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.