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