Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disease derived from biliary dysgenesis secondary to ductal plate malformation; it often coexists with Caroli’s disease, von Meyenburg complexes, autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). Although CHF was first named and described in detail by Kerr et al. in 1961. Its pathogenesis still remains unclear. The exact incidence and prevalence are not known, and only a few hundred patients with CHF have been reported in the literature to date. However, with the development of noninvasive diagnostic techniques such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), CHF may now be more frequently detected. Anatomopathological examination of liver biopsy is the gold standard in diagnosis of CHF. Patients with CHF exhibit variable clinical presentations, ranging from no symptoms to severe symptoms such as acute hepatic decompensation and even cirrhosis. The most common presentations in these patients are splenomegaly, esophageal varices, and gastrointestinal bleeding due to portal hypertension. In addition, in younger children, CHF often is accompanied by renal cysts or increased renal echogenicity. Great variability exists among the signs and symptoms of the disease from early childhood to the 5
th
or 6
th
decade of life, and in most patients the disorder is diagnosed during adolescence or young adulthood. Here, we present two cases of congenital hepatic fibrosis in 2-years-old girl and 12-year-old male who had been referred for evaluation of an abdominal distension with persistent hyper-transaminasemia and cholestasis, the diagnostic was made according to the results of medical imaging (CT or MRI), a liver biopsy, and genetic testing.
Vasa previa is a rare but clinically important obstetrical complication that can be associated with a low-lying placenta or placenta previa. We aim to present one case of vasa previa diagnosed during the placenta examination after the caesarean indicated for triple uterus scar. A 26-year-old female was referred to our hospital at 30 weeks of gestation to provide a scheduled caesarean. Trans-abdominal ultrasound was performed; the placenta was positioned in the posterior side of the fundus. Fetal growth was found to be appropriate for gestational age. A healthy male infant weighing was successfully delivered via cesarean section at 38 weeks of gestation. This operation helped to prevent complications due to acute fetal bleeding. The identification and exclusion of vasa previa using trans-vaginal ultrasound are essential to ensure appropriate and timely treatment.
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