Arvanitis Leonidas D, Sgantzos Markos N, Kotrotsios Anastasios, Vassiou Katerina G
Department of Pathology, Aghia Sophia Children's Hospital, Athens, Greece.
Pediatr Dev Pathol. 2010 Sep-Oct;13(5):419-22. doi: 10.2350/09-08-0690-CR.1. Epub 2010 Feb 16.
In this article we present a case of a male newborn with a CNS malformation that is characterized mainly by complete fusion of the thalami resulting in atresia of the 3rd ventricle accompanied by fusion of the anterior peduncles of the fornix, the presence of a single occult interventricular foramen lying at the midline, absence of the septum pellucidum, hypoplasia of the corpus callosum, disorganization of the head of the left caudate nucleus, and greatly dilated lateral ventricles (hydrocephalus). The patient underwent surgical correction of the meningocele on his 4th postnatal day. On his 13th postnatal day he had projectile vomiting due to a left parietooccipital hygroma that was drained via a shunt. On his 31st postnatal day he developed seizures and marked dilatation of the lateral ventricles, for which he underwent a ventriculoperitoneal shunt (Brown). On the 14th postoperative day the patient developed aspiration pneumonia and died.
在本文中，我们介绍了一例患有中枢神经系统畸形的男性新生儿，其主要特征为丘脑完全融合，导致第三脑室闭锁，同时伴有穹窿前脚融合、中线处单一隐匿性室间孔、透明隔缺如、胼胝体发育不全、左侧尾状核头部结构紊乱以及侧脑室极度扩张（脑积水）。该患者在出生后第4天接受了脑膜膨出的手术矫正。出生后第13天，他因左侧顶枕部脑脊膜膨出出现喷射性呕吐，通过分流术进行了引流。出生后第31天，他出现癫痫发作和侧脑室明显扩张，为此接受了脑室腹腔分流术（布朗手术）。术后第14天，患者发生吸入性肺炎并死亡。
