Fujioka Masaki, Tasaki Isao, Nakayama Riko, Yakabe Aya, Baba Hiroshi, Toda Keisuke, Itoh Masahiro, Hirano Akiyoshi
Department of Plastic and Reconstructive Surgery, National Nagasaki Medical Center, Nagasaki, Japan.
Cleft Palate Craniofac J. 2006 Jan;43(1):112-6. doi: 10.1597/04-131r.1.
OBJECTIVE AND PATIENT: Both nasal cerebral heterotopia and encephalocele are rare congenital benign masses of neurogenic origin caused by an embryonic developmental abnormality. It is generally accepted that nasal heterotopia is a sequelae to encephalocele. This report presents an unusual case of nasal cerebral heterotopia and encephalocele arising in the same patient. The patient had a firm, solid mass measuring 1.5 x 1.0 cm on the bridge of the nose covered with normal skin and another mass in the nasal cavity obstructing the right nasal cavity. Computed tomography (CT) demonstrated that the nasal bone separated these masses. CT also showed a bony defect at the skull base. Surgery consisted of dividing the encephalocele and closure of the skull base fistulae, along with nasal subcutaneous mass enucleation.
Intraoperative examination indicated the existence of a pit on the nasal bone where the pedicle of the nasal subcutaneous mass connected. Microscopic examination of the nasal cavity mass demonstrated meningoencephalocele, and examination of the nasal subcutaneous mass demonstrated nasal cerebral heterotopia, which was confirmed by immunohistochemical staining. After 10 months, complete removal of the subcutaneous nasal mass was recognized and there was no evidence of recurrence.
Findings in this case suggest that the nasal cerebral heterotopias will result from encephalocele.
目的与患者：鼻脑异位和脑膨出均为罕见的先天性神经源性良性肿块，由胚胎发育异常引起。一般认为鼻异位是脑膨出的后遗症。本报告介绍了一例同一患者同时出现鼻脑异位和脑膨出的不寻常病例。患者鼻梁处有一个质地坚硬的实性肿块，大小为1.5×1.0厘米，表面覆盖正常皮肤，鼻腔内还有一个肿块阻塞右鼻腔。计算机断层扫描（CT）显示鼻骨将这些肿块分隔开。CT还显示颅底有骨缺损。手术包括分离脑膨出、封闭颅底瘘管以及摘除鼻腔皮下肿块。
术中检查发现鼻骨上有一个凹陷，鼻腔皮下肿块的蒂与之相连。鼻腔肿块的显微镜检查显示为脑膜脑膨出，鼻腔皮下肿块的检查显示为鼻脑异位，免疫组化染色证实了这一点。10个月后，鼻腔皮下肿块被完全切除，且无复发迹象。
该病例的发现表明鼻脑异位是由脑膨出导致的。
