BACKGROUND:Early fetal herpes simplex virus (HSV) infection is rarely documented. Only the minority of affected fetuses survive this condition.PATIENT AND METHODS:At 19 weeks of gestation the first episode of a genital HSV-infection of a pregnant woman was treated with local interferon beta. At 34 weeks of gestation hydrocephalus with secondary microcephaly and microphthalmia of both eyes was detected by ultrasonography. In the amniotic fluid HSV type 2 (HSV-2) was isolated and HSV-2-DNA was detected by PCR. The serum of the mother proved positive for HSV-2 (glycoprotein G2)-specific IgG-antibodies. No other infectious causes were apparent on further testing. At 35 + 4 weeks gestation a small-for-gestational-age neonate (2130 g) with microcephaly (29 cm head circumference) was born by spontaneous vaginal delivery. Scarce ulcerative skin lesions and vesicles, hepatosplenomegaly and microphthalmia were diagnosed. Furthermore, encephalomalacia with parenchymal destruction, cataract of both eyes and aplasia of the maculae and papillae were found. HSV-2-PCR was tested positive in chorionic cells and an umbilical segment of the placenta as well as in swabs from both eyes, throat, and a herpetic skin lesion collected during the first 5 days of life. HSV-IgM-antibodies were found in the umbilical cord blood. Local and intravenous treatment with aciclovir was started. The infant exhibited signs of a severely malfunctioning central nervous system. At the age of 4 months the boy suffered from generalised cerebral seizures. He died at the age of 9 months as a consequence of respiratory insufficiency with consecutive circulation failure.RESULTS:The case of an intrauterine HSV-2-infection is presented. The time of onset of fetal infection was most probably at the time of the maternal disease (19 weeks of gestation). Inspite of the very early infection the fetus did not die in utero.CONCLUSIONS:Especially, if a primary genital HSV-2-infection of a pregnant woman is suspected, which can be proven by serological means only several weeks after infection, systemic therapy of the mother with aciclovir should be considered since materno-fetal transmission may occur due to the risk of maternal viraemia.

Two cases of non-immunological hydrops fetalis (NIHF) presenting with massive ascites are reported; in both patients an oligosaccharid-pattern in the urine typical for sialidosis resp. galactosialidosis was found. The cerebral sonography of both patients showed streaky echo enhancement in the region of the thalamostriatal vessels, which was interpreted as calcification of the vessels. The courses of the patients were characterised by recurrent infections, hepatosplenomegaly and myoclonus. Relevant Literature reports on a large variability in the clinical appearance of oligosaccharidoses.The diagnosis of sialidosis is confirmed in cultured fibroblasts by the deficiency of alpha-N-acetylneuraminidase and, in case of galactosialidosis by the additional lack of beta-galactosidase. The precise diagnosis in NIHF is of increasing interest for prenatal diagnostic as well as for neonatological management.

In three newborns already during the fetal period hydrocephalus internus had been diagnosed by ultrasound later found to be due to sinus venous thrombosis. After birth two of these newborns had no remarkable neurological signs, the third had seizures, All of them had hydrocephalus internus, intracerebral hemorrhage, and non or reduced doppler ultrasound flow within the venous sinus, Etiopathology is discussed with reference to the literature: damage of the endothelium, venous stasis, hyperviscosity, hypercoagulation slate, protein C-, S- and antithrombin III-deficiencies, trauma.