Sarica Can, Yucetas Cem, Ozen Ali, Ucler Necati, Konca Capan, Akar Selahattin
Division of Neonatal and Developmental Medicine, Department of Neurosurgery, School of Medicine, Adiyaman University, Adiyaman, Turkey.
Division of Neonatal and Developmental Medicine, Department of Pediatrics, School of Medicine, Adiyaman University, Adiyaman, Turkey.
Pediatr Neurosurg. 2018;53(5):337-341. doi: 10.1159/000489856. Epub 2018 Jun 14.
Holoprosencephaly is a rare congenital malformation resulting from an impaired midline division of the prosencephalon into distinct cerebral hemispheres. Hydrocephalus is a frequent problem among the few survivors with alobar holoprosencephaly (aHPE), its most severe form. The literature about neurosurgical management of hydrocephalus in this condition is limited and dispersed, and there are still some points that need to be resolved. We report the case of a newborn with aHPE, hydrocephalus, and central diabetes insipidus. We delineate the complexity of the management of these patients and emphasize the benefits of using an initial programmable shunt valve. Further discussion about management strategies includes reviewing previous reports and the benefits of shunting for hypothalamic osmoreceptor function.
前脑无裂畸形是一种罕见的先天性畸形，由前脑中线分裂受损导致无法形成明显的大脑半球所致。脑积水是最严重的叶状前脑无裂畸形（aHPE）少数幸存者中常见的问题。关于这种情况下脑积水的神经外科治疗的文献有限且分散，仍有一些问题需要解决。我们报告了一例患有aHPE、脑积水和中枢性尿崩症的新生儿病例。我们阐述了这些患者治疗的复杂性，并强调了使用初始可编程分流阀的益处。关于治疗策略的进一步讨论包括回顾既往报告以及分流对下丘脑渗透压感受器功能的益处。
