Branchial anomaly

支气管异常
  • 文章类型: Journal Article
    UNASSIGNED:术语“branch裂囊肿”是指可以被认为与宫颈淋巴上皮囊肿同义的病变。虽然相对罕见,它们是儿童头颈部病变的第二大原因。本研究旨在报告临床表现,诊断,并对病理诊断为腮裂囊肿的小儿患者进行处理。
    UNASSIGNED:本研究是对33例诊断为支气管囊肿的患者的记录的回顾性分析,在两个不同的大学医院,在两个不同的人群中。
    UnASSIGNED:33例患者中有33例分支裂隙囊肿,其中女性17例,男性16例。大多数(16例)是第2分支裂囊肿。在21例(95%)接受术前手术超声成像的患者中,有20例(95%)通过影像学检查对the裂畸形进行了准确诊断。
    未经证实:支气管裂隙囊肿在鉴别诊断中经常被错误地诊断和忽视。因此,诊断经常延迟,导致患者管理不善。任何颈部外侧肿胀的患者都应怀疑有支气管囊肿,不管肿胀是实性的还是囊性的,疼痛或无痛。超声检查的使用可以极大地帮助临床医生区分branch裂囊肿与头颈部的其他类似病变。
    UNASSIGNED: The term \"branchial cleft cyst\" refers to the lesions that can be considered synonymous with cervical lymphoepithelial cysts. Although relatively rare, they constitute the second major cause of head and neck pathologies in childhood. This study aimed to report the clinical presentations, diagnosis, and management of pediatric patients with the pathological diagnosis of branchial cleft cyst.
    UNASSIGNED: This study was a retrospective analysis of the records of 33 patients with the diagnosis of branchial cyst, in two different university hospitals, in two different populations.
    UNASSIGNED: Thirty-three cases of branchial cleft cysts were seen in 33 patients: 17 females and 16 males. The majority (16 patients) were 2nd branchial cleft cysts. Accurate diagnosis of branchial cleft malformation was made via imaging in 20 of the 21 (95%) patients that underwent preoperative surgical ultrasonographic imaging.
    UNASSIGNED: Branchial cleft cysts are frequently incorrectly diagnosed and ignored in the differential diagnosis. Thus, the diagnosis is often delayed, resulting in the mismanagement of affected patients. A branchial cyst should be suspected in any patient with a swelling in the lateral aspect of the neck, regardless of whether the swelling is solid or cystic, painful or painless. The use of ultrasonography can dramatically help clinicians with distinguishing branchial cleft cysts from other similar lesions of the head and neck.
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  • 文章类型: Case Reports
    第四分支裂异常是小儿和成人患者颈部肿块复发的罕见原因。在这份报告中,我们介绍了一例20岁女性感染的第四分支裂隙囊肿的病例,该病例表现为复发性咽喉疼痛和颈部深部脓肿。经过反复的切开和引流程序后,患者接受了直接喉镜检查的明确治疗,左侧梨状窦道消融,左半甲状腺切除术,切除支气管异常而无复发迹象.除了诊断和管理,该病例报告强调了第四分支异常与梨状窝以及喉上神经和喉返神经之间独特的解剖关系。
    Fourth branchial cleft anomalies are an exceptionally rare cause of recurrent neck mass in pediatric and adult patients. In this report, we present a case of an infected fourth branchial cleft cyst in a 20-year-old woman that presented with recurrent throat pain and deep neck abscesses. After undergoing repeated incision and drainage procedures, the patient underwent definitive management with direct laryngoscopy, ablation of the left pyriform sinus tract, left hemithyroidectomy, and excision of the branchial anomaly without evidence of recurrence. In addition to diagnosis and management, this case report highlights the unique anatomical relationship between fourth branchial anomalies and the pyriform fossa as well as the superior and recurrent laryngeal nerves.
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  • 文章类型: Journal Article
    第三和第四分支异常很少见,占所有分支异常的不到10%。梨状窝窦道(PFST)通常表现为左侧化脓性甲状腺炎,尽管它可以在新生儿早期表现为非发炎的囊性颈部肿块。PFST提出了相当大的诊断挑战,具有可变的临床和影像学特征,导致长期延迟到明确的诊断和适当的管理。
    分析PFST患儿的表现模式和影像学表现,特别关注新生儿的表现。
    这是对临床表现的回顾性回顾,在2003年至2018年期间,在我们三级儿童医院就诊的16例PFST的影像学发现和处理。使用相关的国际疾病分类(ICD)-10编码,通过病历和图片存档和通信系统(PACS)搜索来识别病例。
    介绍时的年龄从产前到16岁,男女比例为2:1。所有患者均表现为颈部肿胀。13例患者(81%)在初次就诊时患有化脓性甲状腺炎。两名患者患有严重的甲状腺炎/纵隔炎,需要入住重症监护病房。三名新生儿未受到感染,无症状的大型囊性颈部肿块;其中两个在产前发现,并误诊为淋巴畸形,随后自发临床消退,后来有PFST的证据。15/16(94%)患者的PFST位于左侧。所有患者在明确诊断前均进行了颈部影像学检查。影像学检查包括X光片,超声,计算机断层扫描,磁共振成像和钡食管造影研究。所有患者均未诊断出PFST。75%的患者在诊断前进行了多模态成像。所有PFST均通过内窥镜可视化确认。13例患者通过内镜烧灼治疗PFST,2例患者通过开放手术治疗。一名患者不需要手术矫正。
    我们的研究强调了PFST的复杂性。这种异常并不常见,具有可变的临床和影像学特征,并且可能具有冗长的,复杂的课程,如果不考虑在最初的介绍。儿童化脓性甲状腺炎发作应提示PFST的调查。我们描述了新生儿囊性肿块的非典型表现,这些表现似乎可以解决,但后来表现为PFST的典型临床特征。
    Third and fourth branchial anomalies are rare, accounting for less than 10% of all branchial anomalies. The piriform fossa sinus tract (PFST) typically presents with left-side suppurative thyroiditis, although it can present earlier in neonates as a non-inflamed cystic neck mass. PFST poses a considerable diagnostic challenge with variable clinical and imaging features, leading to long delays to definitive diagnosis and appropriate management.
    To analyse the patterns of presentation and imaging findings in children with PFST, with a particular focus on neonatal presentation.
    This was a retrospective review of the clinical presentation, imaging findings and management in 16 cases of PFST presenting to our tertiary children\'s hospital between 2003 and 2018. Cases were identified by medical records and picture archiving and communication system (PACS) search using relevant International Classification of Diseases (ICD)-10 coding.
    Age at presentation ranged from prenatal to 16 years, with a male-to-female ratio of 2:1. All patients presented with neck swelling. Thirteen patients (81%) had suppurative thyroiditis at initial presentation. Two patients had severe thyroiditis/mediastinitis that required intensive care unit admission. Three neonates presented with noninfected, asymptomatic large cystic neck masses; two of these were detected prenatally and misdiagnosed as lymphatic malformations with subsequent spontaneous clinical resolution that later represented with evidence of PFST. The PFST was on the left side in 15/16 (94%) patients. All patients had neck imaging before definitive diagnosis. Imaging studies included radiographs, ultrasound, computed tomography, magnetic resonance imaging and barium esophagram studies. No single modality was diagnostic of PFST in all patients. Seventy-five percent of patients had multimodal imaging before diagnosis. All PFSTs were confirmed by endoscopic visualisation. Management of PFST was by endoscopic cauterisation in 13 patients and open surgery in 2. One patient did not require surgical correction.
    Our study highlights the complex nature of PFST. The anomaly is uncommon, has variable clinical and imaging features and may have a lengthy, complicated course if not considered at initial presentation. An episode of suppurative thyroiditis in a child should prompt investigation for PFST. We describe atypical presentations with cystic masses in neonates that appear to resolve but represent later as typical clinical features of PFST.
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  • 文章类型: Journal Article
    简介:第四分支异常,最罕见的分支器官异常,经常出现诊断和治疗挑战。我们评估了临床表现和影像学特征,在这种情况下,患者的治疗和长期结局。患者和方法:从2004年到2020年在鲁汶大学医院接受治疗的12例患者中,收集了12个变量:出生日期,性别,症状发作的年龄,最终诊断的年龄,介绍,偏侧性,以前的程序,诊断工具,治疗(开放颈部手术,内窥镜激光切除术,或组合),并发症,复发,和随访期。计算描述性统计数据,并将结果与现有文献进行比较。结果:最常见的临床表现是反复发生的颈部感染,有或没有。使用直接喉镜明确诊断,可视化内部窦口,在所有患者中都是可能的。一项CT研究揭示了9例患者中7例的第四分支异常的典型特征,对9名患者中的5名进行超声研究。所有患者均行颈部开放手术。如果这不够,对梨状窦尖口进行二次内镜激光切除术(n=4).在八名患者中,需要进行甲状腺叶切除术才能安全地进行完整切除。术后并发症很少,长期,没有患者显示进一步复发。平均随访时间为8.6年。结论:直接喉镜和CT是最准确的诊断工具。我们推荐的治疗方案包括通过开放颈部手术完全切除窦道作为主要治疗方法,因为这样可以确保最佳效果。如果以后复发,内镜下激光切除咽口解决了这个问题。
    Introduction: Fourth branchial anomalies, the rarest among anomalies of the branchial apparatus, often present diagnostic and therapeutic challenges. We evaluated the clinical presentation and radiographic features, the treatment and the long-term outcome of patients in this setting. Patients and Methods: Of 12 patients treated in the University Hospitals Leuven from 2004 until 2020, 12 variables were collected: date of birth, gender, age of onset of the symptoms, age at final diagnosis, presentation, laterality, previous procedures, diagnostic tools, treatment (open neck surgery, endoscopic laser excision, or combination), complications, recurrence, and period of follow-up. Descriptive statistics were calculated and results were compared to the existing literature. Results: The most common clinical manifestations were recurrent neck infections with and without abcedation. Definitive diagnosis using direct laryngoscopy, visualizing the internal sinus opening, was possible in all patients. A CT study revealed the typical features of fourth branchial anomalies in seven patients out of nine, an ultrasound study in five out of nine patients. All patients underwent open neck surgery. If this was insufficient, secondary endoscopic laser resection of the ostium at the apex of the piriform sinus was performed (n = 4). In eight patients a thyroid lobectomy was needed for safe complete resection. Postoperative complications were minimal and at long-term, none of the patients showed further recurrence. Average time of follow-up was 8.6 years. Conclusions: Direct laryngoscopy and CT are the most accurate diagnostic tools. Our recommended treatment schedule consists of complete excision of the sinus tract by open neck surgery as the primary treatment because this ensures the best results. In case of recurrence afterwards, endoscopic laser resection of the pharyngeal ostium solved the problem.
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  • 文章类型: Case Reports
    颈瘘是一种非常罕见的II型第一分支裂异常,其中外耳道和颈部之间存在完整的瘘管。由于反复感染,误诊和管理不善可导致长期发病率和并发症。我们介绍了一名18岁女士的病例,她的脖子上有一个反复放电的鼻窦,持续了4年。过去,她曾接受过反复切开和引流以及多种抗生素的治疗。耳镜检查显示左外耳道地板上有一个开口。诊断为感染的结肠瘘。治疗活动性感染后,完全切除瘘管。关于后续行动,1年时无进一步复发.具有良好的病史和检查,对分支异常的胚胎学知识很好,对于做出正确和早期的诊断以预防发病很重要。
    Collaural fistula is a very rare Work Type II first branchial cleft anomaly in which there is a complete fistulous tract between external auditory canal and the neck. Misdiagnosis and mismanagement can lead to prolonged morbidity and complications due to repeated infections. We present a case of an 18-year-old lady with a recurrent discharging sinus on her neck for 4 years. She has been treated with repeated incision and drainage and multiple antibiotics in the past. Otoscopic examination revealed an opening on the floor of the left external auditory canal. A diagnosis of an infected collaural fistula was made. Complete excision of the fistulous tract was done after treatment of the active infection. On follow-up, there was no further recurrence at 1 year. Sound knowledge of embryology of branchial anomalies with good history and examination is important to make correct and early diagnosis to prevent morbidity.
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  • 文章类型: Journal Article
    Observation of a sinus tract during a barium swallow examination is important for the diagnosis of pyriform sinus fistula; however, to our knowledge, no reports have existed regarding the optimal timing of the examination in relation to the onset of symptoms. The purpose of this study was to compare the timing of the examination, patient age, the number of inflammatory episodes that occurred before the examination, and the barium concentration used for examinations with true-positive results versus those with false-negative results for the diagnosis of pyriform sinus fistula.
    Twenty-three children with pyriform sinus fistula were included. The timing of the examination, patient age, the number of the inflammatory episodes that occurred before examination, and the barium concentration used were compared between examinations with true-positive results and examinations with false-negative results, by use of the Mann-Whitney U test.
    The examination had true-positive results for 60.9% (14/23) of patients and false-negative results for 39.1% (9/23) of patients. The mean (± SD) interval since the onset of symptoms was significantly shorter for patients with false-negative examination results than for those with true-positive examination results (26.33 ± 21.17 days vs 48.57 ± 17.67 days; p = 0.020). By 6 weeks after the onset of symptoms, more than half of the examinations had false-negative results. No significant difference in patient age (p = 0.238) or number of previous inflammatory episodes (p = 0.431) existed between examinations with true-positive and false-negative results; however, a significant difference was noted in the mean barium concentration used (88.57% ± 31.53% vs 52.86% ± 18.68% weight/volume, respectively; p = 0.014).
    Barium swallow examinations with false-negative results were significantly more likely when the examination was performed soon after the onset of symptoms. Therefore, early first examinations would not be recommended for the diagnosis of pyriform sinus fistula, especially in terms of radiation exposure. A higher barium concentration may be useful.
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  • 文章类型: Case Reports
    Although 2(nd) Branchial arch fistulae (from incomplete closure of Cervical sinus of His) are well known, 1(st) arch fistulae are much rarer (<10%) and are usually not tackled comprehensively. We present a case of a rare first branchial arch fistula of the type II Arnot classification, which presented with two external openings of more than 20 years duration. Patient had a successful resection of all the concerned fistulous tract. Review of literature and the surgical challenges of the procedure are presented herewith.
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  • 文章类型: Case Reports
    目的:回顾先天性毛状息肉的文献并描述其临床表现,操作管理,以及在反复窒息发作的新生儿中,由腭咽肌引起的先天性毛状息肉的组织学发现。
    方法:对一名2个月大的男性转诊到三级儿科医院进行图表回顾。
    结果:我们介绍了一例2个月大的男性,因反复出现窒息和呕吐而出现在急诊室。患者以前是健康的,没有既往病史或新生儿史。父母注意到患者的口咽部有一个小的肉质肿块,几分钟后他会咀嚼和吞咽。然而,体检时,没有口咽肿块的证据.患者没有呼吸窘迫。成像显示一个22×7×11mm的长方形,下颈和上胸段食管中的脂肪块,细茎向近端延伸至上塌陷的食管。术中记录的喉镜检查显示,有一个带蒂的软腭肿块附着在右上腭咽肌上。组织病理学显示,外胚层和中胚层成分呈息肉状结构,由角化鳞状上皮与附件结构和中央成熟脂肪组织衬砌,与先天性毛状息肉一致,类似于耳朵的副耳屏和分支异常。在6周的随访中,患者情况良好,体重适当增加,没有进一步窒息发作.在随访检查中没有证据表明咽喉功能障碍。手术部位完全愈合,无复发迹象。
    结论:先天性鼻和口咽毛状息肉少见,但可表现为气道或食管肿块,导致儿科患者呼吸窘迫或窒息发作。角化鳞状上皮的病理发现,附件结构,脂肪和软骨组织类似于先天性副耳屏,可能被认为是支气管弓异常。
    OBJECTIVE: To review the literature of congenital hairy polyps and describe the clinical presentation, operative management, and histologic findings of a congenital hairy polyp arising from the palatopharyngeus muscle in a neonate with recurrent choking episodes.
    METHODS: Chart review of a 2-month-old male referred to a tertiary care pediatric hospital.
    RESULTS: We present a case of a 2-month-old male who presented to the emergency room with recurrent episodes of choking and vomiting. The patient was previously healthy with no prior medical or neonatal history. The parents noted a small fleshy mass in the patient\'s oropharynx that he would chew on and swallow after several minutes. However, on physical exam, there was no evidence of oropharyngeal mass. The patient did not have respiratory distress. Imaging revealed a 22×7×11mm oblong, fatty mass in the lower cervical and upper thoracic esophagus with a thin stalk extending proximally to the upper collapsed esophagus. Intraoperative recorded laryngoscopy revealed a pedunculated soft palate mass attached to the right superior palatopharyngeus muscle. Histopathology revealed ectodermal and mesodermal elements in a polypoid structure lined by keratinizing squamous epithelium with adnexal structures and central mature adipose tissue, consistent with congenital hairy polyp resembling an accessory tragus of the ear and branchial anomaly. At 6-week follow up, the patient was doing well and gaining weight appropriately with no further choking episodes. There was no evidence of velopharyngeal dysfunction on follow up exam. The surgical site was completely healed and there was no evidence of recurrence.
    CONCLUSIONS: Congenital hairy polyps of the naso- and oropharynx are rare but may present as airway or esophageal masses, causing respiratory distress or choking episodes in a pediatric patient. The pathologic findings of keratinizing squamous epithelium, adnexal structures, adipose and cartilage tissues resemble congenital accessory tragus and may be considered a branchial arch anomaly.
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  • 文章类型: Journal Article
    OBJECTIVE: To share our experience involving seven patients with type II first branchial cleft anomalies (hereafter, type II anomalies), to determine whether the location of the external fistula openings of the anomalies are associated with the location of the facial nerve tract, and elucidate the relationship between the location of the fistula opening and the facial nerve.
    METHODS: The medical records of seven patients who underwent surgery from 2005 to 2013 for type II anomalies were retrospectively examined. The relationship between the fistula opening and the facial nerve was evaluated in each patient with respect to whether the fistula opening was superior or inferior to the mandibular angle. All patients underwent partial parotidectomy, facial nerve exposure, and total excision of the mass together with connection of a small cuff of the external auditory canal skin to the fistula tract.
    RESULTS: The fistula tracts were located medially to the facial nerve in two patients, and both fistulae had openings inferior to the mandibular angle. The fistula tracts were located laterally to the facial nerve in the remaining five patients: one patient had no external opening, one had an opening inferior to the mandibular angle, and the remaining three had openings superior to the mandibular angle.
    CONCLUSIONS: Because type II anomalies are rare, their diagnosis is difficult. Surgery of such lesions is challenging and associated with a high risk due to their proximity to the facial nerve. We believe that the location of the fistula opening may help to identify the relationship between the anomalous lesion and facial nerve. Studies involving larger series of cases are needed to confirm our hypothesis; however, because of the rarity of this specific anomaly, it will not be easy to compile a large number of cases. We believe that our study will encourage further investigation on this subject.
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