Verney Y, Zanolla G, Teixeira R, Oliveira L C
Service of Paediatric Surgery, University of Santa Maria, Santa Maria, Brazil.
Eur J Pediatr Surg. 2001 Oct;11(5):324-7. doi: 10.1055/s-2001-18547.
Congenital midline nasal masses are rare anomalies that occur in about one in 20,000-40,000 live births. The most common are dermoid/epidermoid tumors, nasal cerebral heterotopias (nasal gliomas), and nasal encephaloceles; some have an actual or potential central nervous system connection. Nasal gliomas are CNS masses of neurogenic origin which have lost their intracranial connections and present as an obvious external or intranasal mass at birth without associated surgical symptoms. Careful evaluation is required to confirm the diagnosis and appropriate management. The interpretation of CT and MR images can be difficult but is useful in differentiating nasal gliomas from other congenital nasal masses. The presence of a fibrous stalk may be associated with cranial defects and CSF leak. Excisional biopsy allows histopathologic diagnosis and is the definitive treatment. They are benign lesions, and recurrences are rare, so conservative cosmetic surgical techniques should be chosen for gliomas where there is no proven intracranial extension. The authors report an illustrative nasal glioma case in a one-year-old male infant with extranasal and intranasal components, and discuss the therapeutic options.
先天性中线鼻部肿物是罕见的异常情况,约每20000 - 40000例活产儿中出现1例。最常见的是皮样/表皮样肿瘤、鼻脑异位(鼻胶质瘤)和鼻膨出;其中一些与中枢神经系统存在实际或潜在联系。鼻胶质瘤是起源于神经的中枢神经系统肿物,已失去其颅内连接,出生时表现为明显的外部或鼻内肿物,无相关手术症状。需要仔细评估以确诊并进行适当处理。CT和MR图像的解读可能有困难,但有助于鉴别鼻胶质瘤与其他先天性鼻部肿物。纤维蒂的存在可能与颅骨缺损和脑脊液漏有关。切除活检可进行组织病理学诊断,是确定性治疗方法。它们是良性病变,复发罕见,因此对于无颅内扩展证据的胶质瘤,应选择保守的美容手术技术。作者报告了1例1岁男婴的典型鼻胶质瘤病例,该病例有鼻外和鼻内成分,并讨论了治疗选择。