Zerris Vasilios A, Annino Don, Heilman Carl B
Department of Neurosurgery, Tufts New England Medical Center, Boston, Massachusetts 02111, USA.
Neurosurgery. 2002 Sep;51(3):811-4; discussion 814.
Nasofrontal dermoid sinus cysts are rare. The embryological origin, presentation, treatment, and genetic associations of two cases of these cysts are discussed. Emphasis is placed on physical findings and the importance of addressing both the intracranial and extracranial components.
The first patient, a 33-year-old woman, sought care for chemical meningitis. As a child, she was differentiated from her identical twin sister by a dimple on the tip of her nose. The second patient, a 34-year-old man, sought care for new-onset seizures. Since birth, he had a dimple on the tip of his nose. As a child, he had undergone resection of a nasal cyst. Imaging studies in both patients indicated a midline anterior cranial base mass within the falx and a defect in the crista galli.
Both patients underwent biorbitofrontal nasal craniotomy. A bifrontal craniotomy was performed first, then removal of the orbitonasal ridge. The dermoid and involved falx were resected. The sinus tract was followed through the crista galli and resected up to the osteocartilaginous junction in the nose. The remainder of the tract was resected via a small incision through the nares. The dura was closed primarily by mobilizing the dura along the sides of the crista galli. After surgery, both patients still possessed their sense of smell.
Nasofrontal dermoid sinus cysts have a unique embryological origin. A midline basal frontal dermoid associated with a dimple on the nasal surface with or without protruding hair and sebaceous discharge is the pathognomonic presentation. It is important to address both the intracranial and extracranial component surgically. Although concomitant anomalies and familial clustering have been described, most cases are spontaneous occurrences.
鼻额部皮样窦囊肿较为罕见。本文讨论了两例此类囊肿的胚胎学起源、临床表现、治疗方法及基因关联。重点关注体格检查结果以及处理颅内和颅外部分的重要性。
首例患者为一名33岁女性，因化学性脑膜炎前来就诊。她小时候鼻尖有一个酒窝，借此与同卵双胞胎姐妹区分开来。第二例患者是一名34岁男性，因新发癫痫前来就诊。自出生起，他鼻尖就有一个酒窝。小时候，他曾接受过鼻囊肿切除术。两名患者的影像学检查均显示在大脑镰内有一个中线前颅底肿物，鸡冠处有缺损。
两名患者均接受了双眶额鼻开颅手术。先进行双额开颅，然后切除眶鼻嵴。切除皮样囊肿及受累的大脑镰。沿鸡冠追踪窦道并切除至鼻内的骨软骨交界处。通过鼻孔处的小切口切除窦道的其余部分。通过沿鸡冠两侧游离硬脑膜，主要对硬脑膜进行缝合。术后，两名患者仍保留嗅觉。
鼻额部皮样窦囊肿有独特的胚胎学起源。中线基底额部皮样囊肿伴有鼻表面酒窝，有或无毛发突出及皮脂分泌，是其典型表现。通过手术处理颅内和颅外部分很重要。虽然已有伴发异常和家族聚集性的描述，但大多数病例是自发发生的。
