Pediatric sinonasal and skull base lesions

Pediatric skull base lesions are complex and challenging disorders. Safe and comprehensive management of this diverse group of disorders requires the expertise of an experienced multidisciplinary skull base team. Adult endoscopic skull base surgery has evolved due to technologic and surgical advancements, multidisciplinary team approaches, and continued innovation. Similar principles continue to advance the care delivered to the pediatric population. The approach and management of these lesions varies considerably based on tumor anatomy, pathology, and surgical goals. An understanding of the nuances of skull base reconstruction unique to the pediatric population is critical for successful outcomes.     

Extraorbital skull base idiopathic pseudotumor

OBJECTIVES/HYPOTHESIS: The term idiopathic pseudotumor (IP) refers to a nonspecific, nonneoplastic inflammatory process without identifiable local or systemic causes, which is one of the most common causes of intraorbital space-occupying lesions. Occasionally, orbital pseudotumors may extend to other areas of the skull base. Rarely, pseudotumors may present as a skull base mass with no involvement of the orbit. The ophthalmology literature has detailed reviews of IP as an intraorbital space-occupying lesion, but lesions involving only the skull base and sparing the orbit are rare in the literature. We present a review of our experience with six patients with extraorbital skull base pseudotumor and a review of the relevant literature. METHOD: Retrospective case study. RESULTS: We treated six patients with extraorbital skull base pseudotumor between 1996 and 2004. Four patients had lesions in the pterygopalatine and/or infratemporal fossae, and two patients had lesions in the superior aspect of the parapharyngeal space. Five patients had excellent initial response to steroids. Two of the pseudotumors recurred. One patient partially responded to steroids and is currently stable with limited disease. Our literature review demonstrated 16 previously published reports of IP of the skull base sparing the orbit. CONCLUSIONS: A well-documented intraorbital lesion, inflammatory pseudotumor may present outside the orbit. Treatment of IP of the skull base is controversial and may involve corticosteroids or surgical resection, or both. Other chemotherapeutic agents and radiotherapy may be considered in steroid-resistant patients. Pathological subtype, ease and safety of resection, safety of high-dose corticosteroid use, the surgeon's comfort, and the patient's preference must be included in the decision-making process for treatment.     

内镜下经鼻确诊眼眶颅底炎性假瘤一例

本文报道了1例中南大学湘雅医院耳鼻咽喉头颈外科收治的眼眶颅底炎性假瘤患者。患者中年男性,以“左侧眼眶疼痛2个月,左眼视力下降1个月”入院,入院诊断为眼眶颅底肿瘤（左）。行鼻内镜下颅底病变活检术,临床表现结合影像学及病理学结果,考虑诊断为眼眶颅底炎性假瘤（左）。术后予以泼尼松口服,密切随访1年无复发。     

Endoscopic skull base surgery for sinonasal malignancy

Management of malignant neoplasms of the sinonasal tract and skull base is hampered by the relative low incidence and pathologic diversity of patient presentations. Many studies have reported successful outcomes in the endoscopic management of malignancy since 1996, and these are summarized in this article. Nonsurgical adjuvant therapies are important for locoregional control because surgery occurs in a restricted anatomic space with close margins to critical structures, and distant disease is an ongoing concern in these disorders. There remains a need for collaborative consistent multicenter reporting, and international registries have been established to assist in such efforts.     

颅底炎性假瘤的诊断和治疗——附2例报道

目的探讨颅底炎性假瘤的诊断和治疗方法。方法报道2004年和2006年在我科诊治的2例颅底炎性假瘤,并结合文献对该病的临床表现、病理学特点、影像学特征、治疗方法及疗效进行总结分析。结果颅底炎性假瘤侵袭性的临床表现易误诊为恶性肿瘤,在病理学上无特异性的表现,在影像学上表现为T2加权低信号,治疗效果不佳,容易复发。结论T2加权低信号是颅底炎性假瘤的影像学特征,颅底炎性假瘤的治疗应根据自身特点,进一步优化治疗方案。     

IgG4-related disease presenting as otogenic skull base osteomyelitis

IgG4-related disease (IgG4-RD) is an emerging clinical disease entity characterized by tumefactive lesions at multiple sites with a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. Although almost any organ can be affected, IgG4-RD is most likely to involve the submandibular, lacrimal, or parotid glands in the head and neck region. However, skull base involvement presenting as otogenic skull base osteomyelitis (SBO) is rare. We encountered a 70-year-old male with IgG4-RD presenting primarily with severe otalgia and otorrhea. He had uncontrolled diabetes mellitus and showed clinical manifestations of otogenic SBO. Tissue immunostaining revealed typical features of increased IgG4-positive plasma cells, and hematological examination showed elevated serum IgG4 concentrations. Treatment with corticosteroids significantly improved well-being and partially resolved the lesion based on computed tomography (CT) scan.     

Minimally invasive endoscopic resection of sinonasal malignancies and skull base surgery

CONCLUSIONS: Sinonasal malignancies without far lateral extension, intracranial invasion or orbital infiltration can be safely excised and reconstructed endoscopically with a satisfactory outcome. OBJECTIVES: To evaluate the safety and efficacy of strictly endoscopic resection of malignant sinonasal neoplasms. PATIENTS AND METHODS: Seven patients with sinonasal malignancies had received a minimally invasive endoscopic resection. Among them, two cases invaded the skull base. All patients were operated with curative intent. RESULTS: All the tumors were removed successfully by strictly endoscopic surgery. Two patients who had invasion of the skull base (28.6%) accepted postoperative radiotherapy. No complications associated with the operation occurred. The patients were followed up for 15-57 months (average 36.4 months). No local recurrence was noted in this series to date. The disease-free survival rate was 100% at a mean follow-up duration of 36.4 months.     

手术治疗鼻窦-颅底区域良性纤维骨性病变

目的:探讨采用经鼻内镜和开放式手术入路治疗鼻窦-颅底区域纤维骨性病变的方法、注意事项及临床疗效。方法:15例鼻窦-颅底区域纤维骨性病变,其中骨瘤6例,骨化纤维瘤2例,骨纤维异常增殖7例。回顾3种病变的病理及CT影像特点,根据病变的位置及范围选择内镜或开放手术入路治疗。采用鼻内镜手术11例,其中单鼻入路7例,双鼻扩大入路4例;开放式手术4例,其中单纯眉弓入路1例,双冠状入路3例。结果:术后随访2个月～4年,病变完全切除10例,无复发;部分切除5例,都是骨纤维异常增殖症。全部患者临床症状及面部畸形消失或较术前明显改善。内镜手术中发生脑脊液鼻漏1例,Ⅰ期修复成功。术前复视3例,2例半年后消失,1例减轻。全部患者无眶内及颅内并发症。结论:手术是治疗临床症状明显的良性鼻窦-颅底区域纤维骨性病变的有效手段。病变位置和范围是决定采用开放式手术还是经鼻内镜入路的决定因素。内镜手术可切除由额窦后壁到斜坡的中线颅底广泛区域内的病变,而开放式手术入路可以处理眶内壁以外前颅底的病变。无论采用何种入路,骨瘤、骨化纤维瘤可作到完全切除。骨纤维异常增殖由于病变广泛且有自限性,故手术以减轻临床症状和改善面部畸形为目的,多行部分切除。即使骨纤...     

带蒂鼻中隔黏膜瓣在侵及前颅底鼻腔鼻窦恶性肿瘤切除术颅底重建中的应用

目的　回顾性分析应用带蒂鼻中隔黏膜瓣修复内镜下切除侵及颅底鼻腔鼻窦恶性肿瘤术后颅底缺损的效果。方法　2008年9月～2016年5月内镜下切除侵及颅底鼻腔鼻窦恶性肿瘤31例,应用以鼻后中隔动脉和筛前-筛后动脉为供血的两种类型带蒂鼻中隔黏膜瓣,修复重建前颅底切除后较大颅底缺损。结果　31例患者前颅底重建均一次性修补成功。1例肿瘤复发二次手术患者术后发生脑脊液漏,给予椎管置管引流1周愈合;1例术后10 d撤出鼻腔填塞物后出现脑脊液鼻漏,颅内感染3例,余无颅内出血或血肿等并发症发生。术后随访3～66个月见黏膜瓣愈合良好,无移植瓣膜坏死和脑膜脑膨出发生。结论　血管化带蒂鼻中隔黏膜瓣是内镜颅底外科的一种首选的、可靠的前颅底修补用材料。     

影像导航技术在鼻窦-颅底内镜手术中的应用

随着影响导航技术的发展和不断应用,鼻内镜下鼻窦、鼻颅底和鼻眼相关等手术取得了进一步发展,鼻内镜手术操作更加精准,安全性更高。本文从影像导航技术的工作原理及其在鼻窦-颅底外科领域的应用历史、适用范围和应用态度进行了系统论述,并对影像导航技术在鼻窦-颅底外科领域的临床应用进展和未来发展方向进行了说明。     

Endoscopically assisted anterior cranial skull base resection of sinonasal tumors.

The traditional approach to sinonasal tumors involving the base of skull has been the anterior craniofacial resection. Endoscopic techniques have created the potential to approach the intranasal aspect of skull base lesions without external incisions and still develop an en bloc resection when removed. We report our initial experience with skull base neoplasms in which the otolaryngic portion of the standard resection was accomplished instead through an endoscopic approach. The nature of lesions favorable for this approach and associated technical issues are discussed. Although we do not consider this approach a replacement for the traditional anterior craniofacial resection, it is an important adjunct in the skull base surgeon's armamentarium.     

IgG4-related disease with pseudotumor formation in the larynx

Mikulicz’s disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery with general anesthesia to improve respiratory symptoms and a resected specimen was submitted for diagnosis. The pathology findings revealed lymph follicle formation, interstitial follicular fibrosis, angiogenesis, and inflammatory cell infiltration with plasmacytosis. Immunohistologic staining at high magnification revealed one hundred or more IgG4-positive plasma cells and fifty percent IgG4/IgG. In addition, obliterative phlebitis was observed. Medical history was positive for retroperitoneal fibrosis with serum IgG4 levels below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The diagnosis of IgG4-RD was established, oral treatment with prednisolone (0.6 mg/kg/day) was initiated, and the tumor reduced in size. IgG4-RD may cause irreversible functional impairment. Early diagnosis and treatment are important in reducing the risk of permanent impairment of vocal fold mobility.     

内镜经鼻入路切除前颅底恶性肿瘤的疗效观察

目的分析内镜经鼻入路切除前颅底恶性肿瘤的可行性,并分析其预后及影响因素。方法回顾性分析2015年6月至2019年4月间在复旦大学附属眼耳鼻喉科医院就诊的42例前颅底恶性肿瘤患者的临床资料,其中男性31例,女性11例;年龄20~78岁,平均49岁。42例患者中包括鼻腔鼻窦鳞状细胞癌(鳞癌)15例(14例T4bN0M0,1例T4bN1M0)、嗅神经母细胞瘤27例(均为Kadish C期)。前颅底切除后采用大腿阔筋膜和带蒂鼻中隔黏膜瓣进行重建。患者术后第1天行头颅CT平扫排除蛛网膜下腔出血、严重颅内积气和脑水肿,同时行鼻窦增强MR检查判定肿瘤切除情况。使用Kaplan-Meier法统计分析总体生存率,Cox回归分析影响预后的因素。结果 42例患者的平均手术时间452 min。术后增强MR明确显示:36例(85.7%)肿瘤全切;2例(4.8%)因侵犯眶内容物且患者强烈要求保留眼球行次全切;1例(2.4%)患者因术中发生颈内动脉损伤而终止手术;1例(2.4%)患者术后MR显示肿瘤残留再次手术切除;2例(4.8%)患者可疑眼眶内侧残留。术后随访2~52个月,平均20个月,中位随访时间17个月;失访2例。术后1、2、3年总体生存率分别为86.5%、76.9%、64.5%;鼻腔鼻窦鳞癌术后1、2、3年的总体生存率分别为86.2%、86.2%、57.4%;嗅神经母细胞瘤1、2、3年的总体生存率分别为86.9%、75.3%、67.8%。多因素分析表明:肿瘤残留(P=0.001)和复发(P<0.01)是影响患者生存的独立危险因素。结论内镜经鼻入路切除前颅底恶性肿瘤可行,预后较好。肿瘤残留和复发是影响患者预后的因素。