6,620 results match your criteria Fibrous Dysplasia


Utility of optical coherence tomography in the diagnosis and management of optic neuropathy in patients with fibrous dysplasia.

J Bone Miner Res 2020 Jul 9. Epub 2020 Jul 9.

Skeletal Diseases and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland.

Optic neuropathy (ON) is a highly disabling complication of fibrous dysplasia (FD). The optimal test for identifying and monitoring ON in FD is unknown. Optical coherence tomography (OCT) is an imaging modality that detects retinal nerve fiber layer (RNFL) thinning, a sign of optic nerve atrophy. Read More

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http://dx.doi.org/10.1002/jbmr.4129DOI Listing

Fibrous dysplasia of the spine: a rare cause of lytic lesions and deformity.

Neurochirurgie 2020 Jul 2. Epub 2020 Jul 2.

Neurosurgery Department, University Hospital Dr. Josep Trueta, Girona, (Girona), Spain; Neurosurgery Department, University Hospital Germans Trias i Pujol, Badalona, (Barcelona), Spain.

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http://dx.doi.org/10.1016/j.neuchi.2020.05.005DOI Listing

A case of presumed monostotic sphenoid fibrous dysplasia and abducens nerve palsy.

J AAPOS 2020 Jul 1. Epub 2020 Jul 1.

Division of Ophthalmology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.jaapos.2020.04.005DOI Listing

Evaluation of natural growth rate and recommended age for shaving procedure by volumetric analysis of craniofacial fibrous dysplasia.

Head Neck 2020 Jul 4. Epub 2020 Jul 4.

Department of Plastic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

Background: We evaluated the preoperative natural growth pattern of craniofacial fibrous dysplasia and postoperative volume changes in patients undergoing shaving procedures.

Methods: Thirty-three patients who underwent serial computed tomography (CT) preoperatively and/or postoperatively were identified. The natural tumor growth rate was assessed using preoperative CT scans. Read More

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http://dx.doi.org/10.1002/hed.26337DOI Listing

Natural History and Progression of Craniofacial Fibrous Dysplasia: A Retrospective Evaluation of 114 Patients From Massachusetts General Hospital.

J Oral Maxillofac Surg 2020 May 30. Epub 2020 May 30.

Walter C. Guralnick Distinguished Professor and Chief, Emeritus, Department of Oral & Maxillofacial Surgery, Massachusetts General Hospital, Harvard School of Dental Medicine, Boston, MA. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.joms.2020.05.036DOI Listing

Teaching Neuroimages: Rare skull base involvement in neurosarcoidosis.

Neurology 2020 Jun 30. Epub 2020 Jun 30.

Department of Neurology, The Royal London Hospital, London, UK

A 57-year-old woman was admitted with a tonic-clonic seizure on a background of systemic sarcoidosis with uveitis and hilar lymphadenopathy. She had previously been well and stopped steroids one year before presentation. CT showed a sclerotic lesion with focal lucent areas in the skull base MRI revealed a frontal FLAIR hyperintense oedematous lesion with meningeal enhancement in gadolinium T1 This appearance on imaging evokes broad differential diagnoses such as sarcoidosis, fungal (although she had increasing lesion size despite six-month treatment with amphotericin B), craniofacial fibrous dysplasia, atypical lymphoma, nasopharyngeal carcinoma, myeloma and tuberculosis, therefore necessitating biopsy for definitive evaluation. Read More

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http://dx.doi.org/10.1212/WNL.0000000000010082DOI Listing

Quantitative evaluation of symmetry after navigation-guided surgical recontouring of zygomatic fibrous dysplasia: a comparative study.

Int J Oral Maxillofac Surg 2020 Jun 25. Epub 2020 Jun 25.

Department of Oral and Maxillofacial Surgery, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China. Electronic address:

Zygomatic fibrous dysplasia is a slowly progressive disorder of bone, which commonly causes facial asymmetry. Precise surgical recontouring can effectively improve facial aesthetics. The aim of this study was to quantitatively evaluate the effectiveness of surgical navigation guidance in correcting zygomatic asymmetry. Read More

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http://dx.doi.org/10.1016/j.ijom.2020.06.001DOI Listing

Clinical features and MRI characteristics of presumptive constrictive myelopathy in 27 pugs.

Vet Radiol Ultrasound 2020 Jun 25. Epub 2020 Jun 25.

Radiology Department, Willows Referral Centre, Solihull, UK.

Constrictive myelopathy has been described in pugs with paraparesis and is characterized by fibrous connective and granulation tissue within the dura mater causing spinal cord compression and focal gliosis. An association between constrictive myelopathy and caudal articular process (CAP) dysplasia is suspected; however, some studies have reported CAP dysplasia as an incidental finding. The imaging appearance of constrictive myelopathy is currently limited to a small number of cases. Read More

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http://dx.doi.org/10.1111/vru.12890DOI Listing

Intranasal proliferative fibro-osseous dysplasia in a domestic longhair cat.

JFMS Open Rep 2020 Jan-Jun;6(1):2055116920917839. Epub 2020 Jun 10.

Lumbry Park Veterinary Specialists, Alton, UK.

Case Summary: A 13-year-old female domestic longhair cat was presented for further investigation of chronic sneezing combined with a right-sided nasal discharge. A CT scan of the head revealed a locally invasive, aggressive right nasal mass radiographically consistent with a malignant neoplastic process. Histopathology on rhinoscopically guided biopsies revealed an unusual pathology consistent with fibro-osseous hyperplasia/dysplasia. Read More

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http://dx.doi.org/10.1177/2055116920917839DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7288824PMC

Bone Tumors: Benign Bone Tumors.

Authors:
Yee Lam

FP Essent 2020 Jun;493:11-21

UNC Department of Family Medicine, 590 Manning Drive, Chapel Hill, NC 27599.

Primary benign bone tumors are uncommon and most often affect children and young adults. They typically are detected incidentally on imaging, though some patients present with pain, swelling, or other symptoms. The four main categories of benign bone tumor are: bone-forming (eg, osteoid osteoma, osteoblastoma, fibrous dysplasia, enostosis), cartilage-forming (eg, osteochondroma, enchondroma), connective tissue, and vascular; the latter two are rare. Read More

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Multiple primary tumors: a case report and review of the literature.

BMC Musculoskelet Disord 2020 Jun 22;21(1):394. Epub 2020 Jun 22.

Musculoskeletal Tumor Center, Peking University People's Hospital, Beijing, 100044, China.

Background: Multiple primary tumors, especially quadruple primary neoplasms is extremely rare. Fibrous dysplasia (FD), osteosarcoma (OS), and giant cell tumor of bone (GCTB) are three bone tumors with low incidence while primary pulmonary meningioma is a rare disease. In this case report, we present a unique synchronous occurrence of these four separate pathological conditions. Read More

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http://dx.doi.org/10.1186/s12891-020-03426-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310446PMC

Bilateral dysplastic gangliocytoma with concurrent polyostotic fibrous dysplasia: a case report and literature review.

World Neurosurg 2020 Jun 16. Epub 2020 Jun 16.

Department of Neurosurgery, Lanzhou University Second Hospital, Lanzhou, Gansu, China; Institute of Neurology, Lanzhou University, Lanzhou, Gansu, China. Electronic address:

Background: Dysplastic gangliocytoma is a sporadic cerebellar benign tumor with the characteristics of hamartoma and true tumor, also known as Lhermitte-Duclos 's disease. Bone fibrous dysplasia is a slowly progressive self-limited benign bone tissue disease. Cowden syndrome, an autosomal dominant genetic disorder caused by germline mutations in the PTEN gene, is considered to be closely related to dysplastic gangliocytoma. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.071DOI Listing

Successful Intravascular Treatment of an Intraosseous Arteriovenous Fistula in Fibrous Dysplasia.

Calcif Tissue Int 2020 Aug 17;107(2):195-200. Epub 2020 Jun 17.

Skeletal Diseases and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, 30 Convent Drive, Building 30, Room 207, Bethesda, MD, 20892-4320, USA.

Fibrous dysplasia (FD) is a benign bone disease characterized by expansile lesions that typically stabilize with age. Rarely, FD can undergo malignant transformation, presenting with atypical, rapid growth and destruction of adjacent bone. Other potential causes of rapid FD expansion include secondary lesions, such as aneurysmal bone cysts. Read More

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http://dx.doi.org/10.1007/s00223-020-00712-4DOI Listing

[Tumor-like bony lesions of the skeleton].

Radiologe 2020 Jul;60(7):655-668

Klinik für Orthopädie und Unfallchirurgie, Comprehensive Cancer Center Freiburg CCCF, Medizinische Fakultät, Universitätsklinikum Freiburg, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland.

Tumor-like bony lesions are, by definition bony lesions, which can be clinically, radiologically and histologically mistaken for real bone tumors. This article presents the aneurysmal bone cyst (ABC), solitary bone cyst (SBC), fibrous dysplasia, osteofibrous dysplasia Campanacci and non-ossifying fibroma (NOF). Many tumor-like bony lesions are often incidental findings. Read More

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http://dx.doi.org/10.1007/s00117-020-00705-2DOI Listing

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

J Orthop Case Rep 2020 ;9(6):19-22

Department of Orthopaedic Surgery, Sir J.J. Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India.

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. Read More

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http://dx.doi.org/10.13107/jocr.2019.v09.i06.1570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7276599PMC
January 2020

Hedgehog Activation Regulates Human Osteoblastogenesis.

Stem Cell Reports 2020 May 27. Epub 2020 May 27.

Center for Disease Biology and Integrative Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo 113-8656, Japan; Department of Bioengineering, Graduate School of Engineering, The University of Tokyo, Tokyo 113-8655, Japan; Department of Cell Biology, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki 852-8521, Japan. Electronic address:

Two genetic diseases, Gorlin syndrome and McCune-Albright syndrome (MAS), show completely opposite symptoms in terms of bone mineral density and hedgehog (Hh) activity. In this study, we utilized human induced pluripotent stem cell (iPSC)-based models of the two diseases to understand the roles of Hh signaling in osteogenesis. Gorlin syndrome-derived iPSCs showed increased osteoblastogenesis and mineralization with Hh signaling activation and upregulation of a set of transcription factors in an osteogenic culture, compared with the isogenic control. Read More

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http://dx.doi.org/10.1016/j.stemcr.2020.05.008DOI Listing

Retrospective Analysis of 61 Cases of Children Died of Viral Pneumonia.

Fa Yi Xue Za Zhi 2020 Apr;36(2):164-168

School of Forensic Medicine, Southern Medical University, Guangzhou 510515, China.

Abstract: Objective To retrospectively analyze the forensic pathological postmortem examination and clinical data of children who died of viral pneumonia in identification of cause of death cases and to discuss the clinical characteristics and pathological features of viral pneumonia in children, in order to provide reference to pathological diagnosis of viral pneumonia in children caused by 2019 novel coronavirus (2019-nCoV) infection. Methods Postmortem examination data from 61 cases of children whose causes of death were identified as viral pneumonia in recent years were collected from the Center of Forensic Identification, Southern Medical University. The gender, age, clinical symptoms and pathological features were comparatively analyzed. Read More

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http://dx.doi.org/10.12116/j.issn.1004-5619.2020.02.003DOI Listing

A signature of circulating miRNAs associated with Fibrous Dysplasia of bone: the mirDys study.

J Bone Miner Res 2020 Jun 11. Epub 2020 Jun 11.

Department of Rheumatology, Edouard Herriot University Hospital, 5 Place d'Arsonval, 69003, Lyon, France.

Fibrous dysplasia (FD) is a rare bone disease caused by activating mutations of GNAS encoding the Gsα protein, enhancing cAMP production by overstimulation of adenylyl cyclase and impairing osteoblastic differentiation. The clinical presentation ranges from asymptomatic to polyostotic forms with severe disability, explained by the mosaic distribution of the GNAS mutation. Physicians have to deal with the gap of knowledge in FD pathogenesis, the absence of prognostic markers and the lack of specific treatment. Read More

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http://dx.doi.org/10.1002/jbmr.4111DOI Listing

The Role of Staged Cryosurgery and Three-Dimensional Computed Tomography Integrated Navigation System in the Surgical Management of Severe Involvement of Craniofacial Polyostotic Fibrous Dysplasia.

J Craniofac Surg 2020 Jun;31(4):1006-1009

Plastic, Reconstructive and Aesthetic Surgeon, Private Practice, Kadikoy, Istanbul, Turkey.

Fibrous dysplasia (FD) is a developmental bone disorder caused by the hamartamatous proliferation of bone-forming cells. A 29-year-old male patient with diagnosis of FD was admitted to our clinic with the symptoms of severe craniomaxillofacial involvement of FD beginning from last year. Neurological examination revealed diplopia, horizontal nystagmus, conductive hearing loss, and partial vision loss. Read More

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http://dx.doi.org/10.1097/SCS.0000000000006348DOI Listing

Florid cemento-osseous dysplasia associated with chronic suppurative osteomyelitis and multiple impacted tooth an incidental finding - A rare case report.

J Family Med Prim Care 2020 Mar 26;9(3):1757-1761. Epub 2020 Mar 26.

Department of Oral Medicine and Radiology, Rajarajeswari Dental College and Hospital, Ramohali Cross, Mysore Road, Bengaluru, Karnataka, India.

Florid cemento-osseous dysplasia (FCOD) is a rare, benign, fibro osseous, and multifocal dysplastic lesion of the jaw that consists of cellular fibrous connective tissue with bone and cementum-like tissue. The word 'florid' was introduced to describe the wide spread, extensive manifestations of the disease in the jaws. FCOD is most commonly found in middle-aged, black women with the prevalence of 5. Read More

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http://dx.doi.org/10.4103/jfmpc.jfmpc_1130_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7266253PMC

New Therapies for Hypophosphatemia-Related to FGF23 Excess.

Calcif Tissue Int 2020 Jun 5. Epub 2020 Jun 5.

Department of Medicine Section of Endocrinology, Yale School of Medicine, PO Box 802080, New Haven, CT, 06520, USA.

FGF23 is a hormone produced by osteocytes in response to an elevation in the concentration of extracellular phosphate. Excess production of FGF23 by bone cells, or rarely by tumors, is the hormonal basis for several musculoskeletal syndromes characterized by hypophosphatemia due to renal phosphate wasting. FGF23-dependent chronic hypophosphatemia causes rickets and osteomalacia, as well as other skeletal complications. Read More

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http://dx.doi.org/10.1007/s00223-020-00705-3DOI Listing

Denosumab for fibrous dysplasia: Promising, but questions remain.

J Clin Endocrinol Metab 2020 Jun 1. Epub 2020 Jun 1.

Skeletal Disorders and Mineral Homeostasis Section, National Institute Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD.

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http://dx.doi.org/10.1210/clinem/dgaa091DOI Listing

Fibrous dysplasia limited to an ossicle.

Auris Nasus Larynx 2020 May 27. Epub 2020 May 27.

Beckman Laser Institute Korea, College of Medicine, Dankook University, Cheonan, South Korea; Department of Otolaryngology-Head & Neck Surgery, College of Medicine, Dankook University Cheonan, South Korea. Electronic address:

Fibrous dysplasia is an unusual pathologic condition caused by abnormal bone metabolism. Temporal bone involvement is often seen, but it is uncommon to find fibrous dysplasia limited to the middle ear, especially originating in and confined to a single ossicle. Here we report a case of osteofibrous dysplasia limited exclusively to an ossicle (malleus) causing gradual conductive hearing loss, which recovered after eventual complete removal of the dysplastic area. Read More

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http://dx.doi.org/10.1016/j.anl.2020.05.005DOI Listing

Limitations in Use of Elastic Stable Intramedullary Nailing (ESIN) in Children with Disorders of Bone Mineralization.

Ortop Traumatol Rehabil 2020 Apr;22(2):77-83

Orthopedic & Trauma Department with Arthrogriposis Ward, University Pediatric Hospital in Cracow, Poland / Department of Pediatric Surgery, Jagiellonian University Collegium Medicum, Cracow, Poland.

Background: Elastic intramedullary nails (ESIN) have been the treatment of choice in many long bone fractures in children for more than 20 years. The introduction of ESIN has drastically reduced tissue traumatization during fracture fixation procedures and decreased the risk of growth cartilage damage, as well as allowing for preservation of the natural biology of closed fracture healing. The objective of the present report is to draw attention to a small group of patients with bone mineralization disorders, who consequently demonstrate decreased mechanical resistance of the skeletal system, in whom indications for using ESIN fixation are limited. Read More

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http://dx.doi.org/10.5604/01.3001.0014.1154DOI Listing

Management of the Temporal Bone Fibrous Dysplasia With External Auditory Canal Stenosis and Secondary Cholesteatoma in an Asian Population: A 11-Case Series.

Ear Nose Throat J 2020 May 22:145561320927922. Epub 2020 May 22.

ENT Institute and Otorhinolaryngology Department, Affiliated Eye and ENT Hospital, Shanghai Medical College, Fudan University, Shanghai, China.

Objective: This article summarizes the experience of diagnosis and treatment of temporal bone fibrous dysplasia (FD) with external auditory canal (EAC) stenosis and secondary cholesteatoma in the Chinese population, in order to improve the quality of life of patients in the future.

Methods: Eleven patients with FD of the temporal bone who underwent surgery were retrospectively reviewed.

Results: All lesions originated from the temporal bone, and all involved of the EAC. Read More

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http://dx.doi.org/10.1177/0145561320927922DOI Listing
May 2020
0.881 Impact Factor

Hemorrhagic Fibrous Dysplasia with Acute Neurological Decline: Case Report and Review of the Literature.

World Neurosurg 2020 May 11;140:71-75. Epub 2020 May 11.

Department of Neurosurgery, University of Utah, Salt Lake City, Utah, USA.

Background: Fibrous dysplasia is a rare, benign fibro-osseous malformation whose occurrence in the craniofacial area can result in optic nerve compression, a cerebral mass effect, and cosmetic deformity. Most lesions will progress slowly, and the risk of malignant progression is rare.

Case Description: We present the case of a 21-year-old woman who had presented with acute worsening visual loss secondary to hemorrhagic fibrous dysplasia with ensuing optic nerve compression. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.249DOI Listing

Rare musculoskeletal diseases in adults: a research priority setting partnership with the James Lind Alliance.

Orphanet J Rare Dis 2020 May 19;15(1):117. Epub 2020 May 19.

The Botnar Research Centre, Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, OX3 7LD, UK.

Background: Osteogenesis imperfecta, fibrous dysplasia/McCune-Albright syndrome and X-linked hypophosphatemia are three rare musculoskeletal diseases characterised by bone deformities, frequent fractures and pain. Little high-quality research exists on appropriate treatment and long-term management of these conditions in adults. This is further worsened by limited research funding in rare diseases and a general mismatch between the existing research priorities and those of the patients. Read More

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http://dx.doi.org/10.1186/s13023-020-01398-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7238497PMC

Successful pregnancy and delivery achieved following intracytoplasmic sperm injection using teratozoospermic sperm exhibiting marked dysplasia of the fibrous sheath: a case report.

Transl Androl Urol 2020 Apr;9(2):800-806

Department of Reproductive Medicine, Kameda IVF Clinic Makuhari, Chiba, Japan.

A husband and his wife, both 34 years old, consulted our clinic because of primary infertility. Sperm analysis revealed that the sperm concentration, motility, and progressive motility were (42.8±22. Read More

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http://dx.doi.org/10.21037/tau.2019.12.14DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7215008PMC

Comparison of Trochanteric Slide and Subtrochanteric Shortening Osteotomy in the Treatment of Severe Hip Dysplasia: Mid-Term Clinical Outcomes of Cementless Total Hip Arthroplasty.

J Arthroplasty 2020 Apr 19. Epub 2020 Apr 19.

Medical Faculty, Department of Orthopaedics and Traumatology, Ondokuz Mayıs University, Samsun, Turkey.

Background: The aim of this study is to compare clinical results of Crowe type III-IV developmental dysplasia of the hip (DDH) patients who underwent total hip arthroplasty with either trochanteric slide osteotomy (TSO) or subtrochanteric shortening osteotomy (SSO).

Methods: The patients who underwent cementless total hip arthroplasty with femoral shortening osteotomy due to Crowe type III/IV DDH between 2004 and 2014 and completed at least 5 years of follow-up were retrospectively analyzed. The patients were grouped according to the type of shortening osteotomy as either TSO or SSO. Read More

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http://dx.doi.org/10.1016/j.arth.2020.04.039DOI Listing

Osteosarcoma of the jaw: report of 3 cases (including the rare epithelioid variant) with review of literature.

Oral Surg Oral Med Oral Pathol Oral Radiol 2020 May 11. Epub 2020 May 11.

Department of Pathology, University of New Mexico, School of Medicine, Albuquerque, NM, USA. Electronic address:

Osteosarcoma of the jaw (OSJ) is a rare malignancy, accounting for less than 1% of head and neck malignancies. OSJ can arise as a primary malignancy or secondary to locoregional radiation treatment. Radiologically, these tumors show large, destructive growth with periosteal reaction, which can suggest the diagnosis of osteosarcoma (OS). Read More

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http://dx.doi.org/10.1016/j.oooo.2020.03.044DOI Listing

Superimposed mosaicism in tuberous sclerosis complex: a key to understanding all of the manifold manifestations?

Authors:
R Happle A Torrelo

J Eur Acad Dermatol Venereol 2020 May 12. Epub 2020 May 12.

Department. of Dermatology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

In patients with tuberous sclerosis we can today distinguish between two different categories of segmental mosaicism. The well-known simple segmental mosaicism is characterized by a unilateral or otherwise localized arrangement of the ordinary lesions of the disorder, reflecting heterozygosity for an early postzygotic new mutation. By contrast, superimposed mosaicism is defined by a pronounced segmental involvement in a patient with ordinary nonsegmental lesions of the same disorder, resulting in a heterozygous embryo from loss of the corresponding wild-type allele that occurred at a very early developmental stage. Read More

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http://dx.doi.org/10.1111/jdv.16603DOI Listing

Computer-guided contouring of craniofacial fibrous dysplasia involving the zygoma using a patient-specific surgical depth guide.

Int J Oral Maxillofac Surg 2020 May 4. Epub 2020 May 4.

Oral and Maxillofacial Surgery Department, Faculty of Dentistry, Cairo University, Cairo, Egypt. Electronic address:

The aim of this study was to introduce a new computer-guided technique for contouring and shaving of craniofacial fibrous dysplasia involving the zygoma. Computer-guided contouring was performed for five patients with unilateral craniofacial fibrous dysplasia involving the zygoma, using a patient-specific surgical depth guide. This patient-specific guide with depth holes was virtually designed for each patient based on mirroring of the unaffected side. Read More

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http://dx.doi.org/10.1016/j.ijom.2020.04.009DOI Listing

Fibrous dysplasia of the orbital region: Series of 12 cases and review of the literature.

J Fr Ophtalmol 2020 Jun 4;43(6):467-476. Epub 2020 May 4.

Service d'ophtalmologie, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.

Purpose: Fibrous dysplasia (FD) is a benign fibro-osseous developmental disorder of growing bone, sometimes involving the craniofacial skeleton (CFD). We wish to present a patient series with CFD of the orbital region and discuss treatment modalities.

Methods: Twelve patients were referred for orbital CFD in the Nantes University Hospital between 2000 and 2018 and studied according to the clinical parameters, radiological features, and modalities of treatment. Read More

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http://dx.doi.org/10.1016/j.jfo.2019.10.012DOI Listing

Protruding Meningioma of the Forehead With Extensive Hyperostosis Mimicking Skull Osteoma.

J Craniofac Surg 2020 May 4. Epub 2020 May 4.

Department of Plastic and Reconstructive Surgery, Medical School of Jeonbuk National University, Jeonju.

Meningioma is the most common intracranial benign tumor in adults. Hyperostosis accompanies about 4.5% of meningiomas. Read More

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http://dx.doi.org/10.1097/SCS.0000000000006472DOI Listing

Total hip arthroplasty in patients with fibrous dysplasia: a modern update.

Can J Surg 2020 May 1;63(3):E202-E207. Epub 2020 May 1.

From the Division of Orthopaedic Surgery, Mount Sinai Hospital, Joseph and Wolf Lebovic Health Complex, Toronto, Ont.

Background: Fibrous dysplasia (FD) results from an abnormality in lamellar bone formation and most frequently involves the proximal femur. This can lead to the development of osteoarthritis requiring total hip arthroplasty (THA). Such cases are challenging, and there is a lack of information guiding best management. Read More

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http://dx.doi.org/10.1503/cjs.007219DOI Listing

Orthodontic treatment in a patient with cherubism: Benefits and limitations.

Spec Care Dentist 2020 May 28;40(3):291-297. Epub 2020 Apr 28.

Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.

Aim: Cherubism is an uncommon hereditary disease that leads to the development of giant cell lesions in the jaws, alterations in the dentition, and malocclusion. The biological behavior of bones to orthodontic forces in these patients is not described in the literature, leading dentists to avoid this management. The aim of this article was to describe a case report of management with orthodontics. Read More

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http://dx.doi.org/10.1111/scd.12457DOI Listing

Frontal osteoplastic flap without frontal sinus obliteration for orbital roof decompression.

Orbit 2020 Apr 27:1-5. Epub 2020 Apr 27.

Division of Rhinology and Anterior Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Columbia University Irving Medical Center, New York, New York, USA.

: We describe a novel surgical approach for bilateral orbital roof decompression using a frontal osteoplastic flap without frontal sinus obliteration. This technique utilizes a combined external and endonasal endoscopic approach for wide exposure to the orbital roof bilaterally. We demonstrate this technique for the resection of a massive frontal fibrous dysplasia lesion in a healthy male with bilateral orbital roof involvement. Read More

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http://dx.doi.org/10.1080/01676830.2020.1757126DOI Listing

Visual Outcomes After Endoscopic Endonasal Resection of Orbital Lesions.

World Neurosurg 2020 Apr 18. Epub 2020 Apr 18.

Division of Neurosurgery, McMaster University, Hamilton, Ontario, Canada. Electronic address:

Background: The endoscopic endonasal approach (EEA) has been increasing in popularity as an alternative to traditional transcranial and transorbital approaches in the treatment of orbital pathological entities. The purpose of the present study was to examine the outcomes of patients who had undergone EEA resection of orbital lesions at our center.

Methods: We performed a retrospective medical record review of patients who had undergone the EEA for resection of orbital lesions and orbital apex decompression from January 1, 2006 to December 1, 2019. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.050DOI Listing

A rare case of polyostotic fibrous dysplasia detected on F-rhPSMA-7 PET/CT.

Eur J Nucl Med Mol Imaging 2020 Apr 16. Epub 2020 Apr 16.

Department of Nuclear Medicine, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Ismaninger Straße 22, 81675, Munich, Germany.

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http://dx.doi.org/10.1007/s00259-020-04751-9DOI Listing

PET imaging of phosphodiesterase-4 identifies affected dysplastic bone in McCune-Albright syndrome, a genetic mosaic disorder.

J Nucl Med 2020 Apr 13. Epub 2020 Apr 13.

Skeletal Disorders and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research, United States.

McCune-Albright syndrome (MAS) is a mosaic disorder arising from gain-of-function mutations in the GNAS gene, which encodes the 3', 5'-cyclic adenosine monophosphate (cAMP) pathway-associated G-protein, Gsα. Clinical manifestations of MAS in a given individual, including fibrous dysplasia, are determined by the timing and location of the GNAS mutation during embryogenesis, the tissues involved, and the role of Gsα in the affected tissues. The Gsα mutation results in dysregulation of the cAMP signaling cascade, leading to upregulation of phosphodiesterase type 4 (PDE4), which catalyzes the hydrolysis of cAMP. Read More

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http://dx.doi.org/10.2967/jnumed.120.241976DOI Listing

A case of recalcitrant fibrous dysplasia treated with denosumab.

Joint Bone Spine 2020 Jul 27;87(4):369-370. Epub 2020 Mar 27.

Department of Endocrinology, Vellore, Tamil Nadu, India. Electronic address:

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http://dx.doi.org/10.1016/j.jbspin.2020.03.007DOI Listing

[Application of 3D computer-assisted printing technique combined with plastic titanium mesh in the reconstruction of maxillary defect].

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2020 Mar;55(3):200-204

Department of Otorhinolaryngology Head and Neck Surgery, Shanghai Ninth People's Hospital, Shanghai JiaoTong University School of Medicine; Ear Institute, Shanghai JiaoTong University School of Medicine; Shanghai Key Laboratory of Translational Medicine on Ear and Nose Diseases, Shanghai 200011, China.

To investigate the application and clinical outcomes of using 3D computer-assisted printing technique combined with plastic titanium mesh in the reconstruction of maxillary defect. Clinical data of 14 patients in Department of Otorhinolaryngology Head and Neck Surgery, Shanghai Ninth People's Hospital from January 2016 to June 2018, who were treated for partial or total removal of the maxilla due to benign or malignant tumors and those acquired maxillary defects caused by severe compound trauma were analyzed retrospectively. Twelve males and 2 females were included, with the age ranging from 16 to 51 years old. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1673-0860.2020.03.003DOI Listing

Surgical resection for advanced bisphosphonate-related osteonecrosis of the jaw associated with fibrous dysplasia: a case report.

J Surg Case Rep 2020 Mar 28;2020(3):rjaa061. Epub 2020 Mar 28.

Department of Oral and Maxillofacial Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

Bisphosphonate-related osteonecrosis of the jaw (BRONJ) is an adverse drug reaction represented by destruction and/or death of bone. Fibrous dysplasia (FD) is a rare bony disorder characterised by abnormal fibro-osseous tissue that has lowered resistance to infection. Effective treatments for BRONJ that follows FD are unclear. Read More

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http://dx.doi.org/10.1093/jscr/rjaa061DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7103417PMC

Lower limb lengthening and deformity correction in polyostotic fibrous dysplasia using external fixation and flexible intramedullary nailing.

J Orthop 2020 Sep-Oct;21:192-198. Epub 2020 Mar 28.

Russian Ilizarov Scientific Centre for Restorative Traumatology and Orthopaedics, 6, M. Ulyanova Street, 640014, Kurgan, Russian Federation.

The study describes preliminary experience of the use of external fixators for limb lengthening and deformity correction in combination with flexible intramedullary nailing in management of polyostotic fibrous dysplasia.

Patients And Methods: The retrospective study included 8 patients (mean age 11.6 ± 3. Read More

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http://dx.doi.org/10.1016/j.jor.2020.03.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7125318PMC

A large maxillary cemento-ossifying fibroma superimposed with solitary bone cyst documented over 18 years: A case report.

Int J Surg Case Rep 2020 7;68:257-262. Epub 2020 Mar 7.

Centre for Oral and Maxillofacial Surgery, University of Dental Medicine and Oral Health, Danube Private University, Steiner Landstraße 124, 3500 Krems-Stein, Austria. Electronic address:

Introduction: Cemento-ossifying fibromas are rare, benign lesions of the head and neck regions. The origin of these lesions can be traced to the periodontal ligament, because its cells are able to form cement, lamellar bone, or fibrous tissue.

Case Presentation: A rare case report of a young Caucasian female with a COF is described including follow-ups. Read More

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http://dx.doi.org/10.1016/j.ijscr.2020.03.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7082601PMC

[Hereditary predispositions to follicular thyroid tumors].

Ann Pathol 2020 Apr 17;40(2):142-147. Epub 2020 Mar 17.

Service d'anatomie et cytologie pathologique, hospices civils de Lyon, centre hospitalier Lyon-Sud, 165, chemin grand revoyet, 69310 Pierre-Bénite, France; Inserm1052 CNRS5286, centre de recherche en cancérologie de Lyon, université Lyon 1, Lyon, France. Electronic address:

Familial thyroid cancers of follicular origin are rare and include syndromic and non-syndromic tumours. In familial adenomatous polyposis, the prevalence of papillary thyroid cancer is 2-12% and in 20-40% of cases it is a cribriform-morular papillary thyroid carcinoma. Morules and cribriform pattern are the two main typical criteria, associated with a nuclear and cytoplasmic immunopositivity for beta catenin. Read More

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http://dx.doi.org/10.1016/j.annpat.2020.02.011DOI Listing

Focal cemento-osseous dysplasia.

J Oral Maxillofac Pathol 2020 Feb 28;24(Suppl 1):S19-S22. Epub 2020 Feb 28.

Department of Oral and Maxillofacial Pathology, Adhiparasakthi Dental College and Hospital, Kanchipuram, Tamil Nadu, India.

Focal cemento-osseous dysplasia (FCOD) is a benign fibro-osseous lesion of bone characterized by the replacement of normal bone by fibrous tissue and subsequently followed by its calcification with osseous and cementum-like material. It is mostly asymptomatic in nature and requires no treatment. When secondarily infected, it becomes symptomatic and intervention is required. Read More

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http://dx.doi.org/10.4103/jomfp.JOMFP_209_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7069129PMC
February 2020

Endoscopic Optic Nerve Decompression for Optic Neuropathy in Sinonasal Fibro-Osseous Tumors.

World Neurosurg 2020 Jun 24;138:e260-e266. Epub 2020 Feb 24.

Department of Otorhinolaryngology-Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea. Electronic address:

Background: Sinonasal fibro-osseous tumors involving the skull base sometimes result in the encasement of the optic canal and can cause the compressive optic neuropathy. This study aimed to elucidate the optimal timing of endoscopic optic nerve decompression (OND) in cases with optic neuropathy caused by fibro-osseous tumors.

Methods: Medical records were reviewed retrospectively from July 2008 through November 2016. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.02.080DOI Listing

Considerations on bone volume normalization in quantifying skeletal burden in fibrous dysplasia using sodium fluoride PET/CT.

Eur J Nucl Med Mol Imaging 2020 Jun 25;47(6):1351-1352. Epub 2020 Feb 25.

Section of Nuclear Medicine, Dept. of Radiology, Leiden University Medical Center (LUMC), Leiden, the Netherlands.

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http://dx.doi.org/10.1007/s00259-020-04730-0DOI Listing

Spectrum of histological features of Denosumab treated Giant Cell Tumor of Bone; potential pitfalls and diagnostic challenges for pathologists.

Ann Diagn Pathol 2020 Apr 14;45:151479. Epub 2020 Feb 14.

Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan. Electronic address:

Objectives: Denosumab is Receptor Activator of Nuclear factor Kappa-B Ligand (RANKL) inhibitor which is being used in the treatment of locally advanced, recurrent and metastatic Giant Cell Tumor of Bone (GCTB). It causes reduction in monocyte recruitment and Osteoclast-Like Giant Cell (OLGC) formation which limits bone destruction. After Denosumab treatment, GCTB exhibit diverse morphological features which can pose diagnostic challenge. Read More

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http://dx.doi.org/10.1016/j.anndiagpath.2020.151479DOI Listing
April 2020
1.112 Impact Factor