1. Bakhshaee M, MD Rhinologist Azar 1388
Patient of The Month
Bakhshaee M, MD
Rhinologist
Azar 1388

2. Presentation
45 man complain from diplopia and headache

3. History Smoking: Pos Job: Driver Smell: Ok
Headache : Six months ago
Diplopia: One month ago
Trauma: Three years ago from car accident.
Background Diseases: Neg
Recurrent URTI: Neg
Epistaxis: Neg
Rhinorrhea: Neg
Nasal Obstruction: Neg
Visual Disturbance: Neg
Smoking: Pos
Job: Driver
Smell: Ok
Facial pain: three months ago
Dizziness: Pos
Hoarseness: Neg
Other cranial nerve: Neg
Long tract sign

4. Examination Craniofacial deformity: Neg
Nasal Endoscopy: Mild SD
Visual acuity: 9/10
Eye movement: Right eye limitation to most lateral gaze
Ptosis & Proptosis: Neg
Neck Mass: Neg
Sinus palpation: No tenderness
Blood Pressure: 13/9
Craniofacial deformity: Neg
Ear, Throat and oral cavity: No noticible point
Retinal Examination: Ok

5. Paraclinic Lab Imaging CBC: Ok FBS: Ok Biochemistry : Ok Conventional
CT Scan
MRI

6. CT Scan

9. MRI

10. Next Step?

11. What is the Best? Diagnostic Endoscopy Angiograghy
Theraputic Endoscopy

12. Diagnostic Endoscopy

13. Diagnosis
Chordoma

14. Management
Surgery
Radiotherapy
Chemotherapy

15. Isolated Sphenoid Sinus Disease

16. 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.

17. Classification Isolated sphenoid sinus disease can be broadly divided
Inflammatory
Neoplastic
Miscellaneous categories

18. Inflammatory lesions Sinusitis Acute Chronic Fungal infections
Invasive: Mucormycoses, disseminated Aspergillosis
Noninvasive: Mycetoma, Aspergilloma
Mucoceles
Pyoceles

19. 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

20. Miscellaneous
Cerebrospinal leaks
Traumatic
Spontaneous
Encephaloceles

21. Clinical signs and symptoms
The most common symptom of sphenoid sinus disease is headache
Visual disturbance is the second most frequently reported symptom
Nasal obstruction
Smell and taste
Cranial nerve palsies
Dizziness

22. Evaluation CT Scan: Air-fluid level Acute & Chronic sinusitis Polyp
Aneurism
Thining & Expansion
Mucocel
tumor
Sclerosis
Fungal
Fibroossous
Bone erosion
Malignant Tumor

23. 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

24. Symptoms
Slow-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,
Hoarseness
CN XII paresis

25. Evaluation CT Scan MRI Reveal destruction of bone in a lytic pattern
T1: Isointense or hypointense lesion
T2: bright signal that may appear heterogeneous.
Gd: Enhancement is typically moderate to high

26. Management
Current and historical management of skull base chordomas has involved :
Surgical excision
Radiation therapy
Both