A Case Series and Literature Review of Craniofacial Fibrous Dysplasia.

Craniofacial fibro-osseous lesions (CFOLs) are a diverse group of relatively rare entities whose etiology ranges from reactive to dysplastic with a potential for malignant transformation. It is distinguished by the replacement of bone with fibrous tissue, that subsequently develops different degrees of calcification. Fibrous dysplasia (FD) is a component of the fibro-osseous lesion spectrum. The clinical spectrum of FD is wide, ranging from minor monostotic lesions affecting a single bone to devastating polyostotic disease involving the entire skeleton. FD produces asymmetry, which impairs face aesthetics. FD leads to bone differentiation, disintegration, and disorganization. It depicts a cellular collagenous stroma lacking mitotic figures and pleomorphism. Blood capillaries are evenly distributed, as are elongated trabeculae of woven or lamellar bone with uneven curves (often referred to as the Chinese letters pattern). Three types of FD patterns can be identified by computed tomography (CT) imaging: a cystic pattern, a homogeneously dense pattern, and a ground-glass pattern. The cornerstone of treatment is surgery, although the method varies depending on the location, size, and symptoms of the lesion. As an alternative to surgery, the use of bisphosphonates to reduce osteoclastic activity is under consideration. In this case series, we present three cases of FD involving the maxilla and mandible. We aim to correlate the clinical presentation, histological features, and radiographic findings, to promote early diagnosis, treatment, and better prognosis of the patient.

Diagnosis and Management of Monostotic Fibrous Dysplasia of the Tibia in an Adolescent Patient: A Case Report.

A rare benign bone condition called monostotic fibrous dysplasia (MFD) is characterized by the growth of fibrous tissue in place of a normal bone. It may lead to deformity in the affected bone, pain, and a pathologic fracture due to bone weakness. Hereunder, a case report of MFD in a 17-year-old male adolescent presenting to the hospital with localized bone pain and swelling in his right tibia is presented. After clinical examination and radiographic imaging, a provisional diagnosis of benign osteolytic lesion was considered. A magnetic resonance imaging (MRI) scan of the leg suggested the possibility of fibrous dysplasia or adamantinoma. The patient was managed with an intralesional curettage of the dysplastic bone and packing the cavity with blocks of a synthetic bone. The excised material was sent for histopathology, which established the diagnosis of fibrous dysplasia.

A Rare Case of Monostotic Fibrous Dysplasia of the Femoral Neck With Pathological Fracture: A Case Report.

Fibrous dysplasia is a rare benign bone disorder characterized by the replacement of normal bone with fibroblastic and osteoblastic tissue. We present a case of monostotic fibrous dysplasia in a 25-year-old male patient. The case highlights the clinical presentation, radiographic features, and management approach for this condition. This report aims to contribute to the understanding of fibrous dysplasia and its management options. A 25-year-old male presented with a chief complaint of persistent left hip pain. The pain was described as a dull ache, associated with difficulty in weight-bearing activities. There was no history of trauma or constitutional symptoms. Physical examination revealed externally rotated left lower limb. Range of motion of the left hip could not be assessed due to pain, with no neurological deficits noted. Initial imaging included plain radiographs of the right femur, which demonstrated a radiolucent lesion with a ground-glass appearance and cortical thinning. Magnetic resonance imaging of both hip joints reveals an irregular T2 hyperintense and T1 hypointense lesion involving the left femoral neck; moreover, few tiny cystic spaces are seen within the lesion. Part of the lesion is extending into the superior-lateral aspect of the femoral head and surrounding bone marrow edema with minimal left hip joint effusion, features suggestive of a primary bony tumour. Plain computed tomography (CT) of the hip joint and pelvis was suggestive of an expansile lytic lesion with thin bony septation within and thick sclerotic margin in the left femoral head and greater trochanter associated with sub-capital femoral neck fracture suggestive of bone neoplasm (? giant cell tumour > simple bone cyst). A bone biopsy was performed, and histopathological examination confirmed the diagnosis of fibrous dysplasia, with characteristic woven bone and fibrous stroma. In this case, after confirming the diagnosis, the patient was managed with total hip arthroplasty on the left side. Monostotic fibrous dysplasia is a rare benign bone disorder that can present with various clinical manifestations. Timely diagnosis through a combination of clinical, radiographic, and histopathological assessments is crucial. Management should be tailored to the patient's symptoms.

Aneurysmal bone cyst arising from the surgically removed craniofacial fibrous dysplasia in the long-term follow-up: a case report.

Fibrous dysplasia (FD) is a rare skeletal disorder characterized by abnormal fibro-osseous connective tissue replacing normal bone. Despite its benign behavior, craniofacial FD can cause morphological disfigurement, headache, and even blindness as a result of the produced mass effect. Surgical resection is recommended when the patient shows apparent clinical symptoms or aggravating facial asymmetry. Postoperative complications have been reported, such as hematoma, surgical site infection, abscess formation, resorption of the bone graft used for reconstruction, and recurrence. An aneurysmal bone cyst (ABC) is a rare benign bony lesion that can occur secondary to preexisting bone tumor. Secondary ABCs in craniofacial FD are extremely rare in the literature, accounting for less than 30, all of which are either case reports or series. We report an extremely rare case of symptomatic secondary ABC arising from craniofacial FD that had been misdiagnosed with abscess formation or recurrence and was surgically removed. Notably, 17 years elapsed between the primary surgery and the complication of secondary ABC. The patient underwent total removal of secondary ABC. After surgery, symptoms were relieved, with no recurrence observed during a 6-month follow-up.

Fibrous Dysplasia of the Clivus: Case Report and Literature Review.

Fibrous dysplasia is a benign, developmental bone disorder that causes fibrous replacement of normal skeletal tissue. This may lead to weakness, distortion, and tissue expansion. Fibrous dysplasia can occur anywhere in the body, including the craniofacial area. The clivus is a central skull bone formed by the bases of the sphenoid and occiput, respectively. The clivus is a rare, usually unrecognized, and seldom reported location for the development of fibrous dysplasia. Although fibrous dysplasia of the clivus (FDC) is usually discovered by incidental findings, it can sometimes present with clinical symptoms. In this case, we discuss a 30-year-old male who presents to the emergency room with headaches, altered mental status, and a prior presentation of location-related symptomatic epilepsy. Magnetic resonance imaging depicted a mass in the clivus, low in signal on T1 and mildly hypointense on T2 imaging. Follow-up computed tomography (CT) imaging, as recommended, revealed the classic presentation of FDC. In this paper, we discuss the significance of this condition and the importance of thorough investigation to rule out differential diagnoses that may present with similar acute symptoms as this patient.

Current concepts of craniofacial fibrous dysplasia: pathophysiology and treatment.

Fibrous dysplasia is an uncommon genetic disorder in which bone is replaced by immature bone and fibrous tissue, manifesting as slowgrowing lesions. Sporadic post-zygotic activating mutations in GNAS gene result in dysregulated GαS-protein signaling and elevation of cyclic adenosine monophosphate in affected tissues. This condition has a broad clinical spectrum, ranging from insignificant solitary lesions to severe disease. The craniofacial area is the most common site of fibrous dysplasia, and nine out of 10 patients with fibrous dysplasia affecting the craniofacial bones present before the age of 5. Surgery is the mainstay of treatment, but the technique varies according to the location and severity of the lesion and associated symptoms. The timing and indications of surgery should be carefully chosen with multidisciplinary consultations and a patient-specific approach.

Monostotic Paget's Disease Involving the Scapula Encountered Incidentally on a Trans-arterial Aortic Valve Implantation Scan.

Paget's disease of the bone is a chronic bone disorder. However, to our knowledge, only a few cases of Paget's disease with isolated scapular involvement have been documented in the literature. In this case study, we describe an 81-year-old male patient who was incidentally diagnosed with monostotic Paget's disease of the left scapula during a computed tomography scan for the placement of a trans-arterial aortic valve. We discuss how crucial it is to recognise typical imaging appearances in order to prevent unnecessary investigations and interventions.

Surgical Treatment Modalities in Pediatric Monostotic Fibrous Dysplasia of Proximal Femur - A Case Series.

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.

[The experience of interdisciplinary interaction in elimination of midface deformation (clinical observation)]. / Opyt mezhdistsiplinarnogo vzaimodeistviya pri ustranenii deformatsii srednei zony litsa (klinicheskoe nablyudenie).

Fibrous dysplasia is a hamartoma appearing as a benign pathologic process in which normal bone tissue formation is replaced by overgrowth of fibrous connective tissue. The problem of diagnosis and treatment of patients with fibrous dysplasia remains unsolved in the absence of a uniform understanding of its etiology and pathogenesis. Treatment and rehabilitation of patients with midface fibrous dysplasia is a complicated medico-social problem, which requires interaction of specialists of different fields to resolve it. The disease presents a practical interest for doctors of more than 20 specialties, as it can lead to disability. The present article describes a clinical case of interdisciplinary interaction of specialists in the fields of maxillofacial surgery, ophthalmology, otorhinolaryngology, plastic surgery and radiology involved in complex treatment of a patient with midface fibrous dysplasia accompanied by eyeball dislocation, decreased vision, as well as progressive impairment of nasal breathing function. The performed treatment resulted in favorable functional and aesthetic outcomes including elimination of exophthalmos, vertical strabismus, lacrimation disorders, nasal breathing disorders, increase of visual acuity and improvement of external appearance.

Wedge Osteotomy with Tens Nailing in Monostotic Fibrous Dysplasia of Tibia - A Case Report.

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.

Tratamiento multidisciplinario en un niño afectado por un quiste dentígero / Multidisciplinaty treatment in a child with dentigerous cyst

Se presenta el caso clínico de un paciente que a los 7 años de edad fue remitido a la Clínica Estomatológica Provincial Docente Mártires del Moncada de Santiago de Cuba por presentar un quiste dentígero y displasia fibrosa monostótica. Desde entonces, y durante casi 10 años, el paciente ha sido atendido por un equipo multidisciplinario odontopediátrico, que ha seguido su evolución y ha aplicado diferentes protocolos diagnósticos y terapéuticos; estos últimos han incluido intervenciones quirúrgicas, rehabilitaciones protésicas, quimioterapia, entre otros. Por la complejidad del caso y la poca frecuencia con que aparecen asociadas ambas entidades clínicas en la infancia, se decidió comunicar este artículo al gremio odontológico nacional y extranjero.

The case report of a seven years old child who was referred to Mártires del Moncada Provincial Teaching Stomatological Clinic in Santiago de Cuba, who had a dentigerous cyst and monostotic fibrous displasia is presented. Since then, and almost during 10 years, the patient has been assisted by a multidisciplinary odontopediatric team, which has followed his clinical course and has applied different diagnostic and therapeutic protocols, including surgical procedures, prosthetic rehabilitations, chemotherapy, among others. Due to the case complexity and the frequency with which both clinical entities are associated in childhood, it was decided to publish this work for the national anf foreing odontological community.

Chondrosarcoma arising in monostotic fibrous dysplasia treated with total femur resection and megaprothesis: A case report.

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.

Imaging characteristics of a rare case of monostotic fibrous dysplasia of the sacrum: A case report.

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.

Displasia fibrosa en seno etmoidal y esfenoidal: a propósito de un caso

Resumen La displasia fibrosa es una enfermedad del hueso, benigna, idiopática, de base genética y de progresión lenta, que se caracteriza por el reemplazo progresivo del hueso normal con tejido fibrótico, entremezclado con trabéculas óseas irregulares. El cráneo también es un sitio frecuente de afectación, los huesos del complejo craneofacial, incluida la mandíbula, el maxilar, la base y la bóveda craneal, son los principalmente afectados. Los huesos etmoidales, esfenoidales, frontales y temporales son afectados con poca frecuencia. En este artículo se presenta un caso de una paciente con cefalea y dolor en hemicara derecha, exoftalmos y edema periorbitario ipsilateral. Luego del examen físico, se realizó tomografía computarizada y biopsia del tumor, llegando al diagnóstico de displasia fibrosa de seno etmoidal y esfenoidal. Se maneja en forma conservadora, con seguimiento cada 6 meses para evaluar evolución. Es un caso con una localización infrecuente, y que debe ser cuidadosamente evaluado para adoptar la conducta terapéutica correcta.

Abstract Fibrous dysplasia is a slowly progressive, genetically based, benign, idiopathic bone disease characterized by progressive replacement of normal bone with fibrotic tissue, interspersed with irregular bone trabeculae. The skull is also a frequent site of involvement, the bones of the craniofacial complex, including the mandible, the maxilla, the base and the cranial vault, are mainly affected. The ethmoid, sphenoid, frontal, and temporal bones are affected at a low rate. This article presents a case of a patient with headache and pain in the right side, exophthalmos, and ipsilateral periorbital edema. After the physical examination, a computed tomography and biopsy of the tumor were performed, reaching the diagnosis of fibrous dysplasia of the ethmoid and sphenoid sinus. Conservative management is given, with follow-up every 6 months to assess evolution. It is a case with an infrequent location, and it must be carefully evaluated to take therapeutic behavior.

New technologies in oral radiology as a diagnostic aid for monostotic fibrous dysplasia: a review.

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.

Displasia fibrosa monostótica craneofacial / Craniofacial monostotic fibrous dysplasia

La displasia fibrosa es una enfermedad benigna del hueso, de base genética y de progresión lenta, que se caracteriza por el reemplazo progresivo del hueso normal por tejido fibrótico. El proceso puede afectar un solo hueso o múltiples huesos (displasia fibrosa monostótica o poliostótica); esta última puede estar asociada con enfermedades sindrómicas, como el síndrome de McCune-Albright. Objetivo: revisar el diagnóstico y tratamiento de los pacientes con displasia fibrosa craneofacial. Materiales y métodos: se realizó una revisión de artículos científicos en revistas indexadas en línea como técnica para la recolección de información relevante en bases de datos como SciELO, Google Académico, Scopus, PubMed y DynaMed, con artículos publicados en inglés y español. El período de revisión tuvo como límite el 1 de junio de 2020. Los términos de búsqueda fueron: "displasia fibrosa", "displasia fibrosa monostótica" y "displasia fibrosa craneofacial". Se obtuvieron 33 artículos. Conclusión: la displasia fibrosa monostótica craneofacial es una enfermedad rara que afecta principalmente la mandíbula, el hueso maxilar y el cornete inferior. Puede cursar asintomática o presentar deformidades faciales, dolor y trastornos visuales. Su diagnóstico es principalmente por estudios de imágenes y la biopsia. El tratamiento quirúrgico debe ser bien planeado en cada uno de los pacientes y enfocado a deformidades faciales, se deben evitar los trastornos visuales y es necesario cuando se maligniza la lesión.

Fibrous dysplasia is a slowly progressive, genetically based, benign bone disease characterized by progressive replacement of normal bone with fibrotic tissue. The process can affect a single bone or multiple bones (monostotic or polyostotic fibrous dysplasia); the latter may be associated with syndromic diseases, such as McCune-Albright syndrome. Objective to review the diagnosis and treatment of patients with fibrous craniofacial dysplasia. Materials and methods a review of scientific articles was carried out in online indexed journals as a technique for collecting relevant information in databases such as SciELO, Google Scholar, Scopus, PubMed, and DynaMed, with articles published in English and Spanish. The review period was limited to June 1, 2020. The search terms were: "fibrous dysplasia", "monostotic fibrous dysplasia", and "craniofacial fibrous dysplasia". Obtaining 33 articles. Conclusion craniofacial monostotic fibrous dysplasia is a rare disease, mainly affecting the mandible, the maxillary bone, and the inferior turbinate. It can be asymptomatic or present facial deformities, pain and visual disorders. Its diagnosis is mainly by imaging studies and biopsy. Surgical treatment must be well planned in each of the patients, focused on facial deformities, avoiding visual disorders and is necessary when the lesion is malignant.

Mickey Mouse Sign on Bone Scan in the Monostotic Form of Paget's Disease Mimicking Osseous Metastasis

Paget's disease is a chronic benign bone disease characterized by excessive and abnormal bone remodeling. Monostotic Paget's disease accounts for only 20% of the cases, and the monostotic form involving the vertebra with the Mickey Mouse sign is very rare. Herein, we report a case of suspected bony metastasis in the second lumbar vertebra that was diagnosed as Paget's disease because of the Mickey Mouse sign on bone scintigraphy, and the diagnosis was confirmed by biopsy. Therefore, bone scintigraphy may provide a positive contribution to the diagnosis, and may help to avoid unnecessary biopsy in cases with specific signs and patterns.

Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case report and its surgical management.

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.

Pathological Fracture of the Tibia in a Monostotic Anterior Cortical Fibrous Dysplasia: Hybrid Fixation with UnicorticalOsteoperiosteal Non-Vascularized Fibula Graft - A Case Report.

INTRODUCTION: Fibrous Dysplasia is a relatively rare Musculoskeletal disorders in which there is a defect in remodelling of immature bone to mature lamellar bone. Fibrous Dysplasia was first described by Von Recklinghausen in 1891, but it was Lichtenstein who labelled it polyostotic Fibrous Dysplasia in 1938. Union of Pathological Fractures in a bone affected by Fibrous Dysplasia poses a difficult set of Challenges for treating Physicians. CASE REPORT: We Report a 24 year-old female patient with a Pathological Fracture of the Tibia in a Monostotic Anterior Cortical Fibrous Dysplasia Hybrid Fixation with a UnicorticalOsteoperisoteal Non Vascularized Fibula Graft with a follow up until the union of fracture site on computed tomography scan and complete integration of unicorticalosteoperiosteal fibular graft . CONCLUSION: We provide additional and independent evidence that removal of the diseased cortex which were parts of the proximal and distal fragment and reconstruction of the defect in the cortex with a press- fitunicortical Non vascularized osteoperiosteal fibular strut graft with a hybrid fixation is a good treatment modality for monostotic fibrous dysplasia. Hybrid fixation includes retrograde intramedullary transarticular rodding extending across the subtalar and ankle joint into the tibia and augmentation of the primary fixation with a mono-planar rail Fixation in compression mode.

O Cripto-1 é um potencial biomarcador para o diagnóstico diferencial dos tumores ósseos benignos e malignos dos maxilares / Cripto-1 is a potential biomarker for the differential diagnosis of benign and malignant jaw bone tumors

O termo lesão fibro-óssea dos maxilares (LFOM) é uma designação inespecífica para um grupo de distúrbios caracterizados, morfologicamente, pela substituição do tecido ósseo por uma matriz de tecido conjuntivo fibrosa, a qual exibe neoformação de tecido ósseo com diferentes graus de mineralização. O diagnóstico preciso das LFOM não é fácil e só pode ser realizado após uma análise minuciosa dos aspectos clínicos, radiológicos e histológicos. No entanto, deve-se admitir que alguns casos desafiam a exatidão na emissão do diagnóstico. Considerando a diversidade do comportamento biológico das lesões e as pesquisas sobre a identificação de potenciais marcadores moleculares, o objetivo deste trabalho foi realizar uma análise imunohistoquímica do cripto-1 (CR-1) e da ß-catenina em uma série de casos diagnosticados microscopicamente como displasia fibrosa (DF) (n=30), fibroma ossificante central (FOC) (n=28) e osteossarcoma (OS) (n=5) armazenados nos arquivos do Serviço de Anatomia Patológica Oral de uma população brasileira. As expressões imuno-histoquímicas foram analisadas através de escore imunorreativo. Os dados obtidos foram inseridos em um arquivo do software Microsoft Excel® e, posteriormente, analisados no software Statistical Package for Social Science. Para todos os testes estatísticos utilizados, o nível de significância foi estabelecido em 5% (p<0,05). O CR-1 exibiu predominância de um padrão fortemente positivo para os casos de FOC e OS, e do padrão moderado para os casos de DF (p<0,001). A ß-catenina exibiu predominância do padrão negativo para os casos de FOC e DF, e do padrão fortemente positivo para os casos de OS (p=0,001). O teste de correlação de Spearman revelou correlação positiva entre os escores imunorreativos de CR-1 e ß-catenina. Os resultados desta pesquisa sugerem a participação do CR-1 na patogênese do FOC e OS, assim como o uso dessa proteína como potencial biomarcador molecular para o diagnóstico diferencial de LFOM (AU).

Fibro-osseous lesions of maxilar (FOLM) is a non-specific designation for a group of disorders characterized, morphologically, by replacement of bone tissue by a matrix of fibrous connective tissue, showing neoformation of bone tissue with varying degrees of mineralization. Precise diagnosis of FOLM is not easy, and requires careful analyisis of clinical, radiological and histological aspects. Even so, some cases still challenge accuracy in diagnosis. Considering the diversity of biological behaviour of the lesions and the research regarding identification of potential molecular markers, this study aims to perform immunohistochemical analysis of crypto-1 and ß-catenin in a series of cases diagnosed microscopically as fibrous dysplasia (FD) (n=30), central ossifying fibroma (COF) (n=28) and osteossarcoma (OS) (n=5), stored in archives of Oral Pathological Anatomy Service of a Brazilian population. Immunohistochemical expressions were analysed by imunorreactive score. All data obtained was inserted into a file of Microsoft Excel® software (Microsoft Corporation, USA) and then transferred to a database of SPSS® for Windows software (Statistical Package for Social Sciences; IBM, USA), version 20.0. For all statistical tests used, the significance level established was p ≤ 0.05. CR-1 showed a predominant pattern of strong positive in COF and OS cases, and a moderate positive in FD cases (p<0,001). ß-catenin showed a predominant negative pattern for COF and FD cases, and a predominant strong positive pattern for OS cases (p=0,001). Spearman correlation tests showed positive correlation of the imunoreative scores of CR-1 and ß-catenin. Those results suggests CR-1 could be involved in the pathogenesis of COF and OS, and this protein could be used as a potential molecular biomarker for diferential diagnosis of FOLM (AU).