Skull base syndromes

Skull base syndromes are caused by malignancies or inflammatory conditions that affect the base of the skull and the cranial nerves exiting the skull. The location of the pathology can often be determined by characteristic features produced by nerve damage and other localizing space-occupying effects (e.g., pain, proptosis). Syndromes primarily involving the cranial nerves are discussed in a separate article (see cranial nerve palsies).

• Etiology: sphenoid wing meningioma; and rarely, trauma
• Clinical features
  • Upper eyelid ptosis
  • Proptosis of the eye
  • Ophthalmoplegia
  • Dilated and fixed pupil
  • Loss of sensation of the upper eyelid and forehead due to damage to the trigeminal nerve

References:^[1][2]

• Etiology
  • Frontal lobe tumors, typically parasellar or subfrontal meningiomas
  • Olfactory groove meningioma; : meningiomas arising from the cribriform plate and frontosphenoidal suture (see meningiomas)
• Clinical features
  • Ipsilateral atrophy of the optic nerve
  • Contralateral papilledema
  • Anosmia

References:^[3][4]

• Etiology: damage to the orbital fissure or the optic canal resulting from an inflammatory condition or space-occupying lesion
• Clinical features
  • Ophthalmoplegia
    • Due to damage to the cranial nerves passing through the orbital fissure
    • Partial/complete paresis of oculomotor nerve (III^rdcranial nerve), trochlear nerve (IV^thcranial nerve), and abducens nerve (VI^thcranial nerve)
  • Blindness
  • Loss of sensation to the upper face may occur (see cranial nerve palsies).

References:^[5][6][7]

• Etiology
  • Cavernous sinus thrombosis (e.g., due to sinusitis and contiguous spread of infection)
  • Carotid-cavernous fistula
  • Cavernous sinus tumors
  • Pituitary tumors producing mass effect
  • Carotid artery fistula or aneurysms
  • Tolosa-Hunt syndrome (idiopathic granulomatous inflammation of the cavernous sinus)
• Clinical features
  • Swelling of the conjunctiva
  • Proptosis
  • Signs of CN palsy due to compression (CN III, IV, V-1, V-2, and VI pass through the cavernous sinus)
    • Painful ophthalmoplegia: partial/complete paresis of oculomotor nerve (third cranial nerve), trochlear nerve (fourth cranial nerve), and abducens nerve (sixth cranial nerve)
    • Absent corneal reflex: paresis of the ophthalmic branch of the trigeminal nerve (V1)
    • Loss of upper facial and corneal sensation may occur due to damage to the trigeminal branches of V1 and V2 (see cranial nerve disorders).
  • Horner syndrome

References:^[8]

• Etiology: : most commonly vestibular schwannomas (acoustic neuroma), although other cerebellopontine angle tumors may result in the same presentation
• Clinical features
  • Unilateral hearing loss
  • Tinnitus
  • Vertigo
  • Headache
  • Loss of facial sensation

References:^[9]

• Etiology: complication of otitis media and mastoiditis that spreads to the petrous apex of the temporal bone, can also be tumor growth in the same area
• Clinical features: typical triad
  • Lateral rectus palsy
  • Retro-orbital pain
  • Otorrhea (ear discharge)

References:^[10]

1. Bienfang DC. Overview and differential diagnosis of papilledema. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/overview-and-differential-diagnosis-of-papilledema.Last updated: June 26, 2016. Accessed: February 19, 2017.
2. Foster Kennedy Syndrome. https://radiopaedia.org/articles/foster-kennedy-syndrome. . Accessed: April 12, 2018.
3. Gappy C, Archer SM, Barza M. Orbital cellulitis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/orbital-cellulitis.Last updated: January 4, 2016. Accessed: February 18, 2017.
4. Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol. 2004; 15 (6): p.490-498.
5. Kauh CY, Nelson CC. Diagnosis and Management of Orbital Mucormycosis. EyeNet Magazine. 2014 .
6. Kattah JC. Cavernous Sinus Syndromes. Cavernous Sinus Syndromes. New York, NY: WebMD. http://emedicine.medscape.com/article/1161710-overview. Updated: February 14, 2014. Accessed: February 19, 2017.
7. Craniomaxillofac Trauma Reconstr. Superior orbital fissure syndrome: a case report. Craniomaxillofac Trauma Reconstr. 2012; 5 (2): p.115-120. doi: 10.1055/s-0032-1313363 . | Open in Read by QxMD
8. Mcavoy CE, Lacey B, Page AB. Traumatic superior orbital fissure syndrome. Eye . 2004; 18 (8): p.844-845. doi: 10.1038/sj.eye.6701320 . | Open in Read by QxMD
9. Maurer J, Ecke U, Schmidt CL, Stoeter P, Mann W. [Vascular origin of cerebellopontine angle syndrome].. HNO. 2000; 48 (2): p.142-6.
10. Limb CJ, Lustig LR, Klein JO. Acute otitis media in adults (suppurative and serous). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/acute-otitis-media-in-adults-suppurative-and-serous.Last updated: November 16, 2016. Accessed: February 19, 2017.