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