The approaches and outcomes of skull base surgery for pediatric sarcoma after initial therapy

Objective

Surgical removal of the residual pediatric sarcoma after initial therapy is common; however, that in the skull base area still presents a formidable challenge. We reviewed the approaches and outcomes of skull base surgery for pediatric sarcoma.

Methods

Thirteen patients with pediatric sarcoma who received skull base surgery were reviewed retrospectively. Tumor sites, surgical approach, complications, regional recurrence after surgery, and survival rate were analyzed.

Results

The residual tumor sites were the infratemporal fossa (8), nasal cavity and paranasal sinus (4) and orbit with anterior skull base invasion (1). Coronal skin incision (1), that with preauricular skin incision (7) and facial dismasking flap (5) were applied to patients as the skin preparation. Following skin preparations, anterior skull base surgery with frontal craniotomy (3), infratemporal fossa approach with temporal craniotomy (5), and anterior-lateral skull base with front-temporal craniotomy (1) were performed. Tumors were removed with a sufficient surgical field in all patients. Facial nerve palsy (9), facial deformity (4), orbital complications (diplopia, decreased visual acuity, narrowing of visual field) (2) and occlusal imbalance (1) occurred. However, facial palsy, diplopia and occlusal imbalance subsided gradually. Local recurrence occurred in 6 cases and distant metastasis was observed in 4 cases. The overall 5-years survival rate was 51.9% (Kaplan-Meier method).

Conclusion

These tumors were safely removed with minimum morbidity. Skull base surgery is recommended to remove residual pediatric sarcoma after the initial treatment.     

Association of skull base and facial fractures.

A retrospective analysis of 268 trauma patients with facial fractures who received computed tomography of the head was undertaken to assess an association with skull base fractures. The incidence of skull base fracture was compared to facial fractures of various anatomic locations. Skull base fractures were significantly increased in orbital wall/rim fractures (36.0%, P = .0823). In contrast, skull base fractures related to orbital floor (27.3%, P = .6191) and maxillary/zygomatic (29.4%, P = .1148) fractures were not significantly greater and were infrequently seen with mandible (4.0%, P = .0454) and nasal (7.7%, P = .0345) fractures. The incidence of skull base fracture was directly associated with the number of facial fractures per patient; one facial fracture (21.0%), two facial fractures (30.4%), and three or more facial fractures (33.3%) (P < .05). The incidence of skull base fractures was related to the location of facial fractures and the number of facial fractures per patient. The results provide additional clinical information to facilitate the prompt detection and diagnoses of skull base fracture.     

Management of combined frontonaso-orbital/skull base fractures and telecanthus in 355 cases.

In regard to the multiple problems of reconstruction concerning this fracture pattern, we developed various methods to achieve optimal results. As various vital regions, apart from the nasoethmoidal fractures and aesthetics, are involved, the proposed classification has direct implication for the surgical procedure. Even in intracranial fragment dislocations, cerebral contusion, and elevated intracranial pressure, the subcranial approach, in contrast with the transfrontal access, enables early definitive management of the skull base and the external facial frame in a one-stage procedure. Another method, the symmetrical centripetal compression of the canthal ligaments and naso-orbital bone fragments, enables correct reduction of the telecanthus. The significant reduction of morbidity and complication rate to a minimum confirms the efficiency of our treatment modalities.     

颅内外沟通性肿瘤的外科治疗

目的 探讨颅内外沟通性肿瘤的外科治疗。方法 统计分析我院自2001年1月至2005年5月治疗的32例颅内外沟通性肿瘤,其中恶性肿瘤22例,良性肿瘤10例,20例进行颅面联合进路肿瘤切除;10例在鼻内镜辅助下开颅手术切除;2例行颅外进路肿瘤切除,对颅底缺损的修复用帽状腱膜-颅骨骨膜瓣、颞肌-肌筋瓣、颅骨骨瓣、前臂皮瓣等。结果 22例恶性肿瘤中随访2年以上6例,1～2年8例,1年以下7例,1例死亡,1例脑转移带瘤生存,其余均健在。10例良性肿瘤无复发。结论 对颅内外沟通性肿瘤,应根据肿瘤的位置、大小及颅内累及的情况,选择合适的手术人路,在保证肿瘤切除彻底的前提下,尽可能的减小损伤,对部分颅内外沟通性肿瘤,鼻内镜辅助下手术,可减小创伤,同时面部无疤痕。     

侵犯颅底的头颈部肿瘤的手术治疗

目的：探讨侵犯颅底的头颈部肿瘤的手术治疗方法。方法：对32例侵犯前、侧颅底的头颈部肿瘤患者进行手术治疗,采用鼻内镜下鼻腔入路5例,颅-面联合入路3例,上颌骨切除入路15例,口腔硬腭入路1例,经下颌入路1例,颈侧-下颌骨切开入路2例,耳后-颈联合入路1例,额颞入路2例,经面入路2例。结果：4例鼻窦黏液囊肿仅作开放引流,1例脊索瘤作次全切除,其余27例患者均全切肿瘤,无手术死亡及严重颅脑并发症。8例良性肿瘤患者随访6个月~8年情况良好,无肿瘤复发及死亡。24例恶性肿瘤患者,术后随访3年以上19例,生存12例;随访5年以上12例,生存5例;术后3、5年生存率分别为63.2%（12/19）和41.7%（5/12）。结论：根据病变性质、部位及范围设计手术入路,力求全切肿瘤并保护重要结构,采用适当的颅底修复方法,可获得良好的治疗效果。     

Management of severe frontobasilar skull fractures

Immediate reconstruction of severely comminuted frontobasilar skull fractures involving the frontal sinus can be accomplished safely if the sinus is cranialized and the floor of the anterior cranial fossa is totally reconstructed. Operative time can be reduced if the pieces of the frontal bone are reassembled and stabilized with rigid fixation devices out of the surgical field and thus made ready for reinsertion as a single unit after any necessary neurosurgical procedures are completed. Immediate reconstruction provides postoperative protection for the brain and eliminates the need to return the patient to the operating room for cranioplasty at a later date.     

儿童侧颅底肿瘤的诊断和治疗

目的:探讨儿童侧颅底肿瘤的临床特点、诊断及治疗方法.方法:回顾性分析8例儿童侧颅底肿瘤的临床表现、影像学特点及治疗方法.其中颞部并中、后颅窝巨大三叉神经鞘膜瘤1例.颞部并中、后颅窝黑色素神经外胚层肿瘤1例,婴幼儿颞骨纤维瘤病1例,斜坡脊索瘤1例,鼻咽部胚胎型横纹肌肉瘤2例,神经母细胞瘤2例.1例经颞下窝Fisch C型及迷路上联合径路、1例经岩骨径路、4例经颞下窝Fisch C型径路将肿瘤全切除,1例经腭径路行肿瘤次全切,1例单纯行化疗未做手术.7例手术患儿中4例术前、术后化疗,3例术后化疗.结果:除1例脊索瘤患儿术后5个月复发死亡外,其余7例患儿均存活(6例手术、1例未手术),其中2例鼻咽部胚胎型横纹肌肉瘤术后小灶复发,1例神经母细胞瘤化疗后未行手术的患儿,6个月后复发.除术前1例外展神经、3例三叉神经受累及外,术后2例出现短暂脑脊液漏,1例同侧听力丧失.1例声嘶(同侧声带外展麻痹),2例吞咽困难,术后3～4个月渐恢复.7例手术患儿均无伤口感染裂开及皮瓣坏死,未出现面瘫、脑膜炎及偏瘫、死亡等严重并发症.结论:儿童侧颅底肿瘤生长部位深在、临床症状复杂多样、隐匿、不典型,往往确诊时已为晚期,积极行CT和MRI检查有助于提高早期诊断率.手术切除肿瘤仍为首选,手术前后配合放、化疗.手术径路的选择应依据病变的部位和范围,颞骨和颞下窝联合径路可以最大限度切除侵及该区域的肿瘤,保存脑神经功能,减少并发症.     

下颌外旋切除咽及颅底肿瘤

目的探讨咽及颅底肿瘤切除的最佳手术入路。方法１３例咽及颅底肿瘤中，鼻咽部２例，口咽部４例，咽旁间隙５例，咽旁颞下区２例。均采用下颌骨切开外旋入路进行了根治性切除。同期行咽后淋巴结清扫术５例，改良根治性颈清扫术２例。咽部缺损以胸大肌皮瓣整复５例。恶性肿瘤术后均接受了辅助性放射治疗。结果１３例患者中恶性肿瘤１０例，良性肿瘤３例。１２例切口愈合良好，１例术后术后胸大肌皮瓣感染坏死，延缓愈合。１例吞咽困难，经锻炼后恢复，１例下颌咬合稍差。随访１５月～３年，３例良性肿瘤情况良好。恶性肿瘤中１例术后半年死于局部复发，１例术后２年死于肺转移。其余患者３年存活２例，２年以上存活２例，１年以上存活４例。结论此入路能充分显露咽、颅底、咽旁间隙、斜坡及颈椎，并能沿颈内动脉向上至颅底，将颈动脉内侧组织与肿瘤整块切除。手术安全，后遗畸形轻微。     

Mucocele after transnasal endoscopic repair of traumatic anterior skull base fistula in children

Objectives

To report the long-term sinonasal complications after endoscopic repair of anterior skull base fractures in children. This study describes mucocele formation in 6 patients treated endoscopically for posttraumatic CSF fistulae. We aim to address possible etiologic factors, specific treatments and follow-up modalities.

Patients and methods

12 children, mean age 5.8 years (3-10), treated endoscopically at our institution between 2004 and 2010 for an anterior cranial base fracture complicated by a CSF fistula. An iatrogenic mucocele was observed in 6 cases. A retrospective review of the files of these 6 patients was carried out demonstrating demographic characteristics, presenting signs/symptoms, site of skull base defect, repair technique, timing and onset of the mucoceles, their presentation and management. A systematic CT-scan and/or MRI was carried out at 3 months, 1 year and then annually.

Results

3 patients presented after cranial trauma with persistent CSF nasal leak, and 4 with meningitis. Posttraumatic defects reached the posterior wall of the frontal sinus and the junction of ethmoid and frontal bone in 3 cases, and the cribriform plate in 3 cases. Endoscopic closure was performed in all cases, with the middle turbinate as an overlay patch. No recurrence of a CSF leak was observed. After a mean period of 16 months, a single iatrogenic mucocele was observed in 4 patients (radiological detection only), and multiple mucoceles in 2 patients. Surgical treatment was advocated in cases of proptosis, quick expansion of the mucocele leading to sinus bony wall remodelling or erosion (2 cases), and meningitis related to an erosion of the cribriform plate by the mucocele. Three mucoceles were successfully treated endoscopically, and 1 required an external approach.

Conclusion

Mucocele incidence after endoscopic repair of skull base fractures in children is not insignificant (50% in our 12 patients series). Paediatric anatomical features, cranial trauma, and the transethmoidal approach may play a role in mucocele pathogenesis. Long-term clinical and radiological follow-up is therefore recommended. These mucoceles may be managed endoscopically with good outcomes.     

Free tissue reconstruction of the anterior skull base: A review

ObjectiveThere has been a significant shift from open craniofacial resection of the anterior skull base to endoscopic approaches that accomplish the same outcomes in tumor ablation. However, when open resection is required, free flap reconstruction is often necessary to provide sufficient well-vascularized tissue for optimal wound healing as well as providing adequate tissue bulk for cosmesis. This articleaims to providea focused review of free flaps most commonly used in anterior skull base reconstruction.MethodsThis is a state-of-the-art review based on expert opinion and previously published reviews and journal articles, queried using PubMed and Google Scholar.Results & conclusionAnterior skull base reconstruction via free tissue transfer is imperative in limiting complications and promoting healing, particularly with large defects, post-radiation, and in at-risk patients. The type of free flap utilized for a particular anterior skull base reconstruction should be tailored to the patient and nature of the disease. This review offers insight into the numerous reconstructive options for the free flap surgeon.     

鼻内镜鼻窦手术前筛窦颅底高度的CT评估价值

目的 探讨内镜鼻窦手术前应用CT评估筛窦颅底高度的价值。 方法 选取就诊于我院的100例共200侧的鼻窦冠状CT扫描图像,测量筛前动脉水平的筛顶中点至眶水平中线的距离,根据测量数据进行分型,同时进行Keros分型,用皮尔森相关系数来确定两者之间是否存在相关性。 结果 筛前动脉管水平的筛顶中点至眶水平中线的距离作为筛窦颅底高度。根据筛窦颅底的高度进行分型,其中Ⅰ型即筛窦颅底高度>7 mm(高位颅底)占44%(88/200),Ⅱ型即筛窦颅底高度介于4~7 mm(中位颅底)占41%(82/200),Ⅲ型即筛窦颅底高度<4 mm(低位颅底)占15%(30/200)。按Keros方法进行测量并分型,Ⅰ型占37%(74/200),Ⅱ型占52%(104/200),Ⅲ型占11%(22/200);Keros分型与筛窦颅底高度之间的皮尔森相关系数为0.384(P<0.001),Keros分型与筛窦颅底高度呈弱相关性。 结论 筛窦颅底高度存在明显差异,术前进行CT影像评估,可有效识别低颅底变异,有助于避免颅底损伤,进而减少内镜手术并发症。     

头颈外科神经外科联合手术治疗颅底沟通肿瘤

目的 介绍头颈外科与神经外科合作处理颅底沟通肿瘤的经验和优势.方法 回顾性分析2005年7月至2008年7月头颈外科和神经外科联合制定手术方案并共同实施手术54例(良性21例,恶性33例)颅底沟通肿瘤的临床资料,其中前颅底19例,侧颅底12例,中央颅底17例,后颅底(颈静脉孔区)6例.结果 本组颅底沟通肿瘤均一期手术切除.良性肿瘤全切除20例,近全切除1例;恶性肿瘤肉眼全切除25例,6例鼻窦、鼻咽、腮腺恶性肿瘤及2例脊索瘤近全切除.无手术死亡病例,恶性肿瘤并发症13例,其中术后出血2例.随访8～43个月,良性与恶性肿瘤中位随访时间分别为19.1及21.0个月,恶性患者失访3例.21例良性肿瘤复发1例.33例恶性肿瘤中复发12例,死亡9例(其中1例死于心脏病),Kaplan-Meier法统计3年生存率与无瘤生存率分别为53.0%及52.7%.结论 头颈外科和神经外科合作有利于提高颅底沟通肿瘤的手术切除率从而提高治疗效果.     

颅底及颈部脊索瘤11例临床分析

目的：探讨发生于耳鼻咽喉科手术部位的颅底及颈部脊索瘤的临床表现,组织学类型,影像学特点,手术方法及远期疗效。方法：回顾性分析11例颅底及颈部脊索瘤患者的临床资料,并介绍1例颈部巨大脊索瘤。11例均行手术治疗。结果：发生于颅底及颈部的脊索瘤临床表现复杂,首发症状以鼻塞、颈部包块、视力下降、耳鸣、耳聋及脑神经受损症状为主。组织学类型：典型脊索瘤8例,软骨性脊索瘤3例。影像学表现：8例行CT检查者表现为软组织肿块影,以膨胀性生长为主,颈椎或颅底多有骨质破坏,肿块与周围软组织边界清楚。2年复发率为36．4％,5年生存率为72．7％。结论：本病临床表现复杂,对颅底及颈椎以外的周围组织以膨胀性压迫为主,对颅底及颈椎骨质以侵蚀性破坏为主;手术治疗效果良好,应根据病变部位及肿瘤大小选择手术径路。复发者仍可再手术,彻底手术可减少复发率。     

Vagus nerve paralysis due to skull base fracture

Lower cranial nerve paralysis originating from skull base fracture is rare and isolated vagus nerve paralysis after close head injury is extremely rare. We, in this study, present the case of a 49-year-old man sustained a right vocal cord paralysis and dysphagia in a fighting accident. Initial examination, including flexible laryngoscope, esophagogram and fine-cut neck computed tomography scan, failed to find out the cause of isolated vagus paralysis. Until high resolution computed tomography of skull base revealed the jugular foramen bony disruption. One individual cranial nerve paralysis may be a significant sign for skull base fracture in closed head injury. Considering the serious consequences of the disease, thorough neurologic and radiologic examination are needed to evaluate the condition of skull base, and to develop reliable effective management for the neurologic sequalae, result from it.     

儿童颅底肿瘤经鼻内镜外科手术治疗分析

摘要：目的探讨分析鼻内镜手术在治疗儿童颅底肿瘤中的应用。 方法回顾性分析中南大学湘雅医院鼻颅底外科 2010年6月—2019年11月收治确诊为颅底肿瘤并行鼻内镜治疗的患儿共44例,其中鼻咽纤维血管瘤17例,朗格汉斯组织细胞增生症7例,骨化纤维瘤5例,原始神经外胚层肿瘤2例,横纹肌肉瘤2例,脑膜瘤2例,间叶性软骨错构瘤1例,颅咽管瘤1例,表皮样囊肿1例,血管纤维脂肪瘤1例,生殖细胞瘤1例,骨母细胞瘤1例,脊索瘤1例,侵袭性垂体腺瘤1例,纤维瘤病1例。 结果44例患儿中43例在鼻内镜下完全切除,仅1例鼻咽纤维血管瘤分期切除。术后44例均进行定期随访,随访时间3个月至9年。5例失访,其中骨母细胞瘤1例,脑膜瘤1例,横纹肌肉瘤1例,鼻咽纤维血管瘤2例;12例术后接受放化疗,其中朗格汉斯组织细胞增生症7例,原始神经外胚层肿瘤 2例,横纹肌肉瘤1例,脊索瘤1例,生殖细胞瘤1例,随访至今患儿状况良好,均无复发及转移;1例鼻咽纤维血管瘤复发;再次手术后治愈;其余26例患儿术后未见复发。结论鼻内镜颅底手术治疗儿童颅底肿瘤是可行、有效及安全的。