Fibrous dysplasia of the temporal bone secondary to ear surgery: a case report

Pardo-Maza, Adriana; Lassaletta, Luis; Ruiz‐Bravo, Elena; Pérez-Mora, Rosa; Peñarrocha, Julio; Gavilán, Javiér

doi:10.1186/s13256-015-0612-x

Cited by 9 publications

(13 citation statements)

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“…Pardo-Maza et al [ 11 ] described a case in which temporal bone FD developed 7 years after middle ear surgery involving cholesteatoma removal. This case emphasizes the importance of close follow-up after otologic surgery.…”

Section: Discussionmentioning

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%

“…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%

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

et al. 2018

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“…[ 19 30 ] Symptoms of aggressive lesions include worsening headaches, severe edema, mass effect, compromised vision, compromised hearing, and altered cosmetic appearance. [ 2 12 16 18 25 ] Our case offers 18 years of insight into the natural history of cranial FD with worsening mass effect and headaches manifesting from a growing lesion in the frontotemporal area.…”

Section: Discussionmentioning

confidence: 99%

“…In addition to our patient, several cases reported in the literature have involved monthly growth with worsening symptoms appearing closer to surgical intervention. [ 12 16 18 25 ] One interesting aspect of this particular case is the aggressive and invasive nature of the lesion observed intraoperatively, in conjunction with growth of the lesion over 18 years. Over years, the patient elected to monitor the lesion radiographically.…”

Section: Discussionmentioning

confidence: 99%

“…[ 2 11 ] The pathogenesis of the disease is known to involve a postzygotic mutation of the GNAS1 gene, resulting in alteration of biochemical pathways ultimately leading to constitutive activation of G proteins and proliferation of undifferentiated mesenchymal cells. [ 9 18 28 ] Mutations occur sporadically and postzygotically resulting in mosaicism and highly variable presentations. [ 13 29 ] The presence of these cells ultimately results in abnormal bone matrix and growth of abnormal tissue.…”

Section: Introductionmentioning

confidence: 99%

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Natural history of cranial fibrous dysplasia revealed during long-term follow-up: Case report and literature review

Penn¹,

Rj²,

Glass³

et al. 2017

Surg Neurol Int

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Background:Fibrous dysplasia (FD) is a rare developmental disease characterized by the replacement of bone marrow with proliferating fibro-osseous tissue. There exist three forms of FD—monostotic, polyostotic, and that associated with McCune-Albright syndrome. The disease can present in different locations and with a variety of symptoms. One of the more common locations of FD occurrence is the craniofacial region. Treatment of asymptomatic FD often involves conservative management with serial imaging. Medical management with bisphosphonates is an option, though long-term efficacy data are lacking. Surgical resection is usually reserved for very large or symptomatic lesions.Case Description:We discuss the most unusual case of a 52-year-old male found to have a left pterional mass while being worked up for sinus headaches. The patient elected to follow this lesion conservatively, and imaging several years later showed obvious growth which accelerated in the last 4 years during an 18-year observational period. He ultimately underwent successful resection of an extradural and intradural FD.Conclusions:The significant growth potential of these lesions was revealed in this patient, in whom conservative management had been adopted. Despite optimal surgical resection and outcome in this case, the importance of surveillance imaging and perhaps earlier intervention cannot be underestimated when managing cranial FD.

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