1

1Case Report
2Idiopathic cervical esophageal webs: A case report
3and literature review

4Oana Cristina Petrea 1,2, Anca Trifan 1,2,*, Catalin Victor Sfarti 1,2, Irina Girleanu
51,2, Laura Huiban 1 and Carol Stanciu 2
6 1 “Grigore T. Popa” University of Medicine and Pharmacy, Department of Gastroenterology, Iasi, Romania;
7 2 Institute of Gastroenterology and Hepatology, Iasi, Romania;
8 * Correspondence: ancatrifan@yahoo.com;
9 Received: date; Accepted: date; Published: date
10Abstract: An esophageal web is a thin and smooth extension of normal esophageal tissue
11consisting of mucosa and submucosa that can occur anywhere along the length of the esophagus
12but is typically located in the cervical segment. The webs can be congenital or acquired, commonly
13associated with Plummer-Vinson syndrome and rarely with celiac disease, dermatological
14disorders or graft-versus-host disease. A 54-year-old man was referred to our hospital with a
15history of high non-progressive dysphagia to solid food, meat impaction and weight loss over last
16ten months. His past history and family history were unremarkable as well as physical
17examination. Complete blood count and basic biochemical tests were normal. Antigliadin and
18antiendomysial antibodies for celiac disease were negative. Barium swallow esophagography and
19upper endoscopy detected cervical esophageal webs. The treatment consisted in endoscopic balloon
20dilation. The patient’s dysphagia resolved shortly after dilation and the endoscope passed easily in
21through the esophagus showing normal esophageal, gastric and duodenal mucosa. This report is
22consisting with a case of esophageal webs rarely documented in males and that is not related with
23commonly causes like Plummer-Vinson syndrome. Thus, the pathogenesis and treatment of
24esophageal webs are evolving.

25 Keywords: esophageal webs; dysphagia; cervical esophagus; endoscopic dilation

261. Introduction
27 An esophageal web is a thin membranous structure which may obstruct the
28esophageal lumen leading to dysphagia. These webs are typically located in the
29cervical esophagus, although they can occur anywhere along the length of the
30esophagus [1]. The prevalence of cervical esophageal webs is not known because
31most of them are asymptomatic. They are seen in 5-15% of patients who had a
32barium swallow for evaluation of dysphagia [1]. Most commonly, the acquired
33cervical esophageal webs are associated with Plummer-Vinson syndrome (PVs),
34also known as Paterson-Brown Kelly syndrome, characterized by the association of
35dysphagia, iron-deficiency anemia and esophageal webs, affecting mainly women
36(80%) [2]. It should be underlined that most proximal webs are considered
37idiopathic, despite several association with other structural lesions such as Zenker

2J. Clin. Med. 2020, 9, x; doi: FOR PEER REVIEW www.mdpi.com/journal/jcm

 3J. Clin. Med. 2020, 9, x FOR PEER REVIEW 2 of 5

38diverticulum, heterotopic gastric mucosa, esophageal duplication cyst, and

39laryngeal carcinoma, as well as dermatological disorders such as epidermolysis
40bullosa, pemphigus or psoriasis [1].
41 Esophagoscopy was demonstrated to be inferior to barium swallow for the
42diagnosis of esophageal webs. Indeed, upper digestive endoscopy can be a reliable
43method regarding evaluation of normal mucosa and has a therapeutic advantage.
44Thus, endoscopy dilation is the method of choice for the treatment of dysphagia
45due to esophageal webs.
46 The case described in this report highlights some aspects like the importance of
47a differential diagnosis between esophageal webs and other structural lesions or
48associated conditions that may lead to dysphagia. Likewise, despite the fact that
49esophageal webs are mostly associated with PVs, this case has allowed us to
50describe a rare presentation of idiopathic esophageal webs.

512. The Case

52 Our patient gave his informed consent regarding publishing personal data in accordance with
53the Declaration of Helsinki.
54 A 54-year-old man was referred to our hospital with a history of high non-progressive
55dysphagia to solid food, meat impaction and ~ 8 kg of weight loss over last ten months because low
56intake due to dysphagia as his appetite was normal. He denied heartburn, acute caustic ingestion,
57any history of chest pain, vomiting or loss of appetite. His past history and family history
58(including atopic disease) were unremarkable as well as physical examination; in particular, the
59patient has no anemia, koilonychia, glossitis or stomatitis.
60 Basic biochemical tests and complete blood count were normal (white blood cell count:
619730/mm3, eosinophils: 70/mm3, hemoglobin level: 14.2 g/dl, mean corpuscular volume: 91.7 fL,
62serum iron level: 91 mcg/dL and ferritin level: 113 mg/L). Antigliadin and antiendomysial
63antibodies for celiac disease were negative.
64 We performed a barium swallow esophagram that showed two linear, circumferential filling
65defects on the anterior aspect of the cervical esophagus, consisting with the diagnosis of esophageal
66webs (Figure 1).
67

(a) (b)
68 Figure 1. Barium swallow esophagram (a). Two cervical esophageal webs (caused by mucosal
69 diaphragm) (b).
4
 5J. Clin. Med. 2020, 9, x FOR PEER REVIEW 3 of 5

70 Likewise, upper gastrointestinal endoscopy confirmed the presence of the cervical webs at 17
71cm from the incisors with the endoscope not able to pass through (Figure 2). In order to exclude any
72other causes of dysphagia, computed tomography was performed. This revealed no abnormality
73around the esophagus, such as a tumor or swollen lymph nodes, which can cause luminal stenosis.
74 Thus, other causes of cervical esophageal webs were ruled out, including celiac disease, PVs
75syndrome, Zenker diverticulum, benign or malignant tumors or extrinsic compression of
76esophagus.

77

78 Figure 2. Endoscopic view of the cervical esophageal web

79 The treatment consisted in endoscopic balloon dilation without any complications (Figure
803). After this procedure the patient’s dysphagia resolved immediately and the endoscope
81passed easily in through the esophagus showing normal esophageal, gastric and duodenal
82mucosa.
83

84
85
86 Figure 3. Endoscopic dilation of cervical esophageal web
87
88 At the first follow-up, twelve months after endoscopic ballon dilation, the patient gained
89weight and he had no complains. Likewise, he remained asymptomatic and recurrence-free for
90three years.
913. Discussion
6
 7J. Clin. Med. 2020, 9, x FOR PEER REVIEW 4 of 5

92 Esophageal webs are rare structural lesions typically located in the cervical esophagus,
93but they may, nonetheless, represent a diagnostic and therapeutic challenge in front of a
94patient presenting with dysphagia and food impaction.
95 The majority of cases of esophageal webs reported in the literature are associated with
96PVs along with dysphagia and iron-deficiency anemia, but the pathogenesis of esophageal web
97is still unknown. However, the incidence of PVs has decreased during the last decades
98probably as the consequence of largely available iron-replacement therapy [3-5].
99 Esophageal webs can also be rarely associated with celiac disease [6, 7], where iron-deficiency
100is usually the cause of anemia, dermatological conditions [8-10] or graft-versus-host-disease [11, 12].
101It should be noted that dysphagia associated with iron-deficiency anemia is not always due to PVs.
102Thus, differential diagnosis is essential and include several others causes such pharyngeal pouch,
103Zenker diverticulum, benign esophageal strictures, vascular rings, retained foreign bodies in the
104esophagus, surgical esophageal anastomosis, benign and malignant esophageal tumors, pernicious
105anemia, rheumatoid arthritis, epidermolysis bullosa, mucous membrane pemphigoid, and several
106others esophageal disorders (i.e. motility disorders) [3, 13].
107 In the current case, clinical and laboratory parameters helped us exclude iron-deficiency
108anemia, PVs and celiac disease. Likewise, computed tomography ruled out any possible causes of
109dysphagia like esophageal tumors or extrinsic compression of esophagus. Barium swallow
110esophagram played an important role in diagnosing cervical esophageal webs. Thus, our case
111emphases the idiopathic etiology of esophageal webs.
112 The therapeutic method of choice in patients with esophageal webs and dysphagia remains
113endoscopic balloon dilation. This procedure is safe and in general without any risk of web
114recurrence [1]. Our patient was successfully treated with a single session of balloon dilation and
115remained asymptomatic for more than two years.

1164. Conclusions
117 Esophageal web is usually seen in women and is rarely documented in males. To the best of
118our knowledge, this is the first report of an idiopathic cervical esophageal web in a patient with no
119other comorbidities and in no association with PVs. The pathogenesis and treatment of esophageal
120webs are evolving. Most of these structural lesions are asymptomatic but can cause dysphagia
121where needs therapy (commonly endoscopic balloon dilation).
122Author Contributions: Conceptualization, C.S. and A.T.; writing—original draft preparation, O.C.P., L.H. and
123I.G.; writing—review and editing, C.S and C.V.S.; supervision, C.S. and A.T.; All authors have read and agreed
124to the published version of the manuscript.
125Conflicts of Interest: The authors declare no conflict of interest.

126References
127
1281. Smith, M.S. Diagnosis and management of esophageal rings and webs. Gastroenterol Hepatol 2010, 6(11),
129 701-704.
1302. Hoffman, R.M.; Jaffe, P.E. Plummer-Vinson syndrome. A case report and literature review. Arch Intern
131 Med 1995, 155, 2008-2011.
1323. Goel, A; Bakshi, S.S.; Soni, N.; Chhavi, N. Iron deficiency anemia and Plummer-Vinson syndrome:
133 current insights. J Blood Med 2017, 8, 175-184, doi: 10.2147/JBM.S127801.
1344. Hefaiedh, R.; Boutreaa, Y.; Ouakaa-Kchaou, A.; Kochlef, A.; Elloumi, H.; Gargouri, D.; Kharrat, J.;
135 Ghorbel, A. Plummer Vinson syndrome association with coeliac disease. Arab J Gastroenterol 2013, 14(4),
136 183-185, doi: 10.1016/j.ajg.2013.10.003.
8
 9J. Clin. Med. 2020, 9, x FOR PEER REVIEW 5 of 5

1375. Tahara, T.; Shibata, T.; Okubo, M.; Yoshioka, D.; Ishizuka, T.; Sumi, K.; Kawamura, T.; Nagasaka, M.;
138 Nakagawa, Y.; Nakamura, M.; et al. A case of Plummer-Vinson syndrome showing rapid improvement of
139 dysphagia and esophageal web after two weeks of iron therapy. Case Rep Gastroenterol 2014, 8(2), 211-215,
140 doi: 10.1159/000364820.
1416. Dutta, U.; Khaliq, A.; Talha Noor, M.; Kochhar, R.; Singh, K. Recurrent multiple cervical esophageal webs:
142 an unusual presentation of celiac disease. Gastroenterology Res 2009, 2(6), 356-357,
143 doi: 10.4021/gr2009.12.1325.
1447. Sinha, S.K.; Nain, C.K.; Udawat, H.P.; Prasad, K.K.; Das, R.; Nagi, B.; Singh, K. Cervical esophageal web
145 and celiac disease. J Gastroenterol Hepatol 2008, 23, 1149-1152, doi: 10.1111/j.1440-1746.2008.05452.x.
1468. Syn, W.K.; Ahmed, M.M. Esophageal involvement in cicatricial pemphigoid: a rare cause of dysphagia.
147 Dis Esophagus 2004, 17, 180-182, doi: 10.1111/j.1442-2050.2004.00398.x.
1489. Fox, L.P.; Lightdale, C.J.; Grossman, M.E. Lichen planus of the esophagus: what dermatologists need to
149 know. J Am Acad Dermatol 2011, 65, 175-183, doi: 10.1016/j.jaad.2010.03.029.
15010. Djuric, Z.; Nagorni, A.; Zivanovic, D. Esophagitis and almost complete esophageal occlusion in a girl
151 with epidermolysis bullosa. Turk J Pediatr 2012, 54, 301-304.
15211. Watari, T.; Nagano, T.; Takinami, Y. Graft-versus-host disease – induced esophageal web. J Gen Fam Med
153 2019, 20, 33-34, doi: 10.1002/jgf2.215.
15412. Trabulo, D., Ferreira, S.; Lage, P.; Lima Rego, R.; Teixeira, G.; Pereira, A.D. Esophageal stenosis with
155 sloughing esophagitis: a curious manifestation of graft-vs-host disease. World J Gastroenterol 2015, 21,
156 9217-9222.
15713. Halland, M.; Baron, T.S.; Ravi, K. Dysphagia in a patient with a Zenker´s diverticulum: there is more to it
158 than meets the eye. Gastroenterology 2014, 146, 590-591, doi: 10.1053/j.gastro.2013.09.051.
© 2020 by the authors. Submitted for possible open access publication under the terms and
conditions of the Creative Commons Attribution (CC BY) license
(http://creativecommons.org/licenses/by/4.0/).
159

10