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Patterson-Kelly Syndrome: A Single Centre Experience

Received: 28 June 2020    Accepted: 16 July 2020    Published: 4 August 2020
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Abstract

Background: Patterson Kelly syndrome is a rare entity, presenting with a triad of Iron deficiency anemia, Dysphagia and Esophageal webs. The syndrome is extremely rare and its incidence is decreasing probably due to improvements in nutritional standards. The syndrome is enigmatic in the sense that not all patients may be having anemia, the syndrome is associated with various autoimmune conditions, webs are located in post-cricoid region only and there is increased prevalence of upper gastrointestinal malignancies in these patients. The symptoms respond to iron replacement but many patients require rupture of web for relief of dysphagia. Savary dilators, TTS balloon, electro-cautery have been variably used to break the web. Objectives: 1. To study the profile of Patterson Kelly syndrome at our center; 2. To study if the endoscope itself can be used as effective dilator for rupturing the web; 3. To study the effect of iron replacement in these patients; 4. To study the occurrence of upper gastrointestinal malignancies in this condition. Material and methods: We report our experience of sixteen patients with Patterson Kelly syndrome over last fifteen years. This is a retrospective, single center, observational study. All patients had demonstrable web on endoscopy. The endoscope itself was used as a dilator to break the web in more than half of the patients. The endoscope being rigid instrument can easily break the thin web during diagnostic endoscopy itself. If the opening was too narrow, TTS balloon or Savary Guilard dilators were used. One patient had a perforation while using endoscope as the dilator. Five patients required more than one sitting of dilatation for achieving relief of dysphagia or for recurrent web formation. Most patients received intravenous iron supplementation. Ten patients had complete relief of dysphagia without recurrence. One patient had concomitant esophageal squamous cell carcinoma in lower esophagus which became evident when the scope was passed distally, post dilatation of the web. Conclusions: Although rare, Patterson Kelly syndrome continues to exist and can be effectively treated by iron replacement, dilatation and rupture of esophageal webs using various methods. The web can be ruptured by careful maneuvering of the endoscope itself. Intravenous iron replacement can be used prior to the procedure which can ameliorate the symptoms and reduce the need for the dilatation or can make it easier. Patients need surveillance for upper gastrointestinal malignancies however exact guidelines in this regard are lacking.

Published in International Journal of Gastroenterology (Volume 4, Issue 2)
DOI 10.11648/j.ijg.20200402.13
Page(s) 41-44
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Patterson Kelly Syndrome, Esophageal Webs, Dysphagia, Iron Deficiency Anemia

References
[1] Paterson DR. A clinical type of dysphagia. Proceedings of the Royal Society of Medicine. 1919 May; 12 (Laryngol_Sect): 235-9.
[2] Brown-Kelly A. Spasm at the entrance of the oesophagus. J Laryngol Otol. 1919 Aug; 34: 285-9.
[3] Bakari G, Benelbarhdadi I, Bahije L, El feydi Essaid A. Endoscopic treatment of 135 cases of Plummer-Vinson web: a pilot experience. Gastrointestinal endoscopy. 2014 Oct 1; 80 (4): 738-41.
[4] Goel A, Lakshmi CP, Bakshi SS, Soni N, Koshy S. Single-center prospective study of Plummer–Vinson syndrome. Diseases of the Esophagus. 2016 Oct 1; 29 (7): 837-41.
[5] Chhabra P, Khurana H. Image Diagnosis: Plummer-Vinson Syndrome: An Unusual Cause of Dysphagia. Perm J. 2018; 22: 18-035. doi: 10.7812/TPP/18-035. PMID: 30005727; PMCID: PMC6045507.
[6] Changela, K., Haeri, N. S., Krishnaiah, M. et al. Plummer-Vinson Syndrome with Proximal Esophageal Web. J Gastrointest Surg 20, 1074–1075 (2016). https://doi.org/10.1007/s11605-015-3051-5.
[7] Chisholm M. The association between webs, iron and post-cricoid carcinoma. Postgraduate Medical Journal. 1974 Apr 1; 50 (582): 215-9.
[8] Makharia GK, Nandi B, Garg PK, Tandon RK. Plummer Vinson syndrome: unusual features. Indian journal of gastroenterology: official journal of the Indian Society of Gastroenterology. 2002; 21 (2): 74.
[9] Salihoun M, Kabbaj N, Raissouni F, Chaoui Z, Mohamed A, Naima A. Safety and effectiveness of endoscopic Savary-Gillaard Bougies dilation in Moroccan Plummer-Vinson syndrome patients. International Scholarly Research Notices. 2013; 2013.
[10] Ohtaka M, Kobayashi S, Yoshida T, Yamaguchi T, Uetake T, SatoT, Hayashi A, Kanai M, Yamamoto T, Hatsushika K, Masuyama K. Use of Sato's curved laryngoscope and an insulated-tip knife for endoscopic incisional therapy of esophageal web. Digestive Endoscopy. 2015 May; 27 (4): 522-6.
[11] Elwood PC, Pitman RG, Jacobs A, Entwistle CC. Epidemiology of the Paterson-Kelly syndrome. The Lancet. 1964 Oct 3; 284 (7362): 716-20.
[12] Hussin A, Din IU, Arif A, Shah SM, Hafeez M. Plummer Vinson Syndrome in Patients Presenting With Dysphagia. Journal of Medical Sciences. 2017 Mar 6; 25 (1): 24-6.
[13] Swain SK, Panigrahy R, Sahu MC. Plummer Vinson syndrome in a male and his chromosomal study–A case report. Egyptian Journal of Medical Human Genetics. 2015; 16 (3): 283-6.
[14] Priyadarshini Karthikeyan, Nalini Aswath, Ramesh Kumaresan Plummer Vinson Syndrome: A Rare Syndrome in Male with Review of the Literature. Case Reports in Dentistry; Volume 2017 | Article ID 6205925|5 pages |https://doi.org/10.1155/2017/6205925.
[15] Bredenkamp JK, Castro DJ, Mickel RA. Importance of iron repletion in the management of Plummer-Vinson syndrome. Annals of Otology, Rhinology & Laryngology. 1990 Jan; 99 (1): 51-4.
[16] Dantas RO, Villanova MG. Esophageal motility impairment in Plummer-Vinson syndrome. Digestive diseases and sciences. 1993 May 1; 38 (5): 968-71.
[17] Dantas RO. Iron deficiency and dysphagia. American Journal of Gastroenterology. 1999 Oct 1; 94 (10): 3072-3.
[18] Jones RM. The Paterson-Brown Kelly syndrome: its relationship to iron deficiency and postcricoid carcinoma. The Journal of Laryngology & Otology. 1961 Jun; 75 (6): 544-61.
[19] Waldenstrom J, Kjellberg SR. The roentgenological diagnosis of sideropenic dysphagia. Acta radiologica. 1939 Jan 1; 20 (6): 618-38.
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    Ramesh Satarkar. (2020). Patterson-Kelly Syndrome: A Single Centre Experience. International Journal of Gastroenterology, 4(2), 41-44. https://doi.org/10.11648/j.ijg.20200402.13

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    Ramesh Satarkar. Patterson-Kelly Syndrome: A Single Centre Experience. Int. J. Gastroenterol. 2020, 4(2), 41-44. doi: 10.11648/j.ijg.20200402.13

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    AMA Style

    Ramesh Satarkar. Patterson-Kelly Syndrome: A Single Centre Experience. Int J Gastroenterol. 2020;4(2):41-44. doi: 10.11648/j.ijg.20200402.13

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  • @article{10.11648/j.ijg.20200402.13,
      author = {Ramesh Satarkar},
      title = {Patterson-Kelly Syndrome: A Single Centre Experience},
      journal = {International Journal of Gastroenterology},
      volume = {4},
      number = {2},
      pages = {41-44},
      doi = {10.11648/j.ijg.20200402.13},
      url = {https://doi.org/10.11648/j.ijg.20200402.13},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijg.20200402.13},
      abstract = {Background: Patterson Kelly syndrome is a rare entity, presenting with a triad of Iron deficiency anemia, Dysphagia and Esophageal webs. The syndrome is extremely rare and its incidence is decreasing probably due to improvements in nutritional standards. The syndrome is enigmatic in the sense that not all patients may be having anemia, the syndrome is associated with various autoimmune conditions, webs are located in post-cricoid region only and there is increased prevalence of upper gastrointestinal malignancies in these patients. The symptoms respond to iron replacement but many patients require rupture of web for relief of dysphagia. Savary dilators, TTS balloon, electro-cautery have been variably used to break the web. Objectives: 1. To study the profile of Patterson Kelly syndrome at our center; 2. To study if the endoscope itself can be used as effective dilator for rupturing the web; 3. To study the effect of iron replacement in these patients; 4. To study the occurrence of upper gastrointestinal malignancies in this condition. Material and methods: We report our experience of sixteen patients with Patterson Kelly syndrome over last fifteen years. This is a retrospective, single center, observational study. All patients had demonstrable web on endoscopy. The endoscope itself was used as a dilator to break the web in more than half of the patients. The endoscope being rigid instrument can easily break the thin web during diagnostic endoscopy itself. If the opening was too narrow, TTS balloon or Savary Guilard dilators were used. One patient had a perforation while using endoscope as the dilator. Five patients required more than one sitting of dilatation for achieving relief of dysphagia or for recurrent web formation. Most patients received intravenous iron supplementation. Ten patients had complete relief of dysphagia without recurrence. One patient had concomitant esophageal squamous cell carcinoma in lower esophagus which became evident when the scope was passed distally, post dilatation of the web. Conclusions: Although rare, Patterson Kelly syndrome continues to exist and can be effectively treated by iron replacement, dilatation and rupture of esophageal webs using various methods. The web can be ruptured by careful maneuvering of the endoscope itself. Intravenous iron replacement can be used prior to the procedure which can ameliorate the symptoms and reduce the need for the dilatation or can make it easier. Patients need surveillance for upper gastrointestinal malignancies however exact guidelines in this regard are lacking.},
     year = {2020}
    }
    

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  • TY  - JOUR
    T1  - Patterson-Kelly Syndrome: A Single Centre Experience
    AU  - Ramesh Satarkar
    Y1  - 2020/08/04
    PY  - 2020
    N1  - https://doi.org/10.11648/j.ijg.20200402.13
    DO  - 10.11648/j.ijg.20200402.13
    T2  - International Journal of Gastroenterology
    JF  - International Journal of Gastroenterology
    JO  - International Journal of Gastroenterology
    SP  - 41
    EP  - 44
    PB  - Science Publishing Group
    SN  - 2640-169X
    UR  - https://doi.org/10.11648/j.ijg.20200402.13
    AB  - Background: Patterson Kelly syndrome is a rare entity, presenting with a triad of Iron deficiency anemia, Dysphagia and Esophageal webs. The syndrome is extremely rare and its incidence is decreasing probably due to improvements in nutritional standards. The syndrome is enigmatic in the sense that not all patients may be having anemia, the syndrome is associated with various autoimmune conditions, webs are located in post-cricoid region only and there is increased prevalence of upper gastrointestinal malignancies in these patients. The symptoms respond to iron replacement but many patients require rupture of web for relief of dysphagia. Savary dilators, TTS balloon, electro-cautery have been variably used to break the web. Objectives: 1. To study the profile of Patterson Kelly syndrome at our center; 2. To study if the endoscope itself can be used as effective dilator for rupturing the web; 3. To study the effect of iron replacement in these patients; 4. To study the occurrence of upper gastrointestinal malignancies in this condition. Material and methods: We report our experience of sixteen patients with Patterson Kelly syndrome over last fifteen years. This is a retrospective, single center, observational study. All patients had demonstrable web on endoscopy. The endoscope itself was used as a dilator to break the web in more than half of the patients. The endoscope being rigid instrument can easily break the thin web during diagnostic endoscopy itself. If the opening was too narrow, TTS balloon or Savary Guilard dilators were used. One patient had a perforation while using endoscope as the dilator. Five patients required more than one sitting of dilatation for achieving relief of dysphagia or for recurrent web formation. Most patients received intravenous iron supplementation. Ten patients had complete relief of dysphagia without recurrence. One patient had concomitant esophageal squamous cell carcinoma in lower esophagus which became evident when the scope was passed distally, post dilatation of the web. Conclusions: Although rare, Patterson Kelly syndrome continues to exist and can be effectively treated by iron replacement, dilatation and rupture of esophageal webs using various methods. The web can be ruptured by careful maneuvering of the endoscope itself. Intravenous iron replacement can be used prior to the procedure which can ameliorate the symptoms and reduce the need for the dilatation or can make it easier. Patients need surveillance for upper gastrointestinal malignancies however exact guidelines in this regard are lacking.
    VL  - 4
    IS  - 2
    ER  - 

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Author Information
  • Satarkar Gastroenterology Centre, Aurangabad, Maharashtra, India

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