scholarly journals Fibrous Dysplasia with Massive Cartilaginous Differentiation (Fibrocartilaginous Dysplasia) in the Proximal Femur: A Case Report and Review of the Literature

2016 ◽  
Vol 9 (1) ◽  
pp. 126-133 ◽  
Author(s):  
Hideo Morioka ◽  
Yasuhiro Kamata ◽  
Kazumasa Nishimoto ◽  
Michiro Susa ◽  
Kazutaka Kikuta ◽  
...  
Keyword(s):  
Fibrous Dysplasia ◽  
Proximal Femur ◽  
Fibrous Tissue ◽  
Bone Lesion ◽  
Pathological Findings ◽  
Review Of The Literature ◽  
Radiographic Findings ◽  
Fibrocartilaginous Dysplasia ◽  
Multiple Enchondromatosis ◽  
Spindle Cell Proliferation

Fibrous dysplasia (FD) is a monostotic or polyostotic benign bone lesion with spindle-cell proliferation in woven bone and stroma. Rarely, cartilaginous differentiation can be seen in the lesions of FD. FD with massive cartilaginous differentiation is called fibrocartilaginous dysplasia (FCD) and is considered a rare variant of FD. Although pathological findings of FD show irregular immature bone formation without osteoblastic rimming in fibrous tissue, and rarely show very small amounts of cartilage, histological images of FCD are said to show that cartilage with a relatively high cell density is present in the majority and that FD-like findings are seen in parts of it. The most characteristic feature of FCD on images is calcification in the lesions reflecting cartilaginous tissue. On the other hand, typical radiographic findings of FD include shadows with a ground-glass appearance and thinning and bulging of the cortical bone, the observation if calcification is not usual. Therefore, in the diagnosis of FCD, differentiation from multiple enchondromatosis, Ollier disease, chondrosarcoma, and chondrosarcoma secondary to FD is necessary, and it seems important to make a careful diagnosis based not only on the pathological findings but also on imaging and clinical findings. Herein, we report on a case of FD of the proximal femur associated with intralesional extensive cartilaginous differentiation in which a pathological fracture occurred during follow-up, with a review of the literature.

scholarly journals Fibrocartilagenous dysplasia in distal femur: A rare histopathological finding

2021 ◽  
Vol 6 (3) ◽  
pp. 222-224
Author(s):  
Shilpa Mishra ◽  
Rakesh Rajiv Patkar ◽  
Amrita Neelakantan
Keyword(s):  
Fibrous Dysplasia ◽  
Rare Variant ◽  
Pathological Fracture ◽  
Distal Femur ◽  
Histopathological Examination ◽  
Corrective Osteotomy ◽  
Bone Lesion ◽  
Bone Tumours ◽  
Fibrocartilaginous Dysplasia ◽  
Malignant Bone Tumours

Fibrocartilaginous dysplasia (FCD) or massive cartilaginous differentiation in fibrous dysplasia are interchangeably used terms. It is a rare variant of fibrous dysplasia (FD) which is benign, lytic, and expansile bone lesion and causes progressive deformity in the bones and may lead to pathological fracture. Radiologically, FCD may confused with cartilaginous benign and malignant bone tumours. FCD usually shows calcification in imaging. Surgical curettage or corrective osteotomy and histopathological examination of these lesions is necessary to differentiate it from other cartilaginous tumours. Here we report case of fibrocartilaginous dysplasia of distal femur in a 4-year-old male child.

Fibrous Dysplasia of the Temporal Bone: The use of Computerized Tomography

1984 ◽  
Vol 92 (4) ◽  
pp. 461-467 ◽  
Author(s):  
Paul R. Lambert ◽  
Derald E. Brackmann
Keyword(s):  
Fibrous Dysplasia ◽  
Temporal Bone ◽  
Computerized Tomography ◽  
Clinical Course ◽  
Fibrous Tissue ◽  
Rare Condition ◽  
Significant Advance ◽  
Review Of The Literature ◽  
Secondary Complications ◽  
Recommended Treatment

Fibrous dysplasia of the temporal bone is a rare condition characterized histologically by proliferation of fibrous tissue with scattered trabeculae of immature bone. Eighteen cases of monostotic fibrous dysplasia of the temporal bone have been reported in the literature. The clinical course of temporal bone fibrous dysplasia is unpredictable. Potential complications include cholesteatoma, recurrence, and malignant transformation. Surgery has been the recommended treatment, but the indications, approach, and extent have not been clearly established. The introduction of computerized tomography with high-resolution bone reconstruction is a significant advance in the therapeutic approach to temporal bone fibrous dysplasia. It accurately defines the extent of the disease within the temporal bone, and periodic scanning will reveal any progression. This information can be used to resolve many surgical dilemmas and to minimize secondary complications. This article includes a comprehensive review of the literature on temporal bone fibrous dysplasia and summarizes a case in which computerized tomography was used.

Hydrocephalus in Spinal Muscular Atrophy: A Case Report and Review of the Literature

2020 ◽  
Author(s):  
Jeetendra P. Sah ◽  
Aaron W. Abrams ◽  
Geetha Chari ◽  
Craig Linden ◽  
Yaacov Anziska
Keyword(s):  
Spinal Muscular Atrophy ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Muscular Atrophy ◽  
Communicating Hydrocephalus ◽  
Type I ◽  
Review Of The Literature ◽  
Radiographic Findings ◽  
Comprehensive Review ◽  
The Relationship

AbstractIn this article, we reported a case of spinal muscular atrophy (SMA) type I noted to have tetraventricular hydrocephalus with Blake's pouch cyst at 8 months of age following intrathecal nusinersen therapy. The association of hydrocephalus with SMA is rarely reported in the literature. Development of hydrocephalus after intrathecal nusinersen therapy is also reported in some cases, but a cause–effect relationship is not yet established. The aim of this study was to describe the clinical characteristics of a patient with SMA type I and hydrocephalus, to review similar cases reported in the literature, and to explore the relationship between nusinersen therapy and development of hydrocephalus. The clinical presentation and radiographic findings of the patient are described and a comprehensive review of the literature was conducted. The adverse effect of communicating hydrocephalus related to nusinersen therapy is being reported and the authors suggest carefully monitoring for features of hydrocephalus developing during the course of nusinersen therapy.

scholarly journals Lesion with blue bone-a case report

2018 ◽  
Vol 8 (1) ◽  
pp. 1323-1325
Author(s):  
Laila Mohamed Ilias ◽  
Babitha Alingal Mohammed ◽  
Roshini PS ◽  
Anupama Ponniah ◽  
Poornima Vijayan
Keyword(s):  
Histopathological Examination ◽  
Fibrous Tissue ◽  
Bone Lesion ◽  
Metacarpal Bone ◽  
Cortical Surface ◽  
Bizarre Parosteal Osteochondromatous Proliferation ◽  
X Ray ◽  
Spindle Cells ◽  
Small Bones ◽  
Hands And Feet

Bizzare parosteal osteochondromatous proliferation, or Nora‘s lesion is a unique bone lesion that most often arises in the small bones of hands and feet.  It is characterised by proliferation of chondroid, bony and fibrous tissue, and is occasionally misdiagnosed as a malignant process.  Our case was a 31 yr old lady, who presented with a painless swelling near the 5th metacarpal bone of right hand.  X-ray showed well marginated mineralised mass arising from the cortical surface of the metacarpal bone.  Histopathological examination revealed bizarre parosteal osteochondromatous proliferation composed of varying amounts of cartilage, bone and spindle cells. Cartilage was hypercellular and chondrocytes were enlarged. Ossification was irregular and had a peculiar blue tinctorial quality. 

scholarly journals Individualized approach to the surgical management of fibrous dysplasia of the proximal femur

2018 ◽  
Vol 13 (1) ◽  
Author(s):  
Bas C. J. Majoor ◽  
Andreas Leithner ◽  
Michiel A. J. van de Sande ◽  
Natasha M. Appelman-Dijkstra ◽  
Neveen A. T. Hamdy ◽  
...  
Keyword(s):  
Fibrous Dysplasia ◽  
Surgical Management ◽  
Proximal Femur ◽  
Individualized Approach

Visual dysfunction: Abnormal sign of benign spheno-ethmoidal lesions

1990 ◽  
Vol 104 (3) ◽  
pp. 217-221 ◽  
Author(s):  
Mohamed Rifai ◽  
Beshr Kenawi
Keyword(s):  
Fibrous Dysplasia ◽  
Review Of The Literature ◽  
Visual Dysfunction ◽  
Visual Defects ◽  
Orbital Region

AbstractIsolated visual dysfunction with or without proptosis is rarely described as a sign of benign sphenoethmoidal lesions. Five patients with benign swelling of the sino-orbital region and related visual defects are reported. Cases include one patient with fibrous dysplasia, two patients with mucocoeles and two patients suffering from aspergillosis of the sinuses. A review of the literature and discussion of the cases is presented.

Hypervitaminosis A in the cat: A case report and review of the literature

2005 ◽  
Vol 7 (6) ◽  
pp. 363-368 ◽  
Author(s):  
Zoe S. Polizopoulou ◽  
George Kazakos ◽  
Michael N. Patsikas ◽  
Nikolaos Roubies
Keyword(s):  
Normal Limit ◽  
Serum Vitamin ◽  
Review Of The Literature ◽  
New Bone Formation ◽  
Hypervitaminosis A ◽  
Nerve Roots ◽  
Canned Food ◽  
Radiographic Findings ◽  
Forelimb Muscles

A case of hypervitaminosis A with secondary entrapment and compression of the left brachial plexus nerve roots is described. A 9-year-old male castrated domestic shorthair, fed a home-made diet based on raw pork liver, was submitted for examination for a left forelimb lameness that evolved to paralysis over a 2-month period. Clinical examination revealed a flaccid paralysis and atrophy of all left forelimb muscles. An ipsilateral Horner's syndrome was also noted. Radiological examination of the cervical and thoracic spine showed massive new bone formation at the ventral aspect of the second cervical to sixth thoracic vertebra. The diagnosis of hypervitaminosis A was made, based on the clinical and radiographic findings, as well as the determination of serum vitamin A concentration, which was 630 μg/dl, three times above the upper normal limit for this species. Despite the unfavourable initial prognosis, the cat progressively regained function of the affected limb approximately 6 months after the diet was changed to a commercial canned food.

scholarly journals Fibrous dysplasia and cherubism

2015 ◽  
Vol 48 (03) ◽  
pp. 236-248 ◽  
Author(s):  
Surajit Bhattacharya ◽  
R K Mishra
Keyword(s):  
Optic Nerve ◽  
Fibrous Dysplasia ◽  
Fibrous Tissue ◽  
Genetic Research ◽  
Young Patients ◽  
Disease Process ◽  
Surgical Excision ◽  
Nerve Compression ◽  
Active Management ◽  
Definitive Treatment

ABSTRACTFibrous dysplasia (FD) is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect in FD is post-natal mutation of GNAS1 gene, which leads to the proliferation and activation of undifferentiated mesenchymal cells arresting the bone development in woven phase and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation. The disease may present in two distinct forms, a less severe and limited monostotic form, and a more aggressive and more widespread polyostotic form. Polyostotic form may be associated with various endocrine abnormalities, which require active management apart from the management of FD. Management of FD is not free from controversies. While total surgical excision of the involved area and reconstruction using newer micro-vascular technique is the only definitive treatment available from the curative point of view, but this can be only offered to monostotic and very few polyostotic lesions. In polyostotic varieties on many occasions these radical surgeries are very deforming in these slow growing lesions and so their indication is highly debated. The treatment of cranio-facial fibrous dysplasia should be highly individualized, depending on the fact that the clinical behavior of lesion is variable at various ages and in individual patients. A more conservative approach in the form of aesthetic recontouring of deformed bone, orthodontic occlusal correction, and watchful expectancy may be the more accepted form of treatment in young patients. Newer generation real-time imaging guidance during recontouring surgery adds to accuracy and safety of these procedures. Regular clinical and radiological follow up is required to watch for quiescence, regression or reactivation of the disease process. Patients must be warned and watched for any sign of nerve compression, especially visual impairment due to optic nerve compression. Rather than going for prophylactic optic canal decompression (which does more harm than good), optic nerve decompression should be done in symptomatic patients only, and preferably be done via minimal invasive endoscopic neuro-surgical approach than the conventional more morbid open craniotomy approach. There is growing research and possibilities that newer generation bisphosphonate medication may change the management scenario, as these medications show encouraging response in not only reducing the osteoclastic activity, but simultaneously also stimulating the osteoblastic and osteocytic activities. The explosion of genetic research and stem cell therapy may lead to better understanding and subsequently better treatment of FD in future.

scholarly journals Fibrous dysplasia of the maxilla: a case report

2016 ◽  
Vol 33 (01) ◽  
pp. 037-040
Author(s):  
R. Sousa ◽  
R. Tavares ◽  
C. Lins
Keyword(s):  
Computed Tomography ◽  
Fibrous Dysplasia ◽  
Maxillary Antrum ◽  
Morphological Changes ◽  
Bone Lesion ◽  
Facial Asymmetry ◽  
Posterior Wall ◽  
Normal Morphology ◽  
Normal Bone ◽  
Benign Bone Lesion

Abstract Introduction: Fibrous dysplasia is a benign bone lesion characterized by replacement of normal bone by fibrous connective tissue, and its diagnosis is based on clinical, radiological and histological findings. Objective: The aim of this study was to report a case of unilateral fibrous dysplasia in the maxilla, in the palate region, by using computed tomography. Results: On examination it was observed: a nodular lesion, with similar staining to the palatal mucosa with varicosities, regular edges, irm and painless. The radiographic indings on computed tomography showed one diffuse and heterogeneous thickening of the bony elements involving the hard palate extending to the posterior wall of the maxillary antrum. We opted for the preservation of the case, considering the age of the patient, the absence of facial asymmetry and lack of aesthetic and functional impairment. Conclusion: Thus, we emphasize that the knowledge of morphological changes is important for the diagnosis of bone pathologies, and the dentist must be familiar with the normal morphology of the structures and their possible abnormalities.

Sign in / Sign up

Export Citation Format

Share Document