juvenile nasopharyngeal angiofibroma

青少年鼻咽血管纤维瘤
  • 文章类型: Case Reports
    一种罕见的局部侵袭性血管肿瘤,青少年鼻咽血管纤维瘤(JNA)主要影响男性青少年。本文描述了一名14岁的男性患者,他表现为嗜睡和复发性鼻出血,这是JNA的症状。CT和MRI扫描证实血管肿块具有明显的局部侵袭,起源于蝶腭孔。CT血管造影后,这揭示了肿瘤的大量血液供应,并有助于有效切除,设计了一个有针对性的手术策略。组织病理学证实了肿瘤的良性性质,手术成功,患者顺利康复。这个案例增加了关于JNA的小文献。它强调了医疗保健专业人员在管理疾病时需要了解早期识别和仔细的术前准备的要求。
    A rare and locally aggressive vascular tumor, juvenile nasopharyngeal angiofibroma (JNA) mostly affects male teenagers. This paper describes a 14-year-old male patient who presented with lethargy and recurrent nasal bleeding, which are symptoms of JNA. CT and MRI scans confirmed a vascular mass with a significant local invasion originating from the sphenopalatine foramen. After a CT angiography, which revealed the tumor\'s large blood supply and helped with efficient excision, a focused surgical strategy was designed. Histopathology verified the benign nature of the tumor, and the operation was successful and the patient had a smooth recovery. This case adds to the little literature on JNA. It highlights the need for healthcare professionals to be aware of the requirement of early identification and careful presurgical preparation in managing the illness.
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  • 文章类型: Journal Article
    背景手术切除是青少年鼻咽血管纤维瘤(JNA)的主要治疗方法,但由于其高血管分布和局部侵略性,该程序具有挑战性。此外,术前栓塞是一个争论的话题。目的本研究的目的是评估疗效,安全,内窥镜辅助切除作为非栓塞性晚期JNA手术干预的可行性。材料与方法本病例系列涉及6名男性JNA患者(平均年龄:16岁),根据Radkowski分类分为Ⅱc至Ⅲb阶段。术前无栓塞。结果2例Ⅱc期患者均行内镜下全鼻孔切除术。1例Ⅲa期患者和1例Ⅲb期患者经内窥镜辅助唇下入路手术。两个病人,一个带有Ⅱc阶段的JNA,另一个带有Ⅲb,进行了两个阶段的手术.术后CT扫描显示六个月时没有残留疾病。平均而言,每个手术需要1.5个单位的输血.一名患者出现术中出血,而其余患者没有任何重大并发症.每个程序的平均手术持续时间为175分钟。每次手术平均住院时间为3.75天。结论内镜辅助或单纯内镜入路可安全有效地用于非栓塞性晚期JNA的完整切除。
    Background Surgical excision is the primary treatment for juvenile nasopharyngeal angiofibroma (JNA), but this procedure is challenging due to its high vascularity and local aggressiveness. Moreover, preoperative embolization is a subject of debate. Objective The objective of this study is to assess the efficacy, safety, and feasibility of endoscope-assisted excision as a surgical intervention for non-embolized advanced JNA. Materials and methods This case series involved six male patients (mean age: 16 years) with JNA, classified as stages Ⅱc to Ⅲb according to the Radkowski classification. None underwent preoperative embolization. Results Two stage Ⅱc cases underwent total endoscopic endonasal excision. One patient with stage Ⅲa and another with stage Ⅲb underwent surgery via an endoscope-assisted sublabial approach. Two patients, one with stage Ⅱc JNA and another with Ⅲb, underwent a two-stage procedure. Postoperative CT scans showed no residual disease at the six-month mark. On average, each procedure required 1.5 units of blood transfusion. One patient experienced intraoperative bleeding, whereas the remaining patients were free of any major complications. The mean operation duration was 175 minutes per procedure. The mean length of stay at the hospital was 3.75 days per procedure. Conclusion Endoscope-assisted or purely endoscopic approaches can be safely and effectively employed for the complete excision of non-embolized advanced JNAs.
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  • 文章类型: Journal Article
    切除青少年鼻咽血管纤维瘤(JNA)的手术可能会导致大量危及生命的出血。麻醉管理旨在维持血流动力学稳定并减少失血。本案例系列描述了捆绑方法作为多模态失血预防束(MBLPB)的应用。20例患者接受了23例MBLPB手术。记录失血量和输血单位数。评估外科医生满意度评分。估计失血量的中位数[四分位距(IQR)]为1300(650-2350)ml。与肿瘤等级较高的患者相比,I期和II期肿瘤患者的中位(IQR)失血量为550(270-750)ml(III期,IV),中位(IQR)失血量为2100(1300-2500)ml。输注的堆积红细胞的中值(IQR)单位为1(0-3)。当MBLPB应用于JNA时,外科医生的满意度得分较高。然而,它似乎并没有显着减少失血。
    Surgery for excision of juvenile nasopharyngeal angiofibroma (JNA) carries the possibility of massive life-threatening haemorrhage. Anaesthetic management aims to maintain haemodynamic stability and reduce blood loss. This case series describes the application of the bundled approach as a multimodal blood loss prevention bundle (MBLPB). Twenty patients underwent 23 surgeries with MBLPB. The blood loss and the number of units of blood transfused were recorded. The surgeon satisfaction score was assessed. The median [interquartile range (IQR)] estimated blood loss was 1300 (650-2350) ml. Patients with tumours in stages I and II had a median (IQR) blood loss of 550 (270-750) ml compared to patients with higher grades of tumours (stages III, IV) with a median (IQR) blood loss of 2100 (1300-2500) ml. Median (IQR) units of packed red cells transfused was 1 (0-3). The surgeon\'s satisfaction score was high when MBLPB was applied for JNA. However, it does not appear to reduce blood loss markedly.
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  • 文章类型: Systematic Review
    背景:青少年鼻咽血管纤维瘤(JNA)是鼻咽的纤维血管肿瘤,典型地出现在青少年男性中。报告的平均发病年龄在13至22岁之间[1-6]。假设显着的雄激素刺激可以解释JNA在年轻的青少年男性中存在的强烈倾向。然而,诊断时存在相当大的年龄差异,很少有非常年轻的患者参与与典型的男性青春期生长模式不一致。
    目的:本系统综述的目的是确定早发性JNA(EOJNA)的病例,(定义为年龄<10岁),并检查该患者组的疾病特征和治疗方法。还描述并介绍了在我们机构中患有EOJNA的7岁男孩的病例。
    方法:我们搜索了Embase,Cochrane数据库和MEDLINE从1996年到2021年2月,用于报道EOJNA病例的研究。相关临床人口统计数据,记录疾病严重程度和治疗结果,并使用描述性统计进行分析.我们将我们的发现与所有年龄段的JNA报告的平均值进行了比较。
    结果:我们确定了29项包含34例EOJNA的研究。绝大多数(31/34)患者为男性,诊断平均年龄为8.15岁。最常见的症状是鼻塞(65.2%)和鼻出血(60.9%)。患者最常见的是RadkowskiII期(39.4%)和III期(39.4%)。主要治疗方式包括开放手术(66.7%),内镜手术(24.2%),和放疗(9.1%)。30%的复发明显。在EOJNA组中,Radkowski分期和治疗类型没有显着差异(分别为p=0.440和p=0.659)。
    结论:本系统综述提示EOJNA的罕见病例具有明显的疾病特征。与报告的平均值相比,该队列中的患者似乎患有更晚期的疾病和更高的复发率。我们希望这篇综述能提高临床对这种潜在更具侵袭性的JNA亚型的认识。随着EOJNA病例的报道越来越多,对该队列进行更有效的统计分析是可行的.
    BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns.
    OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented.
    METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages.
    RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively).
    CONCLUSIONS: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.
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  • 文章类型: Case Reports
    声门下狭窄(SGS),上气管变窄,可能是儿科患者的获得性疾病。表现为不同程度的呼吸困难和喘鸣,获得SGS最常见的原因是插管。气道狭窄通常不被认为是手术并发症。没有关于内窥镜鼻窦手术后获得SGS的文献。我们介绍了一个13岁男性青少年鼻咽血管纤维瘤(JNA)的独特病例,他在鼻内切除肿块后六周出现进行性呼吸困难的情况下出现SGS。他需要在术前栓塞和鼻内手术前紧急插管,延长了他的插管时间.在发现患者获得SGS之后,他最终需要连续扩张来治疗他的狭窄。该患者的表现和手术过程,连同图像和病理结果,正在讨论。基于对PubMed的广泛文献回顾,Medline,和谷歌学者,没有病例讨论经鼻手术后插管后SGS的发展或与JNA相关.获得性SGS可在儿科患者中表现为危及生命的气道阻塞。随着内镜颅底手术的兴起和JNA的普及,本案例研究揭示了SGS术后的检测和管理。
    Subglottic stenosis (SGS), the narrowing of the upper trachea, can be an acquired condition in pediatric patients. Presenting with varying degrees of dyspnea and stridor, acquired SGS is most commonly due to intubation. Airway stenosis is often not considered a surgical complication, and no literature on acquired SGS after endoscopic sinus surgery exists. We present a unique case of a 13-year-old male with juvenile nasopharyngeal angiofibroma (JNA), who developed SGS in the setting of progressive dyspnea six weeks after endonasal resection of his mass. He required urgent intubation prior to preoperative embolization and endonasal surgery, which prolonged his total intubation period. After the patient was found to have acquired SGS, he eventually required serial dilation to treat his stenosis. The presentation and operative course of this patient, along with images and pathologic findings, are discussed. Based on an extensive literature review of PubMed, Medline, and Google Scholar, there have been no cases discussing SGS development post-intubation after endonasal surgery or in association with JNA. Acquired SGS can present as a life-threatening airway obstruction in pediatric patients. With the rise of endoscopic skull base surgery and the prevalence of JNA, this case study sheds light on the detection and management of SGS post-operatively.
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  • 文章类型: Case Reports
    一名54岁的女性出现在耳鼻喉科(ENT)门诊部,有八个月的单侧鼻塞和头痛病史。嗅觉没有变化,鼻漏,面部疼痛,或相关的鼻出血。在检查中,有一个很大的,右侧鼻腔上部红斑肿块,填充鼻中隔之间的空间,中间,和上耳道。其余ENT检查正常。生命体征均在正常范围内。没有明显的既往病史,她尝试了类固醇鼻腔喷雾剂,没有任何好处。她从手术干预中完全缓解了症状,通过组织病理学证实肿块为血管纤维瘤。该病例报告讨论了将鼻咽血管纤维瘤作为单侧鼻腔肿块患者的鉴别诊断的重要性。包括女性患者,不管年龄。
    A 54-year-old female presented to the otolaryngology (ENT) outpatient department with an eight-month history of unilateral nasal obstruction and headache. There was no change in the sense of smell, rhinorrhoea, facial pain, or associated epistaxis. On examination, there was a large, erythematous mass in the superior aspect of the right nasal cavity, filling the space between the nasal septum, middle, and superior meatus. The rest of the ENT examination was normal. Vital signs were all within the normal range. There was no significant past medical history, and she had tried steroid nasal spray without any benefit. She had a complete resolution of symptoms from surgical intervention, and the mass was confirmed to be an angiofibroma through histopathology. This case report discusses the importance of considering nasopharyngeal angiofibroma as a differential diagnosis for patients presenting with unilateral nasal masses, including female patients, regardless of age.
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  • 文章类型: English Abstract
    Objective:To investigate the surgical approach for the resection of juvenile nasopharyngeal angiofibroma(JNA) under nasal endoscopy. Methods:The clinical data of 87 patients undergoing endoscopic resection of nasopharyngeal fibroangioma were retrospectively analyzed. We classified JNA according to tumor site, size, invasion scope and anatomic position relationship between tumor and midline of pupil. Three endoscopic surgical approaches were selected according to the classification, and the postoperative symptoms, complications and recurrence were investigated and analyzed. Results:The tumor resection rate of 87 cases by nasal endoscopic surgery was 100%. Thirty-five cases were approached through the middle nasal passage(small tumors located in the nasal sinuses and pterygopalatine fossa), forty-five cases were approached through the lateral wall of the nasal cavity(tumor invaded the pterygopalatine fossa but did not exceed the midline of the pupil) , and seven cases were approached via the lateral wall of nasal cavity + ipsilateral anterior wall of maxillary sinus(tumor invaded the infratemporal fossa beyond the midline of pupil or invaded the cavernous sinus and the middle cranial fossa epidural), Postoperative patients with nasal congestion, nasal bleeding, headache, dizziness, vision loss and other symptoms showed varying degrees of improvement. No surgical death or intracranial infection occurred. The postoperative follow-up was 6-78 months, and the recurrence rate was 3.44%. Conclusion:Endoscopic resection of nasopharyngeal fibroangioma is the main treatment method for JNA. Selecting suitable endoscopic approach to resect JNA, To maximize the advantage of nasal endoscopic equipment according to the inherent anatomical space of the human nasal cavity, In order to achieve the purpose of JNA resection, reduce intraoperative and postoperative complications, reduce the recurrence rate and improve the prognosis.
    目的:探讨经鼻内镜手术入路切除鼻咽纤维血管瘤(juvenile nasopharyngeal angiofibroma,JNA)的手术经验及效果。 方法:回顾性分析87例行鼻内镜下切除JNA的患者的临床资料。根据肿瘤的部位、大小、侵犯的范围及肿瘤与瞳孔中线的解剖位置关系对JNA进行分类,根据分类选择了3种内镜手术入路方式,并对术后症状、并发症和复发情况进行调查分析。 结果:87例经鼻内镜手术肿瘤切除率100%,经中鼻道入路35例(肿瘤较小,位于鼻腔鼻窦及翼腭窝),经鼻腔外侧壁入路45例(肿瘤侵犯翼腭窝但未超过瞳孔中线),经鼻腔外侧壁+同侧上颌窦前壁入路7例(肿瘤超过瞳孔中线侵及颞下窝或侵犯海绵窦、中颅窝硬脑膜外)。术后患者鼻塞、鼻腔间断流鼻血、头痛头晕、视力下降等症状得到不同程度改善。无手术死亡、颅内感染病例。术后随访6~78个月,复发率3.44%。 结论:经鼻内镜切除JNA手术是治疗JNA的主要手段,术前是否选择行肿瘤营养血管栓塞术可根据肿瘤的位置和分期以及术者的临床操作水平综合考虑;选择合适的鼻内镜入路切除JNA,能够根据人体鼻腔固有解剖空间最大限度的利用鼻内镜设备的优势,以达到切除JNA、减少术中术后并发症、减小复发率、提高预后的目的。.
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  • 文章类型: Case Reports
    幼年性鼻咽血管纤维瘤(JNA)是鼻咽部的组织病理学良性和高度血管性肿瘤。手术是这类肿瘤的首选治疗方法,但其高度血管性质可能导致大量和无法控制的出血。术前经导管动脉栓塞术为减少术中失血提供了一种有效且微创的方式。我们报告了2例JNA患者,分别在17岁男性和14岁男性中进行了术前使用明胶海绵浆液和聚乙烯醇栓塞颈外动脉分支。即使没有颈内动脉分支栓塞,我们报道的两个病例的术中失血量分别为1100mL和1300mL,低于之前报道的颈内动脉和颈外动脉分支栓塞术患者的平均失血量1428mL.
    Juvenile nasopharyngeal angiofibroma (JNA) is a histopathologically benign and highly vascular neoplasm of the nasopharynx. Surgery is the treatment of choice for this type of tumor, but its highly vascular nature could result in profuse and uncontrollable bleeding. Preoperative transcatheter arterial embolization offers an effective and minimally invasive modality for reducing intraoperative blood loss. We report 2 cases of JNA in a 17-year-old male and 14-year-old male who underwent preoperative embolization of external carotid artery branch using gelatin sponge slury and polyvinyl alcohol. Even without internal carotid artery branch embolization, both of our reported cases had intraoperative blood loss of 1100 mL and 1300 mL which are less than the previously reported mean blood loss of 1428 mL in patients who underwent both internal and external carotid artery branch embolization.
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  • 文章类型: Case Reports
    幼年性鼻咽血管纤维瘤(JNA)是一种罕见的良性头颈部肿瘤。我们报道了一个罕见的JNA病例,提供一个简短的文献综述,和治疗选择,并强调了氟他胺作为肿瘤消退术前药物的作用。JNA主要影响14至25岁的青少年男性。有各种理论解释肿瘤的形成。然而,发现性激素在肿瘤的病因中起着至关重要的作用。近年来,已在肿瘤上鉴定出睾丸激素和二氢睾丸激素受体,因此表明激素的强烈影响。这允许使用氟他胺,雄激素受体阻滞剂,作为JNA的辅助治疗。这是一个12岁的男孩,他因右侧鼻塞而出现在医院,鼻出血,流鼻涕,右鼻腔有肿块两个月.诊断鼻内窥镜检查,超声检查,计算机断层扫描,和磁共振成像。这些调查证实了JNAIV期的诊断。患者开始使用氟他胺治疗肿瘤消退。
    Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign head and neck tumor. We report a rare case of JNA, provide a brief literature review, and treatment options, and emphasized the role of flutamide as pre-surgical medication for tumor regression. JNA primarily affects adolescent males aged 14 to 25 years. There are various theories explaining the formation of the tumor. However, sex hormones are found to play a crucial role in the etiology of the tumor. In recent years testosterone and dihydrotestosterone receptors have been identified on the tumor thus suggesting the strong influence of hormones. This permits the use of flutamide, an androgen receptor blocker, as adjuvant therapy for the treatment of JNA. This is a case of a 12-year-old boy who presented to the hospital with right-sided nasal obstruction, epistaxis, watery nasal discharge, and a mass in the right nasal cavity for two months. Diagnostic nasal endoscopy, ultrasonography, computed tomography, and magnetic resonance imaging were done. These investigations confirmed the diagnosis of JNA stage IV. The patient was started on treatment with flutamide for tumor regression.
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  • 文章类型: Journal Article
    用于儿科患者的内窥镜和开放式显微外科手术方法可用于各种颅底病变。混合动力车,头颅内窥镜入路可能有助于改善复杂病变的手术切除。一例13岁男孩,患有大型青少年鼻咽血管纤维瘤,通过鼻咽和翼腭窝延伸到上颌骨,蝶骨,海绵窦通过内窥镜显示,经鼻和额颞叶,扩展中颅窝显微手术入路。通过狭窄的鼻通道管理一个大的儿科肿瘤,在关键的神经血管结构周围进行安全的手术切除,并证明了并发症的避免。该视频可以在这里找到:https://youtu。是/1WqvsOnQCxs。
    Endoscopic and open microsurgical approaches for pediatric patients are useful for a wide variety of skull base pathologies. A hybrid, cranioendoscopic approach may be beneficial in improving surgical resection for complex lesions. A case of a 13-year-old boy with a large juvenile nasopharyngeal angiofibroma extending through the nasopharynx and pterygopalatine fossa into the maxillary, sphenoid, and cavernous sinuses is demonstrated via an endoscopic, transnasal and frontotemporal, extended middle cranial fossa microsurgical approach. Management of a large pediatric tumor via narrow nasal passages, safe surgical resection around critical neurovascular structures, and complication avoidance is demonstrated. The video can be found here: https://youtu.be/1WqvsOnQCxs.
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