Fetal cerebellar hemorrhage: three cases with postnatal follow-up.

Abstract

The cerebellum develops rapidly during late gestation. Cerebellar hemorrhage (CBH) is therefore a relatively common complication of preterm birth, which is associated with a broad spectrum of neurological disabilities1-3. Prenatally, however, CBH is a rare event. Although recent advances in prenatal neuroimaging have led to increased recognition of cerebellar lesions in high-risk fetuses, information about the prognostic consequences is limited4, 5. We report on three cases of isolated fetal CBH, born at our tertiary referral center for antenatal diagnosis and treatment of fetal disorders. Case 1 was a 31-year-old woman referred at 13 + 6 weeks' gestation because of rhesus D alloimmunization. Due to fetal anemia, four uncomplicated intrauterine blood transfusions (IUTs) were performed between 20 and 34 weeks' gestation. Follow-up ultrasound examinations showed mild ventriculomegaly. At 37 + 1 weeks' gestation, a female infant of 3030 g was born by uncomplicated vaginal delivery. Postnatal cranial ultrasound examination, performed 1 day after delivery, showed normal supratentorial structures but relatively small cerebellar vermis and hemispheres with enlargement of the fourth ventricle and cisterna magna, raising suspicion of cerebellar hypoplasia (Figure 1a). Postnatal magnetic resonance imaging (MRI) at 2 months showed hypoplasia of the cerebellar vermis and hemispheres with bilateral hemosiderin deposits, confirming the postnatal diagnosis of fetal CBH with subsequent cerebellar atrophy (Figure 1b and c). Case 2 was a 33-year-old woman who was referred at 21 + 6 weeks' gestation because of rhesus D alloimmunization and fetal hydrops, for which emergency IUT was performed. Ultrasound examination prior to IUT was normal, however when performed the day after IUT abnormal echogenicity in both cerebellar hemispheres was seen, leading to a suspected diagnosis of hemorrhage (Figure 2a). Fetal MRI 7 days after IUT confirmed isolated bilateral CBH (Figure 2b). Except for impaired cerebellar development, fetal growth and development remained normal. At 36 + 5 weeks' gestation a male infant of 2900 g was born by uncomplicated vaginal delivery. Postnatal MRI showed hypoplasia of both cerebellar vermis and hemispheres, with hemosiderin deposits in both hemispheres (Figure 2c and d). Case 3 was a 33-year-old woman, referred at 20 + 2 weeks' gestation because of twin-to-twin transfusion syndrome (Quintero stage III). Laser coagulation of placental anastomoses was complicated by intra-amniotic blood loss. Ultrasound examination on the following day showed fetal demise of the recipient twin. The donor appeared in good condition with no sign of anemia, but ultrasound examination showed suspected hemorrhage in the right cerebellar hemisphere. This was confirmed by MRI at 23 + 3 weeks' gestation. Subsequent ultrasound examinations showed stabilization of the cerebellar lesion. At 33 + 5 weeks' gestation a female infant of 2220 g was delivered by Cesarean section because of signs of intrauterine infection after preterm prelabor rupture of membranes. Postnatal cranial ultrasound examination showed hypoplasia of the right cerebellar hemisphere with a normal vermis. Postnatal MRI was not performed as parents did not give consent. All three cases resulted in liveborn infants who were evaluated subsequently by postnatal neuroimaging and neurodevelopmental follow-up. Despite the fact that there was vermian involvement in Cases 1 and 2, all had unremarkable neurological development at 1–2 years of age. The majority of reports on fetal CBH are described after termination of pregnancy or neonatal death, and therefore information about its prognosis is limited4, 5. Involvement of the vermis seems to be a risk factor, especially for impaired cognitive function and social behavioral deficits5. Findings from our cases suggest that isolated fetal CBH may have a favorable short-term prognosis. However, the long-term prognostic implications need further investigation. We thank all involved subjects from the Departments of Child Neurology, Neonatology and Gynecology & Obstetrics of the LUMC. N.A.A. is supported by a VENI-grant (#91615080) from The Netherlands Organization of Scientific Research. N. A.Aziz†, C. M.Peeters-Scholte†, F. T.de Bruine‡, F. J.Klumper§, P. N.Adama van Scheltema§, E.Lopriore¶ and S. J.Steggerda*¶ †Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands; ‡Department of Neuroradiology, Leiden University Medical Centre, Leiden, The Netherlands; §Department of Obstetrics and Fetal Medicine, Leiden University Medical Centre, Leiden, The Netherlands; ¶Department of Neonatology, Leiden University Medical Centre, Albinusdreef 2, 2333 AZ, Leiden, The Netherlands *Correspondence. (e-mail: [email protected])