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巨大额鼻脑膨出:通过临床病例及文献回顾的手术考量

Giant frontonasal encephalocele: surgical considerations through a clinical case and literature review.

作者信息

Pascuetín Gisela, Gromadzyn Guido, Márquez Yamila Basilotta, Blanco Rodrigo, Asselborn Juan, Argañaraz Romina

机构信息

Department of Neurosurgery, Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina.

出版信息

Childs Nerv Syst. 2025 Mar 19;41(1):136. doi: 10.1007/s00381-025-06780-x.

Abstract

INTRODUCTION

Encephalocele is a congenital malformation of the central nervous system (CNS) characterized by the protrusion of neural tissue through a cranial defect. The frontonasal variant has a reported incidence of approximately 0.8 to 3 cases per 10,000 live births, making it a rare condition.

CASE REPORT

We present the case of a newborn girl prenatally diagnosed with a giant nasofrontal encephalocele without associated pathologies. Following complementary studies for surgical planning, she underwent surgery at 72 h of life, performed by a multidisciplinary team, to prevent significant functional sequelae and promote normal growth and development.

CONCLUSION

Nasofrontal encephalocele is a rare type of neural tube defect, posing a significant challenge for the interdisciplinary team responsible for its management.

摘要

引言

脑膨出是一种中枢神经系统(CNS)的先天性畸形,其特征是神经组织通过颅骨缺损突出。据报道,鼻额部变异型的发病率约为每10000例活产中有0.8至3例,是一种罕见的病症。

病例报告

我们报告了一名产前诊断为巨大鼻额部脑膨出且无相关病变的新生女婴病例。在进行了用于手术规划的补充检查后,她在出生72小时时接受了由多学科团队进行的手术,以预防严重的功能后遗症并促进正常生长发育。

结论

鼻额部脑膨出是一种罕见的神经管缺陷类型,对负责其管理的跨学科团队构成了重大挑战。

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