(MIM 321 100)

Very rare perinatal pathology with antenatal onset. Recent data argue for a process of alloimmunisation of the mother against her fetus liver, which explains the important recurrence rate (> 90 %) in siblings. Accumulation of intra - and extrahepatic of iron which causes liver damage in utero. Acute liver failure in the neonatal period (generally in the first 48 h of life) with high blood ferritin levels. At histology, Fe accumulation in the liver, salivary glands and pancreas. Very poor prognosis, even in case of neonatal liver transplantation. 

Some cases of survival have been described with very early antioxidant treatment: desferoxamine (30 mg/kg/day), selenium, tocopherol. The administration of IV gammaglobulins  to the mother during the second half of pregnancy gives excellent results.

Anesthetic implications: 

acute liver failure.

References : 

-         Whitington PF. 
Neonatal hemochromatosis: a congenital alloimmune hepatitis. 
Semin Liver Dis 2007; 27: 243-50.

-         Sharma A, Cotterell AH, Maluf DG, Posner M, Fisher RA. 
Living donor liver transplantation forr neonatal hemochromatosis using non-anatomically resected segments II and III: a case report.  
J of Medical Case Reports  2010; 4: 372.

-        Baruteau J, Heissat S, Collardeau-Frachon S, Debray D, Broué P, Guigonis V. 
Nouveaux concepts dans l’hémochromatose périnatale. 
Arch Pédiatr 2012 ; 19 : 755-61.

Updated: November 2018