mandible. Based on the radiological findings, the use of further imaging modalities was needed. Posteroanterior (PA) and lateral (LAT) skull and cone beam CT (CBCT) images were taken. Except for the mandibular bone no abnormalities were found in other areas of the skull on the PA and LAT skull radiographs. No chest X-ray acquisition has been taken from the patient within the last five years. On the basis of available medical history, patient’s examination data and radiographic images monostotic fibrous dysplasia has been predicted as radiologic diagnosis. Fibrous dysplasia is a benign developmental failure of the bone, which can involve one (monostotic type) or more bones (polyostotic type). During the bone remodeling the osseus structures are replaced by immature fibro-osseous tissue. A precise diagnosis of fibrous dysplasia usually cannot be made without using appropriate radiologic imaging modalities.
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- 2018
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10. Fibrosus dysplasia differenciál-diagnózisa.
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LÁSZLÓ, SIMONFFY, SZABOLCS, GYULAI-GAÁL, CSABA, DOBÓ NAGY, and TAMÁS, SZABÓ BENCE
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Copyright of Fogorvosi Szemle is the property of Hungarian Dental Association and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2018
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11. Locally aggressive monostotic fibrous dysplasia of the cervical spine mimicking malignancy: a case report and literature review
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Milon Audrey, Polivka Marc, Larousserie Fréderique, Lot Guillaume, Ziza Jean-Marc, and Laredo Jean-Denis
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Monostotic fibrous dysplasia ,Cervical spine ,Pathological fracture ,Aggressive ,Orthopedic surgery ,RD701-811 - Abstract
We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process. Owing to the vertebral instability, a surgical procedure combining C0–C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5–6 laxity below the intervertebral fixation. This case combines three radiological findings, which are unusual in fibrous dysplasia: monostotic presentation involving the spine, some aggressive radiographic features, and a pathological fracture.
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- 2019
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12. Imaging characteristics of a rare case of monostotic fibrous dysplasia of the sacrum: A case report
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Xin Xin, Xiao-Wen Ma, Yu-Hong Yan, and Xin-Xin Liu
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musculoskeletal diseases ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Fibrous dysplasia ,Case Report ,Magnetic resonance imaging ,General Medicine ,musculoskeletal system ,medicine.disease ,Sacrum ,Monostotic fibrous dysplasia ,Computed tomographic ,body regions ,03 medical and health sciences ,0302 clinical medicine ,030220 oncology & carcinogenesis ,Rare case ,medicine ,030211 gastroenterology & hepatology ,Radiology ,business - Abstract
BACKGROUND: Fibrous dysplasia (FD) is a common benign intramedullary fibro-osseous lesion. Involvement of the spine is rare, with the literature including only case reports, and cases of monostotic FD (MFD) in the sacrum are extremely rare. A correct preoperative diagnosis of spinal MFD is important for clinicians to select proper treatment. CASE SUMMARY: We retrospectively assessed a case report of MFD in the sacrum. This patient was examined by computed tomography (CT) and magnetic resonance imaging (MRI), and the diagnosis was confirmed by pathology. A review of the literature was performed to analyze the imaging characteristics and differential diagnoses of spinal MFD. For our patient, the CT scan showed the lesion to be expansile, with ground glass opacity and a sclerotic rim. On MRI, the lesion showed iso-low signal intensity on T1WI and iso-high signal intensity on T2WI. A low signal rim was found on T1WI and T2WI. Our patient was treated by posterior focal excision, decompression, bone grafting, fusion and pedicle screw fixation. A satisfactory result was achieved, with pain disappearance. No complications had occurred at the 1-year follow up. CONCLUSION: MFD is an expansile osteolytic change. Ground glass opacity and a sclerotic margin are obvious characteristics. The lesion often involves the vertebral body and posterior element. Knowledge of these imaging characteristics of spinal FD could be helpful for diagnosis and prevent unnecessary procedures.
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- 2021
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13. Surgical Treatment of Monostotic Craino-Facial Fibrous Dysplasia: Changing the Narratives
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E. A. Dahillo, Oladeji Raheem Quadri, B. F. Bello, I. O. Gbujie, B. I. Egbe, D. F. Fulorunso, B. E. Nwankwo, F. M. Damtong, and Titus S Ibekwe
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medicine.medical_specialty ,business.industry ,Fibrous dysplasia ,Soft tissue ,030206 dentistry ,Cheek ,medicine.disease ,Disfigurement ,Monostotic fibrous dysplasia ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Maxilla ,medicine ,Radical surgery ,030223 otorhinolaryngology ,business ,Nose - Abstract
Background: Fibrous dysplasia mainly presents in its monostotic form in the cranio-facial region with serious cosmetic disfigurement and functional derangement of the affected and adjacent structures putting both patient and the attending surgeon in great dilemma. Surgical treatment is the only rewarding and generally accepted treatment option, however, controversy over the surgical technique to be adopted still exists. While in the past, surgeons generally adopted conservative shaving or contouring technique, over the recent years, advocates of radical surgery are winning more disciples. Objective: To highlight the locally destructive, functionally degrading nature of a neglected or poorly excised (shaved) lesion in patients and highlight the outcome of total excision and surgical technique adopted to obviate the need for autologous bone grafting and two-staged surgery. Subjects and Method: We present case series of three patients with giant monostotic fibrous dysplasia of the maxilla, surgically treated in our Centre, who were part of a total of eight cases managed over the past fifteen years in our department of Ear, Nose and Throat-Head and Neck Surgery. The pre-operative clinical assessment, relevant investigations and post-operative outcome are presented. Our surgical technique is highlighted. All the patients had unilateral lesion of the maxilla with gross cosmetic and functional defects. Two of the patients had ischaemic (pressure) atrophy of the cheek soft tissue and skin leading to skin metaplastic changes including leukoplakia, hyperpigmentation. Post-operative follow-up showed satisfactory cosmetic outcome and significant reversal of malocclusion and dental anarchy. There was no recorded recurrence throughout the follow-up period ranging from four to eleven years. Nasal airway was re-established bilaterally in all the cases. Conclusion: Total or near total excision surgical technique with periosteal preservation is our treatment of choice in the management of monostotic cranio-facial fibrous dysplasia. Given the fact that the growth of the tumours often does not cease after puberty against general belief, shaving or contouring technique should be relegated to the background. Our technique of no grafting which reduced cost and morbidity to the patient should be encouraged.
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- 2021
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14. Monostotic fibrous dysplasia in a 10-year-old patient
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Gayatri Nayanar and MamathaG S. Reddy
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medicine.medical_specialty ,monoostotic ,business.industry ,fibrous dysplasia ,General Medicine ,fibro-osseous lesions ,RM1-950 ,medicine.disease ,Monostotic fibrous dysplasia ,medicine ,woven bone ,Radiology ,Therapeutics. Pharmacology ,business - Abstract
Fibrous dysplasia is a rare congenital bone disorder which manifests as a localized defect in osteoblastic differentiation and maturation with the replacement of normal bone with an abnormal scar-like fibrous connective tissue. It can be classified into monostotic and polyostotic variety with the former seen mostly in the adults and the latter common in children. Craniofacial involvement in case of fibrous dysplasia is very common, affecting maxilla more commonly than the mandible. This report, however, describes a case of fibrous dysplasia in a 10-year-old female patient who presented with a swelling of the left mandible.
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- 2021
15. The Use of Customised Surgical Guide for Bilateral Inferior Mandibulectomy in a Case of Monostotic Fibrous Dysplasia
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Nordin Rifqah, Siti Salmiah Mohd Yunus, Jun Ai Chong, Abd Jabar Nazimi, and Tan Huann Lan
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medicine.medical_specialty ,Mandibulectomy ,business.industry ,Medicine ,Orthodontics ,Oral Surgery ,business ,medicine.disease ,Monostotic fibrous dysplasia ,Surgery - Published
- 2020
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16. Spontaneous heparin induced thrombocytopenia (HIT) following curettage and bone graft of femur in a patient with monostotic fibrous dysplasia
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Seshadri Thirumala, Rachana Yendala, Donald P. Quick, Jonathan Kopel, and Sriman Swarup
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medicine.medical_specialty ,Bone Transplantation ,business.industry ,medicine.medical_treatment ,Hematology ,medicine.disease ,Thrombocytopenia ,Monostotic fibrous dysplasia ,Curettage ,Surgery ,Text mining ,Heparin-induced thrombocytopenia ,medicine ,Humans ,Femur ,business ,Fibrous Dysplasia, Monostotic - Published
- 2020
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17. The surgical management of monostotic fibrous dysplasia of the inferior turbinate
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Camilla Dosio, Matteo Brucoli, Arnaldo Benech, Paolo Boffano, and Massimiliano Garzaro
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Partial Maxillectomy ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Fibrous dysplasia ,030206 dentistry ,medicine.disease ,McCune–Albright syndrome ,Monostotic fibrous dysplasia ,Endoscopy ,03 medical and health sciences ,0302 clinical medicine ,Normal bone ,Otorhinolaryngology ,medicine ,Surgery ,Radiology ,Oral Surgery ,030223 otorhinolaryngology ,business - Abstract
Fibrous dysplasia is a non-neoplastic, sporadic, slowly progressing disease of the bone in which normal bone is replaced by abnormally overgrowing lesions. There are three different types of fibrous dysplasia: monostotic (affecting a single bone), poliostotic (affecting multiple bones) and syndromic, when it is associated with other diseases (such as McCune Albright syndrome). Fibrous dysplasia affects cranio-facial bones in 10% of the cases. However, its occurrence in the inferior turbinate is extremely rare. To the best of our knowledge, only four cases of monostotic form have been reported so far. Hereafter we describe a case of monostotic FD of the inferior turbinate surgically treated with a trans-nasal endoscopic partial maxillectomy type II.
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- 2020
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18. Monostotic fibrous dysplasia at C7 treated with vertebroplasty: a case report and review of the literature
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Jianhong Feng, Xin Xin, Tao Jin, Xinxin Liu, and Chen Yue
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medicine.medical_specialty ,Biopsy ,medicine.medical_treatment ,lcsh:Surgery ,Case Report ,Physical examination ,Fibrous dysplasia ,lcsh:RC254-282 ,03 medical and health sciences ,0302 clinical medicine ,Spinal cord compression ,medicine ,Humans ,Corpectomy ,030222 orthopedics ,Neck pain ,Vertebroplasty ,Neck Pain ,medicine.diagnostic_test ,business.industry ,Bone Cements ,lcsh:RD1-811 ,Middle Aged ,medicine.disease ,lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,Monostotic fibrous dysplasia ,Curettage ,Spine ,Vertebra ,Surgery ,Treatment Outcome ,medicine.anatomical_structure ,Oncology ,Cervical vertebra ,Cervical Vertebrae ,Female ,medicine.symptom ,Tomography, X-Ray Computed ,business ,030217 neurology & neurosurgery ,Fibrous Dysplasia, Monostotic - Abstract
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.
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- 2019
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19. Features of Monostotic Fibrous Dysplasia in Maxilla using Cone Beam Computed Tomography
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Sachin G. Ram, Devika S Pillai, Shruthi Hegde, Vidya Ajila, and G Subhas Babu
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Cone beam computed tomography ,Materials science ,business.industry ,Maxilla ,medicine ,Nuclear medicine ,business ,medicine.disease ,Monostotic fibrous dysplasia - Published
- 2019
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20. Wedge Osteotomy with Tens Nailing in Monostotic Fibrous Dysplasia of Tibia – A Case Report
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Mangal Parihar and Eknath Pawar
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medicine.medical_specialty ,business.industry ,Fibrous dysplasia ,Deformity correction ,Case Report ,Wedge osteotomy ,medicine.disease ,Monostotic fibrous dysplasia ,Surgery ,Male patient ,Radiological weapon ,Medicine ,monostotic ,titanium elastic nailing ,Tibia ,Fibula ,business - Abstract
Introduction: Titanium elastic nailing (TENS) with wedge osteotomy for the management of monostotic fibrous dysplasia (FD) of tibia is done in a 6-year-old male patient. Case Report: This is a case describing monostotic FD of right tibia in a 6-year-old male patient. The patient’s parents noticed a diffuse swelling in the right leg a few days after birth. It was managed conservatively till 3 years of age when the patient started complaining of pain in his right leg and difficulty in walking and running. The patient developed gradual and progressive anterior bowing in the right leg in the next 3 years. The patient was diagnosed with monostotic FD of the right tibia based on clinical and radiological findings. Wedge osteotomies were done in the tibia and fibula with TENS nailing as a definitive procedure for this patient. Discussion: Management of monostotic FD of the right tibia with TENS nailing and wedge osteotomy can be a possible modality of definitive management with complete pain relief and deformity correction. Conclusion: Correction of deformities secondary to FD requires meticulous pre-operative planning and execution. But with proper planning, even major deformities as in our case, procedures as simple as wedge osteotomy and TENS nailing can give excellent outcomes. Keywords: Fibrous dysplasia, monostotic, titanium elastic nailing.
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- 2021
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21. Monostotic Fibrous Dysplasia of the Mandible in a 9-Year-Old Male Patient Treated with a Conservative Surgical Treatment: A Case Report and 15-Year Follow-Up
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Georges Aoun, Georges Aad, Emile Khalaf, and Antoine Berberi
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medicine.medical_specialty ,Osteoplasty ,business.industry ,Fibrous dysplasia ,Mandible ,RK1-715 ,Case Report ,030206 dentistry ,medicine.disease ,Hyperpigmentation ,Monostotic fibrous dysplasia ,Surgery ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Dentistry ,030220 oncology & carcinogenesis ,Maxilla ,medicine ,medicine.symptom ,business ,General Dentistry ,Facial symmetry - Abstract
Fibrous dysplasia is a developmental disorder of the bone that originates from a genetic defect disturbing the osteogenesis leading to the replacement of normal bone with the excess proliferation of fibrous tissue. It can be associated with hyperpigmentation of the skin and endocrine disorders. Fibrous dysplasia can manifest in a monostotic form affecting one bone or in a polyostotic form involving several bones. Approximately 30% of monostotic forms are observed in the maxilla and the mandible. It frequently appears in the posterior region and is usually unilateral. It is found in teenagers and could become static after adulthood. Patients can present with swelling, facial asymmetry, pain, or numbness on the affected side. Treatment modalities vary between conservative surgical treatment, radical surgical approach, and medical treatment based on bisphosphonates. Here, we present a case of a monostotic form of fibrous dysplasia affecting the posterior left region of the mandible in a 9-year-old male complaining of gradually increased swelling on the left mandibular side of one-year duration. The diagnosis of fibrous dysplasia is established based on clinical, radiographical, and histopathological features. Conservative surgery is implemented with surgical shaving and reencountering of the bone excess to reduce the facial asymmetry. Recurrence is reported 10 years later and is also treated with a localized osteoplasty and remodeling of the bone contours. Five years later, the lesion remains stable. In conclusion, a conservative approach should be adopted as the first line of treatment for young patients suffering from monostotic fibrous dysplasia.
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- 2021
22. Doença de Paget óssea monostótica em vértebra lombar: uma localização atípica Monostotic Paget's disease of the lumbar vertebrae: an atypical location
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Alexandre Dias Carvalho, Jerúsia Oliveira Ibiapina, Lina Gomes Santos, Teresinha Castelo Branco Carvalho, and Marcelo Barbosa Ribeiro
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Doença de Paget ,Displasia fibrosa monostótica ,Coluna vertebral ,Paget's disease ,Monostotic fibrous dysplasia ,Spine ,Medicine ,Orthopedic surgery ,RD701-811 - Abstract
Paciente do sexo feminino, 41 anos, cor branca, com queixa de dor lombar há mais de três anos, sem irradiações, submetida há vários exames radiográficos sendo diagnosticada, por biópsia, doença de Paget monostótica na terceira vértebra lombar. Trata-se de uma localização incomum.Female patient, 41 years, Caucasian, with lumbar pain for more than three years, without irradiation, submitted to various radiological exams and diagnosed by biopsy with monostotic Paget's disease of the third lumbar vertebrae, which is an uncommon location.
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- 2010
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23. Monostotic Fibrous Dysplasia Mimicking Metastasis in the Femoral Neck on Bone Scintigraphy and 18F-FDG PET/CT
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Hung-Pin Chan, Ni-Chun Kuo, Hung-Yen Chan, and Wei-Liang Hung
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PET-CT ,medicine.medical_specialty ,lcsh:R5-920 ,medicine.diagnostic_test ,business.industry ,PET/CT ,Fibrous dysplasia ,Clinical Biochemistry ,fibrous dysplasia ,Interesting Images ,medicine.disease ,Scintigraphy ,Monostotic fibrous dysplasia ,Lesion ,medicine.anatomical_structure ,Bone scintigraphy ,Medicine ,metastasis ,Radiology ,bone scintigraphy ,medicine.symptom ,business ,lcsh:Medicine (General) ,Calcification ,Femoral neck - Abstract
A 51-year-old woman who had lung adenocarcinoma was found to have a high uptake area over the right femoral neck by means of bone scintigraphy, suggesting a suspicious bony metastasis. 18F-FDG PET/CT was arranged, and showed an FDG-avid lesion in the same region. However, after augmented CT, a well-defined ground-glass lesion with circular calcification was found, which is the favored benign lesion of fibrous dysplasia. The following imaging of bone scintigraphy and 18F-FDG PET/CT presented no apparent change. This case demonstrates that the treatment scheme should not be solely guided by abnormalities in scintigraphy. Additional imaging is recommended for accurate staging or development of an appropriate treatment plan.
- Published
- 2020
24. Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case report and its surgical management
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Pierre Pascal Girod, Claudius Thomé, Sara Lener, Anna Stocsits, Sebastian Hartmann, and Anto Abramovic
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Adult ,Male ,medicine.medical_specialty ,Monostotic fibrous dysplasia ,03 medical and health sciences ,0302 clinical medicine ,Case Report - Spine - Other ,Deformity ,medicine ,Humans ,Kyphosis ,Minimally invasive ,Neuroradiology ,Lumbar Vertebrae ,medicine.diagnostic_test ,business.industry ,Interventional radiology ,medicine.disease ,Kyphotic deformity ,Second lumbar vertebra ,Surgery ,Spinal Fusion ,Lumbar spine ,030220 oncology & carcinogenesis ,Neurology (clinical) ,Neurosurgery ,medicine.symptom ,business ,030217 neurology & neurosurgery ,Fibrous Dysplasia, Monostotic - Abstract
Monostotic fibrous dysplasia (MFD) of the lumbar spine represents an exceedingly rare lesion. A 26-year-old patient presented with a progressive osteolytic lesion of the vertebral body L2 and the diagnosis of MFD. A minimally invasive left-sided eXtreme Lateral Interbody Fusion (XLIF) approach with resection of the vertebral body L2 with placement of a mesh cage was performed. No complications were observed perioperatively and the symptoms rapidly improved. Minimally invasive piecemeal resection with a combined dorsolateral approach showed a favorable clinical and radiological outcome and seems to be a safe and reliable technique for MFD.
- Published
- 2020
25. Patients With Isolated Craniofacial Dysplasia Report Better Quality of Life Compared With Those With Craniofacial Dysplasia and Extracranial Involvement
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Neveen A. T. Hamdy, P. D. Sander Dijkstra, Cornelie D. Andela, Stijn W. Genders, Natasha M. Appelman-Dijkstra, B.C.J. Majoor, Marlous Hagelstein-Rotman, and Irene C. Notting
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Pediatrics ,medicine.medical_specialty ,Disease ,Fibrous Dysplasia, Polyostotic ,03 medical and health sciences ,0302 clinical medicine ,Quality of life ,Surveys and Questionnaires ,Humans ,Medicine ,In patient ,Craniofacial ,business.industry ,Fibrous dysplasia ,Skull ,Craniofacial dysplasia ,Fibrous Dysplasia of Bone ,General Medicine ,medicine.disease ,Monostotic fibrous dysplasia ,Ophthalmology ,Cohort ,Quality of Life ,030221 ophthalmology & optometry ,Surgery ,business - Abstract
PURPOSE Craniofacial fibrous dysplasia (CFD) is a subtype of fibrous dysplasia/McCune-Albright syndrome (FD/MAS) characterized by FD lesions in one or more of the skull bones. The orbit is often involved, with facial pain, facial deformity, and increased risk of compressive optic neuropathy as associated clinical manifestations possibly leading to altered illness perceptions and impairments in quality of life(QoL). The aim of this study was to evaluate illness perceptions and QoL in patients with CFD among our FD/MAS cohort. METHODS One hundred ninety-one patients were included. Illness perceptions and QoL were assessed by using validated questionnaires, that is, the Illness Perceptions Questionnaire-Revised and the Short-Form 36. Patients were first grouped as CFD versus non-CFD, a second selection was based on the presence of "Isolated CFD" versus "CFD+PFD/MAS." Non-CFD patients were grouped as monostotic fibrous dysplasia "MFD" versus polyostotic "PFD/MAS." RESULTS Patients with isolated CFD attributed less symptoms to their disease compared with patients with CFD+PFD/MAS (p < 0.05). Furthermore, patients with isolated CFD reported better QoL on all domains (except role emotional and mental health) compared with patients with CFD+PFD/MAS (p < 0.05). Patients with isolated CFD also reported better QoL compared with non-CFD groups (on 3 out of 8 subscales) (p < 0.05). CONCLUSIONS Patients with isolated CFD attribute less symptoms to their disease and report better QoL compared with patients with CFD with extracranial involvement or FD without cranial involvement. These findings indicate that craniofacial involvement alone is not sufficient to cause negative illness perceptions and impairments in QoL. Therefore, it can be postulated that isolated CFD should be considered a unique patient subtype within the spectrum of FD/MAS patients.
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- 2020
26. Natural History and Progression of Craniofacial Fibrous Dysplasia: A Retrospective Evaluation of 114 Patients From Massachusetts General Hospital
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Leonard B. Kaban and Kristin Sweeney
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Adult ,Male ,medicine.medical_specialty ,Pathologic fracture ,Fibrous Dysplasia, Polyostotic ,Hospitals, General ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Internal medicine ,medicine ,Craniofacial Fibrous Dysplasia ,Humans ,Craniofacial ,Polyostotic fibrous dysplasia ,Young adult ,Retrospective Studies ,business.industry ,Fibrous dysplasia ,Retrospective cohort study ,030206 dentistry ,Airway obstruction ,medicine.disease ,Monostotic fibrous dysplasia ,Otorhinolaryngology ,Massachusetts ,030220 oncology & carcinogenesis ,Surgery ,Female ,Oral Surgery ,business - Abstract
Purpose The natural history of fibrous dysplasia (FD) is poorly understood. The purpose of this study was to identify differences in demographic, clinical, and radiographic characteristics among patients with craniofacial FD, including McCune-Albright syndrome (MAS), polyostotic fibrous dysplasia (PFD), and monostotic fibrous dysplasia (MFD). We hypothesized that patients with MAS would show higher disease severity, have more complications, and undergo more operations than those with PFD or MFD. Patients and Methods A retrospective cohort study of patients with MAS or FD, evaluated at Massachusetts General Hospital from 2000 to 2018, was implemented. Patients of all ages and genders were identified through Massachusetts General Hospital Data Registries using International Classification of Diseases, Ninth Revision (ICD-9) and International Classification of Diseases, Tenth Revision (ICD-10) codes. Those with adequate clinical and radiographic data were included. Predictor variables were diagnosis of MAS, PFD, or MFD; age; and gender. Outcome variables included severity of disease at initial presentation (aggressive, nonaggressive and slow growing, or quiescent), number of operations, and complications: pain, sensory disturbances, pathologic fracture, airway obstruction, osteomyelitis, and dental findings. Data were analyzed with descriptive statistics and assessed for significance using χ2 tests and analysis of variance (P Results A total of 229 patients were identified: 114 had craniofacial FD, and 70 of these 114 (61.4%) met the inclusion criteria (48 of whom were female patients). The average age at diagnosis was 23.5 years; mean length of follow-up, 5.8 years. Diagnoses included MAS in 9 patients, PFD in 24, and MFD in 37. Signs and symptoms at initial presentation were pain (n = 29), sensory abnormalities (n = 13), facial deformity or swelling (n = 54), and dental findings (n = 25). At presentation, the biological behavior of disease was 77.8% aggressive, 11.1% nonaggressive, and 11.1% quiescent in the MAS group; 41.7%, 41.7%, and 16.7%, respectively, in the PFD group; and 29.7%, 29.7%, and 40.5%, respectively, in the MFD group. Patients with MAS were younger and were more likely to have pain, pathologic fractures, more bones involved, bilateral disease, and visual symptoms than those with PFD or MFD. MAS patients underwent more operations (mean, 4.2 ± 4.18) than those with PFD (mean, 2.6 ± 2.31; P = not significant) or MFD (mean, 1.7 ± 1.28; P = .010). Conclusions The results of this study indicate that patients with MAS, presumably with the same mutation, are more likely to have aggressive disease, complications, and more operations than those with PFD or MFD.
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- 2020
27. New technologies in oral radiology as a diagnostic aid for monostotic fibrous dysplasia: a review
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Ximena Torrico Acha and JHOANA LLAGUNO
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radiografía convencional ,Economics and Econometrics ,tomografía computarizada de haz cónico ,radiografía digital ,Forestry ,cone beam computed tomography ,conventional radiography ,Monostotic fibrous dysplasia ,gammagrafía ósea ,Materials Chemistry ,Media Technology ,magnetic resonance imaging ,bone scintigraphy ,digital radiography ,resonancia magnética ,displasia fibrosa monostótica - Abstract
Monostotic fibrous dysplasia is a benign asymptomatic lesion that affects only one bone, which is replaced by amorphous connective tissue. Clinically there is an increase in the volume of the affected area, which is observed by imaging as a radiopaque area with diffuse non-corticalized limits capable of expanding to neighboring structures, and it is histologically evidenced as “resembling Chinese characters”. The lesion is seen as a radiopaque image with diffuse borders in conventional or digital radiography, while cone beam computed tomography identifies the exact location and extension of an isodense, mixed or hyperdense image of non-corticalized edges. Magnetic resonance imaging is also used when the lesion involves soft tissues or nerves, and bone scintigraphy is performed in order to systemically observe bone quality. The objective of this article was to describe the new technologies in oral radiology for the diagnosis of monostotic fibrous dysplasia and the importance of the current imaging methods in achieving an adequate diagnosis. These techniques range from conventional radiography to bone scans, which provide images of higher quality, clarity and better precision with less invasive techniques to the patient. This review of the literature helps to expand the knowledge of dental professionals in relation to the clinical and imaging characteristics of monostotic fibrous dysplasia. La displasia fibrosa monostótica es una lesión benigna y asintomática que afecta solo a un hueso, que es reemplazado por tejido conectivo amorfo. Clínicamente, existe un aumento del volumen de la zona afectada, que se observa en la imagen como un área radiopaca con límites difusos no corticalizados capaces de expandirse a estructuras vecinas y se evidencia histológicamente como “semejanza de caracteres chinos”. La lesión se ve como una imagen radiopaca con bordes difusos en una radiografía convencional o digital, mientras que la tomografía computarizada de haz cónico identifica la ubicación exacta y la extensión de una imagen isodensa, mixta o hiperdensa de bordes no corticalizados. La resonancia magnética también se usa cuando la lesión involucra tejidos blandos o nervios, y se realiza una gammagrafía ósea para observar sistémicamente la calidad del hueso. El objetivo de este artículo fue describir las nuevas tecnologías en radiología oral para el diagnóstico de la displasia fibrosa monostótica y la importancia de los métodos de imagen actuales para lograr un diagnóstico adecuado. Estas técnicas van desde la radiografía convencional hasta las gammagrafías óseas, que brindan imágenes de mayor calidad, claridad y mejor precisión con técnicas menos invasivas para el paciente. Esta revisión de la literatura ayuda a ampliar el conocimiento de los profesionales de la odontología en relación con las características clínicas y de imagen de la displasia fibrosa monostótica.
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- 2022
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28. A Rare Case of Radiculopathy: Monostotic Fibrous Dysplasia of the Sacrum
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Diogo Simão, Joaquim Cruz Teixeira, Sérgio Livraghi, and José Pimentel
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Sacrum ,lcsh:R5-920 ,medicine.medical_specialty ,Bone disease ,Medullary cavity ,business.industry ,medicine.medical_treatment ,Fibrous dysplasia ,lcsh:R ,lcsh:Medicine ,General Medicine ,medicine.disease ,Monostotic fibrous dysplasia ,body regions ,Foraminotomy ,medicine ,Foramen ,Radiology ,Differential diagnosis ,Radiculopathy ,lcsh:Medicine (General) ,business ,Fibrous Dysplasia, Monostotic - Abstract
Fibrous dysplasia is a bone disease characterized by an osteoblastic dysfunction resulting in a fibrous replacement of the normal medullary bone. We describe the case of a 33-year-old who presented with low back pain irradiating to her right leg. Both the computed tomography scan and magnetic resonance imaging showed an osteolytic, multicystic lesion of the right hemi-sacrum with invasion of the right S1 foramen. She underwent foraminotomy and curettage of the lesion. Histological diagnosis was fibrous dysplasia, without features of malignant transformation. Three years after surgery the patient is asymptomatic and imaging is stable. This is the fifth known case of monostotic fibrous dysplasia involving the sacrum, a rare entity that must be considered in the differential diagnosis when approaching patients with sacral lesions.
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- 2019
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29. Successive generations with inherited craniofacial fibrous dysplasia.
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Charoenlarp, Pornkawee, Cholitgul, Wichitsak, Sinpitaksakul, Phonkit, Dhanuthai, Kittipong, and Sessirisombat, Somchai
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CRANIOFACIAL abnormalities ,FIBROUS dysplasia of bone ,MANDIBULAR condyle ,MEDICAL radiography ,RADIOGRAPHIC contrast media ,HISTOLOGY - Abstract
Craniofacial fibrous dysplasia is a benign fibro-osseous lesion of bone that only affects the bones of the craniofacial complex. Here, we report a case of craniofacial fibrous dysplasia in a 16-year-old Thai male who presented with mild swelling and tenderness at the mandibular right first molar area and ipsilateral nasal congestion. Conventional and cone-beam CT radiographic examinations were performed. The radiographs revealed multiple mixed radiolucent and radiopaque lesions involving most of the craniofacial bones. The first biopsy from the right mandibular area was diagnosed as juvenile ossifying fibroma, whereas a biopsy from the right maxillary area was diagnosed as fibrous dysplasia. The defects appeared to have a genetic basis, because his mother and younger brother had the same clinical and radiological findings. Furthermore, the family history given by his mother revealed that several other members of her family had similar clinical signs and symptoms. We diagnosed this case as inherited craniofacial fibrous dysplasia on the basis of previously reported clinical, radiographic and histologic findings as well as family history. [ABSTRACT FROM AUTHOR]
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- 2012
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30. Chondrosarcoma arising in monostotic fibrous dysplasia treated with total femur resection and megaprothesis: A case report
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Muhammad Wahyudi and Ziad Alaztha
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musculoskeletal diseases ,medicine.medical_specialty ,business.industry ,Fibrous dysplasia ,medicine.medical_treatment ,Chondrosarcoma ,Periosteal reaction ,Case Report ,medicine.disease ,Monostotic fibrous dysplasia ,Curettage ,Primary bone ,Malignant transformation ,Total femur resection ,medicine ,Osteosarcoma ,Surgery ,Femur ,Radiology ,business - Abstract
Introduction Fibrous dysplasia is tumor like lesions of bone which develop as substitution of bone by an expansion of fibrous connective tissue mixed with hard trabeculae. Chondrosarcomas is one of common malignant primary bone tumor derived from heterogenous group of neoplasm producing chondroid matrix. Chondrosarcoma arising in fibrous dysplasia, especially in monostotic fibrous dysplasia is a very rare case. Case report A 54-year-old male presented with chief complaint of pain on left thigh. Patient with history of pathological fracture on left femoral diaphysis 3 years ago due to fibrous dysplasia and had underwent curettage, open reduction, and internal fixation at other hospital. Plain radiography revealed expansive lytic lesion, interrupted periosteal reaction with plate and screw attached to the lesion, and soft tissue mass. MRI T2FS sequence showed hyperintense mass extending from subtrochanteric to distal of left femoral diaphysis. Histopathological result from biopsy suggested chondrosarcoma. Conclusions Malignant transformation of monostotic type was less frequently compared to polyostotic type. Among all malignant transformation cases, alteration to chondrosarcoma was more scarce than other malignancy such as osteosarcoma and fibrosarcoma. Wide surgical margin and reconstruction in chondrosarcoma provide good local control and functional outcome., Highlights • Chondrosarcoma arising in monostotic fibrous dysplasia is very rare. • Wide surgical margin provides good local control, thus become the mainstay treatment of chondrosarcoma. • Limb salvage surgery in chondrosarcoma provides good functional outcome.
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- 2021
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31. Monostotic fibrous dysplasia: a case report with cone-beam computed tomography findings
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Priscila Dias Peyneau, Francielle Silvestre Verner, Solange Maria de Almeida, Eliana Dantas da Costa, and Gláucia Maria Bovi Ambrosano
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Cone beam computed tomography ,business.industry ,Fibrous dysplasia ,Cone-beam computed tomography ,Mandible ,Anatomy ,medicine.disease ,Monostotic fibrous dysplasia ,Maxila ,Lesion ,stomatognathic diseases ,Fibrous dysplasia monostotic ,Maxilla ,Tomografia computadorizada de feixe cônico ,Medicine ,medicine.symptom ,Displasia fibrosa monostótica ,business ,General Dentistry ,Immature Bone ,Facial symmetry - Abstract
Fibrous dysplasia is a benign fibrous-osseous lesion in which normal bone is replaced by fibrous connective tissue and immature bone, affecting only one (monostotic) or several bones (polyostotic) and mainly occurring in children and young adults. When present in facial bones, the maxilla is more frequently involved than the mandible, which can cause facial asymmetry in addition to dental complications. In the image exams, the main characteristic of fibrous dysplasia is its unpolished glass appearance. Computed tomography is the ideal method for evaluating this lesion and its relationship with adjacent structures. The use of conventional radiography, due to the overlapping of anatomical structures, makes it difficult to delineate the extension of the lesion. The present study is aimed at guiding dentist-surgeons on the main imaging characteristics of fibrous dysplasia by describing a case of a female 10-year-old patient presenting with this lesion in the maxilla. RESUMO A displasia fibrosa é uma lesão fibro-óssea benigna em que o osso normal é substituído por tecido conjuntivo fibroso e osso imaturo, podendo afetar um único osso (monostótica) ou vários ossos (poliostótica), ocorrendo principalmente em crianças e adultos jovens. Quando presente nos ossos faciais, a maxila é envolvida com mais frequência que a mandíbula, podendo causar assimetria facial, além de complicações odontológicas. Nos exames por imagem a característica principal da displasia fibrosa é a aparência de vidro despolido. A tomografia computadorizada é o método ideal para avaliação dessa lesão e relação com estruturas adjacentes. A radiografia convencional, devido a sobreposição de estruturas anatômicas, torna difícil a delimitação da extensão da lesão. O presente estudo teve como objetivo apresentar o relato de caso de um paciente do sexo feminino, 10 anos de idade, com presença de displasia fibrosa na maxila, objetivando orientar os cirurgiões-dentistas com relação às principais características imaginológicas dessa lesão.
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- 2017
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32. A case of presumed monostotic sphenoid fibrous dysplasia and abducens nerve palsy
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Kara A. Dolezal and Hersh Varma
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Pathology ,medicine.medical_specialty ,Palsy ,business.industry ,Fibrous dysplasia ,medicine.disease ,Monostotic fibrous dysplasia ,Bone remodeling ,03 medical and health sciences ,Ophthalmology ,0302 clinical medicine ,Pediatrics, Perinatology and Child Health ,030221 ophthalmology & optometry ,Medicine ,Osteodystrophy ,business ,Esotropia ,Abducens nerve ,Subclinical infection - Abstract
Monostotic fibrous dysplasia is a rare osteodystrophy that typically affects adolescents, manifests later in life, and may be associated with subclinical hormonal imbalances. It can lead to serious complications, including visual compromise. We report a case of presumed monostotic sphenoid fibrous dysplasia presenting with transient abducens nerve palsy and esotropia in an otherwise healthy infant. The mechanism of transient, noncompressive abducens nerve palsy is not clearly understood but has been theorized to be secondary to localized inflammation during a cycle of florid bone remodeling that includes cyst formation, microhemorrhage, and resorption.
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- 2020
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33. Locally aggressive monostotic fibrous dysplasia of the cervical spine mimicking malignancy: a case report and literature review
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Audrey Milon, Guillaume Lot, Frédérique Larousserie, Marc Polivka, Jean-Denis Laredo, and Jean-Marc Ziza
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Aggressive ,medicine.medical_specialty ,medicine.medical_treatment ,Radiography ,Review Article ,Bone grafting ,Malignancy ,Monostotic fibrous dysplasia ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,lcsh:Orthopedic surgery ,Cervical spine ,Medicine ,Orthopedics and Sports Medicine ,Pathological ,Neck pain ,business.industry ,Fibrous dysplasia ,Pathological fracture ,medicine.disease ,Spine ,lcsh:RD701-811 ,Radiological weapon ,Surgery ,Radiology ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process. Owing to the vertebral instability, a surgical procedure combining C0–C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5–6 laxity below the intervertebral fixation. This case combines three radiological findings, which are unusual in fibrous dysplasia: monostotic presentation involving the spine, some aggressive radiographic features, and a pathological fracture.
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- 2019
34. Monostotic Fibrous Dysplasia of the Lumbar Spine With Secondary Features of Solid Variant Aneurysmal Bone Cyst
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Konstantinos Linos and Nolan Maloney
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0301 basic medicine ,Microbiology (medical) ,musculoskeletal diseases ,CDH11 ,Pathology ,medicine.medical_specialty ,Histology ,Fibrous dysplasia ,Pathology and Forensic Medicine ,Mazabraud ,03 medical and health sciences ,GNAS ,0302 clinical medicine ,GNAS complex locus ,medicine ,lcsh:Pathology ,Endocrine system ,McCune-Albright ,biology ,business.industry ,Brief Report ,Aneurysmal bone cyst ,medicine.disease ,Monostotic fibrous dysplasia ,030104 developmental biology ,aneurysmal bone cyst ,030220 oncology & carcinogenesis ,biology.protein ,Lumbar spine ,business ,ABC ,lcsh:RB1-214 - Abstract
Fibrous dysplasia is a benign, mass-forming disease of bone composed of abnormal fibrous and osseous elements that can be accompanied by endocrine dysfunction, skin pigmentation, and intramuscular myxomas. It is usually encountered as a solitary lesion in the tibia or femur but can develop in any bone and can be unifocal or multifocal. Difficulty arises when a solitary lesion is identified in an uncommon site or when there are prominent secondary changes, such as aneurysmal bone cyst (ABC). Molecular studies are available as an adjunct to histomorphology to aid distinction from other entities. GNAS mutations, present in greater than 70% of fibrous dysplasia cases, help in the distinction from primary ABC and low-grade osteosarcoma, which exhibit different molecular abnormalities. We report a case of monostotic fibrous dysplasia in a lumbar vertebral body with secondary change consisting of the solid variant of ABC.
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- 2019
35. Surgical Treatment of Polyostotic Craniomaxillofacial Fibrous Dysplasia Associated With Acromegalia: The McCune - Albright Syndrome
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Eliardo Silveira Santos, Jéferson Martins Pereira Lucena Franco, George Ferreira Gomes, Daniel Facó da Silveira Santos, and Roberto Dias Rêgo
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Adult ,medicine.medical_specialty ,Fibrous Dysplasia, Polyostotic ,McCune–Albright syndrome ,03 medical and health sciences ,0302 clinical medicine ,Acromegaly ,medicine ,GNAS complex locus ,Humans ,Polyostotic fibrous dysplasia ,030223 otorhinolaryngology ,Surgical treatment ,biology ,business.industry ,Fibrous dysplasia ,Cafe-au-Lait Spots ,030206 dentistry ,General Medicine ,medicine.disease ,Dermatology ,Monostotic fibrous dysplasia ,Otorhinolaryngology ,biology.protein ,Surgery ,Female ,business ,Congenital disorder ,Fibrous Dysplasia, Monostotic - Abstract
Introduction The McCune-Albright syndrome (MAS) is a complex congenital disorder caused by the embryonic post-zygotic somatic activating mutations in the GNAS1 gene. In such syndrome, phenotypes are heterogeneous and comprised polyostotic/monostotic fibrous dysplasia, cafe au lait macules, and hyperfunctioning endocrinopathies as the excess growth hormone. Likewise, acromegaly, as a manifestation of the endocrine hyperfunction, is unusual and affects about 20% of patients with MAS. Case presentation This research study describes a case of a 31-year-old female subject presenting polyostotic fibrous dysplasia with severe facial involvement, along with acromegaly and the MAS. The case was satisfactorily managed by surgical re-alignment and presented no clinical signs of relapse in a 12-year follow-up period. Finally, a literature review was conducted to discuss the standard protocols and the controversies when treating such cases. Conclusion Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial deformities that can be satisfactorily treated by cosmetic treatment, especially in patients with psychological problems and severe social acceptance.
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- 2019
36. Treatment of diaphyseal pathological fractures in children with monostotic fibrous dysplasia using cortical strut allografts and internal plating: A retrospective clinical study
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Chenjie Xia, Lei Wang, Kejian Lian, Huan Yu, Liangqi Kang, Peng Zhang, Qimiao Hu, and Dasheng Lin
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Male ,medicine.medical_specialty ,Humeral Fractures ,Observational Study ,Bone healing ,monostotic fibrous dysplasia ,03 medical and health sciences ,Fracture Fixation, Internal ,0302 clinical medicine ,Bone plate ,Fracture fixation ,medicine ,internal plating ,Humans ,030212 general & internal medicine ,Child ,Fixation (histology) ,Retrospective Studies ,Bone Transplantation ,business.industry ,Fibrous dysplasia ,Retrospective cohort study ,General Medicine ,diaphyseal pathological fractures ,medicine.disease ,cortical strut allograft ,Monostotic fibrous dysplasia ,Resorption ,Surgery ,Fractures, Spontaneous ,030220 oncology & carcinogenesis ,Female ,Diaphyses ,business ,Bone Plates ,Femoral Fractures ,Fibrous Dysplasia, Monostotic ,Research Article - Abstract
Children with fibrous dysplasia (FD) chronically suffer from pain, pathological fractures, and limb deformities. The most effective methods for managing the associated pathological fractures remain controversial. The purpose of this study was to evaluate the clinical results of the treatment of diaphyseal pathological fractures in children with monostotic fibrous dysplasia (MFD) using cortical strut allografts and internal plating. We retrospectively analyzed outcomes in nine children (5 boys, 4 girls) with diaphyseal pathological fractures due to MFD, who were treated with cortical strut allografts and internal plating (6 femoral fractures and 3 humeral fractures) between July 2007 and November 2012. The median age of patients in our study was 10 years (range 6–14 years). The fracture healing time, pain, extremity function, refracture, graft resorption, and complications were recorded to evaluate treatment effects. The median time of follow-up was 69 months (range 60–75 months). All patients had good postoperative fracture healing with a median healing time of 14 weeks (range 12–16 weeks). None experienced refracture, graft resorption, nerve injury, or limitation of extremity function or other complications. The fixation remained stable in all patients, with no evidence of loosening screws after surgery. In pediatric patients, the described surgical approach is an effective and reliable treatment method for diaphyseal pathological fractures caused by MFD. Cortical strut allografts, which act as biological bone plates, can provide good mechanical support while increasing the rate of fracture union.
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- 2019
37. Surgical Treatment Modalities in Pediatric Monostotic Fibrous Dysplasia of Proximal Femur - A Case Series.
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Tathe PV, Banik S, and Mandal S
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Introduction: Monostotic fibrous dysplasia is a rare genetic non-inherited orthopedic condition presenting at any age with variable presentation. Proximal femur being the most common site, the mechanical factor predisposes it to increased chances of pathological fracture, which makes it challenging to choose an appropriate treatment modality and implant selection in children., Case Presentation: In this retrospective case series, six children aged 7-12 years with monostotic fibrous dysplasia with or without fracture were treated with different treatment modalities from 2015 to 2020. Extended curettage and bone grafting and stabilization were done with extramedullary implants such as locking plates and DHS. Autograft alone or combination with allograft was chosen according to size of lesion. Patients without fracture were treated with curettage and artificial bone graft substitute with hip spica. Patients were followed up to 12 months. Revised Musculoskeletal Tumor Society (MSTS) score at each follow-up and Toronto Extremity Salvage Score (TESS) at final follow-up., Results: Mean fracture healing time was 14.8 ± 2.28 weeks. in patients with fracture. Full weight-bearing was started at average 15.67 ± 2.94 weeks. One patient had shortening of 1 cm but none had any surgical site infection, loss of correction, or varus collapse more than 5°. Mean revised MSTS at 12 months was 24.2 ± 2.28 in patients with curettage, bone grafting, and internal fixation, while it was 27 in patients with curettage, grafting with hip spica. Mean TESS was found to be 90 ± 9.41 in internal fixation group, while it was 95 in patient with curettage, grafting with hip spica., Conclusion: Treatment modality should be chosen wisely after analyzing pre-operative radiograph, lesion size, presence of pathological fracture, and each patient profile. Extramedullary fixation devices can be an alternate choice of implant in children and adolescents for monostotic fibrous dysplasia. Long duration follow-up and patient counseling should be done for recurrence of lesion and deformities., Competing Interests: Conflict of Interest: Nil, (Copyright: © Indian Orthopaedic Research Group.)
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- 2022
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38. Monostotic fibrous dysplasia of fibula: a rare case report
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Bhupes Sil, V Naveen, and Dipen Roy
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medicine.medical_specialty ,Adamantinoma ,business.industry ,Fibrous dysplasia ,Simple Bone Cyst ,medicine.medical_treatment ,medicine.disease ,Monostotic fibrous dysplasia ,Curettage ,Dysplasia ,medicine ,Tibia ,Radiology ,Fibroma ,business - Abstract
Fibrous dysplasia is a developmental anomaly of bone formation that may exist in monostotic or polyostotic form. Monostotic fibrous dysplasia of fibula is a rare case with unusual site and most common sites being facial bones, ribs, proximal femur and tibia. We report a case of 10-year-old boy attended our hospital with occasional pain in left leg while having strenuous activity. On clinical examination there was no obvious swelling with normal appearing skin. Radiographic examination revealed lesion with both radiopaque and radiolucent features showing a “ground-glass” appearing lesion in proximal one third of fibula. Differential diagnosis considered are simple bone cyst, non-ossifying fibroma, osteo-fibrous dysplasia, adamantinoma. As a definitive treatment curettage was done and biopsy sent for histopathological examination, which confirmed he diagnosis. Follow up after 10 months shows normal bone growth as evidenced radiologically and patient is asymptomatic. Case in detail and recent review of literature has been discussed. From our case, we have shown that it is possible to treat uncomplicated fibrous dysplasia with minimally invasive approach of simple curettage. Patient is symptomatically relieved within 2 months without any complications.
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- 2021
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39. Monostotic fibrous dysplasia with Raynaud's phenomenon.
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Kumar, K. V. S. Hari, Aravinda, K., and Narayanan, K.
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FIBROUS dysplasia of bone , *RAYNAUD'S disease , *PERIPHERAL vascular diseases , *MUSCULOSKELETAL system diseases - Abstract
Fibrous dysplasia (FD) is a benign bone disorder characterized by alteration in bone morphology. Monostotic FD is the commonest variant and affects the craniofacial bones. Raynaud's phenomenon is recurrent vasospasm of the fingers and toes due to cold exposure. The disease is usually idiopathic or secondary to connective tissue disorders. Raynaud's phenomenon is not described previously with FD. We recently encountered two interesting patients of craniofacial monostotic FD with Raynaud's phenomenon and report the same in this report. [ABSTRACT FROM AUTHOR]
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- 2015
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40. Maxillo-mandibular Contouring Surgery in Monostotic Fibrous Dysplasia Patients using Simulation Surgery
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You Myoung-Sang, Ahn Kang-Min, and Kim Dong-Young
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medicine.medical_specialty ,Contouring ,business.industry ,Fibrous dysplasia ,Medicine ,Jaw bone ,business ,medicine.disease ,Monostotic fibrous dysplasia ,Surgery - Published
- 2016
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41. Monostotic fibrous dysplasia of the metacarpal: a case report
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Katia Torres Batista, Ulises Prieto y Schwartzman, and Hugo José de Araújo
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medicine.medical_specialty ,Bone disease ,Radiography ,lcsh:Medicine ,Physical examination ,Case Report ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,lcsh:Orthopedic surgery ,Biopsy ,Cystic fibrous osteitis ,medicine ,Disease ,Doença ,Rib cage ,medicine.diagnostic_test ,business.industry ,Fibrous dysplasia ,lcsh:R ,General Medicine ,medicine.disease ,Osteíte fibrosa cística ,Monostotic fibrous dysplasia ,Displasia fibrosa óssea ,Surgery ,Fibrous dysplasia of bone ,lcsh:RD701-811 ,030220 oncology & carcinogenesis ,Differential diagnosis ,Metacarpus ,business ,Metacarpo - Abstract
Fibrous dysplasia is a bone disease characterized by abnormal differentiation of fibrous tissue in the bones; it is often asymptomatic. It may affect one bone (monostotic) or several bones (polyostotic). The monostotic form primarily affects the ribs, but hardly ever affects the hand. It is important to make the differential diagnosis with malignant bone tumors. This article describes the treatment and outcome of a rare case of a patient admitted with a history of tumor growth in the right hand, diagnosed as fibrous dysplasia of the right second metacarpal. Male patient, 14 years of age, admitted to the Sarah Hospital with lesion on the dorsum of the right hand without pain complaints, previous history of trauma, nor local signs of inflammation. Physical examination revealed swelling on the dorsum of the second metacarpal, painless, with unaltered mobility and sensitivity. Radiography, computed tomography, and magnetic resonance imaging indicated the involvement of the entire length of the second metacarpal: only the distal epiphysis was preserved, with areas of bone lysis. After biopsy confirmation, the patient underwent surgery, using a long cortical graft for reconstructing the metacarpal. During the follow-up period of five years there were no signs of recurrence, and proper digital growth and functionality of the operated hand were observed.
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- 2016
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42. MONOSTOTIC FIBROUS DYSPLASIA PRESENTING IN MAXILLA:A CASE REPORT
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Mehmet Ali Erdem, Ilknur Ozcan Duman, Taha Emre Köse, Abdulkadir Burak Cankaya, and Onur Dincer Kose
- Subjects
medicine.medical_specialty ,Radiography ,Monostotic ,Fibrous dysplasia ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Fibrous displasia,monostotic ,maxilla ,medicine ,Maxilla ,Craniofacial ,medicine.diagnostic_test ,business.industry ,Articles ,030206 dentistry ,Biological Sciences ,medicine.disease ,Monostotic fibrous dysplasia ,Surgery ,lcsh:RK1-715 ,Normal bone ,Bone scintigraphy ,Dentistry ,lcsh:Dentistry ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
Fibrous dysplasia (FD) is a bone disorder in which fibrous tissue replaces normal bone. FD can be monostotic (10%–25%) or polyostotic (50–90%) and is typically diagnosed accidentally on radiographs. Craniofacial lesions are typically unilateral and are diagnosed by clinical assessment and radiographic evaluation. This report describes the case of a 50-year old female patient who had presented with painless swelling of the right maxilla and was diagnosed with FD. Subsequent bone scintigraphy identified the lesion as monostotic. Based on the patient’s age and the radiographic data, contour correction was performed. However, 1 year after surgery, the lesion regrew and the treatment was repeated.
- Published
- 2016
43. Recurrent Monostotic Fibrous Dysplasia in the Mandible
- Author
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Naira Figueiredo Deana, Reinaldo José de Oliveira, Denise Takehana, and Nilton Alves
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medicine.medical_specialty ,business.industry ,Fibrous dysplasia ,Mandible ,RK1-715 ,Case Report ,030206 dentistry ,medicine.disease ,Monostotic fibrous dysplasia ,Surgery ,Fibrous connective tissue ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Normal bone ,Dental clinic ,Dentistry ,030220 oncology & carcinogenesis ,medicine ,medicine.symptom ,Radical surgery ,business ,General Dentistry - Abstract
Fibrous dysplasia (FD) is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue. Female patient, white, 20 years old, attended the dental clinic reporting a slow increase in volume in the right mandible region over the last 5 years. She was examined by imaging: the panoramic X-ray revealed a lesion with the appearance of ground glass while the cone-beam computed tomography showed an extensive lesion in the region of the right hemimandible. The histopathological examination was compatible with fibrous dysplasia. Bone gammagraphy was indicated, plus an endocrinological study to eliminate polyostotic forms, which produced a negative result. Monostotic fibrous dysplasia in the right hemimandible was diagnosed. Conservative surgery was carried out and after 1 year recurrence of the tumour was observed. We may conclude that conservative surgery might not be the best choice for treatment for monostotic fibrous dysplasia in the mandible and that other options must be considered, such as radical surgery or the use of bisphosphonates. In our study, we may also conclude that it is very important to explain to the patient the possibility of recurrence of the lesion and the need for monitoring with periodic imaging studies.
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- 2016
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44. Chondrosarcoma arising in monostotic fibrous dysplasia treated with total femur resection and megaprothesis: A case report.
- Author
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Wahyudi, Muhammad and Alaztha, Ziad
- Abstract
Fibrous dysplasia is tumor like lesions of bone which develop as substitution of bone by an expansion of fibrous connective tissue mixed with hard trabeculae. Chondrosarcomas is one of common malignant primary bone tumor derived from heterogenous group of neoplasm producing chondroid matrix. Chondrosarcoma arising in fibrous dysplasia, especially in monostotic fibrous dysplasia is a very rare case. A 54-year-old male presented with chief complaint of pain on left thigh. Patient with history of pathological fracture on left femoral diaphysis 3 years ago due to fibrous dysplasia and had underwent curettage, open reduction, and internal fixation at other hospital. Plain radiography revealed expansive lytic lesion, interrupted periosteal reaction with plate and screw attached to the lesion, and soft tissue mass. MRI T2FS sequence showed hyperintense mass extending from subtrochanteric to distal of left femoral diaphysis. Histopathological result from biopsy suggested chondrosarcoma. Malignant transformation of monostotic type was less frequently compared to polyostotic type. Among all malignant transformation cases, alteration to chondrosarcoma was more scarce than other malignancy such as osteosarcoma and fibrosarcoma. Wide surgical margin and reconstruction in chondrosarcoma provide good local control and functional outcome. • Chondrosarcoma arising in monostotic fibrous dysplasia is very rare. • Wide surgical margin provides good local control, thus become the mainstay treatment of chondrosarcoma. • Limb salvage surgery in chondrosarcoma provides good functional outcome. [ABSTRACT FROM AUTHOR]
- Published
- 2021
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45. Thoracoscopic removal of monostotic fibrous dysplasia of rib
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Harsh Vardhan Puri, Cholenhali Lingaraju Vijay, Belal Bin Asaf, and Arvind Kumar
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Rib cage ,medicine.diagnostic_test ,business.industry ,Fibrous dysplasia ,030204 cardiovascular system & hematology ,medicine.disease ,Monostotic fibrous dysplasia ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,Normal bone ,030228 respiratory system ,Cardiothoracic surgery ,medicine ,Thoracoscopy ,Abnormality ,Cardiology and Cardiovascular Medicine ,business ,Immature Bone - Abstract
Fibrous dysplasia is a non-inherited, skeletal development abnormality, in which normal bone is replaced by fibrous tissue and poorly formed immature bone. It is a tumour which affects ribs in almost half the cases, 75 % are monostotic and 25 % can be polyostotic. These tumours are usually benign but may turn malignant in 0.4% to 4% of cases. Recommended treatment is resection of involved segment with clear margins. Conventionally it is done by open method; we herein report a case of Thoracoscopic removal of such tumor with success and uneventful postoperative course.
- Published
- 2017
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46. Fibrous Dysplasia in the Epiphysis of the Distal Femur
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Jae Woo Lee and Jung Ho Noh
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030222 orthopedics ,business.industry ,Fibrous dysplasia ,Long bone ,Case Report ,030229 sport sciences ,Anatomy ,Metaphysis ,medicine.disease ,Monostotic fibrous dysplasia ,Lesion ,03 medical and health sciences ,Diaphysis ,0302 clinical medicine ,medicine.anatomical_structure ,Epiphysis ,Medicine ,Orthopedics and Sports Medicine ,Surgery ,Femur ,medicine.symptom ,business - Abstract
Fibrous dysplasia is a common benign skeletal lesion that may involve a single bone or multiple bones. Although fibrous dysplasia can affect any bone, monostotic fibrous dysplasia of the long bone typically occurs in the diaphysis or metaphysis. We report a very rare case of monostotic fibrous dysplasia involving the epiphysis of the distal femur in a young man.
- Published
- 2017
47. Inferior alveolar nerve preservation for hemimandibulectomy and bridging plate reconstruction in monostotic fibrous dysplasia case
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Agus Widodo, Muhammad Masykur Rahmat, Cahya Yustisia Hasan, and Prihartiningsih Prihartiningsih
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Bridging (networking) ,Hemimandibulectomy ,business.industry ,Medicine ,Anatomy ,Inferior alveolar nerve ,business ,medicine.disease ,Monostotic fibrous dysplasia - Published
- 2020
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48. Image-guided resection of lumbar monostotic fibrous dysplasia: A case report and technical note
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Adetokunbo A. Oyelese, Ziya L. Gokaslan, Jared Fridley, Rahul A. Sastry, and Albert E. Telfeian
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medicine.medical_specialty ,business.industry ,Fibrous dysplasia ,Radiography ,Case Report ,Technical note ,medicine.disease ,Intraoperative computed tomography ,Asymptomatic ,Navigation ,Spine ,Monostotic fibrous dysplasia ,Resection ,Lumbar ,medicine ,Surgery ,Lumbar spine ,Neurology (clinical) ,Radiology ,medicine.symptom ,business - Abstract
Background: Monostotic fibrous dysplasia rarely involves the lumbar spine. Although its optimal surgical management is unknown, some recommend complete resection to decrease the likelihood of future recurrence. Case Description: A 41-year-old female presented with fibrous dysplasia involving the right L4 lamina and spinous process. Following image-guided en bloc resection, the patient remained asymptomatic without evidence of recurrence 8 months later. Conclusion: Image-guided excision of monostotic fibrous dysplasia involving the right L4 lamina and spinous process was successfully performed without clinical or radiographic evidence of recurrence within 8 postoperative months.
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- 2020
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49. MONOSTOTIC FIBROUS DYSPLASIA: A CLINICAL CASE REPORT
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Hélder Lima Rebelo, Darceny Zanetta Barbosa, Maiolino Thomaz Fonseca Oliveira, Willames Rafael De Jesus Cavalcante, Hugo Franklin Lima De Oliveira, Luiz Fernando Barbosa de Paulo, and Camilla Lins Dos Anjos
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Pathology ,medicine.medical_specialty ,Bone development ,business.industry ,Radiography ,Usually asymptomatic ,medicine.disease ,Monostotic fibrous dysplasia ,Pathology and Forensic Medicine ,Lesion ,Normal bone ,medicine ,Radiology, Nuclear Medicine and imaging ,Dentistry (miscellaneous) ,Surgery ,Clinical case ,Oral Surgery ,medicine.symptom ,business ,Facial symmetry - Abstract
The monostotic fibrous dysplasia is a benign fibro-osseous lesion of bone development, usually asymptomatic, which affects only 1 bone and is characterized by the substitution of the normal bone by an excessive proliferation of the fibrous connective tissue. In almost all cases there are deformities that cause aesthetic and functional disorders. The diagnosis is based on clinical, radiographic, and histologic characteristics. The facial asymmetry is the main clinical change, which is radiographically observed as a radiopaque appearance with frosted glass appearance, and histologically it is verified by the excessive proliferation of bone-forming mesenchymal cells. The objective of the present study is to report a case of a monostotic fibrous dysplasia in the middle third of the face, causing facial asymmetry and treated by a conservative surgery with good aesthetic results.
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- 2020
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50. RADIOGRAPHIC CHARACTERISTICS OF MONOSTOTIC FIBROUS DYSPLASIA: CASE REPORT
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Isabela Moreira Pio, Vânio Santos Costa, Millena Jacinto de Oliveira, Kelly de Moura Ferreira, Eduardo de Oliveira Leão, Regina Fernanda Tavares De Lima, and Douglas Ferreira Da Silva
- Subjects
Pathology ,medicine.medical_specialty ,business.industry ,Fibrous dysplasia ,Radiography ,medicine.disease ,Bone tissue ,Monostotic fibrous dysplasia ,Pathology and Forensic Medicine ,Fibrous connective tissue ,medicine.anatomical_structure ,Dysplasia ,medicine ,Etiology ,Radiology, Nuclear Medicine and imaging ,Dentistry (miscellaneous) ,Surgery ,Oral Surgery ,business ,Facial symmetry - Abstract
Fibrous dysplasia is related to benign fibro-osseous lesions characterized by the progressive replacement of the original bone tissue by fibrous connective tissue. This condition may be present in a single bone, described as monostotic fibrous dysplasia, or in up to 75% of the whole body, described as polyostotic. Its etiology is unknown; however, studies report a relation between the enzymatic and metabolic modifications and genetic mutations. It is more common in the first 2 decades of life, with no prevalence of sex. It is clinically a painless tumefaction, promoting facial asymmetry. Radiographically, it presents as a "glass-matte" aspect opacification. The proposed treatment can be surgical or conservative, depending on the functional and aesthetic impairment. This study aims to report a case and to present the tomographic and anatomic aspects of monostotic fibrous bone dysplasia in a 14-year-old female patient.
- Published
- 2020
- Full Text
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