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<p>Most cases of fibrous dysplasia (FD) of the temporal bone are asymptomatic and do not require intervention. However, concurrent FD with cholesteatoma, facial palsy, and abscess formation is exceptionally rare, with only sporadic cases reported. We report a specific clinical scenario where a patient with FD of the temporal bone developed the squamous variant of chronic otitis media. There was extensive involvement beyond the established boundaries of the temporal bone owing to bone expansion. He developed ipsilateral facial palsy, a mastoid abscess, huge right-sided temporal region swelling, and a neck abscess. We elaborate on the clinical course and management of such a case. A 30-year old male patient with a history of gradually increasing swelling in the right temporal region for the past 10&#xa0;years presented with sudden onset pain, ear discharge, and right-sided facial paralysis for 2&#xa0;weeks. Radiological investigations revealed bony expansion with a ground glass bony matrix involving the right temporo-parieto-occipital bone. The patient was managed surgically by a right-sided radical mastoidectomy and debridement of the bony mass. Intraoperatively, extensive cholesteatoma was noticed extending up to the petrous apex and mastoid tip. Dissection during mastoidectomy revealed a higher placed dural plate, a posteriorly placed sigmoid sinus, and the petrous apex was at a deeper plane. This discussion aims to explore the diagnosis and management strategies for a patient with extensive FD of the temporal bone involving the petrous apex and mastoid tip with a ballooned out mastoid air cell system and altered anatomical landmarks. The cholesteatoma had spread to all the corners of the mega mastoid air cell system. The widespread disease resulted in abscess formation extending into the parapharyngeal and parotid space. Our case is the first case report depicting extensive FD of the temporal bone with mastoiditis and cholesteatoma presenting with facial paralysis, petrous apex involvement, and mastoid tip abscess tracking into the parapharyngeal space. Abscess-induced increases in temporal region swelling remain unnoticed and neglected by patients with FD as they have chronic bone expansion. Hence, they present with widespread abscess and complications of squamous chronic otitis media. Surgical treatment is of paramount importance to ensure disease clearance, cholesteatoma removal, and correction of bony deformities.</p>

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Fibrous Dysplasia of Temporal Bone with Cholesteatoma: A Case Report

  • 期刊论文

  • 发表日期：

    2025年4月30日

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  • Sharmistha Chakravarty,
  • Himanshi Grover,
  • Nitin M. Nagarkar,
  • Anil K. Sharma

摘要

Most cases of fibrous dysplasia (FD) of the temporal bone are asymptomatic and do not require intervention. However, concurrent FD with cholesteatoma, facial palsy, and abscess formation is exceptionally rare, with only sporadic cases reported. We report a specific clinical scenario where a patient with FD of the temporal bone developed the squamous variant of chronic otitis media. There was extensive involvement beyond the established boundaries of the temporal bone owing to bone expansion. He developed ipsilateral facial palsy, a mastoid abscess, huge right-sided temporal region swelling, and a neck abscess. We elaborate on the clinical course and management of such a case. A 30-year old male patient with a history of gradually increasing swelling in the right temporal region for the past 10 years presented with sudden onset pain, ear discharge, and right-sided facial paralysis for 2 weeks. Radiological investigations revealed bony expansion with a ground glass bony matrix involving the right temporo-parieto-occipital bone. The patient was managed surgically by a right-sided radical mastoidectomy and debridement of the bony mass. Intraoperatively, extensive cholesteatoma was noticed extending up to the petrous apex and mastoid tip. Dissection during mastoidectomy revealed a higher placed dural plate, a posteriorly placed sigmoid sinus, and the petrous apex was at a deeper plane. This discussion aims to explore the diagnosis and management strategies for a patient with extensive FD of the temporal bone involving the petrous apex and mastoid tip with a ballooned out mastoid air cell system and altered anatomical landmarks. The cholesteatoma had spread to all the corners of the mega mastoid air cell system. The widespread disease resulted in abscess formation extending into the parapharyngeal and parotid space. Our case is the first case report depicting extensive FD of the temporal bone with mastoiditis and cholesteatoma presenting with facial paralysis, petrous apex involvement, and mastoid tip abscess tracking into the parapharyngeal space. Abscess-induced increases in temporal region swelling remain unnoticed and neglected by patients with FD as they have chronic bone expansion. Hence, they present with widespread abscess and complications of squamous chronic otitis media. Surgical treatment is of paramount importance to ensure disease clearance, cholesteatoma removal, and correction of bony deformities.