Before Old Age-A Rare Case of Werner Syndrome

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Werner syndrome or adult progeria is the most common of the premature ageing disorders. Patients usually present with all the symptoms and signs of old age very early in life, the most common being cataract, development of type II diabetes mellitus, osteoporosis, atherosclerotic changes in the blood vessels, non healing ulcers etc. Secondary complications may necessitate various surgical procedures in these patients subjecting the patient to high risks under anesthesia.

The anesthetic implications in these patients are very challenging as the airway is more often difficult owing to the morphological changes of the oral anatomy. Coexisting diseases like hypertension and ischaemic heart disease may also pose a greater threat to anaesthetize these patients.

A complete knowledge of the anesthetic challenges and preparedness for the management of complications is essential in patients with Werner syndrome coming for elective or emergency surgeries.

A 32 yr old male patient with a diagnosis of Werner syndrome is posted for debridement of non healing ulcer in the left lower limb. He was diagnosed with Werner syndrome 10 yrs back when he had developed cataract bilaterally and surgery under local anaesthesia was done for the same and vision was restored. Six years back he was diagnosed with type II diabetes mellitus and he has been on oral hypoglycemic agents since then. No history of chest pain or breathlessness. The patient has been having multiple ulcers on the lower limbs and frequently visits the dermatology clinic for regular dressings. These ulcers have been painful and so the patient was confined to the bed. Patient gives history of loss of molar teeth on either side. History obtained from the parents revealed that the patient’s sister had the same disease and died of ischaemic heart disease at the age of 25 yrs.

On examination, patient appeared to be 60 yrs of age, cooperative but unable to articulate his speech correctly. The head was large, there was loss of hair extensively over the scalp with premature greying, absent outer eyebrows and pale conjunctiva. The skin appeared to be non-elastic with decreased fat in the subcutaneous tissue and thin extremities. The chest was deformed with prominent lower ribs. Chest was clear on auscultation and cardiac sounds were well heard. Multiple small ulcers were seen on the upper and lower extremities. A large ulcer measuring 7x8 cms was seen in the left ankle which needed debridement under anaesthesia.