Congenital hepatic fibrosis in pregnancy

The purpose of this study was to analyse the course of pregnancy of a 20-year-old woman suffering from congenital hepatic fibrosis (CHF), a genetic disease with recessive autosomic transmission, attributed to the group of hepato-nephro-pancreatic fibropolycystic familial diseases. This was the only time such a case had ever been encountered in a pregnancy in the history of Turin University's Obstetrics and Gynaecological Clinic. The case of a 20-year-old woman is considered. She was admitted to our observation at the Obstetrics and Gynaecological Department of Faculty "B" of the University of Turin, suffering from CHF, a primigravid in the eighth week of gestation. Haematochemical tests carried out on the patient showed a platelet deficiency without evident changes in liver and renal function. Objective examination revealed a slight hepatosplenomegaly confirmed by abdominal echotomography. The haematochemical examinations subsequently carried out confirmed thrombocytopenia due to hepatosplenic hyperhaemocataresis. In agreement with the will of the patient and her family, and after having heard the opinion of our hospital's anaesthetists and internists, a conservative approach was decided on and close monitoring of maternal and foetal conditions was planned, with hospitalisations scheduled for every fortnight. Together with the internists and anaesthetists, it was decided to allow the pregnancy to proceed as far as foetal maturity but not to term, to avoid problems relating to the excessive increase in uterine volume which would have led to pressure on the portal tree and so favoured haemorrhaging due to rupture of the oesophageal varices. Caesarean delivery was planned for the 37th week of gestation. The birth went according to plan and the foetus was a live, vital male with Apgar index of 9/9. The placenta presented normal characteristics and a weight of 590 g. No haemorrhagic complications were observed either in the course of surgery or in the postoperative phase, notwithstanding the preoperative finding of a platelet count of 64,000 platelets/ mm3. Although in our case neither the pregnancy nor the delivery constituted a serious danger for the life of the mother, possible obstetric complications are among the most serious encountered in obstetric pathology and can lead both to maternal death and to the intrauterine death of the foetus. For these reasons we believe it essential to provide correct, clear and adequate information about the risks involved for patients suffering from congenital hepatic fibrosis, especially if they are associated with renal polycystic disease, and who are admitted to our wards with the desire to programme a pregnancy.