Ahmed A, Shah K A.
Peri-operative management of a patient with uncontrolled Polycythemia Vera for above Knee Amputation.
J Pak Med Assoc Jan ;54(1):34-5.

A 34 years old man with a history of Polycythemia Vera (PV) diagnosed in 1991 was admitted with severe pain, swelling and discolouration of right lower leg. His history revealed that his usual hemoglobin (Hb) used to remain above 18g/dl. He used to get a phlebotomy done at irregular intervals. This resulted in his having this thromboembolic event leading to an ischemic lower limb. His co-morbids included hypertension for 8 years and diabetes mellitus type 1 for 3 years both of which were controlled. His current problem started 15 days back when he developed complaints of swelling of his legs. A renal biopsy was done. Then he developed increasing swelling and pain of the right leg which became pulseless. For this a right femoral arterial embolectomy was performed. By the next day his leg developed a compartment syndrome and a fasciotomy was done. After two days he noticed discoloration of the toes of his right foot with increasing pain. On admission he had a temperature of 39°C and was tachypnoeic. He showed signs of congestive cardiac failure (CCF). His right lower limb showed necrotic tissue in the fasciotomy wounds, discolored toes and loss of sensation upto the knee. He had a Hb of 15.9, a hematocrit (Hct) of 50.2% and a markedly deranged coagulation. Atrial fibrillation was detected on the electrocardiogram and the chest radiograph showed cardiomegaly. An echocardiogram showed dilated cardiac chambers with the left ventricular ejection fraction of less than 25%. The rest of the investigations were within normal limits. Intravenous antibiotics were started and hematology and cardiology consults were sought. The patient was started on diuretics, Angiotensin Converting Enzyme Inhibitors, Digoxin, Broad Spectrum Antibiotics, Ranitidine, Insulin (sliding scale), Allopurinol, Isosorbide Mononitrates and Pethidine. The Hematologist advised a phlebotomy to decrease the Hct and a peri-operative transfusion of fresh frozen plasma to correct the coagulopathy. His right leg showed rapidly growing gangrene which needed urgent surgery with as much optimization as possible. Preoperatively he showed no signs of CCF clinically. An arterial cannula and two large bore venous cannulae were passed. Anaesthesia was induced with injection Fentanyl, Midazolam and Propofol and a size 4 laryngeal mask airway was inserted.

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