Neuro-ophthalmology: Case nine

Figure 1

Figure 2

This 75 year-old woman had had this facial appearance following treatment for Raynaud's disease 15 years ago.

a. What do the pictures show and what is the diagnosis?

Figure 1 shows bilateral ptosis and figure 2 shows bilateral sympathetomy scars on the upper chest wall.
The diagnosis is bilateral Horner's syndrome secondary to bilateral sympathetomy for Raynaud's disease.

Raynaud's disease is Raynaud's phenomenon in the absence of underlying disease. It consists of spasm of the arteries precipitated by cold and relieved by heat. There is an initial skin pallor resulting from vasoconstriction which is followed by cyanosis caused by slow blood flow. Redness eventually occurs owing to hyperaemia. The symptoms consist of pain and numbness especially during the re-warming period.

b. How would you manage the ocular problems?
The best corrective procedure for mild ptosis as in Horner's syndrome would be Fasanella Servat procedure in which some of the upper tarsal plate is excised.

c. What treatment is now used for Raynaud's disease?

Sympathetomy is rarely used nowadays for Raynaud's disease. The patient is usually advised to wear gloves during the cold weather. If this were interfering with the patient's work then peripheral calcium channel blockers such as nifidepine or angiotensin converting enzyme inhibitor such as captopril may be useful.
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