Produção Nacional
Revisado por pares
Fetal brain lesion associated with spontaneous twin anemia–polycythemia sequence
2013; Wiley; Volume: 42; Issue: 6 Linguagem: Inglês
10.1002/uog.12574
ISSN1469-0705
AutoresDaniele Luminoso, Camilla Olivares Figueira, Marcos Marins, Cleisson Fábio Andrioli Peralta,
Tópico(s)Hemoglobinopathies and Related Disorders
ResumoA healthy 21-year-old primigravida with monochorionic diamniotic twins was referred to our fetal medicine unit at 23 weeks' gestation for follow-up. Twin A had a middle cerebral artery Doppler peak systolic velocity (MCA-PSV) of 1.72 multiples of the median (MoM), and Twin B had an MCA-PSV of 0.95 MoM (Figure 1)1. Spontaneous Stage 1 twin anemia–polycythemia sequence (TAPS) was suspected2. After discussion about the treatment options, the couple opted for expectant management2. At 24 weeks, an extensive echogenic brain lesion was observed in the left hemisphere of Twin B, in addition to severe ipsilateral ventriculomegaly of 15 mm, which is compatible with a Grade 3 peri-intraventricular hemorrhage (Figure 2). It was suspected that TAPS had progressed to Stage 2 (MCA-PSV MoM values of 1.93 and 0.6 in Twin A (donor) and Twin B (recipient), respectively)2. Fetal brain diffusion-weighted (DW) magnetic resonance imaging (MRI) in Twin B revealed areas of water diffusion restriction around the left ventricle and confirmed the suspicion of a Grade 3 peri-intraventricular hemorrhage (Figure 2). At 31 + 4 weeks the patient sought emergency obstetric care because of regular uterine contractions. Total cervical dilatation prompted a vaginal delivery. The hemoglobin (Hb) levels in the donor and the recipient were 10.7 g/dL and 17.5 g/dL, respectively (intertwin Hb discrepancy of 6.8 g/dL). The platelet counts in the donor and recipient were normal. Twin B was diagnosed with a left Grade 3 peri-intraventricular hemorrhage with mild ipsilateral ventriculomegaly on a day 1 transfontanellar ultrasound scan. On the 30th day of postnatal life, ventriculoperitoneal shunting was performed in response to an increase in the twin's head circumference and deterioration in neurological status. To our knowledge, this is the second case of a brain lesion related to TAPS described in the literature, but it is the first one to be diagnosed prenatally3. The incidence of spontaneous TAPS ranges from 3 to 5%, whereas the incidence of the iatrogenic form (postlaser for twin oligopolyhydramnios sequence) ranges from 2 to 13%4, 5. Based on the prenatal sonographic and MRI findings, we believe that the fetal brain injury was caused by hyperviscosity–polycythemia syndrome3. In fact, DW-MRI revealed areas of water diffusion restriction around the left ventricle in addition to alterations compatible with a Grade 3 peri-intraventricular hemorrhage. Suggested treatments for TAPS are delivery, intrauterine blood transfusion, laser ablation of the placental anastomoses, selective termination or expectant management2. In the present case, the couple chose expectant management, but unfortunately, this decision was not ultimately the best. It is probable that fetoscopic laser treatment at the time of diagnosis would have been the best option. TAPS is a recently described condition that is most probably underdiagnosed. Implementing the serial evaluation of MCA-PSV in monochorionic pregnancies is vital because it is currently the only prenatal technique available for diagnosing, staging and following this disease. Our case shows that even spontaneous cases of TAPS that are mild at the time of diagnosis can rapidly and unexpectedly create complications and cause severe irreversible brain damage in the recipient twin. D. Luminoso†, C. O. Figueira†, M. Marins‡ and C. F. A. Peralta*† †Departamento de Ginecologia e Obstetrícia, Hospital Professor José Aristodemo Pinotti, Centro de Atenção Integral à Saúde da Mulher (CAISM), Universidade Estadual de Campinas (UNICAMP), Rua Alexander Fleming, 101 – Cidade Universitária Zeferino Vaz, Distrito de Barão Geraldo, Campinas, S.P., Brazil; ‡Campinas Radiology Center, Vera Cruz Hospital, Campinas, Brazil *Correspondence. (e-mail: cfaperalta@gmail.com)
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