Lumbar Sympathectomy as a Surgical Approach in Hereditary Sensory Neuropathy (Thevenard Syndrome)

Abstract

Hereditary sensory neuropathy (HSN) as described by Thevenard is characterized by the appearance of a painless corn on the great toe or sole which develops into an ulcer with purulent discharge. This is accompanied by the occurrence of pathologic fractures, and amputation is frequently necessary. Chronic neuritis of the nerve roots and the loss of sensation have been thought to be responsible. Recently, capillary arteriovenous (A-V) anastomosis and a poor capillary network were demonstrated to have caused poor circulation in the skin of the affected feet. Three patients, 1 female and male and female siblings (from 2 unrelated families) whose age ranged from 15-20 years and who suffered from ulcers in the soles and big toe, with purulent discharge and loss of sensation were studied by femoral angiogra phy. Poor vascularization was found in the feet of both females as well as in the male, who had an amputated toe and earlier had undergone surgery because of an A-V fistula in the foot. Macroaggregates (Technecium 99) were injected during angiography and traced with a gamma camera. In the extremities of all 3 patients, the demonstrable material was markedly dimin ished when compared with that seen in the controls. A lumbar sympathec tomy was performed in the 3 patients in order to improve blood flow in the skin and prevent further deterioration. Ten days after surgery the purulent discharge had disappeared and within 5 months the ulcers were healed. After 3 years of follow-up these patients lead normal lives and have no complaints except for the remaining sensory loss. Lumbar sympathectomy is therefore suggested as a new method of treatment for this disabling disease.