Anaesthetic management of a patient posted for liver transplant with CriglerNajjar syndrome type-1

Dr.Nisarg Patel; Dr.Yogeshkumar Jadav

Volume : VII, Issue : XI, November - 2018

Anaesthetic management of a patient posted for liver transplant with CriglerNajjar syndrome type-1

Dr. Nisarg Patel, Dr. Yogeshkumar Jadav

Abstract :

Introduction: Crigler–Najjar syndrome is a rare genetic disorder caused by lack of UDP– lucuronyltransferase enzyme. Type I–characterized by a nearly complete lack of enzyme activity and severe and It has to be immediately treated after birth by exchange transfusion followed by phototherapy. Orthotropic liver transplant is currently preferred type of Liver transplant which is performed at the age of 3–5 years. This syndrome can potentially affect the metabolism of these drugs leading to adverse outcomes.

Case report:A 6 year old male child withCriglerNajjar Syndrome Type–1 was posted for orthotopic liver transplantation. LFT showed total bilirubin 26.5 md/dl, Direct bilirubin 0.9 mg/dl,Indirect bilirubin 25.6 mg/dl. Premedication was with Inj. Glycopyrollate 0.1mg and Inj. Fentanyl 40ug. The patient was maintained intra–operatively with infusions of Atracurium and Noradrenaline. Inj midazolam was given in anhepatic phase. Patient was connected to circle system and maintained with N2O: O2 – 2: 1 and Isoflurane – 0.2% – 1%.The Patient was shifted to Post–Liver Transplant ICU as intubated and kept on SIMV–PC for 2hrsThe patient was then shifted to CPAP–PSV mode for 2hrs and finally extubated after 4hrs and shifted to Post–Liver Transplant ward.

ConclusionAnaesthesiologists should be aware of the medicine decreased glucuronyltransferase activity to prevent intraoperative toxicity of anaesthetic drugs. The anaesthetic goal in cå for patients with Crigler–Najjar disease is prevention of increased free bilirubin in the serum. Halothane should be avoided during anaesthesia because of its high liver metabolism (20%). Isoflurane was the preferred volatile anaesthetic.Paracetamol and morphine were specifically avoided as they are metabolized by conjugation in the liver.Meticulous peri–operative management can result in giving a new life to a moribund patient