Intraventricular Hemorrhage and Multiple Intracranial Cysts Associated with Congenital Cytomegalovirus Infection

Abstract

c Intraventricular hemorrhage with congenital cytomegalovirus (CMV) infection is rare and has been reported only in extremely premature infants or in association with thrombocytopenia. We report the first case of a full-term male infant with congenital CMV infection and intraventricular hemorrhage with a normal platelet count and coagulation profile. The infant also had a left subependymal cyst and bilateral occipital cysts without any other manifestations of CMV infection. CASE REPORTT he mother of our patient was a 31-year-old woman (gravida 2, para 1) who was rubella and varicella immune, and all of her other serologic findings were negative. The parents were nonconsanguineous, and there was no family history of any bleeding disorder. Ultrasound examinations done at 12, 19, and 30 weeks of gestation showed normal fetal anatomy with a fetus appropriate for its gestational age.The pregnancy was uneventful until 38 weeks of gestation, when the mother felt that fetal movement had decreased. An ultrasound examination revealed asymmetric intracranial ventriculomegaly with the left lateral ventricle larger than the right and strands visible across both ventricles. The occipital horn of the right ventricle was also dilated, and there was echogenicity of the left choroid plexus suggestive of a blood clot (Fig. 1A). Fetal biometry was appropriate for the gestational age, the amniotic fluid index was normal, and the biophysical profile was 8/8.As an intraventricular hemorrhage was suspected, delivery was by cesarean section at 39 weeks of gestation with Apgar scores of 9 and 9 at 1 and 5 min, respectively. His birth weight was 2,790 g, his head circumference was 34 cm, and his length was 48 cm, all of which were appropriate for his gestational age. Except for mild hypotonia, the infant was normal upon examination.Magnetic resonance imaging (MRI) done on day 2 of life showed a moderately dilated left lateral ventricle with a subependymal cyst at the left foramen of Munro extending along the caudothalamic notch, consistent with a previous intraventricular hemorrhage. There were bilateral occipital cysts compressing the occipital horns with a thin intervening septation (Fig. 1B). A small-volume intraventricular hemorrhage was visible in the dependent portion of the left lateral ventricle (Fig. 1C). Myelination was appropriate for a term infant, and magnetic resonance spectroscopy was noncontributory.Given his history and neuroimaging findings, the infant was investigated for a bleeding disorder and bacterial and viral infections. Complete blood counts showed a platelet count of 190 ϫ 10 9 /liter (normal range, 150 ϫ 10 9 to 400 ϫ 10 9 /liter) with normal hemoglobin and white cell counts. The partial thromboplastin time was 46.6 s, and the international normalized ratio was 1.4, both of which were within the normal range for a term infant. Bacterial cultures and tests for parvovirus B19-specific IgM and IgG and toxoplasma-specific IgM and IgG were negative. Both the rapid plasma reagin assay and a line immunoassay (Innogene...

Findings and differential diagnosis of fetal intracranial haemorrhage and fetal ischaemic brain injury: what is the role of fetal MRI?

“…Detection of intracranial bleeding in that setting is unlikely to influence pregnancy management but will, at least, reassure the mother that the recurrence risk is low in future pregnancies. [29][30][31] Recent work has identified genetic mutations that may predispose to ICH. 32…”

“…In cytomegalovirus infection, temporal lobe destruction results in a characteristic distribution of intraparenchymal cystic lesions. 18,29 Porencephalic cysts are associated with severe developmental delay and seizure disorders due to the underlying brain destruction (Figures 5 and 6). Arachnoid cysts are simple in architecture and, unless associated with mass effect or other brain malformation, have no adverse impact on the infant.…”

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“…Even though initially he had no neurological symptoms, cranial ultrasound on day 5 of life showed heterogeneous, echogenic bilateral masses within the ventricular system indicative of bilateral germinal matrix hemorrhage; magnetic resonance imaging (MRI) at the age of 2 months revealed hyperintensity on fluid attenuated inversion recovery sequences were suggestive of white matter rarefaction and cystic degeneration. 5,6 Liver involvement presented initially as nonspecific hepatitis associated with cholestasis that worsened over the first month of life with the highest level of direct bilirubin 181 µmol/L. He was successfully managed with ursodiol, Omegaven (Fresenius Kabi AG, Bad Homburg, Germany), phenobarbital, and vitamins A, D, E, and K. Hepatobiliary iminodiacetic acid scan revealed mild-to-moderate excess of blood pool activity that indirectly confirmed hepatic dysfunction secondary to CMV hepatitis.…”

Comparison of Presentation, Course, and Outcome of Congenital and Acquired Cytomegalovirus Infection in Twins

“…Even though initially he had no neurological symptoms, cranial ultrasound on day 5 of life showed heterogeneous, echogenic bilateral masses within the ventricular system indicative of bilateral germinal matrix hemorrhage; magnetic resonance imaging (MRI) at the age of 2 months revealed hyperintensity on fluid attenuated inversion recovery sequences were suggestive of white matter rarefaction and cystic degeneration. 5 6 …”