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
    目的:全面总结鼻窦血管纤维瘤(STA)(俗称青少年鼻咽血管纤维瘤)的影像学特点。
    方法:通过系统评价确定的33篇研究文章提供的41例病例中的44个病灶和来自我们机构的13例与接受MRI检查的STA患者相关的13个病灶被纳入回顾研究。由两名经过董事会认证的经验丰富的放射科医生进行。
    结果:研究参与者均为男性患者,诊断时平均年龄为15.6岁。他们都表现为鼻腔病变(100%),主要在鼻咽部(98.2%)。蝶腭孔/翼腭窝受累占76.0%,上颌窦后外侧壁的压缩移位占一半以上(57.9%)。与骨骼肌(100%)相比,T2加权成像信号强度是异质的,具有混合的高强度和等强度。T1加权成像在61.1%的病例中显示部分高信号强度。几乎所有情况下都存在流动空隙和强烈增强。对比增强MRI上的囊性/非增强改变相对常见(40.8%)。平均表观扩散系数值(2.07×10-3mm2/秒)和一些定量动态对比增强MRI参数较高。根据颅底侵犯(p=0.017)和颅内延伸(p=0.003)的MRI发现,残留/复发病变的频率存在显着差异。
    结论:我们总结了STA的MRI表现,有助于及时诊断和适当处理。
    To comprehensively summarize the radiological characteristics of sinonasal tract angiofibroma (STA) (commonly known as juvenile nasopharyngeal angiofibroma).
    Forty-four lesions from 41 cases provided by 33 study articles identified through a systematic review and 13 lesions from 13 cases from our institution associated with patients with STA who underwent MRI were included in the review study, carried out by two board-certified experienced radiologists.
    The study participants were all male patients with a mean age of 15.6 years at the time of diagnosis. All of them presented with nasal cavity lesions (100%), predominantly in the nasopharynx (98.2%). The sphenopalatine foramen/pterygopalatine fossa was involved in 76.0%, and compressive shift of the posterolateral wall of the maxillary sinus was present in more than half (57.9%). T2-weighted imaging signal intensity was heterogeneous with mixed high and iso intensities as compared to skeletal muscle (100%). T1-weighted imaging showed partial high signal intensity in 61.1% of the cases. Flow void and intense enhancement were present in almost all cases. Cystic/nonenhancement changes on contrast-enhanced MRI were relatively common (40.8%). The mean apparent diffusion coefficient value (2.07 × 10-3  mm2 /second) and some quantitative dynamic contrast-enhanced MRI parameters were high. There was a significant difference in the frequency of residual/recurrent lesions based on the presence of MRI findings of skull base invasion (p = .017) and intracranial extension (p = .003).
    We summarized the MRI findings of STA that can facilitate timely diagnosis and appropriate management.
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  • 文章类型: Journal Article
    青少年鼻咽血管纤维瘤(JNA)是最神秘的,耳鼻喉科医师遇到的血性肿瘤,头颈外科医生,神经外科医生,还有麻醉师.JNA很少见,良性,高血管性肿瘤倾向于侵袭性局部侵袭1手术,开放式或内窥镜检查,去除生长是JNA的主要治疗选择。历史上,手术切除与大量,快速失血,传统上通过输血和故意低血压来管理。采用多模式血液保护策略的预防性管理应该是JNA患者围手术期护理的基本标准。我们描述了一种现代而全面的方法来管理高级别JNA肿瘤患者。这包括手术策略,如抢先颈外动脉栓塞,内镜手术入路,和分阶段的行动,以及麻醉策略,包括抗纤溶治疗和急性等容血液稀释。这些手术,曾经是大量输血的代名词,可能在没有异体输血的情况下进行,或者故意低血压.本文作者从多学科患者血液管理(PBM)的角度重新审视了JNA管理的围手术期方法。
    Juvenile nasopharyngeal angiofibromas are one of the most enigmatic, bloody tumors encountered by otorhinolarygnologists, head and neck surgeons, neurosurgeons, and anesthesiologists. Juvenile nasopharyngeal angiofibromas are rare, benign, highly vascular tumors with a propensity towards aggressive local invasion. Surgery, open or endoscopic, to remove the growth is the primary treatment of choice for Juvenile nasopharyngeal angiofibromas. Historically, surgical resection was associated with massive, rapid blood loss, traditionally managed by blood product transfusion and deliberate hypotension. Preventative management employing multimodal blood conservation strategies should be an essential standard of perioperative care for patients with Juvenile nasopharyngeal angiofibromas.
    We describe a contemporary and comprehensive approach in the management of patients with high grade Juvenile nasopharyngeal angiofibromas. This includes surgical strategies such as preemptive external carotid artery embolization, endoscopic surgical approach, and staged operations, as well as anesthetic strategies including antifibrinolytic therapy and acute normovolemic hemodilution. These surgeries, once synonymous with massive transfusion, may potentially be performed without allogeneic blood transfusion, or deliberate hypotension.
    Using a case series, the authors introduce a contemporary approach to multimodal, multidisciplinary blood conservation strategies for Juvenile nasopharyngeal angiofibromas surgery.
    Here in the authors report on an updated contemporary perioperative clinical approach to patients with Juvenile nasopharyngeal angiofibromas. From an anesthetic perspective, we describe the successful use of normal hemodynamic goals, restrictive transfusion strategy, antifibrinolytic therapy, autologous normovolemic hemodilution, and early extubation in the care of three adolescent males with highly invasive tumors. We demonstrate that new surgical and anesthetic strategies have yielded a significant decrease in intraoperative blood loss and eliminated the need for transfusion of autologous red blood cells, which enable improved outcomes.
    The perioperative approach to elective surgery for Juvenile nasopharyngeal angiofibromas management is presented from a multidisciplinary patient blood management perspective.
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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: Case Reports
    青少年鼻咽血管纤维瘤(JNA)是一种非常罕见的疾病。我们正在介绍一个十几岁的男孩无痛鼻塞和双耳朵丰满两到三个月的情况。在鼻内窥镜检查中,注意到强烈的增生性肿块阻塞了右鼻腔。触摸时流血。在鼻旁窦(CECTPNS)的对比增强计算机断层扫描中,JNA的诊断是基于骨侵蚀和强烈的对比后增强的证据。术前局部栓塞,然后手术切除。关于后续行动,患者病情稳定,无复发迹象.
    Juvenile nasopharyngeal angiofibroma (JNA) is a very uncommon condition. We are presenting a case of a teenage boy with painless nasal blockage and fullness of bilateral ears for two to three months. On nasal endoscopy, a firm proliferative mass obstructing the right nasal cavity was noted. It was bleeding on touch. On contrast-enhanced computed tomography of the paranasal sinuses (CECT PNS), a diagnosis of the JNA was made based on the evidence of bony erosion and intense post-contrast enhancement. He was pre-operatively locally embolized followed by surgical resection. On follow-up, the patient was stable with no signs of recurrence.
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  • 文章类型: Case Reports
    Juvenile nasopharyngeal angiofibroma (JNA) is a relatively uncommon, benign neoplasm of the nasopharynx that can be very difficult to diagnose early due to inconspicuous and seemingly harmless presenting symptoms. Early diagnosis and treatment of JNA are essential for a good prognosis. JNA typically responds well to radiation therapy (RT), but when it does not, the most appropriate next course of action has not been readily defined due to the limited occurrence and experience with this neoplasm. Herein, we describe a JNA patient, who continued to progress after surgery and 36 Gy of adjuvant radiation, but after an additional 14.4 Gy, he has remained in remission for over 2 years. An 11-year-old boy who presented with JNA underwent treatment with embolization and surgical resection. Unfortunately, the tumor progressed within 2 months of surgical intervention and he required RT for adequate local control. While undergoing RT, he again demonstrated signs of progression; so his radiation regimen was increased from 3,600 cGy in 20 fractions to 5,040 cGy in 28 fractions. Since completing RT, the tumor has continued to decrease in size, and the patient is stable and has been without signs of disease progression for over 24 months now. Thus, escalating the radiation regimen to 5,040 cGy may improve local control in rapidly progressive JNA.
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  • 文章类型: Case Reports
    我们提出了一种多系统方法,涉及多种罕见疾病的专家。引入多学科工作的重要性和完整的患者知识,以便适当的临床实践和患者结果。
    We present a multisystemic approach involving diverse specialists of a rare disease. Bringing into the perspective the importance of multidisciplinary work and complete patient knowledge in order to an adequate clinical practice and patient outcome.
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  • 文章类型: Journal Article
    OBJECTIVE: To describe the midfacial degloving approach and compare the varying surgical approaches to juvenile angiofibromas. To demonstrate the efficacy of midfacial degloving in treating large juvenile angiofibromas in a unique patient cohort.
    METHODS: A retrospective case-series between 2006 and 2019.
    METHODS: All patient care was undertaken at a regional skull base referral centre.
    METHODS: Twenty-one male patients with a median age of 18 (range 16-45 years).
    METHODS: Presenting symptoms, imaging, stage, age at operation, residual disease, estimated blood loss and operative time were all recorded. Postoperative outcomes included complications, length of stay and recurrence.
    RESULTS: The median surgical time was 105 minutes (range 55-219 minutes), median estimated blood loss 600 mls (range 150-900 mls) and median length of stay was 4 days (range 2-13 days.). Complications included two episodes of epistaxis, one requiring packing and one return to theatre. 14% (3/21) of patients had residual disease, none requiring further treatment and one patient had recurrence.
    CONCLUSIONS: MFD for JNA in our series resulted in low recurrence rate and no progression of residual disease. The approach has been successful in our cohort of patients and is an option in males over the age of 16 years, with JA extending beyond the nasopharynx and sinuses, involving the infratemporal fossa, cavernous sinus or orbital region.
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