Case report of fetal liver cirrhosis due to gestational alloimmune liver disease in a primigravida female, in the northeast region of India

Abstract

Introduction: Fetal liver failure is a major cause of neonatal morbidity and mortality, presenting as acute liver failure and/or congenital cirrhosis. Gestational alloimmune liver disease associated with neonatal haemochromatosis is a rare cause of fetal liver failure. Case report: 24-year-old primigravida on Level II ultrasound scan showed intrauterine live fetus, with the fetal liver showing nodular architecture and coarse echotexture. Moderate fetal ascites were present. Scalp oedema was present with minimal bilateral pleural effusion. Suspicious fetal liver cirrhosis was raised, and the patient was counseled for a poor prognosis of pregnancy. Surgical termination of pregnancy was performed at 19 weeks through Cesarean section, and postmortem histopathological examination revealed haemochromatosis, hence gestational alloimmune liver disease was confirmed. Discussion: The presence of a nodular echotexture of the liver, with ascites, pleural effusion, and scalp oedema suggested chronic liver injury. Gestational alloimmune liver disease-neonatal haemochromatosis is often diagnosed late and patients are therefore referred late to specialized centers, delaying treatment. Conclusion: This case highlights the consequences of late diagnosis and treatment of gestational alloimmune liver disease-neonatal haemochromatosis and emphasizes the importance of a high grade of suspicion of this disease. Level II ultrasound scan should include scanning of the liver, as a part of the protocol. A high grade of suspicion is key for the diagnosis of gestational alloimmune liver disease-neonatal haemochromatosis, and early use of intravenous immunoglobulin should not be postponed to allow longer survival of the native liver.

Gestational alloimmune liver disease with alpha thalassaemia in a neonate

“…In Indian subcontinent, there is no published report of case of GALD successfully treated with IVIG. One report from north-east India described a case where diagnosis was made postmortem after medical termination of pregnancy3 and a case series from central India where diagnosis was made but subsequently babies succumbed without receiving IVIG or exchange transfusion (ET) treatment 4. Here, we report a unique case of a baby presenting with cholestatic jaundice and diagnosed to have GALD- NH treated successfully with IVIG.…”