Anesthetic management of laparoscopic splenectomy in a case of Evans Syndrome with systemic lupus erythematosis.

Madam,

Evans Syndrome is a rare autoimmune disorder and by definition is the combination of both concurrent and sequential development of idiopathic thrombocytopenic purpura (ITP) and Coombs positive autoimmune hemolytic anemia (AIHA) in the absence of underlying disorder.[1] Although the true incidence is not known, it is estimated to effect 3.7-5% of patients with ITP or AIAH at the onset.[2] The dysregulation of the immune system produces several autoantibodies against red blood cells and platelets leading to early destruction by complement and reticuloendothelial systems.[3] The spleen has been proposed as either as a site of destruction or as a source of autoantibodies production.[4] The presence of associated systemic lupus erythematosis (SLE), warrants a more aggressive line of management than either condition presenting alone with poorer prognosis. We present case of Evans syndrome with ongoing bleeding diathesis, anticipated anesthetic risks and challenges scheduled for elective laparoscopic splenectomy in the presence of depleted platelet count.

A 26-year-old female patient presented with bleeding gums, menorrhagia and petechiae for the last 1-month, and 3 years back she had similar complaints along with jaundice. She was diagnosed to have “steroid-resistant Evans syndrome” associated with SLE. She had remission with two doses of rituximab. The present medication consisted of oral prednisolone 30 mg, hydroxychloroquine 200 mg, injection insulin and calcium supplements. General examination revealed petechiae over trunk and limbs. Her investigations are shown in Table 1. Electrocardiogram, two-dimensional echocardiogram and chest X-ray was normal. Ultrasonography of the abdomen revealed mild splenomegaly and distended gallbladder. She has a short neck with Mallampati Grade II. Vital parameters were normal except mild tachycardia. She was vaccinated to for pneumococcal, meningococcal and haemophilus infections. Premedication consisted of oral pantoprazole; alprazolam and rest of the medications were continued except insulin.

Table 1

Laboratory investigations

On the day of surgery baseline, blood glucose was 65 mg/dL and corrected to 156 mg/dL with 25% dextrose. A large bore 16G intravenous (IV) cannula was secured; 1 unit single donor platelets (SDP) and hydrocortisone 25 mg IV was given prior to the surgery. Anesthesia was induced, and oral endotracheal tube was placed with minimal manipulation. Crepe bandages were applied to the lower limbs. Intraoperative analgesia was with IV fentanyl 80 μg bolus followed by 20 μg slow IV as required. Another unit of SDP and 3 units of platelet rich plasma and 2 units of packed red cells were given to counter the blood loss of approximately 800 mL. The procedure lasted for 5 ½ h with stable hemodynamics, and the urine output was 400 mL. Postoperative course was uneventful; IV hydrocortisone 25 mg was repeated every 6th hourly for 24 h. Platelet count was 70,000 mm3, and there was no bleeding. She was discharged home on 10th postoperative day. Follow-up after 1-month was unremarkable.

Anesthetic management includes a thorough preoperative assessment, perioperative continuation of steroid/immunosuppression and an additional dose of steroid for possible suppression of hypothalamo-pituitary adrenal axis and evaluation of systemic organ involvement. Airway assessment may reveal unanticipated difficult airway, subglottic/laryngeal edema. Avoid trauma and bleeding during airway management. Strict asepsis must be maintained as these patients are at intrinsic susceptibility and also immunosuppressants.[5] A careful preanesthetic evaluation, a definitive anesthetic strategy with special considerations to risk of airway bleed, thrombocytopenia, systemic organ involvement and perioperative steroid replacement is mandatory.