Anesthetic Management with Total Intravenous Anesthesia in Hereditary Spherocytosis: A Case Report

Herediter Sferositoz’da Total İntravenöz Anestezi ile Anestezi Yönetimi

Abstract

Hereditary spherocytosis (HS) is a type of hemolytic anemia. Anesthetic management of any surgery with HS requires close monitoring of hypothermia, hypoxia, acidosis, and postoperative pain. We report a case of a 14-year-old male patient with HS scheduled for laparoscopic cholecystectomy. He was operated for splenectomy 5 years prior to admission. Hemoglobin (Hb) was 11.5 g/dL, platelets were 2,97,000 /mL, and total bilirubin was 4.50 mg/dL with direct bilirubin being 0.41 mg/dl. In peripheral blood smear (PBS) before surgery, spherocytosis percentage was 19%. He was premedicated with midazolam. After induction with propofol, fentanyl, and atracurium, anesthesia was maintained with air and O2 and total intravenous anesthesia (TIVA) was maintained with propofol and remifentanil. Electrocardiography, non-invasive blood pressure, peripheral oxygen saturation, and end-tidal carbon dioxide were monitored. We used an air warmer blanket to avoid hypothermia. Intraoperative arterial blood gas (ABG) values were evaluated at pH 7.37 (PCO2 = 38.1 mm Hg, PO2 = 177 mm Hg, Hb = 11.5 g/dL, and glucose = 109 mg/dL). Furthermore, intraoperative PBS was repeated, and spherocyte percentage was 11%. In post-anesthesia care unit, ABG values at pH 7.36 for PCO2, PO2, and Hb were 37.4 mm Hg, 210 mm Hg, and 11.4 g/dL, respectively. PBS was repeated on the next day, and spherocyte percentage was 16%. Multimodal analgesia was provided with tramadol, paracetamol, and local infiltration of the surgical site. Paracetamol infusion was given postoperatively three times a day for pain control. Intraoperative spherocyte percentage was less than that observed in preoperative PBS (11% and 19%, respectively). We consider that TIVA decreased spherocytes owing to appropriate anesthetic management, ABG stabilization, and normothermia each, either alone or together. On the next day, the spherocyte percentage was increased (16%) but still remained below the first spherocyte count. We consider that this is due to good pain control and well-balanced hydration. In conclusion, TIVA provided a safe anesthetic management.