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菲律宾额筛窦脑膨出的修复:2008年至2013年间30例病例报告

Repair of Frontoethmoidal Encephalocele in the Philippines: An Account of 30 Cases Between 2008-2013.

作者信息

Marshall Amanda-Lynn, Setty Pradeep, Hnatiuk Mark, Pieper Daniel R

机构信息

Oakland University William Beaumont School of Medicine, Rochester Hills, Michigan, USA.

Department of Neurosurgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.

出版信息

World Neurosurg. 2017 Jul;103:19-27. doi: 10.1016/j.wneu.2017.03.063. Epub 2017 Mar 23.

Abstract

BACKGROUND

Frontoethmoidal encephalocele is a congenital abnormality of the anterior skull base involving herniation of cranial contents through a midline skull defect. Patency of the foramen cecum, along with other multifactorial variables, contributes to the development of frontoethmoidal encephaloceles. Because of limited resources, financial constraints, and lack of surgical expertise, repair of frontoethmoidal encephaloceles is limited in developing countries.

METHODS

Between 2008 and 2013 an interdisciplinary team composed of neurosurgeons, craniofacial surgeons, otolaryngologists, plastic surgeons, and nursing personnel, conducted surgical mission trips to Davao City in Mindanao, Philippines. All patients underwent a combined extracranial/intracranial surgical approach, performed in tandem by a neurosurgeon and a craniofacial surgeon, to detach and remove the encephalocele. This procedure was followed by reconstruction of the craniofacial defects.

RESULTS

A total of 30 cases of frontoethmoidal encephalocele were repaired between 2008 and 2013 (20 male; 10 female). The average age at operation was 8.7 years, with 7 patients older than 17 years. Of the 3 subtypes, the following breakdown was observed in patients: 18 nasoethmoidal; 9 nasofrontal; and 3 naso-orbital. Several patients showed concurrent including enlarged ventricles, arachnoid cysts (both unilateral and bilateral), and gliotic changes, as well as orbit and bulbus oculi (globe) deformities. There were no operative-associated mortalities or neurologic deficits, infections, or hydrocephalus on follow-up during subsequent trips.

CONCLUSIONS

Despite the limitations of performing advanced surgery in a developing country, the combined interdisciplinary surgical approach has offered effective treatment to improve physical appearance and psychological well-being in afflicted patients.

摘要

背景

额筛窦脑膨出是一种先天性前颅底异常,涉及颅内容物通过中线颅骨缺损疝出。盲孔通畅以及其他多因素变量导致额筛窦脑膨出的发生。由于资源有限、经济限制以及缺乏手术专业知识,发展中国家额筛窦脑膨出的修复受到限制。

方法

2008年至2013年期间,一个由神经外科医生、颅面外科医生、耳鼻喉科医生、整形外科医生和护理人员组成的跨学科团队前往菲律宾棉兰老岛的达沃市进行手术任务。所有患者均接受了由神经外科医生和颅面外科医生联合进行的颅外/颅内联合手术方法,以分离并切除脑膨出。此程序之后是颅面缺损的重建。

结果

2008年至2013年期间共修复了30例额筛窦脑膨出(男性20例;女性10例)。手术的平均年龄为8.7岁,7例患者年龄超过17岁。在3种亚型中,患者的分布如下:鼻筛型18例;鼻额型9例;鼻眶型3例。一些患者同时存在包括脑室扩大、蛛网膜囊肿(单侧和双侧)、胶质增生改变以及眼眶和眼球畸形。在随后的随访中,没有手术相关的死亡、神经功能缺损、感染或脑积水。

结论

尽管在发展中国家进行高级手术存在局限性,但联合跨学科手术方法为改善患病患者的外貌和心理健康提供了有效的治疗。

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