A 73 y.o. male presents with several weeks of diplopia and L eye ptosis.

He had an MRI at an outside hospital which was normal.

he is unable to look down and in with the L eye

he is unable to look to the R with the L eye

he is unable to look to the L with the L eye

Where is the lesion?

Our patient had palsies of cranial nerves III, IV, and VI.  This localizes the lesion to the cavernous sinus. Since the MRI did not show anything a CTA venogram of the head was done looking for a cavernous sinus thrombosis.  If showed a soft tissue mass in the the L cavernous sinus without thrombosis. At this point since it abutted the pituitary the differential was pituitary adenoma, IgG 4 disease, and lymphoma.   A whole body PET/CT showed intense uptake in R iliac lymph nodes, uptake in the sacrum and also in the L cavernous sinus. 

Cavernous Sinus Anatomy. Cavernous Sinus Anatomy is designed by Dr. Kuybu and Diana for Stat Pearls on 8/2/2018

Until the mass was discovered, our patient had Cavernous Sinus Syndrome (CSS) which can present as unilateral  ophthalmoplegia of cranial nerve III, IV, or VI; Horner’s syndrome  or sensory loss in CN V (1 or 2 distribution). If pressure is high within the cavernous sinus as in cases of carotid cavernous fistula or cavernous sinus thrombosis the eye will become proptotic.

Causes of carotid cavernous syndrome

A Carotid cavernous fistula is an abnormal shunt from the carotid artery to the cavernous sinus. They are divided into low flow and high flow fistulas.   They can be post traumatic (high flow)  or  occur when congenital arteriovenous connections open spontaneously (low flow) in the setting of HT, childbirth, atherosclerosis or collagen vascular disease. Often in traumatic cases, the fistula follows a basilar skull fracture.

A carotid cavernous fistula with the bottom image showing an enlarged draining ophthalmic vein.

CLINICAL PEARL

If a patient complains of diplopia after a blow to the head and has an isolated 4th nerve palsy another diagnosis should be considered. Diplopia  may be the result of direct trauma to the 4th cranial nerve.  It  only cranial nerve to exit the dorsal aspect of the spinal cord and has a long course where it is susceptible to trauma.  The patient may complain of difficulty going down stairs since the 4th cranial nerve allows the eye to look down and in.

Our patient underwent a biopsy of the inguinal lymphadenopathy which showed lymphoma. He is currently undergoing treatment.

 

Ipsalali H, Ciftci A, Kilic D, et al. Variations of the 6th cranial nerve (nervus abducens) in the petroclival region. A microsurgical study. Morphologie. 2019 Jun;103(341Pt 2):103-109.

Fernandez S, et al. Caverous sinus tsyndrome: a series of 126 patients. Medicine. 2007 86(5) :278-81.

Lubomski M et al. Actinomyces cavernous sinus infection: a cans and systematic literature review. Practical neurology  2018 18(5):373-377.  

Dhaliwal A, West A, Trobe J, et al. third, fourth and sixth cranial nerve palsies following closed head injury. J Neuroophalmol. 2006 Mar;26(1):4-10.