Lower-limb intramedullary nailing in patients with polyostotic fibrous dysplasia who had a previous unsuccessful treatment. A report of 48 cases.

BACKGROUND: Intramedullary nailing (IN) seems to be the best primary surgical treatment for patients with either polyostotic fibrous dysplasia or McCune-Albright syndrome (PFD/MAS) when the femur and tibia are totally affected by fibrous dysplasia (FD) and pain, fracture and deformity are likely to occur. However, other management protocols have been applied in these cases, often leading to disabling sequelae. This study sought to evaluate if IN could also have been effective as a salvage procedure to provide patients with satisfactory results, regardless of the poor results due to the improper treatment previously performed. MATERIALS AND METHODS: Twenty-four retrospectively registered PFD/MAS patients with 34 femurs and 14 tibias totally affected by fibrous dysplasia had received various treatments with unsatisfactory results in other institutions. Before the IN performed in our hospital, 3 patients were wheelchair bound; 4 were fractured; 17 limped; and many used an aid for walking. Salvage IN was performed in our hospital at a mean patient age of 23.66 ± 6.06 years (range, 15-37 years). The patients were evaluated before-except for the four fractured ones-and after IN using the validated Jung scoring system, and the data were statistically analyzed. RESULTS: The mean length of follow-up after IN was 9.12 ± 3.68 years (range, 4-17 years). The patients' mean Jung score significantly improved from 2.52 ± 1.74 points before IN to 6.78 ± 2.23 at follow-up (p < 0.05). Ambulation was improved in ambulatory patients and restored in wheelchair users. The complication rate was 21%. CONCLUSIONS: Regardless of the high rate of complications, IN may be considered a reliable surgical procedure to salvage a failed treatment in PFD/MAS, with long-lasting satisfactory results achieved in most patients. Trial registration statement: Not applicable. LEVEL OF EVIDENCE: IV.

Hyperthyroidism in McCune-Albright Syndrome - a case report.

OBJECTIVES: We intend to describe a case of McCune-Albright Syndrome (MAS), a rare disease characterized by fibrous dysplasia (FD), cutaneous hyperpigmentation and hyperfunctioning endocrinopathies (HFE). CASE PRESENTATION: We report the case of a 13-year-old male child who presented with a café-au-lait macule in the lumbosacral region and disabling polyostotic FD, requiring several surgical interventions and bisphosphonates from the age of 3 years (Y) + 9 months (M) due to persistent and severe pain. Hyperthyroidism (HT) became apparent at 5 Y + 1 M with a T3/T4 ratio greater than 20. Treatment with anti-thyroid drugs (ATD) was carried out for 7 Y and there was a progressive improvement in pain complaints 8 M after starting ATD, allowing treatment with pamidronate to be discontinued. Total thyroidectomy was performed at 12 Y + 5 M. CONCLUSIONS: This is a case of MAS-associated HT that reflects the deleterious effect of thyroid hormone excess on FD, reinforcing the need of having a low threshold for suspicion of HFE that may arise.

Surgical correction of valgus deformities of the knee in Polyostotic Fibrous Dysplasia.

Coxa vara and shepherd's crook deformity represent the most common femoral deformities in patients affected by polyostotic fibrous dysplasia (PFD) and McCune Albright syndrome (MAS). The tibia is also commonly affected, with antero-medial bowing and valgus deformity. Surgical treatment of these deformities are technically demanding and, in most cases, intramedullary nails are the preferred method to stabilize corrective osteotomies. Genu valgus may also be present, as a residual defect after the surgical correction of the aforementioned deformities. The aim of our study was to report the outcomes obtained in five patients with PFD or MAS operated on for complex deformities of the lower limbs with an associated residual genu valgum greater than 15°. It was surgically treated by distal femoral or proximal tibial osteotomy stabilized with a screw plate. The osteotomy was performed distal to the intramedullary nail in femurs, while it was performed after removing the intramedullary nail in the proximal metaphysis of the tibia. In latter cases, the intramedullary device was reinserted at least 6 months after surgery. At follow-up, the femoro-tibial angle was corrected in all cases and the patients were satisfied with the final result. Two patients showed mild limping, unrelated to the alignment of the knee. In our opinion, residual valgus deformities of the lower limb greater than 15 degrees should be corrected to improve function and cosmetic appearance of these patients. The osteotomy of the distal femur or of the proximal tibia stabilized by a screw-plate represent the treatment of choice for the correction of these deformities.

Cross-sectional evaluation of FGD-avid polyostotic fibrous dysplasia: MRI, CT and PET/MRI findings.

A 42-year-old male with left hip pain was diagnosed of several right femoral and tibial bone tumours. All lesions were osteolytic with sclerotic margins. The symptomatic lesion in the proximal femur also showed bone expansion and focal cortical thinning. Whole-body [18F]-fluorodeoxyglucose (FDG) PET/CT and segmental PET/MRI of the left hip and femur were performed for metabolic characterization of the lesions and for biopsy guidance. The lesions showed a heterogenous degree of FDG uptake corresponding to different metabolic stages of the disease. A biopsy of the tumour portion showing the highest FDG uptake revealed a fibrous dysplasia (FD). In conclusion, although generally affecting paediatric and adolescent subjects, polyostotic FD may be detected in the adulthood. Despite the benign nature of the disease, increased glucose metabolism can be seen in some lesions. Hybrid imaging combining morphological and functional information may help guide biopsy and better define the treatment strategy.

Pathological Femoral Shaft Fracture With McCune-Albright Syndrome With Hyperthyroidism Managed With Oral Alendronate: A Case Report.

McCune-Albright syndrome (MAS) is a complex endocrinopathy with polyostotic fibrous dysplasia and café-au-lait spots. Pathological femoral shaft fracture along with MAS is not a common occurrence. Bisphosphonates can be used for the management of pathological fractures. A seven-year-old male child presented with pathological femoral shaft fracture with MAS (café-au-lait macules and hyperthyroidism). The patient was managed conservatively with closed reduction along with hip spica and oral alendronate. Fracture consolidation occurred within six to eight weeks with improvement in bone pains. Oral alendronate can be used as an adjuvant to conservative therapy for pathological fracture in MAS in children.

Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review.

BACKGROUND: Surgical correction of femoral deformities in polyostotic fibrous dysplasia (PFD) or McCune-Albright syndrome (MAS), such as coxa vara or shepherd's crook deformity, is a challenge. AIM: To evaluate the treatment of patients with femoral deformities caused by PDF or MAS treated by osteotomies and stabilized with different methods, by analyzing the most relevant studies on the topic. METHODS: A literature search was performed in Medline database (PubMed). Articles were screened for patients affected by PFD or MAS surgically managed by osteotomies and stabilized with different methods. RESULTS: The initial search produced 184 studies, with 15 fulfilling the eligibility criteria of our study. Selected articles (1987-2019) included 111 patients overall (136 femurs). CONCLUSION: Based on our results, the preferred method to stabilize corrective osteotomies is intramedullary nailing with neck cross pinning. When the deformity is limited to the proximal part of the femur, a screw or blade plate may be used, although there is a high risk of fracture below the plate. When the femur is entirely involved, a two-stage procedure may be considered.

Immediate allograft reconstruction of the infraorbital nerve following resection of polyostotic fibrous dysplasia lesion.

Processed nerve allografts (PNA) have increasingly been used as alternative to autogenous nerve grafts to repair nerve injuries in oral-maxillofacial surgeries. This case report describes an immediate PNA reconstruction of infraorbital nerve injury sustained during the ablation of a large expansile polyostotic fibrous dysplasia centered in the left maxilla.

A Patient with neonatal cholestasis.

The patient, a boy born in 1991, showed pronounced polyostotic fibrous dysplasia due to McCune-Albright syndrome, as well as Gilbert syndrome and Charcot-Marie-Tooth neuropathy caused by a DNM2 mutation. In addition, the patient, his sister, mother and maternal grandfather had intermittently increased plasma arginine and lysine levels, most probably due to heterozygosity for a novel pathogenic SLC7A2 variant.

Displasia fibrosa poliostótica - síndrome de McCune-Albright: reporte de un caso / Polyostotic fibrous dysplasia - McCune-Albright syndrome: a case report / Displasia fibrosa poliostótica - síndrome de McCune-Albright: relato de um caso

Resumen: Introducción: la displasia fibrosa poliostótica es una patología de muy baja prevalencia, por lo que su diagnóstico pasa desapercibido en la mayoría de los casos. Cuando se asocia a endocrinopatías o a lesiones cutáneas hiperpigmentadas corresponde al síndrome de McCune-Albright. Caso clínico: escolar de 8 años, sexo femenino, que presentó una fractura patológica de fémur izquierdo traumática, en la cual se diagnosticó una displasia fibrosa poliostótica. Por presentarse acompañada de pubertad precoz periférica configura el denominado síndrome de McCune-Albright. El control y tratamiento fue multidisciplinario. El equipo de traumatología realizó osteosíntesis con placa y tornillos de la lesión ósea con evolución a la consolidación en plazos habituales (tres meses). A los seis meses de seguimiento la niña se encuentra sin dolor y sin repercusiones funcionales para la vida diaria. Del punto de vista endocrinológico se realizó tratamiento de su pubertad precoz con inhibidores de la aromatasa con el fin de mejorar su talla final y evitar repercusiones psicológicas y emocionales. En este estudio se analizan características de esta patología y su pronóstico vital y funcional.

Summary: Introduction: polyostotic fibrous dysplasia is a low prevalence disease, and for this reason, it often goes undetected. When associated to endocrinopathies and/or hyperpigmented skin lesions we speak about McCune Albright syndrome. Clinical case: eight-year old school girl who presented pathological fracture of the left femur, which was diagnosed as polyostotic fibrous dysplasia. As it was accompanied by peripheral precocious puberty it constituted an indicative clinical picture of the so-called McCune Albright. Control and treatment were multidisciplinary. The traumatology team performed osteosynthesis with plaques and nails to fix the bone lesion, and evolution consolidated in a regular time frame (3 months). Upon six months follow-up, the girl has no pain and presents no functional repercussion in daily life. From the endocrinological perspective, the girl received precocious puberty treatment with aromatase inhibitors with the purpose of improving her final size and avoid psychological and emotional implications. The study presents the characteristics of this condition, as well as its vital and functional prognosis.

Resumo: Introdução: a displasia fibrosa poliostótica é uma doença de prevalência muito baixa, por isso seu diagnóstico passa despercebido na maioria dos casos. Quando associada a endocrinopatias e / ou lesões cutâneas hiperpigmentadas, corresponde à síndrome de McCune Albright. Caso clínico: estudante do sexo feminino, 8 anos, com quadro de fratura patológica traumática do fêmur esquerdo, com diagnóstico de displasia fibrosa poliostótica. Por estar acompanhada de puberdade precoce periférica, configura a chamada síndrome de McCune Albright. O controle e o tratamento foram multidisciplinares. A equipe de trauma realizou osteossíntese de placa e parafuso da lesão óssea com progressão à consolidação nos prazos usuais (3 meses). Aos 6 meses de seguimento, a paciente não apresenta dor e tampouco repercussões funcionais no dia a dia. Do ponto de vista endocrinológico, sua puberdade precoce foi tratada com inibidores da aromatase para melhorar sua altura final e evitar repercussões psicológicas e emocionais. Este estudo analisa as características desta patologia, seu prognóstico vital e funcional.

Craniofacial Fibrous Dysplasia Involvements of Mccune-Albright Syndrome: A Review with an Additional Case.

BACKGROUND: McCune-Albright Syndrome (MAS) is a genetic disorder with a triad of endocrine diseases, café-au-lait macules and fibrous dysplasias. Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients. OBJECTIVE: The objective of this review is to determine the involvement frequency of cranial and facial bones in patients with MAS and CFD. METHODS: Articles in PubMed was searched with the following details "(mccune[Title/Abstract] OR albright[Title/Abstract]) OR ("craniofacial fibrous dysplasia"[MeSH Terms] OR ("craniofacial"[ All Fields] AND "fibrous"[All Fields] AND "dysplasia"[All Fields]) OR "craniofacial fibrous dysplasia"[All Fields])". The articles in which the authors did not state the involved bones or did not add any radiographic images were excluded from the study. RESULTS: 26 cases in 25 articles met the inclusion criteria. Among the 26 cases and our case, sphenoid and frontal bones were involved in 17 cases, parietal and occipital bones were involved in 15 cases, mandible and ethmoid bone were involved in 14 cases, maxilla-zygoma-temporal and palate was involved in 13, 11, 6 and 3 cases, respectively. Palate was involved in cases where maxilla was also involved. Our case was the only case that was evaluated with CBCT. CONCLUSION: Routine follow-ups are important since new CFDs can occur in different cranial or facial bones. 2D imaging techniques may not be able to demonstrate early CFDs; thus, an advanced imaging technique should be used after MAS diagnosis.

Discontinuous polyostotic fibrous dysplasia with multiple systemic disorders and unique genetic mutations: A case report.

BACKGROUND: Polyostotic fibrous dysplasia (PFD) is an uncommon developmental bone disease in which normal bone and marrow are replaced by pseudotumoral tissue. The etiology of PFD is unclear, but it is generally thought to be caused by sporadic, post-zygotic mutations in the GNAS gene. Herein, we report the case of a young female with bone pain and lesions consistent with PFD, unique physical findings, and gene mutations. CASE SUMMARY: A 27-year-old female presented with unbearable bone pain in her left foot for 4 years. Multiple bone lesions were detected by radiographic examinations, and a diagnosis of PFD was made after a biopsy of her left calcaneus with symptoms including pre-axial polydactyly on her left hand and severe ophthalmological problems such as high myopia, vitreous opacity, and choroidal atrophy. Her serum cortisol level was high, consistent with Cushing syndrome. Due to consanguineous marriage of her grandparents, boosted whole exome screening was performed to identify gene mutations. The results revealed mutations in HSPG2 and RIMS1, which may be contributing factors to her unique findings. CONCLUSION: The unique findings in this patient with PFD may be related to mutations in the HSPG2 and RIMS1 genes.

Pubertad precoz periférica / Peripheral precocious puberty

Introducción: El síndrome de Mc Cune-Albright (SMA) es una rara entidad asociada con la displasia fibrosa poliostótica, con la presencia de manchas de color café con leche y también con la hiperfunción endocrina. La alteración hormonal más frecuente es la pubertad precoz. El SMA se debe a mutaciones activadoras del gen GNAS1. Objetivo: Describir las características clínicas de una paciente con síndrome de Mc Cune-Albright con una pubertad precoz. Métodos: Se realizó una revisión de la historia clínica como fuente primaria y fueron incorporados todos los elementos clínicos, bioquímicos, imagenológicos y genéticos que conformaron la valoración integral de la paciente. Presentación de caso: Se presenta un caso poco frecuente de síndrome de Mc Cune-Albright en una niña de siete años de edad con mamas Tanner II-III, sangrado vaginal, vello axilar y pubiano escaso, manchas café con leche y lesiones óseas. Lleva tratamiento con tamoxifeno, lo que ha logrado mantener frenada la progresión del desarrollo puberal. Conclusiones: Aunque esta entidad es de carácter benigno y la prevalencia es extremadamente baja, el inicio puberal precoz y el compromiso de la talla final pueden producir impacto psicológico en la calidad de vida y en el desarrollo normal del individuo(AU)

Introduction: Mc Cune-Albright syndrome (SMA, by its acronym in Spanish) is a rare entity associated with polyostotic fibrous dysplasia, with the presence of brown spots with milk and also with endocrine hyperfunction. The most common hormonal alteration is precocious puberty. SMA is caused by GNAS1 gene´s activator mutations. Objective: Describe the clinical characteristics of a patient with Mc Cune-Albright syndrome with precocious puberty. Methods: A review of the medical history was carried out as a primary source and all the clinical, biochemical, imaging and genetic elements that made up the comprehensive assessment of the patient were incorporated. Case presentation: A rare case of Mc Cune-Albright syndrome occurs in a seven-year-old girl with Tanner II-III breasts, vaginal bleeding, axillary and pubic hair, brown spots with milk and bone lesions. She is treated with tamoxifen, which has managed to keep the progression of pubertal development slow. Conclusions: Although this entity is benign in nature and the prevalence is extremely low, early pubertal onset and the compromise of the final size can have a psychological impact on the quality of life and normal development of the individual(AU)

An unusual presentation of non-specific cystic degeneration of craniofacial fibrous dysplasia: a case report and review of literature.

BACKGROUND: Fibrous dysplasia (FD) is a rare, sporadic, and benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. FD localized in the cranial and facial bones is called craniofacial fibrous dysplasia (CFD). Cystic degeneration in CFD cases is rare; cystic degeneration appearing in both the maxilla and the mandible FD lesion is even rarer. The aim of this article was to report a case of fibrous dysplasia of the mandible and maxilla complicated by nonspecific cystic degeneration. CASE PRESENTATION: A 30-year-old woman presented with a rare case of non-specific cystic degeneration in a mandible and maxilla FD lesion that occurred 11 years after surgery. She was diagnosed with polyostotic CFD and underwent maxillary and mandibular bone contouring. Cyst enucleation under general anesthesia was performed in the mandibular region due to pain and discomfort. CONCLUSIONS: In cases involving non-aggressive and non-invasive FD cystic degeneration in focal areas, conservative treatment is recommended. However, if cystic degeneration of FD develops rapidly and causes discomfort, pain, or dysfunction, surgical treatment should be considered.

Jaffe-Lichtenstein syndrome associated with a simple bone cyst: unprecedented rare case report

Abstract Lesions denominated fibro-osseous lesions of the jaw constitute a diversified group of disorders, in which the normal bone architecture is replaced by fibroblasts, collagen fibers and immature bone. At present, the World Health Organization recognizes four variants of these lesions, namely: bone-cement dysplasia, fibrous dysplasia, ossifying fibroma and Familial gigantiform cementoma. Fibrous dysplasia may present in the monostotic form, affecting one single bone or an isolated craniofacial region; and in the Polyostotic form, involving two or more bones of the skeleton, and eventual association with syndromic conditions. The patient, C.P.G., 43 years old, sought attendance due to symptomatic increase in the region of the mandibular body on the right side. Imaging exams revealed craniofacial areas with ground-glass aspect, beyond the extensive mandibular radiolucent lesion. During the physical exam, spots of the Café au lait type disposed on the right side of the body were identified, in addition to uncoordinated gait with distinct shortening of the right leg. Additional radiographic exams showed evidence of skeletal dissemination of the disease. The patient denied any sexual precocity, and the final diagnosis was fibrous dysplasia, expressed by means of the Jaffe-Lichtenstein syndrome, in association with a simple bone cyst.

Resumo As denominadas lesões fibro-ósseas dos maxilares constituem um grupo diversificado de desordens nas quais a arquitetura óssea normal é substituída por fibroblastos, fibras colágenas e osso imaturo. Atualmente a Organização Mundial de Saúde reconhece quatro variantes destas lesões, sendo elas: a displasia cemento-óssea, a displasia fibrosa, o fibroma ossificante e cementoma gigantiforme familiar. A displasia fibrosa pode ser apresentar na forma monostótica, acometendo um único osso ou a região craniofacial isoladamente, e a forma poliostótica, envolvendo dois ou mais ossos do esqueleto, com eventual associação com condições sindrômicas. Paciente C.P.G., 43 anos, procurou atendimento devido aumento volumétrico sintomático na região de corpo mandibular do lado direito. Exames imaginológicos revelaram áreas craniofaciais com aspecto de vidro fosco ou despolido, além de extensa lesão radiolúcida mandibular. Durante o exame físico foram identificadas manchas do tipo café com leite dispostas do lado direito do corpo, além de marcha descoordenada com nítido encurtamento da perna direita. Novos exames radiográficos evidenciaram a disseminação esquelética da doença. O paciente negou qualquer precocidade sexual e o diagnóstico final foi de displasia fibrosa, expressa por meio da síndrome de Jaffe-Linchtenstein, em associação com um cisto ósseo simples.

Diagnosis and conservative treatment of a rare case of femoral intraosseous arteriovenous malformation in a patient with polyostotic fibrous dysplasia: A case report.

Pure intraosseous arteriovenous malformation (AVM) in a limb bone is extremely rare. Furthermore, there is currently insufficient information on the diagnostic and therapeutic strategies for pure intraosseous AVMs. We herein report a case of pure intraosseous AVM of the proximal femur occurring in a patient with polyostotic fibrous dysplasia. The patient was a 39-year-old woman who presented with pain in the right thigh. Plain radiographs and computed tomography scans revealed a medullary lytic lesion with expansion and thinning of the bone cortex in the right proximal femur, mimicking a primary bone tumor. Magnetic resonance imaging (MRI) examination revealed intramedullary signal voids and feeding arteries arising from the deep femoral artery. A non-surgical approach using embolization and denosumab achieved satisfactory results, which included complete obliteration of the AVM, increased cortical thickness of the right proximal femur, and attenuation of the high-turnover bone metabolism 1 year later. Careful review of MRI images is crucial for distinguishing between bone tumors and intraosseous AVM, which exhibit signal voids and feeding arteries, in order to avoid unnecessary interventions such as bone biopsy or surgery.

[Mazabraud and McCune-Albright syndromes in association : A case of two very rare orthopaedic tumour entities]. / Mazabraud- und McCune-Albright-Syndrom in Assoziation : Ein Fall zweier sehr seltener tumororthopädischer Entitäten.

We report on a 47-year-old woman with unilateral fibrous dysplasia and three intramuscular masses. Medical imaging revealed possible intramuscular myxomas, so that the suspected diagnosis was Mazabraud syndrome. After biopsy, the suspected diagnosis was verified by histology and molecular pathology. Due to endocrine abnormalities in the patient's medical history, McCune-Albright syndrome has was also verified.

Mazabraud's syndrome. New clinical case and review of findings. / Síndrome de Mazabraud. Nuevo caso clínico y revisión de los hallazgos.

Intramuscular myxomas are benign and rare tumors that affects predominantly the lower limbs. The association of myxomas and fibrous dysplasia, usually polyostotic, is rarer. This association is known as Mazabraud's syndrome, of which about 81 cases have been described in the literature. We present a new case of this uncommon association to emphasise the importance of recognizing this syndrome in the diagnosis and appropriate management of the patient.

Displasia fibrosa poliostótica: revisión de la literatura y reporte de caso / Polyostotic fibrous dysplasia: literature review and case report

La displasia fibrosa (DF) es una enfermedad rara causada por una mutación genética esporádica y congénita donde existe un trastorno en la maduración del mesénquima óseo. Se presenta un paciente de sexo masculino de 27 años, con antecedentes de fractura de tabique nasal y dolor en la región frontal asociada a náuseas e imposibilidad para caminar desde hace ocho años. Al examen físico se encuentra asimetría craneofacial, disminución de la agudeza visual e hipoacusia bilateral. La resonancia magnética cerebral revela hiperostosis y disminución de volumen del cerebro, cerebelo y senos paranasales. La exploración radiológica revela aumento difuso del volumen del componente medular óseo con compromiso poliostótico. Se diagnostica displasia fibrosa ósea poliostótica sobre la base de los datos clínicos y de imágenes. El diagnóstico de este paciente fue tardío. Se ha reportado que, en pacientes jóvenes, el diagnóstico de displasia fibrosa es radiológico y que, en raras ocasiones se requiere biopsia ósea. (AU)

Fibrous dysplasia (FD) is a rare disease caused by a sporadic genetic mutation that generates a disruption in the maturation of bone mesenchyme. We report a 27 year old male patient, with a history of nasal septum fracture and frontal region pain associated with nausea, and walking disability for eight years. At the physical examination we found craniofacial asymmetry, decreased visual acuity, and bilateral deafness. Brain magnetic resonance imaging revealed hyperostosis and decreased volume of brain volume, cerebellum, and sinuses. Skeletal survey revealed diffuse enlargement of the bone marrow component and polyostotic involvement. Diagnosis of polyostotic fibrous dysplasia bone was based on clinical and imaging data. The diagnosis of this patient was late. It has been reported that in young patients FD is diagnosed by radiological methods, rarely requiring bone biopsy. (AU)

Double-level osteotomy and one-stage reconstruction with long intramedullary femoral nail to correct a severe proximal and diaphyseal femur deformity in a patient with polyostotic fibrous dysplasia: case report and literatures review.

Proximal femur is often involved with varus and retroversion deformity in polyostotic fibrous dysplasia (PFD). Multiple corrective osteotomies with intramedullary nails in two stages is recommended procedure as some authors described. We report a case using double-level osteotomy and one-stage reconstruction with intramedullary nail in a patient with painful proximal femur and diaphysis varus deformity, the neck-shaft angle was corrected from 95° pre-operatively to 125° post-operatively, the patient was free of pain and no evidence of recurrence at the 24-month follow up. The operative design and method were described, and a review of related literatures about the treatment alteration for PFD and relevant operative selection were also performed.

Displasia fibrosa craneofacial avanzada por remodelación ósea / Advanced craniofacial fibrous dysplasia of bone remodeling

La displasia fibrosa es un trastorno del desarrollo esquelético del mesénquima de formación ósea que se manifiesta como un defecto en la maduración y diferenciación de los osteoblastos. La displasia fibrosa representa alrededor de 2.5% de todos los tumores óseos y más del 7% de los tumores benignos. El objetivo de este artículo es presentar un caso de displasia fibrosa poliostótica en la región craneofacial, al cual se le realizaron estudios de imagen, biopsia incisional para diagnóstico definitivo y remodelación ósea para mejorar el contorno facial y la calidad de vida del paciente.

Fibrous dysplasia is an abnormal bone growth, it causes abnormal growth or swelling of bone maturation and differentiation. Fibrous dysplasia represents 2.5% of all bone tumors and more than 7% of benign tumors. The aim of this report is to present the findings of a polyostotic fibrous dysplasia case in the craniofacial skeleton. An incisional biopsy was done to make the diagnosis, after that, a bone remodeling surgery was performed to improve the facial contour and in general to improve the quality of life of the patient.