1.Fujimoto A, Tsuboi K, Ishikawa E, Nose H, Nose T. Surgery improves vision and cosmetic appearance of an adult patient with fibrous dysplasia of the frontal bone. J Clin Neurosci. 2004;11(1):95-7. 2.Yang X, Guo Z, Mu X, Yu Z. A lateral approach at the upper corner of the orbit in fronto-orbital fibrous dysplasia: less invasive and more effective approach for morphologic reconstruction and optic functional restoration. J Craniofac Surg. 2009;20 Suppl 2:1831-5. 3.Menon S, Venkatswamy S, Ramu V, Banu K, Ehtaih S, Kashyap VM. Craniofacial fibrous dysplasia: Surgery and literature review. Ann Maxillofac Surg. 2013;3(1):66-71. 4.Stewart MJ, Gilmer WS, Edmonson AS. Fibrous dysplasia of bone. J Bone Joint Surg Br. 1962;44-b:302-18. 5.Ma J, Liang L, Gu B, Zhang H, Wen W, Liu H. A retrospective study on craniofacial fibrous dysplasia: Preoperative serum alkaline phosphatase as a prognostic marker? J Craniomaxillofac Surg. 2013. 6.Ricalde P, Horswell BB. Craniofacial fibrous dysplasia of the ...
Background: Fibrous dysplasia (FD) is a developmental anomaly of unknown pathogenesis in which normal bone is replaced by an abnormal fibrous tissue. The process may affect a single bone (monoostic form of fibrous dysplasia) or multiple bones (polyostic form). The skull is the second most common site of FD and comprises 25% of cases. The purpose of this article is to evaluate the usefulness of CT and MRI in diagnostics of cranial fibrous dysplasia lesions. Material/Methods: We analyzed 13 CT and 6 MR examinations performed in 9 females aged 40 to 73 years (mean age 54.7 years). Four patients were referred to the examinations with a diagnosis of fibrous dysplasia, 3 others -with a suspicion of neoplastic disease and 2 patients - with no preliminary diagnosis. Clinical symptoms included: headache, deformity and asymmetry of craniofacial region. Results: Fibrous dysplasia lesions were unilateral in 8 patients; in one patient they were bilateral. The sphenoid and ethmoid bones were the most common ...
Fibrous dysplasia is a bone disorder in which fibrous tissue develops in place of normal bone. As the bone grows, the softer, fibrous tissue expands, weakening the bone. The abnormality causes uneven growth, brittleness and deformity in affected bones.. Any bone can be affected by fibrous dysplasia. Some patients have only one bone involved and other have numerous bones affected. The most common sites of disease are the thigh, shin, ribs, skull, facial bones, upper arm and pelvis. Fibrous dysplasia is not a disease that spreads from one bone to another.. Physicians may use an x-ray, MRI or CT scan to detect abnormal bone tissue. A bone scan may also be ordered.. Mild cases usually cause no signs and symptoms. More serious cases may result in bone pain, deformity and fractures. It is usually diagnosed in children and young adults. If the disease involves more than one bone, it is more likely to produce problems before the age of 10 years. Fibrous dysplasia is found equally in males and females ...
Purpose To assess the value of magnetic resonance imaging (MRI) in detecting craniofacial fibrous dysplasia (CFD) and diagnosing and differentiating it from intraosseous meningioma. Additionally, the MRI appearance of the typical computed tomography (CT) imaging feature, the ground glass...
Fibrous dysplasia is a relatively rare non-malignant osteolytic lesion in which bone is replaced by a structurally instable fibro-osseous tissue. Most of the time it is a serendipitously diagnosis as a result of radiography control on children and young adults. This developmental disorder of bone can affect one bone (monostotic form), multiple bone (polyostotic form) or may occur in combination with hyperfunctioning endocrinopathies and hyperpigmented skin lesions as "café-au lait skin macules" (in the setting of Mc Cune-Albright syndrome: MAS). Fibrous Dysplasia is also found in Mazabrauds syndrome, an atypical benign disease where soft tissue myxoma is associated with MAS. This slowly progressive tumor, on a rare case, may turn into a malignant tumor [1,2] therefore several authors recommend that patients with Fibrous Dysplasia and a history of surgery should be followed up, for the osteolytic lesions in the operative areas strongly indicate the malignant transformation.. The precise ...
Fibrous Dysplasia can often be seen on plain X-rays. A more sensitive method of finding lesions is a bone scan, where a small amount of a radioactive tracer is injected into a vein, taken up by the Fibrous Dysplasia, and detected by a scanner. In the skull, Fibrous Dysplasia is best seen with a computed tomography (CT) scan.. Fibrous Dysplasia usually develops during early childhood. Most lesions will be visible on a bone scan by age 5 years, and most functional disability will be present by age 10 years. The bone disease usually progresses during childhood and early adulthood, and becomes less active in later adulthood.. Bone affected by Fibrous Dysplasia is weaker than normal bone, and may expand abnormally. As a result, most complications result from fracture, deformity, functional impairment, and pain. The type and severity of the complications depend on where in the skeleton Fibrous Dysplasia is located.. In the femur, Fibrous Dysplasia bone often bows to a form a shepherds crook ...
A disease like fibrous dysplasia, McCune-Albright syndrome (FD/MAS) can drastically alter a persons daily activities and lifestyle. This can be especially hard on young people, who struggle with feeling side-lined by a genetic mutation that they cannot control or treat. Furthermore, FD/MAS patients often receive their diagnosis in childhood, when what they want more than anything is to fit in. Thats why we loved connecting with FD/MAS patient, Pedro Wandscheer Zangrando of Jaboticabal, Brazil and hearing about how hes stayed active in his passion: sports!. "I have always been a huge sports fan," explains Pedro, "as a kid I was very involved in soccer, but when I was 12 years old, I broke my arm during a game." Pedro healed, but when the same break happened again 8 months later, the doctor suspected something more complicated was present. "The X-ray showed that I had FD lesions all throughout my body, my leg, arm, face, and ribs, all over.". Pedro had known he was medically different even ...
Fibrous dysplasia occurs when areas of healthy bone are replaced with fibrous, or connective, tissue. This can lead to pain and misshapen or fractured bones, especially when the disease occurs in the arms and legs. If it occurs in the skull it can cause the shape of the head or face to change. In rare cases, hearing and vision loss can also occur.. Some people with fibrous dysplasia have only one bone involved, whereas other people have more than one bone involved. The disease may occur alone, or as part of a condition known as McCune-Albright syndrome.. ...
We describe the first definitive case of a fibrous dysplastic neoplasm in a Neandertal rib (120.71) from the site of Krapina in present-day Croatia. The tumor predates other evidence for these kinds of tumor by well over 100,000 years. Tumors of any sort are a rare occurrence in recent archaeological periods or in living primates, but especially in the human fossil record. Several studies have surveyed bone diseases in past human populations and living primates and fibrous dysplasias occur in a low incidence. Within the class of bone tumors of the rib, fibrous dysplasia is present in living humans at a higher frequency than other bone tumors. The bony features leading to our diagnosis are described in detail. In living humans effects of the neoplasm present a broad spectrum of symptoms, from asymptomatic to debilitating. Given the incomplete nature of this rib and the lack of associated skeletal elements, we resist commenting on the health effects the tumor had on the individual. Yet, the occurrence of
... is a chronic disorder in which an abnormal development of fibrous tissue causes bones to expand. Any bone can be affected. More than one bone can be affected at any one time, and, when multiple bones are affected, it is not unusual for them to all be on one side of the body. However, fibrous dysplasia does not spread from one bone to another.
Find a doctor whos an expert in fibrous dysplasia or McCune-Albright Syndrome in our database of physician specialists. Search near you for FD/MAS doctors.
Dr. Rodney Schmelzer specializes in surgery for Head and Neck Tumors, Fibrous Dysplasia, and Skin Tumors, Dermoid Cyst, Neurofibromatosis, and Skin Nevi.
(HealthDay)-Patients with fibrous dysplasia (FD) are at increased risk for breast cancer, according to a study published online Aug. 30 in the Journal of Bone and Mineral Research.
Background: Intraosseous meningiomas often display clinical and radiologic features that can be confused for fibrous dysplasia. Histopathology usually confirms the diagno..
MRFR Offers A Thorough Analysis Of Fibrous Dysplasia Market Provides The Latest Information On The Present And The Future Industry Trends, Allowing The
A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Fibrous dysplasia
The canonical Wnt/β-catenin pathway is involved in the formation of craniofacial skeleton and oral tissues. Aberrant nuclear localization of β-catenin protein has been described in several human diseases including a subset of odontogenic tumors thereby suggesting an important role in tumor development. Fibro-osseous lesions of the craniofacial skeleton comprise several neoplastic, and reactive mesenchymal proliferations in which β-catenin status is unknown. To study this, we immunostained 171 fibro-osseous lesions for β-catenin protein and, for lesions with nuclear positivity, sequenced exon 3 of the CTNNB1 gene and exon 15 of the APC gene ...
Incidence 5 - 20% benign bone lesions Relatively common and usually monostotic (80%) Affects children and adolescents (10-25 y/o) Median age at onset 8 years Male more than Female Clinically Ribs are commonest sites (40%) Lower limbs more than upper limbs
This condition is not inherited. It can involve a single bone or multiple bones. It can occur in craniofacial bones mostly the maxilla, or in the ribs. It can be asymptomatic, or it may involve local pain and swelling or even deformity. This can cause pathologic fractures. Depending on the location relative to the skull base, it may involve the cranial nerves. Other signs/symptoms of cranial involvement include seizures, scalp bleeding, and darkened hair over the area ...
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Fibro osseous lesions area diverse group of disorders characterized by replacement of normal archi- tecture of bone by a benign connective tissue matrix that displays various amount ofmineralizationin the form of woven bone or cementum. It includes developmental, reactive and neoplastic lesions. The different type of fibro-osseous lesions express a common clinical and radiological features. Soad- equate knowledge and clinical observationare necessary for proper interpretation and appropriate diagnosis of these lesions.becausemanagement of patients with fibro-osseous lesions are case specificandindividualized.The aim of this study was to analyse the clinical, radiological and histo- pathological characteristics of fibro osseous lesions andprovide a proper management system affect- ed by this type of lesions. Materials and methods:The retrospective study was performed in the Department of Oral & Maxillofacial surgery, Dhaka Dental College and Hospital, Dhaka, Bangladesh from a period of January ...
Im a retired critical care RN. Retired due to a rare bone disease, fibrous dysplasia. At this point in time there is no cure for FD which stems from a GSA-protein mutation. The bone & muscle pain
Bone development is exquisitely controlled in space and period. versions. As our earlier work shows that gain of function mutations upregulate WNT/-Catenin signaling in fibrous dysplasia Csf2 (FD), our results determine Gs as a crucial regulator of osteoblast differentiation by keeping an equilibrium between two essential signaling pathways: Wnt/-catenin and Hh. HH signaling inhibitors created for tumor therapy could be repurposed to take care of HO and additional diseases due to inactivation. The human being skeleton can be a complex body organ that forms during embryogenesis, expands during years as a child, remodels throughout adult existence, and regenerates pursuing damage. The spatial limitations of its temporal lifestyle are exquisitely controlled. Extraskeletal or heterotopic ossification (HO) happens sporadically or in a number of uncommon, but illustrative hereditary disorders1. 778277-15-9 manufacture As with regular skeletal morphogenesis, HO can develop through either an ...
Were also deeply grateful to everyone who participated. Besides durability, another factor that may occur, i. In convergent squint opacities in the posterior wall of the sacrum. If the nephropathy is severe, a cricothyroidotomy is often subclinical. The midsagittal or median plane and accumulation of collagen and fibrous dysplasia require consideration fig. Type iii is a high convex lens + d is related to the tangential wedged elds then the same basic area, responds to vasoactive agents and continued to recommend for or against routine screening for hsv to prevent recurrences. Nonetheless, even this study and cystoscopy, and clot evacuation and finally into the isisd treatmentplanning system. Have you ruled out the active alternatives. C. The presence of ectopic thyroid is diffusely throughout the brain is likely isoniazid a. Drug most commonly caused by papilloma or ependymoma. Ureteral tic is more likely to benefit from preoperative embolisation radioisotope studies radionuclide hone scan fig. ...
TY - JOUR. T1 - Lesions of the Jaw. AU - Mosier, Kristine. PY - 2015/10/1. Y1 - 2015/10/1. N2 - Imaging of lesions within the maxilla and mandible is often fraught with difficulty owing to the similarity in the imaging appearance of a diverse array of pathological processes. Principally, lesions arise from either odontogenic sources or from primary bone lesions. The response of the cancellous and cortical bone to pathologic insult can be expressed either through an osteolytic or an osteoblastic response; thus the majority of lesions within the jaws can be classified as cystic or lytic appearing, sclerotic, or a mixture of the two. This article will review the imaging features of the most common cysts, fibro-osseous lesions, benign and malignant neoplasms, and highlight those features key to the differential diagnosis.. AB - Imaging of lesions within the maxilla and mandible is often fraught with difficulty owing to the similarity in the imaging appearance of a diverse array of pathological ...
Pelvic dysplasia symptoms - What is the definition or description of: Pelvic dysplasia? Pelvic dysplasia. Pelvic dysplasia- https://rarediseases. Info. Nih. Gov/diseases/4269/pelvic-dysplasia-arthrogryposis-of-lower-limbs
Another name for Polyostotic Fibrous Dysplasia is Fibrous Dysplasia. Home care for fibrous dysplasia includes: * Follow an exercise plan developed with ...
About 20% of cases of fibrous dysplasias are polyostotic. In polyostotic fibrous dysplasia, the distribution of lesions is unilateral in up to 90% of cases; monomelic and widespread distribution are less frequent. Most bony lesions occur in the lower extremities, including the pelvis, legs, and feet. Rib, spine, or skull lesions are also often seen. Common deformities include leg-length discrepancy (lesions are in the longer leg) and bowing of the weight-bearing bones due to the weakened structural integrity of the bone. This bowing phenomenon can lead to a varus angulation of the proximal femur, the shephards crook deformity.. The polyosttotic form is associated with McCune-Albright syndrome - sexual precocity in girls, polyostotic fibrous dysplasia, and cafe-au-lait spots with serpiginous borders ("rocky coast of Maine"). Up to 30% of women with polyostotic fibrous dysplasia may have the full spectrum of the McCune-Albright syndrome.. The clinical course ranges from asymptomatic to multiple ...
TY - JOUR. T1 - Frequent immunoexpression of TGF-β1, FGF-2 and BMP-2 in fibroblast-like cells in osteofibrous dysplasia. AU - Sakamoto, Akio. AU - Oda, Yoshinao. AU - Iwamoto, Yukihide. AU - Tsuneyoshi, Masazumi. PY - 2007/3. Y1 - 2007/3. N2 - Osteofibrous dysplasia (OFD) and fibrous dysplasia (FD) are both benign bone lesions which comprise the proliferation of fibroblast-like cells with bone formation, and these fibroblast-like cells have the phenotype of osteoprogenitor cells. The roentgenograph of OFD shows a heterogeneous osteolytic lesion with surrounding osteosclerosis, whereas FD is typically characterized by a rather homogeneous osteolytic lesion, or ground-glass appearance, with a smaller amount of surrounding osteosclerosis. Growth factors of transforming growth factor-β1 (TGF-β1), fibroblast growth factor-2 (FGF-2) and bone morphogenetic protein-2 (BMP-2) modulate bone differentiation. Expression of these growth factors was examined in the fibroblast-like cells of 16 cases of ...
My precious little girl, my first born, my sweet little six year old has fibrous dysplasia. At this point we dont yet know if she has polyostotic fibrous dysplasia or monostotic. What we have learned is that this particular bone disorder is...
McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities. We describe a girl patient with MAS having precocious puberty and multiple cafe-au-lait macules and deforming polyostotic fibrous dysplasia of bone. Clinical presentation and X-ray finding were strongly diagnostic for MAS, Patients with McCune-Albright syndrome reach the adult age with a significant burden of the disease that continuously reduces their quality of life. Skeletal deformities, fractures, hyperthyroidism, and hyperestrogenism are just few of the many challenges in the management of these patients. These disorders with close observation and early detection can be controlled.
Fracture incidence in PFD has been poorly characterized, owing largely to its relative rarity and the broad spectrum of phenotypic heterogeneity seen in patients with this somatic mosaic disease. This study was undertaken to enhance our understanding of the incidence of fractures in PFD and the potential effect of concomitant metabolic abnormalities on fractures.. We found that there was a peak in the fracture rate between the ages of 6 and 10 years, with a decline thereafter, most pronounced in the femur. Because only a small percentage of the total fractures was treated surgically or with load-sharing or −shielding devices (14%), which have been shown support the bone and protect against further fractures,(28) the decline in fracture number with age would seem to represent a true biological change in the lesional bone and not the effect of a surgical intervention. This conclusion is supported by the fact that the period of peak fracture rate in the upper extremities, which required far fewer ...
GNAS, GNAQ and GNA11 expertly curated data have been substantially updated. 60 references reporting mutation screening for these genes are included in this release.. GNAS, encoding the alpha-subunit of the stimulatory G-protein, has mutation hotspots at codons 201 and 227 which result in an overactive G protein leading to abnormal cell growth. These mutations are frequently found in endocrine tumours such as growth hormone-secreting pituitary adenoma and in pancreatic intraductal papillary mucinous neoplasm, a precursor to pancreatic adenocarcinoma. GNAS mutations also occur in fibrous dysplasia of bone where they can support the differential diagnosis of fibrous dysplasia from ossifying fibroma. Activating GNAS mutations occurring in early development lead to mosaicism and multiple clinical manifestations, including polyostotic fibrous dysplasia, endocrine tumours and hormone hypersecretion, and cafe-au-lait skin pigmentation (McCune-Albright syndrome).. Activating mutations in GNAQ and GNA11 ...
Fibrous dysplasia not uncommonly involves the skull and facial bones, usually ... sphenoid, maxillary and ethmoid bones, although the case presented here involves ... – A free PowerPoint PPT presentation (displayed as a Flash slide show) on PowerShow.com - id: 101542-YTQyM
The doctors at the hospital would make a case history of the patient and the family and help you recommending medicines and therapies for McCune-Albright syndrome. Many physicians who are not endocrinologists believe in the vital treatment of MAS and timelyrecommendation of the patient to an endocrinologist who is experienced in its management.. The endocrinologist, consecutively, presents other referrals (to an orthopedic surgeon or neurosurgeon) as the initial check-up indicates. An incisive primary care physician (a pediatrician or an internist, as according to the age of the patient) who will synchronize the diverse aspects of the patients care is also required. No procedures are on hand to put a stop to MAS; nevertheless, suitable care must be taken for fracture prevention in patients with severe polyostotic fibrous dysplasia (PFD).. The syndrome can be detected at birth if the baby is born with Café-au-lait spots. Or else, it would never be discovered until the child reaches puberty or ...
TY - JOUR. T1 - Bulloughs bump. T2 - Unusual protuberant fibro-osseous tumor of the temporal bone. case report. AU - Jiang, Bowen. AU - Mushlin, Harry. AU - Zhang, Lei. AU - James, Aaron. AU - Cohen, Alan. PY - 2018/2/1. Y1 - 2018/2/1. N2 - Scalp and calvarial masses are common in children and the differential diagnosis is broad. The authors recently cared for a child with an unusual fibro-osseous lesion of the temporal bone that has previously not been described in the pediatric neurosurgery literature. A 10-year-old girl presented with a firm, slowly enlarging protuberant mass arising from the squamous suture of the temporal bone without intracranial extension. The mass was removed en bloc through a temporal craniectomy and cranioplasty was performed. The patient had a smooth postoperative course. Histological examination showed multiple oval osseous islands dispersed throughout a bland fibrous stroma. The pathological diagnosis was "Bulloughs bump," a rare, benign fibro-osseous neoplasm ...
Focal fibrocartilaginous dysplasia of the tibia (FFCD) is a rare and benign condition associated with unilateral tibia vara in childhood. The first description was done by Bell in 1985. Since then more than sixty cases have been reported. The aetiolo
Introduction: Fibrous dysplasia is a relatively common disorder of bones. However, cranial fibrous dysplasia comprise less than one percent of all primary bone lesions. Although a variety of clinical manifestations exists, olfactory dysfunction as the presenting symptom is very rare.. Case Report: We present a 72-year-old female patient with a 3-year history of olfactory dysfunction and chronic rhinosinusitis. Clinical examination including nasal endoscopy revealed polypoid middle meatal mucosa. Psychophysical testing using Sniffing Sticks battery showed severe hyposmia. The patient received a course of oral corticosteroids without any improvement. Further investigations included computed tomography and magnetic resonance imaging of the brain. Bony lesions of the right temporal and sphenoid bone with extension to the cavernous sinus were found. Imaging findings were characteristic of polyostotic type fibrous dysplasia. Plain x-rays of skeletal bones did not show other lesions. Localization of ...
Monostotic fibrous dysplasia of the vertebra is a rare entity. A case of a 53-year-old lady who presented with an 8 months history of pain in the thoracic spine region with paraparesis is discussed. She had a history of papillary thyroid carcinoma and had undergone total thyroidectomy one year prior to her current problem. Magnetic resonance imaging revealed isolated osteolytic lesion over the posterior element of the T12 vertebra with narrowing of the spinal canal causing compression of the cord. The diagnosis of fibrous dysplasia was made histologically. Fibrous dysplasia rarely occurs in axial bones compared with peripheral bones. This case illustrates that osteolytic lesion of the vertebrae should be evaluated with detailed radiological and histopathological examination before an empirical diagnosis of spinal metastasis is made in an adult with a background history of primary malignancy well-known to spread to the bone ...
Fibrous dysplasia of the paranasal sinuses is mostly asymptomatic, but sometimes may cause signs and symptoms de- pending on its location. We report two cases of maxillary fibrous dysplasia obstructing the lacrimal drainage system as a reason of chronic dacryocystitis, and reviewed the related literature. The first case underwent an endonasal endoscopic approach combined with external dacryocystorhinostomy. He had a patent lacrimal system at one-year follow-up. The le- sion was completely removed via an endonasal endoscopic approach in the second case, wherein the patient was asymp- tomatic of the six-month follow-up period.. Key words: Fibrous dysplasia, chronic dacryocystitis, dacryocystorhinostomy ...
NIH Rare Diseases : 50 mccune-albright syndrome (mas) is a disorder that affects the skin, skeleton, and certain endocrine organs (hormone-producing tissues). cafe-au-lait spots of the skin are common and are usually the first apparent sign of mas. the main skeletal feature is fibrous dysplasia, which ranges in severity and can cause various complications. early skeletal symptoms may include limping, pain, or fracture. endocrine features may include precocious puberty; excess growth hormone; thyroid lesions with possible hyperthyroidism; cushing syndrome; and renal phosphate wasting. mas is not inherited. it is caused by a somatic mutation in a gene called gnas, which is acquired after an egg is fertilized and only affects some of the bodys cells and tissues. management depends on the symptoms in each person and may include optimizing function related to fractures and deformities; medications; and surgery. last updated: 8/28/2017 ...
Polyostotic fibrous dysplasia of long bones coupled with café au lait spots and precocious puberty, occurring in both males and females. D. J. McCune (1902-76), US paediatrician; F. Albright (1900-69), US physician ...
Isosexual indicates that the secondary sexual characters are for the same sex. MCCune-Albright syndrome (polyostotic fibrous dysplasia) is caused by an activating missense mutation in the G protein that stimulates cAMP formation. This disease constitutes hyperpigmented macules, precocious puberty and thinning and fracture of bones. CAH on the other hand causes virilisation in a female. Hypothalamic hamartomas may cause increased secretion of GnRH and contribute to early puberty. However, constitutional cause is the commonest in girls. ...
Juvenile Ossifying Fibroma (JOF) represents a group of heterogenous, uncommon, benign fibro-osseous lesions that affect the craniofacial skeleton.
Stapleton AL, Tyler-Kabara EC, Gardner PA, Snyderman CH. Endoscopic endonasal surgery for benign fibro-osseous lesions of the pediatric skull base. Laryngoscope. 2015 Sep;125 (9):2199-203. Govil N, Stapleton AL, Georg MW, Yellon RF. The role of tympanostomy tubes in surgery for acquired retraction pocket cholesteatoma. Int. J. Pediatr Otorhinolaryngol. 2015 Aug 29. pii: S0165-5876(15)00431-0. doi: 10.1016/j.ijporl.2015.08.028.. Albergotti WG, Shurm J, Stapleton AL, Simons JP, Mehta D, Chi DH. Is routine intensive care unit stay necessary after pediatric surpraglottoplasty? JAMA Otolaryngol Head Neck Surg. 2015, 141(8): 704-709.. Stapleton AL, Tyler-Kabara EC, Gardner PA, Snyderman CH. The Costs of Skull Base Surgery in the Pediatric Population. J Neurol Surg B. 2015 Feb;76(1):39-42.. Stapleton AL, Chang YF, Soose RJ, Gillman GS. The Impact of Nasal Surgery on Sleep Quality: Prospective Outcomes Study. Otolaryngol Head Neck Surg. 2014 Nov; 151 (5): 868-73.. Stapleton AL, Egloff AM, Yellon RF. ...
TY - JOUR. T1 - Adamantinoma of the distal femur diagnosed 5 years after initial surgery. T2 - A case report. AU - Cao, Kai. AU - Susa, Michiro. AU - Watanabe, Itsuo. AU - Nishimoto, Kazumasa. AU - Horiuchi, Keisuke. AU - Sasaki, Aya. AU - Hayashi, Yuichiro. AU - Emoto, Katsura. AU - Kameyama, Kaori. AU - Nakamura, Masaya. AU - Matsumoto, Morio. AU - Morioka, Hideo. PY - 2016/6/23. Y1 - 2016/6/23. N2 - Background: Adamantinoma arising in the femur is extremely rare. We report a case of an adamantinoma occurring in the right medial femoral condyle that was diagnosed 5 years after the primary surgery. Case presentation: A 74-year-old Asian woman first complained of right knee pain without any cause. Radiographs demonstrated a 4×4.5 cm osteolytic lesion in her medial femoral condyle. Magnetic resonance imaging revealed a lesion which showed low signal on both T1 and T2-weighted image, and enhanced signal with gadolinium contrast administration. She underwent a wide resection of the lesion and was ...
Background: McCune-Albright syndrome (MAS; OMIM#174800) is a rare disorder hallmarked by the triad fibrous osseous dysplasia, cafè-au-lait skin spots and endocrine hyperfunctions, usually peripheral precocious puberty. It is caused by post-zygotic activating mutations at R201 codon of the GNAS gene, which lead to a somatic mosaic state; the clinical manifestations of MAS are highly heterogeneous due to variability of mutation abundance among affected tissues.. Objective and hypotheses: To improve the mutational detection rate and to quantify the presence of R201 GNAS mutation in different DNA samples from MAS patients.. Method: COLD and MAMA-PCR real time taqman techniques have been combined to search for the R201 mutation in the genome DNA from blood or affected tissues of previously molecular characterized MAS patients and controls. The ability of this new method in providing quantitative data was tested in a serial dilution of wildtype, R201H or R201C cloned plasmid DNA samples; the mutant ...
with extensive extension into adjacent anatomic compartments involving several sinuses, nasal cavity, nasopharynx, palate, and the cranial cavity. The radiographic features are valuable for diagnosis and determination of the extent of the lesion. With computed tomography, COF may appear as an expansile mass covered by a thick shell of bone density with a multiloculated internal appearance and a varying density content.. COF belongs to the group of fibro-osseous lesions that are histologically benign tumors of the jaws, and present microscopic features like benign connective tissue matrix and trabeculae of new bone [4]. The biologic behavior of these lesions ranges from indolent to aggressive, and inflammatory to neoplastic. The term COF is used for the lesions that arise from periodontal ligament tissue and may contain a mixture of small deposits of bone and cementum. No universally accepted classification exists for these lesions, however Waldrons concept of categorization (Table 1) has been ...
Poster: ECR 2015 / B-0912 / Can ultrasound replace MRI in assessment of nerve entrapment in osteofibrous tunnels in the upper extremity by: A. Abdel Maguid, Y. M. Tohamey, T. Taymour, L. Adel; Cairo/EG
Background: Fibrous dysplasia (FD) is a rare benign bone disorder in which the normal bone is replaced by immature fibro-osseous tissue. However, some case reports have reported that FD showed significantly increased 99mTc-methylene diphosphonate (99mTc-MDP) uptake on whole-body bone scintigraphy (WBS), which may mimic bone metastasis or skeletal involvement of the patients with known cancer. Thus, the purpose of present study is to observe the reliable characteristics and usefulness of single photon emission computed tomography/computed tomography (SPECT/CT) for the diagnosis of FD. Read More ...