Anesthetic Management Of VODI (Veno-Occlusive Disease With İmmunodeficiency) Syndrome


Kırova E., İnal D. G., Kaya S. İ., Erdağ T. K., Öçmen E.

Asian Society of Paediatric Anaesthesiologists “ASPA 2022, İstanbul, Türkiye, 14 - 16 Ekim 2022, ss.7

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: İstanbul
  • Basıldığı Ülke: Türkiye
  • Sayfa Sayıları: ss.7
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

Anesthetic management of VODI (veno-occlusive disease with immunodeficiency) syndrome

Purpose/aim: VODI (veno-occlusive disease with immunodeficiency) syndrome is a rare syndrome that occurs in patients who have received chemotherapy. It is a life-threatening complication of allogeneic or autologous hematopoietic stem cell transplantation (HSCT), most commonly associated with high-intensity chemotherapies. We did not find any publication in the literature on the method of anesthesia in vodi syndrome. In our case we reported our anesthesia experience.

Case presentation: Our case was a 13-year-old, 30 kg male patient who was diagnosed with CVID when he was 1 month old. The patient had a runny nose, cough, wheezing and secretory coarse rales. The patient's preoperative laboratory values were within normal limits, except for the minimal elevation in transaminase values. Intravenous immunoglobulin (IVIG) treatment was recommended for the patient in the preoperative period. Sedation was administered by intravenous (iv) 1 mg of midazolam. After anesthesia induction was achieved with IV 100 mg propofol, 0.2 mc/kg/min remifentanil infusion and 15 mg rocuronium, he was intubated with a 6.5 numbered cuffed tube. Anesthesia was maintained with 8 mg/kg/h propofol and 0.25 mc/kg/min remifentanil infusion. At the end of the operation, the patient was awakened and extubated. The patient was discharged home one day after the operation.

Discussion/Conclusion: Its pathogenesis includes multiple thrombotic and inflammatory factors that initially trigger damage to the endothelial cells lining the sinusoids of the hepatic acinus. Diagnosis of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) has historically been based on clinical examination by either Baltimore criteria or modified Seattle criteria. The latest studies reporting VOD/SOS risk factors support the importance of patient-related factors, such as acute kidney injury, increased international normalized ratio, female sex (in children), and platelet refractoriness. In conclusion, we think that TIVA anesthesia with propofol-remifentanil infusion is a safe and usable anesthesia option in a pediatric patient with VODI syndrome.