Werner's syndrome: a quite rare disease for differential diagnosis of scleroderma.

Werner's syndrome (WS) is an autosomal recessive disorder characterized by premature aging. The main features of the disease are scleroderma-like skin appearance, premature atherosclerosis, short stature, diabetes mellitus, early osteoporosis and early aging. Herein, we describe a patient with WS, who has scleroderma-like skin changes and discuss the literature about WS as a disease in the differential diagnosis of systemic sclerosis.

Ophthalmological aspects in patients with Werner's syndrome.

In ten of 18 eyes from nine patients with Werner's syndrome, cataract surgery was complicated by wound dehiscence and its consequences: peripheral anterior synechiae (4), secondary epiretinal gliosis (4), cystoid macular edema (3) in the framework of Hruby-Irvine-Gass syndrome, unplanned filtering bleb (2), and post-operative anterior ischemic optic neuropathy (1). Additionally, corneal endothelial decompensation occurred in eight eyes. In view of the fibroblasts' reduced growth potential, we suggest small surgical incisions, extracapsular cataract surgery using phacoemulsification, intraocular irrigation solutions protecting corneal endothelium, nonabsorbable single knot sutures not removed before 1 year after surgery, and no local or systemic use of cortisone.

[Severe sick sinus syndrome associated with a Werner's syndrome]. / Dysfonction sinusale sévère chez une patiente présentant un syndrome de vieillissement accéléré de Werner.

A 39-year-old woman was explored for recurrent syncopes. She was already known for a Werner's syndrome with symptoms of aging disorders. The explorations revealed an episode of sinusal arrest. A pacemaker was implanted. This case emphasises the relation between Werner syndrome and the development of cardiovascular abnormalities. This pathology should be recalled in young patients with symptoms of aging disorders and cardiovascular abnormalities. Valvular abnormalities, myocardial infarction and stroke are the major complications of Werner's syndrome. This case indicates that a sick sinus syndrome is a rare but possible complication of Werner's syndrome.

Off-pump coronary artery bypass grafting in a patient with Werner's syndrome.

Werner's syndrome is a rare hereditary disorder that is characterized by premature aging. We report a case of off-pump coronary artery bypass grafting (OPCAB) in a 56-year-old man with Werner's syndrome. We used an endoscopic vessel-harvesting system to harvest great saphenous vein grafts (SVGs) because this system helps minimize surgical wounds. This is important because poor wound healing is a prominent feature of Werner's syndrome. Revascularization of the coronary arteries in this case was thought to improve his prognosis, although he had already outlived the average life-span of Werner's syndrome. A detailed examination of the cardiovascular system should be performed in patients with this disorder.

Coverage of elbow ulcers in a patient with Werner's syndrome.

We present the repair of trophic ulcers on both elbows in a patient with Werner's syndrome. There is little information in the surgical literature about the repair of ulcers in patients with this rare entity. The technique used for coverage of the left elbow was an adipofascial turnover flap and on the right elbow an "extended" lateral arm island flap. We analyze the superior result of the latter technique and its advantages over other alternatives. It is a one-stage procedure that is reasonably easy and safe; it avoids elbow and shoulder stiffness; it avoids sacrifice of a principal artery or muscle; the donor site can be closed directly; and the padding obtained is optimum. With the "extended" dissection, we can obtain an additional advance to cover the elbow defect.