Otolaryngology Case Reports最新文献

Clicking larynx syndrome in an adolescent patient following trauma: A case report and review of current literature 青少年创伤后咔嗒喉音综合征一例报告及文献回顾

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2026-01-21 DOI: 10.1016/j.xocr.2026.100729

Lindsey Herberger , Peter Eckard , Soroush Farsi , Olivia Speed , Raghu Ramakrishnaiah , Larry D. Hartzell

Introduction

Clicking Larynx Syndrome (CLS) is an uncommon condition of an audible clicking noise from the larynx with movement. While etiology remains unclear, CLS is often linked to a shortened distance between the hyoid and the thyroid cartilage. This can impact quality of life calling for early diagnosis and treatment. The presented case report and literature review explore etiologies, symptomatology, and impact of CLS on patients and proposed diagnostics and treatments.

Case presentation

A 17-year-old male presented with discomfort and an audible click of the larynx with head turning. This was noticed 2 years after minor laryngeal trauma during football. Bilateral CLS was confirmed both clinically and with dynamic 3D CT scan during lateral head rotation and swallow. The patient underwent surgery to resect the elongated superior cornua of the thyroid cartilage bilaterally. Postoperatively, he had near complete resolution of symptoms with only having abnormal movement of the larynx with extreme turning of his head.

Discussion

Twenty-five case reports were identified in the literature. The common finding is a clicking sound and discomfort that is triggered by swallowing or lateral head movements. Diagnostic and therapeutic challenges are discussed, with emphasis on further research to better determine etiology and the best treatment strategies for CLS.

咔嗒声喉综合征（CLS）是一种罕见的声音咔嗒声从喉部与运动。虽然病因尚不清楚，但CLS通常与舌骨和甲状软骨之间的距离缩短有关。这可能会影响生活质量，需要早期诊断和治疗。本文的病例报告和文献综述探讨了CLS的病因、症状和对患者的影响，并提出了诊断和治疗方法。病例介绍一名17岁男性，以不适和可听到的喉部咔嗒声伴头部转动为主诉。这是在踢足球时轻微喉部损伤2年后发现的。在侧头旋转和吞咽时，通过临床和动态3D CT扫描证实双侧CLS。患者接受手术切除双侧甲状软骨上角。术后，患者症状几乎完全消退，仅出现喉部异常运动和头部极度转动。讨论在文献中确定了25例病例报告。常见的症状是吞咽或头部侧向运动引发的咔哒声和不适。讨论了诊断和治疗方面的挑战，重点是进一步研究以更好地确定CLS的病因和最佳治疗策略。

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引用次数: 0

Late surgical intervention in congenital unilateral aural atresia: Audiological and functional outcomes in an adolescent patient 先天性单侧耳闭锁的晚期手术干预：青少年患者的听力学和功能结果

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2026-01-19 DOI: 10.1016/j.xocr.2026.100728

Ronit Priner , Devora Brand

Background

Congenital aural atresia results in conductive hearing loss due to absent or malformed external auditory canal. In unilateral cases, rehabilitation is often deferred, particularly when contralateral hearing is normal. However, emerging evidence highlights the impact of unilateral hearing loss on auditory processing and the potential for neural reorganization even after prolonged deprivation.

Case presentation

An 18-year-old male with right-sided congenital aural atresia underwent atresiaplasty and tympanoplasty after declining amplification for many years. Preoperative imaging demonstrated favorable middle ear anatomy. Postoperative outcomes included improved air-conduction thresholds, enhanced speech understanding—especially in noisy environments—and successful comprehension of telephone speech via the previously non-functional ear. The patient initially experienced hyperacusis and diplacusis, which resolved over time, suggesting active cortical reorganization.

Conclusion

This case illustrates the potential for meaningful auditory and functional recovery following late surgical intervention in congenital unilateral conductive hearing loss. Restoration of auditory input, even in adolescence, may promote central auditory plasticity and improve real-world listening abilities. Personalized treatment planning should consider anatomical, functional, and motivational factors, regardless of age.

背景：先天性耳闭锁由于外耳道缺失或畸形导致传导性听力损失。在单侧病例中，康复往往被推迟，特别是当对侧听力正常时。然而，新出现的证据强调了单侧听力损失对听觉处理的影响，以及即使在长时间丧失听力后神经重组的潜力。一例18岁男性右侧先天性耳廓闭锁患者，经多年耳廓放大下降后行闭锁成形术及鼓室成形术。术前影像学显示良好的中耳解剖。术后结果包括改善空气传导阈值，增强语音理解（特别是在嘈杂环境中），以及通过先前无功能的耳朵成功理解电话语音。患者最初经历了听觉亢进和重音，随着时间的推移逐渐消退，提示皮层重组活跃。结论本病例说明先天性单侧传导性听力损失晚期手术干预后听觉和功能恢复的可能性。听觉输入的恢复，即使在青春期，也可能促进中枢听觉可塑性，提高现实世界的听力能力。个性化治疗计划应考虑解剖、功能和动机因素，而不考虑年龄。

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引用次数: 0

Surgical planning for paediatric cochlear implantation in Warsaw Breakage Syndrome (WABS) using 3D reconstructed model of the malformed cochlea with a review of the literature 利用畸形耳蜗三维重建模型进行华沙破裂综合征（WABS）儿童人工耳蜗植入手术计划并复习文献

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2026-01-14 DOI: 10.1016/j.xocr.2026.100725

Fiona Anna Molnar , Bence Horvath , Haadi Muhammad Mollabux , Roland Nagy , Balint Posta , Zsolt Geretovszky , Eva Perenyi-Csathi , Livia Ivasko , Miklos Csanady , Laszlo Rovo , Adam Perenyi

Warsaw Breakage Syndrome (WABS) is an ultra-rare autosomal recessive disorder, presenting with multiple abnormalities including sensorineural hearing loss due to cochlear malformation. Cochlear implant is a viable treatment for hearing loss in these patients. We report an implantation in a 2.91 year old girl with WABS who presented 1 year prior with bilateral severe sensorineural hearing loss. Radiological investigations revealed a bilateral Type-I Cochlear Hypoplasia). Using radiological images, we generated a 3D reconstruction of the labyrinth and auditory ossicles using the 3D Slicer image computing platform. The resulting models were 3D printed from a biocompatible resin and insertion of the cochlear implant electrode array was tested using a range of electrode models. Following this pre-surgical planning, an optimal electrode (Med-El Compressed) was selected based on size and positioning within the cochlea. The patient then underwent cochlear implantation and postoperatively has shown signs of hearing assessed through both electrophysiological and subjective approaches. The patient presented with severe bilateral cochlear malformation - hypoplasia of the basal turns, absent modiolus and cyst-like merging of the 2nd and 3rd turns. The preoperative 3D visualization and printed model were instrumental in electrode array insertion, positioning, and selection of the ideal individual electrode. This consequently led to successful cochlear implantation (proper electrode placement, minimal complications, improved hearing thresholds and successful rehabilitation). Our findings, coupled with a review of similar rare cases in the literature, suggest that this approach could be broadly applicable to paediatric cases of cochlear hypoplasia with the future aim of optimizing postoperative outcomes.

华沙破裂综合征（WABS）是一种超罕见的常染色体隐性遗传病，表现为多种异常，包括耳蜗畸形引起的感音神经性听力损失。人工耳蜗是治疗这些患者听力损失的可行方法。我们报告一个2.91岁的WABS女孩的植入术，她在一年前出现了双侧严重的感音神经性听力损失。影像学检查显示双侧1型耳蜗发育不全。利用放射图像，我们使用3D切片图像计算平台生成了迷宫和听小骨的3D重建。所得到的模型由生物相容性树脂3D打印，并使用一系列电极模型测试耳蜗植入电极阵列的插入。根据术前计划，根据耳蜗内的大小和位置选择最佳电极（Med-El Compressed）。患者随后接受了人工耳蜗植入，术后通过电生理和主观方法显示出听力的迹象。患者表现为严重的双侧耳蜗畸形，基底耳蜗发育不全，第二和第三耳蜗缺蜗，囊肿样合并。术前3D可视化和打印模型有助于电极阵列的插入、定位和理想单个电极的选择。结果导致人工耳蜗植入成功（适当的电极放置，最小的并发症，改善听力阈值和成功的康复）。我们的研究结果，加上对文献中类似罕见病例的回顾，表明该方法可以广泛适用于耳蜗发育不全的儿科病例，未来的目标是优化术后结果。

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引用次数: 0

Recurrent congenital cholesteatoma in autism spectrum disorder: A rare and complex case report and literature review 自闭症谱系障碍复发性先天性胆脂瘤：罕见复杂病例报告及文献复习

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2026-01-14 DOI: 10.1016/j.xocr.2026.100727

Bilal Hasan , Zulfiqar Hamdan

Introduction

Congenital cholesteatoma is a rare but locally aggressive middle ear lesion associated with potentially serious complications. In children with autism spectrum disorder (ASD), diagnostic delay is further compounded by communication and behavioral challenges.

Case presentation

A 5-year-old Syrian boy with ASD, previously diagnosed at age two, underwent mastoidectomy for right congenital cholesteatoma discovered incidentally during neuroimaging for developmental delay. Despite prior surgical intervention, he presented three years later with recurrent foul-smelling otorrhea. Imaging revealed a recurrent lesion with ossicular erosion, exposure of the sigmoid sinus, and thinning of the middle cranial fossa plate. Revision surgery via a postauricular approach achieved complete excision, with dural and sinus reconstruction using cartilage and temporalis fascia, along with ossicular chain reconstruction.

Clinical discussion

The coexistence of ASD posed additional challenges during clinical evaluation and follow-up. Nevertheless, tailored surgical management resulted in disease control, with no recurrence during two years of follow-up.

Conclusion

This case highlights the diagnostic and therapeutic challenges of congenital cholesteatoma in children with ASD. Early imaging, vigilant follow-up, and individualized surgical planning are essential to prevent recurrence and life-threatening complications while improving long-term outcomes.

摘要先天性胆脂瘤是一种罕见但局部侵袭性的中耳病变，伴有潜在的严重并发症。在患有自闭症谱系障碍（ASD）的儿童中，沟通和行为方面的挑战进一步加剧了诊断延迟。一名5岁的叙利亚ASD男孩，先前在2岁时被诊断出患有ASD，在神经影像学检查中偶然发现患有发育迟缓的右侧先天性胆脂瘤，接受了乳突切除术。尽管先前的手术干预，他提出三年后复发恶臭耳漏。影像学显示复发性病变伴听骨糜烂，乙状骨窦暴露，中颅窝板变薄。通过耳后入路翻修手术实现了完全切除，使用软骨和颞筋膜重建硬脑膜和鼻窦，同时重建听骨链。ASD的共存给临床评估和随访带来了额外的挑战。然而，量身定制的手术管理导致疾病得到控制，在两年的随访期间没有复发。结论本病例突出了先天性胆脂瘤在ASD患儿中的诊断和治疗挑战。早期成像、随访和个体化手术计划对于预防复发和危及生命的并发症以及改善长期预后至关重要。

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引用次数: 0

Aural myiasis with tympanic membrane perforation and middle ear involvement 伴有鼓膜穿孔和中耳受累的耳膜炎

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2026-01-12 DOI: 10.1016/j.xocr.2026.100726

Elena Seiz, Claudia Scherl, Nicole Rotter, Lena Zaubitzer

引用次数: 0

Oropharyngeal neuroglial heterotopia/choristoma with intracranial and extra-oral extension: A case report 口咽神经胶质异位/脉络瘤伴颅内及口外延伸1例

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2026-01-08 DOI: 10.1016/j.xocr.2026.100724

Yara A. Akkielah , Lulwah S. Alturki , Maraam Al Qout , Wafaa Alshakweer , Ali Alzughbi , Abdulelah A. Alluhaybi , Maqsood Ahmad , Dana S. Aljomaah , Ameen S. Binnasser

Background

Neuroglial heterotopia, also referred to as glial choristoma, is a rare congenital malformation characterized by ectopic glial tissue outside the central nervous system. While most cases are reported in the nasal cavity, non-nasal head and neck presentations are extremely rare. Intracranial extension is even more uncommon, and extra-oral protrusion has not been documented to date.

Case presentation

This case reports a 33-day-old male infant born with a large, non-hemorrhagic mass protruding from the oral cavity, diagnosed antenatally and managed via ex utero intrapartum treatment (EXIT). Imaging revealed a solid and cystic lesion extending from the oral cavity into the right middle cranial fossa through a skull base defect, with remodeling of surrounding bony structures. The mass demonstrated both intracranial and extra-oral components, with prominent vascularity. A two-stage surgical approach was performed: initial resection of the intracranial portion via right pterional craniotomy by neurosurgery, followed by a transpalatal excision of the oral mass by otolaryngology. Reconstruction included an inferiorly based palatal flap and acellular dermal matrix grafting. Histopathology confirmed neuroglial heterotopia, with glial tissue lined by stratified squamous epithelium and choroid plexus structures. Immunohistochemistry showed slight increases in Ki-67 and CD45.

Conclusion

This case represents the first reported instance of oropharyngeal neuroglial heterotopia with both intracranial extension and protrusion into the oral cavity. Multidisciplinary care with timely surgical intervention and histopathological confirmation is essential for optimal outcomes in such rare and complex congenital anomalies.

神经胶质异位，也被称为神经胶质脉络瘤，是一种罕见的先天性畸形，其特征是中枢神经系统外的神经胶质组织异位。虽然大多数病例报告在鼻腔，非鼻腔头颈部的表现是极其罕见的。颅内外伸更为少见，而口外突出至今未见文献记载。该病例报告了一名33天大的男婴，出生时口腔外突出一个大的非出血性肿块，经产前诊断并通过宫内治疗（EXIT）进行治疗。影像学显示一实性囊性病变，从口腔延伸至右颅中窝，经颅底缺损，周围骨结构重塑。肿块可见颅内和口外成分，有明显的血管分布。采用两阶段的手术方法：首先通过神经外科的右翼点开颅术切除颅内部分，然后通过耳鼻喉科的经腭切除口腔肿块。重建包括下基腭瓣和脱细胞真皮基质移植。组织病理学证实神经胶质异位，胶质组织内衬分层鳞状上皮和脉络膜丛结构。免疫组织化学显示Ki-67和CD45轻微升高。结论本病例为首次报道的口咽神经胶质异位伴颅内外伸及口腔外突的病例。多学科的护理，及时的手术干预和组织病理学确认是必要的，以获得最佳的结果，这种罕见的和复杂的先天性异常。

{"title":"Oropharyngeal neuroglial heterotopia/choristoma with intracranial and extra-oral extension: A case report","authors":"Yara A. Akkielah , Lulwah S. Alturki , Maraam Al Qout , Wafaa Alshakweer , Ali Alzughbi , Abdulelah A. Alluhaybi , Maqsood Ahmad , Dana S. Aljomaah , Ameen S. Binnasser","doi":"10.1016/j.xocr.2026.100724","DOIUrl":"10.1016/j.xocr.2026.100724","url":null,"abstract":"<div><h3>Background</h3><div>Neuroglial heterotopia, also referred to as glial choristoma, is a rare congenital malformation characterized by ectopic glial tissue outside the central nervous system. While most cases are reported in the nasal cavity, non-nasal head and neck presentations are extremely rare. Intracranial extension is even more uncommon, and extra-oral protrusion has not been documented to date.</div></div><div><h3>Case presentation</h3><div>This case reports a 33-day-old male infant born with a large, non-hemorrhagic mass protruding from the oral cavity, diagnosed antenatally and managed via ex utero intrapartum treatment (EXIT). Imaging revealed a solid and cystic lesion extending from the oral cavity into the right middle cranial fossa through a skull base defect, with remodeling of surrounding bony structures. The mass demonstrated both intracranial and extra-oral components, with prominent vascularity. A two-stage surgical approach was performed: initial resection of the intracranial portion via right pterional craniotomy by neurosurgery, followed by a transpalatal excision of the oral mass by otolaryngology. Reconstruction included an inferiorly based palatal flap and acellular dermal matrix grafting. Histopathology confirmed neuroglial heterotopia, with glial tissue lined by stratified squamous epithelium and choroid plexus structures. Immunohistochemistry showed slight increases in Ki-67 and CD45.</div></div><div><h3>Conclusion</h3><div>This case represents the first reported instance of oropharyngeal neuroglial heterotopia with both intracranial extension and protrusion into the oral cavity. Multidisciplinary care with timely surgical intervention and histopathological confirmation is essential for optimal outcomes in such rare and complex congenital anomalies.</div></div>","PeriodicalId":37154,"journal":{"name":"Otolaryngology Case Reports","volume":"38 ","pages":"Article 100724"},"PeriodicalIF":0.3,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145976490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Primary parotid angiosarcoma: A case report and literature review 原发性腮腺血管肉瘤1例报告并文献复习

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2025-12-27 DOI: 10.1016/j.xocr.2025.100722

Lucía Honrubia , José Maria Verdaguer , Antonio Rodríguez-Valiente , Miguel Ángel Sánchez-Pérez , José Ramón García-Berrocal , Ángela Maria Harker-Ladino , José Luis Rodríguez-Carrillo

Angiosarcoma is a rare, aggressive vascular malignancy that typically affects older men often following radiotherapy. Primary parotid angiosarcoma is uncommon, particularly in patients without known risk factors. We report the case of an 82-year-old woman who presented a parotid mass and a frontal skin lesion, without prior radiotherapy or relevant medical history. Imaging revealed a 4 cm parotid lesion with mandibular involvement but no nodal disease. Fine needle aspiration suggested a malignant epithelioid neoplasm, and PET confirmed uptake in the parotid gland, mandibular condyle, and frontal lesion. The patient underwent total parotidectomy with en bloc resection of the facial nerve and mandibular condyle, ipsilateral functional neck dissection, and excision of the frontal lesion with deep inferior epigastric perforator (DIEP) flap reconstruction. Histopathology confirmed poorly differentiated angiosarcoma with lymphovascular and perineural invasion. Immunohistochemistry was positive for vimentin, CD31, ERG, and CD34, with loss of H3K27me3 expression but no c-MYC amplification. Despite the adjuvant therapy recommendation, the patient developed pulmonary metastases and died three months postoperatively. Angiosarcoma presents with frequent pulmonary metastases and a dismal prognosis. Surgical resection with negative margins and radiotherapy remains the mainstay of treatment. However, survival outcomes remain poor. This case illustrates its aggressive clinical course and distinctive molecular profile, emphasizing the importance of early diagnosis and multidisciplinary treatment.

血管肉瘤是一种罕见的侵袭性血管恶性肿瘤，通常影响放疗后的老年男性。原发性腮腺血管肉瘤并不常见，特别是在没有已知危险因素的患者中。我们报告一个82岁的妇女谁提出一个腮腺肿块和额部皮肤病变，没有放疗或相关的病史。影像显示一4公分腮腺病变并累及下颌骨，但无淋巴结病变。细针穿刺提示恶性上皮样肿瘤，PET证实腮腺、下颌髁和额部病变有摄取。患者行腮腺全切除术，包括面神经和下颌髁的整体切除，同侧功能性颈部清扫术，以及腹下深穿支皮瓣重建额部病变切除。组织病理学证实低分化血管肉瘤伴淋巴血管和神经周围浸润。免疫组化检测vimentin、CD31、ERG、CD34阳性，H3K27me3表达缺失，c-MYC无扩增。尽管推荐辅助治疗，但患者发生肺转移并在术后3个月死亡。血管肉瘤常发生肺转移，预后差。手术切除阴性切缘和放射治疗仍然是主要的治疗方法。然而，生存结果仍然很差。这个病例说明了其侵袭性的临床过程和独特的分子特征，强调了早期诊断和多学科治疗的重要性。

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引用次数: 0

Retropharyngeal lipoma as a rare etiology of pediatric refractory obstructive sleep apnea 咽后脂肪瘤是小儿难治性阻塞性睡眠呼吸暂停的罕见病因

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2025-12-18 DOI: 10.1016/j.xocr.2025.100721

Maya Andrade , Emlyn Diakow , Michael Bobian , Zachary Kelly

引用次数: 0

Expect the unexpected: A rare case of bilateral lymphoepithelial carcinoma of the parotid gland 意料之外：一个罕见的双侧腮腺淋巴上皮癌病例

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2025-12-11 DOI: 10.1016/j.xocr.2025.100718

Umair Hafeez , Niall James McInerney , Sri Ganesh Kalimuthu , Olena Tkachuk

Lymphoepithelial carcinoma (LEC) of the salivary glands is a rare and aggressive malignancy strongly associated with Epstein–Barr virus (EBV) infection and more frequently reported in endemic regions such as Greenland and Southeast Asia. Bilateral involvement is exceptionally uncommon, particularly in non-endemic populations. We report the case of a 62-year-old male with bilateral, asymptomatic parotid swellings persisting for five years. Initial clinical assessment suggested a benign etiology, with no pain, facial nerve involvement, or other red flag symptoms. However, ultrasound, fine needle aspiration, and contrast-enhanced computed tomography of the left parotid revealed atypical features suspicious for malignancy, and histopathology confirmed LEC. The patient underwent left superficial parotidectomy with clear margins, followed by right superficial parotidectomy, which also confirmed LEC. This case represents a rare presentation of bilateral LEC in a non-endemic region. The indolent course and absence of classic malignant features highlight the diagnostic challenges of this entity. Early multidisciplinary evaluation is essential for persistent or atypical parotid swellings, and further research is warranted into the role of EBV in tumorigenesis outside endemic areas.

唾液腺淋巴上皮癌（LEC）是一种罕见的侵袭性恶性肿瘤，与eb病毒（EBV）感染密切相关，在格陵兰岛和东南亚等流行地区更为常见。双边参与极为罕见，特别是在非流行人群中。我们报告的情况下，62岁的男性与双侧，无症状腮腺肿胀持续五年。初步临床评估提示为良性病因，无疼痛、面神经受累或其他危险症状。然而，超声、细针穿刺和增强ct显示左腮腺不典型特征，怀疑为恶性肿瘤，组织病理学证实LEC。患者行左侧腮腺浅表切除术，边缘清晰，随后行右侧腮腺浅表切除术，也证实LEC。本病例是非流行地区罕见的双侧LEC。惰性过程和缺乏典型的恶性特征突出了这个实体的诊断挑战。早期多学科评估对于持续或非典型腮腺肿胀至关重要，进一步研究eb病毒在流行地区以外的肿瘤发生中的作用是必要的。

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引用次数: 0

Spontaneous catastrophic cervical hemorrhage in the setting of neurofibromatosis 神经纤维瘤病并发自发性灾难性宫颈出血

IF 0.3 Q4 OTORHINOLARYNGOLOGY

Otolaryngology Case Reports

Pub Date : 2025-12-04 DOI: 10.1016/j.xocr.2025.100720

Lavanya Nagappan , Yekaterina Shapiro , Luke Stanisce , Donald Solomon

Neurofibromatosis type 1 (NF1) can manifest in the head and neck in a variety of ways, most commonly presenting as solitary neurofibromas. The management of these lesions require multifaceted consideration of neurovascular involvement and airway compromise. Herein, we describe a 58-year-old male with a history of NF1 and extensive cervical neurofibromas who developed a spontaneous, expanding neck hematoma secondary to his tumors. He was initially stabilized with surgical drainage and endovascular embolization, but then subsequently represented with a second rapidly expanding neck hematoma resulting in acute respiratory distress. Failed intubation due to airway edema necessitated emergent cricothyrotomy and cardiopulmonary resuscitation. Additional embolization was performed but the patient eventually succumbed to global anoxic brain injury.

This case highlights the need for proactive multi-disciplinary management with specific attention to respiratory and hemodynamic stabilization in NF1 patients with large tumor burden. As with all neck hematomas, rapid respiratory decompensation may occur, requiring prompt recognition and early intervention. This case underscores the significance of a multidisciplinary approach involving emergency medicine, critical care, anesthesia, interventional radiology, and otolaryngology to recognize and address the potential for airway compromise in the face of a neck hematoma in patients with NF1.

1型神经纤维瘤病（NF1）可以在头颈部以多种方式表现，最常见的表现为孤立的神经纤维瘤。这些病变的处理需要多方面考虑神经血管受累和气道损害。在此，我们描述了一位58岁的男性，有NF1和广泛的颈部神经纤维瘤的病史，他的肿瘤发展为自发性的，扩大的颈部血肿。他最初通过手术引流和血管内栓塞稳定，但随后出现第二次迅速扩大的颈部血肿，导致急性呼吸窘迫。由于气道水肿插管失败，需要紧急环甲环切开术和心肺复苏。虽然进行了额外的栓塞治疗，但患者最终死于全身性缺氧脑损伤。本病例强调需要积极主动的多学科管理，特别关注具有大肿瘤负担的NF1患者的呼吸和血流动力学稳定。与所有颈部血肿一样，可能发生快速呼吸失代偿，需要及时识别和早期干预。该病例强调了多学科方法的重要性，包括急诊医学、重症监护、麻醉、介入放射学和耳鼻喉科，以识别和解决NF1患者面对颈部血肿时气道损害的可能性。

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引用次数: 0