Fibrous dysplasia of sphenoid bone – diagnostic difficulties

Wardas, Piotr; Piotrowska-Seweryn, Agnieszka; Markowski, Jarosław; Ślaska-Kaspera, Aleksandra; Golonka, Agnieszka; Dorosz, Robert; Sokołowski, Michał

doi:10.5604/20845308.1136064

Cited by 2 publications

(4 citation statements)

References 17 publications

(30 reference statements)

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“…FD shows low signal intensity on T1-weighted MRI and varying signal intensity on T2-weighted MRI. This has been explained by the various amounts of collagen fibers inside the cells and various formation of bony trabeculae [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…Actual data based on genetic and molecular analyses suggest that pathological regulation of intercellular cAMP or protein kinase dysfunction contributes to FD. Other studies revealed a somatic activating mutation within the GNAS1 gene located on chromosome 20 [ 6 , 10 ]. In 2015, Pardo-Maza et al [ 11 ] described a case of FD in which the pathologic process was suggested to be a late complication of temporal bone cholesteatoma surgery.…”

Section: Introductionmentioning

confidence: 99%

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Different clinical presentation and management of temporal bone fibrous dysplasia in children

et al. 2018

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BackgroundFibrous dysplasia is a slowly progressive benign fibro-osseous disorder that involves one or multiple bones with a unilateral distribution in most cases. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Temporal bone involvement is the least frequently reported type, especially in children.We reviewed available articles regarding fibrous dysplasia with temporal bone involvement in children and added four patients aged 7 to 17 years who were diagnosed and treated in our institution from 2006 to 2017. The patients’ clinical picture comprised head deformity, external canal stenosis, headache, progressive conductive and/or sensorineural hearing loss, tinnitus, and sudden deafness. Two patients had experienced severe episodic vertigo with nausea and vomiting. Two were referred to us with external canal obstruction and secondary cholesteatoma formation with broad middle ear destruction. One was diagnosed with acute mastoiditis and intracranial complications. Optimal management of fibrous dysplasia is unclear and can be challenging, especially in children. In our two patients with disease expansion and involvement of important structures, surgical treatment was abandoned and a “wait-and-scan” policy was applied. The other two were qualified for surgical treatment. One patient underwent two surgeries: modified lateral petrosectomy (canal left open) with pathological tissue removal, cavity obliteration, and subsequent tympanoplasty. Another patient with extensive destruction of the left temporal bone underwent canal wall down mastoidectomy with perisinus abscess drainage and revision 12 months later. Tympanoplasty was unsatisfactory in both patients because of slow progression of the middle ear pathology. None of our patients underwent pharmacological treatment.ConclusionsIn younger patients, observation and a “wait-and-scan” protocol is relevant until significant function, or cosmetic deficits are obvious. Surgery is not preferred and should be delayed until puberty because fibrous dysplasia has a tendency to stabilize after adolescence. In patients with severe symptoms medical treatment can be implemented, but safety of this treatment in children remain controversial.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Different clinical presentation and management of temporal bone fibrous dysplasia in children

et al. 2018

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“…Современные данные, основанные на генетическом и молекулярном анализе, свидетельствуют о том, что развитию ФД способствует патологическая регуляция межклеточного цАМФ или дисфункция протеинкиназы. Другие исследования выявили соматическую активирующую мутацию в гене GNAS1, расположенном в 20-й хромосоме [8,12].…”

Section: резюме _____________________________________________________...unclassified

“…Ревизия среднего уха и/или тимпаномастоидэктомия могут выполняться при распространении патологического процесса на среднее ухо или для удаления холестеатомы [2]. По данным обзора литературы, в 56% случаев было отмечено улучшение слуха после закрытой санирующей операции с тимпанопластикой (canal wall up mastoidectomy), в то время как после открытой санирующей операции с тимпанопластикой (canal wall down mastoidectomy) улучшение слуха наблюдалось в 100% случаев [12,26,27,30,31].…”

Section: резюме _____________________________________________________...unclassified

Fibrous Dysplasia of Temporal Bone: Literature Review

Диаб¹,

Дайхес²,

Сайдулаев³

et al. 2022

Оториноларингология. Восточная Европа

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Фиброзная дисплазия (ФД) – это медленно прогрессирующее доброкачественное заболевание костной ткани неизвестной этиологии, неопределенного патогенеза и разнообразной гистопатологической картины, при котором поражается 1 или несколько костей. ФД чаще поражает кости черепа, длинные кости и ребра. Поражение височной кости при фиброзной дисплазии наблюдается реже и обычно бывает односторонним.Клинически ФД височной кости (ВК) характеризуется деформацией пораженной области, стенозом наружного канала, головной болью, прогрессирующей кондуктивной и/или нейросенсорной тугоухостью, шумом в ушах, в некоторых случаях головокружением с тошнотой и рвотой. Диагностика ФД проводится по данным клинических, рентгенологических, гистологических и генетических исследований. Оптимального лечения фиброзной дисплазии не существует, оно зависит от многих факторов. Хирургическое лечение проводится в объеме частичной резекции патологической ткани, каналопластики, мастоидэктомии, радикальной операции на среднем ухе, закрытой санирующей операции, тимпанопластики. Fibrous dysplasia is a slowly progressing benign bony disease of unknown etiology, uncertain pathogenesis and a diverse histopathological picture, in which one or more bones are affected. Fibrous dysplasia more often affects the bones of the skull, tubular bones and ribs. The lesion of the temporal bone with Fibrous dysplasia is observed less often and is usually unilateral.Clinically, fibrous dysplasia of temporal bone is characterized by deformity of the affected area, stenosis of the external auditory canal, headache, progressive conductive and/ or sensorineural hearing loss, tinnitus, in some cases – dizziness, nausea and vomiting. Diagnosis of fibrous dysplasia is carried out according to clinical, radiological, histological and genetic examination. There is no optimal treatment for fibrous dysplasia, it depends on many factors. In some cases surgical treatment is carried out: partial resection of pathological tissue, canaloplasty, canal wall up or canal wall down mastoidectomy, tympanoplasty.

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Fibrous dysplasia of sphenoid bone – diagnostic difficulties

Cited by 2 publications

References 17 publications

Different clinical presentation and management of temporal bone fibrous dysplasia in children

Different clinical presentation and management of temporal bone fibrous dysplasia in children

Fibrous Dysplasia of Temporal Bone: Literature Review

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