Bakhshaee M, MD
Rhinologist
Azar 1388
Presentation
45 man complain from diplopia and headache
HistoryHeadache : Six months
agoDiplopia: One month agoTrauma: Three years
ago from car accident.Background Diseases:
NegRecurrent URTI: NegEpistaxis: NegRhinorrhea: NegNasal Obstruction: NegVisual Disturbance: Neg
Smoking: PosJob: DriverSmell: OkFacial pain: three
months agoDizziness: PosHoarseness: NegOther cranial nerve:
NegLong tract sign
ExaminationNasal Endoscopy: Mild
SDVisual acuity: 9/10Eye movement: Right
eye limitation to most lateral gaze
Ptosis & Proptosis: NegNeck Mass: NegSinus palpation: No
tendernessBlood Pressure: 13/9
Craniofacial deformity: Neg
Ear, Throat and oral cavity: No noticible point
Retinal Examination: Ok
ParaclinicLab ImagingCBC: OkFBS: OkBiochemistry : Ok
ConventionalCT ScanMRI
CT Scan
MRI
What is the Best?Diagnostic EndoscopyAngiograghyTheraputic Endoscopy
Diagnostic Endoscopy
DiagnosisChordoma
ManagementSurgeryRadiotherapyChemotherapy
The sphenoid sinus has often been referred to as the neglected sinus because of its isolated position and difficult accessibility.
Disease restricted to the sphenoid sinus is rare and
often manifests with nonspecific or subtle signs and symptoms.
Typically, patients are referred to the otolaryngologist because of a finding of isolated sinus opacification on CT scans ordered by the patient’s primary care physician or neurologist to evaluate vague symptoms such as headache.
ClassificationIsolated sphenoid sinus disease can be
broadly divided
1.Inflammatory2.Neoplastic3.Miscellaneous categories
Inflammatory lesionsSinusitisAcuteChronicFungal infectionsInvasive: Mucormycoses, disseminated
AspergillosisNoninvasive: Mycetoma, AspergillomaMucocelesPyoceles
Neoplastic lesions
Benign Malignant
Intrinsic: 1. Inverting papillomas 2. Myofibroma 3. Schwannoma 4. Osteochondroma 5. Fibro-osseous disorders
(fibrous dysplasia, ossifying fibroma) Extrinsic: 1. Meningioma 2. Paraganglioma 3. Pituitary macroadenomas
Primary: 1. Squamous cell carcinoma 2. Adenocarcinoma 3. Adenoid cystic carcinoma 4. Mucoepidermoid carcinoma 5. Undifferentiated carcinoma 6. Transitional cell carcinoma Metastatic: 1. Renal cell carcinoma 2. Thyroid carcinoma 3. Prostate adenocarcinoma 4. Breast carcinoma 5. Lung carcinoma 6. Melanoma 7. Multiple myeloma and lymphoma
MiscellaneousCerebrospinal leaks1. Traumatic2. SpontaneousEncephaloceles
Clinical signs and symptomsThe most common symptom of sphenoid
sinus disease is headacheVisual disturbance is the second most
frequently reported symptomNasal obstructionSmell and tasteCranial nerve palsiesDizziness
EvaluationCT Scan:Air-fluid level1.Acute & Chronic
sinusitis2.Polyp3.Aneurism• Thining & Expansion1.Mucocel2.tumor
Sclerosis1.Fungal2.FibroossousBone erosion1.Malignant Tumor
Chordomas
Relatively rare tumors deriving from rests of embryonal notochord tissue located in the skull base, spine, and sacrococcygeal regions.
Skull base chordomas are typically located in the midline, in the clival and basisphenoid regions
SymptomsSlow-growing tumors; therefore, most patients will
relate a fairly long history of symptoms on initial presentation.
Symptoms in a given patient will depend on the exact location of the tumor.
Most commonly, the patient will report headaches and visual changes
Diplopia secondary to cranial nerve (CN)VI paresis, Facial numbness, facial droop, Dysphagia,HoarsenessCN XII paresis
EvaluationCT Scan• Reveal destruction of bone in
a lytic pattern
MRI• T1: Isointense or hypointense
lesion • T2: bright signal that may
appear heterogeneous. • Gd: Enhancement is typically
moderate to high
Management
Current and historical management of skull base chordomas has involved :
1.Surgical excision2. Radiation therapy3. Both