Granulomatous Inflammation of the Skull Base: A Rare Case of Mycobacterium tuberculosis in the United States.

The differential for an ill-defined, bone-destructive, granulomatous lesion of the skull base includes malignancy, as well as autoimmune and infectious processes. Suspicion for tuberculosis of the skull base in high-risk patients is particularly necessary given the difficulty to culture on standard cultures, need for specific and prolonged antibiotic therapy, and dire morbidity if not diagnosed and treated in a timely manner. Repeat biopsies and cultures were necessary to diagnose this case of Mycobacterium tuberculosis of the skull base after initial biopsy was non-diagnostic. Laryngoscope, 134:4023-4027, 2024.

A rare cause of malignant otitis externa and skull base osteomyelitis.

An elderly male with type 2 diabetes presented with a 2-month history of otalgia and severe headaches. He was diagnosed with malignant otitis externa (MOE) and was commenced on empirical treatment with oral ciprofloxacin. Pseudomonas is the most common cause of MOE. A baseline CT scan was undertaken that demonstrated skull base osteomyelitis (SBO) due to findings of bone erosion at the mastoid tip and an infiltrating soft tissue mass eroding the clivus. Eight weeks later, he returned with worsening and bilateral symptoms of otitis externa, hearing loss, temporomandibular pain and dysfunction. Worsening and now bilateral malignant otitis externa were confirmed with an MRI scan that also demonstrated a small fluid collection in his left temporal region. The collection was aspirated and grew scedosporium apiospermum. He was diagnosed with fungal SBO and was commenced on treatment with the antifungal voriconazole, with significant improvement in symptoms and radiological findings. Fungal osteomyelitis is more likely in immunosuppressed patients, particularly those with type 2 diabetes. Fungal aetiology should be suspected in patients with progressive symptoms, despite treatment. A microbiology diagnosis of fungal SBO or MOE can be challenging to obtain and can lead to diagnostic delay. A sampling of the external auditory canal can aid in diagnosing MOE; however, scedosporium may also be isolated as a commensal organism. Aspirations from accessible fluid collections, infratemporal fossa needle sample and bone biopsy can provide material for diagnosis. Scedosporium is a rare cause of disease in humans, however, fungal infections are increasing in humans, due to an increase in susceptible populations. Scedosporium apiospermum is a rare cause of SBO and should be considered in patients not responding to standard treatment.

Skull base osteomyelitis with secondary cavernous sinus thrombosis: a rare presentation of an animate foreign body in the ear.

A 42-year-old woman presented with fever, left ear pain, restricted mouth opening, difficulty in swallowing and inability to open her left eyelid for a period of 10 days. She was treated with antibiotics for the same at a local medical facility; however, a sudden decrease in her left eye vision prompted her to visit our tertiary centre. Her history was insignificant except for having multiple left ear syringing for an insect removal 10 days before onset of her current symptoms. On examination, she had ptosis of the left eye with chemosis, dilated pupil with only perception of light and restricted ocular mobility. Oral examination revealed trismus and bulge in the left peritonsillar region. Left ear examination revealed a large central perforation with mucopurulent discharge. CT of the neck with contrast demonstrated a collection in the left peritonsillar space with left internal carotid artery thrombosis. MRI of the brain with gadolinium revealed left cavernous sinus thrombosis with acute infarcts in the left frontal lobe. An emergency incision and drainage of the left peritonsillar abscess was performed. Culture grew broad aseptate fungal hyphae. Despite starting on antifungal therapy, she succumbed to her illness.

Polymicrobial Skull Base Osteomyelitis Related to Chronic Sphenoiditis and Endoscopic Sinus Surgery.

Skull base osteomyelitis (SBO) secondary to endoscopic surgery for chronic sinusitis and post-operative sphenopalatine artery electrocautery has not been previously reported. This case report details an instance of Central SBO with an unusual microbiology profile and highlights the need to readily consider SBO should patients present with persistent headache and cranial nerve palsies following sinus surgery. Laryngoscope, 131:E1086-E1087, 2021.

Skull Base Fungal Osteomyelitis: A Case Report and Review of the Literature.

Skull base osteomyelitis (SBO) is an invasive infection refractory to therapy, closely linked with malignant otitis externa (MOE). It is characterized by a mild clinical presentation that can delay cross-sectional imaging considered as the key to revealing it. Skull base osteomyelitis typically affects elderly diabetics and immunocompromised patients (>70 years). It most commonly has an otogenic origin due to an extension of MOE. The prognosis can be very poor without the administration of adequate and timely therapy at an early disease stage. Nowadays, Pseudomonas aeruginosa remains the most common pathogen associated with SBO. Fungi are a rare cause of MOE. This report documents a rare case of otogenic SBO caused by Candida parapsilosis in a diabetic patient, with persistent otologic symptoms as clinical onset and resistance to medical treatment. Fungal MOE has more subtle symptoms and is more aggressive than its bacterial counterpart. When MOE is resistant to antibacterial drugs, this should raise the suspicion of a fungal etiology of MOE. The current guidelines do not exhaustively describe the diagnosis, antifungal drugs of choice, and optimum duration of treatment. The description of these rare clinical cases should help with the multidisciplinary management of this disease in order to optimize the diagnosis and therapeutic protocol.

Tracking down the White Plague: The skeletal evidence of tuberculous meningitis in the Robert J. Terry Anatomical Skeletal Collection.

Paleopathological diagnosis of tuberculosis (TB) essentially relies on the identification of macroscopic lesions in the skeleton that can be related to different manifestations of TB. Among these alterations, granular impressions (GIs) on the inner skull surface have been considered as pathognomonic features of tuberculous meningitis (TBM). GIs may be established by pressure atrophy of the tubercles formed on the outermost meningeal layer during later stages of TBM. Although GIs were used as diagnostic criteria for TBM in the paleopathological practice since the late 20th century, their diagnostic value has been questioned. To contribute to strengthening the diagnostic value of GIs, a macroscopic investigation-focusing on the macromorphological characteristics and frequency of GIs-was performed on skeletons of known cause of death from the Terry Collection. The χ2 analysis of our data revealed that GIs were significantly more common in individuals who died of TB than in individuals who died of non-TB causes. Furthermore, GIs were localized on the inner surface of the skull base and of the lower lateral skull vault. The localization pattern and distribution of GIs on the endocranial surface resemble that of the tubercles observed in the affected meninges during the pathogenesis of TBM. Our results strengthen the tuberculous origin of GIs and imply that they can be considered as specific signs of TBM. Therefore, GIs can be used as diagnostic criteria for TBM in the paleopathological practice, and the diagnosis of TBM can be established with a high certainty when GIs are present in ancient human bone remains.

Insidious onset of headache, diplopia and Horner's syndrome: a rare case of petrous bone osteomyelitis.

We present an unusual case of skull base osteomyelitis in an 88-year-old woman. She presented with gradual onset unilateral headache and diplopia. On examination, there was evidence of a left-sided Horner's and ipsilateral sixth nerve palsy. In addition to persistent raised inflammatory markers, an MRI neck identified signal change in the petrous bone confirming a diagnosis of skull base osteomyelitis. Skull base osteomyelitis should be considered in presentations of subacute raised inflammatory markers in the context of ipsilateral cranial nerve signs.

The routes of infection spread in central skull-base osteomyelitis and the diagnostic role of CT and MRI scans.

BACKGROUND: Central skull-base osteomyelitis (CSBO) represents a life-threatening complication of external ear canal infection. Computed tomography (CT) and magnetic resonance imaging (MRI) play key roles in assessment of CSBO progression. METHODS: Twelve patients with CSBO were included in a retrospective clinical study. In total, 62 scans (30 CTs and 32 MRIs) were performed to evaluate the extent of inflammatory changes. The scans were read independently by two radiologists specialised in imaging of the head and neck. The regions under the skull base were specified using the online Anatomy Atlas of the skull base. To clarify the timeline, the time period was divided into four parts, and inflammatory changes in the skull-base regions were tracked. Data were statistically analysed. RESULTS: In early stages of the disease, CT scan detects inflammatory changes closely related to the stylomastoid foramen and medially to the posterior belly of the digastric muscle, changes which have been proved to be crucial for the diagnosis of CSBO. Later the infection spreads to the contralateral side causing demineralisation of the bones. CONCLUSION: Imaging methods play a crucial role not only in establishing the diagnosis, but also in anticipating the direction of infection spread underneath the skull base.

Non-invasive fungal sinusitis resulting in multiple cranial nerve neuropathies.

A 33-year-old man presented to the emergency department with a right-sided facial paralysis and maxillary division (V2, trigeminal nerve) paraesthesia. He had been suffering with upper respiratory tract symptoms in the preceding 2 months, including rhinorrhoea, fever and headache. The patient was otherwise fit and immunocompetent. Urgent radiological investigation revealed extensive fungal sinusitis with sphenoid sinus dehiscence and skull base osteitis. The patient underwent emergency endoscopic sinus surgery revealing concretions and debris in the ethmoid and sphenoid sinuses. He was commenced on systemic antifungal therapy and made a full recovery with resolution of his cranial neuropathies. The fungus Schizophyllum commune was isolated and is a rare cause of fungal sinusitis, but with the potential for invasive disease in immunosuppressed individuals.

Extensive Skull Base Osteomyelitis Secondary to Malignant Otitis Externa.

Skull base osteomyelitis is a severe complication of malignant otitis externa that affects the marrow of the temporal, sphenoid, and occipital bones. Skull base osteomyelitis is usually diagnosed based on clinical, microbiological, and radiological findings. Here, we present the imaging findings of a 76-year-old man who initially presented with right-sided malignant otitis externa, with the involvement of the otomastoid structures and ipsilateral temporal bone. Over the following 3 years, despite specific extended antibiotic therapy, the skull base osteomyelitis entirely involved the skull base, up to the contralateral petrous portion of the temporal bone, and it affected the cervical vertebral processes. This report describes an exceptional extent of unilateral malignant otitis externa with a severe involvement of the skull base on the contralateral side and the cervical spine.

Atypical Form of Cervicofacial Actinomycosis Involving the Skull Base and Temporal Bone.

BACKGROUND:: Cervicofacial actinomycosis is an uncommon indolent infection caused by Actinomyces spp that typically affects individuals with innate or adaptive immunodeficiencies. Soft tissues of the face and neck are most commonly involved. Actinomyces osteomyelitis is uncommon; involvement of the skull base and temporal bone is exceedingly rare. The authors present a unique case of refractory cervicofacial actinomycosis with development of skull base and temporal bone osteomyelitis in an otherwise healthy individual. METHODS:: Case report with literature review. RESULTS:: A 69-year-old man presented with a soft tissue infection, culture positive for Actinomyces, over the right maxilla. Previous unsuccessful treatment included local debridement and 6 weeks of intravenous ceftriaxone. He was subsequently treated with conservative debridement and a prolonged course of intravenous followed by oral antibiotic. However, he eventually required multiple procedures, including maxillectomy, pterygopalatine fossa debridement, and a radical mastoidectomy to clear his disease. Postoperatively he was gradually transitioned off intravenous antibiotics. CONCLUSIONS:: Cervicofacial actinomycosis involves soft tissue surrounding the facial skeleton and oral cavity and is typically associated with a history of mucosal trauma, surgery, or immunodeficiency. The patient was appropriately treated but experienced disease progression and escalation of therapy. Although actinomycosis is typically not an aggressive bacterial infection, this case illustrates the need for prompt recognition of persistent disease and earlier surgical intervention in cases of recalcitrant cervicofacial actinomycosis. Chronic actinomycosis has the potential for significant morbidity.

Skull base aspergillosis in an immunocompetent elderly man with early response to steroid.

We report the case of a previously well 80-year-old man who presented with subacute bilateral painful optic neuropathy with initial response to corticosteroids but ultimately progressed to a fatal skull base syndrome. Initial presentation of steroid-responsive painful bilateral posterior optic neuropathy, preliminary normal enhanced MRI, normal cerebrospinal fluid and inflammatory markers indicated atypical optic neuritis. However, this progressed to a bilateral orbital apex syndrome with ophthalmoplegia and evidence of abnormal skull base enhancement on subsequent MRI. Biopsy of radiologically abnormal dura was non-diagnostic and negative for fungal stains. He deteriorated and died 8 months after initial presentation. At postmortem, fungal skull base infection was diagnosed. This case demonstrates that chronic skull base fungal infection can: (1) present in elderly immunocompetent patients, (2) show initial improvement with corticosteroids and (3) evade diagnosis on biopsy. We encourage a high index of suspicion for fungal skull base infection in similar cases.

Propionibacterium acnes as an emerging pathogen in skull base osteomyelitis: A case series.

Skull base osteomyelitis (SBO) is recognized as a potentially life-threatening infection that has been classically attributed to Pseudomonas aeruginosa. However, growing data shows that the bacteriology of SBO is changing. Traditionally thought of as commensal bacterium, P. acnes has been implicated as a serious pathogen in other specialties. Building on a previous case report from our institution, we have identified three patients with SBO who are culture-positive for Propionibacterium acnes as a single isolate. Herein, we describe the clinical history of the three patients, along with treatment, present outcome, and review of the literature. Laryngoscope, 128:332-335, 2018.

Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base Pseudomonas aeruginosa osteomyelitis.

Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel. Biopsy of the skull base/clivus revealed necrotising granulomatous inflammation with focal vasculitis consistent with GPA, and multiple bone cultures were positive for Pseudomonas aeruginosa This patient was diagnosed concurrently with GPA and P. aeruginosa skull base osteomyelitis. He was started on a 6-week course of cefepime intravenously and oral prednisone, with the plan to initiate rituximab infusion 2 weeks after initiation of antibiotic therapy.

A Treatment Protocol for Management of Bacterial and Fungal Malignant External Otitis: A Large Cohort in Tehran, Iran.

AIMS: High rates of negative microbiologic test results highlight the potential role of empiric antimicrobial agents in management of malignant otitis externa (MOE). This study investigates the clinical presentation, laboratory findings, and response to empiric treatment in a large group of patients admitted to a tertiary academic hospital in Tehran, Iran. METHODS AND MATERIALS: We recruited 224 patients diagnosed with MOE in a prospective observation from 2009 through 2015. All patients received a 2-agent antibacterial regimen at baseline (phase I). Patients with no improvement within 10 days and/or nonresponders to a second course of antibacterials were switched to antifungals (phase II). Response to treatment was observed and documented in both groups. RESULTS: All patients had physical symptoms for more than 12 weeks before admission. In total, 127 patients responded well to antibacterials. Eighty-seven out of 97 patients who were switched to antifungals had complete response to treatment; patients in the latter group had significantly higher A1C levels at baseline. CONCLUSION: Our findings provide evidence to develop clinical guidelines that accelerate diagnosis and treatment of MOE to improve patient outcomes.

Fungal skull base osteomyelitis: Emerging microbial identification techniques.

Culture-based pathogen identification in skull base osteomyelitis, particularly for fungi, is often inaccurate. We report the case of patient with fungal skull base osteomyelitis cured by sustained antifungal therapy after 16 months of debilitating illness. Due to medical complications, a strong clinical rationale was needed to justify long-term antifungal therapy. The offending fungus was identified by experimental molecular technology (Ibis T5000 universal biosensor); invasive fungal disease was corroborated by biochemical assays. Our discussion will help familiarize the otolaryngologist with existing biochemical and molecular diagnostics for invasive fungal disease. We encourage future investigators to study their application in cases of skull base osteomyelitis. Laryngoscope, 127:E5-E7, 2017.

Uncontrolled spread following radiosurgery for a skull base aspergilloma misdiagnosed as schwannoma: is radiosurgery responsible?

BACKGROUND: Little is known about the natural history of skull base fungal lesions in immunocompetent individuals and the effect of high-dose radiation on fungal lesions. METHOD AND RESULTS: We report a case where radiosurgery was given to a skull base aspergilloma, mistaking it to be a trigeminal schwannoma. There was dramatic spread of the lesion to periventricular region with significant increase in the skull base lesion. The patient never received steroids. A stereotactic biopsy established the diagnosis, and treatment with voriconazole helped. CONCLUSIONS: It is possible that radiosurgery decreases local immunity due to vascular sclerosis and aids in spread of the fungal lesion, though it is only speculative. This again highlights the importance of establishing the diagnosis prior to radiosurgery.

[CHRONIC OSTHEOMYELITIS OF THE FACIAL MIDDLE ZONE WITH SPREADING ON THE BONES OF CRANIAL BASE ON BACKGROUND OF NARCOTIC DEPENDENCE].

Own experience of treatment of a narcotic dependence patients, suffering osteomyelitis of jaws, was analyzed. In those patients, who have had exposed themselves towards toxic impact of narcotic substances (even after arrest of the consumption) for a long period of time, the inflammation spreading on the bones of facial middle zone and the skull base with occurrence of a life3threatening purulent3septic, including intracerebral, complications (cerebral аbscess, meningitis, sepsis) were observed. Additional division of cerebral abscess on open and closed forms for optimization of diagnosis of іntracranial complications was proposed.