[Hepatitis and panmyelophthisis].
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Biomedical subjects
Publications and source records attributed to L Wille.
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Fifteen patients with non-immune fetal or neonatal ascites and absence of generalized edema are described. Nine patients were diagnosed prenatally. Nine patients were preterm. Artificial ventilation was necessary in twelve infants immediately after birth. In five cases, including two cases of transitory ascites, the etiology could not be established. In accordance with previous reports, we found the following underlying disorders in our patients: urinary tract obstruction, polycystic disease of the kidneys, chylous ascites, sepsis, intrahepatic atresia of the biliary ducts, hepatic and renal failure following severe asphyxia. 8 out of 15 patients died. A survey of the literature shows a variety of underlying disorders. Therefore, several diagnostic tests are necessary, in order to identify the etiology. Causative treatment is possible in some cases of urogenital , gastrointestinal, infectious and chylous ascites. Sophisticated perinatal management is essential for the outcome of patients with fetal or neonatal ascites; nevertheless the mortality rate is still about 50%.
With the advent of prenatal diagnosis and the feasibility of intrauterine treatment, non-immune hydrops fetalis (NHIF) is gaining increasing importance. 14 patients are reported 10 of whom having been diagnosed prenatally. 13 were born prematurely severe asphyxia being a frequent occurrence. 7 out of 14 infants survived. In 5 cases no definite etiology could be established. In 7 patients NHIF was attributable to cardiovascular diseases, predominantly associated with brady- or tachycardia. A survey of the literature reveals that etiology remains unidentified in about 40%. For the rest fetal, maternal or placental diseases are the primary causes. The abundance of underlying diseases are operative via three pathophysiological pathways: hemodynamic disturbances, decreased oncotic pressure and increased capillary permeability. By ultrasound examination, NHIF can be detected prenatally permitting intrauterine treatment in selected cases. In combination with improved postnatal management, this might contribute to a better outcome of NIHF which is still associated with a high mortality rate. Meticulous diagnostic work-up facilitates genetic counseling and the pursuit of prenatal diagnosis in subsequent pregnancies.