Goel Amit, Bakshi Satvinder Singh, Soni Neetu, Chhavi Nanda
Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Department of Otorhinolaryngology and Head and Neck Surgery, Mahatma Gandhi Medical College and Research Institute, Puducherry, India.
J Blood Med. 2017 Oct 19;8:175-184. doi: 10.2147/JBM.S127801. eCollection 2017.
Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region. It was first described over a century ago. However, literature on this condition remains scanty, and its prevalence appears to be declining worldwide, possibly due to improvements in nutrition over time. The condition has been reported most commonly in thin-built, middle-aged, white women. The esophageal webs in PVS are thin mucosal folds, which are best seen either in lateral views at barium swallow or at esophagoscopy. These are usually semilunar or crescentic, being located most often along the anterior esophageal wall, but can be concentric. The exact cause and pathogenesis of PVS remain unclear, though iron and other nutritional deficiencies, genetic predisposition and autoimmunity have all been implicated in formation of the webs. Treatment includes correction of iron deficiency and endoscopic dilation of the esophageal webs to relieve dysphagia. PVS is associated with an increased risk of hypopharyngeal and esophageal malignancies. Correction of iron deficiency may arrest and reverse the mucosal changes and possibly reduces this risk.
普卢默-文森综合征(PVS)是一种罕见的临床病症,其特征为咽下困难、缺铁性贫血和环状软骨后区域的食管蹼三联征。它在一个多世纪前首次被描述。然而,关于这种病症的文献仍然稀少,而且其在全球的患病率似乎正在下降,这可能是由于随着时间推移营养状况得到改善。这种病症最常报道于体型消瘦的中年白人女性。PVS中的食管蹼是薄的黏膜皱襞,在吞钡造影的侧位片或食管镜检查时最易观察到。它们通常呈半月形或新月形,最常位于食管前壁,但也可能是同心圆形。尽管铁及其他营养缺乏、遗传易感性和自身免疫都被认为与食管蹼的形成有关,但PVS的确切病因和发病机制仍不清楚。治疗包括纠正缺铁以及对食管蹼进行内镜扩张以缓解咽下困难。PVS与下咽和食管恶性肿瘤的风险增加相关。纠正缺铁可能会阻止并逆转黏膜变化,并可能降低这种风险。