Dale Sonia T, Coleman Lee T
Department of Radiology, The Royal Children's and Women's Hospital, Flemington Road, Parkville, Victoria, Australia 3052.
AJNR Am J Neuroradiol. 2002 Oct;23(9):1457-65.
Neonatal alloimmune thrombocytopenia (NAIT) is a maternal-fetal platelet antigen incompatibility disorder estimated to occur in one of 2000 to 5000 neonates. The diagnosis is made serologically by showing parental platelet antigen incompatibility and the presence of maternal platelet antibodies. In the absence of formalized antenatal screening, the radiologist has an important role to play in the recognition of this disorder, which has significant implications for the index case and any subsequent offspring. Our aim was to characterize the neuroradiologic findings and identify, if possible, a consistent pattern of neurologic injury typical of NAIT.
We retrospectively reviewed the ultrasonograms, CT scans, MR images, and medical histories of six patients (21 weeks gestation to 9 years old) with intracranial injury secondary to serologically proved NAIT.
Hemispheric porencephalic cysts (n = 6), primarily located within a temporal lobe with extension into other lobes, were seen on the ultrasonograms, CT scans, and MR images of all six children. In five cases, this was thought to represent encephaloclastic porencephaly and, in one case, schizencephaly (agenetic porencephaly). Six children had ventriculomegaly of varying degrees and severity and asymmetry. Extra-axial hemorrhages (n = 2), intraventricular hemorrhage (n = 1), acute parenchymal hemorrhage (n = 2), and neuronal migrational disorder (n = 1) occurred with varying frequency.
Antenatal or early postnatal neuroradiologic imaging showing hemispheric porencephaly and lateral ventriculomegaly is a recognizable pattern of cerebral injury suggestive of the diagnosis of NAIT. In the absence of a cost-effective screening program of primiparous women and neonates for this disease, the radiologist has an important role to play in the recognition of this disease entity. It is crucial for the reporting radiologist to consider the possibility of NAIT in any child with antenatal hemorrhage and, more importantly, with the pattern of cerebral injury described above. Because a high percentage of subsequent pregnancies might be equally or more severely affected, antenatal management directed at preventing intracranial hemorrhages in utero has become of significant clinical importance.
新生儿同种免疫性血小板减少症(NAIT)是一种母婴血小板抗原不相容性疾病,据估计在2000至5000名新生儿中会有1例发生。通过显示父母血小板抗原不相容以及母体血小板抗体的存在来进行血清学诊断。在缺乏规范化产前筛查的情况下,放射科医生在识别这种疾病方面起着重要作用,这对索引病例及任何后续后代都有重大影响。我们的目的是描述神经放射学表现,并尽可能确定NAIT典型的一致神经损伤模式。
我们回顾性分析了6例经血清学证实为NAIT继发颅内损伤患者(孕周21周-9岁)的超声、CT扫描、MR图像及病史。
所有6例患儿的超声、CT扫描及MR图像均显示半球性脑穿通畸形囊肿(n = 6),主要位于颞叶并延伸至其他脑叶。5例被认为代表脑破坏性脑穿通畸形,1例为脑裂畸形(发育不全性脑穿通畸形)。6例患儿有不同程度和严重程度的脑室扩大及不对称。轴外出血(n = 2)、脑室内出血(n = 1)、急性实质内出血(n = 2)及神经元迁移障碍(n = 1)出现频率各异。
产前或产后早期神经放射学成像显示半球性脑穿通畸形和侧脑室扩大是提示NAIT诊断的可识别脑损伤模式。在缺乏针对初产妇和新生儿的这种疾病的经济有效的筛查项目的情况下,放射科医生在识别这种疾病实体方面起着重要作用。对于报告的放射科医生而言,在任何产前出血的儿童中,更重要的是在具有上述脑损伤模式的儿童中考虑NAIT的可能性至关重要。由于后续妊娠中有很大比例可能受到同等或更严重的影响,旨在预防子宫内颅内出血的产前管理已具有重要的临床意义。