Akpinar Elif, Gürbüz Mehmet Sabri, Okutan Mehmet Özerk, Beşkonakli Ethem
Karatay University Medical Faculty, Medicana Hospital, Department of Neurosurgery, Konya.
Istanbul Medeniyet University, Medical School, Department of Neurosurgery, Istanbul.
J Craniofac Surg. 2019 Sep;30(6):1780-1781. doi: 10.1097/SCS.0000000000005432.
Holoprosencephaly (HPE) is a developmental anomaly of forebrain characterized by a failure of division of the embryonic forebrain into hemispheres. It is associated with a set of facial anomalies at a rate of 80%. Survival rate, particularly in alobar HPE, is quite low. Alobar HPE is usually associated with a large dorsal cyst which might eventually lead to hydrocephalus and raised intracranial pressure. Placement of ventriculoperitoneal (VP) shunt has been reported to be beneficial in symptomatic hydrocephalus accompanying HPE. Here we report a preterm infant born with alobar HPE and undergoing VP shunt placement although there was no sign of raised intracranial pressure. She is 12 months old now having near-normal developmental progress. This case has revealed that the placement of VP shunt, particularly inserting the catheter tip into dorsal cyst of HPE, might be beneficial and contribute to the survival and further brain development even in the absence of the signs of raised intracranial pressure.
前脑无裂畸形（HPE）是一种前脑发育异常，其特征为胚胎前脑未能分裂成两个半球。它与一系列面部异常相关，发生率为80%。存活率，尤其是无脑叶型前脑无裂畸形的存活率相当低。无脑叶型前脑无裂畸形通常与一个大的背侧囊肿相关，这最终可能导致脑积水和颅内压升高。据报道，脑室腹腔（VP）分流术对前脑无裂畸形伴发的症状性脑积水有益。在此，我们报告一名早产的无脑叶型前脑无裂畸形婴儿，尽管当时没有颅内压升高的迹象，但仍接受了VP分流术。她现在12个月大，发育进程接近正常。该病例表明，VP分流术的放置，尤其是将导管尖端插入前脑无裂畸形的背侧囊肿，即使在没有颅内压升高迹象的情况下，也可能是有益的，并有助于存活和进一步的脑发育。
